Review of

THIR D E D ITIO N

Ophthalmology
This pa ge inte ntiona lly le ft bla nk
 Review of THIR D E DITIO N

Ophthalmology
Neil J. Friedm an MD
Adjunct Clinical Associate Professor,
Departm ent of Ophthalm ology, Stanford University School of Medicine
Partner, Mid-Peninsula Ophthalm ology Medical Group
Menlo Park, CA, USA

Peter K. Kaiser MD
Chaney Fam ily Endowed Chair for Ophthalm ology
Research Professor of Ophthalm ology
Cleveland Clinic Lerner College of Medicine
Cole Eye Institute, Cleveland Clinic
Cleveland, OH, USA

William B. Trattler MD
Director of Cornea, Center For Excellence In Eye Care
Departm ent of Ophthalm ology
Florida International University Wertheim College of Medicine
Miam i, FL, USA

Edinburgh • London • New York • O xford • Philadelphia • St Louis • Sydney • Toronto 2018
© 2018, Elsevier Inc. All rights reserved.

First edition 2004

Second edition 2012

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Notices
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m ay becom e necessary.

Practitioners and researchers m ust always rely on their own experience and knowledge in evaluating
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ISBN: 978-0-323-39056-9

Printed in China
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Content Strategist: Russell Gabbedy

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Contents
Preface vi

Acknowledgm ents vii

1 OP TICS 1

2 P HARMACOLOGY 21

3 EMBRYOLOGY/ P ATHOLOGY 35

4 NEURO-OP HTHALMOLOGY 49

5 P EDIATRICS/STRABISMUS 91

6 ORBIT / LIDS / ADNEXA 161

7 CORNEA/ EXTERNAL DISEASE 197

8 UVEITIS 239

9 GLAUCOMA 261

10 ANTERIOR SEGMENT 283

11 P OSTERIOR SEGMENT 301

Answers to Questions 371

Additional Readings 395

Index 397

v
Preface
We are pleased to present the third edition of Review of Ophthalm ology. The basic organization of
the book, with its outline form at and m ultiple choice questions divided into chapters based on
fam iliar written exam sections, is unchanged. What we have changed is the content: we have added
new topics, rewritten entire sections, updated treatm ents, and incorporated over 100 new questions
m any of which represent the style of questions encountered on recertification type exam s
(ie, clinical scenarios rather than esoteric facts). Although standardized test questions are slow to
change, we believe it is im portant to stay current. We feel that the m aterial in the new edition
provides this essential inform ation in the m ost concise and easy to use m anner. We hope that you
agree and will find this book helpful in your review process.
Good luck on your exam s and in your future careers!

Neil J. Friedm an, MD

Peter K. Kaiser, MD
William B. Trattler, MD

vi
Acknowledgm ents
We owe thanks to m any individuals who have assisted us with this third edition of Review of
Ophthalm ology. Our colleagues, fam ily and friends never failed to support us in this endeavor, and
we are forever grateful to you.
A num ber of ophthalm ologists deserve a special thank you for donating countless hours to
reviewing individual chapters and the question and answer sections: Drs. Talm adge Cooper, Blair
Arm strong, Daniel Choi, Andrew Kao, and Yohko Murakam i.
We have been extrem ely fortunate to continue to work with a fantastic group of professionals
at Elsevier: thank you Russell Gabbedy, Nani Clansey and Srividhya Vidhyashankar and your
wonderful staff for always doing such an outstanding job on this book.
And of course, we are grateful to our fam ilies for all their love and understanding: Mae, Jake,
Dawn, Peter Jr, Stephanie, Jennifer, Ali, Jerem y, and Josh.

Neil J. Friedm an, MD

Peter K. Kaiser, MD
William B. Trattler, MD

vii
This pa ge inte ntiona lly le ft bla nk
1 Optics
PROPERTIES OF LIGHT
REFRACTION
PRISMS
VERGENCE
MAGNIFICATION
MIRRORS
EYE AS OPTICAL SYSTEM
PRESCRIBING GLASSES
CONTACT LENSES (CL)
LOW-VISION AIDS
INTRAOCULAR LENSES (IOL)
OPHTHALMIC INSTRUMENTS
EQUATIONS

P ROP ERTIES OF LIGHT Co h er en ce: ability of two light beam s to cause interference
(large white source has a coherence close to zero)
Exa m p le : OCT
Ligh t behaves both as waves and as particles (photons)
Po lar izatio n : each light wave has an electrical field with a
Its speed (velocity) (v) is directly proportional to wavelength
particular orientation
(λ) and frequency (ν): v¼ λν
Nonpolarized light: electrical field of each wave has a
In any given m edium , speed of light is constant
random orientation
(vvacuum ¼ c¼ 3.0 Â 10 10 cm /s); therefore, wavelength
Polarized light: all electrical fields have sam e orientation
and frequency are inversely proportional Exa m p le : Haidinger brushes (polarizing filter rotated in front of blue
Light slows down in any substance other than air or background produces rotating image like a double-ended brush or
vacuum ; am ount of slowing depends on m edium ; propeller; type of entopic phenomenon; test of macular function),
frequency of light rem ains unchanged, but wavelength Titmus stereo testing, polarized microscopy, polarizing sunglasses
changes (becom es shorter) (Fig. 1-1)
Its en ergy is directly proportional to frequency and inversely Diffr actio n : bending of light waves around edges; change in
proportional to wavelength: E¼ hν ¼ h(c/λ) direction of light wave is related to wavelength (the shorter the
wavelength, the less the change in direction); am ount of
In d ex o f r efr actio n (n): ratio of speed of light in a vacuum diffraction is related to size of aperture (the sm aller the
to speed of light in specific m aterial (n ¼ c/v) aperture, the greater the diffraction); interference of new waves
Air ¼ 1.00, water ¼ 1.33, aqueous and vitreous ¼ 1.34, with original rays form s a diffraction pattern
cornea ¼ 1.37, crystalline lens ¼ 1.42, intraocular lens Exa m p le : Airy disc (diffraction pattern produced by a small, circular aperture;
occurs when pupil size is < 2.5 mm; diameter of central disc increases as
(IOL) (silicone ¼ 1.41; polym ethyl m ethacrylate 1.49;
pupilsize decreases); pinhole (reduces refractive error and improves vision by
acrylic¼ 1.55), glass ¼ 1.52, high index lenses ¼ 1.6-1.8 increasing depth of focus, but limited by diffraction; optimal size is 1.2 mm;
may correct for as much as 3 D; smaller aperture limits visualacuity; squinting
In ter fer en ce: overlapping of light waves; m ay be is method of creating a natural pinhole to improve vision; pinhole can also
constructive or destructive improve vision in eyes with corneal or lenticular irregularities; pinhole can
Constructive: peaks of two waves overlap, resulting in reduce vision in eyes with retinal disorders)
m axim um intensity at that wavelength
Destructive: peak of one wave overlaps with trough of Scatter in g: disruption of light by irregularities in light path;
another, obliterating both waves shorter wavelengths scatter to a greater extent
Exa m p le : antireflective coatings (destructive interference, one fourth Exa m p le : Opacity (corneal scar or cataract) scatters light, causing glare and
wavelength apart); interference filters (allow only green light out of the eye image degradation; in atmosphere, scattering involves particles (Rayleigh
during fluorescein angiography); laser interferometry (retinal function test; scattering) and blue light (scattered to the greatest extent; therefore, sky
optical coherence tomography [OCT]) appears blue)

1
CHAPTER 1 • Optics

Fig ure 1-1. The electromagnetic

spectrum. The pictures of mountains,
people, buttons, viruses, and so forth, Vis ible S pe c trum mm
are used to produce a real (i.e., visceral)
feeling of the size of some of the Ra dio 700 600 500 400
wavelengths. (With permission from
1GHz 100GHz
Miller D, Burns SK: Visible light. In:
Infra re d
Yanoff M, Duker J S (eds):
Ophthalmology, ed 2, St Louis, 2004, mm
Mosby.) AM FM
540 –1650kHz 88 –108MHz
Ultra viole t

S oft X-ra ys ha rd

G-ra ys
Fre que ncy (Hz)
3 x10 2 3 x10 4 3 x10 6 3 x10 8 3 x10 10 3 x10 12 3 x10 14 3 x10 16 3 x10 18 3x10 20 3 x10 22 3 x10 24

10 6 10 4 10 2 1 10 –2 10 –4 10 –6 10 –8 10 –1 0 10 –12 10 –14 10 –16

Wa ve le ngth (m)

Mounta ins Fa ctory P e ople Button P oint Dus t Ba cte ria Virus Atom Atomic nucle us
S ize

Atmos phe ric tra ns pa re ncy

Reflectio n : bouncing of light off optical interfaces; the

greater the refractive index difference between the two m edia, REFRACTION
the greater is the reflection; also varies with angle of incidence
Exa m p le : Asteroid hyalosis (asteroids reflect light back into examiner’s eye, Light changes direction when it travels from one m aterial
creating glare; patient is asymptomatic)
to another of different refractive index (e.g., across an
optical in terface); direction of refraction is toward the
Tr an sm issio n : percentage of light penetrating a substance norm al when light passes from a m edium with a lower
(%T); can vary with wavelength index of refraction to a m edium with a higher one, and
away from the norm al when light passes from a m ore
Ab so r p tio n : expressed as optical density (OD) log 1/T dense to a less dense m edium (higher refractive index
m aterials are m ore difficult for light to travel through, so
light takes a shorter path [closer to the norm al]); light does
Illu m in atio n : m easure of incident light not deviate if it is perpendicular to interface (parallel to the
norm al)
Lu m in an ce: m easure of reflected or em itted light (lum en/
m 2 ); apostilb diffusing surface with lum inance of 1 lum en/
Sn ell’s law: n sin (i) ¼ n 0 sin (r); n ¼ refractive index
m 2 (used in Hum phrey and Goldm ann visual field testing)
Exa m p le : contrast sensitivity is the ability to detect small changes in of m aterial; i ¼ angle of incidence (m easured from the
luminance norm al); r ¼ angle of refraction (m easured from the norm al)
(Fig. 1-2)
Laser : light am plification by stim ulated em ission of
radiation; excited m aterial releases photons of sam e Cr itical an gle: angle at which incident light is bent exactly
wavelength and frequency; process is am plified so that released 90° away from the norm al (when going from m edium of higher
photons are in phase (constructive interference); produces to lower n) and after which all light is reflected
m onochrom atic, coherent, high-intensity polarized light; Exa m p le : Glass/air interface has a critical angle of 41°; critical angle of
cornea ¼ 46.5°
power can be increased by increasing energy or decreasing tim e
(P E/t); Q switching and m ode locking (types of shutters that
synchronize light phase) are m ethods of increasing laser power To tal in ter n al r eflectio n : angle of incidence exceeds
by com pressing output in tim e critical angle, so light is reflected back into m aterial with

2
 Prism s

n n'

norma l r n'> n
i
A B
Fig ure 1-4. A, Plus lenses act like two prisms base to base. B, Minus lenses act
like two prisms apex to apex.

Fig ure 1-2. Refraction of light ray.

Dis pla ce me nt Risley p r ism : two right-angle prism s positioned back

to back, which can be rotated to yield variable prism
I diopters from 0 to 30 Δ; used to m easure prism atic correction
De via tion for tropias
O
Fr esn el p r ism s: com posed of side-by-side strips of
A B sm all prism s; prism power is related to apex angle, not the size
Fig ure 1-3. A, Displacement of image toward apex. B, Displacement and
of the prism ; available as lightweight, thin press-on prism to
deviation of light by prism. reduce base thickness of the spectacle prism ; disadvantage is
reflection and scatter at prism interface, causing decreased
visual acuity
higher index of refraction; n sin (ic) ¼ n 0 sin (90°); sin
(ic) ¼ (n 0/n) Â 1 Pr ism atic effect o f len ses (Fig. 1-4): spectacles induce
Exa m p le : Gonioscopy lens is necessary to view angle structures because prism ; all off-axis rays are bent toward or away from axis,
of total internal reflection of the cornea depending on lens vergence
Perceived m ovem ent of fixation target when lens m oves in
P RISMS front of the eye:
Plus lenses: produce ”against” m otion (target m oves
in opposite direction from lens)
Prism s displace and deviate light (because their surfaces are Minus lenses: produce ”with” m otion (target m oves
nonparallel); light rays are deviated toward the base; im age is in sam e direction as lens)
displaced toward the apex (Fig. 1-3) Am ount of m otion is proportional to the power of the lens

Pr ism d io p ter (PD, Δ): displacem ent (in cm ) of light ray Prism atic effect of glasses on strabism ic deviations:
passing through a prism , m easured 100 cm (1 m ) from prism 2.5 Â D ¼ percent difference; m inus lenses m ake deviation
Exa m p le : 15 Δ ¼ ray displaced 15 cm at a distance of 1 m (1 Δ ¼ 1 cm appear larger (”m inus m easures m ore”); plus lenses decrease
displacement/1 m); 1° %2 Δ (this approximation is useful for angles m easured deviation
smaller than 45°)

Pr en tice’s r u le: prism atic power of lens ¼ Δ ¼ hD

An gle o f m in im u m d eviatio n : total angle of deviation is (h ¼ distance from optical axis of lens [cm ], D ¼ power of
least when there is equal bending at both surfaces of prism lens [D])
Plastic prism s are calibrated by angle of m inim um deviation: Prism atic power of a lens increases as one m oves farther
back surface parallel to frontal plane away from optical center (vs. power of prism , which is
Glass prism s are calibrated in Pren tice position : back surface constant)
Exa m p le : Reading 1 cm below optical center: ODÀ 3.00; OS + 1.00
perpendicular to visual axis + 3.00 Â 90
OD (Oculus Dexter – right eye): prism power ¼ 1 cm  3 D¼ 3 Δ BD
Prism placed in front of the eye creates a phoria in the direction
OS (Oculus Sinister – left eye): prism power vertical meridian: 1 cm + 1 D¼ 1 Δ
of the base BU (base-up) (Note: power of cylinder in 90° meridian is zero)
Exa m p le : Base-out (BO) prism induces exophoria; to correct, use prism Net prismatic effect ¼ 4 Δ (either BD over OD, or BU over OS)
with apex in the opposite direction
Treatm ent of vertical prism atic effect of anisom etropia:
Apex is always pointed in direction of deviation: base-out for
1. Contact lenses (CL) (optical center m oves with eyes)
esotropia, base-in for exotropia, base-down (BD) for
2. Lower optical centers of lenses (reduce am ount of
hypertropia
induced prism )
Stacking prism s is not additive; 1 prism in front of each eye is 3. Prescribe slab-off prism (technique of grinding
additive lens [done to the m ore m inus of the 2 lenses] to

3
CHAPTER 1 • Optics

rem ove BD prism [to reduce am ount of

induced prism ])
4. Single vision reading glasses 5P D ba s e up & out a t 37°

Pr ism atic effect o f b ifo cal glasses:

3BU
Image jump: produced by sudden prism atic power at top
of bifocal segm ent; not in fluenced by type of
underlying lens; as line of sight crosses from optical
center of lens to bifocal segm ent, im age position
suddenly shifts up owing to base-down prism atic
effect of bifocal segm ent (m ore bothersom e than 4BO
im age displacem ent; therefore, choose segm ent type to Fig ure 1-5. Addition of base-up and base-out prisms.
m inim ize im age jum p)
Image displacement: displacem ent of im age by total
prism atic effect of lens and bifocal segm ent; m inim ized VERGENCE
when prism atic effect of bifocal segm ent and distance
lens are in opposite directions
Prismatic effect of underlying lens: The am ount of spreading of a bundle of light rays (wavefront)
Hyperopic lenses induce BU prism , causing im age to em erging from a point source
m ove progressively downward in downgaze Direction of light travel m ust be specified (by convention, left
Myopic lenses induce BD prism , causing im age to m ove to right)
progressively upward in downgaze
Prismatic effect of bifocal segment: Co n ver gen ce (converging rays): plus vergence; rare in
Round top (acts like BD prism ): m axim um im age jum p; nature; m ust be produced by an optical system
im age displacem ent less for hyperope than for m yope
Flat top (acts like BU prism ): m inim um im age jum p;
Diver gen ce (diverging rays): m inus vergence
im age displacem ent m ore for hyperope than
for m yope
Executive type or progressive lenses: no im age jum p Par allel r ays: zero vergence
(optical centers at top of segm ent)
Plus lens: choose round top Dio p ter : unit of vergence; reciprocal of distance (in m eters)
Minus lens: choose flat top or executive type (for m yopes, to point at which rays intersect; reciprocal of focal length
im age jum p is very difficult to ignore because it is in sam e of lens
direction as im age displacem ent, so avoid round tops in
m yopes) Len s: adds vergence to light (am ount of vergence ¼ power
of lens [in diopters])
Ch r o m atic effects: prism atic effect varies with wavelength Plus (convex) lens adds vergence; m inus (concave)
Shorter wavelengths are bent farther, causing chrom atic lens subtracts vergence
aberration
White light shines through prism : blue rays closer to base Basic len s fo r m u la: U + D ¼ V (U ¼ vergence of light
(bend farthest), red rays closer to apex entering lens; D ¼ power of lens; V¼ vergence of light
In the eye, blue rays com e to focus closer to lens than do red leaving lens)
rays; difference between blue and red is 1.5 to 3 D Power of a spherical surface in a fluid: Ds ¼ (n 0À n)/r
(n0À n ¼ difference in refractive indices; r ¼ radius of
curvature of surface [in m eters])
Du o ch r o m e test: red and green filters create 0.5 D Exa m p le : Power of corneal surface: back ¼ À 5.7 D; front ¼ + 52.9 D (front:
difference; use to check accuracy of refraction n 0 ¼ 1.37, n ¼ 1.00; back: n 0 ¼ 1.33, n ¼ 1.37; r¼ 0.007)
If letters on red side are clearer, focal point is in front of Power of a thin lens im m ersed in fluid: Dair/Dfluid ¼ (n len s
retina (eye is ”fogged” or m yopic) Àn air)/(n len s À n fluid )
If letters on green side are clearer, focal point is behind Refracting power of a thin lens is proportional to difference
retina (eye is overm inused or hyperopic) in refractive indices between lens and m edium
Technique: start with red side clearer and add m inus sphere
in 0.25 D steps until red and green sides are equal Ob jects an d im ages:
(focal point on retina; m nem onic RAM-GAP [Red Add Object rays: rays that define the object; always on incom ing
Minus – Green Add Plus]); works in color blind (left) side of lens
patients because it is based on chrom atic aberration Image rays: rays that define the im age; always on outgoing
rather than color discrim ination (right) side of lens
Objects and im ages can be on either side of lens: real if on
Vecto r ad d itio n o f p r ism s: prism atic deviations in sam e side as respective rays; virtual if on opposite side
different directions are additive, based on pythagorean from rays (locate by im aginary extension of rays
theorem (a 2 + b2 ¼ c2 ) (Fig. 1-5) through lens)

4
 Vergence

If object is m oved, im age m oves in sam e direction relative SECONDARY (f0): im age point for object at infinity
to light (point on optical axis at which incident parallel
Adding power to system also m oves im age: plus power pulls rays are focused)
im age against light; m inus power pushes im age FOCAL LENGTH: distance between lens and focal points;
with light reciprocal of lens power (f¼ 1/D)
Exa m p le : Focal length of +20 D lens is 1/20 ¼ 0.05 m
NODAL POINT (N): point through which light ray passes
Len ses:
Real (thick) lenses (Fig. 1-6): undeviated; located at center of thin lens (optical
6 CARDINAL POINTS: two principal planes (H and H’);
center)
RAYTRACING: use to determ ine im age size, orientation,
two nodal points (N and N 0); two focal points (F
and F0) and position
THREE PRINCIPAL RAYS:
Refraction occurs at principal planes (U is m easured
from H; V is m easured from H 0) 1. Central ray: undeviated ray passing from tip of
Focal lengths are also m easured from principal planes object through nodal point of lens (or center of
Nodal points coincide with principal planes (exception: curvature of m irror) to tip of im age; gives size and
if different refractive m edia are on opposite sides of orientation of im age (form sim ilar triangles;
the lens, then nodal points are both displaced toward thus, sizes of object and im age are in sam e ratio
the m edium with the higher refractive index) as their distances from the lens)
Central ray: passes through both nodal points (tip of 2. Ray from tip of object through F em erges from
object to N, across to N 0; then, em erges parallel to lens parallel to optical axis
original direction) 3. Ray from tip of object parallel to optical axis
MENISCUS LENSES: difference between anterior and
em erges from lens and passes through F0
posterior curvature determ ines power (Fig. 1-7)
Steeper anterior curvature ¼ convergent lens (+power);
principal planes displaced anteriorly Len s effectivity: function of lens power and distance
Steeper posterior curvature ¼ divergent lens (Àpower); from desired poin t of focus; depends on vertex distance
principal planes displaced posteriorly and refractive
À  index of m edia in which à lens Á
is placed
Power of lens is m easured at posterior surface (posterior Dair ¼ n IOL À n air =n IOL À n aqueous  D aqueous ; m oving a
vertex power) lens forward away from eye increases effective plus power, so
Conjugate points: each pair of object-im age points in an plus lens becom es stronger and m in us lens becom es
optical system is conjugate; if direction of light weaker; when vertex distance decreases, a m ore plus lens is
is reversed, position of object and im age is exactly required to m aintain the sam e distance correction (i.e., as
reversed desired point of focus is approached, m ore plus power is
Conjugate planes: needed); the m ore powerful the lens, the m ore significant is
Exa m p le : Viewing a slide presentation (image of slide is formed on retina of the change in position
each person in audience, and very faint image of each person’s retina is
projected on the screen), direct ophthalmoscope (patient’s retina and Ver tex d istan ce co n ver sio n :
examiner’s retina are conjugate), indirect ophthalmoscope (three conjugate
1. Focal point of original lens ¼ far point
planes [patient’s retina, aerial image plane, examiner’s retina]; any object
placed at far point [aerial image] will be imaged sharply in focus on patient’s 2. Distance of new lens from far point ¼ required focal
retina) length of new lens
Ideal (thin) lenses: special case of thick lens; as lens gets 3. Power of new lens ¼ reciprocal of new focal length
thinner, principal planes m ove closer together; in an Exa m p le : +12.50 D spectacle lens at vertex distance of 13 mm;
calculate CL power:
ideal lens, principal planes overlap at optical center 1. +12.50 D lens has focal point of 0.08 m ¼ 8 cm ¼ far point
TWO FOCAL POINTS: 2. Distance of new lens (CL) from far point is
PRIMARY (f): object point for im age at infinity (point 8 cm À 13 mm ¼ 67 mm ¼ required focal length of CL
on optical axis at which object is placed so parallel 3. Power of CL¼ 1/0.067 m ¼ + 15 D
rays em erge from lens) Approxim ation: D 2 ¼ D 1 + SðD 1 Þ2 ðS ¼ vertex distance
in m etersÞ

H H'

Obje ct 3
N N' F'
1
F Ima ge 2
Obje ct N F'
F Ima ge

Fig ure 1-6. Conjugate points of real lens: each pair of object–image points in an
optical system is conjugate; if direction of light is reversed, positions of object and
image are exactly reversed. Fig ure 1-7. Three principal rays of ideal lens.

5
CHAPTER 1 • Optics

Pu r e cylin d r ical len s: power only in 1 m eridian Therefore, does not change the position of the circle of least confusion with
respect to the retina; normally, have 0.25 D cross cylinder in phoropter; if
(perpendicular to axis of lens); produces focal line
patient’s acuity is 20/40 or worse, must use larger power cross cylinder so
parallel to axis patient can discriminate difference
TIGHT SUTURE AFTER CATARACT OR CORNEAL SURGERY: steepens
Sp h er o cylin d r ical len s: power in 1 m eridian greater cornea in meridian of suture, inducing astigmatism
than other
Spherical equivalent: average spherical power of a Ab e rra t io n s
spherocylindrical lens; equal to sphere plus
one half the cylinder; places circle of least Lenses behave ideally only near optical axis; peripheral to this
confusion on retina paraxial region, aberrations occur
Conoid of Sturm: 3-dim ensional envelope of light rays
refracted by a circular spherocylindrical lens; consists
of: vertical line ! vertical ellipse ! circle (of least
Sp h er ical: shape-dependent aberration; periphery of lens
has increasing prism atic effect; thus, peripheral rays refracted
confusion) ! horizontal ellipse ! horizontal line
farther than paraxial ones, producing a blur interval along the
(Fig. 1-8)
optical axis (Fig. 1-10)
Circle of least confusion: circular cross section of conoid of
Produces bull’s-eye retinoscopic reflex
Sturm , which lies halfway (dioptrically) between the two
Reduce by avoiding biconvex lens shape; use plano-convex,
focal lines at which im age is least blurred; dioptrically
m eniscus, or aspheric lens surface
calculated by spherical equivalent
Eye has three m echanism s for reducing spherical aberration
Interval of Sturm: distance between anterior and posterior
of lens:
focal lines
1. Sm aller pupil size elim inates a greater num ber of
peripheral rays
Cylin d er tr an sp o sitio n : converting cylinder notation 2. Cornea progressively flattens in periphery
(plus ! m inus; m inus ! plus)
3. Nucleus of crystalline lens has higher index of
New sphere ¼ old sphere + old cylinder
refraction
New cylinder ¼ m agnitude of old cylinder but with
opposite power Co m a: com et-shaped im age deform ity from off-axis
New axis ¼ change old axis by 90° peripheral rays
Exa m p le : +3.00 + 1.50 Â 45 ! + 4.50 À 1.50 Â 135

Po wer cr o ss d iagr am : depicts two principal m eridians of

lens with the power acting in each m eridian (90° from axis),
rather than according to axis (Fig. 1-9)
Cros s cylinde r nota tion: ; 2.00! 90; ; 1.00! 180
Co m b in in g cylin d er s at o b liqu e axis: com plex P owe r me ridia n nota tion: ; 2.00 @ 180 / ; 1.00 @ 90
calculation; therefore, m easure with lensom eter
P owe r cros s : ; 1.00
Po wer o f cylin d er at o b liqu e axis: power of the cylinder
+1.00 Â 180° is +1.00 @ 90°; +0.75 @ 60°; +0.50 @ 45°; +0.25
@ 30°; 0 @ 0° ; 2.00
Exa m p le :
J ACKSON CROSS CYLINDER: special lens used for refraction to determine
cylinder power and orientation; combination of plus cylinder in 1 axis and
minus cylinder of equal magnitude in axis 90° away; spherical equivalent is
To conve rt to s phe rocylindrica l nota tion: a dd a nd s ubtra ct
zero (e.g.: À1.00 + 2.00 Â 180)
a cros s e d cylinde r

Origina l cylinde r nota tion: ; 2.00! 90 ; 1.00! 180

Ve rtica l
foca l line Add/s ubtra ct cross e d cylinde r: ; 2.00! 180 : 2.00! 180
Horizonta l
foca l line S phe rocylinde r nota tion: ; 2.00 s phe re : 1.00! 180

Or
Inte rva l of S turm
Origina l cylinde r nota tion: ; 1.00! 180 ; 2.00! 90

Add/s ubtra ct cros s e d cylinde r: ; 1.00! 90 : 1.00! 90

S phe rocylinde r nota tion: ; 1.00 s phe re ; 1.00! 90

Circle of le a s t confus ion
Fig ure 1-8. Conoid of Sturm. Fig ure 1-9. Various lens notations.

6
 Magnification

Blur Obje ctive (; ) Eye pie ce (; )

inte rva l ; 10D ; 20D

15cm
P e riphe ra l ra y P a ra xia l ra y
focus focus fo = 10cm fe = 5cm
Fig ure 1-10. Spherical aberration.

Cu r vatu r e o f field : spherical lens produces curved im age of Fig ure 1-11. Astronomic telescope.
flat object

Astigm atism o f o b liqu e in cid en ce: tilting spherical lens CENTER THICKNESS: every m illim eter of change in
induces astigm atism (oblique rays encounter different thickness will change im age size by one half percent
curvatures at front and back lens surfaces) (m agnification decreases while lens thickness
Exa m p le : pantos copic tilt (amount of induced sphere and cylinder depends decreases).
on power of lens and amount of tilt)
Power factors:
VERTEX POWER (REFRACTIVE POWER): m inus power
Disto r tio n : differential m agnification from optical axis to lenses produce sm aller im ages than do plus lenses.
lens periphery alters straight edges of square objects; shape of VERTEX DISTANCE: an increase in vertex distance will
distortion is opposite of shape of lens (plus lens produces increase the m agnification of plus lenses and decrease
pin cush ion distortion; m inus lens produces barrel distortion); the magnification (increase the m inification) of m inus
effect increases with power of lens lenses.
PLUS LENS: every m illim eter increase in vertex distance
Ch r o m atic: light of different wavelengths is refracted by will increase m agnification by 0.1% per diopter of
different am ounts (shorter wavelengths are bent farther; lens power.
chrom atic interval between blue and red is 1.5 to 3.0 D) MINUS LENS: every m illim eter increase in vertex
Exa m p le : at night with Purkinje shift, chromatic aberration moves focal point
distance will decrease m agnification by 0.1% per
of eye anteriorly, producing myopia
diopter of lens power.
Spectacle lens changes retinal im age size by 2% per diopter
of power at 12 m m vertex distance
MAGNIFICATION
An iso m etr o p ia: the difference in power between the two
Tr an sver se ( lin ear o r later al) : m agnification of image eyes; every 1 D produces approxim ately 2% of aniseikonia.
size (away from optical axis); m ust be able to m easure object and
image size; ratio of image height to object height (or im age An iseiko n ia: difference in im age size between eyes from
distance to object distance); if image is inverted, magnification is unequal m agnification of correcting lenses.
negative ML ¼ I/O Up to 6% to 7% is usually well tolerated; corresponds to
approxim ately 3 D of spectacle anisom etropia; children
Axial: m agnification of depth; m agnification along optical can adjust to m uch larger degrees
axis; equal to square of transverse m agnification MAx ¼ ML2 Exa m p le : unilateral aphakia: 25% enlargement with spectacle lens; 7% with
CL; 2.5% with IOL

An gu lar : m agnification of angle subtended by an im age with

Kn ap p ’s r u le: when proper corrective lens is positioned at
respect to an object; useful when object or im age size cannot be
anterior focal point of eye, retinal im age size will be equal in
m easured
both eyes, no m atter what the degree of anisom etropia (applies
Exa m p le : moon gazing with telescope
only to axial am etropia)
MA ¼ xD¼ D/4 (standardized to 25 cm [one quarter m], the near point of the
average eye)
Exa m p le : with direct ophthalmoscope, eye acts as simple magnifier of Telesco p es: m agnify objects by increasing angle that object
retina: MA ¼ 60/4 ¼ 15 Â magnification subtends on retina
Astronomical telescope (Keplerian): com bination of two
Size o f im age seen th r o u gh glasses: plus lenses; focal points coincide in interm ediate
Shape factors: for any corrective lens, an increase in either im age plane; distance between lenses is sum of focal
front surface curvature or lens thickness will increase the lengths; use higher power as eyepiece; inverted im age
im age size (therefore, equalize the front surface (Fig. 1-11)
curvatures and lens thickness for the 2 lenses) Galilean telescope: com bination of a weak plus lens
FRONT SURFACE CURVATURE: every D of change will (objective) and a strong m inus lens (eyepiece); distance
change im age size by one half percent (m agnification between lenses is difference of focal lengths; erect im age
decreases while plus power decreases) (e.g., surgical loupe) (Fig. 1-12)

7
CHAPTER 1 • Optics

Obje ctive (; ) Eye pie ce (: ) D= ; 60D

; 10D : 20D H
n'= 1.33
n= 1

F N F'
5cm

fe = 5cm

17mm 5.6mm 17mm

fo = 10cm 22.6mm
Fig ure 1-12. Galilean telescope. Fig ure 1-13. Schematic eye.

Cen tr al r ay: passes through center of curvature of m irror,

Angular m agnification is the sam e for both telescopes (power not center of mirror
of eyepiece/power of objective): MA ¼ À D e/D o
Prim ary and secondary focal points coincide
Purkinje-sanson images: four im ages from reflecting
Acco m m o d atio n th r o u gh telesco p e: AT ¼ AN (MA2 ) surfaces of eye
(AN ¼ norm al accom m odation)
Exa m p le : for monocular aphakia, overcorrect aphakic CL by +3.00 D;
1. Front surface of cornea (im age of object at infinity is
then, correct induced myopic error with spectacle of À3.00 D. This located at focal point of m irror ¼ 1/À260 ¼ À 3.85 m m ;
produces an inverse Galilean telescope system that results in significantly thus, this is a virtual, erect, m inified im age 3.85 m m
smaller magnification difference between the two eyes than occurs with posterior to front surface of cornea)
a CL alone Exa m p le : keratometry (see Fig. 1-24)

2. Back surface of cornea (virtual, erect, m inified im age)

3. Front surface of lens (virtual, erect, m inified im age)
MIRRORS 4. Back surface of lens (real, inverted, m inified im age)
(If patient has intraocular lens [IOL], Purkinje-Sanson
Law o f r eflectio n : angle of incidence ¼ angle of reflection im ages 3 and 4 are taken from front and back surfaces of
(m easured from the norm al the IOL, respectively; these are useful in assessm ent of
m ild degrees of pseudophacodonesis)
Ob jects an d im ages: real if located on left side of m irror,
virtual if on right side (inside) of m irror

Fo cal len gth : EYE AS OP TICAL SYSTEM

One quarter the radius of curvature (f¼ r/2)
Mo d e l Eye
Reflectin g p o wer : reciprocal of focal length Gullstrand studied the eye’s optical system and, based on
(Dr ¼ À 1/f¼ À 2/r)
average m easurem ents ðpower ¼ + 58:64 D; F ¼ 17:05 m m Þ, he
created a sim plified m odel: the “reduced” or “sch em atic” eye
Co n vex: positive radius of curvature (R); adds m inus (Fig. 1-13): power ¼ + 60 D; F ¼ 17 m m ; F0 ¼ 22:6 m m
vergence; produces virtual, erect, m inified im age (“VErMin”) Exa m p le : calculate the diameter of the blind spot projected 2 meters in front
Exa m p le : rear view mirror; cornea (reflecting power of cornea ¼ À 1/f¼ À 2/ of the eye (ON ¼ 1.7 mm tall, thus by similar triangles: 1.7/17 ¼ x/2000;
r¼ À 2/0.0077 ¼ À 260 D [much stronger than refracting power]) x¼ 2000(1.7/17)¼ 200 mm)

Co n cave: negative r; adds plus vergence; im age can be virtual

or real, erect or inverted, magnified or m inified, depending on Vis io n Me a s u re m e n t s
object location with respect to center of curvature of mirror: Min im u m visib le: presence or absence of stim ulus;
At twice focal length (center of curvature): real, inverted, depends on am ount of light striking photoreceptors
sam e size
Between center and focal length: real, inverted, m agnified
At focal length: at infinity Min im u m d iscr im in ab le: resolving power of eye; depends
Inside focal length: virtual, erect, m agnified on ability to detect differences in light intensity

Plan o : no change in vergence; im age is virtual, erect, sam e

size; field of view is double the size of the m irror Min im u m sep ar ab le: sm allest angle at which two
Exa m p le : dressing mirror needs to be only half of body length to provide separate objects can be discrim inated; detection of a break in
view of entire self a line

8
 Eye as Optical System

Ver n ier acu ity: spatial discrim ination; ability to detect Emmetropia: focal point on retina
m isalignm ent of two lines (8 seconds of arc; sm aller than Myopia: focal point in front of retina
diam eter of photoreceptor) Hyperopia: focal point behind retina
total hyperopia ¼
Sn ellen acu ity: based on angle that sm allest letter subtends
on retina; each letter subtends 5 m inutes of arc at a specific m anifest hyperopia ðabsolute and facultativeÞ+
distance (represented by the denom inator [i.e., 20/40 letter latent hyperopia ðexposed with cycloplegia Þ
subtends 5 m inutes at 40 feet, 20/20 letter subtends 5 m inutes
at 20 feet]; the num erator is the testing distance); each stroke
width and space subtends 1 m inute (Fig. 1-14) Axial vs r efr active:
Exa m p le : calculate the size of a 20/20 letter at 20 ft (6 m): tan ¼ opposite/ Axial myopia: length of eye too long (refractive power
adjacent; tan 1 0 ¼ 0.0003 therefore, tan 5 0 ¼ 0.0015 ¼ x/6000; x¼ 8.7 mm
norm al)
Refractive myopia: refractive power too strong
ETDRS ( Ear ly Tr eatm en t Diab etic Retin o p ath y (length norm al)
Stu d y) Ch ar t: five letters per line; space between letters is Axial hyperopia: length too short (refractive
equal to size of letter on that line; geom etric proportion of power norm al)
optotype height (changes in 0.1 log unit increm ents) Refractive hyperopia: refractive power too weak
(length norm al)
Near acu ity: m ust record testing distance

Acu ity testin g in ch ild r en : Optokinetic nystagm us

(OKN), CSM (central, steady, m aintain), preferential looking, Emme tropia
Allen pictures, HOTV (letter sym bols used in pediatric visual
acuity testing), visual evoked potential (VEP)

Facto r s o th er th an d isease th at r ed u ce m easu r ed

visu al acu ity: uncorrected refractive error, eccentric viewing, F2
decreased contrast, sm aller pupil size, older age

Legal b lin d n ess (in US): visual acuity (VA) ¼ 20/200 or

worse or visual field (VF) < 20° in better-seeing eye

Visu al acu ity is in flu en ced b y p u p il size: larger pupil Myopia

lim its vision owing to spherical and chrom atic aberrations;
sm aller pupil lim its vision owing to diffraction; optim al pupil
size is 3 m m

Laser in fer o m eter : helium neon laser beam is split and F2

projected onto retina, producing interference fringes; spacing
of fringes can be varied; retinal function is estim ated by
narrowest fringes discernible

Co n tr ast sen sitivity: ability to detect changes in

lum inance Hype ropia

Re fra c t ive Erro r

Secondary focal point (F0) of eye is not located
on retina (accom modation must be completely relaxed)
(Fig. 1-15): F2

x Fig ure 1-15. Emmetropia, myopia, and hyperopia. In emmetropia, the far point
is at infinity and the secondary focalpoint (F2 )is at the retina. In myopia, the far point
is the front of the eye, and the secondary focal point (F2 ) is in the vitreous. In
5' hyperopia, the secondary focal point (F2 )is located behind the eye. (Modified with
6m permission from Azar DT, Strauss L: Principles of applied clinical optics. In Albert
DM, J akobiec FA (eds ): Principles and practice of ophthalmology, vol 6, ed 2,
Fig ure 1-14. Calculation of Snellen letter size. Philadelphia, 2000, WB Saunders.)

9
CHAPTER 1 • Optics

Far p o in t: farthest-away eye can see clearly with Increased axial length: congenital glaucom a, posterior
accom m odation com pletely relaxed (turn light around, start at staphylom a, after scleral buckle, retinopathy of
retina, and trace rays backward through optics of eye; point at prem aturity (ROP)
which rays intersect is far point)
Myopia: far point is centim eters to infinity in front of eye Nigh t m yo p ia: increased m yopia in dark
Hyperopia: far point is behind eye (virtual far point) Pupil dilation: spherical aberration, irregular astigm atism
uncovered
Astigm atism : produces two focal lines rather than a single Purkinje shift: spectral sensitivity shifts toward shorter
focal point (Table 1-1) wavelengths at lower light levels, and chrom atic
Classification: corneal or lenticular; regular (symmetric aberration m oves the focal point anteriorly
[mirror im age axis between eyes] or asymm etric) or Dark focus: no accom m odative target in dark; therefore,
irregular tend to overaccom m odate for distance and
“With-the-rule”: cornea is steepest in vertical m eridian; underaccom modate for near
axis of plus cylinder is 90° (Æ20°); usually young Length of refraction lane: shorter than 20 feet produces one-
patients (elastic lids press on top and bottom of sixth D undercorrection (add m inus 0.25 D to final
cornea) refraction)
“Against-the-rule”: cornea is steepest in horizontal
m eridian; axis of plus cylinder is 180°Æ20°; Acqu ir ed astigm atism : lid lesion (tum or, chalazion,
older patients ptosis), pterygium , lim bal derm oid, corneal degenerations and
ectasias, surgery (corneal, cataract), lenticular, ciliary body
Co r r ectio n o f am etr o p ia: choose lens with focal point (CB) tum or
that coincides with far point of patient’s eye
Ac c o m m o d a t io n
Acqu ir ed h yp er o p ia: Eye gains plus power when crystalline lens becom es m ore
Decreased effective axial length: retrobulbar tum or, convex
choroidal tum or, central serous chorioretinopathy
Decreased refractive power: lens change (posterior lens Accom m odation response can be described as:
dislocation, aphakia, diabetes), drugs (chloroquine,
Am p litu d e o f acco m m o d atio n : total dioptric am ount
phenothiazines, antihistam ines, benzodiazepines),
eye can accom m odate
poor accom m odation (tonic pupil, drugs, traum a),
Near point: only for em m etropes
flattening of cornea (contact lens), intraocular
Prince’s rule: com bines reading card with a ruler calibrated
silicone oil
in centim eters and diopters to measure am plitude of
accom m odation
Acqu ir ed m yo p ia: Technique: place +3.00 D lens in front of distance
Increased lens power: osm otic effect (diabetes, correction to bring far point to one-third m m (33 cm );
galactosem ia, urem ia, sulfonam ides), nuclear sclerotic then, m easure how near patient can read and convert
cataracts, anterior lenticonus, change in lens position or into diopters; subtract far point from near point to
shape (m edication [m iotics]), anterior lens dislocation, determ ine am plitude
excessive accom m odation) Method of spheres: fixate on reading target (e.g., 40 cm ),
Increased corneal power: keratoconus, congenital glaucom a, successively increase m inus sphere until print blurs, then
CL-induced corneal warpage increase plus sphere until blurring occurs again; absolute
difference between the spheres is the am plitude of
accom m odation
Exa m p le : range of À4.00 D to +2.00 D¼ amplitude of 6 D

Ran ge o f acco m m o d atio n : distance between far point

Ta b le 1-1. Classification of astigmatism
and near point; m easured with tape m easure or
Lo c a tio n o f Co rre c tive accom m odative rule
Typ e fo c a l line s le ns Far point: point on visual axis conjugate to retina when
Compound Both in front of Àsph Àcyl; (Àsphere regardless accom m odation is com pletely relaxed
myopic retina Àsph +cyl of notation) Near point: point on visual axis conjugate to retina when
Simple 1 in front, 1 on Àsph +cyl; (Àsphere or plano) accom m odation is fully active
myopic retina plano Àcyl FOR MYOPIA: near point ¼ am ount of m yopia
Mixed 1 in front, 1 Àsph +cyl; (Àsphere or +sphere + am plitude of accom m odation
behind +sph Àcyl depending on FOR HYPEROPIA: near point ¼ difference between
notation)
amplitude of accommodation and amount of hyperopia
Simple 1 on retina, 1 +sph Àcyl; (+sphere or plano)
hyperopic behind plano +cyl
Compound Both behind +sph +cyl; (+sphere regardless
Pr esb yo p ia: loss of accom m odation with age; becom es
hyperopic retina +sph Àcyl of notation) sym ptom atic in early 40s with asthenopic sym ptom s and need
for reading glasses

10
 Prescribing Glasses

Ta b le 1-2. Donder’s table

Age (years): 8 12 16 20 24 28 32 36 40 44 48 52 56 60 64 68
Accommodation (D): 14 13 12 11 10 9 8 7 6 4.5 3 2.5 2 1.5 1 0.5

Th eo r ies o f acco m m o d atio n : Light-adapted eye: yellow, yellow-green, and orange appear
Helmholtz: zonular tension decreases, lens becom es m ore brighter than blues, greens, and reds; peak
spherical, focusing power increases; presbyopia is due to sensitivity ¼ 555 nm
loss of lens elasticity Dark-adapted: peak sensitivity¼ 505 nm (blue)
Tscherning-Schachar: equatorial zonular tension increases,
lens diam eter increases, central lens steepens, focusing Bezo ld -Br u cke p h en o m en o n : as brightness increases,
power increases; lens grows throughout life, decreasing m ost hues appear to change
the working distance between lens and ciliary body; At low intensities, blue-green, green, and yellow-green
presbyopia is result of decreased ciliary m uscle effectivity appear greener; at high intensities, they appear bluer
At low intensities, reds and oranges appear redder; at high
Do n d er ’s tab le: average accom m odative am plitudes for intensities, they appear yellower
different ages (Table 1-2) Exception: blue of 478 nm , green of 503 nm , and yellow of
up to age 40, accom m odation decreases by 1 D every 4 years 578 nm do not change with changes in intensity
(starting at 14 D at age 8)
At age 40, accom m odation is 6.0 D (Æ2 D) Ab n ey effect: as white is added to any hue, desaturating it,
Between ages 40 and 48, accom m odation decreases by 1.5 D the hue appears to change slightly in color; all colors (except
every 4 years yellow) appear yellower
Above age 48, accom m odation decreases by 0.5 D every
4 years After im ages: after a color is stared at for 20 seconds, it
begins to fade (desaturate)
Co n d itio n s th at cau se asth en o p ia (eye fatigue with Then, with gazing at white background, the com plem ent of
sustained near effort): hypothyroidism , anem ia, pregnancy, the color appears (afterim age)
nutritional deficiencies, chronic illness Exa m p le : red is perceived when a greater number of red cones are
stimulated than green or blue cones; after 20 seconds, red cones
fatigue (cannot regenerate pigment fast enough), so color fades; when
Pr em atu r e p r esb yo p ia (subnorm al accom m odation): white background is looked at, there is a relatively greater response by
debilitating illness, diphtheria, botulism , m ercury toxicity, green and blue cones; therefore, a blue-green afterimage is seen
head injury, cranial nerve 3 (CN 3) palsy, Adie’s tonic pupil, (complement of red)
tranquilizers; treat with reading add, base-in prism (helps
convergence) Co lo r p er cep tio n : white wall appears white because white
paint reflects all photons equally well
Co lo r Vis io n Charcoal appears black because it absorbs m ost of the light
3 co m p o n en ts o f co lo r : hue, saturation, lum inosity that strikes it
(lightness, brightness)
Blue flower appears blue because it best absorbs red, yellow,
and green; blue is absorbed least, so a greater num ber of blue
Hu e: m ain com ponent of color perception; depends on which photons are reflected
wavelength is perceived as dom inant; hue discrim ination is the
ability to distinguish between adjacent wavelengths Green leaf appears green because chlorophyll absorbs blue and
red and reflects green
Satu r atio n : richness of color; vivid colors are saturated; Incandescent/tungsten light em its a relatively greater num ber
adding white desaturates color (paler) but does not of photons of longer (red) wavelength than shorter (blue)
change hue; saturation discrim ination is m easured by how wavelength; conversely, fluorescent light em its a relatively
m uch of a specific wavelength m ust be added to white before greater num ber of blue and green wavelengths; therefore, a
the m ixture can be distinguished from white purple dress m ay appear redder under incandescent light and
bluer under fluorescent light
Lu m in o sity: sensation produced by retinal illum ination;
depends on relative lum inous efficiency of the wavelengths;
filters decrease brightness
P RESCRIBING GLASSES
Lu m in o sity cu r ve: illustrates sensitivity to different
wavelengths Use cycloplegia in children and hyperopes to uncover full
Constructed by asking observer to increase lum inance of refractive error
lights of various wavelengths until they appear equal in Infants average 2 D of hyperopia; m yopic shift between ages
brightness to a yellow light of fixed lum inance 8 and 13; m ost adults are em m etropic

11
CHAPTER 1 • Optics

Power of add is difference between accom m odation (1.0 D)

Ta b le 1-3. Guidelines for prescribing glasses for children
and total am ount of accom m odation required (2.5 D)
Hyp e rop ia : ! +5 D With calculated add in front of distance correction, m easure
Anisometropia ! 1.5 D accom m odative range (near point to far point of
Myop ia : accom m odation); if range is too close, reduce add in
Up to age 1 year ! À5 D steps of 0.25 D until correct range found
Ages 1–6 years ! À3 D
Age > 6 years ! À1 D
Kesten b au m ’s r u le: used to estim ate strength of plus lens
required to read newspaper print without accom m odation
Anisometropia ! 3D
Add power ¼ reciprocal of best distance acuity
As tigm a tis m :
Reciprocal of add power ¼ working distance (in m eters)
Up to age 1 year ! 3D
Exa m p le : 20/80 vision; add ¼ 80/20 ¼ + 4.00 D; working distance ¼ 1/4
Ages 1–6 years ! 2D (0.25 m)
Age > 6 years ! 1D
Anisometropia ! 1.5 D
Ap h akic sp ectacles: disadvantages include m agnification
of 25%, altered depth perception, pincushion distortion, ring
scotom a (prism atic effect at edge of lens causes visual field loss
of 20%), “jack-in-the-box” phenom enon (peripherally
invisible objects suddenly appear when gaze is shifted)
Ch ild r en (Table 1-3): give full cycloplegic refraction
Ad u lts: give m anifest refraction; m ay not accept full
astigm atic com ponent, so if cylinder is decreased, adjust sphere CONTACT LENSES (CL)
to keep spherical equivalent constant; be careful about
changing axis To r ic len s: lenses with different radii of curvature in each
m eridian
Min u s cylin d er gr in d in g: placing astigm atic correction on Front toric: anterior surface with 2 different radii of
rear surface (closer to eye) is optically preferable curvature, posterior surface spherical; corrects lenticular
Astigmatic dial: 12 spokes corresponding to clock hours are astigm atism
projected on screen; spokes parallel to principal Back toric: cylinder on back surface only; corrects corneal
m eridians of eye’s astigm atism are sharp (corresponding astigm atism
with focal lines of conoid of Sturm ); the others are Bitoric: m inus cylinder on posterior surface, plus cylinder
blurred on anterior surface; corrects corneal and lenticular
astigm atism
Match b ase cu r ves: when prescribing new glasses, keep
base curve sam e as that of old lenses Ballasted len s: heavier base to orient lens by gravity; two
Geneva lens clock: m easures base curve of lens; direct types:
dioptric power of convex, concave, or aspheric lens Prism: 1.5-2 Δ BD prism added
surface is read on the dial of the clock; calibration is Truncated: flat along inferior edge
based on the refractive index of crown glass (1.52)
Sagittal d ep th /ap ical h eigh t: distance between back
Bin o cu lar b alan ce: equally controls accom m odation in surface of lens center and a flat surface
both eyes (visual acuity m ust be equal)
Methods: Rad iu s o f cu r vatu r e (base curve): curvature of posterior
1. Prism dissociation: 3 Δ BU over one eye and 3 Δ BD over lens surface for a given diam eter; the shorter the radius of
the other (use Risley prism in phoropter) curvature, the greater is the sagittal depth (the steeper the lens)
2. Balanced fogging: fog both eyes and alternate cover until
equally fogged Over all d iam eter : for a given base curve, increasing
3. Duochrome test: red–green balance both eyes (vision diam eter increases the apical vault
m ust be 20/30 or better) Exa m p le : to tighten a lens, reduce radius of curvature or increase diameter

Bifo cal ad d : place segm ents as high as practical in relation to Oxygen tr an sm issio n : DK (relative gas perm eability)
optical centers of the distance lenses value; D ¼ diffusion coefficient; K¼ solubility of oxygen
Measure accom m odation: perform m onocularly, then in m aterial; oxygen transm issibility ¼ DK/L (L¼ lens
binocularly thickness)
Near point of accom m odation (use refractive correction)
Accom m odative am plitude (use Prince rule) Acco m m o d ative d em an d : depends on
Determ ine accom m odative requirem ent for near vision task m agnification, which varies with different lens powers and
Exa m p le : reading at 40 cm ¼ 2.5 D
vertex distances
Hold one-half of m easured accom m odative am plitude in
Hyperopes: decreased accomm odative dem and when CL
reserve to prevent asthenopic sym ptom s
Exa m p le : Prince’s rule measures 2.0 D of amplitude; thus 1.0 D is available are worn com pared with spectacles (presbyopic
to patient sym ptom s appear earlier with spectacles)

12
 Intraocular Lenses (IOL)

Myopes: increased accom m odative dem and when CL Near d evices:

are used (presbyopic sym ptom s appear earlier with High bifocal add or single-vision reading glasses (up to + 20
CL). In high m yopia, spectacles induce base-in prism D): large field of view but short reading distance
with near convergence, lessening requirem ent for Magnifiers: handheld (up to + 20 D; sm all field of view) or
convergence stand (up to + 50 D; bulky)
Loupes: long working distance but sm all field of view
Fittin g r igid CL: SAM-FAP rule (”steeper add m inus, flatter Electronic displays: high m agnification but expensive
add plus”)
Fit steeper than corneal surface (form s a plus tear Distan ce d evices:
m eniscus between cornea and CL, which alters required Telescopes: restricted field of view
power of CL). Therefore, need to subtract power
(add m inus) at end of calculation; for each diopter,
the base curve is m ade ”steeper than K”; subtract 1 D INTRAOCULAR LENSES (IOL)
from the final CL power; if lens is fit flatter than K,
a m inus tear m eniscus is form ed, so m ust add
plus power Form ulas for IOL calculation (see Chapter 10)
Rem em ber, a rigid lens with base curve of 44 D does Empiric: derived from clinical studies by regression
not have a power of 44 D; rather, the radius of the analysis
CL’s central posterior curve is equal to the FIRST GENERATION: SRK, Gills-Lloyd
radius of curvature of a cornea with a calculated SECOND GENERATION: SRK II, Thom pson-M, Donzis
power of 44 D Theoretical: derived from optics by vergence form ulas
FIRST GENERATION: Binkhorst I, Fyodorov,
Colenbrander
Po wer calcu latio n : if trial lens not available for
SECOND GENERATION: Binkhorst II, Sham as
overrefraction
THIRD GENERATION: Hoffer Q, Holladay 1, SRK/T
1. Measure refraction and keratom etry
FOURTH GENERATION: Holladay 2, Haigis
2. Choose base curve steeper than low K (usually
FIFTH GENERATION: Hoffer H-5, Olsen, Barrett
+ 0.50 D steeper to form a tear lens; tear lens prevents
Universal II
apical touch)
Newer formulas: Hill-RBF (pattern recognition algorithm ),
3. Convert refraction to m inus cylinder form and zero
Ladas Super Form ula (am algam of existing form ulas)
vertex distance; disregard the cylinder (m inus cylinder
A rough estim ation of lens power can be quickly obtained
is form ed by the tears)
with the SRK form ula:
4. Power of CL is sphere from refraction adjusted for tear
IOL power for em m etropia ¼ P ¼ AÀ 2.5 LÀ 0.9 K
lens (subtract +0.50); ”SAM-FAP”
A¼ A constant (related to lens type) L¼ axial length
in m m ; 1 m m error ¼ 2.5 D error in IOL power
Evalu atin g fit:
Soft lens: evaluate m ovem ent (poor m ovem ent ¼ too tight
[too steep], excessive m ovem ent ¼ too flat); choose
power based on spherical equivalent
Rigid lens: assess fluorescein pattern (Figs. 1-16–1-18)

LOW-VISION AIDS

Kesten b au m ’s r u le: estim ates strength of plus lens

required to read newspaper print without accom m odation
Fig ure 1-17. Fluorescein pattern of corneal contact lens fitted “on K.”
Note the central alignment. (With permission from White P, Scott C: Contact
lenses. In Yanoff M, Duker J S (eds ): Ophthalmology. London, 1999, Mosby.)

Fig ure 1-16. Fluorescein pattern of corneal contact lens fitted 1 D steeper
than “flat K.” Note the central clearance. (With permission from White P, Fig ure 1-18. Fluorescein pattern of corneal contact lens fitted 1 D flatter than
Scott C: Contact lenses. In Yanoff M, Duker J S (eds ): Ophthalmology. London, “flat K.” Note the central touch. (With permission from White P, Scott C: Contact
1999, Mosby.) lenses. In Yanoff M, Duker J S (eds ): Ophthalmology. London, 1999, Mosby.)

13
CHAPTER 1 • Optics

K¼ average keratom etry value in D; 1.0 D error %1.25 MA ¼ D eye/Dlen s ¼ 60/20 ¼ 3 Â; MAx ¼ 3 2 ¼ 9, but eyepiece
D error in IOL power reduces depth 4 Â; therefore MAx ¼ 9/4 ¼ 2.25 Â
Lens position is im portant: 1 m m error ¼ 1.0 D change
in power
Retin o sco p e: instrum ent to objectively m easure refractive
Calculate IOL power for refractive target other than state of eye
em m etropia: DIO L ¼ P À (R/1.5) (R¼ desired refractive error) The blurred image of the filam ent on the patient’s retina is
considered a new light source returning to exam iner’s
IOL power for a different lens ¼ original IOL
eye; by observing the characteristics of the reflex,
power Ædifference in A constants
Exa m p le : if instead of +20.0 D IOL with A constant of 118, you want to use a exam iner can determ ine patient’s refractive error
different style IOL with A constant of 118.5, equivalent power of the new IOL If exam iner is at far point of patient’s eye, all light rays
is +20.5 D em anating from patient’s pupil pass through retinoscope
and exam iner’s pupil, and patient’s pupil will appear
uniform ly illum inated (neutralization)
OP HTHALMIC INSTRUMENTS If far point is between exam iner and patient (m yopic), reflex
m oves in direction opposite to retinoscope sweep
Dir ect o p h th alm o sco p e (Fig. 1-19): coaxial light (”against” m otion)
and lenses to neutralize patient and exam iner refractive If far point is behind exam iner (hyperopia), reflex has
errors, so retinas becom e conjugate; exam iner uses optics of ”with” m otion
patient’s eye as sim ple m agnifier (MA ¼ 60/4 ¼ 15 Â); field Use correcting lens to determ ine point of neutralization
of view $ 7° Correct for working distance to obtain patient’s final
refraction (add reciprocal of working distance to final
In d ir ect o p h th alm o sco p e (Fig. 1-20): enlarged field of finding)
view (25°) with stereopsis by adding condensing lens between If poor, irregular retinoscopic reflex, try contact lens
patient and exam iner; binocular eyepiece reduces overrefraction or stenopeic slit refraction
interpupillary distance to 15 m m ($ 4 Â);

Obs e rve r P a tie nt Slit-lam p b io m icr o sco p e (Fig. 1-21): illum ination and
m agnification allow stereo viewing of ocular structures;
illum ination and viewing arm s have com m on pivot point

P a rtia l mirror

Light s ource

Conde ns ing
Fig ure 1-19. Optics of the direct ophthalmoscope. By using a mirror (either
le ns
half-silvered or with a central aperture), the directions of the light of S lit
observation and the light incident to the patient are made concentric Filte r/tra y
(coaxial). (With permission from Miller D, Thall EH, Atebara NH: Ophthalmic
instrumentation. In Yanoff M, Duker J S (eds): Ophthalmology, ed 2, St Louis,
2004, Mosby.)

Obs e rve r's e ye P a tie nt's e ye

S lit ima ge
Mirror

Mirror
P upil re tina

Le ns
Ente ring illumina tion be a m Light
Exiting obs e rva tion be a m s ource Micros cope
Fig ure 1-20. Theoretical optics of the indirect ophthalmoscope. The Fig ure 1-21. The slit lamp. Some slit lamps bring the light to a sharp focus
illumination beam enters a small part of the pupil and does not overlap with within the slit aperture, and the light within the slit is focused by the
the observation beam; this minimizes bothersome reflection and condensing lens onto the patient’s eyes. The observation s ystem of a modern slit
backscatter. (With permission from Miller D, Thall EH, Atebara NH: Ophthalmic lamp has many potential reflecting surfaces – antireflection coatings on these
instrumentation. In Yanoff M, Duker J S (eds): Ophthalmology, ed 2, St Louis, surfaces help loss of light. (Modified from Spalton DJ , Hitchings RA, Hunter PA:
2004, Mosby.) Atlas of clinical ophthalmology. New York, 1985, Grower Medical.)

14
 Equations

Len so m eter : m easures power of spectacle or CL using Ocu lar co h er en ce to m o gr ap h y: m easures optical
telescope to detect neutralization point; distance m easurem ent reflectivity to provide cross-sectional im age of ocular
is determ ined from back vertex power; add m easurem ent is structures
taken from front vertex power; prism m easurem ent is
derived from displacem ent of target pattern (Figs. 1-22
and 1-23) EQUATIONS
Ker ato m eter : m easures curvature of anterior corneal Ver gen ce fo r m u la:
surface based on power of reflecting surface; m easures only two
paracentral points 3 m m apart; doubling of im age prevents U+D ¼V
interference from eye m ovem ents (Fig. 1-24)
U ¼ object vergence, D ¼ lens power, V¼ im age vergence

Ap p lan atio n to n o m etr y: direct m easure of IOP as force/ Len s p o wer ( d io p ter s) :
area with split-field prism ; at applanated diam eter of 3.06 m m ,
corneal resistance to deform ation and attractive force of tear D ¼ 1=f
surface tension cancel each other
f¼ focal length (m eters)

Ultr aso n o gr ap h y: m easure acoustic reflectivity of Sn ell’s law:

interfaces to provide axial m easurem ents and 2-dim ensional
im ages of ocular structures; various devices (A-scan, B-scan, nsin ðiÞ¼ n’sin ðr Þ
ultrasound biom icroscope [UBM], pachym eter)
n ¼ refractive index, i ¼ angle of incidence,
r ¼ angle of refraction

P os ition of Illumina te d Fixe d fie ld Unknown

Pr ism atic p o wer :
ta rge t ima ge mova ble le ns le ns Te le s cope
im age deflection ðcm Þ
by fie ld le ns ta rge t Δ¼ ¼ 100 tan ðβÞ
m eters
β ¼ angle of deviation
F
F
Pr en tice’s r u le:
Δ ¼ hD

h ¼ distance from optical axis (cm ), D ¼ lens power

Ba ck foca l le ngth of unknown le ns

Fig ure 1-22. The lensometer resembles an optical bench. The movable
illuminated target sends light to the field lens, with the target in the endpoint
position. Because the focal point of the field coincides with the position of the
unknown lens, all final images are of the same size. (With permission from Miller
D, Thall EH, Atebara NH: Ophthalmic instrumentation. In Yanoff M, Duker J S
(eds): Ophthalmology, ed 2, St Louis, 2004, Mosby.) Illumina te d ring Corne a

Virtua l
ima ge
Fixe d of ring
mirror

Gre e n Be a m S ma ll Be a m White light

filte r forming a pe rture forming s ource
Le ns optics optics 4mm
Ring a pe rture which Be a m pa tte rn in
de fine s de te cte d ra ys de te ctor pla ne
Fixe d Line a r a rra y
mirror photo-de te ctor
(pos ition 1)
Be a m Ima ge (pos ition 2)
forming of ring
le ns a pe rture

Fig ure 1-23. Optics of a typical automated lensometer. Parallel light strikes Fig ure 1-24. Keratometer principle. An illuminated ring is placed in front of
unknown lens. The refracted light rays (which are confined to a pencil beam the cornea, which acts as a convex mirror and produces a virtual image of
within an annulus) ultimately strike an array of electronic photoreceptors. (With the ring approximately 4 mm behind the cornea. (With permission from Miller D,
permission from Miller D, Thall EH, Atebara NH: Ophthalmic instrumentation. Thall EH, Atebara NH: Ophthalmic instrumentation. In Yanoff M, Duker J S
In Yanoff M, Duker J S (eds): Ophthalmology, ed 2, St Louis, 2004, Mosby.) (eds): Ophthalmology, ed 2, St Louis, 2004, Mosby.)

15
CHAPTER 1 • Optics

Red u ced sch em atic eye ( calcu latio n s o f r etin al Telesco p e ( Galilean an d astr o n o m ical) :
im age size) :
MA ¼ ÀD e =D o
I O
¼ D e ¼ power of eyepiece, D o ¼ power of objective
17m m X
I ¼ retinal im age size, O ¼ object size, X¼ distance to object
To tal acco m m o d atio n th r o u gh telesco p e:
Sp h er ical equ ivalen t:
AT ¼ AN ðMAÞ2
spherical equivalent ¼ sphere + ðcylinder=2 Þ
AN ¼ norm al accom m odation required, MA ¼ telescope
m agnification
Refr actin g p o wer o f a sp h er ical su r face:
n 0À n
Ds ¼ IOL p o wer ( SRK) :
r
n0¼ refractive index to right, n ¼ refractive index to left, P ¼ AÀ 2:5 LÀ 0:9K
r ¼ radius of curvature of surface (m), (+¼ convex;
A¼ A constant for type of IOL, L¼ axial length, K¼ average
À ¼ concave)
keratom etry value

Reflectin g p o wer o f a sp h er ical m ir r o r :

À1 À2 AC/A r atio ( acco m m o d ative co n ver ge/
Dr ¼ ¼ acco m m o d atio n ) : (see Chapter 5, Pediatrics/Strabism us)
f r
f¼ focal length of m irror, r ¼ radius of curvature of m irror, norm al ¼ 3 : 1 to 5 : 1 (expressed as PD of deviation per D
(À ¼ convex; + ¼ concave) of accom m odation)

Po wer o f a th in len s im m er sed in flu id : Calcu latio n s:

HETEROPHORIA METHOD: m easure deviation with
D air n IOL À n air fixation target at 6 m and at 1
3 m
¼
D aqueous n IOL À n aqueous
deviation at near À deviation at distance
AC=A ¼ + PD
accom m odative demand
Po wer o f len s at n ew ver tex d istan ce: PD ¼ interpupillary distance ðcm Þ
D 2 ¼ D 1 ¼ SðD 1 Þ2 LENS GRADIENT METHOD: stim ulate accom m odation by
m easurin g deviation with target at 6 m , th en
D 1 ¼ original dioptric power of lens, D 2 ¼ new dioptric
rem easurin g with –1 D sphere in fron t of both eyes; or,
power, S¼ difference in location (m )
relax accom m odation by m easurin g deviation with
target at on e-th ird m , th en rem easurin g with +3 D
Lin ear m agn ificatio n : sphere in fron t of both eyes
ML ¼ I=O deviation with lens À deviation without lens
AC=A ¼
lens power
I ¼ im age distance or height, O ¼ object distance or height

Axial m agn ificatio n :

Max ¼ ðMLÞ2 REVIEW QUESTIONS (Answers start on page 371)

An gu lar m agn ificatio n : 1. Prince’s rule is helpful in determ ining all of the following
except
MA ¼ xD a. am plitude of accom m odation
b. near point of accom m odation
x¼ distance, D ¼ dioptric power of lens
c. far point of accom m odation
d. accom m odative convergence
Sim p le m agn ifier : 2. A m yope who pushes his spectacles closer to his face and
tilts them is
MA ¼ D=4
a. decreasing effectivity, increasing cylinder
(divide by 4 because distance set as reading distance b. decreasing effectivity, decreasing cylinder
of 0.25 m ) c. increasing effectivity, decreasing cylinder
Exa m p le : 1 Â magnifier ¼ + 4 lens; 2 Â magnifier ¼ + 8 lens d. increasing effectivity, increasing cylinder

16
 Review Questions

3. The Prentice position refers to a. im age jum p

a. glass prism perpendicular to visual axis b. im age displacem ent
b. glass prism in frontal plane c. induced prism
c. plastic prism perpendicular to visual axis d. anisophoria
d. plastic prism in frontal plane 13. A refraction with a stenopeic slit gives the following
4. The purpose of Q-switching a laser is to m easurem ents: +1.00 at 90° and À2.00 at 180°. The
a. increase energy, increase power corresponding spectacle prescription is
b. decrease energy, increase power a. À2.00 + 1.00 Â 90
c. decrease energy, decrease power b. À2.00 + 1.00 Â 180
d. increase energy, decrease power c. À2.00 + 3.00 Â 180
5. A 50-year-old wom an with aphakic glasses wants a new d. +1.00 + 3.00 Â 90
pair of spectacles to use when applying m ake-up. How 14. A point source of light is placed 1 3 of a m eter to
m uch power should be added to her distance correction the left of a +7 D lens. Where will its im age com e to
so that she can focus while sitting 50 cm in front of her focus?
m irror? a. 25 cm to the right of the lens
a. À2.00 D b. 25 cm to the left of the lens
b. À1.00 D c. 10 cm to the right of the lens
c. +1.00 D d. 10 cm to the left of the lens
d. +2.00 D 15. What is the equivalent sphere of the following cross
6. How far from a plano m irror m ust a 6-ft-tall m an stand to cylinder: À3.00 Â 180 com bined with +0.50 Â 90?
see his whole body? a. À1.00
a. 2 feet b. À1.25
b. 3 feet c. À1.50
c. 6 feet d. À1.75
d. 12 feet 16. What is the size of a letter on a standard 20-ft
7. A 33-year-old wom an with a refraction of À9.00 Snellen chart if it form s an im age of 1 2 m m on a patient’s
+3.00 Â 90 OD at vertex distance 10 m m and keratom etry retina?
readings of 46.00@90/43.00@180 is fit for a rigid gas- a. 59 cm
perm eable (RGP) contact lens 1 D steeper than flattest K. b. 30 cm
What power lens is required? c. 25 cm
a. À5.00 D d. 18 cm
b. À6.00 D 17. The im age of a distant object is largest in which patient?
c. À7.00 D a. aphake with contact lens
d. À8.00 D b. hyperope with spectacles
8. What is the size of a 20/60 letter on a standard 20-ft c. em m etrope
Snellen chart (tangent of 1 m inute of arc¼ 0.0003)? d. m yope with spectacles
a. 9 m m 18. What type of im age is produced if an object is placed in
b. 15 m m front of a convex lens within its focal length?
c. 18 m m a. erect and real
d. 27 m m b. erect and virtual
9. A Galilean telescope with a +5 D objective and a À20 D c. inverted and real
eyepiece produces an im age with what m agnification and d. inverted and virtual
direction? 19. What is the correct glasses prescription if retinoscopy
a. 4 Â, erect perform ed at 50 cm shows neutralization with a plano
b. 4 Â, inverted lens?
c. 100 Â, erect a. À2.00
d. 100 Â, inverted b. À1.50
10. An object is placed 33 cm in front of an eye. The im age c. plano
form ed by reflection from the front surface of the cornea d. +2.00
(radius of curvature equals 8 m m ) is located 20. An anisom etropic patient experiences difficulty while
a. 4 m m in front of cornea reading with bifocals. Which of the following is not
b. 4 m m behind cornea helpful for reducing the induced phoria?
c. 8 m m in front of cornea a. dissim ilar segm ents
d. 8 m m behind cornea b. slab-off lens
11. A convex m irror produces what type of im age? c. progressive lenses
a. virtual, inverted, m agnified d. fresnel “press-on” prism s
b. real, inverted, m inified 21. A Geneva lens clock is used to m easure what?
c. real, erect, m agnified a. thickness
d. virtual, erect, m inified b. power
12. In general, the m ost bothersom e problem associated with c. index of refraction
bifocals is d. base curve

17
CHAPTER 1 • Optics

22. What is the induced prism when a 67-year-old wom an 31. logMAR equivalent to 20/40 Snellen acuity is
reads 10 m m below the upper segm ent optical center of a. 0.4
her bifocals, which m easure +2.50 + 1.00 Â 90 OD and b. 0.3
À1.50 + 1.50 Â 180 OS add +2.50 OU c. 0.2
a. 4.0 Δ d. 0.1
b. 3.5 Δ 32. A patient who is pseudophakic in one eye and
c. 2.5 Δ phakic in the other eye will have what am ount of
d. 2.0 Δ aniseikonia?
23. The optim al size of a pinhole for m easuring pinhole visual a. 1%
acuity is approxim ately b. 2.5%
a. 2.50 m m c. 5%
b. 2.00 m m d. 7%
c. 1.25 m m 33. A patient with 20/80 vision is seen for a low-vision
d. 0.75 m m evaluation. What add power should be prescribed so the
24. A person looking at an object 5 m away through a 10 Δ patient does not have to use accom m odation to read the
prism placed base-in over the right eye would see the newspaper?
im age displaced a. +3
a. 20 cm to the right b. +4
b. 50 cm to the right c. +8
c. 20 cm to the left d. +16
d. 50 cm to the left 34. The spherical equivalent of a À2.00 + 1.50 Â 90 lens is
25. Calculate the soft contact lens power for a 40-year-old a. +0.50
hyperope who wears +14.00 D glasses at a vertex distance b. À0.50
of 11 m m . c. À1.25
a. +15.00 D d. À3.50
b. +16.00 D 35. After extracapsular cataract extraction a patient is found to
c. +17.00 D have 2 D of with-the-rule astigm atism and a tight suture
d. +18.00 D across the wound at 12 o’clock. Corneal topography is
26. After cataract surgery, a patient’s refraction is À0.75 obtained and the placido disc im age shows an oval pattern
+ 1.75 Â 10, in what m eridian should a suture be cut to with the m ires closest together at
reduce the astigm atism ? a. 12 o’clock with the short axis at 90°
a. 180° b. 12 o’clock with the short axis at 180°
b. 100° c. 6 o’clock with the short axis at 90°
c. 90° d. 6 o’clock with the short axis at 180°
d. 10° 36. A 57-year-old wom an has a 0.25 m m m acular hole in her
27. What is the appropriate correction in the IOL power if the left eye. The size of the corresponding scotom a on a
A constant for the lens to be im planted is changed from tangent screen at 1 m is approxim ately
117 to 118? a. 1.0 cm
a. decrease IOL power by 1.0 D b. 1.5 cm
b. increase IOL power by 1.0 D c. 2.5 cm
c. decrease IOL power by 0.5 D d. 3.0 cm
d. increase IOL power by 0.5 D 37. During retinoscopy, when neutralization is reached, the
28. An IOL labeled with a power of +20 D has a refractive light reflex is
index of 1.5. If this lens were rem oved from the package a. narrowest and slowest
and m easured with a lensom eter, what power would be b. narrowest and brightest
found? c. widest and slowest
a. +10 D d. widest and fastest
b. +13 D 38. A patient undergoing fogged refraction with an
c. +30 D astigm atic dial sees the 9 to 3 o’clock line clearer than all
d. +59 D the others. At what axis should this patient’s m inus
29. The total cylindrical power of a 0.50 D cross cylinder is cylinder correcting lens be placed?
a. plano a. 30°
b. 0.25 D b. 45°
c. 0.50 D c. 90°
d. 1.00 D d. 180°
30. To m inim ize im age displacem ent in a hyperope, the best 39. Myopia is associated with all of the following conditions
type of bifocal segm ent style is except
a. flat top a. nanophthalm os
b. progressive b. pigm ent dispersion syndrom e
c. round top c. spherophakia
e. executive d. nuclear sclerotic cataract

18
 Review Questions

40. What is the ratio of the m agnification from a direct c. increasing nuclear sclerosis
ophthalm oscope to the m agnification from an indirect d. cystoid m acular edem a
ophthalm oscope with a 20 D lens at a distance of 25 cm if 49. The m ost com m on cause of m onocular diplopia is
the patient and exam iner are both em m etropic? a. uncorrected astigm atism
a. 15 :2 b. anterior basem ent m em brane dystropy
b. 10 :3 c. posterior capsular opacity
c. 5 :1 d. epiretinal m em brane
d. 4 :1 50. A patient with a prescription of À5.50 sphere OD and
41. A patient with anisom etropia wears glasses with a À1.00 sphere OS is 20/20 in each eye but glasses cause
prescription of +5.00 OD and +1.25 OS. Which of the headache and double vision. The m ost likely reason is
following actions will not reduce the am ount of a. esophoria
aniseikonia? b. spherical aberration
a. decrease base curve of right lens c. im age jum p
b. decrease center thickness of left lens d. aniseikonia
c. decrease vertex distance of the glasses 51. A hyperopic refraction is m ost likely caused by
d. fit the patient with contact lenses a. steep cornea and short axial length
42. The principal m easurem ent determ ined by a Prince’s rule b. steep cornea and long axial length
and +3 D lens in front of the patient’s eye is the c. flat cornea and short axial length
a. range of accom m odation d. flat cornea and long axial length
b. am plitude of accom m odation 52. How m uch accom m odation is needed for a patient with a
c. near point of accom m odation distance correction of +1.50 to read without correction
d. accom m odative convergence at 40 cm ?
43. The 10 Â eyepiece of the slit-lam p biom icroscope is a. +1.50
essentially a sim ple m agnifier. Using the standard b. +2.50
reference distance of 25 cm , what is the dioptric power of c. +4.00
the 10 Â eyepiece? d. +5.50
a. +2.5 D 53. The lim bal relaxing incision for a patient with a refraction
b. +10 D of +0.75À1.50 Â 180 and no lenticular astigm atism
c. +25 D should be placed at
d. +40 D a. 45 degrees
44. When refracting an astigm atic patient with a Lancaster b. 90 degrees
dial, the exam iner should place the c. 150 degrees
a. circle of least confusion on the retina d. 180 degrees
b. posterior focal line on the retina 54. The denom inator in Snellen visual acuity notation 20/60
c. anterior focal line behind the retina represents:
d. entire conoid of Sturm in front of the retina a. the lowest line the patient was able to read at least 3
45. To increase the m agnification of the im age during indirect letters
ophthalm oscopy, the exam iner should: b. the distance in feet from the patient to the chart
a. m ove closer to the condensing lens c. the angle between the patient and the chart
b. m ove the eyepiece prism s farther apart d. the distance at which the letter subtends the standard
c. use a higher dioptric power condensing lens visual angle
d. rem ove the plus lens in the eyepiece 55. A 26-year-old-wom an with a history of m yopic LASIK
46. A patient with which of the following refractive errors is com plains of blurriness OD. Her uncorrected visual acuity
m ost likely to develop am blyopia? is 20/20 but corneal topography shows a decentered
a. À4.00 OD, À1.50 OS ablation. Which aberration is m ost likely to be found on
b. +1.00 + 0.50 Â 180 OD, À1.00 + 0.50 Â 180 OS wavefront analysis?
c. +5.00 OD, +5.00 OS a. first order
d. À3.00 + 2.00 Â 90 OD, À2.00 + 1.00 Â 90 OS b. second order
47. A 23-year-old m an reports blurry vision at near and wears c. third order
+1.25 reading glasses to see clearly. Which of the d. fourth order
following is m ost likely to be found on exam ination? 56. If a Snellen chart is not present, then which of the
a. latent hyperopia following tests is best for evaluating visual acuity?
b. nuclear sclerosis a. contrast sensitivity
c. prem ature presbyopia b. wavefront aberrom etry
d. exophoria c. Scheim pflug im aging
48. In 1 year, a diabetic 60-year-old wom an has a change in d. optical coherence tom ography
refraction from À5.00 to À6.50 OU, which im proves her 57. A cycloplegic refraction would be m ost helpful for a
vision to 20/20 OU. What is the m ost likely cause of her a. 10-year-old who has headaches
refractive change? b. 19-year-old who cannot see road signs clearly
a. steepening anterior corneal curvature c. 44-year-old who notices m ore trouble reading
b. increasing axial length d. 65-year-old who has trouble seeing a golf ball

19
CHAPTER 1 • Optics

58. The m ost likely cause of a refractive surprise after

uncom plicated cataract surgery is SUGGESTED READINGS
a. incorrect preoperative refraction
b. failure to obtain cycloplegic refraction Basic and Clinical Sciences Course. (2016). Section 3: Clinical Optics.
c. lack of corneal pachym etry m easurem ent San Francisco: AAO.
d. wrong keratom etry readings Benjam in, W. J. (2006). Borish’s clinical refraction (2nd ed.).
59. Decreased color discrim ination is m ost likely to be caused Philadelphia: Elsevier, 2006.
Milder, B., Rubin, M. L., & Weinstein, G. W. (1991). The fine art of
by which type of cataract?
prescribing glasses without making a spectacle of yourself. Gainesville:
a. nuclear sclerotic Triad Scientific Publications.
b. cortical Rubin, M. L. (1993). Optics for clinicians. Gainesville: Triad Scientific
c. anterior subcapsular Publications.
d. posterior subcapsular
60. A 3-piece acrylic IOL intended for the capsular bag is
placed in the sulcus without optic capture through the
capsulotom y. This patient is m ost likely to experience
what type of refractive error?
a. astigm atism
b. em m etropia
c. hyperopia
d. m yopia

20
2 Pharm acology
OCULAR PHARMACOLOGY
ROUTES OF ADMINISTRATION
ANESTHETICS
AUTONOMIC SYSTEM
OCULAR HYPOTENSIVE (GLAUCOMA) MEDICATIONS
ANTIINFLAMMATORY DRUGS
ANTIALLERGY MEDICATIONS
IMMUNOSUPPRESSIVE AGENTS
ANTI-INFECTIVE DRUGS
MISCELLANEOUS
OCULAR TOXICOLOGY

OCULAR P HARMACOLOGY (therapeutic) concentration of antibiotic at target site against a

target organism .
Ph ar m aco d yn am ics: the study of the biochem ical and
physiologic effects of drugs and their m echanism s of action. In h ib ito r y qu o tien t ( IQ) : The m ost potent antibiotic has
the lowest m inim um inhibitory concentration (MIC) (or
Ph ar m aco kin etics: the study of the factors that determ ine highest inhibitory quotient).
the relationship between drug dosage and the change in
concentration over tim e in a biological system .

Bio availab ility: the am ount of drug absorbed (penetration ROUTES OF ADMINISTRATION
into ocular tissues).
Depends on concentration, rate of absorption, tissue To p ical: absorption related to corneal penetration
binding, transport, m etabolism , and excretion. 1 drop ¼ 50 µL (20–40 drops in m ost bottles).
Methods of improving bioavailability: Conjunctival cul-de-sac holds only 10 µL (20% of drop)
INCREASE CONCENTRATION: lim ited by solubility and Drop diluted by reflex tearing and normal tear turnover: only
tonicity (hypertonicity causes reflex tearing, which $ 50% of drug that reaches the cul-de-sac is present
dilutes and washes drug from the eye). 4 m inutes later (10% of drop)
SURFACTANTS: surface-active agents alter cell Corneal barriers to penetration:
m em branes, increasing perm eability of the corneal TIGHT JUNCTIONS (epithelium and endothelium ): lim it
epithelium . passage of hydrophilic drugs
OSMOTICS: alter tonicity. STROMA (water rich): lim its passage of lipophilic drugs
INCREASE PH: increases nonionized (lipid-soluble) Methods of increasing absorption:
form of drug, increasing corneal penetration (pH of 1. Add surfactants (disrupts epithelial integrity; e.g.,
tears ¼ 7.4). benzalkonium chloride, topical anesthetic).
INCREASE VISCOSITY: viscous additives 2. Prom ote punctal occlusion (decreases drainage).
(m ethylcellulose, polyvinyl alcohol) increase contact 3. Close eyelid after drops are placed.
tim e and therefore penetration. 4. Increase lipid solubility of drug (increases pH); m ore
INCREASE CONTACT TIME: gels and oil-based ointm ent im portant than water solubility.
form ulations (m ineral oil, petrolatum ), polym er 5. Increase frequency of drops.
m atrix (DuraSite); m ust be able to release drug. Topical m edications have system ic adverse effects because
the drugs bypass hepatic ”first-pass” m etabolism
Th er ap eu tic in d ex: a m ethod of com paring potency of (cul-de-sac! nasolacrim al duct ! m ucosa
different antibiotics. A m easure of relative effective ! bloodstream ).

21
CHAPTER 2 • Pharm acology

Examples of other delivery systems: To p ical: disturb intercellular junction of corneal epithelium
PRODRUG: dipivalyl epinephrine (Propine; prodrug of (increase perm eability)
epinephrine, less toxicity), nepafenac (Nevanac; Proparacaine (Ophthaine): 10- to 30-m inute duration,
prodrug of am fenac). corneal toxicity; m ay cause allergic derm atitis (also
OINTMENT: drugs that have better uptake in ointm ent com m on with atropine and neom ycin); does not
form , including tetracycline, chloram phenicol, and necessarily have allergic cross-reactivity with tetracaine
fluorom etholone. Tetracaine (Pontocaine): sim ilar to proparacaine but
SUSTAINED RELEASE GEL: pilocarpine (Pilopine HS; longer duration and m ore toxic to corneal epithelium
decreased dosing) Benoxinate: sim ilar to proparacaine; com bined with
INSERT: pilocarpine (Ocusert; m em brane-controlled fluorescein (Fluress) for tonom etry
system ; slow release over 1 week); hydroxypropyl Cocaine: greatest epithelial toxicity; excellent anesthesia,
cellulose (Lacrisert; artificial tear slow-release pellet); sym pathom im etic effect (test for Horner’s syndrom e)
collagen shield (bandage contact lens presoaked in
antibiotic)
Par en ter al: m ay be used with epinephrine (1 :100,000)
to increase duration by preventing systemic absorption;
Su b co n ju n ctival/su b -ten o n ’s: increases duration and also decreases bleeding; hyaluronidase (Wydase) 150 IU
concentration, bypasses conjunctival and corneal barriers, increases tissue penetration, but decreases duration. Side
avoids system ic toxicity; useful if poor com pliance effect of retrobulbar anesthesia ¼ respiratory depression,
bradycardia
Toxicity: hypotension, convulsions, nausea, vom iting
Retr o b u lb ar /p er ib u lb ar : used for anesthesia; alcohol or
Lidocaine (Xylocaine): 1-hour duration (2 hours with
chlorprom azine (to kill pain fibers and optic nerve [ON] in
epinephrine); used for local anesthesia:akinesia
blind, painful eye)
Procaine (Novocain): 30- to 45-m inute duration
Mepivacaine (Carbocaine): 2-hour duration
In tr ao cu lar : direct ocular effects; beware toxicity Bupivacaine (Marcaine): 6-hour duration
(particularly from preservatives), retinal tear, retinal
detachm ent (RD), endophthalm itis; used intraoperatively
Gen er al: all agents decrease intraocular pressure (IOP)except
(intracam eral, intravitreal) and in retinal diseases (intravitreal)
ketam ine, chloral hydrate, N 2 O, and ether
including m acular edem a, choroidal neovascularization
Malignant hyperthermia: rare, autosom al dom inant
(CNV), endophthalm itis, cytom egalovirus (CMV) retinitis
condition that occurs after exposure to inhalation agents
(m ost com m only halothane, also succinylcholine,
System ic: m ust cross blood–ocular barrier (blood–aqueous haloperidol); m ore com m on in children and m ales;
for anterior segm ent, blood–retinal for posterior segm ent); thought to be due to calcium -binding disorder in
penetrance is im proved with decreased m olecular size, sarcoplasm ic reticulum that causes increased intracellular
decreased protein binding, and increased lipid solubility calcium , which stim ulates m uscle contraction
Interference with oxidative phosphorylation causes
hyperm etabolic crisis
Co n c e n t ra t io n Most have defect in ryanidine receptor (RYR-1 gene on
1% solution ¼ 1 g/100 m L¼ 10 m g/m L chrom osom e 19q13.1)
Exa m p le : How much atropine is contained in 5 mL of a 2% solution? FINDINGS: tachycardia (first sign), elevated CO 2 levels,
g mg
tachypnea, unstable BP, arrhythm ias, cyanosis,
2% ¼ 2 mL ¼ 20 ¼ 100 mg in 5 mL sweating, m uscle rigidity (trism us from m asseter
100 mL
rigidity), increased tem perature (later sign);
later, heart failure and dissem inated intravascular
coagulation develop; laboratory tests show respiratory
ANESTHETICS and m etabolic acidosis and increased K, Mg,
m yoglobin, creatine phosphokinase,hypoxem ia,
hypercarbia, m yoglobinuria
Mech an ism : reversible blockade of nerve fiber conduction
SCREENING: elevated creatine phosphokinase, m uscle
(block sodium channels); pH dependent (less effective at low
biopsy/ contracture test, platelet bioassay (decreased
pH [inflam ed tissue])
adenosine triphosphate in platelet exposed to
halothane)
Str u ctu r e: two classes; do not necessarily have allergic TREATMENT: stop anesthesia, hyperventilate with 100%
cross-reactivity oxygen, give sodium bicarbonate, cool patient (iced
Ester: hydrolyzed by plasm a cholinesterase and saline intravenous (IV) and lavage; surface cooling),
m etabolized in liver; cocaine, tetracaine (am ethocaine), m annitol and furosem ide (Lasix), IV dantrolene
proparacaine, procaine, benoxinate (prevents release of calcium from sarcoplasm ic
Amide: longer duration and less system ic toxicity; reticulum ), procainam ide, insulin (do not use lactated
m etabolized in liver; lidocaine, m epivacaine, Ringer’s solution, which increases potassium )
bupivacaine PROGNOSIS: < 5% m ortality

22
 Autonom ic System

Muscarinic: postganglionic parasym pathetic nerves (iris

AUTONOMIC SYSTEM sphincter, ciliary m uscle [inside eye])

S ym p a t h e t ic
Neu r o tr an sm itter : ACh
Extensive system for mass response (“fight or flight”) (Table 2-1) Acetylcholinesterase (AChE) breaks down ACh
Synapses near cord (superior cervical ganglion)
Long postganglionic nerves Ch o lin e rg ic d ru g s
Ad r en er gic r ecep to r s: Dir ect-actin g ago n ists: act on end organ; therefore, do
α 1: sm ooth m uscle contraction (arteries [decrease aqueous not need intact innervation; cause shallowing of anterior
production by reducing ciliary body blood flow], iris cham ber (AC); disruption of blood–aqueous barrier, m iosis,
dilator, Mu€ ller’s m uscle) brow ache, and decrease in IOP
α 2: feedback inhibition, ciliary body (decreases production Acetylcholine (m iochol: very short acting, unstable, used
and/or increases outflow) intracam erally), m ethacholine, carbacholine
β1: cardiac stim ulation (carbam ylcholine, carbachol; direct and indirect acting),
β2: pulm onary and gastrointestinal (GI) sm ooth m uscle pilocarpine (less potent than ACh, but resistant to AChE;
relaxation, ciliary body/trabecular m eshwork (increase no m iosis if IOP > 40 m m Hg)
aqueous production, increase outflow facility)

Neu r o tr an sm itter : acetylcholine (ACh) at preganglionic In d ir ect-actin g ago n ists: anticholinesterases (AChE
term inal, epinephrine and norepinephrine (NE) at inhibitor); strongest agents; cause m iosis and decrease in IOP
postganglionic term inal (contract longitudinal fibers of ciliary m uscle ¼ increased
Monoamine oxidase (MAO) breaks down NE in nerve outflow of trabecular m eshwork).
terminal; blocked by MAO inhibitors (avoid with Reversible: carbacholine, physostigm ine (eserine; treat
phenylephrine, epinephrine, and pseudoephedrine-based lid lice), edrophonium (Tensilon; for diagnosis of
cold remedies) m yasthenia gravis): can cause bradycardia, treat with
Catechol-O-m ethyltransferase breaks down NE in atropine
effector cell Irreversible: echothiophate (phospholine iodide; treat
Reserpine prevents storage of NE in nerve term inal glaucom a and accom m odative esotropia; m ay cause iris
Cocaine, tricyclics block reuptake of NE by nerve term inal cysts and subcapsular cataracts), isofluorophate (very
(thus potentiate its action) long-acting, not used clinically), dem ecarium
Hydroxyam phetam ine increases release of NE from nerve Adverse effects: cataract, RD, pupillary block, blocks
term inal m etabolism of succinylcholine (prolonged respiratory
paralysis) and ester anesthetics, can m im ic acute
P a ra s ym p a t h e t ic abdom en (GI effects); antidote is pralidoxim e (PAM) or
atropine
More lim ited system for discrete response (hom eostatic)
Synapses near end organ (ciliary ganglion)
Short postganglionic nerves Mu scar in ic an tago n ists: anticholinergics; cause
deepening of AC, stabilization of blood–aqueous barrier,
m ydriasis, and cycloplegia
Ch o lin er gic r ecep to r s:
Atropine (1- to 2-week duration; m ost allergenic,
Nicotinic: som atic m otor and preganglionic autonom ic
supersensitivity seen in Down syndrom e), scopolam ine
nerves (extraocular m uscles, levator, orbicularis
(hyoscine; 1-week duration; greater central nervous
[outside eye])
system (CNS) toxicity than atropine), hom atropine
(1- to 3-day duration), cyclopentolate (Cyclogyl;
Ta b le 2-1. Autonomic system responses 24-hour duration; beware CNS side effects with 2%
Sym p a the tic P a ra s ym p a the tic solution, especially in children), tropicam ide (Mydriacyl;
Org a n / Func tio n (fig ht / flig ht) (ho m e o s ta s is ) 4- to 6-hour duration)
Toxicity: m ental status changes, hallucinations,
HR Increase Decrease
tachycardia, urinary retention, dry m outh/skin, fever
BP Increase Decrease
Antidote: physostigm ine (1-4 m g IV)
GI motility Decrease Increase
Bronchioles Dilate Constrict
Bladder Constrict Dilate Nico tin ic an tago n ists:
Vessels Constrict Dilate Nondepolarizing agents: gallam ine, pancuronium ; do not
Sweat Decrease Increase cause m uscle contraction
Pupils Dilate Constrict Depolarizing agents: succinylcholine, decam ethonium ;
Eyelids Elevate Normal
cause m uscle contraction and elevated IOP;
contraindicated for ruptured globe (extraocular m uscle
BP, Blood pressure; GI, gastrointes tinal; HR, heart rate.
contraction can cause extrusion of intraocular contents)

23
CHAPTER 2 • Pharm acology

Ad re n e r g ic d r u g s α 2 -Ag o n is t s
Sym p ath o m im etics: m ay cause m ydriasis, Apr aclon idin e ( Io pidin e), b rimo n idin e ( Alph agan P):
vasoconstriction, decreased IOP Mechanism: reduce aqueous production by decreasing
Direct-acting α-agonists: epinephrine, phenylephrine, episcleral venous pressure
dipivefrin, clonidine, apraclonidine (iopidine), Adverse effects: allergy, superior lid retraction, dry m outh,
brim onidine, m ethyldopa, naphazoline blanching of conjunctival vessels, m iosis, lethargy,
(Naphcon), oxym etazoline (Afrin), tetrahydrozoline headache, stom ach cram ps; avoid in children, especially
(Visine) < 3 years of age: increased risk of som nolence, seizures,
Direct-acting β-agonists: epinephrine, isoproterenol, apnea due to CNS penetration
terbutaline, dopam ine, albuterol (salbutam ol)
Indirect-acting agonists: cocaine, hydroxyam phetam ine Dip ivefr in (Propine): prodrug of epinephrine (converted
(Paredrine), ephedrine into epinephrine in cornea by esterases), lower concentration
and increased solubility vs epinephrine (penetrates cornea
17 Â better); thus less toxicity and fewer adverse effects
Sym p ath o lytics: m ay cause decreased IOP Mechanism: im proves aqueous outflow, slightly reduces
General blockers: guanethidine, bethanidine, protriptyline, aqueous production
6-hydroxydopam ine Adverse effects: allergy, cystoid m acular edem a (CME) in
α-blockers: thym oxam ine, dibenam ine, aphakia, hypertension, tachycardia (fewer system ic
phentolam ine, prazosin, labetalol, dapiprazole adverse effects than epinephrine)
(Rev-Eyes; m iosis)
β-blockers: tim olol, levobunolol, betaxolol, m etipranolol, Ep in ep h r in e: 1%–2% is equivalent to Propine 0.1%; three
carteolol, propranolol, m etoprolol, atenolol, nadolol, salt form s: hydrochloride, borate, bitartrate (first two are
pindolol equivalent, 2% bitartrate is equivalent to 1% hydrochloride
or borate)
Mechanism: improves aqueous outflow, slightly reduces
aqueous production
OCULAR HYP OTENS IVE (GLAUCOMA) Adverse effects: allergy, CME in aphakia (reversible),
MEDICATIONS hypertension, tachycardia, arrhythm ias, adrenochrom e
(black) deposits in conjunctiva
β-Blo c ke rs Mio t ic s
Mech an ism : reduce aqueous production (inhibit Na +/K+ Mech an ism : increase aqueous outflow (contraction of
pum p) by decreasing cyclic adenosine m onophosphate ciliary m uscle opens trabecular m eshwork), decrease
production in ciliary epithelium ; around 20% IOP reduction uveoscleral outflow
Loss of effectiveness over tim e due to downregulation
of β-receptors (long-term drift) Pilo car p in e: only direct cholinergic agonist; peak action at
2 hours, 8-hour duration
Beware for treatm ent of angle closure: causes shallowing
Ad ver se effects: dry eye syndrom e (decreased corneal of AC and narrowing of angle, but m iosis pulls
sensitivity), bradycardia, heart block, bronchospasm , peripheral iris away from angle, balancing out the other
im potence, lethargy, depression, headache, rarely diarrhea effects
and hallucinations, alopecia, derm atitis; m ay m ask Adverse effects: headache, brow ache, accom m odative
hypoglycem ia spasm , increased range of accom m odation, m iosis
(dim m ing, reduction of vision), induced m yopia
(forward shift of lens–iris diaphragm ), pupillary block,
No n selective (β1 and β2 ): tim olol (Tim optic), levobunolol follicular conjunctivitis, derm atitis, nyctalopia, rarely
(Betagan), m etipranolol (Optipranolol), carteolol (Ocupress) retinal tear or even RD
10% do not show a therapeutic effect; inhibit lipoprotein Other effects: breakdown of blood–aqueous barrier,
lipase (break down chylom icrons and very-low-density reduction of uveoscleral outflow
lipoprotein (VLDL)) and cholesterol acyltransferase
(incorporate cholesterol into high-density lipoprotein Car b ach o l: direct and indirect cholinergic agonist; stronger
(HDL)); m ay cause decreased serum HDL levels (except effect and longer duration of action; poor corneal penetration;
carteolol) needs corneal surface disrupter (benzalkonium chloride);
Carteolol has intrinsic sym pathom im etic activity intraocular form ulation (Miostat) used during surgery for
pupillary m iosis

Car d io selective (β1 ) β2 ): betaxolol (Betoptic) Ech o th io p h ate (phospholine iodide): indirect cholinergic
Fewer pulm onary adverse effects agonist (cholinesterase inhibitor); 3-week duration; also used
Indications: patients with pulm onary problem s who in accom m odative esotropia
cannot tolerate nonselective β-blockers; often used for Adverse effects: greater orbicularis, ciliary, and iris m uscle
norm al tension glaucom a; m ay not cause as m uch spasm ; cataracts in adults (therefore, use only in aphakic
vasoconstriction of vessels supplying ON or pseudophakic patients); iris cysts in children

24
 Antiinflam m atory Drugs

(proliferation of iris pigm ent epithelium ; phenylephrine Man n ito l (Osm itrol; IV; 20% solution): m ost potent; m ay
prevents cyst form ation); decreased serum exacerbate congestive heart failure
pseudocholinesterase activity (can accentuate
succinylcholine effects during general anesthesia) Glycer in (Osm oglyn; PO; 50% solution): m ay cause
hyperglycem ia in diabetics (m etabolized by liver into glucose)
Ca rb o n ic a n h yd ra s e in h ib it o rs (CAIs )
Mech an ism : decrease bicarbonate form ation in ciliary body Iso so r b id e (Ism otic; PO): not m etabolized (can be used in
epithelium ; bicarbonate form ation is linked to Na + and fluid diabetics); secreted 95% unchanged in urine
transport, so CAIs reduce aqueous production
Oth er h yp er o sm o tic agen ts: used topically for corneal
Car b o n ic an h yd r ase catalyzes th e edem a
r eactio n : CO 2 + H 2 O $ H 2 CO 3 Glycerin (Ophthalgan; topical; 100% solution): used to
Am ount of carbonic anhydrase is 100 Â that needed for clear corneal edem a for exam ination or laser procedure
aqueous production; so > 99% m ust be inhibited to achieve Muro 128 (hypertonic saline; topical; drops or ointm ent;
IOP decrease 2.5% or 5% strength): used to reduce epithelial edem a of
Sulfonam ide derivative (do not adm inister to patients with cornea, especially in treatm ent of recurrent erosions
sulfa allergy)
Co m b in a t io n s
Or al: acetazolam ide (Diam ox; PO/IV), m ethazolam ide
(Neptazane, PO; m ore lipid soluble, less toxicity) Br im o n id in e-tim o lo l (Com bigan)
Adverse effects: dry eye syndrome (decreased tear production),
metabolic acidosis (IV administration causes greater Br in zo lam id e-b r im o n id in e (Sim brinza)
metabolic acidosis), kidney stones (reduced excretion of
urinary citrate or magnesium), hypokalemia (especially Do r zo lam id e-tim o lo l (Cosopt)
when used with other diuretics; very dangerous with
digoxin), paresthesias (hands/feet /lips), GI upset, diarrhea, Ord e r o f a lle r g y
lethargy, loss of libido, weight loss, metallic taste, aplastic
anemia, Stevens-Johnson syndrome; transient myopia Gr eatest to least: iopidine > epinephrine > dipivefrin
brim onidine > β-blocker > pilocarpine
To p ical: dorzolam ide (Trusopt), brinzolam ide (Azopt)
Adverse effects: metallictaste, paresthesias, malaise, weight loss,
depression, skin rash, corneal endothelial decompensation/ ANTIINFLAMMATORY DRUGS
toxicity (consider stopping prior to cataract surgery)
In fla m m a t o ry p a t h w a y (Fig. 2-1)
P ro s t a g la n d in a n a lo g u e s / p ro s t a n o id s
Mech an ism : increase uveoscleral outflow, around 30% IOP
reduction
P hos pholipids
Latan o p r o st (Xalatan), b im ato p r o st (Lum igan), (ce ll me mbra ne s )
tr avopr ost (Travatan), taflup ro st (Zioptan), un op ro sto n e
P hos pholipa s e A2
iso pr op yl (Rescula) : prostaglandin F2α analogues (inhibite d by s te roids )
Adverse effects: flulike sym ptom s, hyperem ia, eyelash
growth, periocular skin and iris pigmentation (increases Ara chidonic a cid
num ber of m elanosom es, but not melanocytes),
prostaglandin associated periorbitopathy (upper lid Cyclooxyge na s e
ptosis, deepening of upper lid sulcus, involution of 5-Lipoxyge na s e
(inhibite d by NS AIDs )
derm atochalasis, periorbital fat atrophy, m ild
enophthalm os, inferior scleral show, increased Cyclic e ndope roxide s Hydrope roxide s
prom inence of lid vessels, tight eyelids), CME, (P GG 2 , P GH2 ) (5-HPETE, Le ukotrie ne s
reactivation of herpes sim plex virus (HSV) keratitis (C, D, a nd E))

Hyp e ro s m o t ic a g e n t s
Mech an ism : low-m olecular-weight substances that increase Thromboxa ne P ros ta cyclin
serum osm olality to draw fluid out of eye (reduces vitreous (TBX A2 ) (P GI2 )
volum e)
Adverse effects: headache, thirst, nausea, vom iting, P GE 2
diarrhea, diuresis, dizziness, and rebound IOP elevation; P GF 2 a
IV agents can cause subarachnoid hem orrhage P GD2
Ur ea (IV; 30% solution): not com m only used; extravasation Fig ure 2-1. The inflammatory pathway.NSAIDs, nonsteroidal anti-inflammatory
causes tissue necrosis drugs; PG, prostaglandin; 5-HPETE, 5-hydroperoxyeicosate traenoic acid.

25
CHAPTER 2 • Pharm acology

No n s t e ro id a l a n t i-in fla m m a t o ry d ru g s (NS AIDs ) Ta b le 2-3. Common ophthalmic steroids

Mech an ism : inhibit cyclooxygenase pathway Ge ne ric na m e Tra d e na m e
Classes: Top ic a l
Salicylates: acetylsalicylic acid (ASA), diflunisal, Prednisolone acetate 1% Pred Forte, Omni Pred
salicylam ide Fluorometholone acetate 0.1% Flarex
Acetic acids: indom ethacin (indom etacin), diclofenac Dexamethasone alcohol 0.1% Maxidex
(Voltaren), sulindac, etodolac, ketorolac (Acular, Fluorometholone alcohol 0.1% FML
Toradol), nepafenac (Nevanac, Ilevro), brom fenac Prednisolone phosphate 1% Inflamase
(Xibrom , Brom day, Prolensa)
Dexamethasone phosphate 1% Decadron
Phenylalkanoic acids: ibuprofen, suprofen (Profenal),
Difluprednate 0.05% Durezol
flurbiprofen (Ocufen), naproxen, fenoprofen,
Loteprednol etabonate 0.5% Lotemax
ketoprofen
Loteprednol etabonate 0.2% Alrex
Cyclooxygenase-2 inhibitors: celecoxib (Celebrex)
Sub c onjunc tiva l
In d icatio n s: Dexamethasone phosphate Decadron
Prevent miosis during intraocular surgery: Profenal, Ocufen Methylprednisolone acetate Solumedrol, Depomedrol
Allergic conjunctivitis, corneal pain, postsurgical Prednisolone acetate Durapred
inflammation, CME: Voltaren, Acular, Nevanac, Ilevro, Triamcinolone acetonide Kenalog
Xibrom , Brom day, Prolensa Betamethasone Celestone
Scleritis, uveitis: oral agents Intra vitre a l
Triamcinolone acetonide Kenalog, Triesence
S t e r o id s
Mech an ism : anti-inflam m atory and im m unosuppressive by Po ten cy: increased by 1 to 2 double bond(s), 9 fluorination,
inhibiting release of arachidonic acid, inhibiting release of 6 m ethylation, O at C11; IOP elevation from deoxygenation at
lysosom al enzym es, preventing m acrophage m igration, C21 (Table 2-2)
interfering with lym phocyte function, decreasing fibroblast Derivatives of progesterone (weaker): FML, m edrysone
activity, inhibiting neovascularization, and reducing capillary
perm eability Su b co n ju n ctival/su b -Ten o n ’s in jectio n (Table 2-3):
produces higher ocular concentration and longer duration;
Classes: beware in IOP responder
Ester: loteprednol
Ketone: all others Oth er r o u tes o f ad m in istr atio n : oral, IV, intraocular
Oral dose of 7.5 m g dexam ethasone results in intravitreal
Pr ep ar atio n s ( keto n es) : concentration of therapeutic levels
Phosphate: hydrophilic; poor penetration of intact corneal
epithelium (im proved penetration with epithelial defect) In d icatio n s: conjunctivitis, keratitis, scleritis, uveitis,
Alcohol: biphasic; penetrate intact cornea hyphem a, CME, m acular edem a, CNV, endophthalm itis
Acetate: m ore biphasic; best corneal penetration
Ad ver se effects:
Systemic: adrenal insufficiency, hyperglycemia,
hypertension, hypokalemia, peptic ulcers, delayed wound
Ta b le 2-2. Relative anti-inflammatory activity of steroids
healing, superinfection, emotional lability, psychosis,
Re la tive a nti- insomnia, growth retardation, m uscle atrophy,
Ste ro id infla m m a to ry a c tivity osteoporosis, aseptic necrosis of the hip, hirsutism, weight
Hydrocortisone 1 (the standard) gain, cushingoid appearance, pseudotumor cerebri
Cortisone 0.8 Ocular: posterior subcapsular cataract (PSC), cataracts,
Triamcinolone 4
elevated IOP, delayed wound healing/corneal re-
Medrysone 4
epithelialization, secondary infections (e.g., HSV, fungal)
Prednisone 4
Prednisolone 5 IOP-elevatin g p o ten tial: difluprednate >
Dexamethasone 25 dexam ethasone > prednisolone > fluorom etholone >
Betamethasone 25
hydrocortisone > tetrahydrotriam cinolone > m edrysone
(after 6 weeks of dexam ethasone therapy, 42% have
Rimexolone 25
IOP > 20 m m Hg; 6% have IOP > 31)
Loteprednol 25
Fluorometholone 40
Difluprednate 60
Ster o id s with less IOP-elevatin g
Fluocinolone 240
p o ten tial: rim exolone (Vexol), loteprednol
(Lotem ax, Alrex)

26
 Im m unosuppressive Agents

Cyt o t o xic a lkyla t in g a g e n t s

ANTIALLERGY MEDICATIONS
Create cross-linkage between DNA strands, resulting in
inhibition of transcription of m RNA and prevention of DNA
An t ih is t a m in e s / va s o c o n s t ric t o rs
synthesis
(o ve r-t h e -c o u n t e r )
Nap h azo lin e h yd r o ch lo r id e/p h en ir am in e
m aleate: (OcuHist, Opcon-A, Naphcon-A), n aph azolin e Ch lo r am b u cil (Leukeran): interacts with 7-guanine of
h ydroch loride / an tazolin e ph osph ate (Vasocon-A): DNA, resulting in strand breakage or cross-linkage
vasoconstrictor naphazoline tem porarily rem oves redness, but Adverse effects: sterility, bone m arrow suppression
can cause rebound redness with chronic use
Cyclo p h o sp h am id e (Cytoxan):
Ma s t c e ll s t a b iliz e r Adverse effects: hem orrhagic cystitis (oral > IV), renal
transitional cell cancer, sterility, bone m arrow
Cr o m o lyn (crom oglicate; Crolom ; Opticrom ): reduces suppression
perm eability of m ast cell plasm a m em brane, preventing release Prevent hem orrhagic cystitis with high water intake
of histam ine; reduces phosphodiesterase activity (facilitator of (IV or oral)
m ast cell degranulation); inhibits activation of neutrophils,
m onocytes, and eosinophils; does not interfere with binding of
antigen to previously sensitized cells; no antihistam ine activity Cyt o s t a t ic a n t iin fla m m a t o rie s
therefore useful for chronic allergies, not for acute
Ster o id s:
sym ptom atic relief
(see previous page)

Ma s t c e ll s t a b iliz e r + e o s in o p h il s u p p re s s o r
Im m u n o m o d u la t o r
Lo d o xam id e (Alom ide): 2500 tim es m ore potent than
crom olyn; also inhibits eosinophil activation Cyclo sp o r in e: blocks production of interleukin-2 (IL-2)
and IL-2 receptors, inhibits proliferation of lym phocytes,
inhibits T-cell activation and recruitm ent, interferes with
H1 -b lo c ke rs (An t ih is t a m in e s )
production of IL-2 by T cells, prevents form ation of IL-2
Levo cab astin e (Livostin), em ed astin e (Em adine): pure receptors by T cells; natural product of fungi
H 1 -specific receptor antagonists; bind to histam ine receptors Indications:
(inhibit itching and hyperem ia) TOPICAL: necrotizing scleritis, Sjo€ gren’s syndrom e/dry
eye disease, ligneous conjunctivitis, atopic
H1 -b lo c ke rs + m a s t c e ll s t a b ilize rs keratoconjunctivitis
SYSTEMIC: Mooren’s ulcer, uveitis in Behçet’s or
Ned o cr o m il (Alocril) , p em ir o last (Alam ast) , keto tifen sym pathetic ophthalm ia, prevention of corneal
(Zaditor, Alaway), o lo p atad in e (Patanol, Pataday, Pazeo), transplant rejection; also used in ocular cicatricial
azelastin e (Optivar), ep in astin e (Elestat), b ep o tastin e pem phigoid (OCP) and thyroid-related
(Bepreve), alcaftadine (Lastacaft): stabilize m ast cells and ophthalm opathy
binds to H 1 receptors (inhibits itching); binds to H 2 receptors Adverse effects (system ic adm inistration only): renal
at low level (inhibits hyperem ia) toxicity (renal tubular atrophy, interstitial fibrosis),
hypertension, paresthesia, peripheral neuropathy,
elevated erythrocyte sedim entation rate (ESR),
hypertrichosis, hepatotoxicity, hyperuricem ia
IMMUNOSUP P RESSIVE AGENTS
Lifitegr ast ( Xiid r a) : sm all m olecule integrin antagonist,
Cyt o t o xic a n t im e t a b o lit e s inhibits T-cell m ediated inflam m ation (blocks binding of
Inhibit purine-ring biosynthesis lym phocyte function-associated antigen-1 (LFA-1; T-cell
surface receptor) to intercellular adhesion m olecule-1
(ICAM-1; expressed in cornea and conjunctiva)
Meth o tr exate: folate analogue; inhibits folate m etabolism ;
Indications: topical for dry eye disease
inhibits synthesis of deoxythym idine monophosphate
nucleotide; inhibits enzyme dehydrofolate reductase; inhibits
T-cell function Ad alim u m ab ( Hu m ir a) : anti-TNF-alpha agent.
Adverse effects: leukopenia, throm bocytopenia, Indications: subcutaneous for noninfectious interm ediate,
hepatotoxicity, lung or renal toxicity; teratogenic; posterior, and panuveitis in adults
periorbital edem a, hyperem ia Adverse effects: increased risk tuberculosis and
opportunistic infections, lym phom a, anaphylaxis, hepatitis B
Azath io p r in e (Im uran): purine analogue that inhibits reactivation, dem yelinating disease, cytopenia, heart failure,
purine synthesis psoriasis, and lupus-like syndrom e

27
CHAPTER 2 • Pharm acology

Ot h e rs Pen icillin s ( PCN) : least toxic; variable protein binding

Adverse effects: allergy, diarrhea
Co lch icin e: inhibits leukocyte m igration; prevents
Penicillin G:
recurrence of Behçet’s
SPECTRUM: Streptococcus and non–penicillinase-
producing Staphylococcus, Neisseria, Treponema pallidum
On co lytic agen ts: INDICATIONS: syphilis
Antineoplastics: bleom ycin, dactinom ycin, m itom ycin Isoxazolyl PCNs: penicillinase-resistant; m ethicillin,
(topically in glaucom a, corneal and pterygium surgery), nafcillin, oxacillin, cloxacillin, dicloxacillin
daunorubicin (daunom ycin), doxorubicin SPECTRUM: extended to Staphylococcus aureus
(hydroxydaunorubicin, Adriam ycin) Amino PCNs: broader spectrum ; am picillin, am oxicillin
ADVERSE EFFECTS: anem ia, Stevens-Johnson syndrom e SPECTRUM: Gram -positives (except Staph), Neisseria,
(SJS), decreased vision, conjunctivitis, tearing Haemophilus, Proteus, Shigella, Salmonella, Listeria,
Alkylating agents: busulfan, carm ustine, chloram bucil, E. coli
cyclophospham ide INDICATIONS: preseptal cellulitis
ADVERSE EFFECTS: rare Carboxy PCNs: antipseudom onal; carbenicillin, ticarcillin
Vinca alkaloids: vincristine, vinblastine SPECTRUM: extended to Gram -negatives (Pseudomonas
ADVERSE EFFECTS: diplopia, nystagm us, ptosis, CN and Enterobacteriaceae) and anaerobes (Bacteroides)
palsies Ureido PCNs: antipseudom onal; piperacillin, m ezlocillin,
Antiestrogen: tam oxifen azlocillin
ADVERSE EFFECTS: corneal opacities, retinopathy SPECTRUM: extended to Gram -negatives (Pseudomonas
Heavy metals: cisplatin and Enterobacteriaceae) and anaerobes (Bacteroides)
ADVERSE EFFECTS: papilledem a, optic neuritis β-Lactamase Inhibitors: com bined with PCNs to increase
Antimetabolites: m ethotrexate, 5-fluorouracil (5-FU; spectrum of activity; clavulanate
topically in glaucom a surgery) (am ox/ clavulanate ¼ Augm entin), sulbactam
ADVERSE EFFECTS: periorbital edem a, conjunctival (am p/sulbactam ¼ Unasyn)
hyperem ia, photophobia, tearing; corneal epithelial
erosions
Cep h alo sp o r in s: m ore resistant to β-lactam ases; 20%
cross-react with PCN-allergic patients
First generation: cefazolin (Ancef, Kefzol), cefalexin
ANTI-INFECTIVE DRUGS (cephalexin, Keflex), cephalothin (Keflin), cefaclor
(Ceclor)
An t ib io t ic s SPECTRUM: Gram -positives (Staphylococcus,
Streptococcus) and som e Gram -negatives
In h ib it o rs o f In t e rm e d ia ry Me t a b o lis m INDICATIONS: cefazolin for keratitis, endophthalm itis
Second generation: cefam andole (cepham andole, Mandol),
Su lfo n am id es: static; sulfacetam ide, sulfadiazine, cefonicid (Monocid), cefotetan (Cefotan), cefoxitin
sulfisoxizole, sulfam ethoxazole; resistance is problem (Mefoxin), cefuroxim e (Zinacef)
Mechanism: inhibit folic acid synthesis SPECTRUM: better Gram -negative but less Gram -
Spectrum: Gram-positives and Gram -negatives; also, positive, anaerobic (cefoxitin, cefotetan), Haemophilus
toxoplasm osis, Chlamydia, Actinomyces, Pneumocystis influenzae, and Neisseria (cefuroxim e)
Indications: blepharitis, conjunctivitis, toxoplasm osis Third generation: cefoperazone (Cefobid), cefotaxime
Adverse effects: allergy (SJS syndrom e), transient m yopia (Claforan), ceftazidime (Tazidime), ceftizoxime
Pyrimethamine (Daraprim ): (Cefizox), ceftriaxone (Rocephin), moxalactam (Moxam)
ADVERSE EFFECTS: bone m arrow depression (anem ia, SPECTRUM: even better Gram -negative, Pseudomonas
throm bocytopenia; prevent with use of folinic acid (ceftazidim e)
[Leucovorin])
Dapsone:
INDICATIONS: OCP, leprosy Mo n o b actam s (aztreonam ): aerobic Gram -negatives
CONTRAINDICATIONS: patients allergic to sulfa or with
G6PD deficiency Car b ap en em s (im ipenem ): β-lactam ase resistant; very
broad activity (almost all Gram -positives, Gram -negatives, and
Tr im eth o p r im : blocks next step in folate m etabolism ; often anaerobes); does not cover m ethicillin-resistant
com bined with sulfam ethoxazole (Bactrim ) S. aureus (MRSA)
Spectrum: Gram-positives and Gram -negatives Non β-Lactams (cidal)
(Staphylococcus, Streptococcus, Serratia, Proteus,
Haemophilus, Enterobacter, Escherichia coli, Klebsiella)
Po lym yxin B: basic peptides; act as detergents and disrupt
cell m em brane
Indications: conjunctivitis
Spectrum: Gram -negatives (Haemophilus, Enterobacter,
E. coli, Klebsiella, Pseudomonas)
In h ib it o rs o f Ce ll Wa ll S yn t h e s is
Indications: conjunctivitis (com bined with trim ethoprim
β-Lactams (cidal) [Polytrim ])

28
 Anti-Infective Drugs

Bacitr acin : polypeptides; often used in com bination with Ch lo r am p h en ico l: static; inhibits 50S ribosom e
neom ycin or polym yxin to broaden activity Spectrum: Gram -positives and Gram -negatives, anaerobes,
Spectrum: Gram -positives, Neisseria, Haemophilus, Chlamydia, Rickettsia, Mycoplasma, spirochetes
Actinomyces Adverse effects: aplastic anem ia and reversible bone m arrow
Indications: blepharitis, conjunctivitis suppression
Clin d am ycin : static; inhibits 50S ribosom e
Van co m ycin : glycopeptide Spectrum: Gram -positives and anaerobes, toxoplasm osis
Spectrum: very good for Gram -positives; Staphylococcus Indications: toxoplasm osis
(including MRSA), Streptococcus (including PCN- Adverse effects: m ay cause pseudom em branous colitis (due
resistant strains), Bacillus, Propionibacterium acnes, to overgrowth of C. difficile; treat with oral vancom ycin or
Clostridium difficile m etronidazole [Flagyl])
Indications: keratitis, endophthalm itis
Adverse effects: system ic adm inistration is associated with Ot h e rs
ototoxicity and nephrotoxicity Flu o r o qu in o lo n es: cidal; analogues of nalidixic acid;
inhibitors of genetic replication; inhibit DNA gyrase
In h ib it o rs o f P ro t e in S yn t h e s is
(topoisom erase II) and topoisom erase IV; low resistance
second generation: ciprofloxacin (Ciloxan), ofloxacin
Am in o glyco sid es: cidal; inhibit 30S ribosom e; poor GI
(Ocuflox), norfloxacin (Chibroxin)
absorption
third generation: levofloxacin (Quixin, Iquix)
Gentam ycin ([better for Serratia]), tobram ycin (Tobrax
fourth generation: gatifloxacin (Zym ar, Zym axid),
[better for Pseudomonas]), am ikacin (best for Pseudomonas
m oxifloxacin (Vigam ox), besifloxacin (Besivance)
and Mycobacterium; less nephrotoxic than gent),
Spectrum: aerobic Gram -negatives and som e Gram -
streptom ycin (tuberculosis (TB), Streptococcus viridans),
positives (Hemophilus influenzae, Pseudomonas,
neom ycin (Acanthamoeba; allergy com m on),
Enterobacteriaceae, S. aureus); also, Chlamydia,
parom om ycin (Acanthamoeba), kanam ycin
Rickettsia, Mycoplasma, and Mycobacterium. Fourth-
Spectrum: Gram -negative bacilli and som e Staphylococcus
generation agents have extended spectrum with
(gentam ycin and tobram ycin are active against S. aureus
enhanced activity against Gram -positives,
and Staphylococcus epidermidis)
fluoroquinolone-resistant organism s, and atypical
Indications: conjunctivitis, keratitis, endophthalm itis
m ycobacteria
Adverse effects: system ic adm inistration is associated with
Indications: conjunctivitis, keratitis, surgical prophylaxis;
ototoxicity and nephrotoxicity, allergy
prophylaxis in penetrating traum a (oral cipro achieves
high levels in vitreous)
Sp ectin o m ycin : cidal; inhibits 30S ribosom e; not an Adverse effects: GI upset; cartilage dam age in children
am inoglycoside; used for Neisseria
An t i-TB a g e n t s
Tetr acyclin es: static; inh ibit 30S ribosom e; take on
em pty stom ach (ch elates calcium , an tacids; iron , causin g Iso n iazid : cidal; inhibits cell wall synthesis of
decreased absorption ); use with caution in wom en of m ycobacteria
ch ildbearing age; also decreases efficacy of oral con traceptive Adverse effects: hepatotoxicity, vitam in B6 deficiency
m edication s
Tetracycline, doxycycline, m inocycline, m eclocycline Rifam p in : inhibits RNA polym erase of m ycobacteria
Spectrum: Gram -positive and Gram -negative, Chlamydia, Adverse effects: hepatotoxicity, turns body fluids orange-red
Rickettsia, Mycoplasma
Indications: prophylaxis and treatm ent of ophthalm ia Pyr azin am id e: unknown m echanism ; analogue of
neonatorum , Chlamydia; also rosacea, m eibom ianitis, nicotinam ide
scleral m elting (due to anti-inflam m atory and Adverse effects: hepatotoxicity, gout
anticollagenolytic properties)
Adverse effects: GI upset, phototoxic derm atitis, tooth Eth am b u to l: chelates m etals
discoloration in children younger than 8 years of age, Adverse effects: optic neuropathy
teratogenic, nephrotoxicity and hepatotoxicity,
decreased prothrom bin activity (potentiates warfarin Fu m agillin : treatm ent of m icrosporidia keratoconjunctivitis
sodium [Coum adin])
An t ivira ls
Macr o lid es: static; inhibit 50S ribosom e
Erythrom ycin, azithrom ycin (Zithrom ax), clarithrom ycin Mech an ism : static; inhibit genetic replication; m ost are
(Crixan, Biaxin) nucleotide analogues
Spectrum: Gram -positives and a few Gram -negatives,
Chlamydia, Mycoplasma, Legionella To p ical: treatm ent of HSV keratitis
Indications: blepharitis, conjunctivitis, Chlamydia Idoxuridine (IDU, Stoxil): can cause follicular
Adverse effects: GI upset conjunctivitis, corneal epitheliopathy, punctal stenosis

29
CHAPTER 2 • Pharm acology

Vidarabine (Vira-A): adverse effects less severe than IDU Imidazoles:

Trifluorothymidine (Viroptic): inhibits thym idylate MICONAZOLE (topical, iv, intravitreal): broad spectrum
synthetase (virus-specific enzym e) (filam entous fungi and yeast)
Ganciclovir (Zirgan): guanosine analogue; activated only ADVERSE EFFECTS: m ay cause corneal erosions, anem ia
by thym idine kinase (virus-specific enzym e) and KETOCONAZOLE (topical, oral): broad spectrum
selectively interferes with viral DNA replication. Sim ilar ADVERSE EFFECTS: reversible hepatotoxicity
efficacy as topical acyclovir (not available in US); m ore CLOTRIMAZOLE (oral): good for Aspergillus
effective than Viroptic, and less toxic to cornea; best ADVERSE EFFECTS: hepatotoxicity
tolerated Triazoles: safer
ITRACONAZOLE (oral): broad spectrum
System ic: FLUCONAZOLE (oral): broad spectrum
Acyclovir (acycloguanosine, Zovirax; guanosine
analogue; activated only by thym idine kinase (virus- An tim etab o lites: m ost fungi are resistant, except
specific enzym e)), valacyclovir (Valtrex; prodrug of Cryptococcus and som e Candida
acyclovir), pen ciclovir, fam ciclovir (Fam vir; prodrug of Flucytosine: converted to 5-FU, disrupts DNA synthesis
peniclovir) ADVERSE EFFECTS: m yelosuppression, nausea,
INDICATIONS: treatm ent of HSV and varicella zoster vom iting, diarrhea
virus (VZV)
ADVERSE EFFECTS: m ay cause GI upset; high doses can An t ia m o e b ic s
cause nephrotoxicity and neurotoxicity
Ganciclovir (Cytovene): treatm ent of CMV Mech an ism : cidal (am oebae and cysts); cationic
ADVERSE EFFECTS: bone m arrow suppression (cannot surface-active properties; interfere with cell m em branes and
use with azidothym idine [AZT]) inhibit enzym es
Foscamet (Foscavir): treatm ent of CMV
ADVERSE EFFECTS: nephrotoxicity, less
In d icatio n s: topical for Acanthamoeba keratitis
m yelosuppression, electrolyte abnorm alities Bigu an id es: polyhexam ethylene biguanide (PHMB),
chlorhexidine; first-line agents, less corneal toxicity
An t ifu n g a ls
Diam id in es: propam idine (Brolene), hexam idine;
Mech an ism : disrupt cell m em branes
synergistic effect with biguanides, corneal toxicity

Classificatio n :
An t ih e lm in t ic s
Yeasts: form pseudohyphae; Candida, Cryptococcus
Molds: filam entous; form hyphae Meb en d azo le, th iab en d azo le, alb en d azo le: inhibit
SEPTATE: Fusarium, Aspergillus, Penicillium, Curvularia, glucose uptake and m icrotubule synthesis
Paecilomyces, Phialophora Adverse effects: GI upset
NONSEPTATE: Phycomycetes, Rhizopus, Mucor
Dimorphic fungi: grow as yeast or m old; Histoplasma, Pyr an tel p am o ate: neurom uscular junction blocker
Blastomyces, Coccidioides Adverse effects: nausea, vom iting, headache, rash

Dieth ylcar b am azin e: enables phagocytosis of m icrofilaria

Po lyen es: bind to ergosterol; dam ages fungal m em branes
Amphotericin B: system ic Iver m ectin : increases gam m a-am inobutyric acid (GABA)
SPECTRUM: broad (especially Candida; also, release paralyzing m icrofilaria
Cryptococcus, Blastomyces, Histoplasma, Coccidioides, Adverse effects: fever, headache, rash
m ucorm ycosis; not as good for Aspergillus, Fusarium)
INDICATIONS: keratitis, endopthalm itis (intravenous Pr aziqu an tel: causes calcium loss, paralyzing worm
drug abuse [IVDA], im m unosuppression, Adverse effects: GI upset, fever
hyperalim entation)
ADVERSE EFFECTS: fever, hypotension, headache,
phlebitis, GI upset, nephrotoxicity, anem ia MISCELLANEOUS
Natamycin (pim aricin): topical only; too toxic for IV use,
toxic to retina intravitreally Am in o cap r o ic acid (Am icar): synthetic am ino acid sim ilar
SPECTRUM: filam entous fungi (especially Aspergillus, to lysine
Fusarium), not as good for Candida; not effective Mechanism: antifibrinolytic; stabilizes blood clot, delays
against Mucor (nonseptate, branching hyphae) lysis, decreases secondary hem orrhages
INDICATIONS: keratitis (rem oval of epithelium Indications: hyphem a
im proves penetration) Contraindications: hypercoagulable states, pregnancy,
renal disease, liver disease, patients at risk for m yocardial
Azo les: inhibit ergosterol synthesis; second-line agents to infarction (MI), pulm onary em bolism , cerebrovascular
am photericin; also used for Acanthamoeba accident (CVA)
Adverse effects: GI upset, headache, rash Adverse effects: nausea, vomiting, diarrhea, hypotension, rash

30
 Ocular Toxicology

Bo tu lin u m to xin (Botox): neurotoxin that blocks angiography [IVFA]: pruned tree appearance of retinal
release of acetylcholine from nerve term inal; paralyzes m uscle vasculature)
(1–3 m onths) Chloramphenicol: optic neuropathy; peripheral neuritis can
Indications: blepharospasm , hem ifacial spasm , strabism us precede visual com plaints by 1 to 2 weeks
Adverse effects: ptosis, diplopia, exposure keratopathy Penicillin and tetracycline: pseudotum or
Sulfonamides: conjunctivitis, transient m yopia, angle
Flu o r escein d ye: IV for fluorescein angiography closure, optic neuropathy
Adverse effects: nausea, vom iting, dizziness, headache, Isoniazid, rifampin, ethambutol: optic neuropathy
dyspnea, hypotension, skin necrosis, phototoxic
reactions, anaphylaxis An tim alar ials (chloroquine / hydroxychloroquine): cornea
verticillata, fine pigmentary macular changes (bull’s-eye
In d o cyan in e gr een ( ICG) d ye: IV for ICG angiography; maculopathy); patients m ay complain of halos around lights;
contraindicated in patients allergic to iodine visual acuity usually unchanged; dose related
Adverse effects: GI upset, hypotension, urticaria, anaphylaxis
Qu in in e: overdose can result in acute visual loss (to no light
perception [NLP]), tinnitus, weakness, confusion
OCULAR TOXICOLOGY (TABLE 2-4)
Bar b itu r ates (phenobarbital): nystagm us, diplopia, ptosis,
conjunctivitis
An tich o lin er gics (atropine, scopolam ine, donnatal):
toxicity causes flushing, agitation, tachycardia, som nolence,
Ph en o th iazin es (chlorprom azine, thioridazine):
dry m outh, dry eye, m ydriasis, cycloplegia, blurry vision,
pigm entary retinopathy, corneal deposits, cataracts, angle
angle closure; increased sensitivity in albinism , Down
closure
syndrom e, and neonates
Tr icyclic an tid ep r essan ts: m ydriasis, cycloplegia, dry eye,
An tih istam in es (diphenhydram ine): dry eye, m ydriasis, angle closure
cycloplegia, blurry vision, angle closure
Dilan tin : diplopia, nystagm us, papilledem a
An tib io tics:
Aminoglycosides: intraocular adm inistration m ay cause Go ld : deposits in inferior corneal strom a and anterior lens
m acular infarction (intravenous fluorescein capsule (chrysiasis)

Ta b le 2-4. Ocular toxicology

Oc ula r s truc ture Effe c t Drug

Extraocular muscles Nystagmus, diplopia Anesthetics, sedatives, anticonvulsants, propranolol, antibiotics, phenothiazines, pentobarbital,
carbamazepine, monoamine oxidase inhibitors
Lid Edema Chloral hydrate
Discoloration Phenothiazines
Ptosis Guanethidine, propranolol, barbiturates
Conjunctiva Hyperemia Reserpine, methyldopa
Allergy Antibiotics, sulfonamides, atropine, antivirals, glaucoma medications
Discoloration Phenothiazines, chlorambucil, phenylbutazone
Cornea Keratitis Antibiotics, phenylbutazone, barbiturates, chlorambucil, steroids
Deposits Chloroquine, amiodarone, tamoxifen, indomethacin, ibuprofen, naproxen, clofazimine, s uramin,
phenothiazines, gold
Pigmentation Vitamin D
Increased IOP Open angle Anticholinergics, caffeine, steroids, anti-VEGF agents (ranibizumab, bevacizumab, aflibercept)
Narrow angle Anticholinergics, antihistamines, phenothiazines, tricyclic antidepressants, haloperidol, sulfonamides
(Topamax)
Lens Opacities / cataract Steroids, phenothiazines, ibuprofen, allopurinol, long-acting miotics, busulfan, tamoxifen
Myopia Sulfonamides, tetracycline, prochlorperazine, autonomic antagonists, duloxetine (Cymbalta)
Retina Edema Chloramphenicol, indomethacin, tamoxifen, carmustine
Hemorrhage Anticoagulants, ethambutol
Vascular damage Oral contraceptives, oxygen, aminoglycosides, talc, carmustine, interferon
Pigmentary Phenothiazines, indomethacin, nalidixic acid, ethambutol, isotretinoin, chloroquine, hydroxychloroquine
degeneration
Optic nerve Neuropathy Ethambutol, isoniazid, rifampin, sulfonamides, s treptomycin, chloramphenicol, chloroquine, quinine, digitalis,
imipramine, busulfan, cisplatin, vincristine, disulfiram, linezolid, cyclosporine
Papilledema Steroids, vitamin A, tetracycline, phenylbutazone, amiodarone, nalidixic acid, isotretinoin, lithium

31
CHAPTER 2 • Pharm acology

Talc: m ultiple tiny yellow-white glistening particles 3. Which of the following is not an adverse effect of CAIs?
scattered through posterior pole with m acular a. death
edem a, venous engorgem ent, hem orrhages, arterial b. paresthesias
occlusion, retinal nonperfusion, and peripheral c. iris cysts
neovascularization (NV) d. Stevens-Johnson syndrom e
4. Which β-blocker has the least effect on β2 receptors?
Am io d ar o n e (Cordarone): cornea verticillata, occasionally a. levobunolol
anterior subcapsular opacities b. betaxolol
c. carteolol
Digo xin : changes in color vision (xanthopsia [yellow
d. tim olol
vision]), optic neuropathy
5. Which drug has the least effect on uveoscleral outflow?
Diu r etics (hydrochlorothiazide): xanthopsia, transient a. atropine
m yopia, angle closure b. latanoprost
c. pilocarpine
d. dorzolam ide
Ph o sp h o d iester ase 5 in h ib ito r s (Viagra, Cialis, 6. Which enzym e is inhibited by steroids?
Levitra): decreased retinal blood flow by up to À30%; a. cyclooxygenase
altered color and light perception; possibly ischem ic optic b. phospholipase A2
neuropathy c. lipoxygenase
d. endoperoxidase
Car m u stin e: retinal infarction, RPE changes, arterial 7. Which of the following steroid form ulations has the best
occlusions, hemorrhages, m acular edem a, glaucom a, optic corneal penetrability?
neuritis, INO a. prednisolone acetate
b. dexam ethasone phosphate
Nar co tics (opiates): m iosis c. prednisolone phosphate
d. dexam ethasone alcohol
NSAIDs (indom ethacin): corneal deposits, diplopia, optic 8. Adverse effects of foscarnet include all of the following
neuritis, pigm entary m acular changes; m ay have changes in except
vision, dark adaptation, and visual fields a. seizures
b. infertility
Co r tico ster o id s: posterior subcapsular cataracts, increased c. electrolyte abnorm alities
IOP, delayed wound healing, secondary infections, d. m yelosuppression
pseudotum or cerebri 9. Which glaucom a m edication is not effective when IOP is
> 60 m m Hg?
Or al co n tr acep tives: dry eye, vascular occlusions, a. acetazolam ide
perivasculitis, optic neuritis, pseudotum or cerebri b. tim olol
c. pilocarpine
Tam o xifen : deposits in cornea and m acula, posterior d. apraclonidine
subcapsular cataracts, m ay have m acular edem a 10. Which m edicine is not associated with OCP-like
conjunctival shrinkage?
Iso tr etin o in : im pairm ent of dark adaptation a. phospholine iodide
b. pilocarpine
In ter fer o n : reversible vaso-occlusive disease c. epinephrine
d. tim olol
11. Which β-blocker is β1 -selective?
a. carteolol
REVIEW QUESTIONS (Answers start on page 374) b. tim olol
c. betaxolol
1. Which antibiotic results in the highest intravitreal d. levobunolol
concentration when adm inistered orally? 12. The m ost appropriate treatm ent for neurosyphilis is
a. ciprofloxacin a. penicillin G
b. penicillin b. erythrom ycin
c. bactrim c. penicillin VK
d. clindam ycin d. tetracycline
2. Which anesthetic agent would m ost interfere with an 13. Th e correct m ech an ism of action of botulin um toxin
intraocular gas bubble? is
a. isoflurane a. it prevents release of acetylcholine
b. propofol b. it blocks acetylcholine receptors
c. sodium thiopental c. it inhibits reuptake of acetylcholine
d. nitrous oxide d. it is an acetylcholinesterase inhibitor

32
 Review Questions

14. Fluoroquinolones are least effective against 25. All of the following m edications are com bination
a. Klebsiella antihistam ine and m ast cell stabilizers except
b. H. influenzae a. Alom ide
c. anaerobic cocci b. Zaditor
d. Serratia c. Optivar
15. Hydroxychloroquine toxicity depends m ost on d. Patanol
a. patient age 26. The antidote for atropine toxicity is
b. cum ulative dose a. endrophonium
c. patient race b. physostigm ine
d. daily dose c. carbacholine
16. Calculate the am ount of cocaine in 2 m L of a 4% d. pilocarpine
solution 27. Which of the following agents is contraindicated for
a. 2 m g ruptured globe repair?
b. 8 m g a. gallam ine
c. 20 m g b. halothane
d. 80 m g c. pancuronium
17. NSAIDs block the form ation of all of the following d. succinylcholine
substances except 28. The duration of action of 1 drop of proparacaine is
a. throm boxane a. 5 m inutes
b. leukotrienes b. 20 m inutes
c. prostaglandins c. 45 m inutes
d. prostacyclin d. 1 hour
18. System ic effects of steroids m ay include all of the 29. Which of the following m edications is not com m ercially
following except available as a topical form ulation?
a. papilledem a a. ganciclovir
b. hirsutism b. azithrom ycin
c. potassium depletion c. cyclosporine
d. renal tubular acidosis d. vancom ycin
19. Which drug does not produce decreased tear 30. All of the following are com plications of CAIs except
production? a. hypokalem ia
a. pilocarpine b. aplastic anem ia
b. diphenhydram ine (Benadryl) c. m etabolic alkalosis
c. tim olol d. kidney stones
d. atropine 31. Topiram ate is associated with
20. Natam ycin is a a. open-angle glaucom a
a. diam ine b. norm al tension glaucom a
b. im idazole c. angle-closure glaucom a with pupillary block
c. polyene d. angle-closure glaucom a without pupillary block
d. am inoglycoside 32. A patient with ocular hypertension and an allergy to
21. Which glaucom a m edicine does not decrease aqueous sulfonam ides should not be treated with
production? a. bim atoprost
a. aproclonidine b. dorzolam ide
b. pilocarpine c. tim olol
c. acetazolam ide d. brim onidine
d. tim olol 33. Infectious keratitis due to Candida albicans is best treated
22. β-blockers m ay cause all of the following except with topical
a. constipation a. am photericin B
b. im potence b. natam ycin
c. alopecia c. fluconazole
d. depression d. clotrim azole
23. Idoxuridine m ay cause all of the following except 34. Which of the following oral agents should be used to treat
a. filam entary keratitis a patient with ocular cicatricial pem phigoid?
b. punctal stenosis a. pyrazinam ide
c. corneal hypesthesia b. 5-fluorouracil
d. nonhealing epithelial erosion c. cyclophospham ide
24. Which of the following antifungal agents has the broadest d. flucytosine
spectrum against yeast-like fungi? 35. The glaucom a m edication contraindicated in infants is
a. m iconazole a. tim olol
b. natam ycin b. brim onidine
c. ketoconazole c. latanoprost
d. am photericin d. dorzolam ide

33
CHAPTER 2 • Pharm acology

36. Which system ic antibiotic is used to treat Chlamydia during 43. Ocular rosacea is best treated with which of the following
pregnancy? oral m edications?
a. doxycycline a. acyclovir
b. ceftriaxone b. cyclosporine
c. penicillin c. doxycycline
d. erythrom ycin d. tacrolim us
37. The local anesthetic with the longest duration of 44. A patient taking which of the following m edications is at
action is increased risk for com plications at the tim e of cataract
a. m epivacaine surgery?
b. procaine a. beta-adrenergic antagonist
c. bupivacaine b. m uscarinic antagonist
d. lidocaine c. alpha-1 adrenergic antagonist
38. A 33-year-old m an has had follicular conjunctivitis with d. carbonic anhydrase inhibitor
a watery discharge for 5 weeks. Elem entary bodies are 45. Apatient suddenly stops breathing after administration of a
present on a conjunctival sm ear, therefore, the m ost peribulbar injection of anesthetic. The most likely reason is
appropriate treatm ent is a. retrobulbar hem orrhage
a. oral azithrom ycin b. globe perforation
b. oral acyclovir c. injection into the m edial rectus
c. topical crom olyn d. injection into the optic nerve sheath
d. topical prednisolone
39. The m ost appropriate treatm ent for Fusarium keratitis is
topical SUGGESTED READINGS
a. tobram ycin
b. pim aricin
Doughty, M. (2001). Ocular pharmacology and therapeutics: a primary
c. chloram phenicol
care guide. Philadelphia: Butterworth-Heinem ann.
d. ciprofloxacin Fraunfelder, F. T., Fraunfelder, F. W., & Cham bers, W. A. (2014).
40. All of the following are associated with vitam in A toxicity Drug-induced ocular side effects (ed 7). Philadelphia: Butterworth-
except Heinem ann.
a. CN 6 palsy Grant, W. M., & Schum an, J. S. (1993). Toxicology of the eye (ed 4).
b. retinal hem orrhages Springfield, IL: Charles C. Thom as.
c. papilledem a Greenbaum, S. (1997). Ocular anesthesia. Philadelphia: WB Saunders.
d. band keratopathy Physicians’ desk reference for ophthalmic medicines. Montvale, NJ, 2012,
41. Which of the following is a serious adverse effect of a long- Medical Econom ics.
Roy, F. H., & Fraunfelder, F. T. (2007). Roy and fraunfelder’s current
acting cylcoplegic agent?
ocular therapy (ed 6). Philadelphia: WB Saunders.
a. som nolence
Zim m erman, T. J. (1997). Textbook of ocular pharmacology. Philadelphia:
b. bradycardia Lippincott-Raven.
c. urinary retention
d. hypotherm ia
42. An oral NSAID should not be used in a patient with
a. renal insufficiency
b. diabetes m ellitus
c. sulfa allergy
d. hyperthyroidism

34
3 Em bryology /Pathology
EMBRYOLOGY
PATHOLOGY
Microbial Studies
Tissue Stains
Tissue Fixation
Hypersensitivity Reactions
Immunoglobulins
HLA System
Inflammation
Eyelid Epithelial Changes
Aging Changes
Wounds
Ocular Injuries

EMBRYOLOGY Em b r yo n ic fissu r e: on undersurface of optic cups; closes

on day 33, allowing pressurization of globe
Fo r m atio n o f eye: em bryonic plate ! neural plate ! optic Closure occurs first in m idzone/equator, then extends
pits ! optic vesicles ! optic cups posteriorly and anteriorly
Serves as portal for mesoderm to enter eye (i.e., hyaloid
Em b r yo n ic p late (Fig. 3-1): artery)
1. Ectoderm (form s eye and brain): neural ectoderm , Coloboma: failure of closure of em bryonic fissure;
surface ectoderm , and neural crest sporadic or autosom al dom inant (AD); typical
2. Mesoderm (located in inferonasal quadrant) or atypical (located
3. Endoderm elsewhere)
May involve retina and choroid (associated with basal
Op tic p it: form s at day 23 of gestation encephalocele, cleft palate, and CHARGE syndrom e),
iris, and/or optic nerve
Op tic vesicle: anterolateral outpouching of prim itive An eyelid coloboma is not related to closure of
brain stem ; evaginates on day 25 and becom es the globe em bryonic fissure
Optic vesicle induces the lens placode at day 27 Microphthalmos with cyst: sm all, abnorm al eye with cystic
Abnormalities of evagination: may result in anophthalmia, expansion extending posteriorly into orbit
cyclopia (synophthalmia), congenital cystic eye, congenital Arises in area of and external to a choroidal colobom a;
nonattachment of the retina. Apical forebrain lesions such cyst usually contains dysplastic neuroectoderm al
as synophthalmia are associated with arrhinencephaly, tissue and m ay not directly connect with the eye
proboscis, ethmocephaly, trisomy 13 Optic pit: considered an atypical colobom a; associated with
Op tic cu p (Fig. 3-2): develops em bryologically as an basal encephalocele
anterolateral evagination of the forebrain
Inner layer becom es the retina Hyalo id ar ter y (Fig. 3-3): enters through em bryonic fissure
Outer layer becom es the retinal pigm ent epithelium and form s vasa hyaloidea propria (blood supply to prim ary
Potential space between the two becom es the subretinal vitreous)
space (which was the cavity of the neural tube and optic Intravitreal portion regresses by 8 and one-half m onths;
vesicle) intraneural portion becom es central retinal artery
Cells at anterior m argin of optic cup form the posterior Posterior tunica vasculosa lentis supplies the posterior lens
pigm ent epithelium of the iris Retinal vascular developm ent begins during 16th week:
Cells between the future iris and the future retina form the m esenchym al cells next to hyaloid artery form
ciliary body capillary network, then form arteries and veins;

35
CHAPTER 3 • Em bryology/Pathology

Fig ure 3-1. Neural tube formation.

Ne ura l pla te
Ne ura l cre s t ce lls
Ne ura l cre s t
Ectode rm
Me s ode rm Ne ura l tube
Endode rm

Notochord Ga s trointe s tina l

tube

Fig ure 3-2. Optic cup formation.

Ectode rm Optic S urfa ce

s ta lk e ctode rm

Le ns Le ns Le ns
pit pore ve s icle
Optic Le ns Optic
ve s icle pla code cup

PERIPAPILLARY LOOP: vascular loop extending from

optic nerve head; risk of artery obstruction or vitreous
a nterior va scula r ca psule
hem orrhage
(pupilla ry membra ne) MITTENDORF’S DOT: sm all opacity on posterior lens
capsule at which hyaloid artery is attached to posterior
tunica vasculosa lentis, usually inferonasal
PERSISTENT PUPILLARY MEMBRANE: thin iris strands
bridging pupil; m ay attach to anterior lens capsule;
rem nants of anterior tunica vasculosa lentis
posterior
va scula r
ca psule Pr im itive ep ith elial p ap illae: cells from inner layer of
optic cup at superior end of em bryonic fissure, which becom es
ca psulopupilla ry the optic disc
portion
Ganglion cell axons grow through
Myelination starts centrally, reaching the chiasm at 7 and
one half m onths and lam ina cribrosa at birth; com plete
approxim ately 1 m onth after birth
hya loid a rtery Inner limiting membrane of Elschnig: covers ON,
contiguous with ILM
ON m ay show deceptively exaggerated cupping because
Fig ure 3-3. Hyaloid vasculature and primary vitreous during embryologic ocular
nerve fibers posterior to lam ina cribrosa are
development. (With permission from Dass AB, Trese MT: Persistent hyperplastic
primaryvitreous. In YanoffM, Duker JS (eds): Ophthalmology. London, 1999, Mosby.) incom pletely m yelinated at birth
ON hypoplasia is associated with DeMorsier’s syndrom e;
vessels grow centrifugally from optic disc, reach nasal 13% have pituitary abnorm alities
oraserrata during eighth m onth and tem poral ora 1 to
2 m onths later Vitr eo u s: produced by lens, retina, and walls of hyaloid
Three percent of norm al neonates have a patent hyaloid artery; contains m esenchym al cells
artery Primary vitreous: form ed by hyaloid vascular system
Remnants of hyaloid vasculature system: (vasa hyaloidea propria, which includes hyaloid canal,
BERGMEISTER’S PAPILLAE: at optic nerve head; glial hyaloid vessels, and posterior portions of tunica
sheath of Bergm eister envelops posterior third of vasculosa lentis); eventually replaced by secondary
hyaloid artery and begins to atrophy during seventh vitreous; failure to regress causes persistent hyperplastic
m onth; epipapillary veil results if it does not fully prim ary vitreous (PHPV) (Fig. 3-4)
regress Secondary vitreous: form ed by retina

36
 Em bryology

S urfa ce e ctode rm Ante rior P rima ry

va s cula r vitre ous
ca ps ule
Optic
s ta lk

Hya loid
a rte ry P rima ry
vitre ous S e conda ry
vitre ous
Oute r (nucle a te d) Inne r (non-nucle a te d) Re gre s s ing
primitive zone ma rgina l zone hya loid a rte ry
Fig ure 3-4. Posterior segment development.

Area of Martegiani: extends from disc into vitreous to In eighth m onth, form ation is com plete; trabecular
becom e Cloquet’s canal m eshwork appears just before birth
Cloquet’s canal: junction of prim ary and secondary vitreous
Tertiary vitreous: zonule fibers form ed from ciliary Len s: at 27 days, surface ectoderm adjacent to optic vesicle
processes and lens capsule enlarges to form lens placode (lens plate)
Berger’s space: retrolental space Circular indentation then occurs on lens plate, form ing lens
pit, which invaginates the wall of the optic vesicle until it
Retin a: neuroectoderm ; vascularization begins at 4 m onths; closes to form a sphere
tem poral periphery is last portion to becom e vascularized Basem ent m em brane of the surface ectoderm form s the
Developm ent of fovea is not com plete until 4 or m ore surface of the sphere (the lens vesicle) and subsequently
weeks after birth becom es the lens capsule
Retinal dysplasia: abnorm al proliferation of developing Lens epithelial cells on posterior aspect of this sphere
retina produces tubular structures with a rosette-like elongate and m igrate first (prim ary lens fibers); these
appearance; represents nonspecific response to cells fill the core of the lens vesicle at approxim ately
disorganizing influence during developm ent; associated 40 days (em bryonal nucleus)
with m aternal LSD ingestion, Patau’s syndrom e (trisom y At 7 weeks, anterior cells m igrate toward equator and
13), m icrophthalm os, congenital glaucom a, Peter’s proliferate to form secondary lens fibers that encase the
anom aly, uveal and optic nerve colobom as, cyclopia, embryonal nucleus and form the Y sutures; Y sutures
and synophthalm ia represent the m eeting of em bryonal and fetal nuclei
(upright anteriorly, inverted posteriorly)
Ch o r o id : requires retinal pigm ent epithelium (RPE) for After 3 m onths, zonules of Zinn (zonular fibers) develop
developm ent (Fig. 3-5)
Strom a is from neural crest cells
Vascular endothelium is from m esoderm S u m m a ry
Vessel walls are from neural crest cells 3 weeks lens placode from surface ectoderm
6 weeks lens vesicle; further developm ent
Scler a: neural crest cells and m esoderm (tem poral aspect) requires norm al neuroretina
Blue hue at birth due to thinness (see underlying uveal 12 weeks tunica vasculosa lentis
pigm ent) 28 to 38 weeks degeneration of tunica vasculosa lentis

Co r n ea: neural crest cells (two waves) Lens of a newborn is m ore spherical than that of an adult;
First wave grows between epithelium and lens, form ing therefore, anterior cham ber appears shallow
double layer of corneal endothelium
Second wave grows between epithelium and endothelium ; Ir is: rim of optic cup grows around lens and form s iris
this zone is rich in hyaluronic acid and collagen fibrils Epithelial layers (iris pigm ent epithelium [IPE]; anterior
At 4 m onths, Descem et’s m em brane develops pigm ented and posterior nonpigm ented) are from inner
At 5 m onths, Bowm an’s layer develops and outer layers of the optic cup (neuroectoderm ); form s
part of the blood–aqueous barrier
An gle: neural crest cells from peripheral cornea differentiate In seventh week, strom a form s from neural crest cells, and
into chamber angle during seventh week tunica vasculosa lentis form s
In fourth m onth, Schlem m ’s canal form s In sixth m onth, sphincter and dilator m uscles form from
In seventh m onth, angle m oves posteriorly neuroectoderm

37
CHAPTER 3 • Em bryology/Pathology

S urfa ce e ctode rm

Ante rior ce lls Ante rior

ca ps ule

P os te rior ce lls P os te rior ce ll P os te rior

e longa tion ca ps ule

Fully forme d le ns
Fig ure 3-5. Lens development.

In seventh m onth, blood vessels enter iris Em b r yo lo gic tissu es an d th eir co m p o n en ts:
In ninth m onth, tunica vasculosalentis disappears Neural ectoderm: sensory retina, RPE, nonpigm ented ciliary
Newborn iris is usually gray-blue; developm ent of iris color body epithelium , pigm ented CB epithelium (extension
takes weeks to m onths, as strom al chrom atophores of RPE), IPE, iris sphincter and dilator m uscle, optic
(dendritic m elanocytes from neural crest) com plete their nerve (neural and glial elem ents), sym pathetic ganglion,
m igration into uvea shortly after birth lateral geniculate body, ocular pigm ent granules (RPE,
Iris dilator m uscle is im m ature, causing relative m iosis in CB, IPE), peripheral nerves related to eye function,
infancy erector pili m uscle associated with hair follicles of
RPE and posterior pigm ent epithelium of the iris form from the skin
the outer layer of the optic cup (have m ature coloration Surface ectoderm: crystalline lens, corneal and conjunctival
because pigm ent granules develop very early in epithelium , lid epithelium , lacrim al gland, nasolacrim al
gestation) system
Surface and neural ectoderm: vitreous, zonules
Ciliar y b o d y (CB): form ation begins in third m onth; fold in Neural crest cells: corneal strom a and endothelium , iris
optic cup becom es epithelial layers of ciliary processes strom a, trabecular m eshwork (TM), cham ber angle,
In fourth m onth, filam ents from surface cells form zonules; Schlem m ’s canal, sclera (except tem poral portion),
the m ajor arterial circle of the iris (located in CB), the sheaths and tendons of extraocular m uscles, ciliary
longitudinal ciliary m uscle, and the ciliary processes m uscle (nonpigm ented layer of ciliary body), choroidal
develop strom a, m elanocytes, m eningeal sheaths, orbital bones,
In fifth m onth, pars plana develops, and CB strom a and connective tissue of orbit, m uscular and connective
ciliary m uscle develop from neural crest cells adjacent to tissue layers of blood vessels
cornea S-100 STAIN: specific for neural crest–derived structures
In seventh m onth, circular fibers of ciliary m uscle 3 WAVES OF NEURAL CREST CELL MIGRATION ( DURING
differentiate SEVENTH WEEK) : corneal and TM endothelium ,
Patient’s age can be determ ined by analysis of CB keratocytes (corneal strom a), iris strom a
cellularity ANTERIOR SEGMENT DISORDERS DUE TO NEURAL
CREST ABNORMALITIES:
Naso lacr im al system : at 6 weeks, surface ectoderm is ABNORMAL MIGRATION: congenital glaucom a,
buried in m esoderm , between m axillary and lateral nasal posterior em bryotoxin, Axenfeld-Rieger syndrom e,
processes Peter’s anom aly, sclerocornea
During third m onth, the cord canalizes ABNORMAL PROLIFERATION: iridocorneal endothelial
Defects: m ay result in im perforate valve of Hasner; rarely, (ICE) syndrom es
absent puncta or canaliculi ABNORMAL TERMINAL INDUCTION: corneal
endothelial dystrophies
Eyelid s: at 8 weeks, upper lids form by fusion of m edial and Mesoderm: blood vessel endothelium , anterior cham ber
lateral frontonasal processes; lower lids by fusion of m axillary angle outflow apparatus, sclera (sm all area tem porally),
processes and m edial nasal processes EOM, Schlem m ’s canal, portion of vitreous
At 12 weeks, lid folds fuse Mesenchyme: prim itive connective tissue; originates from
At 24 weeks, separation begins from nasal side neural crest cells and m esoderm (Fig. 3-6)

38
 Microbial Studies

Period a fter Period a fter

conception Event conception Event
2 2nd da y O ptic groove a ppea rs 3rd month Differentia tion of precursors of rods a nd cones
2 5th da y O ptic vesicle forms from optic pit Cilia ry body develops
2 6th da y Primordia of superior rectus, inferior rectus, media l Appea ra nce of limbus
rectus, a nd inferior oblique a ppea r Anterior cha mber a ppea rs a s a potentia l spa ce
2 7th da y Forma tion of lens pla te from surfa ce ectoderm Sclera condenses
Primordium of la tera l rectus a ppea rs Eyelid folds lengthen a nd fuse
2 8th da y Embryonic fissure forms 4th month Forma tion of retina l va scula ture begins
Cells destined to become retina l pigment Beginning of regression of hya loid vessels
epithelium a cquire pigmenta tion Forma tion of physiologic cup of optic disc
2 9th da y Primordium of superior oblique a ppea rs Forma tion of la mina cribrosa
5 th week Lens pit forms a nd deepens into lens vesicle Ma jor a rteria l circle of iris forms
Hya loid vessels develop Development of iris sphincter muscle
Prima ry vitreous develops Development of longitudina l cilia ry muscle a nd
O sseous structures of the orbit begin to develop processes of cilia ry body
6 th week Closure of embryonic fissure Forma tion of tertia ry vitreous
Cornea l epithelia l cells develop interconnections Bowma n’s membra ne forms
Differentia tion of retina l pigment epithelium Ca na l of Schlemm a ppea rs
Prolifera tion of neura l retina l cells Eyelid gla nds a nd cilia form
Forma tion of seconda ry vitreous 5th month Photoreceptors differentia te
Forma tion of prima ry lens fibers Eyelid sepa ra tion begins
Development of periocula r va scula ture 6th month Cones differentia te
Appea ra nce of eyelid folds a nd na sola crima l duct Ga nglion cells thicken in ma cula
Cilia ry ga nglion a ppea rs Differentia tion of dila tor pupilla e muscle
7 th week Migra tion of ga nglion cells towa rd optic disc Na sola crima l system becomes pa tent
Forma tion of embryonic lens nucleus 7th month Rods differentia te
Development of choroida l vessels from periocula r O ra serra ta forms
mesenchyme Migra tion of ga nglion cells to form nerve fiber la yer
Three wa ves of neura l crest migra tion: of Henle
first wa ve: forma tion of cornea l a nd Choroid becomes pigmented
tra becula r endothelium
second wa ve: forma tion of cornea l stroma Circula r cilia ry muscle fibers develop
third wa ve: forma tion of iris stroma Myelina tion of optic nerve
Forma tion of tunica va sculosa lentis Posterior movement of a nterior cha mber a ngle
Sclera begins to form O rbicula ris muscle differentia tion
8th month Completion of a nterior cha mber a ngle forma tion
Hya loid vessels disa ppea r
9th month Retina l vessels rea ch the tempora l periphery
Pupilla ry membra ne disa ppea rs
After birth Development of ma cula

Fig ure 3-6. Timeline of ocular embryogenesis. (With permission from Azar NJ , Davis EA: Embryology in the eye. In: Yanoff M, Duker J S (eds): Ophthalmology, ed 2,
St Louis, 2004, Mosby.)

P ATHOLOGY Acid fast (Ziehl-Neelsen): Mycobacterium, Nocardia

Per io d ic acid -Sch iff (PAS): fungi

MICROBIAL STUDIES
Go m o r i’s m eth en am in e silver : fungi
S t a in s
Calco flu o r wh ite: fungi and Acanthamoeba (binds to cell
Gr am : bacteria, fungi wall, visible with fluorescent m icroscopy)
Giem sa: Acantham oeba, fungi, and cytology; best for
intranuclear inclusion bodies KOH: fungi

39
CHAPTER 3 • Em bryology/Pathology

Cu lt u re m e d ia Oil r ed O: stains neutral lipids red-orange in frozen

section; m ust be used on fresh tissue because form alin leaches
Blo o d agar : m ost bacteria; very good for atypical out lipid
Mycobacterium
Su d an b lack: stains phospholipids; m yelin in ON
Blo o d agar in 5% to 10% car b o n d io xid e: Moraxella
Lu xo l fast b lu e: stains m yelin blue; dem yelinated plaques
Ch o co late (contains hem in and nicotinam ide adenine lose affinity for stain
dinucleotide [NAD]): Haemophilus, Neisseria
Bo d ian : stains nerve fibers black
Th io glyco late: anaerobes Mu cicar m in e: stains m ucus pink / red; m ucus-secreting
adenocarcinom as (i.e., m ucoepithelioid variant of squam ous
Sab o u r au d ’s: fungi cell carcinom a, GI, breast)

Lo€ wen stein -Jen sen : Mycobacterium tuberculosis, Nocardia Ver h o eff Van Gieso n : stains elastic tissue black; used for
elastotic degeneration
Lo effler ’s: Corynebacteria
Mo vat’sp en tach r o m e: stains elastic tissue black
No n -n u tr ien t agar with E. coli o ver gr o wth : Wild er : stains reticulin fibers black
Acanthamoeba
Alizar in r ed : stains calcium red-orange
Cyt o lo g y
vo n Ko ssa: stains calcium black; used for band keratopathy
In tr acyto p lasm ic b aso p h ilic in clu sio n s (Giem sa
stain): Chlamydia Pr u ssian b lu e: stains iron (hem osiderin, ferric ions) blue

In tr an u clear eo sin o p h ilic in clu sio n s (Papanicolaou Fo n tan a-Masso n : stains m elanin black; used for
stain): herpes (Tzanck sm ear) am elanotic m elanom a

S-100 p r o tein : stains nevi, m elanom as, schwannom as,

TISSUE STAINS neurofibrom as, and other heterologous cell lines

Hem ato xylin an d eo sin (H&E): hem atoxylin is specific for Po lar izin g filter s: for evaluating structures or deposits that
nucleic acids within nuclei and stains blue (basophilic); eosin have a regular m olecular structure (am yloid, calcium oxalate
is specific for m ost cytoplasm ic organelles (such as crystals), as well as suture granulom as and vegetable foreign
m itochondria) and stains pink (eosinophilic) bodies

Per io d ic acid -Sch iff ( PAS) : stains basem ent m em brane

m aterial m agenta (Descem et’s, lens capsule, Bruch’s TISSUE FIXATION
m em brane, ILM [internal lim iting m em brane], guttae,
drusen); also stains glycogen, fungi, conjunctival goblet cells Or ien tatio n o f glo b e: identify superior oblique (SO)
(useful for differentiation of corneal from conjunctival (tendinous insertion) and inferior oblique (IO) (m uscular
epithelium ) insertion) m uscles

Masso n tr ich r o m e: stains collagen blue or green, and Par affin : em bedding process for histologic exam ination:
hyaline red; used for granular dystrophy water is rem oved, organic solvents leach out lipids;
polym ethylm ethacrylate (PMMA) is dissolved com pletely; to
preserve lipids, fresh or frozen tissue specim ens are used;
Co n go r ed : stains am yloid orange; used for lattice dystrophy
paraffin m ust be rem oved before different stains are applied

Cr ystal vio let: stains am yloid red-purple; used for lattice Glu tar ald eh yd e: for electron m icroscopy
dystrophy
Fo r m alin an d Bo u in ’s fixative: for light m icroscopy; 10%
Alcian b lu e: stains acid m ucopolysaccharide buffered form alin (formalin ¼ 40% solution of form aldehyde in
(glycosam inoglycan) blue; used for m acular dystrophy water); form alin stabilizes protein, lipid, and carbohydrates, and
prevents postm ortem enzymatic destruction of tissue
Co llo id al ir o n : stains acid m ucopolysaccharide
(glycosam inoglycan) blue; used for m acular dystrophy Eth yl alco h o l: cytology

40
 HLA (Hum an Leukocyte Antigen) System

Fixatio n ar tifacts: Typ e III: im m une com plex deposition (Ag–Ab com plex)
Lange’s fold: retinal fold at ora serrata in newborn eyes Exa m p le : Stevens-Johnson, marginal infiltrates, disciform keratitis, subepithelial
infiltrates, Wessely ring, scleritis, retinal vasculitis, phacoanyphlaxis
probably caused by unequal shrinkage of retinociliary
tissues during fixation Typ e IV: cell-m ediated, delayed hypersensitivity
Artifactual RD: com m on histologic finding, differentiated (CD4 lym phocytes)
from true retinal detachm ent by lack of subretinal fluid, Exa m p le : phlyctenule, graft reaction, contact dermatitis, interstitial keratitis,
preservation of photoreceptors, and pigm ent attached to granulomatous disease (tuberculosis [TB], syphilis, leprosy), sympathetic
outer surface of rods and cones (Fig. 3-7) ophthalmia, Vogt-Koyanagi-Harada (VKH) syndrome

Clefts in corneal stroma: clear spaces within strom a; Typ e V: stim ulating antibody
obliterated in corneal edem a Exa m p le : Graves’ disease, myasthenia gravis

HYP ERSENSITIVITY REACTIONS IMMUNOGLOBULINS

(COOMBS AND GELL CLASSIFICATION) IgG: m ost abundant; crosses the placenta; binds com plem ent
Typ e I: anaphylactic/ im m ediate hypersensitivity (IgE) IgA: second m ost abundant; m onom eric or joined by J chain;
Exa m p le : hay fever, vernal, atopic, giant papillary conjunctivitis (GPC) im portant against viral infection; found in secretions

IgM: largest; binds com plem ent; im portant in prim ary

Typ e II: cytotoxic hypersensitivity (com plem ent m ediated)
Exa m p le : OCP, Mooren’s ulcer
im m une response
IgD: present in newborns; not in tear film
IgE: sensitizes m ast cells and tissue leukocytes; role in atopy

Ta b le 3-1. Human leukocyte antigen associations

Uve itis
A11, DR4 Sympathetic ophthalmia
A29 Birdshot retinochoroidopathy (90%)
B7, DR2 Presumed ocular histoplasmosis syndrome
(80%)
B8, B13 Sarcoidosis
B8, B51, DR2, DR15 Intermediate uveitis
B27 (1-5% of population) Adult iridocyclitis (usually unilateral): Reiter’s
syndrome (75%), ankylosing spondylitis (90%),
inflammatory bowel disease (90%), psoriatic
arthritis (also B17), juvenile rheumatoid arthritis
(J RA: subtype V)
B5, B51 Behc¸et’s disease (70%)
DR4, Dw53 Vogt-Koyanagi-Harada syndrome

A DR15 Pars planitis

DQ7 Acute retinal necrosis (50%)

Exte rna l d is e a s e
B5, DR3, DR4 HSV keratitis
B8, DR3 Sjo€gren’s syndrome
B12 Ocular cicatricial pemphigoid
B15 Scleritis
DR3 Thygeson’s superficial punctate keratitis (SPK)

Ne uro -o p htha lm o lo g y
A1, B8, DR3 Myasthenia gravis (MG)
B7, DR2 Multiple sclerosis (MS)
DR3 Graves’ disease

B
HLA (HUMAN LEUKOCYTE ANTIGEN)
SYSTEM
Fig ure 3-7. A, Artifact retinal detachment (RD) with no fluid, pigment adherent to
photoreceptors, and normal retinal architecture. B, True RD with material in
subretinal space and degeneration of outer retinal layers . (With permission from Major histocom patibility com plex (MHC) proteins found on
Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.) surfaces of all nucleated cells

41
CHAPTER 3 • Em bryology/Pathology

In hum ans, MHC proteins are the HLA m olecules Types: B cells, T cells (helper, suppressor, cytotoxic, killer,
null cells)
Gene loci are located on chrom osom e 6
Scanty cytoplasm
Class I: antigen presentation to cytotoxic T cells (CD8
positive); loci A, B, C Plasm a cells: activated B cells
Class II: antigen presentation to helper T cells (CD4 Synthesis and secretion of antibodies
positive); loci DR, DP, DQ Eccentric “cartwheel” nucleus, basophilic cytoplasm
Plasmacytoid cell: granular eosinophilic cytoplasm
Russell body: im m unoglobulin crystals
INFLAMMATION
Macr o p h ages: histiocytes, m onocytes (Fig. 3-8)
Tissue infiltration by inflam m atory cells Prim ary phagocytic cell; second line of cellular defense;
regulation of lym phocytes via Ag presentation and
Typ e s o f in fla m m a t o ry c e lls m onokine production
Transform ation into epithelioid and giant cells
Kidney-shaped nucleus
Neu tr o p h ils: polym orphonuclear leukocytes (PMNs)
Prim ary cell in acute inflam m ation; phagocytosis Ep ith elio id h istio cyte: activated m acrophage with
Multilobed nucleus vesicular nucleus and eosinophilic cytoplasm ; cells resem ble
Abscess: focal collection of PMNs epithelium ; hallm ark of granulom atous inflam m ation; fuse to
Pus: PMNs and tissue necrosis form giant cells

Eo sin o p h ils: Gian t cells: Three types

Allergic and parasite-related reactions (“worm s, wheezes, Langhans: nuclei arranged around periphery in ring /
weird diseases”); m odulation of m ast cell reactions, horseshoe pattern
phagocytosis of Ag–Ab com plexes Exa m p le : TB, sarcoidosis
Bilobed nucleus, granular cytoplasm Touton: m idperipheral ring of nuclei; central eosinophilic
cytoplasm ; nuclei are surrounded by clear zone of foam y
Mast cells: tissue basophils lipid
IgE bound to surface; Ag causes degranulation with release Exa m p le : juvenile xanthogranuloma (J XG)

of histamine and heparin Foreign body: nuclei random ly distributed; surrounds or

Exa m p le : anaphylaxis, allergic conjunctivitis contains foreign body
Looks like plasm a cell
Typ e s o f in fla m m a t io n
Lym p h o cytes: Acu te:
Main cell in hum oral and cell-m ediated im m une Suppurative: neutrophils
reactions Nonsuppurative: lym phocytes

Fig ure 3-8. Macrophage

differentiation. (Modified from Roitt IM,
Brostoff J , Male DK: Immunology, ed 2,
London, 1989, Gower Medical.)

? La ngha ns ce ll
Activa te d
ma cropha ge

?
Monocyte
ma cropha ge Fore ign body
Gia nt ce ll

? ?
Epithe lioid
ce ll

Touton

42
 Eyelid Epithelial Changes

Ch r o n ic: on Bruch’s m em brane, contain lacunae of RPE cells

Nongranulomatous: lym phocytes and plasm a cells [pseudoadenom atous proliferation]); intraocular bone
Granulation tissue in reparative phase; exuberant response from osseous m etaplasia
causes pyogenic granulom a Reactive gliosis
Granulomatous: epithelioidhistiocytes; three patterns
DIFFUSE: epithelioid cells distributed random ly Ph th isis: rectus m uscle traction on hypotonous globe causes
against background of lym phocytes and plasm a cells squared-off appearance; thickened sclera, high incidence of
Exa m p le : sympathetic ophthalmia, fungal infection, J XG, lepromatous retinal detachm ent and disorganization, calcareous
leprosy degeneration of lens
DISCRETE: epithelioid cells form nodules with giant
cells, surrounded by rim of lym phocytes and plasm a
cells
Exa m p le : sarcoidosis, miliary TB, tuberculoid leprosy EYELID EP ITHELIAL CHANGES
ZONAL: palisaded giant cells surround central nidus
Exa m p le : phacoantigenic endophthalmitis (phacotoxic uveitis is
nongranulomatous); rheumatoid scleritis (nidus ¼ scleral collagen);
Hyp er ker ato sis: thickening of the keratin layer; clinically
chalazion appears as white, flaky lesion (leukoplakia)
Endophthalmitis: inflam m ation involving at least one
ocular coat and adjacent cavity, sclera is not involved Par aker ato sis: thickening of the keratin layer with
Panophthalmitis: suppurative endophthalm itis also retention of nuclei; indicates shortened epiderm al regeneration
involving sclera and orbit tim e; granular layer is thin or absent

S e q u e la e o f in fla m m a t io n Dysker ato sis: keratin form ation within the basal cell layer
or deeper
Co r n ea: scarring
Calcific band keratopathy: basophilic granules in Bowm an’s
Acan th o sis: thickening of the squam ous cell layer due to
m em brane
proliferation of prickle cells
Inflammatory pannus: subepithelial fibrovascular and
inflam m atory ingrowth with destruction of Bowm an’s
m em brane
Acan th o lysis: loss of cohesion between epiderm al cells
Exa m p le : trachoma with breakdown of intercellular junctions, creating spaces
Degenerative pannus: fibrous tissue between epithelium within the epiderm is; occurs in pem phigus and produces
and intact Bowm an’s m em brane intraepithelial bullae
Exa m p le : chronic corneal edema
Dysp lasia: disorderly m aturation of epithelium with loss of
An ter io r ch am b er : organization of hypopyon; polarity, cytologic atypia, and m itotic figures found above the
retrocorneal fibrous m em branes basal layer
Peripheral anterior synechiae (PAS): seclusio pupillae Mild: < 50% epiderm al thickness involved
(if 360°) Severe: > 50% involved
Pupillary membrane: occlusion pupillae
Car cin o m a in situ : full-thickness replacem ent of
epithelium by m alignant cells without invasion through the
Len s:
basem ent m em brane
Anterior subcapsular cataract: fibrous plaque beneath
folded anterior capsule, secreted by irritated m etaplastic Squ am o u s cell car cin o m a: m alignant epithelial cells
anterior epithelial cells invade below basem ent m em brane
Posterior subcapsular cataract: bladder cells adjacent to
capsule
An ap lasia: cytologic m alignancy with pleom orphism ,
anisocytosis, abnorm al nuclei, and m itotic figures
Ciliar y b o d y:
Cyclitic membrane: retrolental collagenous m em brane Pap illo m ato sis: proliferation of derm al papillae, causing
attached to CB; contraction leads to detachm ent of pars surface undulation
plana, ciliary m uscle rem ains adherent to scleral spur
attachm ent; due to organization and scarring of vitreous, Pseu d o ep ith elio m ato u s h yp er p lasia: inflam m atory
m etaplastic ciliary epithelium , organized inflam m atory response with hyperplasia of epithelium , which m im ics
residua carcinom a; acanthosis with protrusion of broad tongues of
benign epiderm is into the derm is
Retin a:
CME: retinal vascular leakage or Mu€ ller cell edem a Elasto sis: actinic dam age; seen as blue staining (norm ally
RPE changes: hypertrophy, hyperplasia, and m igration pink) of superficial derm al collagen with H&E stain; dam aged
(pseudoretinitis pigm entosa); fibrous m etaplasia collagen stains with elastic tissue stains but is not susceptible to
(collagen and basem ent m em brane m aterial deposited digestion with elastase (Fig. 3-9)

43
CHAPTER 3 • Em bryology/Pathology

cyst (free-floating or attached to iris); im planted cells tend to be

2 to 4 cell layers thick and have conjunctival characteristics
(m ore than corneal)

Fib r o u s d o wn gr o wth : proliferating fibroblasts originate

from episcleral or corneal strom a; contraction can occur; can
occur with a puncture wound if there is a break in Descem et’s
m em brane

Hem o r r h age:
Corneal blood staining: hem oglobin (Hgb) breakdown
products are forced through endothelial cells by
increased intraocular pressure (IOP); Hgb m olecules are
Fig ure 3-9. Elastosis demonstrating basophilic degeneration of conjunctival
substantia propria in a pinguecula. (With permission from Yanoff M, Fine BS:
rem oved by phagocytic and biochem ical processes
Ocular pathology, ed 5, St Louis, 2002, Mosby.) Hemosiderosis bulbi: hem osiderin contains iron; can
dam age essential intracellular enzym e system s
Ochre membrane: hem orrhage that accum ulates on
posterior surface of detached vitreous
AGING CHANGES Synchysis scintillans: accum ulation of cholesterol within
vitreous following breakdown of red blood cell
Co r n ea: Hassal-Henle warts (excrescences and thickenings of m embranes; angular, birefringent, flat crystalline particles
Descem et’s m em brane in corneal periphery) with golden hue located in dependent portions of globe;
cholesterol dissolves during preparation of tissues in
Ciliar y ep ith eliu m : hyperplasia and proliferation paraffin; cholesterol clefts are negative image of cholesterol
crystals, surrounded by serous fluid
Par s p lan a an d p ar s p licata: clear (teardrop) cysts
Bo x 3-1. Differential diagnostics of intraocular calcification
Retin a: wounds loss of retinal cells and replacem ent with Retinoblastoma
glial tissue; chorioretinal adhesions and pigm entary lesions Choroidal osteoma
in periphery; peripheral m icrocystoid degeneration (Blessig-
Choroidal hemangioma
Iwanoff cysts): located in outer plexiform layer; bubbly
Phthisis
appearance just behind ora serrata; lined by Mu€ ller cells;
Osseous choristoma
contain m ucopolysacharides

WOUNDS Bo x 3-2. Differential diagnostics of intraocular cartilage

Persistent hyperplastic primary vitreous (retrolental plaque)

Wo u n d h e a lin g Medulloepithelioma
Teratoma
Co r n ea: strom al healing is avascular; fibrosis; neutrophils
Trisomy 13 (see Fig. 3-10)
arrive via tears in 2 to 6 hours; wound edges swell and
glycosam inoglycans (keratan sulfate, chondroitin sulfate) Complex choristoma of conjunctiva
disintegrate at edge of wound; activated fibroblasts m igrate
across wound and produce; takes 4 to 6 weeks to return to
Bo x 3-3. Collagen
full corneal thickness collagen and fibronectin; anterior surface
re-epithelializes; endothelium m igrates and regenerates Typ e 1: normal corneal stroma; Bowman’s membrane (highly disorganized
Descem et’s m em brane type 1, basal lamina has type 4)
Typ e 2: vitreous
Scler a: does not heal itself; it is avascular and acellular; Typ e 3: stromal wound healing
ingrowth of granulation tissue from episclera and choroid Typ e 4: basement membranes

Ir is: no healing

Retin a: scars are produced by glial cells rather than OCULAR INJ URIES
fibroblasts
Blu n t t ra u m a
Wo u n d c o m p lic a t io n s
Scler al r u p tu r e (weak spots):
Ep ith elial in gr o wth : sheet of m ultilayered nonkeratinized 1. Lim bus (on opposite side from traum a)
squam ous epithelium over any intraocular surface; m ay form 2. Posterior to rectus m uscle insertions

44
 Ocular Injuries

Co m m o tio r etin ae: tem porary loss of retinal transparency;

due to disruption of photoreceptor elem ents, not true
retinal edem a

P e n e t ra t in g t ra u m a
Pen etr atio n : partial-thickness wound (into)

Per fo r atio n : full-thickness wound (through) (globe

penetration is due to perforation of the cornea or sclera; globe
perforation is a double penetrating injury)

S e q u e la e o f t ra u m a
Fig ure 3-10. Trisomy 13 demonstrating intraocular cartilage and retinal Ph th isis b u lb i:
dysplasia. (Reported in Hoepner J , Yanoff M: Ocular anomalies in Trisomy 13–15:
Atrophia bulbi without shrinkage: initially, size and shape of
an analysis of 13 eyes with two new findings. Am J Ophthalmol 74:729-37, 1972.)
eye are m aintained; with loss of nutrition: cataract
develops, retina atrophies and separates from RPE by
3. Equator serous fluid accum ulation, synechiae cause
4. Lam ina cribrosa (ON) increased IOP
Atrophia bulbi with shrinkage: eye becom es soft owing
Uveal tr act is co n n ected to scler a in 3 p laces: to ciliary body dysfunction; internal structures are
1. Scleral spur atrophic but histologically recognizable; globe becom es
2. Internal ostia of vortex veins sm aller with squared-off shape (because of tension of
3. Peripapillary tissue rectus m uscles); anterior cham ber (AC) collapses;
corneal endothelial cell dam age leads to corneal edem a
Cyclo d ialysis: disinsertion of longitudinal fibers of ciliary and opacification
m uscle from scleral spur Atrophia bulbi with disorganization (phthisis bulbi): globe
shrinks to average diam eter of 16–19 m m ; m ost ocular
An gle r ecessio n : rupture of face of ciliary body; plane of contents are disorganized; calcification of Bowm an’s
relative weakness at ciliary body face extending posteriorly layer, lens, retina, and drusen; bone form ation in
between longitudinal fibers and m ore central oblique and uveal tract
circular fibers; oblique and circular m uscles atrophy, changing
cross-sectional shape of ciliary body from triangular to In t ra o c u la r fo re ig n b o d y
fusiform
Co p p er :
Ir id o d ialysis: disinsertion of iris root from ciliary body ≥ 85%: noninfectious suppurative endophthalm itis
< 85% (chalcosis): copper deposits in basem ent m em branes
Vo ssiu s r in g: com pression and rupture of IPE cells against (Kayser-Fleisher ring, sunflower cataract, retinal
anterior surface of lens deposit ring of m elanin concentric degeneration)
to pupil
Steel (contains iron): siderosis bulbi; follow with
Len s cap su le r u p tu r e: capsule is thinnest at posterior pole; electroretinogram (early increased a wave, norm al b wave; later
cataract can form im m ediately; epithelium m ay be stim ulated decreased b wave leading to extinguished)
by traum a to form anterior lenticular fibrous plaque
Or gan ic (vegetable m atter): severe granulom atous foreign
Descem et’s r u p tu r e: causes acute edem a (hydrops); due to body response
m inor traum a (keratoconus) or m ajor traum a (forceps injury)
Ch e m ic a l in ju ry
Ch o r o id al r u p tu r e: often concentric to optic disc; risk
of CNV Acid : precipitates proteins; zone of coagulative necrosis acts
as barrier to deeper penetration
Sclo p etar ia: high-speed projectiles (e.g. bullet injury);
choroidal rupture with overlying rupture/necrosis of retina Alkali: denatures proteins and lyses cell m em branes; no
effective barrier is created—therefore deeper penetration;
Retin al d ialysis: retina anchored anteriorly to vascular occlusion, ischem ia, corneal dam age during healing
nonpigm ented epithelium of pars plana and reinforced by phase owing to collagenase released by regenerating tissue;
vitreous base, which straddles the ora serrata; circum ferential lim bal bleaching in severe cases (if lim bal stem cells are
tear of retina at point of attachm ent of ora or im m ediately depleted the corneal surface is repopulated with
posterior to vitreous base attachm ent conjunctival cells)

45
CHAPTER 3 • Em bryology/Pathology

Ra d ia t io n
REVIEW QUESTIONS (Answers start on page 375)
No n io n izin g: depends on wavelength
Microwave: cataract
1. Which stain is the m ost helpful in the diagnosis of
Infrared: true exfoliation of lens capsule (glassblower’s
sebaceous gland carcinom a?
cataract)
a. Giem sa
Ultraviolet: keratitis (welder’s flash, snow blindness)
b. hem atoxylin and eosin
Io n izin g: tissue dam age is direct (actively reproducing cells) c. oil-red-O
or indirect (blood vessels); epithelial atrophy and ulceration, d. m ethenam ine silver
derm atitis of eyelids, dysfunction of adnexa, destructive ocular 2. Pagetoid spread is m ost com m only associated
surface disease with keratinization, cataract; retinal necrosis, with
ischem ia, neovascularization, optic atrophy (retina is relatively a. m alignant m elanom a
radioresistant, but retinal blood vessels are vulnerable) b. squam ous cell carcinom a
c. sebaceous gland carcinom a
In fe c t io n (Table 3-2) d. Merkel cell tum or
3. A m elanom a occurring in which of the following locations
Ta b le 3-2. Most common cause of infections has the best prognosis?
a. iris
Endophthalmitis: b. ciliary body
Acute postoperative (< 6 weeks) Coagulase-negative Staphylococcus ,
Staphylococcus aureus
c. choroid, anteriorly
d. choroid, posterior pole
Delayed postoperative Propionibacterium acnes, coagulase-
negative Staphylococcus 4. Calcification in retinoblastom a is due to
From filtering bleb Streptococcus pneumoniae,
a. RPE m etaplasia
Staphylococcus, Haemophilus b. necrosis
influenzae c. hem orrhage
Posttraumatic Staphylococcus species, Bacillus d. m etastasis
cereus, Gram-negative organisms 5. The type of organism that causes Lym e disease
Endogenous (intravenous Candida is a
drug abuse (IVDA)) a. bacillus
Dacryocystitis S. pneumoniae, Staphylococcus b. spirochete
Dacryadenitis Staphylococcus c. protozoan
Canaliculitis Actinomyces d. tick
Orbital cellulitis (children) Staphylococcusaureus 6. Characteristics of ghost cells include all of the
Preseptal cellulites S. aureus following except
Angular blepharitis Staphylococcus, Moraxella a. khaki colored
b. rigid
c. Heinz bodies
Tu m o rs (Box 3-4) d. biconcave
Bo x 3-4. Tumors 7. A gland of Moll is best categorized as
a. m ucin
Congenital: b. apocrine
Hamartoma: composed of tissues normally found in that area c. sebaceous
Exa m p le : hemangioma d. holocrine
Choristoma: composed of tissues not normally found in that area 8. Which of the following is not a Gram -positive
Exa m p le : choroidal osteoma rod?
Most common primary malignant intraocular tumor in adults: uveal a. Corynebacterium
melanoma b. Bacillus
Second most common primary malignant intraocular tumor in adults: c. Serratia
lymphoma d. Listeria
Most common primary malignant intraocular tumor in children: 9. Trantas’ dots are com posed of what cell type?
retinoblastoma
a. m acrophage
Second most common primary malignant intraocular tumor in children: b. neutrophil
medulloepithelioma
c. eosinophil
Most common malignant lacrimal gland tumor: adenocystic carcinoma
d. m ast cell
Most common benign orbital tumor in adults: cavernous hemangioma 10. Types of collagen that can be found in the cornea include
Most common benign orbital tumor in children: capillary hemangioma all of the following except
Most common primary malignant orbital tumor in children: a. I
rhabdomyosarcoma b. II
Most common metastasis to orbit in children: neuroblastoma c. III
d. IV

46
 Review Questions

11. Lens nuclei are retained in all of the following 21. Intraocular calcification m ay occur in all of the
conditions except following except
a. Leigh’s syndrom e a. retinoblastom a
b. Lowe’s syndrom e b. m edulloepitheliom a
c. rubella c. choroidal hem angiom a
d. Alport’s syndrom e d. phthisis
12. Vogt-Koyanagi-Harada syndrom e is best described by 22. The histopathology of which tum or is classically described
which type of hypersensitivity reaction? as a storiform pattern of tum or cells?
a. I a. rhabdom yosarcom a
b. II b. plasm acytom a
c. III c. neurilem om a
d. IV d. fibrous histiocytom a
13. Lacy vacuolization of the iris pigm ent epithelium occurs in 23. Which of the following findings is a histologic fixation
which disease? artifact?
a. central retinal vein occlusion a. Lange’s fold
b. diabetes b. Mittendorf’s dot
c. central retinal artery occlusion c. Berger’s space
d. hypercholesterolem ia d. Cloquet’s canal
14. Antoni A and B cells occur in which tum or? 24. The corneal strom a is com posed of
a. neurilem m om a a. surface ectoderm
b. m eningiom a b. neural crest cells
c. gliom a c. m esoderm
d. neurofibrom a d. neural ectoderm
15. Which tum or is classically described as having a ”Swiss 25. Neisseria is best cultured with which m edia?
cheese” appearance? a. Loeffler’s
a. rhabdom yosarcom a b. Sabaroud’s
b. adenoid cystic carcinom a c. thioglycolate
c. benign m ixed tum or d. chocolate agar
d. m eningiom a 26. Which of the following stains is used to detect
16. Which iris nodule is correctly paired with its am yloid?
histopathology? a. colloidal iron
a. JXG, inflam m atory cells b. Alcian blue
b. Lisch nodule, neural crest ham artom a c. crystal violet
c. Koeppe nodule, strom al hyperplasia d. Masson trichrom e
d. Brushfield spot, histiocytes and Touton giant cells 27. HLA-B7 is associated with
17. Which of the following statem ents is true concerning a. Behçet’s disease
im m unoglobulin b. presum ed ocular histoplasm osis syndrom e
a. IgG crosses the placenta c. iridocyclitis
b. IgA binds com plem ent d. sym pathetic ophthalm ia
c. IgM is present in newborns 28. Which of the following conjunctival lesions should be
d. IgD is the second m ost abundant sent to the pathology lab as a fresh unfixed tissue
18. A retinal detachm ent caused by fixation artifact can specim en?
be differentiated from a true RD by all of the following a. lym phom a
except b. squam ous cell carcinom a
a. a fold at the ora serrata c. Kaposi’s sarcom a
b. no subretinal fluid d. m elanom a
c. norm al retinal architecture 29. Subepithelial infiltrates in the cornea from epidem ic
d. pigm ent adherent to photoreceptors keratoconjunctivitis are thought to be
19. Which of the following epithelial changes in the eyelid a. lym phocytes and dead adenovirus
refers to thickening of the squam ous cell layer? b. polym orphonuclear leukocytes surrounding live
a. parakeratosis adenovirus
b. acanthosis c. m acrophages containing adenoviral particles
c. dysplasia d. lym phocytes and polym orphonuclear leukocytes
d. papillom atosis 30. Which is the correct order of solutions for perform ing a
20. Intraocular hem orrhage m ay cause all of the following Gram stain?
sequelae except a. iodine solution, crystal violet stain, ethanol, safranin
a. synchysis scintillans b. crystal violet stain, safranin, ethanol, iodine solution
b. ochre m em brane c. iodine solution, safranin, ethanol, crystal violet stain
c. asteroid hyalosis d. crystal violet stain, iodine solution, ethanol,
d. hem osiderosis bulbi safranin

47
CHAPTER 3 • Em bryology/Pathology

31. The iris sphincter is derived from what em bryologic tissue?

a. neural crest cells SUGGESTED READINGS
b. surface ectoderm
c. neural ectoderm Em b ryo lo g y
d. m esoderm Mann, I. (1964). The development of the human eye. New York: Grune &
32. Blepharitis is m ost com m only associated with Stratton.
a. Streptococcus pneumoniae
P a t h o lo g y
b. Staphylococcus aureus
c. Haemophilus pneumoniae Apple, D. J. (1998). Ocular pathology: Clinical applications and self-
assessment (5th ed.). St Louis: Mosby.
d. Proprionibacterium acnes
Basic and Clinical Science Course. (2016). Section 2: Fundamentals and
33. Which type of radiation causes lens capsule scrolling?
principles of ophthalmology. San Francisco: AAO.
a. infrared Basic and Clinical Science Course. (2016). Section 4: Ophthalmic
b. ionizing pathology and intraocular tumors. San Francisco: AAO.
c. m icrowave Char, P. H. (2001). Tumors of the eye and ocular adnexa. Ontario,
d. ultraviolet Canada: BC Decker.
34. Which of the following m easures is m ost likely to reduce Eagle, R. C. (2011). Eye pathology: An atlas and basic text (2nd ed.).
the risk of postoperative endophthalm itis following Philadelphia: Lippincott William s & Wilkins.
cataract surgery? Spencer, W. H. (1996). Ophthalmic pathology: An atlas and textbook
a. preoperative povidone-iodine drops (4th ed.). Philadelphia: WB Saunders.
Yanoff, M., & Sassani, J. W. (2014). Ocular pathology (7th ed.).
b. eyelid draping
Philadelphia: Saunders.
c. intracam eral injection of gentam icin
d. subconjunctival injection of gentam icin
35. The m ost com m on causative organism of canaliculitis is
a. Candida albicans
b. HSV
c. Actinomyces israelii
d. Nocardia asteroides

48
4 Neuro-ophthalm ology
ANATOMY OF THE VISUAL PATHWAY
PHYSIO LOGY
DIPLOPIA
EYE MOVEMENT DISORDERS
NYSTAGMUS
CRANIAL NERVE PALSIES
PUPILS
OCULAR MUSCLE DISO RDERS
EYE MOVEMENTS IN COMA
OPTIC NERVE
CHIASM COMPRESSION
RETROCHIASMAL DISORDERS
CORTICAL LESIONS
DISO RDERS DURING PREGNANCY
BRAIN TUMORS
HEADACHES
VISUAL DISTURBANCES
VASCULAR DISORDERS
INTRACRANIAL ARACHNOID CYST
NEURO-O PHTHALMIC MANIFESTATIONS OF AIDS

Intracranial ON: above are found the frontal lobe,

ANATOMY OF THE VISUAL P ATHWAY olfactory tract, and anterior cerebral and anterior
com m unicating arteries; laterally, the internal carotid
artery em erges from the cavernous sinus
Optic nerve ! chiasm ! optic tract ! lateral geniculate
Blood supply (Fig. 4-3)
body ! optic radiation ! occipital lobe (Fig. 4-1)
ORBITAL PORTION: ophthalm ic artery with m eningeal
anastom oses
Op tic n er ve (ON): com posed of 1.2 m illion nerve fibers; INTRACANALICULAR PORTION: pial branches from
approximately 1.5 m m in diam eter, enlarges to 3.5 m m ophthalm ic artery; possibly internal carotid
posterior to lamina cribrosa due to m yelin sheath; located 3 to artery (ICA)
4 m m from fovea; causes absolute scotoma (blind spot) 15° INTRACRANIAL PORTION: sm all vessels from ICA,
tem poral to fixation and slightly below horizontal m eridian; anterior cerebral and anterior com m unicating arteries
approximately 45 to 50 m m in length (1 m m intraocular,
25 m m intraorbital, 9 m m intracanalicular, 10 to 15 m m Ch iasm : 10 m m above pituitary gland
intracranial) (Fig. 4-2); acquires m yelin posterior to lamina 53% of ON fibers cross in chiasm: nasal retinal fibers cross in
cribosa chiasm to contralateral optic tract (decussating nasal
Surrounded by three layers of meninges: dura m ater (outer fibers); inferior fibers (subserving the superior visual
layer; m erges with sclera), arachnoid layer, pia m ater field) are the first to cross; tem poral fibers rem ain
(inner layer, fused to surface of nerve); space between uncrossed; m acular fibers run posteriorly (posterior
arachnoid and pia contains cerebrospinal fluid (CSF) com pression leads to bitem poral defect)
ON runs through annulus of Zinn (ring of tendinous origins “Knee of von Willebrand”: inferonasal retinal fibers cross in
of the rectus m uscles) and enters the optic canal chiasm and course anteriorly approxim ately 4 m m into
Optic canal: 9 m m long and 5 to 7 m m wide; thinnest contralateral ON before running posteriorly; produces
m edially, adjacent to ethm oid and sphenoid sinuses; junctional scotom a
dura of ON fuses with periosteum of canal Carotid arteries course on either side of chiasm (Fig. 4-4)

49
CHAPTER 4 • Neuro-ophthalm ology

Vis ua l fie ld

Le ft Right

L M
P upilla ry fibe rs
Optic ne rve

P A S upe rior colliculus

Optic chia s m

L M

Optic tra ct

L M

Ge niculo-ca lca rine

La te ra l ge nicula te ra dia tion
body

Me ye r's loop
L M

Ca lca rine fis s ure Occipita l lobe

Ante rior optic L M
ra dia tion

S ple num of corpus ca llos um

P os te rior optic
ra dia tion

P os te rior pole of le ft ce re bra l he mis phe re

Fig ure 4-1. Visual pathway.

Blood supply: ICA; occasionally by anterior cerebral and Special fibers run to the hypothalam us, contributing
anterior com m unicating arteries to neuroendocrine system s that control diurnal
rhythm s
Op tic tr act: lower fibers lie laterally (90° rotation of fibers); A m ajor projection leaves the optic tract just before the
tract courses laterally around cerebral peduncle lateral geniculate body (LGB) to form the brachium of
Dam age to optic tract results in contralateral relative the superior colliculus (also called optic tectum)
afferent pupillary defect (RAPD) because 53% Superior colliculus: involved in foveation reflexes
of fibers cross (greater quantity of nasal fibers [nasal to (receives input from pupillary fibers); injury disrupts
foveal]), including the large m onocular crescent eye m ovem ents but does not cause visual field (VF)
(which corresponds with the extrem e nasal retina) defect

50
 Anatom y of the Visual Pathway

Pupillary fibers pass through brachium of superior

colliculus to pretectal area, which innervates both
Edinger-Westphal subnuclei of CN 3
Optic tract provides retinal input to visual nucleus
(pulvinar) in the thalam us
Blood supply: anterior choroidal artery; branches from
Intra ocula r (dis c),
1 mm le ngth
posterior com m unicating artery

Later al gen icu late b o d y: part of the thalam us (Fig. 4-5)

Lower fibers lie laterally in optic tract and LGB (90° rotation
of fibers)
Crossed fibers (contralateral eye): project to layers 1, 4,
and 6
Intra orbita l,
25 mm le ngth
Ante rior communica ting a rte ry
Optic ne rve
Inte rna l
Intra ca na licula r, ca rotid
9 mm le ngth a rte ry

Intra cra nia l,

16 mm le ngth
Optic tra ct Chia s m

P os te rior
Ba s ila r a rte ry communica ting
a rte ry

Fig ure 4-4. Relationship of the optic chiasm, optic nerves, and optic tracts to the
arterial circle of Willis. The chiasm passes through the circle of Willis and receives
its arterial supply from the anterior cerebral and communicating arteries from
Fig ure 4-2. The four portions of the optic nerve. The lengths are given. (With above, and the posterior communicating, posterior cerebral, and basilar arteries
permission from Sadun AA: Anatomy and physiology. In Yanoff M, Duker J S (eds): from below. (Adapted from Reed H, Drance SM: The essentials of perimetry:
Ophthalmology, ed 2, St Louis, 2004, Mosby.) static and kinetic, ed 2, London, 1972,Oxford University Press.)

Circle of S hort pos te rior Optic ne rve S uba ra chnoid

Zinn–Ha lle r cilia ry a rte ry s he a th s pa ce

Optic ne rve

Eye P ia l va s cula ture

Ophtha lmic a rte ry

Ce ntra l a rte ry
Ophtha lmic ve in
a nd ve in
Fig ure 4-3. Anterior optic nerve. The sheath and the vascular supply to the intraocular and intraorbital portions are shown. (With permission from Sadun AA: Anatomy
and physiology. In Yanoff M, Duker J S (eds): Ophthalmology, ed 2, St Louis, 2004, Mosby.)

51
CHAPTER 4 • Neuro-ophthalm ology

m ay also be the only portion of VF spared after

6
occipital lobe dam age
5 Blood supply: m iddle and posterior cerebral arteries

K 4 Visu al asso ciatio n ar eas: areas 18 and 19

K
3
6
Oth er ar eas:
1 6 Ganglia:
2
1. CILIOSPINAL CENTER OF BUDGE: sympathetic fibers
from hypothalamus synapse; located at level C8 to T2
Fig ure 4-5. Lateral geniculate body section. The layers are numbered from 2. SUPERIOR CERVICAL: second-order sym pathetic fibers
ventral to dorsal in this posterior view. K fibers travel between the lamellae. (With synapse
permission from Lawton AW: Retrochiasmal pathways, higher cortical function, 3. CILIARY: sm all parasym pathetic ganglion; 1 cm from
and nonorganic visual loss. In Yanoff M, Duker J S (eds ): Ophthalmology, ed 2,
optic foram en between ON and lateral rectus m uscle
St Louis, 2004, Mosby.)
RECEIVES THREE ROOTS:
Long sensory: sensory from cornea, iris, and ciliary
Uncrossed fibers (ipsilateral eye): project to layers 2, 3, body
and 5 Short parasym pathetic (synapse): m otor to ciliary
Layers of LGB can also be categorized by neuronal size: body and iris sphincter
MAGNOCELLULAR NEURONS (M Cells): layers 1 and 2; Sym pathetic (do not synapse): conjunctival
subserve m otion detection, stereoacuity, and contrast vasoconstrictor fibers and iris dilator
sensitivity; project to layer 4C alpha of visual cortex 4. GENICULATE: traversed by CN 7; contains cell bodies
PARVOCELLULAR NEURONS (P Cells): layers 3 to 6; that provide taste from anterior two-thirds of tongue
subserve fine spatial resolution and color vision; 5. SPHENOPALATINE: parasym pathetic fibers to
project to layer 4C beta of visual cortex lacrim al gland
KONIOCELLULAR NEURONS (KCells): sit in interlam inar Horizontal gaze center: controls gaze to ipsilateral side;
zones and superficial layers; receive input from both located in param edian pontine reticular form ation
retinas and the superior colliculus; m ay m odulate (PPRF) at level of CN 6 nucleus; projects to ipsilateral CN
inform ation am ong other pathways 6 nucleus and (via m edial longitudinal fasciculus [MLF])
BLOOD SUPPLY: anterior com m unicating artery and to contralateral CN 3 nucleus (Fig. 4-6)
choroidal arteries Medial longitudinal fasciculus: extends from anterior
horn cells of the spinal cord to the thalam us; connects
CN 3 nuclei and gaze centers (ipsilateral CN 3 and
Op tic r ad iatio n : m yelinated nerve fibers; connect LGB to contralateral CN 6)
occipital cortex Vertical gaze center: originates in frontal eye fields or
Superior retinal fibers (inferior visual field [VF]): travel in in superior colliculus; requires bilateral cortical input;
white m atter underneath parietal cortex to occipital lobe projections travel to rostral interstitial nucleus of the MLF
Inferior retinal fibers (superior VF): travel around (riMLF) located behind red nucleus in m idbrain; fibers
ventricular system into tem poral lobe (Meyer’s loop); travel to nuclei of CN 3 and 4 (Fig. 4-7)
Meyer’s loop is around 5 cm from tip of tem poral UPGAZE (lateral portion of riMLF): stim ulates
lobe; tem poral lobe in jury causes incongruous CN 3 nucleus (superior rectus [SR] and inferior
hom onym ous superior quadrantanopia, or a ”pie-in- oblique [IO])
the-sky” VF defect DOWNGAZE (m edial portion of riMLF): stim ulates CN 3
Macular fibers: travel m ore centrally than do inferior retinal nucleus (inferior rectus [IR]) and CN 4 nucleus
fibers TORSIONAL MOVEMENTS: via interstitial nucleus
Blood supply: m iddle cerebral arteries of Cajal
Glial cells:
Pr im ar y visu al co r tex (striate cortex, V1, Brodm ann’s area OLIGODENDROCYTES: m yelination (begins at LGB and
17): m edial face of occipital lobe, divided horizontally by reaches lam ina cribrosa after birth)
calcarine fissure ASTROCYTES: support and nutrition
Visual cortex contains a topographic m ap of the MICROGLIAL CELLS: phagocytosis
contralateral hem ifield; central portion of VF is highly
m agnified
Macular region is posterior, extending slightly onto lateral P HYSIOLOGY
aspect of occipital lobe
Peripheral VF is located anteriorly along calcarine fissure
Te s t in g
Tem poral crescent in each VF (from 55° to 100°) is seen
only by nasal retina of ipsilateral eye; located m ost Co lo r visio n tests: Ishihara pseudoisochrom atic or Hardy-
anteriorly; only site posterior to chiasm that, if injured, Rand-Ritter plates; Farnsworth tests
would cause a m onocular VF defect; tem poral crescent Congenital defects: usually red/green

52
 Physiology

Le ft Me dia l Rig ht La te ra l
re ctus Me dia l re ctus
La te ra l re ctus
re ctus Oculomotor (III)
nucle us
Cra nia l
ne rve III Cra nia l
Cra nia l ne rve III Cra nia l
ne rve VI ne rve VI
Me dia l longitudina l
P a ra me dia n pontine fa s ciculus (MLF)
re ticula r forma tion
(P P RF)

Abduce ns (VI) nucle us

Ampulla

Horizonta l
s e micircula r
ca na l
Ve s tibula r
nucle us (VN)

Fig ure 4-6. Horizontal eye movement pathways . (With permission from Bajandas FJ , Kline LB: Neuro-ophthalmology review manual. Thorofare, NJ , 1998, Slack.)

Upg aze Fig u re 4-7. Pathways for vertical gaze. Upgaze

pathways originate in the rostral interstitial nucleus of the
Ros tra l inte rs titia l nucle us of the P os te rior Le s ion Le s ion Le s ion Le s ion medial longitudinal fasciculus and project dorsally to
me dia l longitudina l fa s ciculus commis s ure B A B C innervate the oculomotor and trochlear nerves, traveling
through the posterior commissure. Lesions to both axon
bundles are necessary to produce upgaze paralysis
(lesions B or C). Upgaze paralysis is a feature of the
dorsal midbrain syndrome as a result of the lesion’s
effect on the posterior commissure (lesion A). Downgaze
pathways also originate in the rostral interstitial nucleus
of the medial longitudinal fasciculus but probably travel
more ventrally. Bilateral lesions also are needed to affect
downgaze and usually are located dorsomedial to the
red nucleus. (With permission from Donahue SP, Lavin
PJ M: Disorders of supranuclear control of ocular motility.
In Yanoff M, Duker J S (eds): Ophthalmology. London,
P ons Re d IIIrd, IVth Re d nucle us 1999, Mosby.)
nucle us ce ntra l ne rve s

Do wng aze
Ros tra l inte rs titia l nucle us of the Inte rs titia l P os te rior Ce re bra l P e ria que ducta l
me dia l longitudina l fa s ciculus nucle us of Ca ja l commis s ure a que duct gra y

Le s ion Le s ion

P ons Re d Me dia l longitudina l IIIrd, IVth Re d

nucle us fa s ciculus ce ntra l ne rve s nucle us

53
CHAPTER 4 • Neuro-ophthalm ology

Acquired macular disease: m ay dim inish blue/yellow Op to kin etic n ystagm u s (OKN): presence suggests visual
in early stages (blue cones concentrated in input is present; slow phase is noted in direction of m oving
perifoveal ring) stim ulus
Fovea has m ostly red/green cones, so red/green defects are Parieto-occipital area controls slow pursuit, frontal lobe
detected in optic nerve diseases. Perception of red object controls saccades
indicates gross m acular function Pathway in visual association area term inates in ipsilateral
pontine gaze center, resulting in pursuit m ovem ents to
the sam e side (i.e., right visual association area controls
Ph o to str ess r eco ver y test: determ ine best-corrected pursuit to the right)
vision, shine bright light into eye for 10 seconds, record Can use to diagnose functional visual loss
tim e for vision to recover within 1 line of best-corrected Normal (symmetric) OKN response: occipital lobe, tem poral
vision; test each eye separately; invalid for eyes with vision lobe, LGB, or optic tract lesions do not interfere with
worse than 20/80 pursuit
Optic nerve disease: norm al recovery tim e (< 60 seconds) Deficient pursuit movements to side of lesion (asym m etric
Macular disease: prolonged tim e (> 90 seconds) OKN): parietal lobe lesion
Cogan’s dictum (for hom onom ous hem ianopia):
asym m etric OKN indicates parietal lobe lesion;
Co n tr ast sen sitivity: Pelli-Robson chart; Regan contrast sym m etric OKN indicates occipital lobe lesion
sensitivity chart; VectorVision chart Reversal of OKN response: 60% of patients with congenital
m otor nystagm us
Dorsal midbrain syndrome: downward m oving OKN drum
Visu ally evo ked co r tical p o ten tials/r esp o n ses
causes convergence–retraction nystagm us
(VEP, VER): m easure m acular visual function, integrity of
Congenital ocular motor apraxia: loss of voluntary
prim ary and secondary visual cortex, and continuity of optic
horizontal gaze (vertical gaze intact); abnorm al OKN
nerve and tract radiations; fovea has large area in occipital
(fast phase absent); m aintained tonic deviation; requires
cortex, close to recording electrodes; sm aller area
neuroim aging
representin g m ore peripheral retina lies deep within
calcarine fissure (Fig. 4-8)
Can m easure vision in preverbal infants Red glass test: evaluation of diplopia
Flash VER: strobe light
Pattern VER: checkerboard pattern or bar grating (am acrine Po ten tial acu ity m eter (PAM): projects im age of letter
and ganglion cell layer of retina) chart onto retina to test m acular potential in patients with
P100 wave: positive deflection at 100 m s; am plitude is m edia opacities
height from peak to trough, latency is tim e from onset of
flash to peak of wave Pu r kin je vascu lar p h en o m en a an d b lu e field en to p ic
Toxic or compressive optic neuropathies: reduction of
test: visualization of retinal vasculature; indicates gross
am plitude m ore pronounced than prolongation of
retinal function
latency
Demyelination: latency is prolonged; am plitude m ay be
only m ildly reduced Vis u a l fie ld d e fe c t s (Fig. 4-9)
Typ es:
Blind spot: physiologic due to ON; 15° tem poral to fixation
Am sler gr id : tests central 10° of the visual field (held at
and slightly below horizontal m idline
35 cm ); 10 cm  10 cm grid com posed of 5-m m squares;
Baring of blind spot: glaucom a, norm al patients
prim arily used to evaluate foveal pathology
Cecocentral scotoma: involves blind spot and macula
(within 25° of fixation); can occur in any condition that
P2 produces a central scotoma, dominant optic atrophy,
Leber’s optic atrophy, toxic/ nutritional optic neuropathy,
optic pit with serous retinal detachm ent, optic neuritis
Central scotoma: unilateral (optic neuritis, com pressive
P1 lesion of ON, retinal lesion [m acular edem a, disciform
scar]); bilateral (toxic optic neuropathy, nutritional
deficiency, m acular lesions)
mV
Arcuate scotoma: glaucom a, optic neuritis, anterior
ischem ic optic neuropathy (AION), branch retinal artery
occlusion (BRAO), branch vein occlusion (BVO), ON
N1 N2 drusen
ms e c Altitudinal defect: dam age to upper or lower pole of optic
Fig ure 4-8. Normal visual evoked cortical response. (Reprinted with permission
disc; optic neuritis, AION, hem iretinal artery or vein
from Slamovits TL: Basic and clinical science course. Section 12: Retina and occlusion
Vitreous . San Francisco, 1993, American Academy of Ophthalmology.) Spiraling of VF: suggests m alingering/functional visual loss

54
 Physiology

J unctiona l s cotoma He michia s ma l de fe ct

Bite mpora l de fe ct Ce ntra l bite mpora l

he mia nopia

Me ye r's loop
Incongruous right Le ft homonymous
homonymous he mia nopia horizonta l s e ctora nopia

Incongruous homonymous Right pa rie ta l

s upe rior qua dra nta nopia lobe le s ion
Fig ure 4-9. Visual field defects.

Pseudobitemporal hemianopia (slope and cross-vertical Neu r o lo gic VF d efects:

m eridian): uncorrected refractive error, tilted optic disc, Congruity of VF defect (retrochiasm al lesions): the m ore
enlarged blind spot (papilledem a), large central or congruous the defect, the m ore posterior the lesion
cecocentral scotom a, sector retinitis pigm entosa Superior field: anterior retinal ganglion cells ! lateral
(nasal quadrant), overhanging lid, colobom a portion of optic tract ! tem poral lobe (Meyer’s loop) !
Binasal defect: m ost nasal defects due to arcuate inferior bank of calcarine fissure
scotom as (glaucom a); also, pressure on tem poral Vision not reduced by unilateral lesion posterior to the
aspect of ON and anterior angle of chiasm , aneurysm , chiasm (20/20 acuity with m acula-splitting
pituitary adenom a, infarct hem ianopia)
Constricted field (ring scotom a): retinitis pigm entosa, Chiasm: pituitary tum or, pituitary apoplexy,
advanced glaucom a, thyroid-related ophthalm opathy, craniopharyngiom a, m eningiom a, ON gliom a,
ON drusen, vitam in A deficiency, occipital aneurysm , traum a, infection, m etastatic tum or, m ultiple
stroke, panretin al photocoagulation, functional sclerosis (MS), sarcoid
visual loss ANTERIOR CHIASMAL SYNDROME: lesion at junction of
Neurologic defect: bilateral and respects vertical m idline ON and chiasm ; involves fibers in Willebrand’s knee
(contralateral nasal retinal loop); causes junction al
scotoma (central scotom a in one eye and
Lo calizin g VF d efects: superotem poral defect in the other)
Nerve fiber layer: arcuate, papillom acular, BODY OF CHIASM: bitem poral hem ianopia; vision m ay
tem poral wedge be preserved
Chiasm: bitem poral hem ianopia (junctional scotom a if POSTERIOR CHIASM: bitem poral hem ianopia; prim arily
involving Willebrand’s knee) involves crossing m acular fibers
Optic tract: incongruous hom onym ous hem ianopia LATERAL COMPRESSION (Very rare): binasal
Temporal lobe (Meyer’s loop): ”pie-in-the-sky” (denser hem ianopia (m ore comm only caused by bilateral ON
superiorly, spares central) or retinal lesions)
Parietal lobe: denser inferiorly Optic tract: posterior sellar or suprasellar lesions;
Occipital lobe: congruous; Æm acular sparing hom onym ous hem ianopia, contralateral RAPD

55
CHAPTER 4 • Neuro-ophthalm ology

Retro-LGB lesions: 90% of isolated hom onym ous Vo luntary Purs uit
hem ianopias due to stroke FEM (volunta ry) S EM (purs uit)
Optic radiations: Fronto-me s e nce pha lic Occipito-me s e nce pha lic
TEMPORAL LOBE (Meyer’s loop): ”pie-in-the-sky” pa thwa y pa thwa y
(superior hom onym ous hem ianopia), form ed visual
hallucinations, seizures LE RE
PARIETAL LOBE: inferior hom onym ous hem ianopia,
hem iparesis, visual perception difficulty, agnosia,
apraxia, OKN asym m etry III III
GERSTMANN’S SYNDROME: lesion of dom inant parietal
lobe; acalculia, agraphia, finger agnosia, left–right
confusion, associated with inferior homonym ous
VI VI
hemianopia if optic radiation involved
Occipital lobe: P P Horizonta l
P P
HOMONYMOUS HEMIANOPIA WITH MACULAR R R s e micircula r
SPARING: suggests infarct in area supplied by F F ca na l
posterior cerebral artery; m acular region receives dual VN VN
supply from both m iddle cerebral artery and posterior
cerebral artery
BILATERAL CONGRUOUS CENTRAL ISLANDS WITH
VERTICAL STEP: equivalent to hom onym ous
hem ianopia with m acular sparing; vertical step does Ve s tibular
not occur in retinal or optic nerve lesions S EM (ve s tibula r)
CHECKERBOARD FIELD: bilateral incom plete La byrintho-pontine
hom onym ous hem ianopias, superior on one side and pa thwa y
inferior on opposite side (left upper and right lower Fig ure 4-10. Fast eye movement (voluntary), slow eye movement (SEM)(pursuit),
hom onym ous quadrant defects) and SEM (vestibular). Pathways all converge on paramedian pontine
BILATERAL HOMONYMOUS ALTITUDINAL reticular formation for horizontal eye movements. VN, vestibular nuclei. (With
DEFECTS: infarction or traum a to both occipital permission from Bajandas FJ , Kline LB: Neuro-ophthalmology review manual.
Thorofare, NJ , 1998, Slack.)
lobes, above or below calcarine fissure
MONOCULAR TEMPORAL CRESCENT DEFECT: an terior
occipital in farct; far tem poral field is seen by on ly olivopontocerebellar atrophy, Whipple’s disease,
on e eye Gaucher’s disease, MS, Pelizaeus-Merzbacher disease
Cortical blindness: bilateral occipital lobe destruction; Ocular motor apraxia: failure to initiate a saccade
pupillary response intact, blindsight (rudim entary
visual capacity), unform ed visual hallucinations,
Riddoch ph en om en on (perceive m oving targets but not Sm o o th p u r su it system : slow eye m ovem ents (SEM);
stationary ones); m ay deny blindness (An ton ’s following m ovem ents; ipsilateral parieto-occipital junction
syn drom e) (horizontal); interstitial nucleus of Cajal (vertical)
Tests: Doll’s head, rotation, OKN (pursuit m ovem ent)
Eye m o ve m e n t s u n d e r s u p ra n u c le a r c o n t ro l Abnormalities: dem yelination (young patients),
m icrovascular disease (older patients)
Ho r izo n tal gaze cen ter (Figs. 4-10 and 4-11):

Saccades: contralateral frontal eye fields (frontal lobe) ! Ver gen ce system : m aintains foveal fixation on
superior colliculus ! PPRF! horizontal gaze center approaching object; controlled by frontal and occipital
(CN 6 nucleus) ! ipsilateral lateral rectus (LR) and lobes, and possibly m idbrain
contralateral m edial rectus (MR) (via MLF) Types: voluntary, accom m odative, fusional
Smooth pursuit: ipsilateral parieto-occipital Test: look from distance to near
lobe ! superior colliculus (SC) ! PPRF! horizontal
gaze center ! ipsilateral LR and contralateral MR
(via MLF) Po sitio n m ain ten an ce system (vestibulo-ocular reflex
[VOR]): m aintains specific gaze position during head
Saccad ic system : generates fast eye m ovem ents (FEM) m ovem ents
(refixation); 300° to 700°/s Teleologically oldest eye m ovem ent system ; also fastest
Tests: refixation, rotation, calorics, and optokinetic (shortest latency)
nystagm us (fast saccadic return phase) Sem icircular canals ! CN 8 ! vestibular nucleus !
Abnormalities: progressive external ophthalm oplegia, contralateral horizontal gaze center ! extraocular
m yasthenia gravis, Wilson’s disease, Huntington’s m uscles (EOMs)
disease, ataxia-telangiectasia, spinocerebellar Test: calorics; rotation
degeneration, progressive supranuclear palsy, Abnorm alities cause oscillopsia

56
 Physiology

No n o p tic r eflex system s: integrate eye m ovem ents with

body m ovem ents
Are a 8 Norm al caloric and Doll’s head responses when nuclear and
Are a 19 internuclear connections are intact
Calorics: irrigate water into ears and observe induced
eye m ovements; m nem onic COWS (cold opposite,
warm sam e) refers to direction of fast phase of
nystagm us in awake patient (jerk nystagm us); in
FEM (volunta ry) S EM (purs uit) comatose patient, get tonic deviation in opposite direction
of m nem onic (sustained slow phase m ovement only
Ips ilate ral [Fig. 4-12])
Co ntralate ral Bilateral cold water irrigation produces nystagm us with fast
P P RF phase upward in awake patient and downward tonic
deviation in com atose patient; opposite effects with
warm water
S EM (ve s tibula r) PATHWAY: vestibular nuclei ! contralateral CN 6
nuclei ! ipsilateral LR and contralateral MR (via
MLF); no connection to the PPRF
Doll’s head (oculocephalic reflex): turn head and observe
direction of eye m ovem ents; vestibular system m oves
eyes; use when patient cannot voluntarily m ove eyes
Fig ure 4-11. Composite reminder of the course and lateralization of the three
conjugate horizontal eye movement pathways. (With permission from Bajandas
Eyes should have tonic m ovem ent in direction opposite
FJ , Kline LB: Neuro-ophthalmology review manual. Thorofare, NJ , 1988, Slack, head rotation
NJ , p 55.)

Fa s t Fa s t
S low S low

Le ft Me dia l Rig ht La te ra l
re ctus Me dia l re ctus
La te ra l re ctus
re ctus Oculomotor (III)
nucle us
Cra nia l
ne rve III Cra nia l
Cra nia l ne rve III Cra nia l
ne rve VI ne rve VI
Me dia l longitudina l
P a ra me dia n pontine fa s ciculus (MLF)
re ticula r forma tion
(P P RF)

Abduce ns (VI) nucle us

Ampulla

Horizonta l
s e micircula r
Ve s tibula r ca na l
nucle us (VN)

Wa rm wa te r or
Cold wa te r
right he a d turn

Fig ure 4-12. Vestibulo-ocular reflex demonstrating right beating nystagmus. Right COWS (cold opposite, warm same). (Calorics should be performed with
head back 60°.) (With permission from Bajandas FJ , Kline LB: Neuro-ophthalmology review manual. Thorofare, NJ , 1988, Slack.)

57
CHAPTER 4 • Neuro-ophthalm ology

INTACT DOLL’S HEAD: supranuclear lesion (cranial nerve Differ en tial d iagn o sis ( DDx) :
pathways to m uscles are intact) (i.e., progressive Supranuclear (due to inadequate convergence): skew
supranuclear palsy) deviation, progressive supranuclear palsy, Parkinson’s
ABNORMAL DOLL’S HEAD: lesion is infranuclear paresis disease, Huntington’s disease, dorsal m idbrain
or restrictive disease (perform forced ductions) syndrom e
Bell’s phenomenon: upward turning of eyes with forced Intermittent: MG, MS, migraine, thyroid-related
closure of eyelids ophthalmopathy, convergence spasm, decompensated
INTACT BELL’S PHENOMENON: supranuclear lesion phoria, convergence-retraction nystagmus, ocular myotonia
Vertical: MG, MS, thyroid-related, orbital disease (tum or,
traum a, inflam m ation), CN 3 or CN 4 palsy, Brown’s
syndrom e, skew deviation
Aberrant regeneration: Duane’s syndrom e, Marcus Gunn
DIP LOP IA jaw-winking syndrom e

Typ es: Restr ictive syn d r o m es: IOP elevation > 4 m m Hg when
Comitant: am ount of deviation sam e in all fields of gaze eyes directed into restricted field (thyroid, traum a,
Incomitant: am ount of deviation varies in different fields inflam m atory orbital disease, neoplastic process)
of gaze

Etio lo gy: EYE MOVEMENT DISORDERS

Monocular: high astigm atism , chalazion or lid m ass,
corneal abnorm ality, iris atrophy, polycoria, cataract, Ce ntra l Dis o rd e rs (Sup ra nuc le a r) (Fig. 4-13)
subluxed crystalline lens, decentered IOL, posterior
capsular opacity, retinal pathology, functional Often no sym ptom s or com plaints
Binocular: neuropathic (cranial nerve palsies, MS),
m yopathic (thyroid-related ophthalm opathy, orbital
pseudotum or), neurom uscular junction (MG)
Ho rizo nta l Ga ze P a ls ie s

Co n g e n it a l
S upe rior Must distinguish between supranuclear and infranuclear
Fronta l e ye fie lds colliculus P a rie ta l occipita l causes; often abnorm ality of CN 6 or interneurons; vertical
te mpora l junction m ovem ents usually unaffected

M o€ b iu s ’ s yn d ro m e
Horizontal gaze palsy with CN 6, 7, 8, and 9 palsies (facial
diplegia, deafness, abnorm al digits)

Oc u la r m o t o r a p ra xia
Saccadic palsy; im pairm ent of voluntary horizontal eye
m ovem ents with preservation of reflex m ovem ents;
m ale > fem ale; congenital or acquired (Balin t’s syn drom e);
Inte rs titia l extensive bilateral cerebral disease involving supranuclear
nucle us of Ca ja l pathways (usually bilateral frontoparietal); usually benign and
resolves in congenital disease
Oculomotor nucle us
Abduce ns nucle us
Trochle a r nucle us Asso ciatio n s: Gaucher’s disease, spinocerebellar
Ve s tibula r nucle us
degeneration, MR, ataxia-telangiectasia, Wilson’s disease,
Ros tra l inte rs titia l nucle us of the me dia l longitudina l fa s ciculus hypoplasia of corpus callosum , hydrocephalus; rarely
P a ra me dia n pontine re ticula r forma tion cerebellar m ass lesion (perform m agnetic resonance
im aging [MRI])
Fig ure 4-13. Supranuclear control of eye movements. The pontine horizontal
gaze center (blue) and the vertical gaze center in the midbrain (yellow) receive
input from the frontal eye fields to initiate saccades, and from the parietal occipital Fin d in gs:
temporal junction to control pursuit. These gaze centers control ocular motility by
synapsing upon the ocular motor nerve nuclei (III, IV, and VI). (With permission
Head thrusting: patient m ust m ove head to look at objects;
from Lavin PJ M, Donahue SP: Disorders of supranuclear controlof ocular motility. head thrust toward desired direction of gaze results in
In Yanoff M, Duker J S (eds): Ophthalmology, ed 2, St Louis, 2004, Mosby.) contralateral slow eye m ovem ent so patient m ust

58
 Eye Movem ent Disorders

overshoot target; lessens with age; m ay resolve by age 20; Dr u g-in d u ced : tricyclic antidepressants, phenytoin,
patient m ay also blink to break fixation phenothiazines
Abnormal OKN: fast phase absent
Abnormal vestibular nystagmus: fast phase absent
Normal pursuits
Pseu d o gaze p alsies: m yasthenia gravis, chronic
progressive external ophthalm oplegia (CPEO), Duane’s
Normal vertical saccades
syndrom e

Ac q u ire d
In tern uclear oph th almoplegia (INO) (Fig. 4-14): lesion of
Fr o n to p ar ietal lesio n (stroke, traum a, or infection): tonic MLF; inability to adduct ipsilateral eye with nystagm us of
deviation of eyes to side of lesion (contralateral area fellow eye; m ay have skew deviation, vertical diplopia, and
8 unopposed); seizure will m ove eyes away from lesion gaze-evoked, upbeat nystagm us; rule out m yasthenia gravis
Unilateral: ischem ia (young), dem yelination (older
Do ll’s h ead testin g an d calo r ics: can turn eyes patients), tum or, infection (m eningitis, encephalitis),
contralateral to lesion (intact vestibular pathway) traum a, com pression
Bilateral (involves both MLF near junction with the 3rd
Par ieto -o ccip ital lesio n : ipsilateral pursuit palsy nerve nucleus in m idbrain): demyelination/MS (m ost
(cogwheel pursuit) com m on), traum a, ischem ia, infection, Chiari
m alform ation, toxicity (am itriptyline, ethanol,
benzodiazepine)
Tegm en tal lesio n : ipsilateral pursuit and saccadic palsy Types:
ANTERIOR (m idbrain): preserved convergence
Po n tin e lesio n (PPRF): ipsilateral horizontal gaze palsy POSTERIOR (pons): im paired convergence
(WEBINO ¼ wall-eyed bilateral INO)
Par kin so n ’s d isease: reduced blinking, reduced saccades,
reduced glabellar reflex suppression, blepharospasm , On e-an d -a-h alf syn d r o m e (Fisher syndrom e)
oculogyric crisis (Fig. 4-15): lesion of CN 6 nucleus and ipsilateral MLF;
causes ipsilateral gaze palsy and INO; only m ovem ent is
Hu n tin gto n ’s ch o r ea: abduction of contralateral eye (with nystagm us); supranuclear
Metabolic disorders: hyperglycemia, Wernicke’s lesion therefore convergence intact; called paralytic pontine
encephalopathy, Wilson’s disease exotropia when patient appears exotropic; m yoclo patients

Le ft Me dia l Rig ht
re ctus Me dia l La te ra l
La te ra l re ctus re ctus
re ctus Oculomotor (III)
nucle us
Cra nia l
ne rve III Cra nia l
Cra nia l ne rve III Cra nia l
ne rve VI ne rve VI
1 Me dia l longitudina l
P a ra me dia n pontine fa s ciculus (MLF)
re ticula r forma tion 2
(P P RF)

Abduce ns (VI) nucle us

Ampulla

Horizonta l
s e micircula r
ca na l
Ve s tibula r
nucle us (VN)

Fig ure 4-14. 1. Right internuclear ophthalmoplegia (INO) 2. Bilateral INO. (With permission from Bajandas FJ , Kline LB: Neuro-ophthalmology review manual.
Thorofare, NJ , 1988, Slack.)

59
CHAPTER 4 • Neuro-ophthalm ology

Le ft Me dia l Rig ht
re ctus Me dia l La te ra l
La te ra l re ctus re ctus
re ctus
Cra nia l Cra nia l Cra nia l
Oculomotor (III)
Cra nia l ne rve III ne rve III ne rve VI
nucle us
ne rve VI

Me dia l longitudina l
fa s ciculus (MLF) 1/2 INO

P a ra me dia n pontine Po ntine

re ticula r forma tion 1 c o njug ate
(P P RF) rig ht g aze
pals y
Abduce ns (VI)
nucle us

Ampulla

Horizonta l
s e micircula r
ca na l
Ve s tibula r
nucle us (VN)

Fig ure 4-15. Right acute 1 1⁄2 syndrome (paralytic pontine exotropia). (With permission from Bajandas FJ , Kline LB: Neuro-ophthalmology review manual.
Thorofare, NJ , 1988, Slack.)

with recovery from 1 1/2 syndrom e m ay develop occulopalatal P ro g re s s ive s u p r a n u c le a r p a ls y

m yoclonus (S t e e le -Ric h a rd s o n -Ols z e w s k i s yn d ro m e )
Etiology: stroke (m ost com m on), MS, basilar artery
occlusion, pontine m etastases Progressive vertical (early) and horizontal (late) gaze palsy
(downward gaze usually affected first); no Bell’s
phenom enon; doll’s head m aneuver gives full range of
m ovem ent (ROM), indicating supranuclear nature of
Ve rtic a l Ga ze Ab no rm a litie s disorder; eventually frozen globe to all stim uli, spasm of
fixation, decreased blink rate, blepharitis, blepharospasm ;
P a rin a u d ’s s yn d ro m e occasionally, apraxia of eye opening; also, axial rigidity,
(Do r s a l m id b ra in s yn d ro m e ) dysarthria, and dem entia

Supranuclear gaze palsy with nuclear CN 3 palsy

S ke w d e via t io n
Etio lo gy: 90% due to pineal tum or; also, dem yelination,
infarction, traum a Vertical m isalignm ent of visual axes due to im balance of
prenuclear inputs; com itant or incom itant
Fin d in gs: supranuclear paresis of upgaze (Doll’s Vertical tropia, hyperdeviation usually increases on ipsilateral
h ead in tact), bilateral m iddilated pupils, con vergen ce- downgaze, no cyclodeviation; hypodeviated eye usually
retraction nystagm us on attem pted upgaze (synch ron ous ipsilateral to lesion, except when associated with INO in which
backward jerking m ovem ents of both eyes due hyperdeviated eye is ipsilateral; m ay occur with other brain
to cocon traction of horizontal recti), light-n ear stem sym ptom s or cerebellar disease; MRI of posterior fossa
dissociation, vertical O KN, skew deviation, lid retraction recom m ended
(Collier’s sign)

Diagn o sis: MRI; hum an chorionic gonadotropin Etio lo gy: brain stem infarct, MS, increased intracranial
(Hcg) level pressure (ICP), pseudotum or cerebri, vestibulo-ocular
im balance, cerebellar disease; vertebral–basilar insufficiency
Tr eatm en t: radiation therapy (XRT) m ay cause transient skew deviation

60
 Nystagm us

Tr eatm en t: often transient and requires observation only;

chronic deviations may be treated with prism spectacles or surgery Ac q uire d Nys ta g m us

Wh ip p le ’s d is e a s e Pattern helps localize pathology, m ay have oscillopsia

Oculom asticatory m yorhythm ia (vertical eye m ovem ents and
Bru n s ’
facial activity sim ilar to m yoclonus)
Com bin ation of gaze-paretic nystagm us when looking
Olivo p o n t o c e re b e lla r a t ro p h y toward lesion (fast phase toward the lesion), and vestibular
im balance nystagm us when lookin g away from lesion
Hereditary or sporadic; onset early adulthood; unsteady gait,
(fast phase away from lesion) (i.e., with right-sided lesion:
slurred speech, dem entia, optic atrophy, retinal degeneration
high-am plitude, low-frequency, right-beatin g nystagm us on
Eye m ovem ents progressively slow in all directions, finally right gaze; low-am plitude, high-frequency, left-beating
com plete external ophthalm oplegia nystagm us on left gaze)
Gaze-paretic com ponent is of high am plitude and low
Path o lo gy: cerebellar and pontine atrophy frequency (because of im pairm ent of horizontal gaze
m echanism on side of lesion)
Ke rn ic t e ru s
Vestibular com ponent is of low am plitude and high frequency,
Progressive loss of eye m ovem ents
with fast phase away from dam aged vestibular nuclei on side of
Also, m etabolic diseases (m aple syrup disease, Wernicke’s lesion
encephalopathy), drug-induced
Due to cerebellopontine angle m ass (usually acoustic neurom a
or m eningiom a)

NYSTAGMUS Co n ve rg e n c e -re t ra c t io n
Cocontraction of lateral recti produces convergence m ovem ent
Rhythm ic involuntary oscillations of the eyes due to disorder (abnorm al saccades) on attem pted upgaze
of SEM system . Direction nam ed after fast phase (brain’s
attem pt to correct problem ), even though abnorm ality is noted Due to periaqueductal gray m atter or dorsal m idbrain lesion
with slow phase (Parinaud’s syndrom e, pinealom a, traum a, brain stem
arteriovenous m alform ation [AVM], MS)
Etio lo gy: abnormal slow eye m ovement, high gain instability
(SEM is working at high gain), vestibular tone imbalance, Dis s o c ia t e d
integrator leak (gaze-evoked nystagmus), OKN abnorm ality
Asym m etric between the two eyes (different direction,
am plitude, frequency, etc.); always pathologic
Ch ar acter istics: m ay be fast or slow, pendular or jerk
(designated by direction of rapid phase), unidirectional or Due to posterior fossa disease, MS
m ultidirectional, sym m etric or asym m etric, congenital or
acquired Do w n b e a t
Gain: eye m oves 15° (output) in response to a retinal Eyes drift upward with corrective saccade downward; worsens
im age position error of 15° (gain ¼ 1) in downgaze, im proves in upgaze; oscillopsia
Foveation: bring an im age onto, or m aintain an im age on,
the fovea Due to lesion that affects pathways responsible for downgaze;
Frequency: oscillations per second (Hz) cervicom edullary junction lesion (Arnold-Chiari
Amplitude: excursion of oscillation m alform ation, tum or, syrinx: 33%), spinocerebellar
Intensity: product of am plitude and frequency degeneration, intoxication, lithium , paraneoplastic cerebellar
Null point: position in which intensity of nystagm us is least degeneration, 50% with no identifiable cause
Neutral point: position in which a reversal of the direction
of jerk nystagmus occurs Tr eatm en t: clonazepam

Ga ze -e vo k e d
Child ho o d Nys ta g m us
Nystagm us in direction of gaze, absent in prim ary position, fast
Most com m only, congenital, latent, sensory, and spasm us phase toward lesion (cerebellar)
nutans (see Chapter 5, Pediatrics/Strabism us) Due to extra-axial m ass com pressing brain stem (acoustic
neurom a, cerebellar hem isphere tum or), intoxication (alcohol
or anticonvulsant m edications)
P hys io lo g ic Nys ta g m us
If asym m etric, m ust obtain neuroim aging
Several form s of nystagm us, including end-gaze, optokinetic,
caloric, and rotational Wavefo r m : exponentially decreasing

61
CHAPTER 4 • Neuro-ophthalm ology

P e rio d ic a lt e rn a t in g n ys t a g m u s (P AN) Oc u la r d ip p in g
Changes horizontal direction; jerks right 90 seconds, 5- to Opposite of ocular bobbing; interm ittent rapid upward
10-second pause, jerks left 90 seconds, repeats; rem ains eye m ovem ents followed by slow return to prim ary position
horizontal in vertical gaze; usually acquired
Oc u la r m yo c lo n u s
Due to disease of vestibulocerebellar system (albinism, cranio-
cervico junction lesion [Arnold-Chiari malformation], MS, Vertical pendular nystagm us associated with synchronous
syphilis, tumor, vascular, dilantin, spinocerebellar degeneration) palatal (uvula) beating
Due to bilateral pseudohypertrophy of inferior olives in
Tr eatm en t: baclofen; surgery (large recession of all four recti m edulla, lesion in m yoclonic triangle (red nucleus, ipsilateral
m uscles) inferior olive, contralateral dentate nucleus)

Se e s a w Op s o c lo n u s (S a c c a d o m a n ia )
Vertical and torsional nystagm us with one eye rising and Rapid, chaotic eye m ovem ents in all directions; persists in sleep
intorting while fellow eye falls and excyclotorts, then reverses Associated with dancing hands and feet
Due to suprasellar lesions (can be associated with a visual Abnorm ality of pause cells (norm ally suppress burst cells
field defect, often bitemporal hemianopsia due to a of PPRF)
craniopharyngioma), cerebrovascular accident (CVA), or trauma
Due to rem ote, paraneoplastic effect on cerebellum
MRI: rule out large parasellar tum ors expanding into third from m etastatic neuroblastom a (check urine
ventricle vanillylm andelic acid [VMA]), occasionally with encephalitis

Tr eatm en t: baclofen Wavefo r m : no slow phase; repetitive, random ; no

intersaccadic interval
Up b e a t Os c illo p s ia
Eyes drift downward with corrective saccade upward Illusion of m ovem ent in the seen world
Due to m edullary lesions, m idline cerebellar (verm is) lesions,
Usually occurs in SO m yokym ia and acquired nystagm us;
m edulloblastom a, cerebellar degeneration, MS
rarely in congenital nystagm us

Ve s t ib u la r Due to vestibulo-ocular reflex abnorm ality

Horizontal, rotary, jerk nystagm us in prim ary gaze; sam e in all

fields of gaze; slow com ponent is linear; rem ains horizontal in CRANIAL NERVE P ALSIES (FIG. 4-16)
vertical gaze
Oc u lo m o t o r n e rve (CN 3 ) p a ls y
Due to vestibular disease, infection (labrynthitis), Menière’s
disease, vascular, traum a, toxicity An ato m y: nucleus in rostral m idbrain; fascicle travels
ventrally through m idbrain, traversing red nucleus, and
Per ip h er al vestib u lar d isease: fast phase toward good corticospinal tract in cerebral peduncle; nerve enters
side; slow phase toward lesion; associated with tinnitus, subarachnoid space, passes between posterior cerebral and
vertigo, deafness; fixation inhibits vertigo and nystagm us; superior cerebellar arteries, then courses lateral to posterior
direction of Rom berg fall changes with head turning com m unicating artery and enters lateral wall of cavernous
sinus; receives sym pathetics from internal carotid plexus;
Cen tr al vestib u lar d isease: nonlocalizing; no tinnitus or passes through superior orbital fissure and divides into
deafness superior (supplies SR and levator) and inferior (supplies IR,
MR, IO, iris sphincter, and ciliary m uscle) divisions.
Vo lu n t a ry Only one subnucleus (m idline location) supplies both levator
palpebrae superioris; fibers from superior rectus (SR)
Unsustainable for longer than 30 seconds; associated with
subnucleus supply contralateral SR; Edinger-Westphal nucleus
hysteria and m alingering
supplies both pupils (Fig. 4-18)

Typ es: com plete, partial, pupil sparing

Othe r Eye Mo ve m e nt Dis o rd e rs Fin d in gs: ptosis, hypotropia, exotropia; m ay involve pupil
(fixed and dilated); m ay cause pain
Oc u la r b o b b in g
Seven syn d r o m es (Fig. 4-17):
Interm ittent conjugate rapid downward eye m ovem ents
Nuclear CN 3 palsy (see Fig. 4-17, ): extrem ely rare;
followed by slow return to prim ary position
contralateral SR paresis and bilateral ptosis; pupil
Often secondary to hem orrhage; patient usually com atose involvem ent is both or neither

62
 Cranial Nerve Palsies

Le va tor S upe rior S upe rior Cilia ry Optic Inte rna l P os te rior Oculo- P os te rior S upe rior
pa lpe bra e oblique re ctus ga nglion ne rve (II) ca rotid communi- motor ce re bra l ce re be lla r
s upe rioris a rte ry ca ting ne rve (III) a rte ry a rte ry
a rte ry

Midbra in

Trochle a r
ne rve (IV)

P ons

Trige mina l
ne rve (V)

Me dulla

La te ra l Infe rior Infe rior Me dia l Ophtha lmic Ma xilla ry Ma ndibula r Ga s s e ria n Ba s ila r Abduce ns
re ctus oblique re ctus re ctus divis ion (V1 ) divis ion (V2 ) divis ion (V3 ) ga nglion a rte ry ne rve (VI)

Fig ure 4-16. Cranial nerve pathways. (Copyright Peter K. Kaiser, MD.)

Le va tor S upe rior Cilia ry Inte rna l P os te rior Edinge r-We s tpha l
pa lpe bra e re ctus ga nglion ca rotid communica ting nucle us
s upe rioris a rte ry a rte ry Re d nucle us

Corticos pina l
(pyra mida l)
tra ct
S upe rior
colliculus
Infe rior
1 colliculus
2

4
3

6 5

Infe rior Infe rior Me dia l Oculomotor

oblique re ctus re ctus nucle us
Fig ure 4-17. Seven syndromes of cranial nerve 3 palsy. (Copyright Peter K. Kaiser, MD.)

63
CHAPTER 4 • Neuro-ophthalm ology

Ce ntra l ca uda l nucle us Edinge r–We s tpha l s ubnucle us – Pupil-sparing isolated CN 3 palsy (see Fig. 4-17, ):
to bila te ra l le va tors to ips ila te ra l pupilla ry s phincte r sm all-caliber parasym pathetic pupillom otor fibers
To ips ila te ra l infe rior re ctus
travel in outer layers of nerve closer to blood supply
(but m ore susceptible to dam age by com pression); fibers
To ips ila te ra l infe rior oblique at core of nerve are com prom ised by ischem ia; m ay
explain pupil sparing in 80% of ischem ic CN 3 palsies
To ips ila te ra l me dia l re ctus and pupil involved in 95% of com pressive CN 3 palsies
(traum a, tum or, aneurysm )
ETIOLOGY:
ADULTS: vasculopathic/ischemic (diabetes mellitus
[DM], hypertension [HTN]), trauma, giant cell
Third ne rve arteritis (CGA), occasionally tumor, aneurysm
(very rare)
CHILDREN: congenital, traum a, tum or, aneurysm ,
Infe rior re ctus m igraine
To contra la te ra l Me dia l re ctus 14% of PCom aneurysm s initially spare pupil
s upe rior re ctus S upe rior re ctus 20% of diabetic CN 3 palsies involve pupil
Infe rior oblique Microvascular CN 3 palsies usually resolve in 3 to
Le va tor palpe bra e 4 m onths
Edinge r–We s tpha l s ubnucle us Myasthenia gravis m ay m im ic CN 3 palsy
Fig ure 4-18. Anatomy of the third nerve nucleus. The third nerve nucleus
consists of a single, central, caudally located nucleus for the levator palpebrae,
paired bilateral subnuclei with crossed projections that innervate the superior
recti, and paired bilateral subnuclei with uncrossed projections that innervate the Ab er r an t r egen er atio n : IR and/or MR fibers m ay
medial recti, inferior recti, and inferior oblique muscles. Parasympathetic input to innervate levator and/or iris sphincter; sign of previous CN 3
the ciliary body and iris sphincter arises from the Edinger-Westphal palsy caused by aneurysm or tum or (occasionally traum a);
nucleus. (Redrawn from Warwick R: Representation of the extraocular muscles
never occurs after vasculopathic injury
in the oculomotor nuclei of the monkey. J Comp Neurol, 1953, 98:449-503.)
Findings:
LID-GAZE DYSKINESIS: lid retracts on downgaze
Fascicle syndromes (see Fig. 4-17, ): ischem ic, infiltrative
(tum or), or inflam m atory (rare) (pseudo–von Graefe’s sign) and/or adduction
PUPIL-GAZE DYSKINESIS: m ore pupillary constriction
NOTHNAGEL’S SYNDROME: lesion of fascicle and
superior cerebellar peduncle that causes ipsilateral CN with convergence than to light (pseudo-Argyll-
3 paresis and cerebellar ataxia Robertson pupil) and/or pupillary constriction on
BENEDIKT’S SYNDROME: lesion of fascicle and red
downgaze
nucleus that causes ipsilateral CN 3 paresis, Two forms:
PRIMARY: no preceding acute CN 3 palsy; insidious
contralateral hem itrem or (resting and intentional),
and contralateral decreased sensation development of CN 3 palsy with accompanying signs of
WEBER’S SYNDROME: lesion of fascicle and pyram idal
misdirection; due to intracavernous lesion
tract that causes ipsilateral CN 3 paresis and (meningioma, aneurysm, neuroma)
SECONDARY: occurs m onths after CN 3 palsy from
contralateral hem iparesis
CLAUDE’S SYNDROME: com bination of Nothnagel’s and
traum a, aneurysm (carotid-cavernous [C-C]), or
Benedikt’s syndrom es tum or compression, but never after vasculopathic/
Uncal herniation (see Fig. 4-17, ): supratentorial m ass ischem ic lesion
m ay cause uncal herniation com pressing CN 3
Posterior communicating artery (PCom or PCA) aneurysm
(see Fig. 4-17, ): m ost com m on nontraum atic Oth er cau ses o f CN 3 p alsy:
isolated pupil involving CN 3 palsy; aneurysm at Congenital: variable degrees of aberrant regeneration; 75%
junction of PCom and carotid artery com presses nerve, have sm aller pupil on involved side (due to aberrant
particularly external parasym pathetic pupillom otor regeneration); ptosis and anisocoria
fibers; usually painful TREATMENT: m uscle surgery to straighten eye in
Cavernous sinus syndrome (see Fig. 4-17, ): associated prim ary gaze
with m ultiple CN palsies (3, 4, V1 , 6) and Horner’s; CN 3 Ophthalmoplegic migraine: onset in childhood; positive
palsy often partial and pupil sparing; m ay lead to family history of migraine; paresis of CN 3 occurs as
aberrant regeneration headache abates; usually resolves in 1 month (can be
Orbital syndrome (see Fig. 4-17, ): tum or, traum a, permanent)
pseudotum or, or cellulitis; associated with m ultiple CN Cyclic oculomotor palsy: usually at birth or early childhood;
palsies (3, 4, V1 , 6), proptosis, chem osis, injection; ON very rare; occurs after com plete CN 3 palsy, can be due to
can appear norm al, swollen, or atrophic diabetic ischem ia
After passing through the superior orbital fissure, CN 3 FINDINGS: spastic m ovem ents of m uscles innervated by
splits into superior and inferior divisions; therefore, CN 3 occur at regular intervals for 10 to 30 seconds
CN 3 palsies distal to this point m ay be com plete or (spasm s of lid elevation, adduction, m iosis)
partial MRI: rule out aneurysm

64
 Cranial Nerve Palsies

Wo r ku p : Etio lo gy: congenital (m ost com m on in kids); in adults:

If age < 11: MRI/MRA 40% traum a, 30% ischem ia, 20% m iscellaneous or
If age 11–50 years: MRI/MRA and m edical workup undeterm ined, 10% aneurysm
If age > 50 years:
WITH PUPIL INVOLVEMENT: MRI/MRA; if norm al, Fin d in gs: ipsilateral hypertropia (due to superior rectus
cerebral angiography (catheter) overaction)
WITH PUPIL SPARING: usually m icrovascular; no
invasive investigation needed initially; check blood
pressure (BP) (HTN), blood glucose (DM), Five syn d r o m es:
erythrocyte sedim entation rate (ESR), C-reactive Nuclear/fascicular syndrome (see Fig. 4-19, ):
protein (giant cell arteritis), com plete blood count hem orrhage, infarction, dem yelination, traum a;
(CBC), antinuclear antibody (ANA), Venereal Disease m ay have contralateral Horner’s or INO
Research Laboratory (VDRL), fluorescent treponem al Subarachnoid space syndrome (see Fig. 4-19, ): injury as
antibody absorption (FTA-ABS); if it persists nerve em erges from dorsal surface of brain stem ; traum a
> 3 m onths, MRI/MRA (m agnetic resonance (contracoup forces transm itted to brain stem by free
angiography) with cerebral angiography (aneurysm ) tentorial edge), tum or (pinealom a, tentorial
if negative m eningiom a), m eningitis, neurosurgical traum a
Cavernous sinus syndrome (see Fig. 4-19, ): m ultiple CN
palsies (3, 4, V1 , 6) and Horner’s
Tro c h le a r n e rve (CN 4 ) p a ls y Orbital syndrome (see Fig. 4-19, ): m ultiple CN palsies
(3, 4, V1 , 6) and Horner’s; proptosis, chem osis, injection
An ato m y: nucleus in caudal m esencephalon at level of Isolated CN 4 palsy (see Fig. 4-19, ):
inferior colliculus; decussates in anterior m edullary velum CONGENITAL: m ost com m on; m ay occur in elderly
next to aqueduct of Sylvius; fascicle passes between posterior due to decom pensation; large, vertical fusion
cerebral artery and superior cerebellar artery; nerve travels in am plitudes (10–15 Δ); check old photos for head tilt
lateral wall of cavernous sin us, enters orbit via superior ACQUIRED: acute onset of vertical diplopia; head
orbital fissure outside annulus of Zinn; innervates superior position (chin down, face turn to opposite side, head
oblique m uscle tilt to opposite shoulder)
Longest intracranial course (75 m m ); only CN that exits
dorsally from brain stem ; only CN that decussates (except DDx o f ver tical d ip lo p ia: m yasthenia gravis, thyroid-
for CN 3 subnucleus that innervates SR); m ost com m only related ophthalm opathy, orbital disease (tum or, traum a,
injured CN following closed head injury (owing to long inflam m ation), CN 3 palsy, CN 4 palsy, Brown’s syndrom e,
course) skew deviation, MS

S upe rior Inte rna l S e lla Clivus

oblique ca rotid turcica
a rte ry
Infe rior
colliculus

1 2
5 Trochle a r
ne rve (IV)
4 P ons
3

Me dulla

S upe rior Re gion of

orbita l ca ve rnous
fis s ure s inus
Fig ure 4-19. Five syndromes of cranial nerve 4 palsy. (Copyright Peter K. Kaiser, MD.)

65
CHAPTER 4 • Neuro-ophthalm ology

Diagn o sis: Parks-Bielschowsky three-step test (used for Second m ost com m on CN to be injured following closed head
hypertropia due to weakness of a single m uscle) injury
Step one: Which eye is hypertopic?
CN 6 lesions occur in conditions that cause increased ICP
LEFT HYPER: paresis of OD elevators (RSR or RIO) or
OS (oculus sinister, left eye) depressors (LIR or LSO)
Step two: Worse on right or left gaze? Six syn d r o m es (Fig. 4-20):
If hyper, is worse in left gaze: Brain stem syndromes (see Fig. 4-20, ):
Problem with OS m uscles that have greatest vertical MILLARD-GUBLER SYNDROME: lesion of CN 6 and 7
action during abduction (SR and IR) fascicles and pyram idal tract causing CN 6 and 7
Problem with OD m uscles that have greatest vertical palsies with contralateral hem iparesis
action during adduction (SO and IO) RAYMOND’S SYNDROME: lesion of CN 6 and
SR and IR work best when eye is ABducted pyram idal tract causing CN 6 palsy and contralateral
So if left hyper is worse on gaze left: LIR hem iparesis
SO and IO work best when eye is ADducted FOVILLE’S SYNDROME: lesion of CN 6 nucleus, CN 5 and
So if left hyper is worse on gaze left: RIO 7 fascicles, and sym pathetics causing ipsilateral 5, 6,
If L hyper is worse on gaze right: RSR or LSO and 7 palsies, horizontal conjugate gaze palsy, and
Step three: Worse on right or left head tilt? ipsilateral Horner’s
With head tilt, eyes undergo corrective torsion (with right €
MOBIUS’ SYNDROME: associated with CN 7 lesion,
head tilt, OD intorts and OS extorts) supernum erary digits, skeletal abnorm alities, m ental
If 1 of the intorters is the cause of the hyper, then tilting retardation
head will worsen hyper on side of head tilt Subarachnoid space syndrome (see Fig. 4-20, ):
If worse hyper on left head tilt, problem with left increased ICP can cause downward displacem ent of
intorters (LSR, LSO) or right extorters (RIR, RIO) brain stem with stretching of CN 6 (tethered at exit from
pons and Dorello’s canal); occurs in 30% of patients with
Bilater al CN 4 p alsies: usually due to severe head traum a pseudotum or cerebri; also hem orrhage, m eningitis,
(contusion of anterior m edullary velum ) inflam m ation (sarcoidosis), infiltration (lym phom a,
Vertical deviation in prim ary gaze m ay or m ay not be leukem ia, carcinom a)
present Petrous apex syndrome (see Fig. 4-20, ): portion of CN 6
RHT in left gaze; LHT in right gaze within Dorello’s canal is in contact with tip of petrous
Either eye can be hyper on step 3 of 3-step test pyram id and is susceptible to processes affecting the
V-pattern esotropia (ET > 25 Δ) petrous bone
GRADENIGO’S SYNDROME: localized inflam m ation or
> 10° of torsion with double Maddox rod test
extradural abscess of petrous apex (m astoiditis)
following otitis m edia; CN 6 palsy with ipsilateral
Wo r ku p : check BP, blood glucose, CBC, ESR, VDRL, decreased hearing, facial pain, and facial paralysis
FTA-ABS, ANA; neuroim aging if history of head traum a or PSEUDO-GRADENIGO’S SYNDROME: various etiologies
cancer, signs of m eningitis, young age, other neurologic NASOPHARYNGEALCA: obstruction of eustachian tube
findings, or isolated palsy that does not im prove after 3 to can cause serous otitis m edia, can invade
4 m onths cavernous sinus causing CN 6 palsy
CEREBELLOPONTINE ANGLE TUMOR: CN 5, 6, and 7
Tr eatm en t: palsies; decreased hearing, papilledem a
Glasses: occlude lens or prism s for diplopia PETROUS BONE FRACTURE: m ay have CN 5, 6, 7, or
Muscle surgery: 8 lesions, hem otym panum , Battle’s sign (bruising
KNAPP’S PRINCIPLES FOR CN 4 PALSY: strengthen (tuck) over m astoid bone), CSF otorrhea
the palsied SO, weaken the antagonist (ipsilateral IO), BASILAR ANEURYSM
or weaken the yoke (contralateral IR) to correct CLIVUS CHORDOMA
hyperdeviation Cavernous sinus syndrome (see Fig. 4-20, ):
HARADA-ITO PROCEDURE: anterior and lateral ETIOLOGY: traum a, vascular (diabetes, m igraine,
displacem ent of palsied m uscle to correct aneurysm s, arteriovenous [AV] fistula), tum or,
excyclotorsion inflam m ation, granulom a
FINDINGS: CN 3, 4, V1 , Horner’s, ON, chiasm , and
pituitary involvem ent
Ab d u c e n s n e rve (C N 6 ) p a ls y
Orbital syndrome (see Fig. 4-20, ):
An ato m y: nucleus in dorsal pons m edial to CN 7; travels ETIOLOGY: trauma, tumor, orbital pseudotumor, cellulitis
anterior and lateral to PPRF, then through pyram idal tract, and FINDINGS: proptosis, chem osis, conjunctival injection;
exits lower pons in pontom edullary groove in subarachnoid m ay have ON edem a or atrophy; CN V1 m ay be
space; clim bs over clivus (vulnerable to elevated ICP) over involved
petrous ridge, along base of skull, through Dorello’s canal, Isolated CN 6 palsy (see Fig. 4-20, ):
under Gruber’s ligam ent; enters cavernous sinus and then orbit CHILDREN: often postviral (resolves over weeks),
through superior orbital fissure; passes laterally to supply associated with otitis m edia; consider tum or (pontine
lateral rectus gliom a), traum a

66
 Cranial Nerve Palsies

La te ra l Inte rna l
re ctus ca rotid
a rte ry

P ons

5
6 4 1
3

2 Me dulla

S upe rior Re gion of Dore llo's P e troclinoid Abduce ns

orbita l ca ve rnous ca na l (Grube r's ) ne rve (VI)
fis s ure s inus liga me nt
Fig ure 4-20. Six syndromes of cranial nerve 6 palsy. (Copyright Peter K. Kaiser, MD.)

ADULTS: vasculopathic, undeterm ined, MS, tum or Diagn o sis: neuroim aging
(nasopharyngeal CA, cavernous sinus m eningiom a,
chordom a), traum a, GCA, syphilis
Tr eatm en t: medical (carbamazepine) or surgical
(radiofrequency destruction of trigeminal ganglion through
Pseu d o –CN 6 p alsy: thyroid-related ophthalm opathy, foramen ovale)
convergence spasm , strabism us, m edial wall fracture,
m yasthenia gravis, orbital m yositis, Miller Fisher syndrom e
Fa c ia l n e rve (CN 7 ) p a ls y
Wo r ku p : An ato m y: passes around CN 6 nucleus; exits brain stem
Congenital: usually resolves by 6 weeks ventrally at cerebellopontine angle; enters internal auditory
Acquired: sam e as for CN 4 palsy; consider lum bar canal (of petrous portion of tem poral bone) with nervus
puncture (LP) and Tensilon test interm edius and cochlear and vestibular nerves; enters facial
nerve canal; exits tem poral bone via stylom astoid foram en;
Trig e m in a l n e rve (CN 5 ) p a ls y branches in parotid gland. Supplies facial m uscles and lacrim al
and salivary glands; posterior two-thirds of tongue; external ear
An ato m y: nerve em erges from ventral pons; passes below sensation; dam pens stapedius
tentorium to ganglion; divides into 3 divisions (Fig. 4-21)
Lacrimal gland innervation (parasym pathetic): originates
Ophthalmic (V1 ): passes through lateral wall of
in superior salivatory nucleus, fibers leave brain with
cavernous sinus; divides into lacrim al, frontal, and
nervus interm edius (glossopalatin e) and travel with
nasociliary nerves
CN 7 through geniculate ganglion, em erges from
Maxillary (V2 ): passes through lateral wall of
petrous portion of sphenoid bone as greater superficial
cavernous sinus; exits cranium through foram en
petrosal nerve; enters pterygoid canal and enters
rotundum
sphenopalatine ganglion, where prim ary
Mandibular (V3 ): exits cranium through foram en ovale
parasym pathetic fibers synapse and second-order
Supplies sensory to face and eye, m otor to m uscles of
fibers join zygom atic nerve, which sends a branch to
m astication (Fig. 4-22)
lacrim al gland.
Frontal lobe (precentral m otor cortex) provides input to
Tr igem in al n eu r algia (tic douloureux): dueto compression CN 7 nuclei in pons (control voluntary facial
of CN 5 at root (superior cerebellar artery aneurysm or tumor); m ovem ents); upper face innervation is bilateral (from
facial pain involves entire CN 5 division; lasts seconds; 95% both supranuclear m otor areas), lower-face innervation
unilateral; usually involves maxillary or mandibular distribution, is m ainly from contralateral supranuclear m otor area
ophthalmic distribution alone is rare (Fig. 4-23)

67
CHAPTER 4 • Neuro-ophthalm ology

Cilia ry Inte rna l Optha lmic Ma xilla ry

ga nglion ca rotid ne rve (V1 ) ne rve (V2 )
a rte ry

P ons

Trige mina l
ne rve (V)

Me dulla

S upe rior Infra orbita l Fora me n Fora me n Ma ndibula r P e troclinoid

orbita l ne rve rotundum ova le ne rve (V3 ) liga me nt
fis s ure
Fig ure 4-21. Cranial nerve 5 pathway. (Copyright Peter K. Kaiser, MD.)

Somesthetic cortex (postcentral gyrus)

Thalamus, ventral posterior nucleus

Mesencephalic nucleus

Trigeminal lemniscus
Ophthalmic branch of V
Maxillary branch of V
Mandibular branch of V Main sensory nucleus of V
Trigeminal ganglion

Spinal nucleus of V

Fig ure 4-22. Cranial nerve 5 innervation.

68
 Cranial Nerve Palsies

Motor a re a , Motor a re a ,
fa ce fa ce

S upra -
nucle a r
le s ion
(uppe r
motor)

Fa cia l ne rve Fa cia l ne rve

nucle us nucle us
Corticobulba r Corticobulba r
tra ct tra ct

Lowe r motor
ne uron le s ion
Fa cia l Fa cia l
ne rve ne rve

Fa cia l pa ra lys is Fa cia l pa ra lys is

Fig ure 4-23. Facial weakness due to upper and lower motor neuron lesions. (With permission from Bajandas FJ , Kline LB: Neuro-ophthalmology review manual.
Thorofare, NJ , 1988, Slack.)

Su p r an u clear p alsy: lesion in precentral gyrus of Facial weakness progressing to paralysis over m onths
cerebral cortex results in contralateral paralysis of volitional Associated with progressive twitching or facial spasm ;
facial m ovem ent involving lower face m ore severely suggests neoplasm ; m ost com m on between ages 15
than upper and 45 years; 10% have positive fam ily history
TREATMENT: consider oral steroids
Em otional and reflex m ovem ents (sm iling, spontaneous PROGNOSIS: 70% have com plete recovery in 6 weeks;
blinking) are preserved (extrapyram idal) 85% will fully recover; 10% recurrence (ipsilateral or
contralateral)
Br ain stem lesio n (pons): ipsilateral facial weakness
POOR PROGNOSTIC SIGNS: com plete facial paralysis at
involving both upper and lower face; due to tum or, vascular
presentation, im pairm ent of lacrim ation,
causes
advanced age; if incom plete recovery, aberrant
Associated with CN 5 and 6 palsies, lateral gaze palsy,
regeneration is com m on
cerebellar ataxia, and contralateral hem iparesis
Trauma: fracture of tem poral bone
With CN 5 palsy: cerebellopontine angle tum ors,
FINDINGS: hearing loss, vertigo, hem otym panum ,
infratem poral fossa tum ors
perforated tym panic m em brane, Battle’s sign
With CN 6 palsy: brain stem injury or injury near
(bruising over m astoid bone)
anterom edial portion of tem poral bone (Gradenigo’s
Delayed onset or incom plete paralysis usually due to
syndrom e)
nerve contusion or swelling
Per ip h er al CN 7 lesio n : acute unilateral facial nerve palsy Com plete facial paralysis im m ediately after head traum a
is m ost com m on cranial neuropathy suggests nerve transection
Bell’s palsy: idiopathic facial nerve palsy; m ay be preceded BIRTH TRAUMA WITH FORCEPS: congenital CN 7 lesion;
by preauricular or m astoid pain tends to resolve

69
CHAPTER 4 • Neuro-ophthalm ology

Infection: Oth er fin d in gs: lacrim ation (dam age to greater superficial
RAMSAY-HUNT SYNDROME: herpes zoster virus (HZV) petrosal nerve), im paired stapedius m uscle reflex (dam age to
oticus (infection of outer ear and nerves of inner stapedial nerve), impaired taste (dam age to chorda tym pani
auditory canal); 20% have sensorineual hearing loss nerve), swelling of parotid gland or cervical lym phatics
and dizziness (suggests m alignant tum or or inflam m atory condition of
Preauricular or m astoid pain precedes facial paralysis parotid [sarcoidosis, TB])
by 1 to 3 days; associated with a vesicular rash of
outer ear or on tym panic m em brane Diso r d er s o f CN 7 o ver activity:
TREATMENT: acyclovir and prednisone Benign essential blepharospasm: frequent bilateral
PROGNOSIS: poor; only 10% recovery in patients with blinking proceeds to involuntary spasm s and forceful
com plete paralysis; 66% recovery with partial contractures of orbicularis; m ay cause functional
paralysis blindness; unknown etiology; usually affects wom en
May develop postherpetic neuralgia over age 50; absent during sleep
Also HIV, Lym e disease, otitis m edia, m alignant otitis TREATMENT: botulinum toxin (Botox) injections, rarely
externa (usually elderly diabetics; can progress to surgery (orbicularis m yom ectom y)
cellulitis of inner ear canal and osteom yelitis of tem poral Hemifacial spasm: unilateral contractions of facial m uscles;
bone; usually caused by Pseudomonas) usually due to vascular com pression of CN 7 at brain
Sarcoidosis: m ost frequent cause of bilateral 7th due to stem ; rarely caused by tum or; present during sleep;
infiltration of CN 7, usually at parotid gland obtain MRI
Erosive cholesteatoma: pressure on segm ent of CN 7 that Facial myokymia: fasciculations of facial m uscles; if
travels through m iddle ear m ultifocal and progressive, consider MS
Tumor: m ost intracranial and bone tum ors that cause Eyelid myokymia: benign fasciculations of eyelid
facial paralysis are benign (including acoustic
neurom a, m eningiom a, glom us tum ors [triad of facial
paralysis, pulsatile tinnitus, and hearing loss]); tum ors of Mu lt ip le CN p a ls ie s
parotid gland are usually m alignant (adenoid cystic
CN 3, 4, an d 5: due to lesion of brain stem , cavernous sinus
carcinom a)
(Fig. 4-24), and/or superior orbital fissure
Guillain-Barr e syndrome (Miller Fisher variant): facial
DDx: AV fistula, cavernous sinus throm bosis, m etastases to
diplegia can occur with ophthalm oplegia and ataxia;
cavernous sinus, skin m alignancy with perineural spread
absent deep tendon reflexes; CSF protein elevated with
to cavernous sinus, m eningiom a, m ucorm ycosis, HZV,
norm al cell count; often bilateral
Tolosa-Hunt syndrom e, m ucocele, nasopharyngeal CA,
Melkersson-Rosenthal syndrome: recurrent facial paralysis
carcinom atous m eningitis, pituitary apoplexy (headache
with chronic facial swelling and lingua plicata (furrowing
with bilateral signs and decreased vision)
of tongue); unilateral or bilateral; occurs in childhood or
adolescence
Aberrant facial innervation: Her p es zo ster o p h th alm icu s: CN 3, 4, and 6 palsies
MARCUS-GUNN JAW WINKING: activation of m uscles of occur in approxim ately 15%; pain and skin eruption in
m astication induces orbicularis oculi contraction trigem inal distribution, decreased corneal sensation, pupil
CROCODILE TEARS: lacrim ation evoked by chewing m ay be involved (tonic pupil)

Corona l view La tera l view

Pituita ry gla nd Sympa thetic Abducens Interna l ca rotid Sympa thetic

IIIrd cra nia l
plexus nerve a rtery plexus
nerve IIIrd cra nia l
nerve
IVth cra nia l
nerve
IVth cra nia l
O phtha lmic nerve
nerve
Ga sseria n
VIth cra nia l ga nglion
nerve
Ma ndibula r
nerve
Ma xilla ry
Interna l ca rotid nerve
a rtery Ma xilla ry nerve O ptic nerve

Fig ure 4-24. Anatomy of the cavernous sinus. (With permission from Moster M: Paresis of isolated and multiple cranial nerves and painful ophthalmoplegia. In: Yanoff M,
Duker J S (eds):Ophthalmology. London, 1999, Mosby.)

70
 Pupils

Hutchinson’s rule: if tip of nose involved (nasociliary Path way: posterior hypothalam us ! down spinal
nerve), eye will probably (but not always) be involved cord ! synapse in ciliospinal center of Budge (C8–T2 level) !
Mimics of multiple cranial nerve palsies: MG, CPEO, second-order neuron ascends sym pathetic chain ! over apex of
orbital lesions (thyroid, pseudotum or, tum or), lung ! synapse at superior cervical ganglion ! third-order
progressive supranuclear palsy, Guillain-Barre neuron ascends with ICA and joins CN 6 in cavernous
syndrom e sinus ! enters orbit via long ciliary nerve (through superior
orbital fissure next to CN V1 ) to iris dilator and Mu€ ller’s m uscle

P UP ILS Dis o rd e rs

Inne rva tio n (Fig. 4-25) Re la t ive a ffe re n t p u p illa ry d e fe c t (RAP D;

Ma rc u s -Gu n n p u p il)
Iris s p h in c t e r Large retinal lesion, asym m etric optic nerve disease, chiasm
lesion, optic tract lesion (contralateral RAPD)
Parasym pathetic innervation from Edinger-Westphal nucleus

No RAPD: cataract, acute papilledem a, am blyopia, refractive

Path way o f p u p illar y ligh t r eflex: optic nerve ! chiasm error, functional visual loss, lesions posterior to lateral
(fibers split) ! optic tract ! pretectal nucleus (synapse) ! geniculate body
cross to both EW nuclei (synapse) ! travel via CN 3 though
subarachnoid space and cavernous sinus, then travel in inferior
Lig h t -n e a r d is s o c ia t io n
division of CN 3 to ciliary ganglion ! postganglionic fibers
travel via short ciliary nerves to ciliary body and iris sphincter Pupil does not react to light but near response intact

Etio lo gy: syphilis (Argyll-Robertson), Adie’s pupil,

Iris d ila t o r
fam ilial dysautonom ia (Riley-Day syndrom e),
Sym pathetic innervation Parinaud’s syndrom e, RAPD, physiologic, severe retinal

Arous a l!
S phincte r
pupilla e Iris Optic tra ct
(Input from homonymous ACh
he mire tina s ) P re te cta l nucle us

NE
Inhibitory impuls e s ACh (Excita tory impuls e s )
P upil Hypotha la mus midbra in
Cilia ry ga nglion
Oculomotor ne rve Edinge r–We s tpha l nucle us
S hort cilia ry ne rve
ACh ACh Oculomotor nucle us
'P os tga nglionic ne uron' 'P re ga nglionic ne uron'
P a ra s ympa the tic pa thwa y 'Ce ntra l
Long cilia ry ne uron'
NE ne rve P ons

'P os tga nglionic S ympa the tic pa thwa y Ca rotid

ne uron' ple xus
S upe rior
Dila tor iridis ce rvica l
ga nglion
ACh
Ce rvica l cord
Cilios pina l
ce nte r (Budge )
Ce rvica l
C 8 – T1
s ympa the tic ACh
'P re ga nglionic ne uron'
ACh – Ace tylcholine
NE – Nore pine phrine

Fig ure 4-25. Parasympathetic and sympathetic innervation of the iris muscles. (With permission from Kardon RH: The pupils. In Yanoff M, Duker J S (eds):
Ophthalmology, ed 2, St Louis, 2004, Mosby.)

71
CHAPTER 4 • Neuro-ophthalm ology

disease, aberrant regeneration of CN 3, diabetes, m yotonic SYRINGOMYELIA: spinal cord cavities surrounded by
dystrophy, encephalitis, alcoholism , herpes zoster gliosis involving spinothalam ic tracts; causes
ophthalm icus (HZO) ipsilateral loss of pain/tem perature sensation but
preservation of touch; m uscle wasting and
Ab n o rm a l lig h t a n d n e a r re s p o n s e weakness (especially sm all hand m uscles);
Charcot’s arthropathy (35%)
Pharm acologic, traum a, CN 3 palsy Second-order neuron: m ajority of preganglionic Horner’s;
ciliospinal center of Budge to superior cervical ganglion
An is o c o ria ETIOLOGY: m ediastinal or apical tum or (neuroblastom a
[m ost comm on], Pancoast’s), thyroid disease,
Pupils of unequal size neurofibrom a, pneum othorax, cervical infections,
upper respiratory tract tum ors, brachial plexus
Mio sis: sm all pupil syndrom es, carotid artery dissection, aneurysm ,
DDx: Horner’s syndrom e, pharm acologic (pilocarpine, traum a
brim onidine, narcotics, insecticides), Argyll-Robertson
pupil, iritis, diabetes, spasm of near reflex, senescence
Po stgan glio n ic: superior cervical ganglion to iris dilator
Myd r iasis: large pupil Third-order neuron: anhidrosis lim ited to ipsilateral
DDx: CN 3 palsy, Adie’s tonic pupil, pharm acologic forehead
(m ydriatics, cycloplegics, cocaine), iris dam age (traum a, ETIOLOGY:
ischem ia, surgery [Urrets-Zavalia syndrom e]), INTERNAL CAROTID ARTERY DISSECTION: transient
Hutchinson’s pupil ischem ic attack (TIA), stroke, neck pain,
Both Horner’s and Adie’s are exam ples of denervation am aurosis, dysacusia, bad taste in m outh
hypersensitivity (sym pathetic tone loss in Horner’s, LESIONS INVOLVING MECKEL’S CAVE OR
parasym pathetic tone loss in Adie’s) CAVERNOUS SINUS: associated with CN 3, 4, V1 , V2 ,
and 6 palsies
TRIGEMINAL HERPES ZOSTER
Ho rn e r’s s yn d ro m e
HEADACHE SYNDROMES: m igraine, cluster headaches,
Sym pathetic lesion causing ptosis, m iosis, and anhidrosis; Raeder’s syndrom e (middle-aged m en with
anhidrosis often present in both first- and second-order Horner’s and daily unilateral headaches)
lesions; m ay also have facial num bness, diplopia, and vertigo; TUMOR OF PAROTID GLAND, NASOPHARYNX, SINUSES
m ay have m ild inverse ptosis of lower lid, dilation lag; TRAUMA
anisocoria m ost pronounced in dim light; congenital (forceps
injury, shoulder dystocia); can cause iris heterochrom ia
(ipsilateral lighter iris) Diagn o sis: pharm acologic testing
Cocaine test: topical cocaine (4%, 10%) blocks reuptake of
Pr egan glio n ic: from hypothalam us to superior cervical norepinephrine (NE), causing pupil dilation
ganglion Determ ines presence of Horner’s syndrom e
First-order neuron (central Horner’s): hypothalam us Functioning neuron will release NE, and pupil will
to spinal cord level C8-T2 (ciliospinal center of Budge) dilate
ETIOLOGY: ABNORMAL RESULT: no pupil dilation
MIDBRAIN: neuron located near CN 4 nucleus in Hydroxyamphetamine 1% (Paredrine) test: releases NE from
dorsolateral tegm entum ; lesion at this level nerve term inal
(usually ischem ia) causes Horner’s and superior Distinguishes between preganglionic and
oblique palsy postganglionic lesions
PONS: near CN 6 nucleus; ischem ia, tum or, POSITIVE IN PREGANGLIONIC LESIONS: pupil dilates
demyelination, polio, syringom yelia, and because postganglionic neuron is intact
inflam m ation, Foville’s syndrom e (lesion of CN 5, NEGATIVE IN POSTGANGLIONIC LESIONS: pupil does
6, and 7 and Horner’s) not dilate because neuron does not have NE
MEDULLA: lateral medullary syndrome of Wallenberg
(stroke of vertebral artery or posterior inferior
cerebellar artery (PICA) causing lateral medullary
Tr eatm en t: ptosis surgery to shorten M€u ller’s m uscle
(Putterm an procedure [conjunctival–M€ u ller’s m uscle
infarction; ipsilateral lesion of CN 9, 10, and 11;
resection], Fasanella-Servat procedure [tarsoconjunctival
Horner’s, vertigo, cerebellar signs, skew deviation,
resection])
ipsilateral decreased pain/temperature sensation of
face, contralateral decreased pain/temperature
sensation of trunk and limbs); no extremity weakness
Ad ie ’s t o n ic p u p il
VERTEBROBASILAR STROKES AND VERTEBRAL ARTERY
DISSECTION Dilated, tonic pupil due to postganglionic parasym pathetic
CERVICAL DISC DISEASE: arthritis, dem yelination, pupillom otor dam age; 90% wom en; 20–40 years of age;
inflam m ation, tum ors 80% unilateral

72
 Ocular Muscle Disorders

Fin d in gs: initially, pupil is dilated and poorly reactive; Myo t o n ic d ys t ro p h y (AD)
later becom es m iotic; segm ental contraction of pupil
Mapped to chrom osom e 19; m yotonia of peripheral m uscles;
(verm iform movem ents); light-near dissociation with slow
worsens with cold, excitem ent, fatigue
(tonic) redilation after near stim ulus
Fin d in gs: Christm as tree cataract (presenile cataract with
Ad ie’s syn d r o m e: Adie’s pupil and decreased deep tendon
polychrom atic subcapsular cortical crystals), m ild pigm entary
reflexes; orthostatic hypotension
retinopathy, ptosis, lid lag, light-near dissociation, m iotic
Path o lo gy: loss of ganglion cells in ciliary ganglion, pupils, m ay develop ocular hypotony
degenerated axons in short ciliary nerves
Oth er fin d in gs: myotonia, testicular atrophy, frontal
baldness, cardiacabnorm alities (myopathy, conduction defects),
DDx: HZV, giant cell arteritis (GCA), syphilis, orbital traum a,
bilateral facial weakness, insulin resistance, mental retardation
diabetes, alcoholism , and dysautonom ia associated with
cancer and am yloidosis
Diagn o sis: ERG (low voltage), electrom yogram (EMG)
(m yotonic discharge)
Diagn o sis: dilute pilocarpine (0.125%) or m echolyl 2.5%
will constrict the tonic pupil but not the norm al pupil; false-
In fla m m a t o ry d is o rd e rs
positive test can occur in CN 3 palsy
Thyroid-related ophthalm opathy, orbital pseudotum or,
Arg yll-Ro b e rt s o n p u p il am yloidosis
Bilateral sm all, irregular pupils with light-near dissociation
caused by tertiary syphilis Ot h e rs
Vitam in E deficiency, m uscle tum ors
Hu t c h in s o n ’s p u p il
Unilateral dilated, poorly reactive pupil in com atose patient
Ep is o d ic
due to ipsilateral supratentorial m ass causing displacem ent of Familial periodic paralysis, trauma, ischemia, vasculitis,
hippocam pal gyrus (uncal herniation) entrapping CN 3; disorders of neurom uscular junction (myasthenia gravis, Eaton-
pupillom otor fibers travel in peripheral portion of nerve and Lam bert syndrome, organophosphate poisoning, botulism)
are susceptible to early dam age from com pression
Mya s t h e n ia g ra vis (MG):
S im p le a n is o c o ria
Weakness of voluntary muscles due to autoimmunity to motor
Most com m on cause of relative difference (< 1 m m ) between
end plates (antibodies block ACh receptors), hallm ark is
pupils; occurs in up to 20% of general population
fatigability; 90% have eye symptoms, 50% present with eye
symptoms; females> m ales; onset aged 15–50; generalized
OCULAR MUSCLE DISORDERS disease will develop in 80% with ocular presentation will
develop generalized disease; 15% spontaneously resolve; does
not affect pupillary fibers
Op htha lm o p le g ia
Neo n atal fo r m : transplacental transfer of ACh receptor
S t a t ic antibody

Agenesis of extraocular m uscles, congenital fibrosis syndrom e, Co n gen ital fo r m : genetic defect in ACh receptor
congenital m yopathies
Typ es:
P ro g re s s ive Ocular: consists of ptosis and extraocular weakness only
Systemic: other skeletal m uscles involved; Graves’ disease
Ch ro n ic p ro g re s s ive e xt e rn a l
develops in 5% of patients
o p h t h a lm o p le g ia (CP EO)
Mitochondrial disease, several types Asso ciatio n s: thym ic hyperplasia (70%), thym om a (occurs
in 10% of MG patients; 30% of people with thym om a have
Kear n s-Sayr e syn d r o m e: ophthalm oplegia with ptosis, MG), other autoim m une diseases (rheum atoid arthritis, lupus,
retinal pigm ent degeneration, heart block (workup includes thyroid disease)
electrocardiogram [EKG])
Dr u gs th at exacer b ate MG: steroids, antibiotics
Oc u lo p h a ryn g e a l d ys t r o p h y (AD) (am inoglycosides, clindam ycin, erythrom ycin), β-blockers,
D -penicillam ine, phenytoin, curare, m ethoxyflurane, lidocaine
Onset in fifth to sixth decade; French-Canadian ancestry;
progressive dysphagia followed by ptosis;CPEO develops in most Fin d in gs: asym m etric ptosis, seesaw ptosis (“curtaining”;
when m ore ptotic lid is lifted, other lid falls owing to Hering’s
Path o lo gy: vacuolar m yopathy law), Cogan’s lid twitch (when patient looks from downgaze to

73
CHAPTER 4 • Neuro-ophthalm ology

prim ary position, lid will overshoot), facial m uscle weakness, S u p e rio r o b liq u e m yo kym ia
ophthalm oplegia, pseudo-INO, nystagm us (on extrem e gaze)
Episodic repetitive firing of SO causes interm ittent oscillopsia,
Oth er fin d in gs: jaw weakness, dysphagia, dysarthria, shim m ering vision, and vertical and/or torsional diplopia;
dyspnea, m uscle bulk usually preserved until late chronic; unknown etiology; treat with carbam azepine
(Tegretol) or propranolol; consider sim ultaneous weakening
DDx: Myotonic dystrophy, CPEO, involutional ptosis, toxins of ipsilateral SO and IO
(snake, arthropod, bacteria [botulism ])
Eaton-Lambert syndrome: paraneoplastic syndrom e Oc u la r n e u ro m yo t o n ia
consisting of profound proxim al m uscle pain and
Failure of m uscle to relax; occurs after radiation therapy to
weakness, dysarthria, dysphagia; associated with sm all
parasellar lesions; m ay affect CN 3 or 6; diagnose by having
cell lung cancer
patient look in direction of action of m uscle (up and in for
MECHANISM: im paired presynaptic release of ACh; 50%
CN 3, laterally for CN 6); treat with carbam azepine (Tegretol)
dem onstrate specific antibodies against presynaptic
voltage-gated calcium channels
DIAGNOSIS: m uscle strength and reflexes are enhanced Oc u lo g yric c ris is
with exercise; no im provem ent with edrophonium
Bilateral tonic supraduction of eyes and neck hyperextension
EMG: increased m uscle action potentials with repeated
due to acute effect of phenothiazine overdose; also described
nerve stim ulation
with postinfectious Parkinson’s disease
Diagn o sis:
Tensilon test (edrophonium [IV]; AChE inhibitor that
prolongs action of ACh, resulting in stronger m uscle EYE MOVEMENTS IN COMA
contraction): positive 80% to 90% of the tim e, but high
false-negative rate; m ay cause bradycardia—therefore, No rm a l e ye m o ve m e n t s a n d p u p ils
give test dose of 1 to 2 m g first; other adverse effects
include sweating, nausea, vom iting, salivation, fever, Bilateral cerebral depression
elevated IOP; antidote is atropine sulfate (0.5 m g);
alternative test is neostigm ine m ethylsulfate Lim it e d e ye m o ve m e n t s a n d n o rm a l p u p ils
(Prostigm ine [IM]: useful in children and adults without
ptosis, requires longer observation period Metabolic, drugs
[20–45 m inutes])
ACh receptor antibody: three types (binding Ab, blocking Ho rizo n t a l lim it a t io n
Ab, m odulating Ab); 50% sensitive in ocular MG, 80%
sensitive in general MG, 90% positive in MG with Eyes str aigh t: pontine dam age
thym om a, 99% specific; seronegative MG due to antigen
excess, ocular-specific Ab; Ab against other com ponents Eyes d eviated to sid e: cerebral dam age
of ACh receptor
Emg: fatigue with repetitive nerve stim ulation; single-fiber
Bilater al h o r izo n tal lim itatio n s (gaze palsy, INO, CN 6
EMG is best
palsy): pontine dam age
Photos: check old photos; m orning versus evening for
variability in lid position
Rest test: rest with eyes closed for 30 m inutes; positive if Ip silater al h o r izo n tal d eviatio n with co n tr alater al
im provem ent in ptosis occurs lim b weakn ess: basal ganglia dam age
Ice test: neurom uscular transm ission im proves in cold;
therefore, apply ice pack for 2 m inutes; positive if Co n tr alater al h o r izo n tal d eviatio n with o u t lim b
im provem ent occurs
weakn ess: cerebellar lesions
Others: chest CT/MRI, thyroid function and lupus tests

Tr eatm en t: pyridostigm ine (Mestinon [AChE inhibitor]), Ve rt ic a l lim it a t io n

steroids, plasm apheresis, IV gam m a globulin, cyclosporine Eyes str aigh t: m idbrain or pretectal dam age
(ciclosporin), azothioprine, thym ectom y (33% resolve, 33%
im prove, 33% no benefit); prism spectacles and consider
Up war d d eviatio n : hypoxic encephalopathy (severe
surgery for stable strabism us (> 6–12 m onths)
cerebellar dam age), lithium toxicity, phenothiazine overdose,
heatstroke
P rim a ry Ove ra c tio n Synd ro m e s
Do wn war d d eviatio n (m ore com m on and m ore
am biguous than upward deviation): pretectal, m etabolic,
Co n ve rg e n c e s p a s m
thalam ic hem orrhage; tentorial herniation; pineal tum ors;
Miosis with increasing esodeviation m eningitis; hepatic encephalopathy

74
 Optic Nerve

Ver tical-gaze p ar esis an d b ilater al CN 3 DDx o f o p tic d isc ed em a/p seu d o ed em a: papilledem a,
p alsy: m idbrain dam age ischem ic optic neuropathy, optic neuritis, central retinal vein
occlusion (CRVO), diabetic papillitis, m alignant hypertension,
neuroretinitis (cat-scratch disease), infiltration of the optic disc
To t a l o p h t h a lm o p le g ia (sarcoidosis, tuberculosis, leukem ia, m etastases), optic disc
Midbrain stroke, pituitary apoplexy, m eningitis drusen, optic nerve head tum ors (astrocytic ham artom a,
gliom as, capillary hem angiom a), orbital disease / com pressive
optic neuropathy (thyroid-related ophthalm opathy, orbital
S ke w d e via t io n pseudotum or, orbital m ass), inflam m atory diseases (syphilis,
acute posterior m ultifocal placoid pigm ent epitheliopathy
Posterior fossa dysfunction
[APMPPE], Vogt-Koyanagi-Harada [VKH] syndrom e), Leber’s
hereditary optic neuropathy
Bila t e ra l lid re t r a c t io n
Pretectal dysfunction P a p ille d e m a
Disc edem a due to increased intracranial pressure
Nys t a g m u s
Upbeat (structural), downbeat (hypoxic, Arnold-Chiari Fin d in gs: disc edem a (m ay be asym m etric); loss of
m alform ation, toxic [lithium ], paraneoplastic), convergence- spontaneous venous pulsations (absent in 20% of norm als);
retraction (dorsal m idbrain) transient visual obscurations (visual blackouts lasting
< 10 seconds associated with postural changes); diplopia (due
to unilateral or bilateral CN 6 palsies); norm al color vision,
Ca lo ric s acuity, and pupils; enlarged blind spot on VF; headache,
nausea, vom iting
Water irrigated into ear produces nystagm us; direction of Chronic cases: pale atrophic nerve, arteriolar attenuation,
nystagm us (fast phase) is COWS (cold opposite, warm sam e); poor vision, VF loss, refractile sm all hyaline bodies on
jerk nystagm us in awake patient; tonic deviation in com atose the disk
patient in opposite direction of m nem onic; bilateral cold water
irrigation produces upbeat nystagm us in awake patient;
downward tonic deviation in com atose patient DDx: intracranial tum or (posterior fossa in children),
m eningitis, carcinom atous m eningitis, pseudotum or cerebri
(IIH, see below)

OP TIC NERVE
Diagn o sis: check blood pressure; brain CT/MRI, if no m ass,
perform lum bar puncture (m easure opening pressure)
De ve lo p m e n t a l a n o m a lie s
(See Chapter 5, Pediatrics/Strabism us) Id io p a t h ic in t ra c ra n ia l h yp e rt e n s io n
(IIH; p s e u d o t u m o r c e re b ri)
Op t ic d is c s w e llin g Papilledem a with norm al neuroim aging and CSF
Nerve fibers anterior to lam ina cribrosa swell due to 90% fem ale, m ean age, 33 years old; associated with obesity
obstruction of axoplasm ic flow at level of lam ina choroidalis
or lam ina scleralis Fin d in gs: headache, nausea, vom iting, optic disc
Orthograde transport (ganglion cells to LGB): slow edema (may be asymm etric), transient visual obscurations,
com ponent ¼ 2 m m /day; fast com ponent ¼ 500 m m /day photopsias, VF defect (enlarged blind spot, constriction,
Retrograde transport (LGB to ganglion cells) or arcuate scotoma), retrobulbar pain, pulsatile intracranial
noises; m ay have visual loss, positive RAPD, diplopia
Mech an ism : ischem ia, inflam m ation, increased intracranial (CN 6 palsy)
pressure, com pression In children: irritability; no disc swelling if fontanelles open

Fin d in gs: elevated hyperem ic nerve head (3 diopters ¼ Asso ciatio n s: endocrine disorder (Addison’s; steroid
1 m m ), blurred disc m argins, loss of physiologic cup, withdrawal), chronic obstructive lung disease, throm bosis of
peripapillary nerve fiber layer (NFL) edem a, chorioretinal dural sinus (traum a, childbirth, m iddle ear infection,
folds, dilated / tortuous veins, peripapillary flam e-shaped hypercoagulable state); use of steroids, birth control pills,
hem orrhages, exudates, cotton wool spots (NFL infarcts) vitam in A, tetracycline, nalidixic acid, isotretinoin, lithium ,
am iodarone; m ay be exacerbated with pregnancy
Chronic swelling m ay cause Paton’s lines (radial or concentric
folds of peripapillary retina), gliosis, high water m arks, pale
disc, disc pseudodrusen, attenuated vessels, vision loss DDx: (see Optic Disc Swelling)

75
CHAPTER 4 • Neuro-ophthalm ology

Diagn o sis: norm al brain MRI, elevated CSF pressure with

Re s u lt s :
norm al com position (diagnosis of exclusion)
IV steroids: m ore rapid recovery of visual acuity at
Tr eatm en t: 2 weeks; did not reduce risk of subsequent attacks;
No vision loss: headache treatm ent and weight loss decreased incidence of MS at 2 years versus placebo,
Mild vision loss: acetazolam ide (Diam ox), furosem ide, but no difference at 3 years
weight loss Oral prednisone: associated with higher rate of new
Advanced VF loss: ON sheath decom pression, serial lum bar attacks in either eye at 1 year; highest relapse rate of
punctures (tem porary relief), lum bar–peritoneal shunt optic neuritis at 5 years (41% vs. 25% for IV and
placebo groups)
Op t ic n e u r it is Brain MRI: periventricular white m atter lesions were
Inflam m ation of optic nerve; idiopathic or associated with associated with MS
system ic disease; m ost com m on optic neuropathy in people Lesions in 50% of patients with first attack of optic
< 45 years old; fem ale preponderance neuritis
5-YEAR RESULTS: 30% risk for MS regardless
Fin d in gs: acute unilateral visual loss (can evolve over 1st of treatm ent group
week, then spontaneous im provem ent over weeks [70% WITH NO LESIONS: 16% developed MS

regain 20/20]), ocular pain exacerbated by eye m ovem ents, 3 OR MORE LESIONS: 51% developed MS

decreased color vision and contrast sensitivity, positive Increased risk of developing MS if previous neurologic
RAPD, VF defect (50% diffuse, 20% central scotom a, rarely sym ptom s
altitudinal), optic nerve appears norm al or swollen (35%), disc (regardless of MRI status)
pallor late 10-YEAR RESULTS:
WITH NO LESIONS: 22% developed MS
Oth er fin d in gs: 1 OR MORE LESIONS: 56% developed MS
Pulfrich phenomenon: m otion of pendulum appears If normal MRI, no previous neurologic findings or
elliptical owing to altered depth perception from delayed fellow eye involvement: decreased risk of MS if optic
conduction in the dem yelinated nerve neuritis was painless, if disc swelling was present,
Uhthoff’s symptom: worsening of sym ptom s with heat or and vision loss was only m ild
exercise; present in 50% after recovery No patients developed MS wh o h ad norm al MRI with
Phosphenes: flashes of light induced by eye m ovem ents peripapillary hem orrhage or m acular exudates
or sound Abnormal MRI: 43% progress to MS by 3 years;
Auditory clicks: CN 7 innervates stapedius, and patient progression to MS at 1 year was 7.5% with IV
hears click with effective transm ission steroids, 14.7% with oral steroids, and 16.7% with
placebo
DDx: idiopathic, MS, syphilis, sarcoidosis, Lyme disease,
Wegener’s granulomatosis, systemic lupus erythematosus (SLE), Co n c lu s io n s :
Devic’s syndrome (bilateral optic neuritis and transverse myelitis)
In general, about 30% develop MS within 4 years and
Tr eatm en t: 38% develop MS by 10 years (risk is higher in wom en
Controlled High-risk Avonex Multiple Sclerosis Prevention than m en)
Study (CHAMPS): random ized trial of interferon beta-1a Consider treating optic neuritis with IV steroids; do not
(Avonex) for the prevention of MS after acute initiate treatm ent with oral steroids
dem yelinating episode and abnorm al MRI
3-YEAR RESULTS: risk of MS lower and fewer MRI lesions Regardless of MRI status, there is a higher risk for MS if the
in treatm ent group patient has previous neurologic sym ptom s or a history of
optic neuritis in the fellow eye

MAJ OR CLINICAL STUDY

Op tic Ne uritis Tre a tm e nt Tria l (ONTT)

Ob je c t ive : to evaluate the role of corticosteroids in the Op tic Ne u ro p a thie s

treatm ent of unilateral optic neuritis
Me t h o d s : patients with unilateral optic neuritis were An t e rio r is c h e m ic o p t ic n e u ro p a t h y (AION)
random ly assigned into 3 treatm ent groups: Infarction of optic nerve head just posterior to lam ina cribrosa
IV steroids: 250 m g IV m ethylprednisolone qid  3 days, due to inadequate perfusion by posterior ciliary arteries; results
followed by oral prednisone (1 m g/kg/day) Â 11 days in acute visual loss
Oral steroids: prednisone (1 m g/kg/day) Â 14 days
Oral placebo: 14 days Two fo r m s: arteritic (giant cell arteritis); nonarteritic
(ischem ic)

76
 Optic Nerve

Fin d in gs: decreased color vision and acuity, positive RAPD, Sm okers had earlier m ean onset (age 64) than
VF loss (altitudinal or arcuate defects m ost com m on, central nonsm okers (age 70)
scotom as also occur), unilateral optic disc edem a (often 43% of control patients regained three or m ore lines of
involving one sector), contralateral disc appears crowded vision at 6 m onths (vs. 34% of those having surgery)
(sm all C/D ratio) Surgery conferred higher risk of loss of three lines of
vision (24% vs. 12% with observation)
Ar ter itic: due to giant cell (temporal) arteritis (inflammatory
vasculopathy affecting medium-sized to large vessels) affecting
the posterior ciliary arteries; fem ale > m ale (2:1); aged older Pseu d o -Fo ster -Ken n ed y syn d r o m e: AION is the m ost
than 55 years; m ay have amaurosis fugax or diplopia frequent cause of unilateral disc edem a and contralateral
Other findings: scalp tenderness, jaw or tongue optic atrophy; disc not hyperem ic as in true Foster-Kennedy
claudication, polym yalgia rheum atica (PMR), fever, syndrom e
m alaise, anorexia, weight loss, anem ia, headache, tender
tem poral artery, neck pain, brain stem stroke (due to Re t ro b u lb a r o p t ic n e u ro p a t h y (P o s t e rio r
involvem ent of vertebral artery); cotton wool spots, is c h e m ic o p t ic n e u ro p a t h y)
choroidal ischem ia (seen as patchy choroidal filling on
fluorescein angiography (FA)) Rare; usually bilateral; occurs with severe anem ia and
Pathology: granulom atous inflam m ation with epithelioid hypotension (i.e., m ajor blood loss from surgery, traum a, GI
cells, lym phocytes, giant cells; disruption of internal bleed, dialysis); associated with m edications (antibiotics
elastic lam ina and proliferation of intim a with aneurysm [etham butol, isoniazid, sulfonam ides, chloram phenicol],
form ation (Fig. 4-26) anticancer drugs [cisplatin, vincristine, busulfan])
Diagnosis: elevated ESR (for m en > [age/2]; for wom en >
[(age +10)/2]; m ay be norm al in 10%), C-reactive Fin d in gs: disc swelling m ay occur if ischem ic process
protein (above 2.45 m g/dL), low hem atocrit (anem ia of extends anteriorly
chronic illness), FA (patchy choroidal filling,
nonperfusion), tem poral artery biopsy (inflam m ation in
Tr eatm en t: treat acute cause (prom pt reversal of
artery wall with disruption of internal elastica lam ina,
hypotension, blood transfusion)
skip lesions [specim en at least 3 cm ], perform within
2 weeks of steroid treatm ent)
Treatment (em ergent): steroids (prednisone 60–120 m g Co m p r e s s ive o p t ic n e u ro p a t h y
orally; consider IV initially [1 g for 3 days]) to prevent Intraorbital, intracanalicular, or intracranial (prechiasm al)
fellow eye involvem ent (65% risk of involvem ent of
fellow eye without treatm ent; usually affected within
10 days); som e patients lose vision despite treatm ent Etio lo gy: ON tum or, pituitary tum or or apoplexy, thyroid-
related ophthalm opathy
No n ar ter itic (NAION) (ischem ic): no associated
sym ptom s; usually aged 50 to 75 years; associated with
m icrovascular disease (diabetes, hypertension) and collagen Fin d in gs: slow progressive vision loss, decreased color
vascular disorders; recurrence in sam e eye is rare; 25% to 40% vision, positive RAPD, VF defect (central scotom a that extends
risk of fellow eye involvem ent; norm al ESR: NAION m ay be to periphery), proptosis; disc m ay be norm al, pale, or swollen;
m im icked by am iodarone and phosphodiesterase-5 inhibitors m ay have endocrine abnorm alities (parachiasm al lesions),
chem osis and restricted m otility (thyroid)
Treatment: none
ISCHEMIC OPTIC NEUROPATHY DECOMPRESSION TRIAL
(IONDT): optic nerve sheath fenestration is not Diagn o sis: orbit CT/MRI
effective
In filt ra t ive o p t ic n e u ro p a t h y
Etio lo gy: leukem ia, lym phom a, m ultiple m yelom a,
plasm acytom a, m etastatic carcinom a (breast and lung m ost
com m on), sarcoidosis, TB, cryptococcus, toxoplasm osis,
toxocariasis, CMV, coccidiom ycosis

Fin d in gs: disc m ay appear grayish-white with associated

hem orrhages; m ass m ay be visible

To xic / Nu t rit io n a l o p t ic n e u r o p a t h y
Painless sym m etric progressive vision loss

Fig ure 4-26. Arteric anterior ischemic optic neuropathy demonstrating

Etio lo gy: ethanol, m ethanol, digoxin, etham butol,
vasculitis ofallcoats of temporal artery with giant cells. (Courtesy of MM Rodrigues. chloram phenicol, isoniazid, rifam pin, lead toxicity, tobacco-
From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.) alcohol am blyopia, thiam ine and B12 deficiency, cisplatin

77
CHAPTER 4 • Neuro-ophthalm ology

Fin d in gs: decreased vision (20/200 or worse), VF loss

(cecocentral scotom as), disc hyperem ia then pallor

Tra u m a t ic o p t ic n e u ro p a t h y
ON contusion or com pression due to traum a

Fin d in gs: decreased vision, VF defects, positive RAPD

CT scan : rule out canal fracture, orbital hem atom a, ON

hem atom a

Tr eatm en t: lateral canthotom y for tense orbit, drain

subperiosteal hem atom a
IV steroids (m ethylprednisolone): 30 m g/kg loading dose, Fig ure 4-27. Glioma demonstrating central necrosis. (With permission from
then 5.4 m g/kg/h  48 hours Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.)
If vision improves, switch to 80 mg oral, and taper by 20 mg
every 2 to 3 days; if vision worsens on oral steroids, restart
high-dose IVand consider surgical decompression of canal
Consider surgical decom pression of optic canal if no
response to steroids after 48 hours
If no im provem ent in vision after 3 days, stop steroids

He re d it a ry o p t ic n e u ro p a t h y
(see Chapter 5, Pediatrics/Strabism us)

Op t ic a t ro p h y
Pale white appearance of ON head caused by injury to any part
of pathway from retinal ganglion cells to LGB

Etio lo gy: optic neuropathy, optic neuritis, glaucom a, central

retinal artery occlusion (CRAO), tum ors, aneurysm s Fig ure 4-28. Meningioma demonstrating proliferation of meningothelial
cells. (With permission from Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis,
2002, Mosby.)
Path o lo gy: increased num ber of astrocytes (gliosis) within
nerve; norm al parallel architecture of glial colum ns seen in
sagittal sections is lost; widened space between nerve and ON m e n in g io m a
m eninges; loss of m yelin; thickened pial septa Derived from outer arachnoid; m ay arise from ectopic nests of
m eningeal cells; fem ale > m ale (3:1)

ON Tum o rs Fin d in gs: triad of decreased vision, optic atrophy,

optociliary shunt vessels; also proptosis, ON edem a, retinal
ON g lio m a (Fig. 4-27) vascular occlusions

Lo w-gr ad e astr o cyto m a: usually in children aged < 10 Sp h en o id win g m en in gio m a: fullness of tem poral
”Benign” but 14% m ortality rate (highest with hypothalam us orbital fossa and orbit
involvem ent)
10% to 50% have neurofibrom atosis (15% of patients with NF Path o lo gy: psam m om a bodies and whorls of concentrically
have ON gliom as) packed spindle cells (Fig. 4-28)

50% intraorbital, 50% intracranial

CT/MRI: railroad track sign (due to calcification)
Findings: vision loss, strabism us, nystagm us, disc edem a or
atrophy; m ay have proptosis (intraorbital ON gliom a);
can spread to chiasm and contralateral ON
MRI: fusiform thickening and kinking of ON CHIASM COMP RESSION

Malign an t ON glio m a (glioblastom a m ultiform e): occurs Ch iasm an ato m y: location with respect to pituitary gland
in adults Prefixed: chiasm is forward; pituitary tum or will com press
Painful vision loss; becom es bilateral; death in 3 to 9 m onths the optic tracts

78
 Retrochiasm al Disorders

Normal: chiasm is aligned with pituitary; pituitary tum or S h e e h a n ’s s yn d ro m e

will com press chiasm
Panhypopituitarism in pregnancy due to hem orrhage
Postfixed: chiasm is behind; pituitary tum or will com press
optic nerves
Cra n io p h a ryn g io m a
Etio lo gy: pituitary tum or (m ost com m on), pituitary Usually causes com pression of chiasm from above and behind;
apoplexy, craniopharyngiom a, m eningiom a, gliom a, occurs in children and young adults
aneurysm , traum a, infection, m etastasis, MS, sarcoidosis
Fin d in gs: initial dam age to upper nasal fibers (inferior
VF d efects: central scotom a, bitem poral hem ianopia, bitem poral field defect); later, upper tem poral defect
incongruous hom onym ous hem ianopia, junctional scotom a papilledem a, optic atrophy
(ipsilateral central scotom a with contralateral superotem poral
defect due to lesion at junction of ON and chiasm ) MRI: calcification

DDx o f b item p o r al VF d efect: sector retinitis DDx o f calcificatio n o f sella

pigm entosa, colobom a, tilted disc tu r cica: craniopharyngiom a, supraclinoid aneurysm ,
m eningiom a, AV m alform ation, gliom a of chiasm (very rare),
chordom a (extrem ely rare)
P it u it a ry t u m o r
Most com m only chrom ophobe (often prolactinom a); can also Glio m a
secrete adrenocorticotropin (ACTH) (Cushing’s disease);
Occurs in children and adults; m ay extend into both optic
basophil can secrete ACTH; eosinophil can secrete growth
nerves causing bilateral vision loss with com plicated bilateral
horm one (GH) (acrom egaly); secreting tum ors usually present
VF defects
with endocrine dysfunction, except prolactin-secreting tum or
in m ales (presents with signs of chiasm al com pression); 30%
nonsecreting (present with vision loss) An e u rys m
ICA and anterior cerebral artery; m iddle-aged adults; present
Fin d in gs: VF defect (bitem poral hem ianopia), horm one with vision loss, ophthalm oplegia
im balance, optic atrophy
S u b fro n t a l m a s s le s io n
Diagn o sis: X-ray shows large pituitary fossa, double floor Slow progressive asym m etric bilateral visual loss, late
sign, bony erosion ON pallor

Tr eatm en t: dopam ine antagonist (brom ocriptine) for Ot h e r c h ia s m a l le s io n s

prolactinom a, horm one replacem ent, transsphenoidal surgery
MS, traum a, basal m eningitis, sphenoid sinus m ucocele or
for nonsecreting tum or, XRT
carcinom a

Me n in g io m a
Derived from m eningothelial cells of arachnoid; often at RETROCHIASMAL DISORDERS
sphenoid ridge; occurs in adults, especially m iddle-
aged wom en Cause hom onym ous VF defects (see Fig. 4-9)

Tu b er cu lu m sellae m en in gio m a: com pression of optic Co gan ’s Dictu m : causes hom onym ous hem ianopia
nerve just before chiasm , m ay cause unilateral VF defect With asym m etric OKN ¼ parietal lobe lesion (usually m ass)
Findings: optic atrophy, hyperostosis With sym m etric OKN ¼ occipital lobe lesion (usually
infarction)
Path o lo gy: whorled cellular pattern; m ay see psam m om a
bodies Op t ic t ra c t
Vascular, craniopharyngiom a extending posteriorly
P it u it a ry a p o p le xy
Acute hem orrhage and expansion of a pituitary tum or, usually Fin d in gs: incongruous hom onym ous hem ianopia,
secondary to ischem ic necrosis bilateral optic atrophy (bow-tie appearance),
contralateral RAPD

Fin d in gs: headache, decreased vision, ocular m otility

La t e ra l g e n ic u la t e b o d y
disturbance; progresses to no light perception (NLP) and
com plete ophthalm oplegia Very rare

79
CHAPTER 4 • Neuro-ophthalm ology

Fin d in gs: incongruous hom onym ous hem ianopia or To read, inform ation travels from visual cortex to angular gyrus
congruous hom onym ous sectoranopia (sectoral optic in parietal lobe of dom inant hem isphere (usually on left side);
atrophy), norm al pupils visual inform ation from right hem ifield is transm itted directly
from left occipital lobe to ipsilateral angular gyrus, and
Te m p o r a l lo b e inform ation from left hem ifield is transm itted through
splenium of corpus callosum to contralateral angular gyrus
Gliom a, vascular

Fin d in gs: ”Pie-in-the-sky” VF defect (inferior m acular fibers

do not travel as far anteriorly into Meyer’s loop) Dis o rd e rs
Oth er fin d in gs: d ejà vu sym ptom s, foul odors; m ay have Ale xia
form ed visual hallucinations
Inability to read despite norm al vision
P a rie t a l lo b e
Giom a, m eningiom a, m etastases, m iddle cerebral artery P s e u d o a le xia
throm bosis
Can be caused by inability to read, hem ianopias with split
fixation, and expressive (Broca’s) or conduction aphasias
Fin d in gs: affect fibers from superior retina first (contralateral
inferior homonymous quadrantanopia) or contralateral inferior
homonymous hemianopia denser inferiorly; spasticity of Ale xia w it h a g ra p h ia (In a b ilit y t o w rit e )
conjugate gaze (tonic deviation of eyes to opposite side of
Parietal lesions involving angular gyrus
parietal lesion); OKN asymm etry (nystagmus dampened when
stimuli moved in direction of damaged parietal lobe); may have
contralateral m otor paresis Ale xia w it h o u t a g ra p h ia

Ger stm an n ’s syn d r o m e: lesion of dom inant parietal lobe Large left occipital lesions that also disrupt fibers crossing in
(inferior hom onym ous hem ianopia, acalculia, agraphia, finger splenium of corpus callosum from right occipital cortex to left
agnosia, left–right confusion) angular gyrus; inform ation from left VF cannot travel to left
parietal lobe (angular gyrus); patient is blind in right visual
Oc c ip it a l lo b e field, can see and write but not read, even what they have just
written
Vascular (90%), tum ors, traum a

Fin d in gs: Dys le xia

Central homonymous hemianopia (periphery spared): fibers Central nervous system problem in which letters appear
from m acula term inate at tip of occipital lobes reversed; m ale > fem ale (3:1); visual training does not im prove
Macula sparing: fibers from retinal periphery term inate on academ ic abilities of dyslexic or learning disabled children
m esial surface of occipital lobe (supplied by PCA)
Temporal crescent: hom onym ous hem ianopia with sparing
of far tem poral periphery (supplied by nasal retina and Vis u a l n e g le c t
travels to m ost anterior portion of m esial surface of Patient ignores one side of visual space
contralateral occipital lobe)

Oth er fin d in gs: unform ed visual hallucinations, paliopia Vis u a l e xt in c t io n

(perseveration in hom onym ous field), prosopagnosia
Patient ignores one side of visual space when presented with
(inability to recognize faces; bilateral m edial occipitotem poral
sim ultaneous stim uli to both visual fields; occurs m ore often
lesion), Riddoch phenom enon (ability to perceive m oving
with right parietal lesions
objects but not stationary ones)

Disco n n ectio n syn d r o m e: dom inant occipital lobe and Vis u a l a g n o s ia

splenium of corpus callosum ; usually due to posterior artery
stroke (right hom onym ous hem ianopia with alexia [cannot Bilateral lesions of occipitotem poral area; inability to
read] but not agraphia [can write]) recognize objects by sight, although can recognize by touch,
language, and intellect

CORTICAL LESIONS An o m ia
Inability to nam e objects
Cause disorders of visual integration
Visual inform ation from LGB goes to prim ary visual cortex
P ro s o p a g n o s ia
(V1) of both occipital lobes; further processing occurs in areas
V2–V5 Bilateral infero-occipital lesions; inability to recognize faces

80
 Headaches

Ce re b ra l a c h r o m a t o p s ia Ta b le 4-1. Classification of brain tumors

Unilateral or bilateral lesion in infero-occipital area; loss of By origin:
color vision in opposite hem ifield Glial (gliomas) Astrocytoma
Neuronal Neuroblastoma, medulloblastoma
Connective tissue Sarcoma
Lymphoreticular Primary (non-Hodgkin’s) central nervous
DISORDERS DURING P REGNANCY system (CNS) lymphoma
Blood vessels Hemangioma, angioma
Bone Osteoma
Occur m ore com m only with eclam psia and preeclam psia Neural crest Meningioma (arachnoid cells), primary CNS
Congenital rests: melanoma
Co n d itio n s: central serous retinopathy (CSR), hypertensive Notochord Chordoma
Adipose cells Lipoma
retinopathy, Purtscher’s-like retinopathy, RD, cerebral venous Ectodermal derivatives Craniopharyngioma, teratoma, dermoid
throm bosis, CRAO, carotid cavernous fistula, Sheehan’s Glands: Adenoma
syndrom e (postpartum pituitary infarct causing Pituitary gland Pineocytoma, pineoblastoma,
panhypopituitarism ; secondary hem orrhage can cause CN 2-6 Pineal gland germ cell tumors
By a g e :
palsies)
< 20 years old CNS tumors are second most common
type of malignancy (leukemia is first);
Co n d itio n s th at wo r sen d u r in g p r egn an cy: uveal approximately 66% located in posterior
m elanom a, pituitary adenom a, m eningiom a, DM, Graves’ fossa; gliomas of cerebellum, brain stem,
optic nerve; pinealomas; primitive
disease, orbital and choroidal hem angiom as neuroectodermal tumors;
craniopharyngiomas
Co n d itio n s th at im p r o ve d u r in g p r egn an cy: MS, 20–60 years old Meningiomas, gliomas of cerebral
optic neuritis, sarcoidosis, lupus hemispheres, pituitary tumors
> 60 years old Malignant gliomas, metastases

Migr ain es: m ay im prove or worsen

Pr eeclam p sia: transient visual loss in 50%, cerebral P o s t e rio r fo s s a t u m o r s

blindness in 15%; ischem ic cerebrovascular com plication risk
increases 13 Â during pregnancy and postpartum due to Cause m ost severe papilledem a because encroach on cerebral
hypercoagulable state aqueduct and 4th ventricle with rapid rise in ICP
Arterial abnormalities: occur in 60% to 90%; m ost
com m on during second and third trim esters and first Me t a s t a s e s
week postpartum
In order of frequency at autopsy: m elanom a, lung, breast,
Venous abnormalities: during first 6 weeks postpartum
renal, colorectal, ovarian
(venous sinus throm bosis presents with headache and
papilledem a).

HEADACHES
BRAIN TUMORS
(Fig. 4-29)
(Table 4-1)
Mig ra in e s
Fo s t e r-Ke n n e d y s yn d ro m e
Etio lo gy: cerebral vasospasm ; slow-m oving concentric wave
Frontal lobe m ass (usually olfactory or sphenoid ridge
of suppressed electrical activity
m eningiom a) causing anosm ia (loss of sm ell), ipsilateral optic
atrophy (tum or com pression), and contralateral ON edem a
(elevated ICP) Typ es:
Common (without aura): m ale ¼ fem ale; any age; little or
Pseu d o –Fo ster -Ken n ed y syn d r o m e: bilateral AION no prodrom e; throbbing headache, photophobia,
(pale nerve from old AION and fellow nerve edem a from nausea
new AION) Classic (with aura) (20%): m ale ¼ fem ale; any age; trigger
factors; visual prodrom e followed by hem icranial
pain, nausea, vom iting, dizziness, photophobia
Ce re b e llo p o n t in e a n g le t u m o rs
Scintillating scotom a (5–30 m inutes, half visual field;
Usually acoustic neurom a; associated with neurofibrom atosis; m ay have m acropsia, m icropsia, hallucinations,
peripheral CN 7 lesion (orbicularis weakness, inability to achrom atopsia); m ay have tingling on one side of
wrinkle forehead), CN 6 lesion, CN 5 lesion (decreased corneal face and/or ipsilateral hand; rarely, hem iparesis,
sensation), nystagm us (vestibular to contralateral side, gaze vertigo, m ood changes, distortion of hearing
paretic to ipsilateral side) and sm ell

81
CHAPTER 4 • Neuro-ophthalm ology

Sinusitis Tension

1 kg

Migraine Trigeminal neura lgia

Cluster

most frequent site

less frequent site
lea st frequent site

Fig ure 4-29. Location of pain for the common headache syndromes. (With permission from Weinstein J M: Headache and facial pain. In Yanoff M, Duker J S (eds):
Ophthalmology. London, 1999, Mosby.)

Complicated: hem iplegic or hem iparetic; m ostly children Lifestyle: excessive sleep, fasting or dieting, exertion,
OPHTHALMOPLEGIC: headache and recurrent paresis of fatigue, stressful events, depression
one or m ore ocular m otor nerves (usually CN 3) Hormonal: supplem ental estrogen, m enses, oral
May last longer than 1 week; rarely, perm anent contraceptives, ovulation
ophthalm oplegia Environmental: sun exposure, loud noise, bright lights,
Childhood: brief attacks of nausea/vom iting; usually no glare, flickering lights, strong odors
headache
Acephalgic (m igraine equivalent): aura without headache
Most com m on after age 40 years; history of m igraines
with or without aura; rule out TIA Tr eatm en t:
Retinal: tem porary scotom a or m onocular blindness; General: alter diet, lifestyle, horm onal and environm ental
headache m ay precede or occur within an hour factors
Exam ination during attack reveals venous constriction Abortive: ice pack, cold sh ower, exercise, m assage, local
Migraine infarction: focal signs persist for longer than scalp pressure; analgesics; oral ergotam ine or
1 week; corresponding lesion on neuroim aging dih ydroergotam ine (DHE); an tiem etics; sum atriptan
Status migrainosus: attack lasting longer than 72 hours ([Im itrex], seroton in agonist; works on periph eral
(with or without treatm ent); continuous headache or trigem in al n erve term in als that supply pain-sensitive
interm ittent with interruptions at less than 4 hours vascular and m eningeal structures; also causes
constriction of certain intracranial blood vessels; oral
Pr ecip itatin g facto r s: and in jectable form s; 83% obtain relief within
Diet: tyram ine (bananas, avocado, yogurt, aged cheese), 4 h ours, 45% have recurrence 24 h ours later; adverse
phenylethylam ine (chocolate, cheese, wine), sodium effects in clude flushin g or tingling, ch est pain )
nitrite (preservatives, food coloring, processed m eats and Prophylactic: consider if attacks occur once every
fish), m onosodium glutam ate (Chinese food, processed 2 m onths or m ore often, if severity lim its norm al
m eats, frozen dinners, canned soup), caffeine, alcohol, activity, or if acute therapies have failed or are
artificial sweeteners contraindicated

82
 Visual Disturbances

β-BLOCKERS: reduce frequency of attacks by half

(in 70% of patients) VISUAL DISTURBANCES
TRICYCLIC ANTIDEPRESSANTS: am itriptyline,
nortriptyline, im ipram ine, and doxepin
SEROTONIN REUPTAKE INHIBITORS: fluoxetine Func tio na l Vis ua l Lo s s
CALCIUM CHANNEL BLOCKERS: decrease frequency of
attacks, do not affect severity Diagnosis of exclusion; trick patient with visual tests

Te n s io n h e a d a c h e s Bo tto m -u p acu ity: start with 20/20 line and slowly

progress up chart
Dull, persistent pain like tight band around head
Decep tio n o f eye tested : fogging with phoropter; trial
Clu s t e r h e a d a c h e s frame with two high-power cylinders of opposite sign in same
axis (so net power is zero), then spin one of the cylinders thus
Severe, unilateral, orbital, supraorbital, and/or tem poral pain fogging good eye; red-green glasses used with duochrome filter
lasting from 15 m inutes to 3 hours; m ay awaken patient from of projection chart (red letters are seen with eye behind red lens),
sleep; usually occur in m en 30 to 40 years of age; m ore prism test (10 PD lens base out in front of a seeing eye causes
com m on in sm okers; attacks occur in groups and last weeks or gaze shift)
m onths
Ster eo acu ity: m inim um am ount of acuity is needed to
Fin d in gs (ipsilateral): conjunctival injection, lacrim ation, distinguish different stereo im ages
nasal congestion, rhinorrhea, forehead/facial sweating, m iosis,
ptosis, and/or eyelid edem a; may develop postganglionic
Bin o cu lar in tegr ated m u ltico lo r ed visio n
Horner’s syndrom e
assessm en t test (BIMVAT): red and blue filters placed over
near correction in trial fram e; blue filter over ”bad” eye, then
Glo s s o p h a ryn g e a l n e u ra lg ia show letters m ade up of orange, brown, and blue com ponents
Unilateral pain in region of larynx, tongue, tonsil, and ear; m ay (orange com ponents seen through blue filter but not red filter);
have hoarseness and coughing; can be stim ulated by som e letters designed so that they can be read as two different
swallowing or pungent tastes letters (e.g., P or R) depending on what com ponents are seen,
so any letter with all orange com ponents read correctly was
Tr eatm en t: carbam azepine, baclofen, phenytoin read by the ”bad”eye

Visu al field testin g: spiral field

Ca ro t id yn ia
Pain arising from cervical carotid artery, radiates to ipsilateral With co m p lete b lin d n ess: OKN, signature (truly blind
face and ear; rule out carotid dissection patients can do this without difficulty), touch nose or other
finger (patient m ay mistake this for a visual task and be unable to
Te m p o r o m a n d ib u la r jo in t (TMJ ) s yn d ro m e perform ), mirror test, shock value tests (startle or menace), ERG

Unilateral ear or preauricular pain, radiates to tem ple, jaw, or

neck; worse with chewing Tra ns ie nt Vis ua l Lo s s
Fin d in gs: lim itation of norm al jaw m ovem ent, audible click Vis u a l o b s c u ra t io n s
on jaw opening
Last seconds; occur in papilledem a (change in posture or eye
Ot h e r c a u s e s o f h e a d a c h e / fa c ia l p a in m ovem ent) or optic disc drusen

Acu te: subarachnoid hem orrhage, m eningitis, encephalitis, Am a u ro s is fu g a x

focal scalp inflam m ation, sinusitis, dental disease, acute
uveitis, acute glaucom a, scleritis, HZV, cervical Monocular loss or dim m ing of vision lasting from 2
spondylitis, GCA to > 30 m inutes; com m only due to carotid or cardiac disease,
also occurs in GCA, vertebrobasilar insufficiency (bilateral),
Recu r r en t : asthenopia, cerebral aneurysm or angiom a, hypotension, hyperviscosity (anticardiolipin), m igraine,
severe hypertension eclam psia; brief episodes m ay indicate im pending CRAO

Ch r o n ic: m uscle tension, depression, cerebral tum or, Uh t h o ff’s p h e n o m e n a

pituitary or nasopharyngeal tum or, m etastatic carcinom a, Blurring of vision with activity or heat; due to optic neuritis
Paget’s disease, increased intracranial pressure, chronic
subdural hem orrhage, postherpetic neuralgia, trigem inal
S c in t illa t in g s c o t o m a
neuralgia (tic douloureux), Costen’s syndrom e
(tem porom andibular osteoarthritis) Ocular m igraine

83
CHAPTER 4 • Neuro-ophthalm ology

Wh it e o u t o f vis io n o r “Ch ic ke n -w ire ” p a t t e rn Fin d in gs:

Anterior communicating artery: ON com pression, chiasm
Occipital ischem ia
com pression, paraplegia
Origin of PCA: sudden-onset severe headache, com plete
Gra d u a l p e rip h e ra l c o n s t ric t io n o f vis io n w it h CN 3 palsy
vis u a l p h e n o m e n a Bifurcation of MCA: hem iparesis, aphasia
Cerebrovascular disease or occipital m igraine Bifurcation of ICA: ON com pression, chiasm com pression,
hem iparesis
Subarachnoid hemorrhage: neurosurgical em ergency; severe
Othe r Vis ua l P he no m e na headache (”worst headache of life”), nausea, vom iting,
stiff neck; Terson ’s syn drom e (vitreous and
Vis u a l h a llu c in a t io n s subarachnoid hem orrhages [when ICP in ON sheath
exceeds ocular venous pressure])
Release h allu cin atio n s (Charles Bonnet Sentinal bleed: headache with transient neurologic
syndrom e): form ed (faces, objects) or unform ed (flashes of sym ptom s before m ajor rupture
light); occur in areas of absent vision; usually continuous and
variable; com m on in age-related m acular degeneration (AMD)
and patients with large VF defects; associated with lesions Diagn o sis:
anywhere in visual pathway; stop with eye m ovem ent Cerebral arteriogram: 4-vessel study of both carotid and
vertebral arteries
Ictal h allu cin atio n s: stereotyped, paroxysm al visual MRI: detects aneurysm s > 5 m m in size
hallucinations; unform ed (occipital lobe lesion) or form ed Magnetic resonance angiography (MRA): can detect 3-m m
(associated with strange odor; tem poral lobe lesion) aneurysm
CT scan: acutely to screen for subarachnoid and
Palin o p sia: abnorm al perseveration of visual im ages; can be intraparenchym al bleed; unacceptable screen for
hallucinatory (creation of im age that does not exist; either unruptured aneurysm s (if negative, perform LP to
sim ple/unformed or com plex/form ed; caused by dysfunction determ ine presence of subarachnoid blood)
of visual m em ory from seizures or cortical lesions) or illusory
(altered perception of real im age; caused by m igraine, head
traum a, drug/m edication) Tr eatm en t:
Medical (sym ptom atic, unruptured): stabilize, lower ICP
Phos phe ne s with hyperventilation and m annitol; prevent vasospasm
Unstructured flashes of light with calcium channel blockers and blood volum e
expansion; control blood pressure
P h o t o p s ia s Surgery: clip aneurysm ; if unable, m ay need to ligate
feeding artery
Structured geom etric figures Prognosis: risk of bleed is 1%/year
30% m ortality at tim e of rupture; if untreated, 33%
m ortality at 6 m onths; survivors have neurologic deficits
VASCULAR DISORDERS Rebleed risk is highest in first 24 hours; untreated patients
have 25% risk of rebleed during first 2 weeks
Ce re b ra l a n e u rys m Vasospasm is m ajor cause of m orbidity and death; 30%
Occurs in 5% of population; rarely sym ptom atic before age 20; with in first 2 weeks, high est risk between days 4
associated with hypertension and 10

Risk facto r s: HTN, AV m alform ation, coarctation of the

aorta, polycystic kidney disease, fibrom uscular dysplasia, Art e rio ve n o u s m a lfo rm a t io n (AVM)
Marfan’s syndrom e, Ehlers-Danlos syndrom e
Congenital, m ay be fam ilial; sym ptom s usually before age 20;
Typ es: fusiform or saccular (”berry”; m ost com m on at 90% supratentorial, 70% cortical, 20% deep, 10% in posterior
arterial bifurcations; 90% supratentorial; > 10 m m have fossa or dura m ater; 6% have intracranial aneurysm
highest risk of rupture)

Lo catio n : Fin d in gs: intracranial bleed (50%), som etim es with

Internal carotid artery (85%): m ain trunk (PCA, subarachnoid hem orrhage; neurologic sym ptom s before bleed
ophthalm ic artery, cavernous sinus), anterior (50%) (seizures, headaches, other neurologic deficits); m ay
com m unicating artery, m iddle cerebral artery (MCA) hear bruit
trifurcation, anterior cerebral artery. Most com m on site is Cortical AVM in occipital lobe: visual sym ptom s and
at origin of posterior com m unicating artery leading to m igraines
third CN palsy with pupil involvem ent Hemispheric AVM: can get hom onym ous hem ianopia
Basilar artery (5%) Brain stem AVM: diplopia, nystagm us, gaze palsy, pupil
Vertebral artery (5%) abnorm ality

84
 Vascular Disorders

Diagn o sis: Ve rt e b ro b a s ila r in s u ffic ie n c y (VBI)

CT scan: hem orrhage; calcified AVMs visible on plain X-ray
Posterior circulation ischem ia; vertebrobasilar system
MRI: better for sm all AVMs
(vertebral, basilar, posterior cerebral arteries) supplies occipital
Cerebral angiogram: dem onstrate anatom y
cortex and areas involved with ocular m otility in brain stem
and cerebellum
Tr eatm en t: resection, ligation of feeding vessel,
em bolization, stereotactic radiosurgery Etio lo gy: throm bus, em boli, hypertension, arrhythm ias,
arterial dissection, hypercoagulable state, subclavian steal
Pr o gn o sis: 20% m ortality when bleeding begins; rebleed syndrom e
rate is 2.5%/year
Fin d in gs: ataxia, vertigo (m ay also have tinnitus, deafness,
or vom iting), dysarthria, dysphagia, hem iparesis, hem iplegia,
Ca ro t id a rt e ry d is s e c t io n drop attack (patient suddenly drops to ground without
Intracranial or extracranial warning, no loss of consciousness), bilateral dim m ing of vision
lasting seconds to m inutes, photopsias, hom onym ous VF loss
without other neurologic findings
Etio lo gy:
Trauma: blunt (head, neck), carotid artery com pression,
hanging, m anipulative neck therapy, surgery, carotid
Ce re b ra l b lin d n e s s / c o rt ic a l b lin d n e s s
artery cannulation during angiography Bilateral occipital lobe lesions; pupils react norm ally; m ay deny
Spontaneous: fibrom uscular dysplasia, Marfan’s syndrom e, blindness (Anton’s syndrom e)
Ehlers-Danlos, polycystic kidney disease, syphilis,
atherosclerosis, m oyam oya, idiopathic
Ce re b ra l ve n o u s a n d d u ra l s in u s t h ro m b o s is
Occlusion of cortical and subcortical veins produces
Fin d in gs: neurologic sym ptom s; m ost com m only, cavernous sinus,
Traumatic: ipsilateral headache and ophthalm ic signs, lateral sinus, and superior sagittal sinus
contralateral neurologic deficits; m ay hear bruit
Sym ptom s can be delayed (weeks to m onths); severe cases
can present with cerebral ischem ia and com a Etio lo gy: inflam m ation (Behçet’s, SLE), infection, traum a,
Spontaneous: transient or perm anent neurologic defects; invasion of vessel wall by tum or (leukem ia, lym phom a,
am aurosis fugax, m onocular visual loss, or ipsilateral m eningiom a), altered blood flow (hypoperfusion,
Horner’s syndrom e; intracranial extension m ay cause CN hem atologic disorders, venous em boli, hypercoagulability,
palsies, diplopia, tongue paralysis, facial num bness oral contraceptives, pregnancy, sickle cell disease, protein C or
Visual loss from em bolic occlusion of ophthalm ic S deficiency, antithrom bin III deficiency, lupus anticoagulant)
artery, central retinal artery, or short posterior ciliary
arteries Caver n o u s sin u s th r o m b o sis: aseptic or septic (infection
Rarely, ocular ischem ia from reduced blood flow of sinus or face; rarely otitis or orbital cellulitis)
Findings: usually unilateral; orbital congestion,
lacrim ation, chem osis, eyelid swelling, ptosis, proptosis,
Diagn o sis: MRI ophthalm oplegia (CN 6 m ost com m on); m ay have
corneal anesthesia, facial num bness, Horner’s syndrom e
Tr eatm en t: controversial Other findings: headache, nausea, vom iting, som nolence,
fever, chills, evidence of m eningitis or sepsis
Treatment: antibiotics, anticoagulants, corticosteroids,
Ve rt e b ro b a s ila r d is s e c t io n surgery
40% of all dissecting aneurysm s; basilar m ore com m on than
vertebral Later al sin u s th r o m b o sis: usually septic from chronic
otitis m edia
Findings: CN 6 palsy m ost com m on (severe facial pain
Gen er al fin d in gs: headache, neck pain, signs of brain stem [Gradenigo’s syndrom e] if com pressed against
and cerebellar dysfunction petroclinoid ligam ent), papilledem a
Basilar artery dissection: ocular m otor palsies, progresses to Other findings: sym ptom s of infection, neck pain,
com a and death tenderness of ipsilateral jugular vein, retroauricular
edem a; m ay have facial weakness
Ver teb r al ar ter y d issectio n (various
presentations): fatal brain stem infarction (usually young Su p er io r sagittal sin u s ( SSS) th r o m b o sis: usually
adults); subarachnoid hem orrhage; aneurysm al dilation with aseptic
brain stem and lower cranial nerve signs from m ass effect; Aseptic: occurs during pregnancy, im m ediately
chronic dissection with recurrent TIAs, strokes, and postpartum , or with oral contraceptives; risk factors
subarachnoid hem orrhages include vasculitis and system ic inflam m atory disorders

85
CHAPTER 4 • Neuro-ophthalm ology

Septic: m ost com m only from m eningitis; also paranasal

sinus infection, pulm onary infection, tonsillitis, REVIEW QUESTIONS (Answers start on page 376)

dental infection, pelvic inflam m atory disease, and

otitis m edia 1. The visual field defect m ost characteristic of optic
Findings: neuritis is
THROMBOSIS OF ANTERIOR THIRD OF SINUS: m ild a. altitudinal
sym ptom s b. central
THROMBOSIS OF POSTERIOR SINUS: m ay cause c. centrocecal
pseudotum or cerebri (headaches and papilledem a; d. arcuate
consider in pseudotum or that occurs in thin 2. Which cranial nerve is m ost prone to injury in the
patients), seizures, altered m ental status, focal cavernous sinus?
neurologic signs; m ay be fatal from brain a. 3
hem orrhage and herniation b. 4
Treatment: anticoagulation, fibrinolytic agents, c. 5
ICP-lowering agents d. 6
3. Which of the following agents is least toxic to the optic
Diagn o sis: CT, MRI, MRA (venous phase) nerve?
a. isoniazid
b. dapsone
INTRACRANIAL ARACHNOID CYST c. m ethanol
d. etham butol
Congenital m alform ation: CSF-filled cyst m ost com m only in 4. Seesaw nystagm us is produced by a lesion located in
m iddle cranial fossa (Sylvian fissure) which area?
a. chiasm
Fin d in gs: seizures, headaches, CN palsy, exophthalm os, b. posterior fossa
hydrocephalus (com pression of foram en of Munroe, aqueduct, c. suprasellar
or fourth ventricle), increased intracranial pressure, d. cervicom edullary junction
papilledem a 5. What is the location of a lesion that causes an
ipsilateral Horner’s syndrom e and a contralateral CN 4
Cyst rupture m ay cause subdural hem atom a palsy?
a. m idbrain
Tr eatm en t: fenestration, shunt b. pons
c. cavernous sinus
d. orbit
NEURO-OP HTHALMIC 6. The least useful test for functional visual loss is
MANIFESTATIONS OF AIDS a. confrontation VF
b. OKN
c. HVF
CNS lym p h o m a d. tangent screen at 1 m and 2 m
High-grade B-cell non-Hodgkin’s lym phom a; second m ost 7. Optociliary shunt vessels m ay occur in all of the following
com m on m alignancy in AIDS conditions except
a. chronic glaucom a
Fin d in gs: diplopia (from CN 3, 4, 6 involvem ent; m ay have b. CVO
disc swelling from infiltration of orbit and optic nerve) c. m eningiom a
d. ischem ic optic neuropathy
P ro g re s s ive m u lt ifo c a l 8. Which is not a sym ptom of pseudotum or cerebri?
le u ke n c e p h a lo p a t h y (P ML) a. diplopia
b. entoptic phenom ena
Papovavirus destroys oligodendrocytes; gray m atter
c. visual obscurations
relatively spared; can affect central visual pathway and ocular
d. headache
motor fibers
9. A 63-year-old wom an reports sudden onset
of jagged lines in the righ t peripheral vision .
Fin d in gs: ataxia, altered m ental status, dem entia, She h as experienced three episodes in th e past
hem iparesis, focal neurologic defects m on th , which lasted approxim ately 10 to
20 m inutes. She denies headaches and any
Diagn o sis: MRI (dem yelination; usually parieto-occipital history or fam ily h istory of m igraines. The m ost
areas, typically involves subcortical white m atter with likely diagnosis is
focal or confluent lesions; m ay see focal enhancem ent with a. vertebrobasilar insufficiency
contrast) b. occipital AVM
c. m igraine variant
Tr eatm en t: none; death com m on within 6 m onths d. posterior vitreous detachm ent

86
 Review Questions

10. A 60-year-old m an with optic disc swelling in the right 21. Which of the following syndrom es is
eye and left optic atrophy m ost likely has characterized by abduction deficit and contralateral
a. ischem ic optic neuropathy hem iplegia?
b. left sphenoid ridge m eningiom a a. Foville’s
c. Leber’s hereditary optic neuropathy b. Gradenigo’s
d. left optic nerve gliom a c. Millard-Gubler
11. Which of the following findings m ay not be present in a d. Weber’s
patient with an INO? 22. All of the following are features of progressive
a. dissociated horizontal nystagm us supranuclear palsy except
b. lim itation of adduction a. hypom etric saccades
c. absent convergence b. loss of oculovestibular reflex
d. abnorm al abduction saccades c. full m otility with doll’s head m aneuver
12. A paradoxical pupillary reaction is not found in which d. lim itation of downgaze
condition? 23. Pituitary apoplexy is characterized by all of the following
a. achrom atopsia except
b. albinism a. nystagm us
c. Leber’s congenital am aurosis b. facial num bness
d. optic nerve hypoplasia c. headache
13. Inheritance of Leber’s optic neuropathy is d. diplopia
a. sporadic 24. Which of the following is m ost likely to produce a
b. autosom al dom inant junctional scotom a?
c. m itochondrial DNA a. craniopharyngiom a
d. X-linked recessive b. m ultiple sclerosis
14. An OKN strip m oved to the left stim ulates what part c. pituitary adenom a
of the brain? d. m eningiom a
a. right frontal, left occipital 25. All of the following are characteristics of an optic tract
b. right frontal, right occipital lesion except
c. left frontal, left occipital a. relative afferent pupillary defect
d. left frontal, right occipital b. decreased vision
15. The sm ooth pursuit system does not involve the c. hom onym ous hem ianopia
a. PPRF d. optic nerve pallor
b. prestriate cortex 26. The saccade system does not involve the
c. occipital m otor area a. occipital m otor area
d. frontal m otor area b. prem otor cortex
16. Dorsal m idbrain syndrom e is not associated with c. frontal m otor area
a. absent convergence d. PPRF
b. gaze palsy 27. A 22-year-old m an sustains traum a resulting in a
c. light-near dissociation transected left optic nerve. Which of the following is true
d. nystagm us regarding the right pupil?
17. The location of Horner’s syndrom e is best differentiated by a. it is larger than the left pupil
which drug? b. it is sm aller than the left pupil
a. cocaine c. it is equal in size to the left pupil
b. hydroxyam phetam ine (Paredrine) d. it reacts consensually
c. pilocarpine 0.125% 28. Characteristics of spasm us nutans include all of the
d. pilocarpine 1% following except
18. The blood supply to the prelam inar optic nerve is a. spontaneously disappears within 3 years
a. m eningeal arteries b. m ay m im ic chiasm al gliom a
b. ophthalm ic artery c. begins before 1 year of age
c. short posterior ciliary arteries d. signs present during sleep
d. central retinal artery 29. A congenital CN 4 palsy can be distinguished from an
19. Optic nerve hypoplasia is associated with all of the acquired palsy by
following except a. vertical fusional am plitude > 10 Δ
a. paradoxic pupillary response b. incyclotropia of 5°
b. m idline abnorm alities c. excyclotropia of 5°
c. m aternal ingestion of LSD d. spontaneous head tilt to the opposite side
d. spasm us nutans 30. Characteristics of a diabetic CN 3 palsy m ay include all of
20. A lesion in the pons causes the following except
a. anisocoria a. pain
b. m iosis b. spontaneous recovery within 90 days
c. light-near dissociation c. sluggish pupillary response
d. m ydriasis d. aberrant regeneration

87
CHAPTER 4 • Neuro-ophthalm ology

31. A CN 3 lesion m ay cause all of the following except c. abduction of contralateral eye
a. contralateral ptosis d. adduction of contralateral eye
b. ipsilateral ptosis 41. A pineal tum or is m ost likely to cause
c. bilateral ptosis a. Ballint’s syndrom e
d. no ptosis b. Fisher syndrom e
32. Optic nerve drusen is associated with all of the following c. Nothnagel’s syndrom e
except d. Parinaud’s syndrom e
a. RP 42. Metastatic neuroblastom a is m ost likely to be associated
b. CRAO with
c. CME a. opsoclonus
d. CNV b. ocular bobbing
33. A lesion causing lim ited upgaze with an intact Bell’s c. ocular dipping
phenom enon is located where? d. ocular m yoclonus
a. supranuclear 43. Which of the following statem ents regarding pupillary
b. nuclear innervation is true?
c. tract a. sym pathetic innervation of the iris sphincter involves
d. cavernous sinus three neurons from the Edinger-Westphal nucleus
34. An acute subarachnoid hem orrhage due to a ruptured b. parasym pathetic innervation of the iris sphincter
aneurysm m ay produce all of the following except involves two neurons and the ciliospinal center
a. ptosis of Budge
b. orbital hem orrhage c. parasym pathetic innervation of the iris dilator
c. vitreous hem orrhage involves two neurons from the Edinger-Westphal
d. efferent pupillary defect nucleus
35. The length of the canalicular portion of the optic nerve is d. sym pathetic innervation of the iris dilator involves
approxim ately three neurons and the ciliospinal center of Budge
a. 5 m m 44. The m ost im portant test to order in a patient with chronic
b. 10 m m progressive external ophthalm oplegia is
c. 15 m m a. ERG
d. 20 m m b. EKG
36. Findings in ocular m otor apraxia include all of the c. EMG
following except d. ESR
a. abnorm al OKN response 45. Pseudotum or cerebri is m ost likely to cause a palsy of
b. abnorm al pursuits which cranial nerve?
c. head thrusting a. 3
d. abnorm al vestibular nystagm us b. 4
37. Which of the following statem ents is true regarding the c. 5
optic chiasm ? d. 6
a. 53% of tem poral retinal fibers rem ain uncrossed in the 46. CT scan of a patient with visual loss shows a railroad-track
ipsilateral optic tract sign. The m ost likely diagnosis is
b. 47% of tem poral retinal fibers cross to the contralateral a. optic nerve gliom a
optic tract b. pituitary adenom a
c. 53% of nasal retinal fibers cross to the contralateral c. optic nerve m eningiom a
optic tract d. craniopharyngiom a
d. 47% of nasal retinal fibers cross to the contralateral 47. The m ost likely etiology of hom onym ous hem ianopia
optic tract with m acular sparing is
38. Which of the following statem ents is false regarding the a. vascular
lateral geniculate body (LGB)? b. infectious
a. M cells are im portant for stereoacuity c. neoplastic
b. LGB is part of the thalam us d. traum atic
c. There is a 90° rotation of optic nerve fibers in 48. All of the following findings are associated with optic
the LGB neuritis except
d. P cells are im portant for m otion detection a. abnorm al color perception
39. Apatient with a hom onymous hemianopia is found to have b. abnorm al depth perception
an asymmetric OKN response. The location of the lesion is c. flashes of light with eye m ovem ents
a. parietal lobe d. m etam orphopsia
b. occipital lobe 49. Which of the following findings is not associated with an
c. tem poral lobe acoustic neurom a
d. lateral geniculate body a. lagophthalm os
40. The only intact eye m ovem ent in 1½ syndrom e is b. light-near dissociation
a. abduction of ipsilateral eye c. decreased corneal sensation
b. adduction of ipsilateral eye d. nystagm us

88
 Suggested Readings

50. A superior oblique m uscle palsy is m ost com m only 57. A 68-year old diabetic patient reports double vision. Exam
caused by is norm al except for a right abducens palsy. Further
a. tum or questioning reveals recent weight loss and scalp and jaw
b. m ultiple sclerosis pain. Which of the following tests is m ost useful?
c. aneurysm a. fasting blood glucose
d. traum a b. C-reactive protein
51. A 29-year-old obese wom an with headaches, papilledem a, c. Tensilon test
and a norm al head CT scan is diagnosed with idiopathic d. CT of orbits
intracranial hypertension. All of the following findings are 58. A patient is found to have anisocoria, which is greater in a
consistent with her diagnosis except dim room . The m ost likely etiology is
a. visual obscurations a. CN 3 palsy
b. hom onym ous hem ianopia b. Horner's syndrom e
c. enlarged blind spot c. pharm acologic
d. incom itant esotropia d. traum atic
52. Transection of the left optic nerve adjacent to the chiasm 59. A m iddle-aged m an relates a history of double vision and
results in hearing loss from his left ear. On exam he has a left
a. visual field defect in the right eye esotropia and facial palsy. The m ost likely location of his
b. decreased corneal sensation in the right eye lesion is
c. afferent pupillary defect in the right eye a. Cavernous sinus
e. pseudoproptosis of the right eye b. Inferior colliculus
53. The Am sler grid viewed at 30 cm tests how m any degrees c. Sella turcica
of central vision? d. Cerebellopontine angle
a. 5 60. A risk factor for nonarteritic anterior ischem ic optic
b. 10 neuropathy is
c. 15 a. obesity
d. 30 b. hypertension
54. Aberrant regeneration of CN 3 m ay cause all of the c. polym yalgia rheum atica
following except d. MS
a. lid elevation on abduction
b. pupillary constriction on adduction
c. m onocular dam pening of the OKN response
d. lid elevation on down gaze SUGGESTED READINGS
55. A 42-year-old wom an adm itted to the hospital with
severe headache and neck stiffness suddenly becom es Basic and Clinical Sciences Course. (2016). Section 5: Neuro-
disoriented and vom its. On exam ination her left ophthalmology. San Francisco: AAO.
pupil is dilated and does not react to light. She m ost Burde, R. M., Savino, P. J., & Trobe, J. D. (2002). Clinical decisions in
likely has neuro-ophthalmology (3rd ed.). Philadelphia: Mosby.
a. encephalitis Kline, L. B., & Foroozan, R. (2012). Neuro-ophthalmology review manual
(7th ed.). Thorofare, NJ: Slack.
b. m eningitis
Liu, G. T., Volpe, N. J., & Galetta, S. (2011). Neuro-ophthalmology
c. brainstem herniation
diagnosis and management (2nd ed.). Philadelphia: WB Saunders.
d. subarachnoid hem orrhage Loewenfeld, I. E., & Lowenstein, O. (1999). The pupil anatomy:
56. Ahealthy 19-year-old wom an presents with gradual loss of Physiology and clinical applications (2nd ed.). Philadelphia: Butter-
vision OD and pain when looking side to side. Her past worth-Heinem an.
m edical history and review of system s are negative. Exam Miller, N. R., Newm an, N. J., Biousse, V., & Kerrison, J. B. (2007). Walsh
shows visual acuity of 20/50, reduced color vision, a & Hoyt’s clinical neuro-ophthalmology: The essentials (2nd ed.).
relative afferent pupillary defect, and a norm al appearing Philadelphia, PA: Lippincott William s and Wilkins.
optic nerve OD. The m ost im portant test to obtain is
a. visual field
b. lum bar puncture
c. MRI
d. blood work (ANA, ACE, VDRL)

89
This pa ge inte ntiona lly le ft bla nk
5 Pediatrics/Strabism us
PEDIATRICS
Anatomy
Physiology
Orbital Disorders
Craniofacial Disorders
Lid Disorders
Lacrimal Disorders
Conjunctival Disorders
Corneal Disorders
Iris Disorders
Lens Disorders
Glaucoma
Uveitis
Metabolic Disorders
Retinal Disorders
Phakomatoses
Optic Nerve Disorders
STRABISMUS
Anatomy and Physiology
Nystagmus
Ocular Alignment
Special Forms of Strabismus
Strabismus Surgery

P EDIATRICS P HYSIOLOGY

Visual acuity levels (see Strabismus section)

ANATOMY Docum ented by visual evoked potentials in infancy and
preferential looking tests in first m onths of life
Prem ature infants reach landm arks later
At birth, diam eter of eye is 66% that of the adult eye Decreased vision in infants and children
Eye enlarges until 2 years of age, then further growth in puberty
(Table 5-1)
Histo r y: fam ily history, com plications in pregnancy,
Infants have variable levels of astigm atism perinatal problem s
Majority of children are hyperopic; increases upto 7 years of Examination: visual response (assess each eye
age, then dim inishes independently), pupillary reactions (paradoxical
response suggests achrom atopsia, congenital stationary
Eye color darkens during first few m onths of life night blindness (CSNB), optic nerve hypoplasia),
ocular m otility (strabism us, nystagm us, torticollis),
Dilator pupillae poorly developed at birth
cycloplegic refraction, fundus exam ination
Fovea m atures during first few m onths of life Additional tests: optokinetic (OKN) response, forced
Myelinization of optic nerve com pleted shortly after birth preferential looking, electroretinogram (ERG),

91
CHAPTER 5 • Pediatrics/Strabism us

Ta b le 5-1. Changes in ocular measurements with age

Na n o p h t h a lm o s
Oc ula r d im e ns io ns Infa nt (m m ) Ad ult (m m ) Sm all eye with norm al features

Axial length 17 24 Norm al-sized lens and thickened sclera

Corneal diameter 9.5–10.5 12
Associated with hyperopia and angle-closure glaucom a
Corneal radius of curvature 6.6–7.4 7.4–8.4 Increased risk of choroidal effusion during intraocular surgery
Scleral thickness Half of adult

visual evoked response (VEP, VER), ultrasonography,

Infe c tio ns
im aging studies
Differential diagnosis of infant with poor vision and normal P re s e p t a l c e llu lit is
ocular structures: Leber’s congenital am aurosis, Infection anterior to orbital septum ; spares globe
achrom atopsia, blue cone m onochrom atism , CSNB,
albinism , optic nerve hypoplasia, optic atrophy, Penetrating skin traum a, dacryocystitis, sinusitis (m ost
congenital infection (TORCH syndrom es), cortical visual com m on pediatric cause)
im pairm ent (due to extensive occipital lobe dam age),
delay in visual m aturation Mo st co m m o n o r gan ism : Staphylococcus aureus

Fin d in gs: lid edem a, erythem a, and pain; m ay have fever;

ORBITAL DISORDERS m ay progress to orbital cellulitis

Tr eatm en t: CT scan if questioning orbital involvem ent,

Co ng e nita l Ano m a lie s system ic antibiotics (IV in severe cases, children < 5 years old,
and failed oral treatm ent)
An o p h t h a lm o s
Orb it a l c e llu lit is
Bilateral absence of eye due to failure of prim ary optic vesicle
to form ; extrem ely rare Infection posterior to orbital septum ; involves globe
Most com m on cause of proptosis in children
Fin d in gs: hypoplastic orbits
Usually secondary to sinusitis (ethm oid sinus is m ost
com m on)
Bu p h t h a lm o s
Associated with subperiosteal abscesses
Large eye due to increased intraocular pressure in congenital
glaucom a
Etio lo gy: extension of infection from periorbital structures,
sinusitis, dacryocystitis, dacryoadenitis, endophthalm itis, dental
Cryp t o p h t h a lm o s
infections, intracranial infections, trauma or previous surgery,
Failure of differentiation of lid and anterior eye structures endogenous (bacteremia with septic embolization)
Partial or com plete absence of the eyebrow, palpebral fissure,
Or gan ism s: S. aureus (m ost com m on organism in children),
eyelashes, and conjunctiva
Streptococcus pneumoniae, fungi (Phycom ycetes, m ost
Attenuation of the levator, orbicularis, tarsus, and conjunctiva aggressive)

Often associated with severe ocular defects Fin d in gs: fever, decreased vision, positive RAPD, proptosis,
restriction of ocular m otility, pain on eye m ovem ent,
Mic ro p h t h a lm o s periorbital swelling, chem osis, optic disc swelling
Sm all, disorganized eye
CT scan : diagnosis, localization, and involvement of adjacent
Disruption of ocular developm ent occurs after budding of structures
optic vesicle
Usually unilateral Tr eatm en t: IV antibiotics, surgical drainage, observation of
subperiosteal abscess unless any of the following are present
Mic ro p h t h a lm o s w it h c ys t (then em ergent drainage): 1. age > 9 years old, 2. frontal
sinusitis, 3. nonm edial location, 4. large size, 5. anaerobic
Due to failure of em bryonic fissure to close infection (gas on CT scan), 6. recurrence after prior drainage, 7.
chronic sinusitis (polyps), 8. optic neuropathy, 9. dental origin
Usually blue m ass of lower lid
Associated with congenital rubella, congenital toxoplasm osis, Co m p licatio n s: subperiosteal abscess, cavernous sinus
m aternal vitam in Adeficiency, m aternal thalidom ide ingestion, throm bosis, or intracranial extension causing blindness
trisom y 13, trisom y 15, and chrom osom e 18 deletion or death

92
 Orbital Disorders

Be nig n Le s io ns

De rm o id c ys t (Ch o ris t o m a )
Arises from derm al elem ents (neural crest origin)
Lined by keratinizing epithelium with derm al appendages
Most com m on orbital m ass in childhood
Usually located in superotem poral quadrant near brow, often
adjacent to bony suture
Often filled with keratin
Generally do not enlarge after 1 year of age
May induce bony erosion Fig ure 5-1. Capillary hemangioma demonstrating abnormal proliferation of
blood vessels and endothelial cells. (From Yanoff M, Fine BS: Ocular pathology,
Rupture can cause intense inflam m atory reaction ed 5, St Louis, 2002, Mosby.)

CT scan : well circum scribed with bony m olding Predilection for the superior nasal quadrant of the orbit and
m edial upper eyelid
Tr eatm en t: com plete excision, rem ove en bloc because Fem ale > m ale (3:2)
contents m ay cause granulom atous inflam m ation
Diffuse irregular m ass of plum p endothelial cells and sm all
vascular channels
Ep id e rm o id c ys t (Ch o ris t o m a )
High-flow lesion
Arises from epiderm al elem ents
Lined by epiderm is only (no derm al appendages) Fin d in gs:
Strawberry nevus: skin involvem ent; appears as red,
Usually filled with keratin irregularly dim pled, elevated surface; blanches with
Rupture can lead to an acute inflam m atory process direct pressure (port wine stain does not blanch)
Orbital location: can present with proptosis; bluish
appearance of eyelids and conjunctiva
Lip o d e rm o id
Solid tum or usually located beneath the conjunctiva over
lateral surface of globe Path o lo gy: num erous blood-filled channels lined by
endothelium ; little contribution from larger vessels or strom a;
May appear sim ilar to prolapsed orbital fat, prolapsed lacrim al unencapsulated (Fig. 5-1)
gland, or lym phom a
Usually no treatm ent is needed CT/MRI scan : well-circum scribed lesion

Difficult to excise com pletely Tr eatm en t: required if tum or causing ptosis or astigm atism
with resultant anisom etropia, strabism us, or am blyopia.
Te ra t o m a Options include observation, intralesional steroid injection,
system ic steroids, interferon, topical tim olol, propanolol
Rare, cystic tum or arising from two or m ore germ inal layers (requires m onitoring), laser, radiation, or excision
Usually com posed of ectoderm along with either endoderm or
m esoderm (or both)
Co m p licatio n s:
Can cause dram atic proptosis at birth Eyelid involvement may cause: ptosis with occlusion
am blyopia, astigm atism and anisom etropia with
Rarely m alignant
refractive am blyopia, strabism us with strabism ic
am blyopia
Ca p illa ry h e m a n g io m a Kassabach-Merritt syndrome: consum ptive coagulopathy
with platelet trapping, resulting in throm bocytopenia
Most com m on benign tum or of the orbit in children
and cardiac failure; acute hem orrhage is possible;
Often m anifests in the first few weeks of life and enlarges over m ortality approxim ately 30%
the first 6–12 m onths, with com plete regression by age High-output congestive heart failure can occur with m ultiple
5–8 years in 80% of cases visceral capillary hem angiom as
Spontaneous involution over the next few years Respiratory com prom ise with subglottic hem angiom as

93
CHAPTER 5 • Pediatrics/Strabism us

Lym p h a n g io m a (Lym p h a t ic m a lfo r m a t io n ) Rad io gr ap h : phlebolith present in 30% of cases

Rare, lym phatic-filled choristom a
Tr eatm en t: surgery
Often superonasal Indications: cosm etic, severe proptosis, optic nerve
com pression, pain; surgery is often difficult because of
Appears in first decade of life
intertwining of norm al structures and direct
Involves the eyelids, conjunctiva, and deeper orbital tissues com m unication with cavernous sinus
Lesion waxes and wanes, but does not involute
Fib ro u s d ys p la s ia
Tum or of fibrous connective tissue, cartilage, and bone
Fin d in gs: acute pain, proptosis (often increasing with upper
respiratory infection), bluish hue to overlying lid, m ay Progressive disease of childhood and young adulthood
hem orrhage into channels (chocolate cyst)
Monostotic (in young adults) or polyostotic

Path o lo gy: lym ph-filled vascular channels lined by

Or b ital in vo lvem en t: usually m onostotic; frontal (m ost
endothelium ; unencapsulated (Fig. 5-2)
com m on), sphenoid, and ethm oid bones; causes unilateral
proptosis during first 2 decades of life
CT scan : m ay show layered blood in a lobular, cystic m ass
with infiltrative pattern
Po lyo sto tic: m ultiple bones involved; can cause narrowing
of optic canal and lacrim al drainage system
MRI: infiltrating pattern with indistinct m argins; grapelike
cystic lesions with fluid-fluid layering of serum and red blood Alb r igh t’s syn d r o m e: polyostotic fibrous dysplasia, short
cells stature, prem ature closure of epiphysis, precocious puberty,
and hyperpigm ented m acules
Tr eatm en t: observation (spontaneous regression com m on); Findings: diplopia, proptosis, headaches, facial asym m etry,
rarely surgical evacuation because com plete excision difficult decreased visual acuity (com prom ised optic canal),
and recurrence com m on hearing loss (com prom ised external ear canal)
Pathology: norm al bone is replaced by im m ature woven
Co m p licatio n s: anisom etropia, am blyopia, strabism us bone and osteoid in a cellular fibrous m atrix, strom a of
the bone is highly vascularized, no osteoblasts present
Va rix
Ne u ro fib ro m a
Most com m on vascular abnorm ality
Ham artom a
Dilations of preexisting venous channels
18% have neurofibrom atosis (NF) type 1
Fin d in gs: proptosis (increases with crying or straining; can Nearly all adults with NF 1 have neurofibrom as
occur when head is in a dependent position), orbital
hem orrhage (especially after traum a), m otility disturbance
(usually restricted upgaze), disc swelling, optic atrophy
Plexifo r m n eu r o fib r o m a: m ost com m only involves the
upper lid (S-shaped deform ity); tortuous, fibrous cords
infiltrate orbital tissues

No d u lar : firm and rubbery consistency

Fin d in gs: often involves eyelid or causes proptosis, m ay

cause glaucom a; absence of greater wing of sphenoid bone

Path o lo gy: well-circum scribed, nonencapsulated

proliferation of Schwann cells, perineural cells, and axons;
stains with S-100 (specific for neural crest–derived structures)
(Fig. 5-3)

Tr eatm en t: surgical excision

Op t ic n e r ve g lio m a (Gr a d e I As t ro c yt o m a )
Considered pilocytic astrocytom a of the juvenile type
Fig ure 5-2. Lymphangioma demonstrating lymph-filled spaces, some
with blood. (From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, Slow-growing ham artom a derived from interstitial cells,
2002, Mosby.) astroglia, and oligodendroglia

94
 Orbital Disorders

Oth er fin d in gs: constitutional sym ptom s in 50%

Tr eatm en t: system ic steroids

Gra ve s ’ d is e a s e
Occasionally occurs in adolescents (see Chapter 6, Orbit/Lids/
Adnexa)

Ma lig na nt Ne o p la s m s

Rh a b d o m yo s a rc o m a
Fig ure 5-3. Neurofibroma demonstrating proliferation of Schwann cells. Most com m on prim ary orbital m alignancy of children
(From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.)
Most com m on soft tissue m alignancy of childhood
Most com m on m esenchym al tum or of orbit
Usually does not m etastasize Malignant spindle cell tum or with loose m yxom atous m atrix
Usually appears during first decade of life Average age at diagnosis is 8 years old (90% before age 16)
Associated with neurofibrom atosis I (25%–50%) Cell of origin is an undifferentiated, pluripotent cell of the soft
tissue; does not originate from the extraocular m uscles
Fin d in gs: unilateral proptosis, loss of vision, strabism us, Unilateral; tends to involve superonasal portion of orbit
and papilledem a; m ay develop retinal vascular occlusions,
optociliary shunt vessels; orbital tum ors m ay cause More com m on in m ales (5:3)
chorioretinal folds, optic disc swelling, or atrophy; chiasm al
Aggressive local spread through orbital bones; hem atogenous
tum ors m ay cause pituitary or hypothalam ic dysfunction, and
spread to lungs and cervical lym ph nodes; m ost com m on
nystagm us and head nodding from com pression of 3rd
location for m etastasis is chest
ventricle

Fin d in gs: rapidly progressive proptosis, reddish

Path o lo gy: circum scribed astrocytic tum or from neural crest discoloration of eyelid; m ay have ptosis; later develop tortuous
tissue, glial hypercellularity, Rosenthal fibers, m yxom atous, retinal veins, choroidal folds, and optic nerve edem a
arachnoid hyperplasia; reactive m eningothelial hyperplasia
m ay occur, which can lead to a false diagnosis of m eningiom a
Typ es:
Embryonal: m ost com m on (70%), usually occurs in
CT scan : fusiform enlargem ent of ON, enlargem ent of optic children; tum or appears circum scribed, but often there is
foram en, bony erosion m icroscopic evidence of invasion of nearby structures
PATHOLOGY: elongated spindle cell with central
Tr eatm en t: observation; consider surgery if tum or spreads hyperchrom atic nucleus, eosinophilic granular
posteriorly into the chiasm ; radiation alone for nonresectible cytoplasm ; cells arranged in parallel palisading bands;
lesions with neurologic sym ptom s; chem otherapy obvious cross-striations are rare (Fig. 5-4)
Botryoid: subtype of em bryonal; can occur in anterior orbit,
em bryonal rhabom yosarcom a abutting a m ucosal
Pr o gn o sis: in adults, tum or is m alignant, with death surface, m ultiple polypoid m asses protruding from
occurring in 6 to 12 m onths; better prognosis in children or in hollow viscera
adults with neurofibrom atosis Pleomorphic: least com m on, usually occurs in adults, rare
involvem ent of orbit, m ost differentiated, best prognosis
Id io p a t h ic o rb it a l in fla m m a t io n (IOI) (Orb it a l PATHOLOGY: dense spindle cell arrangem ent with
enlarged cells containing hyperchrom atic nuclei,
p s e u d o t u m o r)
deeply eosinophilic cytoplasm ; m ay see cross-
Idiopathic inflam m atory disorder of orbit striations on m icroscopic exam ination
Alveolar: poorest prognosis, usually found in inferior orbit,
Com m only bilateral with episodic recurrence
generally arises in extrem ities (adolescents)
PATHOLOGY: dense cellular proliferations of loosely
Fin d in gs: decreased vision, diplopia, red eye, headache; cohesive sm all round cells separated by connective
acute, painful presentation in children; resem bles orbital tissue septa; lack of cohesiveness m ay explain
cellulitis m etastatic potential

95
CHAPTER 5 • Pediatrics/Strabism us

40% develop Orbital m etastases

Average age of presentation with m etastatic neuroblastom a to
orbit is 2 years old
Spontaneous regression is rare

Fin d in gs: sudden proptosis and periorbital ecchym osis

(raccoon eyes); m ay have ipsilateral Horner’s syndrom e and
opsoclonus (better prognosis)
Paraneoplastic syndrome associated with metastatic
neuroblastoma: opsoclonus (saccadom ania; random , rapid
eye m ovem ents in all directions that disappear
during sleep)
A
Path o lo gy: sheets of indiscrete round cells with scant
cytoplasm and high m itotic figures, areas of tum or necrosis;
can have bony invasion; usually positive for neuronal m arkers
(synaptophysin and neuron-specific enolase) (Fig. 5-5)

CT scan : bony destruction

Tr eatm en t: chem otherapy and local XRT

Pr o gn o sis: poor if age of onset is over 1 year with m etastases

to bone;however, m etastases to just liver, bone m arrow, or spleen
can be associated with a survival rate as high as 84%

B Accounts for 15% of all childhood cancer deaths

Fig ure 5-4. A, Embryonal type demonstrating primitive rhabdomyoblasts. B, In adults, neuroblastom a usually m etastasizes to the uveal tract
Cross-striations in cytoplasm of some cells. (From Yanoff M, Fine BS: Ocular
pathology, ed 5, St Louis, 2002, Mosby.)
Ew in g ’s s a rc o m a
CT scan : well-circum scribed orbital m ass with possible Second m ost frequent m etastatic tum or to the orbit
extension into adjacent orbital bones or sinuses, bony
Prim ary interm edullary m alignancy of bone; originates in long
destruction
bones of extrem ities or axial skeleton

A-scan u ltr aso u n d : orbital m ass with m edium internal Frequently m etastasizes to bone and lungs
reflectivity Occurs in 2nd–3rd decade of life

Tr eatm en t: urgent biopsy, radiation (100% local control

with 6000 cGy; 30% m ortality due to m etastases),
chem otherapy for any m icroscopic m etastases, surgical
debulking

Pr o gn o sis: with chem otherapy and XRT, 3-year

survival ¼ 90%; cure rate close to 100% with localized orbital
tum or; 60% if invasion of adjacent structures
Tumors arising in the orbit, bladder, and prostate: 77%
disease-free survival at 2 years
Intrathoracic tumors: worst prognosis, 24% disease-free
survival at 2 years

Ne u r o b la s t o m a
Most com m on m etastatic orbital tum or of childhood
Fig ure 5-5. Neuroblastoma. (Case presented by Dr E. Torcynski at the meeting
Usually originates in the adrenal gland or sym pathetic of the Eastern Ophthalmic Society, 1994. From Yanoff M, Fine BS: Ocular
ganglion chain, also m ediastinum or neck pathology, ed 5, St Louis, 2002, Mosby.)

96
 Craniofacial Disorders

Fin d in gs: acute proptosis, hem orrhage, and inflam m ation Bu rkit t ’s lym p h o m a
from tum or necrosis
Prim arily affects the m axilla in black children, with secondary
invasion of the orbit
Tr eatm en t: chem otherapy
Related to Epstein-Barr virus infection

Gra n u lo c yt ic s a rc o m a (“Ch lo r o m a ”)
Fin d in gs: proptosis and bony destruction
Patients with m yelogenous leukem ia m ay present with orbital
signs before hem atologic evidence of leukem ia
Path o lo gy: m alignant B cells, “starry sky” appearance due to
histiocytes interspersed am ong uniform background of
Fin d in gs: proptosis, m ay have subcutaneous lym phocytes
periocular m ass

Path o lo gy: infiltration of involved tissues by leukem ic cells CRANIOFACIAL DISORDERS

Tr eatm en t: radiotherapy, chem otherapy Structural developm ent of head and face occur during 4th–8th
week of gestation
His t io c yt o s is X (La n g e rh a n s c e ll h is t io c yt o s is ) Ocular m otility disturbances occur in 75% of patients with
Group of disorders resulting from abnorm al proliferation of craniofacial disorders
histiocytes (Langerhans cells) that m ay involve orbit
Cr an io facial cleft syn d r o m es: 1 or m ore facial fissures
Spectrum of disease, from isolated bone lesions with excellent
fail to close during the 6th–7th week of gestation
prognosis (eosinophilic granulom a) to system ic spread with
rapid death (Letterer-Siwe disease)
Ocu lar co m p licatio n s: Corneal exposure from proptosis
Children under age 2 with m ultifocal disease have a poor or lid defects
prognosis (50% survival rate) Refractive errors from lid anom alies
Ocular m otility disturbances, m ost com m only exotropia
Fin d in gs: m ost frequent orbital presentation is lytic defect of Papilledem a and optic atrophy from increased intracranial
orbital roof causing progressive proptosis pressure (ICP) or fibrous dysplasia involving optic canal
Ocular anom alies from em bryogenesis
Path o lo gy: granulom atous histiocytic infiltrate, Birbeck
granules (central, dense core, and thick outer shell), stains
positively for S-100 and vim entin Synd ro m e s

Eo sin o p h ilic gr an u lo m a: Benign, local, solitary bone Tre a c h e r-Co llin s s yn d ro m e (Ma n d ib u lo fa c ia l
lesion d ys o s t o s is )
Most likely to involve superior tem poral bony orbit in Hypoplasia of the m idface
childhood or adolescence
CT SCAN AND X-RAY: sharply dem arcated osteolytic Associated with dental and ear abnorm alities, m icrotia,
lesions m icrognathia, hypoplastic m alar bones, and low sideburns
TREATMENT: incision and curettage, intralesional
steroids, or radiotherapy Fin d in gs: lateral lid defects, absent lateral canthal tendon,
absent m edial lashes, antim ongoloid slant, ectropion, poorly
Han d -Sch u€ ller -Ch r istian d isease: developed puncta and m eibom ian glands
Triad of proptosis, lytic skull defects, and diabetes insipidus
More aggressive Go ld e n h a r’s s yn d ro m e (Oc u lo a u ric u lo ve rt e b ra l
Multifocal bony lesions; frequently involves orbit, usually d ys p la s ia )
superolaterally
TREATMENT: system ic steroids and chem otherapy
Abnorm alities of the 1st and 2nd brachial arches
PROGNOSIS: good
Associated with Duane’s syndrom e

Letter er -Siwe d isease: Fin d in gs: lim bal derm oids and derm olipom as; m ay have
Most severe and m alignant lower lid colobom as
Progressive and fatal
Multisystem involvem ent Oth er fin d in gs: hypoplastic facial bones, pretragal
Rarely ocular/orbital involvem ent auricular appendages, vertebral abnorm alities; m ay have
PROGNOSIS: system ic steroids and chem otherapy fistulas between the m outh and ear

97
CHAPTER 5 • Pediatrics/Strabism us

Hyp e rt e lo ris m Me d ia n fa c ia l c le ft s yn d ro m e
Fin d in gs: increased interpupillary distance due to increased Fin d in gs: hypertelorism , exotropia
distance between m edial orbital walls
Associated with blepharophim osis, frontal m eningoceles, Oth er fin d in gs: m edial cleft nose, lip, palate; widow’s peak;
encephaloceles, m eningoencephaloceles, and fibrous dysplasia cranium bifidum occultum

Wa a rd e n b u r g ’s s yn d ro m e (AD)
Cra n io s yn o s t o s e s
Fin d in gs: lateral displacem ent of inner canthi/puncta,
Prem ature closure of bony sutures; inhibits growth of the confluent eyebrows, heterochrom ia iridis, fundus
cranium perpendicular to the axis of the suture; growth can hypopigm entation
continue parallel to the suture
Oth er fin d in gs: sensorineural deafness, white forelock
Fin d in gs: hypertelorism ; proptosis can also occur
He m ifa c ia l m ic ro s o m ia
Plagio cep h aly: prem ature closure of one-half of the Fin d in gs: upper lid colobom a, strabism us
coronal sutures
Skull develops norm ally on one side and is underdeveloped on Oth er fin d in gs: facial asym m etry with m icrotia,
the other side (resulting in a flattened face) m acrostom ia, m andibular anom alies, orbital dystopia, ear
tags, vertebral anomalies
Oth er fin d in gs: m idfacial hypoplasia, proptosis,
telecanthus, V-pattern XT, oral and dental abnorm alities, and Ha lle rm a n n -S t re iff s yn d ro m e
respiratory problem s Sporadic

Cro u z o n ’s s yn d ro m e (Cra n io fa c ia l d ys o s t o s is ) Fin d in gs: bilateral cataracts, glaucom a, m icrophakia,

(Au t o s o m a l d o m in a n t [AD] o r s p o ra d ic ) m icrocornea

Absence of forward developm ent of the cranium and m idface

Oth er fin d in gs: m andibular hypoplasia, beaked nose
Multiple com binations of suture closure can occur
P ie rre Ro b in s e q u e n c e
Fin d in gs: proptosis, V-pattern exotropia, nystagm us, Fin d in gs: retinal detachm ent, congenital glaucom a and/or
hypertelorism; shallow orbits; optic atrophy (in 25%–50%; cataracts, high m yopia
due to narrowing of optic canal, kinking or stretching of
optic nerve)
Oth er fin d in gs: m icrognathia, glossoptosis, cleft palate
Associated with Stickler’s syndrom e
Oth er fin d in gs: m ental retardation, hypoplasia of m axilla,
parrot’s beak nose, high arched palate, external auditory canal Fe t a l a lc o h o l s yn d ro m e
atresia, anodonia
Fin d in gs: short palpebral fissures, telecanthus, epicanthal
folds, com itant strabism us, optic disc anom alies; m ay have
Ap e rt ’s s yn d ro m e (AD) high m yopia, anterior segm ent anom alies
Crouzon’s plus syndactyly
Oth er fin d in gs: thin verm ilion border of upper lip, variable
Anterior m egalophthalm os m ental retardation, sm all birth weight and height,
Associated with increased paternal age cardiovascular and skeletal abnorm alities

P fe iffe r’s s yn d ro m e (AD)

LID DISORDERS
Fin d in gs: hypertelorism and pointy head; m ay have shallow
orbits, syndactyly, and short digits
Ab le p h a ro n
Absence of lids
Ca rp e n t e r’s s yn d ro m e
(Au t o s o m a l re c e s s ive [AR])
An kylo b le p h a r o n
Severe m ental retardation
Partial or com plete fusion of lid m argins; usually tem poral,
Involvem ent of sagittal, lam bdoidal, and coronal sutures often bilateral

98
 Lid Disorders

Often AD Co n g e n it a l Ho rn e r’s s yn d ro m e
Associated with craniofacial abnorm alities Ptosis, m iosis, anhydrosis
Can be secondary to therm al or chem ical burns, inflam m ation, May cause am blyopia
ocular cicatricial pem phigoid, Stevens-Johnson syndrom e
Tr eatm en t: surgical repair (levator resection), severe cases
Ble p h a ro p h im o s is require frontalis sling

Horizontally and vertically shortened palpebral fissures with

poor levator function
Co n g e n it a l e c t ro p io n
Eversion of eyelid m argin due to vertical shortening of anterior
Absent lid crease
lam ella
May be part of autosom al dom inant syndrom e (chrom osom e
3q) with ptosis, telecanthus, epicanthus inversus, lower lid Etio lo gy: inclusion conjunctivitis, anterior lam ella
ectropion, hypoplasia of nasal bridge and superior orbital rim , inflam m ation, Down syndrom e
anteverted ears, hypertelorism
Associated with blepharophim osis syndrom e
Tr eatm en t: surgical correction requires m ultiple procedures
Tr eatm en t: usually not required; otherwise, treat as the
cicatricial form
Co lo b o m a
Em bryologic cleft involving lid m argin; unilateral or bilateral; Co n g e n it a l e n t ro p io n
partial or full thickness
Inversion of eyelid m argin
Ranges from notch to absence of entire lid
Etio lo gy: lid retractor dysgenesis, tarsal plate defects, vertical
Up p er lid : usually m edial third, not associated with system ic shortening of posterior lam ella
abnorm alities, usually full thickness
Usually involves lower eyelid

Lo wer lid : usually lateral third, associated with other

Tr eatm en t: m ay require surgical repair
abnorm alities (Treacher-Collins, Goldenhar’s syndrom es),
usually partial thickness
Co n g e n it a l t a rs a l kin k
May have exposure keratitis
Upper eyelid bent back and open with com plete fold of
tarsal plate
Tr eatm en t: m ethod of surgical repair depends on size of
defect (see section on Lid avulsion) May cause corneal abrasion and exposure keratopathy

Co n g e n it a l b le p h a ro p t o s is Dis t ic h ia s is
Droopy eyelid; 75% unilateral; nonhereditary Partial or com plete accessory row of eyelashes growing from or
Rarely causes am blyopia posterior to m eibom ian orifices

Associated with blepharophim osis syndrom e (AD) Due to im proper differentiation of pilosebaceous units

Myogenic is m ost com m on Usually well tolerated, but trichiasis m ay develop

Etio lo gy: Tr eatm en t: lubrication, cryoepilation, surgical epilation

Myogenic:
Dysgenesis of levator m uscle Ep ib le p h a ro n
Fibroadipose tissue in m uscle belly
Poor levator function, loss of lid crease, eyelid lag, Pretarsal skin and orbicularis override the lid m argin, causing a
som etim es lagophthalm os horizontal fold of tissue to push cilia vertically; no entropion
Aponeurotic: Most com m on am ong Asians
Rare, possibly due to birth traum a
Good eyelid excursion, high or indistinct lid crease Usually occurs in lower lid and resolves spontaneously
Neurogenic: Rarely requires surgery (excision of skin and m uscle for
Congenital CN 3 palsy significant trichiasis)
Marcus Gunn jaw-winking (aberrant connections
between m otor division of CN V3 (to the external
Ep ic a n t h u s
pterygoid m uscle) and levator m uscle; jaw
m ovem ents cause elevation of ptotic lid) Medial canthal vertical skin folds

99
CHAPTER 5 • Pediatrics/Strabism us

Due to im m ature facial bones or redundant skin Presents at birth as bluish swelling inferior and nasal to m edial
Usually bilateral canthus
Produces pseudoesotropia Infection (dacryocystitis) develops if condition does not
resolve spontaneously
Typ es: Common organisms: Haemophilus influenzae,
Epicanthus tarsalis: S. pneumoniae, Staphylococcus, Klebsiella,
Fold m ost prom inent in upper eyelid Pseudomonas
Com m only associated with Asian eyelid
Epicanthus inversus:
Fold m ost prom inent in lower eyelid Tr eatm en t: digital m assage, lacrim al probing, or surgical
Associated with blepharophim osis syndrom e decom pression (for dacryocystitis)
Epicanthus palpebralis:
Fold is equally distributed in upper and lower eyelids Na s o la c rim a l d u c t o b s t ru c t io n (NLDO)
Epicanthus supraciliaris:
Fold arises from eyebrow and extends to lacrim al sac Up to 5% of infants have obstruction of the NLD, usually due
to m em brane covering valve of Hasner
Eu ryb le p h a ro n Most open spontaneously within 4–6 weeks of birth; ⅓ are
bilateral
Horizontal widening of palpebral fissure due to inferior
insertion of lateral canthal tendon
Fin d in gs: tearing, discharge; m ay develop dacryocystitis or
Associated with ectropion of lateral third of lid conjunctivitis
Poor lid closure with exposure keratitis Digital pressure over lacrim al sac producing m ucoid reflux
indicates obstruction of nasolacrim al duct
Tr eatm en t: lubrication, m ay require surgical repair with
resection of excess lid, lateral canthal tendon repositioning, May have a dacryocystocele (dilated lacrim al sac), or am niotic
and vertical eyelid lengthening fluid or m ucus trapped in tear sac

Mic ro b le p h a ro n Tr eatm en t: lacrim al sac m assage, topical antibiotic, probing

and irrigation by 13 m onths of age (95% cure rate); consider
Vertical shortening of lids turbinate infracture and silicone intubation if probing
May have exposure keratitis unsuccessful; dacryocystorhinostom y (DCR) after m ultiple
failures
Te le c a n t h u s
Widened intercanthal distance due to long m edial canthal
tendons
CONJ UNCTIVAL DISORDERS
Associated with fetal alcohol, Waardenburg’s, and Oc u la r m e la n o c yt o s is
blepharophim osis syndrom es
Unilateral excessive pigm ent in uvea, sclera, and episclera
Tr eatm en t: surgery with transnasal wiring Increase in num ber of norm al m elanocytes
More com m on am ong Caucasians

LACRIMAL DISORDERS
Fin d in gs: slate grey appearance of sclera, darkened
appearance of fundus
De ve lo p m e n t a l a n o m a lie s
When associated with pigm entation of eyelid skin, called nevus
Atr esia o f lacr im al p u n cta: ranges from thin m em brane of Ota (congenital oculoderm al m elanocytosis; m ore com m on
over punctal site to atresia of canaliculus am ong African Am ericans and Asians; m alignant potential
only in Caucasians)
Su p er n u m er ar y p u n cta: no treatm ent needed
Oth er fin d in gs: heterochrom ia iridis due to diffuse nevus
Lacr im al fistu la: usually located inferonasal to the m edial of uvea; m ay develop glaucom a (due to m elanocytes in
canthus trabecular m eshwork), rarely uveal, orbital, or m eningeal
m elanom a
Da c ryo c ys t o c e le
Cystic swelling of lacrim al sac accom panies obstruction of Path o lo gy: spindle-shaped pigm ent cells in subepithelial
lacrim al drainage system above and below the sac tissue

100
 Conjunctival Disorders

Ot h e r in fe c t io n s
Co njunc tivitis
Pediatric conjunctivitis is usually bacterial (50%–80%) versus
adult infectious conjunctivitis, which is usually viral
Op h t h a lm ia n e o n a t o ru m
Age dependent; m ore com m on in younger children (< 3 years
Conjunctivitis within first m onth of life
old)
Papillary conjunctivitis (no follicular reaction in neonate due
to im m aturity of im m une system )
Or gan ism s: H. influenzae (50%–65%), S. pneumoniae
(20%-30%), Moraxella catarrhalis (10%); rarely Streptococcus
Etio lo gy: pyogenes or S. aureus
Chemical: caused by silver nitrate 1% solution (Crede’s
prophylaxis); occurs in first 24 hours, lasts 24 to 36 hours Fin d in gs: purulent discharge (80%), red eye (50%)
(therefore, prophylaxis is now with erythrom ycin or
tetracycline ointm ent)
Oth er fin d in gs: otitis m edia (30% in < 3-year-olds; H.
FINDINGS: usually bilateral, bulbar injection with clear
influenzae), preseptal cellulitis (> 3-year-olds with sinusitis,
watery discharge
fever, and elevated white blood cells (WBCs); S. pneumoniae)
No treatm ent necessary
Neisseria gonorrheae: days 1–2; can occur earlier with
prem ature rupture of m em branes Diagn o sis: culture refractory cases and neonates
FINDINGS: severe purulent discharge, chem osis, eyelid
edem a; can be hem orrhagic; m ay develop corneal Tr eatm en t: topical antibiotics; oral antibiotics (cefixim e)
ulceration or perforation for extraocular involvem ent (otitis m edia, sinusitis)
DIAGNOSIS: Gram ’s stain (Gram -negative intracellular
diplococci) Ve rn a l ke ra t o c o n ju n c t ivit is
TREATMENT: IV ceftriaxone  7 days; bacitracin ointm ent
Form of seasonal (warm m onths), allergic conjunctivitis
topically
High incidence of Chlamydia coinfection; therefore, Male > fem ale (2:1); onset by age 10 years, lasts 2 to10 years,
also use oral erythrom ycin syrup and treat m other and usually resolves by puberty
sexual partners
Associated with atopic derm atitis (75%) or fam ily history of
Other bacteria: days 4 to 5; Staphylococci, Streptococci,
atopy (66%)
Haem ophilus, Enterococci
TREATMENT: bacitracin, erythrom ycin, or gentam icin
ointm ent; fortified antibiotics for Pseudomonas Lim b al ver n al: m ore com m on am ong African Am ericans
HSV: days 5 to 14; type 2 in 70%
FINDINGS: serous discharge, conjunctival injection, Sym p to m s: intense itching, photophobia, pain
keratitis; m ay have vesicular lid lesions
Can have system ic herpetic infection Fin d in gs: large upper tarsal papillae (cobblestones),
DIAGNOSIS: conjunctival scrapings with m ultinucleated m inim al conjunctival hyperem ia, Horner-Trantas’ dots
giant cells; cultures take 5 to7 days (elevated white accum ulations of eosinophils at lim bus),
TREATMENT: Zirgan, 5 Â/d or Viroptic q2h  1 week; lim bal follicles (gelatinous nodules at lim bus), copious ropy
acyclovir, 10 m g/kg IV, tid  10 days m ucus; m ay have pseudom em brane, keratitis, m icropannus,
Chlamydia (neonatal inclusion conjunctivitis): days 5–14; shield ulcer (central oval epithelial defect with white fibrin
m ost comm on infectious cause of neonatal conjunctivitis coating); high levels of histam ine and im m unoglobulin (Ig)E
FINDINGS: acute, m ucopurulent, papillary (no follicles in tears
until 3 m onths of age); m ay have pseudom em branes
Associated with pneum onitis, otitis, nasopharyngitis, Path o lo gy: chronic papillary hypertrophy, epithelial
gastritis hypertrophy then atrophy, conjunctiva contains m any m ast
DIAGNOSIS: intracytoplasm ic inclusions on Giem sa stain cells, eosinophils, and basophils
TREATMENT: topical erythrom ycin, oral erythrom ycin
syrup  2–3 weeks (125 m g/kg/day qid) to prevent Tr eatm en t: topical allergy m edication and steroids; consider
pneum onitis (onset 3–13 weeks later); treat m other topical ciclosporin (cyclosporine) or tacrolim us
and sexual partners with doxycycline,100 m g
bid  1 week (do not use in nursing m others)
Rule out concom itant Gonococcus infection Lig n e o u s
Rare, bilateral, pseudom em branous conjunctivitis in children;
com m only young girls
DDx: traum a, foreign body, corneal abrasion, congenital
glaucoma, NLDO, dacryocystitis Acute onset, chronic course

Pr o p h ylaxis: tetracycline, 1% or erythrom ycin, 0.5% Etio lo gy: appears to be exaggerated response to tissue injury
ointm ent at birth following infection, surgery, or traum a

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CHAPTER 5 • Pediatrics/Strabism us

Fin d in gs: unilateral or bilateral highly vascularized, raised,

friable lesion; with continued inflam m ation, a white, Tum o rs
thickened avascular woody (ligneous) m ass appears above the
neovascular m em brane, usually on palpebral conjunctiva; Ep ib u lb a r o s s e o u s c h o ris t o m a
easily rem oved but bleeds; m ay recur
Hard m ass, com posed of m ature bone
Process affects all m ucous m em branes (m outh, vagina, etc.)
Usually located on bulbar conjunctiva (superotemporal fornix)
Does not enlarge
Path o lo gy: acellular eosinophilic hyaline m aterial
(im m unoglobulins, prim arily IgG), granulation tissue, cellular Not associated with other osteom as
infiltration (T cells, m ast cells, eosinophils) No m alignant potential

Tr eatm en t: com plete excision (expect significant bleeding); Tr eatm en t: observe or excise
any rem aining portion of lesion results in rapid recurrence
because retained lesion acts as physical barrier to topical Co m p le x c h o ris t o m a
m edications; ENT evaluation before surgery to ensure May contain cartilage, ectopic lacrim al gland tissue, sm ooth
respiratory tract not involved because m any require general m uscle, sweat glands, sebaceous glands, hair
anesthesia
Postexcision: topical steroids every hour; hyaluronidase, Isolated or associated with linear nevus sebaceous syndrom e
acetylcysteine, and α-chym otrypsin every 4 hours; topical
antibiotic; topical cyclosporine (ciclosporin) 2% Ec t o p ic la c rim a l g la n d
(applied to surgical bed with sterile cotton-tipped
Fleshy, vascularized tum or with raised translucent nodules
applicator); oral prednisone (1 m g/kg/day); daily
d ebridem ent of any recurrence Usually extends into corneal strom a
Mild growth during puberty
Ka w a s a ki’s d is e a s e (Mu c o c u t a n e o u s lym p h
n o d e s yn d ro m e ) Path o lo gy: lacrim al gland parenchym a
System ic childhood inflam m atory disease/vasculitis with Tr eatm en t: excision
prom inent m ucocutaneous m anifestations
Occurs in children < 5 years old
CORNEAL DISORDERS
More com m on am ong individuals of Japanese descent
Epidem ics suggest exposure to causal agent; siblings have 10Â An t e rio r m e g a lo p h t h a lm o s
increased risk
Large anterior segm ent of eye
More than 50% of fam ilial cases occur within 10 days after
onset of first case Asso ciatio n s: Marfan’s syndrom e, m ucolipidosis type II,
Apert’s syndrom e
Diagn o stic cr iter ia (requires 5 of 6): fever, bilateral
Fin d in gs: high m yopia and astigm atism ; enlarged cornea,
conjunctivitis (90%), mild bilateral nongranulom atous uveitis
lens, iris, and ciliary ring; iris transillum ination defects, lens
(80%), rash, cervical lym phadenopathy, oral lesions (fissures,
dislocation, cataract
strawberry tongue), lesions of extrem ities (edem a, erythem a,
desquam ation)
Co n g e n it a l c o rn e a l s t a p h ylo m a
Associated with polyarteritis
Protuberant corneal opacity due to intrauterine keratitis

Fin d in gs: bilateral conjunctival injection, iridocyclitis, Fin d in gs: atrophic iris adheres to back of m arkedly
punctate keratitis, vitreous opacities, papilledem a, and thickened, scarred, and vascularized cornea; lens m ay be
subconjunctival hem orrhages adherent to posterior cornea; cornea m ay perforate

Co rn e a p la n a (AD o r AR)
Tr eatm en t: aspirin; system ic steroids contraindicated
(increased risk of coronary artery aneurysm ) Mapped to chrom osom e 12q

Asso ciatio n s: sclerocornea, m icrocornea, and angle-closure

Pr o gn o sis: 0.3% m ortality
glaucom a

Co m p licatio n s: 15% develop coronary arteritis (can lead to Fin d in gs: flat cornea with curvature equaling that of sclera
coronary artery aneurysm or m yocardial infarction) (usually 20–30 D), diffuse scarring and vascularization; m ay

102
 Corneal Disorders

have colobom a, cataract, sclerocornea, shallow anterior An t e rio r s e g m e n t d ys g e n e s is (Me s o d e r m a l

cham ber (AC), refractive error d ys g e n e s is s yn d ro m e s )
Bilateral, congenital, hereditary disorders affecting anterior
Path o lo gy: thickened epithelium , absent basem ent
segm ent structures
m em brane, very thin Descem et’s, anterior third of strom a is
scarred and vascularized
Axen feld ’s an o m aly: posterior em bryotoxon (anteriorly
Me g a lo c o rn e a displaced Schwalbe’s line; found in 15% of healthy
individuals) with iris processes to scleral spur; 50% develop
Horizontal diam eter of cornea greater than 12 m m in glaucom a; AD; m apped to chrom osom es 4q25 (RIEG1),
newborns (> 13 m m in adult) 13q14 (RIEG2), 6p25(FOXC1), 11p13 (PAX6)
Nonprogressive; m ost comm only X-linked (associated with
anterior m egalophthalm os), bilateral, 90% m ale; also AR Alagille’s syn d r o m e: Axenfeld’s plus pigm entary
retinopathy, corectopia, esotropia, and system ic abnorm alities
Asso ciatio n s: Marfan’s syndrom e, Alport’s syndrom e, (absent deep tendon reflexes, abnorm al facies, pulm onic
Down syndrom e, dwarfism , craniosynostosis, facial valvular stenosis, peripheral arterial stenosis, biliary
hem iatrophy hypoplasia, and skeletal abnorm alities); m apped to
chrom osom e 20p12 (JAG1); ERG and EOG are abnorm al
Fin d in gs: large cornea; m ay have weak zonules, lens
subluxation, hypoplastic iris, and ectopic pupil Rieger ’s an o m aly: Axenfeld’s plus iris hypoplasia with
holes; glaucom a; m apped to chrom osom es 4q25 (RIEG1),
Co m p licatio n s: ectopia lentis (enlarged lim bal ring 13q14 (RIEG2), 6p25 (FOXC1), and 11p13 (PAX6)
stretches the zonular fibers), glaucom a (due to angle
abnorm alities), cataract (PSC) Rieger ’s syn d r o m e: Rieger’s anom aly plus m ental
retardation and system ic abnorm alities (dental, craniofacial,
Mic ro c o rn e a genitourinary, and skeletal)
Corneal diam eter less than 9 m m in newborns (< 10 m m
in adult) Peter ’s an o m aly: central corneal leukom a (opacity due to
defect in Descem et’s m em brane with absence of endothelium )
Autosom al dom inant or sporadic; unilateral or bilateral with iris adhesions; lens involvem ent (cataract) m ay occur and
50% develop glaucoma; also associated with cardiac,
Etio lo gy: arrested corneal growth after 5th month of fetal craniofacial, and skeletal abnorm alities; usually sporadic,
development bilateral (80%); AR or AD; m apped to chrom osom es 11p13
(PAX6), 4q25-q26 (PTX2), 2p21-p22 (CYP1B1), and
Asso ciatio n s: dwarfism , Ehlers-Danlos syndrom e 6p25 (FOXC1)

Fin d in gs: sm all cornea; often hyperopic (relatively flat DDx: m nem onic STUMPED (Box 5-1)
corneas); m ay have cataract, colobom a, persistent hyperplastic
prim ary vitreous (PHPV), m icrophakia; m ay develop angle-
closure glaucom a or prim ary open-angle glaucom a (POAG) S c le r o c o rn e a
Scleralized (white vascularized opacification) cornea;
DDx: nanophthalm os (sm all but structurally norm al eye), peripheral or entire
anterior m icrophthalm os (sm all anterior segm ent),
Nonprogressive; sporadic (50%) or hereditary (50%; AD or
m icrophthalm os (sm all m alform ed eye)
AR), m apped to chrom osom e 14; 90% bilateral

P o s t e rio r ke ra t o c o n u s Corneoscleral lim bus indistinct

Discrete posterior corneal indentation with strom al haze and

Bo x 5-1. Differential diagnosis of congenital cloudy cornea: STUMPED
thinning
Sclerocornea
Fem ale > m ale Tears in Descemet’s membrane
Nonprogressive, usually central and unilateral Ulcers
Metabolic disease
Anterior corneal surface is norm al
P eter’s anomaly
Vision usually good; causes irregular astigm atism Edema (CHED)
Dermoids
Path o lo gy: loss of strom al substance; Descem et’s Others: Congenital stromal corneal dystrophy (CSCD), rubella, posterior ulcer
m em brane m ay be thinned, but both it and endothelium of von Hippel, posterior keratoconus, congenital corneal staphyloma
are intact

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CHAPTER 5 • Pediatrics/Strabism us

Asso ciatio n s: persistent pupillary m em brane, congenital Fin d in gs: flaky, feathery white strom al opacities clear
glaucom a, cornea plana (80%) peripherally; no corneal edem a
Poor prognosis for corneal transplant
May cause strabism us, nystagm us, and am blyopia
De s c e m e t ’s t e a r/ ru p t u re
De rm o id
May be caused by forceps traum a (vertical or oblique) or
glaucom a (horizontal or concentric to lim bus; Haab’s striae) Sm ooth, solid, yellow-white choristom a that m ay extend into
corneal strom a to cover visual axis or cause astigm atism
Acutely, edem a occurs, scarring develops later
No hereditary pattern; 25% bilateral
May cause astigm atism and am blyopia
Typ es:
Conjunctival: (lim bal) derm oid: Straddles lim bus, m ost
Me ta b o lic Dis o rd e rs com m only in inferotem poral quadrant
(Muc o p o lys a c c ha rid o s e s , Muc o lip id o s e s ) Isolated or associated with Goldenhar’s syndrom e (30%;
includes accessory auricular appendages, aural
(See Table 5-4) fistulas, vertebral body abnorm alities)
May cause astigm atism and am blyopia
Co n g e n it a l h e re d it a ry e n d o t h e lia l Also associated with linear sebaceous nevus syndrom e
d ys t r o p h y (CHED; fo rm e rly CHED2 ) (AR) Dermolipoma of the conjunctiva: Usually located in
superotem poral fornix; can extend deep into orbit
Rare, bilateral, nonprogressive m apped to chrom osom e 20p13
Consists of adipose and connective tissue
(SLC4A11)
High surgical com plication rate, with risk of m arked
Onset at or shortly after birth ptosis, lateral rectus m uscle paresis, and dry eye
Corneal clouding due to edem a from defect of corneal
endothelium and Descem et m em brane Fin d in gs: derm oid, layer of lipid at leading edge in cornea;
m ay cause proptosis, astigm atism , restricted m otility,
No association with other system ic abnorm alities am blyopia

Path o lo gy: thickened collagen fibers covered by skinlike

Fin d in gs: cloudy corneas, (often asym m etric), epithelium ; contains hair, sebaceous and sweat glands, and fat;
corneal edem a, nystagm us, no pain or tearing
lined by squam ous epithelium
Path o lo gy: thickened edem atous strom a, m assively
thickened Descem et’s m em brane, atrophic or nonfunctioning
Tr eatm en t: observation; excision for cosm esis or if visual
axis is blocked (caution: m ay be full thickness; granulom atous
endothelium
reaction if ruptures)
X-lin k e d e n d o t h e lia l c o rn e a l d ys t ro p h y (XECD)
(X-lin k e d d o m in a n t ) Ot h e r c a u s e s o f c o rn e a l o p a c it y

Rare, bilateral, m inim ally progressive (m ales), nonprogressive

In ter stitial ker atitis: congenital syphilis causes keratitis
with edem a followed by strom al vascularization (“salm on
(fem ales); m apped to chrom osom e X
patch”), blood flow stops and ”ghost” vessels rem ain with
Onset at or shortly after birth corneal haze

Fin d in gs: Riley-Day syn d r o m e ( Fam ilial d ysau to n o m ia; AR) :

Males: cloudy corneas (diffuse haze to ground-glass, m ilky autonom ic nervous system dysfunction due to block of NE
appearance), m oon crater-like endothelial changes; production; Eastern European Jews
decreased vision; m ay develop band keratopathy, m ay Findings: decreased corneal sensation, lack of tearing,
have nystagm us poorly reactive pupils, light-near dissociation; m ay
Females: only m oon crater-like endothelial changes develop neurotrophic keratitis with risk of perforation
Other findings: poor pain, tem perature, and taste sensation;
Path o lo gy: m oon crater-like endothelial changes, spinal curvature; increased sweating; constipation;
subepithelial keratopathy, thinning of epithelium am d hypotension
Bowm an layer, thickening of Descem et m em brane with pits, Crisis (lasts 1–10 days): em otional lability, profuse
loss of endothelial cells sweating, postural hypotension, vom iting; treat with
diazepam (Valium ) and hydration
Co n g e n it a l s t ro m a l c o rn e a l d ys t ro p h y Increased risk with general anesthesia: exquisite sensitivity to
(CS C D) (AD) thiopental sodium (Pentothal) (hypotension, cardiac
arrest)
Rare, bilateral nonprogressive, diffuse opacification of cornea;
Diagnosis: high urinary hom o-vanillic acid (HVA) and
m apped to chrom osom e 12q21.33 (DCN)
vanillylm andelic acid (VMA), low
Onset at or shortly after birth hexam ethylphosphorous triam ide (HMPT)

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 Iris Disorders

Infe c tio ns IRIS DISORDERS

S yp h ilis An irid ia
Co n gen ital: m aternal transm ission after fourth m onth of Bilateral absence of iris, com m only a rudim entary iris stum p
gestation (50% for prim ary or secondary syphilis, 30% for exists
untreated or late syphilis)
Incidence 1 in 100,000
Fin d in gs: interstitial keratitis (33%), ectopia lentis, Argyll- Hereditary or sporadic; m apped to chrom osom e 11p13 (PAX6)
Robertson pupil, optic atrophy, panuveitis with various retinal
pigmentary changes (usually salt and pepper or pseudo RP Typ es:
pattern) AN1 (85%): AD, only eye involvem ent
AN2 (13%): sporadic, associated with Wilm s’tum or (Miller
Oth er fin d in gs: syndrom e and WAGR [Wilm s’ tum or, aniridia,
Early: stillbirth, failure to thrive, rhinitis (”snuffles”), genitourinary abnorm alities, and m ental retardation])
osteochondritis, pneum onia, hepatosplenom egaly AN3 (2%): AR, associated with m ental retardation and
Late: Hutchinson’s teeth (peg-shaped), m ulberry m olars, ataxia (Gillespie’s syndrom e)
saber shins, frontal bossing, saddle nose, deafness (5%),
tabes dorsalis Fin d in gs: visual acuity usually 20/100 or worse, foveal and
optic nerve hypoplasia, nystagm us, photophobia, am blyopia,
Hu tch in so n ’s tr iad : interstitial keratitis, Hutchinson’s strabism us
teeth, deafness
Interstitial keratitis (IK): im m une response to treponem al May have cataracts (50%–85%), glaucom a (30%–50%),
antigens ectopia lentis, and corneal pannus
Starts between ages of 5 and 20 years; triggered by m inor
corneal traum a Tr eatm en t: consider cosm etic/ painted contact lenses for
Bilateral with 2nd-eye involvem ent at 1–2 m onths in 50%, photophobia; peripherally painted intraocular lenses (IOLs),
12 m onths in 75% opaque polym ethyl m ethacralate (PMMA) rings, and artificial
3 STAGES: iris im plants have been used with cataract surgery but are not
PROGRESSIVE: pain, photophobia, poor vision; Food and Drug Adm inistration approved
blepharospasm , fine keratic precipitates (KP),
perilim bal injection, diffuse or sectoral corneal haze Co lo b o m a
(ground-glass appearance)
FLORID: acute inflam m atory response; salm on patch Iris sector defect due to incom plete closure of em bryonic
of Hutchinson (cornea appears pink due to deep fissure; usually located inferonasal
vascularization) May have other colobom as (lid, ciliary body, choroid, retina,
RETROGRESSIVE: vessels m eet at center of cornea and optic nerve)
LATE FINDINGS: ghost vessels, Descem et’s folds, guttata,
secondary glaucom a (iris/angle dam age) Associated with trisomy 13, 18, and 22; chromosome 18 deletion;
PATHOLOGY: strom al blood vessels just anterior to Klinefelter’s syndrome, Turner’s syndrome, CHARGE, basal cell
Descem et’s nevus syndrome, Goldenhar’s syndrome, Meckel’s syndrome,
TREATMENT: steroids (do not prevent involvem ent of Rubinstein-Taybi syndrome, linear sebaceous nevus syndrome
fellow eye); system ic penicillin
Tr eatm en t: consider surgical repair in sym ptom atic cases
DDx: acquired syphilis (IK usually sectoral and less severe),
leprosy (superficial avascular keratitis, usually superotem poral Co n g e n it a l iris e c t ro p io n
quadrant; later, leprous pannus of blood vessels, beading of
corneal nerves) Ectr o p io n u veae: ectropion of posterior pigm ent
epithelium onto anterior surface of iris
He rp e s s im p le x viru s (HS V) Associated with neurofibrom atosis, Prader-Willi syndrom e
Often an asym ptom atic prim ary infection before age of 5; 3- to
5-day incubation period Co n gen ital ir is ectr o p io n syn d r o m e: unilateral
congenital iris ectropion, high iris insertion, sm ooth cryptless
Co n gen ital: ocular involvem ent in 10% of iris surface, dysgenesis of angle, and glaucoma
dissem inated cases
Co n g e n it a l iris h yp o p la s ia
Fin d in gs: vesicular skin eruption, conjunctivitis, epithelial
Thin iris strom a with transillum ination of entire iris
keratitis, strom al im m une reaction, cataracts, necrotizing
chorioretinitis Associated with albinism

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CHAPTER 5 • Pediatrics/Strabism us

Co n g e n it a l m io s is Lis c h n o d u le s
May be associated with other anterior segm ent abnorm alities Neural crest ham artom as
Associated with neurofibrom atosis type I
Etio lo gy: absence or m alform ation of dilator pupillae
m uscle or contracture of fibrous m aterial on pupil m argin from Num ber and frequency increase with age
tunica vasculosa lentis rem nant or neural crest cell
Appear as tan nodules, usually in inferior iris
abnorm alities
J u ve n ile xa n t h o g ra n u lo m a (J XG;
Co n g e n it a l m yd ria s is Ne vo xa n t h o e n d o t h e lio m a )
Etio lo gy: iris sphincter traum a, pharm acologic, neurologic Histiocytic proliferation usually of skin
disease
Yellow-orange nodules appear before 1 year of age
Co re c t o p ia Orange because of vascularity (red) com bined with high lipid
Displacem ent of pupil content (yellow)

Isolated or associated with ectopia lentis et pupillae, Axenfeld- May involve iris (m ay cause spontaneous hyphem a)
Rieger syndrom e, iridocorneal endothelial syndrom e, uveitis, May involve m uscles, salivary glands, stom ach, and other
or traum a internal organs

Dys c o ria Rarely associated with an orbital granulom a (which causes

proptosis)
Abnorm ally shaped pupil
Lesions often spontaneously regress by 5 years of age
Isolated or associated with posterior synechiae, Axenfeld-
Reiger syndrom e, ectopia lentis et pupillae Path o lo gy: diffuse non-necrotizing proliferation of
histiocytes with scattered touton giant cells (ring of nuclei
separating a central eosinophilic cytoplasm from peripheral
P e rs is t e n t p u p illa ry m e m b ra n e
foam y [or clear] cytoplasm )
Rem nants of anterior tunica vasculosa lentis that appear as fine
iris strands Tr eatm en t: iris lesions are treated with steroids, XRT, and
excision
Com m on congenital ocular anom aly
Me d u llo e p it h e lio m a (Dikt yo m a )
Rarely visually significant
Prim ary neoplasm of ciliary body neuroectoderm (arises in
Typ e I: iris to iris, bridging pupil nonpigmented epithelium); arises from primitive m edullary
epithelium that lines neural tube; can also arise in retina and ON
Typ e II: iris to lens; m ay have associated anterior polar Occurs in both benign and m alignant form s (locally invasive
cataract but lim ited m etastatic potential)
May have heterotopic elem ents; no calcification
P rim a r y iris c ys t s
Unilateral, unifocal, usually arises before 6 years of age; no
Due to spontaneous separation of pigm ented and hereditary pattern
nonpigm ented epithelium
Occur anywhere between pupil and ciliary body
Typ es:
Nonteratoid or simple: pure proliferation of em bryonic
Miotics can cause cysts at pupillary border nonpigm ented ciliary epithelium
Teratoid: contains heterotopic elem ents such as cartilage,
Congenital strom al cysts occur in infants and young children
brain tissue, and rhabdom yoblasts
from sequestration of epithelium during fetal developm ent
Fin d in gs: decreased vision, pain, strabism us, leukocoria, iris
Bru s h fie ld ’s s p o t s m ass, fleshy pink to white peripheral fundus tum or; rubeosis,
hyphem a, glaucom a, lens colobom a occurs in som e
Focal areas of iris strom al hyperplasia surrounded by relative congenital cases
hypoplasia
Appear as ring of peripheral, elevated, white-gray spots Path o lo gy: undifferentiated round to oval cells containing
(10–20/eye) little cytoplasm, organized into ribbon-like structures that have
distinct cellular polarity; lined on one side by thin basem ent
Occur in 85% of Down syndrom e patients
m em brane; stratified sheets of cells are capable of form ing
May be found in norm al individuals (Kunkm ann-Wolffian m ucinous cysts that are clinically characteristic; Flexner-
bodies) Wintersteiner and Hom er-Wright rosettes can be seen; called

106
 Lens Disorders

teratoid m edulloepith eliom a when com posed of cells from 2 Co n g e n it a l c a t a ra c t s

different em bryonic germ layers; m ay contain cartilage, brain
tissue, and rhabdom yoblasts
Ch ar acter istics:
Bilateral: usually AD; consider diabetes, galactosem ia, or
Lowe’s syndrom e; require m etabolic workup and
Tr eatm en t: resection (iridocyclectomy) or enucleation (not treatm ent by age 3 m onths, or irreversible nystagm us
radiosensitive) with poor visual acuity ( 20/200) occurs. Opacities
greater than 3 m m can be visually significant. Surgery
Pr o gn o sis: good if no extraocular extension; rarely often perform ed on the better-seeing eye first:
m etastasizes lensectom y, anterior vitrectom y, and contact lens fitting
for infants; posterior capsulotom y is necessary due to
significant postoperative inflam m ation, which causes
LENS DISORDERS posterior capsular opacification
Unilateral: generally not m etabolic or genetic; therefore,
laboratory testing is not needed. Usually local dysgenesis
Co n g e n it a l a n o m a lie s
(PHPV, anterior polar or posterior lenticonus), often
Mitten d o r f’s d o t: sm all white opacity on posterior lens presents with leukocoria and strabism us. Requires
capsule that represents a rem nant of the posterior vascular treatm ent by 6–8 weeks of life
capsule (tunica vasculosa lentis) where hyaloid artery is
inserted
Typ es: classified by location or etiology
Polar (subcapsular cortex and capsule): anterior or
Ch icken tr acks (epicapsular star): brown or golden flecks posterior, sporadic, or AD
on anterior lens capsule; rem nant of anterior tunica vasculosa ANTERIOR: usually small, bilateral, symm etric,
lentis nonprogressive; good visual prognosis; 90% are
idiopathic. Remnant of the hyaloid system. Associated
Len tico n u s: cone-shaped lens deform ity due to central with m icrophthalm os and anterior lenticonus
bulge in area of thin capsule; causes irregular m yopic PATHOLOGY: fibrous plaque beneath folded anterior
astigm atism ; ”oil-droplet” sign on retinoscopy; associated with capsule secreted by irritated m etaplastic epithelial
cataract cells
Anterior: bilateral, m ale > fem ale; associated with Alport’s POSTERIOR: larger, usually stable, but m ay progress;
syndrom e (anterior lenticonus, hereditary nephritis, and m ore visually significant; AD (bilateral) or sporadic
deafness) (unilateral); often with associated defect of posterior
Posterior: unilateral, sporadic, fem ale > m ale, m ore capsule
com m on than anterior type, am blyopia com m on Associated with rem nants of the tunica vasculosa lentis,
posterior lenticonus, or lentiglobus
Len s co lo b o m a: focal flattening of lens edge due to absence PATHOLOGY: posterior m igration of lens epithelium
of inferior zonules from ciliary body colobom a; not a true Sutural (AR): bilateral opacities of Y sutures; rarely
colobom a affects vision. Occurs during developm ent of fetal lens
nucleus
Len tiglo b u s: generalized hem ispherical deform ity, very rare Nuclear (usually AD): bilateral, opacification of
em bryonic fetal nucleus; typically axial, dense, bilateral,
Micr o p h akia: sm all lens due to arrested developm ent; and larger than 3 m m . Associated with sm all eye
associated with Lowe’s syndrom e Anterior pyramidal: congenital anterior subcapsular
Lamellar/zonular: bilateral, sym m etric, appears like a sand
dollar; circum scribed zone of opacity within lens,
Micr o sp h er o p h akia: sm all, spherical lens, usually surrounding the nucleus. May be due to transient toxic
bilateral; zonules visible on pupillary dilation; iridodenesis;
exposure during em bryogenesis (neonatal tetany); can
zonule rupture is com m on, pupillary block m ay occur
be AD. Usually does not interfere with vision
especially with use of m iotics (treat with cycloplegic to tighten
Complete: no red reflex; unilateral or bilateral
zonules, flattening the lens and pulling it posteriorly)
Membranous: lens proteins resorb following traum a;
Associated with Weill-Marchesani syndrom e, hyperlysinem ia, anterior and posterior capsules fuse into a dense white
Lowe’s syndrom e, Alport’s syndrom e, congenital rubella, and m em brane
Peter’s anom aly Crystalline: rare, bilateral congenital cataract, with refractile,
Treatment: cycloplegic (tightens zonules, flattening lens and rhom boid crystals (containing tyrosine and cysteine)
pulling it posteriorly) radiating outward from the center of the lens into the
juvenile nucleus
Co n gen ital ap h akia: rare, absence of lens Anterior axial embryonic: m ost com m on type of congenital/
infantile cataract. White, clustered, punctate opacities
near the Y sutures; not visually significant
Ec t o p ia le n t is
Pulverulent (AR): central, translucent, ovoid, and cluster of
(See Chapter 10, Anterior Segm ent) dot-like opacities in the fetal nucleus

107
CHAPTER 5 • Pediatrics/Strabism us

Coronary (AR): wreath of peripheral cortical opacities that OTHER FINDINGS: angiokeratom as, cardiovascular
encircle the nucleus in a radial fashion; sm aller punctate abnorm alities, renal disorders, bouts of pain in digits
bluish opacities within the nucleus. Associated with Hypocalcemia: either idiopathic or following surgery of
Down syndrom e parathyroid glands
Punctate iridescent opacities in anterior and posterior
Etio lo gy: One-third hereditary; one-third associated with cortex
system ic syndrom es; one-third of unknown origin Lowe’s (oculocerebrorenal) syndrome (X-linked): defect of
am ino acid m etabolism ; m ale > fem ale
Etio lo gy o f b ilater al catar acts: Idiopathic (60%) FINDINGS: congenital cataract (100%), usually bilateral;
Intrauterine infection (3%): TORCH syndrom es sm all, thin, discoid lens (m icrophakic) associated
(Toxoplasm osis, Other infections (syphilis; also with retained lens nuclei; glaucom a (50%);
hepatitis B, Varicella-Zoster virus, HIV, parvovirus B19), congenital cataract and glaucom a are very rare
Rubella, CMV, HSV) the alternate acronym is TORCHES Fem ale carriers have white, punctate cortical opacities
(TOxoplasm osis, Rubella, CMV, HErpes sim plex, and subcapsular plaquelike opacities
Syphilis) OTHER FINDINGS: renal tubular acidosis, am inoaciduria,
Associated with ocular disorders: Leber’s congenital renal rickets, m ental retardation, m uscular hypotonia,
am aurosis, retinitis pigm entosa (RP), PHPV, retinopathy failure to thrive
of prem aturity (ROP), aniridia, Peter’s anom aly, ectopia Alport’s syndrome (X-linked): triad of anterior lenticonus,
lentis, posterior lenticonus, uveitis, tum ors deafness, and hem orrhagic nephropathy/renal failure
(retinoblastom a, m edulloepitheliom a) FINDINGS: conjunctival calcium crystals, corneal
Metabolic: galactosem ia, hypocalcem ia, Lowe’s syndrom e, endothelial pigment, juvenile arcus, spherophakia,
congenital hem olytic jaundice, hypoglycem ia, anterior polar cataract, retinal changes similar to retinitis
m annosidosis, Alport’s syndrom e, Fabry’s disease pigmentosa or fundus albipunctatis, optic nerve drusen
Hereditary (30%, usually AD): Fem ale carriers have lenticular changes
WITHOUT SYSTEMIC ABNORMALITIES: AD, AR, X-linked DIAGNOSIS: renal or skin biopsy (lack of α-5 type IV
WITH CHROMOSOMAL ABNORMALITIES: trisomy 18, collagen in glomerular and epidermal basement
trisomy 21 (Down syndrome), Turner’s syndrome, membranes)
trisomy 13 (Patau’s syndrome),”‘cri-du-chat” syndrome Hallermann-Streiff syndrome (mandibulo oculofacial
CRANIOFACIAL SYNDROMES: Crouzon’s syndrom e, dysmorphia): hypoplasia of m andible with birdlike facies;
Apert’s syndrom e, Hallerm ann-Streiff syndrom e, one of the few syndrom es with com bined cataract and
Pierre Robin sequence glaucom a
CNS ABNORMALITIES: Zellweger syndrom e, Torsten- FINDINGS: m icrophakia, m icrocornea, glaucom a, and
Sjo€ gren syndrom e, Marinesco-Sjo€ gren syndrom e, cataract (can develop within 1st few weeks of life);
Lawrence-Moon-Biedl-Bardet syndrom e, Norrie spontaneous rupture of lens capsule with absorption
disease, neurofibrom atosis of lens proteins can occur; im m une response to lens
SKIN ABNORMALITIES: Cockayne’s syndrom e, proteins resem bles phacoanaphylactic uveitis
Rothm und-Thom son syndrom e, Werner’s syndrom e, Intrauterine infections: usually occur early in first trim ester
atopic derm atitis, ichthyosis, incontinentia pigm enti because lens capsule is form ed during week 5 of
Maternal drug ingestion / malnutrition em bryogenesis; rubella, HSV, m um ps, toxoplasm osis,
Trauma vaccinia, CMV
Hypoglycemia during pregnancy: congenital lenticular
Sp ecific en tities: opacities; associated with optic atrophy, mental retardation
Galactosemia (AR): defect in 1 of 3 enzym es (galactose-1-P- Down syndrome: snowflake cataract, keratoconus
uridyl transferase [m ost com m on], galactokinase, or
Diagn o sis o f b ilater al catar acts: if AD pattern
uridine diphosphate [UDP] galactose-4-epim erase)
determ ined, no workup is necessary
causes inability to convert galactose into glucose;
Urinalysis: am ino acids (Lowe’s syndrom e), reducing
galactose is converted into galactitol, which serves as
substance after m ilk feeding (galactosem ia)
osm otic agent for influx of fluid
Blood tests: calcium (hypocalcem ia/hyperparathyroidism ),
FINDINGS: oil-droplet cataract (reversible early on)
glucose (hypoglycem ia), red blood cell galactokinase,
OTHER FINDINGS: m ental retardation, hepatom egaly,
TORCH titers
jaundice, and m alnutrition within first few weeks
Karyotyping: trisom y 13 (Patau’s syndrom e), 18, and 21
of life
(Down syndrom e); Turner’s syndrom e, ”cri-du-chat”
TREATMENT: elim inate lactose from diet; fatal if untreated
syndrom e
Mannosidosis: α-m annosidase deficiency causes Hurler-like
Bilateral audiograms: congenital rubella, Alport’s syndrome
syndrom e
B-scan ultrasound: if no view of fundus
FINDINGS: posterior spoke-like opacity; no corneal
changes (unlike Hurler’s) Etio lo gy o f u n ilater al catar acts:
OTHER FINDINGS: m ental retardation, short stature, Idiopathic (80%):
skeletal changes, hepatosplenom egaly Ocular abnormalities (10%): PHPV, posterior lenticonus
Fabry’s disease: α-galactosidase A deficiency (90% unilateral), anterior segm ent dysgenesis, tum ors
FINDINGS: cornea verticillata; spoke-like cataract in 25% (retinoblastom a, m edulloepitheliom a)

108
 Glaucom a

Trauma (9%): Prima ry infa ntile gla ucoma (congenita l gla ucoma , tra beculodysgenesis)
Intrauterine infection: rubella; 33% unilateral
Seconda ry infantile glaucoma
Associa ted with mesoderma l Iridocorneotra beculodysgenesis
Diagn o sis o f u n ilater al catar acts: rule out traum a neura l crest dysgenesis • Rieger’s a noma ly or syndrome
(child abuse), TORCH titers
• Axenfeld’s a noma ly or syndrome
• Peters’ a noma ly
DDx o f leu ko co r ia (white pupil): cataract, retinoblastom a, • systemic hypopla stic mesoderma l
toxoplasm osis, toxocariasis, RD, ROP, PHPV, Coats disease, dysgenesis (Ma rfa n’s syndrome)
colobom a, m yelinated nerve fibers, retinal dysplasia, Norrie • systemic hyperpla stic mesoderma l
dysgenesis (W eill–Ma rchesa ni syndrome)
disease, incontinentia pigm enti, retinoschisis, cyclitic
m em brane, m edulloepitheliom a Iridotra beculodysgenesis (a niridia )

Associa ted with pha ko- Neurofibroma tosis (Von Recklingha usen’s disea se)
ma toses a nd ha ma rtoma s
DDx o f co n gen ital catar acts an d glau co m a: Lowe’s Encepha lotrigemina l a ngioma tosis
(Sturge–W eber syndrome a nd va ria nts,
syndrom e, rubella, Hallerm ann-Streiff syndrom e
e.g., Klippel–Tréna una y–W eber syndrome)
Rubella: due to m aternal infection late in the first trim ester
Angioma tosis retina e et cerebelli
of pregnancy
O culoderma l mela nocytosis
FINDINGS (in 50%): cataract (usually bilateral, pearly
white nuclear opacification; retention of lens nuclei in Associa ted with O culocerebrorena l syndrome (Lowe’s syndrome)
meta bolic disea se
em bryonic nucleus; virus rem ains viable in lens for Homocystinuria
years: viral shedding following cataract surgery can Associa ted with Ma terna l rubella syndrome (congenita l rubella )
lead to intense and persistent AC inflam m ation), salt infla mma tory disea se Herpes simplex iridocyclitis
and pepper fundus (norm al ERG) (Fig. 5-24),
Associa ted with Juvenile xa nthogra nuloma (nevoxa nthoendothelioma )
glaucoma (usually either cataract or glaucom a, rarely
mitotic disea se Retinobla stoma
both), m icrophthalm os (15%), necrotizing
iridocyclitis, corneal clouding Trisomy 1 3–1 5 syndrome (Pa ta u’s syndrome)
Associa ted with other
OTHER FINDINGS (especially during 1st trim ester): congenita l disea se Rubinstein–Ta ybi syndrome
cardiac defects (patent ductus arteriosis), deafness Persistent hyperpla stic prima ry vitreous
(infection of the organ of Corti [90%]), and m ental Congenita l ca ta ra ct
retardation • in pha kic eyes
• in a pha kic eyes following surgery
Co m p licatio n s o f su r ger y: chronic aphakic glaucom a
(15%). Usually discovered 5–6 years after surgery; increased Fig ure 5-6. Classification of the congenital and infantile glaucomas.
(From Yanoff M, Duker J S [eds ]: Ophthalmology, London, 1999, Mosby.)
risk with traum a, m icrocornea, PHPV, preexisting anterior
segm ent abnorm alities, and retained lens m aterial

Pr o gn o sis: P rim a r y c o n g e n it a l g la u c o m a
Good: lam ellar cataracts (later onset and less risk of
Etio lo gy: m apped to chrom osom es 1p36 (GLC3B),
glaucom a)
2p21-p22 (GLC3A, CYP1B1); a m utation in the CYP1B1 gene
Intermediate: nuclear cataracts
accounts for $ 85% of congenital glaucom a. AR in 10%;
Poor: total cataracts (high incidence of m icrocornea, poor
affected parent has 5% chance of having a child with infantile
pupillary dilation, and increased risk of glaucom a).
glaucom a
Microphthalm os, strabism us, nystagm us, and
am blyopia; 90% of patients with visually significant
congenital bilateral cataracts will develop nystagm us if Ep id em io lo gy: occurs in 1 of 12,500 births; 40% present at
not treated by 2 m onths of age; nystagm us and birth; 86% present during first year of life; 70% bilateral;
am blyopia m ay not resolve even after cataract surgery 70% m ales

Sym p to m s: tearing, photophobia, blepharospasm , eye

GLAUCOMA rubbing. If younger than age 3 m onths, usually presents with
corneal clouding or tearing; older than 3 years of age, usually
asym ptom atic with progressive m yopia and insidious VF loss
Ch ild h o o d g la u c o m a
Several types of glaucom a typically categorized by age of onset Fin d in gs: IOP > 21 m m Hg; C/D ratio > 0.3 (present in only
Congenital (occurs in infants < 3 m onths old; m ay be prim ary, 2.6% of normal infants; cupping is reversible in childhood);
secondary, or associated with a syndrom e) buphthalm os (“bull’s-eye”; horizontal corneal diam eter
> 13 m m ; lim bal ectasia; stretching of zonules can lead to lens
Infantile (between 3 m onths and 3 years of age) subluxation; irreversible); corneal clouding/edem a/Haab’s
striae (circum ferential or horizontal Descem et’s ruptures [vs.
Juvenile (between 3 and 35 years of age)
oblique or vertical with forceps injury]; m ay result in chronic
Types (Fig. 5-6) corneal edem a, scarring, and astigm atism ); m yopia

109
CHAPTER 5 • Pediatrics/Strabism us

Angle is of neural crest origin, as are facial bones, teeth, Tr eatm en t: definitive treatm ent is surgical; m edication is a
cartilage, and m eninges; therefore, congenital glaucom a m ay tem porizing m easure
be associated with m alform ation of these structures Goniotomy: perform in child < 1 1 2 years of age; TM incised
under direct gonioscopic visualization; requires clear
cornea; 77% success rate
Oth er o cu lar asso ciatio n s: m icrocornea, cornea plana,
Trabeculotomy (ab externo): if cornea hazy, if > 1 1 2 years
sclerocornea, Axenfeld’s/Reiger’s/Peter’s anomaly (50%),
aniridia (50–75% develop glaucoma by teens due to increased old, or if goniotom y fails twice; Schlem m ’s canal is
episcleral venous pressure, iris stump blocking TM, or angle entered via an external incision, and the trabeculotom e
malformation or agenesis), microspherophakia, rotates into the AC and tears the TM; 77% success rate
If goniotom y and trabeculotom y fail, consider
nanophthalm os, PHPV, ROP, tumors (retinoblastoma [RB], JXG,
trabeculectom y with m itom ycin C, drainage im plant,
medulloepithelioma), inflamm ation, traum a, steroid-induced
cycloablation of CB

Asso ciated syn d r o m es: Lowe’s syndrom e (50%); Sturge-

Weber syndrom e (50%, especially if nevus flam m eus involves UVEITIS
upper lid, due to prim ary defect in angle and increased
episcleral venous pressure); neurofibrom atosis (25% if
plexiform neurofibrom a involves upper lid; m ay have Ante rio r Uve itis
ham artom atous infiltration of angle); congenital rubella (2%–
15%); also Marfan’s syndrom e, hom ocystinuria, Weill- DDx: JRA traum a, infection, tum or, sym pathetic ophthalm ia,
Marchesani syndrom e, Rubenstein-Taybi, Pierre Robin, nevus sarcoidosis, phacoantigenic
of Ota, trisom y 13 (Patau’s), Hallerm ann-Streiff, Stickler’s
syndrom e, m ucopolysaccharidoses (Hurler’s and Hunter’s) J u ve n ile rh e u m a t o id a rt h rit is (J RA)
Most com m on cause of uveitis in children: seronegative (RFÀ),
Diagn o sis: ANA-positive, pauciarticular (< 5 joints) arthritis in children
Examination under anesthesia (EUA): usually required for < 16 years old; m ostly girls
com plete evaluation. Rem em ber, ketam ine and
Typ es (Table 5-2)
succinylcholine raise IOP, general anesthesia (halothane,
Pauciarticular (< 5 joints), early onset: 80% fem ale; 30%
thiopental, tranquilizers, and barbituates) lowers IOP.
with chronic iridocyclitis; 25% of all JRA; RFÀ; 60%
Best tim e for IOP m easurem ent is just as patient goes
ANA+
under and is not too deep
Pauciarticular, late onset: 90% m ale; 15% of all JRA; RFÀ;
Gonioscopy: landm arks are often poorly recognized due to
ANAÀ; 75% HLA-B27; 15% acute iridocyclitis
”Barkan’s m em brane” covering TM (but no histologic Polyarticular (> 5 joints): 85% fem ale; iridocyclitis rare;
evidence of such a structure) 40% of all JRA; 75% are RFÀ
1. Open-angle with anterior iris insertion above scleral
Still’s disease: throughout childhood; 60% m ale; sm all and
spur (usual configuration is flat iris insertion into
large joints; iridocylitis rare; 20% of all JRA; RFÀ; ANAÀ;
trabecular m eshwork; less com m only, concave
high fever, rash, organom egaly, polyserositis
insertion with plane of iris posterior to scleral spur
and anterior iris sweeping upward and inserting Fin d in gs: chronic anterior uveitis (30%, usually bilateral;
into TM) eyes are white and quiet even with active ocular inflam m ation
2. Thickening of TM [lots of flare]), glaucom a (20%), cataract (40%), band
3. Peripheral iris strom al hypoplasia keratopathy (40%)

Ta b le 5-2. Classification of juvenile rheumatoid arthritis

P a uc ia rtic ula r P a uc ia rtic ula r Sys te m ic

Typ e P o lya rtic ula r RF-Ne g a tive P o lya rtic ula r RF-P o s itive typ e I typ e II (Still’s d is e a s e )
Age Any Late childhood Early childhood Late childhood Any
Sex 90% girls 80% girls 80% girls 90% boys 60% boys
RF Negative Positive Negative Negative Negative
ANA 25% 75% 60% Negative Negative
HLA-B27 N/A N/A N/A 75% N/A
J oints Any Any Large joints (knee, Large joints (hip, Any
elbow, ankle) SI joints)
Uveitis Rare No 30% Chronic 15% Acute Rare
Other findings Low-grade fever, delayed Low-grade fever, anemia, Few Few High fever, rash,
growth, anemia, malaise malaise, rheumatoid nodules organomegaly,
polyserositis
antinuclear antibody (ANA), xx; HLA, human leukocyte antigen; RF, rheumatoid factor; SI, sacroiliac.

110
 Uveitis

Oth er fin d in gs (30%): arthritis, fever, lym phadenopathy,

m aculopapular rash, m yocarditis, hepatosplenom egaly

Tr eatm en t: topical steroids and cycloplegic; consider

system ic steroids or sub-Tenon’s steroid injection; treat
com plications. May require im m unosuppressive agents, EDTA
chelation for band keratopathy, treatm ent of secondary
glaucom a, cataract surgery (eyes should be without AC reaction
for at least 3 months; intraoperatively, anterior vitrectom y,
synechialysis, rem oval of cyclitic m em branes; no IOL; m ay be
com plicated by hypotony)

Inte rm e d ia te Uve itis

Fig ure 5-7. Congenital toxoplasmic retinitis. Note inactive satellite scars at the
P a rs p la n it is macula, the inferior juxtapapillary scar, and the temporal pallor of
the disc. (From Khanna A, Goldstein DA, Tessler HH: Protozoal posterior uveitis.
(See Chapter 8, Uveitis) In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

P o s te rio r Uve itis Oth er fin d in gs:

Congenital toxoplasmosis: stillbirth, m ental retardation,
seizures, hydrocephalus, m icrocephaly, intracranial
DDx: toxoplasm osis, toxocariasis, presum ed ocular calcifications, hepatosplenom egaly, vom iting, diarrhea
histoplasm osis syndrom e (POHS), HSV, syphilis, Lym e
Acquired toxoplasmosis: rash, m eningoencephalitis, flu-like
disease, sym pathetic ophthalm ia, m asquerade syndrom es (RB,
syndrom e
leukem ia, lym phom a, m elanom a, JXG, intraocular foreign
body, RRD, RP, MS)
Path o lo gy: round Toxoplasma cysts (Fig. 5-8); chronic
granulom atous choroiditis
To xo p la s m o s is
Due to infection with Toxoplasma gondii (obligate intracellular Diagn o sis: ELISA or im m unofluorescence assay (IFA) for
parasitic protozoan), usually congenital (m aternal infection Toxoplasma IgG or IgM
during gestation)
70% of wom en are seronegative
Tr eatm en t:
Indications: decreased vision; m oderate to severe vitreous
Prim ary retinal infection with coagulative necrosis and inflam m ation; lesions that threaten m acula,
secondary granulom atous choroiditis with vitritis; intraretinal papillom acular bundle, or optic nerve; sm all peripheral
cysts cause recurrent disease lesions m ay be observed (heal spontaneously)
Antibiotics: kill tachyzoites in retina during active retinitis,
Most com m on cause of posterior uveitis (25%); 98% congenital
but do not affect cysts
Most com m on cause of pediatric uveitis (50% of posterior CLINDAMYCIN: 300 m g, qid (risk of pseudom em branous
uveitis in children) colitis)
Tachyzoite form is responsible for inflam m ation

Co n gen ital in fectio n : transplacental transm ission of

T. gondii
First-trimester infection: neonatal convulsions, intracerebral
calcifications, retinitis
Later infection: m ay have retinitis only

Fin d in gs: inactive chorioretinal scar in posterior pole, often

in m acula (Fig. 5-7); active focal white fluffy lesion (“headlight
in fog” appearance) occurs adjacent to old scar with
granulom atous uveitis and vitritis; m ay have white spots along
arterioles (Kyrieleis’ plaques); m ay have m icrophthalm ia,
nystagm us, strabism us
In AIDS: head CT m ay show ring-enhancing lesions;
m inim al AC reaction and vitritis because Fig ure 5-8. Viable and necrotic cysts of Toxoplasma gondii in the necrotic
im m unocom prom ised host is unable to m ount norm al retina. (From Khanna A, Goldstein DA, Tessler HH: Protozoal posterior uveitis. In
im m une response Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

111
CHAPTER 5 • Pediatrics/Strabism us

Ta b le 5-3. Clinical presentations of toxocariasis

Chro nic Lo c a lize d P e rip he ra l

e nd o p htha lm itis g ra nulo m a g ra nulo m a
Age range 2–9 years 6–14 years 6–40 years
Lesion Exudation filling Single localized Peripheral
vitreous cavity, granuloma in granuloma with
cyclitic membrane macula or dense fibrotic
peripapillary strand, often to
region disc
Symptoms Pain, photophobia, Quiet eye, Decreased
lacrimation, decreased vision,
decreased vision, vision, strabismus
acute inflammation strabismus
Course Often leads to Nonprogres sive Nonprogressive
destruction of globe

Fig ure 5-9. Typical toxocara granuloma located over the optic nerve. (In: Yanoff
SULFADIAZINE: 2 g loading, then 1 g qid M, Duker J S [eds ]: Ophthalmology, London, 1999, Mosby, chapter 173.)
PYRIMETHAMINE (DARAPRIM): 75 m g loading, then
25 m g daily (bone m arrow depression; prevent with
use of folinic acid [leucovorin, citrovorum factor;
3 to5 m g, 2 times/week]) RETINAL DISORDERS
ALTERNATIVE: trim ethoprim -sulfam ethoxazole
(Bactrim )
Steroids: system ic and periocular added after a few days of P e rs is t e n t h yp e rp la s t ic p r im a ry vit re o u s (P HP V;
antibiotic therapy and should never be given without p e rs is t e n t fe t a l va s c u la t u re [P FV])
concom itant antibiotics Unilateral m icrophthalm ia with spectrum of findings from
prom inent hyaloid vessel rem nant with large Mittendorf’s dot
To xo c a ria s is and Bergm eister’s papillae to angle-closure from fibrovascular
Due to infection with 2nd-stage larval form of dog invasion of lens through posterior lens capsule
roundworm Toxocara canis (ocular larva m igrans) Due to incom plete regression of tunica vasculosa lentis and
Acquired by ingestion of contam inated soil prim ary vitreous
Sporadic, 90% unilateral
Ocu lar lar va m igr an s: usually unilateral, solitary lesion;
does not com plete life cycle (stool exam ination unnecessary),
no other findings Fin d in gs: m icrophthalm ia, vascularized retrolental plaque
(m ay contain cartilage), elongated ciliary processes, prom inent
radial vessels on iris surface, shallow AC, iris vascularization;
Viscer al lar va m igr an s: fever, lym phadenopathy,
m ay have cataract, angle-closure glaucom a, vitreous
hepatom egaly, pneum onitis, eosinophilia, no eye involvem ent
hem orrhage, retinal detachm ent
Fin d in gs: 3 clinical presentations depending on patient age
(endophthalm itis, localized granulom a, or peripheral
granulom a) (Table 5-3); vitreous abscess; dragging of m acula
DDx o f in tr ao cu lar car tilage: PHPV,
m edulloepitheliom a, teratom a, trisom y 13
tem porally owing to peripheral lesion results in apparent XT;
often presents with leukocoria; traction RD can occur (Fig. 5-9)
Tr eatm en t: observation, lensectom y with or without
Oth er fin d in gs: m ay affect lungs and liver vitrectom y
DDx: as for leukocoria
Pr o gn o sis: depends on degree of am blyopia; visual
Diagn o sis: AC tap for eosinophils; ELISA for Toxocara prognosis variable after surgery, often depends on status of
antibody titers; no ova/parasites in stool
posterior segm ent

Tr eatm en t: topical steroid and cycloplegic for active uveitis;

vitrectom y, m ay require surgical repair of RD Co lo b o m a
Yellow-white lesion with pigm ented m argins due to
incom plete closure of em bryonic fissure; usually located
METABOLIC DISORDERS inferonasal
Retina is reduced to glial tissue; no retinal pigm ent epithelium
(Table 5-4, Box 5-2) (RPE); m ay have colobom as of other ocular structures

112
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Retinal Disorders
CHAPTER 5 • Pediatrics/Strabism us

Bo x 5-2. Ocular manifestations of childhood cerebral degenerations Extent: m easured in clock hours of involvem ent
Stage:
Conjunc tiva l te la ngie c ta s ia : ataxia telangiectasia, Fabry’s disease
1. Dem arcation line between vascular and avascular
Gla uc oma : MPS I-Scheie, Zellweger’s disease (rare)
retina (flat, white) (Fig. 5-10)
Corne a l op a c ity: MPS I, III, IV, VI; mucolipidoses III, IV; Fabry’s 2. Ridge (elevated, pink/white) (Fig. 5-11)
diseas e, sialidosis with chondrodystrophy, Cockayne’s disease, xeroderma
pigmentosum, Zellweger’s disease (occasionally), Wilson’s disease
3. Ridge with tufts of extraretinal blood vessels
(Kayser-Fleischer ring) (popcorn) (Figs. 5-12 and 5-13)
Le ns op a c ity: Wilson’s disease, galactosemia, Marines co-Sjo€ gren 4. Subtotal retinal detachm ent (Fig. 5-14)
syndrome, Lowe’s disease, cerebrocutaneous xanthomatosis, sialidos is, A. Extrafoveal
mannosidosis B. Including fovea
Che rry re d s p ot: Tay-Sachs disease, s ialidosis, Niemann-Pick disease 5. Total RD with funnel (open or closed)
(50%), GM gangliosidosis (50%), Farber’s disease, multiple sulfatase
deficiency (metachromatic leukodys trophy variant)
Ma c ula r a nd p igm e nta ry d e ge ne ra tion: ceroid lipofuscinosis, MPS 1-H,
1-S, II, III; mucolipidoses IV, Refsum’s disease (phytanic acid lipidosis), Plu s d isease: engorged, tortuous vessels around disc,
Bass en-Kornzweig (abetalipoproteinemia), Kearns-Sayre syndrome vitreous haze, and iris vascular congestion; progressive vascular
Op tic a trop hy: Krabbe’s disease, metachromatic leukodystrophy, incom petence throughout eye; poor prognostic sign
sphingolipidoses, adrenoleukodystrophy, Alexander’s disease, spongy
degeneration, Pelizaeus-Merzbacher disease, neuraxonal dystrophy, Alpers’
diseas e, spinocerebellar degeneration, diseases with retinal pigmentary
degeneration
Nys ta g mus : diseases with poor vision (searching nystagmus), Pelizaeus-
Merzbacher disease, metachromatic leukodystrophy, Friedreich’s ataxia,
other spinocerebellar degenerations and cerebellar atrophies, neuraxonal
dys trophy, ataxia telangiectasia, Leigh’s syndrome, Marinesco-Sjo€ gren
syndrome, opsoclonus-myoclonus syndrome, Ch ediak-Higashi disease
Op htha lmop le gia : Leigh’s syndrome, Kearns-Sayre syndrome,
Niemann-Pick variant with vertical ophthalmoplegia, Gaucher’s
diseas e, Bass en-Kornzweig syndrome, ataxia-telangiectasia, Tangier’s
diseas e

Re t in o p a t h y o f p re m a t u rit y
(Re t ro le n t a l fib ro p la s ia )
Vasoproliferative retinopathy occurring alm ost exclusively in
prem ature infants; occasionally in full-term infants Fig ure 5-10. Stage 1 retinopathy of prematurity. The flat, white border between
the avascular and vascular retina seen superiorly is called a
demarcation line. (Reproduced from Earl A. Palmer, MD, and the Multicenter Trial
Risk facto r s: low birth weight (< 1.5 kg; if < 1.25 kg, 65%
of Cryotherapy for Retinopathy of Prematurity. From Yanoff M, Duker J S [eds]:
develop som e degree of ROP early gestational age (50 days; Ophthalmology, London, 1999, Mosby.)
controversial), coexisting illness
Risk increases exponentially the m ore prem ature and the
sm aller the infant: < 2000 g (4 pounds, 7 ounces), earlier than
36 weeks (at 36 weeks, nasal retina is com pletely vascularized;
at 40 weeks, tem poral retina is fully vascularized)

Two p h ases:
Acute: abnorm al vessels develop in association with fibrous
proliferation; > 85%–90% spontaneously regress
Chronic: retinal detachm ent, tem poral displacem ent of
m acula, severe vision loss; occurs in 15%

Classificatio n : (International Classification of

ROP [ICROP])
Zone:
1. Posterior pole, area enclosed by 60° diam eter circle
centered on optic nerve
Fig ure 5-11. Stage 2 retinopathy of prematurity. The elevated mesenchymal
2. Area between edge of zone 1 and circle centered on
ridge has height. Highly arborized blood vessels from the vascularized retina dive
optic disc and tangent to nasal ora serrata into the ridge. (Reproduced from Palmer EA, MD, and the Multicenter Trial of
3. Rem aining crescent of tem poral peripheral retina Cryotherapy for Retinopathy of Prematurity: In: Yanoff M, Duker J S [eds]:
anterior to zone 2 Ophthalmology, London, 1999, Mosby.)

114
 Retinal Disorders

12 12

zone 3 zone 3
zone 2 zone 2
zone 1 zone 1
9 3 9 3

ma cula 6 ora serra ta 6 ma cula

right eye left eye

Fig ure 5-15. Definition of “threshold” retinopathy of prematurity. (From Sears J ,

Capone A: Retinopathy of prematurity. In Yanoff M, Duker J S [eds]:
Ophthalmology, London, 1999, Mosby.)
Fig ure 5.12. Stage 3 retinopathy of prematurity. Vessels on top of the ridge
project into the vitreous cavity. The extraretinal proliferation carries with it a
fibrovascular membrane. Note the opalescent avascular retina anterior to
the ridge. (From Sears J , Capone A: Retinopathy of primaturity. In Yanoff M,
Duker J S [eds]: Ophthalmology, London, 1999, Mosby.) Th r esh o ld d isease: (level at which 50% go blind without
treatment) ¼ stage 3 in zone 1 or 2 with Plus disease and at
least five contiguous or eight cumulative clock hours of
involvement; usually develops at 27 weeks’ gestational age
(Fig. 5-15)

Aggr essive p o ster io r ( AP) ROP o r Ru sh d isease: plus

disease in zone 1 or posterior zone 2; rapidly progressive

Pr eth r esh o ld : approxim ately one-third progress to

threshold disease
1. Zone 1, any stage
2. Zone 2, stage 2 + or 3
3. Zone 2, stage 3 + for > 5 clock hours

DDx o f p er ip h er al vascu lar ch an ges an d r etin al

d r aggin g: FEVR, incontinentia pigm enti (Bloch-Sulzberger
syndrom e), X-linked retinoschisis
Fig ure 5-13. Stage 3 retinopathy of prematurity. Note finger-like projections of
extraretinal vessels into the vitreous cavity. Hemorrhage on the ridge is not
uncommon. (From Sears J , Capone A: Retinopathy of prematurity. In Yanoff M, DDx o f tem p o r ally d r agged d isc: FEVR, Toxocara,
Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)
congenital falciform fold

Diagn o sis: screen infants < 1500 g, on supplem ental oxygen

during first 7 days of life, or < 30 weeks’ gestation
sta ge 4a sta ge 4b

zone 3 zone 3 Tr eatm en t: observation, laser, cryotherapy, anti-VEGF

zone 2 zone 2 injections, surgery
Cryotherapy for ROP (Cryo-ROP) Study: determ ined
zone 1 zone 1
whether treatm ent for ROP would prevent poor
outcom es (see below)
Treat with laser to peripheral avascular retina: when patient
reaches type 1 ROP, defined as:
Zone 1, any stage with Plus disease
Zone 1, stage 3 without Plus disease
retina l retina l Zone 2, stage 2 or 3 with Plus disease
deta chment deta chment (Early Treatm ent for ROP [ETROP] Study)
(extra fovea l) (involving fovea ) Indirect argon green or diode laser photocoagulation: 500 µm
Fig ure 5-14. Stage 4a detachment spares the fovea. Stage 4b detachment
spots to entire avascular retina in zone 1 and peripheral
involves the fovea. (From Sears J , Capone A: Retinopathy of prematurity. In zone 2 (at least as effective as cryotherapy; Laser-
Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.) ROP study)

115
CHAPTER 5 • Pediatrics/Strabism us

Cryotherapy: to entire avascular retina in zone 2, but not

However, the rate of recurrence in zone 2 posterior
ridge (cryo-ROP study)
disease did not differ significantly between the 2 groups
Anti-VEGF (BEAT-ROP Study): intravitreal injection of
(12% vs 5%). There was also a difference in the
0.625 m g in 0.025 m l bevacizum ab for zone I disease
tim eline for recurrence between the 2 groups (16.0 ± 4.6
Serial exams: with type 2 ROP, defined as:
weeks for bevacizum ab vs 6.2 ± 5.7 weeks for laser
Zone 1, stage 1 or 2
therapy). There was no evidence of system ic or local
Zone 2, stage 3
toxicity
Surgery: vitrectom y with or without lensectom y,
m em brane peel, and possible scleral buckle for
tractional retin al detachm ent (TRD) or Co n c lu s io n s :
rhegm atogenous retin al detachm ent (RRD) (cicatricial
ROP, stages 4 and 5) Intravitreal bevacizum ab had a lower recurrence rate in
infants with stage 3+ retinopathy of prem aturity for
zone 1 but not zone 2 disease com pared with
Pr o gn o sis: depends on extent of disease; m ost cases resolve conventional laser therapy
spontaneously without visible residua
Some develop cicatricial changes: (retrolental fibroplasia)
GRADE I: m yopia and peripheral glial scar (VA > 20/40)
GRADE II: dragged retina, m acular heterotopia Cryo the ra p y fo r ROP (Cryo -ROP ) Stud y
(VA 20/50-20/200)
GRADE III: retinal fold (VA < 20/200)
Ob je c t ive :
GRADE IV: partial retrolental m em brane and RD
(VA¼ HM or LP) To evaluate whether cryotherapy for ROP in preterm
GRADE V: com plete retrolental m em brane and RD infants with birth weights < 1251 g prevents
(VA¼ LP or NLP) unfavorable outcom es (posterior retinal fold, stage 4B or
5 retinal detachm ent, or visual acuity of 20/200
or worse)
Co m p licatio n s:
Grade I-II: m yopia (80%; due to forward displacem ent
of lens–iris diaphragm ), anisom etropia, strabism us, Re s u lt s :
am blyopia, m acular heterotopia, pseudo-XT
(change in angle k due to m acular dragging from The m ore posterior the zone and the greater the extent of
peripheral cicatrization), increased risk of retinal tear stage 3 + ROP, the poorer the outcom e
or RD All unfavorable fundus structural outcom es and alm ost all
Grade III-V: nystagm us, glaucom a, cataracts, heterochrom ia unfavorable visual acuity outcom es occurred in eyes with
irides, rubeosis iridis, anterior uveitis, RD, band zone 1 or 2 ROP and m ore than six sectors of stage 3 +
keratopathy, phthisis bulbi disease
Fewer unfavorable outcom es occurred in treated versus
control eyes (44.4% vs. 62.1% for visual acuity; 27.2% vs
47.9% for fundus status)
Retinal detachm ents rem ained stable in treated eyes (22%)
MAJ OR CLINICAL STUDY but continued to occur in control eyes (from 38.6% at
5.5 years to 41.4% at 10 years)
The Be va c izum a b Elim ina te s the After 10 years, treated eyes were m uch less likely than
Ang io g e nic Thre a t o f Re tino p a thy o f control eyes to be blind
P re m a turity (BEAT-ROP ) Stud y
Co n c lu s io n s :
Ob je c t ive :
Cryotherapy preserves visual acuity in eyes with threshold
To com pare intravitreal bevacizum ab to conventional laser disease
therapy in cases of stage 3+ retinopathy of prem aturity Initial examination: 4 to 6 weeks after birth or
(ROP) with zone 1 or 2 posterior disease at 30 weeks’ gestational age (whichever is later), then
every 2 weeks until vessels reach zone 3
Re s u lt s : Prethreshold disease: exam ine every week until regression
or threshold disease develops
150 infants (300 eyes) were enrolled; 143 infants survived Threshold disease: perform cryotherapy within
to 54 weeks’ postm enstrual age. Significantly higher 72 hours of diagnosis
rate of recurrence in zone 1 disease with conventional Laser photocoagulation or cryotherapy to avascular
laser therapy com pared with bevacizum ab (42% vs 6% ). retina, 90% regression rate

116
 Retinal Disorders

After 1 week, plus disease is usually less if

treatm ent will work, fibrovascular proliferation
often takes 2 weeks to begin regressing; consider
retreatm ent if ROP is worse after 1 week; retreat
untreated areas
Stage 4 disease: 60% reattachm ent rate with scleral buckle;
5% obtain useful vision
Stage 5 disease: vitrectom y (very poor success rate)

Ea rly Tre a tm e nt fo r Re tino p a thy o f

P re m a turity (ETROP ) Ra nd o m ize d Tria l

Ob je c t ive : Fig ure 5-16. Fundus view of a patient who has familial exudative
vitreoretinopathy. Note abnormally straightened retinal vasculature. (From
To determ ine if early treatm ent with laser to avascular Kimura AJ : Hereditary vitreoretinopathies. In Yanoff M, Duker J S [eds]:
retina in high-risk pre-threshold ROP results in better Ophthalmology, London, 1999, Mosby.)
vision and anatom ic outcom es vs conventional laser
treatm ent

Re s u lt s :
307 eyes were random ized. “Unfavorable” visual outcom es
were reduced by early treatm ent (19.5% vs 14.5%);
“unfavorable” anatom ic outcom es were reduced with
early treatm ent (15.6% vs 9.1%) at 9 m onths

Co n c lu s io n s :
Early laser therapy for type1 RO P defined as zone 1, any
stage RO P with plus disease; zone 1, stage 3 without
plus; or zone 2, stage 2 or 3 without plus disease.
Observation for type 2 RO P defined as zone 1, stage 1
or 2 without plus or zone 2, stage 3 without plus
Fig ure 5-17. Fluorescein angiogram of a patient who has familial exudative
disease
vitreoretinopathy. (From Kimura AJ : Hereditary vitreoretinopathies. In Yanoff M,
Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

strabism us or nystagm us; m ost asym ptom atic; m ay

Fa m ilia l e xu d a t ive vit re o re t in o p a t h y (FEVR)
progress to stage 2
(AD, AR o r X-lin ke d re c e s s ive ) Stage 2: fibrovascular proliferation with neovascularization,
AD m apped to chrom osom e 11q13-q23 (EVR1, FZD4, LRP5), exudates, dragging of disc and m acula, retinal folds and
11p13-p12 (EVR3); X-linked (NDP); AR (m ost severe form ) detachm ents; visual loss after 2nd–3rd decade of life rare
gene not identified unless progression to stage 3
Stage 3: cicatrization with rhegm atogenous retinal
Most are unaware that they have this disorder detachm ents (10%–20%; usually in third–fourth decade
Rare, progressive developm ental abnorm ality of peripheral of life), m ay develop proliferative vitreoretinopathy;
retinal vasculature (especially tem porally) prognosis poor

Tr eatm en t: prophylactic laser treatm ent to avascular retina

Fin d in gs: sim ilar to ROP (Figs. 5-16 and 5-17) (controversial); m ay require RD repair
Stage 1: avascular peripheral retina, white without
pressure, vitreous bands, peripheral cystoid
Co a t s d is e a s e (Le b e r’s m ilia ry a n e u rys m s )
degeneration, m icroaneurysm s, telangiectasia,
straightened vessels, vascular engorgem ent; m ay have Nonhereditary, proliferative exudative vascular disease

117
CHAPTER 5 • Pediatrics/Strabism us

More com m on in m ales (10:1); bim odal distribution (peak in Treatm en t: cryotherapy or laser to stop leaking blood vessels
first decade and sm all peak in young adulthood [associated
with hypercholesterolem ia])
No rrie d is e a s e (X-lin ke d re c e s s ive )
90% unilateral; 50% progressive
Defect of retinal developm ent

Fin d in gs: usually presents with leukocoria and strabism us; Fin d in gs: bilateral leukocoria (white, often hem orrhagic
telangiectatic blood vessels leak (with large am ount of retrolental m ass), retinal dysplasia, peripheral
subretinal lipid in outer plexiform layer), noncalcified neovascularization (NV), hem orrhagic retinal detachm ent
yellow subretinal lesions, exudative RD in 66% (especially in (RD), and retinal necrosis
patients < 4 years old), m icroaneurysm s, capillary
nonperfusion
Oth er fin d in gs: deafness, m ental retardation

Path o lo gy: triad of retinal vascular anom alies, S h a k e n b a b y s yn d ro m e

subretinal and intraretinal cholesterol deposits, and
intraretinal periodic acid-Schiff (PAS)-positive deposits; 30–40% of children subjected to child abuse will have
loss of vascular endothelium and pericytes; gliotic retina ophthalm ic sequelae
over subretinal fluid with cholesterol clefts and
Most com m on in children younger than 3 years old
hem osiderin-laden m acrophages. Aspiration of
subretinal exudates reveals cholesterol and pigm ent-laden
m acrophages Fin d in gs: diffuse retinal hem orrhages, papilledem a,
vitreous hemorrhage, retinal tissue disruption (retinoschisis,
retinal breaks, folds); m ay resem ble central retinal vein
DDx: retinoblastom a, angiom atosis retinae (von Hippel- occlusion (CRVO), Terson’s syndrom e, or Purtscher’s
Lindau syndrom e), PHPV retinopathy

B-scan u ltr aso u n d : consider ruling out retinoblastom a Asso ciated with o th er in ju r ies: subdural hematoma,
subarachnoid hemorrhage, bruises, fracture of long bones or ribs
Flu o r escein an gio gr am (FA): blood-fluid levels; saccular
aneurysm s (“light bulb” dilatations) of retinal arterioles and Pr o gn o sis: poor due to m acular scarring, vitreous
venules (Fig. 5-18) hem orrhage, RD

A B
Fig ure 5-18. A, The classic fundus picture of Coats disease with massive lipid exudation that causes an exudative retinal detachment. B, Fluorescein angiogram from the
same patient with large telangiectatic vessels and numerous leaking aneurysms. (From Mittra RA, Mieler WF, Pollack J S: Retinal arterial macroaneurysms. In Yanoff M,
Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

118
 Retinal Disorders

Inhe rite d Re tina l Dis e a s e s

Fu n d u s fla vim a c u la t u s (AR)

Mapped to chrom osom e 1p21-p13 (ABCA4)

Fin d in gs: bilateral pisciform , yellow-white flecks at level

of RPE
Predom inantly involves peripheral retina with m acula
involved to a lesser degree; flecks, then m acular degeneration
“s ile nt” choroid pis ciform fle cks
Central vision preserved until m acula involved
Fig ure 5-20. Fluorescein angiogram of Stargardt disease demonstrating dark
choroid and hyperfluorescent pisciform flecks. (From Kaiser PK, Friedman NJ ,
Path o lo gy: lipofuscin deposits within hypertrophied Pineda II, R: Massachusetts eye and ear infirmary illustrated manual of
RPE cells ophthalmology, ed 2, Philadelphia, 2004,WB Saunders.)

ERG: degree of abnorm ality correlates with am ount of

fundus involvem ent
FA: dark choroid (85%) due to accum ulation of lipofuscin in
S t a rg a rd t d is e a s e RPE; areas of hyperfluorescence do not directly correspond to
flecks; pigm entary changes in m acula appear as window defects
AR or less often AD; m apped to chrom osom es 1p21-p22 (Fig. 5-20)
(STGD1; ABCA4, typical form , AR), 4p (STGD4, AD), 6q14
(STGD3, Stargardt-like macular dystrophy) FAF: hyper-autofluorescence due to excessive lipofuscin
Juvenile m acular degeneration with flecks accumation in RPE; hypo in areas of RPE atrophy

Most com m on hereditary m acular dystrophy

ERG an d EOG: norm al or subnorm al in widespread disease;
Onset in first 2 decades of life with decreased vision delayed dark adaptation

Sym p to m s: decreased vision, nyctalopia Tr eatm en t: gene therapy being tested; vitam in A
supplem entation accelerates accum ulation of lipofuscin
Fin d in gs: bilateral pisciform , yellow-white flecks at level of pigm ents in RPE. Long-term vitam in supplem entation
RPE; change with tim e (new ones appear, others disappear); increases form ation of vitam in Adim ers which favor lipofuscin
beaten-m etal appearance of fundus; foveal atrophy; bull’s-eye synthesis and deposition. Therefore, avoid vitam in A
m aculopathy; m ay have salt and pepper pigm entary changes supplem entation
of peripheral retina (Fig. 5-19)
Pr o gn o sis: 50% have at least 20/40 vision in one eye by age
Path o lo gy: RPE m assively thickened by accum ulation of 19; only 22% have 20/40 vision by age 39; rapid progression to
lipofuscin, ABCA4 localizes to the disc m em branes in cone and 20/200 – Counting fingers (CF)
rod outer segm ents; part of the retinoid cycle
Be s t d is e a s e (Vit e llifo rm d ys t ro p h y) (AD)
Variable penetrance; hereditary dystrophy m apped to
chrom osom e 11q13 (VMD2 [BEST1], encodes bestrophin1
located on basolateral aspect of RPE)
Second m ost com m on hereditary m acular dystrophy
Progressive with onset in first decade of life
Macular dystrophy in which RPE is prim arily affected; form
of exudative central m acular detachm ent in which
pigm entation can occur in end stages with atrophic scarrin g
and/ or CNV
Associated with strabism us and hyperopia
pis ciform fle cks “bull’s -e ye ” ma culopa thy
Fig ure 5-19. Stargardt diseas e. (From Kaiser PK, Friedman NJ , Pineda II, R: Sym p to m s: none or m ild decreased vision initially; later
Massachusetts eye and ear infirmary illustrated manual of ophthalmology, ed 2, decreases to 20/30-20/100; better vision than expected by
Philadelphia, 2004,WB Saunders.) clinical appearance

119
CHAPTER 5 • Pediatrics/Strabism us

Fin d in gs: progressive m acular changes; can get CNV Fa m ilia l d ru s e n (Do yn e ’s h o n e yc o m b
Stage 1 - Previtelliform stage: sm all round subm acular d ys t r o p h y) (AD)
yellow dot, subtle RPE changes; norm al vision
Stage II - Vitelliform stage: yellow-orange egg yolk/fried egg Mapped to chrom osom e 2p16-p21 (EFEMP1)
appearance; can be m ultiple; usually between ages 3 and Sm all yellow-white, round to oval deposits on Bruch’s
15 (Fig. 5-21) m em brane; decreased vision after age 40
Stage III - Pseudohypopyon stage: layering of lipofuscin, RPE
atrophy
Co m p licatio n s: m acular edem a, hem orrhage, CNV
Stage IV - Scrambled egg stage: irregular subretinal spots;
vision usually still good
Stage V - Round chorioretinal atrophy stage: atrophic scar;
Ma t e rn a l in h e rit e d d ia b e t e s
vision usually stabilizes at 20/100 a n d d e a fn e s s (MIDD)
Stage VI - CNV stage: occurs in 20% Mitochondrial disease (m aternal inheritance); point m utation
at position 3243 of m aternal m tDNA
DDx: central serous chorioretinopathy, RPED,
toxoplasm osis, m acular colobom a, solar retinopathy, old Eye findings occur in 5th decade with ptosis, external
foveal hem orrhage, adult foveom acular vitelliform dystrophy, ophthalmoplegia, ragged red fibers in extraocular
syphilis, North Carolina dystrophy, autosom al recessive muscles, chorioretinal atrophy, iris atrophy, pigmentary
bestrophinopathy, dom inant drusen, AMD, m acular hole, retinopathy(annular perifoveal RPEatrophyin macula or pattern-
m yopic degeneration type dystrophy appearance); associated with pattern dystrophies

FA: blockage by egg yolk lesion; window defect when cyst VF: annular defect
ruptures
FA: autofluorescence around dark pigm ent atrophy area
OCT: localizes vitelliform lesion, thickening of outer
segm ents, evaluate for CNV OCT: photoreceptor dropout

FAF: hyper-autofluorescence of lesion in early stages; m ottled ERG: norm al

hypo during later stages; hypo in atrophic stage
No rt h Ca ro lin a m a c u la r d ys t ro p h y (AD)
ERG: norm al Mapped to chrom osom e 6q14-q16 (MCDR1)

EOG: abnorm al (Arden ratio 1.5; also in carriers) Onset in first decade with drusen progressing to chorioretinal
atrophy with staphylom a of m acula (Fig. 5-22)
Dar k ad ap tatio n : norm al May develop CNV

Pr o gn o sis: good vision (20/30-20/100 range) ERG, EOG, an d d ar k ad ap tatio n : norm al

Fig ure 5-21. Best disease. Vitelliform stage. (Courtesy of Ola Sandgren, Fig ure 5-22. North Carolina macular dystrophy. (From Parnell J R, Small KW:
University Hospital of Umea, Sweden. From Parnell J R, Small KW: Macular Macular dystrophies. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999,
dystropies. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.) Mosby.)

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 Retinal Disorders

P s e u d o in fla m m a t o ry m a c u la r d ys t ro p h y
(S o rs b y’s ) (AD)
Mapped to chrom osom e 22q12-q13 (SFD [TIMP-3])
Atrophy, edem a, hem orrhage, and exudate
Decreased acuity and color vision occurs between ages 40 and
50 years

ERG an d EOG: subnorm al late

Dar k ad ap tatio n : delayed

P a t t e rn d ys t ro p h ie s (Us u a lly AD)

Group of diseases with central pigm entary disturbance, good
central vision, norm al ERG, abnorm al EOG
Fig ure 5-23. The “bull’s-eye” maculopathy in this 5-year-old male who has a
May develop CNV cone–rod dystrophy is not found in all cases of this entity. (From Sieving PA:
Retinis pigmentosa and related disorders. In Yanoff M, Duker J S [eds]:
Ophthalmology, London, 1999, Mosby.)
Sj o€ gr en ’s r eticu lar d ystr o p h y: fishnet configuration;
fishnet is hypofluorescent on FA
VF: norm al

Bu tter fly-sh ap ed d ystr o p h y: bilateral gray-yellow FA: m ay show window defects early
butterfly wing-shaped lesion with surrounding
depigm entation; onset between ages 20 and 50 years with ERG: absent photopic, norm al scotopic
slightly reduced vision; m apped to chrom osom e 6p21 (RDS
[peripherin]); butterfly lesion is hypofluorescent on FA EOG: norm al
Ad u lt o n set fo veo m acu lar vitellifo r m Dar k ad ap tatio n : rod phase only
d ystr o p h y: onset between ages 30 and 50; early ringlike area
of RPE clum ping that develops into sym m etric, solitary yellow Pr o gn o sis: poor vision (20/200 level) by 4th decade of life
m acula lesions (like Best egg yolk lesions but sm aller [½ DD in
central fovea] and do not break up); often have central area of
pigm ent; CNV is m ore com m on than in Best disease; S t a t io n a ry c o n e d is o rd e rs
norm al EOG Present at birth; nonprogressive

Fu n d u s p u lver u len tu s: rarest form , coarse pigm ent Co m p lete r o d m o n o ch r o m atism ( co n gen ital
m ottling in m acula; associated with pseudoxanthom a ach r o m ato p sia) (AR): m apped to chrom osom e 14
elasticum (ACHM1); absent or abnorm al cones; nonprogressive
Findings: decreased vision (20/200 level), com plete color
P ro g re s s ive c o n e d ys t ro p h y Autosom al dom inant or blindness, photophobia, nystagm us, norm al retinal
less often X-linked; m apped to chrom osom es 6p21 (COD3), exam ination
Xp21 (COD1) [RPGR]), Xq27 (COD2) VF: central scotom a
Progressive dysfunction of cones with norm al rod function Erg: norm al scotopic, abnorm al photopic

Onset during first 3 decades of life with decreased vision, Blu e co n e m o n o ch r o m atism (X-linked recessive):
dyschrom atopsia, photophobia have only blue-sensitive cones
Findings: decreased vision (20/40-20/200), photoaversion,
Sym p to m s: visual loss, photophobia (usually precede nystagm us
visible m acular changes) ERG: absent cone response, norm al rod response

Fin d in gs: decreased vision (to 20/200), decreased color

vision, central scotoma, nystagm us (25%), nerve fiber layer Infe c tio ns
(NFL) loss, optic atrophy, macular degeneration (granular
appearance early, then beaten-metal appearance); can have fine,
Co n g e n it a l ru b e lla s yn d ro m e
golden subretinal deposits, bull’s-eye m aculopathy (Fig. 5-23);
can develop a pattern that mimics Stargardt or fundus Sensorineural hearing loss, salt and pepper retinopathy; m ay
flavim aculatus develop cataract or glaucom a (rare to have both) (Fig. 5-24)

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CHAPTER 5 • Pediatrics/Strabism us

Retinopathy only: Hunter (type II), Sanfilippo (type III)

Corneal clouding only: Morquio (type IV), Sly (type VII)
No corneal clouding or retinopathy: Sly (type VII)

S p h in g o lip id o s e s (AR)
Accum ulation of sphingolipids due to lysosom al enzym e
defects
Sphingolipids accum ulate in retinal ganglion cells; result in
cherry red spot (m acula has highest concentration of ganglion
cells) (see Table 5-4)

Ch er r y r ed sp o t: Tay-Sachs, Sandhoff’s, Neim ann-Pick,

and Gaucher’s disease
Fig ure 5-24. Rubella retinopathy. (Courtesy of George S. Novalis, MD. From Tay-Sachs: m ost com m on; hexosam inodase A deficiency;
Hudson HL, Boyer DS, Martin DF, et al: Viral posterior uveitis. In Yanoff M, death by 3 years of age; cherry red spot and m ental
Duker J S [eds]: Ophthalmology, London, Mosby, 1999.)
retardation
Vision and electrophysiologic testing are usually norm al Sandhoff’s: sim ilar to Tay-Sachs; extensive visceral
involvem ent
Live virus in an infant is found in the lens, as well as Niemann-Pick: cherry red spot, m acular halo, optic atrophy;
conjunctival swab, pharyngeal swab, and urine cultures hepatosplenomegaly, infiltration of lungs, foam cells in
bone m arrow, no m ental retardation
Me a s le s
Pigm entary retinopathy due to infection acquired in utero DDx o f ch er r y r ed sp o t: CRAO, com m otio retinae,
Acute blindness 6–12 days after m easles rash appears m acular hole, m acular hem orrhage, ocular ischem ic syndrom e,
subacute sclerosing panencephalitis, quinine toxicity,
Fin d in gs: keratoconjunctivitis, retinal edem a, vascular m ethanol toxicity
attenuation, m acular star, no hem orrhages; Koplik’s spots in
m outh No ch er r y r ed sp o t: Fabry’s and Krabbe’s diseases
Fabry’s (X-linked dominant): α-galactosidase A deficiency;
Tr eatm en t: none; m ost infants recover, som e develop accum ulation of trihexosylceram ide in sm ooth m uscle of
pigm entary degeneration blood vessels
FINDINGS: cornea verticillata (whorl-like opacities in
basal epithelium ), tortuous conjunctival and retinal
Su b acu te scler o sin g p an en cep h alitis ( SSPE) : fatal vessels, cataracts (posterior lenticular spoke-like
m easles slow virus infection of CNS
changes); m anifest in heterozygous fem ales (carriers)
Findings: m acular or perim acular chorioretinitis,
and 90% of affected m ales
pigm entary changes with bone spicules, papilledem a,
OTHER FINDINGS: paresthesias in extrem ities,
optic atrophy, nystagm us, cortical blindness
cutaneous telangiectases, angiokeratom as, poor
Other findings: personality or behavioral changes,
tem perature regulation, abdom inal pain, anem ia,
dem entia, seizures, m yoclonus
renal failure, hypertension; vascular anom alies
Pathology: eosinophilic nuclear inclusions in neuronal
of heart, kidney, and brain; renal failure is leading
and glial cells
cause of death
DIAGNOSIS: failure to detect α-galactosidase in tears
Me ta b o lic Dis e a s e s

Mu c o p o lys a c c h a r id o s e s (AR e xc e p t Hu n t e r Cys t in o s is

[X-lin k e d re c e s s ive ]) Mapped to chrom osom e 17p13
Accum ulation of acid m ucopolysaccharides due to lysosom al Am ino acid disorder; lysosom es cannot excrete cystine
enzym e defects
Fin d in gs: iridescent fusiform cystine crystals in conjunctiva,
Syndrom es in which heparin sulfate accum ulates are cornea, iris, lens, and retina; photophobia
associated with pigm entary retinopathy Infantile form (nephropathic form ) (AR): Fanconi’s
syndrom e: polyuria, growth retardation, rickets,
Fin d in gs: m ay have corneal clouding (strom al; progressive), progressive renal failure, salt and pepper fundus changes
retinopathy (RPE degeneration), and / or optic atrophy (see (but no visual disturbance); the only form with
Table 5-4) retinopathy; m ost die before puberty
Corneal clouding and retinopathy: Hurler (type Ia), Scheie Adolescent form (AR): less severe nephropathy than in
(type Ib), Maroteaux-Lam y (type VI) Fanconi’s syndrom e

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 Retinal Disorders

Adult form: benign; no renal problem s; deposits lim ited to

anterior segm ent

Tr eatm en t: cysteam ine (system ically for renal disease,

topically for corneal crystals)

Lo n g -c h a in 3 -h yd r o xya c yl-Co A d e h yd ro g e n a s e
d e fic ie n c y (LC HAD d e fic ie n c y)
Disorder of m itochondrial fatty acid beta-oxidation due to
m utation of guanine to cytosine at position 1528

Fin d in gs: norm al fundus at birth, followed by RPE pigm ent

dispersion; eventually develop chorioretinal atrophy and
occlusion of choroidal vessels; deterioration of central vision;
Fig ure 5-25. “Typical” retinitis pigmentosa changes. (From Sieving PA: Retinis
m ay have posterior staphylom as, developm ental cataracts, pigmentosa and related disorders. In Yanoff M, Duker J S [eds ]: Ophthalmology,
progressive m yopia London, 1999, Mosby.)

Tr eatm en t: low-fat, high-carbohydrate diet with carnitine

supplem entation
Pathology: photoreceptor atrophy including outer nuclear
layer; inner retina well preserved; RPE cells invade retina
Pr o gn o sis: usually fatal by 2 years of age (hepatic or and surround retinal vessels (bone spicules)
cardiorespiratory failure) unless dietary treatm ent is started VF: inferotem poral scotom a, enlarges to form ring/annular
scotom a; constricted
ERG: early, increased rod threshold with norm al cone
Ta p e to re tina l De g e ne ra tio n
response and decreased scotopic b-wave; late,
nonrecordable; abnorm alities precede retinal changes
Processes that involve the outer half of the retina and visual com plaints
(photoreceptor / RPE); RPE¼ tapetum nigran (black carpet) EOG: abnorm al
Dark adaptation: prolonged
Re t in it is p ig m e n t o s a (RP ) RP carriers:
FUNDUS: bone spicules, salt and pepper changes,
Group of progressive dystrophies caused by abnorm al bronze sheen in m acula
photoreceptor protein production ERG: decreased scotopic am plitude and delayed cone
Most com m on hereditary degeneration, incidence 1:5000 B-wave im plicit tim e in X-linked carriers
VITREOUS FLUOROPHOTOMETRY: abnorm al in X-linked
carriers
RP typ e I ( r o d –co n e) :
DDx of tunnel vision: glaucom a, functional, gyrate atrophy,
Inheritance patterns:
vitam in A toxicity, occipital lobe stroke
AR (37%): m ost com m on form ; m ay be due to defects in
DDx of nyctalopia: uncorrected m yopia, vitam in A
2 RPE genes (which provide instructions for vitam in A
deficiency, zinc deficiency, choroiderem ia, gyrate
transport proteins): RPE 65, CRALBP
atrophy, congenital stationary night blindness (CSNB),
X-LINKED RECESSIVE (4%): rarest and m ost severe form ;
Goldm an-Favre disease
poor prognosis; vision < 20/100 by 3rd decade of
DDx of salt and pepper fundus: rubella retinopathy, Leber’s
life; carriers m ay have fundus changes
congenital am aurosis, carrier states (albinism , RP,
AD (20%): least severe form , later onset; m ost likely to
choroiderem ia), syphilis, cystinosis, phenothiazine
retain good vision
toxicity, pattern dystrophy, following resolution of an
SPORADIC (30%–50%)
exudative RD
Associations: keratoconus, m acular cystoid degeneration,
Macular complications: CME (no leakage on FA), epiretinal
Coats disease, optic disc drusen, astrocytic ham artom a of
m em brane, atrophy
ON, m yopia
Symptoms: nyctalopia, ”blue blindness,“ decreased
vision late RP typ e II ( co n e–r o d ) : AD, AR, or X-linked
Findings: pigmentary retinal changes (bone spicules), Findings: less pigm ent deposition; 50% are sine pigm ento
attenuated arteries, waxy pallor of optic nerve ERG: cones m ore affected than rods
(due to glial m em brane form ation over disc), retinal
atrophy with increased visibility of choroidal vessels, Tr eatm en t: low vision aids, dark glasses; vitam in A slows
vitreous cells, cataract; som e form s can have reduction of ERG (controversial)
prom inent subretinal exudation (Coats-like disease)
(Fig. 5-25) Pr o gn o sis: poor

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CHAPTER 5 • Pediatrics/Strabism us

RP va ria n t s TYPE 2: night blindness (second-fourth decade),

partial deafness, intelligible speech, no ataxia
Tr eatab le RP: Bassen-Kornzweig (abetalipoproteinem ia), DDx of deafness and RP: Usher’s, Hallgrenn’s, Alstrom ’s,
Refsum ’s (elevated phytanic acid), gyrate atrophy (elevated Laurence-Moon-Biedl-Bardet, and Cockayne’s
ornithine) syndrom es
Other eye syndromes associated with hearing loss: Cogan’s (IK
Leb er ’s co n gen ital am au r o sis (AR): m apped to plus hearing loss), Stickler’s (vitreous changes, joint and
chrom osom es 1p31 (LCA2), 14q11 (RPGRIP1), 17p13 (LCA1, orofacial abnorm alities, hearing loss), Waardenburg-
LCA4), 19q13 (CRX) Klein (iris heterochrom ia, white forelock), Duane’s (15%
have hearing loss)
Infantile form of RP; blind or severe visual im pairm ent in
infancy or early childhood; responsible for approxim ately 10%
of childhood blindness
Refsu m ’s d isease (AR): deficiency of phytanic acid oxidase
interferes with fatty acid m etabolism
Associations: keratoconus, hyperopia, cataract, m acular
colobom a, m ental retardation, deafness, seizures, renal Phytanic acid accum ulates in RPE cells, sensory retina
and m usculoskeletal abnorm alities deteriorates; onset in childhood
Findings: decreased vision (HM-CF [hand m ovem ents–
counting fingers]), nystagm us, hyperopia, poorly Mapped to chrom osom es 7q21-q22 (PEX1), 10p15-p12
reactive pupils, range of fundus appearance from norm al (PNYH)
(m ost com m on) to variety of pigm entary changes Findings: atypical RP (often sine pigm ento or starts in
(granular, fleck, salt and pepper, sheen, or atrophic) m acula), cataracts, prom inent corneal nerves
OCULODIGITAL SIGN: child rubs eyes to elicit entopic
Other findings: ataxia, peripheral neuropathy, deafness,
stim ulation of retina anosm ia, ichthyosis, cardiac abnorm alities, hypotonia,
Pathology: diffuse absence of photoreceptors hepatom egaly, m ental retardation
ERG: low or com pletely flat Diagnosis: increased serum copper and ceruloplasm in;
increased CSF protein without pleocytosis
Treatment: dietary restriction of anim al fats, m ilk products,
Secto r RP (AD or AR): retinal changes lim ited to focal area, leafy green vegetables
usually inferonasal quadrant
VF: abnorm al Bassen -Ko r n zweig syn d r o m e (AR): hereditary
ERG: abnorm al; norm al b-wave im plicit tim e reflects abetalipoproteinem ia; m apped to chrom osom e 4q24 (MTP)
nonprogression
Prognosis: good Inability to transport and absorb lipids because apolipoprotein
B is a m ajor protein of chylom icrons; deficiency in fat-soluble
vitam ins (A, D, E, and K)
Un ilater al RP: very rare
Findings: RP (usually without bone spicules); m ay have
Bitot’s spots on conjuctiva
In ver se RP: posterior pole affected (rather than Other findings: ataxic neuropathy, growth retardation,
m idperiphery): bone spicules in m acula, norm al periphery coagulopathy
FA: dark choroid (probably a variant of Stargardt) Diagnosis: CBC, cholesterol (low), stool sam ple; elevated
Retinitis punctata albescens (AR): sm all white spots in CSF protein; no apolipoprotein B-48 (low chylom icrons)
m idperiphery of retina, no bone spicules or apolipoprotein B-100 (low LDL and VLDL)
Treatment: vitam in A and E supplem ents
RP sin e p igm en to : no retinal pigm entary changes Other causes of treatable RP due to vitamin A deficiency:
chronic pancreatitis, cirrhosis, bowel resection
Pseu d o r etin itis p igm en to sa: m igration of RPE m elanin
into sensory retina leading to bone spicule pattern Lawr en ce-Mo o n -Bar d et-Bied l syn d r o m e
Etiology: traum a, drug toxicity (chloroquine, (AR): m apped to chrom osom es 2q31 (BBS5), 3p13-p12
chlorprom azine), infection / inflam m ation (syphilis, (BBS3), 11q13 (BBS1), 15q22-q23 (BBS4), 6q21 (BBS2),
toxoplasm osis, m easles, rubella), post-CVO, resolved 20p12 (BBS6)
exudative RD, ophthalm ic artery occlusion L-M and B-B: pigm entary retinopathy with flat ERG, m ental
retardation, hypogonadism , short stature
RP s yn d ro m e s B-B: polydactyly and obesity (Fig. 5-26)
L-M: spastic paraplegia
Ush er ’s syn d r o m e (AR): m ost com m on syndrom e
associated with RP; m apped to chrom osom e 1q41 (USH2A)
Sp ielm eyer -Vo gt-Batten -Mayo u syn d r o m e: neuronal
Findings: RP and deafness; ataxia, MR, low phosphate
ceroid lipofuscinosis
(rickets), m uscle wasting, 10%–20% of RP patients are
deaf; 5% of congenitally deaf individuals have Usher’s Onset between 2 and 4 years of age in Jewish fem ales
type I Findings: RP-like retinal degeneration with bull’s-eye
TYPE 1: night blindness (first-second decade), m aculopathy
profound deafness, unintelligible speech, ataxia Other findings: seizures, progressive dem entia, ataxia

124
 Retinal Disorders

ce ntra l a re ola r choroida l dys trophy

Fig ure 5-27. Central geographic atrophy in a patient with central areolar
Fig ure 5-26. Bardet-Biedl syndrome with extensive peripheral retinal pigment choroidal dystrophy. (From Kaiser PK, Friedman NJ , Pineda II, R: Massachusetts
epithelium and parafoveal retinal pigment epithelium atrophy. (From Sieving PA: eye and ear infirmary illustrated manual of ophthalmology, ed 2, Philadelphia,
Retinis pigmentosa and related disorders. In Yanoff M, Duker J S [eds]: 2004,WB Saunders.)
Ophthalmology, London, 1999, Mosby.)

Pathology: vacuolization of peripheral lym phocytes;

m etachrom asia of skin fibroblasts in cell culture
(carriers only)

Ch r o n ic p r o gr essive exter n al o p h th alm o p legia

( CPEO) : m itochondrial inheritance; 50% with positive
fam ily history
Findings: retinal degeneration (salt and pepper changes
with norm al retinal function; progresses to RP-like
disease), ptosis, ophthalm oplegia, strabism us
Other findings: facial weakness, dysphagia, sm all stature,
lim b girdle m yopathy, cardiac conduction defects
(Kearns-Sayre syndrom e)
ce ntra l a re ola r choroida l dys trophy
Olivo p o n to cer eb ellar atr o p h y: retinal degeneration;
trem ors, ataxia, dysarthria Fig ure 5-28. Left eye of same patient as shown in Figure 5-27 demonstrating
similar central geographic atrophy. (From Kaiser PK, Friedman NJ , Pineda II, R:
Massachusetts eye and ear infirmary illustrated manual of ophthalmology, ed 2,
Alstr o m ’s d isease: m apped to chrom osom e 2p13 Philadelphia, 2004,WB Saunders.)
(ALMS1)
RP with profound visual loss in first decade of life; diabetes,
obesity, deafness, renal failure, baldness, acanthosis nigricans, FA: window defect of central lesion
hypogenitalism
ERG: norm al or subnorm al
Co ckayn e’s syn d r o m e: RP with profound visual loss by
second decade of life; dwarfism , deafness, m ental retardation,
EOG, d ar k ad ap tatio n : norm al
prem ature aging, psychosis, intracranial calcifications

Neu r o n al cer o id lip o fu scin o sis ( Batten ’s

Bie t t i’s c rys t a llin e re t in o p a t h y (AR)
d isease) : juvenile or adult onset of seizures, dem entia,
ataxia, m ental retardation Decreased vision in fifth decade

Ce n t ra l a re o la r c h o ro id a l d ys t ro p h y (AD) Fin d in gs: yellow-white refractile spots throughout fundus,

geographic atrophy; m ay have crystals in peripheral corneal
Mapped to chrom osom e 6p (RDS [peripherin]), 17p13
strom a (Fig. 5-29)
(CACD)
Decreased vision in fourth decade FA: crystals hyperfluoresce, window defects and areas of
blockage
Fin d in gs: RPE m ottling in m acula progressing to geographic
atrophy (choroidal vessels visible) (Figs. 5-27 and 5-28) ERG: reduced

125
CHAPTER 5 • Pediatrics/Strabism us

Bie tti’s crys ta lline re tinopa thy

Fig ure 5-29. Bietti’s crystalline retinopathy. (From Kaiser PK, Friedman NJ , Fig ure 5-30. Fundus albipunctatus. This posterior pole and beyond show
Pineda II, R: Massachusetts eye and ear infirmary illustrated manual of multiple small, discrete, round, white dots that spare the fovea. (From Noble KG:
ophthalmology, ed 2, Philadelphia, 2004,WB Saunders.) Congenital stationary night blindness. In Yanoff M, Duker J S [eds]:
Ophthalmology, London, 1999, Mosby.)
Co n g e n it a l s t a t io n a ry n ig h t b lin d n e s s (CS NB)
Poor night vision (nyctalopia)
Group of nonprogressive rod disorders classified by fundus
appearance

Fin d in gs: norm al vision, VF, and color vision; m ay show

paradoxical pupillary dilation to light; no Purkinje shift
(always m ost sensitive to 550 nm )

ERG: scotopic and photopic im plicit tim es are identical;

decreased scotopic ERG; photopic ERG alm ost norm al

No r m al fu n d u s:
Nougaret type (AD): no rod function; m apped to
chrom osom e 3p21 (GNAT1)
Riggs type (AR): som e rod function
Schubert-Bornschein type (X-linked or AR): som e or no rod
function, m yopia
A
Ab n o r m al fu n d u s:
Fundus albipunctatus (AR): m apped to chrom osom e 12q13-
q14
FINDINGS: m idperipheral deep yellow-white spots spare
m acula (Fig. 5-30)
ERG: norm alization of scotopic after 4–8 hours of dark
adaptation
ALPORT’S SYNDROME (AD): kidney failure, deafness,
anterior lenticonus with anterior polar cataract; m ay
have retinal appearance sim ilar to fundus
albipunctatus
Oguchi’s disease: m apped to chrom osom es 2q37 (Oguchi 1,
Arrestin, SAG), 13q34 (Oguchi 2 [RHOK])
MIZUO-NAKAMURA PHENOMENON: golden-brown
fundus (yellow / gray sheen) in light-adapted state,
norm al-colored fundus in dark-adapted state (takes B
around 12 hours) (Fig. 5-31)
Fig ure 5-31. Oguchi’s disease. A, The yellowish metallic sheen is apparent nasal
ERG: absent b-wave; only scotopic a-wave to the optic disc. B, After 3 hours of dark. (From Noble KG: Congenital stationary
Kandori’s flecked retina (AR): yellow-white spots scattered in night blindness. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999,
equatorial region; spares m acula Mosby.)

126
 Retinal Disorders

Fig ure 5-32. Fundus changes in the right eye from a patient with late-stage gyra te a trophy
choroideremia. (From Grover S, Fishman GA: Choroidal dystrophies. In Yanoff
Fig ure 5-33. Gyrate atrophy. (From Kaiser PK, Friedman NJ , Pineda II, R:
M, Duker J S [eds ]: Ophthalmology, London, 1999, Mosby.)
Massachusetts eye and ear infirmary illustrated manual of ophthalmology, ed 2,
Philadelphia, 2004,WB Saunders.)

Ch o ro id e re m ia (X-lin ke d re c e s s ive )
Diagn o sis: blood tests (increased ornithine, decreased
Mapped to chrom osom e Xq21 (CHM); basic defect lysine), urinalysis (increased ornithine)
localized to the RPE (not the choriocapillaris as previously Carrier: decreased levels of ornithine ketoacid transam inase
believed)
Progressive degeneration of the RPE and choriocapillaris;
ERG an d ECG: abnorm al
considered a form of rod–cone degeneration
Dar k ad ap tatio n : prolonged
Onset during late childhood with nyctalopia, photophobia,
constricted visual fields in affected m ales Tr eatm en t: restrict arginine and protein in diet; consider
vitam in B6 supplem entation (pyridoxine; reduces ornithine)
Fin d in gs: early—degeneration of RPE and choriocapillaris
in periphery (scalloped RPE atrophy); late – absence of RPE
and choriocapillaris except in m acula (Fig. 5-32) Vitre o re tin a l Dys tro p hie s
Fem ale carriers have salt and pepper fundus
Vitreous abnorm alities associated with schisis cavity at level of
Path o lo gy: defect in choroidal vasculature NFL and ganglion cell layer

ERG: m arkedly reduced; undetectable late J u ve n ile re t in o s c h is is (X-lin ke d re c e s s ive )

Mapped to chrom osom e Xp22 (XLRS1 [retinoschisin])
Gyra t e a t ro p h y (AR)
Males; bilateral; present at birth; progresses rapidly during first
Mapped to chrom osom e 10q26 5 years of life; stable by age 20
Deficiency of ornithine am inotransferase; elevated ornithine Cleavage of retina at NFL (in senile retinoschisis, cleavage is at
levels (10–20 Â norm al), low lysine levels; chorioretinal outer plexiform layer)
dystrophy itself is not due to high ornithine levels
Progressive retinal degeneration; starts peripherally and Fin d in gs:
spreads toward posterior pole Foveal retinoschisis (only abnorm ality in 50%):
pathognom onic; earliest change is radial, spoke-like ILM
Onset by second decade of life with decreased vision, folds centered on fovea because of dehiscence of NFL;
nyctalopia, and constricted visual fields appears like cystoid m acular edem a (CME). Later, cystoid
structure appears in fovea with round m icrocysts in
Fin d in gs: scalloped areas of absent choriocapillaris and RPE perifoveal area and m arked pigm entary degeneration;
in periphery with abrupt transition between norm al and bullous schisis cavities develop; retinal vessels are only
atrophic areas (Fig. 5-33); generalized hyperpigmentation of rem aining structure within inner layer and m ay bleed
rem aining RPE (vs choroiderem ia); eventually lose causing vitreous hem orrhage
choriocapillaris and m edium -sized choroidal vessels; m yopia Marked sclerosis/sheathing of blood vessels with
(90%), cataracts, vitreous degeneration, CME appearance of vitreous veils
Vitreous cells (30%)
Oth er fin d in gs: seizures, structural changes in m uscle and True retinoschisis (NFL) in periphery in 50% (usually
hair fibers inferotem poral); does not extend to ora

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CHAPTER 5 • Pediatrics/Strabism us

Vitreous syneresis
Hyperopia
Reduced vision (20/50-20/100 level)
Carriers have norm al retinal appearance and function

VF: absolute scotom a

FA: m icrocysts with no leakage

ERG: norm al a-wave until late, reduced b-wave (especially

scotopic) in proportion to am ount of retinoschisis, m arkedly
reduced oscillatory potentials

EOG: norm al

Co m p licatio n s: RD and VH (uncom m on)

Fig ure 5-34. Fundus view ofthe eye ofa Stickler’s syndrome patient. Note the radial
perivascular pigmentary changes. (From Kimura AJ: Hereditary vitreoretinopathies.
Go ld m a n n -Fa vre d is e a s e (AR) In Yanoff M, Duker J S (eds): Ophthalmology, London, 1999, Mosby.)

Mapped to chrom osom e 15q23 (PNR)

Rare, vitreotapetoretinal degeneration with nyctalopia
and constricted visual fields (like RP plus juvenile S t ic k le r’s s yn d r o m e (AD)
retinoschisis)
Mapped to chrom osom es 1p21 (COL11A1), 6p21 (COL11A2),
12q13 (COL2A1)
Fin d in gs: decreased vision, nyctalopia, optically em pty
vitreous with strands and veils, bilateral central and peripheral Progressive arthro-ophthalm opathy; Wagner-like ocular
retinoschisis, attenuated retinal vessels, peripheral bone– changes with severe m yopia and m arfinoid habitus
spicule retinal pigm entary changes, lattice degeneration, optic
disc pallor, cataracts
Fin d in gs: optically em pty vitreous, lattice degeneration, RD
(50%), optic atrophy, cataract, glaucom a, high degree of
ERG: m arkedly reduced m yopia (Fig. 5-34)

EOG: abnorm al (distinguishes from juvenile retinoschisis) Oth er fin d in gs: orofacial abnorm alities (Pierre Robin
anom aly: m idfacial flattening and cleft palate), m arfinoid
Tr eatm en t: m ay require retinal surgery for retinal tears or habitus, hearing loss, m itral valve prolapse, joint abnorm alities
detachm ents (hyperextensibility, enlargem ent, arthritis)

Vitre o re tin a l De g e ne ra tio ns ERG: decreased b wave

Wa g n e r s yn d ro m e (AD) He re d it a ry a rt h ro -o p h t h a lm o p a t h y
Mapped to chrom osom es 5q13-q14 (WGN1), 12q13
(We ill-Ma rc h e s a n i-lik e va rie t y)
(COL2A1) Sim ilar to Stickler’s but short stature with stubby hands and
feet; increased risk of ectopia lentis
Fin d in gs: optically em pty vitreous (abnorm al vitreous
structure; vitreous liquefaction and fibrillar condensation S n o w fla ke d e g e n e ra t io n (AD)
result in a clear vitreous space with m em branes, veils, and
strands), no RD, equatorial and perivascular pigm ented lattice- Very rare, progressive vitreoretinal degeneration
like changes, peripheral vessel sheathing, RPE atrophy, cataract Manifests after 25 years of age
(wedge and fleck opacities between ages 20 and 40), m oderate
m yopia, optic atrophy
Fin d in gs: optically em pty vitreous; associated with
peripheral NV, increased risk of RD, early cataracts
ERG: m ay be abnorm al Stage 1: Extensive white without pressure
Stage 2: Snowflakes (white dots in superficial retina) within
Tr eatm en t: cataract extraction; genetic counseling white without pressure
Stage 3: Sheathing of retinal vessels and early peripheral
pigm entation
J a n s e n s yn d ro m e (AD)
Stage 4: Disappearance of retinal vessels in periphery and
Wagner syndrom e + RD increased retinal pigm entation

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 Retinal Disorders

ERG: decreased scotopic b-wave am plitude AUTOSOMAL RECESSIVE: both m ales and fem ales
affected
EOG: norm al ERG and EOG: supranorm al
Dar k ad ap tatio n : elevated rod threshold later in disease
Aic a rd i’s s yn d ro m e (X-lin ke d d o m in a n t )
Only fem ales, lethal in m ales
Mis c e lla ne o us Re tina l Dis o rd e rs
Lacunar defects in RPE
Alb in is m
Fin d in gs: widespread depigm ented round chorioretinal
Alb in o id ism : only m inim al reduction in vision; no lesions, ON head colobom a, m icrophthalm os
nystagm us
Alb in ism : AR or less frequently AD disorder characterized by Oth er fin d in gs: infantile spasm s, severe m ental
retardation, agenesis of corpus collosum
decreased m elanin and congenitally subnorm al vision (foveal
hypoplasia and nystagm us)
Co lo r b lin d n e s s / d e fic ie n c y (Tables 5-5 and 5-6)
Oculocutaneous: lack of pigm entation of skin, hair, and eyes
TYROSINASE-NEGATIVE (no pigm entation): Defect in Classificatio n : Norm al individuals are trichrom ats: can
chrom osom e 11 m atch any color by m ixing three prim ary colors (red, green,
FINDINGS: decreased vision, iris transillumination, and blue).
foveal hypoplasia, hypopigmented fundus, 3 types of cones (L (long wavelength sensitive; red), M (middle
nystagmus, photophobia, high myopia, strabismus wavelength sensitive; green), S(short wavelength sensitive; blue))
OTHER FINDINGS: hypopigm entation of skin and hair
Congenital color blindness/deficiency caused by absence or
(white hair, pink skin)
abnorm ality of one or m ore photopigm ents
TYROSINASE-POSITIVE (som e pigm entation): Defect in
chrom osom e 15 (paternal ¼ Prader-Willi Prot ¼ Red; Deuter ¼ Green; Trit ¼ Blue (Greek for 1st, 2nd, 3rd;
syndrom e; m aternal ¼ Angelm an’s syndrom e) order in which these deficits were described)
Ocular and system ic characteristics less severe
Anopia ¼ absent; Anom aly ¼ abnorm al; An ¼ unspecified
POTENTIALLY LETHAL VARIANTS:
CH EDIAK-HIGASHI SYNDROME: large m elanosom es on Congenital color vision defect is typically red-green; acquired
skin biopsy, reticuloendothelial dysfunction with color vision defect is typically blue-yellow (depends on
pancytopenia, recurrent infections, and
m alignancies (leukem ia, lym phom a)
Ta b le 5-5. Color vision abnormalities
HERMANSKY-PUDLAK SYNDROME: clotting disorder
due to abnorm al platelets, com m only of Puerto Typ e Ge ne tic s Ma le Fe m a le
Rican descent Deuteranomaly X-linked recessive 5% 0.35%
Ocular: abnorm al m elanogenesis lim ited to eye (decreased Deuteranopia X-linked recessive 1% 0.01%
num ber of m elanosom es)
Protonanomaly X-linked recessive 1% 0.03%
X-LINKED RECESSIVE: iris transillum ination less
Protonanopia X-linked recessive 1% 0.01%
prom inent, fem ale carriers with variable retinal
Tritanomaly Autosomal 0.0001% 0.0001%
pigm entation (m osaic pattern)
Dominant
Giant m elanosom es in skin, norm al pigm entation
Tritanopia Autosomal 0.001% 0.001%
of skin Recessive
Associated with deafness

Ta b le 5-6. Comparison of dyschromatopsias

Co ng e nita l d ys c hro m a to p s ia Ac q uire d d ys c hro m a to p s ia

Deuteranomalous trichromats are most common Ocular pathology, intracranial injury, medication
(5% of male population)
Usually red-green axis (protan or deutan) Usually blue-yellow axis (tritan)
Type 1 (red-green; similar to protan): progressive cone dystrophies, RPE dystrophies
Type 2 (red-green; similar to deutan): optic neuritis, Leber’s optic atrophy, tobacco/toxic amblyopia, lesions of
optic nerve and visual pathway, papillitis
Type 3 (tritan): glaucoma, AMD, CSR, rod and rod-cone dystrophies, retinal detachment, retinal vascular
occlus ion, diabetic retinopathy, myopic degeneration, papilledema, autosomal dominant optic atrophy
Males > females Females ¼ males
Nonprogressive May be slowly progressive
Bilateral and symmetric Unilateral or a symmetric
Normal eye examination (normal visual acuity and Associated with abnormality on eye examination (usually have decreased visual acuity and visual field defects)
visual fields)
X-linked recessive No heritability
Deep blue is perceived as purple Deep blue is perceived as gray

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CHAPTER 5 • Pediatrics/Strabism us

location of lesion: outer retinal layer lesions and ocular m edia Tr itan co lo r d efect an d sligh tly su b n o r m al
changes produce blue-yellow defect; inner retinal layer, optic visio n : consider Kjer’s dom inant optic atrophy
nerve, and visual pathways lesions produce red-green defect,
except glaucom a (blue-yellow defect); S cones m ost susceptible Diagn o sis: color vision testing
to sensitivity loss (occurs with increased blood glucose Farnsworth-Munsell 100 hue test: consists of 85 hue caps
levels (diabetes) and glaucom a (test with SWAP (short contained in 4 separate racks with 2 end caps fixed;
wavelength autom ated perim etry with blue flash on yellow patient arranges caps between fixed ends in order of hue;
background)) tests for both red-green and blue-yellow defects
Congenital color vision defects do not cause decreased vision, Farnsworth’s panel D-15: derived from 100-hue test, but
except for red and blue cone m onochrom atism uses only 15 caps
City university test: derived from 100-hue test, but uses 10
Red-green disorders are X-linked recessive (m ale charts with a central color and 4 peripheral colors; select
preponderance; chrom osom e Xq28 OPN1LW (opsin 1 long outer color that m atches central color
wave; encoding red sensitive pigm ent) and OPN1MW (opsin 1 Pseudoisochromatic plates: Ishihara plates (for protanopes
m iddle wave; encoding green sensitive pigm ent)); incidence: and deuteranopes; detect only red-green defects), and
8% m ales, 0.5% fem ales (Northern European ancestry); 3-4% Hardy-Rand-Ritter (HRR) polychrom atic plates (test for
African m ales; 3-6% Asian m ales; m en and wom en can have both red-green and blue-yellow defects)
tritan disorders (AD) Anomaloscope: tests severity of color blindness and
Blue-yellow disorders are linked to chrom osom e 7 distinguishes between dichrom ats and anom alous
trichrom ats; based on Rayleigh m atch (adjust m ixture of
red and green light to m atch yellow light source) and
An o m alo u s tr ich r o m atism : all three cones present but m ay also include Moreland m atch (blue-green test for
one with abnorm al proportions; photopigm ent (altered tritan defects)
spectral sensitivity causing slight shift in m axim a of absorption Ko lln er ’s r u le: errors m ade by persons with optic nerve
curve); difficulty distinguishing shades of colors, especially disease tend to resem ble those m ade by protans and
colors of low saturation deutans (red and green), whereas errors m ade by
According to which pigm ent is abnorm al, the disorders individuals with retinal disease resem ble those m ade by
are called: tritans (blue)
Protanomaly: abnorm al red sensitive pigm ent
Deuteranomaly: abnorm al green sensitive pigm ent
Tritanomaly: abnorm al blue sensitive pigm ent
Re tina l Tum o rs
Co n gen ital d ich r o m atism : absence of one type of
Co n g e n it a l h yp e rt ro p h y o f t h e RP E (CHRP E)
color photopigm ent; severe color defects
Protanopia: absence of red sensitive pigm ent (no functional Usually unilateral, congenital, asym ptom atic
L (red) cones)
Deuteranopia: absence of green sensitive pigm ent (no
Fin d in gs: flat, well-circum scribed black lesion with
surrounding halo; larger lesions often contain depigm ented
functional M (green) cones)
lacunae (choroid visible through lacunae); m ultiple
Tritanopia: absence of blue sensitive pigm ent (no
patches with sector distribution called “bear tracks”
functional S (blue) cones)
(Figs. 5-35 to 5-37)
Monochromatism (achromatism): absence of 2 or 3
types of color photopigm ent; unable to distinguish
colors
Two form s of congenital dyschrom atopsia associated with low
vision:
Cone monochromatism (X-linked recessive): failure of 2
of the 3 cone cell photopigm ents to work; only one
type of functional cone; can be red cone
m onochrom atism , green cone m onochrom atism ,
or blue cone m onochrom atism (atypical
achrom atopsia; m ost severe form ; nystagm us, decreased
vision, photophobia, m yopia; vision helped by
m agenta filter)
Rod monochromatism (AR): no cones have functional
photopigm ent therefore com plete achrom atopsia;
poor vision (20/60-20/200) and nystagm us in
infants, m acular pigm entary changes (light fundus,
granularity, Bull's-eye m aculopathy); vision helped by
red filter Fig ure 5-35. Typical congenital hypertrophy of retinal pigment
ERG: norm al under scotopic conditions; reduced during epithelium. (From Augsburger J J , Bolling J J : Hypertrophy of retinal pigment
photopic conditions epithelium. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

130
 Retinal Disorders

Bo x 5.3. Differential diagnosis of congenital hypertrophy of the

retinal pigment epithelium.

Reactive hyperplasia of retinal pigment epithelium

Massive gliosis of retina
Combined hamartoma of retina
Melanotic choroidal nevus or melanoma
Bilateral diffuse uveal melanocytic proliferation ass ociated with systemic
carcinoma syndrome
Adenoma or adenocarcinoma of retinal pigment epithelium
Metastatic melanoma to retina
(From Augsburger J J , Bolling J J : Hypertrophy of retinal pigment epithelium.
In Yanoff M, Duker J S [eds]: Ophthalmology, London, Mosby, 1999.)

Fig ure 5-36. Congenital hypertrophy of retinal pigment epithelium with

prominent depigmented lacunae. (From Augsburger J J , Bolling J J : Hypertrophy
of retinal pigment epithelium. In Yanoff M, Duker J S [eds]: Ophthalmology,
Re t in o b la s t o m a (RB)
London, 1999, Mosby.)
Most com m on intraocular m alignancy in children
Incidence 1 in 20,000
No sex or race predilection
90% diagnosed by 5 years of age; fatal in 2 to 4 years if
untreated
30% bilateral, 30% m ultifocal

Gen etics: (Table 5-7)

Mapped to chrom osom e 13q14, m ust have gene defect on
both chrom osom es
94% sporadic (75% som atic, 25% germ inal m utation); 6%
autosom al dom inant
40% heritable, 80% penetrance
Most occur sporadically in infants with no fam ily history;
bilateral cases are usually fam ilial (risk that additional
Fig ure 5-37. Congenital hypertrophy of retinal pigment epithelium (grouped by offspring will have retinoblastom a is 40%)
pigmentation of retina). (From Augsburger J J , Bolling J J : Hypertrophy of retinal
pigment epithelium. In Yanoff M, Duker J S [eds]: Ophthalmology, ed 2, St Louis, 75% are caused by m utation (inactivation of both RB genes) in
2004, Mosby. a single retinal cell; these tum ors are unilateral and unifocal;
the chance of inactivation of both RB genes is very sm all
Parents with 1 affected child: 6% risk of producing m ore
Path o lo gy: m elanosomes in RPE cells are larger and more affected children
spherical than norm al; densely packed round m elanocytes and
Parents with 2 or more affected children: 40% risk (because
increased thickness of RPE; focal areas of RPE loss (lacunae)
only 80% penetrance)
Atyp ical m u ltifo cal b ilater al var ian t: occurs in 75% of Retinoblastoma survivor with hereditary form: 50% chance of
patients with fam ilial adenom atous polyposis (FAP: AD, transferring to children (but children have only 40%
m apped to chrom osom e 5q; high incidence of m ultiple chance of m anifesting a tum or)
adenom as of colon and rectum ; eventually undergo m alignant
Pr esen tatio n : leukocoria (60%), strabism us (22%),
transform ation. Gardner’s syndrom e [variant of FAP]:
decreased vision (5%)
com bination of colonic polyps and extracolonic
m anifestations [osteom as, derm oid tum ors])
Fin d in gs: yellow-white retinal m ass, rubeosis,
DDx: (Box 5-3) pseudohypopyon, hyphem a, angle-closure glaucom a, uveitis

Ta b le 5-7. Chances of having a baby with retinoblastoma

NEGATIVE FH P OSITIVE FH
Offs p ring o f Unila te ra l (% ) Bila te ra l (% ) Unila te ra l (% ) Bila te ra l (% )
Parents with an affected child 1 6 40 40
Affected patient 8 40 40 40
Normal sibling of affected patient 1 <1 7 7

131
CHAPTER 5 • Pediatrics/Strabism us

Typ es:
Endophytic: arises from inner retina and grows toward
vitreous; can sim ulate endophthalm itis; can have
pseudohypopyon (Fig. 5-38)
Exophytic: arises from outer retina and grows toward
choroid causing solid RD; can extend through sclera; can
sim ulate Coats disease or traum atic RD
Tum or necrosis when outgrows blood supply, then
calcification (seen on CT and ultrasound); m ay develop
NV glaucom a (17%); no m acular tum ors after
1.5 m onths of age

ON in vo lvem en t: present in 29% of enucleated eyes

Fig ure 5-39. Homer-Wright rosettes. (From Augsburger J J , Bornfeld M, Giblin
Risk fo r ON in vasio n : exophytic tum ors > 15 m m , and ME: Retinoblastoma. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999,
eyes that have secondary glaucom a Mosby.)

Seco n d tu m o r s: com m only present around age 17;

osteogenic sarcom a of fem ur (m ost com m on),
m alignant m elanom a of eye or orbit, leiom yosarcom a of eye or
orbit, lym phom a, leukem ia, rhabdom yosarcom a,
m edulloblastom a

Tr ilater al r etin o b lasto m a: bilateral RB with a

pinealoblastom a or parasellar neuroblastom a; occurs in 3%
of children with unilateral RB and 8% with bilateral RB; 95%
have a positive fam ily history and/or other tum ors;
spontaneous regression occurs with subsequent necrosis and
phthisis

Path o lo gy: rosettes are histologic m arkers for tum or

differentiation
In order of increasing differentiation: Fig ure 5-40. Flexner-Wintersteiner rosettes. (From Augsburger J J , Bornfeld M,
Homer-Wright rosette: no lum en; nuclei surround tangle of Giblin ME: Retinoblastoma. In Yanoff M, Duker J S [eds]: Ophthalmology,
London, 1999, Mosby.)
neural filam ents; reflects low-grade neuroblastic
differentiation; can be found in other types of
neuroblastic tum ors (adrenal neuroblastom a, Fleurettes: bouquet of pink bulbous neoplastic
m edulloblastom a) (Fig. 5-39) photoreceptor inner segm ents; highest degree of
Flexner-Wintersteiner rosette: ring of single row of differentiation in retinoblastom a; found in relatively
colum nar cells around central lum en; cells have eosinophilic areas of tum or (photoreceptor
eosinophilic cytoplasm and peripheral nuclei; differentiation)
photoreceptors contain cilia with a 9 + 0 pattern; Pseudorosettes: circum ferential arrangem ents of viable
represent early retinal differentiation; attem pt of tum or cells surrounding a central vessel
outer photoreceptor production; special stains Cells have round, spindle-shaped hyperchrom atic nuclei
show hyaluronidase-resistant acid m ucopolysaccharides and very little cytoplasm ; high m itotic activity; as tum or
in lum en; also present in m edulloepitheliom as grows, outgrows blood supply creating necrosis with
(Fig. 5-40) areas of calcification (80%)
H&E stain:
BLUE AREAS: represent viable tum or; differentiated
cells that have basophilic nuclei and little cytoplasm
PINK AREAS: represent necrotic tum or; lose basophilic
nuclei and appear eosinophilic (pink)
PURPLE AREAS: represent calcified tum or; occur in
necrotic areas; calcium stains purple (Fig. 5-41)

DDx: (DDx of leukocoria): cataract, retrolental m ass (PHPV,

ROP, Norrie disease, RD), tum or (choroidal m etastases, retinal
astrocytom a), exudates (FEVR, Coats disease, Eales’ disease),
re tinobla s toma change in retinal pigm ent (incontinentia pigm enti, high
Fig ure 5-38. Retinoblastoma demonstrating discrete round tumor. (From Kaiser m yopia, m yelinated nerve fiber, retinal dysplasia,
PK, Friedman NJ , Pineda II, R: Massachusetts eye and ear infirmary illustrated choroiderem ia, colobom a), infections (toxoplasm osis,
manual of ophthalmology, ed 2, Philadelphia, 2004,WB Saunders.) toxocariasis, endophthalm itis)

132
 Retinal Disorders

Bilateral involvement does not worsen prognosis;

prognosis depends on status of tumor in worse eye; degree
of necrosis and calcification does not influence prognosis
Reese-Ellsworth classification: predicts visual prognosis (not
survival) in eyes treated with radiation
International Classification of Retinoblastoma (ICRB): better
predicts outcom es:
GROUP A: sm all intraretinal tum ors (< 3 m m ) not
located near m acula or disc
GROUP B: tum ors > 3 m m , m acular or juxtapapillary
location, or with subretinal fluid
GROUP C: tum or with focal subretinal or vitreous
seeding within 3 m m of tum or
GROUP D: tum or with diffuse subretinal or vitreous
seeding > 3 m m from tum or
Fig ure 5-41. RB demonstrating necrosis and optic nerve invasion. (From Yanoff
GROUP E: extensive tum or occupying > 50% of the
M, Fine BS: Ocular pathology, ed 5, Mosby, 2002, Philadelphia.)
globe with or without NVG, hem orrhage, extension of
Diagn o sis: tum or to optic nerve or anterior cham ber
LDH levels: ratio of aqueous:plasm a lactate dehydrogenase
(LDH) > 1.0
Ultrasound: acoustic solidity and high internal reflectivity; Re t in o c yt o m a / re t in o m a
calcium appears as dense echoes Benign tum or with sam e appearance and genetics as RB
FA: early hyperfluorescence with late leakage of lesions
MRI: look for pineal tum or, ON involvem ent
Path o lo gy: num erous fleurettes am ong cells with varying
CT scan (less preferred due to radiation exposure):
degrees of photoreceptor differentiation
calcification;
Metastatic workup: bone scan, bone m arrow aspirate, LP
(cytology) Differ en tiatio n fr o m RB: m ore cytoplasm , m ore
evenly dispersed nuclear chromatin, no m itoses,
Tr eatm en t: calcification m ay be present, and necrosis is usually absent in
Chemotherapy: systemic chemotherapy often indicated for retinocytom a
bilateral disease, intra-arterial chemotherapy for unilateral
disease, intravitreal chemotherapy for vitreous seeds 1 3 q d e le t io n s yn d ro m e
Photocoagulation/cryotherapy: eyes with one or a few sm all
tum ors, not involving ON or m acula, tum ors that have Associated with retinoblastom a, m icrocephaly, hypertelorism ,
been reduced by chem otherapy m icrophthalm os, ptosis, and epicanthus
Enucleation: all blind and painful eyes; affected eye in m any
unilateral cases; worse eye in m ost asym m etric cases; Le u ke m ia
both eyes in m any sym m etric cases; excise at least 10 m m
Most com m on m alignancy of childhood
of ON to prevent spread
External beam radiation: salvageable eyes with vitreous Usually affects choroid with retinal hem orrhages; usually
seeding or large tum or; m ost eyes with m ultifocal unilateral
tum ors; eyes that have failed coagulation therapy; RB is
very radiosensitive; used to treat m ost 2nd tum ors Sym p to m s: blurred vision, floaters
Episcleral plaque radiation: salvageable eyes with single
m edium -sized tum or that does not involve ON or
m acula, even with localized vitreous seeding Fin d in gs (Figs. 5-42 and 5-43): cellular infiltration of
vitreous; infiltrative lesions of retina, optic nerve, or uvea;
m ultiple hem orrhages, Roth spots, cotton wool spots (CWS);
Pr o gn o sis: 90–95% of children with retinoblastom a survive heterochrom ia irides, pseudohypopyon, spontaneous
(sam e with either growth pattern); 3% spontaneously regress hyphem a, uveitic glaucom a, cataract
Metastases: m ost com m only to CNS along ON; 50% are
to bone Optic nerve infiltration causes loss of vision and papilledem a
Familial RB: location related to age, earliest in m acula, later
Orbital infiltration (rare) causes proptosis, lid swelling,
in periphery; second eye tumor develops up to 44 months
ecchym osis (1%-2% of patients)
later; 45% m ortality by age 35 (vs. 19% long-term survival
for all patients with RB by age 35); m ultiple primary
tumors in an eye does not worsen prognosis Tr eatm en t: em ergent XRT for ON infiltration; patient is
Increased risk of second unrelated m alignancy in 25% of m ore susceptible to developing radiation optic neuropathy
children with heritable retinoblastom a when chem otherapy is used concurrently
Poor prognostic signs: ON invasion, uveal invasion,
extrascleral extension, m ultifocal tum ors (represent Pr o gn o sis: poor; high m ortality; can be rapidly fatal if
seeding); delay in diagnosis, degree of differentiation untreated

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CHAPTER 5 • Pediatrics/Strabism us

Ta b le 5-8. Genetics of the phakomatoses

Dis o rd e r Chro m o s o m e
Neurofibromatosis:
Type 1 17
Type 2 22
Sturge-Weber None
von Hippel-Lindau 3
Tuberous sclerosis 9
Ataxia-telangiectasia 11
Wyburn-Mason None

Tr eatm en t: often requires CT scans and m edical

consultations; m ay require treatm ent of elevated IOP
Fig ure 5-42. Leukemic infiltrate in optic nerve. (From Yanoff M, Fine BS: Ocular Ne u ro fib ro m a t o s is (AD)
pathology, ed 5, Mosby, 2002, Philadelphia.)
Variable expressivity

P HAKOMATOSES Disorder of Schwann cells and m elanocytes with ham artom as

of nervous system , skin, and eye
Group of disorders (neurocutaneous syndrom es) characterized Typ es:
by ocular and system ic ham artom as. Most are AD with variable NF-1 (von Recklinghausen’s syndrome): m apped to
penetrance except Sturge-Weber and Wyburn-Mason (no chrom osom e 17q11 (neurofibrom in), 50% due to new
hereditary pattern) and ataxia-telangiectasia (AR) (Table 5-8) m utation; 80% penetrance

A B

C D
Fig ure 5-43. Acute leukemia. A, A patient presented with a large infiltrate of leukemic cells positioned nasally within the conjunctiva of the right eye, giving this
characteristic clinical picture. These lesions look similar to those caused by benign lymphoid hyperplasia, lymphoma, or amyloidosis. B, A biopsy of the lesion
shows primitive blastic leukocytes. C, In another case, the iris is infiltrated by leukemic cells. A special stain (Lader stain) shows that some of the cells stain red, better
seen when viewed under increased magnification in D. This red positiveness is characteristic of myelogenous leukemic cells. (From Augsburger J J , Tsiaras WG:
Lymphoma and leukemia. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

134
 Phakom atoses

More com m on form (prevalence 1 in 3000 to 5000) angiom as of iris), angiom as of episclera and CB, diffuse
CRITERIA (two or m ore of the following): six or m ore cafe cavernous choroidal hem angiom a (“tom ato-ketchup” fundus
au lait spots > 5 m m in diam eter in prepubescent [50%]), peripheral retinal AV m alform ations, m ay get RD or
or > 15 m m in postpubescent individuals severe RPE alterations (pseudo-RP)
two or m ore neurofibrom as or one plexiform Mechanism of glaucoma: neovascular, increased episcleral
neurofibrom a venous pressure, im m ature angle structures
Freckling of intertriginous areas
Optic nerve gliom a
Oth er fin d in gs: leptom eningeal vascular m alform ations
two or m ore Lisch nodules
(ipsilateral to port wine stain), central calcifications, m ental
Osseous lesion (sphenoid bone dysplasia,
retardation, seizures, pheochrom ocytom a
thinning of long bone cortex)
First-degree relative with NF-1
FINDINGS: plexiform neurofibrom a (plexus of abnorm al Klip p el-Tr en au n ay-Web er : variant of Sturge-Weber with
m arkedly enlarged nerves; occurs in 25%; 10% cutaneous nevus flam m eus, hem angiom as, varicosities,
involve face, often upper eyelid or orbit; “bag of intracranial angiom as, and hem ihypertrophy of lim bs
worm s” appearance and S-shaped upper lid; Findings (uncom m on): congenital glaucom a, conjunctival
congenital glaucom a in ipsilateral eye in up to 50%), telangiectasia; can have AV m alform ation sim ilar to
fibroma molluscum, plexiform neurofibroma of Wyburn-Mason
conjunctiva, prominent corneal nerves, Lisch nodules
(glial/melanocytic iris ham artom as), diffuse uveal An g io m a t o s is re t in a e (Vo n Hip p e l–Lin d a u
thickening due to excess m elanocytes and neurons d is e a s e ) (AD)
(similar to ocular melanocytosis), ectropion uveae,
retinal astrocytic hamartom a (less likely to be calcified Incom plete penetrance; m apped to chrom osom e 3p26-p25
than in tuberous sclerosis), increased incidence of (VHL)
myelinated nerve fibers and choroidal nevi (33%), ON
Prevalence 1 in 100,000
glioma (juvenile pilocytic astrocytoma in > 30%; may
cause visual loss, hypothalamic dysfunction or 50% bilateral
hydrocephalus; neuroim aging shows fusiform
Ham artom as of eye, brain (cerebellum ), kidney/adrenal gland
enlargement of nerve with kinking; if have glioma,
25% have NF), meningioma, orbital plexiform (Note: disease has three nam es, three locations for tum ors, and
neurofibroma, schwannoma, absence of sphenoid defect on chrom osom e 3)
wing (pulsating exophthalmos)
OTHER FINDINGS: cafe au lait spots, intertriginous Fin d in gs: retinal angiom a (hem angiom a or
(axillary) freckling, cutaneous peripheral nerve sheath hem angioblastom a; round orange-red m ass fed by dilated
tum ors tortuous retinal artery and drained by engorged vein; m ay be
NF-2: m apped to chrom osom e 22q m ultifocal as well as bilateral; often in m idperiphery, m ay be
Prevalence 1 in 50,000 near disc; leaks heavily causing serous RD and/or m acular
CRITERIA: edem a; treat with yellow dye laser if enlarges)
Bilateral cerebellar–pontine angle tum ors
(acoustic neurom as; cause hearing loss, ataxia,
headache) Oth er fin d in gs: 25% of retinal capillary hem angiom as
First-degree relative with NF-2 and either a unilateral associated with CNS tum or (hem angioblastom a of cerebellum
acoustic neurom a or two of the following: [60%]; pons, m edulla, and/or spinal cord is less com m on);
m eningiom a, schwannom a, neurofibrom a, visceral lesions (cysts and tum ors of kidney, pancreas, liver,
gliom a, posterior subcapsular cataract adrenal glands, including renal cell carcinom a [25%],
May have pheochromocytoma and other malignant pheochrom ocytom a [5%])
tumors
No Lisch nodules Vo n Hip p el d isease: only ocular involvem ent
En c e p h a lo t rig e m in a l a n g io m a t o s is
(S t u rg e -We b e r s yn d ro m e ) Tr eatm en t: observation, cryotherapy, or laser
photocoagulation
Nonhereditary
No racial or sex predilection
Tu b e ro u s s c le ro s is (Bo u rn e ville ’s d is e a s e )
(AD o r s p o ra d ic )
Facial h em an gio m a: nevus flam m eus (port wine stain)
lim ited to first two divisions of CN 5; 5%–10% bilateral Mapped to chrom osom es 9q34 (TSC1 [ham artin]), 16p13
(TSC2 [tuberin])
Fin d in gs: dilated tortuous vessels of the conjunctiva and
Prevalence 1 in 10,000 to 100,000
episclera, congenital or juvenile glaucom a (25% risk, especially
if upper lid is involved), heterochrom ia irides (due to Triad of adenom a sebaceum , m ental retardation, and epilepsy

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CHAPTER 5 • Pediatrics/Strabism us

Fin d in gs: astrocytic ham artom a of the retina (flat or

m ulberry-shaped with calcifications; usually in posterior pole;
consists of nerve fibers and undifferentiated glial cells; occurs
in 50%; bilateral in 15%), astrocytic ham artom a of the ON
(“giant drusen”; benign)

Oth er fin d in gs:

Cutaneous: facial angiofibrom a (adenom a sebaceum ;
vascularized red papules in butterfly distribution, not
present at birth, becom e visible between ages 2 and 5),
ash leaf spots (hypopigm ented spots that fluoresce under
Wood’s light, considered pathognom onic), shagreen
patches (25%; areas of fibrom atous infiltration, usually
on trunk), periungual fibrom as, m ay have cafe au
lait spots
CNS: subependym al ham artom as (calcify, form ing “brain Fig ure 5-44. Incontinentia pigmenti. The peripheral retina of a patient who has
incontinentia pigmenti demonstrates somewhat elevated vessels with vessel
stones” with rootlike appearance; concentrated in walls. A majority of these vessels show nonperfusion. The more posterior retina
periventricular area), m ental retardation (60%), seizures was perfused, and the anterior retina was ischemic. (From Ebroom DA, J ampol
(80%), cerebral calcification J M: Proliferation retinopathies. In Yanoff M, Duker J S [eds ]: Ophthalmology,
Other: cardiac rhabdom yom a, spontaneous pneum othorax London, 1999, Mosby.)
(from pleural cyst), renal angiom yolipom as,
pheochrom ocytom a Vitreous hem orrhage is rare
Early m ortality
May be associated with cutaneous and CNS hem angiom as

At a xia -t e la n g ie c t a s ia (Lo u is -Ba r s yn d ro m e ) (AR) FA: fluid levels without leakage within lesions
Mapped to chrom osom e 11q22 (ATM)
In c o n t in e n t ia p ig m e n t i (Blo c h -S u lz b e rg e r
Prevalence 1 in 40,000
s yn d ro m e ) (X-lin ke d d o m in a n t )
Fin d in gs: prom inent dilated conjunctival vessels, im paired Occurs exclusively in fem ales (lethal in m ales)
convergence, nystagm us, oculom otor apraxia
Fin d in gs: proliferative retinal vasculopathy (resem bles
Oth er fin d in gs: cutaneous telangiectasia in butterfly ROP); m ay have retinal detachm ent and retrolental m em brane
distribution during 1st decade of life, mental retardation, (Fig. 5-44)
cerebellar ataxia (due to cerebellar atrophy), thymic hypoplasia
with defective T-cell function and IgA deficiency with increased Oth er fin d in gs:
risk of infections, malignancy (leukemia/lymphoma) Skin lesions:
STAGE 1 (at birth): erythem atous m acules, papules, and
bullae on trunk and extrem ities (intraepithelial
Ra c e m o s e h e m a n g io m a t o s is vesicles containing eosinophils)
(Wyb u rn -Ma s o n s yn d ro m e ) STAGE 2 (2 m onths of age): vesicles are replaced by
verrucous lesions
Nonhereditary, usually unilateral
STAGE 3 (3-6 m onths of age): lesions take form of
pigm ented whorls on trunk
Fin d in gs: racem ose hem angiom a of retina (arteriovenous
STAGE 4: skin is atrophic with hypopigm ented patches
m alform ation with m arkedly dilated and tortuous shunt
Hair abnormalities: alopecia
vessels); m ay have intraocular hem orrhage or glaucom a
CNS abnormalities: m icrocephaly, hydrocephalus, seizures,
Oth er fin d in gs: arteriovenous m alform ations in brain m ental deficiency
(m ay cause seizures, paresis, m ental changes, VF defects), orbit Dental abnormalities: m issing and cone-shaped teeth
and facial bones; m ay have sm all facial hem angiom as Tr eatm en t: photocoagulation (variable results)

Othe rs OP TIC NERVE DISORDERS

Re t in a l c a ve rn o u s h e m a n g io m a
Ap la s ia
Cluster of intraretinal aneurysm s filled with venous blood
Com plete absence of optic nerve; very rare; m ay occur with
Appears as “cluster of grapes” anencephaly or m ajor cerebral m aldevelopm ent

136
 Optic Nerve Disorders

Hyp o p la s ia Fin d in gs: cup filled with glial tissue surrounded by pigm ent
ring, adjacent retinal folds com m on; also described as funnel-
Variable visual com prom ise
shaped, enlarged, excavated disc with central white connective
Etiology unclear tissue; vessels radiate in spoke-like fashion; m ay develop
usually poor vision; peripapillary RD
No sex predilection
Op t ic p it
Asso ciatio n s: aniridia, Goldenhar’s syndrom e, m idline Gray-white depression in optic disc, usually in
anom alies, endocrine abnorm alities, congenital intracranial inferotem poral area
tum ors (craniopharyngiom a, optic gliom a), m aternal diabetes 85% unilateral
or drug ingestion during pregnancy (alcohol, LSD, quinine,
dilantin) Associated with peripapillary RPE disturbances
May develop serous retinal detachm ent extending from
Fin d in gs: double ring sign (thin ring of pigm ent pit (40%)
surrounding nerve tissue; halo of retina and RPE partially Tilt e d d is c
covering lam ina cribrosa), strabism us, am blyopia, nystagm us,
positive RAPD, VF defects Fin d in gs (any of the following m ay occur, alone or in
com bination): apparent tilting (usually inferiorly with
superior pole of disc elevated), scleral crescent, situs inversus
Oth er fin d in gs: CNS abnorm alities (45%), growth arteriosus (vessels em erge tem porally from optic nerve [rather
retardation, endocrine abnorm alities than nasally] and course nasally before sweeping tem porally),
m yopia and/or astigm atism , reduced visual acuity, visual field
defects (usually bitem poral and do not respect vertical
De Mo r sier ’s syn d r o m e ( sep to -o p tic d ysp lasia) : m idline)
bilateral ON hypoplasia, septum pellucidum abnorm ality,
pituitary and hypothalam us deficiency; associated with
agenesis of the corpus callosum ; m ay have chiasm al
developm ental anom alies with VF defects; at risk for Mye lin a t e d n e rve fib e rs
sudden death Myelination begins at lateral geniculate body and usually
ceases at lam ina cribrosa; however, som e retinal fibers m ay
acquire m yelin sheath during first m onth of life
Tr eatm en t: brain MRI, endocrine workup
More com m on in m ales

Co lo b o m a Unilateral or bilateral (20%)

Due to incom plete closure of em bryonic fissure; usually Vision generally good unless m acula involved; increased risk of
located inferonasal am blyopia

Unilateral or bilateral Fin d in gs: superficial white flam e–shaped patches with
feathery m argins; usually peripapillary; can be extensive
Variable visual acuity and visual field defects
Relative or absolute scotom a corresponds to area of
Associated with other ocular colobom as
m yelination

P e rs is t e n c e o f h ya lo id s ys t e m
Fin d in gs: large anom alous discs, deep excavation with
abnorm al vascular pattern (ranges from com plete chorioretinal Com m on; ranges from tuft of glial tissue on disc (Bergm eister’s
colobom a to involvem ent of proxim al portion of em bryonic papillae) to patent artery extending from disc to lens
fissure causing only optic nerve deform ity); m ay resem ble
physiologic cupping if m ild
Me g a lo p a p illa
Enlarged optic disc
Mo rn in g g lo ry d is c
Probably represents a dysplastic colobom a
P e rip a p illa ry s t a p h ylo m a
Posterior bulging of sclera in which optic disc occupies bottom
Generally unilateral
of bulge
Fem ale > m ale (2:1)
Often seen with high m yopia Op t ic n e r ve d ru s e n
May be associated with cranial defects or other ocular Superficial or buried hyaline bodies in prelam inar portion of
anom alies optic nerve

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CHAPTER 5 • Pediatrics/Strabism us

Sporadic or AD; 75% bilateral Kjer ’s syn d r o m e (AD): dominant optic atrophy; most
common form of heritable optic atrophy; mapped to
Incidence 0.3%–1% clinically; 2% histopathologically
chromosome 3q28 (OPA1); insidious onset between ages of 4
More com m on in Caucasians; no sex predilection and 8; bilateral and symmetric; decreased vision (20/40-20/200),
blue–yellow dyschromatopsia, temporal wedge of disc pallor
Asso ciatio n s: angioid streaks, retinitis pigm entosa,
Alagille’s syndrom e (fam ilial intrahepatic cholestasis, posterior Wo lfr am syn d r o m e (AR): onset between ages of 5 and 21;
em bryotoxin, bilateral optic disc drusen [80%]) slowly progressive; decreased vision (< 20/400), diffuse optic
atrophy; DIDMOAD (Diabetes Insipidus, Diabetes Mellitus,
Optic Atrophy, Deafness), ataxia, seizures, m ental retardation
Fin d in gs: disc m argins m ay show irregular outline, bum py
nodular chunky appearance to nerve head
(pseudopapilledem a); VF defects (especially with deep drusen;
Beh r ’s syn d r o m e (AR): complicated hereditaryinfantileoptic
atrophy; onset before age 10, male > female, nonprogressive;
enlarged blind spot, arcuate scotom a, sectoral scotom a); m ay
moderate to severe decreased vision, nystagmus, diffuse optic
have transient visual obscurations, positive RAPD (unilateral
atrophy; ataxia, spasticity, hypotonia, mental retardation
ON drusen); often calcify with age
Oth er s: congenital recessive optic atrophy, apparent
Path o lo gy: hyaline bodies that becom e calcified; stain sex-linked optic atrophy
positively for am ino acids, calcium , acid m ucopolysaccharides,
and hem osiderin; stain negatively for am yloid DDx: acquired optic neuropathies: com pressive (tum ors
[craniopharyngiom a, optic nerve/chiasm al gliom as],
Diagn o sis: B-scan ultrasound, CT scan, autofluorescence hydrocephalus), toxic (anoxia at birth or in neonatal period),
traum atic, infiltrative (leukem ia, m etabolic storage diseases)
Co m p licatio n s: rarely visual loss due to axonal
com pression, AION, CNV, subretinal or vitreous hem orrhage, Tr eatm en t: no treatm ent; genetic counseling
vascular occlusion
Mit o c h o n d ria l d is e a s e s
Me la n o c yt o m a (Ma g n o c e llu la r n e vu s o f t h e Maternal inheritance; children of both sexes affected; only
o p t ic d is c ) fem ale offspring can pass on
Deeply pigm ented tum or with feathery border located over ON
Fin d in gs: optic atrophy, CPEO, pigm entary retinopathy,
Derived from uveal dendritic m elanocytes retrochiasm al visual loss
May have choroidal and NFL involvem ent
Diso r d er s:
15% show m inim al enlargem ent over 5 years Maternal inherited diabetes and deafness (MIDD): see earlier
Leber’s hereditary optic neuropathy (LHON): see earlier
Fin d in gs: VF defect; m ay have positive RAPD (even with Kearns-Sayre syndrome: onset before age 20
good vision) FINDINGS: chronic progressive external ophthalm oplegia
with ptosis, pigm entary retinopathy (salt and pepper,
bone spicules, and/or RPE atrophy), m ild visual loss
Path o lo gy: benign, plum p, round polyhedral m elanocytes
(50%)
Malignant transform ation very rare OTHER FINDINGS: neck and lim b weakness, cardiac
conduction defects (arrhythm ias, heart block,
He re d it a ry o p t ic n e u ro p a t h y cardiom yopathy), cerebellar ataxia
PATHOLOGY: “ragged red” fibers (contain degenerated
Group of disorders with optic nerve dysfunction (isolated or m itochondria) on m uscle biopsy
part of system ic disease) Most com m on are: EKG: heart block
CSF: elevated protein
Leb er ’s h er ed itar y o p tic n eu r o p ath y MELAS: Mitochondrial Encephalopathy, Lactic Acidosis,
( LHON) : m aternal m itochondrial DNA; point m utations in and Stroke-like episodes
m itochondrial gene for NADH subunit 4 (position 11778 Onset before age 15; point m utations (3242, 3271) or
[m ost com m on], 3460, 14484); m ale > fem ale (9:1); m aternal deletions
transm ission to all sons (50% affected) and all daughters (15% FINDINGS: retrochiasmal visual loss; recurrent attacks
of daughters affected, 85% are carriers); onset between ages of of headache, vom iting, seizures; transient focal
15 and 30; subacute sequential bilateral vision loss ( 20/200) neurologic deficits (hemiplegia, hem ianopia/cortical
over days; tobacco or alcohol can trigger decom pensation blindness), CPEO, optic neuropathy; pigmentary
Findings: disc hyperem ia, peripapillary telangiectatic vessels retinopathy, dem entia, hearing loss, short stature,
(do not leak fluorescein, also found in 60% of muscle weakness
asym ptom atic fam ily m em bers), tortuous vessels, PATHOLOGY: “ragged red” fibers on m uscle biopsy,
peripapillary NFL edem a, late optic disc pallor; m ay have abnorm al mitochondria in blood vessels (Fig. 5-45)
cardiac conduction abnorm alities DIAGNOSIS: Elevated serum and CSF lactate

138
 Anatom y and Physiology

Fa t cus hion
S upe rior outs ide Orbita l
s e ptum
Le va tor re ctus mus cle cone

Annulus
of Zinn
Te non's
ca ps ule
Conjunctiva

Fig ure 5-45. MELAS (Mitochondrial Encephalopathy, Lactic Acidosis, and

Strokelike episodes) syndrome demonstrating degenerated extraocular La te ra l
muscle (EOM)with trichome stain shows “ragged red” fibers. (Case presented by re ctus
Dr. R. Folberg to the meeting of the Verhoeff Society, 1993, and reported by Infe rior
Rummett V, Folberg R, Ionasescu V et al: Ocular pathology of MELAS syndrome re ctus
with mitochondrial DNA nucleotide 3243 point mutation. Ophthalmology, Orbita l
100:1757-66, 1993.) s e ptum
Fa t cus hion
Inte rmus cula r Infe rior
ins ide
CT SCAN: basal ganglia calcification s e ptum oblique
mus cle cone
MRI: posterior cortex lesions, spare deep white m atter;
can resolve Fig ure 5-46. Muscle cone. (From Campolattaro BN, Wang FM: Anatomy and
physiology of the extraocular muscles and surrounding tissues. In Yanoff M,
MERRF: Myoclonus, Epilepsy, and Ragged Red Fibers Duker J S [eds]: Ophthalmology, ed 2, St Louis, 2004, Mosby.) 2004.)
FINDINGS: encephalopathy with m yoclonus, seizures,
ataxia, spasticity, dem entia; m ay have dysarthria,
optic neuropathy, nystagm us, short stature,
hearing loss 3. Hardest to find if slipped because no fascial
PATHOLOGY: “ragged red” fibers on m uscle biopsy attachm ent to an oblique m uscle
(Gom ori trichrom e stain)
Later al r ectu s (LR):
STRABISMUS 1. Pure abductor
2. Easiest exposure
3. Connected to inferior oblique

ANATOMY AND P HYSIOLOGY

Su p er io r r ectu s (SR):
1. Elevates, adducts, and incyclotorts
S u b c o n ju n c t iva l fa s c ia (Fig. 5-46) 2. Inserts 23° tem poral to visual axis in prim ary position;
Ten o n ’s cap su le: pure elevator only in 23° abduction
Anterior: fuses with conjunctiva just behind lim bus 3. Fascial connections: SO, upper eyelid elevators.
Posterior: separates orbital fat from m uscles and globe Therefore, SR recession m ay cause lid retraction, and
resection may cause fissure narrowing
In ter m u scu lar sep tu m : extension of Tenon’s, connects 4. Passes over superior oblique
m uscles
Ch eck ligam en ts: connect m uscles to overlying Tenon’s In fer io r r ectu s (IR):
and inserts on orbital walls to support globe
1. Depresses, adducts, and excyclotorts
Lo ckwo o d ’s ligam en t: fusion of sheaths of inferior rectus 2. Inserts 23° tem poral to visual axis in prim ary position;
and inferior oblique; attaches to medial and lateral retinaculi and pure depressor only in 23° abduction
supports globe 3. Fascial connections: IO, lower eyelid retractors
(Lockwood’s ligam ent). Therefore, IR recession m ay
Ext ra o c u la r m u s c le s (Table 5-9, Figs. 5-47 to 5-49) cause lid retraction, and resection m ay cause fissure
All rectus m uscles have two accom panying ciliary arteries narrowing
except lateral rectus, which has one

Sp ir al o f Tillau x: Continuous curve that passes through Su p er io r o b liqu e (SO):

insertions of rectus muscles; the temporal aspect of vertical 1. Incyclotorts, abducts, and depresses
muscles lies farther from lim bus than the nasal aspect (Fig. 5-50) 2. Inserts 51° to visual axis; pure depressor only in 51°
adduction
Med ial r ectu s (MR): 3. Passes inferior to SR
1. Pure adductor 4. Arises from orbital apex above annulus of Zinn (lesser
2. Closest to lim bus wing of sphenoid)

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CHAPTER 5 • Pediatrics/Strabism us

Ta b le 5-9. Extraocular muscles

Mus c le Te nd o n Arc o f Ac tio n fro m

le ng th le ng th c o nta c t Ana to m ic ins e rtio n p rim a ry
Mus c le (m m ) (m m ) (m m ) fro m lim b us p o s itio n Orig in Inne rva tio n
Medial rectus 40 4.5 7 5.5 mm Adduction Annulus of Zinn CN 3, (inferior
(MR) division)
Lateral rectus 40 7 12 6.9 mm Abduction Annulus of Zinn CN 6
(LR)
Superiorrectus 40 6 6.5 7.7 mm Elevation Annulus of Zinn CN 3,
(SR) Intorsion (superior
Adduction division)
Inferior rectus 40 7 6.5 6.5 mm Depres sion Annulus of Zinn CN 3, (inferior
(IR) Extorsion division)
Adduction
Superior 32 26 7-8 Posterior to equator Intorsion Orbital apex CN 4
oblique (SO) insuperotemporal quadrant Depres sion above annulus of
Abduction Zinn
Inferior oblique 37 1 15 Posterior to equator Extorsion Behind lacrimal CN 3, (inferior
(IO) ininferotemporal quadrant Elevation fossa division)
Abduction
Levator 40 14-20 — Septa of pretarsal orbicularis Lid elevation Orbital apex CN 3,
palpebrae and anterior surface of tarsus above annulus of (superior
Zinn division)

Annulus of Zinn Optic ne rve Me dia l Infe rior

re ctus re ctus Optica l Mus cle
a xis 23 pla ne

Trochle a r ne rve

Fronta l ne rve

La crima l ne rve
Ophtha lmic
ve in Ophtha lmic
a rte ry
S upe rior Infe rior oblique
oblique

S upe rior divis ion Infe rior divis ion

oculomotor ne rve oculomotor ne rve S upe rior re ctus

Abduce ns Na s ocilia ry
ne rve ne rve

Fig ure 5-47. The annulus of Zinn and surrounding structures. (From
Campolattaro BN, Wang FM: Anatomy and physiology of the extraocular muscles La te ra l re ctus
and surrounding tissues. In Yanoff M, Duker J S [eds]: Ophthalmology, ed 2, St
Louis, 2004, Mosby.)

Fig ure 5-48. The extrinsic mucles of the right eyeball in the primary position, seen
from above. The muscles are shown as partially transparent. From Campolattaro
BN, Wang FM: Anatomy and physiology of the extraocular muscles and
In fer io r o b liqu e (IO): surrounding tissues. In Yanoff M, Duker J S [eds]: Ophthalmology, ed 2, St Louis,
1. Excyclotorts, abducts, and elevates 2004, Mosby.)
2. Inserts 51° to visual axis; pure elevator only in 51°
adduction
P e d ia t ric e ye e xa m in a t io n
3. Passes inferior to IR
4. Originates from periosteum of m axillary bone Visu al d evelo p m en t:
5. Inserts near m acula At birth: blinking response to bright light
6. Avoid inferotem poral vortex vein during surgery At 7 days: vestibulo-ocular response
7. The inferior division of CN 3 up to the inferior At 2 months: fixation well developed
oblique carries parasym pathetic supply to iris At 6 months: VER acuity at adult level
constrictor; injury to these fibers results in m ydriasis At 2 years: Snellen acuity at adult level

140
 Anatom y and Physiology

S upe rior oblique S upe rior re ctus S upe rior te mpora l At 7 years: stereoacuity at adult level
te ndon mus cle vorte x ve in Hyperopia (average of 2 D) increases during first year of life;
50% have > 1 D with-the-rule astigm atism ; decreases after
7 years of age

La te ra l re ctus Visio n testin g:

mus cle Infancy: optokinetic (OKN) response, forced preferential
looking (Teller acuity cards), visual evoked
response (VER)
Older children: Allen pictures, HOTV, tum bling E, Snellen
acuity

S e n s o ry t e s t in g
Bin o cu lar visio n :
Horopter: the set of object points im aged on corresponding
retinal points
Me dia l re ctus Panum’s fusional space: region around horopter in which
mus cle binocular vision exists (Fig. 5-51)
Types of binocularity:
SIMULTANEOUS PERCEPTION: ability to see two im ages,
one on each retina (superim posed, not blended)
FUSION: sim ultaneous perception of two sim ilar im ages
Infe rior te mpora l Infe rior oblique
blended as 1 (Table 5-10)
ve rte x ve in mus cle
STEREOPSIS: perception of two slightly dissim ilar im ages
Fig ure 5-49. Posterior view of the eye with Tenon’s capsule removed. (From blended as one with appreciation of depth
Campolattaro BN, Wang FM: Anatomy and physiology of the extraocular muscles STEREOACUITY: occurs when retinal disparity is too
and surrounding tissues. In YanoffM, Duker JS [eds]: Ophthalmology, ed 2, St Louis,
2004, Mosby.)
sm all to cause diplopia, but too great to allow
superim position of fusion of the two visual
directions. Norm al is 20 to 50 seconds of arc
TITMUS STEREOTEST: readily available, m onocular
clues present. Determ ines norm al retinal
correspondence (NRC) in binocular patient.
Exa m p le : Fly¼ 3000 arc seconds; animals ¼ 400, 200, 100 arc
seconds; circles ¼ 800 À 40 arc seconds
S upe rior RANDOM DOT STEREOGRAMS: m onocular clues absent
re ctus but m ore difficult for children to understand

10.6mm
horopter
region
of single
fixa tion vision
7mm 7mm
7.7mm ta rget
10.3mm 9.2mm
5.5mm 6.9mm 20 min a rc
8
min
Me dia l La te ra l a rc
re ctus re ctus Pa num fusiona l
6mm 6.5mm 8mm a rea on retina

9.8mm

Infe rior Fig ure 5-51. Panum’s fusional area. The left eye fixates a square target, and a
search object visible only to the right eye is moved before and behind this
re ctus
target. The ellipse of retinal area, for which typical dimensions are given for the
Fig ure 5-50. Spiral of Tillaux. The structure of the rectus muscle parafoveal area, is the projection of Panum’s fusional area. Diplopia is not
insertions. (From Campolattaro BN, Wang FM: Anatomy and physiology of the perceived for 2 targets within this area. (From Diamond GR: Sensory status
extraocular muscles and surrounding tissues. In Yanoff M, Duker J S [eds ]: in strabismus . In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999,
Ophthalmology, ed 2, St Louis, 2004, Mosby.) Mosby.)

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CHAPTER 5 • Pediatrics/Strabism us

Ta b le 5.10. Normal fusional amplitudes (prism diopters [Δ])

Es otropic le ft e ye with ARC a nd s uppre s s ion or with
monofixa tion s yndrome , fixing with right e ye
Te s ting Co nve rg e nc e Dive rg e nc e Ve rtic a l
Dis ta nc e (Δ) (Δ) (Δ)
Dis ta nt
6m 14 6 2.5 ta rge t
25 cm 38 16 2.6

Ne a r
ta rge t
Sen so r y p h en o m en a asso ciated with str ab ism u s:
Diplopia: sim ultaneous perception of sim ilar im ages falling Right e ye Le ft e ye P e rce ive d ima ge
on noncorresponding retinal points
Visual confusion: sim ultaneous perception of dissim ilar Es otropic right e ye with ARC a nd s uppre s s ion or with
im ages falling on corresponding retinal points monofixa tion s yndrome , fixing with le ft e ye

Dis ta nt
Sen so r y ad ap tatio n s to d ip lo p ia an d visu al ta rge t
co n fu sio n :
Suppression (scotoma): im age from one eye is inhibited or Ne a r
does not reach consciousness (Fig. 5-52) ta rge t
CENTRAL: adaptation to avoid confusion
PERIPHERAL: adaptation to avoid diplopia Right e ye Le ft e ye P e rce ive d ima ge
OBLIGATORY: present all the tim e
Fig ure 5-53. Possible Worth 4 Dot percepts in binocular patients. Note the
FACULTATIVE: present only when eyes are deviated similar distant responses in patients who have esotropia with abnormal retinal
TESTS: correspondence (ARC) and suppression and in those who have monofixation
WORTH 4 DOT: with red lens over 1 eye and green lens syndrome. Patients who have exotropia with ARC and suppression give the same
over other eye, patient views red, green, and white responses, but the suppression scotoma is larger and shaped somewhat
differently. The red lens is over the right eye, and the green lens is over the
lights. Green light is visible to eye under green lens,
left eye. (From Diamond GR: Sensory status in strabismus. In Yanoff M, Duker J S
red light visible to eye under red lens, and white [eds]: Ophthalmology, ed 2, St Louis, 2004, Mosby.)
light visible to both eyes. Determ ines binocularity
(Figs. 5-53 and 5-54). May be used to define the
size and location of a suppression scotom a in Any stra bismus in pa tient fixing right eye
patients with strabism us
Perform ed at distance (central fusion) and at near a ll ta rget
(peripheral fusion) dista nces
Results:
Suppression: two red or three green lights seen right eye left eye perceived ima ge
Fusion: four lights seen Any stra bismus in pa tient fixing left eye
Diplopia: five lights seen
4-PRISM DIOPTER BASE-OUT PRISM TEST: for sm all a ll ta rget
suppression scotom as. 4-prism diopter (Δ) prism dista nces
placed base-out over one eye; if suppression
scotom a is present, eye will not m ove right eye left eye perceived ima ge
HORROR FUSIONIS: intractable diplopia with absence of
Fig ure 5-54. Possible Worth 4 Dot responses in patients who do not have
central suppression binocularity. The red lens is over the right eye, and the green lens is over the
Abnormal retinal correspondence (ARC): (see following) left eye. (From Diamond GR: Sensory status in strabismus. In Yanoff M, Duker J S
Monofixation syndrome: binocular sensory state in patients [eds]: Ophthalmology, London, 1999, Mosby.)
with sm all angle strabism us (< 8 Δ; m icrotropia)

CHARACTERISTICS: central scotom a and peripheral

fusion present with binocular viewing
Usually esotropic (ET), m ay appear exotropic (XT) or
orthophoric
Am blyopia com m on, usually m ild
Stereoacuity reduced
Central or eccentric fixation
Typically occurs when preexisting strabism us is
controlled nonoperatively or after surgery, but m ay
occur in nonstrabism ic patients, also from m acular
lesions and anisom etropia
ET XT
Decom pensation occurs when the deviation changes
Fig ure 5-52. Suppression scotomas for esotropia (ET) and exotropia (XT). from a latent to a m anifest one

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 Anatom y and Physiology

DIAGNOSIS: 4 Δ base-out prism test (used to shift im age Accom panied by scotom a
outside scotom a to cause eye m ovem ent). In norm al No fusional am plitudes and no stereopsis
patient, when prism is placed in front of one eye, eyes Fovea in deviating eye and fovea in fixing eye do not
turn and then refixate have the sam e visual direction
In m onofixation syndrom e, when prism is placed in Fovea of fixing eye shares com m on visual direction with
front of norm al eye, there is no refixation m ovem ent, peripheral area of the nonfixing eye
and when prism is placed in front of eye with HARMONIOUS ARC: subjective angle is zero
scotom a, there is no initial eye turn UNHARMONIOUS ARC: subjective angle is greater than
zero but less than objective angle
Retin al co r r esp o n d en ce: the ability of the sensory system TESTS FOR ARC:
to appreciate the perceived direction of the fovea and other AFTERIMAGE TEST: label fovea of each eye with a
retinal elem ents in each eye relative to each other linear light afterim age (vertical im age for
Corresponding retinal points: two retinal points (one in each deviating eye and horizontal im age for fixing eye).
eye) that, when stim ulated sim ultaneously, result in the Each eye is stim ulated individually (m onocular),
subjective sensation that the stim ulating target com es then patient draws perceived afterim ages
from the sam e direction in space. If diplopia occurs, then (Fig. 5-55):
the two points are noncorresponding NRC: cross with central gap
Normal retinal correspondence: corresponding areas of the ET with ARC: afterim ages crossed
retina that have identical relationships to the fovea of XT with ARC: afterim ages uncrossed
each eye BAGOLINI LENSES: glasses have no dioptric power but
CHARACTERISTICS: occurs in straight eyes under have narrow striations running at 45° and 135°.
binocular conditions With glasses on, patient fixes on a light and draws
Occurs in eyes in which the objective and subjective angles perceived im age. Allows determ ination of
of strabismus are the same; measured with amblyoscope strabism us as well as retinal correspondence. Break
(patient superimposes dissimilar targets) in line is proportional to size of suppression
OBJECTIVE ANGLE: m easured angle scotom a (Fig. 5-56)
SUBJECTIVE ANGLE: the am ount of prism required to RED GLASS TEST: with red glass in front of deviating
superim pose the im ages or prom ote fusion eye, patient fixes on light; angular deviation can be
Other dissim ilar target tests: Lancaster red-green test and m easured with a Maddox rod to determ ine retinal
Hess screen; require NRC; used to m easure paretic correspondence
strabism us ET: uncrossed im ages
Abnormal retinal correspondence (ARC): corresponding areas
XT: crossed im ages
of the retina that have dissimilar relationships to their
Harm onious ARC: patient sees pink light
respective foveas. Sensory adaptation eliminates peripheral
AMBLYOSCOPE: device with which patient views two
diplopia and confusion by permitting fusion of similar
dissim ilar targets and attem pts to
images projecting onto noncorresponding retinal areas
superim pose them
CHARACTERISTICS: change in visual direction of retinal
points NRC: objective and subjective angles of strabism us
Manifests only during binocular viewing are equal
Objective and subjective angles are not equal Harm onious ARC: subjective angle equals zero
Sensory adaptation of im m ature visual system to (arm s of am blyoscope are parallel)
strabism us Unharm onious ARC: subjective angle is between
Prevents diplopia zero and objective angle

Te s t
Horizonta l fla s h be fore fixing e ye , ve rtica l fla s h be fore nonfixing e ye . Fla s h one e ye a t
a time . Afte r both e ye s fla s he d, a s k pa tie nt to clos e e ye s ; pos itive a fte rima ge is s e e n.
Whe n e ye s ope n, ne ga tive a fte rima ge note d.
Re s ults

Norma l re tina l corre s ponde nce Abnorma l re tina l corre s ponde nce (a s s ume right
s tra bis mus , ve rtica l fla s h be fore right e ye )

e s otropia e xotropia

Fig ure 5-55. Afterimage test percepts, central fixation. Shown are those possible in patients who have central fixation and binocular vision. (From Diamond GR: Sensory
status in strabismus. In Yanoff M, Duker J S [eds ]: Ophthalmology, ed 2, St Louis, 2004, Mosby.)

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CHAPTER 5 • Pediatrics/Strabism us

Test Ta b le 5-11. Agonists, synergists, and antagonists

Right eye lens a t 1 35 ° in tria l fra me, left eye lens a t 45 °. Fixa te on dista nt light in Ag o nis t Syne rg is ts Anta g o nis ts
semida rkened room.
Closest sensory test to norma l viewing, first to exhibit a bnorma l retina l correspondence Medial rectus (MR) SR, IR LR, SO, IO
(ARC) stra bismus, first to revert to norma l retina l correspondence (NRC) when Lateral rectus (LR) SO, IO MR, SR, IR
eyes a ligned.
Superior rectus (SR) IO, MR IR, SO
Results Inferior rectus (IR) SO, MR SR, IO

Cover–uncover No binocula rity, No binocula rity, Superior oblique (SO) IR, LR IO, SR
test irreleva nt right eye fixing left eye fixing Inferior oblique (IO) SR, LR SO, IR

No shift on NRC bifovea l NRC monofixa tion,

cover–uncover left eye fixing Mo t o r t e s t in g
testing (no tropia )
Du ctio n s: m onocular rotations of eye
Shift on ≤8D NRC monofixa tion, left eye fixing
cover–uncover >8D ARC, left eye fixing esotropia
testing (tropia ) Ver sio n s: conjugate binocular eye m ovem ents
>8D ARC, left eye fixing exotropia
Ver gen ce: disconjugate binocular eye m ovem ent

>8D NRC, NRC, exotropic Ago n ist: prim ary m uscle m oving eye in given direction
esotropic diplopia diplopia (Table 5-11)

Syn er gist: secondary m uscle that acts with agonist to m ove

Fig ure 5-56. Possible Bagolini lens percepts, central fixation. (From Diamond eye in given direction
GR: Sensory status in strabismus. In Yanoff M, Duker J S [eds]: Ophthalmology,
1999, London, Mosby.)
An tago n ist: m uscle that acts to m ove eye in opposite
direction as agonist
Am b lyo p ia
Unilateral or bilateral reduction of visual acuity that cannot be Yo ke m u scles: two m uscles, one in each eye, that act to
attributed directly to any structural abnorm ality of the eye or m ove respective eyes into a cardinal position
visual system . Due to disuse of fovea
Car d in al p o sitio n s: six positions of gaze in which one
Ch ar acter istics: prevalence 2% to 4%; preventable or m uscle of the eye is the prim e m over (Fig. 5-57)
reversible with appropriately tim ed intervention

Typ es: Mid lin e p o sitio n s: straight up and straight down from
Strabismic: crowding phenom enon (letters or sym bols are prim ary position
m ore difficult to recognize if closely surrounded by
sim ilar form s; therefore, visual acuity m ay substantially Her in g’s law (equal innervation): equal and sim ultaneous
im prove with isolate letters); neutral density filters do innervation to synergistic m uscles; am ount of innervation to
not reduce acuity as m uch in the am blyopic eye as in the eyes is determ ined by the fixating eye; therefore, the am ount of
norm al eye deviation depends on which eye is fixating:
Refractive: high am etropia (hyperopia of +5 D, m yopia of Primary deviation (paralytic strabismus): deviation
À8 D, astigm atism of 2.5 D) or anisom etropia m easured with norm al eye fixing
(1 D for hyperopia, 3 D for m yopia, 1.5 D for Secondary deviation (paralytic strabismus): deviation
astigm atism ) m easured with paretic eye fixing; larger than prim ary
Deprivation: m edia opacity, ptosis, occlusion deviation
Hering’s law also explains the term ”inhibitional paresis of
Diagn o sis: reduced visual acuity that cannot be entirely the contralateral antagonist”
explained by physical abnorm alities
Right Le ft
Tr eatm en t: treat am blyopia before strabism us surgery RS R RIO
ga ze ga ze
(elim inate any obstacle to vision, rem ove significant LIO LS R
congenital lens opacity by 2 m onths of age). Force use of
poorer eye by lim iting use of better eye (full-tim e occlusion, RLR RMR
part-tim e occlusion, optical degradation of better eye, LMR LLR
atropinization of better eye). Full-tim e occlusion for 1 week per
year of age at most prior to re-exam ination; continue until no
RIR RS O
further im provem ent. Re-evaluate until 10 years of age
LS O LIR

Pr o gn o sis: good for strabism ic, poor for deprivation Fig ure 5-57. Cardinal positions and yoke muscles.

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 Anatom y and Physiology

This is the incorrect impression that a muscle in the

norm al eye is responsible for the ocular m isalignment and Fove a
may occur in an SO palsy when the paretic eye fixes (eg, in
SO palsy, less innervation is required by the antagonist P upilla ry a xis
[IO], and therefore less innervation is directed to the yoke
[contralateral SR]). The contralateral antagonist is actually
the antagonist of the yoke of the paretic muscle
k°
Sh er r in gto n ’s law (reciprocal innervation): innervation to
ipsilateral antagonist decreases while innervation to the
agonist increases Optic a xis

Op tic/Op tical axis: extends from anterior to posterior pole

of eye through nodal point and geom etric centers of lens and Fig ure 5-58. The angle κ. This is the displacement in degrees of the pupillary axis
cornea from the visualaxis. The positive-angle κ provides the illusion of exotropia in the left
eye. (Adapted from Diamond GR: Sensory status in strabismus. In Yanoff M,
Duker J S [eds]: Ophthalmology, ed 2, St Louis, 2004, Mosby.)
Visu al axis: extends from fovea through nodal point of eye
to object of fixation Negative-angle κ: nasal position of fovea relative to
pupillary axis; causes slight nasal rotation of globe to
An gle alp h a ( α ) : angle between visual axis and optic axis as keep im age in focus; light reflex appears tem poral. May
they cross at the nodal point of the eye; m easure of tilt of eye, cause a pseudoesotropia or increase the apparent degree
approxim ately 5.2 degrees horizontally; im portant for of an ET, and lessen or m ask an XT
pseudophakic IOL centration
Eccen tr ic fixatio n : consistent use of a nonfoveal region of
An gle kap p a (κ): angle between visual axis and center of the retina for m onocular viewing in an am blyopic eye; a
pupil (Fig. 5-58); approxim ately 2.6 degrees horizontally, m onocular phenom enon
m easured as distance between center of pupil and corneal light
reflex (near visual axis); im portant for centration of corneal
refractive procedures Detectin g d eviatio n s:
Positive-angle (κ): tem poral position of fovea relative to Corneal light reflex: if equal, no m anifest deviation (tropia)
pupillary axis; causes slight tem poral rotation of globe to but m ay have latent deviation (phoria); if unequal,
keep im age in focus; light reflex appears nasal. May cause tropia is present
a pseudoexotropia or increase the apparent degree of an Monocular cover–uncover test: tests for phoria or tropia by
XT, and lessen or m ask an ET detecting m ovem ent of eyes when 1 eye is covered and
Exa m p le : ROP, Toxocara then uncovered (Fig. 5-59)

Exotropia Esotropia Hypertropia Exophoria

A B C D
Fig ure 5-59. Cover test for tropias and phorias. A, For exotropia, covering the right eye drives inward movement of the left eye to take up fixation; uncovering the right
eye shows recovery of fixation by the right eye and leftward movement of both eyes; covering the left eye discloses no shift of the preferred right eye. B, For
esotropia, covering the right eye drives outward movement of the left eye to take up fixation; uncovering the right eye s hows recovery of fixation by the right eye and
rightward movement of both eyes; covering the left eye disclos es no shift of the preferred right eye. C, For hypertropia, covering the right eye drives downward
movement of the left eye to take up fixation; uncovering the right eye shows recovery of fixation by the right eye and upward movement of both eyes; covering the
left eye shows no shift of the preferred right eye. D, For exophoria, the left eye deviates outward behind a cover and returns to primary position when the cover is removed.
An immediate inward movement denotes a phoria, a delayed inward movement denotes an intermittent exotropia. (From Diamond G, Eggers H: Strabismus and
Pediatric Ophthalmology, London, Mosby, 1993.)

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CHAPTER 5 • Pediatrics/Strabism us

Measu r in g d eviatio n s: Oth er tests:

Modified Krimsky’s method: place prism over fixating eye to Double Maddox rod test: m easures torsion
center the corneal light reflex over the pupil; place over Parks-Bielschowsky three-step test: identifies cyclovertical
deviating eye in incom itant or paralytic deviations palsy
(Fig. 5-60) 1. Which eye has hyperdeviation?
Hirschberg’s method: estim ate each m illim eter of Identify weak depressors in higher eye (IR, SO) and
decentration of corneal light reflex from the center of elevators in lower eye (IO, SR)
the pupil (1 m m equals 7° or 15 Δ of deviation) 2. Is hyperdeviation greater in right or left gaze?
(Fig. 5-61) Identify m uscles that act in direction as
Simultaneous prism cover test or cover–uncover test: m easures hyperdeviation increases
tropia Right gaze: SR and IR of right eye, IO and SO of left eye
Alternate cover testing: m easures tropia and phoria Left gaze: IO and SO of right eye, SR and IR of left eye
Measurements are affected by corrective lenses: m inus 3. Is hyperdeviation greater with right or left head tilt?
(concave lenses; m yopia) m easures m ore, and plus Identify torting m uscles that act in direction as
(convex lenses; hyperopia) m easures less, due to the hyperdeviation increases
prism atic effect of the lenses. Difference ¼ 2.5% Â D Right head tilt:
Intorsion of right eye (SR and SO)
Extorsion of left eye (IO and IR)
Left head tilt is reversed
(Rem em ber: supinate–superiors intort)

Norma l
A

15° ET
B

30° ET
C

f
f
C

Obs e rve r 45° ET

D
D
Fig ure 5-60. Modified Krimsky’s method of estimating deviation. (From von
Noorden GK: Von Noorden-Maumenee’s Atlas of Stabismus, 3rd edn, St Louis, Fig ure 5-61. Hirschberg’s method of estimating deviation. (From von Noorden
Mosby, 1977.) GK: Von Noorden-Maumenee’s Atlas ofStabismus, 3rd edn, St Louis, Mosby, 1977.)

146
 Nystagm us

1 accom m odative convergence do not dam pen nystagm us); if

head posture > 50% of tim e, surgical correction (Kestenbaum
procedure)
RS R RIO LIO LS R
3 Distin gu ish co n gen ital n ystagm u s fr o m laten t
2 3 2
n ystagm u s:
RIR RS O LS O LIR Cover one eye: latent nystagm us worsens
Reversal of normal OKN response: congenital nystagm us
Dampens with convergence: congenital nystagm us
1
Examine slow-phase velocity: increases (congenital),
Fig ure 5-62. Parks-Bielschowsky 3-step test for RSO palsy. decreases (latent)

La t e n t
Circle involved m uscles at each step described previously.
Jerk nystagm us during m onocular viewing away from
Muscle with 3 circles is the one with palsy
covered eye
Exa m p le : RSO palsy (Fig. 5-62)
Associated with congenital esotropia and DVD
Forced duction test: determ ines deviation due to m uscle
Pure form is rare
restriction
Forced generation test: m easures m uscle strength
Etio lo gy: abnorm al cortical binocularity, proprioceptive
im balance, defective egocentric localization

NYSTAGMUS Ch ar acter istics: cover one eye and uncovered eye, fast
phase to side of fixing eye develops nystagm us; long-standing;
Oscillation of eyes norm al OKN response; nulls with adduction; norm al vision
when both eyes open, vision decreases when each eye is tested
Childhood form s are m ost com m only congenital, latent, separately; m ay have strabism us, especially infantile esotropia,
sensory, and spasm us nutans DVD (50%)

Co n g e n it a l Wavefo r m : exponentially decreasing velocity of slow phase

Benign disorder of eye m ovem ent calibration system (between Alexan d er ’s r u le: intensity increases when looking toward
sensory and m otor system s) fast phase and decreases when looking toward slow phase (ie,
adduction nulls; therefore, no head posture)
Ch ar acter istics: long-standing, horizontal in all positions,
m ay have rotary com ponent, increases intensity with fixation, Tr eatm en t: surgery for strabism us or head turn
null point, no oscillopsia, m ay have head posture, dam pened
by convergence (better near than distance vision),
S e n s o ry
exponentially increasing velocity of slow phase, OKN reversal
in 60%, strabism us in 33%, sim ilar m onocular and binocular Pendular nystagm us due to visual loss; m ore com m on than
vision (20/20–20/70), absent during sleeping, latent congenital nystagm us
nystagm us can be present (cover one eye, nystagm us converts
to jerk away from covered eye), m ay develop head oscillations Etio lo gy: aniridia, albinism , rod m onochrom atism
at 3 m onths of age, wide-swinging eye m ovem ents; at 1 year, (achrom atopsia), CSNB, optic nerve colobom a, cataracts, optic
sm all pendular m ovem ents; at 2 years, jerk nystagm us with nerve hypoplasia, Leber’s congenital am aurosis, bilateral
null zone, discover convergence dam pens nystagm us and m ay m acular colobom a
develop nystagm us blockage syndrom e
Spa s m us nuta ns
Wavefo r m : exponentially increasing velocity of slow phase
Benign form of nystagm us consisting of fine, rapid, often
m onocular or m arkedly asym m etric eye m ovem ents
Asso ciatio n s: albinism , aniridia, Leber’s congenital
am aurosis, ON hypoplasia, congenital cataracts, Diagnosis of exclusion
achrom atopsia
Onset during first year of life with spontaneous resolution by
Tr eatm en t: base-out prism s in glasses dam pen age 3 years
nystagm us by forcing patient to converge (only fusional
convergence [overcom ing exophoria] dim inishes the Tr iad o f fin d in gs: eye m ovem ents, head nodding/bobbing,
nystagm us; therefore, m inus lenses that stim ulate torticollis (diagnosis can be m ade in absence of head nodding)

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CHAPTER 5 • Pediatrics/Strabism us

Wavefo r m : high frequency, low am plitude, pendular, Bo x 5-4. Classification of esotropia

asym m etric (dissociated form of nystagm us)

In fa ntile
DDx: chiasm al gliom a, subacute necrotizing
Classic congenital
encephalom yopathy
Early-onset accommodative
Duane’s syndrome type I
Neu r o im agin g: rule out tum or
Abducens paralysis from birth trauma
Nystagmus blockage syndrome
Ac q uire d Nys ta g m us Mo€bius’ syndrome

(See Chapter 4, Neuro-ophthalm ology) Ac q u ire d

Ac c om m od a t ive
Refractive

OCULAR ALIGNMENT Nonrefractive

Mixed (partially accommodative and partially basic)

Ho rizo nta l De via tio ns Decompensated

Non a c c om m o d a t ive
Stress-induced acquired
At birth, one-third of babies are orthophoric and two-thirds are
Cyclic
slightly XT
Acute comitant
Sensory deprivation
Or th o p h o r ia: norm al alignm ent; eyes are straight; no latent
or m anifest deviation Divergence insufficiency
Divergence paralysis
Spasm of near synkinetic reflex
Co m itan t: deviation is equal in all positions of gaze
Restrictive (thyroid-related, medial orbital wall fracture, overresected MR)
Lateral rectus weakness (CN 6 palsy, iatrogenic / surgical [slipped,
In co m itan t: deviation varies in different positions of gaze detached, overrecessed LR])
(paralytic or restrictive etiologies)

EXOPHORIA ( X) : latent deviation; detected by alternate

Eso d eviatio n s: latent ormanifest convergenceofthevisualaxes
cover testing; m ay be related to asthenopia
Most com m on deviation (50%–75%) INTERMITTENT EXOTROPIA ( X( T) ) : deviation that is
Types: som etim es latent and som etim es m anifest; partially
PSEUDOESOTROPIA: patient is orthophoric but has controlled by fusional m echanism s
appearance of esotropia. Due to broad nasal bridge, EXOTROPIA ( XT) : m anifest deviation
prom inent m edial epicanthal folds, or negative-angle κ BASIC: exodeviation equal at distance and near
ESOPHORIA ( E) : latent deviation controlled by fusional DIVERGENCE EXCESS: exodeviation greater at distance
m echanism s; m ay m anifest under certain conditions than near by at least 10 Δ
(fatigue, illness, stress, or tests that dissociate eyes Types:
[alternate cover test]) Sim ulated (pseudodivergence excess):
INTERMITTENT ESOTROPIA ( E( T) ) : deviation that is enhanced fusional convergence at near
som etim es latent and som etim es m anifest; partially related to accom m odation 30-m inute
controlled by fusional m echanism s patch test: binocular fusional im pulses
ESOTROPIA ( ET) : m anifest deviation (Box 5-4) suspended and exodeviation becom es
equal at distance and near. If near
Exo d eviatio n s: latent or m anifest divergence of the deviation increases to close to distance
visual axes deviation with +3.00 lenses, then high
Less com m on than ET AC/A ratio exists
Account for 25% of strabism us in children True: after 30-m inute patch test, still has
In neonates, up to 60% have a constant, transient divergence excess at distance
exodeviation CONVERGENCE INSUFFICIENCY: near exotropia is
Most com m on form is interm ittent exotropia greater than distance exotropia
Types:
Co n g e n it a l e s o t ro p ia
PSEUDOEXOTROPIA: patient is orthophoric but has
appearance of exotropia. Due to positive-angle κ 1%–2% of all strabism us
without other abnorm alities, m acular heterotopia Equal sex distribution
(tem porally dragged m acula [PHPV, ROP, Present by 6 m onths of age
toxocariasis] with large angle κ), wide interpupillary Fam ily history of strabism us is com m on
distance Increased frequency in cerebral palsy or hydrocephalus

148
 Ocular Alignm ent

Fin d in gs: deviation usually ! 30 Δ, low amounts of Associated surgery of inferior obliques if overaction
hyperopia, often cross-fixation with equal visual acuity in each present
eye, rarely develop normal binocular vision (even with surgery) Three or four m uscle surgeries for large deviation

Asso ciatio n s: DVD in 70%

Overaction of inferior obliques in 70% Ac c o m m o d a t ive e s o t ro p ia
Involved eye elevates with adduction Onset 6 m onths to 7 years
Onset usually in second year of life; greatest occurrence
between ages 3 and 7 years May be interm ittent at onset
Treat with IO weakening procedure
Often, positive fam ily history
Latent nystagmus
Congenital, conjugate, horizontal jerk nystagm us that Associated with am blyopia (generally from anisom etropia)
occurs under m onocular conditions
May be precipitated by traum a or illness
When one eye is occluded, nystagm us develops in both
eyes with fast phase toward the fixing eye and slow
phase toward the occluded eye Typ es: (Table 5-12)
MANIFEST LATENT NYSTAGMUS: nystagm us present when
both eyes are open
Because of induced nystagm us in the uncovered eye with Acco m m o d ative co n ver gen ce to acco m m o d atio n
latent nystagm us, binocular visual acuity is better ( AC/A) r atio : norm ally between 3:1 and 5:1 prism diopters
than when each eye is tested individually per diopter of accom m odation
NYSTAGMUS BLOCKAGE SYNDROME: High AC A ratio: usually present when near deviation
overaccom m odation to dam pen nystagm us results in exceeds distance deviation by > 10 to 15 Δ
ET at near (usually large). Can differentiate from Calculation (two m ethods):
essential infantile esotropia (EIE) with m anifest latent HETEROPHORIA METHOD: AC/A¼ IPD + [(N À D)/
nystagm us because nystagm us does not increase with Diopt]
occlusion of 1 eye. Nystagm us dam pens in IPD ¼ interpupillary distance (cm )
convergence or adduction and increases in prim ary or N ¼ near deviation
lateral gaze. Adduction continues even when one eye D ¼ distance deviation
is occluded, and head turn occurs toward the Diopt ¼ accom m odative dem and at fixation distance
uncovered eye when the fellow eye is occluded. With
adduction effort, the pupil constricts, dem onstrating
that accom m odative m echanism s are present. May Ta b le 5-12. Types of accommodative esotropia
treat with large (6 m m +) bim edial resections using a
Faden suture Typ e Cha ra c te ris tic s Tre a tm e nt
Asym m etry of the m onocular, optokinetic m otion– Refractive Esotropia at distance Full cycloplegic refraction
processing response accommodative and near within 10 Δ
Nasal to tem poral sm ooth pursuit less well developed (normal AC / A
ratio)
than tem poral to nasal pursuit
High hyperopia (range Treat amblyopia
Can establish congenital nature of ET in an older patient
+3.00 to +10.00,
Asym m etry also seen in healthy newborns and average ¼ + 4.00)
disappears by 6 m onths of age Nonrefractive Lower amount of Consider bifocal when
accommodative hyperopia (average ¼ esotropia at near exceeds
Diagn o sis: dem onstrate potential for full abduction with (high AC / A ratio) + 2.25) distance by 10 Δ
vestibular ocular reflex and doll’s head m aneuver to rule out (executive type)
congenital CN 6 palsy Esotropia greater at Consider miotics
Look for synkinetic lid or eye m ovem ents with attem pted near than at distance (phospholine iodide
(may resolve with age) 0.125%)
horizontal gaze to rule out Duane’s syndrom e
Esotropia reduced at Surgery if needed for
Rule out accom m odative com ponent with glasses if older
near with plus lens residual esotropia
than age 1 year or phospholine iodide 0.125%
Mixed Partially Full cycloplegic refraction
mechanism accommodative
Tr eatm en t: Angle of deviation Surgery for
Nonsurgical: correct am blyopia before surgery; cross reduced but not nonaccommodative
fixation suggests equal visual acuity of both eyes eliminated with component
Early surgery (as early as 6 m onths): potential for sensory spectacles
binocular fusion; aim for alignm ent within 10 Δ of Decompensate d Residual esotropia Full cycloplegic refraction
orthophoria accommodative after correction with full
cycloplegic refraction
PROCEDURES:
Occurs more Surgery
Bilateral m edial rectus recession commonly with high
Recess m edial rectus m uscle and resect lateral rectus AC / A ratio
m uscle of 1 eye

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CHAPTER 5 • Pediatrics/Strabism us

Exa m p le : near deviation ¼ 35 Δ; distance deviation ¼10 Δ; accommodative is norm al; large difference between m anifest and
demand ¼ 20 cm (5 D); IPD¼ 60 mm (6 cm). Therefore, AC / A¼ 6 cycloplegic refraction
+ [(35 À 10)/5]¼ 11 : 1
Treatment: cycloplegia m ay break spasm ; on
LENS GRADIENT METHOD: AC / A¼ (WLÀ NL)/D
postcycloplegic refraction push plus
WL¼ deviation with lens in front of eye
NL¼ deviation without lens in front of eye
D ¼ dioptric power of lens used In co m itan t ET:
Exa m p le : near deviation with a +1.00 lens is 50 Δ and without a lens is 35 Δ. Etiology: MR restriction: thyroid, orbital wall fractures, or
Therefore, AC/A¼ (50 À 35)/1.00 ¼ 15:1
excessively resected MR m uscle
Slipped m uscle following strabism us surgery
Natu r al h isto r y: hyperopia m ay decrease with age, AC/A Neurogenic: CN 6 palsy (spontaneous or due to
ratio m ay norm alize, and fusional divergence can im prove; intracranial lesions [33%], infections, or birth
m ay begin to reduce strength of glasses and bifocals slowly as traum a)
child gets older Myasthenia gravis

Tr eatm en t: correct refractive error and treat am blyopia; Co n secu tive eso tr o p ia: follows surgery for exotropia; rule
consider surgery for residual com ponent out slipped m uscle
Satisfactory: residual esotropia < 10 Δ; peripheral fusion
and expansion of fusional am plitudes possible
Unsatisfactory: try atropine with glasses or long-acting Pr ism ad ap tatio n test: prisms are used to help patients
cholinesterase inhibitors; recheck refraction for increased obtain binocular fusion while awaiting surgical alignment of the
or latent hyperopia eyes. Used to preoperatively predict which patients will develop
Echothiophate (phospholine iodide) causes residual esotropia after surgery. Patients who respond with
accom modation that rem oves convergence with increasing deviation are given stronger prism until orthophoria is
accom modative effort obtained. The largest angle is the target for surgical correction

No n a c c o m m o d a t ive a c q u ire d e s o t ro p ia In t e rm it t e n t e xo t ro p ia
Str ess-in d u ced acqu ir ed ET: breakdown of fusional Most com m on form of XT
divergence that occurs after illness, em otional traum a, or Onset varies from infancy to age 4 years
injury; requires surgery May be progressive
Often have reflex closure of one eye in bright light
Cyclic ET: interm ittent, usually present every other day Suppression only when eyes are deviated (facultative
(48-hour cycle) and becom es constant with tim e suppression)
Findings: V-pattern ET is com m on; am blyopia can develop; Am blyopia is uncom m on
on days when ET is not present, norm al binocular visual
acuity with good stereovision Natu r al h isto r y:
Treatment: prescribe full hyperopic correction; m ay also Phase 1: X(T) at distance and straight at near
require surgery if glasses do not fully correct deviation; Present when fatigued
phenobarbital and am phetam ines can alter the May see double
frequency of the cycles Most m aintain excellent stereovision
Phase 2: X(T) becom es m ore constant at distance with X(T)
Sen so r y ET: due to loss of vision (sensory deprivation) in at near
one or both eyes, usually during childhood (age < 6 years); Suppression increases
identify and treat obstruction to vision Phase 3: XT at distance and near
Often no diplopia because of suppression
Diver gen ce in su fficien cy: ETgreater at distance than near; Most com m on cause of a constant XT
fusional divergence is reduced
Treat with base-out prism s (to correct diplopia) or surgery Tr eatm en t: treat am blyopia, alternate occlusion therapy,
Rule out divergence paralysis (associated with pontine induce accom m odative convergence by prescribing overm inus
tum ors, head traum a, or other neurologic abnorm alities; spectacles (also for consecutive exotropia), prism therapy with
m ay m im ic bilateral CN 6 paralysis) base-in prism s, fusional convergence training (progressive
base-out prism to induce convergence)
Sp asm o f n ear syn kin etic r eflex ( ciliar y Surgery for increased tropic phase, poor recovery of fusion
sp asm ) : interm ittent episodes of sustained convergence with once tropic, increasing ease of dissociation, XT greater
accom m odative spasm and m iosis than 50% of tim e at hom e
Findings: headache, blurred distance visual acuity (recent PROCEDURES:
onset of m yopia is present in history [pseudom yopia]), Bilateral lateral rectus recession
abnorm ally close near point, and fluctuating visual Recess lateral rectus and resect m edial rectus of 1 eye
acuity; variable angle of deviation; m onocular abduction Three of four m uscle surgeries for large deviation

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 Ocular Alignm ent

Co n g e n it a l e xo t ro p ia
Ve rtic a l De via tio ns
Rare
May be prim ary (otherwise healthy patients) or secondary Dis s o c ia t e d ve rt ic a l d e via t io n (DVD; d is s o c ia t e d
(from ocular or system ic abnorm alities) h o rizo n t a l d e via t io n [DHD]; d is s o c ia t e d
Usually large angle of deviation (average > 35 Δ) t o rs t io n a l d e via t io n [DTD])

Am blyopia is uncom m on; sim ilar refractive error to general Interm ittent deviation of nonfixing eye consisting of upward
population excursion, extorsion, excyclotorsion, abduction, or lateral
deviation
Most resolve by age 6 m onths; if not, consider surgery
Exact etiology unknown but associated with early disruption of
binocular developm ent
Asso ciatio n s: DVD and oblique m uscle overaction; orbital
or skull defects, neurologic disease, or other ocular, genetic, or Usually asym ptom atic because of poor fusion and
system ic conditions suppression
Often asym m etric
Co n ve rg e n c e in s u ffic ie n c y
Does not obey Hering’s law (fellow eye does not exhibit
Exophoria greater at near than at distance (not exotropic at near) refixation m ovem ent in opposite direction)
Rem ote near point of convergence and reduced fusional Occurs with eye occlusion or visual inattention
convergence am plitudes at near
May be latent or m anifest
Fem ale > m ale
Usually presents before 12 m onths of age
Com m on in teenagers and young adults; incidence increases
with age; prevalence 2-8% Usually m ore m arked when patient is fatigued, daydream ing,
under stress, or sick
Asso ciatio n s: can be exacerbated by fatigue, drugs, uveitis,
or Adie’s tonic pupil; m ay also follow head traum a. Also Asso ciatio n s: nystagm us (latent or m anifest latent), inferior
associated with system ic illnesses, or as a conversion reaction oblique overaction, often occurs in patients with history of
congenital ET; isolated in 40% of patients
Fin d in gs: asthenopia, diplopia; reduced fusional
convergence am plitudes and rem ote near point of
convergence, m ay have exophoria at near
Fin d in gs: slow m ovem ent of eye in the characteristic
direction, am ount of deviation variable (accurate
m easurem ents difficult to obtain), dissociated deviation occurs
Tr eatm en t: observation; orthoptic exercises to im prove
in all directions of gaze
fusional am plitudes, base-out prism s; rarely surgery (m edial
rectus resection)
Diagn o sis:
Co n ve rg e n c e p a ra lys is s e c o n d a ry t o in t ra c ra n ia l Bielschowsky’s phenomenon: occurs in 50% of patients
le s io n with DVD
Elevated eye will drift downward when light in fixing eye
Norm al adduction and accom m odation is reduced; conversely, increased illum ination in an
XT and diplopia on attem pted near fixation eye with DVD will cause it to drift up
Red lens phenomenon: place red lens over either eye while
patient fixates on light source
Asso ciatio n s: Parinaud’s syndrom e
Red im age is always seen below the white im age
In patients with a true hypertropia, the red im age is seen
Tr eatm en t: base-in prism s or occlusion of 1 eye to relieve above or below the prim ary im age, depending on
diplopia whether the red filter is placed in front of the
hyperdeviated or hypodeviated eye
S e n s o ry e xo t ro p ia
Due to loss of vision or long-standing poor vision in 1 eye Tr eatm en t:
Children age < 6 years with unilateral vision loss; may develop ET Increase fusional mechanisms: give optim al spectacle
or XT; adults usually develop XT correction; switch fixation to nonpreferred eye
Angle of deviation m ay be variable and usually increases Surgery:
with tim e INDICATIONS: increasing size or frequency of m anifest
DVD, abnorm al head position
PROCEDURES: SR recession, IR resection, IO weakening
Co n s e c u t ive e xo t ro p ia
or anterior transposition (in patients with IOOA
Follows previous strabism us surgery for esotropia and DVD)

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CHAPTER 5 • Pediatrics/Strabism us

CAUTIONS: perform bilateral surgery (unilateral surgery Co n gen ital (75%): large fusional am plitudes (as m uch as
often reveals occult contralateral DVD); often recurs 15 Δ or m ore)
or persists after surgery SO tendon often long or floppy; som etim es absent
Long-standing CN 4 palsy results in ocular torticollis, which
In fe rio r o b liq u e o ve ra c t io n (IOOA) m ay lead to facial asym m etry
Exam ine old photographs to determ ine duration of head tilt
Occurs in two-thirds of patients with congenital ET Can m im ic a double elevator palsy (if fixation preference is
Usually bilateral and asym m etric for the affected eye, the contralateral SR m uscle can
appear to underact [inhibitional palsy of the
Early surgery for ET is im portant for developm ent of binocular contralateral antagonist] and the contralateral IR can
vision but does not reduce incidence of IO dysfunction undergo contracture, leading to double elevator palsy)
May be prim ary (due to paralysis of the antagonist [SO]) or
secondary Acqu ir ed : often due to traum a (long course of fourth nerve
m akes it especially vulnerable)
Fin d in gs: when fixing eye is abducted, adducting eye is
elevated; when fixing eye is adducted, abducted eye is Fin d in gs: diplopia (vertical, horizontal, or torsional). In
depressed; V-pattern deviation; prim ary position extorsion of long-standing cases of SO palsy, com itance develops and the
fundus on indirect ophthalm oscopy deviation becom es m ore difficult to localize (hypertropia can
be present in all fields of gaze)

Tr eatm en t: IO weakening procedure (recession, m yectom y,

Bilater al: objective torsion is often > 10° of excyclotorsion
anteriorization)
(m easure with double Maddox rod), often have inferior
oblique overaction with V-pattern ET and chin-down head
In fe rio r o b liq u e p a ls y posture, alternate hypertropia with head tilt
Rare
Diagn o sis: Parks-Bielschowsky three-step test (i.e., right
Usually idiopathic but m ay follow orbital traum a, viral illness, superior oblique palsy produces right hypertropia in prim ary
or other neurologic problem s gaze, worsens in left gaze and in right head tilt)
May be bilateral
Tr eatm en t: surgery
Indications: significant head tilt, large hypertropia in
Fin d in gs: eye is hypotropic when fixing with norm al eye, prim ary gaze, diplopia
hypertropia of norm al eye when fixing with involved eye,
Procedures:
deviation worsens with gaze into field of action of involved
If deviation in prim ary gaze is 15 Δ, operate on 1
inferior oblique, deviation is worse with head tilt opposite of
m uscle; if deviation > 15 Δ, operate on 2 or m ore m uscles
paretic eye, poor elevation in adduction, norm al forced
With IO overaction, weaken ipsilateral IO (corrects
duction (distinguishes from Brown’s syndrom e), A pattern
approxim ately 15 Δ of vertical deviation)
If hyperdeviation is > 15 Δ in prim ary gaze, strengthen
Tr eatm en t: ipsilateral IR or weaken contralateral SR
With associated SO overaction: SO weakening procedure. With no inferior oblique overaction, consider weakening
If hyperdeviation develops in downgaze, then weaken ipsilateral SR or recessing contralateral IR; SO tuck
contralateral IR m ay also be considered if there is laxity of the tendon
IO palsy with hypotropia in primary gaze and no SO Knapp classification for treatm ent of superior oblique
overaction: IR recession (2.5 Δ per m m of recession) palsy offers guidance
General principle is to m atch the fields of greatest
deviation to the m uscles that work m ost in those
S u p e rio r o b liq u e o ve ra c t io n
fields to guide surgical approach
Less com m on than IO overaction or DVD Check traction test in operating room to assess laxity of
superior oblique tendon
Torsional symptoms: m ost people can tolerate 7° of torsion
Fin d in gs: a pattern; associated with depression on
Consider Harada-Ito procedure: lateral transposition of
attem pted adduction if fixing with uninvolved eye; m ay have
anterior portion of SO tendon; corrects excyclotorsion
associated horizontal deviation
only; no effect on vertical deviation or fusion

Tr eatm en t: weaken overacting SO with tenotom y In fe rio r re c t u s p a ls y

(uncontrolled) or silicone spacer (controlled)
Due to traum a to CN 3 or IR
S u p e rio r o b liq u e p a ls y May occur at tim e of injury or repair of an orbital fracture
Most com m on isolated cyclovertical m uscle palsy May resolve with tim e

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 Ocular Alignm ent

Do u b le e le va t o r p a ls y (Mo n o c u la r e le va t io n Tr eatm en t:
d e fic ie n c y) Observation: spontaneous im provem ent m ay occur
Surgery:
Sporadic, unilateral defect of upgaze associated with ipsilateral INDICATIONS: abnorm al head position, large hypotropia
ptosis in prim ary position, constant deviation causing
There are two elevators of the eye: the SR (provides m ost of am blyopia and threatening binocularity
elevation) and the IO PROCEDURE: superior weakening procedure (SO
tenectom y, tenotom y, or silicone spacer)
May be supranuclear (m ay not involve both elevators) COMPLICATION: SO palsy
May be congenital or acquired (cerebrovascular disease, tum or,
or infection) A a n d V p a t t e rn s
Change in horizontal deviation as eyes m ove between upgaze
Typ es: and downgaze
IR restriction: unilateral fibrosis syndrom e
Up to 50% of all strabism us has an associated A or V pattern
Positive forced ductions to elevation
Positive force generation (no m uscle paralysis) May present as com pensatory head posture (chin-up or chin-
Norm al saccades of SR down) in child with binocular function
Elevator weakness: (SR and IO)
Free forced ductions to elevation A p atter n : increasing convergence or decreasing divergence
Reduced force generation (m uscle paralysis) in upgaze, increasing divergence in downgaze
Reduced velocities of upgaze m ovem ents
Combination: IR restriction plus weak elevators Clinically significant when eyes diverge > 10 Δ from upgaze to
Positive forced ductions to elevation downgaze
Reduced force generation (m uscle paralysis)
Reduced velocities of upgaze m ovem ents V p atter n : increasing convergence or decreasing divergence
in downgaze, increasing divergence in upgaze
Fin d in gs: unilateral lim itation of upgaze above m idline Clinically significant when eyes converge > 15 Δ from upgaze
with accom panying ptosis in both adduction and abduction, to downgaze
variable head position (norm al or chin-up), hypotropia
increases on upgaze, fixing with involved eye causes large Etio lo gy: oblique m uscle dysfunction (obliques are
secondary hypotropia in nonparetic eye abductors, IO overaction creates a V pattern), horizontal rectus
m uscle dysfunction, vertical rectus m uscle dysfunction,
Tr eatm en t: surgery structural factors (e.g., craniosynostosis is associated with a V
Indications: chin-up head position, large vertical deviation pattern, abnorm al insertions of rectus m uscles, absence of SO
in prim ary position, poor fusion in prim ary position tendon)
Procedures:
DOUBLE ELEVATOR PALSY WITH TIGHT IR: IR recession Diagn o sis: m easure deviation in prim ary position, then
DOUBLE ELEVATOR PALSY WITH SR WEAKNESS: Knapp with eyes directed 25° in upgaze and downgaze
procedure (elevation and transposition of MR and LR
to the side of SR) Tr eatm en t: surgery
DOUBLE ELEVATOR PALSY WITH PTOSIS: correct Indications: abnorm al head position; to im prove m otor
strabism us then residual ptosis alignm ent
Procedures:
Bro w n ’s s yn d ro m e (S O t e n d o n s h e a t h s yn d ro m e ) If no oblique m uscle overaction is present, vertical
transposition of horizontal m uscles (Fig. 5-63)
Inability to elevate the eye in adduction, both actively and Corrects about 15 Δ of the A and V patterns, m uscles are
passively on forced duction testing transposed one half to 1 tendon width up or down,
May be congenital or acquired (traum atic, inflam m atory, transposition is in the direction of the desired
iatrogenic [following SO tuck, glaucom a drainage im plant, weakening
scleral buckle])
LR LR MR MR
Fin d in gs: lim itation of elevation in adduction, Less
elevation deficiency in m idline, Minim al or no elevation
deficiency in abduction, V-pattern divergence in upgaze,
Restricted forced ductions, Minim al or no superior oblique
overaction, “Clicking” m ay occur and suggests a trochlear
MR MR LR LR
problem , Anom alous head posture or hypotropia in prim ary
position m ay be present Fig ure 5-63. Direction of muscle transposition for A and V patterns.

153
CHAPTER 5 • Pediatrics/Strabism us

Mnem onic: MALE (Medial rectus to Apex of pattern; M o€ b iu s ’ s yn d ro m e

Lateral rectus to the Em pty space)
CN 6 and 7 palsies
If oblique m uscle overaction present, oblique m uscle
weakening Due to aplasia of involved brain stem nuclei
IO WEAKENING: corrects ! 15 Δ of eso shift in upgaze,
with no effect on horizontal alignm ent in
prim ary gaze Asso ciatio n s: congenital facial diplegia (m ask facies);
BILATERAL SO TENOTOMIES: corrects up to 30 to 40 Δ com bined CN 6, 7, 9, and 12 palsies; gaze palsy (PPRF
of eso shift in downgaze; torsional sym ptom s m ay involvem ent); chest, lim b, tongue defects
occur in patients with good fusion
Fin d in gs: esotropia with lim itation of abduction, exposure
keratitis from poor lid closure
SP ECIAL FORMS OF STRABISMUS
Co n g e n it a l fib ro s is s yn d ro m e
Du a n e ’s re t ra c t io n s yn d ro m e
Group of congenital anom alies characterized by variable
Usually sporadic, m ay be inherited am ounts of restriction of extraocular m uscles and replacem ent
of the m uscles with fibrous tissue
Cocontraction of m edial and lateral rectus m uscles causes
retraction of the globe with secondary narrowing of the Nonprogressive
palpebral fissure
One or m ore m uscles m ay be involved
Vertical deviations m ay be present with characteristic upshoot
Positive forced duction testing
or downshoot (leash phenom enon)
Bilateral in 15%–20%
Typ es:
Head turn com m on (for fusion) Generalized fibrosis:
Most severe
Etio lo gy: abnorm al innervation of lateral rectus by a branch Usually autosom al dom inant, m ay be recessive
of CN 3; electrom yography shows decreased firing of lateral All extraocular m uscles involved, including levator (with
rectus during abduction and paradoxical innervation of the ptosis)
lateral rectus during adduction Congenital fibrosis of inferior rectus:
Exact etiology unclear, proposed m echanism s include Sporadic or fam ilial
hypoplasia of sixth nerve nucleus, m idbrain pathology, Levator m ay be involved
fibrosis of lateral rectus Strabismus fixus:
Severe esotropia
Horizontal rectus m uscles involved (usually MR,
Typ es:
occasionally LR)
Type 1: (m ost com m on): lim itation of abduction, retraction
Vertical retraction syndrome:
and narrowing of palpebral fissures in adduction;
SR fibrosis (inability to depress eye)
appears esotropic
Congenital unilateral fibrosis:
Type 2: lim itation of adduction; appears exotropic
Enophthalm os and ptosis
Type 3: lim itation of abduction and adduction
Fibrosis of all m uscles, including levator

Asso ciatio n s: deafness, crocodile tears, syndrom es Ch ro n ic p ro g re s s ive e xt e rn a l

(Goldenhar’s syndrom e, Klippel-Feil syndrom e, Wildervanck’s
syndrom e [Duane’s syndrom e associated with Klippel-Feil],
o p h t h a lm o p le g ia (CP EO)
thalidom ide toxicity, fetal alcohol syndrom e, cat-eye Sporadic or m aternal inheritance (m itochondrial DNA)
syndrom e)
May present at any age
Tr eatm en t: correct any refractive error, treat am blyopia
Surgery Asso ciatio n s:
INDICATIONS: abnorm al head position or deviation in Kearns-Sayre syndrome: triad of CPEO, retinal pigm entary
prim ary gaze changes, and cardiac conduction defects
PROCEDURES: for esotropia: recession of m edial rectus Bassen-Kornzweig syndrome (Abetalipoproteinemia): retinal
m uscle pigm entary changes sim ilar to RP, diarrhea, ataxia, and
For globe retraction: sim ultaneous lateral rectus other neurologic signs
recession Refsum’s disease: RP-like syndrom e with elevated phytanic
For upshoot or downshoot: splitting of lateral rectus or acid levels
Faden procedure Oculopharyngeal dystrophy (AR and AD): difficulty
Avoid m uscle resection (increases globe retraction, swallowing atrophy of tongue, and proxim al m uscle
upshoots and downshoots) (hips and upper legs) weakness

154
 Strabism us Surgery

Fin d in gs: severe ptosis with com plete ophthalm oplegia, Limbal: conjunctival/Tenon’s flap at lim bus
absent Bell’s phenom enon, no restrictions on forced ductions, Swan: over the m uscle (causes m ore scarring)
large angle strabism us (often exotropia)

We a ke n in g p ro c e d u re s
Orb it a l flo o r fra c t u re
Recessio n : m ost com m on technique used to weaken rectus
Fin d in gs: ecchymosis, diplopia in some or all positions of
m uscles by m oving m uscle posteriorly; also used to weaken
gaze im mediately after injury, paresthesia or hypesthesia in
inferior oblique
distribution of infraorbital nerve, entrapment of inferior rectus
muscle or inferior oblique m uscle; m ay have associated ocular
dam age, m ay have associated wall fractures (e.g., medial wall Myo to m y (cutting across m uscle) o r m yecto m y (excising a
with medial rectus entrapment) portion of m uscle): used to weaken inferior oblique

Diagn o sis: forced duction testing Den er vatio n an d extir p atio n : used to weaken inferior
orbital im aging oblique by ablating all m uscle within Tenon’s capsule

Tr eatm en t: observation (usually 5 to 10 days until edem a Ten o to m y (cutting across tendon) o r ten ecto m y (excising
and hem atom a resolve, then reevaluate), surgery
a portion of tendon): m ost com m only used to weaken
superior oblique
Fo llo w in g s u rg e ry
Com m only after cataract, scleral buckle, and glaucom a Po ster io r fixatio n su tu r e ( Fad en p r o ced u r e) : suture
drainage im plant procedures placed 11–18 m m from insertion through belly of m uscle and
sclera to weaken m uscle only in its field of action; decreases
May be transient and resolve spontaneously m echanical advantage of m uscle acting on globe; often
Horizontal, vertical, or torsional com bined with recession

Etio lo gy:
Mechanical: adhesions, m ass effect (im plant or buckle) S t re n g t h e n in g p r o c e d u re s
Motor: traum a, ischem ia, slipped or disinserted m uscle, Resectio n : used to strengthen rectus m uscles by
toxicity (local anesthetic; m ost com m only IR fibrosis excising portion of m uscle and reattaching m uscle at its
with hypotropia in prim ary gaze that worsens in insertion site
abduction; positive forced ductions)
Sensory: fusion breakdown, anisom etropia, aniseikonia,
im age distortion Ad van cem en t (m oving m uscle forward): often used
for m uscles previously recessed, also in Harada-Ito
procedure
Tr eatm en t: observation, prism spectacles, surgery

Tu ck: technique used to strengthen superior oblique by

Ot h e r fo rm s o f s t ra b is m u s shortening the tendon (m ay produce iatrogenic Brown’s
Myasthenia gravis, thyroid-related ophthalm opathy, CN 3 syndrom e)
palsy, skew deviation

Ot h e r t e c h n iq u e s
STRABISMUS SURGERY Ad ju stab le su tu r e: slipknot or noose suture to enable
m uscle adjustm ent postoperatively under topical anesthesia
In d icatio n s: establish binocularity, im prove fusion,
im prove sym ptom s or appearance Tr an sp o sitio n : m oving m uscle out of original plane of
action; used in cases of paralysis, double elevator palsy, or A
An esth esia: and V patterns
Topical: m ay consider for recession procedures
Retrobulbar: use shorter-acting anesthetic for adjustable
sutures Har ad a-Ito p r o ced u r e: anterior tem poral displacem ent of
General: for children; consider in adults undergoing anterior half of superior oblique tendon; used to correct
bilateral surgery excyclotorsion

In cisio n s: Kesten b au m p r o ced u r e: bilateral resection / recession to

Fornix: in superior or inferior cul-de-sac on bulbar dam pen nystagm us in patients with nystagm us and head turn;
conjunctiva eyes are surgically m oved toward direction of head turn

155
CHAPTER 5 • Pediatrics/Strabism us

Bo tu lin u m to xin ( Bo to x) : interferes with release of noninflamed, translucent mass under conjunctiva. May
acetylcholine to paralyze m uscle into which it is injected. When resolve spontaneously or require excision if symptomatic
an extraocular m uscle is paralyzed by Botox, the antagonist
contracts to change the alignm ent of the eyes Co n ju n ctival scar r in g: can occur with orbital fat
Used in cases of paralytic strabism us to prevent contracture of encroaching under bulbar conjunctiva because of inadvertent
m uscles or postoperative residual strabism us, and when openings through Tenon’s capsule
surgery is inappropriate
Fat ad h er en ce syn d r o m e: violation of Tenon’s capsule
with prolapsed orbital fat into sub-Tenon’s space can lead to
Ge n e ra l p rin c ip le s fibrofatty scar. May have positive traction test and restricted
m otility
Need les: use spatulated needles (cutting surface on the side)
to decrease risk of perforation (sclera is thinnest just posterior
to insertion of rectus m uscles [0.3 m m ]) Delle: thin, dehydrated area of cornea caused by elevated
lim bal conjunctiva that prevents adequate corneal lubrication
during blinking
Ver tical d eviatio n s: in general, surgery should be
perform ed on m uscles whose field of action is in sam e field as
the vertical deviation (e.g., left hypertropia that is greatest An ter io r segm en t isch em ia: m ost blood to anterior
down and to patient’s right m ight be addressed by either segm ent supplied by anterior ciliary arteries that travel in rectus
weakening right inferior rectus or strengthening left superior m uscles. Usually occurs with surgery on 3 or m ore m uscles.
oblique) Corneal edem a and anterior uveitis are present. Treat with
topical steroids
Ho r izo n tal in co m itan cy: can be treated by adjusting
am ount of surgery perform ed on each m uscle (i.e., if exotropia Dip lo p ia
increases from right gaze to prim ary gaze to left gaze,
consider larger recession on left lateral rectus to have a Ch an ge in eyelid p o sitio n : m ost com m only with surgery
greater effect on exotropia to left [in field of action of that of vertical rectus m uscles
m uscle])
Lo st o r slip p ed m u scle: when only the capsule is sutured
Distan ce–n ear d isp ar ity: perform surgery on lateral to insertion site, m uscle can slip back; prevented with locking
rectus m uscles if exodeviation is greater at distance than at near. bites during suturing
Perform surgery on m edial rectus m uscles if esodeviation is
greater at near than at distance
Ocu lo car d iac r eflex: slowing of heart rate with traction on
extraocular m uscles

Co m p lic a t io n s o f s t ra b is m u s s u rg e ry
Malign an t h yp er th er m ia: acute m etabolic disorder that
Resid u al str ab ism u s (m ost com m on): alignm ent in m ay be fatal if diagnosis or treatm ent is delayed. Triggered by
postoperative period can change owing to poor fusion, inhalation agents and succinylcholine. Signs include
poor vision, altered accom m odation, and contracture of scar tachycardia, unstable BP, arrhythm ias, increased tem perature,
tissue m uscle rigidity, cyanosis, dark urine

Scler al p er fo r atio n : usually creates only a chorioretinal

scar but can also lead to vitreous hem orrhage, retinal tear, REVIEW QUESTIONS (Answers start on page 379)
retinal detachm ent, or endophthalm itis. If suspected during
surgery, perform indirect ophthalm oscopy to determ ine 1. The approxim ate age of onset for accom m odative ET is
whether a perforation is present; consider retinal laser closest to
treatm ent, cryotherapy, or observation a. 1 year old
b. 3 years old
In fectio n : uncom m on; includes endophthalm itis, cellulitis, c. 5 years old
and subconjunctival abscess d. 7 years old
2. A 15-year-old girl with strabism us is exam ined,
and the following m easurem ents are recorded:
Fo r eign b o d y gr an u lo m a: m ay develop weeks after
distance deviation of 10 Δ, near deviation of 35 Δ at
surgery, often at suture site. Localized, elevated, slightly
20 cm , and interpupillary distance of 60 m m . Her AC / A
hyperem ic, tender m ass, usually < 1 cm ; m ay require surgical
ratio is
excision
a. 11 :1
b. 10 :1
Co n ju n ctival in clu sio n cyst: may occur when conjunctiva c. 5 :1
is buried during closure of incision. Appears days to weeks later as d. 3 :1

156
 Review Questions

3. Duane’s syndrom e is thought to be due to a c. posterior zone 2

developm ental abnorm ality of the d. zone 3
a. trochlear nucleus 14. The least com m on finding of congenital ET is
b. m otor endplate a. dissociated vertical deviation
c. oculom otor nucleus b. cross fixation
d. abducens nucleus d. am blyopia
4. The m ost helpful test in a patient with aniridia is d. inferior oblique overaction
a. EKG 15. The contalateral antagonist of the right superior rectus
b. abdom inal ultrasound a. passes under another m uscle
c. chest X-ray b. causes incyclotorsion when paretic
d. CBC c. adducts the eye
5. The best test for an infant with a norm al fundus and d. is innervated by the inferior division of CN 3
searching eye m ovem ents is 16. With respect to Panum ’s area, physiologic diplopia occurs
a. VER at what point?
b. ERG a. on the horopter
c. EOG b. within Panum ’s area
d. OKN c. in front of Panum ’s area
6. The m ost com m on congenital infection is d. none of the above
a. toxoplasm osis 17. The best treatm ent of an A-pattern ET with m uscle
b. HSV transposition is
c. CMV a. MR resection with upward transposition
d. rubella b. LR resection with upward transposition
7. ARC is m ost likely to develop in a child with c. MR recession with downward transposition
a. congenital esotropia d. LR resection with downward transposition
b. am blyopia and suppression 18. A superior rectus Faden suture is used for the treatm ent of
c. alternating exotropia which condition?
d. sm all-angle esotropia a. Duane’s syndrom e
8. Which of the following m ost accurately reflects what a b. dissociated vertical deviation
patient with harm onious ARC reports when the angle of c. Brown’s syndrom e
anom aly is equal to the objective angle? d. double elevator palsy
a. fusion 19. Which m edication should be adm inistered
b. crossed diplopia to a child who develops trism us under general
c. sim ultaneous m acular perception anesthesia?
d. uncrossed diplopia a. atropine
9. The inferior oblique m uscle is weakened m ost by which b. edrophonium chloride (Tensilon)
procedure? c. lidocaine
a. disinsertion d. dantrolene
b. recession 20. Congenital superior oblique palsy is characterized by all of
c. m yotom y the following except
d. anteriorization a. excyclotorsion < 10°
10. The test that gives the best dissociation is b. head tilt
a. Maddox rod c. facial asym m etry
b. Worth 4 Dot d. < 10 D of vertical vergence am plitudes
c. Bagolini glasses 21. Which of the following statem ents regarding
d. Red glass m onofixation syndrom e is false?
11. The 3-step test shows a left hypertropia in prim ary position a. th ere is n o d ip lo p ia with th e 4 Δ b ase-o u t
that worsens on right gaze and with left head tilt. The best p rism test
surgical procedure is b. fusion with the Worth 4 Dot test is absent at
a. RIR resection distance
b. RSO tuck c. fusional vergence am plitudes are absent
c. LIR recession d. titm us test for fly is norm al
d. LIO weakening 22. Iridocyclitis is m ost com m only associated with which
12. In the treatm ent of a superior oblique palsy, Knapp form of JRA?
recom m ended all of the following except a. Still’s disease
a. SO plication b. polyarticular
b. recession of the contralateral IR c. spondyloarthropathy
c. IO weakening d. pauciarticular
d. resection of the contralateral SR 23. Congenital rubella is m ost com m only associated with
13. The best results of cryotherapy for ROP occur for treatm ent a. retinal pigm ent epitheliopathy
of disease in which location? b. cataracts
a. zone 1 c. glaucom a
b. anterior zone 2 d. strabism us

157
CHAPTER 5 • Pediatrics/Strabism us

24. The m ost com m on cause of proptosis in a child is c. optic nerve gliom a
a. idiopathic orbital inflam m ation d. lym phosarcom a
b. orbital cellulitis 35. Retinitis pigm entosa and deafness occur in all of the
c. cavernous hem angiom a following disorders except
d. Graves’ disease a. Usher’s syndrom e
25. Which form of rhabdomyosarcoma has the worst prognosis? b. Alstrom ’s syndrom e
a. botryoid c. Refsum ’s disease
b. pleom orphic d. Cockayne’s syndrom e
c. alveolar 36. α-galactosidase A deficiency is associated with
d. em bryonal a. cornea verticillata
26. Which of the following conditions is the least com m on b. corneal clouding
cause of childhood proptosis? c. corneal vascularization
a. cavernous hem angiom a d. no corneal changes
b. rhabdom yosarcom a 37. Congenital cataracts and glaucom a m ay occur in all of the
c. lym phangiom a following disorders except
d. m ucocele a. Hallerm an-Streiff syndrom e
27. A child with retinoblastom a is born to healthy parents b. Alport’s syndrom e
with no fam ily history of RB. The chance of RB occurring in c. rubella
a second child is approxim ately d. Lowe’s syndrom e
a. 5% 38. RPE degeneration and optic atrophy are found in all of the
b. 25% following m ucopolysaccharidoses except
c. 40% a. MPS type I
d. 50% b. MPS type II
28. The best chronologic age to exam ine a baby for ROP is c. MPS type III
a. 28 weeks d. MPS type IV
b. 32 weeks 39. Which vitam in is not deficient in a patient with
c. 36 weeks abetalipoproteinem ia (Bassen-Kornzweig syndrom e)?
d. 40 weeks a. A
29. All of the following are associated with trisom y 13 except b. C
a. anophthalm os c. D
b. retinal dysplasia d. E
c. epiblepharon 40. Hearing loss is not found in
d. intraocular cartilage a. Cogan’s syndrom e
30. Paradoxical pupillary response does not occur in b. Refsum ’s disease
a. achrom atopsia c. Duane’s syndrom e
b. CSNB d. Stickler’s syndrom e
c. Leber’s congenital am aurosis 41. Pheochrom ocytom a m ay occur in all of the following
d. albinism phakom atoses except
31. An infant with bilateral cataracts is diagnosed with a. Louis-Bar syndrom e
galactosem ia. Which enzym e is m ost likely to be defective? b. von Hippel-Lindau disease
a. galactokinase c. Sturge-Weber syndrom e
b. galactose-1-P-uridyl transferase d. Bourneville’s disease
c. galactose-6-sulfatase 42. Maternal ingestion of LSD is m ost likely to result in which
d. UDP galactose-4-epim erase congenital optic nerve disorder
32. All of the following are associated with optic nerve drusen a. colobom a
except b. optic pit
a. peripapillary hem orrhage c. hypoplasia
b. inferior nasal visual field loss d. m orning glory disc
c. increased risk of intracranial tum ors 43. A patient with strabism us wearing À6 D glasses is
d. autosom al dom inant inheritance m easured with prism and cover test. Com pared with
33. The etiology of torticollis and interm ittent, fine, rapid, the actual am ount of deviation, the m easurem ent
pendular nystagm us of the right eye in a 10-m onth-old would find
baby is m ost likely a. m ore esotropia and less exotropia
a. m etastatic neuroblastom a b. m ore esotropia and m ore exotropia
b. posterior fossa tum or c. less esotropia and m ore exotropia
c. optic nerve m eningiom a d. less esotropia and less exotropia
d. none of the above 44. Prism glasses are least helpful for treating
34. The m ost com m on m alignant tum or of the orbit in a a. incom itant esotropia
6-year-old boy is b. divergence insufficiency
a. neuroblastom a c. sensory esotropia
b. rhabdom yosarcom a d. interm ittent exotropia

158
 Review Questions

45. A 4-year-old boy has bilateral lateral rectus recessions for a. urine am ino acids
exotropia. Two days after surgery he has an esotropia b. calcium
m easuring 50 Δ. The m ost appropriate treatm ent is c. TORCH titers
a. atropinization d. urine reducing substances
b. prism glasses 56. Genetics of aniridia are best sum m arized as
c. alternate patching a. ¼ AR, ¾ AD
d. surgery b. ¼ sporadic, ¾ AR
46. The m ost com m on cause of a vitreous hem orrhage in a c. ¼ AD, ¾ sporadic
child is d. ¼ sporadic, ¾ AD
a. ROP 57. A pigm entary retinopathy occurs in which m esoderm al
b. shaken baby syndrom e dysgenesis syndrom e?
c. FEVR a. Axenfeld’s anom aly
d. Coat’s disease b. Alagille’s syndrom e
47. A 5-year-old girl with 20/20 vision OD and 20/50 vision c. Rieger’s syndrom e
OS is diagnosed with an anterior polar cataract OS. The d. Peter’s anom aly
m ost appropriate treatm ent is 58. Which of the following laboratory tests is m ost com m only
a. start occlusion therapy found in JRA-related iritis?
b. observe and reexam ine in 6 m onths a. RF–, ANA–
c. perform cataract surgery and use aphakic contact lens b. RF+, ANA–
d. perform cataract surgery with lens im plant c. RF–, ANA+
48. Chronic iritis in a child is m ost com m only caused by d. RF+, ANA+
a. JRA 59. The size of an esodeviation is m easured with
b. traum a a. Cover-uncover test
c. sarcoidosis b. Double Maddox rod test
d. Lym e disease c. Alternate prism and cover test
49. All are features of ataxia-telangiectasia except d. Worth four dot test
a. sinopulm onary infections 60. Toxoplasm osis is m ost likely to be acquired from
b. thym ic hyperplasia a. cat scratch
c. IgA deficiency b. undercooked m eat
d. autosom al recessive inheritance c. needle stick
50. All of the following vitreoretinal disorders are inherited in d. eating dirt
an autosom al dom inant pattern except 61. A 10-day-old infant develops an acute, papillary
a. fam ilial exudative vitreoretinopathy conjunctivitis with m ucoid discharge. Which of the
b. Wagner’s syndrom e following is the m ost likely cause?
c. Stickler’s syndrom e a. Neisseria gonorrhoeae
d. Goldm ann-Favre disease b. Escherichia coli
51. The m ost com m on location for an iris colobom a is c. Chlam ydia
a. superotem poral d. Herpes sim plex
b. superonasal 62. An infant is brought to the ER after a fall. There is a bruise
c. inferotem poral on the forehead and num erous retinal hem orrhages. There
d. inferonasal are also bruises on the back. An X-ray shows previous rib
52. Von Hippel-Lindau disease has been m apped to which fractures. The m ost likely diagnosis is
chrom osom e? a. Coats disease
a. 3 b. m alnutrition
b. 9 c. anem ia
c. 11 d. non-accidental traum a
d. 17 63. A child undergoes uncom plicated cataract surgery with
53. Which X-linked disorder is not associated with an ocular phacoem ulsification and insertion of an acrylic posterior
abnorm ality in the fem ale carrier? cham ber intraocular lens. What is the m ost likely
a. choroiderem ia com plication to develop in the future?
b. albinism a. capsular opacification
c. juvenile retinoschisis b. cystoid m acular edem a
d. retinitis pigm entosa c. hyperopia
54. Which tum or is not associated with von Hippel-Lindau d. retinal detachm ent
disease? 64. On a routine eye exam , a 5-year-old girl is found to have
a. hepatocellular carcinom a m ild iritis in both eyes. What is the m ost helpful test to
b. pheochrom ocytom a order?
c. renal cell carcinom a a. ANA
d. cerebellar hem angioblastom a b. ANCA
55. The m ost useful diagnostic test in an infant with an oil- c. HLA typing
droplet cataract is d. PPD

159
CHAPTER 5 • Pediatrics/Strabism us

65. The m ost com m on color vision defect is Del Monte, M. A., & Archer, S. M. (1991). Atlas of pediatric ophthalmology
a. protanom aly and strabismus surgery. Philadelphia: Butterworth-Heinem ann.
b. protanopia Hoyt, C. S., & Taylor, D. (2012). Pediatric ophthalmology and strabismus
c. deuteranom aly (4th ed.). Philadelphia: Saunders.
Nelson, L. B., & Catalano, R. A. (1997). Atlas of ocular motility.
d. deuteranopia
Philadelphia: WB Saunders.
Nelson, L. B., & Olitsky, S. E. (2013). Harley’s pediatric ophthalmology
(6th ed.). Philadelphia: Lippincott William s & Wilkins.
Von Noorden, G. K. (1983). Atlas of strabismus (4th ed.). St Louis:
SUGGESTED READINGS Mosby.
Von Noorden, G. K., & Cam pos, E. C. (2002). Binocular vision and
Basic and Clinical Sciences Course. (2016). Section 6: Pediatric ocular motility: Theory and treatment of strabismus (6th ed.). St Louis:
ophthalmology and strabismus. San Francisco: AAO. Mosby.
Burian, H. M., & von Noorden, G. K. (1974). Binocular vision and ocular Wright, K. W., & Strube, Y. N. J. (2012). Pediatric ophthalmology and
motility: Theory and treatment of strabismus. St Louis: Mosby. strabismus (3rd ed.). New York: Oxford University Press.

160
6 Orbit/Lids/Adnexa
ANATOMY
IMAGING
ORBITAL DISO RDERS
EYELID DISORDERS
NASOLACRIMAL SYSTEM DISORDERS
ORBITAL SURGERY

ANATOMY Naso lacr im al can al:

Runs from lacrim al sac fossa to inferior m eatus under the
inferior turbinate
Dim e n s io n s
Contains the nasolacrim al duct
Or b it: pear-shaped (widest diam eter is 1 cm posterior to Lateral wall ¼ m axillary bone
orbital rim ); 40 m m wide, 35 m m high, 45 m m deep, Medial wall ¼ lacrim al bone and inferior turbinate
volum e ¼ 30 cc (Table 6-1)
Zygo m atico facial an d zygo m atico tem p o r al can als:
Op tic n er ve: orbital length ¼ 25-30 m m ; length from globe Transmit: vessels and branches of the zygom atic nerve
to optic foram en ¼ 18 m m ; width ¼ 1.5 m m in globe, 3.5 m m through the lateral wall to cheek and tem poral fossa
posterior to lamina cribrosa (due to m yelin), 5.0 m m with the
addition of the optic nerve sheath (see Fig. 4-2)
Eth m o id al fo r am in a:
Transmit: anterior and posterior ethm oid arteries
Pr o p to sis: m easure with Hertelexophthalm om eter Potential route for spread of infectious sinusitis
(Table 6-2)

Ap e rt u re s Fr o n to sp h en o id al fo r am in a:
Transmit: anastom osis between the m iddle m eningeal and
(Figs. 6-1 to 6-3) lacrim al arteries, which provides collateral blood supply
to the orbit
Su p er io r o r b ital fissu r e:
Separates greater and lesser sphenoid wings Fo r am en o vale:
Between roof and lateral wall Transmits: CN V3
Transmits: CN 3, 4, V1 , and 6, superior ophthalm ic vein,
and sym pathetic fibers to iris dilator Fo r am en r o tu n d u m :
Transmits: CN V2
Op tic can al (orbital foram en):
Within lesser wing of sphenoid: 10 m m long Fo r am en lacer u m :
Enlarged with ON gliom a Transmits: internal carotid artery
Transmits: Optic nerve (CN 2), ophthalm ic artery, and
sym pathetic nerves to ocular and orbital blood vessels
Va s c u la r s u p p ly t o e ye
In fer io r o r b ital fissu r e: (Figs. 6-4 to 6-6)
Bordered m edially by m axillary bone, anteriorly by
zygom atic bone, and laterally by greater wing of sphenoid Op h th alm ic ar ter y: first branch of internal carotid
Transmits: CN V2 , zygom atic nerve, inferior ophthalm ic within skull
vein, venous com m unication between ophthalm ic Central retinal artery:
vein and pterygoid plexus, sphenopalatine ganglion Enters optic nerve 13 m m posterior to globe
branches Supplies blood to inner two-thirds of retina

161
CHAPTER 6 • Orbit/Lids/Adnexa

Frontal Optic Supraorbital Superior Zygomaticotemporal Zygomatric

Ta b le 6-1. Osteology
bone canal foramen orbital fissure foramen bone
Re la te d s truc ture s /
Orb it Bo ne s m is c e lla ne o us
Roof Sphenoid (lesser wing) Lacrimal gland fossa
Frontal Trochlea
Supraorbital notch (medial)
Lateral wall Sphenoid (greater wing) Lateral orbital tubercle of
Whitnall
Zygomatic Strongest orbital wall
Lateral orbital rim at equator of
globe
Floor Maxilla Contains infraorbital nerve and
canal
Palatine Forms roof of maxillary sinus
Zygomatic
Medial wall Sphenoid Lacrimal sac fossa
Maxilla Adjacent to ethmoid and
sphenoid sinuses
Ethmoid Posterior ethmoidal foramen
Lacrimal Weakest orbital wall
Nasal Lacrimal Ethmoid Infraorbital Maxillary Inferior Sphenoid Zygomatico-
bone bone bone foramen bone orbital bone facial
fissure foramen

Ta b le 6-2. Hertel exophthalmometry measurements Fig ure 6-1. Bony anatomy of the orbit in frontal view. (From Dutton J J : Atlas of
clinical and surgical orbital anatomy, Philadelphia, 1994, WB Saunders.)
Up p e r lim it o f
Me a n (m m ) no rm a l (m m )
Caucasian male 16.5 21.7
Caucasian female 15.4 20
African American male 18.5 24.7
African American female 17.8 23.0

Fronta l ne rve Trochle a r ne rve (CN4)

La crima l ne rve Optic ne rve (CN2)

L
S upe rior orbita l
fis s ure SR
SO
Na s ocilia ry ne rve

Oculomotor ne rve Ophtha lmic a rte ry

(CN3, s upe rior divis ion) MR

Abduce ns ne rve (CN6) LR Oculomotor ne rve

IR (CN3, infe rior divis ion)

S upe rior ophtha lmic ve in Ma xilla ry ne rve (CNV2 )

P te rygopa la tine
ga nglion ne rve

P te rygoid ne rve
Infe rior orbita l
fis s ure Infe rior ophtha lmic ve in

Fig ure 6-2. Orbital apex, superior and inferior orbital fissure. MR, medial rectus; IR, inferior rectus; LR, lateral rectus; SR, superior rectus; L, levator; SO, superior oblique.
Note that the trochlear nerve lies outside the muscle cone.

162
 Anatom y

P la num Chia s ma tic P e troclinoid

s phe noida le groove (Grube r's liga me nt)
S upe rior
Clivus
Optic Tube rculum colliculus
fora me n s e lla e
Midbra in
Ante rior Fourth
clinoid ve ntricle

S phe noid
ridge P ons
Fa cia l
S upe rior colliculus
orbita l
fis s ure

Fora me n
Me dulla
rotundum
Fora me n Fora me n Fora me n
la ce rum ova le ma gnum
Floor of Trige mina l P e trous portion Inte rna l
middle fos s a impre s s ion of te mpora l bone a uditory
(floor of me a tus
Me cke l's ca ve )
Fig ure 6-3. Schematic representation of the landmarks, temporal view. (From Bajandas FJ , Kline BK: Neuro-Ophthalmology review manual, Thorofare, NJ , 1998,
Slack, 1988.)

Supra orbita l
La tera l posterior
a rtery
cilia ry a rtery

Dorsa l na sa l
a rtery Zygoma ticotempora l
a rtery

Zygoma ticofa cia l

Anterior a rtery
ethmoida l
a rtery

Centra l retina l
a rtery
O phtha lmic
a rtery

Media l posterior
cilia ry a rtery

Fig ure 6-4. Arterial supply to the orbit, in coronal view. (From Dutton J J : Atlas of clinical and surgical orbital anatomy, Philadelphia, 1994, WB Saunders.)

163
CHAPTER 6 • Orbit/Lids/Adnexa

Superior ophtha lmic vein

Superior la tera l vortex vein

Media l
orbita l vein La crima l vein

La tera l
colla tera l vein

Centra l
retina l vein

Inferior la tera l
vortex vein

Media l colla tera l vein

Inferior ophtha lmic vein

Fig ure 6-5. Orbital veins.Venous drainage from the orbit, in coronal view. (From Dutton J J : Atlas of clinical and surgical orbital anatomy, Philadelphia, 1994, WB
Saunders.)

Posterior ciliary arteries: Short ciliary nerves pass through ciliary ganglion
Long posterior ciliary arteries supply anterior segm ent Long ciliary nerves supply iris, cornea, and ciliary m uscle
Short posterior ciliary arteries supply choroid Frontal nerve:
Optic nerve head is prim arily supplied by blood from Enters orbit above annulus of Zinn
the vascular circle of Zin n -Haller (collection of Divides into supraorbital nerve and supratrochlear nerve
anastom otic arteries arising from the short posterior Innervates m edial canthus, upper lid, and forehead
ciliary arteries) Lacrimal nerve:
Enters orbit above annulus of Zinn
Ven o u s system : Orbital veins do not have valves Innervates upper eyelid and lacrim al gland
Vortex veins:
4-8 per eye; 1-2 per quadrant
Maxillar y n er ve (V2 ):
Exit posterior to the equator
Passes through foram en rotundum , then passes through
Drain choroid and m erge into the superior or inferior
inferior orbital fissure
ophthalm ic veins
Divides into infraorbital nerve, zygom atic nerve, and
Superior ophthalmic vein:
superior alveolar nerve
Exits via superior orbital fissure into the cavernous sinus
Two superior vortex veins
Interior ophthalmic vein: Par asym p ath etic in n er vatio n :
Exits via inferior orbital fissure Controls accom m odation, pupillary constriction, and
Two inferior vortex veins lacrim al gland stim ulation
Central retinal vein: Enters eye as short posterior ciliary nerves after synapsing in
Joins superior or inferior ophthalm ic vein the ciliary ganglion
Leaves nerve 10 m m behind globe

In n e r va t io n o f e ye Sym p ath etic in n er vatio n :

Sensory innervation provided by ophthalm ic and m axillary Controls pupillary dilation, vasoconstriction, sm ooth
division of trigem inal nerve (CN 5) (Fig. 6-7) m uscle function of eyelids and orbit, and hidrosis
Dysfunction: Horner’s syndrom e (ptosis, m iosis,
Op h th alm ic d ivisio n (V1 ): anhidrosis, and vasodilation)
Nasociliary nerve: Nerve fibers follow arterial supply as well as long ciliary
Enters orbit within the annulus of Zinn nerves

164
 Anatom y

Minor a rte ria l circle of iris Supra trochlea r Supra orbita l Fronta l Na socilia ry La crima l
nerve nerve nerve nerve nerve
Ma jor a rte ria l circle of iris
Conjunctiva l ve s s e ls
Ante rior cilia ry
ve s s e ls

Ve s s e ls of
cilia ry body

O phtha lmic
division
of the
Choroida l ve s s e ls trigemina l
nerve
Long
Re tina l ve s s e ls posterior
cilia ry
nerves

Epis cle ra l
ve s s e ls
Infra orbita l Ma xilla ry nerve Pterygopa la tine
Vorte x ve in nerve ga nglion

Fig ure 6-7. Sensory nerves of the orbit, in lateral view. (From Dutton J J : Atlas of
clinical and surgical orbital anatomy, Philadelphia, 1994, WB Saunders.)
Long pos te rior
cilia ry a rte ry
S hort pos te rior
S o ft t is s u e s
cilia ry a rte rie s
Dura l ve s s e ls (Fig. 6-9)
Ce ntra l ve s s e ls
of re tina P ia l ve s s e ls
Per io r b ita:
Fig ure 6-6. Vascular supply to the eye. All arterial branches originate with
the ophthalmic artery. Venous drainage is through the cavernous sinus and
Periosteum is attached firm ly at the orbital rim and
the pterygoid plexus. suture lines
Arcus marginalis: fusion of periosteum and orbital septum
at orbital rim
S in u s e s
Fuses with the dura covering the optic nerve
(Fig. 6-8)
An n u lu s o f Zin n :
Fr o n tal: Fibrous tissue ring arising from periorbita that surrounds
Not radiographically visible before age 6 the optic canal
Drains into anterior portion of the m iddle m eatus Origin of the 4 recti m uscles
Fuses with dura covering ON at apex
Eth m o id :
Multiple thin-walled cavities Ad ip o se tissu e:
First sinus to aerate Surrounds m ost of orbital contents; divided by fine
Anterior and m iddle air cells drain into the m iddle m eatus fibrous septa
Posterior air cells drain into the superior m eatus Preaponeurotic fat pads: lie im m ediately posterior to
Ethm oidal sinusitis is the m ost com m on source of infection orbital septum
that leads to orbital cellulitis 2 FAT PADS IN UPPER LID: anterior to levator
aponeurosis; m edial pad sm aller and paler than
Sp h en o id : central pad; with aging, m edial pad m oves anteriorly
Rudim entary at birth and m ay cause bulging of upper nasal orbit
Reaches full size after puberty 3 FAT PADS IN LOWER LID: anterior to capsulopalpebral
Optic canal is superior and lateral to sphenoid sinus fascia; inferior oblique muscle separates the m edial
Drains into sphenoethm oid recess of each nasal fossa and central pads; lateral pad is sm all and m ore
inferior
Maxillar y:
Largest sinus Lacr im al glan d :
Roof contains the infraorbital nerve 2 lobes: orbital (larger) and palpebral, separated by levator
Drains into m iddle m eatus aponeurosis

165
CHAPTER 6 • Orbit/Lids/Adnexa

FS FS

ES SS
ES

MS MS

Fig ure 6-8. Relationship of the orbits to the paranasal sinuses: FS, frontal sinus, ES, ethmoid sinus; MS, maxillary sinus; SS, sphenoid s inus. (Reprinted with permission
from Slamovits TL: Basic and clinical science course, Section 7: Orbit, eyelids and lacrimal system. American Academy of Ophthalmology, 1993, San Francisco.)

Fa t cus hion S upe rior Le va tor P os te rior Te non's Ante rior Blood supply: lacrim al artery
outs ide re ctus mus cle mus cle ca ps ule Te non's ca ps ule Orbita l
Accessory lacrimal glands: glands of Wolfring located in
mus cle cone s e ptum
tarsus and glands of Krause located in the conjunctival
fornices (10% of production)

Eye lid
Lam ellae o f u p p er eyelid : (Fig. 6-10)
Anterior: skin, orbicularis
S upe rior Conjunctiva Posterior: tarsus, levator aponeurosis, Mu€ ller’s m uscle,
oblique Fus e d Te non's
ca ps ule a nd
palpebral conjunctiva
te ndon
inte rmus cula r
s e ptum
Fa t cus hion Skin :
ins ide mus cle
cone Thinnest skin of the body, no subcutaneous fat layer
Upper eyelid crease approxim ates attachm ents of
Infe rior the levator aponeurosis to the pretarsal orbicularis
re ctus mus cle and skin
Histology of epidermis:
Conjunctiva
BASILAR LAYER (stratum basalis): cuboidal cells with
scant cytoplasm ; responsible for generating
Orbita l
s e ptum
superficial layers
P os te rior Te non's PRICKLE CELL LAYER: m ultiple layers of cells with
ca ps ule Ante rior
abundant cytoplasm attached to each other by
Infe rior oblique mus cle Te non's ca ps ule
desm osom es
Fig ure 6-9. Sagittal section of orbital tissues through the vertical recti. (Adapted GRANULAR CELL LAYER (stratum granulosum ): cells
from Parks MM: Extraocular muscles. In Duane TD: Clinical ophthalmology, contain granular m aterial that stains blue with H&E
Philadelphia, 1982, Harper and Row.) KERATIN LAYER: superficial, acellular keratin devoid of
nuclei; stains pink with H&E

Ducts from both lobes pass though the palpebral lobe and Or b icu lar is o cu li: (Fig. 6-11)
em pty into the superior fornix Main protractor of the eyelid; acts as lacrim al pum p;
Innervation: secretom otor from superior salivary nucleus innervated by CN 7
via CN 7; sensory (afferent) from CN 5; sym pathetic 3 anatomic parts: palpebral portion (pretarsal and
from superior cervical ganglion via deep petrosal preseptal) involved with involuntary blinking; orbital
nerve, pterygopalatine ganglion, and zygom atic nerve portion involved with voluntary, forced lid closure

166
 Anatom y

Orbita l Le va tor Mue lle r's mus cle Gla nds

s e ptum mus cle of Kra us e Arcus
ma rgina lis
Fa t
Orbicula ris S upe rior
P e riphe ra l
mus cle orbita l
a rca de
s e ptum

Gla nds of Le va tor

S kin Wolfring a pone uros is

Conjunctiva

Ta rs a l
gla nds

Ma rgina l
Ha ir a rca de Ante rior Inte rme dia te P os te rior Infe rior orbita l
follicle la ye r la ye r la ye r s e ptum

Ze is Mucocuta ne ous Fig ure 6-12. The orbital septum. (From Dutton J J : Atlas of clinical and surgical
junction orbital anatomy, Philadelphia, 1994, WB Saunders.)
gla nd
Me ibomia n gla nd
Gla nds of Moll ope ning
PRESEPTAL: overlies orbital septum
Fig ure 6-10. Cross-section of upper eyelid. Note position of cilia, tarsal gland
orifices, and mucocutaneous junction. (Reprinted with permission from Grand
Originates from anterior lim b of the m edial canthal
MG: Basic and clinical science course, section 2: fundamentals and principles of tendon, and from posterior lacrim al crest and
ophthalmology. American Academy of Ophthalmology,1993, San Francisco.) lacrim al sac fascia
Form s lateral palpebral raphe overlying the lateral
orbital rim
ORBITAL: lies beneath skin
Fronta lis mus cle
Thickest portion of orbicularis
P roce rus mus cle
Inserts at m edial canthal tendon
Orbita l portion Interdigitates with the frontalis m uscle superiorly at
of orbicula ris eyebrow
mus cle
S upe rior
pre s e pta l portion
Oth er m u scles o f fo r eh ead an d eyeb r o w:
of orbicula ris
mus cle Frontalis: m oves scalp anteriorly and posteriorly; raises
La te ra l horizonta l eyebrows; innervated by CN 7
ra phe Corrugator: pulls m edial eyebrow inferiorly and m edially
S upe rior producing vertical glabellar wrinkle; originates from
pre ta rs a l portion nasal process of frontal bone; inserts laterally into
of orbicula ris subcutaneous tissue; innervated by CN 7
mus cle Procerus: pulls forehead and m edial eyebrow inferiorly
Ante rior a rm producing horizontal lines in nose; interdigitates with
of me dia l
inferior edge of frontalis; innervated by CN 7
ca ntha l te ndon

Fig ure 6-11. The orbicularis and frontalis muscles. (From Dutton J J : Atlas of
clinical and surgical orbital anatomy, Philadelphia, 1994, WB Saunders.) Or b ital sep tu m : (Fig. 6-12)
Dense fibrous sheath that acts as barrier between the
PRETARSAL: overlies tarsus, originates from lateral orbit and eyelid; stops spread of infection
orbital tubercle, form s m uscle of Riolan (seen as gray Originates from periosteum of the superior and inferior
line at lid margin) orbital rim s
SUPERFICIAL HEAD: m edially inserts into anterior Inserts into levator aponeurosis superiorly (2–5 m m above
lacrim al crest, contributes to m edial canthal superior tarsal border in non-Asians) and into lower
tendon, laterally inserts into zygom atic bone eyelid retractors inferiorly (capsulopalpebral fascia just
DEEP HEAD: m edially inserts into posterior lacrim al below inferior tarsal border)
crest, called Horner’s muscle, surrounds canaliculi Medially inserts into lacrim al crest
facilitating tear drainage, laterally inserts into Structures posterior to the septum : palpebral lobe of
lateral orbital tubercle, contributes to lateral lacrim al gland, lateral canthal tendon, trochlea of
canthal tendon superior oblique m uscle

167
CHAPTER 6 • Orbit/Lids/Adnexa

W hitna ll's Leva tor

liga ment
pa lpebra e
superioris
muscle

Leva tor
a poneurosis

Prea poneurotic La tera l horn

fa t pa ds
La tera l ca ntha l
tendon

Müller‘s muscle
O rbita l septum

Eyelid crea se

Leva tor Media l Media l Fa scia l slips to Ca psulopa lpebra l

a poneurosis ca ntha l tendon horn orbicula ris muscle fa scia

Fig ure 6-14. The levator aponeurosis, and the medial and lateral canthal
tendons. (From Dutton J J : Atlas of clinical and surgical orbital anatomy,
Ta rsus Philadelphia, 1994, WB Saunders.)

Whitnall’s ligament (superior transverse ligam ent):

condensation of levator m uscle sheath
Visible as horizontal white line 10 m m above superior
edge of tarsus
Arises from the sheath of anterior portion of levator
m uscle
Medially attaches to trochlea
Laterally form s septa through strom a of lacrim al gland
and attaches to inner aspect of lateral orbital wall at
Fig ure 6-13. The orbitalseptum inserts into the levator aponeurosis (arrows). The the frontozygom atic suture (10 m m above orbital
preaponeurotic fat pads are located posterior to the septum. In downgaze, the lid
crease becomes attenuated (weakened), and in a normal young eyelid, the fold is
tubercle [Whitnall’s tubercle]) and at the orbital
absent. From Zide BW, J elks BW: Surgical anatomy of the orbit. New York, tubercle
1985, Raven Press.) Acts to change direction of pull of levator and
serves as check ligam ent to prevent excessive lid
In Asians, orbital septum fuses with levator aponeurosis elevation
between the eyelid m argin and the superior border of the Mu€ller’s muscle (superior tarsal m uscle):
tarsus Posterior to levator aponeurosis
Up p er eyelid r etr acto r s: (Fig. 6-13) Sym pathetic innervation
Levator palpebrae: Originates from undersurface of levator approxim ately
Originates from lesser wing of sphenoid above annulus at level of Whitnall’s ligam ent
of Zinn Inserts into upper border of tarsus
Innervated by CN 3 12–15 m m long; raises eyelid 2 m m
MUSCULAR PORTION: 40 m m Peripheral arterial arcade found between aponeurosis
APONEUROTIC PORTION: 14–20 m m and has lateral and Mu€ ller’s m uscle
and m edial extensions (horns) (Fig. 6-14)
LATERAL HORN: Lo wer eyelid r etr acto r s: (Fig. 6-15)
Inserts onto lateral orbital tubercle Capsulopalpebral fascia: analogous to levator aponeurosis
Divides lacrim al gland into orbital and palpebral Originates from inferior rectus m uscle sheath, divides as
portions it encircles inferior oblique m uscle, and then joins to
MEDIAL HORN: form Lockwood’s ligam ent
Inserts onto posterior lacrim al crest Fuses with septum and inserts into inferior tarsus
More delicate and weaker than lateral horn Lockwood’s suspensory ligament: analogous to Whitnall’s
Anterior portion inserts into septa between pretarsal ligam ent
orbicularis m uscle bundles Arises posteriorly from fibrous attachm ents to inferior
Posterior portion inserts onto anterior surface of side of the IR m uscle and continues anteriorly as
lower half of tarsus; form s eyelid crease capsulopalpebral fascia
Disinsertion of aponeurosis results in loss of eyelid Medial and lateral horns attach to retinacula form ing
crease a suspensory ham m ock for the globe

168
 Anatom y

O rbicula ris Arte ria l s upply

muscle Fornix Tenon's Ca psulopa lpebra l Inferior S upe rior
Ta rsus ca psule fa scia rectus S upra orbita l pa lpe bra l
a rte ry a rte ry
S upra trochle a r s upe rior
a rte ry P e riphe ra l
a rte ria l a rca de
Me dia l S upe rior
pa lpe bra l ma rgina l
a rte ry a rte ria l a rca de
La te ra l
Angula r
pa pe bra l a rte ry
a rte ry
Infe rior
ma rgina l
Fa cia l a rte ry a rte ria l a rca de
Ca psulopa lpebra l hea d Ve nous dra ina ge

Inferior oblique muscle S upra orbita l S upe rior

O rbita l fa t ve in pa lpe bra l
ve in
Inferior
Na s ofronta l
Septum Lockwood's liga ment ve in S upe rior
orbita le ta rsa l muscle
Me dia l pe riphe ra l
Fig ure 6-15. Anatomy of the lower eyelid retractors. (Adapted from Hawes MJ , pa lpe bra l ve nous
Dortzbach RK: The microscopic anatomy of the lower eyelid retractors, Arch ve ins a rca de
Ophthalmol 100:1313-1318, 1982.) La te ra l
Angula r ve in pa lpe bra l
ve in
Inferior tarsal muscle: analogous to Mu€ ller’s m uscle
Sym pathetic innervation Infe rior
pe riphe ra l
Arises from capsulopalpebral fascia Ante rior
ve nous a rca de
fa cia l ve in
Tar su s:
Dense connective tissue plate in each eyelid; not cartilage Fig ure 6-16. Arterial supply to, and venous drainage from, the eyelids. (From
Dutton J J : Atlas of clinical and surgical orbital anatomy, Philadelphia, 1994,
Contains m eibom ian glands
WB Saunders.)
29 m m long and 1 m m thick; tapered at ends
Rigid attachm ents to periosteum m edially and laterally
Superior tarsal plate: 10 m m in m axim al height centrally Vascu lar su p p ly: (Fig. 6-16)
Inferior tarsal plate: 4 m m in m axim al height Upper lid: internal carotid artery ! ophthalm ic artery !
superior m arginal arcade (deep to orbicularis and
anterior surface of tarsus)
Med ial can th al ten d o n :
Lower lid: external carotid artery ! facial artery! angular
Fusion of tendinous insertions of pretarsal and preseptal
artery! inferior m arginal arcade
orbicularis m uscles
Angular artery is 6 to 8 m m m edial to m edial canthus,
Attaches to tarsal plates
posterior to orbicularis, 5 m m anterior to lacrim al sac
Anterior limb: attaches to frontal process of m axillary bone
Peripheral and m arginal arcades allow for anastom osis
and serves as origin of superficial head of pretarsal
between the ICA and ECA
orbicularis; passes in front of lacrim al sac and attaches to
Venous drainage:
anterior lacrim al crest
PRETARSAL: angular vein m edially, superficial tem poral
Posterior limb: attaches to posterior lacrim al crest; passes
vein tem porally
behind lacrim al sac. More im portant than anterior lim b
POST-TARSAL: orbital vein, anterior facial vein, and
for norm al m edial canthal appearance and function
pterygoid plexus
(m aintaining apposition of lids to globe)

Later al can th al ten d o n :

Broad band of connective tissue from lateral borders of In n er vatio n :
upper and lower tarsal plates Sensory: CN V1 (upper lid), CN V2 (lower lid)
Inserts onto lateral orbital tubercle of Whitnall; 3 m m Motor: CN 3, CN 7, sym pathetics
superior to m edial canthal tendon insertion

Eyelid m ar gin : 3 distinguishing landm arks Lym p h atic d r ain age:

Lash line: 2 to 3 rows of lashes; 100 in upper lid, 50 in lower Submandibular nodes: drain m edial one-third of upper lid,
lid; 10-week growth, 5-m onth resting phase m edial two-thirds of lower lid
Gray line: border of pretarsal orbicularis (m uscle of Preauricular nodes: drain lateral two-thirds of upper lid,
Riolan); junction of anterior and posterior lam ellae; lateral one-third of lower lid
vascular watershed No lym phatic vessels or nodes are found within the orbit
Meibomian gland orifices: 30 in upper lid, 20 in lower lid Lym phatic vessels are found in the conjunctiva

169
CHAPTER 6 • Orbit/Lids/Adnexa

Ta b le 6-3. Glands of the eyelids

Gla nd Lo c a tio n Typ e Func tio n P a tho lo g y

Lacrimal Superotemporal orbit Eccrine Reflex tear (aqueous) secretion Dacryoadenitis
Tumors
Access ory lacrimal: Fornix Eccrine Basal tear Sjo€ gren’s syndrome
Krause J ust above tarsus (aqueous) s ecretion GVH disease
Wolfring Rare tumors (BMT)
Meibomian Within tarsus Holocrine Lipid secretion Chalazion
Retards tear evaporation Sebaceous carcinoma
Zeis Near lid margin Holocrine Lipid secretion External hordeolum
Caruncle Lubricates cilia Sebaceous carcinoma
Associated with cilia
Moll Near lid margin Apocrine Modified sweat glands Ductal cyst
Lubricates cilia Apocrine carcinoma
Sweat Eccrine Electrolyte balance Ductal cyst
Syringoma
Sweat gland carcinoma
Goblet cells Conjunctiva Holocrine Mucin secretion Dry eye
Plica Enhances corneal wetting
Caruncle

Glan d s o f th e eyelid s: (Table 6-3) 8 mm

Eccrine: secretion by simple exocytosis
Holocrine: secretion by release of entire cellular contents
(disruption of cell)
Apocrine: secretion by pinching or budding off of a portion Fundus of s a c
of cellular cytoplasm
2 mm 10 mm
Na s o la c rim a l s ys t e m Body of s a c
(Fig. 6-17)

Pu n cta:
Upper and lower; 6 m m from m edial canthus, lower is
slightly m ore tem poral Na s ola crima l duct Inte ros s e ous
Slightly inverted against globe 15 mm pa rt
Open into am pulla (2 m m long) oriented perpendicular to
eyelid m argin

Can alicu li: Me a ta l pa rt

10 m m long (2 m m vertical segm ent [am pulla] and 8 m m
horizontal portion parallel to lid m argin)
Com bine to form single com m on canaliculus in 90% of
Infe rior me a tus
individuals
~20 mm
Valve of Rosenm u€ ller prevents reflux from lacrim al sac into
canaliculi

Naso lacr im al sac: Fig ure 6-17. Excretory lacrimal system. (From Grand MG: Basic and clinical
science course, section 2, fundamentals and principles of ophthalmology, San
10 m m long, occupies the lacrim al fossa Francisco, 1993, American Academy of Ophthalmology.)
Lies between anterior and posterior crura of the m edial
canthal tendon, anterior to orbital septum
Lateral to m iddle m eatus of nose
Lacr im al p u m p :
Naso lacr im al d u ct: Lids close: pretarsal orbicularis contracts, com presses
15 m m long am pulla, shortens canaliculus; punctum m oves
Passes inferiorly, posteriorly, and laterally within canal m edially; lacrim al sac expands, creates negative pressure,
form ed by m axillary and lacrim al bones draws fluid from canaliculus into sac
Extends into inferior m eatus, which opens under inferior Lids open: m uscles relax, lacrim al sac collapses, tears forced
turbinate (2.5 cm posterior to naris) into nose, punctum m oves laterally, tears enter
Partially covered by valve of Hasner canaliculus

170
 Im aging

Dem yelinating plaques of MS are best seen with

IMAGING T2-weighted im ages (hyperintense foci, usually in
periventricular white m atter)
Ult ra s o u n d
Ad van tages: better for soft tissues and posterior fossa,
Optim al sound wave frequency is 10 MHz sagittal sections, no artifacts from bone or teeth, no ionizing
Higher frequencies give better resolution; lower frequencies radiation
provide better penetration (Table 6-4) Disad van tages: poor bony detail, longer im aging tim e,
m ore expensive, certain contraindications
Ta b le 6-4. Ultrasound characteristics of lesions
Co n tr ain d icatio n s: patients with retained m etallic foreign
Go o d s o und tra ns m is s io n P o o r s o und tra ns m is s io n
body, pacem aker, aneurysm clip
Cavernous hemangioma Metastatic cancer
Lymphangioma Orbital pseudotumor CT s c a n
Mucocele Glioma
Dermoid Neurofibroma Use with orbital traum a to detect foreign body or calcification,
High Re fle c tivity Low Re fle c tivity or for evaluation of orbital soft tissue lesion with suspicion of
Neurofibroma Metastatic cancer bony erosion
Fresh hemorrhage Orbital pseudotumor
Hemangioma Cyst Bone and m etal appear bright
Thyroid eye disease Mucocele
Varix
Ad van tages: better bony detail and for acute hem orrhage,
Dermoid
Lymphoma sinuses, and traum a; shorter im aging tim e; less expensive

Disad van tages: poor posterior fossa detail; no sagittal

MRI sections, ionizing radiation
(Table 6-5)
Strong m agnetic field results in alignm ent of nuclei of atom s
with odd num bers of protons or neutrons Ta b le 6-5. CT and MRI characteristics of lesions
Radiofrequency pulse disturbs the alignm ent by energizing Mo s t c o m m o n o rb ita l
protons or neutrons le s io ns with we ll- Mo s t c o m m o n o rb ita l le s io ns
c irc um s c rib e d a p p e a ra nc e with Ill-d e fine d a p p e a ra nc e
When pulse term inates, protons return to previous alignm ent o n CT a nd MRI o n CT a nd MRI
and em it absorbed energy as radiofrequency signal, generating
Child re n: Child re n:
an im age
Dermoid cyst Capillary hemangioma
Can provide axial, coronal, and sagittal views Lymphangioma Orbital pseudotumor
Rhabdomyosarcoma Plexiform neurofibroma
ON glioma Leukemic infiltrate
Relaxatio n tim es: Eos inophilic granuloma
Longitudinal relaxation time (T1): tim e required for net Ad ults : Ad ults :
bulk m agnetization to realign itself along original axis Cavernous hemangioma Orbital pseudotumor
Blood, orbital fat, m elanin, and m ucus appear bright Neurofibroma Metastasis
Neurilemmoma Leukemic infiltrate
Acute hem orrhage, vitreous, optic nerve sheath, and Fibrous histiocytoma Primary malignant tumor
extraocular m uscles appear dark (Lymphoproliferative disorders) Lymphoproliferative disorders
Most orbital tum ors are hypointense to fat on CT, Computed tomography; MRI, magnetic resonance imaging.
T1-weighted im ages
EXCEPTIONS: m ucinous lesions (orbital derm oid cyst,
m ucocele), lipom a, liposarcom a, m elanom a, Ra d io g ra p h s
subacute hemorrhage Specific views can be helpful, especially when CT is unavailable
Gadolinium : bright white on T1-weighted im ages or to quickly rule out a m etallic foreign body
Must perform fat suppression on T1-weighted im ages
when gadolinium is used Plain film radiography also detects sinus pathology,
Fat suppression renders orbital fat hypointense and calcification, hyperostosis, and lytic lesions
perm its better visualization of optic nerve and
extraocular m uscles
Can th o m eatal lin e: radiographic baseline of skull (line
between lateral canthal angle and tragus of ear)
Transverse relaxation time (T2): m ean relaxation tim e
Based on interaction of hydrogen nuclei within a given Water s view: occipitom ental; head extended until
tissue canthom eatal line lies at 37° to the central beam ; best view of
Provides m ore inform ation about pathologic processes orbital roof and floor
Helps differentiate m elanotic lesions from hem orrhagic
process Cald well view: posteroanterior view with central beam
Vitreous is bright on T2-weighted im ages tilted 25° toward feet; best view of superior and lateral orbital
Blood and fat appear dark rim , m edial wall, and ethm oid and frontal sinuses

171
CHAPTER 6 • Orbit/Lids/Adnexa

Later al view: best for evaluation of sella turcica Or b ital ap ex:

Can involve optic canal and superior orbital fissure
Su b m en tal ver tex view: basal or Hirtz view with head Complications: CSF rhinorrhea, carotid-cavernous fistula,
tilted back until canthom eatal line is perpendicular to central direct dam age to the optic nerve with vision loss
beam ; best view of sphenoid and ethm oid sinuses, nasal
cavities, and zygom atic arch Or b ital r o o f:
Uncom m on, entrapm ent rare
Ob liqu e ( × 2) views: PA view with central beam tilted 32° May affect frontal sinus, cribriform plate, and brain
toward feet and 37° from perpendicular; best views of optic Complications: CSF rhinorrhea, pneum ocephalus,
foram ina pulsating exophthalm os (delayed com plication)

ORBITAL DISORDERS Zygo m atic (tripod):

Fracture of zygom a at 3 sites (zygom aticom axillary suture,
zygom aticofrontal suture, and zygom atic arch) and
Co ng e nita l a nd De ve lo p m e nta l orbital floor
Ano m a lie s Findings: discontinuity of orbital rim , flattening of
m alar em inence, enophthalm os, infraorbital
(See Chapter 5, Pediatrics/Strabism us) hypesthesia, trism us, orbit or lid em physem a, downward
displacem ent of lateral canthus

Tra um a LeFo r t (I, II, III):

I: low transverse fracture of m axillary bone above teeth;
Orb it a l c o n t u s io n no orbital involvem ent
II: pyram idal fracture of nasal, lacrim al, and m axillary
Bruising from blunt traum a bones; involves m edial orbital floors
Pain, decreased vision III: craniofacial dysjunction; involves orbital floor, lateral
and m edial walls; m ay involve optic canal
Often have associated lid, orbit, and ocular injuries CT scan: identify and localize fracture and coexisting injuries
Tr eatm en t: ice com presses, system ic NSAIDs
Tr eatm en t: system ic steroids and antibiotics; Surgical repair
Orb it a l fra c t u re s depends on type, location, and severity of fracture and
associated findings
Fractures of orbital bones, often associated with ocular or
intracranial injuries
In t ra o rb it a l fo re ig n b o d ie s
Or b ital flo o r (blow-out): Maxillary bone Com m only associated with intraocular and / or optic nerve
Posterior m edial floor is weakest area injury
Findings: m ay have enophthalm os, diplopia, infraorbital
hypesthesia, orbit or lid em physem a, and m edial wall Inert m aterial can be well tolerated and m ay be observed
fracture Copper and organic m aterial are poorly tolerated and m ust be
Complications: orbital contents may becom e entrapped in rem oved
maxillary sinus with resultant restriction of ocular motility, Often asym ptom atic, but m ay have pain or decreased vision
diplopia, and globe ptosis. Entrapment is comm on in
children with a “white-eyed blow-out fracture” in which CT scan (MRI is contraindicated for m etallic foreign bodies)
the pliable bone creates a “trapdoor” with ensnarem ent of
the inferior rectus or perimuscular tissue resulting in Tr eatm en t: system ic antibiotics, tetanus booster, consider
positive forced ductions and nausea and bradycardia surgical rem oval
(oculocardiac reflex); this requires urgent surgery
Orb it a l (Re t ro b u lb a r) h e m o rrh a g e
Med ial wall:
Maxillary, lacrim al, and ethm oid bones Com partm ent syndrom e due to bleeding in orbit with globe
Direct (naso-orbito-ethmoidal). Type 1: central bone fragm ent, and nerve com pression
tendon attached; Type II: com m inuted fracture of bones; Ocular em ergency
Type III: avulsed tendon (m iniplate fixation, transnasal
wiring)
Indirect (blow-out) Fin d in gs: pain, decreased vision, subconjunctival
Findings: m ay have depressed nasal bridge, telecanthus, hem orrhage, proptosis, restriction of ocular m otility, increased
m edial canthal rounding, floor fracture, enophthalm os IOP, tense orbit
Complications: epistaxis from injury of anterior ethm oidal
artery, CSF rhinorrhea, lacrim al drainage injury, orbit or Tr eatm en t: lateral canthotom y, inferior and possibly
lid em physem a superior cantholysis

172
 Orbital Disorders

Asp er gillo sis: Septated branching hyphae

De g e ne ra tio n Disseminated form: occurs in im m unosuppressed hosts;
widespread necrotizing angiitis, throm bosis,
At ro p h ia b u lb i endophthalm itis
Local form: sclerosing granulom atous infiltrative m ass that
Progressive degeneration and decom pensation of globe
originates in the sinus; proptosis, periorbital pain,
following severe injury
visual loss
3 stages: atrophia bulbi without shrinkage, atrophia bulbi Treatment: surgical d ebridem ent, am photericin B,
with shrinkage, atrophia bulbi with disorganization flucytosine, rifam pin (IV and oral)
(phthisis bulbi)
Increased risk of intraocular m alignancy
Vira l

Annual B scan to rule out m alignancy

Den gu e fever :
Flavivirus
75% with ocular soreness and pain on eye m ovem ent
Fin d in gs: cataract, retinal detachm ent, hypotony, corneal
edem a, globe shrinkage, intraocular hem orrhage, P a ra s it ic
inflam m ation, calcification, and disorganization
Tr ich in o sis:
Trichinella spiralis
Tr eatm en t: topical steroid and cycloplegic for pain; consider Larvae from ingestion of raw m eat m igrate to m uscle and
retrobulbar alcohol injection or enucleation for severe pain brain and becom e encysted
Associated with fever, m yalgia, diarrhea, eosinophilia,
and periorbital edem a
Infe c tio ns
Cysticer co sis:
Taenia solium
Ba c t e ria l Larvae penetrate intestinal wall, enter bloodstream , and
m igrate to eyes, lungs, brain, m uscle, and connective
Pr esep tal cellu litis/Or b ital cellu litis: (See Chapter 5,
tissue; becom es encysted
Pediatrics/Strabism us). In adults, preseptal m ost com m ly due
to cutaneous traum a, dacryocystitis

Infla m m a tio n
Necr o tizin g fasciitis:
Superficial and deep fascia; rapid evolution, potentially fatal Id io p a t h ic o rb it a l in fla m m a t io n
Organisms: group A ß-hem olytic Streptococcus m ost
(Orb it a l p s e u d o t u m o r)
com m on
Findings: anesthesia, disproportionate pain, rose to blue- Idiopathic inflam m atory disease of orbital tissues
grey skin discoloration, cutaneous necrosis, system ic shock
Treatment: early d ebridem ent, IV antibiotics Fin d in gs: acute orbital pain, lid erythem a and edem a,
(clindam ycin has activity against group A Streptococcus toxins), lacrim al gland enlargem ent, restricted eye m ovem ents,
adjuvant steroid therapy proptosis, diplopia, increased IOP; m ay have im paired vision
from optic nerve involvem ent
Fu n g a l
Ad u lts: usually unilateral; bilateral cases need workup for
Most com m only Phycomycetes system ic vasculitis and lym phoproliferative disorders

Mu co r m yco sis: Ch ild r en : bilateral in 33%; commonly have headache, fever,

Most com m on and virulent fungal orbital disease vomiting, and lethargy; may have associated papillitis or iritis;
Nonseptated, large, branching hyphae, with invasion of workup usually not needed (see Chapter 5, Pediatrics/
blood vessels, throm bosis, and necrosis Strabismus)
Black eschar m ay be visible in nose or palate
Intracranial spread is hem atogenous via ophthalm ic artery Path o lo gy: enlargement of extraocular muscles and tendons;
Risk factors: diabetes m ellitus (70%), im m unosuppression patchy infiltrate of lymphocytes, plasma cells, and eosinophils
(18%), renal disease (5%), leukem ia (3%)
Findings: painful orbital apex syndrom e, proptosis, ptosis, DDx: thyroid-related ophthalm opathy, orbital cellulitis,
ophthalm oplegia, decreased vision, corneal anesthesia; tum or, vasculitis, traum a, AV fistula, cavernous sinus
m ay develop retinal vascular occlusions; stains with H&E throm bosis, CN palsy
Treatment: requires im m ediate and em ergent m anagem ent
Surgical d ebridem ent, IV am photericin B; control Diagn o sis:
underlying illness Lab tests: eosinophilia, elevated ESR, positive ANA, CSF
Complications: intracranial involvem ent, death pleocytosis

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CHAPTER 6 • Orbit/Lids/Adnexa

CT scan: enlargem ent of extraocular m uscles and tendons,

ring sign (contrast enhanced sclera), enlarged
lacrim al gland
B-scan ultrasound: m ay show acoustically hollow area
corresponding to edem atous Tenon’s capsule

Tr eatm en t: system ic NSAIDs, system ic steroids (1 m g/kg

oral prednisone, taper slowly over m onths); biopsy for atypical
presentation, poor therapeutic response, recurrence; consider
radiation if unresponsive to steroids, chem otherapy
(cyclophospham ide)

Or b ital m yo sitis: localized to extraocular m uscles MR and

LR m ost com m only involved (33% each), IR 10%
Findings: diplopia, pain, proptosis, ptosis, conjunctival Fig ure 6-18. Thyroid-related ophthalmopathy demonstrating thickened
injection, and chem osis extraocular muscles with inflammatory cell infiltrate and fluid. (Courtesy of Dr. RC
Complications: fibrosis with strabism us Eagle, J r. Reported in Hufnagel TJ , Hickey WF, Cobbs WH et al:
Immunohistochemical and ultrastructural studies on the exenterated orbital
tissues of a patient with Graves’ disease, Ophthalmology 91:1411-9, 1984.)
To lo sa-Hu n t syn d r o m e: localized to superior orbital
fissure, optic canal, and cavernous sinus
Werner Classification of Eye Findings in Graves’ Disease
Findings: painful ophthalm oplegia, decreased vision
(m nem onic NO SPECS):
No signs or sym ptom s
Scler o sin g o r b ital p seu d o tu m o r : fibrosis of orbit and
Only signs
lacrim al gland
Soft tissue involvem ent (signs and sym ptom s)
Insidious onset
Proptosis
More steroid resistant; typically requires
Extraocular m uscle involvem ent
im m unom odulating therapy
Corneal involvem ent
Pain is less com m on, and eye is often white and quiet
Sight loss (optic nerve com pression)
Associated with retroperitoneal fibrosis
A-scan ultrasound: low reflectivity (seen with both orbital
pseudotum or and lym phom a) Path o lo gy: enlargem ent of extraocular m uscles; patchy
infiltrates of lym phocytes, m onocytes, m ast cells, and
fibroblasts; fibroblasts produce m ucopolysaccharides, which
Th yro id -re la t e d o p h t h a lm o p a t h y leads to increased water content of m uscles; inflam m ation
Autoim m une disease with spectrum of ocular m anifestations spares tendons (Fig. 6-18)
Most com mon cause of unilateral or bilateral proptosis in adults
CT scan : enlargem ent of extraocular m uscles sparing
Most com m on cause of acquired diplopia in adults tendons; inferior rectus > m edial rectus > superior
Wom en affected 8-10 Â m ore often than m en rectus > lateral rectus > obliques (IMSLO )

Patient can be hyperthyroid ($ 90%), hypothyroid ($ 5%), or

euthyroid ($ 5%); m ost com m only associated with Graves’
Tr eatm en t: control underlying thyroid abnorm ality; ocular
lubrication; consider lid taping or tarsorrhaphy; system ic
disease; increased incidence in Graves’ patients who sm oke
steroids; radiation (effects take 2–4 weeks)
(7 Â m ore likely)
Surgery:
Associated with m yasthenia gravis (in 5% of patients with ORBITAL DECOMPRESSION: perform before strabism us
Graves’ disease) surgery; 2, 3, or 4 walls; 80% will experience
postoperative diplopia
Usually asym ptom atic
STRABISMUS SURGERY: perform before lid surgery
INDICATIONS: diplopia, abnorm al head position,
Fin d in gs: eyelid retraction (90%), proptosis (63%), large-angle strabism us
restrictive m yopathy with diplopia; overaction of levator Strabism us m ust be stable for at least 6 m onths
(secondary to restrictive m yopathy of inferior rectus m uscle); Avoid if anterior inflam m atory signs present
pseudoretraction (secondary to proptosis); lagophthalm os; lid Recession of affected m uscles is m ainstay of
lag on downgaze (von Graefe’s sign); dry eye (due to corneal treatm ent, avoid resections (can exacerbate
exposure and infiltration of lacrim al glands); conjunctival restrictions already present)
injection and chem osis; corneal exposure (from com bination Vertical m uscle surgery can affect eyelid position
of eyelid retraction, proptosis, and lagophthalm os); increased (recession of inferior rectus can cause increased lid
IOP on upgaze; choroidal folds or disc hyperem ia; com pressive retraction due to connection of IR to lower lid
optic neuropathy (< 5%) with decreased acuity and color retractors; recession of IR can decrease upper lid
vision, RAPD, and VF defect retraction in that the SR now has to work less

174
 Orbital Disorders

against the tight IR [thus, the associated levator AV Fis t u la

muscle is less stimulated, causing less eyelid
Direct or indirect com m unication between previously norm al
retraction])
carotid artery and venous structures of the cavernous sinus
Beware late slippage of inferior rectus
Consider adjustable sutures
LID SURGERY: eyelid retraction repair; perform after Dir ect car o tid -caver n o u s sin u s fistu la: high flow;
orbital decom pression and strabism us surgery associated with head traum a, especially basal skull fracture;
due to spontaneous rupture of aneurysm
S a rc o id o s is Findings: dilated corkscrew scleral / episcleral vessels,
conjunctival injection and chem osis, elevated IOP on
(See Chapter 8, Uveitis) involved side, proptosis (m ay be pulsatile), orbital bruit
Chronic, idiopathic, m ultisystem , granulom atous disease that m ay be abolished by ipsilateral carotid com pression,
prim arily affecting lung, skin, eye dilated tortuous retinal veins; m ay develop ischem ic
m aculopathy or retinal artery occlusion, enlarged cup-to-
More com m on am ong young African Am ericans or disc ratio, ophthalm oplegia (often CN 6), anterior
Scandinavians segm ent ischem ia, blood in Schlem m ’s canal
CT/ MRI scan: dilated superior ophthalm ic vein
Fin d in gs: bilateral panuveitis, corneal abnorm alities Treatment (for severely affected patients): em bolization,
(thickening of Descem et’s m em brane, calcific band surgical ligation
keratopathy, num m ular keratitis, and deep strom al
vacuolation), pars planitis, chorioretinitis, optic nerve
Du r al-sin u s fistu la: low-flow com m unication between
involvement, orbital apex syndrome, ptosis, conjunctival
m eningeal branches of carotid artery and dural walls of
nodules, dacryoadenitis; most frequently involves lacrimal gland
cavernous sinus; often asym ptom atic; associated with
hypertension, atherosclerosis, and connective tissue diseases;
Path o lo gy: noncaseating granulom as and Langhans’
m ay close spontaneously; need MRI / MRA
giant cells

Diagn o sis: chest X-ray (CXR), purified protein derivative Tum o rs

(PPD) and controls, angiotensin-converting enzym e (ACE),
serum lysozym e, gallium scan, pulm onary function tests
(Table 6-6)

Tr eatm en t: system ic steroids, chem otherapy; treat ocular

Ham ar to m as: growth arising from tissue norm ally found at
com plications
that site (e.g., nevus, neurofibrom a, neurilem m om a,
schwannom m a, gliom a, hem angiom a, hem angiopericytom a,
We g e n e r’s g ra n u lo m a t o s is (Gra n u lo m a t o s is lym phangiom a, trichoepitheliom a)
w it h p o lya n g iit is )
System ic disease of necrotizing vasculitis and granulom atous Ch o r isto m as: growth arising from tissue not norm ally
inflam m ation involving sinuses, respiratory system , kidneys, found at that site (e.g., derm oid cyst, derm atolipom a, ectopic
and orbit lacrim al gland)

Cys t ic Tu m o rs
Fin d in gs: painful proptosis, reduced ocular m otility,
(See Chapter 5, Pediatrics/Strabism us)
chem osis, scleritis (25%), keratitis, optic nerve edem a,
nasolacrim al duct obstruction
Ta b le 6.6. Common orbital tumors
Path o lo gy: triad of vasculitis, granulom atous
In c hild re n* In a d ults
inflam m ation, and tissue necrosis
90% are benign; 10% are malignant Mucocele
Rhabdomyosarcoma (most common Cavernous hemangioma
Diagn o sis: antineutrophil cytoplasm ic antibodies (ANCA) primary orbital malignancy)
(positive in 67%) Capillary hemangioma (most common Meningioma
benign orbital tumor)
Tr eatm en t: system ic steroids, im m unosuppressive therapy Lymphangioma Fibrous histiocytoma
Neuroblastoma (most common Neurilemmoma
metastatic orbital tumor)
Co m p licatio n s: fatal if untreated Dermoid (most common orbital mass) Pleomorphic adenoma benign
mixed tumor (BMT)
Teratoma Lymphoid tumors
Va s c ula r Ab no rm a litie s Optic nerve glioma
Granulocytic sarcoma (“chloroma”)
Metastatic tumors

Burkitt’s lymphoma
Va rix Histiocytic tumors
*See Chapter 5, Pediatrics/Strabismus)
(See Chapter 5, Pediatrics/Strabism us)

175
CHAPTER 6 • Orbit/Lids/Adnexa

CT scan : well-circum scribed, encapsulated m ass

A-scan u ltr aso u n d : low to m edium internal reflectivity

Tr eatm en t: com plete surgical excision because it m ay

m etastasize

Ne u ra l Tu m o r s

Op t ic n e r ve g lio m a
(See Chapter 5, Pediatrics/Strabism us)

Ne u ro fib ro m a
(See Chapter 5, Pediatrics/Strabism us)
Fig ure 6-19. Cavernous hemangioma demonstrating large, blood-filled spaces
and fibrous septa. (Case presented by Dr. WC Frayer to the meeting of the Ne u rile m m o m a (S c h w a n n o m a )
Verhoeff Society, 1989. From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis,
2002, Mosby.) Encapsulated tum or consisting of benign proliferation of
Schwann cells
Va s c u la r Tu m o rs No m alignant potential

Lym p h a n g io m a Occurs in m iddle-aged individuals

(See Chapter 5, Pediatrics/Strabism us) Lesion can be painful due to perineural spread and
com pression of nerve
Ca p illa ry h e m a n g io m a Usually located in the superior orbit causing gradual proptosis
and globe dystopia
(See Chapter 5, Pediatrics/Strabism us)
Schwannom as can grow along any peripheral or cranial nerve,
Ca ve rn o u s h e m a n g io m a m ost com m only CN 8 (acoustic neurom a)

Most com m on benign orbital tum or in adults (usually m iddle- Rarely associated with neurofibrom atosis
aged wom en)
Path o lo gy: stains with S-100
Fin d in gs: slowly progressive proptosis; m ay induce Antoni A: spindle cells arranged in interlacing cords,
hyperopia, m ay also have retinal striae, IOP elevation, whorls, or palisades; contains Verocay bodies (collections
strabism us, or optic nerve com pression of cells resem bling sensory corpuscles) (Fig. 6-20)
Antoni B: loose, m yxoid; stellate cells with a m ucoid
Growth m ay accelerate during pregnancy strom a (Fig. 6-21)
CT scan : well-circum scribed, fusiform m ass
Path o lo gy: encapsulated lesion com posed of blood-filled
cavernous spaces, lined by endothelial cells (Fig. 6-19)
Tr eatm en t: com plete surgical excision; can recur after
CT scan : well-dem arcated, encapsulated, intraconal m ass com plete surgical rem oval

MRI: hypointense to fat on T1-weighted im ages, hyperintense

to fat and equivalent to vitreous on T2-weighted im ages

A-scan u ltr aso u n d : high internal reflectivity

Tr eatm en t: observation, surgical excision

He m a n g io p e ric yt o m a
Rare tum or of abnorm al pericytes surrounding blood vessels
Occurs in m iddle-aged adults
More com m on in wom en
Often located in superior orbit
Can m etastasize to lung, bone, liver
Fig ure 6-20. Antoni A pattern demonstrating palisading spindle cells with
Fin d in gs: slowly progressive proptosis, pain, decreased Verocray bodies (areas that appear acellular). (From Yanoff M, Fine BS: Ocular
vision, diplopia pathology, ed 5, St Louis, 2002, Mosby.)

176
 Orbital Disorders

All lym phoid lesions of orbit m ust have a workup for system ic
lym phom a, including CBC with differential, serum protein
electrophoresis (SPEP), physical exam ination for
lym phadenopathy, CT of thoracic and abdom inal viscera, and
bone scan
Perform every 6 m onths for 2 years
Bone m arrow biopsy (better than bone m arrow aspirate)

Be n ig n re a c t ive lym p h o id h yp e rp la s ia
Fin d in gs: bilateral painless lacrim al gland enlargem ent,
lim itation of ocular m otility, visual disturbances

Fig ure 6-21. Antoni B pattern demonstrating necrosis with inflammatory

Path o lo gy: m ature lym phocytes with reactive germ inal
cells. (From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.) centers; T cells, 60–80% with scattered polyclonal B cells; high
degree of endothelial cell proliferation
Me n in g io m a
Op tic n er ve sh eath m en in gio m a: At yp ic a l lym p h o id h yp e rp la s ia
More com m on in wom en Low-grade lym phom a, no m itotic activity
Accounts for 33% of all prim ary optic nerve tum ors
Increased incidence in neurofibrom atosis Path o lo gy: follicles and polym orphous response consistent
Arises from arachnoid cells in the m eninges with benign process
Findings: decreased visual acuity and color vision, visual
field loss, proptosis, disc edem a or pallor, optociliary 40% develop system ic disease within 5 years
shunt vessels
Spencer’s triad: optociliary shunts, optic atrophy, optic
Orb it a l lym p h o m a
nerve m eningiom a
Pathology: sheets of cells or whorls; psam m om a bodies Prim ary orbital lym phom a and usually involves superior orbit
(calcified) in the center of whorls (only Burkitt’s occurs in children)
CT scan: tubular enlargem ent of optic nerve that enhances 50% are m ucosa-associated lym phoid tissue (MALT), 15% to
(railroad track sign); optic foram en enlargem ent 20% of MALT transform to m ore aggressive form
Treatment: observation, surgical excision to prevent
Secondary orbital lym phom a is typically interm ediate of high
involvem ent of optic nerve or chiasm
grade
Sp h en o id win g m en in gio m a:
Most com m on tum or to spread to orbit from intracranial Fin d in gs: lim itation of ocular m otility, painless lacrim al
space gland swelling, conjunctival salm on patches, visual changes;
Findings: tem poral fullness, proptosis, lid edem a 17% bilateral
CT scan: hyperostosis and calcifications
Treatment: observation, surgical excision Path o lo gy: atypical im m ature lym phocytes with m itoses;
diffuse or follicular growth; m onoclonal B-cell proliferations
60–90% with scattered or reactive T cells; involves
Rh a b d o m yo s a rc o m a
reticuloendothelial system , including retroperitoneal lym ph
(See Chapter 5, Pediatrics/Strabism us)
nodes (Fig. 6-22)

Lym p h o id Tu m o r s
Spectrum of disorders characterized by abnorm al proliferation CT scan : putty-like m olding of tum or; orbital tissues not
of lym phoid tissue displaced

10% of all orbital tum ors; 90% are non-Hodgkins low-grade Tr eatm en t: radiotherapy for localized orbital disease,
B-cell lym phom a chem otherapy for system ic involvem ent
Usually occur in adults 50–70 years old; rare in children Pr o gn o sis: 50% risk of system ic involvem ent; lower for
Wom en > m en (3:2) MALT; location appears m ore im portant than histopathology
for determ ining system ic involvem ent: lym phom a of eyelids
Frequency of periocular involvem ent: orbit (up to 75%), (67% have system ic involvem ent) > orbit (35%) > conjunctiva
conjunctiva (20-33%), eyelid (5-20%) (20%); 90% 5-year survival
Cause painless proptosis
Tissue biopsy with im m unohistochem ical studies required for P la s m a c yt o m a
diagnosis Tum or com posed of plasm a cells

177
CHAPTER 6 • Orbit/Lids/Adnexa

Os s ifyin g fib ro m a
Variant of fibrous dysplasia
Occurs in second and third decades of life
More com m on in wom en
Well-circum scribed, slow-growing, m onostotic lesion

Path o lo gy: vascular strom a containing lam ellar bone with a

rim of osteoid and osteoblasts

Os t e o m a
Dense bony lesions originating in the frontal and ethmoid sinus
Well-circum scribed, slow-growing m ass
A Sym ptom s secondary to sinus obstruction and intracranial or
intraorbital extension

Path o lo gy: lam ellar bone with variable am ounts of fibrous

strom a

Fib ro u s h is t io c yt o m a
Firm orbital m ass com posed of fibroblasts and histiocytes
Usually benign (m alignant in 10%)
Distinguished from hem angiopericytom a only on biopsy
Most com m on m esenchym al orbital lesion of adults

Path o lo gy: storiform (cartwheel or spiral), nebular pattern

B of tum or cells (fibroblasts); both fibrous histiocytom a and
hem angiopericytom a are spindle cell tum ors, and they can be
Fig ure 6-22. A, Nodular lymphoid infiltrate. B, Higher magnification shows uniform difficult to differentiate (Fig. 6-23)
lymphocyte infiltrate with mitotic figures. (From Yanoff M, Scheie HG: Malignant
lymphoma ofthe orbit – difficulties in diagnosis, SurvOphthalmol12:133-140, 1967.)

CT scan : well-circum scribed m ass anywhere in the orbit

Path o lo gy: plasm acytoid lym phocytes, im m unoblasts, (upper nasal m ost com m on)
m ature plasm a cells
Tr eatm en t: surgical excision; m oderate risk of recurrence
DDx: Waldenstro€ m ’s m acroglobulinem ia, m ultiple
m yelom a, lym phom a with im m unoglobulin production
His t io c yt o s is X
Gra n u lo c yt ic s a rc o m a (“Ch lo r o m a ”) (Langerhans cell histiocytosis) (See Chapter 5, Pediatrics/
(See Chapter 5, Pediatrics/Strabism us) Strabism us)

Bu rkit t ’s lym p h o m a
(See Chapter 5, Pediatrics/Strabism us)

S ys t e m ic lym p h o m a a n d Wa ld e n s t r o€ m ’s
m a c r o g lo b u lin e m ia
Solid infiltrating tum or with putty-like m olding of tum or to
pre-existing structures

Path o lo gy: Dutcher bodies (intranuclear PAS-positive

inclusions of im m unoglobulin)

Fib ro -o s s e o u s Tu m o rs
Fig ure 6-23. Histiocytes (on left) and fibrous (on right, with storiform [matted]
Fib ro u s d ys p la s ia appearance) components. (From J ones WD 3rd, Yanoff M, Katowitz J A:
(See Chapter 5, Pediatrics/Strabism us) Recurrent facial fibrous histiocytoma, Br J Plast Surg 32:46-51, 1979.)

178
 Orbital Disorders

J u ve n ile xa n t h o g ra n u lo m a (J XG; Tr eatm en t: com plete en bloc excision without biopsy;

n e vo xa n t h o e n d o t h e lio m a ) incom plete excision m ay result in recurrence and m alignant
transform ation into pleom orphic adenocarcinom a
(See Chapter 5, Pediatrics/Strabism us)
P le o m o rp h ic a d e n o c a rc in o m a
Ad u lt -o n s e t xa n t h o g ra n u lo m a
(Ma lig n a n t m ixe d t u m o r)
Often associated with system ic m anifestations Long history of m ass in lacrim al fossa
4 syndrom es (in order of frequency):
1. Necrobiotic xanthogranulom a (NBX) Occurs in elderly individuals
2. Adult-onset asthm a with periocular Fin d in gs: rapid, progressive, painful proptosis
xanthogranulom a (AAPOX)
3. Erdheim -Chester disease (ECD) Path o lo gy: sim ilar to benign m ixed tum or but with foci of
4. Adult-onset xanthogranulom a (AOX) m alignant change

Ep it h e lia l La c rim a l Gla n d Tu m o r s Tr eatm en t: radical orbitectom y and bone rem oval
50% of lacrim al gland lesions are inflam m atory and
lym phoproliferative; contour around the globe Ad e n o id c ys t ic c a rc in o m a
50% of lacrim al gland tum ors are of epithelial origin Most com m on m alignant tum or of the lacrim al gland
50% of epithelial tum ors are benign pleom orphic adenom as
Highly m alignant
50% of m alignant tum ors are adenoid cystic carcinom as
Presents in 4th decade of life
P le o m o rp h ic a d e n o m a (Be n ig n m ixe d t u m o r ) Rapidly progressive proptosis, pain and paresthesia due to
Most com m on epithelial tum or of the lacrim al gland perineural invasion and bony destruction

Occurs in 4th–5th decade of life Path o lo gy: sm all, benign-appearing cells arranged in nests,
More com m on in m en tubules, or in a ”Swiss-cheese” (cribriform ) pattern (Fig. 6-25)
Slow onset (6–12 m onths) CT scan : poorly circum scribed m ass, bony destruction,
Firm m ass in lacrim al fossa with painless proptosis; globe often calcifications
displaced m edially and downward
Tr eatm en t: rem oval of any bone that is involved,
Progressive expansile growth m ay indent bone of lacrim al fossa exenteration, adjunctive radiation and chem otherapy
Tum or growth stim ulates periosteum to deposit a thin layer of
new bone (cortication) Pr o gn o sis: poor; survival rate of 20–70%
S in u s Tu m o rs
Path o lo gy: proliferation of epithelial cells into a double
layer, form ing lum ina with ductal and secretory elem ents; S in u s m u c o c e le
ductal inner cells secrete m ucus; outer strom al cells give rise to
Cystic, slowly expanding sinus lesion
fibrous strom a and osteoid and cartilaginous m etaplasia,
pseudoencapsulated with surface bosselations (Fig. 6-24) Entrapm ent of m ucus in aerated space due to obstruction of
sinus ostia
CT scan : well-circum scribed but m ay have nodular Exerts pressure on surrounding bony structures
configuration

Fig ure 6-24. Benign mixed tumor (BMT) demonstrating ductal structures in a Fig ure 6-25. Adenoid cystic carcinoma demonstrating characteristic “Swiss-
myxoid stroma. (From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, cheese” appearance. (From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis,
2002, Mosby.) 2002, Mosby.)

179
CHAPTER 6 • Orbit/Lids/Adnexa

May becom e infected (m ucopyocele) P ro s t a t e c a rc in o m a

Causes of ostial obstruction: inflam m ation with scarring, May present sim ilarly to acute pseudotum or
congenital narrowing of ostia, traum a, osteom as, polyps,
Less aggressive than lung carcinom a
septal deviation, m ucus retention cysts
Associated with cystic fibrosis
Must rule out encephalocele and m eningocele EYELID DISORDERS
Findings:
Frontoethmoid sinus (m ost com m on location): outward
Co ng e nita l Ano m a lie s
and downward displacem ent of globe, fullness in
superonasal and m edial canthal regions, above the
(See Chapter 5, Pediatrics/Strabism us)
m edial canthal tendon
Sphenoid and posterior ethmoid sinus: visual sym ptom s,
retrobulbar pain; m ay have cranial nerve palsies; 50% Tra um a
have nasal sym ptom s
Maxillary sinus (rare): upward displacem ent of globe,
Lid la c e ra t io n
erosion of orbital floor m ay cause enophthalm os
Partial- or full-thickness cut in eyelid that m ay involve the lid
CT scan : hom ogeneous opacified cyst with bowing of sinus m argin, canthus, or canaliculus
wall and attenuation/erosion of bone
Tr eatm en t: antibiotics, tetanus booster; surgical repair;
MRI: highly variable signal intensities technique depends on severity and location of injury
Tr eatm en t: surgical excision with IV antibiotics; reestablish
norm al drainage; obliteration of sinus (only if frontal) Lid a vu ls io n
Com plete or partial tearing of eyelid with or without tissue loss
S in u s c a rc in o m a
Usually squam ous cell
Tr eatm en t: technique of repair depends on severity and
location of injury
Most com m only from m axillary sinus Small defect (< 25%): direct closure
Moderate defect (25–50%): Tenzel’s flap
No early signs except for sinusitis
Large defect (> 50%): bridge flap reconstruction (Cutler-
Late fin d in gs: nonaxial proptosis, epiphora, epistaxis, Beard [for upper lid] or Hughes’[for lower lid] procedure
infraorbital anesthesia with flap advancem ent)

Me t a s t a t ic Tu m o rs
In contrast to adults, pediatric tum ors m etastasize to the orbit Infla m m a tio n
m ore frequently than to the uvea
Orbital m etastases produce rapid painful proptosis with Ho rd e o lu m
restricted ocular m otility
Obstruction and infection of Zeiss (external hordeolum , stye)
Ne u r o b la s t o m a or m eibom ian (internal hordeolum , chalazion) glands

(See Chapter 5, Pediatrics/Strabism us) Painful erythem atous eyelid swelling

Accum ulation of secretions causes acute inflam m atory
Le u ke m ia
response
(See Chapter 5, Pediatrics/Strabism us)
Lipogranulom a form ation (chalazion) often evolves from
Ew in g ’s s a rc o m a internal hordeolum

(See Chapter 5, Pediatrics/Strabism us) Path o lo gy: epithelioid and giant cells surrounding em pty
lipid vacuoles, zonal granulom atous inflam m ation (zonal
Bre a s t c a r c in o m a lipogranulom a) (Fig. 6-26)
Most com m on prim ary source of orbital m etastasis in wom en
Tr eatm en t: warm compresses, topical antibiotic ointm ent, lid
May occur m any years after prim ary diagnosis scrubs (for associated blepharitis); if no response, consider local
May elicit a fibrous response and cause enophthalm os and steroid injection, or incision and curettage (subm it specimen
ophthalm oplegia to pathology, especially for atypical or recurrent lesions)
May respond to horm onal m anipulation
Ble p h a rit is
Lu n g c a r c in o m a Inflam m ation of lid m argin
Most com m on prim ary source of orbital m etastasis in m en
An ter io r lid m ar gin d isease: seborrheic or staph
Very aggressive blepharitis

180
 Eyelid Disorders

acids), topical azithrom ycin, lubrication (for associated dry

eye); topical m etronidazole to facial skin; consider topical
steroid

Tr eatm en t: Lid scrubs, oral tetracycline/doxycycline, oral

fish oil (om ega-3 fatty acids), lubrication (for associated dry
eye), topical m etronizadole to facial skin

Co n t a c t d e rm a t it is
Inflam m ation of lid skin due to exogenous irritant or allergic
hypersensitivity reaction

Sym p to m s: red, swollen, itchy periorbital skin

Fig ure 6-26. Chalazion demonstrating epithelioid and giant cells surrounding
clear areas that contained lipid. (From Yanoff M, Fine BS: Ocular pathology, Fin d in gs: erythem atous, scaling lesions; m ay have vesicles
ed 5, St Louis, 2002, Mosby.) or weeping areas

Po ster io r lid m ar gin d isease: m eibom itis, Meibom ian Tr eatm en t: elim inate inciting agent; use m ild steroid cream
gland dysfunction (MGD) or tropical tacrolim us 0.1% (Protopic)

An gu lar b lep h ar itis: associated with Moraxella

Infe c tio ns
Sym p to m s: burning, itching, redness, tearing
Mo llu s c u m c o n t a g io s u m
Fin d in gs: lid thickening, crusting, scurf, collarettes, clogged Shiny, white-yellow papule with central um bilication
m eibom ian glands, thickened/cloudy m eibum , foam y tears,
lash loss, redness; decreased tear breakup tim e (< 10 seconds) Infection by the poxvirus MCV (m olluscum contagiosum
in MGD virus)

Associated with dry eye, acne rosacea, and chalazia Spread by direct contact; consider HIV in healthy adult
Usually asym ptom atic
Tr eatm en t: lid scrubs, topical antibiotic ointm ent,
lubrication (for associated dry eye), oral supplem ents (om ega- Can cause follicular conjunctivitis and punctate keratitis
3 fatty acids): consider oral tetracycline/doxycycline, topical
azithrom ycin, topical steroid Path o lo gy: lobular acanthosis, large basophilic poxviral
intracytoplasm ic inclusions com posed of nucleic acids from
Ac n e ro s a c e a DNA virus (Fig. 6-27)

Idiopathic, chronic skin disorder affecting sebaceous glands of

face (including m eibom ian glands)
Type IV hypersensitivity m ay play a role
Com m on between ages 40 and 60 years
Ocular involvem ent in > 50%

Fin d in gs: chronic blepharitis, m eibom itis, lid m argin

telangiectasia, conjunctival injection, abnorm al tear film
(foam , m ucin particles, excess oil), decreased tear breakup
tim e, m eibom ian gland plugging, recurrent chalazions; m ay
develop keratitis with peripheral corneal infiltrates,
vascularization, scarring, thinning, ulceration, and rarely
perforation

Oth er fin d in gs: acne-like skin changes, telangiectatic

vessels, papules, and rhinophym a

Path o lo gy: granulom atous inflam m ation

Fig ure 6-27. Intracytoplasmic, eosinophilic molluscum bodies in epidermis; they
become larger and more basophilic near the surface. (Courtesy of Dr. WC
Tr eatm en t: warm com presses and lid scrubs, oral Frayer. From Skin and lacrimal drainage system. In Yanoff M, Fine BS: Ocular
doxycycline or erythrom ycin, oral fish oil (om ega-3 fatty pathology, ed 5, St Louis, 2002, Mosby.)

181
CHAPTER 6 • Orbit/Lids/Adnexa

Tr eatm en t: surgical excision, cryo, incision, and curettage Etiology unknown but m ay be due to abnorm ality of basal
ganglia
P h t h ir ia s is p a lp e b ru m / p e d ic u lo s is Usually occurs in 5th–7th decade of life; fem ale > m ale (3 :1);
Lice infection of lashes associated with Parkinson’s disease

Spread by direct contact, usually sexually transm itted Absent during sleep

Produces blepharoconjunctivitis Meige’s syn d r o m e: blepharospasm , cervical dystonia, and

facial orom andibular dystonia (involuntary jaw and tongue
Fin d in gs: follicles, injection, sm all white eggs (nits) and lice contractions)
attached to lashes
DDx: secondary blepharospasm (due to ocular irritation),
Tr eatm en t: rem ove nits and lice, topical ointm ent to hem ifacial spasm (usually unilateral, due to com pression
suffocate lice, delousing cream s and sham poo of CN 7, can be caused by cerebellopontine angle [CPA]
tum or, present during sleep), m yokym ia, Tourette’s
Ve rru c a vu lg a ris (P a p illo m a ) syndrome, trigem inal neuralgia, basal ganglia disease,
tardive dyskinesia
Pink, pedunculated or sessile m ass
Consider CT/ MRI scan to rule out posterior fossa lesion
Associated with papillom avirus (HPV)
Usually asym ptom atic Tr eatm en t: Botox (botulinum toxin type A) injections,
Often m ultiple and resolve spontaneously surgery (excision of lid protractors or differential section of
CN 7), m edications (tetrabenazine, lithium , carbidopa,
clonazepam ); also deep brain stim ulation for Meige’s
He rp e s in fe c t io n
syndrom e
Her p es sim p lex vir u s (HSV): Botox: derived from Clostridium botulinum, freeze-dried
Prim ary infection causes vesicular derm atitis solution; subcutaneous injection, no m ore than 200
May be associated with follicular conjunctivitis and keratitis units/m onth
Treatment: cold com presses, antibiotic ointm ent to skin MECHANISM: blocks neurom uscular conduction by
lesions, system ic antiviral; topical antiviral if eye binding to receptors on nerve term inals and stops
involved release of ACh by disrupting calcium m etabolism ;
does not cross blood–brain barrier
Var icella zo ster vir u s (VZV): INDICATIONS: blepharospasm (effective in 90%, lasts
Vesicular derm atitis with ulceration, crusting, and scarring 3 m onths), hem ifacial spasm (lasts 4 m onths),
Reactivation of latent infection along distribution of strabism us (lasts 1–8 weeks)
trigem inal nerve (m ost often 5 1 ) ADVERSE EFFECTS (no system ic toxicity): exposure
Derm atom al distribution, does not cross m idline keratopathy, ptosis, diplopia, flaccid lower lid
May have constitutional sym ptom s and ocular involvem ent ectropion
Treatment: cold com presses, antibiotic ointm ent to skin
lesions, system ic antiviral; (see Chapter 7, Cornea/
Ble p h a ro c h a la s is
External Disease) for ocular involvem ent), m ay require
treatm ent of postherpetic neuralgia (PHN) Idiopathic inflam m atory edem a of the eyelids
Fam ilial, m ost often in young wom en
Le p ro s y
Recurrent attacks of transient, painless eyelid edem a resulting
Organism is acid-fast bacillus Mycobacterium leprae in atrophy, wrinkling, and redundancy of eyelid skin
Tuberculoid and leprom atous leprosy can affect lids May develop ptosis and herniation of orbital lacrim al gland

Fin d in gs: loss of lashes, trichiasis, ectropion, and exposure

De rm a t o c h a la s is
keratitis
Redundancy of eyelid skin with orbital fat prolapse due to
Tr eatm en t: system ic antibiotics (dapsone, rifam pin) involutional changes

Ma lp o s itio n a nd Othe r Dis o rd e rs Ble p h a ro p t o s is (Ac q u ire d )

Eyelid m alposition characterized by drooping upper eyelid
Ble p h a ro s p a s m
Myo gen ic:
Bilateral, interm ittent, involuntary contractions of orbicularis uncom m on; due to local or system ic m uscular disease
and facial m uscles causing uncontrolled blinking; m ay cause (e.g., m yotonic dystrophy, chronic progressive external
functional blindness ophthalm oplegia, m yasthenia gravis)

182
 Eyelid Disorders

Treat underlying condition; usually requires frontalis sling DDx: thyroid-related ophthalm opathy (m ost com m on,
lateral upper eyelid m ore retracted than m edial eyelid, fibrous
In vo lu tio n al (aponeurotic): contraction of eyelid retractors), orbital pseudotum or
m ost com m on form of ptosis; disinsertion of levator (idiopathic orbital inflam m ation [IOI]), pharm acologic
aponeurosis (phenylephrine, α-agonists, cocaine), resection of superior
due to aging or chronic inflam m ation; m ay be exacerbated by rectus, overcorrection of ptosis, contralateral ptosis
eye surgery or traum a (Hering’s law)
High eyelid crease with good levator function; ptotic lid in all
positions of gaze (no lid lag on downgaze as in Tr eatm en t: lubrication; surgical repair (levator
congenital cases); m ay have thinning of eyelid above aponeurosis recession, levator m yotom y, spacer insertion,
tarsal plate full-thickness skin graft for lower eyelid, hard palate grafts
for lower eyelid)
Neu r o gen ic:
due to CN 3 palsy, aberrant regeneration of CN 3 (Marcus
Gunn jaw-winking ptosis), Horner’s syndrom e, m ultiple
Ec t ro p io n
sclerosis, ophthalm oplegic m igraine Eversion of eyelid m argin; m ay cause keratinization and
Treatment for Horner’s: shorten Mu€ ller’s m uscle hypertrophy of conjunctiva
(Putterm an [conjunctival-Mu€ ller’s m uscle resection]) or
Fasanella-Servat [tarsoconjunctival resection] procedure)
Sym p to m s: tearing, foreign body sensation, redness
Mech an ical:
due to m ass effect of orbital or eyelid tum ors, derm atochalasis, In vo lu tio n al:
blepharochalasis, cicatrix m ost com m on cause of ectropion; often in lower eyelid
Etiology: horizontal laxity, disinsertion of lower eyelid
Tr au m atic: retractors
due to traum a to levator aponeurosis; m ay have Diagnosis: snap-back test, distraction test
lagophthalm os from cicatricial changes Treatment: horizontal eyelid shortening (lateral tarsal
strip), lateral canthoplasty, repair of lower eyelid
May im prove spontaneously, therefore observe for 6 m onths retractors

Exam in atio n : Par alytic:

Palpebral fissure height (PF): norm ally 10 to 11 m m in occurs after CN 7 palsy
prim ary gaze Frequent com plaints of tearing from chronic reflex secretion
Marginal reflex distance (MRD): MRD1 is distance between Treatment: lubrication, taping of tem poral lower eyelid,
corneal light reflex and upper lid m argin in prim ary m oisture cham ber goggles, lateral tarsorrhaphy,
gaze; norm ally 4 m m . MRD2 is distance to lower lid horizontal tightening procedure, hard palate m ucosal
m argin graft for lower eyelid elevation, gold weight im plantation
Levator function (LF): distance of upper lid excursion while to aid in closure of upper eyelid
frontalis is im m obilized; norm al > 12 m m , fair if 6 to
11 m m , poor if < 5 m m
2.5% phenylephrine test: activates sym pathetic fibers of Cicatr icial: shortening of anterior lam ella
Etiology: burns, traum a, tum or, infection, chronic
Mu€ ller’s m uscle; resulting lid elevation sim ulates
inflam m ation causing secondary anterior lam ellar
position after Mu€ ller’s m uscle resection
contraction (acne rosacea, atopic derm atitis, eczem a,
herpes zoster derm atitis, scleroderm a, epiderm olysis
DDx: pseudoptosis (nanophthalm os, m icrophthalm os,
bullosa, porphyria, xeroderm a pigm entosum )
phthisis bulbi, hypotropia, derm atochalasis, enophthalm os,
Treatment: revision and relaxation of cicatrix, horizontal
contralateral eyelid retraction or proptosis)
tightening procedure, m ay require vertical lengthening
with full-thickness graft
Tr eatm en t: eyelid crutches, levator aponeurosis
advancem ent, levator m uscle resection, Mu€ ller’s m uscle
resection, frontalis suspension with autogenous fascia lata, Mech an ical:
banked fascia lata, or synthetic m aterials Etiology: tum ors of eyelid, herniated orbital fat, poorly
fitted spectacles, chronic edem a
Su r gical co m p licatio n s: overcorrection or Treat underlying condition
undercorrection, lid lag, lagophthalm os, exposure keratopathy
En t ro p io n
Eye lid re t r a c t io n Inversion of eyelid m argin
Upper eyelid at or above superior lim bus Lower eyelid entropion usually involutional
Lower eyelid exposing sclera Upper eyelid entropion usually cicatricial

183
CHAPTER 6 • Orbit/Lids/Adnexa

Sym p to m s: tearing, foreign body sensation, redness Flo p p y e ye lid s yn d ro m e

Co n gen ital: Easily everted upper eyelids

Very rare; due to epiblepharon or tarsal kink (see Chapter 5, Chronic papillary conjunctivitis due to lid autoeversion during
Pediatrics/Strabism us) sleep with resultant m echanical irritation from bed sheets
Usually does not require treatm ent
Associated with obesity, keratoconus, eyelid rubbing, and
Sp astic: sleep apnea
Associated with ocular inflam m ation, traum a, and
prolonged patching Tr eatm en t: lubrication, tape/patch/shield lids during sleep;
Squeezing of eyelids causes inward rolling of eyelid m argin; consider surgical correction (upper eyelid lateral tarsal strip,
ocular surface irritation perpetuates cycle m edial and lateral canthal plication, or m edial tarsal strip;
Treatment: taping of eyelid, cautery, Botox injection, wedge excision is less effective)
Quickert suture

In vo lu tio n al: Ma d a r o s is
Etiology: canthal tendon laxity (horizontal lid laxity, Loss of eyelashes and/or eyebrows due to local or system ic
diagnose with snap-back test), eyelid retractor dehiscence disorders
(vertical lid laxity), overriding preseptal orbicularis
m uscle, involutional enophthalm os
Treatment: Quickert suture, horizontal lid-shortening
DDx: eyelid neoplasm s, chronic blepharitis, traum a, burns,
trichotillom ania, alopecia, seborrheic derm atitis,
procedure (Bick, lateral tarsal strip, m arginal wedge
chem otherapy agents, m alnutrition, lupus, leprosy
resection), vertical lid-shortening procedure (Jones,
Hotz, Wies m arginal rotation), retractor advancem ent,
excision of preseptal orbicularis P o lio s is / vit ilig o
Prem ature whitening of the eyelashes / eyebrows (poliosis) or
Cicatr icial: Shortening of posterior lam ella skin (vitiligo) due to local system ic disorders
Etiology: ocular cicatricial pem phigoid, Stevens-Johnson
syndrom e, trachom a, herpes zoster derm atitis, surgery, Associated with Vogt-Koyanagi-Harada syndrom e,
traum a, chem ical burns, m iotics sym pathetic ophthalm ia, Waardenburg syndrom e, tuberous
Digital pressure on inferior border of tarsus corrects eyelid sclerosis, radiation, and derm atitis
position in involutional entropion but not cicatricial
entropion
Treatment: lubrication, avoid surgery during acute phase of Eye lid Tum o rs
autoim mune disease, rem ove lashes in contact with
cornea, use tarsal fracture operation, apply
Be n ig n Ep it h e lia l Tu m o rs
tarsoconjunctival grafts or hard palate m ucosal grafts to
replace scarred tarsus; m ay require sym blepharon ring
S q u a m o u s p a p illo m a
and am niotic m em brane graft to prevent recurrent
scarring Keratinized epiderm al fronds with fibrovascular cores
Most com m on benign lesion of eyelid
Tric h ia s is
Associated with papovavirus (HPV) infection
Misdirection of eyelashes and contact with ocular surface
May be sessile or pedunculated
Etiology: OCP, Stevens-Johnson syndrom e, chronic blepharitis,
burns, traum a, trachom a, herpes zoster, vernal Path o lo gy: papillary configuration, proliferating
keratoconjunctivitis fibrovascular tissue covered by hyperplastic prickle cell layer of
the epiderm is, hyperkeratosis and parakeratosis m ay be
present, vacuolated cells containing virus particles m ay be seen
DDx: Pseudotrichiasis (entropion, epiblepharon) or in the upper squam ous layer (Fig. 6-28)
distichiasis (abnorm al growth of lashes from Meibom ian
gland orifices) Tr eatm en t: surgical excision, cryo or laser ablation

Sym p to m s: tearing, foreign body sensation, redness S e b o rrh e ic ke ra t o s is

Papillom atous proliferation of suprabasalar (prickle) cells
Tr eatm en t: lubrication, epilation, electrolysis,
cryodestruction with double-freeze/thaw technique, Pigm ented keratin crust with greasy ”stuck-on” appearance
radiofrequency or laser (argon, diode, or ruby) ablation, Occurs in elderly
trephination, full-thickness wedge resection, tarsal fracture, or
entropion repair; oral azithryom ycin to reduce recurrence in Path o lo gy: acanthosis, hyperkeratosis, parakeratosis, and
cases of trachom a squam ous eddies m ay be present (Fig. 6-29)

184
 Eyelid Disorders

Trapped but viable epithelium proliferates and produces

keratin
Rupture can cause a foreign body granulom atous reaction

De rm o id c ys t
Lined by keratinized squam ous epithelium and derm al
appendages such as hair shafts and sebaceous glands
Contains keratin, cilia, and sebum
Often congenital; m ost com m on orbital tum or in children
Can be found in eyelid or orbit

Hyd ro c ys t o m a (S u d ifo ro u s c ys t )
Fig ure 6-28. Squamous papilloma demonstrating acanthosis and Multilocular, branching lum en that appears em pty or contains
hyperkeratosis. (From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis,
2002, Mosby.)
serous fluid
Lined by a double row of cuboidal epithelium (resem bling
sweat duct)
Most are eccrine hydrocystom as (can be apocrine if arising
from the glands of Moll)
Occur m ost com m only at eyelid m argin or lateral canthus

S e b a c e o u s Gla n d Tu m o rs

Co n g e n it a l s e b a c e o u s g la n d h yp e rp la s ia
Proliferation of norm al sebaceous glands
20% degenerate into basal cell carcinom a

Ac q u ire d s e b a c e o u s g la n d h yp e rp la s ia
Multiple well-circum scribed yellow nodules
Fig ure 6-29. Seborrheic keratosis appears as blue lesion above skin surface
with blue basaloid cells and keratin cysts. (From Yanoff M, Fine BS: Ocular
Lobules of m ature sebaceous glands around a dilated duct
pathology, ed 5, St Louis, 2002, Mosby.)
Mu ir-To rre s yn d ro m e
Multiple sebaceous neoplasm s, keratoacanthom as, and
In ve rt e d fo llic u la r ke ra t o s is visceral tum ors (especially GI)

Nodular or verrucous lesion S w e a t Gla n d Tu m o rs

Looks like a cutaneous horn S yrin g o m a
Inflam ed seborrheic keratosis Waxy, yellow nodules on lower lid

Path o lo gy: acanthosis, squam ous and basal cell Usually occur in young wom en
proliferation Benign proliferation of eccrine ductal structures

Cys t s Hyd ro c ys t o m a
Epithelial lined cham bers filled with debris
Translucent, bluish cyst

Ep id e rm a l in c lu s io n c ys t (Ep id e rm o id c ys t ) Arises from eccrine or apocrine glands

Single-lum en, round or oval lesion lined by keratinized, Tu m o rs o f Ha ir Fo llic le Orig in

stratified squam ous epithelium (epiderm is) and filled with
Tric h o e p it h e lio m a
cheesy keratin debris
Firm , skin-colored nodule
Occurs after a fragm ent of epiderm is is carried into the
subepithelium by traum a More com m on in wom en

185
CHAPTER 6 • Orbit/Lids/Adnexa

Usually occurs on forehead, eyelids, nasolabial fold, and

upper lip

Path o lo gy: basaloid cells surrounding a keratin center

Mu ltip le lesio n s: Brooke’s tum or (AD)

Tric h o fo llic u lo m a
A keratin-filled dilated cystic hair follicle, surrounded by
im m ature hair follicles
Appears as a sm all um bilicated nodule usually with central
white hairs

Tric h o le m m o m a
Sm all crusty lesion with rough ulcerated surface
Usually occurs on face
Arises from glycogen-rich clear cells of the outer hair sheath
May resem ble basal, squam ous, or sebaceous gland carcinom a

Co wd en ’s d isease (AD): m ultiple facial tricholem m om as;

m arker for breast (40%) or thyroid cancer
Fig ure 6-30. Actinic keratosis appears as pink papillomatous lesion with marked
hyperkeratosis and acanthosis about skin surface. (From Yanoff M, Fine BS:
Ocular pathology, ed 5, St Louis, 2002, Mosby.)
P ilo m a t rixo m a (“Ca lc ifyin g e p it h e lio m a
o f Ma lh e r b e ”) Tr eatm en t: surgical excision, cryo or freezing with liquid
Solitary, firm , deep nodule with overlying norm al, pink, or nitrogen
bluish skin Ne vu s
Freely m ovable subcutaneous pink-purple nodule Congenital or acquired ham artom a
Most com m on cystic lesion of childhood Can be flat, but is usually elevated and pigm ented
In young adult, arises from hair m atrix of upper lid or brow Arises from neural crest cells

Occurs on the eyelid, face, neck, or arm s Pigm entation and size tend to increase during puberty

Can range from 5 to 30 m m in diam eter With tim e, nevi tend to m ove deeper, m igrating into the derm is
Contains benign-appearing derm al m elanocytes
May resem ble an epiderm al cyst
Malignant transform ation is rare
Associated with m yotonic dystrophy and Gardner’s syndrom e
Classified by location
P re c a n c e ro u s Le s io n s

Ac t in ic ke ra t o s is Ju n ctio n al n evu s: occurs at epiderm al/derm al

junction; flat
Most com m on precancerous lesion Greatest m alignant potential
Related to sun exposure
Occurs in m iddle-aged individuals Co m p o u n d n evu s:
Scaly, white, flat-topped lesion with surrounding erythem a Both intraderm al and junctional com ponents (Fig. 6-31);
slightly elevated or papillom atous
Com m on on face, eyelid, and scalp Often pigm ented
May be single or m ultiple Junctional com ponent gives m alignant potential
12% evolve into squam ous cell carcinom a (less aggressive than
if it arises de novo); 25% spontaneously resolve In tr ad er m al n evu s:
May also evolve into basal cell carcinom a Most com m on
Most benign
Path o lo gy: elastotic degeneration in the derm is, overlying Can be papillom atous, dom e-shaped, or pedunculated
hyperkeratosis, focal parakeratosis, clefts in dyskeratotic areas Slightly pigm ented or am elanotic
(Fig. 6-30) Hair shafts indicate intraderm al variety

186
 Eyelid Disorders

Ma lig n a n t Ep it h e lia l Tu m o rs
Risk facto r s: increased age, sun exposure, fair skin, previous
history of skin cancer, positive fam ily history

Ch ar acter istics: irregularity, induration, and altered lid

anatom y

Ba s a l c e ll c a rc in o m a (BCC )
Most com m on m alignancy of the eyelid (90%)
40 tim es m ore com m on than squam ous cell carcinom a
Develops on sun-exposed skin in elderly patients; sm oking is
also a risk
Fig ure 6-31. Compound nevus with cells at junction and in dermis. (From Yanoff
M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.)
Lo catio n (in order of frequency): lower lid (50–60%),
m edial canthus (20–30%), upper lid (15%), outer canthus
Kissin g n evu s: (5%)
Congenital
Involves both upper and lower lids Poorest prognosis when found in m edial canthus because tum or
Secondary to fusion of lids during em bryonic developm ent often extends deeper and can involve lacrimal drainage system
Morbidity and m ortality occur from local invasion of skull
Sp in d le cell n evu s: and CNS
Com pound nevus of childhood Rarely m etastasizes
Bizarre cellular com ponents
No m alignant potential Path o lo gy: blue basaloid tum or cells arranged in nests and
cords (H&E stain), peripheral palisading com m only seen
Gian t h air y n evu s o f th e face an d scalp : (Fig. 6-32)
congenital; thickened eyelids can cause am blyopia
No d u lar b asal cell car cin o m a: m ost com m on form ; firm ,
raised, pearly, discrete m ass, often with telangiectases over
Ne vu s o f o t a (Oc u lo d e rm a l m e la n o c yt o s is ) tum or m argin; if center is ulcerated, called a rodent ulcer
(See Chapter 5, Pediatrics/Strabism us)
Mo r p h eafo r m b asal cell car cin o m a: less com m on, but
m uch m ore aggressive; firm , flat lesion with indistinct borders;
Le n t ig o m a lig n a (Me la n o t ic fre c kle o f penetrates into derm is, pagetoid spread can occur; tum or
Hu t c h in s o n ) cells m ay line up in single cell layer (”Indian-file” pattern)
(Fig. 6-33)
Acquired cutaneous pigm entation
Often periocular Tr eatm en t: excisional biopsy, wide excision with frozen
section, Mohs’ m icrographic surgery, m ay require
Occurs in m iddle-aged or older individuals
supplem ental cryotherapy or radiation therapy, exenteration
Conjunctival pigm entation m ay be noted for orbital extension
No episcleral pigm entation
S q u a m o u s c e ll c a rc in o m a (S CC)
Melanom a arises in approxim ately 30%
Flat, keratinized, ulcerated, erythem atous plaque
Cutaneous counterpart of prim ary acquired m elanosis (PAM)
of the conjunctiva Can arise de novo or from preexisting actinic keratosis
May spread by direct extension or m ay m etastasize via local
lym phatics or hem atogenously
Xe ro d e rm a p ig m e n t o s a (AR)
May be associated with HIV and HPV infection
Defect in DNA repair (UV light endonuclease)
More aggressive than basal cell carcinom a
Freckles and scaling at early age Usually occurs on lower eyelid
Susceptible to a variety of malignant tumors (basal cell carcinoma,
Path o lo gy: pink dyskeratotic cells form ing keratin pearls
squamous cell carcinoma, malignant melanoma, sarcoma)
(H&E stain), cord-like infiltrating strands into the derm is
3% incidence of skin m alignant m elanom a containing atypical anaplastic cells (Fig. 6-34)

187
CHAPTER 6 • Orbit/Lids/Adnexa

A A

B B
Fig ure 6-32. A, Basal cell carcinoma appears as blue nests of basal cells Fig ure 6-34. Squamous cell carcinoma demonstrating A, pink epithelial cells
proliferating over pale pink desmoplasia (dermal fibroblast proliferation). B, Basal invading dermis with overlying ulceration. B, squamous cells in dermis making
cell nests with peripheral palisading and mitotic figures. (Courtesy of HG Scheie. keratin. (From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.)
From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.)

Ke ra t o a c a n t h o m a
Dom e-shaped squam ous lesion
Rapid onset (4–8 weeks); occurs in elderly
Central keratin-filled crater and elevated rolled edges; clinically
resem bles basal cell carcinom a
Previously considered a form of pseudoepitheliom atous
hyperplasia, now classified as a squam ous carcinom a
Spontaneous involution
May cause perm anent dam age to lid m argin and m adarosis
(loss of lashes)

Path o lo gy: acanthotic, hyperkeratotic, dyskeratotic

Fig ure 6-33. Morpheaform type demonstrating thin cords of basal cells. (From
Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.) epithelium and inflam m atory cells
(Fig. 6-35)
Tr eatm en t: excisional biopsy, wide excision with frozen
section, Mohs’ m icrographic surgery, m ay require
supplem ental cryotherapy or radiation therapy, exenteration Tr eatm en t: observation, surgical excision, local steroid
for orbital extension injection

188
 Eyelid Disorders

Fig ure 6-35. Keratoacanthoma appears as cup-shaped lesion with central

A
keratin core above skin surface. (From Yanoff M, Fine BS: Ocular pathology,
ed 5, St Louis, 2002, Mosby.)

S e b a c e o u s g la n d c a rc in o m a
Orange-yellow nodule
Second m ost com m on m alignancy of the eyelid (after BCC)
Occurs in the elderly (sixth-seventh decade of life); higher
incidence am ong Asian populations
Usually arises from m eibom ian glands; can also arise from
glands of Zeis and glands of caruncle
Upper lid m ore com m only involved (greater num ber of
m eibom ian glands)
Can m asquerade and be m isdiagnosed as a recurrent chalazion B
or chronic blepharitis Fig ure 6-36. Sebaceous adenocarcinoma A, Large, foamy tumor cells in
epidermis demonstrating pagetoid spread. B, Oil-red-O stains fat in
Often associated with m adarosis cells. (From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.)
Highly m alignant and lethal tum or; regional lym ph node and
hem atogenous m etastasis; 5-year m ortality rate ¼ 30%

Path o lo gy: lobules of anaplastic cells with foam y, lipid-

laden, vacuolated cytoplasm , large hyperchrom ic nuclei, skip
areas and pagetoid invasion (spread of tum or into conjunctival
epithelium ), positive lipid stains (oil-red-O stain) (Fig. 6-36)

Tr eatm en t: wide excision with frozen section and

conjunctival m ap biopsy, exenteration for orbital extension or
pagetoid spread, radiotherapy for palliation

Mu ir -To r r e syn d r o m e: m ultiple sebaceous neoplasm s,

keratoacanthom as, and visceral tum ors (especially GI)

Ma lig n a n t m e la n o m a (MM) Fig ure 6-37. Malignant melanoma. (From Yanoff M, Fine BS: Ocular pathology,
ed 5, St Louis, 2002, Mosby.)
< 1% of all eyelid cancers (Fig. 6-37)
Len tigo m align a m elan o m a ( Hu tch in so n ’s Su p er ficial sp r ead in g m elan o m a (80%): occurs in sun-
m align an t fr eckle) (10%): occurs in sun-exposed areas of exposed and nonexposed skin (this form is not directly related
elderly patients; arises from lentigo m aligna (see Precancerous to sun exposure) of younger individuals
lesions) Initial horizontal growth phase before invading deeper
Findings: flat, pigm ented m acule with irregular borders Findings: spreading m acule with irregular outline, variable
Pathology: fascicles of spindle-shaped cells pigm entation, invasive phase m arked by papules and
10% m etastasize; 5 year survival ¼ 90% nodules

189
CHAPTER 6 • Orbit/Lids/Adnexa

Pathology: pagetoid nests in all levels of epiderm is Usually occurs in upper lid
5-year survival ¼ 69%
Occurs in older individuals
No d u lar m elan o m a (10%): Metastasis and death in 30% of patients
Most com m on type of melanom a in the eyelid
Tr eatm en t: wide excision with im m unohistochem ical
Usually occurs in fifth decade of life
stains, lym ph node dissection, radiation therapy
More com m on in m en (2:1)
Always palpable Va s c u la r Tu m o rs

More aggressive, with early vertical invasion; 5-year Lym p h a n g io m a

survival ¼ 44%
(See Chapter 5, Pediatrics/Strabism us)
20% of nodular and 50% of superficial spreading arises
from nevi
Ca p illa ry h e m a n g io m a
Sign s o f tr an sfo r m atio n : change in color, change in (See Chapter 5, Pediatrics/Strabism us)
shape or size, unduration, ulceration, bleeding
Ca ve rn o u s h e m a n g io m a
Tr eatm en t: wide surgical excision, lym ph node dissection if
Appears as a port wine stain (nevus flam m eus)
m icroscopic evidence of lym phatic or vascular involvem ent
Associated with Sturge-Weber syndrom e
Pr o gn o sis: depends on depth of vertical invasion; Grows with the patient; does not involute
< 0.75 m m indicates favorable prognosis
Path o lo gy: dilated capillaries without endothelial cell
Ne u r o g e n ic Tu m o r s proliferation
Ne u r o fib ro m a
Ka p o s i’s s a rc o m a
(See Chapter 5, Pediatrics/Strabism us)
Malignant soft tissue sarcom a (solitary or m ultiple)
No d u lar fo r m (fibrom a m olluscum ): More com m on in im m unocom prom ised patients and those of
Usually not associated with neurofibrom atosis (NF) Mediterranean descent

Plexifo r m fo r m : Var io u s typ es: epidem ic (AIDS related), classic

Associated with NF (Mediterranean), endem ic (African), and iatrogenic
Produces S-shaped lid deform ity (transplant-associated)
May involve orbit
Path o lo gy: com bined proliferation of axons, Schwann cells,
and endoneural fibroblasts Nontender, violaceous nodules and plaques
May cause lid distortion with edem a and entropion
Tr eatm en t: observation or resection
Tr eatm en t: com plete surgical excision, also responsive to
Ne u r ile m m o m a (S c h w a n n o m a ) radiotherapy and chem otherapy
(See Orbit section) Ot h e r Le s io n s
Solitary eyelid nodule com posed of Schwann cells
Xa n t h e la s m a
Usually located near m edial canthus
Soft, flat or slightly elevated yellow plaques
Usually not associated with NF
More com m on on the m edial aspect of the eyelids
Path o lo gy: proliferation of Schwann cells 2
of patients have norm al serum lipids
3
Can occur in hyperlipid syndrom es such as fam ilial
Tr eatm en t: local excision
hypercholesterolem ia, juvenile xanthogranulom a,
histiocytosis X
Me r ke l c e ll t u m o r
Associated with Erdh eim -Ch ester disease (lipoid
Rare, vascular, red-blue, sausage-shaped lesion
granulom atosis): m ultisystem disease with lipogranulom a
Rapid growth from Merkel cells (m echanical receptors for form ation in the liver, heart, kidneys, lungs, and bones
touch; am ine precursor uptake and decarboxylation [APUD] Findings: proptosis and xanthelasm a-like lesions
system ) Pathology: histiocytes and Touton giant cells

190
 Nasolacrim al System Disorders

Path o lo gy: aggregates of lipid-containing m acrophages indicates functional occlusion of nasolacrim al duct;
(foam cells) with surrounding inflam m ation clear saline recovery indicates canalicular occlusion or
nonfunctioning lacrim al pum p
Tr eatm en t: observation; m ay recur after excision DACRYOSCINTOGRAM: instillation of technetium 99,
physiologic test
DACRYOCYSTOGRAM: injection of Lipiodol (Ethiodol;
S a rc o id o s is
radiopaque) outlines drainage system
(See Orbit section) PROBING AND IRRIGATION
Slightly elevated, um bilicated papules
P u n c t a l o b s t ru c t io n
Noncaseating granulom as
Etio lo gy: senile, cicatrizing (ocular cicatricial pem phigoid,
Am ylo id o s is Stevens-Johnson syndrom e), traum a, tum or, drug-induced

Confluent, yellow, waxy papules Tr eatm en t: dilation, punctoplasty, reapposition of puncta

May hem orrhage with m inor traum a
Ca n a lic u la r o b s t ru c t io n
Lid lesions are associated with system ic involvem ent
Etio lo gy: traum a, toxic m edications (antivirals, strong
Path o lo gy: heterogeneous group of substances that stains m iotics, epinephrine, chem otherapeutics), infections (HSV,
with Congo red
EBV, trachoma), dacryolith, inflam m ation (ocular cicatricial
pem phigoid, Stevens-Johnson syndrom e), allergy, radiation,
Ne c ro b io t ic xa n t h o g ra n u lo m a tum or, canaliculitis
Zonal granulom a with necrobiotic center
Tr eatm en t:
Associated with JXG, m onoclonal gam m opathies, and plasm a Repair of canalicular system (in traum a)
cell dyscrasias Partial obstruction: Crawford tubes, Viers rods,
Path o lo gy: Touton giant cells and xanthom a cells m onocanalicular stents
Obstruction within 8 mm of
punctum: conjunctivodacryocystorhinostom y (CDCR)
Myc o s is fu n g o id e s
with Jones tubes
Cutaneous T-cell lym phom a Obstruction > 8 mm from punctum: dacryocystorhinostom y
(DCR) with O’Donoghue tubes
Path o lo gy: Lutzner cells and Pautrier abscesses
Na s o la c rim a l d u c t o b s t ru c t io n
Co n gen ital: (See Chapter 5, Pediatrics/Strabism us)
NASOLACRIMAL SYSTEM DISORDERS
Acqu ir ed :
Ob s truc tio n s Etiology: involutional stenosis (m ost com m on cause, m ore
com m on in wom en [2 :1], inflam m atory infiltrates
Co n gen ital tear in g: (See Chapter 5, Pediatrics/Strabismus) com press nasolacrim al duct), traum a, chronic sinus
Must rule out congenital glaucom a; also consider disease, nasal polyps, dacryocystitis, granulom atous
corneal traum a, trichiasis, and superficial foreign disease (e.g., sarcoidosis, Wegener’s granulom atosis)
body Treatment: silicone intubation, DCR

Acqu ir ed tear in g: La c rim a l s a c o b s t ru c t io n

Etiology: prim ary idiopathic hypersecretion, ocular
surface irritation with reflex tearing, outflow Etio lo gy: traum a, acute or chronic dacryocystitis
obstruction Tr eatm en t: DCR
Diagnosis:
DYE DISAPPEARANCE TEST: instill fluorescein in inferior
fornix of both eyes, wait 5 m inutes, then evaluate for
Infe c tio ns
asym m etric clearance of dye from tear m eniscus
JONES I TEST: perform dye disappearance test, then
attem pt to recover fluorescein in inferior nasal Ca n a lic u lit is
m eatus with cotton-tipped applicator; abnorm al Infection of canaliculus, usually chronic
result (no fluorescein) occurs in 33% of norm al
individuals Or gan ism s: Actinomyces israelii is m ost com m on
JONES II TEST: perform Jones I test, then irrigate saline (filam entous Gram -positive rod). Also, Candida albicans,
into nasolacrim al system ; dye recovery from nose Aspergillus, Nocardia asteroides, HSV, and VZV

191
CHAPTER 6 • Orbit/Lids/Adnexa

Ta b le 6.7. Differential diagnosis of common disorders

Ep ithe lia l Eye lid Le s io ns Cavernous sinus thrombosis

Slow-growing: Orbital varices
Squamous cell papilloma AV malformations
Actinic keratosis Acromegaly
Seborrheic keratosis Histiocytosis X
Basal cell carcinoma Multiple myeloma
Squamous cell carcinoma Orbital fractures
Fast-growing: P igm e nte d Eye lid Le s ions
Molluscum contagiosum Nevus
Keratoacanthoma Pigmented basal cell carcinoma
Sub e p ithe lia l Eye lid Le s io ns Lentigo maligna (Hutchinson’s freckle)
Cystic Malignant melanoma
Cyst of Moll Oculodermal melanocytosis (nevus of Ota)
Cyst of Zeiss Enla rge d Extra oc ula r Mus c le s on Com p ute d Tom ogra p hy Sc a n
Sebaceous cyst Thyroid-related ophthalmopathy (muscle tendons spared)
Solid Orbital pseudotumor (muscle tendons involved)
Meibomian cyst Metastatic cancer
Hordeolum Lymphoma
Chalazion Infection
Xanthelasma Carotid-cavernous sinus fistula
Sebaceous adenocarcinoma Acromegaly
P s e ud op rop tos is Amyloid
High myopia (axial myopia) Nodular fasciitis
Contralateral enophthalmos Fibrous histiocytoma
Shallow orbits (Crouzon’s, Apert’s syndrome) Tenon’s cyst
Buphthalmos Wegener’s granulomatos is
Contralateral ptosis La c rima l Gla nd Infla mma tio n/Swe lling
Upper lid retraction Mumps
Infa ntile P rop tos is Glandular fever
Capillary hemangioma (most common) Suppurative adenitis
Orbital cellulitis Extension of conjunctivitis
Dermoid Tuberculosis
Encephalocele Sarcoidosis
Histiocytosis X Malignant lymphoma
Leukemia Lacrimal gland tumor
Retinoblastoma Thyroid-related ophthalmopathy
Craniofacial disorders Orbital pseudotumor
Child ho od P rop tos is Unila te ra l P e riorb ita l Infla mma tion
Orbital cellulitis (most common) Ruptured dermoid cyst
Capillary hemangioma Rhabdomyosarcoma
Dermoid cyst Orbital pseudotumor
Inflammatory pseudotumor Leukemia
Lymphangioma Eosinophilic granuloma
Metastatic neuroblastoma Infantile cortical hyperostosis
Granulocytic sarcoma Enop htha lm os
Orbital extension of retinoblastoma Orbital floor fracture
Optic nerve glioma Metastatic tumors with sclerosis (breast carcinoma)
Rhabdomyosarcoma P a inle s s P rop tos is
Lymphoma/leukemia Cavernous hemangioma
Meningioma Optic nerve tumor
Neurofibromatosis Neurofibroma
Spheno-orbital encephalocele Neurilemmoma
Optic nerve glioma Benign lacrimal gland tumors
Plexiform neurofibroma in the orbit Rhabdomyosarcoma
Ad ult P rop tos is Fibrous histiocytoma
Thyroid-related ophthalmopathy (most common) Lymphoma
Orbital pseudotumor Osteoma
Orbital cellulitis/abscess P a inful P rop tos is
Mucocele Orbital pseudotumor
Wegener’s granulomatos is Posterior scleritis
Sarcoidosis Dacryoadenitis
Optic nerve glioma Orbital hemorrhage
Meningioma Malignant lacrimal gland tumor
Hemangioma Nasopharyngeal carcinoma
Orbital lymphoma Lymphangioma
Metastatic tumor Orbital abscess
Orbital cysts Wegener’s granulomatos is
Fibrous histiocytoma Primary orbital tumors
Fibro-osseous tumors Hemangioma
Leukemia Lacrimal gland tumors
Chloroma Optic nerve meningioma or glioma
Crouzon’s disease Rhabdomyosarcoma

192
 Review Questions

More com m only occurs in m iddle-aged wom en Squ am o u s p ap illo m a: m ost com m on prim ary lacrim al
sac tum or
Sym p to m s: tearing, redness, pain, discharge
Squ am o u s cell car cin o m a: m ost com m on prim ary
Fin d in gs: erythem atous, dilated, tender, pouting punctum ; m alignant lacrim al sac tum or
expressible discharge, recurrent conjunctivitis; m ay have
bloody tears Lym p h o m a: second m ost com m on prim ary m alignant
lacrim al sac tum or
Grating sensation with probing
Lacrim al sac can be invaded by other m alignant tum ors of
Often find sulfur granules eyelids and conjunctiva
Tr eatm en t: warm com presses, probing and irrigation with
penicillin, canalicular curettage, incision and d ebridem ent
ORBITAL SURGERY
Da c ryo c ys t it is
Evis c e ra t io n
Infection of nasolacrim al sac
Rem oval of intraocular contents leaving the sclera intact
Or gan ism s: Staphylococcus, Streptococcus, Pseudomonas, H.
influenzae (young children), Klebsiella, Actinomyces, Candida In d icatio n s: blind painful eye, process not involving sclera
Fin d in gs: Co m p licatio n s: im plant extrusion due to epithelial
Acute: edem a, erythem a, and distention below the m edial downgrowth and poor closure
canthal tendon; m ay result in m ucocele form ation,
chronic conjunctivitis, or orbital cellulitis En u c le a t io n
Chronic: distended lacrim al sac, m inim al inflam m ation
Rem oval of entire globe and portion of optic nerve
Tr eatm en t: warm com presses, topical and system ic
Im p lan t m ater ial: PMMA, silicone, hydroxyapatite, derm is
antibiotics, incision and drainage if abscess present, avoid
fat graft
irrigation and probing during acute infection, DCR after acute
inflam m ation subsides In tegr ated im p lan t: painted prosthesis fits into im plant
via peg
Da c ryo a d e n it is
In d icatio n s: blind painful eye, intraocular m alignancy, after
Acute or chronic inflam m ation of lacrim al gland severe traum a to avoid sym pathetic ophthalm ia
Etio lo gy: Exe n t e ra t io n
Acute: infection (Staphylococcus, N. gonorrheae, m um ps,
Epstein-Barr virus [EBV], VZV) Rem oval of all orbital contents
Chronic: in flam m ation or in fection (IO I, sarcoidosis,
In d icatio n s: adenoid cystic carcinom a, m ucorm ycosis,
Mikulicz’s syn drom e, lym ph oid lesion s,
orbital extension of sebaceous cell, basal cell, m alignant
syph ilis, TB)
m elanom a, and squam ous cell carcinom as
Sym p to m s: swelling, redness; m ay have pain, tearing,
discharge with acute infection Da c ryo c ys t o rh in o s t o m y (DCR)
Creation of passage between lacrim al sac and nasal cavity
Fin d in gs: enlarged lacrim al gland; m ay have tenderness,
fever, preauricular lym phadenopathy, globe dystopia, Osteotom y is m ade by rem oving the lacrim al sac fossa and
restricted ocular m otility superior nasal wall of NLD at level of m iddle turbinate; bony
window should m easure 15 m m  15 m m
Diagn o sis: CT scan; consider culture, laboratory tests
(serologies), and biopsy In d icatio n s: focal distal canalicular obstructions
and NLDOs
Tr eatm en t: m ay require system ic antibiotics, incision and
drainage, or excision Co m p licatio n s: failure due to obstruction at com m on
canaliculus or at bony ostom y site

Tum o rs o f the La c rim a l Sa c

REVIEW QUESTIONS (Answe rs start on page 381)
Rare; secondary to dacryocystitis
Fin d in gs: painless m ass located above m edial canthal 1. Which organism is most com monly associated with angular
tendon, tearing, m ay have bloody tears; bleeding with blepharitis?
probing a. S. epidermidis
b. Moraxella
Must be differentiated from dacryocystitis
c. S. aureus
Dacryocystogram outlines tum or d. Demodex folliculorum

193
CHAPTER 6 • Orbit/Lids/Adnexa

2. Sequelae of a CN 7 palsy m ay include all of the following 12. Which m uscle is m ost com m only responsible for vertical
except diplopia after 4-lid blepharoplasty?
a. filam ents a. superior oblique
b. ptosis b. inferior oblique
c. decreased vision c. superior rectus
d. dry eye d. inferior rectus
3. Which procedure is the best treatm ent option for the repair 13. Congenital and involutional ptosis can be distinguished
of a large upper eyelid defect? by all of the following except
a. Cutler-Beard a. degree of levator function
b. Bick b. presence of lid crease
c. Hughes c. width of palpebral fissure
d. Fasanella-Servat d. presence of jaw wink
4. The extraocular m uscle with the largest arc of contact is 14. Congenital obstruction of the lacrim al drainage system
the usually occurs at the
a. LR a. valve of Rosenm u€ ller
b. IO b. com m on canaliculus
c. MR c. lacrim al sac
d. SO d. valve of Hasner
5. The risk of system ic involvem ent is highest for an ocular 15. What is the correct order of structures that would be
lym phoid tum or in which location? encountered when the upper eyelid is penetrated 14 m m
a. orbit above the lid m argin?
b. eyelid a. preseptal orbicularis m uscle, orbital septum , levator
c. conjunctiva aponeurosis, Mu€ ller’s m uscle
d. bilateral orbit b. preseptal orbicularis m uscle, orbital septum , levator
6. The rectus m uscle with the shortest tendon of insertion m uscle
is the c. pretarsal orbicularis m uscle, levator m uscle,
a. IR conjunctiva
b. SR d. pretarsal orbicularis m uscle, levator aponeurosis,
c. MR orbital septum , fat
d. LR 16. What is the best treatm ent option for a child who
7. Which of the following bones does not m ake up the develops recurrent proptosis after upper respiratory
m edial orbital wall? infections?
a. lacrim al a. observation
b. m axilla b. XRT
c. sphenoid c. chem otherapy
d. palatine d. surgery
8. Which of the following clinical features is least com m only 17. All of the following are features of m ucorm ycosis
associated with a tripod fracture? except
a. restriction of the inferior rectus a. internal ophthalm oplegia
b. flattening of the m alar em inence b. ipsilateral CN 7 palsy
c. hypesthesia c. diplopia
d. displacem ent of the lateral canthus d. involvem ent of the first branch of the
9. A carotid-cavernous fistula is com m only differentiated trigem inal nerve
from a dural-sinus fistula by all of the following 18. All of the following are associated with blepharophim osis
characteristics except except
a. proptosis a. trisom y 18
b. afferent pupillary defect b. ectropion
c. bruit c. AR inheritance
d. CN 6 palsy d. wide intercanthal distance
10. Basal cell carcinom a is least likely to occur at which 19. Which of the following is the m ost im portant
site? test to perform in a patient with a capillary
a. upper eyelid hem angiom a?
b. m edial canthus a. ECHO
c. lower eyelid b. hearing test
d. lateral canthus c. EKG
11. All of the following are sites of attachm ent of the lim bs of d. bleeding tim e
the m edial canthal tendon except 20. For entropion repair, the lateral tarsal strip is sutured
a. frontal process of the m axillary bone a. below and anterior to the rim
b. anterior lacrim al crest b. below and posterior to the rim
c. orbital process of the frontal bone c. above and anterior to the rim
d. posterior lacrim al crest d. above and posterior to the rim

194
 Review Questions

21. Staged surgery for a patient with severe thyroid-related 31. Blepharospasm is associated with
ophthalm opathy is best done in what order? a. m yotonic dystrophy
a. decom pression, strabism us, lid repair b. syphilis
b. strabism us, decom pression, lid repair c. vertebrobasilar insufficiency
c. lid repair, decom pression, strabism us d. Parkinson’s disease
d. decom pression, lid repair, strabism us 32. The anatom ic boundaries of the superior orbital
22. Which of the following best explains why when a ptotic lid fissure are
is lifted, the contralateral lid falls? a. the greater wing of the sphenoid and the
a. inhibition of Mu€ ller’s m uscle zygom a
b. Sherrington’s law b. the greater and lesser wings of the sphenoid
c. relaxation of the frontalis m uscle c. the lesser wing of the sphenoid and the m axilla
d. Hering’s law d. the lesser wing of the sphenoid and the zygom a
23. Which study is m ost helpful in the evaluation of a patient 33. Which of the following is m ost likely to
with opsoclonus? exacerbate the sym ptom s of thyroid-related
a. EKG ophthalm opathy?
b. MRI a. alcohol
c. ERG b. cigarettes
d. angiogram c. aspirin
24. What is the m ost appropriate treatm ent for a benign m ixed d. caffeine
tum or of the lacrim al gland? 34. A 44-year-old wom an develops a left lower eyelid
a. radiation ectropion following a severe facial burn. The m ost
b. excision appropriate procedure includes
c. observation a. horizontal tightening
d. biopsy b. vertical shortening
25. What is the m ost appropriate treatm ent for a biopsy- c. repair of lower eyelid retractors
positive basal cell carcinom a of the lower eyelid? d. levator m yotom y
a. cryotherapy to the cancer and m argins 35. All of the following are m ethods of treating spastic
b. local antim etabolite treatm ent entropion except
c. radiation with 2500 rads to the lesion and m argins a. eyelid taping
d. excision with frozen section control of the m argins b. Botox injection
26. Which of the following CT enhancing lesions has a c. Wies m arginal rotation
pathognom onic appearance? d. Quickert suture
a. rhabdom yosarcom a 36. The m ost com m on com plication of a hydroxyapatite
b. gliom a orbital im plant is
c. lym phangiom a a. im plant m igration
d. m eningiom a b. infection
27. Which of the following factors is least likely to contribute c. orbital hem orrhage
to the developm ent of entropion? d. conjunctival erosion
a. preseptal orbicularis override 37. Which collagen vascular disease is associated with
b. horizontal lid laxity m alignancy?
c. posterior lam ella foreshortening a. derm atom yositis
d. capsulopalpebral fascia disinsertion b. scleroderm a
28. A 24-year-old wom an presents after blunt traum a to the c. Wegener’s granulom atosis
left orbit with enophthalm os and restriction of d. system ic lupus erythem atosus
upgaze. Which plain film radiographic view would be 38. Oral antibiotics are indicated for
most helpful? a. canaliculitis
a. Caldwell view b. dacryocystitis
b. lateral view c. dacryoadenitis
c. Waters view d. nasolacrim al duct obstruction
d. axial view 39. The levator m uscle inserts onto all of the following
29. All of the following m ay cause enophthalm os except structures except
a. breast carcinom a a. tarsus
b. lym phom a b. lateral orbital tubercle
c. orbital floor fracture c. posterior lacrim al crest
d. phthisis bulbi d. trochlea
30. All of the following nerves pass through the superior 40. When perform ing a DCR, the osteum is created at the level
orbital fissure except of the
a. CN 3 a. superior turbinate
b. CN 4 b. m iddle turbinate
c. CN V2 c. inferior turbinate
d. CN 6 d. none of the above

195
CHAPTER 6 • Orbit/Lids/Adnexa

41. An adult with a com plete nasolacrim al duct obstruction 48. A72-year-old m an has bilateral ptosis and levator function
and patent puncta and canaliculi is best treated with which m easuring 14 m m OU. The m ost likely diagnosis is
procedure? a. chronic progressive external ophthalm oplegia
a. silicone stent intubation b. Horner’s syndrom e
b. Jones tube c. levator aponeurotic dehiscence
c. dacryocystectom y d. m yasthenia gravis
d. dacryocystorhinostom y 49. The sensory nerve m ost likely to be affected by an orbital
42. The m ost effective procedure for involutional ectropion is fracture is
a. lateral tarsal strip a. lacrim al
b. plication of the orbital septum b. nasociliary
c. shortening of the m edial canthal ligam ent c. infraorbital
d. wedge resection of the tarsus d. infratrochlear
43. A patient presents with follicular conjunctivitis and a 50. Which of the following findings is m ost helpful for m aking
cluster of um bilicated papules are noted near the eyelashes the diagnosis in a patient with suspected thyroid-related
of the left eye. The m ost effective treatm ent for this orbitopathy?
condition is a. abnorm al forced ductions
a. acyclovir b. com itant esotropia
b. antibiotics c. presence of ptosis
c. cryotherapy d. norm al thyroid function tests
d. radiation
44. The m ost com m on cause of unilateral proptosis in a
m iddle-aged wom an is SUGGESTED READINGS
a. cavernous hem angiom a
b. thyroid-related orbitopathy
Basic and Clinical Sciences Course. (2016). Section 7: Orbit, eyelids and
c. derm oid
lacrimal system. San Francisco: AAO.
d. m etastatic breast carcinom a Black, E. H., & Nesi, F. A. (2012). Smith and Nesi’s Ophthalmic Plastic and
45. The m ost com m on cause of involutional entropion of the Reconstructive Surgery (3rd ed.). New York: Springer.
lower eyelid is Chen, & Kahn. (2009). Color atlas of cosmetic oculofacial surgery (2nd
a. inflam m ation ed.). Philadelphia: Saunders.
b. laxity and retractor disinsertion Collin, J. R. O. (2006). Manual of systematic eyelid surgery (3rd ed.).
c. orbicularis spasm Philadelphia: Butterworth-Heinem ann.
d. posterior lam ella scarring Dutton, J. S. (2011). Atlas of Clinical and Surgical Orbital Anatomy (2nd
46. An elderly wom an with chronic unilateral blepharitis, ed.). Philadelphia: Saunders.
Dutton, J. S. (2013). Atlas of Oculoplastic and Orbital Surgery (2nd ed.).
thickening of the left upper eyelid, and subm andibular
Philadelphia: Lippincott William s and Wilkins.
lym phadenopathy is m ost likely to have
Fagien, S. (2007). Putterman’s cosmetic oculoplastic surgery (4th ed.).
a. basal cell carcinom a Philadelphia: Saunders.
b. keratoacanthom a Levine, M. R. (2010). Manual of oculoplastic surgery (4th ed.). Thorofare:
c. m olluscum contagiosum SLACK.
d. sebaceous gland carcinom a Rootm an, J. (2002). Diseases of the orbit (2nd ed.). Philadelphia:
47. A 56-year-old diabetic wom an presents with pain, Lippincott William s and Wilkins.
swelling, and redness of the left upper eyelid. Orbital Rootm an, J. (2013). Orbital Surgery: A Conceptual Approach (2nd ed.).
involvem ent is m ost likely if she also has Philadelphia: Lippincott William s and Wilkins.
a. headache Sm ith, B. C., Nesi, F. A., Cantarella, V. H., et al. (1998). Smith’s
ophthalmic plastic and reconstructive surgery(2nd ed.). St Louis: Mosby.
b. discharge
c. ptosis
d. pain with eye m ovem ent

196
7 Cornea /External Disease
ANATOMY/ PHYSIOLOGY
CONJUNCTIVAL DISORDERS
CORNEAL DISORDERS
SCLERAL DISO RDERS
SURGERY

ANATOMY/ P HYSIOLOGY Co rn e a (Fig. 7-1)

Aver age m easu r em en ts:
Co n ju n c t iva Diameter: vertical ¼ 11.5 m m , horizontal ¼ 12.5 m m ; at
birth, horizontal diam eter ¼ 9.5–10.5, reaches adult size
Nonkeratinized stratified colum nar epithelium with goblet
by age 2
cells (m ost num erous in fornices) underlying loose strom al
Thickness: central ¼ 550 µm , peripheral ¼ 1.0 m m ;
tissue (substantia propria)
typically, inferotem poral paracentral cornea is thinnest,
superior paracentral cornea is thickest
Palp eb r al co n ju n ctiva: firm ly adherent to tarsus Radius of curvature: 7.8 m m anteriorly; 6.2–6.8 m m
posteriorly (peripheral cornea is flatter)
Bu lb ar co n ju n ctiva: loosely adherent to globe except at Power: anterior surface ¼ +49 D, posterior surface ¼ À 6D,
lim bus where it fuses with Tenon’s capsule total ¼ 43 D (75% of total power of eye)
Refractive index: 1.36
Plica sem ilu n ar is: narrow fold of m edial bulbar
conjunctiva near caruncle; rudim entary structure analogous to
nictitating m em brane in certain anim als Ep ith eliu m : 50 µm thick (10% of corneal thickness);
hydrophobic (hydrophilic m olecules penetrate poorly)
Car u n cle: tissue at m edial canthus interm ediate between Sm ooth refractive surface; protects against infection
conjunctiva and skin; contains accessory derm al appendages Aerobic m etabolism (accounts for 70% of ATP production)
Oxygen is obtained via diffusion from the tear film when
the eye is open and from the lid vasculature when the eye
P re c o rn e a l t e a r film
is closed; also, sm all am ount from the aqueous
3 Layer s: Regenerates from lim bal stem cells (turnover ¼ 6–7 days)
Lipid: outer layer; reduces evaporation; cholesterol and Approxim ately 4–6 cells thick centrally and 7–10 cells thick
lipids; produced by m eibom ian (holocrine), Zeis’ at lim bus
(sebaceous), and Moll’s (apocrine) glands Layers:
Aqueous: middle layer; provides oxygen to epithelium; 98% TOP: 3–4 layers of squam ous cells (upperm ost are
water, 2% protein, pH ¼ 7.2; osm olarity $ 302 m Osm/L; apical cells)
produced by lacrim al and accessory lacrimal (exocrine) MIDDLE: 1–3 layers of wing cells (flattened
glands (Krause’s and Wolfring’s), basal secretion polygonal shape)
rate ¼ 2 µL/m in; lysozyme (antibacterial enzyme) DEEP: 1 layer of basal cells
constitutes 30% of total protein in tear film ; also, Cells:
lactoferrin, IgA and IgG (not IgD), electrolytes, oxygen APICAL CELLS: secrete proteins that form the glycocalyx
Mucin: inner layer; reduces surface tension and allows extending from epithelial surface into m ucin layer of
aqueous tear film to be spread evenly; helps structure the tear film
tear film ; glycoproteins; 3 types: secreted m ucins (MUC4 WING CELLS: tightly packed, linked by desm osom es;
and MUC7) produced by lacrim al gland, gel-form ing form protective barrier
m ucins (MUC5-AC) produced by conjunctival goblet BASAL CELLS: anchor epithelium to strom a by
cells (glands of Manz and crypts of Henle, prim arily in hem idesm osom es; secrete basem ent m em brane
fornix), and m em brane-associated m ucins (MUC1 and NERVE CELLS: epithelium contains sensory nerve
MUC16) that protect the ocular surface; 2–3 m L/day. endings

197
CHAPTER 7 • Cornea /External Disease

play a critical role in restructurin g the cornea after

in jury
MMP-1 (collagenase-1): breaks down collagen types I, II,
and III
MMP-2 (gelatinase A): breaks down collagen types IV, V,
and VII, as well as gelatins and fibronectin
MMP-3 (stom alysin): breaks down proteoglycans and
fibronectins
MMP-9 (gelatinase B): breaks down collagen types IV, V,
and VII, as well as gelatins and fibronectin.
MMP-1, 2, and 3 are m ade by the strom a; MMP-9 is m ade
by the epithelium ; only MMP-2 is found in healthy
cornea, the others are found only after injury
Pathology: during processing, strom al lam ellae separate
form ing clefts (artifact); if these are absent, suggests
Fig ure 7-1. Normal cornea. (From Yanoff M, Fine BS: Ocular Pathology, 5th ed,
St Louis, Mosby, 2002.)
corneal edem a (lam ellae are sam e thickness, but space
between fills with fluid)

Pathology: Descem et’s m em b r an e: 3 (birth) to 12 µm (adults) thick;

CORNEAL EPITHELIAL EDEMA: PAS-positive basem ent m em brane
INTRACELLULAR: due to epithelial hypoxia and Anchors endothelium to strom a
nutritional comprom ise; associated with contact Type IV collagen secreted by endothelial cells
lens use; fine, frosted-glass appearance Layers:
(Sattler’s veil) FETAL BANDED LAYER: anterior layer (closer to strom a),
INTERCELLULAR: due to elevated IOP; causes striated pattern; organized collagen lam ellae (like
m icrocystic edem a and epithelial bullae strom a); no change with age (3 µm )
CORNEAL FILAMENTS: com posed of m ucus and ADULT NONBANDED LAYER: posterior layer (closer to
desquam ated epithelial cells; due to increased m ucus endothelium ), no striations; nonorganized; thickens
production and abnorm al epithelial turnover with age (2–10 µm )
Regenerates after dam age as long as endothelium is
intact
Basal lam in a: scaffold for epithelium ; adjacent to Pathology:
Bowm an’s layer; com posed of type IV collagen secreted by
BREAKS: edges tend to coil or roll into a scroll shape
basal epithelium (use PAS stain)
(Haab’s striae, forceps injury, hydrops) (Fig. 7-2)
2 layers: lam ina lucida and lam ina densa
FOCAL THICKENING: Fuchs dystrophy, iridocorneal
Basal epithelium is secured by hem idesm osom es; adheres
touch, vitreocorneal touch, guttata (Fig. 7-3)
to strom a by anchoring fibrils
En d o th eliu m : 4–6 µm thick
Bo wm an ’s layer ( Bo wm an ’s m em b r an e) : 10 µm thick; Monolayer of interdigitating hexagonal cells joined by tight
type I collagen enm eshed in GAG m atrix, rich in fibronectin, junctions
acellular; form ed from secretion from both basal epithelial Transports nutrients into cornea, pum ps fluid out of cornea
cells and strom al keratocytes Rich in m itochondria; m etabolizes carbohydrates at 5–6
Not a true basem ent m em brane tim es the rate of epithelium
Heals with scarring; does not regenerate

Str o m a: 480 µm thick centrally, 900 µm peripherally; 78%

water by weight
Type I, IV, V collagen in m ucopolysaccharide m atrix
Collagen lamellae: 250 sheets com posed of parallel
sm all-diam eter (20-30 nm ) fibrils
Glycosaminoglycans: m aintain lam ellar spacing,
contain water
Keratan sulfate:
Cells: keratocytes (produce tropocollagen; in wound
repair, tropocollagen is different, resulting in nonparallel
collagen fibrils and opacity), Langerhans cells,
pigmented m elanocytes, lym phocytes, m acrophages,
histiocytes
Matrix metalloproteinases (MMP): fam ily of enzym es Fig ure 7-2. Hydrops demonstrating corneal edema (thickening) with breaks in
that break down com pon ents of the extracellular Descemet’s membrane. (From Yanoff M, Fine BS: Ocular Pathology, 5th ed,
m atrix; help m aintain th e norm al corneal structure; St Louis, Mosby, 2002.)

198
 Conjunctival Disorders

INFERIOR PUNCTATE: blepharitis

PERILIMBAL (360°): soft contact lens wear
NEGATIVE STAINING: thickened irregular epithelium
(i.e., epithelial basem ent m em brane dystrophy,
healing epithelial defect)
Rose bengal: stains tissue deficient of album in and m ucin,
including devitalized cells
Lissamine green: devitalized cells, CIN; m ore com fortable
than rose bengal

Sp ecial tech n iqu es:

Fig ure 7-3. PAS stain demonstrating guttata as wart-like excrescences of Pachymetry: m easures corneal thickness
Descemet’s membrane. (From Yanoff M, Fine BS: Ocular Pathology, ed 5,
Keratometry: m easures corneal curvature (only 2 points
St Louis, 2002, Mosby.)
3 m m apart in paracentral region)
Topography: m easures curvature of entire cornea
Functions: Schirmer’s test: m easures tear production (basal + reflex
1. Barrier between strom a and anterior cham ber (without anesthesia); basal (with anesthesia))
2. Keeps cornea dehydrated and clear; 1 m illion cells at Specular microscopy: m easures endothelial cell count and
birth ($ 3800 endothelial cells/m m 2 ); loss of m orphology
approxim ately 50% with aging; adjacent cells stretch Confocal microscopy: im ages corneal layers
to fill gaps (no regeneration), insufficient pum p when
< 500 cells/m m 2
Morphology: at least 60% of cells should be hexagonal; less Lim b u s
than this represents unhealthy cornea Area 1–2 m m wide at which cornea and sclera m eet; contains
Pathology: corneal epithelial stem cells, goblet cells, lym phoid cells,
PLEOMORPHISM: variation in cell shape Langerhans cells, mast cells (see Ch. 10, Anterior Segment)
POLYMEGATHISM: variation in cell size
Responses to stress (large, unusually shaped cells); surgery,
contact lens wear, certain drugs m ay cause an osmotic S c le r a
challenge or inhibition of the Na+/K+ pum p causing White fibrous layer com posed of collagen and elastin
strom al edema
Exa m p le : diabetes (buildup of sorbitol in endothelial cells with
hyperglycemia); contact lens (inhibits Na +/K+ pump and cells Th ickn ess: 0.66 m m at m uscle insertion, 0.33 m m beneath
swell); earliest endothelial change (endothelial bleb response) recti, 1.0 m m posteriorly
occurs within minutes of insertion of a thick, soft or rigid contact lens
(resolves rapidly after lens removal or slowly after 30 minutes of
3 layer s (ill defined): episclera, sclera proper, and
lens wear)
lam ina fusca
SCHWALBE’S LINE: term ination of corneal endothelium
(junction between endothelium and trabecular
m eshwork)
SCHWALBE’S RING: peripheral term ination of
Descem et’s m em brane CONJ UNCTIVAL DISORDERS
POSTERIOR EMBRYOTOXIN: thickening and anterior
displacem ent of Schwalbe’s line
Infla m m a tio n
In n er vatio n : Innervation to cornea occurs via CN V1
Approxim ately 70–80 branches of long posterior ciliary
Fo llic le s
nerves enter peripheral cornea after m yelin sheath is lost
1–2 m m before lim bus Gray-white round elevations with avascular center and vessels
at periphery
Stain s: Well-circum scribed focus of lym phoid hypertrophy
Fluorescein: stains epithelial defects; negative staining
(lym phocytes with a germ inal center) due to reactive
occurs in areas of epithelial irregularity
hyperplasia
PATTERN GIVES CLUE TO ETIOLOGY:
INTERPALPEBRAL: dry eye Generally m ost prom inent in inferior fornix (except in
HORIZONTAL BAND ACROSS INFERIOR ONE-THIRD OF trachom a)
CORNEA: lagophthalm os / exposure
SUPERIOR PUNCTATE: SLK, floppy eyelid syndrom e
P a p illa e
CENTRAL PUNCTATE: focal epithelial keratitis
(Thygeson’s SPK, epidem ic keratoconjunctivitis Sm all to large elevations with central vascular tuft and pale
[EKC], m olluscum ) avascular valleys

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CHAPTER 7 • Cornea /External Disease

Epithelial proliferation, hypertrophy, and infoldings; Path o lo gy: acellular eosinophilic m aterial that stains with
hyperplasia of vascular strom a with chronic inflam m atory cells Congo red, thioflavin T, m etachrom atic with crystal violet,
apple-green birefringence and dichroism with polarization
Usually upper eyelid
m icroscopy
Nonspecific reaction to conjunctival inflam m ation (edem a
and leakage of fluid from vessels)
Co n c re t io n s (Lit h ia s is )
Subepithelial substantia propria of tarsal and lim bal
conjunctivae contains fibrous tissue septa that interconnect to Sm all, round, yellow-wh ite deposits in palpebral
form polygonal lobules with a central vascular bundle conjunctiva
May contain calcium
Giant papillae: > 1 m m in diam eter May erode through conjunctiva and abrade ocular surface
causing foreign body sensation
Treatment: rem ove with needle if erodes through
Ch e m o s is
conjunctiva
Conjunctival edem a; m ay be caused by allergy or infection, can
occur after eyelid surgery, or m ay be idiopathic
Co n ju n c t ivo c h a la s is
P h lyc t e n u le Redundant, loose, nonedem atous inferior bulbar conjunctiva
interposed between globe and lower eyelid
Usually unilateral; m ore com m on in children
Exact etiology unknown; thought to be due to elastoid
degeneration and loss of adhesion between conjunctiva and
Etio lo gy: type IV hypersensitivity reaction to Staphylococci, sclera as Tenon’s capsule thins with age, possible m echanical
coccidioidomycosis, Candida, HSV, lym phogranulom a factor from dry eye or blepharitis (lid rubbing, dry
venereum (LGV), TB conjunctiva); often coexists or m isdiagnosed as dry eye disease;
m ore com m on in elderly, contact lens wearers, and patients
with autoim m une thyroid disease
Fin d in gs: round, elevated, focal, sterile infiltrate on bulbar
conjunctiva, lim bus, or cornea; overlying epithelium breaks
down (stains with fluorescein); m ay have corneal Sym p to m s: tearing (interference with tear m eniscus and
vascularization lacrim al drainage), irritation, foreign body sensation, redness,
tearing
Path o lo gy: infiltration of lym phocytes; atypical ulceration;
fibrosis Fin d in gs: excess folds of inferior bulbar conjunctiva,
epiphora; m ay have conjunctival ulceration, subconjunctival
hem orrhage, and signs of dry eye disease
Tr eatm en t: topical steroids and antibiotic
Classificatio n :
S ym b le p h a ro n Grade 1 ¼ no persistent fold
Grade 2 ¼ single, sm all fold
Adhesion between conjunctival surfaces (palpebral and Grade 3 ¼ m ore than two folds and not higher than tear
bulbar) m eniscus
Due to inflam m ation, traum a, or surgery Grade 4 ¼ m ultiple folds and higher than tear m eniscus

Bilateral in ocular cicatricial pem phigoid and Stevens-Johnson

Tr eatm en t: if sym ptom atic treat with lubrication ,
syndrom e
con sider topical steroids or surgery (conjun ctivoplasty
(con jun ctival resection ) with or without am niotic
m em bran e tran splantation, con jun ctival fixation to sclera,
cautery)
De g e ne ra tio ns

Am ylo id o s is P in g u e c u lu m
Yellow-salm on, subepithelial, interpalpebral plaque in Sm all nodule com posed of abnorm al subepithelial collagen;
prim ary localized disease m ay calcify
System ic am yloidosis is not associated with conjunctival Located at lim bus, nasal m ore com m on than tem poral; does
am yloid but is associated with am yloid of lid not involve cornea

200
 Conjunctival Disorders

Caused by actinic (UV light) exposure

Alle rg y
Path o lo gy: elastoid degeneration (basophilic
degeneration of collagen)
Typ e I h yp er sen sitivity r eactio n : airborne allergens
(pollen, m old, dander) cross link IgE receptors on m ast cells
causing degranulation with release of histam ine, eosinophil
Pin gu ecu litis: inflam ed pingueculum due to dryness and chem otactic factors, platelet-activating factor, m ajor basic
irritation protein, and prostaglandin D 2
Treatment: lubrication, short course of topical steroids
Sym p to m s: itching (H 1 receptors), hyperem ia
P t e ryg iu m (H 2 receptors)

Interpalpebral, wing-shaped, fibrovascular tissue that invades

cornea Co n ju n ctival scr ap in g: abundant eosinophils (norm ally,
there are very few)
Associated with actinic exposure
Tr eatm en t: topical antihistam ine (em edastine [Em adine],
levocabastine [Livostin]), m ast cell stabilizer (pem irolast
Fin d in gs: Stocker’s line (corneal iron line at head of [Alam ast], nedocrom il [Alocril], lodoxam ide [Alom ide],
pterygium ); m ay induce astigm atism with flattening in crom olyn sodium [Crolom ]), antihistam ine / m ast cell
m eridian of pterygium ; m ay decrease vision if crosses stabilizer com bination (azelastine [Optivar], olopatadine
visual axis [Patanol, Pataday], ketotifen [Zaditor, Alaway], epinastine
[Elestat], bepotastine [Bepreve], alcaftadine [Lastacaft]),
Path o lo gy: elastoid degeneration with destruction of NSAID (ketorolac [Acular]), steroid, artificial tears, cold
Bowm an’s layer; m ay have epithelial dysplasia; in recurrences com presses
after excision m ay have fibrotic response not elastoid
degeneration
Alle rg ic c o n ju n c t ivit is
Tr eatm en t: observation or surgical excision
20% of the US population has allergies
Up to 50% recurrence rate with prim ary excision (50%
within 4 m onths, 95% within 1 year) 90% of patients with system ic allergies will have ocular
Decreased risk of recurrence with am niotic m em brane sym ptom s
(15%), conjunctival autograft (5%), or m itom ycin
Most com m only seasonal or perennial allergic conjunctivitis
application at surgery
Associated with allergic rhinitis

De p o s its Sym p to m s: itching, tearing, redness

Exo g e n o u s Fin d in gs: lid swelling, conjunctival injection, chem osis

Argyrosis, m ascara, adenochrom e (topical epinephrine is
m etabolized to m elanin) Gia n t p a p illa ry c o n ju n c t ivit is
Etio lo gy: allergic reaction to m aterial coating a foreign body
En d o g e n o u s
(e.g. contact lens [CL, especially extended wear], exposed
Addison’s disease, Nelson’s syndrom e, alkaptonuria (AR, suture, ocular prosthesis); true giant papillae also occur in
absence of hom ogentisic acid oxidase) vernal and atopic keratoconjunctivitis
Atopic individuals are at higher risk
Bio p sy: for cystinosis or oxalosis, fix in 50% alcohol so
crystals do not dissolve; if urate suspected, use absolute alcohol
Sym p to m s: itching, tearing, m ucus discharge, CL
discom fort, then intolerance
Co njunc tiva l te la ng ie c ta s ia
Fin d in gs: giant papillae (> 1.0 m m ) on upper palpebral
Associated with Louis-Barr syndrom e (ataxia telangiectasia), conjunctiva
Osler-Weber-Rendu (hereditary telangiectasia), Sturge-Weber
syndrom e, diabetes, sickle cell disease, Fabry’s disease, Tr eatm en t: rem oval of inciting factor (e.g., CL, suture),
increased orbital pressure (C-C fistula), blood-filled lym phatic topical allergy m edication; sym ptom s resolve m onths before
tissue, irradiation the giant papillae resolve; avoid thim erosal

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CHAPTER 7 • Cornea /External Disease

At o p ic ke ra t o c o n ju n c t ivit is (AKC) Fin d in gs: conjunctival injection hyperem ia, thickening,

redundancy of superior bulbar conjunctiva with fine punctate
Ato p y: hereditary allergic hypersensitivity (10–20% of
staining (rose bengal), velvety papillary hypertrophy of upper
population)
palpebral conjunctiva, m icropannus, filam entary keratitis
Types I < IV hypersensitivity reactions (50%), decreased Schirm er’s test (25%)
Onset usually between ages 30 and 50 years Path o lo gy: thickening and keratinization of superior bulbar
conjunctiva with loss of goblet cells
Clinical diagnosis (atopic skin disease [eczem a], hay fever,
asthm a) Co n ju n ctival scr ap in g: neutrophils, lym phocytes,
plasm a cells
Eczem a (atopic derm atitis): 3% of population; AKC in 15% Tr eatm en t: variety of options, usually requires scarring of
to 40% of those with atopic derm atitis superior bulbar conjunctiva
Steroids of little use
Sym p to m s: itching, burning, photophobia, tearing, blurred Pressure patch
vision Large-diam eter bandage contact lens
Mechanical scraping of affected area m ay provide tem porary
relief
Fin d in gs: atopic derm atitis of eyelids (m ay have blepharitis, Topical silver nitrate solution 0.5% to1.0% to produce
m adarosis [loss of lashes], punctal ectropion), papillary
chem ical burn, can retreat for recurrence (never use silver
conjunctivitis (sm all or m edium -sized papillae in inferior
nitrate stick)
fornix); m ay develop sym blepharon, corneal vascularization,
Therm ocauterization of upper bulbar conjunctiva
and scarring
Recession or resection of upper bulbar conjunctiva
Associated with keratoconus (10%), subcapsular cataracts
(Maltese cross pattern), bilateral HSV keratitis, pellucid
m arginal degeneration
Co njunc tivitis a s s o c ia te d with
s ys te m ic d is e a s e s
Path o lo gy: m ast cell infiltration of conjunctival epithelium
Mu co cu tan eo u s d iso r d er s: Stevens-Johnson syndrom e,
ocular cicatricial pem phigoid (OCP), bullous pem phigoid,
Tr eatm en t: topical allergy m edication; consider system ic pem phigus, epiderm olysis bullosa, derm atitis herpetiform is,
antihistam ine, cyclosporine (up to 5 m g/kg/day) arthropathies (Reiter’s syndrom e, psoriatic arthritis), infections
(Parinaud’s oculoglandular syndrom e, Kawasaki’s disease),
Ve rn a l ke ra t o c o n ju n c t ivit is (VKC) Wegener’s granulom atosis

(See Chapter 5, Pediatrics/Strabism us)

Infe c tio us c o njunc tivitis
To xic ke ra t o c o n ju n c t ivit is
May be hyperacute, acute, or chronic; usually viral in adults
Etio lo gy: direct contact of m edication or chem ical substance and bacterial in children
with ocular surface
Fin d in gs: papillae, follicles, conjunctival injection,
Fin d in gs: conjunctival injection, follicles, papillae, keratitis chem osis, discharge; m ay have preauricular lym phadenopathy,
(SPK, occasionally pseudodendrite) lid swelling, m em branes, keratitis, corneal infiltrates, AC
reaction
Subepithelial infiltrates (SEI): collections of inflam m atory
S u p e rio r lim b ic ke ra t o c o n ju n c t ivit is (S LK) cells (m ostly lym phocytes) at level of Bowm an’s layer
Recurrent inflam m ation of superior bulbar and palpebral and anterior strom a
conjunctiva; unknown etiology Typically occur 2 weeks after onset of EKC, can last
m onths
Associated with CL wear and thyroid dysfunction (50%) Thought to be im m unologic response to viral antigens
trapped in strom a
Fem ale preponderance (70%), onset usually between ages
May cause decreased vision, glare, and photophobia
30 and 55 years
DDx: hypoxia (contact lens overwear), infectious
Recurrent episodes; lasts 1 to 10 years, eventually resolves keratitis, EKC, Thygeson’s SPK, hypersensitivity
perm anently (staph m arginal keratitis), m edication (postsurgical
topical NSAID without concom itant topical steroid),
70% bilateral; sym ptom s worse than signs
corneal graft rejection, Reis-Bucklers dystrophy,
Cogan’s dystrophy
Sym p to m s: foreign body sensation, burning, photophobia, TREATMENT: topical steroids (SEIs fade but m ay return if
redness, blurred vision steroids abruptly discontinued)

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 Conjunctival Disorders

Subconjunctival hemorrhages: hem orrhagic conjunctivitis Ne w c a s t le ’s d is e a s e

ETIOLOGY: coxsackie A24, Picorna (enterovirus 70),
Unilateral follicular conjunctivitis, pneum onitis, preauricular
EKC (adenovirus types 8 and 19)
lym phadenopathy
True membrane: fibrin exudation , inflam m atory cells,
an d in vasion by vessels; firm ly adh erent to Occurs in poultry handlers; self-lim ited, lasts 1 week
epithelium , bleedin g occurs wh en peeled (diph theria,
Gonococcus, β-hem olytic streptococcus, Stevens-Johnson
Etio lo gy: RNA virus; causes fatal disease in turkeys and
syndrom e)
other birds
Pseudomembrane: less adherent fibrin exudate (HSV, EKC,
pharyngoconjunctival fever [PCF], bacterial, chlam ydial,
VKC, chem ical burn, OCP, foreign body, ligneous, Me a s le s
Kawasaki’s disease, GVH disease)
Conjunctivitis, epithelial keratitis (associated with vitam in A
xerosis)
DDx o f co n ju n ctivitis with p r eau r icu lar
lym p h ad en o p ath y: EKC, HSV, Gonococcus, Chlamydia,
Mo llu s c u m c o n t a g io s u m
Parinaud’s oculoglandular syndrom e, Newcastle’s disease
Chronic follicular conjunctivitis associated with elevated
um bilicated lid lesions
DDx o f acu te fo llicu lar co n ju n ctivitis: EKC, PCF,
chlam ydial, prim ary HSV, m edicam entosa (antivirals, Due to release of toxic viral products
atropine, Propine, apraclonidine [Iopidine], brim onidine
[Alphagan], neom ycin), viral lid infections (verruca,
m olluscum ), Newcastle’s disease, acute hem orrhagic Tr eatm en t: excision of lesions or cryosurgery
(enteroviral) conjunctivitis
Ba c t e ria l
DDx o f ch r o n ic fo llicu lar co n ju n ctivitis
Hyp e ra c u t e (< 2 4 h o u r s )
(> 4 weeks): chlam ydial, m edicam entosa, viral lid lesion,
HSV, psittacosis, Lym e disease, Parinaud’s oculoglandular Copious purulent discharge, m arked conjunctival injection
syndrom e, chronic fiber granulom a (nylon in fornix), type I and chem osis
hypersensitivity (atopic), m olluscum , trachom a Neisseria gonorrheae: preauricular lym phadenopathy,
corneal infiltrates; can penetrate intact corneal
Vira l epithelium ; can perforate within 48 hours

Ad e n o viru s
Diagn o sis: Gram ’s stain (Gram -negative intracellular
Most com m on; double-stranded DNA; prim ary infection diplococci)
provides lifelong im m unity; 51 stereotypes, one-third
associated with ocular infection
Tr eatm en t: system ic ceftriaxone (1 g IM if no corneal
Fin d in gs: initially diffuse epithelial keratitis with norm al involvem ent; 1 g IV or IM qd  5 days with corneal
vision; later, focal epithelial keratitis, coalescence of fine spots involvem ent); topical antibiotic (fluoroquinolone or
that becom e subepithelial infiltrates bacitracin); erythrom ycin or doxycycline  1 week (to cover
concurrent Chlamydia infection)

Ep id em ic ker ato co n ju n ctivitis (EKC): adenovirus types

8, 19, and 37; bilateral in 75% to 90% Ac u t e (h o u rs t o d a ys )
Findings: preauricular lym phadenopathy, follicular
Purulent discharge, not as severe as hyperacute
conjunctivitis, lid swelling, watery discharge,
pseudom em brane, subconjunctival hem orrhage, Streptococcus pneumoniae, Staphylococcus, Haemophilus influenzae,
conjunctival scarring (sym blepharon), SEIs; rarely, Pseudomonas
corneal edem a, AC reaction, hypopyon; pharyngitis and
rhinitis in 50%; can cause corneal ulceration
Treatment: steroids are useful prim arily with a true Tr eatm en t: topical antibiotic (Polytrim , tobram ycin,
m em brane or vision worse than 20/40 from SEIs azithrom ycin, or fluoroquinolone); add system ic antibiotic for
H. influenzae
Ph ar yn go co n ju n ctival fever ( PCF) :
adenovirus types 3, 4, 5, and 7; young children; spread by
Ch ro n ic
respiratory secretions
Findings: fever, pharyngitis, follicular conjunctivitis; m ay Staphylococcus, Moraxella (chronic angular
have punctate keratitis, rarely SEIs blepharoconjunctivitis), occasionally Gram -negative rods

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CHAPTER 7 • Cornea /External Disease

Diagn o sis: culture Tr eatm en t: 3 weeks of system ic and several m onths of

topical antibiotics (tetracycline) during active disease;
m anagem ent of dry eyes; rem oval of m isdirected lashes
Tr eatm en t: topical antibiotic (Polytrim )

Ch la m yd ia l
Othe r Co njunc tivitis
In c lu s io n c o n ju n c t ivit is (Tr a c h o m a in c lu s io n
c o n ju n c t ivit is [TRIC]) Lig n e o u s
Chlam ydia trachom atis serovars D to K (See Chapter 5, Pediatrics/Strabism us)
Chronic, follicular conjunctivitis
P a rin a u d ’s o c u lo g la n d u la r s yn d ro m e
Associated with urethritis (5%)
Monocular granulom atous conjunctivitis, with necrosis and
ulceration of follicles; fever, m alaise, lym phadenopathy; m ay
Fin d in gs: bulbar follicles, subepithelial infiltrates, no have rash
m em branes

Path o lo gy: follicles and granulom as

Tr eatm en t: doxycycline, also need to treat sexual partners
DDx: cat-scratch disease (Bartonella henselae), tularem ia,
Tra c h o m a sporotrichosis, TB, syphilis, LGV, Actinomyces, m ononucleosis,
Bilateral keratoconjunctivitis; leading cause of preventable Rickettsia, coccidioidom ycosis
blindness
Diagn o sis: PCR assay
Chlam ydia trachom atis serovars A to C

Re it e r’s s yn d r o m e (Re a c t ive a rt h rit is )

Fin d in gs (progressive): bilateral infection of upper tarsal
and superior bulbar conjunctiva, papillary reaction, Triad of urethritis, arthritis and conjunctivitis/uveitis;
conjunctival follicles, repeated infections, conjunctival scarring m ucopurulent conjunctivitis is the m ost com m on ocular
(Arlt’s line), tarsal shortening, entropion, trichiasis, corneal finding (30% to 50%), uveitis occurs in approxim ately 10%;
abrasion, superior corneal pannus, corneal scarring, cicatrized m ay have keratoderm a blenorrhagicum
lim bal follicles (Herbert’s pits)
Etio lo gy: non-gonocococcal urethritis, Chlamydia, Shigella
Classificatio n : and Salmonella bowel infection; 85% HLA-B27 positive
MacCallan (old):
STAGE 2 ¼ sim ple conjunctivitis with im m ature follicles S t a p h ylo c o c c a l d is e a s e
Stage 2A¼ m ostly follicles
STAGE 2B¼ m ostly follicles Blepharitis, conjunctivitis, keratitis (SPK, m arginal infiltrates),
STAGE 3 ¼ cicatrizing with trichiasis, entropion, horizontal phlyctenule
palpebral conjunctival scar (Arlt’s line)
STAGE 4 ¼ inactive with varying degrees of scarring, ptosis, Flo p p y e ye lid s yn d ro m e
xerosis
World Health Organization (See Chapter 6, Orbit/Lids /Adnexa)
TF5 TRACHOMATOUS INFLAMMATION (follicular):> 5
Autoeversion of eyelids during sleep with m echanical irritation
follicles larger than 0.5 m m on upper tarsus on bedsheets causes papillary reaction on tarsal conjunctiva
TI 5 TRACHOMATOUS INFLAMMATION (intense):
inflam m atory thickening obscuring > 50% of large, Associated with obesity, keratoconus, eyelid rubbing, and
deep tarsal vessels sleep apnea
TS 5 TRACHOMATOUS CICATRIZATION (scarring):
visible white lines or sheets of fibrosis (Arlt’s line) Tr eatm en t: lubrication, tape / patch / shield lids during
TT5 TRACHOMATOUS TRICHIASIS: at least one sleep; consider horizontal eyelid tightening
m isdirected eyelash
CO 5 CORNEAL OPACITY: obscuring at least part of pupil
m argin, causing vision worse than 20/60 Tum o rs

Path o lo gy: epithelial cells contain initial bodies (basophilic Ham ar to m a: growth arising from tissue norm ally found at
intracytoplasm ic inclusions of Halberstaedter and Prowazek); that site (e.g., nevus, neurofibrom a, neurilem m om a,
Leber’s cells (m acrophages in conjunctival strom a with schwannom m a, gliom a, hem angiom a, hem angiopericytom a,
phagocytosed debris) lym phangiom a, trichoepitheliom a)

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 Conjunctival Disorders

Ch o r isto m a: growth arising from tissue not norm ally found Tr eatm en t:
at that site (e.g., derm oid cyst, derm atolipom a, ectopic Adults: excisional biopsy with cryotherapy to rule out
lacrim al gland) dysplastic or carcinom atous lesion; incom plete excision
m ay result in m ultiple recurrences
Co n g e n it a l Tu m o rs Rare risk of m alignant transform ation
(See Chapter 5, Pediatrics/Strabism us)
Co n ju n c t iva l in t ra e p it h e lia l n e o p la s ia (CIN)
Cys t ic Tu m o rs Prem alignant lesion
S im p le Cys t Replacem ent of conjunctival epithelium by atypical dysplastic
Serous squam ous cells
Usually translucent or gelatinous appearance; < 10% exhibit
In c lu s io n c ys t leukoplakia (keratinization)

Clear cyst lined by norm al epithelium

Congenital or acquired (post surgery or traum a) Car cin o m a in situ : total replacem ent of epithelium by
m alignant cells; BM intact; no invasion into substantia propria;
Dislodged epithelium undergoes cavitation within strom a characterized by leukoplakia, thickened epithelium , and
abnorm al vascularization
Lined by nonkeratinized stratified squam ous epithelium ;
contains m ucin (goblet cells) Usually begins at lim bus and spreads onto cornea
Associated with HPV subtype 16 and 18 (check HIV in young
Tr eatm en t: com plete excision; recurs if not com pletely patient), and actinic exposure
excised
Men > wom en; occurs in older, fair-skinned individuals
S q u a m o u s Tu m o r s
Path o lo gy: dysplastic epithelium spreads anterior to
S q u a m o u s p a p illo m a Bowm an’s layer, fine vascularity with hairpin configuration
(sim ilar to papillom a), anaplastic cells, dyspolarity (Fig. 7-5)
Benign proliferation of conjunctival epithelium , appears as
sessile or pedunculated fleshy m ass with prom inent
vascular tufts Tr eatm en t: wide local excision with cryotherapy; rem ove
involved corneal epithelium (use fluorescein or rose bengal
to delim it); excise until m argins are clear. Consider topical
Sessile: broad base, usually older patients, often located at
5-fluorouracil, topical m itom ycin C or interferon alpha-2b
lim bus
(topical or subconjunctival)

Ped u n cu lated : usually caused by HPV, occurs in children, S q u a m o u s c e ll c a rc in o m a

often located near caruncle; frequently recurs after excision; can
regress spontaneously Malignant cells have broken through epithelial basem ent
m em brane
Path o lo gy: vascular cores covered by acanthotic, Most com m on m alignant epithelial tum or of conjunctiva;
nonkeratinized, stratified squam ous epithelium (see Fig. 7-4) rarely m etastasizes

Fig ure 7-4. Squamous papilloma demonstrating acanthotic epithelium with

blood vessels. (From Yanoff M, Fine BS: Ocular Pathology, ed 5, St Louis, 2002, Fig ure 7-5. CIN demonstrating full-thickness atypia and loss of polarity. (From
Mosby.) Yanoff M, Fine BS: Ocular Pathology, ed 5, St Louis, 2002, Mosby.)

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CHAPTER 7 • Cornea /External Disease

Most com m on in pigm ented individuals

No m alignant potential

Fre c k le
Congenital; increased m elanin in basal epithelium ; norm al
num ber of m elanocytes

Ne vu s
Congenital nests of benign nevus cells along basal epithelium
and / or substantia propria
20% to 30% am elanotic
Fig ure 7-6. Squamous cell carcinoma demonstrating cells in substantia propria Freely m ovable over globe
forming keratin pearls. From Yanoff M, Fine BS: Ocular Pathology, ed 5, St Louis,
2002, Mosby.) 50% have epithelial inclusion cysts
Often enlarges or becom es m ore pigm ented during puberty or
Appearance sim ilar to carcinom a in situ pregnancy

Associated with ultraviolet radiation, HPV, and sm oking Typ es (classified by location):
90% caucasian, 80% m ale Junctional: nevus cell confined to epithelial / subepithelial
junction (anterior to basem ent m em brane); often seen
Path o lo gy: invasive m alignant squam ous cells with during 1st and 2nd decades of life; appearance sim ilar to
penetration through basem ent m em brane (Fig. 7-6) PAM with atypia
Compound (m ost com m on): nevus cells in epithelial and
subepithelial locations; cystic or solid epithelial rests are
Tr eatm en t: wide excision (4 m m m argin) with rem oval of
very com m on; epithelial inclusion cysts com m on
surrounding conjunctiva, episclerectom y and corneal
Subepithelial: nevus cells confined to substantia propria;
epitheliectom y with 100% alcohol and cryotherapy (reduces
m alignant transform ation possible (Fig. 7-7)
recurrence rate from 40% to < 10%); consider topical
5-fluorouracil or m itom ycin-C or interferon-alpha2b
(topical or subconjunctival); enucleation for intraocular P rim a r y a c q u ire d m e la n o s is (P AM, a c q u ire d
involvem ent; exenteration and radiation therapy for m e la n o s is o c u li)
intraorbital spread
Unilateral, flat, diffuse, patchy, brown pigm entation; waxes
and wanes
Pr o gn o sis: 2% to 8% intraocular invasion, 12% to 16%
introrbital invasion, m etastases rare; up to 8% m ortality rate Proliferation of intraepithelial m elanocytes; no cysts (Fig. 7-8)
Most frequently on bulbar conjunctiva or in fornices, but can
Mu c o e p id e rm o id c a rc in o m a occur on palpebral (tarsal) conjunctiva
Rare, aggressive variant of squam ous cell carcinom a with Analogous to lentigo m aligna of skin
m alignant goblet cells
Occurs in m iddle-aged to elderly whites
Typically occurs in individuals > 60 years old
Very aggressive, can invade globe through sclera
Suspect in cases of recurrent squam ous cell carcinom a

Path o lo gy: epiderm oid and m ucinous com ponents; stains

with m ucicarmine, Alcian blue, and colloidal iron

Tr eatm en t: wide local excision with cryotherapy; high

recurrence rate

Me la n o c yt ic Tu m o rs
Ra c ia l m e la n o s is
Bilateral, light brown, flat, perilim bal pigm entation; increased Fig ure 7-7. Nevus with subepithelial rests of nevus cells. (From Yanoff M, Fine
m elanin in basal epithelium BS: Ocular Pathology, ed 5, St Louis, 2002, Mosby.)

206
 Conjunctival Disorders

Fig ure 7-8. PAM with pigmentation throughout the epithelium. (From Yanoff M, Fig ure 7-9. Malignant melanoma appears as pigmented tumor with los s of
Fine BS: Ocular Pathology, ed 5, St Louis, 2002, Mosby.) polarity. (From Yanoff M, Fine BS: Ocular Pathology, ed 5, St Louis, 2002,
Mosby.)

Low risk of m alignant transform ation, nodular thickening is Pr o gn o sis: 25% to 40% m ortality; 25% risk of m etastases
indication for excisional biopsy (regional lym ph nodes and brain); m ore likely to invade sclera
than SCC
PAM with o u t atyp ia an d PAM with m ild atyp ia: no
risk for progression to m elanom a If m ore than 2 m m thick, increased risk of m etastasis and
m ortality
PAM with sever e atyp ia: 13% risk for progression to
Involvem ent of caruncle, fornices, palpebral conjunctiva has
m elanom a
worst prognosis
Greatest risk factor is extent in clock hours
Better prognosis than cutaneous m elanom a
Tr eatm en t: observe with photographs; biopsy thickened Exenteration does not im prove survival
areas (does not increase risk of m etastasis); excisional biopsy
with cryotherapy, consider topical interferon alpha-2b or
Va s c u la r Tu m o rs
m itom ycin C for recurrence; com plete excision if m alignant
P yo g e n ic g ra n u lo m a
Co n g e n it a l o c u la r m e la n o s is Exuberant proliferation of granulation tissue
(See Chapter 5, Pediatrics/Strabism us) Vascular m ass with sm ooth convex surface occurring at site of
previous surgery (usually strabism us or chalazion excision)
S e c o n d a ry a c q u ire d c o n ju n c t iva l m e la n o s is
Path o lo gy: loose fibrous strom a containing m ultiple
Addison’s disease, radiation, pregnancy, topical epinephrine capillaries and inflam m atory cells

Tr eatm en t: topical steroids; excision with conjunctival graft

Ma lig n a n t m e la n o m a or cryotherapy
Rare, variably pigm ented, elevated m ass m ost com m only on
bulbar conjunctiva Ka p o s i’s s a rc o m a

May see feeder vessel Red m ass, often m ultifocal

Arises from PAM (70%) or preexisting nevi (20%), or de novo Stages 1 an d 2: patchy and flat, < 3 m m in height,
(10%); 2% of ocular m alignancies < 4 m onths in duration

Stage 3: m ore nodular, > 3 m m in height, longer duration

Path o lo gy: intraepithelial pagetoid spread; need to bleach Associated with AIDS (20%)
specim en to determ ine am ount of atypia (Fig. 7-9), stain with
S-100 and HMB-45 Path o lo gy: proliferation of capillaries, endothelial cells, and
fibroblast-like cells (Fig. 7-10)
Tr eatm en t: docum ent with photos; com plete excision
(no touch technique) with clear m argins, episclerectom y, Tr eatm en t: excision, XRT, paclitaxel (Taxol) (inhibits
corneal epitheliectom y with 100% alcohol and cryotherapy; m itosis)
consider adjuvant topical m itom ycin C; adjuvant radiotherapy
(plaque or proton beam ); exenteration now rare Ca ve rn o u s h e m a n g io m a
Recurrence m ay be am elanotic Red patch; m ay bleed

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CHAPTER 7 • Cornea /External Disease

Ot h e r Tu m o rs
Fib ro u s h is t io c yt o m a
Yellow-white m ass com posed of fibroblasts and histiocytes
Extends from lim bus to peripheral cornea

Path o lo gy: storiform pattern

Tr eatm en t: local excision

Be n ig n h e re d it a ry in t ra e p it h e lia l d ys ke ra t o s is
(BHID) (AD)
Mapped to chrom osom e 4q35
Fig ure 7-10. Kaposi’s sarcoma demonstrating neoplastic cells and vascular
spaces. (From Yanoff M, Fine BS: Ocular Pathology, ed 5, St Louis, 2002, Originally seen in triracial fam ilies in Halifax County, North
Mosby.) Carolina (Haliwa Indians)

Associated with other ocular hem angiom as or system ic disease Usually presents in 1st decade of life

Path o lo gy: endothelial lined canals with RBCs Sym p to m s: itching, burning, photophobia

Lym p h a n g io m a Fin d in gs: bilateral dyskeratotic lesions (plaques with

gelatinous base and keratinized surface) involving bulbar
Cluster of clear cysts; m ay have areas of hem orrhage
conjunctiva near lim bus; m ay have sim ilar plaques on buccal
m ucosa or oropharynx
Path o lo gy: dilated lym phatic vessels

Tr eatm en t: excision if sm all Path o lo gy: acanthosis, dyskeratosis, prom inent rete pegs;
no m alignant potential
Lym p h o id Tu m o r s
DDx: Bitot’s spot, pingueculum , squam ous papillom a,
(See Chapter 6, Orbit/Lids /Adnexa) squam ous cell carcinom a
Sm ooth, flat, fleshy, salm on-colored m ass; single or m ultiple
Tr eatm en t: observe; topical steroids (severe sym ptom s);
Occurs in substantia propria; overlying epithelium is sm ooth;
excision (diagnostic; lesions recur)
can be bilateral
Most com m only in fornix, m ay involve orbit P s e u d o e p it h e lio m a t o u s h yp e rp la s ia
20% associated with system ic disease (but system ic lym phom a Benign proliferation of conjunctiva onto corneal epithelium
rarely presents in conjunctiva)
Usually occurs away from lim bus (in contrast to squam ous
Spectrum of disease from benign to m alignant (non-Hodgkin’s; hyperplasia or carcinom a)
less aggressive MALT [mucosa-associated lym phoid tissue] or
m ore m alignant non-MALT); cannot distinguish clinically; Develops over weeks to m onths
requires biopsy with imm unohistochemical studies (fresh,
unfixed tissue specimen)for diagnosis Fin d in gs: raised, whitened hyperkeratotic surface (cannot
differentiate clinically from dysplasia)
Usually m iddle-aged adults
Requires system ic workup, including CT scan, bone scan, SPEP, Path o lo gy: thickened squam ous epithelium ; occasional
m edical consultation m itotic figures; no atypia; no clear dem arcation between
norm al and abnorm al cells
DDx: leukem ia, m etastases

Tr eatm en t: low-dose XRT, surgery, local chem otherapy Tr eatm en t: excision

Me t a s t a t ic Tu m o rs Ca ru n c le Tu m o r s
Very rare to conjunctiva
Be n ig n
Fleshy yellow-pink m ass
Papillom a (30%), nevus (25%), inclusion cyst, sebaceous
Breast, lung, cutaneous m elanom a (usually pigm ented) hyperplasia, sebaceous adenom a, pyogenic granulom a

208
 Corneal Disorders

On co cyto m a (oxyphilic adenom a): arises from m etaplasia Associated with epithelial basem ent m em brane dystrophy
of ductal and acinar cells of accessory lacrim al glands; (EBMD) in 50% or previous corneal abrasion; also occurs in
com posed of polyhedral cells arranged in nests, cords, or other corneal dystrophies
sheets; eosinophilic cytoplasm correlates with abundance of
Abnorm al adhesion of epithelium to Bowm an’s layer allows
m itochondria; more com m on in wom en
spontaneous sloughing

Ma lig n a n t (5 % ) Sym ptom s usually occur in m orning upon awakening

(epithelium swells 4% overnight, and m echanical force of lids
Squam ous cell carcinom a, m alignant m elanom a, sebaceous rubbing across corneal surface when open eyes or blink can
adenocarcinom a dislodge epithelium )
Duration of sym ptom s depends on size of erosion
CORNEAL DISORDERS
Fin d in gs: epithelial defect (m ay be partially healed),
conjunctival injection; m ay have m ild AC reaction and signs of
Co ng e nita l corneal dystrophy (i.e., ABMD with irregular epithelium , areas
of negative stain)
(See Chapter 5, Pediatrics/Strabism us)
Tr eatm en t: lubrication, hypertonic saline (Muro 128 5%
ointm ent qhs  3-12 m onths); if recurs, consider bandage
Tra um a contact lens, epithelial d ebridem ent, anterior strom al
puncture / reinforcem ent, diam ond burr (d ebride and polish
Ab ra s io n Bowm an’s layer), laser (phototherapeutic keratectom y [PTK]
with excim er laser, or m icropuncture with Nd :YAG laser (1 m J,
Epithelial defect, m ost com m only traum atic (e.g., fingernail,
aim just below epithelium )); also, consider treatm ent with
plant branch)
m atrix m etalloproteinase-9 inhibitors (doxycycline 50 m g PO
Increased risk of infection, especially in contact lens wearer bid  2 m onths and topical steroids tid  2-3 weeks);
d ebridem ent for subepithelial scarring
Sym p to m s: foreign body sensation, pain, tearing, redness,
photophobia
Burns
Fin d in gs: epithelial defect stains with fluorescein,
conjunctival injection, ciliary flush; m ay have m ild AC reaction
Acid :
denatures and precipitates tissue proteins
Tr eatm en t: topical antibiotic, consider topical NSAID,
Sulfuric (batteries): m ost com m on
cycloplegic, bandage contact lens, or patching for pain control
Sulfurous (bleach): penetrates m ore easily than other acids
(never patch CL wearer because of increased risk of m icrobial
Hydrofluoric (glass polishing / etching): increased
keratitis)
penetration
Hydrochloric: severe burn only with high concentration
Fo re ig n b o d y (FB) Acetic: m ild burn if concentration < 10%
May be superficial or deep Pathology: superficial coagulative necrosis of conjunctiva
and corneal epithelium
Often m etal (usually associated with adjacent rust ring), glass,
or organic m aterial
Alkali: denatures but does not precipitate proteins, also
Tr eatm en t: rem oval of FB, topical antibiotic; old deep inert saponifies fat; therefore penetrates deeply
m aterial m ay be observed Lime (plaster, cem ent): m ost com m on; toxicity increased
by retained particulate m atter; less penetration
La c e ra t io n Ammonia (fertilizers), lye (drain cleaners): produce m ost
serious injury
Partial- or full-thickness cut in cornea Also, MgOH (fireworks), KOH
Pathology: conjunctival and corneal epithelial loss with
Requires surgical repair and topical antibiotic and steroid
corneal clouding and edem a; conjunctival and corneal
Com plications include scarring and irregular astigm atism , necrosis; ischem ia from throm bosis of conjunctival and
which if affect vision, m ay be treated with rigid contact lens or episcleral vessels
keratoplasty

Rad iatio n : therm al burn (sim ilar to acid burn)

Re c u r re n t e ro s io n
UV light (snow blindness, arc-welding): punctate epithelial
Spontaneous epithelial defect keratitis 8 to 12 hours after exposure; com pletely resolves

209
CHAPTER 7 • Cornea /External Disease

Ionizing radiation: superficial keratitis; then strom al Aq u e o u s d e fic ie n c y

disruption from keratocyte dam age; corneal drying Lacrim al gland dysfunction (aplasia, surgical rem oval),
secondary to keratinization of conjunctiva infiltration (sarcoidosis, thyroid disease, lym phom a,
Gr ad in g system s: am yloidosis, TB), inflam m ation (m um ps, Sjo€ gren syndrom e),
Grade I: corneal epithelial dam age, no ischem ia; full denervation (Riley-Day [fam ilial dysautonom ia], Shy-Drager
recovery [adult dysautonom ia, idiopathic autonom ic dysfunction],
Grade II: strom al haze but iris details seen, ischem ia < one- Mo€ bius’ syndrom e), drugs that decrease lacrim al secretion
third of lim bus; good prognosis, som e scarring (antihistam ines, diuretics, anticholinergics, psychotropics),
Grade III: total corneal epithelial loss, strom al haze, androgen deficiency
obscuration of iris; ischem ia from one-third to one-half
of lim bus; guarded prognosis
Grade IV: cornea opaque; ischem ia > one-half of lim bus; Ke ra t o c o n ju n c t ivit is s ic c a
poor prognosis, risk of perforation Adult wom en (95%), often associated with Sjo€ gren syndrom e
McCulley Classification:
1. IMMEDIATE PHASE: extent of ocular surface
Diagn o stic cr iter ia fo r Sj o€ gr en
involvem ent
syn d r o m e: Keratoconjunctivitis sicca
2. ACUTE PHASE: 0–7 days
Xerostom ia (decreased parotid flow rate)
3. EARLY REPARATIVE PHASE: 7–21 days
Lym phocytic infiltration on labial salivary gland biopsy
4. LATE REPARATIVE PHASE: 3 weeks to several m onths
Laboratory evidence of system ic autoim m une disease
Tr eatm en t: Copious irrigation; d ebridem ent of necrotic (positive ANA titer, rheum atoid factor, or SS-A or SS-B
conjunctiva and particulate m atter; check pH level antibody; associated with HLA-B8 [90%])
Topical (10%) and oral sodium ascorbate (aids collagen
synthesis and scavenges superoxide radicals)
Sym p to m s: burning, dryness, foreign body sensation,
Collagenase inhibitors (acetyl cysteine or EDTA)
redness, tearing; worse later in day, with prolonged use of eye,
Steroids for first 5 to 10 days only (reduce corneal and
and in dry or windy environm ents
intraocular inflam m ation, help prevent sym blepharon
but can enhance collagenase-induced corneal m elting,
which often begins 1–2 weeks after injury) Fin d in gs: decreased tear meniscus height (< 0.2 mm),
Topical antibiotic decreased tear breakup time (< 10 seconds), increased mucus,
Ocular hypotensive m edication for elevated IOP filaments in severe cases, interpalpebral corneal and conjunctival
Cycloplegic staining with rose bengal/lissam ine green or fluorescein,
Citrate (chelates calcium ) Schirmer’s test (< 10 mm/5 m in) or Phenol red thread test (< 10
During first 1 to 3 weeks: mm/15 seconds), increased tear osmolarity (> 316 mOsm/L),
PROMOTE EPITHELIAL HEALING: artificial tears, increased matrix m etalloproteinase-9 level (> 40 ng/m L)
tarsorrhaphy, bandage CL; consider am niotic
m em brane, Prokera or Boston ocular surface Path o lo gy: lym phocytic infiltration of lacrim al gland
keratoprosthesis (PROSE lens)
LIMIT ULCERATION AND SUPPORT Tr eatm en t: lubrication with artificial tears and ointm ents,
REPAIR: ascorbate  21 days, steroids  1 week; if lacrisert, punctal occlusion, hum idifier, m oisture cham ber
epithelium does not heal, consider conjunctival flap, goggles; consider acetylcysteine (Mucom yst) or bandage
tissue glue, or keratoplasty to prevent perforation contact lens for filam ents; topical cyclosporine (Restasis),
TREAT SYMBLEPHARON: lyse with glass rod; consider topical lifitegrast (Xiidra), short course of topical steroid;
vitam in A to im prove goblet cell function autologous serum drops or Boston ocular surface
TREAT GLAUCOMA: topical m edication; m ay eventually keratoprosthesis (PROSE lens)
require surgery
SURGERY: limbal autograft or keratoepithelioplasty Miku licz’s syn d r o m e: lacrim al and parotid gland swelling
(if epithelium not healed after 3 weeks); penetrating and keratoconjunctivitis sicca due to sarcoidosis, TB,
keratoplasty (poor results; better if perform ed 1–2 years lym phom a, leukem ia
after injury; may need limbal stem cell transplant)
Co m p licatio n s: cataract, glaucom a, uveitis, sym blepharon, Mu c in De fic ie n c y
entropion, xerosis, retrocorneal m em brane, neurotrophic Goblet cell dysfunction due to conjunctival scarring and
keratitis, corneal ulceration, anterior segm ent ischem ia, and keratinization (vitam in A deficiency, OCP, Stevens-Johnson
neovascularization syndrom e, alkali burns, trachom a, GVH disease); im pression
cytology m easures num ber of goblet cells
Oc ula r Surfa c e Dis e a s e
Xe ro p h t h a lm ia
Causes tear film disturbance and dry eye Epithelial keratinization due to vitam in A deficiency
Due to deficiency in tear film com ponent(s); usually Vitam in A required for conjunctival goblet cell m ucin
m ultifactorial production, corneal strom al m etabolism , retinal

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 Corneal Disorders

photoreceptor m etabolism , norm al iron m etabolism , norm al Fin d in gs: sym blepharon, ankyloblepharon, trichiasis,
growth, resistance to m easles entropion, subconjunctival fibrosis, severe dry eye, corneal
ulcers, vascularization, and scarring
Incidence 5 m illion new cases / year; affects 20-40 m illion
children worldwide
Oth er fin d in gs:
Usually in developing countries (50% in India); in developed Skin: recurrent vesiculobullous lesions of inguinal region
countries, due to lipid m alabsorption (short bowel syndrom e, and extrem ities, scarring lesions on scalp and face
chronic liver dysfunction, cystic fibrosis) and poor diet Mucous membranes (nose, pharynx, larynx, esophagus):
(chronic alcoholism ) strictures, dysphagia

Fin d in gs: night blindness (nyctalopia), conjunctival xerosis Path o lo gy: occurs at level of basem ent m em brane
(leathery appearance) and Bitot’s spots (gray foam y plaques), (subepithelial bullae); epithelial thinning, loss of goblet cells,
corneal xerosis with keratom alacia, ulceration, and scarring, keratinization, subepithelial inflam m ation and fibrosis; IgA in
xerophthalm ic fundus (fine white m ottling) conjunctival basem ent m em brane zone; m ostly polys; antigen-
antibodies are deposited below epiderm is
Pemphigus vulgaris: antigen-antibodies located within
Tr eatm en t: 200,000 IU vitam in A (IM or PO) epithelium (intraepithelial acantholysis with epithelial
bullae)
S t e ve n s -J o h n s o n s yn d ro m e
Young people; 25% recurrence, 10–33% m ortality Co n ju n ctival scr ap in g: lym phocytes, plasm a cells,
eosinophils
Etio lo gy: drugs (sulfa, antibiotics, barbituates, phenytoin
[Dilantin]), infection (herpes, Mycoplasma), idiopathic Tr eatm en t: lubrication, im m unosuppressive therapy,
dapsone, cyclophospham ide, surgery; consider punctal
Fin d in gs: acute pseudom em branous conjunctivitis, occlusion, tarsorrhaphy, Boston ocular surface
sym blepharon, persistent epithelial defects, dry eye, trichiasis, keratoprosthesis (PROSE lens)
corneal scarring, vascularization, erosions, and ulcers Penetrating keratoplasty: poor success rate
Keratoprosthesis: lim ited success for end-stage disease
Oth er fin d in gs:
Erythema multiforme: cutaneous bullous eruptions (target Pr o gn o sis: rem issions and excacerbations
lesions), crusting, rash involves face, m ucosal ulceration
and strictures, sore throat, fever, arthralgias Lip id De fic ie n c y
ERYTHEMA MULTIFORME MINOR: no m ucous
m em brane involvem ent
ERYTHEMA MULTIFORME MAJOR: m ucous m em brane Me ib o m ia n g la n d d is e a s e / d ys fu n c t io n (MGD,
ulceration; 20% m ortality from secondary infection p o s t e rio r b le p h a rit is , m e ib o m it is , a c n e ro s a c e a )
(see Chapter 6, Orbit / Lids / Adnexa)
Path o lo gy: occurs at m ucocutaneous junction
Early: epithelial thinning with fibrinous exudate and Approxim ately 86% of patients with dry eye disease have
strom al lym phocytic infiltration signs of MGD
Late: patchy epiderm alization with keratinization and
subepithelial fibrosis Ot h e r Fa c t o rs

Tr eatm en t: topical antibiotic, steroid, lubrication, Lid ab n o r m alities: increased evaporative loss
sym blepharon lysis, surgery; consider oral steroids, punctal (lagophthalm os, Bell’s palsy, ectropion, entropion, thyroid
occlusion, tarsorrhaphy, Boston ocular surface disease); treat with lid taping, tarsorrhaphy, surgical repair of
keratoprosthesis (PROSE lens) underlying abnorm ality

Oc u la r c ic a t ric ia l p e m p h ig o id Agin g: reduced basal tear secretion

Bullous disease of m ucous m em branes resulting in scarring
En vir o n m en t: wind, low hum idity
Usually wom en age > 60 years old
Med icatio n s: antihistam ines, phenothiazines,
Etio lo gy: probably autoim m une (im m une com plexes anticholinergics, β-adrenergic blocking agents, diuretics,
present in basem ent m em brane zone) retinoids, estrogens, preservatives (benzalkonium chloride,
thim erosal, polyquad)
Associated with HLA-B12 and with m edications (pilocarpine,
phospholine iodide, tim olol, epinephrine, idoxuridine) Contact lens wear: can cause reduced tear production

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CHAPTER 7 • Cornea /External Disease

Th yg e s o n ’s s u p e rfic ia l p u n c t a t e ke ra t o p a t h y
Infla m m a tio n
Associated with HLA-DR3; 90% bilateral; spontaneous
In t e rs t it ia l ke ra t it is rem issions and exacerbations for years
Etio lo gy:
Bacteria: syphilis (congenital [90%; m ost com m on cause Sym p to m s: photophobia, foreign body sensation, burning,
of bilateral IK] or acquired), relapsing fever (Borrelia), TB tearing
(unilateral, sectoral), leprosy, LGV
Virus: HSV, EBV, m easles, m um ps, rubella, influenza, Fin d in gs: coarse, punctate, gray-white snowflake opacities
sm allpox, vaccinia in corneal epithelium with faint subepithelial haze; raised
Protozoa: leishmaniasis, African sleeping sickness (Taenia center breaks through epithelial surface and stains with
cruzi), malaria, onchocerciasis, cysticercosis (Taenia solium) fluorescein during acute attack; during rem issions, inactive
Other causes: sarcoidosis, Hodgkin’s disease, Kaposi’s lesions appear flat and do not stain; no associated
sarcom a, m ycosis fungoides, incontinentia pigm enti, conjunctivitis or iritis
hidradenitis suppurativa, Cogan’s syndrom e (vertigo,
tinnitus, hearing loss, IK)
Can be initiated by m inor corneal traum a in patients with Tr eatm en t: topical steroids (sym ptom atic relief and
congenital syphilis rapid resolution of lesions, but m ay prolong course of
disease), therapeutic contact lens, trifluoridine (Viroptic; m ay
Fin d in gs: neovascularization appears as salm on patch, help, but idoxuridine does not work and can cause
results in hazy corneal scarring, regression of vessels leaves subepithelial scarring), topical cyclosporine (m ay also be
ghost vessels (clear branching pattern within the scar); m ay beneficial)
develop secondary glaucom a from iris/angle dam age Without treatment sym ptom s last for 1–2 m onths, then
rem ission; recurrences can begin 6–8 weeks later
Path o lo gy: diffuse lym phocytic infiltrate with thickened
corneal strom a
Fila m e n t a r y ke ra t it is
Tr eatm en t: topical steroids Strands com posed of m ucus and desquam ated epithelial cells
adherent to cornea at one end
Co g a n ’s s yn d ro m e Due to increased m ucus production and abnorm al epithelial
Ocular inflam m ation (usually IK) with Meniere’s-like turnover
vestibular dysfunction
Most likely autoim m une Etio lo gy: keratoconjunctivitis sicca, SLK, recurrent erosions,
bullous keratopathy, prolonged patching, HSV keratitis,
Affects young adults, average age of onset is 29 years neurotrophic keratitis, neuroparalytic keratopathy, ectoderm al
Associated with antecedent upper respiratory illness (50%) and dysplasia, traum a, atopic derm atitis, adenoviral
vascular inflam m atory disease (10%; aortic insufficiency, keratoconjunctivitis, topical m edication toxicity
aortitis, necrotizing large vessel [Takayasu’s-like], and (m edicam entosa), ptosis
polyarteritis nodosa) No filam ents in OCP because m ucus production is dim inished
in this disorder
Sym p to m s: pain, redness, photophobia

Fin d in gs: SEIs, IK, conjunctivitis, iritis, scleritis, episcleritis Tr eatm en t: acetylcysteine (Mucom yst), lubrication, rem ove
filam ents, bandage contact lens, treat underlying disorder
Oth er fin d in gs: sudden onset of Meniere’s-like sym ptom s
(nausea, em esis, tinnitus, decreased hearing, severe vertigo;
m ay have nystagm us); 80% progress to deafness without De g e ne ra tio ns
system ic steroids
Pannus
DDx o f ker atitis with vestib u lo au d ito r y
Peripheral ingrowth of subepithelial fibrovascular tissue,
sym p to m s: syphilis, polyarteritis nodosa, Wegener’s usually superiorly
granulom atosis, sarcoidosis, VKH, sym pathetic ophthalm ia,
cerebellopontine angle tum or
In flam m ato r y: destruction of Bowm an’s layer (i.e.,
Au d io gr am : m ost pronounced loss at extrem e frequencies, trachom a)
relative sparing of m idrange
Degen er ative: Bowm an’s layer rem ains intact; m ay contain
Tr eatm en t: system ic steroids to prevent perm anent fatty plaque deposits (i.e., chronic edem a)
hearing loss Most com m only due to contact lens wear

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 Corneal Disorders

Wh it e lim b a l g ird le o f Vo g t S p h e ro id a l d e g e n e ra t io n (La b ra d o r ke ra t o p a t h y,

Sm all, white, fleck and needle-like deposits at tem poral and a c t in ic ke ra t o p a t h y, lip id d ro p le t d e g e n e ra t io n ,
nasal lim bus Bie t t i’s h ya lin e d e g e n e ra t io n , ke ra t in o id
Pathology: subepithelial elastotic degeneration of collagen d e g e n e ra t io n )
(som etim es with calcium particles)
Bilateral; m ale > fem ale

Co rn e a l a rc u s
Etio lo gy: com bination of genetic predisposition, actinic
Ar cu s sen ilis: hazy white peripheral corneal ring with exposure, and age
intervening clear zone between lim bus
Typ es:
Ar cu s ju ven ilis: arcus in person < 40 years old; associated Type 1 (m ost com m on): involves peripheral cornea in
with hyperlipoproteinem ia types 2, 3, and 4 horizontal m eridian; occurs after age 30
Type 2: associated with other corneal pathology; m ay
Path o lo gy: lipid deposition in strom a involve central cornea; occurs earlier in life
Type 3: involves conjunctiva
DDx: lecithin cholesterol acyltransferase (LCAT) deficiency, Usually asym ptom atic
fish eye disease, Tangier’s disease
Fin d in gs: translucent, golden brown, spherical, superficial,
Car o tid u ltr aso u n d : for unilateral arcus senilis (m ay have strom al and conjunctival proteinaceous deposits
stenosis on uninvolved side)
Path o lo gy: extracellular basophilic m aterial
Fu rro w d e g e n e ra t io n
Thin area peripheral to arcus senilis, m ore apparent than real; Co rn e a fa rin a t a (AD)
nonprogressive; asym ptom atic Tiny, dot- and com m a-shaped, deep strom al opacities; m ay
contain lipofuscin (degenerative pigm ent)
Cro c o d ile s h a g re e n Involutional change
Mosaic, polygonal, hazy, gray opacities separated by clear
zones; ”cracked-ice” appearance Ha s s a ll-He n le b o d ie s
Extends to periphery Sm all, peripheral, wart-like excrescences (gutatta) of
Descem et’s m em brane (protrude toward AC)
An ter io r cr o co d ile sh agr een : occurs at level of
Norm al senescent change; rare before age 20 years
Bowm an’s layer

Po ster io r cr o co d ile sh agr een : occurs at level of

Descem et’s m em brane
De p o s itio ns

S a lz m a n n ’s n o d u la r d e g e n e ra t io n Ba n d ke ra t o p a t h y
Interpalpebral band of subepithelial hazy white opacities with
Blue-white elevated nodules; m ore com m on in older fem ales
ground-glass, Swiss-cheese appearance; begins at lim bus

Etio lo gy: chronic inflam m ation (e.g., old phlyctenulosis,

trachom a, IK, staph hypersensitivity) Etio lo gy: uveitis, interstitial keratitis (IK), superficial
keratitis, phthisis, sarcoidosis, traum a, intraocular silicone oil,
system ic disease (hypercalcem ia, vitam in D intoxication,
Path o lo gy: replacem ent of Bowm an’s layer by hyaline and Fanconi’s syndrom e, hypophosphatem ia, gout, “m ilk-alkali”
fibrillar m aterial
syndrom e, m yotonic dystrophy, chronic m ercury exposure)

Tr eatm en t: superficial keratectom y, PTK; m ay recur after

Path o lo gy: calcification of epithelial basem ent m em brane,
Bowm an’s layer, and anterior strom a, with destruction of
P o lym o r p h ic a m ylo id d e g e n e ra t io n Bowm an’s layer; calcium salts are extracellular when process is
Bilateral, sym m etric, sm all stellate flecks or filam ents in deep due to local ocular disease, calcium salts are intracellular when
strom a process is due to alteration of system ic calcium m etabolism

Slowly progressive; usually seen in patients > 50 years old;

Tr eatm en t: chelation with topical disodium EDTA
asym ptom atic
Gout and hyperuricem ia cause brown band from deposition
Not associated with system ic am yloid of urate

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CHAPTER 7 • Cornea /External Disease

Lip id ke ra t o p a t h y Copper deposition in

Basal ganglia: spasticity, dysarthria, trem or, ataxia
Diffuse or crystalline yellow strom al deposits
Liver: cirrhosis
Due to lipid exudation from corneal vascularization Eye: Kayser-Fleischer ring (copper deposition in peripheral
Descem et’s; starts superiorly, then inferiorly, m edially,
Co a t ’s w h it e rin g and tem porally); sunflower cataract

Sm all, discrete, gray-white dots

DDx o f Kayser -Fleisch er r in g: prim ary biliary cirrhosis,
Follows m etallic foreign body chronic active hepatitis, m ultiple m yelom a, chronic cholestatic
jaundice
Mu c o p o lys a c c h a r id o s e s
(See Chapter 5, Pediatrics/Strabism us) Tr eatm en t: oral tetrathiom olybdate (penicillam ine),
followed by oral zinc m aintenance; Kayser-Fleischer rings
resolve with adequate treatm ent
S p h in g o lip id o s e s
(See Chapter 5, Pediatrics/Strabism us)
Ch a lc o s is
Dys lip o p r o t e in e m ia s Copper (Descem et’s), due to intraocular foreign body
com posed of < 85% copper
Fish eye d isease (AR): m apped to chrom osom e 16q22;
diffuse corneal clouding, denser in periphery
S id e ro s is
Hyp er lip o p r o tein em ia typ es 2, 3, an d 4: arcus Iron (strom a)

LCAT d eficien cy: m apped to chrom osom e 16; dense arcus Iro n lin e s
and diffuse, fine, gray strom al dots
Due to stagnation of tears (basal epithelium )
Tan gier ’s d isease (AR): HDL deficiency, relapsing
polyneuropathy, sm all deep strom al opacities Fleisch er r in g: base of cone in keratoconus

Hyp e rg a m m a g lo b u lin e m ia Sto cker ’s lin e: head of pterygium

Crystals
Fer r y’s lin e: adjacent to filtering bleb
Cys t in o s is
(See Chapter 5, Pediatrics/Strabism us) Hu d so n -St €
a h li lin e: horizontal at lower ⅓ of cornea;
norm al aging, nocturnal exposure
Oc h ro n o s is (Alka p t o n u ria ) (AR)
Arg yr o s is
Mapped to chrom osom e 3q2
Silver (deep strom a / Descem et’s)
Melanin-like pigm ent (alkapton; peripheral epithelium and
superficial strom a)
Ch rys ia s is
Tyro s in e m ia t yp e II (Ric h n e r-Ha n h a rt Gold (peripheral deep strom a)
s yn d ro m e ) (AR)
Mapped to chrom osom e 16q22 Kru ke n b e rg ’s s p in d le
Deposits in epithelium and subepithelial space due to tyrosine Melanin (on endothelium )
am inotransferase deficiency
Refractile branching linear opacities, m ay have dendritic P la n t s a p
pattern
From Dieffenbachia
Triad of painful hyperkeratotic skin lesions (on palm s and
soles), keratitis, and m ental retardation Co rn e a ve rt ic illa t a (Vo r t e x ke ra t o p a t h y)

Wils o n ’s d is e a s e (He p a t o le n t ic u la r Brown deposits in whorl pattern (epithelium ) due to Fabry’s

disease and m edications (m ost com m only am iodarone,
d e g e n e ra t io n ) (AR)
chloroquine, indom ethacin, ibuprofen, naproxen, tam oxifen,
Increased copper levels due to deficiency in ceruloplasm in suram in, clofazim ine, and phenothiazines)

214
 Corneal Disorders

Fin d in gs: leading edge of lipid, steep central edge, sloping

Ulc e rs peripheral edge, intact epithelium , superficial vascularization;
can progress circum ferentially or centrally; induces ”against-
Co rn e a l Me lt (No n in fe c t io u s Ulc e r) the-rule” astigm atism ; m ay perforate with m ild traum a, rarely
spontaneously

No n im m u n e m ed iated : traum atic, eyelid abnorm alities S t a p h m a rg in a l u lc e r

(entropion, ectropion, trichiasis, exposure, lagophthalm os),
neurotrophic cornea, acne rosacea, keratom alacia, gold toxicity Hypersensitivity reaction

Fin d in gs: initial subepithelial infiltrate with peripheral clear

System ic im m u n e m ed iated : prim ary zone, progresses to shallow ulcer; adjacent conjunctival
keratoconjunctivitis sicca, Sjo€ gren syndrome, ocular cicatricial injection; blepharitis
pemphigoid, Stevens-Johnson syndrome, rheumatoid arthritis,
SLE, Wegener’s granulomatosis, polyarteritis nodosa Tr eatm en t: resolves spontaneously, topical steroids m ay
help; treat blepharitis

Lo calized im m u n e m ed iated : Mooren’s ulcer, Staph

m arginal ulcer, vernal keratoconjunctivitis
Ma rg in a l ke ra t o lys is / P e rip h e ra l u lc e ra t ive
ke ra t it is (P UK)
P e rip h e ra l Co rn e a l Ulc e rs Ulceration is typically peripheral and unilateral, can be central
and bilateral
Mo o re n ’s u lc e r Due to elevated collagenase; melting stops when epithelium heals
Chronic, very painful, progressive ulceration Associated with dry eyes (Sjo€ gren’s) and sytem ic disease:
Typically begins nasally or tem porally and spreads Rheumatoid arthritis: painless guttering or acute painful
circum ferentially (up to 360°) ulceration; also m ay have scleritis
Type II hypersensitivity reaction Wegener’s granulomatosis: 60% have ocular involvem ent,
Associated with hepatitis C, Crohn’s disease, and hydradenitis m ost com m only PUK; also, m ay have scleritis,
conjunctivitis, orbital involvem ent with proptosis,
Sym p to m s: photophobia, decreased vision (irregular retinal vasculitis, and AION
astigm atism ) Polyarteritis nodosa: m ay be presenting feature; also m ay
have pale yellow, waxy, raised, friable conjunctival
vessels, scleritis, conjunctivitis, and retinal vasculitis
Fin d in gs: underm ined leading edge with overhanging SLE: rare m anifestation
m argin, absent epithelium in active areas; m ay have Scleroderma: also m ay have keratoconjunctivitis sicca and
conjunctival injection trichiasis
Others: relapsing polychondritis, inflam m atory bowel
disease, Behçet’s disease
Var ian ts:
USA: unilateral disease of elderly (m ales ¼ fem ales), Diagn o sis: blood tests as for scleritis (CBC, ESR, ANA,
usually less aggressive; perforation rare RF, ANCA)
Africa: severe bilateral disease in young patients; rapidly
progressive with risk of perforation Tr eatm en t: lubrication, punctal occlusion, tarsorrhaphy;
Diagnosis of exclusion (rule out rheum atologic and consider im m unosuppressive agents (topical cyclosporine),
autoim m une diseases) conjunctival recession or resection; treat underlying disease

Path o lo gy: adjacent conjunctiva contains increased plasm a Fu c h s ’ s u p e rfic ia l m a rg in a l ke ra t it is

cells, im m unoglobulin, and com plem ent Marginal infiltrates initially, then pseudopterygium with severe
corneal thinning underneath
Tr eatm en t: steroids, im m unosuppressive agents Risk of perforation with surgery or traum a
(m ethotrexate, cyclosporine), NSAIDs, bandage contact lens,
conjunctival recession or resection; m ay require corneal glue or Mic ro b ia l Ke ra t it is (In fe c t io u s Ulc e r)
penetrating or lam ellar keratoplasty for perforation
Ba c t e ria l
Most com m on
Te rrie n ’s m a rg in a l d e g e n e ra t io n
Painless, progressive, bilateral, trough-like strom al thinning; Pen etr atio n o f in tact ep ith eliu m : Neisseria,
starts superiorly Corynebacterium diphtheriae, Shigella, Haemophilus aegyptus,
Young to m iddle-aged m en (75%), unknown etiology Listeria monocytogenes

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CHAPTER 7 • Cornea /External Disease

Th r o u gh ep ith elial d efect: any organism ; m ost Often asym ptom atic prim ary infection before age 5 years, 3- to
com m only Staphylococcus, Streptococcus, and Pseudomonas 5-day incubation period
Generally unilateral but can be bilateral (i.e.,
Risk facto r s: corneal traum a or surgery, contact lens wear,
im m unocom prom ised host)
epithelial ulceration, dry eye, lid abnorm alities
Seropositivity to HSV is 25% by age 4 years and 100% by age
Sign s o f im p r o vem en t: decrease in infiltrate (density/ 60 years
size), strom al edem a, endothelial plaque, AC reaction,
hypopyon; and reepithelialization Co n gen ital: (see Chapter 5, Pediatrics/Strabism us)

Tr eatm en t: Vesicu lar b lep h ar itis: prim ary or secondary HSV;

Empiric: topical fortified antibiotics (vancom ycin or lym phadenopathy does not occur in recurrences; perilim bal
cefazolin, plus tobram ycin alternating q1h) or involvem ent is atypical, dendrite uncom m on
fluoroquinolone (Besivance, Zym axid or Vigam ox
q15m in for 2–3 hours, then q1h); consider Acu te u n ilater al fo llicu lar co n ju n ctivitis: prim ary or
subconjunctival antibiotic injection (if noncom pliant) recurrent HSV; m ay m im ic and often m isdiagnosed as EKC;
If no response to em piric therapy: antibiotic resistance usually punctate epithelial keratitis near lim bus, preauricular
(change regim en based on culture results); fungal, lym phadenopathy; m ay develop dendrite and
protozoal, or viral process; poor com pliance (adm it to pseudom em brane, m ay have vesicular skin eruption; bulbar
hospital); anesthetic abuse conjunctival ulceration is rare but specific
Topical cycloplegic
Pr im ar y HSV ep ith elial ker atitis: one or m ultiple
Topical steroid (should be avoided until im provem ent is
sm all dendrites, strom al infiltrates; no conjunctivitis; heals in
noted [usually after 48–72 hours], then dosed at lower
1–2 weeks with corneal scarring; lid lesions occur (uncom m on
frequency than topical antibiotic)
in recurrences except in children) but without scarring; m ay
Steroids for Corneal Ulcers Trial (SCUT): evaluated topical
have lid m argin ulceration (ulcerative blepharitis); associated
m oxifloxacin m onotherapy or with adjunctive 1%
with decreased corneal sensation and patchy iris atrophy near
prednisolone phosphate at initial dose of qid after 48
pupillary m argin
hours of topical antibiotic; no difference in BCVA at 3
Cultures: positive in 75%
m onths, but steroid was safe, no difference in rate of
Pathology: intranuclear viral inclusions and m ultinucleated
healing, rate of corneal perforation, or rate of worsening
giant cells with Giem sa stain
of keratitis. At 12 m onths, patients with non-Nocardia
Prognosis: risk of recurrence is 30% within 2 years; can be
species had slightly better visual acuity in steroid group.
induced by m any stim uli (stress, sun exposure,
horm onal changes, fever, corneal traum a or surgery)
Co m p licatio n s:
Spread to adjacent structures: sclera (Pseudomonas), Recu r r en t HSV: due to reactivation of latent virus in
intraocular (rare in absence of corneal perforation; trigem inal (Gasserian) ganglion; 4 presentations:
filam entous fungi m ay penetrate intact Descem et’s Epithelial keratitis: infectious dendritic ulcer caused by live
m em brane) virus in basal epithelium (Table 7-1)
Corneal damage: scarring, neovascularization, endothelial 4 lesions:
dysfunction (corneal edema), descemetocele, perforation VESICLES: clear cystic lesions that coalesce to form
Synechiae and secondary glaucoma dendrite
Cataract DENDRITIC ULCER: branching linear lesion with central
trough (stains with fluorescein), swollen heaped-up
S yp h ilis borders containing active virus (stain with rose bengal
(See Chapter 5, Pediatrics/Strabism us) or lissam ine green), and term inal bulbs
(accum ulations of vesicular cells); m ay have strom al
edem a or infiltrate, iritis (few KP).
Crys t a llin e ke ra t o p a t h y
GEOGRAPHIC ULCER: enlarged, nonlinear dendritic
Most com m only caused by Streptococcus viridans, also Candida, ulcer with scalloped borders containing live virus
Staphylococcus epiderm idis, Streptococcus pneumoniae, MARGINAL ULCER: strom al infiltrate under a lim bal
Haemophilus, and Enterococcus dendrite with adjacent lim bal injection (no clear zone
as seen in staph m arginal keratitis); m ay have
Associated with chronic topical steroid use (post corneal graft)
conjunctival dendrite(s)
Branching, cracked-glass appearance without epithelial defect TREATMENT: topical antiviral (trifluridine [Viroptic] 9 Â/
day, ganciclovir [Zirgan 5 Â .day, or vidarabine [Vira
Treat strep with vancom ycin
A] or idoxuridine 5 Â/day; use until ulcer heals (1–
2 weeks), then taper for 1 week; beware of toxicity
He rp e s s im p le x (HS V)
from overuse [> 2–3 weeks]), cycloplegia, consider
Most com m on cause of infectious blindness and second m ost debridem ent and oral antivirals (acyclovir,
com m on cause of corneal blindness in United States (traum a is fam ciclovir, or valacyclovir)
first); HSV-1 is m ore com m on for ocular infections than HSV-2 Dendrite usually resolves in 1 week; if still present after
(genital) 10 days, possible resistance, so switch m edication. As

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 Corneal Disorders

TREATMENT: steroid and antiviral; consider

Ta b le 7-1. Comparison of herpetic epithelial keratitis
cyclosporine or am niotic m em brane transplant
Le s io n HSV d e nd rite HZV p s e ud o d e nd rite Endotheliitis: inflam m atory reaction to live virus in the
Appearance Delicate, fine, lacy Coarse, ropy, elevated, endothelium with varying degrees of isolated strom al
ulcer ”painted-on” lesion edem a and stellate KP; can be disciform (round), linear,
Smaller, less branching than HSV or diffuse (lim bus to lim bus); m ay develop hypopyon,
dendrite rubeosis, spontaneous hyphem a, elevated IOP
Terminal bulbs Blunt ends (no terminal bulbs) TREATMENT: topical steroid plus oral antiviral; consider
Epithelial cells Epithelial cells are swollen and oral steroid
slough heaped-up Neurotrophic keratopathy (m etaherpetic ulcer): chronic
Staining Base with Poor with fluorescein and rose sterile m acroulceration due to im paired corneal
fluorescein bengal sensation and decreased tear production
Edges with rose Oval-shaped with sm ooth edges, no staining with rose
bengal bengal at edge; no strom al inflam m ation; m ay have
Treatment Do not use steroids Good response to steroids thinning and scarring
Note: Active viral replication in epithelia l lesions occurs in both HSV and HZV TREATMENT: lubrication; m ay take m onths to heal owing
Other causes of pseudodendrite: Acanthamoeba, tyrosinemia II, epithelial to damaged basem ent m embrane, impaired corneal
healing ridge
innervation, and abnorm al tear film ; m ay require
tarsorrhaphy, bandage contact lens, autologous serum
the lesion heals, a dendritic shape epitheliopathy drops, am niotic m embrane, conjunctival flap
persists for several weeks (which should not be treated
with antiviral m edication)
Diagn o sis:
Papanicolaou smear: intranuclear inclusion bodies
Antiviral toxicity (idoxuridine > vidarabine >
(Lipsch u€ tz bodies), Cowdry type A (eosinophilic)
trifluridine > acyclovir / ganciclovir): punctate
intranuclear inclusions
epithelial keratopathy, follicular conjunctivitis,
Tzanck’s prep: m ultinucleated giant cells with Giem sa stain
indolent corneal ulceration, preauricular
Culture: only 60% positive if swab active dendrite
lym phadenopathy with idoxuridine, punctal stenosis
Immunofluorescence: detects HSV antigen
Steroids reduce scarring; taper very slowly, critical dose
variable (m ay need steroids for years); potentiate but
Tr eatm en t: depends on lesion (see earlier); preferred topical
do not activate live virus
antiviral is now ganciclovir (Zirgan); corneal tranplant (PK) for
Without treatm ent, 25% of epithelial keratitis resolves in
visually significant scarring
1 week and 50% resolves in 2 weeks but increased risk
Herpetic Eye Disease Study (HEDS):
of strom al involvem ent with scarring,
STROMAL DISEASE: treatm ent with topical steroids and
neovascularization, and decreased vision
trifluridine (Viroptic) is better than Viroptic alone
Stromal keratitis (im m une or interstitial keratitis):
(prednisolone acetate 8 Â/day and viroptic qid for
im m unologic reaction due to recurrent infection in
1 week, then 10-week taper; supports im m une
20–30% of dendritic ulcer patients within 5 years.
m ediated m echanism for strom al disease); no benefit
Hallm arks are strom al infiltrate, neovascularization, and
to concom itant oral acyclovir
scarring
IRITIS: acyclovir (400 mg 5 Â/day), in addition to topical
NON-NECROTIZING ( DISCIFORM) KERATITIS:
Viroptic and steroid, is better than topicals alone
inflam m atory reaction within endothelium with
PROPHYLAXIS: acyclovir (400 m g bid for 1 year)
secondary focal num m ular strom al edem a due to
decreases by 50% risk of recurrent HSV keratitis
delayed hypersensitivity reaction
Localized fine keratic precipitates (KP), no necrosis;
Co m p licatio n s: uveitis, glaucom a, episcleritis, scleritis,
m ay have iritis, increased IOP (trabeculitis)
secondary bacterial keratitis, corneal scarring and
If severe: diffuse strom al edem a, Descem et’s folds;
neovascularization, corneal perforation, iris atrophy,
m ay have hypopyon, rarely corneal vascularization
punctal stenosis
Culture negative
PATHOLOGY: granulom atous reaction, som etim es
with retrocorneal m em brane
TREATMENT: topical steroid (cover with antiviral to MAJ OR CLINICAL STUDY
prevent epithelial recurrence and antibiotic
ointm ent to prevent secondary bacterial infection); He rp e tic Eye Dis e a s e Stud y (HEDS)
consider topical cyclosporine
NECROTIZING KERATITIS: rare; antigen-antibody
Ob je c t ive : Five trials to evaluate the role of steroids and
com plem ent m ediated reaction to rapid viral
antiviral m edication in the treatm ent and prevention of
replication in strom a
ocular HSV disease:
Ulceration with infiltrate and thinning, strom al
inflam m ation, epithelial defect, AC reaction; 1. Efficacy of oral acyclovir (ACV) in treating strom al
m ay perforate, m ay be difficult to distinguish keratitis (non-necrotizing [disciform ] and necrotizing):
from bacterial or fungal keratitis

217
CHAPTER 7 • Cornea /External Disease

10-week course of ACV 400 m g 5 Â/day or placebo, in Co n c lu s io n s :

addition to topical steroids and trifluridine
2. Efficacy of topical corticosteroids in treating strom al 1. Oral ACV is not useful for the treatm ent of strom al
keratitis: 10-week tapering course of prednisolone keratitis
phosphate or placebo in addition to topical trifluridine 2. Topical corticosteroids are beneficial for the treatm ent
(prednisolone acetate 8 Â/day and trifluridine of strom al keratitis
[Viroptic] qid  1 week, then gradual taper over 3. Results were not statistically significant but suggest a
10 weeks) possible benefit of oral ACV for the treatm ent of
3. Efficacy of oral ACV in treating iridocyclitis: 10-week iridocyclitis
course of ACV 400 m g 5 Â/day or placebo, in addition 4. In patients with epithelial keratitis, a 3-week course of
to topical steroids and trifluridine oral ACVhas no apparent benefit in preventing strom al
4. Efficacy of oral ACV in preventing strom al keratitis or disease or iridocyclitis
iridocyclitis in patients with epithelial keratitis: 3-week 5. Oral ACV prophylaxis significantly reduces the risk of
course of ACV 400 m g 5 Â/day or placebo, in addition recurrent ocular and orofacial HSVdisease, especially in
to topical trifluridine patients with previous strom al keratitis
5. Efficacy of oral ACV in preventing recurrent ocular 6. In patients with ocular HSVdisease in the previous year,
HSV disease: 12-m onth course of ACV 400 m g bid a history of epithelial keratitis is not a risk factor for
or placebo and 6-m onth observation period for recurrent epithelial keratitis, but a history of strom al
patients with a history of ocular HSV within the keratitis increases the risk of subsequent strom al
preceding year keratitis, and this risk is strongly associated with the
6. Determ inants of recurrent HSV keratitis: an alyzed num ber of previous episode
the placebo group from th e acyclovir prevention
trial
He rp e s zo s t e r o p h t h a lm ic u s (HZO)
Re s u lt s : Herpes zoster involvem ent of first branch of trigem inal nerve
1. Oral ACV, in addition to topical steroids and CN 5 (V1 )
trifluridine (Viroptic), for the treatm ent of strom al Acute, painful, unilateral derm atom al vesicular eruption
keratitis did not im prove the num ber of treatm ent (obeys midline) with prodrom e; new lesions occur for
failures, tim e to resolution of keratitis, or 6-m onth best- $ 1 week with resolution in 2–6 weeks
corrected visual acuity
2. Topical corticosteroids reduced the risk of persistent or May occur without rash (zoster sine herpete)
progressive strom al keratitis by 68% and shortened the After chickenpox, 20–30% risk of developing herpes zoster
duration of keratitis (shingles); increased incidence and severity with age > 60 years,
3. Trial was stopped due to slow recruitm ent. Treatm ent up to 50% at age 85 years; 10–20% have HZO and 50% of these
failure occurred in 50% of the ACVgroup vs 68% of the have ocular involvem ent if untreated
placebo group
4. Oral ACV did not reduce the risk of strom al keratitis Most com m on single derm atom e is CN 5: ophthalm ic (V1 ) >
or iridocyclitis developm ent in patients with m axillary (V2 ) > m andibular (V3 )
epithelial keratitis. Strom al keratitis or iridocyclitis 3 branches of ophthalm ic division: frontal nerve > nasociliary
developed in 11% of patients in the ACV group vs nerve > lacrim al nerve
10% in the placebo group, and such an occurrence Hutchinson’s sign: skin lesion on tip or side of nose
was m ore com m on in those with a previous history (nasociliary nerve) is a strong indication of ocular
of strom al keratitis or iritis (23% vs 9% without involvem ent
previous history)
5. Oral ACV reduced the risk of recurrent ocular disease
during the treatm ent period (19% vs 32%), especially Sym p to m s: 2- to 3-day prodrom e (fever, m alaise, headache,
in the strom al keratitis subset (14% vs 28%). paresthesia)
Recurrence of nonocular HSV disease was also lower in
the treated group (19% vs 36%). No rebound in the rate Fin d in gs:
of disease was seen during the 6-m onth observation Lid: vesicles, cicatricial changes (ectropion, entropion,
period after treatm ent m adarosis, ptosis), trichiasis, lagophthalm os, blepharitis
6. In the placebo group of the previous trial, 18% Conjunctiva: follicular conjunctivitis, vesicles,
developed epithelial keratitis and 18% developed pseudom em branes, sym blepharon
strom al keratitis. Previous epithelial keratitis did not Cornea: keratitis (65%) (Table 7-1)
significantly affect the subsequent risk of epithelial PUNCTATE EPITHELIAL: precursor to pseudodendrite;
keratitis; previous strom al keratitis significantly viral replication with epithelial destruction
increased the subsequent risk of strom al keratitis PSEUDODENDRITIC: blunt ends, no term inal bulbs, no
(10 Â) ulceration, m inim al staining; self-lim ited (appears
within a few days of rash, resolves in 4–6 days)

218
 Corneal Disorders

INFLAMMATORY ULCERATION: im m une response; often Oral steroid: prednisone 60 m g qd  1 week, then 30 m g
peripheral; m ay lead to perforation qd  1 week, then 15 m g qd  1 week
DENDRITIFORM EPITHELIAL PLAQUES (delayed m ucous Reduces tim e course, duration and severity of acute pain,
plaques): can occur at any tim e even weeks later; and incidence of postherpetic neuralgia
linear or branching, sharply dem arcated gray-white For intraocular and ocular surface involvement: topical
lesion on epithelial surface; m ild fluorescein staining, steroid (m ay require indefinitely to prevent recurrence),
vivid rose bengal staining; interference with norm al cycloplegic, and antibiotic; m ay require IOP control and
m ucous–epithelial interaction, no active virus treatm ent of ocular com plications
replication For postherpetic neuralgia: capsaicin cream (Zostrix;
STROMAL: occurs in 50% with epithelial disease within first depletes substance P), cim etidine, tricyclic antidepressants
1–3 weeks; nummular subepithelial infiltrates and (amitriptyline, desipramine), carbamazepine (Tegretol),
disciform keratitis beneath initial keratitis; immune gabapentin (Neurontin), pregabalin (Lyrica), lidocaine
response (may develop ring) (Lidoderm patch), opioids, benadryl; consider nerve
ENDOTHELIITIS: occurs in 10% with epithelial disease; block, botox map injections
bullous keratopathy (corneal edem a out of
proportion to strom al infiltrate), few KP, iritis
NEUROTROPHIC KERATOPATHY: occurs in 20%; due to Co m p licatio n s: occur in 50%; m ost com m on is
decreased corneal sensation; leads to ulceration postherpetic neuralgia
Also, strom al scarring, pannus, exposure keratopathy Postherpetic neuralgia (PHN): neuropathic pain syndrom e
(due to lid abnorm alities) that persists or occurs after resolution of rash; risk factors
Episcleritis/ scleritis: lim bal vasculitis, sclerokeratitis, scleral include age > 60 years, severity of prodrom al and acute
atrophy, posterior scleritis pain, severity of rash, and HZO. Occurs in 10% of all
Iris: segm ental atrophy due to vasculitis with ischem ia and zoster patients, 10% to 20% with HZO; 50% resolve
necrosis; Argyll-Robertson pupil (ciliary ganglion within 1 m onth, 80% within 1 year
involvem ent) Meningoencephalitis and myelitis: rare; occurs 7–10 days
Uveitis: can occur m onths later; m ay have elevated IOP, after rash with fever, headache, cranial nerve palsies,
45% develop glaucom a; m ay develop cataract hallucinations, altered sensorium ; m ost com m on in
Retina: central retinal artery occlusion, acute retinal patients with cranial zoster, dissem ination, or
necrosis (ARN); progressive outer retinal necrosis im m unocom prom ised (m ay be fatal in AIDS)
(PORN) in patients with AIDS Granulomatous arteritis: contralateral h em iplegia weeks
Optic nerve: ischem ic optic neuropathy to m onths after acute disease; involves m iddle
CN palsies: CN 3, 4, or 6 palsyoccurs in 25%;self-limited;may cerebral artery; 65% are > 60 years old; 25%
involve pupil; may have orbital apex syndrome (optic m ortality
neuropathy, ophthalmoplegia, and anesthesia) from
vasculitis Ch ic ke n p o x
Syndromes:
Fin d in gs: papillary conjunctivitis, ”pock” lesions on bulbar
RAMSAY HUNT SYNDROME: CN 5 and 7 involvem ent
conjunctiva and at lim bus, keratitis (dendritic, disciform ,
with facial paralysis
interstitial)
ZOSTER SINE HERPETAE: zoster-type derm atom al pain
without rash
Fu n g i

Diagn o sis: characteristic skin lesions, Tzanck’s prep, Typ es:

serology (IgG antibodies), culture, ELISA Molds (filam entous): form hyphae
SEPTATE: m ost com m on cause of fungal keratitis;
m ost com m on in south ern / south western United
Pr even tio n : vaccination in patients age 50 years or older States – Fusarium (F. solani is m ost virulent),
(was previously 60 years or older; safety and efficacy Aspergillus, Cuvularia, Paecilomyces, Penicillium,
dem onstrated in Shingles Prevention Study); reduces incidence Phialophora
(51%), severity and duration of zoster; reduces incidence of NONSEPTATE: Mucor (rare cause of keratitis)
pain (61%) and postherpetic neuralgia (67%) Yeast: Candida (most com m on cause of m ycotic ocular
infections in northern United States), Cryptococcus
Tr eatm en t: (associated with endogenous endophthalm itis; rarely
Oral antiviral within 72 hours of rash: acyclovir (800 m g keratitis)
5 Â /day), fam ciclovir (Fam vir, 500 m g tid), or Dimorphic fungi: grow as yeast or m old – Histoplasma,
valacyclovir (Valtrex, 1000 m g tid) Â 7 days; IV Blastomyces, Coccidioides
acyclovir  10-14 days if im m unocom prom ised
Reduces tim e course (form ation of new lesions, healing Risk facto r s:
of lesions, period of viral shedding), and risk and Molds: corneal injury especially from tree branch or
severity of ocular involvem ent. Fam vir and Valtrex vegetable m aterial, soft contact lens wear
reduce risk, duration, and severity of postherpetic Yeast: therapeutic soft contact lens wear, topical steroid use,
neuralgia nonhealing epithelial defect, decreased host resistance

219
CHAPTER 7 • Cornea /External Disease

Fin d in gs: satellite infiltrates, feathery edges, endothelial Sym p to m s: blurred vision, photophobia, foreign body
plaque; can penetrate Descem et’s m em brane sensation

Tr eatm en t: topical antifungal (natam ycin 5% Fin d in gs: diffuse punctate epithelial erosions and
intraepithelial opacities; m ay have sinusitis
[filam entous], clotrim azole [Aspergillus], am photericin B
0.15% [yeast], flucytosine 1%, ketoconazole 2%, m iconazole
1%, itraconazole 1%, fluconazole 0.5%); cycloplegic Diagn o sis: epithelial cells with cytoplasm ic inclusions on
Giem sa stain
Corneal epithelium is significant barrier to natam ycin
penetration Tr eatm en t: fum agillin (10 m g/m L), itraconazole,
albendazole
On c h o c e rc ia s is
Ac a n t h a m o e b a
(See Chapter 8, Uveitis)
22 species; exists as trophozoite or cyst
Le p ro s y
90% initially m isdiagnosed as HSV
(See Chapter 8, Uveitis)

Risk facto r s: cleaning soft contact lenses with hom em ade Ep s t e in -Ba r r viru s (EBV)
saline solution; swim m ing or hot tubbing while wearing
Fin d in gs: m ultifocal strom al keratitis; m ay rarely have
contact lenses
episcleritis, follicular conjunctivitis, uveitis, optic neuritis

Pr even tio n : heat disinfectant (75 °C) Diagn o sis:

Antibodies: viral capsid antigen IgM and IgG and early
antigen-diffuse (EA-D) peak at 6–12 weeks; viral capsid
Fin d in gs: epithelial cysts (can look like EKC with antigen (VCA)-IgM and Epstein-Barr nuclear antigen
subepithelial infiltrates and punctate keratopathy), perineural (EBNA) are detectable for life; heterophile antibodies
infiltrate (enlarged corneal nerves, decreased corneal sensation, (IgM that reacts to horse and sheep blood cells) peak at
pain [perineuritis]), ring-shaped infiltrate, corneal edem a, 2–3 weeks and last 1 year (Monospot test)
pseudodendrite, iritis, hypopyon (30%–40%), scleritis; m ay Lymphocytosis: occurs in 70%; peaks at 2–3 weeks
perforate Liver enzymes: elevated

Diagn o sis: calcofluor white, Giem sa stain, culture (non- Ec ta s ia s

nutrient agar with E. coli overlay; can grow on blood or
chocolate agar but not as well), confocal m icroscopy Ke ra t o c o n u s
90% bilateral; onset typically around puberty
Path o lo gy: oval am oebic double-walled cysts in strom a
Asso ciatio n s:
Systemic conditions: atopy (eye rubbing), Down syndrom e,
Tr eatm en t: d ebridem ent if infection lim ited to epithelium connective tissue disease (Ehlers-Danlos syndrom e,
(m ay be curative), com bination of topical agents Marfan’s syndrom e, osteogenesis
Antibacterial: neom ycin or parom om ycin (Hum atin) 1% im perfecta)
q2 hours initially Ocular conditions: aniridia, retinitis pigm entosa, VKC,
Antifungal: m iconazole 1% or clotrim azole 1% q1 hour Leber’s congenital am aurosis, ectopia lentis, floppy
initially, and oral voriconazole, ketoconazole or eyelid syndrom e, CHED, posterior polym orphous
itraconazole dystrophy (PPCD)
Antiparasitic: polyhexam ethylene biguanide (PHMB,
Baquacil; swim m ing pool cleaner) 0.02% or Fin d in gs: inferior corneal protrusion, strom al thinning at
chlorhexidine 0.02%, and propam idine isethionate cone apex (apical thinning), apical scarring, Fleischer ring
(Brolene) 0.1% or hexam idine (Desom edine) 0.1% q1 (epithelial iron deposition at base of cone), Vogt’s striae (fine
hour initially deep striae anterior to Descem et’s m em brane; disappear with
Steroid: controversial external pressure), prom inent corneal nerves, scissors reflex on
30% recurrence after penetrating keratoplasty retinoscopy, Munson’s sign (angulation of lower lid on
downgaze due to corneal protrusion), Rizzutti’s sign (conical
reflection of light from tem poral light source on nasal iris);
Mic ro s p o rid ia m ay develop hydrops
Hydrops: acute corneal edem a and clouding due to break in
Obligate intracellular spore-form ing parasite (Encephalitozoon
Descem et’s m em brane; painful or painless; decreased
hellem, E. cuniculi)
IOP; heals over 6–10 weeks; scarring can occur
Keratitis in HIV-positive patients occasionally with strom al neovascularization

220
 Corneal Disorders

Path o lo gy: breaks in Bowm an’s layer, strom al be AD and include granular, lattice type 1 and variants, Reis-
folds, superficial scarring, strom al thinning; Descem et’s is Bucklers, and Thiel-Behnke
norm al unless hydrops has occurred (breaks with curled
Onset by age 20 years, except epithelial basem ent m em brane
edges covered by adjacent endothelial cells and new
dystrophy (ages 30–40), pre-Descem et (ages 30–40), and
Descem et’s)
Fuchs (ages 40–50)

DDx: contact lens-induced corneal warpage, pellucid Not associated with system ic diseases, except Schnyder
m arginal degeneration, keratoglobus (associated with hyperlipoproteinem ia), and lattice type 2 (not
considered a corneal dystrophy; Meretoja syndrom e associated
Diagn o sis: corneal topography (irregular astigm atism , with system ic am yloidosis)
central or inferior steepening), keratom etry (steep readings, Affect central cornea only, except Meesm ann’s, m acular, fleck,
irregular m ires) CSCD, and CHED, which extend to lim bus

Tr eatm en t: contact lenses (rigid, piggyback, scleral) for visual The classification of corneal dystrophies has traditionally been
improvement; rigid contact lenses do not stop progression of anatom ic according to the corneal layer m ost affected
disease. Corneal collagen crosslinking (to stop progression), (anterior, strom al, and endothelial). This was changed in 2008
Intacs; penetrating keratoplasty for advanced cases; supportive for by the International Com m ittee for Classification of Corneal
hydrops (topical steroids, cycloplegic, bandage contact lens) Dystrophies (IC3D), which divided the m ajor corneal
dystrophies into 4 categories: Epithelial and Subepithelial
P o s t e rio r ke ra t o c o n u s dystrophies, Bowm an Layer dystrophies, Strom al dystrophies,
and Descem et Mem brane and Endothelial dystrophies. In
(See Chapter 5, Pediatrics/Strabism us)
2015, the IC3D revised the classification to reflect the
Ke ra t o g lo b u s involvem ent of cellular layers (excluding Bowm an’s layer and
Descem et's m em brane): Epithelial and Subepithelial
Rare, sporadic, globular corneal deform ity dystrophies, Epithelial-strom al (TGFB1) dystrophies, Strom al
Associated with connective tissue disorders, Leber’s congenital dystrophies, and Endothelial dystrophies.
am aurosis

Fin d in gs: bilateral corneal thinning, m axim al in Ep it h e lia l a n d S u b e p it h e lia l Dys t ro p h ie s

m idperiphery at base of protrusion
Ep it h e lia l b a s e m e n t m e m b ra n e d ys t ro p h y
Tr eatm en t: CL (rigid), penetrating keratoplasty (EBMD; Co g a n ’s m ic ro c ys t ic e p it h e lia l
d ys t ro p h y; m a p -d o t -fin g e rp rin t d ys t ro p h y;
P e llu c id m a rg in a l d e g e n e ra t io n a n t e rio r b a s e m e n t m e m b ra n e d ys t ro p h y)
(m a jo rit y d e g e n e r a t ive ; AD in is o la t e d fa m ilia l
Onset age 20–40 years old; no sex predilection; m ore com m on case s)
in Europeans and Japanese Most com m on anterior corneal dystrophy

Fin d in gs: bilateral, inferior corneal thinning with protrusion Usually bilateral, can be asym m etric; prim arily affects m iddle-
above thinnest area; no vascularization; acute hydrops rare aged wom en
Sym ptom s m ore com m on in those > 30 years old
Path o lo gy: resem bles keratoconus
Sym p to m s: usually asym ptom atic; m ay have pain, redness,
tearing, irritation (recurrent epithelial erosions), or decreased
Diagn o sis: corneal topography (irregular, against-the-rule
vision (subepithelial scarring)
astigm atism with inferior annular/claw/lazy-c pattern of
steepening) Fin d in gs: irregular and often loose epithelium with
characteristic appearance (m ap, dot, fingerprint):
Tr eatm en t: contact lenses for visual im provem ent; corneal Map lines: subepithelial connective tissue
collagen crosslinking to stop progression. If severe, a large Microcysts: white, putty-like dots (degenerated epithelial
penetrating keratoplasty m ay be required cells trapped in abnorm al epithelium )
Fingerprints: parallel lines of basem ent m em brane
separating tongues of reduplicated epithelium
Dys tro p hie s May develop recurrent erosions (10% with the dystrophy get
erosions, 50% with erosions have the dystrophy) or decreased
Inherited genetic disorders (usually defective enzym e or vision or m onocular diplopia from subepithelial scarring
structural protein) (irregular astigm atism )
AD except m acular, gelatinous drop-like, and CHED, which Path o lo gy: epithelial reduplication with excess
are AR subepithelial and intraepithelial production of basem ent
Many dystrophies have a m utation of the TGFB1 (also known m em brane m aterial and collagen (due to poor epithelial
as BIGH3) gene on chrom osom e 5. These dystrophies tend to adhesion to basem ent m em brane) (Fig. 7-11)

221
CHAPTER 7 • Cornea /External Disease

Me e s m a n n c o rn e a l d ys t ro p h y (MECD) (AD)
Mapped to chrom osom es 12q13 (KRT3) and 17q12 (KRT12)
(Stocker-Holt variant)
Appears early in life
Minim al sym ptom s; m ay have m ildly reduced vision,
photophobia
Stocker-Holt variant m ore severe

Fin d in gs: tiny epithelial vesicles, m ost numerous

interpalpebrally, extend to lim bus, m ay have epithelial erosions

Fig ure 7-11. ABMD demonstrating cysts and aberrant production of basement Path o lo gy: epithelial cells contain PAS-positive m aterial
membrane material in the epithelium. (From Yanoff M, Fine BS: Ocular (peculiar substance); thickened epithelial basem ent
Pathology, ed 5, St Louis, 2002, Mosby.) m em brane (Fig. 7-12)
No treatm ent
Tr eatm en t: none unless sym ptom atic. For erosions, treat
with lubrication, hypertonic saline (Muro 128 5% ointm ent
qhs  3–12 m onths); if recurs, consider bandage contact lens, Lis c h e p it h e lia l c o rn e a l d ys t r o p h y (LECD;
epithelial d ebridem ent, anterior strom al puncture / Ba n d -s h a p e d o r w h o rle d m ic ro c ys t ic d ys t ro p h y
reinforcem ent, diam ond burr (d ebride and polish Bowm an’s o f t h e c o rn e a l e p it h e liu m ) (X-lin ke d d o m in a n t )
layer), laser (PTK with excim er laser, or m icropuncture with
Nd :YAG laser (1 m J, aim just below epithelium )); also, Mapped to chrom osom e Xp22.3
consider treatment with m atrix m etalloproteinase-9 inhibitors Onset in childhood, slowly progressive
(doxycycline 50 m g PO bid  2 m onths and topical steroids
tid  2–3weeks); d ebridem ent for subepithelial scarring Sym p to m s: asym ptom atic or blurred vision

Ep it h e lia l re c u rre n t e ro s io n d ys t ro p h ie s Fin d in gs: various patterns of gray opacities (whorl, radial,
(EREDs ) (AD) band, flam e, feather, or club-shaped)
Onset early childhood
Path o lo gy: vacuolated cells containing glycogen

3 var ian ts: Franceschetti Corneal Dystrophy (FRCD), Ge la t in o u s d ro p -like c o rn e a l

Dystrophia Smolandiensis (DS), Dystrophia d ys t r o p h y (GDLD) (AR)
Helsinglandica (DH)
Mapped to chrom osom e 1p32 (TACSTD2; form erly M1S1)
Sym p to m s: decreased vision, photophobia, tearing Rare; onset in 1st to 2nd decade of life, m ore com m on
in Japanese; not associated with system ic am yloidosis
Fin d in gs: recurrent epithelial erosions lasting 1 to 7 days; less
frequent and severe by age 30 to 40 years, but develop central, Sym p to m s: decreased vision, pain, photophobia, tearing
subepithelial opacity, fibrosis and keloid-like elevated scar
Path o lo gy: destruction and absence of Bowm an's layer with
connective tissue pannus (FRCD); keloid-like deposit of
am yloid (stains with Congo red) (DS)

S u b e p it h e lia l m u c in o u s c o rn e a l d ys t ro p h y
(S MC D) (AD)
Onset in first decade of life

Sym p to m s: pain, photophobia, tearing from epithelial

erosions; decreased vision in adolescence

Fin d in gs: recurrent corneal erosions, diffuse subepithelial

opacities and haze

Path o lo gy: subepithelial deposits of fin e fibrillar Fig ure 7-12. Meesmann’s dystrophy with Periodic Acid-Schiff stain. (From
substance Yanoff M, Fine BS: Ocular Pathology, ed 5, St Louis, 2002, Mosby.)

222
 Corneal Disorders

Fin d in gs: progressive central, m ulberry-like, subepithelial Classic fo r m o f lattice d ystr o p h y: onset in 1st decade
deposits and strom al opacity; recurs after treatm ent of life; starts centrally, spares periphery, no system ic am yloid
(keratectom y and keratoplasty)
4 var ian ts ( p r esen t later in life) : type IIIA (5th-7th
Path o lo gy: absence of Bowm an’s layer; am yloid decade of life, epithelial erosions, thicker lattice lines extending to
(subepithelial and strom al)
limbus), type I/IIIA (thinner lattice lines), type IV (7th-9th
decade of life, smaller lattice lines in deep stroma, no epithelial
Ep it h e lia l-S t ro m a l (TGFB1 ) Dys t ro p h ie s erosions), and polymorphic amyloidosis (no lattice lines)

Mer eto ja syn d r o m e ( Fin n ish fam ilial am ylo id o sis;

Re is -Bu c k le rs c o rn e a l d ys t ro p h y (Co rn e a l
fo r m er ly Lattice co r n eal d ystr o p h y typ e II ( LCD2;
d ys t r o p h y o f Bo w m a n ’s la ye r t yp e I (CDB1 )) (AD)
Gelso lin typ e) ) ( AD) : not a true corneal dystrophy.
Mapped to chrom osom e 5q31 (TGFB1) Mapped to chrom osom e 9q34 (GSN); Finnish descent;
system ic am yloidosis syndrom e (facial m ask; dry, lax skin;
Onset in childhood
blepharochalasis, derm atochalasis; pendulous ears; cranial
Sym p to m s: pain (recurrent epithelial erosions), decreased and peripheral nerve palsies); corneal lattice lines m ore
vision peripheral and m oves centrally

Fin d in gs: irregular, diffuse, geographic gray-white opacities Sym p to m s: pain (recurrent epithelial erosions), decreased
at Bowm an‘s layer and strom a, begin centrally with clear vision by 4th decade of life
interruptions, then m ore confluent extending to lim bus and
deeper strom a; epithelial erosions, corneal scarring Fin d in gs: branching refractile lines in anterior strom a (best
seen in retroillum ination), central subepithelial white dots,
Path o lo gy: absence of Bowm an’s layer and replacem ent by subepithelial diffuse haze with ground-glass appearance,
granular highly reflective irregular deposits extending into peripheral cornea typically clear (depends on type); eventually
strom a; stain with Masson’s trichrom e epithelial erosions with scarring

Tr eatm en t: penetrating keratoplasty (recurrence is Path o lo gy: amyloid deposits, stain with Congo red, thioflavin
T, metachromatic with crystal violet, apple-green birefringence
com m on)
and dichroism with polarization microscopy (Fig. 7.13)

Th ie l-Be h n k e c o rn e a l d ys t ro p h y (TBCD; Co rn e a l Tr eatm en t: penetrating keratoplasty (recurrence m ore likely

d ys t r o p h y o f Bo w m a n ’s la ye r t yp e II (CDB2 ); than in m acular or granular, but less likely than in Reis-
h o n e yc o m b -s h a p e d c o rn e a l d ys t ro p h y; c u rly Bucklers)
fib e rs c o rn e a l d ys t ro p h y; Wa a rd e n b u rg -J o n ke rs
c o rn e a l d ys t ro p h y) (AD)
Mapped to chrom osom es 5q31 (TGFB1)
Onset in early childhood
Less visual loss and does not recur in graft as early and
extensively (com pared to Reis-Bucklers (CDB type I))

Sym p to m s: pain (recurrent epithelial erosions), gradual

decreased vision

Fin d in gs: flecks and irregular scattered opacities at

Bowm an’s layer progress to reticular or honeycom b pattern;
epithelial erosions, corneal scarring

Path o lo gy: Bowm an’s layer is replaced by fibrocellular

m aterial in a pathognom onic wavy “sawtooth” pattern; curly Fig ure 7-13. Deposits in lattice dystrophy. (From Yanoff M, Fine BS: Ocular
fibers on electron m icroscopy Pathology, ed 5, St Louis, 2002, Mosby.)

Gra n u la r c o rn e a l d ys t ro p h y t yp e I (GCD1 ;
La t t ic e c o rn e a l d ys t ro p h y t yp e I (LC D1 ;
Gro e n o u w c o rn e a l d ys t ro p h y t yp e I) (AD)
Bib e r-Ha a b -Dim m e r c o rn e a l d ys t ro p h y) a n d
va ria n t s (AD) Most com m on strom al dystrophy

Mapped to chrom osom e 5q31 (TGFB1) Mapped to chrom osom e 5q31 (TGFB1)

223
CHAPTER 7 • Cornea /External Disease

S t ro m a l Dys t ro p h ie s

Ma c u la r c o rn e a l d ys t ro p h y (MC D; Gro e n o u w
c o rn e a l d ys t ro p h y t yp e II) (AR)
Mapped to chrom osom e 16q22 (CHST6); error in synthesis of
keratan sulfate
3 variants (type I, type IA, type II) based on im m unoreactivity
of keratan sulfate in cornea and serum
Most severe but less common than granular or lattice dystrophies
Deposits present in 1st decade of life

Fig ure 7-14. Deposits in granular dystrophy stain red with trichrome. (From Sym p to m s: severe decreased vision in 2nd–3rd decade of
Yanoff M, Fine BS: Ocular Pathology, ed 5, St Louis, 2002, Mosby.) life

Deposits present in 1st decade of life, can rem ain

Fin d in gs: gray-white strom al fleck-like opacities at all levels
asym ptom atic for decades
extending to periphery with diffuse haze (no intervening clear
spaces), develop central corneal thinning and guttata;
Sym p to m s: glare, photophobia, decreased vision; pain epithelial erosions rare
(recurrent epithelial erosions)
Path o lo gy: m ucopolysaccharide (glycosam inoglycan)
Fin d in gs: discrete focal, white, granular deposits deposits, stain with Alcian blue and colloidal iron (Fig. 7-15)
with breadcrum b or snowflake appearance in central
anterior strom a, intervening clear areas; epithelial erosions Tr eatm en t: penetrating keratoplasty; PTK (less useful,
lesions extend though entire strom a and erosions rare)
Path o lo gy: hyaline deposits (from deep epithelium to
Descem et’s m em brane), stain with Masson trichrom e S c h n yd e r c o rn e a l d ys t ro p h y (S CD; S c h n yd e r
(Fig. 7-14)
c rys t a llin e c o rn e a l d ys t ro p h y) (AD)
Tr eatm en t: penetrating keratoplasty, PTK Mapped to chrom osom e 1p36 (UBIAD1)
Onset usually 2nd–3rd decade
Gra n u la r c o rn e a l d ys t r o p h y t yp e II (GCD2 ;
Slowly progressive, rarely reduces vision enough to require
Ave llin o d ys t ro p h y; c o m b in e d g ra n u la r-la t t ic e corneal transplantation
d ys t r o p h y) (AD)
Mapped to chrom osom e 5q31 (TGFB1)
Sym p to m s: decreased vision, glare
Later identified in fam ily from Avellino, Italy
Onset usually in teens or early adulthood, m ay occur in 1st Fin d in gs: age dependent, only 50% have corneal crystals
decade of life; slowly progressive (m ay be unilateral, rarely regress, occur late)
≤ 23 years old: ring-like central corneal opacity, com m a-
shaped subepithelial crystals
Fin d in gs: white dots with sm all spokes, discoid or ring- 23–38 years old: arcus lipoides
shaped patches, spiky deposits (star, icicle, spider-shaped), and
short lines or dashes (rarely cross); dots and rings appear early
in anterior strom a, dashes occur later in deeper strom a; strom al
haze; m ay get erosions

Path o lo gy: hyaline and am yloid deposits from basal

epithelium to deep strom a; stain with Masson trichrom e and
Congo red

Mn em o n ic: Marilyn Monroe Always Gets Her Men in LA

County
Refers to nam e of dystrophy, substance deposited in cornea,
special stain (for the 3 classic “strom al” dystrophies)
Macular, Mucopolysaccharide, Alcian blue
Granular, Hyaline, Masson trichrom e Fig ure 7-15. Deposits in macular dystrophy stain with Alcian blue. (From Yanoff
Lattice, Am yloid, Congo red M, Fine BS: Ocular Pathology, ed 5, St Louis, 2002, Mosby.)

224
 Corneal Disorders

> 38 years old: diffuse m idperipheral strom al haze P re -De s c e m e t c o rn e a l d ys t ro p h y (P DCD; De e p

May have hyperlipoproteinem ia (type IIa, III, or IV) filifo r m d ys t ro p h y) (AD)
Onset in 4th–5th decade of life; several subgroups
Path o lo gy: cholesterol and neutral fat deposits, stain with
Oil-red-O and Sudan black Isolated PDCD m ay be hereditary or degenerative
Can recur in graft
PDCD subtype (punctiform and polychrom atic) is AD
Co n g e n it a l s t r o m a l c o rn e a l d ys t ro p h y (CS CD) PDCD associated with X-linked ichthyosis is m apped to
chrom osom e Xp22.31 (STS)
(See Chapter 5, Pediatrics / Strabism us)
Usually asym ptom atic
Fle c k c o rn e a l d ys t ro p h y (FCD; Fra n c o is -Ne e t e n s
c o rn e a l d ys t ro p h y) (AD) Fin d in gs: central, annular, or diffuse fine gray deep strom al
Mapped to chrom osom e 2q34 (PIKFYVE (previously known polym orphic opacities
as PIP5K3))
Congenital, nonprogressive m aybe asym m etric or unilateral Path o lo gy: lipid deposits in posterior strom al keratocytes

Vision usually not affected

En d o t h e lia l Dys t ro p h ie s
Fin d in gs: discrete, flat, gray-white, dandruff-like speck and Fu c h s e n d o t h e lia l c o rn e a l d ys t ro p h y (FECD) (AD)
ring-shaped opacities, scattered throughout strom a extending Early onset (m apped to chrom osom e 1p34.3-32 (FECD1))
to periphery; m ay have decreased corneal sensation or late onset (m apped to chrom osom es 13pter-q12.13
Associated with lim bal derm oid, keratoconus, central cloudy (FECD2), 18q21 (FECD3), 20p13-12 (FECD4), 5q33.1-35.2
dystrophy of Francois, punctate cortical lens changes, (FECD5), 10p11.2 (FECD6), 9p24.1-22.1 (FECD7), and 15q25
pseudoxanthom a elasticum , atopy (FECD8))
Variable expressivity and incom plete penetrance, m ay be
Path o lo gy: glycosam inoglycan and lipid deposits, stain
sporadic; m ost com m only inheritance unknown
with Alcian blue and colloidal iron (GAGs), Sudan black and
Oil-red-O (lipid) Onset usually in 4th decade of life or after, early variant
onset in 1st decade; fem ale > m ale (approxim ately 3:1);
P o s t e rio r a m o rp h o u s c o rn e a l d ys t ro p h y progressive
(P ACD) (AD)
Mapped to chrom osom e 12q21.33 (KERA, LUM, DCN, and Sym p to m s: blurred vision (initially, only in m orning; later,
EPYC) all day); m ay have pain, photophobia, tearing (ruptured
Early in life, m inim ally progressive or nonprogressive bullae)

Vision m ildly affected

Fin d in gs: cornea guttata starts centrally, m ay spread
peripherally (stage 1); endothelial decom pensation, strom al
Fin d in gs: diffuse gray-white sheet-like strom al opacities edem a, Descem et’s folds, endothelial beaten m etal appearance
concentrated in posterior strom a, m ostly centrally but extends with or without pigm ent dusting (stage 2); epithelial bullae,
to periphery bullous keratopathy (stage 3); subepithelial fibrosis, scarring,
Associated with corneal thinning (as thin as 380 m icrons), and peripheral superficial vascularization (pannus) (stage 4)
corneal flattening (< 41 D), hyperopia, prom inent Schwalbe
line, fine iris processes, pupillary rem nants, iridocorneal Path o lo gy: hyaline excrescences (guttae) on thickened
adhesions, corectopia, pseudopolycoria; no association with Descem et’s m em brane, atrophic endothelium with areas of cell
glaucom a dropout, endothelial cell polym egathism and pleom orphism

Ce n t ra l c lo u d y d ys t ro p h y o f Fra n c o is (CCDF) Tr eatm en t: hypertonic ointm ent, PK, contact lens or

Unknown inheritance (m ay be AD); m ay be posterior conjunctival flap (Gunderson) for com fort; m ay require
crocodile shagreen (corneal degeneration) Descem et’s stripping endothelial keratoplasty (DSEK) or
penetrating keratoplasty
Nonprogressive, asym ptom atic; onset in 1st decade of life
P o s t e rio r p o lym o rp h o u s c o rn e a l d ys t ro p h y
Fin d in gs: m ultiple, nebulous, polygonal gray areas
(P P CD) (AD)
separated by cracklike intervening clear zones
(indistinguishable from posterior crocodile shagreen); m ost PPCD1 m apped to chrom osom e 20p11.2-q11.2; PPCD2
dense centrally and posteriorly, fade anteriorly and m apped to chrom osom e 1p34.3-32.3 (COL8A2); PPCD3
peripherally m apped to chrom osom e 10p11.22 (ZEB1)

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CHAPTER 7 • Cornea /External Disease

Minim ally or nonprogressive, often asym m etric; occurs early Bu llo u s ke ra t o p a t h y

in life
Corneal edem a with epithelial bullae, thickened strom a, but
Vision usually norm al no guttata
Endothelium behaves like epithelium , form ing m ultiple Due to loss or dysfunction of endothelial cells
layers with occasional m igration of cells into angle causing
May develop fibrovascular pannus
glaucom a (15%)
Fin d in gs: isolated grouped vesicles, geographically shaped Etio lo gy: aphakia, pseudophakia (rigid AC IOL),
discrete gray lesions, broad bands with scalloped and flaky vitreocorneal touch, iridocorneal touch, sequela of severe or
m aterial (railroad tracks) at level of Descem et’s m embrane and chronic keratitis
endothelium ; corneal steepening (> 48 D in m ost PPCD3); m ay
develop stromal edem a, corectopia, peripheral iridocorneal
adhesions (25%), and increased intraocular pressure (15%) Path o lo gy: blister-like elevations of corneal epithelium ,
dam aged hem idesm osom es
Path o lo gy: irregular excrescences at level of Descem et’s
m em brane surrounded by gray opacification, thickened DDx o f co r n eal ed em a: aphakic bullous keratopathy
Descem et’s m em brane, abnorm al endothelial cells (resem ble (ABK), PBK, inflam m ation, infection, Fuchs dystrophy, PPCD,
epithelial cells; contain keratin, m icrovilli, desm osom es) with CHED, hydrops, acute angle-closure glaucom a, congenital
blebs, discontinuities, reduplication glaucom a, graft failure, contact lens overwear, hypotony, birth
traum a, ICE syndrom e, anterior segm ent ischem ia, Brown-
DDx: Haab’s striae in congenital glaucom a, ICE syndrom e McLean syndrom e
Brown-McLean Syndrome: peripheral corneal
Tr eatm en t: penetrating keratoplasty for endothelial decom pensation in aphakic patients, central cornea clear
decom pensation, can recur in graft ETIOLOGY: uncertain, possibly m echanical irritation
from floppy peripheral iris
Co n g e n it a l h e re d it a ry e n d o t h e lia l d ys t ro p h y
(CHED)
Ne u ro t ro p h ic ke ra t it is
(See Chapter 5, Pediatrics/Strabism us)
Corneal anesthesia due to CN 5 lesion

X-lin k e d e n d o t h e lia l c o rn e a l d ys t ro p h y (XECD) Disruption of reflex arc between CN V1 and lacrim al gland in
anesthetic eye causes epithelial abnorm alities
(See Chapter 5, Pediatrics / Strabism us)

Fin d in gs: conjunctival injection, corneal edem a, nonhealing

Mis c e lla ne o us epithelial defects, risk of infection and perforation; m ay have
AC reaction
Co rn e a g u t t a t a
Associated with aging and with corneal endothelial dystrophy
DDx: fam ilial dysautonom ia, topical anesthetic abuse,
”crack” cornea (anesthesia from sm oking crack cocaine),
Beaten m etal appearance HSV, HZV
Most never develop corneal edem a
Diagn o sis: decreased corneal sensation (test with cotton-
Path o lo gy: thickened Descem et’s with localized tipped applicator or anesthesiom eter)
excrescences of collagen; variable endothelial size and shape
and reduced num ber on specular m icroscopy Tr eatm en t: lubrication; consider prophylactic antibiotic,
bandage CL, punctal plugs, tarsorrhaphy, Boston ocular surface
De lle (n ) keratoprosthesis (PROSE lens)

Focal thinning(s) from dehydration due to adjacent area of

elevation (i.e., pingueculum , pterygium , filtering bleb) Fa m ilia l d ys a u t o n o m ia (Rile y-Da y s yn d ro m e )
(See Chapter 5, Pediatrics/Strabism us)
Tr eatm en t: lubrication; punctal occlusion to raise tear film ;
m ay require rem oval of inciting lesion
Exp o s u re ke ra t o p a t h y
De s c e m e t o c e le
Desiccation of corneal epithelium due to CN 7 lesion (failure
Extrem e focal thinning of cornea in which only Descem et’s
to close eyelids)
m em brane rem ains
Due to corneal m elt (see Corneal m elt section)
Fin d in gs: epithelial defects; m ay develop infection, m elt,
High risk of perforation perforation

226
 Corneal Disorders

Tr eatm en t: lubrication, m oisture cham ber goggles, lid (m oves across cornea destroying endothelium , resulting
taping at bedtim e; consider tarsorrhaphy, lid weights in strom al edem a)
Other findings: subepithelial infiltrates, new KP, increased
Co n t a c t le n s -re la t e d p ro b le m s IOP (trabeculitis from AC reaction)

SPK: m echanical or chem ical (solutions) Tr eatm en t:

Endothelial rejection: IV steroids (m ethylprednisone
3 an d 9 o ’clo ck stain in g: due to poor wetting of 500 m g; reduces risk of subsequent rejection episodes
peripheral cornea; refit lens [33% vs 67% for oral steroids]), topical steroids (40%
reversal rate alone), oral steroids
Epithelial rejection or stromal infiltrates with no graft
Ab r asio n : increased risk of infection in CL wearers
swelling: treat with topical therapy alone
Treatment: topical antibiotics, never patch

In filtr ates: hypoxia, antigenic reaction to preservatives in CL Pr o gn o sis: graft survival rate for treating endothelial
rejections with a single pulse of IV steroids is 90% when
solutions, infection
presents within 1 week and 67% when presents later than
Treatment: stop CL wear, topical antibiotics
1 week (probably due to irreversible dam age to endothelial
cells despite reversal of rejection)
Ed em a: epithelial m icrocystic edem a due to altered
m etabolism ; associated with extended wear CL
Treatment: decrease wear tim e; switch to daily wear lenses Ep it h e lia l d o w n g ro w t h
Can occur following alm ost any intraocular surgery; increased
Gian t p ap illar y co n ju n ctivitis (GPC): sensitivity to CL risk with com plicated surgery associated with hem orrhage,
m aterial, deposits on CL, or m echanical irritation inflam m ation, vitreous loss, or incarcerated tissue
Treatment: stop CL wear, topical allergy m edication
Surface epithelium grows through wound into eye, covering
anterior segm ent structures
Su p er io r lim b ic ker ato co n ju n ctivit is (SLK): m ay be
due to hypersensitivity or toxic reaction to thim erosal Appears as advancing line on corneal endothelium
Treatment: resolves with discontinuation of lens wear
Epithelium can cover the endothelium (leading to edem a) and
Neo vascu lar izatio n : superficial, deep, sectoral, or 360°; the angle (resulting in glaucom a); contact inhibition by
due to chronic hypoxia healthy endothelium m ay inhibit this
Treatment: refit lens with increased oxygen transm ission;
stop lens wear if significant vascularization Path o lo gy: m ultilayered nonkeratinized squam ous
epithelium ; PAS stains conjunctival goblet cells (differentiates
Co r n eal war p age: irregular astigmatism related to lens between corneal and conjunctival epithelium ) (Fig. 7-16)
material (hard > rigid gas-permeable [RGP] > soft), fit, and
length of time of wear; not associated with corneal edema.
Usually asymptomatic; may have contact lens intolerance, DDx o f r etr o co r n eal m em b r an e: m esoderm al
blurred vision with glasses, loss of best spectacle-corrected visual dysgenesis, ICE syndrom e, traum a, posterior polym orphous
acuity, or change in refraction (especially axis of astigmatism); dystrophy, disciform keratitis (HSV or HZV), alkali burn
corneal topography is abnorm al (irregular astigmatism)
Treatment: discontinue lens wear; repeat refraction and Diagn o sis: argon laser application to surface of iris produces
corneal topography until stable white burn if iris involved; helps delineate extent of epithelial
m em brane
Co rn e a l t ra n s p la n t fa ilu re
Ear ly (prim ary): poor tissue or surgery

Late: allograft rejection (delayed onset [at least 2 weeks]);

hom ograft reaction

Risk facto r s: young age, strom al vascularization, previous

graft failure, post chem ical burns, inflam m atory disease

Sym p to m s: decreased vision, redness, pain, irritation

Fin d in gs:
Epithelial rejection line (“battle” line of host-versus-donor
epithelium ): occurs in 10%, often precedes m ore
destructive rejection events Fig ure 7-16. Epithelial downgrowth with epithelium present on corneal
Endothelial rejection: anterior cham ber reaction, keratitic endothelium, angle, and iris, and into vitreous. (From Yanoff M, Fine BS:
precipitates, graft edem a, Khodadoust rejection line Ocular Pathology, ed 5, St Louis, 2002, Mosby.)

227
CHAPTER 7 • Cornea /External Disease

Tr eatm en t: excision of epithelial m em brane and involved and attack host’s organs; m inor histocom patability
tissue with cryotherapy to rem aining corneal m em brane; antigens are the inciting antigens, cytotoxic T cells are the
high-power laser ablation of entire m em brane m ay effectors)
occasionally work; com plete excision is difficult
Occu r s p r im ar ily in allo gen eic gr afts: 50% develop
Pr o gn o sis: poor, often develop secondary glaucom a acute GVHD (50% m ortality); 20% to 40% develop
chronic GVHD
Fib ro u s in g ro w t h
Acute GVHD is biggest risk factor for chronic GVHD
Fibrous proliferation through wound into AC
Less progressive and destructive than epithelial downgrowth Fin d in gs: hem orrhagic cicatricial conjunctivitis,
pseudom embranes, sym blepharon, punctate keratitis,
Fibroblasts originate from episclera or corneal strom a
keratoconjunctivitis sicca, lagophthalm os, entropion,
m icrovascular retinopathy (4%–10%), optic neuropathy (2%;
Lim b a l s t e m c e ll d e fic ie n c y thought to be due to cyclosporine [ciclosporin], resolves with
Etio lo gy: destruction of stem cells (chem ical burn, Stevens- discontinuation)
Johnson syndrom e, ocular cicatricial pem phigoid, atopic
keratoconjunctivitis, corneal infections, m ultiple ocular Oth er fin d in gs:
surgeries) or dysfunction of stem cells (aniridia, m ultiple Acute GVHD: m aculopapular rash on palm s and soles,
endocrine deficiency, neurotrophic, contact lens overwear) pruritus, GI involvem ent (diarrhea), elevated liver
enzym es
Sym p to m s: decreased vision, photophobia, pain, tearing, Chronic GVHD: scleroderm atoid or lichenoid skin changes,
blepharospasm Sjo€ gren-like syndrom e

Fin d in gs: conjunctivilization of cornea, irregular corneal Diagn o sis: skin, liver, intestinal biopsy
epithelium (stream ing/swirling pattern from area of lim bal
dam age) corneal vascularization, poor epithelial integrity, Tr eatm en t: cyclosporine, high-dose system ic steroids; treat
chronic inflam m ation ocular com plications

Path o lo gy: Bowm an’s layer destruction, fibrous tissue Pr o gn o sis: acute GVHD (stage 2 or higher) occurs in 12%
ingrowth
and has a 90% m ortality; chronic GVHD 10-year
survival ¼ 40%
Diagn o sis: im pression cytology (presence of goblet cells on
cornea); corneal epithelium takes up fluorescein Irid o c o rn e a l e n d o t h e lia l (ICE) s yn d ro m e
Tr eatm en t: lim bal stem cell transplant (autograft [from (See Chapter 10, Anterior Segm ent)
other eye if unilateral], allograft [am niotic m em brane]); poor
candidate for penetrating keratoplasty; risk of recurrent Mu lt ip le e n d o c rin e n e o p la s ia (MEN)
conjunctivilization
MEN 1 ( Wer n er ’s syn d r o m e) : m apped to
Gra ft -ve rs u s -h o s t d is e a s e (GVHD) chrom osom e 11
Om inous com plication of bone m arrow transplant (BMT) Neoplasias of pituitary, parathyroid, and islet cells of
pancreas
Findings: visual field defects from pituitary tum ors
3 r equ ir em en ts: Graft m ust contain im m unologically
com petent cells
Host m ust possess im portant transplantation isoantigens MEN 2a ( Sip p le’s syn d r o m e) ( AD) : m apped to
lacking in graft (so host appears foreign) chrom osom e 10
Host m ust not be able to m ount im m unologic reaction Medullary thyroid carcinom a, pheochrom ocytom a,
against graft parathyroid adenom as
Findings: prom inent corneal nerves
Typ es o f b o n e m ar r o w tr an sp lan ts: Pathology: thickened conjunctival nerves contain
Autologous: patient’s own stored m arrow num erous Schwann cells and partially m yelinated axons;
Syngeneic: m arrow from identical twin thickened corneal nerves are due to an increased num ber
Allogeneic: m arrow from HLA-m atched donor of axons per nerve fiber bundle
Diagnosis: elevated calcitonin and vanillylm andelic acid
Co m p licatio n s:
Acute: hem orrhage and infection (first 100 days following MEN 2b ( Sip p le-Go r lin syn d r o m e) ( AD) : m apped to
BMT [period of aplasia]) chrom osom e 10
Late: GVHD (T lym phocytes in transplanted m arrow Medullary thyroid carcinom a, pheochrom ocytom a,
recognize antigens of host as foreign, becom e sensitized, m ucosal and GI neurom as, m arfanoid habitus

228
 Scleral Disorders

Eye findings: prom inent corneal nerves, conjunctival Classificatio n :

neurom a (87%), eyelid neurom a (80%), dry eye (67%), Anterior: 98%
prom inent perilim bal blood vessels (40%) DIFFUSE (40%): m ost benign; widespread involvem ent
DDx of prominent corneal nerves: leprosy, Acanthamoeba, PATHOLOGY: sclera thickened, diffuse granulom atous
Down syndrom e, neurofibrom atosis, keratoconus, inflam m ation of scleral collagen
congenital glaucom a, Refsum ’s disease, Fuchs NODULAR (44%): focal involvem ent; nodule is
corneal endothelial dystrophy, failed corneal graft, im m obile
keratoconjunctivitis sicca, traum a, advanced PATHOLOGY: zonal necrotizing granulom a, fibrinoid
age, ichthyosis, posterior polym orphous dystrophy necrosis, chronic inflam m ation, fusiform
thickening, im m une com plex deposition with
Tu m o rs com plem ent activation; once collagen has been
destroyed, inflam m ation recedes, uvea herniates
Co rn e a l in t ra e p it h e lia l n e o p la s ia (CIN) into defect
(See Conjunctiva section) NECROTIZING (14%): associated with life-threatening
autoim m une disease (5-year m ortality rate ¼ 25%)
S q u a m o u s c e ll c a rc in o m a (S CC) WITH INFLAMMATION: focal or diffuse, progressive,
pain and redness; vascular sludging and occlusion;
(See Conjunctiva section) underlying blue uveal tissue m ay be visible giving
redness a ”violaceous” hue; 40% lose vision; 60%
have ocular com plications: anterior uveitis,
SCLERAL DISORDERS sclerosing keratitis, cataract, glaucom a, peripheral
corneal m elt, scleral thinning
Ep is c le rit is WITHOUT INFLAMMATION (sclerom alacia perforans):
Inflam m ation of episclera m inim al sym ptom s, m inim al injection; scleral
thinning, m arked ischem ia; seldom perforate;
Self-lim ited, usually young adults fem ales with severe rheum atoid arthritis; 8-year
m ortality rate ¼ 21%
33% bilateral; 67% recurrent
Posterior: 2%; usually unilateral; very painful
Associated with HZV, RA, gout Usually no associated system ic disorder
80% have anterior scleritis
Sign s: sectoral (70%) or diffuse (30%) injection with m ild or FINDINGS: thickened and inflam ed posterior sclera,
no discomfort; m ay have nodule (20%), chem osis, AC reaction chorioretinal folds, am elanotic fundus m ass, ON
edem a; m ay have vitritis, m acular edem a, lim itation
Path o lo gy: nongranulom atous vascular dilatation, of m otility, ptosis, proptosis, exudative RD, elevated
perivascular lym phocytic infiltration; diffuse or nodular IOP (forward shift of lens-iris diaphragm due to
(sim ilar to rheum atoid scleritis but lim ited to episclera); ciliochoroidal detachm ent)
palisade of epithelioid cells bordering central fibrinoid B-SCAN ULTRASOUND: pocket of fluid in Tenon’s space
necrosis (sclerotenonitis), T sign, thickened sclera
FA: early patchy hypofluorescence, late staining, disc
Diagn o sis: blanches with topical 2.5% phenylephrine; if leakage
lasts for > 3 weeks, perform system ic workup CT SCAN: thickening of posterior sclera with contiguous
involvem ent of orbital fat (T sign)
Tr eatm en t: topical vasoconstrictor (Naphcon A), m ild DDX: choroidal tum or, orbital pseudotum or (IOI),
topical steroid (FML, loteprednol [Alrex, Lotem ax]); consider uveal effusion syndrom e, VKH, thyroid-related
oral NSAID ophthalm opathy, orbital tum ors
S c le r it is Sym p to m s: pain, redness, photophobia
Inflam m ation of sclera
Fin d in gs: diffuse or sectoral area of deep injection (violaceous
More com m on in fem ales; onset age 30–60 years hue), tender to touch, chemosis, scleral edema; may have scleral
nodule, scleral thinning, AC reaction (33%), keratitis (acute
> 50% bilateral stromal keratitis, sclerosing keratitis, marginal keratolysis);
Etio lo gy: 50% associated with system ic disease chorioretinal folds and serous RD in posterior scleritis
Collagen vascular diseases (RA [20% of all cases of scleritis;
only 3% of patients with RA develop scleritis; 75% of RA- Diagn o sis: does not blanch with topical phenylephrine
related scleritis is bilateral], SLE, polyarteritis nodosa), Lab tests: CBC, ESR, RF, ANA, ANCA (antineutrophil
vascular disease (Wegener’s granulom atosis, giant cell cytoplasm ic antibody), VDRL, FTA-ABS, uric acid, blood
arteritis, Takayasu’s disease), relapsing polychondritis, urea nitrogen (BUN), PPD, CXR
sarcoidosis, ankylosing spondylitis, Reiter’s syndrom e, ANCA (antineutrophil cytoplasm ic antibodies): positive
Crohn’s disease, ulcerative colitis, infections (syphilis, in Wegener’s; also polyarteritis nodosa, Churg-
TB, leprosy, HSV, HZV), gout, rosacea Strauss, and crescentic glom erulonephritis

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CHAPTER 7 • Cornea /External Disease

2 TYPES: Fin d in gs: necrotizing sclerokeratitis, vascular occlusions,

C-ANCA (cytoplasm ic; positive in Wegener’s) ischem ic optic neuropathy, rarely aneurysm s of retinal vessels
P-ANCA (perinuclear; positive in polyarteritis
nodosa [PAN]) Oth er fin d in gs: kidneys (nephritic or nephrotic syndrom e,
Anterior segment fluorescein angiogram: m ay detect hypertension, renal failure), heart (MI, angina, pericarditis),
nonperfusion and vaso-obliteration intestines (pain, infarction), arthritis, peripheral neuropathy

Re la p s in g p o lyc h o n d rit is
Tr eatm en t: oral NSAID, consider oral steroids or
im m unosuppressive agents; treat underlying disorder; topical Multisystem disorder characterized by recurrent episodes of
steroids are not effective; sub-Tenon’s steroid injection is inflam m ation in cartilaginous tissues throughout body
contraindicated; m ay require patch graft for perforation
Fin d in gs (60%): episcleritis, scleritis, iritis, conjunctivitis,
keratoconjunctivitis sicca (KCS), exudative RD, optic neuritis,
Co m p licatio n s: keratitis, cataract, uveitis, glaucom a proptosis, CN 6 palsies

We g e n e r’s g ra n u lo m a t o s is Oth er fin d in gs: m igratory polyarthritis, fever, diffuse

inflam m ation of cartilage (nose, ear, trachea, larynx)
System ic disease of necrotizing vasculitis and granulom atous
inflam m ation involving sinuses, respiratory system , kidneys,
Tr eatm en t: topical or system ic steroids; dapsone
and orbit
Males > fem ales Dis c o lo ra t io n
Blu e scler a: sclera appears blue owing to thinning and
Fin d in gs (60%): nasolacrim al duct obstruction, proptosis,
visualization of underlying uvea
painful ophthalm oplegia, conjunctivitis, chem osis, KCS,
DDx: Ehlers-Danlos, Hurler’s, Turner’s, and Marfan’s
episcleritis, necrotizing scleritis (m ost com m on type of ocular
syndrom es; osteogenesis im perfecta, sclerom alacia
involvem ent), keratitis, cotton-wool spots, arterial narrowing,
perforans, congenital staphylom a
venous tortuosity, choroidal thickening, CME, CRAO, optic
nerve involvem ent
Scler al icter u s:
yellow sclera caused by hyperbilirubinem ia
Oth er fin d in gs: upper respiratory tract (hem optysis), renal
failure (m ajor cause of death), hem orrhagic derm atitis,
cerebral vasculitis, weight loss, peripheral neuropathy, fever, Och r o n o sis ( alkap to n u r ia) (AR): hom ogentisic acid
arthralgia, saddle nose (destruction of nasal bones) oxidase deficiency
Hom ogentisic acid accum ulates, m etabolized into m elanin-
like com pound with brown pigm entation of cartilage
Diagn o sis: C-ANCA positive in 67% (ear, nose, heart valves) and sclera; excreted in
urine (dark)
Findings: triangular pigm ent deposits anterior to rectus
Tr eatm en t: system ic steroids and im m unosuppressives
m uscle insertions, pinhead-sized peripheral corneal
(cyclophospham ide)
strom al deposits, pigm entation of tarsus and lids

Co m p licatio n s: fatal if untreated Co gan ’s sen ile scler al p laqu e: blue-gray hyalinization of
sclera anterior to horizontal rectus muscle insertions
Ta ka ya s u ’s d is e a s e
Narrowing of large branches of aorta
Dim inished pulsations in upper extrem ities
SURGERY
Decreased blood pressure in upper extrem ities, increased BP in P e n e t ra t in g ke ra t o p la s t y (P K; P KP )
lower extrem ities
Full-thickness corneal graft / transplant

Fin d in gs: scleritis, am aurosis fugax, ocular ischem ia, AION Mo st co m m o n in d icatio n s: bullous keratopathy
(pseudophakic, aphakic), keratoconus, graft failure, corneal
P o lya rt e rit is n o d o s a scar; Peter’s anom aly (children)

System ic vasculitis affecting m edium -sized and sm all arteries

Su ccess r ate: 90% of grafts are clear at 1 year (> 90% for
Males > fem ales keratoconus; 65% for HSV)

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 Surgery

Co m p licatio n s: rejection (20%), failure, glaucom a, Co m p licatio n s: graft rejection, suture-related

suprachoroidal or expulsive hem orrhage, endophthalm itis, com plications, and interface opacification can occur with both
recurrence of pathology procedures; intraoperative Descem et’s m em brane tear or
perforation can occur with DALK requiring conversion to
Co llab o r ative Co r n eal Tr an sp lan t Tr eatm en t Stu d y penetrating keratoplasty.
(CCTS): ABO blood type incom patability is a possible risk
factor for rejection En d o t h e lia l ke ra t o p la s t y (EK; DLEK, DS EK,
HLA m atching is not cost-effective or advantageous DS AEK, DMEK, DMAEK)
High-risk status: vascularization of cornea in 2 quadrants,
Techniques have evolved from Deep Lam ellar EKto Descem et’s
history of previous graft rejection, glaucom a, extensive
Stripping and Descem et’s Mem brane EK and Autom ated EK
PAS, traum atic or hereditary ocular surface disorders
m aking the procedure faster and safer with better visual
outcom es. Descem et’s Stripping and Autom ated Endothelial
Do n o r scr een in g: Keratoplasty (DSAEK) m anually stripping the endothelium
Contraindications for donor corneas: septicem ia, lym phom a and replacing with donor lenticule com posed of endothelium ,
or leukem ia, system ic m alignancy with ocular m etastasis, Descem et’s m em brane, and thin layer of strom a prepared with
anterior segm ent tum ors (choroidal m elanom a is not a a m icrokeratom e. Descem et’s Mem brane Endothelial
contraindication), Creutzfeldt-Jakob disease, rabies, Keratoplasty (DMEK; requires peripheral iridotom y/
subacute sclerosing panencephalitis, PML, HIV, hepatitis iridectom y) and Descem et’s Mem brane and Autom ated
B and C (neither HIV nor hepatitis C has ever been Endothelial Keratoplasty (DMAEK; lenticule has peripheral
transm itted) ring of strom a) have faster recovery, better vision, and lower
rejection rate
Tissu e p r eser vatio n :
4°C moist chamber: use within 48 hours
In d icatio n s: corneal edem a due to Fuchs dystrophy and
M-K medium (McCarey-Kaufm an): TC-199 m edium with
bullous keratopathy
dextran (5% concentration) and gentam icin stored at 4 °
C; use within 48 hours
Chondroitin sulfate: acts as antioxidant and m em brane Co m p licatio n s: endothelial rejection, lenticule separation
growth factor or displacem ent requiring rebubble (SF6 gas)
K-Sol (Kaufm an): chondroitin sulfate in TC-199 at 4 °C;
discontinued Ke ra t o p h a kia
Corneal storage media (CSM): from Minnesota Lions Eye
Donor lenticule is placed between the cornea and a
Bank researchers plus m ercaptoethanol (antioxidant)
m icrokeratom e created lam ellar section to correct aphakia
and chondroitin sulfate; discontinued with introduction
of dexsol and optisol
Dexsol: m inim al essential m edium with chondroitin Co n tr ain d icatio n s: thinned corneas, infants, glaucom a
sulfate and dextran 1% (osm otic agent to keep
cornea thin) Ke ra t o m ile u s is
Optisol: hybrid of K-sol, Dexsol, and CSM, plus vitam ins,
Lam ellar section is rem oved, frozen, shaped on a cryolathe,
am ino acids, and antioxidants; as effective as dexsol but
then replaced in strom al bed to correct m yopia or hyperopia.
keeps cornea thinner
Donor lenticule can be used instead (Fig. 7-17)
Many m edia contain pH indicators that change color with a
change in pH (i.e., m icrobial contam ination)
Co n tr ain d icatio n s: glaucom a

La m e lla r ke ra t o p la s t y (ALK, DALK) Ep ike ra t o p h a kia (Ep ike r a t o p la s t y)

Partial or full-thickness strom al graft com posed of epithelium , Epithelium is rem oved and a lathed donor lenticule is placed to
Bowm an’s layer, and strom a. Anterior lam ellar keratoplasty correct m yopia or hyperopia (Fig. 7-18)
(ALK) involves a strom al dissection, while deep anterior
lam ellar keratoplasty (DALK) entails replacem ent of strom al Can be perform ed in children; reversible
tissue down to the level of Descem et’s m em brane. DALK is
safer, has increased wound strength and less chance of Co n tr ain d icatio n s: blepharitis, dry eye, lagophthalm os
rejection. The dissection can be perform ed m anually, with an
air bubble or viscoelastic; intraoperative OCT can be helpful
Path o lo gy: 2 Bowm an’s layers (donor lenticule and patient)
(OCT-guided DALK)

As t ig m a t ic ke ra t o t o m y (AK)
In d icatio n s: strom al pathology (scar, dystrophy) and
99% depth arcuate or straight incisions typically placed at the 7
keratoconus (DALK)
or 8 m m optical zone (OZ) to correct astigm atism
Co n tr ain d icatio n s: corneal endothelial dysfunction Various nom ogram s exist (i.e., Lindstrom ARC-T)

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CHAPTER 7 • Cornea /External Disease

Myopic kera tomileusis Hyperopic kera tomileusis

cornea l button cornea l button

sha ved sha ved

cornea l button cornea l button

ra ised ra ised

sha ved sha ved

cornea l button cornea l button
lowered lowered

Fig ure 7-17. Keratomileusis. In a myopic keratomileusis, a corneal button is raised using a microkeratome, and it is reshaped using a cryolathe (upper part). When the
button is replaced, the central cornea is flattened (lower part). (From Chang SW, Azar DT: Automated and manual lamellar surgical procedures and epikeratoplasty. In
Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

Myopic correction Donor Hyperopic correction

Fig ure 7-18. Epikeratoplasty. A preshaped donor lenticule is sutured to the recipient stromal bed to correct myopia and hyperopia. (From Chang SW, Azar DT:
Automated and manual lamellar surgical procedures and epikeratoplasty. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

Do not m ake arcuate incisions > 90° (decreased efficacy, Number of incisions: the greater the num ber of incisions,
increased instability) the greater the effect; never use m ore than 8 incisions
(additional incisions have lim ited effect and cause
When com bined with RK, do not cross incisions (creates
greater destabilization of the cornea)
wound gape and instability)
Incision depth: the deeper the incision, the greater the
Lim bal/corneal relaxing incisions (LRI/CRI) are perform ed at effect; diam ond knife is set at 100% of thinnest
10–12 m m OZ, usually at tim e of cataract surgery with pachym etry m easurem ent (usually paracentral
preset diam ond knife (500–600 µm depth) inferotem poral region;
tem poral < inferior < nasal < superior)
Ra d ia l ke ra t o t o m y (RK) Optical zone: the sm aller the diam eter of the OZ
(the longer the incision), the greater the effect;
Deep radial corneal incisions to correct low to m oderate
do not m ake OZ < 3 m m (visual aberrations
m yopia
[starburst])
Effect dependent on num ber of incisions, depth of incisions, Patient age: the older the patient, the greater the effect
size of optical zone, patient age Patient sex: greater effect in m ales than in fem ales

232
 Surgery

Tech n iqu e: Am erican (downhill cut [toward lim bus], safer Tr eatm en t o f co m p licatio n s:
but shallower) vs Russian (uphill cut [toward central cornea], Enhancements: it is recom m ended to wait at least 3 m onths
deeper but risk of invading OZ); com bined technique with or until the refraction and corneal topography are stable
special knife gives deep cut with safety before perform ing an enhancem ent for overcorrection or
undercorrection
Co m p licatio n s: undercorrection or overcorrection, Haze: treat with topical steroids; consider application of
perforation, infection, scarring, irregular astigm atism , m itom ycin C 0.02% for 2 m inutes along with scar
progressive hyperopia (in PERK study, 43% had ! 1.00 D of d ebridem ent with either a diam ond burr or a superficial
hyperopia after 10 years), glare / starburst, fluctuating vision excim er laser treatm ent (PTK). Once the haze is reduced,
the refraction can change dram atically, so do not treat
residual refractive error at tim e of haze rem oval
Resu lts: 85% achieved 20/40 or better vision after initial Irregular astigmatism: rigid gas-perm eable contact lens,
procedure; 96% achieved 20/40 or better after enhancem ent,
wavefront-guided retreatm ent
85% achieved 20/25 or better after enhancem ent

La s e r in s it u ke ra t o m ile u s is (LAS IK)

P h o t o r e fra c t ive ke ra t e c t o m y (P RK)
Com bination of keratom ileusis and excim er laser ablation to
Laser ablation of corneal surface to correct m yopia, hyperopia,
correct m yopia, hyperopia, and astigm atism
and astigm atism
Corneal flap created with m echanical or laser m icrokeratom e
Excim er ( excited d im er ) laser : argon-fluoride and laser ablation perform ed on underlying strom al bed
(wavelength ¼ 193 nm ; far ultraviolet), energy ¼ 64 eV
Functions as a “cold” laser (breaks molecular bonds to ablate Ad van tages o f LASIK: m ore rapid healing, less discom fort,
tissue; no therm al dam age) less risk of haze, less postoperative m edications
Each pulse rem oves approxim ately 0.25 µm of corneal tissue
Disad van tages o f LASIK: flap com plications
Depth of ablation is related to diam eter of optical zone and
am ount of intended correction
Co m p licatio n s:
Mu n n er lyn equ atio n : depth ¼ (refractive error/3) Â OZ2 Intraoperative: flap com plication (buttonhole, incom plete,
Exa m p le : 12 µm for 6 mm OZ and 1 D of myopia or irregular flap, free cap) epithelial abrasion or
sloughing, program m ing error, decentered ablation.
Ad van tages o f PRK: precise rem oval of tissue, m inim al Immediate postoperative: diffuse lam ellar keratitis (DLK,
adjacent tissue dam age sands of Sahara), flap dislocation, flap striae, epithelial
ingrowth, central island, irregular astigm atism , dry eye,
undercorrection or overcorrection, night vision problem s
Disad van tages o f PRK: involves visual axis, risk of haze /
(glare, halos)
scarring, regression, requires rem oval of epithelium
Delayed postoperative: dry eye, late DLK, late flap slip,
undercorrection or overcorrection, keratectasia,
Co n tr ain d icatio n s: collagen vascular, autoim m une, or photophobia (from laser keratom e, 1–3 weeks to
im m unodeficiency disorders; pregnancy; corneal ectasia; 3 m onths postop, treat with topical steroids)
keloid form ation; isotretinoin (Accutane), am iodarone,
sum atriptan (Im itrex)
Tr eatm en t o f co m p licatio n s:
Buttonhole (increased risk with steep corneas), incomplete or
Cau tio n s: unstable refraction (change of > 0.5 D/year), irregular flap: replace flap, do not perform ablation; when
system ic diseases that affect wound healing (diabetes, atopy, cornea stabilizes, either cut a new flap or perform surface
connective tissue diseases, im m unocom prom ised status), ablation (PRK/ PTK)
herpes keratitis (HSV or HZV), ocular inflam m ation (keratitis, Free cap (increased risk in cases of flat corneas): place free
acne rosacea, pannus that extends into visual axis) cap in m oisture cham ber epithelial side down, perform
ablation, then replace cap and let dry
Co m p licatio n s: undercorrection or overcorrection, haze/ Epithelial slough or corneal abrasion: increased risk with
scarring, delayed reepithelialization, sterile infiltrates, certain m icrokeratom es and in eyes with epithelial
infection, decentered ablation, central island, irregular basem ent m em brane dystrophy; increases risk of
astigm atism , keratectasia, regression, glare, halos, dry eye, developing DLK and epithelial ingrowth. Treat with
glaucom a or cataract (from steroids) bandage contact lens and close follow-up. If large defect
Corneal haze: risk and severity increases with deeper occurs in first eye, consider postponing treatm ent of
ablations and postoperative ultraviolet (UV) radiation second eye
exposure; results in decreased vision with regression of Flap striae or flap dislocation: replace flap and stretch;
effect. Oral vitam in C 1000 m g/day for 1 week before consider use of epithelial d ebridem ent, hypotonic saline
surgery and 2 weeks postoperatively, and avoidance of or sterile water, ironing with a warm spatula, or suturing
UV exposure m ay help prevent haze (interrupted radials or antitorque)

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CHAPTER 7 • Cornea /External Disease

DLK: inflam m ation within flap interface; etiology often Disad van tages o f LASEK: slower visual recovery than
unknown; increased risk with epithelial defect. Late DLK with PRK.
m ay result from increased intraocular pressure, epithelial
defect, and cornea ulcer Co m p licatio n s: sam e as with PRK: undercorrection or
Treat with frequent topical steroids for grades I and II, and overcorrection, haze / scarring, delayed reepithelialization,
flap lifting and interface irrigation for grades III infection, decentered ablation, central island, irregular
and IV consider a short pulse of oral steroids astigm atism , regression, glare/halos, dry eye, strom al incursion
Central toxic keratopathy: noninflam m atory central corneal (with m echanical flap device)
opacity and hyperopic shift; appears on postop day 3–5,
usually preceded by DLK. No treatm ent. Spontaneously Risk of haze can be reduced with oral vitam in C and avoidance
resolves in 2–18 m onths. May also occur after PRK of UV exposure
Epithelial ingrowth: nests of epithelium growing under the
flap; active areas of ingrowth detected with fluorescein P h o t o t h e ra p e u t ic ke ra t e c t o m y (P TK)
staining of adjacent flap edge; m ore com m on in eyes
Use of excim er laser to ablate corneal pathology lim ited to
with epithelial basem ent m em brane dystrophy and after
anterior 1 3 of cornea
enhancem ents. Ingrowth that is progressive or affects
the vision is treated with flap lifting and d ebridem ent of In d icatio n s: superficial scars, dystrophies, irregularities,
the strom al bed and flap undersurface. Consider radial residual band keratopathy, recurrent erosions
sutures if ingrowth recurs, Consider Nd :YAG laser
treatm ent Can use confocal m icroscopy to determ ine depth of pathology
Irregular ablation (decentered, irregular
astigmatism): requires custom or wavefront-guided
Co m p licatio n s: recurrence of pathology, reactivation of
HSV, induced hyperopia or irregular astigm atism
retreatm ent
Corneal ectasia (keratectasia): treatm ent of eyes with a In t ra c o rn e a l in la ys
preexisting ectasia (keratoconus, form e fruste
keratoconus, pellucid m arginal degeneration) or A thin, contact lens-like, im plant placed in the central
excessive depth of ablation in norm al corneas (residual optical zone beneath a corneal flap or within a corneal
strom al depth < 250 µm ) can result in progressive pocket to correct m yopia, hyperopia, astigm atism , or
m yopic astigm atism and corneal ectasia. Treat with presbyopia without rem oving corneal tissue
corneal collagen cross-linking to halt progression. A Presbyopic im plants are inserted in the patient’s nondom inant
rigid gas-perm eable contact lens can help with visual eye to im prove near vision. Strategies for presbyopia
im provem ent, but will not stop progression. Consider correction: im plant thickness and shape alters the corneal
Intacs to im prove the shape of the cornea, or penetrating curvature (Raindrop Near Vision Inlay), im plant has power, or
keratoplasty for severe cases. im plant acts as a pinhole (KAMRA Inlay).
Dry eye: can worsen postoperatively if untreated prior to
surgery; can also occur in patients without pre-existing Ad van tages: reversible, adjustable, no rem oval of tissue
dry eye. Superior hinged flap m ay cause greater dryness
than is caused by nasal hinged flap. Treat with frequent Co m p licatio n s: undercorrection or overcorrection,
lubrication; consider punctal occlusion and topical irregular astigm atism, glare, halos, flap striae, epithelial
cyclosporine ingrowth, DLK, infection, im plant decentration or extrusion
Glare/halos: often due to residual refractive error. The role
In t ra s t ro m a l c o rn e a l rin g s e g m e n t s (In t a c s )
of scotopic pupil size as a contributing factor is
controversial (m ay have been a risk with older laser Ring segm ent im plants placed in to peripheral corneal
ablations, but is not a risk factor with m odern laser chann els outside the visual axis to correct low to
ablations). Treatm ent options include pharm acologic m oderate m yopia. Im plants flatten the cornea without
m iosis (Alphagan-P or iilocarpine), or retreatm ent with cuttin g or rem oving tissue from the central optical zone. Also
larger optical zone or blend zone used to reshape corneas with keratoconus or post-LASIK
Infectious keratitis: uncom m on but serious com plication; ectasia (Fig. 7-19)
m ost com m on organism s are Gram -positive cocci and
atypical Mycobacteria. Suspected infection requires lifting Ad van tages: reversible, adjustable, spares visual axis, no
flap and culturing; treat with fortified topical antibiotics rem oval of tissue
(vancom ycin and am ikacin); m ay require flap
am putation Co m p licatio n s: undercorrection or overcorrection,
irregular astigm atism, glare, halos, infection, im plant
La s e r-a s s is t e d e p it h e lia l decentration or extrusion, strom al deposits
ke ra t o m ile u s is (LAS EK)
Variation of PRK; epithelial flap is created with alcohol, Co n d u c t ive ke ra t o p la s t y (CK)
excim er laser is applied, and epithelial flap is replaced A contact probe directly delivers radiofrequency (therm al)
energy to the peripheral cornea in a ring pattern to steepen the
Ad van tages o f LASEK: m ay have less risk of haze than PRK central corneal and correct low to m oderate hyperopia

234
 Review Questions

c. sebaceous gland carcinom a

d. m alignant m elanom a
7. All of the following m ay cause follicular conjunctivitis
except
a. Chlamydia
b. Neisseria
c. lopidine
d. EKC
8. Which of the following tests is least helpful in determ ining
the etiology of enlarged corneal nerves?
a. EKG
b. calcitonin
c. urinary VMA
d. acid-fast stain
9. Corneal filam ents are least likely to be present in which
Fig ure 7-19. Slit-lamp photograph of the intrastromal corneal ring segments in condition?
position in patient’s cornea. (Courtesy of Mr. Thomas Loarie. From Friedman NJ ,
Husain SE, Kohnen T et al: Investigational refractive procedures. In: Yanoff M,
a. keratoconjunctivitis sicca
Duker J S [eds]: Ophthalmology, London, 1999, Mosby.) b. Thygeson’s SPK
c. SLK
d. m edicam entosa
Ad van tages: spares visual axis, no rem oval of tissue
10. Which of the following is not an appropriate treatm ent for
SLK?
Co m p licatio n s: undercorrection or overcorrection, a. bandage contact lens
irregular astigm atism , regression b. conjunctival resection
e. silver nitrate stick
d. conjunctival cautery
REVIEW QUESTIONS (Answe rs start on page 383) 11. In what level of the cornea does a Kayser-Fleischer ring
occur?
1. Which is the least desirable m ethod for corneal graft a. epithelium
storage? b. Bowm an’s layer
a. m oist cham ber at 4°C e. strom a
b. glycerin d. Descem et’s m em brane
c. Optisol 12. Cornea verticillata-like changes are associated with all of
d. cryopreservation the following except
2. Presently in the United States, phlyctenule is m ost a. indom ethacin
com m only associated with b. haloperidol
a. HSV e. chloroquine
b. tuberculosis d. am iodarone
c. Staphylococcus 13. The least com m on location for a nevus is
d. fungus a. bulbar conjunctiva
3. Which blood test is m ost helpful in the b. palpebral conjunctiva
evaluation of a patient with Schnyder corneal dystrophy? c. caruncle
a. calcium d. lid skin
b. uric acid 14. All of the following ions m ove across the corneal
c. im m unoglobulins endothelium by both active transport and passive
d. cholesterol diffusion except
4. Which disease has never been transm itted by a corneal a. ClÀ
graft? b. K+
a. CMV c. Na +
b. Creutzfeldt-Jakob d. H +
c. rabies 15. Which organism is associated with crystalline
d. Cryptococcus keratopathy?
5. Which corneal dystrophy does not recur in a corneal a. S. aureus
graft? b. H. influenzae
a. granular c. Enterococcus
b. m acular d. S. viridans
c. lattice 16. Which of the following conditions is associated with the
d. PPCD best 5-year prognosis for a corneal graft?
6. A conjunctival m ap biopsy is typically used for which a. PBK
m alignancy? b. Fuchs dystrophy
a. squam ous cell carcinom a c. ABK
b. basal cell carcinom a d. HSV

235
CHAPTER 7 • Cornea /External Disease

17. The best strategy for loosening a tight contact lens is to c. VKC
a. increase the diam eter d. GPC
b. increase the curvature 28. Which of the following is m ost likely to be associated with
c. decrease the diam eter m elanom a of the uvea?
d. decrease the curvature a. dysplastic nevus syndrom e
18. The type of contact lens that causes the least endothelial b. m elanosis oculi
pleom orphism is c. nevus of Ota
a. soft daily wear d. secondary acquired m elanosis
b. soft extended wear 29. Which of the following is not associated with N. gonorrheae
c. rigid gas perm eable conjunctivitis?
d. hard/PMMA a. pseudom em brane
19. Which of the following conditions is associated with the b. preauricular lym phadenopathy
worst prognosis for a corneal graft? c. purulent discharge
a. PBK d. corneal ulcer
b. Fuchs dystrophy 30. Even spreadingofthe tear film depends most on which factor?
c. Reis-Bucklers dystrophy a. lipid
d. keratoglobus b. aqueous
20. Which is not a treatm ent of acute hydrops? c. m ucin
a. steroid d. epithelium
b. hom atropine 31. Neurotrophic ulcer should not be treated with
c. bandage contact lens a. tarsorrhaphy
d. corneal transplant b. antiviral
21. Which organism cannot penetrate intact corneal epithelium? c. antibiotic
a. Corynebacterium diphtheriae d. bandage contact lens
b. N. gonorrhoeae 32. Which layer of the cornea can regenerate?
c. Pseudomonas aeruginosa a. Bowm an’s layer
d. H. aegyptius b. strom a
22. Which of the following m edications would be the best c. Descem et’s m em brane
choice in the treatm ent of m icrosporidial d. endothelium
keratoconjunctivitis? 33. The m ost appropriate treatm ent for a patient with
a. fum agillin sclerom alacia is
b. chloram phenicol a. topical steroid
c. galardin b. sub-Tenon’s steroid injection
d. parom om ycin c. oral NSAID
23. All of the following agents are used in the treatm ent of d. oral im m unosuppressive agent
Acanthamoeba keratitis except 34. The HEDS recom m endation for treating strom al
a. parom om ycin (disciform ) keratitis is
b. natam ycin a. topical steroid alone
c. chlorhexidine b. topical steroid and topical antiviral
d. m iconazole c. topical steroid and oral antiviral
24. Goblet cells are m ost abundant in which location? d. topical steroid, topical antiviral, and oral antiviral
a. fornix 35. Feathery edges and a satellite infiltrate are m ost
b. plica characteristic of a corneal ulcer caused by
c. bulbar conjunctiva a. Pseudomonas
d. lim bus b. Acanthamoeba
25. Thygeson’s superficial punctate keratopathy is best treated c. Microsporidia
with topical d. Fusarium
a. cyclosporine 36. PTK would be m ost appropriate for treating which of the
b. idoxuridine following corneal disorders?
c. loteprednol a. superficial granular dystrophy
d. trifluridine b. anterior strom al neovascularization
26. EKC is typically contagious for how m any days? c. m id-strom al herpes scar
a. 5 days d. Fuchs dystrophy
b. 7 days 37. A62-year-old wom an with keratoconjunctivitis sicca is most
c. 10 days likely to dem onstrate corneal staining in which location?
d. 14 days a. superior third
27. A shield ulcer is associated with b. m iddle third (interpalpebral)
a. AKC c. inferior third
b. SLK d. diffuse over entire cornea

236
 Review Questions

38. Which of the following findings is m ost com m only a. sym blepharon
associated with SLK? b. corneal perforation
a. filam ents c. irregular astigm atism
b. giant papillae d. corneal neovascularization
c. pseudom em brane 48. After a corneal alkali burn, which of the following signs is
d. follicles associated with the worst prognosis?
39. Which lab test is m ost helpful to obtain in a 38-year-old a. conjunctival chem osis
m an with herpes zoster ophthalm icus? b. com plete corneal epithelial defect
a. ANCA c. corneal edem a
b. Lym e titer d. com plete lim bal blanching
c. chest x-ray 49. The m ost likely cause of a corneal keratom etry
d. HIV test m easurem ent 2.5 D steeper inferiorly than superiorly at
40. A patient with conjunctival intraepithelial neoplasia is the 3 m m zone is
m ost likely to have a. pterygium
a. CMV b. Mooren’s ulcer
b. EBV c. keratoconus
c. HSV d. Terrien’s m arginal degeneration
d. HPV 50. The nasociliary branch of the trigem inal nerve innervates
41. Which of the following disorders is m ost the tip of the nose and the
likely to be found in a patient suffering from sleep a. frontal sinus
apnea? b. lacrim al gland
a. iritis c. upper eyelid
b. interstitial keratitis d. cornea
c. follicular conjunctivitis 51. The m ost com m on com plication of pterygium surgery is
d. trichiasis a. dellen
42. A patient with graft-vs-host disease is m ost likely to b. scleral m elt
have which eye finding? c. pyogenic granulom a
a. scleritis d. recurrence
b. sym blepharon 52. The refractive effect of Intacs is titrated by the
c. optic neuropathy a. num ber of ring segm ents inserted
d. iritis b. optical zone of the ring segm ents
43. Topical corticosteroids should not be used in a patient c. thickness of the ring segm ents
with which form of herpes sim plex keratitis? d. curvature of the ring segm ents
a. epithelial 53. At the 4-week postop exam of a patient who underwent
b. endothelial uncom plicated PRK for m oderate m yopia, the m anifest
c. disciform strom al refraction is À0.25 D OD and À1.25 D OS. Slit lam p exam
d. necrotizing strom al shows m oderate anterior strom al haze OS. How would
44. What is the m ost appropriate m anagem ent for a patient you m anage this patient?
who reports recurrent foreign body sensation when a. add topical antibiotic
waking up but on exam does not have a discrete epithelial b. increase topical steroids
defect? c. bandage contact lens
a. epithelial debridem ent d. PRK enhancem ent with m itom ycin-C
b. lubrication 54. A buttonhole flap is m ost likely to occur with a
c. anterior strom al puncture m icrokeratom e if the patient has
d. phototherapeutic keratectom y a. axial length > 26 m m
45. A 24-year-old wom an reports a painful, red left eye and b. corneal pachym etry < 520 m icrons
blurry vision after sleeping with her contact lens for several c. keratom etry > 47 D
days. Exam ination shows a corneal ulcer with edem a and a d. white-to-white > 12.5 m m
hypopyon. The m ost likely diagnosis is 55. The best surgical option for a patient with Fuchs
a. HSV dystrophy, corneal edem a, and a visually significant
b. Candida cataract is
c. Acantham oeba a. phacoem ulsification and IOL
d. Pseudomonas aeruginosa b. phacoem ulsification, IOL, and penetrating
46. The best test to m easure basal tear secretion is keratoplasty
a. dye disappearance test c. phacoem ulsification, penetrating keratoplasty, and
b. Jones I test contact lens
c. Schirm er’s test with anesthesia d. phacoem ulsification, IOL, and endothelial
d. tear film breakup tim e keratoplasty
47. Adenoviral m em branous conjunctivitis is m ost likely to
cause which of the following?

237
CHAPTER 7 • Cornea /External Disease

Kaufm an, H. E., Barron, B. A., & McDonald, M. B. (1997). The cornea
SUGGESTED READINGS (2nd ed.). Philadelphia: Butterworth-Heinem ann.
Krachm er, J. H., Mannis, M. J., & Holland, E. J. (2011). Cornea
Arffa, R. C. (1998). Grayson’s diseases of the cornea (4th ed.). St Louis: (3rd ed.). St Louis.
Mosby. Krachm er, J. H., & Palay, D. A. (2013). Cornea color atlas (3rd ed.).
Basic and Clinical Sciences Course. (2016). Section 8: External Disease Philadelphia: Saunders.
and Cornea. San Francisco: AAO. Foster, C. S., Azar, D. T., & Dohlm an, C. H. (2005). Smolin and Thoft’s
Brightbill, F. S., McDonnell, P. J., McGhee, C. N. J., et al. (2008). the cornea: Scientific foundations and clinical practice (4th ed.).
Corneal surgery theory, technique and tissue (4th ed.). St Louis: Mosby. Philadelphia, PA: Lippincott William s & Wilkins.

238
8 Uveitis
PATHOPHYSIOLOGY
CLASSIFICATION
ANTERIOR UVEITIS
INTERMEDIATE UVEITIS
POSTERIOR UVEITIS
ENDOPHTHALMITIS
PANUVEITIS
MASQUERADE SYNDROMES
DIFFERENTIAL DIAGNOSIS OF UVEITIS AND ASSOCIATED SIGNS
SURGERY AND UVEITIS

P ATHOP HYSIOLOGY Lo catio n : sclerouveitis, keratouveitis, anterior uveitis (iritis),

iridocyclitis, lens-induced uveitis, interm ediate uveitis,
endophthalmitis (infection or inflam m ation of the vitreous,
In flam m ato r y r eactio n :
anterior cham ber, ciliary body, and choroid), posterior uveitis
Acute: chem ical m ediators (histam ine, serotonin, kinins,
(retinitis, choroiditis, vasculitis), panuveitis (endophthalm itis
plasm in, com plem ent, leukotrienes, prostaglandins)
and involvem ent of the sclera)
Chronic: cellular infiltrates
NONGRANULOMATOUS: lym phocytes, plasm a cells
GRANULOMATOUS: epithelioid and giant cells ANTERIOR UVEITIS
(Langhans’, foreign body, Touton)
3 PATTERNS: diffuse (e.g., VKH syndrom e), discrete Inflam m ation of iris (iritis) and ciliary body (cyclitis)
(e.g., sarcoidosis), zonal (e.g., lens-induced)
Most com m on cause of anterior uveitis in adults is idiopathic
Retin al S-an tigen : (50%) followed by HLA-B27 associated uveitis
Protein found in hum an retinal photoreceptors and
Most com m on cause of acute, noninfectious, hypopyon iritis is
pineal gland
HLA-B27-associated iritis
Most potent uveitic antigen; thought to be an enzym e
located in rod outer segm ents Etio lo gy:
In animal model: produces uveitis when injected into Children: JRA, ankylosing spondylitis, psoriatic arthritis,
nonocular sites (experim ental autoim m une uveitis acute interstitial nephritis, Fuchs’ heterochrom ic
[EAU]; type 4 hypersensitivity); rem oval of thym us iridocyclitis, sarcoidosis, postviral, HSV, Lym e disease,
prevents EAU (supports T cell-m ediated basis of traum a, Kawasaki’s disease
inflam m atory response) 50% have posterior com ponent (toxoplasm osis in 50%
Human diseases that resemble EAU: VKH, sym pathetic with posterior com ponent)
ophthalm ia, birdshot retinochoroidopathy; Young adults: HLA-B27 associated, sarcoidosis, HSV,
lym phocytes from these patients develop an in vitro syphilis, Fuchs’, Behçet’s disease, spillover from
proliferative response to retinal S-antigen, suggesting interm ediate or posterior uveitis
an autoim m une basis for these diseases Older adults: idiopathic, sarcoidosis, HSV, HZO,
m asquerade syndrom es (peripheral RD [Schwartz-
Matsuo syndrom e], intraocular foreign body, JXG,
CLASSIFICATION m ultiple sclerosis, m alignancies [retinoblastom a,
leukem ia, large cell lym phom a, m alignant m elanom a])
Path o lo gy: nongranulom atous, granulom atous Classificatio n :
Nongranulomatous:
Etio lo gy: infection, im m une response, m alignancy, traum a, ACUTE: idiopathic, HLA-B27 associated (5% of general
chem ical, idiopathic population; associated with acute, recurrent uveitis;

239
CHAPTER 8 • Uveitis

usually starts unilaterally; associated with the

following 4 disorders: ankylosing spondylitis, Reiter’s
syndrom e, psoriatic arthritis, inflam m atory bowel
disease), Behçet’s disease, glaucom atocyclitic crisis
(Posner-Schlossm an syndrom e), HSV, HZO,
Kawasaki’s disease, Lym e disease, Ebola (convalescent
phase; anterior, posterior, or panuveitis) traum atic,
postoperative, other autoim m une diseases (lupus,
relapsing polychondritis, Wegener’s granulom atosis,
interstitial nephritis), drugs (rifabutin, cidofovir,
sulfonam ides, bisphosphonates, diethylcarbam azine,
m etipranolol, system ic m oxifloxacin)
CHRONIC (duration > 6 weeks): JRA, Fuchs’
heterochrom ic iridocyclitis, HSV, HZO
Granulomatous:
INFECTIOUS: syphilis, TB, leprosy, brucellosis, Fig ure 8-2. Keratic precipitates in anterior uveitis. (From Forster DJ : General
toxoplasm osis, P. acnes, fungal (Cryptococcus, approach to the uveitis patient and treatment strategies. In Yanoff M, Duker J S
[eds]: Ophthalmology, London, 1999, Mosby.)
Aspergillus), HIV
IMMUNE-MEDIATED: sarcoidosis, VKH syndrom e, May be white or pigm ented, sm all or large,
sym pathetic ophthalm ia, lens induced nongranulom atous (fine) or granulom atous
(phacoanaphylactic/phacoantigenic), MS (m utton fat), diffuse or focal
NONGRANULOMATOUS: com posed of lym phocytes
and PMNs
Sym p to m s: pain, photophobia, decreased vision, redness GRANULOMATOUS: com posed of m acrophages,
lym phocytes, epithelioid cells, and m ultinucleated
giant cells
DDX OF DIFFUSE KP: Fuchs’ heterochrom ic iridocyclitis,
Fin d in gs: conjunctival and episcleral injection, ciliary sarcoidosis, syphilis, keratouveitis, toxoplasm osis
injection (circum corneal flush from branches of anterior ciliary (rarely)
arteries), m iosis (iris sphincter spasm ), AC reaction; m ay have Iris nodules:
hypopyon, keratic precipitates, iris nodules, dilated iris vessels KOEPPE: located at pupil m argin; occur in
(occasionally, rubeosis), synechiae (posterior [iris adhesions to granulom atous and nongranulom atous uveitis
lens; seclusio pupillae is a com plete adhesion that can result in BUSACCA: located on anterior iris surface; occur only in
iris bom be] or anterior [iris adhesions to cornea and angle])
granulom atous uveitis (Fig. 8-3)
(Fig. 8.1) BERLIN: located in anterior cham ber angle; occur in
Keratitic precipitates (KP): aggregates of white blood cells granulom atous uveitis
on corneal endothelium ; typically located inferiorly and
centrally (Fig. 8-2)
DDx o f h yp o p yo n :
May occur with any intraocular inflam m ation, m ost
HLA-B27 associated, infection (keratitis, endophthalm itis),
com m only with uveitis
foreign body, JRA Behçet’s disease, VKH syndrom e,
m alignancy (leukem ia, lym phom a, retinoblastom a),
toxic (rifabutin)

Fig ure 8-1. Severe idiopathic anterior uveitis with fibrinoid reaction. (From Fig ure 8-3. Chronic granulomatous uveitis demonstrating Busacca
Hooper P.L. (1999). Idiopathic and other anterior uveitis. In Yanoff M. Duker J .S. nodules. (From Forster DJ : General approach to the uveitis patient and treatment
[eds]. Ophthalmology. London: Mosby.) strategies. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

240
 Anterior Uveitis

DDx o f u veitic glau co m a: Lum bar puncture

HSV, HZV, Posner-Schlossm an syndrom e, Fuchs’ Brain MRI
heterochrom ic iridocyclitis, sarcoidosis; rarely Audiom etry
toxoplasm osis, syphilis, sym pathetic ophthalm ia ELDERLY FEMALE WITH VITRITIS: rule out intraocular
lym phom a or infection
Vitreal biopsy for culture or cytology
Diagn o sis: UNILATERAL IRIDOCYCLITIS, FINE WHITE KP, LIGHTER
Minimum: CBC with differential, ESR, VDRL and FTA-ABS IRIS IN AFFECTED EYE: rule out Fuchs’ heterochrom ic
Expanded (for recurrent anterior [3 or m ore attacks], iridocyclitis
granulom atous, positive review of system s, or posterior IOP
involvem ent): erythrocyte sedim entation rate, ACE, Gonioscopy (fine-angle vessels)
ANA, ANCA, IL-10, PPD plus anergy panel, CXR or chest
CT; consider Lym e test in endem ic areas, HLA typing Tr eatm en t: topical steroids, cycloplegic; m ay require
(25% with HLA-B27 develop sacroiliac disease, so obtain system ic steroids, im m unosuppressive agents, antibiotics
sacroiliac X-ray), HIV Ab test; for endophthalm itis, Poor response to steroids: Fuchs’, syphilis, toxoplasm osis,
culture by tap to determ ine organism keratouveitis, Lym e disease, chronic postoperative
Targeted approach: endophthalm itis, ARN, CMV
RECURRENT UVEITIS, WITH BACK STIFFNESS UPON
AWAKENING: rule out ankylosing spondylitis
Co m p licatio n s: iris atrophy, band keratopathy, cataract,
HLA-B27 glaucom a, cystoid macular edem a
Lum bosacral spine im aging (CT)
GRANULOMATOUS UVEITIS: rule out TB and sarcoidosis
CXR An kylo s in g s p o n d ylit is
Upper body gallium scan 90% HLA-B27-positive; typically young m en
Angiotensin-converting enzym e ([ACE]; elevated in
60% with sarcoidosis; also in Gaucher’s disease, Sym p to m s: lower back pain and stiffness with inactivity
m iliary tuberculosis, silicosis) (upon awakening in m orning)
Lysozym e
Serum calcium
Fin d in gs: anterior uveitis (30%; recurrent in 40%),
Liver function tests and bilirubin
episcleritis, scleritis
PPD
CHILD WITH RECURRENT OR CHRONIC
IRIDOCYCLITIS: rule out JRA (usually ANA-positive, Oth er fin d in gs: arthritis (sacroiliac and peripheral joints),
RF-negative) heart (aortic insufficiency, heart block), colitis (10%), lungs
ANA (restricted chest expansion, apical fibrosis)
RF
HLA-B8 Sacr o iliac X-r ay: sclerosis and narrowing of joint space;
RETINAL VASCULITIS, RECURRENT APHTHOUS ULCERS, ligam entous ossification can occur
PRETIBIAL SKIN LESIONS: rule out Behçet’s disease
Skin lesion biopsy Re it e r’s s yn d ro m e (Re a c t ive a rt h rit is )
HLA-B51 and -B27
PARS PLANITIS AND EPISODIC PARESTHESIAS: rule out
Triad of conjunctivitis, urethritis, and arthritis
m ultiple sclerosis (MS) 75% HLA-B27; m ales > fem ales
Brain MRI Associated with infections: Chlamydia, Ureaplasma urealyticum,
Lum bar puncture Yersinia, Shigella, Salmonella
RETINOCHOROIDITIS ADJACENT TO PIGMENTED CR
SCAR: rule out toxoplasm osis Fin d in gs: m ucopurulent conjunctivitis, keratitis, acute
Toxoplasm a IgM and IgG titers anterior uveitis
RETINAL VASCULITIS AND SINUSITIS: rule out Wegener’s
granulom atosis Diagn o sis: 3 m ajor criteria, or 2 m ajor and 2 m inor criteria
ANCA Major:
CXR Urethritis
Sinus film s Polyarthritis (knees, sacroiliac joints)
Urinalysis Conjunctivitis
RECURRENT UVEITIS AND DIARRHEA: rule out Keratoderm a blenorrhagicum (scaling skin lesion,
inflam m atory bowel disease (IBD) often on feet and hands [20%]; sim ilar to pustular
GI consult psoriasis)
Endoscopy with biopsy Minor:
CHOROIDITIS, EXUDATIVE RD, EPISODIC Plantar fasciitis and Achilles tendinitis
TINNITUS: rule out Harada’s disease/VKH syndrom e Circinate balanitis
FA Painless m outh ulcers

241
CHAPTER 8 • Uveitis

Prostatitis DDx o f ir is h eter o ch r o m ia: traum a (intraocular m etallic

Cystitis foreign body), inflam m ation, congenital Horner’s syndrom e,
Spondylitis/sacroiliitis iris m elanom a, Waardenburg’s syndrom e (iris heterochrom ia,
Tendinitis telecanthus, white forelock, congenital deafness), Parry-
Recent history of diarrhea Rom berg syndrom e (iris heterochrom ia, Horner’s syndrom e,
Iritis/keratitis ocular m otor palsies, nystagm us, facial hem iatrophy),
glaucom atocyclitic crisis, m edication (topical prostaglandin
P s o ria t ic a rt h rit is analogues [Xalatan, Lum igan, Travatan]), nevus of Ota

Associated with HLA-B17 and HLA-B27

Path o lo gy: plasm a cells in ciliary body
Uveitis does not occur in psoriasis without arthritis
Tr eatm en t: poor response to steroids
Fin d in gs: conjunctivitis, dry eyes, anterior uveitis
Co m p licatio n s: glaucom a (60%), cataract (PSC; 50%)
Oth er fin d in gs: arthritis (hands, feet, sacroiliac joints),
psoriatic skin and nail changes
J u ve n ile rh e u m a t o id a rt h rit is (J RA)
In fla m m a t o ry b o w e l d is e a s e (IBD) (See Chapter 5, Pediatrics/Strabism us)
Uveitis occurs in ulcerative colitis (10%) and Crohn’s
disease (3%) Ka w a s a ki’s d is e a s e
(See Chapter 5, Pediatrics/Strabism us)
Fin d in gs: conjunctivitis, keratoconjunctivitis sicca,
episcleritis, scleritis, anterior uveitis, orbital cellulitis, optic
neuritis
Lym e d is e a s e
Due to Borrelia burgdorferi (spirochete)
Oth er fin d in gs: arthritis, erythem a nodosum , pyoderm a Ocular involvem ent is usually bilateral
gangrenosum , hepatitis, sclerosing cholangitis
Affected organ system s: skin, CNS, cardiovascular,
m usculoskeletal
Fu c h s ’ h e t e r o c h ro m ic irid o c yc lit is
Occurs in young adults; unilateral
Fin d in gs (in stage 2 and 3 disease): cranial nerve palsies
Associated with chorioretinal scars (toxo) (CN 7 m ost com m on), orbital m yositis, follicular
conjunctivitis, sym blepharon, episcleritis, keratitis (m ultiple
Sym p to m s: blurred vision num m ular strom al infiltrates), chronic granulom atous
iridocyclitis with posterior synechiae and vitreous cells,
interm ediate or posterior uveitis, pars planitis, chorioretinitis,
Fin d in gs: diffuse sm all white stellate KP, m inim al AC exudative RD, CME, BRAO, retinal vasculitis, papilledem a,
reaction, no posterior synechiae, iris heterochrom ia (diffuse pseudotum or cerebri, optic neuritis, optic atrophy
atrophy of strom a, loss of iris crypts; involved iris is usually
lighter; 15% bilateral), fine-angle vessels (m ay bleed during
gonioscopy, cataract surgery, or paracentesis) (Fig. 8-4) P o s n e r-S c h lo s s m a n s yn d ro m e
(g la u c o m a t o c yc lit ic c ris is )
Recurrent anterior uveitis and increased IOP; episodes are
typically self-lim ited

Sym p to m s: unilateral pain

Fin d in gs: m ild AC reaction, few or no KP, elevated IOP

Tr eatm en t: m ay require topical glaucom a m edications to

control IOP, steroids to control inflam m ation

P h a c o a n a p h yla c t ic e n d o p h t h a lm it is
(p h a c o a n t ig e n ic u ve it is )
Fig ure 8-4. Fuchs’ uveitis. (From Hooper PL: Idiopathic and other anterior Im m une com plex disease (type 3 hypersensitivity reaction)
uveitis. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.) when norm al tolerance to lens protein is lost

242
 Posterior Uveitis

Previous rupture of lens capsule in sam e or fellow eye, followed

by latent period; with re-exposure to lens protein during
surgery, zonal granulom atous uveitis occurs
Associated with traum a (80%)
May develop severe granulom atous uveitis with hypotony,
secondary open-angle glaucom a

Path o lo gy: zonal pattern (PMNs infiltrate central lens

m aterial; epithelioid histiocytes and m ononuclear cells
around nidus)

Tr eatm en t: rem ove lens

In t e rs t it ia l n e p h rit is
Fig ure 8-5. Inferior pars plana s nowbank with attached snowballs. (From
Rare; can be idiopathic or triggered by allergic reaction to Zimmerman PL: Pars planitis and other intermediate uveitis. In Yanoff M, Duker J S
m edicines (antibiotics, NSAIDs) [eds]: Ophthalmology, London, 1999, Mosby.)

Associated with acute anterior uveitis

Multiple sclerosis: bilateral pars planitis can occur (80%
bilateral, 20% unilateral); m ay present with band
Path o lo gy: interstitial edem a with m ononuclear
keratopathy, m ild AC reaction, vitreous cells in a young
inflam m atory cells; eosinophils can be present
patient; can also have cataract (PSC), epiretinal
m em brane, CME, retinal phlebitis

INTERMEDIATE UVEITIS
Tr eatm en t: (m ain indication is CME with reduced vision):
periocular and oral steroids; consider im m unosuppressive
Etio lo gy: m ultiple sclerosis (5–25% have periphlebitis and agents, vitrectom y, cryotherapy to areas of peripheral NV
interm ediate uveitis), Lym e disease, sarcoidosis, Fuchs’ (controversial; can decrease leakage and m acular edem a, but
heterochrom ic iridocyclitis can lead to retinal tears)

P a rs p la n it is
Pr o gn o sis: 10% self-lim ited, 90% chronic ( 1 3 with
Most com m on type of interm ediate uveitis (85–90%) exacerbations)
Usually young adults; fem ales > m ales; 75% bilateral
Co m p licatio n s: cataract, retrolenticular cyclitic m em brane,
Accounts for 25% of uveitis in children
VH (from peripheral retinal neovascularization), tractional
Associated with HLA-DR15 and MS RD, CME (prim ary cause of vision loss, followed by cataract),
band keratopathy
Etio lo gy: unknown; diagnosis of exclusion

Sym p to m s: floaters, decreased vision P OSTERIOR UVEITIS

Fin d in gs: light flare with a few KP, anterior vitritis, Most com m on cause of posterior uveitis in adults is
snowballs (white vitreous cellular aggregates near ora serrata; toxoplasm osis (followed by retinal vasculitis)
m ay coalesce to form peripheral fibrovascular accum ulation
[snowbank] over inferior pars plana and vitreous base),
peripheral retinal periphlebitis, hyperem ic disc, no Sign s: vitreous cells, m em branes, opacities, inflam m atory
chorioretinitis, no synechiae (Fig. 8-5) exudates (“snowballs”), vasculitis (opacification around
vessels [sheathing {whole vessel}, cuffing {segm ent
Path o lo gy: of vessel}]), exudates (candlewax drippings), retinitis,
Snowballs: epithelioid cells and m ultinucleated giant cells pigm entary changes (due to RPE inflam m ation), choroiditis
Snowbank: preretinal m em brane of fibroglial and vascular (focal, m ultifocal, or diffuse), choroidal detachm ent
elem ents
Peripheral retinal veins: often have perivascular cuff of DDx o f vitr itis:
lym phocytes Panuveitis: sarcoidosis, TB, syphilis, VKH, sym pathetic
ophthalm ia, Behçet’s disease, Ebola
DDx: sarcoidosis, toxoplasm osis, toxocariasis, syphilis, Lym e Postsurgical/trauma: Irvine-Gass syndrom e
disease, MS Endophthalmitis

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CHAPTER 8 • Uveitis

Choroiditis: acute posterior m ultifocal placoid pigm ent

epitheliopathy (APMPPE), serpiginous, birdshot,
m ultifocal choroiditis, Toxocara, POHS
Retinitis: ARN, CMV, toxoplasm osis, candidiasis,
cysticercosis, onchocerciasis
Vasculitis: Eales disease
Other infections: nem atodes, Whipple’s disease, EBV, Lym e
disease
Other: am yloidosis, ocular ischem ia, m asquerade
syndrom es, spillover from anterior uveitis
Intermediate uveitis: pars planitis, MS

Co m p licatio n s: NV, CME, ON swelling and atrophy

CME: com m on cause of vision loss in pars planitis,
birdshot retinochoroidopathy, retinal vasculitis, and any
iridocyclitis (especially chronic, recurrent cases); CME Fig ure 8-6. Cytomegalovirus retinitis. (From Hudson HL, Boyer DS, Martin DF,
does not occur in VKH et al: Viral posterior uveitis. In Yanoff M, Duker J S [eds]: Ophthalmology,
London, 1999, Mosby.)

In d icatio n s fo r cyto to xic th er ap y: Behçet’s disease,

Fin d in gs: well-circum scribed necrotizing retinitis
sym pathetic ophthalm ia, VKH, pars planitis, Eales disease,
(2 appearances), m ild AC and vitreous reaction
retinal vasculitis, serpiginous choroidopathy, OCP, necrotizing
Brushfire: indolent, granular, yellow-white advancing edge
scleritis, inflam m ations unresponsive to m axim um steroid
with peripheral atrophic “burned out” region
therapy
Pizza-pie fundus: thick, yellow-white necrosis; hem orrhage,
vascular sheathing (Fig. 8-6)
Zone 1: 1 disc diam eter surrounding the disc and 2 disc
Infe c tio ns diam eter around the fovea: im m ediately sight
threatening
Co n g e n it a l ru b e lla s yn d ro m e Zone 2: anterior to zone 1 and posterior to vortex vein
am pullae
(See Chapter 5, Pediatrics/Strabism us)
Zone 3: peripheral to zone 2

Me a s le s
Path o lo gy: infected retinal cells are m arkedly enlarged, then
(See Chapter 5, Pediatrics/Strabism us) necrotic, finally atrophic; large owl’s eye intranuclear
inclusions
To xo p la s m o s is
(See Chapter 5, Pediatrics/Strabism us) Tr eatm en t: antiviral therapy (induction during first
2 weeks); intravitreal injections for sight threatening lesions
Ganciclovir (Cytovene): virostatic
To xo c a ria s is TOXICITY: m yelosupression with neutropenia and
(See Chapter 5, Pediatrics/Strabism us) throm bocytopenia (5–10% of patients)
INDUCTION: 5–7.5 m g/kg IV bid  2–4 weeks
MAINTENANCE: 5–10 m g/kg IV qd
P re s u m e d o c u la r h is t o p la s m o s is
INTRAVITREAL INJECTION: 2 m g/0.1 m L 2–3 tim es a
s yn d ro m e (P OHS ) week  2–3 weeks
(See Chapter 11, Posterior Segm ent) Foscarnet: virostatic
TOXICITY: renal, seizures, anem ia
INDUCTION: 90 m g/kg IV bid or 60 m g/kg IV
Cyt o m e g a lo viru s (CMV)
tid  2 weeks
Progressive hem orrhagic necrotizing retinitis involving all MAINTENANCE: 90–120 m g/kg IV qd
retinal layers INTRAVITREAL INJECTION: 2.4 m g/0.1 m L 2–3 tim es a
week  2-3 weeks, then 1–2 tim es a week
Four-year incidence rate is 12% of AIDS patients; usually when Cidofovir (Vistide): longer half-life
CD4 count < 50 cells/m m 3 TOXICITY: renal, anterior uveitis (50%), hypotony
40% bilateral at presentation INDUCTION: 3–5 m g/kg IV once a week  2 weeks
MAINTENANCE: 3–5 m g/kg IV once every 2 weeks
Rare syndrom e of neonatal cytom egalic inclusion disease Adm inistered through peripheral line (central line
required for ganciclovir and foscarnet)
Sym p to m s: often asym ptom atic; m ay have floaters, INTRAVITREAL INJECTION: 165–330 µg once a
scotom a week  3 weeks, then every 2 weeks

244
 Posterior Uveitis

Associated with lowering of IOP (about 2 m m Hg), 20– Path o lo gy: necrosis occurs from virally induced cytolysis,
50% develop iritis (about 5 days after last infusion) arteriolar and choriocapillaris occlusion; necrotic cells slough
Probenecid and hydration with each dose to reduce renal into vitreous, leaving large areas devoid of retina
toxicity and decrease iritis
Median tim e to progression ¼ 120 days FA: focal areas of choroidal hypoperfusion early; late staining
B-scan ultrasound in patients with severe hypotony
revealed CB atrophy Tr eatm en t: treatm ent needs to be individualized
Ganciclovir implant (Vitrasert): Antiviral agents which can be tapered slowly over m onths
Median tim e to progression ¼ 194 days (vs 72 days with following resolution of the acute herpetic phase:
IV ganciclovir and 15 days with no treatm ent); lasts Acyclovir (15 m g/kg/day divided every 8 h IV for 7 days,
approxim ately 8 m onths followed by 800 m g five tim es daily orally for 3–4 m onths)
May be increased risk of RD with im plantation Fam ciclovir (500 m g orally q8h)
Oral valganciclovir: L-valyl ester of ganciclovir (prodrug), Valacyclovir (1000–2000 m g orally q8h for induction)
60% bioavailability, fatty foods increase bioavailability, Ganciclovir (500 m g IV q12h)
reaches peak concentration after 2 hours Valganciclovir (900 m g twice daily orally for 3 weeks
INDUCTION: 900 m g BID for 21 days induction, then 450 m g twice daily po for m aintenance)
MAINTENANCE: 900 m g QD Intravitreal injections for sight threatening retinitis:
Surgery: vitrectom y with long-acting tam ponade for Ganciclovir (2m g per 0.1 m L)
RRD repair (occurs in 5–29% of patients) Foscarnet (1.2-2.4 m g per 0.1 m L)
Prednisone (0.5-2.0 m g/kg/day orally for up to 6-8 weeks)
Co m p licatio n s: rhegm atogenous RD initiated 24-48 hours after the start of antiviral therapy or
once regression of retinal necrosis has been dem onstrated

Ac u t e re t in a l n e c ro s is (ARN)
Pr o gn o sis: watch fellow eye closely (usually develops ARN
Acute self-lim ited confluent peripheral necrotizing retinitis due within 4 weeks); anti-viral agents reduce risk by 50%
to infection with VZV, HSV, or rarely CMV
65–90% develop rhegm atogenous RD (usually within
Usually occurs in im m unocom petent individuals; 33% 3 m onths)
bilateral (BARN), com m only in im m unosuppressed
Association with HLA-DQw7, HLA-Bw62, and HLA-DR4 in
P ro g re s s ive o u t e r re t in a l n e c ro s is (P ORN)
Am erican Caucasian populations and HLA-Aw33, HLA-B44,
and HLA-DRw6 in Japanese populations Variant of ARN in AIDS but painless with m inim al intraocular
inflam m ation

Sym p to m s: rapid onset of ocular/periocular pain, pain on Often have history of cutaneous zoster
eye m ovem ent, redness, photophobia, floaters, decreased
74% unilateral at presentation, 70% becom e bilateral
vision, constriction of visual field

Fin d in gs: m ultiple discrete peripheral or central areas of

Fin d in gs: diffuse episcleral injection, m ild iritis with retinal opacification/infiltrates (deep with very rapid
granulom atous KP, vitritis; “thum bprint” num m ular infiltrates progression), “cracked m ud” appearance after resolution;
posterior to equator with isolated peripheral patches of vasculitis is not prom inent (Fig. 8-8)
necrotizing retinitis that becom es confluent; sawtooth
dem arcation line between necrotic and healthy retina,
generalized obliterative retinal arteritis (with peripheral vaso- Tr eatm en t: sam e as above; poor response to antivirals
occlusion), pale disc edem a (Fig. 8-7); within 2 m onths,
retinitis gradually resolves and necrotic retina sloughs; coarse Pr o gn o sis: 67% becom e NLP with in 4 weeks; RD
salt and pepper pigm entation in 90%

Fig ure 8-7. Acute retinal necrosis. (From Kaiser

PK, Friedman NJ , Pineda II, R: Massachusetts eye
and ear infirmary illustrated manual of
ophthalmology, ed 2, Philadelphia, 2004, WB
Saunders.)

intra re tina l he morrha ge s re tina l ne cros is

245
CHAPTER 8 • Uveitis

Fig ure 8-8. Progressive outer retinal necrosis, early stage. (From Hudson HL, Fig ure 8-9. Multiple choroidal lesions in Pneumocystis choroiditis. (From Cowan
Boyer DS, Martin DF, et al: Viral posterior uveitis. In Yanoff M, Duker J S [eds]: CL: Sarcoidosis. In Yanoff M, Duker J S [eds ]: Ophthalmology, London, 1999,
Ophthalmology, London, 1999, Mosby.) Mosby.)

He rp e s zo s t e r Associated with use of inhaled pentam idine (which is

Uveitis typically develops during convalescence from acute prophylaxis for pulm onary Pneumocystis only)
Varicella infection Cys t ic e rc o s is
Reactivated uveitis (anterior and/or posterior), m ay have Due to infection with pork tapeworm Taenia solium or
keratitis (epithelial or strom al) T. saginata; hum ans are definitive host, pigs are
May require chronic topical steroid treatm ent to prevent interm ediate host
recurrence Adult worm lives in sm all intestine; larvae travel to eye,
Ca n d id ia s is producing cystic subretinal or intravitreal lesion (cysticercus)

Yeast-like form (blastoconidia), or pseudohyphae or elongated

Fin d in gs: m ass lesion or exudative RD (Fig. 8-10)
branching structures (pseudom ycelia)
Occurs in debilitated patients on hyperalim entation and in IV Tr eatm en t: vitrectom y required for posterior segm ent
drug abusers disease
Most cases occur without positive blood cultures or ongoing Le p ro s y
fungem ia
Due to Mycobacterium leprae
Approxim ately 10% with candidem ia develop
endophthalm itis 5–15 m illion infected; 250,000 blind

Fin d in gs:
Fin d in gs: anterior uveitis, retinal hem orrhages, perivascular Lids: lagophthalm os, m adarosis, blepharochalasis,
sheathing, chorioretinitis with fluffy white lesions (puff balls; nodules, trichiasis, entropion, ectropion, reduced
m ay be joined by opaque vitreous stands [“string of pearls”]), blinking
vitreous abscess; m ay have subretinal abscess

DDx o f p ale su b r etin al m ass: m etastasis, am elanotic

m elanom a, choroidal osteom a, old subretinal hem orrhage,
granulom a

Cu ltu r e: blood agar or Sabaraud’s glucose (large, cream y

white colonies)

Tr eatm en t: IV am photericin B

Pr o gn o sis: 70% m ortality within 1 year

P n e u m o c ys t is c h o ro id it is
Due to Pneumocystis carinii
Choroiditis with m ultifocal orange num m ular lesions; lesions Fig ure 8-10. Cysticercus in the eye. (From Cowan CL: Sarcoidosis. In Yanoff M,
contain cysts of Pneumocystis carinii (Fig. 8-9) Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

246
 Posterior Uveitis

Lacrimal: acute and chronic dacryocystitis

Cornea: anesthesia, exposure keratopathy, band
keratopathy, corneal leprom a, interstitial keratitis,
thickened nerves, superficial strom al keratitis
Sclera: episcleritis, scleritis, staphylom a, nodules
Iris: m iosis, iritis, synechiae, seclusio pupillae, atrophy, iris
pearls, leprom a
Ciliary body: loss of accom m odation, hypotony, phthisis
Fundus: peripheral choroidal lesions, retinal vasculitis

Co m p licatio n s: cataract, glaucom a

Diffu s e u n ila t e ra l s u b a c u t e
n e u ro re t in it is (DUS N)
Due to infection with sm all roundworm in subretinal space: Fig ure 8-11. Sclerosing keratitis as a result of onchocerciasis. (Courtesy of
Professor HR Taylor. From Cowan CL: Sarcoidosis. In Yanoff M, Duker J S [eds]:
dog hookworm (Ancylostoma caninum) or racoon nem atode
Ophthalmology, London, 1999, Mosby.)
(Baylisascaris procyonis)

Fin d in gs: deep, gray-white retinal lesions; optic nerve optic neuritis and atrophy; m ay develop cataract, PAS, and
swelling; vitreous cells; late findings include retinal vascular glaucom a (Fig. 8-11)
narrowing, diffuse RPE pigm entary changes, optic atrophy;
results in unilateral “wipe-out” syndrom e
Oth er fin d in gs: pruritus, pigm entary changes (leopard-
skin appearance), chronic inflam m ation, scarring,
ERG: decreased m aculopapular rash, subcutaneous nodules

Tr eatm en t: laser nem atode Diagn o sis: skin snip (place piece of skin in tissue m edia,
20–100 m icrofilariae em erge)
Ca t -s c ra t c h d is e a s e
Due to Bartonella henselae (also causes oculoglandular Tr eatm en t: iverm ectin, suram in, diethylcarbam azine (DEC)
syndrom e)
Op h t h a lm o m yia s is
Fin d in gs: retinal granulom a or neuroretinitis Due to direct ocular invasion by fly larvae (m aggots) that can
invade AC, posterior segm ent, or subretinal space
Tr eatm en t: oral antibiotics (doxycycline 100 m g bid, and
rifam pin 300 m g bid  4–6 weeks) Su b r etin al in vasio n : larvae travel through fundus, leaving
criss-crossing tracks of atrophied RPE; death of organism causes
On c h o c e rc ia s is (Rive r b lin d n e s s ) ocular inflam m ation

Due to Onchocerca volvulus; larvae m ature in hum ans, form ing

Wh ip p le ’s d is e a s e
adult worm s that live in fibrous, subcutaneous nodules
(usually in joints); fem ale worm s (100 cm long, live for up Due to Tropheryma whipplei; Gram -positive bacillus,
to 20 years) and m ale worm s (5 cm long, live for m uch Actinom ycetes fam ily; found intracellularly and extracellularly
shorter tim e) reproduce sexually, fem ales give birth to $ 2000
m icrofilariae/day; m icrofilariae (300 µm long, live for Fin d in gs: uveitis, retinal vasculitis
1–2 years) m igrate all over body by direct invasion and
hem atogenous spread, prefer skin and eyes; live m icrofilariae Oth er fin d in gs: m alabsorption (diarrhea, steatorrhea),
induce little or no inflam m ation; death of organism causes m igratory arthralgias
severe granulom atous inflam m ation and scarring
Transm itted by black fly: breeds along fast-m oving stream s; Path o lo gy: PAS-positive m acrophages within lam ina
bites infected host and acquires m icrofilariae, which m ature propria of “clubbed” (abnorm al) m icrovilli of intestinal wall,
into infectious larvae; transm its larvae to hum ans with bite; 17 or vitreous biopsy
m illion infected; 1–2 m illion blind (Africa and Latin Am erica)
Second leading cause of corneal blindness in world (trachom a
Tr eatm en t: Bactrim m ay be helpful; m ay require a year of
therapy as shorter courses lead to relapses
is first)

P ro p io n ib a c t e riu m a c n e s
Fin d in gs: intraocular m icrofilariae (in AC), anterior
uveitis, SPK, sclerosing keratitis, scleritis, chorioretinitis, Gram -positive rod; anaerobic

247
CHAPTER 8 • Uveitis

May becom e sequestered in capsular bag following cataract AP MP P E le s ions

surgery

Fin d in gs: delayed onset, chronic granulom atous uveitis,

often with fibrin or hypopyon; white plaque on posterior
capsule is characteristic

Tr eatm en t: intravitreal antibiotics (vancom ycin,

cephalosporins; resistant to am inoglycosides); usually also
requires partial or total rem oval of capsular bag with/without
IOL rem oval/exchange

Infla m m a tio ns ( Table 8-1)

Fig ure 8-12. Acute posterior multifocal placoid pigment epitheliopathy
demonstrating multiple posterior pole lesions. (From Kaiser PK, Friedman NJ ,
Ac u t e p o s t e rio r m u lt ifo c a l p la c o id p ig m e n t Pineda II R: Massachusetts eye and ear infirmary illus trated manual of
ophthalmology, ed 2, Philadelphia, 2004, WB Saunders.)
e p it h e lio p a t h y (AP MP P E)
Occurs in young, healthy adults; fem ale ¼ m ale; usually FA: initial blockage with late hyperfluorescent staining;
bilateral window defects in old cases (Figs. 8-13, 8-14)
Acute, self-lim ited; m ay be nonspecific choroidal
hypersensitivity reaction
FAF: hyper autofluoresence of acure lesions, older lesions are
Possible HLA-B7 association hypo autoflourescent
Associated with cerebral vasculitis
Flu-like prodrom e (33%) followed by decreased vision
ICG: early and late hypofluorescence of placoid lesions

Fin d in gs: m ultiple cream y yellow-white placoid lesions

(usually < 1 DD) at level of RPE or choriocapillaris (possibly OCT: hyperreflectivity in outer plexiform layer; focal areas of
due to choroidal hypoperfusion); lesions fade over 2–6 weeks, discontinuous outer segm ent ellipsoid zone, relatively healthy
leaving geographically shaped RPE changes photoreceptor layers
(hypopigm entation and hyperpigm entation); m ay have
vitreous cells, m ild AC reaction; rarely vascular sheathing,
disc edem a, choroidal neovascularization (CNV) (Fig. 8-12) Tr eatm en t: none

Oth er fin d in gs: thyroiditis, erythem a nodosum , cerebral Pr o gn o sis: vision recovers in m ost patients to > 20/40;
vasculitis, regional enteritis rarely recurs

Ta b le 8-1. White dot syndromes

AP MP P E MEWDS Se rp ig ino us Bird s ho t Multifo c a l c ho ro id itis P IC

Age 20–40 15–50 30–50 40–60 20–50 20–40
Sex F¼M F> M F¼ M F> M F> M Female
Laterality Bilateral Unilateral Bilateral Bilateral
HLA B7, DR2 None B7 A29 None None
Vitritis Mild Mild Mild Chronic, moderate Chronic, moderate None
Lesions Large, geographic, Small, soft, gray- Active, geographic, Deep, creamy spots; 50–350 µm gray-white 100–300 µm
gray-white shallow white dots; no gray-white patches; indistinct margins; yellow s pots; mixture of old yellow or gray
pigmented scars scarring deep scars with yellow scars without scars and new spots spots; punched-
within 1–2 weeks fibrosis pigmentation out scars
Macula Rare CNV Granularity Subretinal scars CME CME Atrophic scars
Rare CNV 25% CNV Rare CNV 35% CNV 40% CNV
Prognosis Good Good Poor Fair Fair Good
Treatment None None Steroids Steroids Steroids None
Cyclosporine Anti-VEGF for CNV Anti-VEGF for
CNV

248
 Posterior Uveitis

e a rly hypofluore s ce nce multiple e va ne s ce nt white dot s yndrome

Fig ure 8-13. Fluorescein angiogram of same patient as shown in Fig. 8-13 Fig ure 8-15. Multiple evanescent white dot syndrome demonstrating faint
demonstrating early hypofluorescence of the lesions. (From Kaiser PK, Friedman white spots. (From Kaiser PK, Friedman NJ , Pineda II, R: Massachusetts eye and
NJ , Pineda II, R: Massachusetts eye and ear infirmary illustrated manual of ear infirmary illustrated manual of ophthalmology, ed 2, Philadelphia, 2004, WB
ophthalmology, ed 2, Philadelphia, 2004, WB Saunders.) Saunders.)

la te s ta ining dot s yndrome e a rly hype rfluore s ce nce

Fig ure 8-14. Fluorescein angiogram of same patient as shown in Fig. 8-14 Fig ure 8-16. Same patient as s hown in Fig. 8-16 demonstrating early fluorescein
demonstrating late staining of the lesions. (From Kaiser PK, Friedman NJ , Pineda angiogram appearance. (From Kaiser PK, Friedman NJ , Pineda II, R:
II, R: Massachusetts eye and ear infirmary illus trated manual of ophthalmology, Massachusetts eye and ear infirmary illustrated manual of ophthalmology, ed 2,
ed 2, Philadelphia, 2004, WB Saunders.) Philadelphia, 2004, WB Saunders.)

S e rp ig in o u s c h o ro id o p a t h y
Mu lt ip le e va n e s c e n t w h it e d o t
Chronic, recurrent, indolent disease of unknown etiology
s yn d ro m e (MEWDS )
Onset between age 40–60; fem ale ¼ m ale; usually bilateral
Onset between age 15 and 50; fem ale (80%) > m ale;
unilateral > bilateral Affects inner chorioretinal pigm ent epithelium

Sym p to m s: decreased vision, dark spots in the periphery Sym p to m s: painless loss of vision
(peripheral scotom as), shim m ering photopsias
Fin d in gs: geographic (m ap-like) pattern of scars with active
Fin d in gs: granular retinal appearance with sm all
edges (yellow-gray, edem atous), usually beginning in posterior
(100–200 µm) white spots in posterior pole at level of RPE;
pole (often extending from disc); active areas becom e atrophic
m ay have vitreous cells, positive RAPD, m ild optic disc swelling
over weeks to m onths; new lesions occur contiguously or
(Fig. 8-15)
elsewhere (often in snake-like/serpiginous pattern); m ay have
VF: enlarged blind spot m ild AC reaction, vitritis, vascular sheathing, RPE detachm ent,
NVD, CNV (rare) (Fig. 8-17)
FA: early hyperfluorescence in wreath-like configuration; late
staining of lesions and optic nerve (Fig. 8-16) VF: absolute scotom as corresponding to atrophic scars
OCT: norm al
FA: acute lesions stain
ERG: reduced a wave (involvem ent of RPE and outer retina)
ICG: hypofluoresence of lesions
Pr o gn o sis: vision recovers over weeks but m ay be
perm anently decreased due to pigm entary changes in fovea; FAF: hyper autofluorescence of lesions with hypo
10% have recurrent episodes in sam e or fellow eye autofluorescent surrounding area

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CHAPTER 8 • Uveitis

Fig ure 8-17. Fundus view of the right eye of a 57-year-old woman with early Fig ure 8-18. Birdshot retinochoroidopathy. (From Moorthy RS, J ampol LM:
serpiginous choroiditis. (From Moorthy RS, J ampol LM: Posterior uveitis of Posterior uveitis of unknown cause. In Yanoff M, Duker J S [eds ]: Ophthalmology,
unknown cause. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, London, 1999, Mosby.)
Mosby.)

OCT: loss of photoreceptors in lesions; outer retinal swelling

in acute lesions

Tr eatm en t: m ay respond to oral steroids,

im m unosuppressive drugs

Pr o gn o sis: fair to poor; com m only recurs m onths to

years later

Bird s h o t c h o ro id o p a t h y (vit ilig in o u s

c h o rio re t in it is )
Occurs after 4th decade of life; fem ale > m ale; usually bilateral
Associated with HLA-A29 (90%)

Fig ure 8-19. Fundus view of the left eye of a 50-year-old woman who has
Sym p to m s: decreased vision, nyctalopia, decreased color progressive subretinal fibrosis and uveitis syndrome. (From Moorthy RS, J ampol
vision, peripheral visual field loss LM: Posterior uveitis of unknown cause. In Yanoff M, Duker J S [eds]:
Ophthalmology, London, 1999, Mosby.)

Fin d in gs: cream -colored depigm ented spots scattered FA: acute lesions block or fill early and stain late; older lesions
throughout fundus; m ay have m ild AC reaction, vitritis, retinal behave like window defects with early hyperfluorescence and
vasculitis, disc edem a, optic atrophy, CME, epiretinal late fading
m em brane, CNV (Fig. 8-18)
FAF: old lesions are hypo autofluorescent
FA: pronounced perifoveal capillary leakage with CME
OCT: outer retinal swelling; discontinuous outer segm ent
ERG: dim inished scotopic response (rod dysfunction) ellipsoid zone

Mu lt ifo c a l c h o ro id it is Tr eatm en t: steroids

Onset between age 20–50; fem ale > m ale
Co m p licatio n s: CNV (m ost com m on cause of vision
loss), CME
Fin d in gs: m ultiple gray-white to yellow lesions
(50–350 µm ) at level of choroid or RPE; vitreous and AC cells;
P u n c t a t e in n e r c h o ro id o p a t h y (P IC)
chronic lesions becom e atrophic with punched-out m argins,
variable am ounts of pigm entation, and occasionally fibrosis Onset between age 20 and 40; healthy, m oderately
(Fig. 8-19) m yopic wom en

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 Posterior Uveitis

Fig ure 8-20. Punctate inner choroidopathy. (From Moorthy RS, J ampol LM:
Posterior uveitis of unknown cause. In Yanoff M, Duker J S [eds ]: Ophthalmology,
London, 1999, Mosby. Fig ure 8-21. Retinal pigment epitheliitis. (From Moorthy RS, J ampol LM:
Posterior uveitis of unknown cause. In Yanoff M, Duker J S [eds ]: Ophthalmology,
London, Mosby, 1999.)
Sym p to m s: acute scotom as and photopsias

Fin d in gs: sm all (100–300 µm ) yellow or gray inner

choroidal lesions; resolve over weeks to form atrophic scars
that m ay enlarge and becom e pigm ented; new lesions do
not appear; may have serous RDs; 40% risk of CNV; no AC
or vitreous cells (Fig. 8-20)

Tr eatm en t: none

Ac u t e re t in a l p ig m e n t e p it h e liit is (Krill’s d is e a s e )
Rare, occurs in young adults; usually unilateral

Sym p to m s: sudden decrease in vision

Fin d in gs: clusters of hyperpigm ented spots (300–400 µm ) Fig ure 8-22. “Frosted branch angiitis” secondary to cytomegalovirus
in m acula surrounded by yellow-white halos; with resolution, retinitis. (From Hudson HL, Boyer DS, Martin DF, et al: Viral posterior uveitis.
the spots lighten or darken, but halos rem ain; no vitritis In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)
(Fig. 8-21)
Sym p to m s: rapid loss of function in 1 or m ore regions of
DDx: central serous retinopathy, acute m acular visual field, photopsias
neuroretinopathy (wedge-shaped or cloverleaf dark red
perifoveal dots; paracentral scotom a; norm al ERG) Fin d in gs: m inim al changes initially; retinal degeneration/
pigm entary changes late
FA: blockage of spots with halos of hyperfluorescence DDx: MEWDS, acute m acular neuroretinopathy, m ultifocal
choroiditis
Tr eatm en t: none
VF: scotom as (m ay enlarge)
Pr o gn o sis: resolves com pletely in 6–12 weeks ERG: decrease rod and cone

Fro s t e d b ra n c h a n g iit is Id io p a t h ic u ve a l e ffu s io n s yn d ro m e

White spots along retinal arterioles (Fig. 8-22) Occurs in healthy, m iddle-aged m ales
Chronic, recurrent disorder

DDx: CMV retinitis, toxoplasm osis (Kyrieleis’ plaques) Sym p to m s: decreased vision, m etam orphopsia, scotom as
Fin d in gs: serous retinal, choroidal, and ciliary body
Ac u t e zo n a l o c c u lt o u t e r re t in o p a t h y (AZOOR)
detachm ents; m ild vitritis, RPE changes (leopard spots),
Usually bilateral; fem ales > m ales conjunctival injection; key finding is shifting subretinal fluid

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CHAPTER 8 • Uveitis

B-scan u ltr aso u n d : thickened sclera, serous RD, choroidal 25% CULTURE POSITIVE: Bacillus (30%), S. epidermidis
detachm ents (25%), Streptococci (13%), S. aureus (8%),
Gram -negative and m ixed flora
FA: no discrete leakage Poor visual outcom e
Endogenous:
Tr eatm en t: steroids and im m unosuppressive agents are not RISK FACTORS: im m unosuppression, indwelling
effective; consider scleral resection in nanophthalm ic eyes, or venous catheters, IVDA, following intra-abdom inal
quandrantic partial-thickness scleral windows surgery
30% of patients with Candida septicem ia develop
endophthalmitis
Sterile: culture negative
ENDOP HTHALMITIS Tr eatm en t:
rule out infection (AC and vitreous taps)
Inflam m ation involving 1 or m ore coats of the eye and Intravitreal antibiotics: vancom ycin 1 m g/0.1 m L,
adjacent ocular cavities ceftazidim e 2.25 m g/0.1 m L (consider intravitreal
dexam ethasone 400 µg/0.1 m L)
Etio lo gy: infectious or sterile; m ust rule out infection Systemic antibiotics: in severe cases, m ost traum atic cases,
and endogenous bacterial cases; vancom ycin 1 g IV
Sym p to m s: pain, decreased vision q12 hours or cefazolin 1 g IVq8 hours; ceftazidim e 1 g IV
q12 hours
Fin d in gs: lid edem a, chem osis, AC reaction, hypopyon Topical fortified antibiotics: vancom ycin 25–50 m g/m L,
(pink hypopyon ¼ Serratia), vitritis ceftazidim e 50 m g/m L q1 hour
Topical steroids and cycloplegics
Classificatio n :
Acute postoperative (< 6 weeks after surgery; 90% in 1st
week):
MAJ OR CLINICAL STUDY
RISK FACTORS: blepharitis, wound leak, iris prolapse,
vitreous loss, contam inated IOL, diabetes, chronic End o p htha lm itis Vitre c to m y
alcoholism Stud y (EVS)
ORGANISMS: S. epidermidis, S. aureus, Streptococci,
Pseudomonas Ob je c t ive : to evaluate the treatm ent of acute (< 6 weeks)
INCIDENCE: postoperative (cataract or secondary IOL surgery)
Extracapsular extraction (ECCE) or endophthalm itis with im m ediate vitrectom y vs “tap and
intracapsular cataract extraction (ICCE) (with or inject,” and whether intravenous antibiotics (ceftazidim e
without IOL): 0.072% and am ikacin) are necessary
Secondary IOL: 0.3%
PPV: 0.51%
PK: 0.11% Me t h o d s : Patients with clinical evidence of acute
Glaucom a filter: 0.061% (Streptococcus [Enterococcus] (< 6 weeks) postoperative (cataract or secondary IOL
faecalis, Streptococci [57%], H. influenzae [23%]) surgery) endophthalm itis and visual acuity of LP or better,
PREVENTION: and sufficient clarity to see at least som e part of the iris were
TREAT BLEPHARITIS: 85% of responsible organism s random ly assigned to em ergent AC and vitreous taps alone
(S. epidermidis and S. aureus) are found on lids; or with vitrectom y and with injection of intravitreal
hot com presses, lid hygiene, Polysporin antibiotics (0.4 m g am ikacin and 1.0 m g vancom ycin).
ointm ent  1 week preop; add doxycycline 100 m g Patients were also given subconjunctival injections of
bid  1 week for acne rosacea or seborrheic antibiotics (25 m g vancom ycin, 100 m g ceftazidim e, and
derm atitis steroid [6 m g dexam ethasone phosphate]), topical fortified
STERILIZE OPERATIVE FIELD: 5% povidine iodine antibiotics (50 m g/m L vancom ycin and 20 m g/m L
(Betadine); preoperative topical antibiotics am ikacin) and steroid (prednisolone acetate), and oral
(fluoroquinolone) steroid (prednisone 30 m g bid  5–10 days). Patients were
AVOID INOCULATION: drape eyelashes also random ly assigned to receive system ic IV antibiotics
ANTIBIOTICS: intracam eral (injection or in irrigating (2 g ceftazidim e IV q8h and 7.5 m g/kg am ikacin IV
solution); topical preop for 1–3 days and postop followed by 6 m g/kg q12h) or no system ic antibiotics.
for 7–10 days; consider subconjunctival antibiotics Intravitreal steroids were not used
with broken posterior capsule or vitreous loss
Delayed postoperative: Propionibacterium acnes (anaerobic Re s u lt s :
gram + rod) or fungal (Aspergillus, Candida)
420 patients enrolled
Trauma: Incidence of 2%–7% after penetrating traum a
RISK FACTORS: retained intraocular FB, delayed surgery On average, signs and sym ptom s occurred 6 days after
(> 24 hours), rural setting (soil contam ination), surgery (75% presented within 2 weeks of surgery)
and disruption of the crystalline lens

252
 Panuveitis

69% had positive cultures; 94% were Gram -positive Bo x 8-1. Systemic findings in sarcoidosis
bacteria (70% coagulase-negative Staphylococcus, 10% S. 1. Lung (most commonly involved organ): hilar adenopathy (most
aureus, 9% Streptococci species) common), diffuse fibrosis
2. Skin: erythema nodosum, skin granulomas (subcutaneous nodules)
IV antibiotics were of no benefit
3. Bones: arthralgias
Im m ediate vitrectom y had significant benefits only when 4. Hepatosplenomegaly
patients presented with light perception vision or worse 5. Peripheral neuropathy
6. Diabetes insipidus
Co n c lu s io n s :
7. Hypercalcemia (vitamin D metabolism abnormality)
For endopthalm itis after cataract or secondary IOL surgery, 8. Elevated serum gamma globulin (abnormality in immunoregulation)
perform em ergent treatm ent with AC tap and injection of 9. Parotid gland infiltration (may cause facial nerve palsy from
intravitreal antibiotics when vision is better than LP compression)

Vitrectom y should be reserved for patients presenting with

light perception vision or worse
IV antibiotics do not im prove the outcom e

P ANUVEITIS

S a rc o id o s is
Multisystem granulom atous disease characterized by
noncaseating granulom as; unknown etiology
Fem ales > m ales; m ore com m on am ong African Am ericans
(10 :1)
25%–50% have system ic sarcoidosis
Ocular disease: 30% unilateral, 70% bilateral; 40% acute, 60%
chronic Fig ure 8-23. Sarcoidosis demonstrating conjunctival granuloma with giant cells
surrounded by lymphocytes and plasma cells. (From Yanoff M, Fine BS: Ocular
Fin d in gs (25%–30%): uveitis in 60% of patients with pathology, ed 5, St Louis, 2002.)
ocular involvem ent; 66% of uveitis is anterior (2 form s:
acute granulom atous [responds well to corticosteroids]
and chronic recurrent [difficult to control with
corticosteroids]), KP, iris nodules, lacrim al gland infiltration
(25%; painless bilateral enlargem ent), conjunctival follicles,
keratoconjunctivitis sicca, episcleritis and scleritis with
nodules, choroiditis with yellow or white nodules, retinal
periphlebitis with candlewax drippings (granulom as
along retinal venules), pars planitis, vitritis, retinal
neovascularization, optic nerve granulom a; secondary
cataracts, glaucom a, and band keratopathy

Oth er fin d in gs: pulm onary (50%), constitutional (40%;

m alaise, fever, weight loss), skin (15%), lym phadenopathy
(20%) (Box 8-1)

Path o lo gy: noncaseating granulom as (caseating

granulom as occur in TB) with Langhans’ m ultinucleated giant
cells (Fig. 8-23)

Diagn o sis:
Lab tests: angiotensin-converting enzym e (ACE; elevated in
any diffuse granulom atous disease affecting the lung),
serum lysozym e (m ore sensitive than ACE, but less Fig ure 8-24. Bilateral hilar adenopathy in a patient without pulmonary
specific) symptoms. (From Cowan CL: Sarcoidosis. In Yanoff M, Duker J S [eds]:
Chest X-ray or chest CT: hilar adenopathy (Fig. 8-24) Ophthalmology, London, 1999, Mosby.)

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CHAPTER 8 • Uveitis

Gallium scan: look for parotid, lacrim al, or pulm onary

involvem ent
Biopsy: skin lesion, conjunctival nodule, lym ph node,
salivary or lacrim al gland, lung; if elevated ACE and
positive CXR, 60%–70% of blind conjunctival biopsies
will be positive

Tr eatm en t: topical, periocular, and system ic steroids,

im m unosuppressive therapy

Lo fg re n ’s s yn d ro m e
Hilar lym phadenopathy, erythem a nodosum , anterior uveitis,
arthralgia

Miku lic z’s s yn d ro m e

Lacrim al and parotid gland swelling, sicca syndrom e
Fig ure 8-25. Fundus view of a patient who has Behc¸et’s disease. (From Yanoff
Etio lo gy: sarcoidosis, TB, lym phom a/leukem ia M, Fine BS: Ocular Pathology, ed 5, St Louis, Mosby, 2002.)

He e rfo rd t ’s s yn d ro m e
Bo x 8-2. Criteria for Behc¸et’s disease
Fever, parotid gland enlargem ent, anterior uveitis, facial
nerve palsy
Ma jo r c rite ria

Be h c¸e t ’s d is e a s e 1. Oral aphthous ulcers (round, discrete borders; heal without scarring)
2. Genital ulcers
Chronic recurrent m ultisystem condition characterized by
3. Skin lesions (erythema nodosum)
relapsing inflam m ation and occlusive vasculitis
4. Ocular disease (75%): nongranulomatous iridocyclitis with sterile
Triad of oral ulcers, genital ulcers, and inflammatory eye disease hypopyon, necrotizing retinal vasculitis (may cause vascular occlusion),
posterior synechiae, glaucoma (pupillary block, uveitic), cataract, traction
Associated with HLA-B51/B5; m ales > fem ales, usually young retinal detachment (TRD)
adults; m ore com m on in Japan and Mediterranean countries
Mino r c rite ria

Fin d in gs (75%): recurrent, explosive inflam m atory episodes 1. Arthritis (50%)

with active episodes lasting 2–4 weeks 2. GI lesions
3. Occlusive vascular lesions of major vessels (vena cava)
Uveitis (posterior m ore com m on than anterior); can present
4. Migratory thrombophlebitis (33%)
with nongranulom atous anterior uveitis (usually bilateral; m ay
5. CNS involvement (25%; neuro-Behc¸et’s; meningoencephalitis, involve-
have transient hypopyon); occasionally, conjunctivitis,
ment of brain stem, spinal cord, peripheral nerves)
episcleritis, or keratitis can occur; posterior involvem ent with
6. Pulmonary artery aneurysm (pathognomonic CXR finding)
recurrent vascular occlusions, retinal hem orrhages, exudates,
7. Interstitial lung changes
CME, vitritis, traction RD, ischem ic optic neuropathy; m ay
develop glaucom a and cataract (Fig. 8-25)
Tr eatm en t: system ic steroids, cytotoxic agents
Oth er fin d in gs: oral (aphthous) ulcers, genital ulcers, skin (chloram bucil m ost effective for retinal vasculitis,
lesions (erythem a nodosum , acne-like lesions, folliculitis), m eningoencephalitis), colchicine (prevents recurrences),
arthritis (50%; especially wrists and ankles), throm bophlebitis, cyclosporine (ciclosporin); plasm apheresis
large vessel occlusion; GI pain, diarrhea, constipation; CNS
involvem ent (25%; m eningoencephalitis, strokes, palsies,
confusional states) Vo g t -Ko ya n a g i-Ha ra d a s yn d ro m e (VKH)
Uveoencephalitis: bilateral diffuse granulom atous panuveitis,
Path o lo gy: obliterative vasculitis with activation of both serous RDs, disc edem a, m eningeal irritation, skin pigm entary
cellular and hum oral lim bs of im m une system ; circulating changes, and auditory disturbance
im m une com plexes in > 50%

Diagn o sis: Har ad a’s d isease: if only eye findings

Clinical: (Box 8-2) Presum ed autoim m une process against m elanocytes
Behcetine skin test (cutaneous hypersensitivity):
Typically occurs in Asians, Am erican Indians, and Hispanics
intraderm al puncture; positive test ¼ pustule form ation
between ages 30 and 50 years; fem ale > m ale
within m inutes
HLA-B51/B5 Associated with HLA-DR4 and HLA-Dw53

254
 Panuveitis

Sym p to m s: decreased vision, pain, redness, photophobia, Pr o gn o sis: 60% retain vision > 20/30
stiff neck, headache, deafness, tinnitus, vertigo
Co m p licatio n s: cataracts (25%), glaucom a (33%), CNV
Fin d in gs: bilateral diffuse granulom atous panuveitis, serous (10%)
RDs, vitritis, exudative choroiditis, CB detachm ent, CME, ON
hyperem ia and edem a, poliosis; later develop perilim bal S ym p a t h e t ic o p h t h a lm ia
vitiligo (Sugiura’s sign), Dalen-Fuchs nodules (yellow-white
Bilateral granulom atous panuveitis following penetrating eye
retinal spots), sunset fundus (RPE disturbance with focal areas
traum a
of atrophy and hyperpigm entation [healing phase])
Due to immune sensitization to melanin or melanin-associated
Oth er fin d in gs: tem porary deafness, tinnitis, vertigo, proteins in uveal tissues;T-cell mediated (delayed hypersensitivity
m eningeal irritation, skin changes (30%; alopecia, vitiligo, reaction); latency of 10 days to 50 years after injury
poliosis) Incidence: 0.1–0.3% of penetrating injuries; 0.015% of
intraocular surgery
Clin ical co u r se: Associated with HLA-A11 and HLA-DR4
Prodrome: headache, m eningism us, seizures, bilateral
decreased vision with pain, redness, photophobia
Fin d in gs: Koeppe nodules, m utton fat KP, retinal edem a,
Syndrome: uveitis with serous RDs
Dalen-Fuchs nodules; m ay have disc edem a (Fig. 8-27)
Chronic stage: sunset fundus, Dalen-Fuchs nodules,
perilim bal vitiligo Path o lo gy: diffuse lym phocytic infiltration of choroid with
Recurrent stage: AC reaction, pigm ent changes; 60% retain ill-defined patchy accum ulations of epithelioid (giant) cells
vision > 20/30 that contain phagocytosed uveal pigm ent; inflam m ation can
extend into optic nerve, causing granulom atous optic neuritis;
Path o lo gy: inflam m ation of choriocapillaris and retina; Dalen-Fuchs nodules (epithelioid giant cells between Bruch’s
Dalen-Fuchs nodules (epithelioid cells between Bruch’s m em brane and RPE that appear as sm all, round, yellow
m em brane and RPE) (Fig. 8-26) depigm ented spots in peripheral retina [also seen in
VKH, sarcoidosis, TB]); no involvem ent of choriocapillaris
(Figs. 8-28, 8-29)
DDx: sym pathetic ophthalm ia, posterior scleritis, syphilis,
lupus choroiditis, hypotony, uveal effusion syndrom e
FA: m ultiple hyperfluorescent sites of leakage
Diagn o sis: Tr eatm en t: steroids, im m unosuppressive agents; consider
LP: CSF pleocytosis enucleation of injured eye within 10–14 days if NLP vision, but
FA: m ultiple focal areas of subretinal leakage (“1000 points once inflam m ation has started in fellow eye, rem oval of
of light”) inciting eye is controversial
OCT: grossly thickened choroid

Tr eatm en t: steroids (6 m onths), cycloplegic,

im m unosuppressive agents

Da le n-Fuchs nodule s
Fig ure 8-26. VKH demonstrating granulomatous inflammation in choroid Fig ure 8-27. Dalen-Fuchs nodules in a patient with sympathetic ophthalmia.
extending into choriocapillaris and through RPE. (From Yanoff M, Fine BS: (From Kaiser PK, Friedman NJ , Pineda II, R: Massachusetts eye and ear infirmary
Ocular Pathology, ed 5, St Louis, Mosby, 2002.) illustrated manual of ophthalmology, ed 2, Philadelphia, 2004, WB Saunders.)

255
CHAPTER 8 • Uveitis

Fig ure 8-30. Extensive chorioretinal damage with hyperplasia of retinal pigment
epithelium due to syphilis. (From Dugel PU: Syphilitic uveitis. In Yanoff M, Duker
J S [eds]: Ophthalmology, London, 1999, Mosby.)

Other findings:
SECONDARY: chancre, rash, lym phadenopathy,
condylom a lata
Fig ure 8-28. SO demonstrating thickened choroid with epithelioid cells and
lymphocytes; the choriocapillaris is spared. (From Yanoff M, Fine BS: Ocular TERTIARY: CNS, aortic aneurysm , gum m as
Pathology, ed 5, St Louis, Mosby, 2002.) Diagnosis: serology (VDRL or RPR, and FTA-ABS); m ust
rule out neurosyphilis with LP
FALSE-POSITIVE VDRL: rheum atoid arthritis,
anticardiolipin antibody, SLE, leprosy, hepatitis,
m ononucleosis, HIV, Lym e disease, rheum atic fever,
m alaria, pregnancy, and certain drugs
Treatment: as for neurosyphilis
Penicillin G 12–24 m illion units/day IVÂ 2 weeks,
followed by penicillin G 2.4 m illion units/week
IM Â 3 weeks
In penicillin-allergic patients: doxycycline, erythrom ycin

Co n gen ital:
Findings: interstitial keratitis (new vessels m eet in center of
cornea [salm on patch]); then atrophy (ghost vessels),
anterior uveitis, ectopia lentis, Argyll-Robertson pupil,
optic atrophy, chorioretinitis (salt and pepper fundus)
Other findings: death (in utero or perinatal), inflam m ation
Fig ure 8-29. Higher magnification of Fig. 8-29 shows a Dalen-Fuchs nodule
of internal organs, dental abnorm alities (Hutchinson’s
between RPE and Bruch’s membrane. (From Yanoff M, Fine BS: Ocular teeth), facial deform ities (saddle nose), saber shins
Pathology, ed 5, St Louis, Mosby, 2002.)

Tu b e rc u lo s is
Due to infection with Mycobacterium tuberculosis
Pr o gn o sis: m any patients retain very good vision

S yp h ilis Fin d in gs: lupus vulgaris on eyelids, phlyctenule, prim ary

conjunctival TB, interstitial keratitis, scleritis, lacrim al gland
Panuveitis (“great m im ic”) due to infection with spirochete
involvem ent, orbital periostitis, granulom atous panuveitis,
Treponema pallidum
secondary glaucom a and cataract, chorioretinal plaque or
nodule (tuberculom a), exudative RD, cranial nerve palsies
Acqu ir ed : (often due to basal m eningitis)
Findings (secondary and tertiary): panuveitis, iris papules
and gum m ata (yellow-red nodules), chorioretinitis (salt
and pepper changes), optic neuritis, optic atrophy, Tr eatm en t: isoniazid, rifam pin, etham butol, pyrazinam ide;
Argyll-Robertson pupil, ectopia lentis, interstitial system ic steroids m ay cause a flare-up; etham butol and
keratitis (Fig. 8-30) isoniazid can cause a toxic optic neuropathy

256
 Differential Diagnosis of Uveitis and Associated Signs

Diagn o sis: cytology (aqueous and vitreous)

MASQUERADE SYNDROMES
Tr eatm en t: radiation therapy (ocular and CNS),
Conditions that present as uveitis: peripheral RD (Schwartz- intrathecal chem otherapy
Matsuo syndrom e), intraocular foreign body, JXG, m ultiple
sclerosis, m alignancies (retinoblastom a, acute lym phoblastic
Ma lig n a n t m e la n o m a
leukem ia [ALL], large cell lym phom a, m alignant
m elanom a), RP Necrotic tum or m ay seed tum or cells into the vitreous and
anterior segm ent, causing an inflam m atory response
In t ra o c u la r fo r e ig n b o d y
Fin d in gs: AC reaction, iris heterochrom ia, vitreous
Fin d in gs: AC reaction; m ay have signs of ocular traum a, hem orrhage; m ay have brown pseudohypopyon (m elanin-
visible FB, iris heterochrom ia
laden m acrophages) and m elanom alytic glaucom a

Diagn o sis: X-ray, B-scan ultrasound Diagn o sis: FA, B-scan ultrasound

J u ve n ile xa n t h o g ra n u lo m a Re t in it is p ig m e n t o s a
Fin d in gs: sm all fleshy iris tum ors, AC reaction, spontaneous Fin d in gs: vitreous cells, pigm entary retinopathy with
hyphem a vascular attenuation and optic nerve pallor

Diagn o sis: skin lesions, iris biopsy Diagn o sis: ERG, electro-oculography (EOG), visual fields

Mu lt ip le s c le r o s is
Fin d in gs: periphlebitis, pars planitis, optic neuritis
DIFFERENTIAL DIAGNOSIS OF UVEITIS
AND ASSOCIATED SIGNS
Diagn o sis: neurologic exam ination, head MRI
Ban d ker ato p ath y: JRA, sarcoidosis, m ultiple sclerosis

Re t in o b la s t o m a Hyp h em a: Fuchs’ heterochrom ic iridocyclitis, traum a,

Fin d in gs: pseudohypopyon, vitreous cells JXG, HZV

Diagn o sis: Vitr eo u s h em o r r h age: VKH, POHS

AC tap: lactate dehydrogenase (LDH) levels, cytology
B-scan ultrasound: calcifications Ir is n o d u les: TB, syphilis, sarcoidosis, leprosy, sym pathetic
ophthalm ia, VKH, m ultiple sclerosis, Fuchs’ heterochrom ic
iridocyclitis
Le u ke m ia
Retina is m ost com m on ocular tissue affected clinically Ir is atr o p h y: herpetic (diffuse or sectoral), Fuchs’
heterochrom ic iridocyclitis (diffuse), traum atic (focal, usually
Choroid is m ost com m on ocular tissue affected
surgical)
histopathologically

Bell’s p alsy (bilateral): Lym e disease, sarcoidosis

Fin d in gs: AC reaction, iris heterochrom ia, Roth spots,
retinal hem orrhages, cotton wool spots, peripheral NV, serous
RDs, vascular dilation and tortuosity, optic nerve infiltration Gen ito u r in ar y in vo lvem en t: Reiter’s syndrom e,
gonococcal disease, Behçet’s disease

Diagn o sis: bone m arrow, peripheral blood sm ear, aqueous Jau n d ice: leptospirosis, inflam m atory bowel disease (IBD),
cytology CMV, schistosom iasis
FA with serous retinal detachment: m ultiple areas of
hyperfluorescence (sim ilar to VKH)
Liver en lar gem en t: toxocariasis, toxoplasm osis, CMV

P rim a r y in t ra o c u la r lym p h o m a CNS in vo lvem en t: TB, VKH, congenital toxoplasm osis,

(re t ic u lu m c e ll s a rc o m a ) congenital CMV, Behçet’s disease, large cell lym phom a
Fin d in gs: bilateral vitreous cells, retinal hem orrhage and
exudates, retinal and choroidal infiltrates often; CNS Skin r ash : secondary syphilis, sarcoidosis, Behçet’s disease,
involvem ent psoriasis, Reiter’s syndrom e, VKH, POHS

257
CHAPTER 8 • Uveitis

Er yth em a n o d o su m : sarcoidosis, TB, IBD, POHS, Behçet’s 5. The m ost com m on organism causing endopthalm itis
disease, APMPPE following cataract surgery is
a. S. pneumoniae
Or al u lcer s: Behçet’s disease, Reiter’s syndrom e, HSV, IBD, b. H. influenzae
OCP, Stevens-Johnson syndrom e (SJS), SLE c. S. aureus
d. S. epidermidis
6. MEWDS can be differentiated from APMPPE by
Gen ital u lcer s: syphilis, Reiter’s syndrom e, Behçet’s a. age of onset
disease, OCP
b. fem ale predilection
c. paracentral scotom as
Pu lm o n ar y in vo lvem en t: TB, sarcoidosis, Churg-Strauss d. viral prodrom e
syndrom e, Toxocara, aspergillosis, coccidioidom ycosis, POHS, 7. All of the following disorders are correctly paired with
Wegener’s granulom atosis their HLA associations except
a. POHS, B9
b. Behçet’s disease, B51
c. Birdshot retinochoroidopathy, A29
SURGERY AND UVEITIS d. Reiter’s syndrom e, B27
8. Decreased vision in a patient with interm ediate uveitis is
Usually wait at least 3 m onths for cataract surgery, 6 m onths for m ost likely due to
corneal transplant a. cataract
b. m acular edem a
c. papillitis
JRA: no IOL; can develop cyclitic m em branes and CB
d. glaucom a
detachm ents; consider lensectom y with partial vitrectom y
9. A 71-year-old wom an with a 6-m onth history of
fatigue, anorexia, and 10-pound (4 kg) weight loss is
Par s p lan itis: increased risk of com plications, higher risk for found to have left-sided weakness, visual acuity of 20/80
CME; vitreous opacities m ay lim it vision, therefore, consider OD and 20/60 OS, and vitreous cells. The m ost helpful
lensectom y with pars plana vitrectom y workup is
a. LP and vitrectom y
Fu ch s’ h eter o ch r o m ic ir id o cyclitis: cataract surgery is b. ESR and tem poral artery biopsy
safe; increased risk of transient postoperative hyphem a c. CBC and lym ph node biopsy
d. PPD and chest X-ray
10. The m ost com m on organism s causing endophthalm itis
following traum a are
REVIEW QUESTIONS (Answers start on page 385) a. Enterococcus species and S. aureus
b. Bacillus species and S. epidermidis
1. The m ost effective antibiotic for the treatm ent of P. acnes c. Pseudomonas species and S. aureus
endophthalm itis is d. S. aureus and S. epidermidis
a. am ikacin 11. All of the following are features com m on to both
b. gentam icin sym pathetic ophthalm ia and Vogt-Koyanagi-Harada
c. vancom ycin syndrom e except
d. ceftazidim e a. serous retinal detachm ents
2. For the diagnosis of granulom atous inflam m ation, b. Dalen-Fuchs nodules
which cell type m ust be present? c. pathology localized to choroid
a. Langhans’ cell d. vitritis
b. lym phocyte 12. Which disorder is m ore com m on in m ales?
c. Touton giant cell a. MEWDS
d. epithelioid histiocyte b. uveal effusion syndrom e
3. All of the following are true concerning sarcoidosis c. APMPPE
except d. Birdshot choroidopathy
a. Touton giant cells are com m on 13. EVS findings include all of the following except
b. lym phocytes surround the granulom a a. vitrectom y was beneficial only in patients with LP
c. histiocytes are abundant vision
d. necrosis is rare b. intravitreal corticosteroids were helpful
4. Which of the following is not characteristic of Fuchs’ c. IV antibiotics were not helpful
heterochrom ic iridocyclitis? d. the m ost com m on organism was
a. iris neovascularization S. epidermidis
b. cataract 14. Which of the following is not characteristic of MEWDS?
c. posterior synechiae a. enlargem ent of the blind spot
d. vitreous opacities b. bilaterality

258
 Review Questions

c. flu-like illness 25. Features of Harada’s disease include all of the following
d. fem ale preponderance except
15. The m ost com m on cause of posterior uveitis is a. vitritis
a. sarcoidosis b. deafness
b. syphilis c. serous retinal detachm ents
c. CMV d. Dalen-Fuchs nodules
d. toxoplasm osis 26. Larva cause all of the following infections except
16. All of the following are causes of HLA-B27-associated a. cysticercosis
uveitis except b. diffuse unilateral subacute neuroretinitis
a. ankylosing spondylitis c. onchocerciasis
b. ulcerative colitis d. cat-scratch disease
c. Crohn’s disease 27. Which of the following signs of pars planitis is m ost
d. psoriasis associated with m ultiple sclerosis?
17. Which of the following is not part of the classic triad of a. subretinal neovascularization
findings in Reiter’s syndrom e? b. snowbank
a. iritis c. periphlebitis
b. arthritis d. CME
c. conjunctivitis 28. CSF abnorm alities are associated with all of the following
d. urethritis disorders except
18. Phacoantigenic endophthalm itis is characterized by which a. VKH syndrom e
pattern of granulom atous inflam m ation? b. ocular sarcoidosis
a. zonal c. APMPPE
b. diffuse d. pars planitis
c. discrete 29. All of the following can present as uveitis except
d. necrotizing a. retinoblastom a
19. A 35-year-old m an with decreased vision OD is found to b. choroidal hem angiom a
have optic nerve edem a and a m acular star. The causative c. leukem ia
organism m ost likely is d. juvenile xanthogranulom a
a. Onchocerca volvulus 30. Which of the following is not associated with
b. Bartonella henselae inflam m atory bowel disease?
c. Treponema pallidum a. conjunctivitis
d. Borrelia burgdorferi b. episcleritis
20. A person living in which area of the US would be m ost c. interstitial keratitis
likely to develop POHS? d. iritis
a. southwest 31. Anterior vitreous cells are least likely to be found in
b. northwest a. retinitis pigm entosa
c. m idwest b. CMV
d. southeast c. serpiginous choroidopathy
21. All of the following are true of birdshot choroidopathy d. chronic cyclitis
except 32. Gastrointestinal disorders associated with uveitis include
a. m ore com m on in m ales all of the following except
b. usually bilateral a. ulcerative colitis
c. CME is com m on b. Whipple’s disease
d. associated with HLA-A29 c. diverticulitis
22. Which of the following is least com m only associated with d. Crohn’s disease
Treponema pallidum infection? 33. All of the following m ay occur in ocular sarcoidosis
a. interstitial keratitis except
b. chorioretinitis a. optic disc nodules
c. ectopia lentis b. pars planitis
d. glaucom a c. CN palsies
23. The HLA association for pars planitis with m ultiple d. low serum gam m a globulin
sclerosis is 34. The choroid is the prim ary location of the pathologic
a. B8 process in
b. B51 a. toxoplasm osis
c. DR4 b. CMV
d. DR15 c. Coat’s disease
24. Retinal S antigen is found in d. VKH syndrom e
a. ganglion cells 35. Which of the following is least likely to be found in a
b. retinal pigm ent epithelium patient with sym pathetic ophthalm ia?
c. photoreceptors a. onset after a latent period of 40 years
d. Mueller cells b. granulom atous nodules in the retina

259
CHAPTER 8 • Uveitis

c. history of evisceration of the traum atized eye b. do not delay cataracst surgery and treat with oral
d. iris nodules in the sym pathizing eye cyclosporine
36. Band keratopathy is least likely to occur in a patient with c. delay cataract surgery for 6 weeks and treat with oral
a. sarcoidosis cyclosporine
b. JRA d. delay cataract surgery until the eye has been quiet for at
c. Behçet’s disease least 3 m onths
d. m ultiple sclerosis 43. All of the following are risk factors for traum atic
37. A patient with APMPPE is m ost likely to have endophthalm itis except
a. unilateral involvem ent a. delayed surgery
b. enlarged blind spot b. disruption of the lens
c. viral prodrom e c. retained intraocular foreign body
d. CNV d. double penetrating injury
38. A patient with a m ild anterior cham ber reaction, increased 44. A pseudophakic patient develops granulom atous
intraocular pressure, and iris heterochrom ia is m ost likely inflam m ation 8 m onths after cataract surgery, and a white
to also exhibit which other finding? plaque is present on the posterior capsule. The organism
a. anisocoria m ost likely to be causing this condition is
b. ptosis a. A. fum igatus
c. posterior synechiae b. C. albicans
d. fine vessels in the angle c. P. acnes
39. Which of the following cell types is found in both d. B. cereus
granulom atous and nongranulom atous keratic 45. A 28-year-old wom an acutely develops reduced vision,
precipitates? pain, redness, and floaters OS. Exam shows a m ild iritis
a. epithelioid cells with granulom atous KP and discrete patches of peripheral
b. giant cells necrotizing retinitis. The m ost appropriate m anagem ent is
c. lym phocytes to begin treatm ent with system ic
d. m acrophages a. acyclovir
40. All of the following are m asquerade syndrom es except b. foscarnet
a. vitreous hem orrhage c. ranibizum ab
b. retinal detachm ent d. vancom ycin
c. intraocular foreign body
d. m ultiple sclerosis
41. A false-positive VDRL test is least likely to occur in a SUGGESTED READINGS
patient with
a. Wegener’s granulom atosis
Basic and Clinical Sciences Course. (2012). Section 9: Intraocular
b. lupus
inflammation and uveitis (Vol. 9). San Francisco: AAO.
c. rheum atoid arthritis Foster, C. S., & Vitale, A. T. (2013). Diagnosis and Treatment of Uveitis
d. anticardiolipin antibody (2nd ed.). New Delhi: Jaypee Brothers Medical Publishers.
42. A 54-year-old m an with chronic recurrent uveitis OS Jones N.P. (2012) Uveitis (2nd ed.). JP Medical Ltd, 2012.
controlled with topical steroids has developed a visually Michelson, J. B. (1992). Color Atlas of Uveitis (2nd ed.). St Louis: Mosby.
significant cataract. What is the m ost appropriate Nussenblatt, R. B., & Whitcup, S. M. (2010). Uveitis: Fundamentals and
treatm ent? Clinical Practice (4th ed.). Philadelphia: Mosby.
a. do not delay cataract surgery and treat with oral
steroids

260
9 Glaucom a
ANATOMY/PHYSIOLOGY
TESTING
PATHOLOGY
DISO RDERS
TREATMENT

ANATOMY/P HYSIOLOGY adenylcyclase to inhibit the Na +/K+ pum p; glucose enters

by passive diffusion.
Rate ¼ 2–3 µL/min, AC volume ¼ 200 µL, posterior
Cilia r y b o d y (CB) chamber volume ¼ 60 µL (AC volume turnover is
6-m m -wide structure located between the scleral spur approximately 1%/min). Rate measured by
anteriorly and the ora serrata posteriorly; com posed of the pars fluorophotometry (direct optical measurement of
plicata (anterior 2 m m with ciliary processes) and the pars decreasing fluorescein concentration) or tonography
plana (posterior 4 m m , flat) (indirect calculation from outflow measurement).
Pars plicata consists of Diurnal production of aqueous (may be related to
Ciliary muscle: longitudinal fibers (insert into scleral spur cortisol levels) decreases with sleep (45%), age
and affect outflow facility), circular fibers (anterior inner (2%/decade; counterbalanced by the decreased outflow
fibers oriented parallel to lim bus and affect with age), inflammation, surgery, trauma, and drugs
accom m odation), and radial fibers (connect AQUEOUS COMPOSITION: slightly hypertonic and acidic
longitudinal and circular fibers) (pH 7.2) com pared with plasm a; 15 Â m ore
Ciliary vessels: m ajor arterial circle of the iris (in CB near iris ascorbate than plasm a; lower protein (0.02% vs 7%
root) form ed by anastom osis of branches of anterior and in plasm a); lower calcium and phosphorus (50% of
long posterior ciliary arteries the level in plasm a); chloride and bicarbonate vary
Ciliary processes: 70 finger-like projections com posed of (from 25% above or below plasm a levels); sodium ,
pigm ented and nonpigm ented epithelial cell bilayer, potassium , m agnesium , iron, zinc, and copper levels
capillaries, and strom a sim ilar to those in plasma
FUNCTIONS OF AQUEOUS: m aintains intraocular
pressure, provides m etabolic substrates (glucose,
Fu n ctio n s: oxygen, electrolytes) to the cornea and lens, and
Suspends and alters shape of lens: zonular fibers that rem oves m etabolic waste (lactate, pyruvate, carbon
originate between the ciliary processes of the pars plicata, dioxide)
attach to the crystalline lens, and suspend it. Helm holtz’s Affects aqueous outflow: contraction of ciliary m uscle
theory of accom m odation explains the changes in lens longitudinal fibers causes traction on the trabecular
shape and thus refractive power with contraction and m eshwork, increasing outflow
relaxation of the ciliary m uscle. Contraction of the Synthesizes acid mucopolysaccharide component of
longitudinal fibers pulls the lens forward, shallowing the vitreous: occurs in nonpigm ented epithelial cells of pars
anterior cham ber. Contraction of the circular fibers plana and enters vitreous body at its base
relaxes the zonules m aking the lens m ore spherical with Maintains blood–aqueous barrier: within the ciliary
greater focusing power. Relaxation of circular fibers (or processes, plasm a enters from thin fenestrated
cycloplegia) tightens the zonules, stretching the lens and endothelium of capillary core ! passes through
m aking it thinner with less focusing power. strom a ! 2 layers of epithelium with apposing apical
Produces aqueous humor: production of aqueous is surfaces (which form s the ciliary epithelial bilayer):
prim arily by active secretion (also diffusion and OUTER PIGMENTED LAYER: continuous with the RPE
ultrafiltration); both an Na+/K+ pum p and carbonic (basal lam ina continuous with Bruch’s m em brane),
anhydrase are involved. β-blockers act through contains zonula occludens

261
CHAPTER 9 • Glaucom a

INNER NONPIGMENTED LAYER: equivalent to the Canal of Schlem m is lined by a single layer of endothelial cells
sensory retina (basal lam ina continuous with ILM), (m esothelial cells) and connects to the venous system by 30
site of active secretion collector channels
Both layers have basal basem ent mem branes with their
apices facing each other. During passage from the
bloodstream to the posterior cham ber, a m olecule Uveo scler al o u tflo w (15–20% of total outflow): pressure
m ust pass through capillary basem ent m em brane, independent; aqueous passes through face of ciliary body in
pigm ented epithelium basem ent m em brane, pigm ented the angle, enters the ciliary m uscle and suprachoroidal space,
epithelium , nonpigm ented epithelium , nonpigm ented and is drained by veins in the ciliary body, choroid, and sclera
epithelium basem ent m em brane Cyclodialysis cleft increases aqueous outflow through the
Cryodestruction and inflam m ation cause loss of barrier uveoscleral pathway (without reducing aqueous production);
function (tight junctions open), resulting in flare cycloplegics and prostaglandin analogues increase uveoscleral
(proteins in aqueous); atropine reduces flare by closing outflow; m iotics decrease uveoscleral outflow and increase
tight junctions trabecular m eshwork outflow

Ou t flo w p a t h w a ys
An g le s t r u c t u re s
Tr ab ecu lar m esh wo r k (traditional pathway): pressure
dependent;represents major aqueous drainage system;uveoscleral Visible only by gonioscopy because of total internal reflection
meshwork ! corneoscleral meshwork ! juxtacanalicular at the air/cornea interface (Fig. 9-1)
connective tissue ! Schlemm’s canal ! collector
channels ! aqueous veins! episcleral and conjunctival Sch walb e’s lin e: peripheral/posterior term ination of
veins ! anterior ciliary and superior ophthalmic Descem et’s m em brane, which corresponds to apex of corneal
veins ! cavernous sinus light wedge (optical cross section of the cornea with narrow slit
The pore size of the m eshwork decreases towards Schlem m ’s beam reveals 2 linear reflections, 1 from external and 1 from
canal: internal corneal surfaces, which m eet at Schwalbe’s line)
Uveal meshwork: collagenous core surrounded by
endothelial cells; pore size up to 70 µm ; linked to ciliary
m uscle Tr ab ecu lar m esh wo r k (TM): anterior nonpigm ented
Corneoscleral meshwork: sheet-like beam s insert into scleral portion appears as a clear white band; posterior pigm ented
spur; pore size up to 30 µm portion has variable pigm entation (usually darkest
Juxtacanalicular tissue: links corneoscleral trabeculae with inferiorly). Increased pigm entation of trabecular m eshwork
Schlem m ’s endothelium ; pore size ¼ 4–7 µm ; site of occurs with pseudoexfoliation syndrom e (Sam paolesi’s
greatest aqueous outflow resistance line), pigm ent dispersion syndrom e, uveitis, m elanom a,

Gra de 4 pigme nt Gra de 2 pigme nt No pigme nt

S chwa lbe 's line

Tra be cula r me s hwork
S chle mm's ca na l
S chwa lbe 's line
S cle ra l s pur Tra be cula r
Cilia ry me s hwork
body (a nte rior
nonpigme nte d
a nd pos te rior
pigme nte d)
S cle ra l s pur
Cilia ry body

Longitudina l Circula r Cilia ry

mus cle mus cle proce s s e s
Fig ure 9-1. Composite drawing of the microscopic and gonioscopic anatomy. (From Becker B, Shaffer RN: Diagnosis and therapy of the glaucomas , St Louis,
1965, Mosby.)

262
 Testing

An gle r ecessio n : tear between longitudinal and circular

fibers of the ciliary m uscle. Because longitudinal fibers are still
attached to the scleral spur, m iotics still work, but because they
decrease uveoscleral outflow, IOP m ay actually increase. Breaks
in posterior TM result in scarring and a nonfunctional TM;
aqueous drains prim arily through uveoscleral outflow;
60–90% of patients with traum atic hyphem as have angle
recession; 5% of eyes with angle recession will develop
glaucom a
Gonioscopic findings: widened ciliary body band, increased
visibility of scleral spur, torn iris processes, sclera visible
through disrupted ciliary body tissue, m arked variation
in CB width in different quadrants of sam e eye

Cyclo d ialysis cleft: separation of ciliary body from scleral

Fig ure 9-2. Vascular supply and anatomy of the anterior optic nerve. (From spur; often from traum a. Results in direct com m unication
Hart WM J r: In Podos SM, Yanoff M [eds ]: Textbook of ophthalmology, vol 6, between AC and suprachoroidal space causing hypotony;
London, 1994, Mosby.) spontaneous closure m ay occur (unlikely after 6 weeks) with
m arked IOP rise; shortly thereafter, the TM should begin to
traum a, surgery, hyphem a, darkly pigm ented individuals, and function norm ally again
increasing age Gonioscopic findings: cleft at junction of scleral spur and
CB band
Sch lem m ’s can al: usually not visible or only faintly visible Treatment: cycloplegic to relax ciliary body in an attem pt to
as a light gray band at the level of posterior TM; elevated venous close cleft (avoid pilocarpine, which m ay open the cleft
pressure or pressure from the edge of the gonioscopy lens m ay through ciliary m uscle traction); laser (argon induces
cause blood to reflux, m aking Schlem m ’s canal visible as a faint inflam m ation to close the cleft; spot size ¼ 50–100 µm ,
red band duration ¼ 0.1–0.2 s; power ¼ 0.5–1.0 watts to uvea and
1–3 watts to sclera); cryotherapy; suture CB to sclera
DDx o f b lo o d in Sch lem m ’s can al: elevated episcleral (direct cyclopexy); intravitreal air bubble for a superior
venous pressure, oculoderm al m elanocytosis (nevus of Ota), cleft; YAG laser can be used to open a closed cleft
neurofibrom atosis, congenital ectropion uveae, hypotony,
secondary to gonioscopy Ir id o d ialysis: tear/disinsertion of iris root. If large or
sym ptom atic, consider surgical repair with m attress sutures
Scler al sp u r (SS): narrow white band that corresponds to
the site of insertion of longitudinal fibers of ciliary m uscle to
sclera Op t ic n e r ve (Fig. 9-2)
Approxim ately 1.2 m illion axons; cell bodies of ganglion cells
Ciliar y b o d y (CB): pigm ented band that represents the are located in the ganglion cell layer
anterior face of the ciliary body; iris processes m ay be seen as Four layers of optic nerve head based on blood supply:
lacy projections crossing this band but not the scleral spur Nerve fiber: supplied by branches of central retinal artery
(occur in 33% of population) Prelaminar: supplied by capillaries of the short posterior
ciliary arteries
An g le a b n o rm a lit ie s Laminar (lam ina cribrosa): supplied by dense plexus from
short posterior ciliary arteries
Per ip h er al an ter io r syn ech ia (PAS): any pigm ented Retrolaminar: supplied by both ciliary (via recurrent pial
structure that crosses scleral spur vessels) and retinal (via centripetal branches from pial
Etiology: angle-closure, uveitis, neovascularization, flat region) circulations
anterior cham ber, ICE syndrom e, ciliary body tum ors, Optic nerve blood flow is influenced by m ean blood pressure,
m esoderm al dysgenesis IOP, blood viscosity, blood vessel caliber, and blood vessel
length
No r m al vessels: radial iris vessels, portions of arterial circle
of CB, and rarely, vertical vessels deep in CB; do not branch or
cross the scleral spur; present in 7% of patients with blue irides
and 10% with brown TESTING
Ab n o r m al vessels: fine, often branch, no orientation, In t ra o c u la r p re s s u re
cross-scleral spur
DDx: neovascularization (rubeosis iridis), iris neoplasm , Go ld m an n equ atio n : IOP ¼ F=C + EVP relates 3 factors
Fuchs’ heterochrom ic iridocyclitis (sparse, faint, and im portant in determ ination of IOP
delicate; bleed easily on decom pression of AC) F ¼ rate of aqueous form ation ¼ 2 À 3 µL=m in

263
CHAPTER 9 • Glaucom a

C ¼ facility of outflow ¼ 0:28 µL=m in =m m Hg; < 0:20 is No n co n tact:

abnorm al; decreases with age, increases with Air puff tonometer: noncontact device; tim e required for air
m edication; m easured by tonography jet to flatten cornea is proportional to IOP; varies with
cardiac cycle
EVP ¼ episcleral venous pressure ¼ 8À12 m m Hg;
increases with venous obstruction or AÀ V shunt;
m easured by m anom etry
IOP ¼ 8À21 m m Hg is considered norm al; average ¼ 16 Go n io s c o p y
+ =À 2:5 m m Hg; distribution is not Gaussian and is skewed Classificatio n system s:
to higher IOPs Scheie:
Grade I ¼ wide open (CB visible)
IOP is influenced by age (m ay increase with age), genetics, Grade II ¼ SS visible; CB not visible
race (higher in African Am ericans), season (higher in winter, Grade III ¼ only anterior TM visible
lower in sum m er), blood pressure, obesity, exercise (lower Grade IV¼ closed angle (TM not visible)
after exercise), Valsalva, tim e of day (diurnal variation Schaffer: opposite of Scheie (Grade 0 is closed; Grade IV is
[2–6 m m /day]; peak in m orning), posture (higher when lying wide open) (Fig. 9-3)
down vs sitting up), various horm ones, and drugs. Also ocular Grade I ¼ 10% open
factors: refractive error (higher in m yopes) and eyelid closure Grade II ¼ 20% open
Grade III ¼ 30% open
To n o m e t ry Grade IV¼ 40% open
Spaeth: m ost descriptive; 4 elem ents
IOP m easurem ent can be perform ed with a variety of devices
FIRST ELEMENT: level of iris insertion (capital letter A-E)
(tonom eters)
A¼ Anterior to TM
B¼ Behind Schwalbe’s line or at TM
In d en tatio n :
C ¼ At scleral spur
Schiøtz tonometer: known weight indents cornea and
D ¼ Deep angle, CB visible
displaces a volum e of fluid within the eye; am ount of
E¼ Extrem ely deep, large CB band
indentation estim ates pressure; falsely low readings
Perform indention gonioscopy: if true insertion is m ore
occur with a very elastic eye (low scleral rigidity as with
posterior, place original im pression in parentheses
high m yopia, buphthalm os, retinal detachm ent,
followed by true insertion location
treatm ent with cholinesterase inhibitors, thyroid disease,
SECOND ELEMENT: num ber that denotes the
and previous ocular surgery) or a com pressible
iridocorneal angle width in degrees from 5 to 45
intraocular gas (e.g., SF6 and C3 F8 ); falsely high readings
THIRD ELEMENT: peripheral iris configuration (lower
occur with scleral rigidity and hyperopia
case letter r, s, or q)
r ¼ regular (flat)
Ap p lan atio n : based on the Im bert-Fick principle: P ¼ F/A s ¼ steep (convex)
(for an ideal thin-walled sphere, pressure inside sphere equals q ¼ queer (concave)
force necessary to flatten its surface divided by the area of FOURTH ELEMENT: pigm entation of posterior TM
flattening). The eye is not an ideal sphere: the cornea resists (graded from 0 [none] to 4 [m axim al])
flattening, and capillary action of the tear m eniscus pulls the Exa m p le : (A)B15 r, 1 + (appositionally clos ed 15° angle that opens to
tonom eter to the eye. However, these 2 forces cancel each other TM with indentation, regular iris configuration, and mildly pigmented
when the applanated diam eter is 3.06 m m posterior TM)
Goldmann tonometer: biprism attached to a spring;
fluorescein sem icircles align when area applanated has a
diam eter ¼ 3.06 m m ; error can occur with squeezing,
Valsalva, vertical gaze, irregular or edem atous cornea, gra de IV
corneal thickness (thick corneas overestim ate IOP gra de I
= 40 °
[$ 5 m m Hg per 70 µm ], and thin corneas underestim ate = 10 °
IOP [$ 5 m m Hg per 70 µm ]), am ount of fluorescein,
external pressure on or restriction of globe, and
astigm atism > 1.5 D (m ust align red m ark on tip with
axis of MINUS cylinder)
Perkins tonometer: portable form of Goldm ann device
Mackay-Marg tonometer: applanates sm all area; good for
corneal scars and edem a. Exam ples of this style
tonom eter include the tonopen and the
pneum otonom eter
TONOPEN: probe indents cornea, and m icroprocessor
calculates IOP and reliability Fig ure 9-3. Shaffer’s angle-grading system. (From Fran M, Smith J , Doyle W:
PNEUMOTONOMETER: central sensing device is Clinical examination of glaucoma. In Yanoff M, Duker J S [eds]: Ophthalmology,
controlled by air pressure ed 2, St Louis, 2004, Mosby.)

264
 Testing

Typ es o f len ses: Hu m p h r ey (static):

Koeppe lens: direct view Test distance ¼ 0.33 m ; background illum ination ¼ 31.5
Goldmann 3-mirror lens: requires coupling solution apostilbs (asb); stim ulus size ¼ III; stim ulus
(Goniosol) duration ¼ 0.2 s; various program s (i.e., central 30°, 24°,
Zeiss, Posner, and Sussman 4- or 6-mirror lenses: can perform 10°, neuro fields, ptosis fields, etc.)
indentation gonioscopy to determ ine whether angle Reliability indices:
closure is appositional or synechial FIXATION LOSS: patient responds when a target is
displayed in blind spot. There is also a gaze tracking
printout at the bottom of the page that shows the
Gr ad in g system o f sh allo w/flat an ter io r ch am b er :
deviation of fixation during each stim ulus
Grade I: Contact between cornea and peripheral iris
presentation
Grade II: Contact between cornea and iris up to pupil
FALSE-POSITIVE: patient responds when there is no
(consider reform ing AC with BSS or viscoelastic)
stim ulus (nervous or trigger-happy; causes
Grade III: Contact between cornea and crystalline lens
white areas)
(surgical em ergency)
FALSE-NEGATIVE: patient fails to respond to a
superthreshold stim ulus at a location that was
Vis u a l fie ld s previously responded to (indicates loss of attention or
fatigue; causes cloverleaf pattern)
Perim etry m easures the ”island of vision” or topographic
Global indices:
representation of differential light sensitivity. Peak ¼ fovea;
MEAN DEVIATION (MD): average departure of each test
depression ¼ blind spot; extent ¼ 60° nasally, 60° superiorly,
point from the age-adjusted norm al value. This
70–75° inferiorly, and 100–110° tem porally
represents the overall deviation (m ean elevation or
Central field tests points only within a 30° radius of fixation
depression) of the visual field from the norm al
reference field
Typ es: PATTERN STANDARD DEVIATION (PSD): standard
(Fig. 9-4) deviation of the differences between the threshold
Kinetic: uses a m oving stim ulus of constant intensity to and expected values for each test point. This
produce an isopter or points of equal sensitivity represents the change in shape of the field from the
(horizontal cross section of the hill of vision) expected shape for a norm al field
Static: uses a fixed stim ulus with constant or variable SHORT-TERM FLUCTUATION (SF): variability in
intensity to produce a profile (vertical cross section of the responses when the sam e 10 points are retested;
hill of vision) m easure of consistency
CORRECTED PATTERN STANDARD DEVIATION
(CPSD): PSD adjusted for patient reliability
Go ld m an n (kinetic and static):
(correcting for SF)
Test distance: 0.33 m
Patient vision 20/80 will cause a scotom a to appear larger
Test object size: I–V (each increm ent doubles the diam eter
and deeper
[quadruples the area] of test object; III4e test object will
Pupil < 3 m m will cause reduction in total deviation
have 2 Â the diam eter and 4 Â the area of II4e)
Light filters: 1–4 (increm ents of 5db), a–e (increm ents
of 1db)
Tan gen t scr een (usually kinetic): test distance is 1 m , test
object m ay vary in size and color; tests only central field.
Kine tic (is opte r) pe rime try Magnifies scotom a and is of low cost; however, poor
reproducibility and lack of standardization

VF d efect: a scotom a is an area of partial or com plete

blindness
Corresponds to a defect on Hum phrey testing that is at least
3° wide and 6 decibels (dB) deep; also 1 point that is
depressed > 10 dB or at least 2 points that are depressed
at least 5 dB
Typical localized glaucomatous scotomas: (Fig. 9-5)
PARACENTRAL: within central 10°
ARCUATE (Bjerrum ): isolated, nasal step of R€ o nne and
Seidel (connected to blind spot)
TEMPORAL WEDGE:
Glaucom atous scotom as do not respect the vertical
S ta tic (profile ) pe rime try m eridian (vs neurologic VF defects, which do)
Fig ure 9-4. Kinetic and static perimetry. (From Bajandas FJ , Kline LB: VF should correlate with optic nerve appearance; otherwise,
Neuro-ophthalmology review manual, ed 3,Thorofare, NJ , 2004, Slack.) consider refractive error, level of vision, m edia opacities,

265
CHAPTER 9 • Glaucom a

Na s a l s te p of Rönne pupil size, and other causes of VF defects (tilted ON

135
120 105 90 75 60 120 105 90 75 60
45
head, ON head drusen, retinal lesions, etc.)
150 30

Op t ic n e r ve h e a d (ONH) a n a lyze rs
165 15

180 0
Various digital and video cam eras that capture ONH im age;
Te mpora l com puter then calculates cup area in an attem pt to objectively
195 345
NFB quantify ONH appearance (Table 9-1)
de fe ct of
ra dia ting 210 330
na s a l fibe rs 225 315 Co n fo cal scan n in g laser o p h th alm o sco p y (CSLO;
240 255 270 285 300 240 255 270 285 300
Le ft Right heidelberg retinal tom ograph [HRT]; TopSS): low-power laser
Infe rior a ltitudina l
de fe ct ("fa t" Bje rrum
produces digital 3D picture of ON head by integrating coronal
Bje rrum s cotoma ) scans of increasing tissue depth; indirectly m easures nerve fiber
a rcua te 120 105 90 75 60 120 105 90 75 60 layer (NFL) thickness (Figs. 9-6, 9-7)
s cotoma 135 45

150 30
Op tical co h er en ce to m o gr ap h y (OCT): m easures
165 15
optical backscattering of light to produce high-resolution,
180 0 cross-sectional im age of the NFL (Fig. 9-8)
195 345

Scan n in g laser p o lar im etr y ( SLP; n er ve fib er

210 330
an alyzer , GDx) : uses a confocal scanning laser
225 315
240 255 270 285 300 240 255 270 285 300
ophthalm oscope with an integrated polarim eter to detect
Le ft Right changes in light polarization from axons to m easure the NFL
Is ola te d s cotoma within S e ide l s cotoma
Bje rrum's a re a
thickness; quantitative analysis of NFL thickness to detect early
glaucom atous dam age (Fig. 9-9)
Fig ure 9-5. Composite diagram depicting different types of field defects. (From
Bajandas FJ , Kline LB: Neuro-ophthalmology review manual, ed 3,Thorofare, NJ , Op tic n er ve b lo o d flo w m easu r em en t: color Doppler
2004, Slack.) imaging and laser Doppler flowmetry (Fig. 9-10)

Ta b le 9-1. Summary of techniques for retinal nerve fiber layer analysis

Te c hniq ue Eq uip m e nt ne e d e d Go ve rning p rinc ip le s Ad va nta g e s Dis a d va nta g e s

Ophthalmoscopy Direct ophthalmoscope or Nerve fiber layer visibility is Easy to perform using readily May be difficult without clear
slit lamp and 78D or 90D enhanced with shortwavelength available equipment media
lens Red-free light light Nerve fiber layer not easily
seen in lightly pigmented
fundi
Red-free, high Fundus camera with red- Nerve fiber layer visibility is Nerve fiber layer defects may be Requires skilled
contrast fundus free filter enhanced with shortwavelength easy to detect photographer
photography High contrast black-and- light Requires dilated pupil
white film and paper Limitations of
ophthalmoscopy apply
Retinal contour Scanning laser Three-dimensional construction Easy to perform through Equipment is expensive
analysis ophthalmoscope that can of retinal surface can measure undilated pupil Height measurements
perform tomographic retinal height above a reference No discomfort to patient depend upon location of
topography plane – height is related to nerve Can image through most media reference plane
fiber layer thickness opacities unless very dense Retinal thickness may not
be true indirect measure of
nerve fiber layer thickness
Optical coherence Optical coherence Uses reflected and Can differentiate layers within Equipment is expensive
tomography tomography unit backscattered light to create the retina, including the nerve Requires dilated pupil
images of various retinal layers fiber layer, with a 10 µm Resolution may not be high
(analogous to the use of sound resolution Correlates with enough to detect small
waves in ultrasonography) known histology changes
Scanning laser Scanning laser polarimeter Birefringent properties of the Easy to perform through Equipment is expensive
polarimetry nerve fiber layer caus e a undilated pupil Measurements not
measurable phase shift of an No discomfort to patient correlated histologically in
incident polarized light Can image through most media humans
proportional to the tissue opacities, unless very dense Requires compensation for
thickness Resolution limited to size of a other polarizing media, e.g.,
pixel (possibly as small as 1 µm) cornea
Reproducibility 5–8 µm
From Chopin NT: Retinal nerve fiber layer analysis. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.

266
 Disorders

Path o lo gy:
P ATHOLOGY atrophy of neural elem ents with cystic spaces containing
hyaluronic acid (m ucopolysaccharides), which stains with
Gla u c o m a colloidal iron and Alcian blue (Fig. 9-13)
Dropout of ganglion cells, replacem ent of NFL with dense
gliotic tissue and som e glial cell nuclei; partial preservation of Glio s is o f t h e o p t ic n e rve
inner nuclear layer with loss of Mu€ ller’s and am acrine cells With optic atrophy, glial cells replace nerve cells and assum e a
(norm al 8–9 cells high; in glaucom a, 4–5 cells high); earliest random distribution throughout the optic nerve
histologic changes occur at level of lam ina cribrosa; advanced
cases m ay show backward bowing of lam ina or ”beanpot”
appearance (Figs. 9-11, 9-12)
DISORDERS

S c h n a b e l’s c a ve rn o u s o p t ic a t ro p h y Child ho o d Gla uc o m a

Histologic finding in eyes with increased IOP or atherosclerosis
and norm al IOP; hyaluronic acid infiltration of nerve (See Chapter 5, Pediatrics/Strabism us)
(originally thought to be from vitreous; m ay be from in situ
production); also occurs in eyes with ischem ic optic
neuropathy
P rim a ry Op e n-Ang le Gla uc o m a (P OAG)

Progressive, bilateral, optic neuropathy with open angles,

typical pattern of nerve fiber bundle visual field loss, and
increased intraocular pressure (IOP > 21 m m Hg) not caused by
another system ic or local disease

Ep id em io lo gy: second leading cause of blindness in US; most

com mon form of glaucom a (60%–70%); 7% of population has
ocular hypertension; 3% of population in Baltim ore Eye Study
had glaucomatous VF defects. If damage in one eye, untreated
fellow eye has 29% risk over 5 years. Steroid responders have 31%
risk of developing glaucom a within 5 years

Gen etics: juvenile-onset POAG has been m apped to

chrom osom e 1q21-q31 (GLC1A, MYOC/TIGR). Adult POAG
has been m apped to chrom osom es 2qcen-q13 (GLC1B), 2p15-
p16 (GLC1H), 3q21-q24 (GLC1C), 8q23 (GLC1D), 10p14
Fig ure 9-6. Confocal scanning laser ophthalmoscopy. (Adapted from Schuman (GLC1E, OPTN [optineurin]). A m utation in the OPTN gene
J S, Noeker RJ : Imaging of the optic nerve head and nerve fiber layer in glaucoma. accounts for $ 17% of POAG. Congenital glaucom a m apped to
Ophthalmol Clin North Am 8:259–279, 1995.) chrom osom e 2p21 (GLC3A) and chrom osom e 1p36 (GLC3B).

Fig ure 9-7. Confocal scanning laser ophthalmoscopy printed report.

(From Zangwill L, de Souza K, Weinrob RN: Confocal scanning laser
ophthalmoscopy to detect glaucomatous optic neuropathy. In Shuman
J S [ed]: Imaging in glaucoma. Thorofare, NJ , 1997, Slack.)

267
CHAPTER 9 • Glaucom a

B
Fig ure 9-8. Optical coherence tomography. (From Pedut-Kloizman TP, Schuman J S: Disc analysis. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

Risk facto r s: increased IOP, increased cup-to-disc ratio,

thinner central corneal thickness (less than approxim ately 550
µm ), positive family history (6 Â increase in first-degree
relatives), age (increased in patients > 60 years old; 15% of
those > 80 years old have glaucom a), race (6 Â increase in
African Am ericans vs Caucasians); other possible risk factors
include DM, m yopia, hypertension, m igraines

Path o gen esis: unknown

Theories of optic nerve dam age:
Mechanical: resistance to outflow, trabecular m eshwork
dysfunction, difference between IOP and CSF pressure
Vascular: poor optic nerve perfusion; ischem ia occurs with
system ic hypotension; possible contribution of
vasospasm ; increased IOP reduces blood flow to optic
nerve
NERVE FIBER ANALYSIS Ganglion cell necrosis or apoptosis: excitotoxicity
(glutam ate), neurotrophin starvation, autoim m unity,
Actua l va lue Sta tus Proba bility
abnorm al glial-neuronal interactions (tum or necrosis
Symmetry 0 .9 7 W ithin norma l factor [TNF]), defects in endogenous protective
Superior ra tio 2 .3 0 W ithin norma l m echanism s (heat shock proteins)
Inferior ra tio 2 .3 6 W ithin norma l
Superior/ na sa l 2 .1 2 W ithin norma l Fin d in gs: increased IOP, large cup-to-disc ratio (especially
Ma ximum modula tion 1 .3 6 W ithin norma l vertical elongation of optic cup; notching of rim ; asym m etry of
Ellipse modula tion 2 .1 3 W ithin norma l C/D ratio), nerve fiber layer loss (evaluate with red-free light),
The number 10
disc splinter hem orrhage (often followed by notching in region
Avera ge thickness 64 W ithin norma l
of hem orrhage), characteristic visual field defects; m ay have
beta-zone peripapillary atrophy (atrophy of RPE and
Ellipse a vera ge 69 W ithin norma l
choriocapillaris between optic disc and alpha-zone)
Superior a vera ge 78 W ithin norma l
With optic nerve dam age, loss of yellow-blue color axis
Inferior a vera ge 80 W ithin norma l
occurs first
Superior integra l 0 .2 2 7 W ithin norma l
Com bined sensitivity of tonom etry and disc exam ¼ 67%

Fig ure 9-9. Printout from the GDx software of the Nerve Fiber Analyzer;
normal eye. (From Chopin NT: Retinal nerve fiber layer analysis. In Yanoff M, Visu al field s: sensitivity (percent of diseased properly
Duker J S [eds]: Ophthalmology, 1999, London, Mosby.) identified) ¼ 85%; specificity (% of norm als

268
 Disorders

Fig ure 9-10. Color Doppler imaging of the ophthalmic

artery. (From O’Brien C, Harris A: Optic nerve blood flow
measurement. In Yanoff M, Duker J S [eds ]:
Ophthalmology, London, 1999, Mosby.)

Fig ure 9-11. POAG demonstrating cupping of the optic nerve head. (From Yanoff
M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.)

i a

rc

rpe
c

A B
Fig ure 9-12. POAG demonstrating atrophy of the inner retinal layers. A, low power, B, higher magnification. (From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis,
2002, Mosby.)

269
CHAPTER 9 • Glaucom a

A B
Fig ure 9-13. Schnabel’s cavernous optic atrophy demonstrating cystic spaces in optic nerve parenchyma. A, Hemoxylin and eosin stain, B, colloidal iron stain. (From
Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.)

properly identified) ¼ 85%. Risk of initial field loss is 1%–2%/ Lyt ic g la u c o m a s

year for ocular hypertensive patients, risk of field loss increases
Macrophages laden with various m aterials
with increasing IOP

Hem o lytic: hem osiderin-laden m acrophages deposit in TM

Se c o nd a ry Op e n-Ang le Gla uc o m a
Ph aco lytic: denatured lens proteins escape from
Mech an ism /Etio lo gy: hyperm ature or m orgagnian cataract; with intact lens capsule;
Clogging of TM: RBCs (hyphem a, sickle cell, ghost cells), proteins and macrophages that have ingested lens m aterial clog
m acrophages (hem olytic, phacolytic, m elanocytic, the outflow tract; appears as m ilky substance in AC; requires
m elanom alytic), neoplastic cells (m alignant tum ors, surgical rem oval of lens. Reduce the inflam m ation
neurofibrom atosis, juvenile xanthogranulom a), pigm ent preoperatively with topical steroid, cycloplegic, and ocular
(pigm entary glaucom a, pseudoexfoliation, chronic uveitis, hypotensive m edications
m alignant m elanom a), lens protein (lens-particle glaucom a),
photoreceptor outer segm ents (Schwartz-Matsuo syndrom e), Melan o m alytic: m elanin from m alignant m elanom a is
zonular fragm ents (α-chym otrypsin induced), viscoelastic engulfed by m acrophages, which clog TM
Toxic/medication: steroid-induced, siderosis, chalcosis
Inflammation: uveitis, IK
Melan o cyto m alytic: m elanin from necrotic
Increased episcleral venous pressure
m elanocytom a
Trauma: angle recession, chem ical injury, hem orrhage,
postoperative
Tu m o r c e lls
Hyp h e m a Deposition of tum or cells, inflam m atory cells, and cellular
27% risk of glaucom a if hyphem a > 50% of AC volum e; 52% debris; also m acrophages laden with m elanin from
risk with total hyphem a m elanom as, direct infiltration of trabecular m eshwork, or
Surgical intervention for uncontrolled IOP, corneal blood neovascularization with subsequent intraocular hem orrhage
staining, prolonged presence of clot (> 15 days), rebleed, 8-ball or neovascular glaucom a (NVG). 45% of anterior uveal
hyphem a (see Ch. 10, Anterior Segm ent) m elanom as and 15% of choroidal m elanom as are
associated with glaucom a. Retinoblastom a causes glaucom a
in 25%–50% from NVG, pupillary block, or tum or m aterial;
S ic kle c e ll
also lym phom a
Sickled cells are rigid; therefore, even sm all hyphem as can
obstruct TM and raise IOP; acetazolam ide (Diam ox) P ig m e n t a ry g la u c o m a (P G) (AD)
contraindicated (increases ascorbic acid in AC causing greater
sickling, also causes hem oconcentration and system ic acidosis, Mapped to chrom osom e 7q35-q36 (GLC1F)
both of which favor sickling) Typically young m yopic m ales; up to 50% of patients
with pigm ent dispersion syndrom e (PDS) develop
Gh o s t c e lls glaucom a
Weeks to m onths after vitreous hem orrhage, degenerated RBCs
pass through hyaloid face. These khaki-colored, spherical cells Mech an ism : reverse pupillary block bows iris against
are less pliable than RBCs and do not pass through trabecular zonules, and iris m ovem ent results in pigm ent liberation;
m eshwork; layer of cells form s a pseudohypopyon pigm ent obstructs TM

270
 Disorders

Fin d in gs: halos and blurry vision with IOP spikes Fin d in gs: iridopathy (blood-aqueous barrier defect,
(pigm ent m ay be released with exercise); Krukenberg pseudouveitis, iris rigidity, posterior synechiae, poor pupillary
spindle (m elanin phagocytized by corneal endothelium ); dilation), keratopathy (reduced endothelial cell count,
heavy TM pigm entation; iridodonesis; iris transillum ination endothelial decom pensation, corneal endothelial proliferation
defects (radial m idperipheral spoke-like appearance); over trabecular m eshwork), Sam paolesi’s line (scalloped band
associated with lattice degeneration (20%) and retinal of pigm entation anterior to Schwalbe’s line), weak zonules
detachm ent (5%) (Fig. 9-14) (phacodonesis, risk of lens dislocation during cataract surgery,
and angle closure from anterior m ovem ent of lens), lens
capsule (white fibrillar m aterial)
Tr eatm en t: m iotics m inim ize iris-zonule touch; very good
response to laser trabeculoplasty; laser peripheral iridotom y
m ay help reduce posterior bowing of iris Tr eatm en t: very good response to laser trabeculoplasty.
PXS usually presents with higher initial IOP and is m ore
difficult to control with m edical treatm ent alone vs POAG

P s e u d o e xfo lia t io n g la u c o m a (P XG)

Mapped to chrom osom e 15q24 (LOXL1).
Le n s -p a rt ic le g la u c o m a
Lens m aterial in setting of violated capsule blocks TM
High incidence (up to 50%) of secondary open-angle
following traum a or cataract surgery. Greater inflam m ation
glaucom a in patients with pseudoexfoliation syndrom e
than with phacolytic; PAS, posterior synechiae, and
(PXS); m ore com m on am ong Scandinavians. Am yloid-like
inflam m atory m em branes are com m on; IOP can be very high
substance deposits in eye and clogs TM, also found in other
organs (Fig. 9-15)
Tr eatm en t: cycloplegics, steroids (careful because steroids
slow absorption of lens m aterial), and ocular hypotensive
m edications

S c h w a rt z-Ma t s u o s yn d ro m e
High IOP associated with rhegm atogenous RD. (usually
shallow and anterior involving ora serrata or nonpigm ented
epithelium of ciliary body). Photoreceptor outer segm ents
m igrate transvitreally into aqueous and block trabecular
m eshwork; outflow obstruction is also due to pigm ent released
from RPE and glycosam inoglycans released from
photoreceptors. Usually resolves after repair of RD

Alp h a -c h ym o t ryp s in in d u c e d
Fig ure 9-14. Gonioscopic view of pigmentary glaucoma. (From Ball SF: Zonular fragm ents accum ulate in trabecular m eshwork
Pigmentary glaucoma. In Yanoff M, Duker J S [eds]: Ophthalmology, London, after intracapsular cataract extraction (ICCE) with enzym atic
1999, Mosby.) zonulysis. Alpha-chym otrypsin itself does not cause dam age

A B
Fig ure 9-15. Pseudoexfoliative syndrome demonstrating exfoliative material on lens capsule. (From Samuelson TW, Shah G: Pseudoexfoliative glaucoma. In: Yanoff M,
Duker J S [eds]: Ophthalmology, London, 1999, Mosby.)

271
CHAPTER 9 • Glaucom a

Co rt ic o s t e ro id -in d u c e d After co r n eal tr an sp lan t: distortion of TM

Risk factors include open-angle glaucom a, fam ily history of
glaucom a, increasing age, diabetes, and high m yopia. Topical Ele va t e d e p is c le ra l ve n o u s p re s s u re
steroids have greater pressure-raising effects than do system ic Causes resistance to aqueous outflow
steroids:
In response to topical dexam ethasone 0.1% qid for
Etio lo gy: carotid-cavernous sinus fistula, cavernous sinus
6 weeks, 65% had IOP rise of < 5 m m Hg, 30% had a rise
throm bosis, Sturge-Weber syndrom e, neurofibrom atosis,
of 5–14 m m Hg, and 5% had a rise of at least 15 m m Hg
orbital m ass (tum or, varices), thyroid-related ophthalm opathy
Fluorom etholone, rim exolone (Vexol), and loteprednol
(false elevation of IOP can be caused by IR fibrosis with
(Lotem ax, Alrex) are less likely to increase IOP
increased resistance in upgaze), superior vena cava obstruction,
m ediastinal tum ors and syndrom es, scleral buckle, idiopathic.
S id e ro s is (iro n ) o r c h a lc o s is (c o p p e r ) May see blood in Schlem m ’s canal
TM toxicity and scarring from intraocular foreign body
P rim a ry Ang le -Clo s ure Gla uc o m a
Uve it ic
Inflam m ation of the ciliary body (cyclitis) often decreases Glaucom a caused by peripheral iris obstructing the trabecular
aqueous production however, aqueous outflow m ay also be m eshwork, m ost com m only due to pupillary block; classified
acutely im paired by trabeculitis and inflam m atory cells causing as acute, interm ittent, or chronic. Plateau iris syndrom e is a
TM obstruction. Chronic inflam m ation m ay cause PAS form of prim ary angle-closure without pupillary block

Mech an ism o f p u p illar y b lo ck: in susceptible

JRA-asso ciated u veitis: glaucom a occurs in about 20% patients, iridolenticular touch causes resistance of aqueous
of cases flow from posterior to anterior cham ber, causing increased
posterior pressure. When the pupil is m id-dilated (i.e., stress,
Glau co m ato cyclitic cr isis (Posner-Schlossm an low am bient light levels, sym pathom im etic or
syndrom e): episodic trabeculitis (m ononuclear cells in anticholinergic m edications), the elevated posterior cham ber
TM) with high IOP for hours to weeks; m inim al pressure causes peripheral iris tissue to bow forward and
inflam m atory signs; prolonged use of steroid is not occlude the TM
recom m ended. Association with herpes virus
Ep id em io lo gy: acute form is m ost com m on in Eskim os and
Asians, followed by Caucasians, then African Am ericans;
Fu ch s’ h eter o ch r o m ic ir id o cyclitis: up to 60% highest risk is between age 55 and 65 years old; m ore com m on
develop high IOP (glaucom a occurs m ore com m only in in wom en only am ong Caucasians. Chronic form is m ore
patients with bilateral disease and spontaneous hyphem a); com m on in African Am ericans than Asians, who have a higher
m ay persist after resolution of uveitis; do not develop PAS. risk than Caucasians. 5% of population older than 60 years of
Association with rubella, herpes, and toxoplasm osis age have angles that can be occluded; 0.5% of these individuals
develop angle closure. Usually bilateral (75% risk in untreated
Ph aco an tigen ic u veitis ( p h aco an ap h ylactic fellow eye within 5 years)
en d o p h th alm itis) : type 3 hypersensitivity reaction to lens
m aterial following traum a or surgery causing zonal An ato m ic featu r es p r ed isp o sin g to an gle
granulom atous reaction after latent period clo su r e: sm all anterior segm ent (hyperopia,
nanophthalm os, m icrocornea, m icrophthalm os); hereditary
narrow angle; anterior iris insertion (Eskim os, Asians, and
Uveitis-glau co m a-h yp h em a African Am ericans); shallow AC (large lens, plateau iris
syn d r o m e: nongranulom atous inflam m ation. IOL configuration, loose or dislocated lens)
physically irritates iris and ciliary body
Ac u t e a n g le c lo s u re
Tra u m a t ic Sym p to m s: blurred vision, colored halos around lights,
An gle r ecessio n : glaucom a develops in 10% of cases pain, redness, nausea and vom iting, headache
with > 180° of involvem ent due to scarring of the angle and TM
Fin d in gs: high IOP, corneal epithelial edem a, conjunctival
injection, m id-dilated pupil, shallow anterior cham ber, m ild
Ch em ical b u r n : can dam age trabecular m eshwork or uveal
AC cell and flare, closed angle (perform indentation
circulation, or cause shrinkage of scleral collagen
gonioscopy to differentiate between appositional and
synechial angle closure; glycerin can be used to clear corneal
After vitr ecto m y: m ost com m on com plication is edem a; evaluate angle in other eye), ON swelling and
glaucom a; caused by intraocular gas, hyphem a, ghost cells, hyperem ia; with rapid rise in IOP, m ay see arterial pulsations
uveitis, silicone oil (retinal ischem ia can occur)

272
 Disorders

Sequ elae o f isch em ia: segm ental iris atrophy (focal iris P la t e a u iris
strom a necrosis), dilated irregular pupil (sphincter and dilator
Co n figu r atio n : angle anatom y resulting in deep central AC
necrosis), glaukom flecken (focal anterior lens opacities due to
and shallow peripheral AC
epithelial necrosis)

Syn d r o m e: angle closure in an eye with plateau iris

Late fin d in gs: decreased vision, PAS, chronic corneal edem a configuration; usually occurs in 4th to 5th decade of life, and in
individuals with less hyperopia than typical angle-closure
Pr o vo cative tests: prone test, darkroom test, prone patient
darkroom test, pharm acologic pupillary dilation; positive if
IOP rises > 8 m m Hg Fin d in gs: m ay present with acute or chronic angle
closure; anteriorly positioned ciliary processes force the
Th ym o xam in e test: α-adrenergic antagonist used to peripheral iris m ore anteriorly than norm al; deep cham ber
distinguish angle closure from glaucom a with narrow angles; centrally; flat iris contour with sharp dropoff peripherally;
thym oxam ine blocks iris dilator m uscle producing m iosis; with dilation, peripheral iris folds into the angle and
however, it does not affect trabecular m eshwork and norm ally occludes TM; with com pression gonioscopy, angle is m ore
does not decrease IOP; therefore, decrease in IOP suggests difficult to open and does not open as widely as in prim ary
m iosis has rem oved iris from the outflow channel reversing angle closure
angle closure
Tr eatm en t: laser peripheral iridotom y, laser iridoplasty, and
m iotics; plateau iris appearance rem ains
Tr eatm en t o f acu te an gle-clo su r e
glau co m a: peripheral iridotom y or iridectom y
(PI, definitive treatm ent); com pression gonioscopy
(m ay force aqueous through block and open angle);
pilocarpine (m ay not be effective at IOP > 40 m m Hg due to Se c o nd a ry Ang le -Clo s ure Gla uc o m a
sphincter ischem ia; m ay also cause lens–iris diaphragm to
m ove forward, worsening pupillary block); reduce IOP with
β-blocker, α2 -agonist, topical or oral CAI, or hyperosm otic
agent (isosorbide, glycerin [contraindicated in diabetics], Me c h a n is m / Et io lo g y
or IV m annitol [risk of cardiovascular adverse effects]); With p u p illar y b lo ck: phacom orphic (lens enlargem ent in
topical steroids for inflam m ation; laser PI in fellow elderly; urgent surgery needed to rem ove lens); dislocated lens;
eye (75% chance of attack in untreated fellow eye; seclusio pupillae; nanophthalm os; aphakic and pseudophakic
pilocarpine lowers risk to 40% but m ay lead to chronic pupillary block; silicone oil (prevent by perform ing an inferior
angle closure) PI because oil is lighter than water)
If attack of angle closure is broken m edically, consider waiting Without pupillary block:
POSTERIOR ”PUSHING” MECHANISM (m echanical/
a few days before perform ing laser PI because corneal edem a,
iridocorneal touch, and iris congestion m ake the procedure anterior displacem ent of lens–iris diaphragm )
ANTERIOR ROTATION OF CILIARY BODY: inflam m ation
m ore difficult
(scleritis, uveitis, after scleral buckle or panretinal
Persistently increased IOP following laser PI m ay be due to photocoagulation [PRP]), congestion (postscleral
PAS, incom plete iridotom y, underlying open-angle glaucom a, buckling, nanophthalm os), choroidal effusion
or secondary angle closure (hypotony; uveal effusion), suprachoroidal
hem orrhage
AQUEOUS MISDIRECTION (MALIGNANT
In t e rm it t e n t a n g le c lo s u re
GLAUCOMA)
2 :1 ratio of nonacute-to-acute presentations; m ay be PRESSURE FROM POSTERIOR SEGMENT: tum or,
asym ptom atic or have sim ilar presentation as acute angle expanding gas, exudative retinal detachm ent (RD)
closure but less severe, and occurs over days to weeks; often CONTRACTION OF RETROLENTAL TISSUE: persistent
com plain of headaches, episodes resolve spontaneously, hyperplastic prim ary vitreous (PHPV), retinopathy
especially by entering a well-lit area (induces m iosis); IOP m ay of prem aturity (ROP)
be norm al, glaukom flecken and PAS are evidence of previous ANTERIOR ”PULLING” MECHANISM (adherence of iris to
attack. Treat with laser PI trabecular m eshwork/m em branes over TM)
EPITHELIAL: epithelial downgrowth, fibrous ingrowth
ENDOTHELIAL: iridocorneal endothelial (ICE)
Ch ro n ic a n g le c lo s u r e
syndrom e, posterior polym orphous dystrophy
Gradual closure of angle by apposition or developm ent of PAS (PPMD)
leads to slow rise in IOP; variable IOP, but less than with acute NEOVASCULAR: neovascular glaucom a (NVG)
angle closure. Often asym ptom atic; cornea usually clear due to PERIPHERAL ANTERIOR SYNECHIAE (PAS)
gradual rise in IOP, but can have extensive visual field loss ADHESION FROM TRAUMA

273
CHAPTER 9 • Glaucom a

As s o c ia t e d w it h RD s u rg e ry iridotom y in both eyes (elim inate any com ponent of

pupillary block); m iotics are contraindicated
Anterior rotation of CB around the scleral spur secondary to
50% resolve within 5 days with m edical therapy alone
swelling from excessive PRP or tight scleral buckle
Surgical: argon laser photocoagulation of ciliary
processes (requires shrinkage of at least 2–4 ciliary
Tr eatm en t: cycloplegics, PI, consider cutting encircling band processes), Neodym ium :Yttrium Alum inium Garnet
(Nd :YAG) laser rupture of hyaloid face
Na n o p h t h a lm o s (for pseudophakic and aphakic patients; 4–6 m J; best
> 10 D hyperopia; sm all eye (< 20 m m ) with sm all cornea to perform peripherally [through PI]), com bined
(m ean diam eter ¼ 10.5 m m ), shallow AC, narrow angle, and iridectom y/zonulectom y/hyaloidectom y/vitrectom y
high lens/eye volum e. Pupillary block or uveal effusion often curative (for phakic patients)
produces angle-closure glaucom a. Thick sclera (about 2 Â
thicker than norm al) m ay im pede vortex venous drainage, as In t ra o c u la r t u m o rs
well as decrease uveoscleral outflow, and can also result in
Can push angle closed from posteriorly
spontaneous uveal effusion with anterior rotation of CB
Malignant m elanom a of the anterior uveal tract can cause
leading to angle closure
glaucom a by direct extension of tum or into trabecular
m eshwork, inducing neovascularization of the angle,
Tr eatm en t: weak m iotics, cycloplegics (if no angle obstructing TM with m elanin-laden m acrophages
crowding), laser PI, laser iridoplasty, trabeculectom y after (m elanom alytic), or seeding of tum or cells in outflow
prophylactic sclerotom y. Because there is a high com plication channels; other m echanism s include pigm ent dispersion,
rate with surgery (uveal effusion), first use m edical therapy, inflam m ation, and hem orrhage
then laser
Increased risk of com plications with cataract surgery (RD, P HP V
choroidal effusion, angle-closure glaucom a, flat AC, CME,
corneal decom pensation, m alignant glaucom a, IOL Contraction of retrolenticular m em brane and swelling of
m iscalculations). If shallow AC and thickened choroid cataract can cause angle closure
preoperatively, perform prophylactic anterior sclerotom ies
with surgery Tr eatm en t: rem ove lens and m em brane via lim bal
approach (pars plana approach m ay be dangerous in that
retina can extend up to the pars plicata)
Ma lig n a n t g la u c o m a (a q u e o u s m is d ire c t io n
s yn d ro m e , c ilio le n t ic u la r o r c ilio vit re a l b lo c k )
ROP
Mech an ism : tips of ciliary processes rotate forward against
lens (ciliolenticular block) causing anterior displacem ent of Due to contraction of retrolental tissue
lens-iris diaphragm
Ep it h e lia l d o w n g ro w t h
Risk facto r s: uveitis, angle closure, nanophthalm os,
Due to epithelium growing over angle
hyperopia; occurs postoperatively (usually 5 days) following a
variety of laser or incisional surgeries; usually in patients with
PAS or chronic angle closure following intraocular surgery if Fib ro u s in g ro w t h
the hyaloid face has not been broken (follows 2% of surgical
Due to fibrous proliferation through wound into AC
cases for angle closure); can also occur in unoperated eye when
m ydriatics are stopped or m iotics are added
ICE s yn d ro m e
Fin d in gs: entire AC shallow (versus angle closure in which Due to descem etization of TM and angle closure from
the AC is deeper centrally than peripherally), IOP higher than contraction of endothelial m em brane; PAS are prom inent but
expected, presence of patent iridectom y, absence of less responsible for glaucom a
suprachoroidal fluid or blood

DDx: pupillary block (no patent iridectom y; m oderate depth P P MD

of central AC), suprachoroidal hem orrhage (acute severe pain Due to abnorm al corneal endothelial cells that m igrate into
and choroidal elevation), choroidal effusion (usually low IOP angle causing glaucom a (15%)
and choroidal elevation), annular peripheral choroidal
detachm ent
NVG
Tr eatm en t: Due to widespread retinal or ocular ischem ia; clinically
Medical: cycloplegic (relaxes ciliary m uscle and pulls transparent fibrovascular m em brane flattens anterior iris
lens-iris diaphragm posteriorly; continued indefinitely to surface; m yofibroblasts provide m otive force for angle closure
prevent recurrence), aqueous suppressants, peripheral and ectropion uveae

274
 Treatm ent

Etio lo gy: proliferative retinopathy (diabetes [33% of all toxicity. These entities are m ore likely to have early loss of
form s of NVG], ischem ic CRVO [33%], carotid occlusive central vision and color vision; pallor m ay be worse than
disease [13%], ciliary artery occlusion, sickle cell, Norrie cupping
disease, ROP); intraocular inflam m ation (uveitis,
postoperative); neoplasm s (retinoblastom a [50% develop Diagn o sis: diurnal curve, neurologic workup (CBC, ESR,
NVG], m alignant m elanom a, large cell lym phom a [reticulum ANA, VDRL and FTA-ABS, carotid evaluation, brain
cell sarcom a], m etastatic); chronic retinal detachm ent neuroim aging)

Tr eatm en t: aqueous suppressants and hyperosm otics; Pr o gn o sis: m ore difficult to treat than POAG
increase uveoscleral outflow (atropine; avoid m iotics);
panretinal photocoagulation; peripheral retinal cryotherapy
(if poor visualization of retina; 50% develop phthisis with TREATMENT
cyclocryotherapy); glaucom a drainage im plant (70%
success rate)
Generally, m edications are tried first, followed by laser
treatm ent, and then surgery; however, the choice and tim ing of
various treatm ent m odalities are dependent on the type of
No rm a l Te ns io n Gla uc o m a (NTG) glaucom a, severity of optic nerve dam age, level of control, and
m any other factors. Therapy m ust also be directed to any
Glaucom a with open angles and IOP < 22 m m Hg preexisting or underlying process

Pr o p o sed m ech an ism s:

Nocturnal systemic hypotension: diurnal curve of BP sim ilar Me d ic a tio n
to IOP. 66% of patients will have a BP drop of greater
than 10% during early m orning hours (”dippers”); Ocular hypotensive agents (see Chapter 2, Pharm acology)
patients with HTN have an even greater swing in BP
(26% average drop); patients with HTN treated with
β-blockers can have diastolic BP < 50 m m Hg, which m ay La s e r
com prom ise blood supply to optic nerve
Autoimmune: increased incidence of proteinem ia and Arg o n la s e r t ra b e c u lo p la s t y (ALT) (Fig. 9-16)
autoantibodies in patients with NTG
Vasospasm Power 400–1200 m W; spot size 50 µm ; duration 0.1 s; titrate
Previous hemodynamic crisis: excessive blood loss or shock power to generate sm all bubble at junction of nonpigm ented
and pigm ented TM; 50 applications/180°; average of 30%
Fin d in gs: VF defects in NTG have steeper slopes, greater reduction in IOP; can be repeated
depths, and closer proxim ity to fixation than in POAG; splinter Iopidine im m ediately postoperatively to avoid pressure spike,
hem orrhages are m ore com m on and treat with steroids for 1 week. Assess efficacy of treatm ent
at 6 weeks postoperatively
Anterior burns have poor effect; posterior burns m ore likely to
DDx: POAG with large diurnal variation, ”burned-out” develop PAS
secondary open-angle glaucom a, chronic angle closure. Must
rule out intracranial processes and other causes of optic
neuropathy
Resu lts: 25% fail to control IOP at 1 year; 10% per year
failure rate thereafter. Repeating ALT m ay provide IOP control
Cupping can occur with neurologic disease: AION (arteritic in 33–50%, but sustained elevation in 10%
50%; nonarteritic 10%), chiasm al com pressive lesions (5%), Best predictor of success is type of glaucom a: PXG (best) >
optic neuritis (< 5%), hereditary optic neuropathies, m ethanol PG > POAG > NTG > aphakic (worst)

cornea
Schwa lbe's
too
line post
a nterior
nonpigmented trea tment
tra becula r correct excessive
meshwork pla cement trea tment
pigmented
tra becula r too correct
meshwork posterior trea tment

sclera l spur insufficient

trea tment
iris

Fig ure 9-16. ALT. (From Schwartz AL: Argon Laser Trabeculoplasty in Glaucoma: What’s Happening (Survey Results of American Glaucoma Society Members),
J Glaucoma 2:329–336, 1993.)

275
CHAPTER 9 • Glaucom a

The greater the am ount of pigm ent in the angle, the better the TOXICITY: intraocular causes corneal decom pensation
result; poorer response in patients < 50 years of age. ALT is (dam ages endothelium ), AC inflam m ation, and
ineffective (and m ay worsen IOP) in inflam m atory glaucom a, necrosis of ciliary body and iris; can develop scleral
angle recession, angle-closure glaucom a (m em branes in necrosis; retinal toxicity with intravitreal injection
angles), congenital glaucom a, and steroid-induced glaucom a. 5-FU: specifically affects the S-phase of the cell cycle;
Contraindicated in patients with large am ounts of PAS requires postoperative injections (30–35 m g in 5 m g
doses over 1–3 weeks); corneal epithelial toxicity
Co m p licatio n s: IOP spike (increases with energy and m ay occur
num ber of laser burns), iritis, PAS
Co m p licatio n s: block ostium during surgery in hyperopia,
nanophthalm os, or chronic angle closure, the ciliary processes
S e le c t ive la s e r t ra b e c u lo p la s t y (S LT) can roll anteriorly
Tim e and spot size (400 µm ) are fixed, power 0.6–0.9 m J; 50
Tr eatm en t: suture wound and reinflate eye allowing ciliary
confluent applications/180°, straddling TM
processes to revert to norm al position; if still present, use
Produces less tissue destruction than ALT, and is
cautery to rem ove the processes
repeatable. Beware IOP spikes in patients with pigm ent
High IOP immediately postoperatively: attem pt digital
dispersion syndrom e and pigm entary glaucom a
m assage or laser suture lysis
Shallow or flat AC: (Table 9-2)
La s e r irid o t o m y Blebitis: photophobia, discharge, m arked conjunctival
Perform in eyes with narrow angles (prophylactically), iris injection around an opalescent filtering bleb; often
bom be, synechial angle closure, pigm entary glaucom a (due to Seidel-positive, m oderate AC cells and flare, but no
configuration of iris), plateau iris, and m alignant glaucom a involvem ent of the vitreous. Organism usually
Staphylococcus
TREATMENT: intensive topical antibiotics, repair wound
YAG: power 1–12 m J; bleed m ore, but close less than leak, observe daily for endophthalm itis
with argon Bleb-associated endophthalmitis: pain, decreased vision, AC
cells and flare, hypopyon, and vitreous cells; often Seidel-
Ar go n : reaction is pigm ent-related and requires m ore energy positive. Organism usually Streptococcus species or H.
and total applications than with YAG; less bleeding because of influenzae
therm al effect; extensive tissue destruction at m argins of
treatm ent; iritis m ore pronounced
Ta b le 9-2. Treatment of shallow or flat anterior chamber
following trabeculectomy
Irid o p la s t y
Ble b Intra o c ula r De finitive
For narrow angles; aim at peripheral iris; stretch iris away he ig ht p re s s ure d ia g no s is Tre a tm e nt
from angle; power 200–400 m W; spot size 500 µm ; duration Elevated Low Excessive Bleb revision
0.5–1.0 s filtration
Flat Low Choroidal Cycloplegic, steroid, drainage
detachment
Surg e ry Bleb leak Antibiotic, aqueous
suppressants, stop steroid, AC
reformation, pressure patch,
Tra b e c u le c t o m y Simmons’ scleral compression
shell, large diameter soft
Consider use of antim etabolite in patients at risk for bleb contact lens, trichloroacetic
failure acid, glue, laser, or autologous
blood injection. Consider
surgical intervention (drainage
Risk facto r s fo r b leb failu r e: previous surgical failure, of choroidals and repair of
darker skin pigm entation, history of keloid form ation, wound) for impending failure of
neovascular changes, younger age, intraocular inflam m ation, bleb, flat anterior chamber with
scarred conjunctiva, high hyperopia, inability to use corneal decompensation,
kissing choroidals, progressive
corticosteroids, shallow AC cataract
Flat Elevated Suprachoroidal Drainage
An tim etab o lites: hemorrhage
Mitomycin-C (MMC): antineoplastic antibiotic (isolated Pupillary block Cycloplegic, steroid, peripheral
from Streptomyces caespitosus) iridotomy (PI)
MECHANISM: intercalates with DNA and prevents Malignant Cycloplegic, aqueous
replication; suppresses fibrosis and vascular glaucoma suppressants, PI, YAG anterior
vitreolysis or vitrectomy
ingrowth after exposure to the filtration site. Toxic
Elevated Elevated Encapsulated Needling, aqueous
to fibroblasts in all stages of cell cycle; 100Â m ore
bleb suppressants, bleb revision
potent than 5-FU

276
 Treatm ent

TREATMENT: em ergent, as for endophthalm itis Mech an ism : reduced aqueous production by destruction of
Suprachoroidal hemorrhage: usually occurs several days ciliary epithelium
after trabeculectom y surgery with acute pain, often while Procedure is painful; therefore, requires peribulbar or
straining; also, nausea, vom iting, and decreased vision retrobulbar block
MECHANISM: progressive serous choroidal detachm ent,
stretching the long posterior ciliary artery until it
ruptures Cyc lo c ryo t h e ra p y
RISK FACTORS: aphakia, hypertension, cardiovascular
disease, and increased age Fir st tr eatm en t: 2.5 m m cryo tip, with anterior edge 1 m m
Hypotony: generally seen in young m yopic patients, from inferior lim bus and 1.5 m m from superior lim bus.
especially with antim etabolites Provide 6 freezes on the clock hours of the inferior 180°.
DDx: wound leak, overfiltration, iridocyclitis, cyclodialysis, Maintain each freeze for 60 seconds at À80 °C. The iceball will
ciliochoroidal effusion, RD encroach upon cornea for about 0.5 m m . Wait 1 m onth for the
TREATMENT: wound revision IOP to reach new baseline
COMPLICATIONS: corneal edem a, cataract, choroidal
effusion, optic disc edem a, chorioretinal folds, Seco n d tr eatm en t: if first treatm ent does not lower IOP
m aculopathy sufficiently, treat superior tem poral quadrant with 2–4 freezes.
Exuberant bleb: bleb can enlarge, spread onto cornea, and Always leave at least 1 quadrant (usually superonasal) free to
interfere with vision due to astigm atism , dellen, or prevent hypotony. Give subconjunctival steroid
encroachm ent into visual axis postoperatively
TREATMENT: recess or am putate bleb; usually a cleavage Procedure is painful; therefore, requires peribulbar or
plane is present retrobulbar block

Dra in a g e im p la n t s (s e t o n s / t u b e s )
Seton is from Latin ”seta” or bristle (original surgery used S u rg ic a l irid e c t o m y
horse hair)
Perform through 3 m m clear corneal wound for angle-closure
glaucom a
Typ es:
Nonvalve: Molteno (single or double plate), Schocket,
Baerveldt; m ust tem porarily occlude tube lum en with In d icatio n s: if severe corneal edem a precludes adequate iris
suture or biodegradable collagen plug visualization, AC is extrem ely shallow, or patient is unable to
Valve: Ahm ed (1-way valve m aintains IOP at 8 m m Hg or cooperate for laser iridotom y
higher), Krupin, and Baerveldt (pressure-sensitive valve)
Anterior chamber glaucoma drainage implant: for patients
with scleral buckles who do not have adequate scleral
surface for placem ent of a seton; a silicone im plant MAJ OR GLAUCOMA
shunts fluid from AC or PC to the fibrous capsule CLINICAL STUDIES
surrounding the episcleral encircling elem ent
Ad va nc e d Gla uc o m a Inte rve ntio n
No n -p e n e t r a t in g filt ra t io n s u rg e ry
Stud y (AGIS)
Less Invasive Alternatives

Ab exter n o : Ex-Press m inishunt, canaloplasty, Ob je c t ive : to evaluate argon laser trabeculoplasty (ALT)
viscocanalostomy vs trabeculectom y as the initial surgery in patients with
advanced open-angle glaucom a not controlled by m edical
Ab in ter n o : iStent, CyPass, DeepLight Gold m icro-shunt, treatm ent
trabectom e Main outcom e variable was visual function (field
and acuity)
Go n io s yn e c h ia lys is
Direct lysis of PAS with cyclodialysis spatula or other Re s u lt s
m icroinstrum ent; consider for angle-closure glaucom a of less
7-year data
than 12 m onths’ duration
In African Am ericans, the best results were obtained
when ALT was perform ed 1st, followed by
Cyc lo p h o t o c o a g u la t io n trabeculectom y (ATT sequence)
Laser treatm ent of ciliary body with argon (transpupillary or In Caucasians, the best results occurred when
endoprobe), Nd :YAG (contact or noncontact), or diode trabeculectom y was perform ed 1st, followed by
(contact); useful in m any types of refractory glaucom as, ALT, and finally by a 2nd trabeculectom y (TAT
including aphakic, neovascular, and inflam m atory, and in eyes sequence)
with failed filtering blebs

277
CHAPTER 9 • Glaucom a

Visual field defects were m ore severe in African Am erican

patients
Co lla b o ra tive Initia l Gla uc o m a
Eyes with IOP < 18 m m Hg at all visits had alm ost no Tre a tm e nt Stud y (CIGTS)
progression of VF loss
Trabeculectom y increased the risk of cataract by 78% Ob je c t ive : to evaluate m edicine (stepped regim en)
(47% if no com plications occurred vs 104% if vs trabeculectom y (with or without 5-fluorouracil)
com plications occurred, especially severe for the treatm en t of n ewly diagn osed open -angle
inflam m ation and flat anterior cham ber) glaucom a (prim ary, pigm entary, or pseudoexfoliative)
The prim ary outcom e variable was progression of visual
Co n c lu s io n s field loss
Secondary outcom es were quality of life, visual acuity,
In patients with advanced POAG, ALT should be
and intraocular pressure
considered as the first surgical treatm ent in African
Am erican patients; trabeculectom y should be
considered first in Caucasians Re s u lt s
Risk factors for ALT failure are younger age and 5-year data
higher IOP VF loss was not significantly different with either
Risk factors for trabeculectom y failure are younger treatm ent
age, higher IOP, diabetes, and postoperative Surgery had an initially increased risk of significant visual
com plications (particularly elevated IOP and loss, but by 4 years, the average VA was equal in the
m arked inflam m ation) 2 groups
Risk factors for bleb encapsulation include m ale sex IOP averaged 17–18 m m Hg in the m edicine group vs
and previous ALT (but this was not statistically 14–15 m m Hg in the surgery group
significant) The rate of visually significant cataract was greater in the
Low IOP reduces VF deterioration surgery group
Trabeculectom y is associated with an increased risk of
cataract form ation
Co n c lu s io n s

Oc ula r Hyp e rte ns io n Tre a tm e nt Initial treatm ent of open-angle glaucom a with m edicine
or surgery results in sim ilar VF outcom e
Stud y (OHTS) Although VA loss was initially greater in the surgery
group, the differences converged with tim e
Ob je c t ive : to evaluate the effect of topical m edication
in delaying or preventing POAG in patients with ocular
hypertension (IOP between 24 and 32 m m Hg in one Ea rly Ma nife s t Gla uc o m a Tria l (EMGT)
eye and between 21 and 32 m m Hg in the fellow eye;
norm al visual fields and optic nerves)
Ob je c t ive : to evaluate the effectiveness of reducing IOP
The goal in the treatm ent group was to reduce the IOP (vs no treatm ent) on the progression of newly diagnosed
by at least 20% from baseline and obtain a target open-angle glaucom a
pressure of 24 m m Hg
The treatm ent group received argon laser trabeculoplasty
The prim ary outcom e variable was visual field loss
plus topical betaxolol
or optic nerve dam age
The prim ary outcom e m easures were progression of
visual field loss and optic disc changes
Re s u lt s A secondary aim was to assess risk factors for
Mean IOP reduction in the m edicine group was 22.5% vs progression
4.0% in the observation group
At 5 years, the cum ulative risk of developing POAG Re s u lt s
was 4.4% in the m edicine group vs 9.5% in the
observation group 6-year data
53% of patients progressed
Treatm ent reduced the IOP on average 5.1 m m Hg (25%)
Co n c lu s io n s
Progression was less com m on in the treatm ent group
Topical ocular hypotensive m edication is effective (45% vs 62%) and occurred later
in delaying or preventing POAG in eyes with Each 1 m m Hg of IOP lowering from baseline to the first
ocular hypertension follow-up visit (3 m onths) reduced the risk of
Predictive factors for developing POAG include progression by approxim ately 10%
baseline IOP, age, cup-to-disc ratio, and central Increased n uclear lens opacity occurred with
corneal thickness (CCT 555 µm ) treatm ent

278
 Review Questions

2. Which of the following statem ents is true? Uveoscleral

Co n c lu s io n s outflow is
Treatm ent of early glaucoma halves the risk of progression a. inversely proportional to intraocular pressure
Risk factors for progression included higher baseline b. m easured clinically by fluorophotom etry
IOP, exfoliation, bilateral disease, older age, and c. increased by atropine
frequent disc hem orrhages d. responsible for about 10% of total outflow
3. Risk factors for angle-closure glaucom a include all of the
Gla uc o m a La s e r Tria l (GLT) following except
a. pseudoexfoliation
b. m yopia
Ob je c t ive : to evaluate the efficacy and safety of starting
c. Eskim o ancestry
treatm ent for POAG with ALT vs topical m edication
d. nanophthalm os
(Tim optic 0.5% bid)
4. Which of the following would cause the greatest elevation
Re s u lt s in IOP?
a. blinking
Eyes treated initially with ALT had lower IOP and better b. decreased blood cortisol levels
VF and optic nerve status than fellow eyes treated c. change from supine to sitting position
initially with topical m edication d. darkening the room
5. The m ost likely cause of a large filtering bleb and a shallow
Co n c lu s io n s cham ber is
Initial treatm ent of POAG with ALT is at least as a. aqueous m isdirection
efficacious as initial treatm ent with Tim optic b. bleb leak
c. pupillary block
d. overfiltration
Co lla b o ra tive No rm a l Te ns io n 6. A change in Goldm ann visual field stim ulus from I4e to
Gla uc o m a Stud y (CNTGS) II4e is equivalent to
a. 1 log
Ob je c t ive : to evaluate whether IOP is a causative factor b. 2 log
in NTG c. 3 log
d. 4 log
The goal in the treatm ent group was to reduce the IOP by 7. An Am sler grid held at 33 cm m easures approxim ately
30% with m edication, laser, and/or surgery how m any degrees of central vision?
a. 5
Re s u lt s b. 10
Lowering IOP by 30% or m ore reduced the rate of visual c. 20
field loss in NTG. However, the rate of progression d. 30
without treatm ent is variable and usually slow since 8. The m ost decreased sensitivity in an arcuate scotom a
half of untreated patients showed no progression in occurs in which quadrant?
5 years a. inferotem poral
Factors that increase the rate of progression include: b. superonasal
fem ale gender, m igraine headaches, and presence of c. superotem poral
disc hem orrhage d. inferonasal
9. The best gonioscopy lens for distinguishing appositional
Co n c lu s io n s from synechial angle closure is
a. Goldm ann 3-m irror
IOP is a factor in the pathogenesis of NTG and lowering
b. Zeiss
the IOP by 30% is beneficial
c. Koeppe
d. Goldm ann 1-m irror
10. Which is not a risk factor for POAG?
a. m yopia
b. CVO
REVIEW QUESTIONS (Answe rs start on page 386) c. diabetes
d. CRAO
1. What is the m ost appropriate initial treatm ent of pupillary 11. ALT would be m ost effective in a patient with which type
block in a patient with m icrospherophakia? of glaucom a?
a. acetazolam ide a. congenital
b. laser iridotom y b. inflam m atory
c. pilocarpine c. pigm entary
d. cyclopentolate d. aphakic

279
CHAPTER 9 • Glaucom a

12. In which direction should a patient look to aid the 22. The location of the greatest resistance to aqueous outflow is
exam iner’s view of the angle during Zeiss gonioscopy? a. corneoscleral m eshwork
a. up b. uveal m eshwork
b. toward the m irror c. juxtacanalicular connective tissue
c. away from the m irror d. Schlem m ’s canal
d. down 23. Facility of aqueous outflow is best m easured by
13. The best param eter for determ ining the unreliability of a a. tonography
Hum phrey visual field is b. m anom etry
a. fixation losses c. tonom etry
b. false-positives d. fluorophotom etry
c. false-negatives 24. A patient recently had an acute angle closure attack in the
d. fluctuation right eye. What is the m ost appropriate treatm ent for her
14. Which of the following does not cause angle-closure left eye?
glaucom a? a. synechiolysis
a. ICE syndrom e b. laser peripheral iridotom y
b. PHPV c. laser iridoplasty
c. RD d. pilocarpine
d. choroidal effusion 25. The m ost likely gonioscopic finding in a patient with
15. Which of the following is least likely to cause increased glaucom a and radial m idperipheral spoke-like iris
IOP 2 days postoperatively? transillum ination defects is
a. retained viscoelastic a. concave peripheral iris
b. red blood cells b. anterior iris insertion
c. m acrophages c. peripheral anterior synechiae
d. steroid drops d. plateau iris configuration
16. Treatm ent of m alignant glaucom a m ay include all of the 26. A 60-year-old m yope with early cataracts and enlarged
following except cup-to-disc ratios of 0.6 OU is found to have an abnorm al
a. laser iridotom y Hum phrey visual field test OS. He has no other risk factors
b. pilocarpine for glaucom a. What is the m ost appropriate next step for
c. atropine this patient?
d. vitrectom y a. repeat visual fields
17. The m ost com m on organism associated with bleb-related b. diurnal curve (serial tonom etry)
endophthalm itis is c. m onocular trial of latanoprost
a. Streptococcus species d. laser trabeculoplasty
b. S. epidermidis 27. Bilateral scattered PAS in an elderly hyperope with no past
c. H. influenzae ocular history is m ost likely due to
d. Gram -negative organism s a. ICE syndrom e
18. Which visual field defect is least characteristic of b. uveitis
glaucom a? c. chronic angle-closure glaucom a
a. paracentral scotom a d. Axenfeld’s anom aly
b. nasal defect 28. According to the CIGTS 5-year results, initial treatm ent of
c. central scotom a POAG with which two m ethods had sim ilar visual field
d. enlarged blind spot outcom es?
19. The type of tonom eter m ost greatly affected by scleral a. m edicine or laser trabeculoplasty
rigidity is b. m edicine or trabeculectom y
a. Goldm ann c. laser trabeculoplasty or trabeculectom y
b. tonopen d. trabeculectom y or drainage im plant
c. Schiøtz 29. Which of the following m edications should not be used to
d. pneum otonom eter treat a patient with HSV keratouveitis and elevated IOP?
20. As com pared with plasm a, aqueous has a higher a. pilocarpine
concentration of b. tim olol
a. calcium c. brim onidine
b. protein d. dorzolam ide
c. ascorbate 30. A patient undergoes m ultiple subconjunctival injections
d. sodium of 5-FU after glaucom a filtration surgery. The m ost
21. The rate of aqueous production per m inute is com m on reason for discontinuing these injections is if the
approxim ately patient develops toxicity of which tissue?
a. 0.26 µL a. lens
b. 2.6 µL b. sclera
c. 26 µL c. cornea
d. 260 µL d. conjunctiva

280
 Suggested Readings

31. A patient with retinoblastom a develops glaucom a. The 38. The m ost likely risk factor for phacomorphic glaucoma in a
m ost likely m echanism is patient with brunescent cataracts and narrow angles is
a. secondary angle closure a. traum a
b. uveitic b. pseudoexfoliation syndrom e
c. neovascular c. pigm ent dispersion syndrom e
d. tum or cell d. hyperopia
32. Glaucom a due to elevated episcleral venous pressure 39. A patient with an anterior cham ber IOL presents with
occurs in all of the following except ciliary block. Exam shows a patent iridectom y. Which of
a. carotid-cavernous fistula the following is the m ost appropriate treatm ent option?
b. hyphem a a. topical cycloplegic
c. Sturge-Weber syndrom e b. topical beta-blocker and carbonic anhydrase inhibitor
d. thyroid-related ophthalm opathy c. laser iridoplasty
33. The earliest color deficit in glaucom a is loss of d. anterior vitrectom y
a. yellow-green axis 40. A m echanic presents one week after an eye injury with
b. red-green axis decreased vision, redness, and eye pain. Exam reveals a
c. red-blue axis self-sealing corneal laceration, elevated IOP, and white
d. blue-yellow axis fluffy m aterial in the anterior cham ber. The m ost likely
34. Blood in Schlem m ’s canal is not associated with diagnosis is
a. Fuchs’ heterochrom ic iridocyclitis a. phacolytic glaucom a
b. thyroid-related ophthalm opathy b. lens-particle glaucom a
c. hypotony c. phacom orphic glaucom a
d. Sturge-Weber syndrom e d. ghost cell glaucom a
35. According to the OHTS conclusions, a predictive factor for
developing POAG is a central corneal thickness of less than
or equal to how m any m icrons? SUGGESTED READINGS
a. 565
b. 555 Allingham , R. R., & Moroi, S. E. (2010). Shields textbook of glaucoma
c. 545 (6th ed.). Philadelphia: Lippincott William s and Wilkins.
Anderson, D. R., & Patella, V. M. (1999). Automated static perimetry
d. 535
(2nd ed.). St Louis: Mosby.
36. Which index on Hum phrey visual field testing is
Basic and Clinical Sciences Course. (2016). Section 10: Glaucoma.
m ost helpful for determ ining progression of visual San Francisco: AAO.
field loss? Choplin, N. T., & Traverso, C. E. (2014). Atlas of glaucoma (3rd ed.).
a. false-positive Boca Raton: CRC Press.
b. m ean deviation Higginbotham , E. J., & Lee, D. A. (2003). Clinical guide to glaucoma
c. pattern standard deviation management. Am sterdam : Butterworth-Heinem ann.
d. short-term fluctuation Netland, P. A., & Mandal, A. K. (2006). Pediatric glaucoma.
37. Which optic disc finding is m ost likely to indicate Philadelphia: Elsevier Butterworth-Heinem ann.
progression of glaucom a? Ritch, R., Shields, M. B., & Krupin, T. (1996). The glaucomas (2nd ed.).
St Louis: Mosby.
a. peripapillary atrophy
Weber, J., & Caprioli, J. (2000). Atlas of computerized perimetry.
b. focal notch
Philadelphia: WB Saunders.
c. pallor Zim m erman, T. J., & Kooner, K. S. (2001). Clinical pathways in glaucoma.
d. splinter hem orrhage New York: Thiem e Medical.

281
This pa ge inte ntiona lly le ft bla nk
10 Anterior Segm ent
IRIS, CILIARY BODY, AND ANTERIOR CHAMBER (AC) ANGLE
LENS

IRIS, CILIARY BODY, AND ANTERIOR Fin d in gs: layer of blood and/or clot with suspended RBCs in
AC; called microhyphem a if only suspended cells; dam age to
CHAMBER (AC) ANGLE other structures m ay be seen

Diagn o sis: sickle prep, consider hem oglobin

Ana to m y electrophoresis, rule out ruptured globe, gonioscopy
(wait 4–6 weeks in traum atic cases)
Lim b u s
Tr eatm en t: cycloplegic, topical steroid, control IOP (avoid
Transition zone between cornea and sclera; 1–2 m m wide m iotics); consider Am icar (am inocaproic acid), protective
Conjunctiva and Tenon’s capsule are fused over this area; shield; elevate head, bed rest, no aspirin-containing products,
contains corneal epithelial stem cells, goblet cells, lym phoid control system ic BP, antiem etics (if needed); m ay require
cells, Langerhans’ cells, m ast cells surgery (AC washout)
Indications for surgical intervention: large hyphema that
Defin itio n s: persists for longer than 10 days, total hyphema that
Anatomist’s: term ination of Descem et’s and Bowm an’s persists for longer than 5 days, corneal blood staining,
m em branes uncontrolled IOP, 8-ball hyphema; best to perform
Pathologist’s: anterior lim bus corresponds to term ination washout 4–7 days after injury (clot has time to solidify,
of Bowm an’s and Descem et’s m em branes, and posterior reducing rate of rebleed)
lim bus corresponds to line between iris root and
Schlem m ’s canal Co m p licatio n s:
Surgeon’s: anterior blue zone (1 m m ) and posterior white 8-Ball hyphema: hyphem a that has clotted and taken on a
zone (1 m m ); a perpendicular incision through the black or purple color because of im paired aqueous
conjunctival insertion enters the AC through Descem et’s circulation and deoxygenated blood, which prevents
m em brane; a perpendicular incision through the resorption; total hyphem a is differentiated from 8-ball
posterior aspect of the lim bus will enter the AC through hyphem a by retention of bright red color, which
the trabecular m eshwork (Fig. 10-1) indicates aqueous circulation
Corneal blood staining: passage of erythrocyte breakdown
Cilia r y b o d y a n d a n g le s t ru c t u r e s products into stroma, creates yellow-brown discoloration;
occurs in about 5%, especially with recurrent hemorrhage,
(See Chapter 9, Glaucom a) compromised endothelial cell function, large hyphemas
that remain for extended periods, elevated IOP
Recurrent hemorrhage: usually larger than initial hyphem a;
Dis o rd e rs incidence 5–35% with greatest risk at 2–5 days
Secondary Glaucoma:
Tra u m a EARLY: due to TM obstruction, pupillary block by clot,
hem olytic, steroid-induced
Hyp h e m a LATE: due to angle recession, ghost cell, PAS form ation,
Blood in anterior cham ber posterior synechiae with iris bom be
Central retinal artery occlusion
Etio lo gy: traum a, surgery (incisional or laser), spontaneous
(neovascularization, Fuchs’ heterochrom ic iridocyclitis, Sickle cell an d h yp h em a: higher elevation in IOP (sickled
intraocular tum ors, juvenile xanthogranulom a), clotting RBCs cannot pass through TM), and higher risk of central
abnorm alities (leukem ia, hem ophilia, anem ia, coum adin, retinal artery occlusion and optic nerve infarction due to
aspirin, ethanol) vascular sludging

283
CHAPTER 10 • Anterior Segm ent

Ante rior
limba l borde r

Blue zone

White zone

P os te rior Te rmina tion of

limba l Bowma n's me mbra ne
borde r

Te rmina tion of
De s ce me t's me mbra ne
S cle ra l (S chwa lbe 's line )
s pur

Tra be cula r
me s hwork

S chle mm's
ca na l

Iris root

Fig ure 10-1. Anatomy of the surgical limbus.

Factors that increase sickling: acidosis, hypoxia, Cyclo d ialysis: separation of ciliary body from scleral spur;
hem oconcentration allows free passage of aqueous into suprachoroidal space; can
Ocular hypotensive medications in sickle cell: β-blockers are result in hypotony; treat with atropine, laser, and/or
safe; avoid CAIs (increases concentration of ascorbic acid surgical repair
in aqueous, decreasing pH and leading to sickling),
epinephrine and α-agonists (cause vasoconstriction with Op e n g lo b e / in t ra o c u la r fo re ig n b o d y
subsequent deoxygenation and sickling); hyperosm otics
(lead to hem oconcentration with vascular sludging and Full-thickness defect in cornea or sclera
sickling) Usually due to traum a (penetrating or blunt), m ay also occur
from m elt (chem ical injury, autoim m une disorder, infection)
Iris a n d a n g le t ra u m a (Fig. 10-2)
Pen etr atio n : entrance wound only

Ir is sp h in cter tear : sm all tear at pupillary m argin; Per fo r atio n : entrance and exit wounds (double penetrating
asym ptom atic injury)
Risk of infectious endophthalm itis (3–7%) increases if
Tr au m atic m yd r iasis: dilated, poorly reactive pupil; m ay retained foreign body, delayed surgery (> 24 hours), rural
cause glare or photophobia; consider cosm etic contact lens or setting (soil contam ination), and lens disruption
surgical repair
S. aureus is the most com m on organism to cause post-traum atic
endophthalm itis; Bacillus cereus is also com m on and causes
Ir id o d ialysis: tear in iris root; consider cosm etic contact severe dam age
lens or surgical repair if large or sym ptom atic
Fin d in gs: wound with positive Seidel test, hem orrhage
(conjunctiva, hyphem a, vitreous, retina), decreased IOP,
An gle r ecessio n : tear in anterior face of ciliary body shallow or flat AC, peaked pupil (toward wound), cataract,
between the longitudinal and circular ciliary m uscles; m ost retinal tear/detachm ent, prolapsed intraocular contents (uvea,
com m on source of hem orrhage in blunt traum a, occurs in vitreous), foreign body (sm all high-velocity FB m ay cause self-
m ore than 60% of hyphem as; 10% of patients with greater sealing wound or interm ittently positive Seidel; iris
than 180° of angle recession will develop chronic glaucom a transillum ination defect, capsule/lens disruption)

284
 Iris, Ciliary Body, and Anterior Cham ber (AC) Angle

3. Ante rio r c iliary bo dy – ang le re c e s s io n

2. Iris bas e – irido dialys is 4. Ciliary bo dy to s c le ral s pur – c yc lo dialys is c le ft

2
4

1. Iris s phinc te r – radial te ars 5. Trabe c ular me s hwo rk – TM te ars

6
5

7. Re tina to o ra s e rrata – re tinal de tac hme nt and dialys is 6. Zo nule s – le ns s ubluxatio n and dis lo c atio n

Fig ure 10-2. Seven areas of traumatic ocular tears (shown in yellow) with the resultant findings. (From Campbell DG: Traumatic glaucoma. In Shingleton BJ , Hersh PS,
Kenyon KR [eds]: Eye trauma. St Louis, 1991, Mosby.)

Typ es o f fo r eign b o d ies: degeneration with sclerosis of vessels, retinal

Inert: glass, plastic, sand, stone, ceram ic, gold, platinum , thinning, and atrophy
silver, alum inum ERG: initial a wave increased, m ay eventually
Reactive: becom e flat
COPPER: severity of inflam m ation is directly WOOD: significant inflam m ation; plant m atter has
proportionate to am ount of copper in foreign body higher risk of endophthalm itis
(! 85% copper causes severe endophthalm itis; < 85%
causes chalcosis; < 70% is relatively inert) Tr eatm en t: rule out FB with CT or MRI (contraindicated for
CHALCOSIS: m ild intraocular inflammation, m etallic FB); surgical exploration and repair, rem ove reactive
deposition of copper in anterior lens capsule m aterial as soon as possible
(sunflower cataract) and Descemet’s membrane Prophylactic antibiotics: IV (am in oglycoside
(Kayser-Fleisher ring), retinal degeneration, iris may plus ceph alosporin or vancom ycin; consider
become green and sluggishly reactive to light clin dam ycin) and intravitreal (vancom ycin
ERG: decreased am plitude (suppressed by [1 m g/ 0.1 m L], am ikacin [0.4 m g/ 0.1 m L], or
copper ions) ceftazidim e [2.25 m g/ 0.1 m L]) reduces risk of
IRON (siderosis): iris heterochrom ia (hyperchrom atic on en dophthalm itis; consider in travitreal dexam eth ason e
involved side), m id-dilated m inim ally reactive pupil, 0.4 m g/ 0.1 m L
lens discoloration (brown-orange dots from iron
deposition in lens epithelium , generalized yellowing Pr o gn o sis: variable; poor if endophthalm itis or PVR
from involvem ent of cortex), vitritis, pigm entary RPE develops (7–21 days later)

285
CHAPTER 10 • Anterior Segm ent

Co n g e n it a l Ab n o rm a lit ie s Fin d in gs: pigm ent deposits on lens capsule, anterior iris,
angle structures, and corneal endothelium (Krukenberg
(See Chapter 5, Pediatrics/Strabism us)
spindle); m idperipheral, radial, iris transillum ination defects;
Me s o d e rm a l Dys g e n e s is S yn d ro m e s pigm entary glaucom a m ay develop

(See Chapter 5, Pediatrics/Strabism us) Irid o c o rn e a l e n d o t h e lia l (ICE) s yn d ro m e

Ot h e r Dis o rd e rs Nonhereditary, progressive abnorm ality of corneal
endothelium
Co re c t o p ia
Abnorm al corneal endothelium grows across angle and iris,
Displacem ent of pupil
producing m em brane that obstructs trabecular m eshwork,
Isolated or associated with Axenfeld-Rieger syndrom e, ICE distorts iris, and m ay contract around iris strom a to form
syndrom e, uveitis, traum a, or ectopia lentis et pupillae nodules
Unilateral, m ostly wom en, occurs during m iddle age
Iris h e t e ro c h ro m ia
Iris of different colors Fin d in gs: fine, beaten m etal appearance of endothelium ;
secondary angle-closure glaucom a m ay develop due to angle
May be congenital or acquired, unilateral (heterochrom ia
endothelialization and PAS
iridis) or bilateral (heterochrom iairidum )
Syn d r o m es: com m on features of iris distortion, corneal
DDx: edem a, secondary angle-closure glaucom a
Congenital: Iris nevus (Cogan-Reese) syndrome: flattening and
INVOLVED IRIS HYPOCHROMIC: Horner’s syndrom e, effacem ent of iris strom a, pigm ented iris nodules
Waardenburg’s syndrom e, Hirschsprung’s disease, (pseudonevi) com posed of norm al iris cells that are
Parry-Rom berg hem ifacial atrophy bunched up from the overlying m em brane, corectopia,
INVOLVED IRIS HYPERCHROMIC: ocular or oculoderm al ectropion uveae
m elanocytosis, iris pigm ent epithelium ham artom a Chandler’s syndrome: corneal edem a often with norm al
Acquired: IOP, m ild or no iris changes (m inim al corectopia, iris
INVOLVED IRIS HYPOCHROMIC: Horner’s syndrom e, atrophy, peripheral anterior synechiae)
Fuchs’ heterochrom ic iridocyclitis, iris atrophy, Essential iris atrophy: proliferating endothelium produces
m etastatic carcinom a, juvenile xanthogranulom a broad PAS, corectopia, ectropion uveae, and iris holes
INVOLVED IRIS HYPERCHROMIC: iris nevus or (stretch holes [area away from m axim al pull of
m elanoma, ICE syndrome, rubeosis, siderosis, endothelial m em brane is stretched so thin that holes
hemosiderosis, medication (prostaglandin analogues) develop] and m elting holes [holes in areas without iris
thinning due to iris ischem ia])
Tr eatm en t: rule out tum or or intraocular foreign body
Path o lo gy: growth of endothelium and Descem et’s
Ru b e o s is irid is m em brane over trabecular m eshwork and onto iris
Neovascularization of the iris
DDx: posterior polym orphous dystrophy, m esoderm al
Etio lo gy: ischem ia (usually proliferative diabetic dysgenesis syndrom es
retinopathy [PDR], CRVO, carotid occlusive disease; also
CRAO, sickle cell retinopathy, anterior segm ent ischem ia), Irid o s c h is is
tum or, uveitis, chronic RD. Separation of iris strom a due to senile changes
Bilateral; onset in 6th-7th decade of life
Fin d in gs: abnorm al iris vessels; perform gonioscopy to Glaucom a in 50%
assess presence of angle neovascularization; m ay have elevated
IOP (neovascular glaucom a [NVG]) Iris No d u le s
Bru s h fie ld ’s s p o t s
Tr eatm en t: often requires panretinal photocoagulation
(PRP) for ischem ia; NVG often needs glaucom a drainage (See Chapter 5, Pediatrics/Strabism us)
im plant for IOP control
J u ve n ile xa n t h o g ra n u lo m a (J XG)
P ig m e n t d is p e rs io n s yn d ro m e
(See Chapter 5, Pediatrics/Strabism us)
Pigm ent liberation from posterior iris surface (due to contact
with zonules)
Ep it h e lia l in va s io n , s e ro u s c ys t , im p la n t a t io n
More com m on in young Caucasian m ales m e m b ra n e , s o lid o r p e a rl c ys t
Associated with m yopia and lattice degeneration (20%) Serous or solid cysts after surgery or injury

286
 Iris, Ciliary Body, and Anterior Cham ber (AC) Angle

Ko e p p e n o d u le s Fin d in gs su ggestive o f m elan o m a: growth (only 6.5%

of iris m elanom as enlarge over 5-year period), spontaneous
Along pupillary border in granulom atous uveitis
hyphem a, large size, vascularity, ectropion uveae, iris
Path o lo gy: inflam m atory cells and debris heterochrom ia, elevated IOP, angle involvem ent, glaucom a,
sector cataract
Bu s a c c a n o d u le s
On anterior surface of the iris in granulom atous uveitis
Path o lo gy: low-grade spindle B cells; epithelioid cells
are rare
Path o lo gy: inflam m atory cells and debris
Tr eatm en t: resection (sector iridectom y), m ay require
Be rlin n o d u le s enucleation if extensive, radiotherapy (plaque or proton beam )

In anterior cham ber angle in granulom atous uveitis Pr o gn o sis: 3% m ortality; 2–10% overall risk of m etastasis
(associated with tum or extension into TM, glaucom a, older
Path o lo gy: inflam m atory cells and debris
age, poorly defined m argins)
Iris n e vu s s yn d ro m e (C o g a n -Re e s e )
Tu m o rs o f iris p ig m e n t e p it h e liu m
(See above)
Adenom a (benign) or adenocarcinom a (m alignant)
Iris Tu m o rs Rare com pared with m elanom as
Perform transillum ination to differentiate cyst from
solid tum or Deeply pigm ented, circum scribed or m ultinodular m ass; can
cause secondary glaucom a by involvem ent of angle
Fre c k le
Tr eatm en t: chem otherapy, radiation, or excision
No distortion of iris architecture

Ne vu s Lis c h n o d u le s
Localized or diffuse variably pigm ented lesion of strom a; Benign, tan, iris ham artom as; associated with
obscures crypts neurofibrom atosis type 1

Path o lo gy: usually low-grade spindle cells Me t a s t a s is

Sm all risk of transform ation into m elanom a ($ 5% of
Most com m only, breast, lung, lym phom a
suspicious iris lesions grow during first 5 years after detection)
Fin d in gs: fluffy, friable iris m ass; m ay have pseudohypoyon,
Me la n o c yt o s is anterior uveitis, hyphem a, rubeosis, and glaucom a
Co n gen ital o cu lar : usually unilateral with diffuse iris
nevus causing iris heterochrom ia Le io m yo m a

Ocu lo d er m al (nevus of Ota): ocular plus periorbital skin Well localized or pedunculated, often diffuse and flat
involvem ent
Path o lo gy: very sim ilar to am elonotic spindle cell
Me la n o c yt o m a m elanom a
Form of nevus; darkly pigm ented, very discohesive (like black,
wet sand) Le u ke m ia
May have necrotic center Rare nodular or diffuse m ilky lesions with intense hyperem ia
5% risk of m elanom a
Fin d in gs: iris thickening with loss of norm al architecture;
Ma lig n a n t m e la n o m a iris heterochrom ia and pseudohypopyon

5% of ocular m elanom as
Cys t ic le s io n s
Elevated, vascular, darkly pigm ented or am elanotic lesion;
usually located inferiorly Ir is p igm en t ep ith eliu m ( IPE) : can be AD; flocculi
around pupillary m argin; associated in certain fam ilies with
Can be diffuse (appearing as iris heterochrom ia) and aortic dissection; m idperipheral cysts, peripheral cysts (usually
associated with glaucom a, localized, annular, or tapioca (dark clear; can push iris forward)
tapioca appearance)
Com m on in Caucasians with light irides Str o m a: congenital or acquired (after traum a or surgery)

287
CHAPTER 10 • Anterior Segm ent

Cilia ry Bo d y Tu m o rs prolifera tive ca pa city

increa ses
a nterior
Me la n o m a
pregermina tive epithelia l centra l pregermina tive
10% of ocular m elanom as zone cells zone cortex zone
germina tive germina tive
Pigm ented m ass zone zone

May be very large before detection, producing lenticular

equa tor equa tor
astigm atism , cataract, or shallow AC; m ay have classic sentinel
vessel or nodule of extrascleral extension
tra nsitiona l tra nsitiona l
Path o lo gy: spindle, epithelioid, or m ixed cells zone zone

embryonic nucleus ca psule bow

Tr eatm en t: local resection, XRT, enucleation feta l nucleus posterior
infa ntile nucleus
Pr o gn o sis: poorer than choroidal m elanom a because a dult nucleus
usually diagnosed at later stage; risk of m etastasis is 25%
Fig ure 10-3. Typical photographic procedure. (From Mandava N, Reichel E,
at 5 years and 34% at 10 years Guyer D, et al: Fluorescein and IGC angiography. In Yanoff M, Duker J S [eds]:
Ophthalmology, London, 1999, Mosby.)
Cilia r y b o d y le io m yo m a
Sim ilar to fibroid; m yogenic origin a nterior pole
14 m
Sc hwa nnom a 21 m 21 m
Looks like am elanotic m elanom a
17 m 17 m
Cilia r y b o d y a d e n o m a o r a d e n o c a rc in o m a
Arises from neuroepithelial cells
23 m 23 m
Occurs during adulthood
4 m
May m etastasize
posterior pole

Fu c h s ’ a d e n o m a (Fu c h s ’ re a c t ive h yp e rp la s ia , Fig ure 10-4. Thickness of the lens capsule. (From Saxby LA: Anatomy. In Yanoff
M, Duker J S [eds ]: Ophthalmology, London, 1999, Mosby.)
b e n ig n c ilia ry e p it h e lio m a )
Proliferation of basem ent m em brane m aterial (type IV organelles. Lens fibers do not contain nuclei except in rubella,
collagen and lam inin) and nonpigm ented ciliary body Lowe’s syndrom e, and trisom y 13. The em bryonal nucleus
epithelial cells located at ciliary crest develops by proliferation and m igration of epithelial cells from
the equator. Acute IOP elevation m ay cause patches of
Occurs in 25% of older patients
epithelium degeneration and necrosis beneath the capsule,
Rarely causes localized occlusion of anterior cham ber angle which appear as white flecks (glaukom flecken). Chronic iritis
m ay cause liquefaction of the nucleus, peripheral cortical
Me d u llo e p it h e lio m a changes, and degeneration and necrosis, as well as
(See Chapter 5, Pediatrics/Strabism us) proliferation of anterior lens epithelium

Su tu r es: upright Y suture anteriorly and inverted Y suture

LENS posteriorly represent the ends of lens fibers; sutures appear
during the 2nd m onth of gestation because of unequal growth
of new lens fibers
Ana to m y/P hys io lo g y (Figs. 10-3 and 10-4)
Zo n u les: attach from pars plicata to anterior and posterior
Len s cap su le: basem ent m em brane secreted by epithelium ; lens capsule in midperiphery (inserting more centrally on
thickest peripherally near the equator, thinnest posteriorly. anterior capsule). During the 5th month of gestation,
Thickening of lens capsule can occur with anterior cham ber nonpigmented ciliary epithelium of ciliary body secretes collagen
inflam m ation or pathologic proliferation of lens epithelium fibers that become zonules. Equatorial zonules are lost with aging

Ep ith eliu m : present anteriorly, ending just posterior to the Tu n ica vascu lo sa len tis: vascular network that surrounds
lens equator. Derived from cells of original lens vesicle. In the lens during em bryogenesis; derived from hyaloid and long
germ inative zone, just anterior to equator, lens epithelial cells ciliary arteries. Rem nants of tunica vasculosa lentis include
divide, elongate, and differentiate into lens fibers. During this Mittendorf’s dot, epicapsular star, persistent pupillary
transform ation, epithelial cells lose their nuclei and m ost m em brane, and capsular whorls

288
 Lens

Pr o p er ties o f th e cr ystallin e len s: Ca t a ra c t s

Composed of proteins: highest protein content in body
(up to 60% wet weight and m ost of dry weight)
Co n g e n it a l c a t a ra c t s
WATER SOLUBLE:
ALPHA CRYSTALLINS: largest of all water-soluble pro- (See Chapter 5, Pediatrics/Strabism us)
teins, com posed of 4 subunits; approxim ately 35%
of lens protein by weight; involved in transform ing
epithelial cells into lens fibers Ac q u ire d c a t a r a c t s
BETA CRYSTALLINS: m ost abundant lens protein
by weight; com posed of 3 subunits; approxim ately Classified by location or etiology
55% of lens protein by weight; first appears in
cortex
GAMMA CRYSTALLINS: sm allest lens protein Co r tical: lens fiber fragm ents, degenerated protein,
WATER INSOLUBLE: liquefaction
MAIN INTRINSIC POLYPEPTIDE: correlates with nuclear Spokes and vacuoles: radial lines and dots (Fig. 10-5)
brunescence Mature: com pletely white
Potassium-rich tissue: due to Na+/K+ pum ps found on Hypermature: leakage of degenerated cortical m aterial,
lens epithelial cells, which pum p Na+ out and K+ in; wrinkled capsule; m ay have calcium deposits
concentrations of Na + and K+ in aqueous are Morgagnian: total liquefaction of lens cortex; nucleus floats
inverse to those in lens (10 Â as m uch Na + in aqueous freely within capsule; lens m aterial can leak through
as in lens) intact capsule (Fig 10-6)
Grows throughout life: weight at m aturity is 3 Â that at Pathology: hydropic swelling of lens fibers; m orgagnian
birth, anterior-posterior diam eter increases from 3.5 m m globules (eosinophilic, globular m aterial between lens
at birth to 5 m m at m aturity, equatorial length fibers)
increases from 6.4 m m at birth to 9.0 m m at
m aturity; also increase in curvature with greater
refractive power
Refractive index: increase in lens curvature and thickness
with age is offset by change in gradient of refractive index
due to increased concentration of m ain intrinsic
polypeptide; associated with increased brunescence,
resulting in greater absorption of blue and violet
wavelengths, as well as UV light (aiding in retinal
protection)
Metabolism: anaerobic; 80% of glucose m etabolized by
glycolysis, 10% m etabolized by pentose phosphate
pathway, 5% reduced to sorbitol, and 5% converted to
glucuronic acid; highest m etabolic rate is in cortex;
energy is required for ion transport and glutathione
production Fig ure 10-5. Cortical cataract. (From Collin J : The morphology and visual effects
Lens fiber structure: fibers contain few intracellular of lens opacities. In Yanoff M, Duker J S [eds]: Ophthalmology, London, 1999,
organelles and no nuclei; appear hexagonal in cross Mosby.)
section
Average lens power: at birth ¼ 37 diopters (D), at age
2 years ¼ 23 D, at adulthood ¼ 20 D
Protective mechanisms against free radical damage and
oxidation: glutathione peroxidase, superoxide dism utase,
catalase, and vitam ins C and E
Accommodation: parasym pathetic fibers of CN 3
Generally accepted theory is that of von Helm holtz: ciliary
m uscle contraction causes zonules to relax, allowing the
lens to becom e m ore spherical and increasing its
focusing power; presbyopia is therefore due to loss of
lens elasticity with age

Dis o rd e rs

Co n g e n it a l An o m a lie s Fig ure 10-6. Morgagnian cataract. (From Collin J : The morphology and visual
effects of lens opacities. In Yanoff M, Duker J S [eds]: Ophthalmology, London,
(See Chapter 5, Pediatrics/Strabism us) Mosby, 1999.)

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CHAPTER 10 • Anterior Segm ent

Nu clear scler o sis: increased nuclear density, then Mercurial lentis: m ercury deposits in lens capsule
opacification occurs with aging; lenticular m yopia results from (occupational)
increased index of refraction; ”second sight” (presbyopes can Electrical: lens vacuoles in anterior m idperiphery;
often read again without spectacles) linear opacities (stellate pattern) in anterior
Cataract brunescens (poor blue discrim ination), cataract subcapsular cortex; burns > 200 volts cause conjunctival
nigrans, calcium oxalate crystals in nucleus hyperem ia, iritis, hyphema, iris atrophy, sphincter changes
Pathology: inwardly sequestered lens fibers degenerate Argon laser: blue light absorbed by yellow sclerotic
(analogous to desquam ating skin); hom ogenous loss of nucleus
cellular lam inations Nonionizing radiation: infrared causes true exfoliation
(glassblower’s cataract) with splitting and scrolling of
anterior lens capsule; ultraviolet (UV-B) causes cortical
Po ster io r su b cap su lar (PSC): posterior m igration of lens cataracts
epithelium , and bladder (Wedl) cell form ation (eosinophilic
Ionizing radiation: a threshold level of radiation is required
globular cells with nuclei; swell to 5–6 Â norm al size)
to induce cataract developm ent (PSC)
Etiology: age, traum a, steroids, inflam m ation, ionizing
Medications:
radiation, retinitis pigm entosa, atopic derm atitis,
CORTICOSTEROIDS: PSC from long-term use of any form
Werner’s syndrom e, Rothm und’s syndrom e, diabetes
of steroids, m ost com m only topical; develop in 33%
Symptoms: glare and poor vision from bright lights; affects
of patients on long-term daily dose of 10 m g; also
near vision m ore than distance vision
associated with inhaled steroids
Pathology: posterior m igration of lens epithelial cells,
MIOTICS: echothiophate iodide and dem ecarium
which swell along posterior capsule (swollen cells are
brom ide cause anterior subcapsular vacuoles in
called Wedl cells)
adults (not children)
PHENOTHIAZINES AND AMIODARONE: pigm ented
An ter io r su b cap su lar (ASC): fibrous plaque beneath deposits in anterior lens capsule; axial, spoke-like
folded anterior capsule, secreted by irritated m etaplastic configuration; dose and duration dependent
anterior epithelial cells (due to traum a or uveitis), can also BUSULFAN: PSC
occur in atopic derm atitis TAMOXIFEN: PSC
Pathology: cells surrounded by basem ent m em brane

Catar acts asso ciated with system ic d isease:

Glau ko m flecken : necrosis of lens epithelial cells due to
Atopic dermatitis: PSC and ASC shieldlike plaque
ischem ia from elevated IOP (angle closure); appears as central
Also associated with chronic keratoconjunctivitis,
subcapsular sm all white dots and flecks
keratoconus, vernal keratoconjunctivitis
Diabetes: osm otic cataract due to high glucose levels
Tr au m atic an d to xic catar acts: and aldose reductase, wh ich results in high
Contusion: petalliform or rosette pattern due to separation sorbitol levels
of lens fibers around lens sutures Snowflake cataract (punctate white subcapsular
Vossius’ring: with blunt injury, pigm ent from pupillary ruff opacities) can occur rapidly with h igh blood sugar
is im printed onto anterior lens capsule Myotonic dystrophy (AD): m apped to chrom osom e 19
Soemmering’s ring: doughnut of residual equatorial cortex; FINDINGS: characteristic presenile “Christm as tree”
after cataract surgery or traum a; can be associated with an cataract consisting of polychrom atic cortical crystals;
adherent leukom a ptosis, lid lag, light-near dissociation, m ild
Siderosis lentis: iron deposits in lens epithelium ; anterior pigm entary retinopathy; ERG – low voltage
subcapsular orange deposits; photoreceptor and RPE OTHER FINDINGS: m yotonia (bilateral facial
degeneration resulting in pigm entary retinopathy with weakness, difficulty relaxing grip, m uscle wasting),
peripheral visual field loss; iris heterochrom ia (involved testicular atrophy, frontal baldness, cardiac
iris darker) abnorm alities, m ental retardation; excessive
Chalcosis lentis: copper deposits in lens capsule; green contractility of muscles
discoloration of anterior lens capsule and cortex in Neurofibromatosis type 2: presenile PSC opacities
petaloid configuration occurs in Wilson’s disease, Werner’s syndrome: syndrom e of prem ature aging,
form ing a sunflower cataract; also Kayser-Fleischer ring scleroderm a-like m atting of skin, and bilateral PSC
(copper deposition in peripheral Descem et’s cataracts in 3rd to 4th decade of life
m em brane); can also occur in m ultiple m yelom a and Wilson’s disease (hepatolenticular degeneration): deficiency
lung carcinom a of ceruloplasm in (alpha-2 globulin)
Other intraocular foreign bodies: gold, silver, platinum , FINDINGS: green sunflower cataract due to deposition of
alum inum are inert; lead and zinc m ay cause m ild copper in anterior lens capsule and cortex in petaloid
nongranulom atous reaction configuration; Kayser-Fleischer ring (copper in
COPPER: if concentration ! 85%, an acute inflam m atory peripheral Descem et’s m em brane)
reaction occurs; if concentration between 70% and OTHER FINDINGS: cirrhosis, renal im pairm ent,
85%, chalcosis develops; lesser concentrations are degeneration of basal ganglia
relatively inert TREATMENT: penicillam ine to lower serum copper

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 Lens

Le n s Ca p s u le Ab n o r m a lit ie s DDx: traum a (m ost com m on acquired cause), Marfan’s

syndrom e, hom ocystinuria, aniridia, congenital glaucom a,
Tru e e xfo lia t io n m egalocornea, Ehlers-Danlos syndrom e, hyperlysinem ia,
Delam ination/schisis of anterior lens capsule, form ing scrolls; sulfite oxidase deficiency, hereditary ectopia lentis (AD > > AR;
often due to infrared radiation (glassblowers), can be an aging bilateral; m ay not present until 3rd–5th decade), ectopia lentis
change; not associated with glaucom a et pupillae (AR; lens and pupil are displaced in opposite
directions; bilateral, asym m etric), tertiary syphilis, Weill-
P s e u d o e xfo lia t io n s yn d ro m e (P XS ) Marchesani syndrom e, m edulloepitheliom a, Stickler’s
syndrom e, pseudoexfoliation (rare)
Material produced by lens epithelial cells and extruded through
lens capsule
Ma rfa n ’s s yn d ro m e (AD)
Appears to be an ocular sign of system ic elastosis; also found in
conjunctiva, skin, lung, and liver Mapped to chrom osom e 15q; defect in fibrillin (elastic
m icrofibrillar glycoprotein, m ajor constituent of zonules);
Usually elderly, Caucasian fem ales; increased incidence in 15% have no fam ily history
Scandinavians and with increasing age
Fin d in gs: ectopia lentis (65%; usually superotem poral);
Fin d in gs: white fibrillar m aterial on anterior lens capsule, glaucom a, keratoconus, cornea plana, axial m yopia, retinal
iris, ciliary body, zonules, anterior vitreous with characteristic degeneration (salt and pepper fundus), high risk of retinal
pattern of deposition on lens surface (central disc, clear zone, detachm ent
then peripheral annulus, often with bridging strands to central
disc); m ay see flakes on pupillary m argin; Sam paolesi’s line
(pigm ent band anterior to Schwalbe’s line) on gonioscopy; up Oth er fin d in gs: tall stature, spidery digits, arm span is
to 50% develop glaucom a (PXG); poor dilation due to iris larger than height, cardiac disease, dissecting aneurysm of
m uscle degeneration or lack of iris strom al elasticity; the aorta
peripupillary transillum ination defects; weak zonules
(phacodonesis, increased incidence of angle closure, lens Ho m o c ys t in u ria (AR)
subluxation, and com plications during and after cataract
Deficiency of cystathionine β-synthase, which converts
surgery [vitreous loss, IOL and capsular dislocation])
hom ocysteine to cystathionine; m ethionine and hom ocysteine
accum ulate; zonules are deficient in cysteine and weakened
P o s t e rio r c a p s u la r o p a c ific a t io n (P CO;
because of reduced sulfhydryl cross-linkage; degeneration of
s e c o n d a ry c a t a ra c t ) entire zonule occurs
Proliferation of residual lens epithelial cells (Elschnig’s pearls)
and fibrosis Fin d in gs: bilateral ectopia lentis (90%; usually inferonasal;
30% in infancy, 80% by age 15), enlarged globe, m yopia,
In cid en ce: up to 50% of patients within 2 years of peripheral RPE degeneration, increased risk of retinal
extracapsular cataract surgery; influenced by IOL optic m aterial detachm ent after cataract surgery, early loss of accom m odation
(acrylic< silicone < PMMA) and edge design (square due to disintegration of zonules
edge < round edge); with newer IOLs, the incidence has
decreased to $ 10%. Incidence approaches 100% in children Oth er fin d in gs: blonde, tall (m arfinoid habitus with
and patients with uveitis arachnodactyly), osteoporosis, fractures, seizures, m ental
retardation (50%), cardiom egaly, platelet abnorm ality with
Tr eatm en t: Nd:YAG laser posterior capsulotom y when hypercoagulability (risk of throm boem bolic problem s,
visually significant especially with general anesthesia); infants appear norm al at
birth; 75% m ortality by age 30 years
In young children, prim ary posterior capsulotom y and
anterior vitrectom y are perform ed at the tim e of cataract
surgery Hyp er h o m o cystin em ia o ccu r s in p atien ts
h eter o zygo u s fo r h o m o cystin em ia: high serum
hom ocysteine increases risk of arterial and venous throm bosis
Ec t o p ia Le n t is
(CRAO and CVO) and cardiovascular disease, especially in
Displacem ent of lens
type 2 diabetic patients. In nondiabetic patients younger than
55 years of age who have had a stroke or MI, 25% have elevated
Su b lu xatio n : partial displacem ent, rem ains in hom ocysteine levels (5% in norm al population)
pupillary axis
Diagn o sis: increased urinary excretion of hom ocysteine
Dislo catio n (luxation): com plete displacem ent from pupil (nitroprusside urine test), am ino acid assays; check renal
function (homocysteine levels rise with elevated creatinine
Fin d in gs: decreased vision, astigm atism , m onocular levels); subnorm al or low levels of folate are associated with
diplopia, iridodonesis, phacodenesis, m alpositioned lens elevated levels of hom ocysteine

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Tr eatm en t: vitam in B6 (folate), m ethionine-restricted diet, Co m p licatio n s: increased IOP (glaucoma patients – 17%
supplem entary cysteine (this diet can reduce lens dislocation); have rise after 2 hours; 14% with high IOP at 1 week;
certain m edications (m ethotrexate, phenytoin, nonglaucoma patients – 6% have rise after 2 hours, 3% with
carbam azepine) can elevate hom ocysteine levels by interfering high IOP at 1 week), retinal detachment (RD; increased risk with
with folate m etabolism axial length > 25 m m), cystoid m acular edema (CME), rupture of
the anterior hyaloid face, IOL dislocation (increased risk with
silicone plate IOL), iritis, pitting of IOL, corneal or retinal burn
We ill-Ma r c h e s a n i s yn d ro m e (AR)
Fin d in gs: ectopia lentis (usually inferiorly or anteriorly), Vit re o lys is
m icrospherophakia, high lenticular m yopia, cataract, Disrupt anterior vitreous face in aphakic and pseudophakic
m icrocornea, glaucom a (pupillary block) eyes with m alignant glaucom a
Power: 3–11 m J, focused on anterior hyaloid; deepening of
Oth er fin d in gs: short stature; short, stubby fingers with anterior cham ber signifies success
broad hands; hearing defects; inflexible joints; m ental Treatment: topical steroid qid  1 week and cycloplegic;
retardation vitrectom y if regim en described here fails and in
phakic eyes
Relieve CME due to vitreous wick
Eh le rs -Da n lo s s yn d ro m e (AD, AR, o r X-lin ke d ) Power: 5-10 m J bursts, often best to aim near wound
Defect in type III collagen; at least 9 types or pigmented area of incarcerated vitreous; success
occurs with a change in the pupil shape back to round
Treatment: pretreat with pilocarpine to induce m iosis and
Fin d in gs: ectopia lentis, easy lid eversion (Metenier’s sign), stretch the strand, then topical steroid  1 week
epicanthal folds, m yopia, m icrocornea, blue sclera, Ca t a ra c t S u rg e ry
keratoconus, angioid streaks, retinal detachm ent In d icatio n s: altered vision (based on patient’s functional
impairment; when patient is having visual difficulty performing
Oth er fin d in gs: hyperextensible joints and skin, poor tasks and does not achieve adequate improvement from corrective
wound healing, easy bruising lenses), also medical indications (phacolytic glaucoma,
phacomorphic glaucoma, phacoantigenic uveitis, dense cataract
S u lfit e o xid a s e d e fic ie n c y (AR) that obscures view of fundus in patients who require regular retinal
evaluation [i.e. diabetes, glaucoma])
Enzym atic defect causing m olybdenum deficiency and
increased urinary sulfite IOL c a lc u la t io n s (see Chapter 1, Optics):
Fo r m u las: rough estim ation, use IO L power (D) ¼ A
Fin d in gs: ectopia lentis (50%), enophthalm os, constant of IOLÀ 2.5 (axial length) À 0.9 (average
Brushfield’s spots keratom etry)
ACD (anterior cham ber depth) approxim ately 3.5 m m
First generation: ACD is constant
Oth er fin d in gs: seizures, m ental retardation, Second generation: ACD is based on axial length (AL)
frontal bossing Third generation (Holladay 1, Hoffer Q, SRK/T): ACD is
based on AL and keratom etry (K)
Hyp e rlys in e m ia (AR) Fourth generation (Holladay 2): variables include AL, K,
corneal diam eter, ACD, lens thickness, refraction, and
Deficiency of lysine dehydrogenase
patient age. (Haigis): ELP (effective lens position)
derived from a function rather than a single num ber
Fin d in gs: ectopia lentis, m icrospherophakia Fifth generation (Olsen, Barrett Universal II): used in
IOLMaster and Lenstar instrum ents. (Hoffer H-5): based
on Holladay 2 and racial variations
Oth er fin d in gs: growth, m otor, and m ental retardation Newer formulas (Hill-RBF): pattern reccognition algorithm.
(Ladas Super Form ula): amalgam of existing formulas
Most accurate third and fourth generation form ulas according
Surg e ry to axial length
Long eyes (AL> 26.5 m m ): SRK/T, Holladay 2, Haigis
Medium-long eyes (AL 24.5–26.5 m m ): Holladay 1 or 2
Nd :YAG La s e r Medium eyes (AL 22.0–24.5 m m ): Hoffer Q, Holladay 1 or
2, SRK/T, Haigis
P o s t e rio r c a p s u lo t o m y Short eyes (AL< 22.0 m m ): Hoffer Q, Holladay 2
Open visually significant posterior capsule opacity
Power: 1.0–2.0 m J; m ake an opening equal to the size of A scan : error of 0.1 m m ¼ 0.3 D error in lens power;
the pupil in am bient light however, the shorter the axial length, the greater the effect of
Treatment: pretreat with Iopidine (apraclonidine), then a m easurem ent error
topical steroid qid  1 week Myopia: 1.75 D error per m m of AL error

292
 Lens

Emmetropia: 2.35 D error per m m of AL error

Hyperopia: 3.75 D error per m m of AL error
Ultrasound waves travel faster through lens (1640 m /s) than
either aqueous or vitreous (1532 m /s)
To correct any AL, use the form ula:
ALcorrected ¼ ALm easured  (Vcorrect /Vm easured )

Aver age m easu r em en ts:

Axial length: 23.5 m m (22.0–24.5 m m )
Keratometry: 43.0 to 44.0 D (anterior corneal curvature)
Posterior corneal curvature: contributes approxim ately 0.4
D against-the-rule astigm atism ; m easured directly with
Scheim pflug im aging and ray-tracing technologies
Anterior chamber depth (ACD): 3.25 m m
Lens thickness: 4.6 m m
Ultrasound speeds:
PHAKIC EYE ¼1550 m /s
APHAKIC EYE ¼1532 m /s
PSEUDOPHAKIC EYE (depends on IOL m aterial and Fig ure 10-7. A-scan pattern of a phakic eye with the initial spike removed
thickness) ¼ 1556 m /s for PMMA lens, 1487 m /s for from the screen display. Identified are the 2 cornealpeaks (CIand C2), the anterior
silicone lens, and 1549 m /s for acrylic lens lens spike (L1), the posterior lens spike (L2), and the retinal spike (R). (From
More accurate m ethod for calculatin g pseudoph akic Shammas HJ : Intraocular lens power calculations. In Azar DT [ed]: Intraocular
lenses in cataract and refractive s urgery, Philadelphia, 2001, WB Saunders.)
AL is to m easure at 1532 m /s an d to use correction
factors: Ker ato m etr y in p atien t fo llo win g r efr active
PMMA: ALcorrected ¼ AL1532 + 44% IOL thickness + 0.04
su r ger y: need to determine accurate central corneal curvature;
SILICONE: ALcorrected ¼ AL1532 À 56% IOL
keratometer measures at 3 mm from center of cornea
thickness + 0.04 Methods:
ACRYLIC: ALcorrected ¼ AL1532 + 75% IOL
CLINICAL HISTORY: K¼ preopK + (refraction
thickness + 0.04
preop À refraction postop)
SILICONE OIL ¼980 to 1040 m /s (this is only for the
Convert for vertex distance: refraction (corneal
portion of the eye containing silicone); thus to find
surface) ¼ refraction (spectacle plane)/(1–0.012
the correct AL, the previous form ula can be used, or
refraction [spectacle plane])
the eye can be m easured using an average velocity:
CONTACT LENS: use plano rigid gas-perm eable contact
phakic eye with silicone oil ¼ 1140 m /s. Alternatively,
lens with known base curve
use optical biom etry (IOLMaster or Lenstar)
K¼ base curve of CL+ power + refraction (with
If silicone oil is to be left in the eye, the index of refraction
CL) À refraction (without CL)
of the oil will cause a hyperopic refraction; therefore,
Convert for vertex distance
adjust the IOL power by adding 3.0 to 3.5 D
CORNEAL TOPOGRAPHY: use central 1 m m effective
Im m ersion technique is m ore accurate than contact
power reading from the Holladay diagnostic
because of corneal com pression (0.1–0.2 m m ) for the
sum m ary m ap (not sim K readings)
later m ethod. Optical biom etry is m ost accurate
FORMULAS: num erous m ethods; online calculators
available
Co n tact A scan : 5 spikes corresponding to beam reflection Presence of cataract m ay induce m yopia, causing error in
from interfaces: cornea, anterior lens surface, posterior lens first m ethod, and m ay cause poor vision with inability to
surface, retina, sclera (Fig. 10-7) perform adequate CL overrefraction; therefore, a variety
Causes of falsely long axial length: of m ethods should be used, then take flattest K to use in
Posterior staphylom a in high m yopia; fovea m ay be IOL calculation and consider using a final IOL 1–2 D
located m ore anteriorly than the staphylom a stronger to avoid a hyperopic result
Measurem ent of the sclera rather than the retina
Wrong ultrasonic velocity (too fast) P ig g yb a c k IOL (s e c o n d IOL in s u lc u s )
Fluid m eniscus between probe and cornea
Should be different m aterial from the IOL in the bag to
Wrong gate position (for ultrasound devices in which
reduce the risk of interface m em brane form ation between
gates are m anually set for the position of the different
the two lenses, and should have round edge to reduce risk
ocular structures)
of iris chafing with possible pigm ent dispersion, elevated IOP,
Causes of falsely short axial length:
or UGH syndrom e
Excessive indentation of cornea with contact probe
Nonperpendicular m easurem ent
Hyp er o p ia: Prim ary for patient requiring IOLpower > +34 D
Choroidal thickening or effusion
Vitreous opacity Secondary for refractive surprise; correct piggyback IOL
Wrong ultrasonic velocity (too slow) power ¼1.5 Â spherical equivalent (i.e., if patient is +2.00 D,
Wrong gate position place a +3.00 D piggyback IOL).

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CHAPTER 10 • Anterior Segm ent

Myo p ia: Secondary for refractive surprise; correct piggyback affected by changes in tip occlusion; indirectly contacts
IOLpower ¼ 1.2 Â spherical equivalent (i.e., if patient is À3.00, aspiration line fluid by the induced vacuum in rigid
place a À3.50.00 D piggyback IOL) drainage cassette between line and pum p
VENTURI: com pressed air flows through tube; rapid,
precise aspiration and vacuum response; requires
Vis c o e la s t ic d e vic e (o p h t h a lm ic vis c o s u r g ic a l external air supply
d e vic e [OVD]) DIAPHRAGM: flexible m em brane m oves like a piston;
outm oded, large noisy system
Variety of clear gel-like m aterials com posed of sodium
ROTARYVANE: wheel with vanes rotates; does not require
hyaluronate and chondroitin sulfate
external air supply
Used intraocularly to m aintain and preserve space, displace Hybrid: program m able like peristaltic or Venturi
and stabilize tissue, and coat and protect corneal endothelium
Vary in m olecular weight, viscosity, clarity, ease of rem oval, Vacu u m r ise tim e: tim e for vacuum to reach preset lim it;
and potential for IOP spike if retained in eye (peak usually affected by aspiration rate; requires tip occlusion with
4–6 hours postoperatively) peristaltic pum p

Classificatio n : Po sto cclu sio n su r ge (vacuum rush): collapse of AC due to

Cohesive agent (ability to adhere to itself): long-chain, increase in aspiration rate to level of pum p speed; during
high-m olecular-weight, high surface tension (low occlusion, vacuum builds to preset level, no flow occurs, and
coating capability), high degree of pseudoplasticity tubing collapses; when occlusion breaks, pum p pulls fluid at
(ability to transform from high viscosity at rest [zero preset rate and rebound of tubing pulls additional fluid
shear rate] to lower viscosity at high shear rate) through tip. Risk reduced by lowering m axim um vacuum
ADVANTAGES: capsulorhexis, insert IOL, ease of setting; prevented by venting, rigid tubing, m icroprocessor
viscoelastic rem oval in pum p
Exa m p le s : Healon, Healon GV, Amvis c, Amvisc Plus, Provisc
Dispersive agent: short-chain, low-m olecular-weight, low
surface tension (high coating ability), low to m oderate Fo llo wab ility: ability to bring m aterial to tip; function of
pseudoplasticity flow and vacuum
ADVANTAGES: corneal coating, less IOP rise if retained,
m aintain space, m ove tissue Ho ld ab ility: ability to keep m aterial at tip; function of
Exa m p le s : Viscoat, Vitrax, Ocucoat vacuum and power
Viscoadaptive agent: ultrahigh-m olecular-weight,
superviscous and cohesive at low shear rates, m ore
dispersive at increasing shear rates; thus com bines the
Ph aco p o wer (0–100%): depends on frequency and stroke
length; surgeon (linear) or panel control; pushes m aterial away
advantages of cohesive and dispersive agents
Exa m p le : Healon 5 from tip; generates heat.
Power factors: am plitude (fixed vs linear) and duration
(continuous vs interm ittent)
P h a c o d yn a m ic s
Power modulation: pulse, burst, bim odal, dual linear
Ir r igatio n : rate of fluid delivery; determ ined by bottle height
above eye (34–110 cm ); not adjusted by m achine; depends on Ultr aso u n d effect:
aspiration rate and wound leakage; m aintains anterior Fragmentation: m echanical (jackham m er effect)
cham ber depth Cavitation: sound waves expand and com press liquid,
creating shock wave and m icrobubbles, which im plode,
Flo w (aspiration rate [AFR]): rate of fluid rem oval (0–60 m L/ form ing cavity and dissolving nucleus in front of
m in); determ ines how quickly vacuum is created; attracts needle tip
m aterial to tip
Ultr aso u n d in str u m en t:
Vacu u m (aspiration level): negative pressure created at tip by Handpiece: produces vibration of needle
pum p (0–500 m m Hg); holds m aterial onto occluded tip MAGNETORESTRICTIVE: converts m agnetic energy into
m otion
Pu m p system s: PIEZOELECTRIC CRYSTAL: converts electricity into
Flow-based: independent, direct control of AFR and m otion
vacuum ; vacuum rise requires tip occlusion; physically Needle: oscillation produces phaco power
contacts and regulates aspiration line fluid FREQUENCY: rate of oscillation (25–62 kHz)
PERISTALTIC: rollers com press tubing; AFR not sm ooth STROKE LENGTH: distance of travel (0.002–0.004 inch
because of peristaltic wave; less accurate, especially at [0.05–0.1 m m ])
higher vacuum levels BEVEL: occlusion vs cutting (0–45°)
SCROLL: scroll elem ent orbits against housing; m ore TIP SIZE/BORE: 0.9, 1.1, flared
accurate; can act clinically like Venturi pum p SHAPE: straight, angled
Vacuum-based: direct control of vacuum only; AFR INNOVATIONS: m icroflow, m icroseal, aspiration bypass
indirectly controlled by vacuum level; vacuum not (ABS), torsional, elliptical

294
 Lens

Footpedal: usual settings; can be program m ed differently Re t ro b u lb a r h e m o rrh a g e

KICK LEFT: reflux
May occur following retrobulbar injection or traum a; risk of
POSITION 0: resting
CRAO as orbital pressure rises
POSITION 1: irrigation
POSITION 2: aspiration
POSITION 3: ultrasound
Fin d in gs: m arked proptosis, rapid orbital swelling and
Audible clues: congestion, lim ited extraocular m otility, hem orrhagic
METALLIC CLICK: irrigation (plunger opens) chem osis, lid ecchym osis and edem a
HUMMING: aspiration
BUZZING: ultrasound (harm onic overtones)
BELLS: occlusion (no flow) Tr eatm en t: consider em ergent surgical decom pression with
Custom ized tones lateral canthotom y and cantholysis, hyperosm otic agents to
Alternative systems: Sonic, Fluid, Laser lower IOP

S t ra b is m u s
Co m p lic a tio ns o f Ca ta ra c t Surg e ry
Due to inferior rectus dam age
Paralysis or fibrosis result from anesthetic injection
Pr eo p er ative ( r etr o b u lb ar /p er ib u lb ar in jectio n ) :
Globe perforation
Retrobulbar hem orrhage Ce n t ra l a n e s t h e s ia fo llo w in g re t ro b u lb a r b lo c k
Strabism us (inferior rectus fibrosis or m yotoxicity)
Central anesthesia Agent spreads along m eningeal cuff of optic nerve to enter CSF
Increased risk with 4% lidocaine

In tr ao p er ative:
Wound burn Fin d in gs: m ental confusion, dysphagia, dyspnea, apnea,
Iris prolapse am aurosis of fellow eye
Iris dam age
Intraoperative floppy iris syndrom e (IFIS)
Descem et’s m em brane detachm ent Wo u n d b u rn
Capsule dam age (radial tear, posterior rupture, zonulolysis) Therm al injury from phaco needle due to lack of irrigation
Retained m aterial (nucleus, cortex)
Vitreous loss
Choroidal effusion Etio lo gy: excessively tight incision, low or em pty infusion
Suprachoroidal hem orrhage bottle, low-flow settings, occluded phaco tip (with dispersive
Phototoxicity viscoelastic agent or im paled nucleus), phaco needle without
therm al protective design

Po sto p er ative: Fin d in gs: wound whitening, distortion, and astigm atism ;
Wound leak warning sign is lens m ilk/dust (m ilky substance) near phaco tip
Inadvertent filtering bleb
Epithelial downgrowth
Corneal edem a Tr eatm en t: im m ediately stop phaco, check for incision
Corneal m elt tightness and phaco tip obstruction; scleral relaxing incision,
Toxic anterior segm ent syndrom e (TASS) horizontal suture, bandage contact lens; if severe, m ay require
Secondary glaucom a astigm atic surgery after stabilization or patch graft
Uveitis, glaucom a, hyphem a (uveitis-glaucom a-hyphem a
[UGH]) syndrom e
Sputtering hyphem a (vascularization of posterior wound Iris p ro la p s e d u rin g p h a c o
lip; m alpositioned IOL)
Pupillary capture of IOL Wound too large or too posterior, bottle too high, posterior
IOL decentration pressure, suprachoroidal hem orrhage
Capsular block syndrom e
Anterior capsular contraction syndrom e
In t ra o p e ra t ive flo p p y iris s yn d ro m e (IFIS )
Posterior capsular opacification (PCO)
Cystoid m acular edem a (CME) Altered iris dilator tone due to system ic α-1 blocker
Retinal detachm ent (RD) m edications (m ost com m only tam sulosin [Flom ax]) with
Endophthalm itis varying degrees of poor iris dilation and atonic floppy iris tissue
Mydriasis (billows and prolapses), which m ay increase the risk of
Ptosis intraoperative com plications during cataract surgery

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CHAPTER 10 • Anterior Segm ent

Tr eatm en t: one or a com bination of approaches depending To xic a n t e rio r s e g m e n t s yn d ro m e (TAS S )

on the severity of IFIS including preoperative topical atropine,
Acute, severe intraocular inflam m ation that presents within 24
intraoperative use of preservative free epinephrine, Healon 5,
hours of uncom plicated surgery with corneal edem a, anterior
pupil expansion devices (i.e., Malyugin ring, Graether
cham ber reaction, and variable pain, m ay have increased
expander, Morcher dilator), iris hooks; m anual pupil stretching
intraocular pressure
does not help. Stopping the m edication does not prevent
IFIS and m ay lead to urinary retention

De s c e m e t ’s m e m b r a n e d e t a c h m e n t Etio lo gy: form of sterile endophthalm itis due to toxicity

from noninfectious exogenous contam inant (i.e., biofilm ,
Due to inadvertent stripping of Descem et’s from instrum ent surgical instrum ent, intraocular solution)
insertion during surgery or viscoelastic injection; causes
corneal edem a or decom pensation

Tr eatm en t: rule out infectious endophthalm itis; treat with

Tr eatm en t: air or gas bubble, m ay require suture repair topical steroids, consider topical NSAID; m ay require ocular
hypotensive m edications
Exp u ls ive s u p ra c h o ro id a l h e m o rrh a g e
May occur during intraocular surgery or days later; incidence of
0.5% Uve it is -g la u c o m a -h yp h e m a (UGH) s yn d ro m e
Due to repeated traum a to angle structures and iris by IOL;
Risk facto r s: elderly patient, hypertension, atherosclerosis, scarring and degeneration occur
diabetes, sudden drop in IOP during surgery, high m yopia,
vitreous loss or previous vitrectom y, previous expulsive
hem orrhage in fellow eye, glaucom a, aphakia, postoperative
Valsalva m aneuver Tr eatm en t: atropine, topical steroids, ocular hypotensive
m edications; consider argon laser of bleeding site; usually
requires IOL explantation
Fin d in gs: forward m ovem ent of intraocular contents during
surgery and loss of red reflex (om inous sign), subretinal
hem orrhage, choroidal detachm ent, and extrusion of
intraocular contents; delayed hem orrhage presents with severe Hyp h e m a
eye pain, nausea, vom iting, and rapid loss of vision
Result of fine neovascularization of scleral incision (Swan
syndrom e) or m echanical traum a from IOL haptics
Tr eatm en t: close wound rapidly; m ay require surgical
drainage Occurs with m ore posteriorly located incisions
IOP can becom e dangerously elevated
Pr even tio n : decom press eye preoperatively, sm all wound,
control BP during surgery, prevent bucking in patients under
general anesthesia
P u p illa ry c a p t u re
Portion of IOL optic anterior to iris
P h o t o t o xic it y
Occurs in up to 39% for surgical tim e > 100 m inutes
Risk facto r s: large or incom plete capsulorhexis, sulcus
Pr even tio n : dim light, oblique light, pupil shield haptic placem ent, nonangulated IOL, upside-down IOL
placem ent, vitreous pressure
Co rn e a l e d e m a
Etio lo gy: traum a to endothelium , elevated IOP, endothelial
chem ical toxicity, Descem et’s detachm ent, decom pensation in Tr eatm en t: observe if long-standing and stable without
patient with Fuchs’ corneal endothelial dystrophy sym ptom s; dilate and place patient supine if occurs early
postoperatively; m ay require lens repositioning with
m icrohook
Co rn e a l u lc e r a t io n a n d m e lt in g
Occurs m ost com m only after cataract surgery in patients with
keratoconjunctivitis sicca and rheum atoid arthritis; m elting
IOL d e c e n t r a t io n
several weeks after uncom plicated cataract surgery due to
collagenase release from surrounding inflam m atory cells Causes diplopia or polyopia because of prism atic effect

296
 Review Questions

Su n set syn d r o m e: inferior displacem ent of IOL within Rh e g m a t o g e n o u s re t in a l d e t a c h m e n t

capsular bag; occurs with unrecognized zonular dialysis,
In cid en ce: 1–2%
capsular tears, or asym m etric IOL haptic im plantation (1 in
bag and other in sulcus)
Risk facto r s: high m yopia, lattice degeneration, history of
RD in fellow eye, vitreous loss
Su n r ise syn d r o m e: superior displacem ent of IOL; occurs
with asym m etric IOL haptic placem ent or capsular contraction Myd ria s is
Due to sphincter dam age (m echanical from iris
Tr eatm en t: consider pilocarpine; IOL repositioning or stretching or ischem ic from elevated IOP (Urrets-Zavalia
exchange m ay be required syndrom e))

Ca p s u la r b lo c k s yn d ro m e P t o s is
Due to retained viscoelastic behind IOL Due to traum a from lid speculum

Causes m yopic shift

REVIEW QUESTIONS (Answe rs start on page 388)

Tr eatm en t: Nd :YAG laser anterior capsulotom y
1. The m ost helpful test for evaluating m acular function in
a patient with advanced cataract is
An t e rio r c a p s u la r c o n t ra c t io n s yn d r o m e
a. blue field entoptic phenom enon
(c a p s u la r p h im o s is ) b. 2-light separation
Due to sm all capsulorhexis with retention of lens c. red-light discrim ination
epithelial cells d. directional light projection
2. Ectopia lentis is least likely to be associated with
a. cleft palate
Tr eatm en t: Nd :YAG laser radial anterior capsulotom ies b. pectus excavatum
c. short stature
d. m ental retardation
CME (Irvin e -Ga s s s yn d ro m e ) 3. Anterior segm ent signs of ciliary body m elanom a include
More com m on with intracapsular (ICCE) than extracapsular all of the following except
cataract extraction (ECCE) a. corneal edem a
b. increased IOP
50% angiographically for ICCE vs 15% for ECCE c. astigm atism
d. cataract
Clinically significant (visual loss) in < 1% of patients
4. Which of the following is least characteristic of ICE
with ECCE
syndrom e?
a. corneal edem a
Risk facto r s: capsular rupture, dislocated IOL, iris- b. increased IOP
supported IOL, iris tuck, vitreous adhesion to pupil or wound; c. ectropion uveae
also patients with diabetes, epiretinal m em brane, and retinal c. PAS
vein occlusion 5. A stellate anterior subcapsular cataract is m ost likely to be
found in a patient with
a. Fabry’s disease
FA: petalloid hyperfluorescence, late leakage; late staining of b. atopic derm atitis
optic nerve helps to differentiate postoperative CME from c. m yotonic dystrophy
other form s of CME d. electrical injury
6. Which of the following does not occur in siderosis bulbi?
a. glaucom a
OCT: increased foveal thickness; intraretinal cystoid spaces b. retinal atrophy
c. sunflower cataract
d. fixed pupil
Tr eatm en t: topical steroid and NSAID, consider posterior 7. A patient has a history of increased IOP with exercise;
sub-Tenon’s steroid injection with kenalog which finding is associated with this condition?
a. Krukenberg spindle
b. PAS
En d o p h t h a lm it is
c. phacodenesis
(See Chapter 8, Uveitis) d. pars plana snowbank

297
CHAPTER 10 • Anterior Segm ent

8. Separation between the longitudinal and circum ferential 18. After finishing phacoem ulsification on a dense cataract,
fibers of the ciliary m uscle is called the surgeon notes whitening of the clear corneal
a. iridoschisis incision. The m ost likely cause is
b. angle recession a. tight incision
c. iridodialysis b. high aspiration flow rate
d. cyclodialysis c. high phaco power
9. Characteristics of pigm ent dispersion syndrom e include d. 45° bevel phaco needle
all of the following except 19. Nuclear brunescence increases with higher concentrations
a. increased IOP with exercise of which lens protein?
b. Krukenberg’s spindle a. alpha crystallin
c. phacodenesis b. betacrystallin
d. radial iris transillum ination defects c. gam m a crystallin
10. A sickle cell patient with a hyphem a develops d. m ain intrinsic polypeptide
increased IOP; which of the following treatm ent choices 20. Lens fibers contain nuclei in all of the following
is best? conditions except
a. pilocarpine a. trisom y 13
b. Diam ox b. syphilis
c. hyperosm otic c. rubella
d. Tim optic d. Lowe’s syndrom e
11. Of the following causes of iris heterochrom ia, the involved 21. The m ost likely cause of an intraoperative com plication
iris is hyperchrom ic in during cataract surgery in a patient with pseudoexfoliation
a. ICE syndrom e syndrom e is
b. Horner’s syndrom e a. sm all pupil
c. Waardenburg’s syndrom e b. thin posterior capsule
d. Fuchs’ heterochrom ic iridocyclitis c. weak zonules
12. Which of the following iris lesions is a true tum or? d. shallow anterior cham ber
a. Kunkm ann-Wolffian body 22. The m ajority of glucose m etabolism in the lens is by
b. Koeppe nodule a. glycolysis
c. Lisch nodule b. pentose phosphate pathway
d. juvenile xanthogranulom a c. reduction to sorbitol
13. At which location is the lens capsule thinnest? d. conversion to glucuronic acid
a. anterior capsule 23. The m ost appropriate system ic treatm ent for a patient
b. posterior capsule with a sunflower cataract is
c. equatorial capsule a. insulin
d. anterior paracentral capsule b. penicillam ine
14. Which of the following is not associated with sunset c. steroids
syndrom e? d. m ethotrexate
a. asym m etric IOL haptic placem ent 24. Which of the following is the least likely cause of
b. polyopia decreased vision 2 years after cataract surgery?
c. pseudoexfoliation a. subluxed IOL
d. hyphem a b. retinal detachm ent
15. Lens epithelial cells differentiate into lens fibers c. posterior capsular opacity
a. anterior to the equator d. cystoid m acular edem a
b. at the equator 25. Which type of cataract is m ost closely associated with
c. posterior to the equator UV-B exposure?
d. in the fetal nucleus a. anterior subcapsular
16. Light of which wavelength is absorbed greatest by a b. cortical
dense nuclear sclerotic cataract? c. nuclear sclerotic
a. red d. posterior subcapsular
b. green 26. Which of the following strategies is least likely to be
c. yellow effective for treating IFIS?
d. blue a. atropine
17. A patient with background diabetic retinopathy and b. Healon 5
clinically significant m acular edem a desires cataract c. m anual pupil stretching
surgery. The m ost appropriate m anagem ent is d. Malyugin ring
a. cataract surgery 27. Ocular m elanom a is least likely to occur in which portion
b. focal laser treatm ent then cataract surgery of the uvea?
c. cataract surgery then panretinal photocoagulation a. conjunctiva
d. cataract surgery with intraoperative laser b. iris
treatm ent c. ciliary body
d. choroid

298
 Review Questions

28. A patient reports acute pain while ham m ering m etal c. near vision affected m ore than distance vision
and presents with 20/20 vision, subconjunctival hem or- d. reduced vision from bright lights
rhage, and sm all hyphem a OD. The m ost appropriate test 38. A piggyback IOL is m ost likely to be necessary in which
to obtain is of the following conditions?
a. FA a. status post vitrectom y
b. OCT b. pseudoexfoliation syndrom e
c. MRI c. high hyperopia
d. CT d. diabetes
29. A patient with a traum atic cataract requires cataract 39. A 77-year-old wom an with cataracts and m acular degen-
surgery. Which of the following is m ost helpful? eration is interested in cataract surgery. The m ost
a. laser interferom eter helpful test for evaluating this patient is
b. Am sler grid a. Am sler grid
c. brightness acuity test b. brightness acuity test
d. visual field c. contrast sensitivity
30. Pseudoexfoliation syndrom e and pigm ent dispersion d. potential acuity m eter
syndrom e share which of the following findings? 40. In which situation does optical biom etry have the largest
a. deep anterior cham ber angles advantage over ultrasound biom etry in determ ining
b. pigm ented trabecular m eshwork an accurate m easurem ent?
c. poor pupillary dilation a. keratoconus
d. phacodonesis b. posterior subcapsular cataract
31. The m ost likely cause of m onocular diplopia after c. high m yopia
blunt traum a is d. after LASIK
a. orbital fracture 41. During phacoem ulsification a m ilky substance
b. hyphem a (lens m ilk) appears. What is the m ost appropriate
c. lens subluxation next step?
d. m acular hole a. check for tight incision
32. Which of the following tests is m ost helpful in a patient b. raise irrigation bottle
with a Morgagnian cataract? c. increase aspiration and vacuum settings
a. ANCA d. add m ore OVD
b. HbA1C 42. Which of the following is the m ost likely com plication
c. HLA-B27 of Nd:YAG laser posterior capsulotom y?
d. RPR a. hypotony
33. A truck driver desires the best range of vision after b. corneal burn
cataract surgery. Which IOL strategy is m ost appropriate? c. retinal detachm ent
a. apodized diffractive lens d. epiretinal m em brane
b. zonal refractive lens 43. A patient with vitreous to the wound is m ost at risk for
c. m ini-m onovision with accom m odating lens which postoperative com plication?
d. m onovision with m onofocal lens a. endopthalm itis
34. One week after cataract surgery, a patient has increased b. retinal detachm ent
anterior cham ber cells and flare, IOP of 36 m m Hg, and c. increased IOP
a nuclear fragm ent in the inferior angle. The m ost d. cystoid m acular edem a
appropriate treatm ent option is 44. On postop day 1 after cataract surgery, exam shows
a. increase the topical antibiotic wound gape and a flat anterior cham ber. The m ost
b. increase the topical steroid appropriate treatm ent is
c. perform a a paracentesis a. cycloplegia
d. rem ove the retained fragm ent b. bandage contact lens
35. Which of the following tools is m ost helpful when c. suture wound
perform ing surgery on a patient with a m ature cataract? d. inject OVD
a. Trypan blue 45. A silicone IOL should not be used in a patient with
b. intracam eral epinephrine a. Fuchs’ corneal dystrophy
c. cohesive OVD b. proliferative diabetic retinopathy
d. Malyugin ring c. pseudoexfoliation syndrom e
36. Glaukom flecken is caused by d. m acular degeneration
a. traum a 46. A patient with a posterior subcapsular cataract desires
b. steroids surgery. What is the m ost appropriate reason for
c. inflam m ation perform ing cataract surgery?
d. ischem ia a. patient com plains of glare and difficulty reading
37. Which of the following sym ptom s is least associated b. visual acuity at near is worse than at distance
with posterior subcapsular cataracts? c. distance visual acuity is reduced in a bright
a. increased glare room
b. poor blue discrim ination d. visual acuity im proves after dilation

299
CHAPTER 10 • Anterior Segm ent

47. An anterior subcapsular cataract is m ost likely to c. lower irrigation bottle height
develop in a patient with which skin condition? d. increase tubing com pliance
a. pem phigous 56. Which of the following IOL designs is m ost likely to be
b. Stevens-Johnson Syndrom e associated with a com plication of laser posterior
c. epiderm olysis bullosa capsulotom y?
d. atopic derm atitis a. polym ethlym ethacrylate 1-piece
48. Reduced color discrim ination is m ost likely caused by b. acrylic 1-piece
which type of cataract? c. silicone plate haptic
a. anterior subcapsular d. silicone 3-piece
b. cortical 57. A patient with previous RK surgery undergoes uncom pli-
c. nuclear sclerotic cated phacoem ulsification. Two weeks after the cataract
d. posterior polar surgery, the patient states that her vision is blurry and
49. Which of the following is m ost likely to occur unchanged. Her refraction is +2.00 + 0.50x30, which
following cataract surgery in a patient with previous im proves her vision to 20/20. At this point, the m ost
m yopic LASIK? appropriate m anagem ent options is
a. flap dislocation a. observation
b. hyperopia b. conductive keratoplasty
c. DLK c. PRK with m itom ycin-C
d. corneal edem a d. piggyback IOL
50. A patient with no visual com plaints is noted to have 58. What is the m ost appropriate treatm ent for a patient with a
pupillary capture of an IOL. What is the m ost appropriate hyphem a and persistent elevated IOP for 1 week despite
m anagem ent? m axim al m edical therapy?
a. observation a. oral steroids and am inocaproic acid
b. cycloplegia b. hem oglobin electrophoresis
c. reposition IOL c. paracentesis
d. IOL exchange d. anterior cham ber washout
51. One day after uncom plicated cataract surgery with pha- 59. A32-year-old m an presents 1 week after blunt traum a with
coem ulsification, the exam shows counting fingers vision, 20/20 visual acuity and a dilated, unresponsive pupil OD.
3 + corneal edem a with Descem et’s folds, and severe The m ost appropriate next step is
anterior cham ber reaction with fibrinous m aterial. The a. careful slit lam p exam of iris
m ost likely diagnosis is b. hydroxyam phetam ine test
a. vitreous in anterior cham ber c. VDRL
b. angle-closure d. MRI
c. toxic anterior segm ent syndrom e 60. Which of the following IOLdesigns is m ost appropriate for
d. endophthalm itis placem ent in the sulcus?
52. Apatient with high hyperopia is at increased risk for which a. 1-piece acrylic
of the following com plications of cataract surgery? b. truncated optic
a. posterior capsule tear c. 5 m m optic
b. CME d. 13.5 m m polym ethylm ethacrylate
c. retinal detachm ent
d. choroidal effusion
53. The largest risk for capsule rupture during hydrodissection
occurs with SUGGESTED READINGS
a. sm all pupil
b. pseudoexfoliation syndrom e Abelson, M. B. (2001). Allergic diseases of the eye. Philadelphia: WB
c. posterior polar cataract Saunders.
d. m ature cataract Azar, D. T., Gatinel, D., & Hoang-Xuan, T. (2006). Refractive surgery
54. After hydrodissection you notice capsular block. The next (2nd ed.). Philadelphia: Mosby.
step is to Basic and Clinical Sciences Course. (2016). Section 11: Lens and cataract.
a. raise the irrigation bottle San Francisco: AAO.
b. add OVD Basic and Clinical Sciences Course. (2016). Section 13: Refractive surgery.
San Francisco: AAO.
c. burp the wound
Boruchoff, S. A. (2001). Anterior segment disorder: A diagnostic color atlas.
d. decom press the nucleus and lift the anterior capsule
Boston: Butterworth-Heinem ann.
55. Which of the following m easures is m ost likely to reduce Henderson, B. A. (2014). Essentials of Cataract Surgery (2nd ed.).
the risk of post-occlusion surge during Thorofare: SLACK.
phacoem ulsification? Mackie, I. A. (2003). Externaleyedisease. Boston:Butterworth-Heinem ann.
a. increase aspiration flow rate setting Watson, P., Hazlem an, B., & Pavesio, C. (2003). Sclera and systemic
b. reduce m axim um vacuum setting disorders. Philadelphia: Butterworth-Heinem ann.

300
11 Posterior Segm ent
ANATOMY
PHYSIO LOGY
ELECTROPHYSIOLOGY
RETINAL IMAGING
DISO RDERS
LASER TREATMENT

ANATOMY 3. Gan glion cell layer: usually a single cell layer with cells
packed tightly near the optic disc and m ore scattered
in the periphery; nuclei are m ultilayered in m acula
Vit re o u s 4. In n er plexiform layer: synaptic processes between
Com posed of 99% water and a few type II (m ainly) and bipolar and ganglion cells, as well as am acrine with
type IX collagen fibers; viscous, gel-like quality from bipolar cells; outerm ost layer com pletely nourished
m ucopolysaccharide and hyaluronic acid that is folded into by retinal arteries
coiled chains and holds water like a sponge; 5. In n er n uclear layer: outerm ost retinal layer; inner ⅔
volum e $ 4 cm 3 ; syneresis (liquefaction) occurs with aging of retina receives its nourishm ent from retinal
(Figs. 11-1, 11-2). vasculature (outer ⅓ from choroid); contains cell
bodies of bipolar, am acrine (confined to inner
Vitr eo u s b ase: portion of vitreous that attaches to surface), horizontal (confined to outer surface), and
peripheral retina and pars plana; 6 m m width – 2 m m anterior Mu€ ller’s (span from ILM [foot processes] to ELM
and 4 m m posterior to the ora serrata; straddles ora serrata; [m icrovilli, which point toward RPE]) cells
avulsion is pathognom onic for traum a 6. Outer plexiform layer: synaptic processes between
photoreceptors and dendritic processes of bipolar
Vitr eo r etin al ju n ctio n s: arise from footplates of Mu€ller’s cells; cone axons ¼ Henle fibers
cells at internal lim iting m em brane; provide firm vitreoretinal Middle limiting membrane (MLM): synapses; form s
attachm ent, especially at vitreous base, m acula, optic nerve, approxim ate border of vascular inner portion
and retinal vessels; also at edge of lattice degeneration, and avascular outer portion of the retina
chorioretinal scars, degenerative rem odeling, enclosed ora Central retinal artery supplies retina internally
bays; weak attachm ents at fovea and disc, and over areas of (MLM to ILM)
lattice Choriocapillaris supplies retina externally (MLM
to RPE)
Interior to MLM: dendrites of bipolar cells along
Re t in a (Fig. 11-3) with horizontally coursing processes of
Neu r o sen so r y r etin a ( 9 layer s) : inner refers to proxim al horizontal cells
or vitreous side of retina External to MLM: basal aspect of photoreceptors
1. In tern al lim itin g m em bran e (ILM): foot processes of In m acula, outer plexiform layer is called Henle’s
Mu€ ller’s cells (PAS-positive basem ent m em brane); fiber layer; radial orientation of fibers
true basem ent m em brane; clinically visible as sm all (responsible for m acular star configuration and
yellow-white spots (Gunn’s dots) at ora serrata; cystic spaces in cystoid m acular edem a)
continuous with inner bordering m em brane of 7. Outer n uclear layer: photoreceptor cell bodies and
ciliary body nuclei; m ost cone nuclei lie in single layer
2. Nerve fiber layer (NFL): unm yelinated ganglion cell im m ediately internal to ELM
axons; also contains glial cells (astrocytes); 8. Extern al lim itin g m em bran e (ELM): fenestrated
m yelination by oligodendrites occurs at lam ina intercellular bridges, situated external to
cribrosa; axons synapse with nuclei of cells in LGB photoreceptor nuclei; interconnects photoreceptor

301
CHAPTER 11 • Posterior Segm ent

Egge r's line forming Ana tomica l ve rtica l me ridia n

Wie ge r's liga me nt (hya loide o- Ca na l of
ca ps ula r liga me nt) Ha nnove r
P a rs plica ta S hort cilia ry
Be rge r's s pa ce a rte rie s
(re trole nta l s pa ce P a rs pla na
of Ergge le t)

Ora Vorte x a mpulla e

s e rra ta
S hort cilia ry
ne rve s
S cle ra Long cilia ry a rte ry Long cilia ry ne rve
Ca na l of Ante rior Vitre ous
P e tit vitre ous ba s e
Choroid corte x

Inve rte d
Re tina S hort cilia ry ne rve s
vorte x ve in Vorte x ve in

Cloque t's ca na l

S e conda ry
Are a of Ma rte gia ni vitre ous

Fig ure 11-1. Vitreous anatomy according to classic anatomic and histologic
studies . (From Schepens CL, Neetens A: The vitreous and vitreoretinal interface.
Ana tomica l ve rtica l me ridia n
New York, 1987, Springer-Verlag.)
Fig ure 11-3. Normal fundus as seen through indirect
ophthalmoscope. (From J ames A. Richard (ed). (1980). Practical
Ophthalmology: A Manual for Beginning Residents (3rd ed., p. 122, Fig. 60.).
San Francisco: AAO.

cells to Mu€ ller’s cells; not a true basem ent m em brane

but an illusion caused by tight junctions between
Mu€ ller’s cells and photoreceptors
9. Ph otoreceptor layer: rods and cones; num ber equal
in m acula; 11-cis retinal converted to all-trans retinol
in PR outer segm ents (not in RPE where it is converted
back) (Fig. 11-4)
120 million rods: rods account for 95% of
A photoreceptors; rhodopsin; density m axim al in a
ring 20–40° around fovea, no rods in center of
fovea; when dark adapted, rods are 1000 tim es
IF
m ore sensitive than cones; rod disks are not
attached to cell m em brane, discrete structures;
provide dark adapted vision
6 million cones (50% in m acula): 3 types of visual
pigm ent (S [short-wavelength sensitive, blue, m ake
up only 2%], M [m iddle-wavelength sensitive,
green, 33%], and L [long-wavelength sensitive, red,
hya luronic a cid colla gen fibril
65%]); density m axim al in fovea; cone disks are
C
attached to cell m em brane and undergo
m em branous replacem ent; provide high acuity
and color vision

Per ip h er al r etin a: extends from m acula to ora serrata,

nonpigm ented epithelium is contiguous with pars plana;
defined as any area of the retina with a single layer of ganglion
chondroitin interconnecting cells (Fig. 11-5)
sulfa te fila ment

Fig ure 11-2. Ultrastructure of hyaluronan–collagen interaction in the vitreous. C, Macu la: area of retina where ganglion cell layer is m ore than
collagen; IF, interconnecting filament. (Courtesy of Dr Akiko Asakura. From
1 cell thick (5–6 m m in diam eter)
Askura A: Histochemistry of hyaluronic acid of the bovine vitreous body as studied
by electron micoscopy. Acta Soc Ophthalmol J pn 89:179–191, 1985.) Centered 4 m m tem poral and 0.8 m m inferior to optic nerve

302
 Anatom y

Inte rna l limiting

Fove a l dia me te r 1500 µm Ma rgin
me mbra ne
Inte rna l limiting
Ga nglion Fove ola 350 µm me mbra ne
ce ll
Mülle r ce lls in
inne r nucle a r la ye r
Exte rna l limiting
me mbra ne
Re tina l pigme nt
Ama crine ce ll Inne r nucle a r e pithe lium
Bipola r Ca pilla ry De clivity Umbo Fove a
Horizonta l ce ll la ye r a rca de e xte rna
ce ll
Fig ure 11-6. Foveal margin, foveal declivity, foveola, and umbo. (From Schubert
HD: Structure and function of the neural retina. In: Yanoff M, Duker J S (eds ):
Ophthalmology. London, 1999, Mosby.)
Middle limiting
me mbra ne

Exte rna l limiting

me mbra ne
Mülle r's
fibe r (glia )

Cone

Rod
Fig ure 11-4. Neuronal connections in the retina and participating
cells. (From Schubert HD: Structure and function of the neural retina.
In: Yanoff M, Duker J S (eds): Ophthalmology. London, 1999, Mosby.)

A
Inte rna l
limiting me mbra ne Ora s e rra ta

Mülle r ce lls Vitre ous

Ba s e a tta chme nts
P e rifove a l
Ba s e me nt a re a
me mbra ne P a ra fove a l
a re a
Ape x Nonpigme nte d Fove a
Ape x e pithe lium Fove ola
P igme nte d Umbo
Ba s e
e pithe lium
Exte rna l limiting me mbra ne Bruch's (ba s e me nt) me mbra ne 1.5 0.5 0.5 1.5
mm mm 0.35 mm mm mm
Fig ure 11-5. Transition of neural retina to nonpigmented epithelium at the ora 1.5 mm
serrata. (From Schubert HD: Structure and function of the neural retina. In: Yanoff
M, Duker J S (eds ): Ophthalmology. London, 1999, Mosby.) B
Fig ure 11-7. Normal fundus with macula encompassed by major vascular
Differentiation of the m acula does not occur until age arcades. (From Schubert HD: Structure and function of the neural retina.
4–6 m onths old In: Yanoff M, Duker J S (eds ): Ophthalmology. London, 1999, Mosby.)

Predom inantly red and green cones in m acula

Contains taller RPE cells and xanthophyll pigm ent (blocks
High levels of carotenoids (100-1000 Â m ore than anywhere
choroidal fluorescence during fluorescein angiography)
else): lutein (antioxidant) and zeaxanthin (light screening)
Initially, ganglion cell nuclei are present in fovea but are
gradually displaced peripherally, leaving fovea devoid of
accessory neural elem ents
Fo vea (Figs. 11-6, 11.7): central depression of inner retinal
Floor of the fovea is m ade up of specialized Mu€ ller cells in
surface (1.5 m m in diam eter) within m acula
inverted cone shape (Mu€ ller cell cone), likely a reservoir for
Corresponds to the foveal avascular zone (FAZ) concentrated xanthophyll and structural support for foveola

303
CHAPTER 11 • Posterior Segm ent

Fo veo la: central area of fovea (350 µm in diam eter) further differentiation of retinal layers (m ay explain why
m acula fails to develop properly in albinism ); m elanin
Absence of ganglion cells and other nucleated cells, no rods; helps absorb excess light; m acular RPE cells contain
avascular larger and a greater num ber of m elanosom es than those
Mechanism s to prevent foveal detachm ent: in periphery
Microvilli of RPE, which surround tips of photoreceptors Contributes to adhesion of sensory retina
Viscous mucopolysaccharides, which bathe photoreceptors ATP-dependent Na + / K+ pum p on apical surface
and RPE m aintains environm ent of subretinal space
Intraocular pressure Heat exchange
Light absorption
In ter cellu lar ju n ctio n s: RPE cells m ay undergo hypertrophy, hyperplasia, m igration,
Blood–retinal barrier: atrophy, and m etaplasia
INNER BARRIER: tight junctions (zonula occludens) HYPERTROPHY: flat, jet-black subretinal lesion
between retinal vascular endothelial cells HYPERPLASIA: intraretinal pigm ent deposition
OUTER BARRIER: tight junctions between RPE cells Migrate into retina to form bone spicules around
Other intercellular junctions: blood vessels in retinitis pigm entosa
ZONULA ADHERENS (external lim iting m em brane): Migrate through retinal holes to form preretinal
no barrier to passage of fluids m em branes in proliferative vitreoretinopathy
MACULA ADHERENS (desm osom es): no barrier to METAPLASIA: nonspecific reaction, observed m ost often
passage of fluids in phthisis; fibrous (com ponent of disciform scars in
AMD) or osseous (intraocular ossification)
Clin ical co r r elatio n :
Collateral vessels: occur at site of obstruction, across
horizontal raphe, and at disc Br u ch ’s m em b r an e:
Perm eable to sm all m olecules
Hem o r r h ages: Layers:
Flame or splinter: superficial; blood tracks along NFL 1. Basem ent m em brane (inner basal lam ina of RPE)
Blot or dot: deep; blood confined by axons oriented 2. Collagen
perpendicular to Bruch’s m em brane 3. Elastic tissue
Boat-shaped (scaphoid): 4. Collagen
SUB-ILM: hem orrhagic detachm ent of ILM
5. Basem ent m em brane (outer basal lam ina of
SUB-HYALOID: blood between ILM and
choriocapillaris)
posterior hyaloid RPE and Bruch’s m em brane are continuous with the
Dark hemorrhage: sub-RPE; can be confused with choroidal pigm ented ciliary epithelium
m elanom a
Neu r o glial cells:
RPE: m onolayer of hexagonal cells with apical m icrovilli and Astrocytes: branching neural cells in retina and CNS;
basem ent m em brane at base adherent to each other by system proliferation leads to gliosis; cell of origin for optic nerve
of tight junctions or term inal bars that m ake up blood retinal gliom a; provide structural support to optic nerve and
barrier retina; contribute to nourishm ent of neuronal elem ents;
RPE and outer segm ents of photoreceptors have apex-to-apex foot processes ensheath blood vessels within nerve,
arrangem ent, resulting in a potential subretinal space contributing to blood–brain barrier
Mu€ller’s cells: m odified astrocytes; footplates form ILM,
Merges anteriorly with pigm ented epithelium of nuclei in inner nuclear layer; provide skeletal support;
ciliary body; m ore highly pigm ented in central m acula contribute to gliosis; extend from ILM to ELM
Functions: Microglia: phagocytic cells of the CNS
Helps with developm ent of photoreceptors during Arachnoidal cells: cell of origin for m eningiom a
em bryogenesis Oligodendrocytes: produce m yelin in the CNS; cell of origin
Involved in vitam in A cycle (uptake, transport, storage, for oligodendrogliom a
m etabolism , and isom erization) Schwann cells: produce m yelin in peripheral nervous
Provides nourishm ent for outer half of retinal cells system
Receives waste products: phagocytoses photoreceptor
outer segm ents; photoreceptors renew outer segm ents
Ch o ro id
every 10 days
Form s outer blood–retinal barrier (tight junctions between Posterior part of uveal tract that extends from ora serrata (outer
RPE cells) layers end before inner) to optic nerve. Attached to sclera by
Secretes basem ent m em brane m aterial (deposits on strands of connective tissue at optic nerve, scleral spur, vortex
inner basal lam ina of Bruch’s m em brane) veins, and long and short posterior ciliary vessels; derived from
Produces m elanin granules in 6th week of gestation (first m esoderm and neuroectoderm ; 0.22 m m thick posteriorly and
cells of body to m elanize); m elanization m ay induce 0.1–0.15 m m thick anteriorly

304
 Electrophysiology

Layer s: Ta b le 11-1. Visual pigments

1. Bruch ’s m em bran e (innerm ost layer): PAS stain
positive, 2 µm thick centrally and increases in Peak
thickness with age P ho to re c e p to r P ig m e nt s e ns itivity
2. Ch oriocapillaris: 40–60 µm diam eter capillary layer Rod Rhodopsin 505 nm
with 600–800 Å wide fenestrations (leak fluorescein L (long-wavelength Erythrolabe (red 560 nm
dye); fenestrations have thin covering diaphragm sensitive; red) cones sensitive)
m ore num erous on internal side; lobular pattern in M (middle-wavelength Chlorolabe (green 530 nm
posterior pole, m ore parallel in periphery, at right sensitive; green) cones sensitive)
angles anteriorly form ing ladder-like pattern S (short-wavelength Cyanolabe (blue 420 nm
(Fig. 11-8) sensitive; blue) cones sensitive)
3. Strom a: m ainly blood vessels; outer, unfenestrated
large vessel layer (Haller’s layer) and unfenestrated,
m edium vessel layer (Sattler’s layer) rhodopsin is then resynthesized, and vitam in Ais stored in liver
4. Suprach oroidal space (outerm ost layer): 30 µm and transported to RPE by serum retinol–binding protein and
thick, darkly pigm ented pre-album in
Blood supply from 1–2 long and 15–20 short posterior ciliary Rods shed outer segm ents during the day
arteries (from internal carotid to ophthalm ic artery)
Cones shed outer segm ents during the night
Endothelium is perm eable to large m olecules
Choriocapillaris arranged in segm ental pattern; m ajor source
of nutrition for RPE and outer retinal layers Lu m in o sity cu r ves:
Drains via vortex veins to superior and inferior Light-adapted: cone peak sensitivity is to light of 555 nm ;
ophthalm ic veins yellow, yellow-green, and orange appear brighter than
blue, green, and red
Contain both parasym pathetic and sym pathetic Dark-adapted: rod peak sensitivity is to light of
nerves (autoregulatory function to keep blood flow constant) 505 nm (blue)
via short (m ainly) and long posterior ciliary nerves Purkinje shift: shift in peak sensitivity that occurs from
light- to dark-adapted states (Fig. 11-9)

P HYSIOLOGY

Visu al p igm en ts: 4 types, each com posed of 11-cis-retinal ELECTROP HYS IOLOGY
(vitam in A aldehyde) + a protein (opsin); 3 cone pigm ents and
1 rod pigm ent (Table 11-1) Ele c t ro r e t in o g ra m (ERG)
Electrical potential generated by retina in response to flash of
Ro d p h o to r ecep to r m em b r an es: lipid bilayer in which light; m easures m ass retinal response; useful for processes
rhodopsin is an integral com ponent affecting large areas of retina
Chrom ophore 11-cis-retinaldehyde is oriented parallel to the Photoreceptors, bipolar and Mu€ ller’s cells contribute to flash
lipid bilayer (perpendicular to the path of photons) ERG; ganglion cells do not
Bleaching releases all-trans-retinaldehyde from the visual Light is delivered uniform ly to entire retina in Ganzfeld bowl,
pigm ent (opsin) with conversion to all-trans-retinol; this and electrical discharges are m easured with a corneal contact
initiates an electrical im pulse that travels to visual cortex; lens electrodes and a ground electrode placed over ears

Fig ure 11-8. Human choriocapillaris, retinal view. (From Fryczkowski AW: Anatomical and functional choroidal lobuli. Int Ophthalmol 18:131–141, 1994.)

305
CHAPTER 11 • Posterior Segm ent

4
s
0°
t
r
e
b
As ce nding
m
2° fie ld in diffe re nt b-wa ve
a
l
i
l
re tina l pos itions
l
3
i
m
R1
o
r
c
i
m
2 1/2 °
n
i
2
I
5°
g
a -wa ve
o
L
10° 50 µV 50 µV R2

1 1.0 ms e c 0.5 ms e c
0 10 20 30 Fig ure 11-11. Normalhuman early receptor potential(ERP). This rapid response is
complete within 1.5 ms and is believed to be generated by the outer segments. An
Minute s in da rk
intense, bright stimulus in the dark-adapted state is needed for an ERP to be
Fig ure 11-9. Normaldark-adaptation curves measuring retinalsensitivityto a small obtained. (Redrawn from Berson EL, Goldstein EG: Early receptor potential in
spot of light whose intensity is varied until the threshold value is found; a 2° test spot dominantly inherited retinitis pigmentosa. Arch Ophthalmol 83:412–420, 1970.
was placed at different distances from the fovealcenter. Note the cone–rod break at From Slamovits TL: Basic and Clinical Science Course: section 12: orbit, eyelids,
9 minutes, middle graph. (From Hecht S, Haig C, Wald G: The dark adaptation of and lacrimal system. San Francisco,1993, American Academy of Ophthalmology.)
retinal fields of different size and location. J Gen Physiol 19:321–337, 1935.)

b-wa ve Ne utra l De ns ity 2.0

OD b-wa ve

OS
Amplitude ba s e
line Amplitude

OD ba s e
a -wa ve 100 µV
Implicit time 20 ms line
Implicit time OS
Fig ure 11-10. The photopic (cone-mediated) ERG is a light-adapted, bright
flash-evoked response from the cones of the retina; the rods do not respond in 100 µV
the light-adapted state. (From Slamovits TL: Basic and clinical science cours e:
section 12: orbit, eyelids, and lacrimal system. San Francisco, 1993, American
20 ms
Academy of Ophthalmology, 1993.) Fig ure 11-12. The scotopic (rod-mediated) ERG is a dark-adapted, dim
flash (below cone threshold)-evoked response that records the signal from the
rods. The test s hould be performed after at least 30 minutes of dark
adaptation. (From Slamovits TL: Basic and clinical science course: section 12:
Co m p o n en ts (Fig. 11-10): orbit, eyelids, and lacrimal system. San Francisco, 1993, In: Yanoff M, Duker J S
a-wave: photoreceptor cell bodies (negative waveform ) (eds) Ophthalmology. American Academy of Ophthalmology.)
b-wave: Mu€ ller’s and bipolar cells (positive waveform )
Amplitude: bottom of a-wave to top of b-wave; m easured in c-wave: RPE; late (2–4 seconds) positive deflection; occurs
m icrovolts in dark-adapted state
Measures response of entire retina; proportionate to area Early receptor potential (ERP) (Fig. 11-11): outer segm ents
of functioning retina of photoreceptors; com pleted within 1.5 m s
Decreased in anoxic conditions (diabetes, CRAO, Represents bleaching of visual pigm ent; requires intense
ischem ic CVO) stim ulus in dark-adapted state
Implicit time: tim e from light flash to peak of b-wave; Ganglion cells are not m easured; therefore, flash ERG is not
m easured in m illiseconds useful in glaucom a
Increased in various hereditary conditions
Oscillatory potential: four to ten high-frequency, low- Ph o to p ic (light adapted): strong flash in light adapted for 10
am plitude wavelets superim posed on ascending b-wave m in state (bleach out rods); m easures cone function
of scotopic and photopic bright flash ERG Flicker ERG: flashing light at 30 flashes/second (30 Hz)
Generated in m iddle retinal layers (inner plexiform isolates cone response; photopic response to
layer): m ay be inhibitory potentials from superstim ulation
am acrine cells Sm all wave follows each flash
Reduced in conditions of retinal hypoxia or Cone response because rods cannot recycle rhodopsin this
m icrovascular disease quickly

306
 Electrophysiology

Poor response at 30 cycles/second indicates abnorm al cone Ne utra l De ns ity 0.0

function; rods can respond up to 20 Hz
Cones also respond to repetitive light
b-wa ve
Sco to p ic (dark adapted): weak flash in dark adapted for
30 m in state (Fig. 11-12); m easures rod function
Dim white or blue flash below cone threshold
At low intensity: sm all a- and b-waves
At increasing intensity: im plicit tim e shortens, b-wave OD
ba s e
am plitude increases line
Bright-flash ERG (in scotopic state; m easures com bined
m aximal rod and cone response): deep a-wave and large OS
b-wave; oscillatory potentials are present (Fig. 11-13) Amplitude

In d icatio n s: 100 µV
Diagnose generalized retinal degeneration 20 ms
Assess fam ily m em bers for heritable retinal degeneration
Assess decreased vision and nystagm us present at birth a -wa ve
Assess retinal function in presence of opaque ocular m edia Implicit time
or vascular occlusion
Evaluate functional visual loss Fig ure 11-13. The bright flash, dark-adapted ERG stimulates both the cone and
rod systems and gives large a and b-waves, with oscillatory potentials in the
ascending b-wave. Some testing centers call this a “scotopic ERG,” but the rods
Disease states (Fig. 11-14, Table 11-2): are not isolated by this method. (From Slamovits TL: Basic and clinical science
CRAO: norm al a-wave (perfused by choroid), absent course: section 12: orbit, eyelids, and lacrimal system. San Francisco, American
b-wave Academy of Ophthalmology, 1993. In Yanoff M, Duker J S (eds): Ophthalmology.
Ischemic CVO: reduced b-wave am plitude, reduced b : London, Mosby, 1999.)
a-wave ratio, prolonged b-wave im plicit tim e
Retinitis pigmentosa: early, reduced am plitude (usually Female carriers of X-linked RP: prolonged photopic b-wave
b-wave) and prolonged im plicit tim e; later, extinguished im plicit tim e, reduced scotopic b-wave am plitude
with no rod or cone response to bright flash Sector RP: norm al b-wave im plicit tim e

Da rk Ada pte d Flicke r

Condition P hotopic S cotopic Bright Fla s h 30/s e cond

Norma l

Cone de ge ne ra tion blink blink

re s pons e re s pons e

P a rtia l cone
de ge ne ra tion

Cone -rod
de ge ne ra tion
Rod-cone
de ge ne ra tion
Conge nita l s ta tiona ry
night blindne s s
(X-linke d re ce s s ive )
X-linke d
re tinos chis is

X-linke d incomple te
blue cone
monochroma t 100 µV
20 ms
Fig ure 11-14. Electroretinogram patterns. (From Slamovits TL: Basic and Clinical Science Course: Section 12: Orbit, Eyelids, and Lacrimal System. San Francisco,
American Academy of Ophthalmology, 1993.)

307
CHAPTER 11 • Posterior Segm ent

Ta b le 11-2. ERG patterns for various ocular diseases

P 50

No rm a l
Exting uis he d ERG a -wa ve , Ab no rm a l
a b no rm a l p ho to p ic , re d uc e d p ho to p ic , no rm a l
no rm a l ERG b -wa ve s c o to p ic ERG
RP CSNB; Oguchi’s Achromotopsia
disease
Ophthalmic artery X-linked juvenile Cone dystrophy
occlusion retinoschisis
N35
DUSN CVO
Metallosis CRAO
RD Myotonic
dystrophy
2 µV
Drug toxicity Quinine toxicity
(phenothiazine;
50 ms N95
chloroquine)
Cancer-associated
Fig ure 11-15. Pattern ERG with components labeled. This waveform measures
retinopathy
retinal ganglion cell function and is not related to the flash ERG test. (After Treat
GL: The pattern electroretinogram in glaucoma and ocular hypertension. In:
Heckenlively J R, Arden GB (eds): Principles and Practice of Clinical
Cone dystrophy: abnorm al photopic and flicker, norm al
Electrophysiology of Vision. St Louis, 1991, Mosby-Yearbook.)
scotopic
X-linked foveal retinoschisis: norm al a-wave until late,
Ele c t ro -o c u lo g ra m (EOG)
reduced b-wave (especially scotopic)
Retinal detachment: reduction in am plitude corresponds to Indirect m easure of standing potential of eye (voltage
extent of neurosensory loss (50% decrease in difference between inner and outer retina) (Fig. 11-16)
am plitude ¼ 50% of neurosensory retina is functionally
Depolarization of basal portion of RPE produces light peak;
detached)
norm al result requires that both RPE and sensory retina be
Diffuse progressive retinal disease: increased b-wave
norm al
im plicit tim e
Nonprogressive retinal disease: decreased b-wave am plitude
MEWDS: reduced a-wave Ar d en r atio : ratio of light-to-dark peak (2 :1 is norm al;
Chalcosis: reduced am plitude (suppressed by intraocular < 1.65 is abnorm al); decreased ratio is due to photoreceptor or
copper ions) RPE disorder
Retinal microvascular disease (diabetes, hypertension,
CVO): loss of oscillatory potentials Pr o ced u r e: light adaptation  15 m inutes, recording during
Achromotopsia: absent cone function; norm al rod function dark adaptation  20 m in, and another recording under
Leber’s congenital amaurosis: flat ERG standard light adaptation  10 m in; electrode placed on cornea
CSNB: norm al a-wave, poor b-wave and another on body in Ganzfeld bowl, and electrodes placed
Congenital rubella: norm al ERG on canthi of eye; m easurem ent is based on eye m ovem ent
Glaucoma: norm al ERG dependent voltage generation from patient looking back and
Optic neuropathy/atrophy: norm al ERG forth between two fixation lights; both eyes are recorded
sim ultaneously
Patter n ERG (PERG): waveform sim ilar to flash ERG, but Dark adaptation causes progressive decrease in response
different test to m easure ganglion cell activity; stim ulus is an reaching a trough (dark trough) at 8–12 m inutes
alternating checkerboard pattern that gives a constant Light adaptation causes progressive rise in am plitude over
illum ination to the retina (Fig. 11-15) 6–9 m inutes (light peak)
Norm al response is com posed of 3 waves: Measure lowest voltage with dark adaptation and highest
N35: cornea-negative wave at 35 m s voltage with light adaptation
P50: cornea-positive wave peak at 50 m s Am plitude is higher with light adaptation than dark
N95: negative trough at 95 m s adaptation
ERG is abnorm al in all cases in which EOG
is abnorm al except:
Mu ltifo cal ERG(m fERG): topographic representation of
Best disease and carriers: norm al ERG but
electrophysiologic function; retina stim ulated with array of
abnorm al EOG
hexagonal elem ents that are illum inated in pseudorandom
Pattern dystrophies
pattern; m athem atical extraction of recorded signal is
Chloroquine toxicity
displayed as three peak waveform (N1, P1, and N2); N1
Abnorm al ERG, norm al EOG: conditions with abnorm al
includes sam e cells as full field cone ERG a-wave; P1 includes
bipolar region but norm al rods
sam e cells as cone b-wave and oscillatory potentials
CSNB
Useful in disorders of posterior pole such as cone dystrophy X-linked retinoschisis

308
 Retinal Im aging

Vie wing s cre e n

(Ba ckground is lighte d s e cond ha lf of te s t)
La rge s t pote ntia l in light
Arde n ra tio = –––––––––––––––– µ 100
Lowe s t pote ntia l in da rk

Tra cing of Tra cing of

30˚ a rc s ta nding s ta nding
pote ntia l pote ntia l
in da rk light-a da pte d

OD
//
Re corde r
OS
//
 12 min˜  12 min˜

Fig ure 11-16. Diagram illustrating technique used in recording the electro-oculogram test; the patient is positioned so that the eyes will traverse a 30° arc between
2 blinking red lights. The skin electrodes are positioned at the lateral and inner canthi. The standing potential is measured as the patient moves his eyes between the lights,
first in the dark and then in the light. The maximum amplitude from the light condition is compared with minimum value from the dark to give a light peak-to-dark
trough ratio. (From Heckenlively J R: Retinitis pigmentosa. Philadelphia, 1988, J B Lippincott.)

analyzed so that the tissue reflectivity (sim ilar to ultrasound)

RETINAL IMAGING and density can be determ ined; m ore reflective areas
create greater interference; with tim e-dom ain OCT (TDOCT),
Op t ic a l c o h e re n c e t o m o g ra p h y (OCT) the reference m irror m oves; with spectral-dom ain or
spatially-encoded frequency dom ain OCT (SDOCT) the m irror
Creates cross-sectional im age of tissue from reflections of does not m ove and the broadband interference is evaluated by
broad bandwidth light based on low-coherence interferom etry a Fourier transform to obtain reflectance inform ation (this
Provides retinal thickness measurements, optic nerve m akes SDOCT m uch faster than TDOCT). Swept-source OCT
measurements, and cross-sectional retinal imaging to $ 3–10 µm (tim e-encoded frequency dom ain) uses even longer
depending on light source; anterior segment spectral domain OCT wavelength laser source and photodetectors to further increase
is useful to image anterior segment, in particular the cornea scan speed
and angle. Using specialized techniques evaluation of flow can Useful for optic nerve (glaucom a) and m acular pathology
produce images of vasculature (OCT angiography [OCTA]) (edem a, hole, pucker, CNV); can com pare thickness in cases of
Superlum inescent diodes or short-pulse lasers create beam s of m acular edem a from one visit to next; can diagnose and
infrared light through a Michelson interferom eter at both the differentiate vitreom acular pathology e.g., stage 1 m acular
eye and a reference m irror; the reflected light from the retina is hole/vitreom acular traction vs full-thickness hole
com pared with the light from the reference m irror and vs pseudohole or lam ellar holes (Figs. 11-17, 11-18)

super-
luminescent fiber
diode bea msplitter
tra nsverse
sca nning reference mirror
detector
78 D
slit-la mp condensing
ima ge pla ne lens
electronics computer

slit-la mp biomicroscope hea d

bea msplitter

Fig ure 11-17. Optical coherence tomography principle. (Adapted From Shuman J S, Hee MR, Puliafito CAet al: Quantification of nerve fiber layer thickness in normal and
glaucomatous eyes using optical coherence tomography. Arch Ophthalmol 113:586-596, 1995. From: Yanoff M, Duker J S (eds):Ophthalmology. London, 1999, Mosby.)

309
CHAPTER 11 • Posterior Segm ent

high inte rna l re fle ctivity

Fig ure 11-19. A-scan ultrasound demonstrating high internal reflectivity. (From
Friedman NJ , Kaiser PK, Pineda R II: The Massachusetts eye and ear infirmary
illustrated manual of ophthalmology, ed 3, Philadelphia, 2009, Elsevier.)

te mpora l na s a l

370 µm

250
µm

me dium inte rna l re fle ctivity

Fig ure 11-20. A-scan ultrasound demonstrating medium internal

log re fle ction
reflectivity. (From Friedman NJ , Kaiser PK, Pineda R II: The Massachusetts eye
and ear infirmary illustrated manual of ophthalmology,ed 3, Philadelphia, 2009,
B Elsevier.)

Fig ure 11-18. Full-thickness macular hole. A, fundus photograph, B, time

domain OCT. (From: Yanoff M, Duker J S (eds): Ophthalmology. London, 1999,
Mosby.)

He id e lb e rg re t in a l t o m o g ra p h (HRT)
Laser tom ography
Confocal scanning laser produces 3-dim ensional sections of
optic nerve and retina through undilated pupil
670 nm diode laser that is periodically deflected by oscillating
m irrors; laser scans retina, and instrum ent m easures reflectance
and constructs series of 2-dim ensional im ages at different
depths, which are com bined to create a m ultilayer 3D low inte rna l re fle ctivity
topographic im age Fig ure 11-21. A-scan ultrasound demonstrating low internal reflectivity. (From
Friedman NJ , Kaiser PK, Pineda R II: The Massachusetts eye and ear infirmary
Measures surface height to within 20 µm illustrated manual of ophthalmology, ed 3, Philadelphia, 2009, Elsevier.)

Re t in a l t h ic kn e s s a n a lyze r (RTA)
Creates thickness contour m ap Ult ra s o u n d
HeNe laser scans central 2 Â 2 m m area; receives 2 reflections, Acoustic im aging of globe and orbit
1 from ILM and 1 from RPE, then m aps distance between these
layers
A-scan : 1-dim ensional display (am plitude of echoes plotted
S c a n n in g la s e r o p h t h a lm o s c o p e (S LO) as vertical height against distance) (Figs. 11-19 to 11-21)
Modulated red light laser (633 nm )
Perform s funduscopy and autom ated perim etry B-scan : 2-dim ensional display (am plitude of echoes
sim ultaneously represented by brightness on a gray scale im age) (Fig. 11-22)

310
 Retinal Im aging

After m o vem en t: dynam ic features of lesion echoes

Observe B-scan echoes for m otion after cessation of eye
m ovem ents (i.e., rapid m ovem ent of vitreous
hem orrhage distinguished from slower, undulating
m ovem ent of rhegm atogenous retinal detachm ent)

Vascu lar ity: spontaneous m otion of echoes within lesion

corresponds to blood flow; m ay be visualized with Color
Doppler
choroida l de ta chme nt
Fig ure 11-22. B-scan ultrasound demonstrating choroidal detachment. (From Sp ecific lesio n s: (Tables 11-3 and 11-4) (Figs. 11-22
Kaiser PK, Friedman NJ , Pineda R II: The Massachusetts eye and ear infirmary
to 11-27)
illustrated manual of ophthalmology, ed 2, Philadelphia, 2004, Saunders.)
Asteroid hyalosis:
A-SCAN: m edium to high internal reflectivity
Reflectivity: height of spike on A-scan and signal brightness B-SCAN: bright echoes in vitreous due to calcium soaps;
on B-scan area of clear vitreous usually present between
Internal reflectivity refers to am plitude of echoes within a opacities and posterior hyaloid face
lesion or tissue Intraocular foreign body:
B-SCAN: high reflectivity when ultrasound probe is
In ter n al str u ctu r e: degree of variation in histologic perpendicular to reflective surface of foreign body;
architecture within a m ass lesion bright echo persists when gain turned down;
Regular internal structure indicates hom ogenous shadowing often present
architecture (m inim al or no variation in the heights of Intraocular calcification:
spikes in A-scan and uniform appearance of echoes on A-SCAN: high am plitude peak due to strong acoustic
B-scan) interface
B-SCAN: white echoes; partial or com plete shadowing
So u n d atten u atio n : occurs when acoustic wave is DDX: tum ors (retinoblastom a, choroidal osteom a, optic
scattered, reflected, or absorbed by tissue nerve m eningiom a, choroidal hem angiom a,
Decrease in strength of echoes within or posterior to lesion; choroidal m elanom a), Toxocara granulom a, chronic
m ay produce a void posterior to lesion called retinal detachm ent, optic nerve head drusen, vascular
“shadowing” (caused by dense substances [bone, occlusive disease of optic nerve, phthisis bulbi,
calcium , foreign body]) intum escent cataractous lens

Ta b le 11-3. Ultrasound characteristics of select retinal lesions

P a tho lo g y Lo c a tio n Sha p e Inte rna l re fle c tivity Inte rna l s truc ture Va s c ula rity
Melanoma Choroid and / or CB Dome or collar button Low to medium Regular Yes
Choroidal hemangioma Choroid; posterior pole Dome High Regular Yes
Metastasis Choroid; posterior pole Diffuse, irregular Medium to high Irregular No
Choroidal nevus Choroid Dome or flat High Regular No
Choroidal hemorrhage Choroid Dome Variable Variable No
Disciform lesion Macula Dome, irregular High Variable No

Ta b le 11-4. Ultrasound characteristics of different types of retinal detachments

P a tho lo g y To p o g ra p hic (B-s c a n) Qua ntita tive (A-s c a n) Afte r m o ve m e nt

Retinal detachment Smooth or folded surface Steep spike (100% high) Moderate to none
Open or closed funnel
Inserts at ON and ora serrata
May see intraretinal cysts
Posterior vitreous detachment Smooth surface Variable spike height (< 100%) Marked to moderate
Open funnel
With or without ON or fundus insertion
Inserts at ora serrata or ciliary body
Choroidal detachment Smooth, dome, or flat surface Steeply rising, thick, double peaked spike (100% high) Mild to none
No ON insertion
Inserts at ciliary body and vortex veins

311
CHAPTER 11 • Posterior Segm ent

choroida l ma ligna nt me la noma

s e rous re tina l choroida l s cle ra l
Fig ure 11-26. B-scan ultrasound demonstrating dome-shaped
de ta chme nt de ta chme nt thicke ning choroidal mass. (From Kaiser PK, Friedman NJ , Pineda R II: The Massachusetts
Fig ure 11-23. B-scan ultrasound demonstrating serous retinal detachment with eye and ear infirmary illustrated manual of ophthalmology, ed 2, Philadelphia,
shifting fluid, shallow peripheral choroidal detachment, and diffuse scleral 2004, Saunders.)
thickening. (From Kaiser PK, Friedman NJ , Pineda R II: The Massachusetts eye
and ear infirmary illustrated manual of ophthalmology, ed 2, Philadelphia, 2004,
Saunders.)

s e rous re tina l de ta chme nt choroida l me ta s ta s is

Fig ure 11-27. B-scan ultrasound of a patient with choroidal metastasis

demonstrating elevated choroidal mass with irregular surface and overlying
serous retinal detachment. (From Kaiser PK, Friedman NJ , Pineda R II: The
Massachusetts eye and ear infirmary illustrated manual of ophthalmology, ed 2,
Philadelphia, 2004, Saunders.)

s cle ra l thicke ning T-s ign

Fig ure 11-24. B-scan ultrasound demonstrating scleral thickening and the Flu o re s c e in a n g io g ra m (FA)
characteristic peripapillary T sign. (From Kaiser PK, Friedman NJ , Pineda R II:
The Massachusetts eye and ear infirmary illustrated manual of ophthalmology, ed Ph ases: choroidal filling, arterial, venous, recirculation
2, Philadelphia, 2004, Saunders.) Sodium fluorescein in a hydrocarbon based yellow-red dye
with a m olecular weight of 376 daltons (Da). Dye enters
choroidal circulation via short posterior ciliary arteries
10-15 seconds after injection; choroidal flow is very
rapid; choriocapillaris filling is usually com pleted in the
AV or early venous phase; cilioretinal artery is filled at
tim e of choroidal filling; central retinal artery takes m ore
circuitous route, resulting in dye arriving 1–2 seconds
after choroidal filling; AV phase occurs 1–2 seconds after
arterial phase; veins fill in 10–12 seconds (Figs. 11-28 to
11-32)
Fluorescein absorbs light at 465–490 nm (blue), em its at
520–530 nm (yellow-green); to produce an im age two
filters are required: an exciter filter that em its blue light
choroida l he ma ngioma which stim ulates fluorescein to em it yellow-green light
(barrier filter transm its only green light) so im age
Fig ure 11-25. B-scan ultrasound demonstrating elevated mass with underlying
thickened choroid. (From Kaiser PK, Friedman NJ , Pineda R II: The
produced by what returns through filter
Massachusetts eye and ear infirmary illustrated manual of ophthalmology, ed 2, 80% bound to album in and other serum proteins
Philadelphia, 2004, Saunders.) 90% excreted from kidney (also liver) within 24–36 hours

312
 Retinal Im aging

Fig ure 11-28. FA demonstrating choroidal and early arterial filling. Fig ure 11-31. FA demonstrating peak AV trans it.

Fig ure 11-29. FA demonstrating arterial phase. Fig ure 11-32. FA demonstrating late phase.

Transient yellowing of skin and conjunctiva that lasts

8–12 hours; m ost com m on adverse events: nausea
(3–15%), vom iting (5%), pruritus (5%); anaphylactic
reaction in 1 :100,000; death occurs in 1 :220,000;
pregnancy in first trim ester is a relative contraindication

Ch ar acter istics:
Hyperfluorescence: leakage (fenestrated choriocapillaris, iris
vessels), staining (structures such as collagen), pooling
(pockets of fluid), window defects (RPE defects)
Hypofluorescence: blockage (opacity that reduces
fluorescence; e.g. RPE, blood, xanthophyll) or filling
defect (ischem ia)
Macular dark spot is due to blockage by xanthophyll in
outer plexiform layer and tall RPE cells with increased
m elanin and lipofuscin
Fluorescein passes through Bruch’s m em brane, cannot
Fig ure 11-30. FA demonstrating early venous phase with laminar filling. pass through RPE or retinal capillaries

313
CHAPTER 11 • Posterior Segm ent

A B
Fig ure 11-33. Serous retinal pigment epithelial detachment seen on FA: A, with associated hot spot seen on ICG (B). (From Reichel E: Indocyanine green angiography.
In: Yanoff M, Duker J S (eds ): Ophthalmology. London, 1999, Mosby, 1999.)

Autofluorescence: fluorescence prior to fluorescein Rarely affects vision, but m ay prevent visualization of posterior
dye injection; seen in optic nerve drusen, pole, use FA to look for abnorm alities in these patients
astrocytic ham artom as, and large deposits of
lipofuscin Path o lo gy: gray spheres with “Maltese cross” birefringence
Choroidal filling: choroidal lesions (m alignant m elanom a, on polarization
cavernous hem angiom a) and cilioretinal artery
Arterial phase filling: retinal lesions (capillary S yn c h is is s c in t illa n s (Ch o le s t e ro l b u lb i)
hem angiom a, NVD)
Cholesterol crystals derived from old vitreous hem orrhage;
with PVD crystals settle inferiorly
In d o c ya n in e g re e n (ICG)
Rare, unilateral
Sterile, water-soluble, tricarbocyanine dye with a m olecular
weight of 775 Da. Absorbs light in near-infrared range (790– Occurs after blunt or penetrating traum a in blind eyes
805 nm ), em its at 770–880 nm (peak at 835 nm ) range, which
Crystals sink to bottom of globe because no fixed vitreous
penetrates RPE, blood, and other ocular pigm ents to greater
fram ework
extent than visible light and fluorescein (60–75% of blue light
absorbed by RPE and choroid)
P rim a r y a m ylo id o s is
98% bound to serum proteins (80% to globulins such as
alpha-lipoproteins); therefore, less dye escapes through Vitreous involvem ent in fam ilial am yloidotic
choriocappilaris fenestrations allowing enhanced im aging of polyneuropathies (FAP I and II get system ic m anifestations)
the choroidal circulation; CNV often appears as hot spot Am yloid enters via retinal vessels
(bright area; occurs within 3–5 m in, lasts 20 m in) (Fig. 11-33)
Patients have cardiac disease and am yloid neuropathy
Excreted via liver into bile
Safer than fluorescein angiography; nausea and vom iting less P o s t e rio r vit re o u s d e t a c h m e n t (P VD)
com m on; should not be done in patients allergic to iodine,
Separation of posterior hyaloid face from retina
urem ic, or with liver disease
Mech an ism : vitreous syneresis (liquefaction) and
contraction with age
DISORDERS
Sym p to m s: floaters; m ay see flashes (due to traction on
retina)
Vitre o us Ab no rm a litie s
Fin d in gs: acute sym ptom atic PVD m ay have retinal tear
As t e ro id h ya lo s is (10–15% of acute sym ptom atic PVDs), vitreous hem orrhage
(hem orrhagic PVD; 7.5% of PVDs) if vessel is torn during
Refractile particles (calcium soaps) suspended in vitreous
vitreous separation (70% risk of retinal tear), retinal
More com m on in older patients and those with diabetes; 25% detachm ent (RD) especially when pigm ented vitreous cell is
bilateral present (Schaffer's sign)

314
 Disorders

Vit re o u s h e m o rr h a g e (VH) Ch o ro id a l ru p t u re
Etio lo gy: diabetes (m ost com m on), other proliferative Tear in choroid, Bruch’s m em brane, and RPE due to blunt or
retinopathies, traum a, PVD, Terson’s syndrom e (blood from penetrating traum a
subarachnoid hem orrhage travels along optic nerve and into
eye), ruptured retinal arterial m acroaneurysm , retinal angiom a; Mech an ism : m echanical deform ation results in rupture of
in children, consider child abuse, pars planitis, X-linked choroid; sclera is resistant due to high tensile strength, retina is
retinoschisis resistant due to elasticity; Bruch’s m em brane is less elastic and
breaks with choroid and RPE
Och r e m em b r an e: results from chronic hem orrhage
accum ulating on posterior surface of detached vitreous Dir ect: occurs anteriorly at site of im pact; oriented parallel to
ora serrata
P e rs is t e n t h yp e rp la s t ic p rim a ry vit re o u s (P HP V)
(See Chapter 5, Pediatrics/Strabism us) In d ir ect: occurs posteriorly away from site of im pact; usually
crescent-shaped, concentric with and tem poral to optic disc;
often associated with VH
Re tina l Ab no rm a litie s
Fin d in gs: choroidal neovascular m em brane (CNV) can
Co n g e n it a l develop during healing process (m onths to years after traum a;
can regress spontaneously), scar form s by 3–4 weeks,
(See Chapter 5, Pediatrics/Strabism us)
hyperplasia of RPE at m argin of lesion (Fig. 11-35)
Tra u m a
Re t in a s c lo p e t a ria
Co m m o t io re t in a e (Be rlin ’s e d e m a )
Traum a to retina and choroid caused by transm itted shock
Transient retinal whitening at level of deep sensory retina due waves and necrosis from high-velocity projectile
to disruption (probably photoreceptor outer segm ents) with
photoreceptor loss and thinning of outer nuclear and
plexiform layers; not true edem a Fin d in gs: rupture of choroid and retina with hem orrhage
and com m otio; vitreous hem orrhage can occur; lesion heals
Pigm entary changes can occur (RPE hyperplasia); traum atic with white fibrous scar and RPE changes (Fig. 11-36)
m acular hole m ay develop; usually resolves without sequelae
(Fig. 11-34) Low risk of RD in young patients with form ed vitreous;
posterior vitreous face usually intact; choroid and retina tightly
adherent
Co n t u s io n o f RP E
Blunt traum a can cause RPE edem a with overlying serous RD Tra u m a t ic re t in a l t e a r
Most patients are young with form ed vitreous that tam ponades
the tear

Fig ure 11-34. Berlin’s edema (commotio retinae) in a patient after blunt ocular Fig ure 11-35. Choroidal rupture after blunt trauma. (From Rubsamen PE:
trauma. (From Rubsamen PE: Posterior segment ocular trauma. In: Yanoff M, Posterior segment ocular trauma. In: Yanoff M, Duker J S (eds): Ophthalmology.
Duker J S (eds): Ophthalmology. London, 1999, Mosby, 1999.) London, 1999, Mosby.)

315
CHAPTER 11 • Posterior Segm ent

P u rt s c h e r’s re t in o p a t h y
Due to head traum a or com pressive injury to trunk
Unilateral or bilateral

Fin d in gs: retinal whitening, hem orrhages, cotton wool

spots, papillitis; m ay have positive RAPD (Fig. 11-37)

DDx: pancreatitis, fat em boli, lupus, leukem ia, am niotic

fluid em boli, derm atom yositis

FA: leakage from retinal vasculature, late venous staining

May take up to 3 m onths to resolve

Te rs o n ’s s yn d ro m e
A
Vitreous hem orrhage following subarachnoid or subdural
hem orrhage due to intracranial hypertension blocking venous
return from eye; patients have acute neck stiffness
20% of patients with spontaneous or traumatic
subarachnoid hemorrhage will present with vitreous hemorrhage;
bleeding can also occur between ILM and NFL (Fig. 11-38)

B
Fig ure 11-36. Gunshot wound to the periocular region demonstrating
appearance of retina sclopetaria: A, acute; B, chronic. (From Rubsamen PE:
Posterior segment ocular trauma. In: Yanoff M, Duker J S (eds ): Ophthalmology.
London, 1999, Mosby.)

As vitreous liquefies over tim e, fluid passes through breaks,

causing retina to detach
Fig ure 11-37. Purtscher’s retinopathy. (From Regillo CD: Distant trauma with
Traum a is associated with 10% to 20% of all phakic RDs posterior segment effects. In: Yanoff M, Duker J S (eds): Ophthalmology. London,
1999, Mosby.)

4 typ es o f tr au m atic r etin al b r eaks: horseshoe tear,

operculated tear, retinal dialysis, m acular hole (rare)
Dialysis: m ost com m on type of retinal tear associated with
traum atic RD; usually inferotem poral (31%) or
superonasal (22%); 50% have dem arcation line; 10% of
dialysis-related RDs are present on initial exam ination,
30% occur within 1 m onth, 50% within 8 m onths, 80%
within 2 years
Avulsion of vitreous base: separation of vitreous base from
ora serrata; pathognom onic for trauma
Oral tear: at ora serrata; due to split of vitreous; fish-m outh
appearance
Preoral tear: anterior border of vitreous base; m ost often
superotem poral

Tr eatm en t: laser therapy or cryotherapy for horseshoe tear,

operculated tear, and retinal dialysis without RD; vitrectom y Fig ure 11-38. Terson’s syndrome. (From Regillo CD: Distant trauma with
with gas for m acular hole; scleral buckle for RD due to retinal posterior segment effects. In: Yanoff M, Duker J S (eds): Ophthalmology.
tear; proliferative vitreoretinopathy (PVR) is uncom m on London, 1999, Mosby.)

316
 Disorders

Va ls a lva re t in o p a t h y pa rtia l-thickne s s hole

1b 2
Rise in intrathoracic or intra-abdom inal pressure against a
closed glottis (Valsalva m aneuver) causes superficial veins to
rupture with hem orrhage under ILM
Preretinal hem orrhage in m acula causes sudden decreased
vision

hya loida l tra ction ope rculum

Fa t e m b o li s yn d ro m e
3 4
Follows fracture of m edullated bones; occurs in 5% of patients
with long-bone fractures
Affects m ultiple organ system s

Fin d in gs (in 50%): cotton wool spots, sm all blot

hem orrhages; rarely intravenous fat or CRAO
full-thickne s s hole
20% m ortality
Fig ure 11-39. Optical coherence tomography scans demonstrating cross-
Wh ip la s h re t in o p a t h y sectional image of all stages of macular hole formation and the full-thickness
retinal defect. (From Kaiser PK, Friedman NJ , Pineda R II: The Massachusetts
Associated with severe flexion / extension of head and neck eye and ear infirmary illustrated manual of ophthalmology, ed 2, Philadelphia,
without direct eye injury 2004, Saunders.)

Fin d in gs: m ild reduction in vision (to 20/30); gray swelling Gass classificatio n (Fig. 11-39):
of fovea, foveal pit (50–100 µm ) 1. Prem acular or im pending hole with foveal
detachm ent and m acular cyst (yellow spot [1a] or
FA: m ay have tiny focal area of early hyperfluorescence ring [1b])
2. Full-thickness eccentric hole; usually < 400 µm
Ma c u la r Dis e a s e s in width
3. Full-thickness hole with operculum, cuff of subretinal
Ep ir e t in a l m e m b r a n e (Ce llo p h a n e fluid, yellow deposits at base, positive Watzke-Allen sign
m a c u lo p a t h y, m a c u la r p u c ke r) 4. Full-thickness hole with PVD
Proliferations at vitreoretinal junction, m ay contract and cause
retinal striae, folds and m acular edem a
Watzke-Allen sign : shine narrow-slit beam over m acular
12% prevalence in individuals 43–86 years old; 20% bilateral; hole, positive if patient perceives “break” in slit beam
2% associated with retinal folds (decreased vision)
Associated with diabetes, retinal vascular occlusions, DDx: epiretinal m em brane with pseudohole, lam ellar hole,
anom alous, PVD, high m yopia, retinal hole / tear, previous vitreom acular traction / detachm ent
ocular or laser surgery, and increasing age

FA: window defect corresponding to the full-thickness

OCT: epiretinal m em brane visible on surface of retina, m acular hole (hyperfluorescence during choroidal filling)
distortion of retinal surface evident in 3D m ap; m ay produce
intraretinal cysts and fluid as well as localized traction retinal
detachment OCT: differentiates between various stages and other disease,
hole always evident on scan; often have rim of SRF and cysts
within edges of hole, occasionally operculum can be seen over
Tr eatm en t: consider surgery for decreased vision (< 20/50), the hole
m arked retinal distortion, or m etam orphopsia

Fu ll-t h ic kn e s s m a c u la r h o le Tr eatm en t: stage 1 also known as vitreom acular traction so

no full-thickness hole present m ay close spontaneously so
Due to tangential traction on foveal region by posterior cortical observation; if it worsens can consider intravitreal injection of
vitreous recom binant m icroplasm in (ocriplasm in (Jetrea)); consider
vitrectom y with peeling of posterior hyaloid and gas
Most com m only idiopathic (senile); m ay develop after traum a,
tam ponade for stage 2 through stage 4; ocriplasm in (Jetrea)
surgery, CME, or inflam m ation
can also close sm aller MH in presence of vitreom acular traction
Fem ale > m ale; bilateral in 25–30%; prevalence ¼ 0.33%; in up to 40% of cases
average age of onset is 67 years; risk of developing in fellow eye Complications of surgery: increased size of hole, RPE
< 1% (no risk if PVD present); if traction is present risk rises to m ottling, light toxicity, cataract, retinal tear, retinal
40% (also known as Stage 0 m acular hole) detachm ent, endophthalm itis

317
CHAPTER 11 • Posterior Segm ent

Pr o gn o sis: good if recent onset and hole width < 400 µm ,

poor if > 1 year duration and larger holes

Tra u m a t ic m a c u la r h o le
Rare (5%); due to disruption and necrosis of retinal
photoreceptors with subsequent loss of retinal tissue; results
from pre-existing com m otio retinae in m acula

S o la r re t in o p a t h y
Photochem ical retinal dam age can occur after $ 90 seconds or
longer of sungazing, thought to be caused by blue (441 nm )
and near UV light (325–250 nm )
With foveal fixation, retinal im age of sun is 160 µm and is
usually within the foveola and FAZ
Associated with solar eclipse, psychiatric disorders, religious
Fig ure 11-41. Fluorescein angiography of s olar retinopathy in the
rituals, or ingestion of hallucinogens
left eye. (Courtesy of William E. Benson. From Baumal CR: Light toxicity and laser
burns. In: Yanoff M, Duker J S (eds ): Ophthalmology. London, 1999, Mosby.)
Sym p to m s: vision can range from norm al to 20/100;
usually returns to 20/20 to 20/40 within 6 m onths

Fin d in gs: yellow-white spot in fovea; later, red foveolar

depression or lam ellar hole (Fig. 11-40)

FA: intense staining of dam aged RPE, particularly in acute

phase of injury, but no leakage; as RPE heals, window defects
develop (Fig. 11-41)

Ce n t ra l s e ro u s re t in o p a t h y / c h o rio re t in o p a t h y
(CS R; id io p a t h ic c e n t ra l s e ro u s c h o ro id o p a t h y
(ICS C))
Serous retinal detachm ent Æretinal pigm ent epithelium
detachm ent (PED) (Fig. 11-42) pigme nt e pithe lia l de ta chme nt

Males (80%), typically in 4th–5th decade Fig ure 11-42. Idiopathic central serous retinopathy with large serous retinal
detachment. (From Kaiser PK, Friedman NJ , Pineda R II: The Massachusetts eye
Associated with hypertension, steroid use, psychiatric and ear infirmary illustrated manual of ophthalmology, ed 2, Philadelphia, 2004,
m edication use, and type A personality Saunders.)

A B
Fig ure 11-40. Solar retinopathy of both eyes. (Courtesy of William E. Benson. From Baumal CR: Light toxicity and laser burns. In: Yanoff M, Duker J S (eds):
Ophthalmology. London, 1999, Mosby.)

318
 Disorders

Fin d in gs: blurred vision, m icropsia, paracentral scotom a; Pr o gn o sis: 90% spontaneous resorption in 1–6 m onths;
poor color vision; induced hyperopia, absent foveal reflex; after 50% recur; 66% achieve 20/20; 14% with bilateral vision loss
resolution, m ay have yellow subretinal deposits, RPE changes over 10 years

DDx: AMD, VKH syndrom e, uveal effusion syndrom e, Re t in a l p ig m e n t e p it h e lia l d e t a c h m e n t (P ED)

toxem ia of pregnancy, optic nerve pit, pigm ent epithelial
detachment from other causes (CNV) Appears as discrete, blister-like elevation; som etim es outlined
by orange-pink rim of subretinal fluid

FA: sm all focal hyperfluorescent dot (leakage of dye from

choroid through RPE); later, dye accum ulates beneath DDx:
neurosensory detachm ent; “sm okestack” appearance in 10%; In patient < 50 years old: probably due to central serous
expanding dot of hyperfluorescence in 80%, diffuse leakage in retinopathy
the rest (Fig. 11-43) In patient > 50 years old: PED associated with drusen m ay
indicate occult CNV, especially if PED has a notch

OCT: sensory retinal elevation, m ay have PED (usually

within or near subretinal fluid accum ulation); thickened FA: discrete early hyperfluorescence of entire serous PED with
choroid late pooling into PED

ICG: can be useful in helping to distinguish atypical diffuse Co m p licatio n s: 33% develop CNV(FA shows slower, m ore
CSR in older patients from occult CNV in exudative AMD and hom ogenous filling with sharp border) term ed pachychoroidal
idiopathic polypoidal choroidal vasculopathy neovascularization

FAF: teardrop-shaped area of hyperautofluoresence Ag e -re la t e d m a c u la r d e g e n e ra t io n (ARMD, AMD)

corresponding to dependent m igration of fluid
Leading cause of central visual loss in patients > 60 years old in
the United States and Western world
Tr eatm en t: observation in m ost cases, consider laser to focal
hot spots outside fovea or verteporfin (Visudyne) ocular
photodynam ic therapy (PDT) for subfoveal spots or diffuse
Risk facto r s: age, heredity, sex (fem ale), race (white),
sm oking, nutrition, photic exposure, hypertension, light iris
leakage (off label); recently m ineralocorticoid antagonists have
color, hyperopia
been shown to be beneficial, especially in chronic cases
including spironolactone or eplerenone (off-label)
Treatment indications: Sym p to m s: decreased vision, central scotom a,
1. Persistent serous detachm ent (> 3 m onths) m etam orphopsia
2. Previous episode of CSR, with perm anent vision
reduction
3. Episode in fellow eye with vision loss
Fo r m s:
1. Non exudative or dry (80–90%): drusen, pigm ent
4. Dem and for rapid recovery of binocular function for
changes, RPE atrophy (Figs. 11-44 to 11-47)
occupational reasons
2. Exudative, n eovascular, or wet (10-20%):
Laser and PDT accelerate resolution of fluid, but do not result
characterized by CNV (Figs. 11-48 to 11-50)
in better final vision or reduce rate of recurrence; laser m ay
rarely cause CNV

pigme nt e pithe lia l “s moke s ta ck”

de ta chme nt le a ka ge
Fig ure 11-43. Fluorescein angiogram demonstrating classic smokestack
appearance in central serous chorioretinopathy. (From Kaiser PK, Friedman NJ , Fig ure 11-44. Nodular “hard” drusen. (From Edwards MG, Bressler NM,
Pineda R II: The Massachusetts eye and ear infirmary illustrated manual of Raja SC: Age-related macular degeneration. In: Yanoff M, Duker J S (eds):
ophthalmology, ed 2, Philadelphia, 2004, Saunders.) Ophthalmology. London, 1999, Mosby.)

319
CHAPTER 11 • Posterior Segm ent

drus e n s ubre tina l choroida l

he morrha ge ne ova s cula r
me mbra ne
ha rd drus e n s oft drus e n
Fig ure 11-48. Exudative age-related macular degeneration, demonstrating
Fig ure 11-45. Dry, age-related macular degeneration demonstrating drusen and
subretinal hemorrhage from classic choroidal neovascular membrane. (From
pigmentary changes (category 3). (From Kaiser PK, Friedman NJ , Pineda R II:
Kaiser PK, Friedman NJ , Pineda R II: The Massachusetts eye and ear infirmary
The Massachusetts eye and ear infirmary illustrated manual of ophthalmology,
illustrated manual of ophthalmology, ed 2, Philadelphia, 2004, Saunders.)
ed 2, Philadelphia, 2004, Saunders.)

choroida l he morrha ge
ne ova s cula r
me mbra ne
ge ogra phic a trophy
Fig ure 11-49. Fluorescein angiogram of same patient as in Fig. 11-48,
Fig ure 11-46. Advanced, atrophic, nonexudative, age-related macular demonstrating leakage from the CNV and blockage from the surrounding
degeneration demonstrating subfoveal geographic atrophy (category 4). (From subretinal blood. (From Kaiser PK, Friedman NJ , Pineda R II: The Massachusetts
Kaiser PK, Friedman NJ , Pineda R II: The Massachusetts eye and ear infirmary eye and ear infirmary illustrated manual of ophthalmology, ed 2, Philadelphia,
illustrated manual of ophthalmology, ed 2, Philadelphia, 2004, Saunders.) 2004, Saunders.)

Dr u sen : focal deposits of extracellular debris located

between basal lam ina of retinal pigm ent epithelium (RPE) and
inner collagenous layer of Bruch's m em brane; clinical m arker
for sick RPE; PAS-positive, m ildly eosinophilic hyaline
excrescences of abnorm al basem ent m em brane m aterial;
found to contain lipid, m ainly esterified cholesterol,
unesterified cholesterol, and phosphatidylcholine;
carbohydrates, zinc, and nearly 150 proteins, including
vitronectin, apolipoproteins E and B, and num erous
com ponents of com plem ent system

ge ogra phic a trophy Typ es o f d r u sen :

Hard (nodular / cuticular / hyaline): sm all yellow-white
Fig ure 11-47. Fluorescein angiogram of same patient as in Fig. 11-46, spots 50 µm in diam eter
demonstrating well-defined window defect corresponding to the area of
geographic atrophy. (From Kaiser PK, Friedman NJ , Pineda R II: The
Soft: larger (63–1000 µm ), less dense, m ore fluffy, with
Massachusetts eye and ear infirmary illustrated manual of ophthalmology, ed 2, tapered edges; m ay resem ble PED; associated with
Philadelphia, 2004, Saunders.) thickened inner Bruch’s m em brane and wet AMD

320
 Disorders

A B

Fig ure 11-50. A-C, Neovascular, age-related macular degeneration. (From Edwards MG, Bressler NM, Raja SC: Age-related macular degeneration. In: Yanoff M, Duker
J S (eds): Ophthalmology. London, 1999, Mosby.)

Basal laminar (cuticular): diffuse, confluent, densely by 20 drusen each having a diam eter of 100 µm . If no soft
packed sm all yellow-white (blocks early, stains late indistinct drusen are present, interm ediate drusen are
on FA, “starry sky” appearance) produces sawtooth considered to be extensive when they occupy an area
pattern of sm all blunted triangles that sit on Bruch's equivalent to at least 1 ⁄5 of a disc area (approxim ately 65,
m em brane on OCT 100 µm diam eter drusen)
Calcific: sharply dem arcated, glistening; associated with
RPE/geographic atrophy DDx o f yello w fo veal sp o t: solar m aculopathy,
Reticular pseudodrusen or subretinal drusenoid adult vitelliform dystrophy, Best disease, stage 1a
debris: located in sub-retinal space not sub-RPE m acular hole, CSR, old subfoveal hem orrhage, CME, pattern
dystrophy

Dr u sen size classificatio n : FA: hyperfluorescence of drusen due to window defects (from
Small: < 64 µm in diam eter degeneration of overlying RPE) and uptake of dye within
Intermediate: 64–124 µm in diam eter drusen (staining)
Large: ! 125 µm in diam eter (approxim ately equal to vein
width at disk m argin)
Also classified based on drusen extent:
OCT: drusen appear as elevations of RPE; useful to
differentiate dry from wet AMD where the CNV as well as
Sm all drusen considered extensive if the cum ulative area
intraretinal, subretinal, or sub-RPE fluid is evident
within 2 disc diam eters of the center of the m acula equal
to at least that of the AREDS standard circle C-1 (with
diam eter 1 ⁄12 that of the average disc) – this corresponds to Sign s o f CNV: subretinal blood, fluid, and / or
approxim ately 15 sm all drusen from stereo photographs lipid; RPE detachm ent (PED); gray-green subretinal
or 5–10 sm all drusen discoloration
Interm ediate drusen considered extensive if soft, indistinct
drusen are present and the total area occupied by the Typ es o f CNV: historically defined by appearance of leakage
drusen is equivalent to the area that would be occupied on FA

321
CHAPTER 11 • Posterior Segm ent

Classic: treated eyes had > 6 lines of vision loss; > 50%
EARLY PHASE: bright, fairly uniform hyperfluorescence recurrence rate. However, m any patients do not
that progressively intensifies throughout the qualify owing to subfoveal CNV and occult lesions
transit phase Treatment of Age-Related Macular Degeneration with
LATE PHASE: progressive leakage of dye that extends Photodynamic Therapy (TAP) Trial:
beyond the m argins of the CNV seen in early stages Photodynamic therapy with verteporfin (Visudyne) can
Occult (2 types): prevent vision loss in eyes with subfoveal, predomi-
FIBROVASCULAR PED: nantly classic and occult AMD, with no classic CNV
EARLY PHASE: irregular m ottled hyperfluorescence at Verteporfin in Photodynamic Therapy (VIP) Trial:
level of RPE Photodynam ic therapy with verteporfin (Visudyne) can
LATE PHASE: stippled hyperfluorescent leakage that is prevent vision loss in eyes with occult and no classic
not as bright or extensive as classic lesions CNV, especially if less than 4 MPS Da in size or
LATE LEAKAGE OF UNDETERMINED ORIGIN: baseline vision worse than 20/50
EARLY PHASE: no apparent leakage Pegaptanib (Macugen) VISION Trial:
LATE PHASE: stippled hyperfluorescence at level FDA-approved aptamer that is a selective VEGF antagonist
of RPE binding to the 165 isoform of VEGF-A while sparing
Can also be defined by lesion location: other isoforms; no longer commonly used; intravitreal
TYPE 1: CNV under RPE; PCV is a variant of type 1 injection every 6 weeks.
TYPE 2: CNV above RPE Ranibizumab (Lucentis) MARINA and ANCHOR Trials:
TYPE 3: retinal angiomatous proliferation (RAP) ¼ often FDA-approved, hum anized, antigen-binding fragm ent
bilateral with small intraretinal hemorrhages and (Fab) designed to bind and inhibit all VEGF isoform s;
associated PED; develop retinal-choroidal anastamosis intravitreal injection m onthly or as needed (CATT
finding was equal efficacy when delivered m onthly or
Lo catio n o f CNV: as needed).
Extrafoveal: posterior border of CNV is 200–2500 µm from Bevacizumab (Avastin): off-label, full-length antibody that
center of foveal avascular zone (FAZ) binds and inhibits all VEGF isoforms (FDA approved for
Juxtafoveal: 1–199 µm from center of FAZ, or CNV colorectal cancer): the Complications of Age Related
200–2500 µm from center of FAZ with blood or blocked Macular Degeneration Treatment Trials (CATT) study
fluorescence within 200 µm of FAZ center reported that monthly Avastin was non-inferior to monthly
Subfoveal: under center of FAZ lucentis, but as needed Avastin was not equal. There were
significantly greater serious systemic adverse events in the
Avastin groups than lucentis;intravitreal injection monthly.
ICG: helpful for visualizing CNV that is not well seen on FA; Other comparison studies including IVAN, MANTA,
CNV appears as focal hot spots or plaque of late GEFAL, LUCAS have reported similar finding that Lucentis
hyperfluorescence; can dem onstrate CNV under thin and Avastin efficacy are very similar.
hem orrhage that would be blocked on FA; high-speed Aflibercept (VEGF Trap Eye) VIEW 1&2 Trials: fusion
ICG useful to delineate feeder vessels of CNV. Also receptor decoy containing dom ain 2 from VEGF receptor
useful to visualize polyps and branching vascular network 1 and dom ain 3 from VEGFR2 fused to hum an Fc
of PCV fragm ent that blocks all isoform s of VEGF-A, VEGF-B and
placental growth factor (PLGF); labeled indication is
Tr eatm en t: follow Am sler grid, low-vision aids, vitam in injected every two m onths after a loading dose of
supplem ents; consider intravitreal injections (anti-VEGF 3 m onthly injections.
[vascular endothelial growth factor] agents), focal laser, or PDT Anti-VEGF safety: all anti-VEGF agents can get into the
for CNV system ic circulation putting patients at risk of
Age-related Eye Disease Study (AREDS): arteriothrom botic events (ATE) such as hypertension,
Supplements with high-dose antioxidants and zinc are MI, and stroke.
helpful in reducing vision loss and the progression of Combination treatment: Com bination therapy with PDT
disease in patients with category 3 and 4 AMD; and anti-VEGF agents has been shown to be safe with
AREDS2 study evaluated lutein, zeaxanthin, and sim ilar visual results and decreased injections (DENALI
om ega-3 fatty acids in addition to the original AREDS and MT BLANC Studies). In particular, the best therapy
form ulation; form ulations had alm ost equal for PCV is com bination therapy PDT and anti-VEGF
efficacy so AREDS2 recom m ended for sm okers to agents (EVEREST Study). Com bination therapy with
avoid beta-carotene other drugs such as antiplatelet derived growth factor
Vitam in C and E can increase risk of m yocardial (PDGF) is being evaluated in Phase 3.
infarction (MI) in postm enopausal wom en Radiation: internal (strontium -90)(CABERNET Study -
Zinc is associated with benign prostate hypertrophy in failed) or external (low-dose X-ray radiation) com bined
m en and stress incontinence in wom en with anti-VEGF agents (experim ental)
Macular Photocoagulation Study (MPS): Surgery: in certain cases, consider subm acular surgery to
Treatm ent of well-dem arcated extrafoveal or juxtafoveal rem ove CNV or displace hem orrhage in cases with large
CNV with uniform laser. After 5 years, 64% of subm acular hem orrhage, or m acular translocation
untreated eyes had > 6 lines of vision loss; 46% of (experim ental)

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 Disorders

Pr o gn o sis: entire lesion had to m easure less than 3.5 MPS disc
Rate of Progression to Advanced AMD (AREDS) over 5 years: areas in size and could be classic or occult CNV.
1.3% m any sm all or few m edium drusen (if both eyes Patients were random ly assigned to argon green or
have m any interm ediate drusen, but no large drusen, krypton red treatm ent of entire CNV and 100 µm
then patient score ¼ 1 in scale below) beyond the borders of the lesion vs observation
18% m any m edium or any large drusen Subfoveal Recurrent CNV Study: well-dem arcated CNV
43% unilateral advanced AMD due to AMD under the center of foveal avascular zone
AREDS Clinical Severity Scale (AREDS) for AMD: (FAZ) contiguous with previous laser treatm ent scar
Based on giving 1 point for the presence of ! 1 large and with vision between 20/40 and 20/320. The
drusen and / or pigm ent changes (hyper, hypo, or non- entire lesion had to m easure less than 6MPS disc areas
central GA) per eye, or 2 points for advanced AMD in in size and could be classic or occult CNV. Patients
1 eye, then total 2 eyes to com e up with a point score that random ly assigned to argon green or krypton red
shows patient’s risk of developing advanced AMD in treatm ent of entire CNV and 100 µm beyond the
5 years: borders of the lesion vs observation
0 points ¼ 0.5% DEFINITIONS:
1 point ¼ 3% EXTRAFOVEAL: 200–2500 µm from center of foveal
2 points ¼ 12% avascular zone (FAZ)
3 points ¼ 25% JUXTAFOVEAL:, 1–199 µm from center of FAZ
4 points ¼ 50% SUBFOVEAL: extends beneath center of fovea
Risk of CNV in fellow eye at 5 years: if drusen is present MAIN OUTCOME MEASURES: visual acuity, contrast
Hard (nodular) ¼ 10% sensitivity, reading speed, persistent and / or
Soft ¼ 30% recurrent CNV, treatm ent com plications
Pigm ented ¼ 30%
Soft and pigm ented ¼ 60%
Re s u lt s :
Risk of fellow eye developing CNV: 4–12% per year;
increased risk with m ultiple soft drusen, RPE clum ping, Ext ra fo ve a l s t u d y:
densely packed drusen, PED
After 18 months:
AMD: 25% of treated vs 60% of untreated eyes had
MAJ OR AGE-RELATED MACULAR lost ! 6 lines of vision (a quadrupling of the visual
angle [i.e., 20/50 to 20/200])
DEGENERATION CLINICAL STUDIES POHS: 9.4% of treated vs 34.2% of untreated eyes
had lost ! 6 lines of vision
Ma c ula r P ho to c o a g ula tio n Stud y (MP S) IDIOPATHIC: sam ple size (67) was too sm all to
reach clinical significance, but trend was sim ilar to
Ob je c t ive : to evaluate efficacy of laser photocoagulation results for AMD and POHS
in preventing visual loss from CNV Recurrence of CNV after argon laser was 52% with AMD,
28% with POHS, 28% with idiopathic CNV
Me t h o d s : patients were random ly assigned to laser After 3 years:
photocoagulation vs observation in the following groups:
Relative risk of severe vision loss (! 6 lines) for no
Extrafoveal Study: well-dem arcated CNV due to AMD or treatm ent vs treatm ent was 1.4 for AMD, 5.5 for
ocular histoplasm osis syndrom e (OHS), or POHS, and 2.3 for idiopathic CNV
idiopathic, that was 200–2500 µm from center of After 5 years:
foveal avascular zone (FAZ) with vision better than AMD: treated eyes lost 5.2 lines of vision vs 7.1 lines in
20/100. Patients who received previous untreated eyes; recurrence in 54% of treated eyes;
photocoagulation were excluded. Patients were 26% developed CNV in fellow eye
random ly assigned to argon blue-green treatm ent to POHS: treated eyes lost 0.9 line of vision vs 4.4 lines in
the entire CNV and 100–125 µm beyond the borders untreated eyes; recurrence in 26% of treated eyes
of the lesion vs observation IDIOPATHIC: treated eyes lost 2.7 lines of vision vs
Juxtafoveal (Krypton) Study: well-dem arcated CNV due 4.4 lines in untreated eyes; recurrence in 34% of
to AMD or OHS, or idiopathic, that was 1–199 µm treated eyes
from center of foveal avascular zone (FAZ), or with a
CNV m ore than 200 µm with blood or pigm ent
J u xt a fo ve a l s t u d y:
extending within 200 µm and with vision better than After 3 years:
20/400. Patients were random ly assigned to Krypton POHS: 4.6% of treated vs 24.6% of untreated eyes had
red treatm ent of entire CNV and 100 µm beyond the lost ! 6 lines of vision
borders of the lesion except on the foveal side of the IDIOPATHIC: 10% of treated vs 37% of untreated eyes
lesion vs observation had lost ! 6 lines of vision
Subfoveal, New CNV AMD Study: well-dem arcated CNV After 5 years:
due to AMD under the center of foveal avascular zone Relative risk of severe vision loss (! 6 lines) for no
(FAZ) with vision between 20/40 and 20/320. The treatm ent vs treatm ent was 1.2 for AMD

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CHAPTER 11 • Posterior Segm ent

(hypertensive patients had little or no benefit), 4.26 period. All patients were then irradiated with a 689-nm
for POHS, and interm ediate between AMD and diode laser (light dose: 50 J/cm 2 ; power density:
POHS for idiopathic CNV 600 m W/cm 2 ; duration: 83 seconds) 15 m inutes after the
start of infusion
S u b fo ve a l n e w AMD s t u d y:
Re s u lt s : 609 patients enrollm ent (311 in Study A and
After 3 months:
298 in Study B). Vision was stabilized or im proved in 61.4%
AMD: 20% of treated vs 11% of untreated eyes had
of patients treated with Visudyne OPT com pared with
lost ! 6 lines of vision
45.9% treated with placebo at 12 m onths. The difference
After 2 years:
was sustained at 24 and 60 (TAP Extension Study) m onths.
AMD: 20% of treated vs 37% of untreated eyes had
In subgroup analysis, visual acuity benefit was m ost
lost ! 6 lines of vision; recurrence in 51% of
pronounced for lesions in which area of classic CNV
treated eyes
occupied m ore than 50% of entire area of lesion
After 4 years:
(predom inantly classic). Specifically, 33% of Visudyne-
AMD: 22% of treated vs 47% of untreated eyes had lost
treated eyes com pared with 61% of placebo-treated eyes
! 6 lines of vision
sustained moderate visual loss. No difference in visual
S u b fo ve a l re c u rre n t AMD s t u d y: acuity was noted when area of classic CNV was greater than
0% but less than 50% of entire lesion (m inim ally classic).
After 3 years: 12% of treated vs 36% of untreated eyes Sixteen percent of patients experienced an im provem ent in
had lost ! 6 lines of vision vision (1 or m ore lines) in Visudyne-treated group
com pared with 7.2% in control group. Overall, Visudyne
Co n c lu s io n s : group was 34% m ore likely to retain vision. Most patients
Treat extrafoveal CNV in patients with AMD, POHS, and required periodic retreatm ents with an average of 3.4 (of a
idiopathic lesions possible 4) being required in first year, 2.1 in second year
(5.5 total over 24 m onths), and 1.5 in third year (7 total
Patients with juxtafoveal CNV (AMD, POHS, and over 36 m onths)
idiopathic) benefit from krypton laser photocoagulation,
except for hypertensive AMD patients Co n c lu s io n s : Visudyne ocular photodynam ic therapy
AMD patients with subfoveal CNV(new or recurrent) benefit is recom m ended for subfoveal, predom inantly classic, CNV
equally from argon green or krypton red laser treatm ent
For unilateral CNV, large drusen and focal hyperpigmentation Ve rte p o rfin in P ho to d yna m ic The ra p y
are risk factors for development of CNV in fellow eye (VIP ) Tria l; Ve rte p o rfin in P ho to d yna m ic
Treatm ent of AMD patients with juxtafoveal CNV is The ra p y–P a tho lo g ic Myo p ia
beneficial when the lesion is classic, even though CNV (VIP -P M) Tria l
recurs; treatm ent of classic CNV alone in lesions with both
classic and occult CNV was not beneficial Ob je c t ive : to evaluate Visudyne ocular photodynam ic
therapy (OPT) in the m anagem ent of subfoveal CNV not
Tre a tm e nt o f Ag e -Re la te d Ma c u la r included in original TAP investigation

De g e ne ra tio n with P ho to d yna m ic Me t h o d s : patients with evidence of AMD, age > 50 years,
The ra p y (TAP ) Tria l evidence of subfoveal “occult” only CNV by FA with recent
disease progression defined as evidence of hem orrhage, loss
Ob je c t ive : to evaluate verteporfin (Visudyne) ocular of ! 1 line of vision, or increased size of the lesion by 10%
photodynam ic therapy (OPT) in the m anagem ent of during the preceding 3 m onths, and Early Treatm ent
subfoveal CNV with som e classic characteristics Diabetic Retinopathy Study (ETDRS) visual acuity ! 20/
100; or subfoveal “classic” CNV with ETDRS visual acuity of
Me t h o d s : patients with evidence of AMD, age > 50 years, ! 20/40, greatest linear dim ension of CNV < 5400 µm (9
evidence of new or recurrent subfoveal “classic” (can have MPS disc areas), and the ability to return every 3 m onths for
occult features) CNV by fluorescein angiography with 2 years. Patients were excluded who had other ocular
greatest linear dim ension of CNV < 5400 µm (9MPS diseases that could com prom ise visual acuity, history of
disc areas), ETDRS visual acuity of 20/40 to 20/200, and previous experim ental treatm ent for CNV, porphyrin
the ability to return every 3 m onths for 2 years. Patients were allergy, liver problem s, or intraocular surgery within
excluded who had other ocular diseases that previous 2 m onths. Two-thirds of the patients in both
could com prom ise visual acuity, history of previous studies were random ly assigned (2 :1 random ization
experim ental treatm ent for CNV, porphyrin allergy, liver schem e) to receive verteporfin (Visudyne 6 m g/m 2 ) and one
problem s, or intraocular surgery within the previous third to control vehicle (D 5 W IV infusion) infused over a
2 m onths. Two thirds of patients in both studies were 10-m inute period. All patients were then irradiated with the
random ly assigned (2:1 random ization schem e) to use of a 689 nm diode laser (light dose: 50 J/cm 2 ; power
receive verteporfin (Visudyne 6 m g/m 2 ) and one third to density: 600 m W/cm 2 ; duration: 83 seconds) 15 m inutes
control vehicle (D 5 W IV infusion) infused over a 10-m inute after the start of the infusion

324
 Disorders

Re s u lt s : 459 patients enrolled. O ne-year results showed Zinc alone: reduced risk of vision loss by 21%
no statistically significant difference between Visudyne- Antioxidants alone: reduced risk of vision loss by 17%
treated patients and placebo (difference 4.2%). However,
by 24 m onths, a statistically significant difference was seen Co n c lu s io n s :
that was due to a decline in vision in control group
(difference 13.7%). Moreover, this difference was m ost High-dose supplements are beneficial in reducing risk of
pronounced in patients with “occult” only CNV lesions vision loss in patients with high-risk AMD (categories 3
m easurin g < 4MPS disk areas in size at baselin e, or who and 4). Caution should be exercised in smokers or recent
had baseline visual acuity of 20/50 or worse smokers in use of high-dose beta-carotene because of
possible increased risk of lung cancer
Few ocular or other system ic adverse events were seen with
Visudyne therapy. In 4.4% of patients, an im m ediate severe Treatm ent is of no benefit in patients with no AMD or early
visual decrease within 7 days of treatm ent was observed AMD (several sm all or interm ediate drusen)
Treatm ent does not affect developm ent or progression of
Co n c lu s io n s : Visudyne ocular photodynamic therapy
cataract
(OPT) is recommended in the m anagement of subfoveal
occult but not classic CNV when there is evidence of recent
disease progression, especially if baseline lesion size is smaller
Ag e -Re la te d Eye Dis e a s e Stud y
than 4 MPS DA, or the baseline vision is worse than 20/50 (AREDS2)
Ob je c t ive : to evaluate the effect of high-dose
Ag e -Re la te d Eye Dis e a s e supplem ents, m acular carotenoids, and om ega-3-fatty acid
Stud y (AREDS) on the progression of AMD

Ob je c t ive : to evaluate the effect of high-dose Me t h o d s : 4203 patients random ized to receive
supplem ents on the progression of AMD, and on the various com binations of vitam in C (500 m g), vitam in E
developm ent and progression of cataracts (400 IU), beta-carotene (vitam in A, 15 m g), zinc (80 m g
plus 2 m g copper), lutein (10 m g), zeaxanthin (2 m g),
Me t h o d s : patients aged 55–80 years with 20/32 or better om ega-3 long-chain polyunsaturated fatty acids
vision OU, or 20/32 or better in one eye and AMD in fellow (LCPUFA) in the form of docosahexaenoic acid
eye, received antioxidants (vitam in C [500 m g], vitam in E (DHA) (350 m g) and eicosapentaenoic acid (EPA)
[400 IU], beta-carotene [vitam in A, 15 m g]), zinc (80 m g (650 m g)
plus 2 m g copper), both, or placebo
Re s u lt s : original AREDS form ula reduced risk of
Categorized into 4 groups: progression to advanced AMD. Adding lutein and
Category 1: fewer than 5 sm all (< 63 µm ) drusen zeaxanthin provided about 20% reduction in progression
Category 2 (m ild AMD): m ultiple sm all drusen or single beyond original AREDS, in those who had the lowest dietary
or nonextensive interm ediate (63–124 µm ) drusen, or intake of lutein and zeaxanthin. Addition of om ega-3 did
pigm ent abnorm alities not reduce risk of progression
Category 3 (interm ediate AMD): extensive
interm ediate-sized drusen, or 1 or m ore large Co n c lu s io n s : National Eye Institute recom m ends
(> 125 µm ) drusen, or noncentral geographic atrophy AREDS form ula be adjusted by rem oval of betacarotene and
Category 4 (advanced AMD): vision loss (< 20/32) addition of lutein and zeaxanthin
due to AMD in 1 eye (due to either central / subfoveal
geographic atrophy or exudative m acular degeneration VEGF Inhib itio n Stud y in Oc ula r
Prim ary outcom es:
Progression to advanced AMD: treatm ent for CNV or Ne o va s c ula riza tio n (VISION) Tria l
photographic evidence (geographic atrophy of center
of m acula, nondrusenoid RPE detachm ent, serous or Ob je c t ive : to evaluate intravitreal pegaptanib for
hem orrhagic RD, subretinal hem orrhage or fibrosis) subfoveal CNV due to neovascular AMD
Moderate vision loss: ! 15-letter loss (doubling of Me t h o d s : 2 concurrent random ized, double-m asked
visual angle) clinical trials, 1208 patients received either pegaptanib
Development and progression of lens opacities intravitreal injection (0.3 m g, 1.0 m g, or 3.0 m g) or a
Re s u lt s : 4757 patients enrolled sham injection into study eye every 6 weeks for total of
48 weeks. Patients were eligible for trial if they were 50 years
Antioxidants plus zinc: reduced the risk of progression to old or older and had subfoveal classic, m inim ally classic,
advanced AMD and vision loss over 6 years in 25% of and / or occult CNV due to wet AMD with best-corrected
high-risk patients visual acuity of 20/40 to 20/320 in study eye.
HIGH-RISK PATIENTS: intermediate AMD (many inter-
mediate drusen [63–124 µm] or 1 large drusen Re s u lt s : on average, patients treated with pegaptanib
[! 125 µm] in 1 or both eyes) or advanced AMD (in 1 0.3 m g and sham -treated patients continued to
eye only) experience vision loss. However, rate of visual acuity decline

325
CHAPTER 11 • Posterior Segm ent

in pegaptanib-treated group was slower than rate in com pared ranibizumab with the active control verteporfin
patients who received sham treatm ent; 70% of patients PDTin subfoveal predominantly classic CNVdue to wet AMD
treated with pegaptanib sodium injection (0.3 m g; n ¼ 294)
lost fewer than 15 letters of visual acuity com pared with 55% Re s u lt s : enrolled 423 patients with predom inantly
in control group (n ¼ 296; P < 0.001); 10% of patients treated classic subfoveal CNV associated with AMD. Prim ary
with pegaptanib sodium injection (0.3 m g; n ¼ 294) had outcom e was prevention of m oderate visual loss ( 15
severe visual acuity loss (30 letters or m ore) com pared with letters loss of vision), which was seen in 94.3% with
22% in the control group (n ¼ 296; P < 0.001). Beneficial ranibizum ab 0.3 m g, 96.4% with ranibizum ab 0.5 m g and
effect was observed for all subtypes of neovascularization 64.3% of patients receiving PDT. Vision im proved by ! 15
(NV) and was sustained for up to 2 years of follow-up letters in significantly m ore ranibizum ab-treated patients
(35.7% for 0.3 m g and 40.3% for 0.5 m g) than PDT-treated
Co n c lu s io n s : pegaptanib was better than sham and patients (5.6%). At 12 m onths, m ean change in VA
PDT for neovascular AMD increased by +8.5 letters in ranibizum ab 0.3 m g group and
by +11.3 letters in 0.5 m g group, but decreased by À9.5
letters in sham group
Minim a lly Cla s s ic / Oc c ult Tria l o f the
Anti-VEGF Antib o d y Ra nib izum a b in the Co n c lu s io n s : ranibizum ab was superior to verteporfin
for treatm ent of predom inantly classic CNV due to
Tre a tm e nt o f Ne o va s c ula r AMD neovascular AMD
(MARINA) Tria l
Aflib e rc e p t fo r Ag e Re la te d Ma c ula r
Ob je c t ive : pivotal phase III, m ulticenter, double-blind De g e ne ra tio n Stud y (VIEW 1/2)
24-m onth study, which com pared m onthly intravitreal
injections of ranibizum ab 0.3 or 0.5 m g or sham injections
(n ¼ 716) in patients with subfoveal occult only or Ob je c t ive : phase 2 studies to evaluate three different
m inim ally classic CNV due to wet AMD dosing regim ens of aflibercept, 0.5 m g every four weeks,
2 m g every four weeks, and 2 m g every eight weeks
Re s u lt s : enrolled 716 patients with m inim ally classic (following three initial m onthly injections), com pared to
and occult subfoveal CNV associated with AMD. Prim ary ranibizum ab 0.5 m g every four weeks. 2-year study
outcom e was prevention of m oderate visual loss ( 15
letters loss of vision), which was seen in 94.5% with Re s u lt s : integrated analysis of VIEW 1 and VIEW 2
ranibizum ab 0.3 m g, 94.6% with ranibizum ab 0.5 m g and studies, visual acuity gain from baseline in aflibercept 2 m g
62.2% of patients receiving sham injections (P < 0.001). every eight week group at week 96 was 7.6 letters com pared
Vision im proved by ! 15 letters for a significantly greater to 8.4 letters at week 52, with an average of 11.2 injections
num ber of ranibizum ab-treated patients (24.8% for 0.3 m g over two years and 4.2 injections during the second year.
and 33.8% for 0.5 m g) versus sham -treated patients (5.0%). Visual acuity gain from baseline in m onthly ranibizum ab
Mean increases in VA from baseline were +6.5 letters for group at week 96 was 7.9 letters com pared to 8.7 letters at
ranibizum ab 0.3 m g group and +7.2 letters for ranibizum ab week 52, with an average of 16.5 injections over two years
0.5 m g group, whereas sham -injected patients had a m ean and 4.7 injections during the second year. Safety results
decrease of À10.4 letters. This benefit in VAin ranibizum ab- between drugs was sim ilar
treated patients was m aintained through 24 m onths. At
24 m onths, 90% of ranibizum ab-treated patients in
Co n c lu s io n s : VIEW studies found that aflibercept
injected every two m onths was equivalent to ranibizum ab
MARINA study lost less than 15 letters of visual acuity; 33%
injected m onthly after 3 m onthly loadiung doses. 94%
gained 15 or m ore letters of visual acuity. Ranibizum ab-
(VIEW 1) and 95% (VIEW 2) of patients treated with
treated patients exhibited a statistically significant
aflibercept or ranibizum ab m aintained vision (< 15 letters
im provem ent com pared with sham -treated patients in all
loss) at two years
subgroups for all outcom e m easures.

Co n c lu s io n s : ranibizum ab was better than sham for

occult with no classic and m inim ally classic CNV due to Co m p a ris o n o f AMD Tre a tm e nt Tria ls
neovascular AMD (CATT)

Anti-Va s c ula r End o the lia l Gro wth Fa c to r Ob je c t ive : CATT was designed to com pare Avastin and
(VEGF) Antib o dy fo r The Tre a tm e nt o f Lucentis with m onthly and as-needed treatm ent schedules
for 2 years. After year one, patients initially assigned to
P re do m ina ntly Cla s s ic Cho ro ida l m onthly treatment were random ly reassigned to m onthly or
Ne o va s c ula riza tion (CNV) in Age -Re la te d as needed treatm ent without changing their drug
Ma c ula r De g e ne ra tio n (ANCHOR) Tria l assignm ent

Ob je c t ive : second pivotal phase III, m ulticenter, Re s u lt s : 1107 patients included. After 2-years,
randomized, double-m asked 24-month clinical trial, which differences in visual acuity outcom es between drugs when

326
 Disorders

given m onthly or as needed were not statistically significant; treating neovascular AMD at up to two years. However,
m ean gain in visual acuity was sim ilar for both drugs there are concerns that bevacizum ab m ay have worse side
(bevacizum ab-ranibizum ab difference, 1.4 letters). Mean effect profile due to its greater system ic bioavailability. In
gain was greater for m onthly treatm ent than for as-needed addition, PRN dosing was not as efficacious as fixed dosing,
treatm ent (difference, -2.4 letters). However, m any of especially with bevacizum ab
secondary anatom ic outcom es suggested that bevacizum ab
is biologically inferior to ranibizum ab. Proportion of
patients who were com pletely dry as m easured by OCT was
13.9% with PRN bevacizum ab vs 30.2% with m onthly Ot h e r Dis o rd e rs As s o c ia t e d w it h Ch o ro id a l
bevacizum ab, and 22.3% with PRN ranibizum ab vs 45.5% Ne o va s c u la r Me m b ra n e (CNV)
with m onthly ranibizum ab. Eyes receiving PRN
bevacizum ab required m ean of 14.1 injections vs 12.6 with
Tr eatm en t fo r all CNV: consider laser only for extrafoveal
lesions (MPS showed that laser treatm ent for juxtafoveal and
PRN ranibizum ab. Macular atrophy was found to be greater
extrafoveal CNV was beneficial); juxtafoveal and subfoveal
in groups that received m onthly treatm ent (19% with
lesions are treated with anti-VEGF agents or photodynam ic
bevacizum ab and 30% with ranibizum ab) than PRN
therapy (see above)
treatm ent groups (14% with bevacizum ab and 16% with
ranibizum ab). The significance of this is unknown. Arterial
throm botic event rates were sim ilar between the 2 drugs, but P re s u m e d o c u la r h is t o p la s m o s is
proportion of patients with 1 or m ore serious system ic s yn d ro m e (P OHS )
adverse event was significantly greater with bevacizum ab
Due to Histoplasma capsulatum, a dim orphic fungus (m old in
than with ranibizum ab: 39.9% vs 31.7%
soil, yeast in anim als and birds) endem ic to Mississippi and
Ohio River valleys; rare in Europe; rare am ong African
The a lte rna tive tre a tm e nts to Inhib it Am ericans. Age of onset com m only 20–45 years; no sex
VEGF in Ag e -re la te d c ho ro id a l predilection; 90% of patients with ocular signs have positive
skin reaction (> 5 m m ) to intracutaneous 1 :100 histoplasm in
Ne o va s c ula riza tio n (IVAN) Stud y (test usually not used because it m ay incite m acular disease)
Macular involvem ent associated with HLA-B7, HLA-DRw2;
Ob je c t ive : ranibizum ab versus bevacizum ab to treat however, HLA typing is not com m only used
neovascular age-related m acular degeneration. Patients
random ized into 4 groups: ranibizum ab or bevacizum ab, Prim ary infection involves inhalation of spores into respiratory
given either every m onth (continuous) or as needed tract and a self-lim ited flu-like illness; dissem ination of the
(discontinuous), with m onthly evaluations. Perform ed in fungus then occurs to spleen, liver, and choroid. Prim ary
the UK by National Health Service. Unlike CATT, patients choroidal infection causes granulom atous, clinically
received three m onthly injections at outset of trial unapparent inflam m ation that resolves into a sm all, atrophic
scar (“histo spot”) that can disrupt Bruch’s m em brane
Re s u lt s : 610 patients included. 1-year results reported
that com parison between bevacizum ab and ranibizum ab
Fin d in gs: POHS consists of the triad of peripapillary
was “inconclusive”. Discontinuous treatm ent was
atrophy, m ultiple punched-out chorioretinal scars (“histo
equivalent to continuous treatm ent. Fewer participants
spots,” m ay enlarge, 5–10% develop new spots), and
receiving bevacizum ab had an arteriothrom botic event or
m aculopathy. No anterior or posterior segm ent cell; CNV can
heart failure. There was no difference between drugs in
occur (different from that in AMD in that vessels penetrate
proportion experiencing a serious system ic adverse event.
Bruch’s m embrane and extend over RPE; a second layer of RPE
Serum VEGF levels were lower with bevacizum ab and
form s [basal side up] and attem pts to encircle the CNV)
higher with discontinuous treatm ent; bevacizum ab was less
(Fig. 11-51)
costly for both treatm ent regim ens. 2-year results showed no
difference in vision between the drugs. For BCVA,
bevacizum ab was neither non-inferior nor inferior to Risk o f CNV: 1% if no signs of POHS in fellow eye; 4% if
ranibizum ab. Discontinuous treatm ent was neither non- peipapillary atrophy; 25% if histo spots in m acula
inferior nor inferior to continuous treatm ent. Monthly
treatm ent resulted in slightly better levels of vision, detected CXR: calcifications
through testing of near visual acuity and contrast sensitivity.
Discontinuous group received on average 13 injections over An g io id s t re a ks
the 2-year period com pared to 23 for m onthly treatm ent Peripapillary linear cracks in thickened, degenerated, and
group. Frequency of arterial throm botic events or hospital calcified Bruch’s m em brane (Fig. 11-52)
adm ission for heart failure did not differ between drugs, but
m ortality was lower with continuous than discontinuous Subretinal hem orrhage can occur with m inor traum a; patients
treatm ent should consider safety glasses

Co n c lu s io n s : CATT, IVAN, MANTA LUCAS, and GEFAL

Etio lo gy: 50% associated with system ic condition, 50%
found that either m onthly or PRN treatm ent with
idiopathic
bevacizum ab or ranibizum ab was equally effective in
Mnem onic PEPSI:
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CHAPTER 11 • Posterior Segm ent

P a t h o lo g ic m yo p ia
High m yopia ¼ Axial length > 26 m m ; > À6 D of m yopia;
Pathologic m yopia ¼ Axial length > 32.5 m m ; > À8 D of
m yopia;
Approxim ately 2% of US population; fem ale > m ale
CNV due to PM com m only occurs in young patients; bilateral
com m on (12–40%)

Fin d in gs: long, oval disc, m ay be tilted, cup usually shallow;

tem poral crescent; posterior staphylom a; tigroid fundus with
visible choroidal vessels; lacquer cracks (breaks in Bruch’s
m em brane); Foerster-Fuchs’ spot (m acular hem orrhage);
cataract; retinal holes and high risk of rhegm atogenous RD
Lacquer crack: sudden decrease in vision, m etam orphopsia,
often in teenagers; focal subretinal hem orrhage, dense,
Fig ure 11-51. POHS demonstrating peripapillary scarring and macular,
juxtafoveal choroidal neovascular membrane with surrounding subretinal round, deep, and often centered on fovea; blood m ay
hemorrhage. (From Noorthy RS, Fountain J S: Fungal uveitis. In: Yanoff M, Duker obscure crack
J S (eds): Ophthalmology. London, 1999, Mosby.)

Co m p licatio n s: CNV often near fovea (65%) worse

prognosis; 60% with vision < 20/200 at 2 years

Tr eatm en t: Sam e as CNV due to AMD

Ot h e r c a u s e s o f m a c u la r CNV
Idiopathic, optic nerve head drusen, choroidal rupture,
choroidal nevus, sym pathetic ophthalm ia, Vogt-Kayanagi-
Harada disease, serpiginous choroiditis, other posterior
uveitides (choroidal inflam m ation m ay enhance production of
angiogenic factors; when coupled with RPE-Bruch’s m em brane
disruption, CNV can develop)

Va s c u la r Dis e a s e s
Fig ure 11-52. Peripapillary angioid streaks. (From Vander J F: Angioid streaks. Dam age to vessel walls causes leakage of serum and blood into
In: Yanoff M, Duker J S (eds ): Ophthalmology. London, 1999, Mosby.)
plexiform layers, causing edem a, exudates, and hem orrhages
Pseudoxanthoma elasticum (PXE) (AR > > AD):
fem ale > m ale; peau d’orange appearance to retina; Ed em a: histologically appears as clear cystoid spaces
redundant skin with waxy, yellow, papule-like lesions
(“plucked chicken” skin); increased elastic tissue; Lip id : appears as yellow lesion; histologically, hard exudates
vascular m alform ations; abnorm al m ucosal vasculature are eosinophilic and PAS-positive
m ay cause GI bleeds; m ay have optic nerve head drusen; DDx: diabetes, hypertensive retinopathy, CNV, vein
angioid streaks present in 85% occlusion, m acular telangiectasia, Coats disease,
Ehlers-Danlos syndrome (fibrodysplasia hyperelastica) (AD): radiation retinopathy, CSR, traum a, m acroaneurysm ,
hyperextensible skin due to deficient collagen m atrix; papilledem a, angiom atosis retinae
other eye findings include subluxed lens, high m yopia,
keratoconus, blue sclera, retinal detachm ent
Micr o an eu r ysm : fusiform outpouching of capillary wall
Paget’s disease: increased bone production and destruction;
increased serum alkaline phosphatase; prone to basal
skull and long bone fractures; other bone disorders Co tto n wo o l sp o t: m icroinfarction of NFL (usually
(acrom egaly, calcinosis) secondary to occlusion of retinal arteriole) with cessation of
Sickle cell: risk for autoinfarction of spleen and throm botic axoplasm ic flow, m itochondria accum ulate (resem ble a
episodes; other hem atologic diseases (thalassem ia, nucleus, so lesion appears like a cell [“cytoid body”])
hereditary spherocytosis, acanthocytosis [Bassen-
Kornzweig syndrom e]); angioid streaks present in 1% Hem o r r h age: shape of intraretinal blood depends on layer
Idiopathic: 50% in which it occurs (dot / blot in plexiform layer where cells are
oriented vertically; flam e-shaped / feathery border in NFL
DDx: lacquer cracks in m yopia where cells are oriented horizontally)

328
 Disorders

Ro th sp o t: white-centered hem orrhage

DDx: ischem ia (anem ia, anoxia, carbon m onoxide
poisoning), elevated venous pressure (birth traum a,
shaken baby syndrom e, intracranial hem orrhage),
capillary fragility (hypertension, diabetes), infection
(bacterial endocarditis, HIV), leukem ia, collagen
vascular disease

Neo vascu lar izatio n : growth of new vessels on vitreous

side of ILM; new vessels grow along posterior hyaloid

Vascu lar to r tu o sity: m ay be congenital (arterial and

venous) or acquired (venous) intra re tina l cys ts dis c e de ma
DDx: hypertension, high venous pressure (occlusion), Fig ure 11-53. Cystoid macular edema with decreased foveal reflex, cystic
papilledem a, high viscosity, AV fistula; associated with changes in fovea, and intraretinal hemorrhages. (From Kaiser PK, Friedman NJ ,
fetal alcohol syndrom e, Peter’s anom aly, optic nerve Pineda R II: The Massachusetts eye and ear infirmary illustrated manual of
hypoplasia ophthalmology, ed 2, Philadelphia, 2004, Saunders.)

Re t in a l va s c u lit is
Involvem ent of retinal arterioles (arteritis), veins (phlebitis), or
both (periphlebitis)

Fin d in gs: sheathing of vessels, hem orrhage

DDx: tem poral arteritis, polyarteritis nodosa, lupus, Behçet’s

disease, inflam m atory bowel syndrom e, m ultiple sclerosis,
pars planitis, Wegener’s granulom atosis, Eales disease,
sarcoidosis, syphilis, toxoplasm osis, viral retinitis (HSV, HZV),
IV drug abuse, Lym e disease, tuberculosis
pe ta lloid le a ka ge dis c le a ka ge
Cys t o id m a c u la r e d e m a (CME)
Fig ure 11-54. Fluorescein angiogram of same patient as in Fig. 11.53,
Intraretinal edem a in honeycom b-like spaces; flower-petal demonstrating characteristic petalloid appearance with optic nerve
pattern due to Henle’s layer leakage. (From Kaiser PK, Friedman NJ , Pineda R II: The Massachusetts eye and
ear infirmary illustrated manual of ophthalmology, ed 2, Philadelphia, 2004,
Saunders.)
Etio lo gy: m nem onic DEPRIVEN:
Diabetes
Epinephrine FA: m ultiple sm all focal fluorescein leaks early; late pooling
Pars planitis of dye in cystoid spaces; classically, flower-petal (“petalloid”)
Retinitis pigm entosa pattern; staining of optic nerve (Fig. 11-54)
Irvine-Gass syndrom e OCT: cystic intraretinal spaces (Fig. 11-55)
Venous occlusion
E2 prostaglandin
Nicotinic acid m aculopathy (does not leak)
DDx o f cystic m acu lar ch an ges ( lo o ks like CME
Others: uveitis, hypertensive retinopathy, retinal vasculitis,
clin ically, b u t n o flu o r escein fillin g o f cysts) :
epiretinal m em brane, radiation retinopathy, post laser or
1. Juvenile retinoschisis
cryo treatm ent, hypotony, occult rhegm atogenous retinal
2. Goldm ann-Favre syndrom e
detachm ent, intraocular tum ors, m acular telangiectasia,
3. Som e types of retinitis pigm entosa
CNV (rare), juvenile retinoschisis (does not leak),
4. Nicotinic acid m aculopathy
Goldm ann-Favre syndrom e (does not leak), latanoprost
(Xalatan), vitreous wick
Tr eatm en t: depends on etiology; focal laser treatm ent,
Path o p h ysio lo gy: abnorm al perifoveal retinal capillary topical steroids and NSAID, oral Diam ox, sub-Tenon’s or
perm eability; initial fluid accum ulation m ay be within Mu€ ller’s intravitreal steroid injection
cells (rather than in spaces of outer plexiform and inner nuclear
layers)
Co n g e n it a l re t in a l t e la n g ie c t a s ia / Co a t s
d is e a s e (Le b e r’s m ilia ry a n e u rys m s )
Fin d in gs: CME, optic nerve swelling, vitreous cell
(Fig. 11-53) (See Chapter 5, Pediatrics/Strabism us)

329
CHAPTER 11 • Posterior Segm ent

cys toid ma cula r e de ma RPE hyperplasia; occasionally, yellow lesion m easuring ⅓

DD centered on FAZ (pseudovitelliform macular
degeneration); may develop m acular edema that is due to
ischemia (not amenable to laser treatment); ⅓ have
abnormal glucose tolerance test
FA: parafoveal capillary leakage; risk of CNV
Type 3: bilateral, idiopathic; m ale ¼ fem ale; capillary
occlusion predom inates
TYPE 3A: occlusive idiopathic
TYPE 3B: occlusive idiopathic; associated with central
nervous system vasculopathy
Structural abnorm alities in types 2 and 3 are sim ilar to diabetic
m icroangiopathy (but no risk of NVE)

DDx: diabetes, vein occlusion, radiation retinopathy, Coats

disease, Eales’ disease, Best disease, sickle cell, Irvine-Gass
Fig ure 11-55. Optical coherence tomography of cystoid macular edema,
demonstrating intraretinal cystoid spaces and dome-shaped configuration
syndrome, ocular ischem ia
of fovea. (From Kaiser PK, Friedman NJ , Pineda R II: The Massachusetts eye and
ear infirmary illustrated manual of ophthalmology, ed 2, Philadelphia, 2004, Co m p licatio n s: m acular edem a, exudates, CNV,
Saunders.)
intraretinal neovascularization, retinal–retinal anastam osis
Ma c u la r t e la n g ie c t a s ia (Ma c Te l)
Re t in a l a rt e ria l m a c ro a n e u rys m (RAM)
Microaneurysm al and saccular dilation of parafoveal vessels
May bleed, then autoinfarcts
Form erly called parafoveal/juxtafoveal telangiectasia
Usually, elderly fem ales; m ost com m on along tem poral
arcades; 10% bilateral
Classificatio n :
Type 1: unilateral, m ale > fem ale (10 :1); onset during
Mech an ism : arteriosclerosis (fibrosis, thinning, decreased
m iddle age; in spectrum of Coats disease
elasticity of vessel wall), hypertension (increased pressure on
TYPE 1A: congenital; confined to tem poral half of fovea;
thin wall)
m acular edem a and exudation (lipid)
TYPE 1B: idiopathic; capillary telangiectasia confined to
1 clock hour at edge of FAZ; m inim al leakage on FA; Fin d in gs: blood in every retinal layer, lipid exudate, artery
occasional hard exudates; vision usually better than occlusion downstream (especially following laser treatm ent),
20/25; can be treated with laser CME (Fig. 11-56)
Type 2A (m ost com m on): bilateral, acquired;male ¼ fem ale;
5th–6th decade of life; symmetric, involving area < 1 DD; DDx o f b lo o d in ever y r etin al layer ( su b r etin al,
minim al macular edema; occasionally, superficial in tr ar etin al, an d p r er etin al) : m acroaneurysm , traum a,
glistening white dots (Singerman’s spots); right-angle sickle cell retinopathy, choroidal m elanom a, vein occlusion
retinal venules dive deep into choroid; eventually develop (rare), CNV (rare)

A B
Fig ure 11-56. Macroaneurysm with s urrounding dilated and telangiectatic capillary bed. A, fundus photograph; B, FA. (Courtesy Susan Fowell, MD. From Mittra RA,
Mieler WF, Pollack J S: Retinal arterial macroaneurysms . In: Yanoff M, Duker J S (eds ): Ophthalmology. London, 1999, Mosby.)

330
 Disorders

Path o lo gy: overall thickening of vessel wall with

hypertrophy of m uscularis

Tr eatm en t: observation; consider focal laser (risk of

hem orrhage)

Hyp e rt e n s ive re t in o p a t h y
Focal or generalized vasoconstriction, breakdown of blood–
retinal barrier with subsequent hem orrhage and exudate
Associated with m icroaneurysm s or m acroaneurysm s

Classificatio n system s:
Keith-Wagener-Barker Classification
GROUP I: m inim al constriction and tortuosity of
arterioles
GROUP II: Moderate constriction of arterioles; focal Fig ure 11-57. Nonproliferative diabetic retinopathy.
narrowing and arteriovenous nicking
GROUP III: Group II plus cotton wool spots,
hem orrhages, and exudates
GROUP IV: Group III plus optic disc edem a
Scheie Classification
GROUP 0: no visible changes
GROUP I: Diffuse arteriolar narrowing
GROUP II: Pronounced arteriolar narrowing and focal
constriction
GROUP III: Grade II plus retinal hem orrhages
GROUP IV: Grade III plus optic disc edem a

Fin d in gs:
Retinopathy: AV nicking, “copper or silver wire” arterial
changes, hem orrhages, exudates, cotton wool spots lipid exuda te intra re tina l he morrha ge s
Choroidopathy: fibrinoid necrosis of choroidal arterioles; Fig ure 11-58. Severe nonproliferative diabetic retinopathy with extensive
m ay have Elschnig’s spots (zone of nonperfusion of hemorrhages, microaneurysms, and exudates. (From Kaiser PK, Friedman NJ ,
choriocapillaris; pale white or red patches of RPE), Pineda R II: The Massachusetts eye and ear infirmary illustrated manual of
Siegrist streak (reactive RPE hyperplasia along sclerosed ophthalmology, ed 2, Philadelphia, 2004, Saunders.)
choroidal vessel), and exudative RD; due to acute
hypertensive episode (pre-eclam psia, eclam psia, or Severe NPDR (“4-2-1 rule”) (15% progress to PDR in 1 year):
pheochrom ocytom a); FA shows early hypoperfusion Defined as any one of the following:
and late staining 4 quadrants of hem orrhages / MAs
Optic neuropathy: florid disc edem a with m acular exudate, 2 quadrants of venous beading
linear flam e hem orrhages 1 quadrant of IRMA (Fig. 11-58)
Path o lo gy: thickening of arteriolar walls leads to nicking of Very severe NPDR (50% progress to PDR in 1 year):
venules; endothelial hyperplasia Defined as 2 or m ore of the above
Proliferative (PDR): NV of disc or elsewhere (Fig. 11-59)
Co m p licatio n s: retinal vein occlusion, retinal High-risk proliferative (HR-PDR):
m acroaneurysm , nonarteritic AION, ocular m otor nerve Defined as any one of the following:
palsies, worsening of diabetic retinopathy 1. NVD ! ¼ to ⅓ disc area
2. Any NVD with vitreous hem orrhage
Dia b e t ic re t in o p a t h y (DR) 3. NVE! ½ disc area with vitreous hem orrhage

Leading cause of new blindness in United States, adults aged Ep id em io lo gy:

20–74 years Type 1 DM:
AT DIAGNOSIS: no NPDR
Classificatio n : AT 5 YEARS: 25% NPDR, PDR rare
Background or nonproliferative (BDR, NPDR): AT 20 YEARS: 98% NPDR, 60% PDR, 30% CSME
hem orrhages, exudates, cotton wool spots, Type 2 IDDM:
m icroaneurysm s (MA), intraretinal AT DIAGNOSIS: 30% NPDR
m icrovascular abnorm alities (IRMA), venous beading AT 5 YEARS: 40% NPDR, 2% PDR
(Fig. 11-57) AT 20 YEARS: 90% NPDR, 25% PDR, 40% CSME

331
CHAPTER 11 • Posterior Segm ent

ne ova s cula riza tion ne ova s cula riza tion

of the dis c e ls e whe re

Fig ure 11-59. Proliferative diabetic retinopathy demonstrating florid

neovascularization of the disc and elsewhere. (From Kaiser PK, Friedman NJ , Fig ure 11-61. Lacy vacuolization of iris pigment epithelium. (Modified from
Pineda R II: The Massachusetts eye and ear infirmary illustrated manual of Yanoff M et al: Diabetic lacy vacuolization of iris pigment epithelium. Am J
ophthalmology, ed 2, Philadelphia, 2004, Saunders.) Ophthalmol 69:201–210, 1970. From Yanoff M, Fine BS: Ocular pathology, ed 5,
St Louis, 2004, Mosby.)

Main cau se o f visio n lo ss in NPDR: m acular edem a or

ischem ia

Main cau ses o f visio n lo ss in PDR: tractional RD

(TRD), neovascular glaucom a (NVG), vitreous
hem orrhage (VH)

Oth er sequ elae:

Diabetic cataract: aldose reductase pathway
converts glucose into sorbitol and fructose; causes
ne ova s cula riza tion ca pilla ry nonpe rfus ion
osm otic effect; aldose reductase also converts
Fig ure 11-60. Fluorescein angiogram of a patient with proliferative diabetic galactose into galactitol (which causes cataracts in
retinopathy, showing extensive capillary nonperfusion, neovascularization galactosem ia)
elsewhere, and vascular leakage. (From Kaiser PK, Friedman NJ , Pineda R II: The Diabetic iridopathy: iris NV; lacy vacuolization of iris
Massachusetts eye and ear infirmary illustrated manual of ophthalmology,ed 2,
pigm ent epithelium in 40%; glycogen-filled cysts in iris
Philadelphia, 2004, Saunders.)
pigm ent epithelium (PAS+)
Papillitis: acute disc swelling; vision usually ! 20/50;
Type 2 NIDDM: 50% bilateral; m ay have VF defect; m ost recover
AT DIAGNOSIS: 20% NPDR
to ! 20/30
AT 5 YEARS: 30% NPDR, 2% PDR
Isolated cranial nerve palsies: CN 3, 4, 6 (including pupil-
AT 20 YEARS: 50% NPDR, 10% PDR, 20% CSME
sparing CN 3 palsy)
Pupillary abnormalities: light-near dissociation
Fin d in gs: cotton wool spots, lipid exudates (m ay appear as Fluctuation in refractive error: due to osm otic effect on
circinate exudate [ring of hard exudate surrounding leaky crystalline lens from unstable blood sugar levels
focus] or m acular star [pattern reflects radial orientation of
Henle’s fibers]), hem orrhages (blot [outer plexiform layer], Path o lo gy: selective loss of pericytes, no endothelial cells or
flam e [tracks along NFL]), m icroaneurysm s, IRMA (intraretinal pericytes in nonperfused areas; thickening of retinal capillary
m icrovascular abnorm alities; shunts [arteriole to venule]), basem ent membranes; m icroaneurysm formation; retinal
venous beading and loops, neovascularization (disc [NVD], capillary closure; breakdown of blood–retinal barrier; lacy
elsewhere in retina [NVE], iris [NVI]) (Fig. 11-60) vacuolization of iris pigment epithelium; intraepithelial
vacuoles contain glycogen; gitter cells (lipid-laden macrophages)
(Figs. 11-61, 11-62)
Clin ically sign ifican t m acu lar ed em a ( CSME)
d efin itio n : one of the following: DDx: ocular ischem ic syndrom e, radiation retinopathy,
1. Thickening within 500 µm of the m acular center hypertensive retinopathy, retinal vein occlusion,
2. Hard exudate within 500 µm of the m acular center proliferative retinopathies (sarcoidosis, sickle cell), m acular
with associated thickening of adjacent retina telangiectasia
3. Zone of retinal thickening 1 disc area in size, any part
of which is within 1 disc diameter of the m acular center FA: to identify m acular ischem ia, localize m icroaneurysm s to
Asym m etric diabetic retinopathy is usually due to carotid guide focal laser treatm ent, identify areas of NVE, identify areas
disease (on either side) of capillary nonperfusion

332
 Disorders

tear and rhegm atogenous or traction RD), central

scotom a (worsening of CSME), progression of
cataract
Up to 33% of patients will not respond to PRP
Anti-VEGF agents: for center-involving m acular edem a
(no CSME definition in the anti-VEGF trials)
bevacizum ab, ranibizum ab or aflibercept injections
with im m ediate or deferred laser were significantly
better than laser therapy alone (DRCR.net, RISE, RIDE,
VIVID, VISTA, RESOLVE, and RESTORE studies,
see below)
Steroids: triamcinolone acetonide intravitreal injections
found to be effective in pseudophakic patients, but not
in overall group (see DRCR.net Protocol Bbelow). Iluvien is
a sustained drug delivery system that uses a drug matrix in a
Fig ure 11-62. Microaneurysms, pericyte dropout, and acellular capillaries tiny cylindrical tube that is injected intravitreally via a 25 g
are seen. (From Benson WE: Diabetic retinopathy. In: Yanoff M, Duker J S (eds):
Ophthalmology. London, 1999, Mosby.)
needle, releasing 0.2 µg/day of the corticosteroid fluoci-
nolone acetonide over 2–3 years (found to be effective in
the Phase 3 FAME studies); dexamethasone polymer
OCT: m acular edem a appears as increased retinal implant (Ozurdex) releases dexamethasone from PLGA
thickness, cysts, and subretinal fluid; can identify vitreous polymer that is completely biodegradable over several
traction months
Diabetic Retinopathy Vitrectomy Study (DRVS):
B-scan u ltr aso u n d : to identify TRD if VH present
Early vitrectomy for VH in type 1 diabetics only,
can defer in type 2 (however indications for vitrectomy
Tr eatm en t: have changed from these DRVS study conclusions
Based on results from several im portant studies: largely owing to better vitrectomy systems,
Diabetes Control and Complications Trial (DCCT): im proved instrumentation, and the use of endolaser)
Tight control of blood sugar slows progression of INDICATIONS FOR VITRECTOMY:
retinopathy, diabetic m acular edem a, and visual Nonclearing vitreous hem orrhage
loss in type 1 diabetic patients; patients with Com bined tractional-rhegm atogenous RD
HbA1c < 8% had a significantly reduced risk of Traction retinal detachment of the fovea. If tractional
retinopathy detachment is extramacular, watch closely; if macula
Rapid norm alization and tight control of blood sugar is threatened or detaches, perform vitrectomy
after a period of prolonged hyperglycem ia can lead to Anterior hyaloidal fibrovascular proliferation
worsening of retinopathy Refractory macular edema: patients with taut posterior
United Kingdom Prospective Diabetes Study (UKPDS): hyaloid face can have chronic m acular edema that
Tight control of blood sugar and blood pressure does not resolve with laser. Chronic traction of
slows progression of retinopathy and vitreous face on m acula appears to produce
developm ent of m acular edem a in type 2 diabetic persistent leakage; can resolve after traction is
patients relieved with vitrectomy
Early Treatment Diabetic Retinopathy Study (ETDRS): Progressive fibrovascular proliferation despite
Focal laser decreases vision loss from m acular edem a com plete PRP
by 50% Ghost cell glaucom a
No benefit from aspirin Consider for refractory diabetic m acular edem a (also
Focal laser: for CSME (visual acuity is not part of consider injecting steroids, or intravitreal anti-
treatm ent criteria); argon green preferred; yellow VEGF agents)
(577 nm ) is also used because it is well absorbed by
hem oglobin within m icroaneurysm s; re-exam ine Pr o gn o sis: risk of progression without treatm ent from
every 3–4 m onths preproliferative to proliferative DR over 2 years is 50%
Diabetic Retinopathy Study (DRS):
PRP reduces incidence of severe vision loss in high-risk Severe NPDR has 50% risk of progression to proliferative
PDR by 60% disease in 12–18 m onths
CRITERIA: HR-PDR
Conditions that exacerbate diabetic retinopathy:
1. NVD ! ¼ to ⅓ disc area
hypertension, puberty, pregnancy (at conception, if no BDR,
2. Any NVD with vitreous hem orrhage
88% have no retinopathy; if m ild BDR, 47% worsen, 5%
3. NVE! ½ disc area with vitreous hem orrhage
develop PDR; if PDR, 46% have progression), renal disease,
COMPLICATIONS: decreased night vision (destruction of
anem ia
extram acular rods), angle-closure glaucom a
(choroidal effusion), retinal detachm ent (regression Follow HbA1c (serum glycosylated hem oglobin [provides
of NV fronds can cause contracture, leading to retinal 3-m onth view of blood sugar levels])

333
CHAPTER 11 • Posterior Segm ent

MAJ OR DIABETIC RETINOP ATHY Me t h o d s : patients had m ild, m oderate, or severe NPDR
CLINICAL STUDIES or early PDR (does not m eet high-risk criteria of PDR) in
both eyes, and 20/200 or better vision in each eye, and were
random ly assigned to receive 650 m g daily aspirin or not
Dia b e tic Re tino p a thy Stud y (DRS) AND 1 of the following:
1. Moderate to severe NPDR or early PDR with no
Ob je c t ive : to evaluate whether photocoagulation m acular edem a ¼ im m ediate PRP (further random ly
prevents severe visual loss in eyes with diabetic retinopathy assigned to full [1200–1600, 500 µm spots]
scatter or m ild [400–650, 500 µm spots] scatter PRP)
Me t h o d s : patients had PDR in at least 1 eye or severe vs deferred PRP until high-risk characteristics
NPDR in both eyes, or 20/100 or better vision in each eye, developed
and were random ly assigned to treatm ent with scatter laser 2. Mild or m oderate NPDR and m acular
photocoagulation (random ly assigned to either xenon arc edem a ¼ im m ediate laser (further random ly
[200–400, 4.5° spots] or argon blue-green laser [800–1600, assigned to im m ediate focal laser photocoagulation
500 µm spots]) in 1 eye and no treatm ent in fellow eye and deferred full scatter or m ild scatter when
(protocol later am ended to allow deferred laser high-risk characteristics developed; or im m ediate
photocoagulation). Surface neovascularization (NVE) m ild or full scatter PRP and deferred focal laser
treated directly with confluent burns, and NVD treated photocoagulation) vs deferred laser
directly only in argon laser–treated eyes. Patients were photocoagulation
excluded if they had previous panretinal photocoagulation 3. Severe NPDR or early PDR and m acular
or traction retinal detachm ent threatening m acula edem a ¼ im m ediate laser (further random ly assigned
to im m ediate m ild or full PRP and im m ediate or
S e ve re NP DR d e fin e d a s h a vin g a t le a s t 3
deferred focal laser photocoagulation) vs deferred laser
o f t h e fo llo w in g : photocoagulation
Cotton wool spots Patients were excluded if they had high-risk proliferative
Venous beading diabetic retinopathy
Intraretinal m icrovascular abnorm alities (IRMA) in ! 2 of Mild NPDR defined as:
4 contiguous overlapping photographic fields At least 1 m icroaneurysm , but not enough to qualify as
Moderate to severe retinal hem orrhages and / or m oderate NPDR
m icroaneurysm s in ! 1 standard photographic field Moderate NPDR defined as:
Extensive intraretinal hem orrhages and / or
Re s u lt s : 1727 patients enrolled m icroaneurysm s, cotton wool spots, IRMA,
PRP reduced the risk of severe vision loss (VA < 5/200 on venous beading, but not enough to qualify as
2 consecutive visits 4 m onths apart) by 50% to 60% in severe NPDR
patients with high-risk characteristics (see later) Severe NPDR defined with “4-2-1 rule” as any one
of the following:
Co n c lu s io n s : perform PRP for patients with high-risk Intraretinal hem orrhages and / or m icroaneurysm s in all
proliferative retinopathy (HR-PDR) regardless of vision
4 quadrants
NVD (new vessels on or within 1 disc diam eter of the Venous beading in at least 2 quadrants
disc) ! ¼À⅓ of disc area (standard photo 10A) IRMA in at least 1 quadrant
CSME defined as one of the following:
Any NVD with vitreous or preretinal hem orrhage
1. Retinal thickening at or within 500 µm of the center
NVE (new vessels elsewhere) ! ½ of disc area (standard of the m acula
photo 7) with vitreous or preretinal hem orrhage 2. Hard exudates at or within 500 µm of the center of
the m acula with associated thickening of the
PRP is also indicated for NVI
adjacent retina
DRS did not report a clear benefit for im m ediate PRP in 3. Retinal thickening ! 1 disc area in size 1 disc
patients with severe nonproliferative diabetic retinopathy diameter from the center of the m acula
and proliferative diabetic retinopathy without high-risk
characteristics. However, older-onset diabetic patients Re s u lt s : 3711 patients enrolled
should be considered for earlier PRP
Im m ediate focal laser decreased m oderate vision loss in
Ea rly Tre a tm e nt Dia b e tic Re tino p a thy patients with clinically significant m acular edem a
by $ 50%
Stud y (ETDRS) Early PRP reduced the risk of developm ent of
high-risk PDR in patients with NPDR and early PDR,
Ob je c t ive : to evaluate but difference in severe visual loss was m inim al
1. Whether photocoagulation is effective for diabetic Im m ediate focal laser and deferred scatter PRP
m acular edem a reduced m oderate visual loss by 50% in
2. Effect of aspirin on the course of diabetic retinopathy patients with m ild or m oderate NPDR and
3. When to initiate PRP treatm ent for diabetic retinopathy m acular edem a

334
 Disorders

Im m ediate focal laser and scatter PRP reduced severe Type 1 diabetic patients had an increased chance of
visual loss by 50% in patients with severe NPDR or obtaining good vision with early vitrectom y
early PDR and m acular edem a Previous PRP increased chances of good vision
Aspirin had no effect on progression or com plications of With increasing severity of vessels, early
diabetic retinopathy intervention was better than deferred intervention
Group H:
Co n c lu s io n s : treat all patients with CSME regardless of 2-YEAR RESULTS: patients in early vitrectom y group
vision had better vision than those in deferral group
Patients with type 1 DM had superior outcom es from
Im m ediate PRP should be reserved for patients with
early vitrectom y, no advantage seen in type 2 DM
high-risk PDR and possibly those with severe NPDR in
both eyes Co n c lu s io n s : early vitrectomy for eyes with severe visual
loss due to nonclearing vitreous hemorrhage (at least
No benefit from aspirin (650 m g/day) 1 m onth) is helpful in type 1 diabetic patients and monocular
patients despite the type of diabetes. Early vitrectomy is also
Dia b e tic Re tino p a thy Vitre c to m y recomm ended for eyes with useful vision and advanced active
PDR, especially when extensive neovascularization is present.
Stud y (DRVS) NLP was seen in 20% of eyes regardless of intervention once
severe vitreous hemorrhage had occurred. Eyes with traction
Ob je c t ive : to observe patients with severe DR in type 1 and retinal detachment not involving fovea can be observed until
type 2 diabetes over 2 years to determine visual outcomes
fovea becomes detached, provided that fibrovascular
Me t h o d s : patients with severe NPDR or early PDR (do proliferation is not severe
not m eet high-risk criteria of PDR) were placed into 1 of 3
groups
Dia b e te s Co ntro l a nd Co m p lic a tio ns
Tria l (DCCT)
Group N: natural history study with 744 eyes of 644
patients enrolled Ob je c t ive : to evaluate effect of tight vs conventional
Group NH: to evaluate early surgical intervention vs control of blood sugar on diabetic com plications in type 1
delayed surgery (for at least 1 year) in eyes with severe diabetic patients
diabetic retinopathy and active neovascularization or
fibrovascular proliferation but without severe visual Me t h o d s : patients with insulin dependence defined by C-
loss (vision better than 10/200). 370 eyes enrolled. peptide secretion were random ly assigned to either
Am ount of new vessel severity was quantified
NVC-1 (least severe): new vessel severity no worse Intensive therapy: 3 or m ore daily insulin injections or
than m oderate in only 1 photographic field im plantation of an insulin pum p
NVC-2 (m oderately severe): m oderate new vessels in Self-m onitoring of glucose levels at least 4 tim es a day
2 or m ore fields, but not severe in any field Adjust insulin dose based on glucose level
NVC-3 (severe): severe NVD or NVE in at least 1 field Preprandial glucose ¼ 70–120 m g/dL (3.9 and
NVC-4 (very severe): severe NVD and NVE in at least 6.7 m mol/L)
1 field Postprandial glucose < 180 m g/dL (10.0 m m ol/L)
Group H: to evaluate early surgical intervention (within Weekly 3 AM glucose > 65 m g/dL (3.6 m m ol/L)
days) vs delayed surgery (for at least 1 year, unless Monthly HbA1c < 6.05% (nondiabetic range)
TRD of m acula detected on ultrasound exam ination) Conventional therapy: 1 or 2 daily insulin injections
in eyes with severe diabetic retinopathy and active Daily self-m onitoring of glucose levels
neovascularization or fibrovascular proliferation, Did not adjust insulin dose daily
with history of sudden visual loss from severe vitreous Education about diet and exercise
hem orrhage within 6 m onths (vision worse than 5/ Followed every 3 m onths
200, not NLP). 616 eyes enrolled. (Note: No glycosuria or ketonuria
endophotocoagulation was not used in DRVS) Absence of hyperglycem ic or hypoglycem ic sym ptom s
Patients were excluded if they had previous vitrectom y,
Re s u lt s : 1441 patients included, study stopped after
photocoagulation within 3 m onths, IOP > 29 m m Hg
average follow-up of 6.5 years by independent safety and
on m edications, severe iris neovasularizataion, or
m onitoring com m ittee
neovascular glaucom a
Average difference in HbA1c was > 2% between groups;
however, < 5% of intensive group kept < 6.05%
Re s u lt s : Incidence of DR was the sam e up to 36 m onths
Group NR: At baseline, 726 patients had no retinopathy, and 715
4-YEAR RESULTS: patients in early vitrectom y group had m ild retinopathy. After 5 years, incidence of
had better vision than those in deferral group retinopathy was approxim ately 50% less with
Up to 18 m onths, early vitrectom y group had a intensive therapy
higher risk of no light perception vision; this was Patients with HbA1c < 8% had significantly reduced risk
not seen at later tim e points of retinopathy

335
CHAPTER 11 • Posterior Segm ent

Intensive control: The reduction in HbA1c was associated with a 25% overall
Reduced the risk of developm ent of DR by 76% reduction in m icrovascular com plications, including
Slowed the progression of DR by 54% and reduced the retinopathy (21% reduction) and nephropathy (34%
developm ent of severe NPDR or PDR by 47% reduction). 37% of patients had m icroaneurysm s in 1 eye at
Reduced the risk of m acular edem a by 23% diagnosis and random assignm ent
Reduced the risk of laser treatm ent by 56%
Reduced album inuria by 54% Co n c lu s io n s : type 2 diabetic patients benefit from
Reduced clinical neuropathy by 60% intensive glycem ic control, as do type 1 diabetic patients
Adverse effect: 2–3 Â increase in severe hypoglycem ia
The Unite d King d o m P ro s p e c tive
Co n c lu s io n s : tight control is beneficial; however, rapid Dia b e te s Stud y – Hyp e rte ns io n in
norm alization and tight control of blood sugar after a Dia b e te s Stud y (UKP DS-HDS)
period of prolonged hyperglycem ia can lead to an initial
worsening of retinopathy Ob je c t ive : to com pare effects of intensive blood pressure
control on the risk of m icrovascular and m acrovascular
Ep id e m io lo g y o f Dia b e te s Inte rve ntio ns com plications
a nd Co m p lic a tio ns (EDIC) Tria l Me t h o d s : 1148 patients with type 2 diabetes and m ild to
moderate hypertension to determine if “tight blood pressure
Ob je c t ive : to gain further follow-up of patients control (150/85 m mHg)” using an angiotensin-converting
from DCCT enzyme (ACE) inhibitor or a β-blocker vs “less tight control
(< 180/105 m mHg)” would prevent diabetic complications.
Me t h o d s : 1208 patients from DCCT, all of whom
Patients were random ly assigned to captopril (ACE
received intensive therapy and were followed for an
inhibitor) or atenolol (β-blocker) and followed for a
additional 4 years
m edian of 8.4 years.
Re s u lt s : intensive therapy reduced the risk of Re s u lt s : tight control of blood pressure in patients with
Progression of retinopathy by 75% hypertension and type 2 diabetes reduced risk of death
Any m acular edem a by 58% related to diabetes by 32%.
Laser treatm ent by 52%
In addition, there was a 34% reduction in risk of deterioration
of retinopathy by 2 or m ore ETDRS steps from baseline, and a
Co n c lu s io n s : tight control is still beneficial
47% reduction in risk of deterioration of visual acuity by 3
ETDRS lines with tight blood pressure control.
Unite d King d o m P ro s p e c tive Dia b e te s
Lowering of blood pressure with captopril or atenolol was
Stud y (UKP DS)
sim ilarly effective in reducing incidence of diabetic
com plications, suggesting that blood pressure reduction in
Ob je c t ive : to com pare effects on risk of m icrovascular itself m ay be m ore im portant than the treatm ent used
and m acrovascular com plications of intensive blood
glucose control with oral hypoglycem ics and / or insulin and Co n c lu s io n s : tight blood pressure control reduced the
conventional treatm ent with diet therapy risk of com plications from diabetic retinopathy

Me t h o d s : 4209 newly diagnosed patients with type 2 A Stud y o f Ra nib izum a b Inje c tio n in
diabetes, median age 54 years (range, 25–65 years), were Sub je c ts With CSME With Ce nte r
randomly assigned to intensive therapy with a sulfonylurea
(chlorpropamide, glibenclamide (glyburide), or glipizide) or
Invo lve m e nt Se c o nd a ry to Dia b e te s
with insulin, or conventional therapy using diet control (RIDE)

Re s u lt s : after m edian duration of therapy of 11 years, Ob je c t ive : to com pare efficacy of ranibizum ab versus
intensive treatm ent with sulfonylurea, insulin, and / or sham with laser rescue in patients with diabetic
m etform in was equally effective in reducing fasting plasm a m acular edem a
glucose concentrations.
Me t h o d s : multicenter, random ized, double-masked,
Over 10 years, HbA1c was 7.0% (range 6.2–8.2) in intensive sham injection-controlled, 36-month (sham
group com pared with 7.9% (range, 6.9–8.8) in injection-controlled for 24 m onths) Phase III study designed
conventional group – an 11% reduction. No difference in to assess the efficacy and safety profile of ranibizumab in 382
HbA1c was seen am ong agents in intensive group. patients with diabetic m acular edema (DME). Patients
Com pared with conventional group, risk in intensive group randomized to receive m onthly injections of either 0.3 mg
was 12% lower for any diabetes-related end point; 10% ranibizumab (n ¼ 125), 0.5 mg ranibizumab (n ¼ 127) or
lower for any diabetes-related death; and 6% lower for all- monthly sham injections (n ¼ 130). Beginning at three
cause m ortality. months, m acular laser rescue treatment was m ade available

336
 Disorders

to all patients, if needed, based on pre-specified criteria. After followed by PRN ranibizumab alone; focal laser and
month 24, patients in the sham injection group were eligible ranibizumab 0.5 mg with 3 monthly loading dose followed
to receive m onthly injections of 0.5 mg ranibizumab, and all by PRN ranibizum ab; or focal laser alone. Prim ary outcome
patients will continue to be followed and dosed m onthly for was m ean change in vision from baseline at month 12.
a total of 36 months. The study then continues in an open-
label extension phase. Re s u lt s : at m onth 12, ranibizum ab alone group
(n ¼ 115) gained +6.1 letters, ranibizum ab plus laser
Re s u lt s : at 24 m onths, 33.6% of patients who received (n ¼ 118) gained +5.9 letters, and the laser alone (n ¼ 110)
0.3 m g ranibizum ab and 45.7% of patients who received gained +0.8 letters. There was a m ean of 2.1 laser treatm ents
0.5 m g ranibizum ab were able to read ! 15 letters com pared in the laser alone group. The groups gained ! 15 letters in
to baseline, com pared to 12.3% of patients who received 22.6%, 22.9% and 8.2% respectively. Central retinal
sham injections with laser rescue. thickness im proved in all groups: the ranibizum ab alone
group decreased À118.7 µm , ranibizum ab plus laser
Co n c lu s io n s : ranibizum ab injections are effective in decreased À128.3 µm , and the laser group decreased by
diabetic m acular edem a À61.3 µm . A com panion study RESOLVE reported that
ranibizum ab-treated patients achieved an average +11.7
A Stud y o f Ra nib izum a b Inje c tio n in letters gain in visual acuity at 12 m onths com pared with
sham -treated patients, som e of who received laser
Sub je c ts With CSME With Ce nte r treatm ent.
Invo lve m e nt Se c o nd a ry to Dia b e te s
(RISE) Co n c lu s io n s : ranibizum ab therapy with or without laser
resulted in significant visual gain over laser treatm ent alone.
Ob je c t ive : to com pare efficacy of ranibizum ab versus
sham with laser rescue in patients with diabetic
Dia b e tic Re tino p a thy Clinic a l Re s e a rc h
m acular edem a
Ne two rk (DRCR.ne t) Stud ie s : Ma jo r
Me t h o d s : m ulticenter, random ized, double-m asked, P ro to c o ls Only
sham injection-controlled, 36-m onth phase III study
designed to assess the safety and efficacy profile of
P ro t o c o l B
ranibizum ab in 377 patients with DME. Prim ary endpoint
com pared the proportion of ranibizum ab and sham -treated
Ob je c t ive s : to com pare intravitreal triam cinolone
patients who gained ! 15 letters in best corrected visual
acetonide (TA) injections at doses of 1 m g or 4 m g and
acuity (BCVA) at m onth 24, relative to baseline. Patients
m acular laser photocoagulation in treatm ent of diabetic
were random ized to receive m onthly injections of either
m acular edem a
0.3 m g ranibizum ab (n ¼ 125), 0.5 m g ranibizum ab (n
¼125), or m onthly sham injections (n ¼ 127). At 3 m onths, Me t h o d s : patients age ! 18 years. Study eye with center-
rescue laser treatm ent was m ade available to all patients, if involved DME present on clinical exam and on OCT based
needed based on pre-specified criteria. After m onth 24, on m ean retinal thickness on two OCT m easurem ents ! 250
patients in the control group are eligible to receive m onthly m icrons in central subfield, and best corrected E-ETDRS
injections of 0.5 m g ranibizum ab and all patients will acuity ! 24 letters (20/320 or better) and 73 letters (worse
continue to be followed for 36 m onths. than 20/40). Patients random ized to either 1 or 4 m g TA or
focal laser photocoagulation. Prim ary outcom e was ! 15-
Re s u lt s : patients receiving m onthly ranibizum ab
letter im provem ent in visual acuity from baseline at 2 years
achieved an im provem ent in vision (BCVA) of ! 15 letters at
24 m onths, com pared to those in control group, who Re s u lt s : 840 eyes (693 subjects) enrolled. Primary outcome
received placebo (sham ) injection. was mean improvement in vision at 24 months:+1 letters in the
laser group (n ¼ 330); À2 letters in the 1 mg triamcinolone
Co n c lu s io n s : ranibizum ab injections are effective in
group (N ¼ 256); -3 letters in the 4 mg triamcinolone group
diabetic m acular edem a
(n ¼ 254). Increased IOP > 30 mmHg was seen in 4% of laser
treated group, 9% of1 mgTAgroup and 21% in 4 mgTAgroup.
Sa fe ty a nd Effic a c y o f Ra nib izum a b in Cataract surgery was required in 13% of laser treated group,
Dia b e tic Ma c ula r Ed e m a (RESTORE/ 23% of 1 mg TA patients and 51% in 4 mg TA group.
RESOLVE) Stud ie s
Co n c lu s io n s : triam cinolone acetonide injections were
not superior to focal / grid photocoagulation, and resulted
Ob je c t ive : to com pare efficacy of ranibizum ab with or
in m ore adverse events
without laser versus laser alone in patients with diabetic
m acular edem a
P ro t o c o l I
Me t h o d s : randomized, double-masked, multicenter, laser-
controlled phase III trial (n ¼ 345) with patients randomized Ob je c t ive s : to evaluate safety and efficacy of (1)
to either ranibizum ab 0.5 m g with 3 monthly loading dose intravitreal ranibizum ab in com bination with focal laser

337
CHAPTER 11 • Posterior Segm ent

photocoagulation, (2) intravitreal ranibizum ab treatm ent Me t h o d s : eyes were random ly assigned to receive
alone, and (3) intravitreal triam cinolone acetonide in PRP treatm ent, com pleted in 1 to 3 visits (n = 203 eyes),
com bination with focal laser photocoagulation in eyes with or ranibizum ab, 0.5 m g, by intravitreous injection at
center-involved DME baseline and as frequently as every 4 weeks based on a
structured re-treatm ent protocol (n = 191 eyes). Eyes
Me t h o d s : patients age ! 18 years. Study eye with center- in both treatment groups could receive ranibizum ab
involved DME present on clinical exam and on OCT based for DME
on m ean retinal thickness on two OCT m easurem ents
! 250 µm in central subfield, and best corrected acuity Re s u lt s : m ean visual acuity letter im provem ent at 2 years
20/32 or worse. Patients were random ized to one of was +2.8 in the ranibizum ab group vs +0.2 in the PRP
4 groups: Group A: sham injection plus focal (m acular) group. Mean peripheral visual field sensitivity loss was
photocoagulation; Group B: 0.5 m g injection of intravitreal worse (À23 dB vs À422 dB), vitrectom y was m ore frequent
ranibizum ab plus focal photocoagulation; Group C: 0.5 m g (15% vs 4%), and DME developm ent was m ore frequent
injection of intravitreal ranibizum ab plus deferred focal (28% vs 9%) in PRP group vs ranibizum ab group,
photocoagulation; Group D: 4 m g intravitreal respectively. Eyes without active or regressed
triam cinolone plus focal photocoagulation. Prim ary neovascularization at 2 years were not significantly different
outcom e was ! 15-letter im provem ent in visual acuity from (35% in the ranibizum ab group vs 30% in the PRP group).
baseline at 1 year One eye in ranibizum ab group developed endophthalm itis.
No significant differences between groups in rates of m ajor
cardiovascular events
Re s u lt s : 854 patients random ized. Prim ary outcom e was
m ean im provem ent in vision at 12 m onths: +3 letters in the Co n c lu s io n s : am ong eyes with PDR, treatm ent with
laser group (n ¼ 293); +9 letters in the ranibizum ab with ranibizum ab resulted in visual acuity that was non-inferior
prom pt laser group (n ¼ 187); +9 letters in the ranibizumab to (not worse than) PRP treatm ent at 2 years
with deferred laser group (n ¼188); and +4 letters in the
triamcinolone plus laser group (n ¼ 186). Only 28% of
patients in ranibizumab plus deferred laser group received P ro t o c o l T
laser in first year and 42% by year 2. At 24 months, mean
improvement in vision was +2 letters in laser group; +7 letters Ob je c t ive : to com pare aflibercept, bevacizum ab, and
in ranibizum ab with prompt laser group; +10 letters in ranibizum ab for the treatm ent of center involving DME
ranibizumab with deferred laser group; and +0 letters in
triamcinolone plus laser group. Mean change in retinal Re s u lt s : 660 patients random ly assigned and
thickness was À102 µm in laser group; À131 µm in followed for 2 years. At 1 year, m ean visual-acuity score
ranibizumab with prompt laser group; À137 µm in (range, 0 to 100, with higher scores indicating better
ranibizumab with deferred laser group; and À 127 µm in visual acuity; a score of 85 is approxim ately 20/20)
triamcinolone plus laser group. 5 year extended follow up im proved by 13.3 with aflibercept, by 9.7 with
report noted that number of visits declined in years 2 bevacizum ab, and by 11.2 with ranibizum ab. When initial
through 5 (interval could be extended up to 16 weeks starting visual-acuity letter score was 78 to 69 (equivalent to
in year 2). Of m axim um of 65 visits, prompt laser group approxim ately 20/32 to 20/40) (51% of participants), m ean
had m ean of 38 visits and deferred laser group had 40, im provem ent was 8.0 with aflibercept, 7.5 with
and m edian of 13 injections was given in prompt laser group bevacizum ab, and 8.3 with ranibizum ab. When initial letter
and 17 in deferred laser group. score was less than 69 (approxim ately 20/50 or worse),
m ean im provem ent was 18.9 with aflibercept, 11.8 with
bevacizum ab, and 14.2 with ranibizum ab. There were no
Co n c lu s io n s : intravitreal ranibizum ab with prom pt or
significant differences am ong study groups in rates of
deferred (! 24 weeks) focal / grid laser had superior VA and
serious adverse events, hospitalization, death, or m ajor
OCT outcom es compared with focal / grid laser treatm ent
cardiovascular events. Median num bers of injections were 5,
alone. Although intravitreal triam cinolone com bined with
6, and 6 in year 2 and 15, 16, and 15 over 2 years in
focal / grid laser did not result in superior VA outcom es
aflibercept, bevacizum ab, and ranibizum ab groups,
com pared with laser alone, an analysis lim ited to
respectively. Focal/grid laser was adm inistered in 41%,
pseudophakic eyes showed that triam cinolone group’s
64%, and 52%, respectively. At 2 years, m ean VA im proved
outcom e for VA appeared to be of sim ilar m agnitude to that
by 12.8, 10.0, and 12.3 letters, respectively. With worse
of 2 ranibizum ab groups.
baseline VA (20/50 to 20/320), m ean im provem ent was
18.3, 13.3, and 16.1 letters, respectively. With better
P ro t o c o l S baseline VA (20/32 to 20/40), m ean im provem ent was 7.8,
6.8, and 8.6 letters, respectively. Anti-Platelet Trialists’
Ob je c t ive : to evaluate noninferiority of intravitreous Collaboration (APTC) events occurred in 5% with
ranibizum ab com pared with PRP for visual acuity outcom es aflibercept, 8% with bevacizum ab, and 12% with
in patients with proliferative diabetic retinopathy ranibizum ab

338
 Disorders

occlusions [wedge sign], CRAO, RVO, choroid; CRAO can

Co n c lu s io n s : aflibercept, bevacizum ab, or ranibizum ab develop in patients with hyphem a), venous tortuosity,
im proved vision in eyes with center-involved diabetic
com m a-shaped conjunctival vessels, vitreous hem orrhage,
m acular edem a, but the relative effect depended on baseline
rhegm atogenous and traction RD (due to contracture of NV
visual acuity. When initial visual-acuity loss was m ild, there
fronds) (Figs.11-63 to 11-65)
were no apparent differences, on average, am ong study
Stage 1: Peripheral arterial occlusions
groups. At worse levels of initial visual acuity, aflibercept
Stage 2: Peripheral anastom oses
and ranibizum ab was m ore effective at im proving vision
Stage 3: Neovascularization (sea fan) at posterior border
than bevacizum ab
of areas of non-perfusion
Stage 4: Vitreous hem orrhage
Intra vitre a l Aflib e rc e p t fo r Dia b e tic Stage 5: Vitreous traction with RD
Ma c ula r Ed e m a (VIVID/VISTA) Tria ls
Ob je c t ive : to evaluate aflibercept versus laser therapy for Diagn o sis: sickle cell prep, Hgb electrophoresis
center involving DME

Re s u lt s : 872 eyes random ized to receive aflibercept 2q4 FA: capillary nonperfusion, AV anastom oses,
(n = 155), aflibercept 2q8 (n = 152), or laser neovascularization (Fig. 11-66)
photocoagulation (n = 154). Mean vision gains from
baseline to week 52 in aflibercept 2q4 and 2q8 groups
versus laser group were 12.5 and 10.7 versus 0.2 letters in
VISTA, and 10.5 and 10.7 versus 1.2 letters in VIVID. Mean
reductions in central retinal thickness were 185.9 and 183.1
versus 73.3 µm in VISTA, and 195.0 and 192.4 versus
66.2 µm in VIVID. After 2 years, m ean vision im proved 11.5
letters in aflibercept 2q4 group and 11.1 letters in 2q8
group. Patients in laser photocoagulation group had m ean
BCVA im provem ent of 0.9 letters. After 3 years, patients
receiving aflibercept every m onth had m ean gain in BCVA
from baseline of 10.3 letters, patients receiving aflibercept
every two m onths had m ean gain in BCVA from baseline of
11.7 letters. Patients in the laser photocoagulation
treatm ent group had m ean change in BCVAfrom baseline of
1.6 letters. Safety was sim ilar am ongst groups

Fig ure 11-63. An equatorial “salmon patch” intraretinal hemorrhage with

Co n c lu s io n s : at week 52, aflibercept dem onstrated periarteriolar hemorrhage. (Courtesy of William Tasman, MD. From Ho AC:
significant superiority in functional and anatom ic Hemoglobinopathies. In: Yanoff M, Duker J S (eds): Ophthalmology. London,
1999, Mosby.)
endpoints over laser, with sim ilar efficacy in 2q4 and 2q8
groups. Results held for 3 years of follow up

S ic kle c e ll (S C) re t in o p a t h y
Proliferative retinopathy, usually equatorial or pre-equatorial
Most severe in HbSC disease; SC > S Thal > SS> SA
Incidence in African Am erican population: SS¼ 1%; sickle
trait ¼ 8%; SC < 0.5%;
20–60% autoinfarct, unlike DR, neovascularization can
regress spontaneously and involute

Fin d in gs: salm on patch (intraretinal hem orrhage following

peripheral retinal arteriolar occlusion), black sunburst
(chorioretinal scar with RPE proliferation due to old
hem orrhage), sea fan (peripheral NV), refractile spots (old, Fig ure 11-64. A black “sunburst” retinal lesion. (From Ho AC:
resorbed hemorrhages), silver wiring of peripheral vessels, Hemoglobinopathies. In: Yanoff M, Duker J S (eds): Ophthalmology.
angioid streaks, vascular occlusions (m acular arteriolar London, 1999, Mosby.)

339
CHAPTER 11 • Posterior Segm ent

form ation around occluded vessels, peripheral nonperfusion;

m ay have AC reaction, KP, vitreous cells, m acular edem a
Stage 1: sheathing of retinal venules, retinal edem a,
hem orrhages
Stage 2: m ore severe involvem ent and vitreous haze
Stage 3: peripheral NV
Stage 4: proliferative retinopathy with VH and TRD

Oth er fin d in gs: vestibulocochlear involvem ent

(sensorineural hearing loss and vestibular dysfunction),
PPD-positive, cerebral vasculitis (rare), epistaxis

DDx o f p er ip h er al n eo vascu lar izatio n :

hem oglobinopathies, diabetes, retinal vein occlusions, ROP,
FEVR, retinal em boli (talc), hyperviscosity syndrom es, carotid-
cavernous sinus fistula, ocular ischem ia, sarcoidosis, lupus,
Fig ure 11-65. A sea fan in a sickle SC patient. (From Ho AC:
Hemoglobinopathies. In: Yanoff M, Duker J S (eds): Ophthalmology.
inflam m atory bowel disease, retinal vasculitis, uveitis, VKH
London, 1999, Mosby.) syndrom e, pars planitis, Norrie disease, incontinentia pigm enti

Re t in o p a t h y o f p re m a t u rit y
(ROP ; re t ro le n t a l fib ro p la s ia )
(See Chapter 5, Pediatrics/Strabism us)

Fa m ilia l e xu d a t ive vit re o re t in o p a t h y (FEVR)

(See Chapter 5, Pediatrics/Strabism us)

Bra n c h re t in a l ve in o c c lu s io n (BVO)
Site of occlusion is at AV crossing (usually thickened artery
com presses vein in com m on adventitial sheath); generally
superotem poral (63%)
Associated with increased age (> 60 years), cardiovascular
disease, hypertension, increased BMI at age 20, glaucom a,
papilledem a, optic disc drusen, and high serum alpha 2 -
globulin. Note: diabetes is not a risk factor. Decreased incidence
with high levels of HDL cholesterol and light to m oderate
Fig ure 11-66. Fluorescein angiography of s ea fans with peripheral
alcohol consum ption (especially after ophthalm ology
nonperfusion. (From Ho AC: Hemoglobinopathies. In: Yanoff M, Duker J S (eds):
Ophthalmology. London, 1999, Mosby.) board exam s)

Typ es:
Tr eatm en t: laser (PRP) or consider cryotherapy Nonischemic: < 5 DD of capillary nonperfusion
(controversial) for neovascularization; surgery for RD and Ischemic: ! 5 DD of capillary nonperfusion
chronic VH; risk of anterior segm ent ischem ia if encircling
scleral buckle placed or large volum e of intraocular gas; try to Fin d in gs: num erous deep and superficial hem orrhages (in a
avoid epinephrine in infusion fluid during surgery wedge-shaped distribution with the tip pointing toward the
etiologic crossing), cotton wool spots, CME, disc edem a
(Figs. 11-67, 11-68)
Ea le s ’ d is e a s e
Idiopathic retinal perivasculitis and peripheral nonperfusion Tr eatm en t: sector PRP for NV and VH, grid laser for
with NV m acular edem a when vision < 20/40 for at least 3 m onths and
Occurs in healthy young m en; 90% bilateral no m acular ischem ia on FA; recent studies with intravitreal
More com m on in Middle East and India anti-VEGF and steroids agents have shown better results
Increased risk of BVO than laser
Branch Vein Occlusion Study (BVOS):
Perform grid argon laser for m acular edem a and vision
Fin d in gs: NV (80%, NVD or NVE), recurrent VH, < 20/40 for > 3 m onths (treated eyes m ore likely to
rubeosis, neovascular glaucom a, cataract, vascular sheathing have im proved vision: +6.7 letters vs. +1 letter with
(80%) with leakage on FA, vascular tortuosity, collateral observation)

340
 Disorders

Fig ure 11-69. Nonischemic central retinal vein occlusion. (From Heier J S,
Morley MG: Venous obstructive disease of the retina. In: Yanoff M, Duker J S (eds):
Fig ure 11-67. BVO. Early leakage. Ophthalmology. London, 1999, Mosby.)

Co m p licatio n s:
Nonischemic BVO: m acular edem a can develop
Ischemic BVO: m acular edem a, ischem ic m aculopathy,
neovascularization, vitreous hem orrhage, tractional or
rhegm atogenous RD; 40% risk of developing NV
(NVI rare)

Ce n t ra l re t in a l ve in o c c lu s io n (CVO)
Throm bosis of central retinal vein at or posterior to the lam ina
cribrosa

Typ es:
Perfused/non-ischemic (70%): vision > 20/200, 16%
progress to nonperfused; 50% resolve com pletely
Fig ure 11-68. BVO. Late leakage.
without treatm ent; defined as < 10 DD of capillary
nonperfusion (Fig. 11-69)
Non-perfused / ischemic (30%): ! 10 DD non-perfusion;
Perform PRP for ! 5 DD of nonperfusion if patients are older and have worse vision; 60%
neovascularization develops (treated eyes less likely develop iris NV; up to 33% develop neovascular
to develop NV and VH) glaucom a (NVG; “90-day” glaucom a since it occurs
Branch Retinal Vein Occlusion (BRAVO) Study: 3–5 m onths after occlusion); extensive hem orrhage
Intravitreal injection of ranibizum ab (Lucentis) for with m arked venous dilation and cotton wool spots;
m acular edem a very poor prognosis with only 10% having better than
Intravitreal Aflibercept for Macular Edema Following Branch 20/400 vision (Fig. 11-70)
Retinal Vein Occlusion (VIBRANT) Study: 10% com bined with BRAO (usually cilioretinal artery due to
Intravitreal injection of aflibercept for m acular edem a low perfusion pressure of choroidal system )
Global Evaluation of Implantable
Dexamethasone in Retinal Vein Occlusion with Macular
Edema (GENEVA) Study:
Intravitreal injection of sustained release Risk facto r s: age (> 50 years old in 90%), hypertension
dexam ethasone intravitreal im plant (Ozurdex) for (61%), diabetes (unlike BVO), heart disease, glaucom a,
m acular edem a increased ESR in wom en, syphilis, sarcoidosis, vasculitis,
increased intraorbital or intraocular pressure, hyphem a,
Pr o gn o sis: 30% have spontaneous recovery; > 50% hyperviscosity syndrom es (m ultiple m yelom a, Waldenstro€ m ’s
m aintain vision better than 20/40 after 1 year; 10% have m acroglobulinem ia, leukem ia), high hom ocysteine levels,
episode in fellow eye sickle cell, HIV

341
CHAPTER 11 • Posterior Segm ent

Global Evaluation of Implantable Dexamethasone in Retinal

Vein Occlusion with Macular Edema (GENEVA) Study:
Intravitreal injection of dexam ethasone sustained-release
intravitreal im plant (Ozurdex) for m acular edem a [as
of 2011 the only FDA-approved treatm ent for m acular
edem a from vein occlusion]; tim e to first gain of ! 15
letters was faster with the biodegradable im plant
com pared with observation with 20–30% 3-line
gainers. Watch IOP as 25% eyes increased.

Pr o gn o sis: > 75% have disease progression.

Co m p licatio n s: iris NV(m ore com m on than NVD or NVE),

NVG, TRD, VH, and m acular edem a

He m ire t in a l ve in o c c lu s io n (HRVO)
Fig ure 11-70. Ischemic central retinal vein occlusion. (From Heier J S, Morley
MG: Venous obstructive disease of the retina. In: Yanoff M, Duker J S (eds):
Risk facto r s: hypertension, diabetes, glaucom a
Ophthalmology. London, 1999, Mosby.)
Isch em ic HRVO: higher risk of NVD (30%) or NVE (40%)
Fin d in gs: venous dilation and tortuosity, hem orrhages in all than with either an ischem ic CVO or a BVO; NVI in 10%
4 quadrants, m ay have disc edem a and m acular edem a
High-risk characteristics: m arked vision loss, num erous MAJ OR RETINAL VEIN OCCLUSION
cotton wool spots, positive RAPD, dense central scotom a
CLINICAL STUDIES
with peripheral field changes on VF, widespread capillary
nonperfusion on FA, reduced b-wave-to-a-wave ratio
on ERG Bra nc h Ve in Oc c lus io n Stud y (BVOS)

Path o lo gy: m arked retinal edem a, focal retinal Ob je c t ive : to evaluate photocoagulation in patients with
necrosis, gliosis; subretinal, intraretinal, and preretinal branch vein occlusion (BVO) for:
hem orrhages 1. Prevention of neovascularization
2. Prevention of vitreous hem orrhage
Wo r ku p : younger patients require m ore extensive workup 3. Im provem ent of vision in eyes with m acular edem a,
including system ic vascular disease (hypertension, diabetes reducing vision to 20/40 or worse
m ellitus, cardiovascular disease), blood dyscrasias
(polycythemia vera, lym phom a and leukem ia), clotting Me t h o d s :
disorders (activated protein C resistance, lupus anticoagulant, Group I (at risk for neovascularization): patien ts with
anticardiolipin antibodies, protein C and protein S, BVO occurring within 3–18 m on ths with an area
antithrom bin III) paraproteinem ia and dysproteinem ias, of retinal in volvem en t at least 5 DD in size,
m ultiple m yelom a, cryoglobulinem ia, vasculitis, syphilis, with n o n eovascularization present and vision
sarcoidosis, autoim m une disease, system ic lupus better than 5/ 200, were random ly assigned to
erythem atosus, oral contraceptive use in wom en, other rare peripheral scatter laser ph otocoagulation vs
associations (closed-head traum a, optic disc drusen, observation
arteriovenous m alform ations of retina) Group II (at risk for vitreous hem orrhage): patients with
BVO occurring within 3–18 m onths with disc or
Tr eatm en t: follow every m onth for first 6 m onths, including peripheral neovascularization and vision better than
gonioscopy; Intravitreal steroids or anti-VEGF agents for 5/200 were random ly assigned to peripheral scatter
m acular edem a laser photocoagulation vs observation
Central Vein Occlusion Study (CVOS): Group III (at risk for vision loss due to m acular edem a):
No benefit from early PRP (to prevent iris NV) in patients with BVO occurring within 3–18 m onths
ischemic CVO; therefore, wait until first sign of NV with m acular edem a involving the fovea and vision
No benefit from focal laser for m acular edem a worse than 20/40 were random ly assigned to grid
Central Retinal Vein Occlusion (CRUISE) Study: pattern laser photocoagulation with argon laser vs
Intravitreal injection of ranibizum ab (Lucentis) for observation
m acular edem a Group X (at high risk for neovascularization): patients
Intravitreal Aflibercept Injection for Macular Edema with BVO occurring within 3–18 m onths with at least
Resulting from Central Retinal Vein Occlusion 5 DD of capillary nonperfusion were followed. Note:
(COPERNICUS/GALILEO) Studies: This group was recruited after group I recruitm ent
Intravitreal injection of aflibercept (Eylea) for had ended
m acular edem a

342
 Disorders

Re s u lt s : group and 35% in the 4 m g TA group. Cataract progression

was seen in 18% in standard of care group, 26% in 1 m g TA
Group 1: 319 patients enrolled. After average follow-up
group and 33% in 4 m g TA group.
of 3.7 years, developm ent of neovascularization
was significantly less in laser-treated eyes. Treated
eyes were less likely to develop NV (12% vs 24% of Co n c lu s io n s : triam cinolone acetonide was superior to
untreated) observation in CVO (5 tim es greater chance of gaining
Group II: 82 eyes enrolled. After average follow-up 3 lines), but not to laser in BVO. Com plications were higher
of 2.8 years, developm ent of vitreous hem orrhage in the 4 m g TA group than the 1 m g TA group, so 1 m g is
was significantly less in laser-treated eyes. Treated preferred.
eyes were less likely to develop VH (29% vs 61% of
untreated)
Group III: 139 patients enrolled. After an average follow- Stud y o f COm p a ra tive Tre a tm e nts fo r
up of 3.1 years, treated eyes were m ore likely to have REtina l Ve in Oc c lus io n 2 (SCORE2)
im proved vision (gain of ! 2 lines in 65% vs 37% of
untreated), vision > 20/40, and final average vision
Ob je c t ive : non-inferiority 6 m onth trial to com pare
better than untreated eyes
bevacizum ab vs aflibercept for treatm ent of center involving
Group X: eyes with ! 5 DD of nonperfusion were
m acular edem a due to central retinal vein occlusion
considered nonperfused and showed greater risk of
developing neovascularization Me t h o d s : 362 patients enrolled with results expected in
4Q 2016. Study eyes random ized 1:1 to intravitreal
Co n c lu s io n s : bevacizum ab (1.25 m g) every 4 weeks vs. intravitreal
Grid pattern laser photocoagulation recom m ended aflibercept (2.0 m g) every 4 weeks for treatm ent of CRVO.
for eyes with a BVO of 3–18 m onths duration Ozurdex (dexam ethasone) will be offered as rescue therapy
if visual acuity is 20/40 or worse and if fluorescein in one study arm
angiography docum ents m acular edem a without
foveal hem orrhage as cause of visual loss BRAnc h Re tina l Ve in Oc c lus io n (BRAVO)
Perform PRP if retinal neovascularization develops,
especially if ! 5 DD of nonperfusion exists Stud y
Ob je c t ive : to assess the safety and efficacy profile of
The Sta n d a rd Ca re vs Co rtic o s te ro id fo r ranibizum ab in m acular edem a secondary to branch retinal
Re tina l Ve in Oc c lus io n (SCORE) Stud y vein occlusion.

Ob je c t ive : to com pare intravitreal triam cinolone to Me t h o d s : m ulticenter, random ized, double-m asked,
observation in retinal vein occlusion sham injection-controlled phase III study of 397 patients
designed to assess safety and efficacy profile of
Me t h o d s : Patients > 18 years old with center-involved ranibizum ab in m acular edem a secondary to branch-RVO .
m acular edem a due to CVO or BVO of at least 3 m onths’ Patients were included if they were ! 18 years age with
duration but no longer than 18 m onths with central foveal center-involved ME secondary to branch / hem i RVO
retinal thickness > 250 µm on OCT and ETDRS visual diagnosed within 12 m onths prior to screening. BCVA
acuity score 20/40–20/200 random ized to either 1 m g 20/40 to 20/400 and retin al thickness ! 250 µm . Patients
or 4 m g doses of preservative-free intravitreal were random ized to 0.3 m g ranibizum ab, 0.5 m g, or sham
triam cinolone acetonide (TA) versus standard of care injections. Laser rescue after 3 m onths. Prim ary endpoint
(observation in CVO, and focal laser in BVO). The prim ary was m ean change in vision from baseline at 6 m onths. In
outcom e was ! 3 line gainers at m onth 12. All patients will next 6 m onths, m onthly ranibizum ab PRN was allowed for
be followed and retreated as needed every 4 m onths for all patients with rescue laser at m onth 9.
3 years
Re s u lt s : At 6 m onths, m ean change in vision (prim ary
Re s u lt s : 271 patients enrolled. For CVO, patients with outcom e) was +7.3 letters in the sham group (n ¼ 132),
! 3 lin e gain in vision at m onth 12 (prim ary outcom e) was +16.6 letters in 0.3 m g ranibizum ab group (n ¼ 134), and
7% in observation group (n ¼ 73), 27% in 1 m g TA group +18.3 letters in 0.5 m g ranibizum ab group (n ¼ 131).
(n ¼ 83) and 26% in 4 m g TA group (n ¼ 82). Mean change In addition, 55% (74/134) of patients who received 0.3 m g
in vision at 12 m onths was À12.1 letters in observation of ranibizum ab and 61% (80/131) who received 0.5 m g of
group, À1.2 letters in the 1 m g TA group and À1.2 letters in ranibizum ab had their vision im proved by ! 15 letters,
4 m g TA group. For BVO, the patients with ! 3 line gain in com pared with 29% (38/132) of patients receiving sham
vision at m onth 12 (prim ary outcom e) was 29% in the injections. Mean gain in BCVA was observed beginning at
laser group, 26% in the 1 m g TA group and 27% in the day seven with a +7.6 and +7.4 letter gain in the 0.3 m g and
4 m g TA group. Overall, IO P lowering m edications were 0.5 m g study arm s of ranibizum ab, respectively (com pared
required in 8% in the laser group, 20% in the 1 m g TA with +1.9 letters in the sham injection arm ).

343
CHAPTER 11 • Posterior Segm ent

Co n c lu s io n s : ranibizum ab injections are safe and Group M (m acular edem a): patients with CVO occurring
effective in branch retinal vein occlusion for at least 3 m onths with m acular edem a that
involves the fovea and with visual acuity of 20/50 to
5/200 were random ly assigned to grid pattern laser
Intra vitre a l Aflib e rc e p t fo r Ma c ula r photocoagulation or observation; 155 eyes enrolled
Ed e m a Fo llo wing Bra nc h Re tina l Ve in
Re s u lt s :
Oc c lus io n (VIBRANT) Stud y
Group P: 34% becam e nonperfused at 3 years
Ob je c t ive : to com pare efficacy and safety of intravitreal RISK FACTORS FOR PROGRESSION TO NONPERFUSED:
aflibercept injection (IAI) with m acular grid laser CVO of less than 1 m onth duration, visual acuity less
photocoagulation for treatm ent of m acular edem a after than 20/200, presence of greater than 5 disc areas of
branch retinal vein occlusion (BRVO) nonperfusion
Group N: prophylactic PRP did not prevent iris or angle
Me t h o d s : eyes that gained ! 15 ETDRS letters from NV (18 of 90 eyes [20%] with prophylactic PRP
baseline at 6 months was 52.7% in IAI group com pared developed iris or angle NV, vs 32 of 91 eyes [35%] that
with 26.7% in laser group. Mean im provem ent from did not receive prophylactic PRP), eyes
baseline BCVA at week 24 was 17.0 ETDRS letters in IAI prophylactically treated with PRP did not respond
group and 6.9 ETDRS letters in laser group. Mean reduction well to supplem ental PRP (18 of 32 [56%]
in CRT from baseline at week 24 was 280.5 µm in IAI group observation eyes responded to PRP following
and 128.0 µm in laser group developm ent of iris or angle NV, vs 4 of 18 eyes [22%]
Co n c lu s io n s : m onthly aflibercept provided that received prophylactic PRP responded to
significantly greater visual benefit and reduction in CRT at supplem ental PRP).
RISK FACTORS FOR DEVELOPING NVI: am ount of
24 weeks than grid laser photocoagulation in eyes with
m acular edem a after BRVO nonperfused retina, extent of retinal hem orrhages,
m ale sex, CVO of less than 1-m onth duration
Group M: grid pattern photocoagulation did not preserve
Ce ntra l Ve in Oc c lus io n Stud y (CVOS) or im prove visual acuity (but reduced angiographic
evidence of m acular edem a)
Ob je c t ive : to evaluate patients with central retinal vein
occlusion (CVO) for: Co n c lu s io n s :

1. Natural history of eyes with perfused (< 10 disc areas of No benefit from early PRP in nonperfused CVO;
nonperfusion) CVO therefore, wait until first sign of NVI before
2. Im provem ent of vision in eyes with perfused initiating PRP
m acular edem a PRP for nonperfused CVO when 2 clock hours of NVI or
3. Eyes with nonperfused CVO; does early PRP any angle NV present
prevent NVI? Monthly follow-up with gonioscopy during first
4. Eyes with nonperfused CVO; is early PRP m ore effective 6 m onths after CVO
than delayed PRP in preventing NVG? No benefit from focal laser for treatm ent of m acular
edem a after CVO
Me t h o d s :
Group P (perfused): patients with CVO occurring for less
than 1 year with intraretinal hem orrhages in all 4 Ce ntra l Re tina l Ve in Oc c lus io n
quadrants and with capillary nonperfusion less than (CRUISE) Stud y
10 DD in size and with no iris or angle
neovascularization. These patients were observed; 547 Ob je c t ive : to assess safety and efficacy profile of
patients enrolled ranibizum ab in m acular edem a secondary to central-RVO.
Group N (nonperfused): patients with CVO occurring for
less than 1 year with intraretinal hem orrhages in all 4 Me t h o d s : m ulticenter, random ized, double-m asked,
quadrants and with capillary nonperfusion greater sham injection-controlled phase III study designed to
than 10 DD in size and with no iris or angle assess safety and efficacy profile of ranibizum ab in 392
neovascularization were random ly assigned to patients with m acular edem a secondary to central-RVO.
im m ediate PRP or observation; 180 patients enrolled Patients were included if they were ! 18 years age with
Group I (indeterm inate): patients with CVO occurring foveal center-involved ME secondary central RVO
for less than 1 year with intraretinal hem orrhages in diagnosed within 12 m onths prior to screening. BCVA 20/
all 4 quadrants and with retinal hem orrhages that 40 to 20/400 and baseline retinal thickness ! 250 µm .
prevent m easurem ent of the area of capillary Patients were random ized to 0.3 m g ranibizum ab, 0.5 m g,
nonperfusion and with no iris or angle or sham injections. Prim ary endpoint was m ean change in
neovascularization. These patients were followed; 52 vision from baseline at 6 m onths. In the next 6 m onths,
eyes enrolled m onthly ranibizum ab PRN was allowed for all patients.

344
 Disorders

Re s u lt s : at 6 m onths, m ean change in vision (prim ary Intra vitre a l Aflib e rc e p t Inje c tio n fo r
outcom e) was +0.8 letters in sham group (n ¼ 130), +12.7
Ma c ula r Ed e m a Re s ulting fro m Ce ntra l
letters in 0.3 m g ranibizum ab group (n ¼ 132), and +14.9
letters in 0.5 m g ranibizum ab group (n ¼ 130). In Re tina l Ve in Oc c lus io n (GALILEO)
addition, 46% (61/132) of patients given 0.3 m g of
ranibizum ab and 48% (62/130) given 0.5 m g of Ob je c t ive : phase 3 studies to determ ine efficacy of
ranibizum ab had their vision im proved by ! 15 letters aflibercept injected into the eye on vision function in
com pared with 17% (22/130) of patients receiving sham subjects with m acular edem a due to central retinal vein
injections. Mean gain in BCVA was observed beginning at occlusion (CRVO)
day seven with a +8.8 and +9.3 letter gain in 0.3 m g and
0.5 m g study arm s of ranibizum ab, respectively, com pared
Me t h o d s : patients received either 2 m g intravitreal
with +1.1 letters in sham injection arm
aflibercept or sham injections every 4 weeks for 20 weeks.
From week 24 to 48, aflibercept group received aflibercept
Co n c lu s io n s : ranibizum ab injections are safe and PRN, and sham group continued receiving sham injections
effective in central retinal vein occlusion

Re s u lt s : 177 patients in GALILEO. At week 52, m ean

Intra vitre a l Aflib e rc e p t Inje c tio n fo r percentage of patients gaining 15 letters or m ore was 60.2%
Ma c ula r Ed e m a Re s ulting fro m Ce ntra l in aflibercept group and 32.4% in sham group. Aflibercept
patients, com pared with sham patients, had significantly
Re tina l Ve in Oc c lus io n (COP ERNICUS)
higher m ean im provem ent in BCVA (+16.9 letters vs. +3.8
letters, respectively) and reduction in central retinal
thickness (-423.5 µm vs. -219.3 µm , respectively) at week
Ob je c t ive : phase 3 studies to determ ine efficacy of 52. Aflibercept patients received a m ean of 2.5 injections
aflibercept injected into the eye on vision function in (standard deviation, 1.7 injections) during PRN dosing
subjects with m acular edem a due to central retinal vein
occlusion (CRVO)
Co n c lu s io n s : treatm ent with intravitreal aflibercept
Me t h o d s : 189 patients random ized in COPERNICUS. provided significant visual and anatom ic benefits after 52
Patients received IAI 2 m g (IAI 2Q4) (n = 114) or sham weeks as com pared with sham . Im provem ents achieved
injections (n = 74) every 4 weeks up to week 24. During after 6 m onthly doses at week 24 largely were m aintained
weeks 24 to 52, patients from both arm s were evaluated until week 52 with PRN dosing
m onthly and received IAI as needed, or pro re nata (PRN)
(IAI 2Q4 + PRN and sham + IAI PRN). During weeks 52 to
100, patients were evaluated at least quarterly and received
IAI PRN
Bra n c h re t in a l a rt e ry o c c lu s io n (BRAO)
Re s u lt s : proportion of patients gaining ! 15 letters was 90% caused by em boli (cholesterol, calcium , fibrin, platelets)
56.1% versus 12.3% at week 24, 55.3% versus 30.1% at
week 52, and 49.1% versus 23.3% at week 100 in the Most com m only at retinal arterial bifurcations
aflibercept 2Q4 + PRN and sham + IAI PRN groups, 10% risk of episode in fellow eye
respectively. Mean change from baseline BCVA was also
significantly higher in IAI 2Q4 + PRN group com pared with
sham + IAI PRN group at week 24 (+17.3 vs. -4.0 letters), Ce n t ra l re t in a l a rt e ry o c c lu s io n (C RAO)
week 52 (+16.2 vs. +3.8 letters), and week 100 (+13.0 vs.
+1.5 letters). Mean reduction from baseline in central retinal Most com m on cause of cherry red spot (thin transparent foveal
thickness was 457.2 versus 144.8 µm at week 24, 413.0 tissue surrounded by opacified ischem ic retina)
versus 381.8 µm at week 52, and 390.0 versus 343.3 µm at
week 100 in IAI 2Q4 + PRN and sham + IAI PRN groups, Etio lo gy:
respectively. Mean num ber (standard deviation) of PRN Atherosclerosis: of CRA at lam ina cribrosa
injections in IAI 2Q4 + PRN and sham + IAI PRN groups was Emboli: cholesterol (73%), platelet fibrin (15%) from
2.7 Æ1.7 versus 3.9 Æ2.0 during weeks 24 to 52 and carotid plaque, calcific (from heart especially valves),
3.3 Æ2.1 versus 2.9 Æ2.0 during weeks 52 to 100, tum or (atrial m yxom a), lipid em boli (pancreatitis), talc
respectively Vasculitis: GCA in elderly patient (always check ESR)
Trauma: retrobulbar injection, orbital surgery, penetrating
Co n c lu s io n s : visual and anatom ic im provem ents after injury
fixed IAI dosing through week 24 and PRN dosing with Coagulopathy: oral contraceptives, pregnancy, sickle cell
m onthly m onitoring from weeks 24 to 52 were dim inished (especially after hyphem a), platelet or factor
after continued PRN dosing, with a reduced m onitoring abnorm alities, hom ocystinuria, hyperhom ocysteinem ia,
frequency from weeks 52 to 100 protein S deficiency, antiphospholipid antibody (check
anticardiolipid antibody panel; also at risk for DVT,

345
CHAPTER 11 • Posterior Segm ent

pulm onary em bolism , cardiac vessel blockage, and FA: delayed filling, increased AV transit tim e, focal staining
spontaneous abortion; treat with coum adin; steroids do at obstruction, collateral or retrograde flow (Fig. 11-72)
not decrease risk of throm boem bolism ) ERG: depressed b-wave (inner retina), norm al /
Ocular abnormalities: optic disc drusen, prepapillary loops, supernorm al a-wave, loss of oscillatory potentials
elevated IOP
Collagen vascular disease: lupus, polyarteritis nodosa, Tr eatm en t: irreversible dam age occurs after 90 m inutes;
Wegener’s granulom atosis therefore, em ergent lowering of IOP to allow arterial pressure
Migraine: in young patients to re-establish blood flow: paracentesis, ocular m assage,
Orbital mucor carbogen (95% O 2 , 5% CO 2 ) or breath into paper bag, oral and
Fibromuscular hyperplasia topical ocular hypotensive agents m ay be tried; PRP for
Behçet’s disease neovascular com plications
Leukemia
Pr o gn o sis: 10% recover vision (usually from cilioretinal
Fin d in gs: severe visual loss (LP to CF vision in 90%), retinal artery [in 25% of population]), 18% have vision ! 20/40, 66%
whitening in posterior pole with cherry red spot, box-carring have vision 20/400; < 5% develop neovascular glaucom a; 1%
(interruption of blood colum n), em boli, positive RAPD; with bilateral disease; 10% risk of episode in fellow eye
whitening resolves after 4–6 weeks, and pale disc and
attenuated vessels develop Co m p licatio n s: rubeosis (15%)

Path o lo gy: atrophy of inner retinal layers (those supplied by Op h t h a lm ic a rt e ry o c c lu s io n

retinal circulation) (Fig. 11-71)
NLP vision and no cherry red spot; RPE pigm entary changes
develop
DDx: ophthalm ic artery occlusion, inadvertent intraocular
injection of gentam icin, arteritic AION (check ESR and C- May occur with orbital m ucorm ycosis
reactive protein)
FA: absent choroidal and retinal filling
DDx o f ch er r y r ed sp o t: sphingolipidosis (Tay-Sachs,
Niem ann-Pick, Gaucher’s, Farber’s disease), quinine toxicity,
com m otio retinae, m acular hole with surrounding RD,
ERG: absent a-wave
m acular hem orrhage, subacute sclerosing panencephalitis,
ocular ischem ia, m ethanol toxicity Oc u la r is c h e m ic s yn d ro m e

Diagn o sis: Reduced blood flow to globe produces anterior and / or

Lab tests: CBC, ESR, com plete cardiovascular workup; posterior segm ent ischem ia
consider workup for clotting disorders: serum protein
electrophoresis, protein C, activated protein C resistance Etio lo gy: carotid occlusion (m ost com m on, usually > 90%
(factor 5 Lieden deficiency), protein S, antithrom bin III, obstruction), carotid dissection, arteritis (rare)
anticardiolipin antibodies, lupus anticoagulant, plasm a Associated with diabetes (56%), hypertension (50%), coronary
hom ocysteine, C-ANCA artery disease (38%), CVA/ TIA (31%)

Re tina l e de ma P a te nt P a te nt Abs e nt flow

ciliore tina l ciliore tina l
a rte ry a rte ry

Fig ure 11-71. Cilioretinal artery-sparing central retinal artery occlusion with Fig ure 11-72. Fluorescein angiogram of the same patient as in Fig. 11.71,
patent cilioretinal artery, allowing perfusion (thus no edema) in a small section demonstrating no filling of retinal vessels except in cilioretinal artery and
of the macula. (From Kaiser PK, Friedman NJ , Pineda R II: The Massachusetts surrounding branches. (From Kaiser PK, Friedman NJ , Pineda R II: The
eye and ear infirmary illustrated manual of ophthalmology, ed 2, Philadelphia, Massachusetts eye and ear infirmary illustrated manual of ophthalmology, ed 2,
2004, Saunders.) Philadelphia, 2004, Saunders.)

346
 Disorders

Fig ure 11-73. Retinal vascular changes in ocular ischemic syndrome. (From
Fox GM, Sivalingham A, Brown CG: Ocular ischemic syndrome. In: Yanoff Fig ure 11-74. Fluorescein angiography, ocular ischemic syndrome. (From
M, Duker J S (eds ): Ophthalmology. London, 1999, Mosby.) Fox GM, Sivalingham A, Brown CG: Ocular ischemic syndrome. In: Yanoff M,
Duker J S (eds):Ophthalmology. London, 1999, Mosby.)

Sym p to m s: am aurosis fugax, gradual or sudden visual loss, rads (150 Gy); occurs 36 m onths after external beam radiation
dull pain (im proves on lying down); m ay experience transient with m ean dose of 5000 rads (50 Gy)
visual loss, som etim es precipitated by exposure to bright light
(due to im paired photoreceptor regeneration)
Fin d in gs: hem orrhage, exudate, m icroaneurysm s, capillary
nonperfusion, cotton wool spots, NV, optic disc swelling, optic
Fin d in gs:
Anterior segment: chronic conjunctival injection, rubeosis neuropathy; gradual occlusion of larger retinal vessels,
eventually proliferative retinopathy
and neovascularization of the iris (NVI) com m on (IOP
often not elevated due to ciliary body shutdown), PAS,
AC cells and flare, corneal edem a, cataract, altered IOP, Path o lo gy: vascular decom pensation with focal loss of
hypopyon (rare) capillary endothelial cells and pericytes; radiation-induced
Posterior segment: vitreous cells, NV, optic disc pallor optic neuropathy: ischem ic dem yelination with obliterative
(40%), optic nerve swelling (8%), superficial endarteritis of nerve sheath vasculature
hem orrhages in m idperiphery, CME, cherry red spot
(18%), altered vessels (attenuated, box-carring, dilated, Tr eatm en t: focal laser for m acular edem a, PRP for NV;
nontortuous veins) (Fig. 11-73) hyperbaric O 2 (controversial)
Certain types of chem otherapy and pre-existing vascular
DDx: aortic arch disease, Takayasu’s disease
com prom ise (e.g., diabetic or hypertensive retinopathy) can
worsen radiation retinopathy
Diagn o sis: digital pressure on eye causes arterial pulsation
FA: delayed filling (retina and choroid), diffuse leakage
(Fig. 11-74) Hyp e rc o a g u la b le s t a t e s
Ophthalmodynamometry: decreased retinal arterial pressure Etio lo gy: antiphospholipid antibodies (lupus
Carotid ultrasound: severe stenosis (> 80%) anticoagulant) and anticardiolipins, Waldenstro€ m ’s
m acroglobulinem ia, leukem ia
Tr eatm en t: lower IOP to increase perfusion pressure,
carotid surgery; PRP (controversial because anterior Fin d in gs: cotton wool spots, blot hem orrhages, box-carring,
segm ent ischem ia can possibly be due to uveal ischem ia capillary nonperfusion, arteriolar attenuation
without retinal ischem ia); carotid endarterectom y (CEA)
beneficial when carotid stenosis 70–99% (not possible
when 100%) – m ay lead to increased IOP from im proved
Dis s e m in a t e d in t ra va s c u la r c o a g u la t io n
ciliary body perfusion May cause fibrinoid necrosis of choriocapillaris, serous retinal
detachm ents, and m ultiple areas of RPE changes
Ra d ia t io n re t in o p a t h y
P h a ko m a t o s e s
Slowly progressive m icroangiopathy following exposure to
(See Chapter 5, Pediatrics/Strabism us)
radiation (6 m onths to 3 years after radiation treatm ent)
Threshold dose is 300 rads (3 Gy); occurs 4–32 m onths In fla m m a t o r y/ Im m u n e Dis e a s e
following plaque brachytherapy with m ean dose of 15,000 (See Chapter 8, Uveitis)

347
CHAPTER 11 • Posterior Segm ent

In fe c t io n s DIAGNOSIS: stool for ova and parasites, CBC with

(See Chapter 8, Uveitis) differential (eosinophilia som etim es present);
LDH and SGOT som etim es elevated
Ne u r o re t in it is FA: early hypofluorescence and late staining of lesions,
perivascular leakage, and disc staining; m ultifocal
Usually unilateral window defects in late disease
ERG: subnorm al, loss of b-wave
50% have viral prodrom e TREATMENT: laser photocoagulation of the worm ;
rarely subretinal surgery to rem ove worm
(controversial)
Sym p to m s: decreased vision; colors appear washed out; System ic antihelm intic m edications
rarely, retrobulbar pain, pain associated with eye m ovem ent, or (thiabendazole, diethylcarbam azine,
headache pyrantel pam oate) are controversial and
often not effective; steroids are usually added
because worm death m ay increase
Fin d in gs: optic nerve swelling (optic atrophy can develop), inflam m ation
peripapillary NFL hem orrhages, serous RD; iritis, vitritis, and / PROGNOSIS: poor without treatm ent; variable if worm
or scleritis (rare); vascular occlusions m ay develop can be killed with treatm ent

Etio lo gy: DDx o f o p tic n er ve swellin g an d m acu lar star : Leber’s

Noninfectious: cerebral AV m alform ation, elevated ICP, idiopathic stellate neuroretinitis, syphilis, hypertension,
m alignant hypertension, ischem ic optic neuropathy, traum a, acute febrile illness (m easles, influenza),
polyarteritis nodosa, Leber’s idiopathic stellate TB, coccidiom ycosis, cat-scratch disease, papillitis,
neuroretinitis papilledem a, AION (m acular star rare), DUSN (m acular
LEBER’S IDIOPATHIC STELLATE NEURORETINITIS star rare)
(LISN):
Affects individuals age 8–55 years old (usually in 3rd
decade); m ale ¼ fem ale; > 70% unilateral Diagn o sis:
Viral prodrom e in 5% Lab tests: ESR, VDRL, FTA-ABS, Lym e titer, Bartonella
FINDINGS: decreased vision; positive RAPD (75%); serology, Toxoplasma and Toxocara titers, PPD
cecocentral or central scotom a; vitreous cells; optic FA: diffuse leakage from disc, peripapillary capillary
disc edem a (resolves over 8–12 weeks); optic staining; 10% have disc leakage in fellow eye; no leakage
atrophy is rare; m acular star develops in 2nd week; in m acula
exudative peripapillary RD can occur; RPE defects
late; rarely develop chorioretinitis with elevated
yellow-white spots in deep retina Tr eatm en t: treat underlying disease, observe idiopathic
FA: hot disc; no perifoveal capillary leakage or m acular form ; m ost recover ! 20/50 after 3 m onths; only 3–5% have
abnorm alities perm anent severe visual loss
PROGNOSIS: excellent
Infectious (suggested by m ultiple areas of retinitis): HIV Re t in o p a t h y
syphilis, Lym e disease, viral (influenza A, m um ps,
coxsackie B, EBV), cat-scratch disease (Bartonella Microangiopathy in up to 50% of hum an im m unodeficiency
henselae), TB, Salmonella typhi, parasites (Toxocara, virus (RNA retrovirus) infected individuals due to com plem ent
Toxoplasma, DUSN) deposition (not infectious)
DIFFUSE UNILATERAL SUBACUTE NEURORETINITIS Asym ptom atic, non-progressive
(DUSN):
Due to subretinal nem atode: Ancylostoma caninum
(dog hookworm ), Baylisascaris procyonis
(raccoon worm ) Fin d in gs: cotton wool spots, Roth spots, hem orrhages, and
Chronic course m icroaneurysm s in posterior pole; m any HIV-positive patients
Does not have characteristic neuroretinitis have early presbyopia caused by inflam m ation of ciliary body
appearance with loss of accom m odative am plitude
FINDINGS: m ultifocal pigm entary changes due to
m ovem ent of the worm , m inim al intraocular
inflam m ation, decreased vision (typically out of Diagn o sis: HIV antibody test, CD4 count, HIV viral load
proportion to findings); m ay have positive RAPD.
Late findings include visual field defects, optic
nerve pallor, chorioretinal atrophy, and narrowed Tr eatm en t: none; spontaneous resolution within
retinal vessels 1–2 m onths

348
 Disorders

To xic Re t in o p a t h ie s Th io r id a zin e (Me lla ril)

Concentrates in uveal tissue with RPE dam age, decreased
Am in o g lyc o s id e s (Ge n t a m ic in /
vision, nyctalopia, and som etim es altered color vision
To b ra m yc in / Am ika c in )
Fin d in gs: acute, severe, perm anent visual loss after To xic d o se: > 1200 m g/day
intraocular injection of toxic doses with m arked retinal
whitening (especially in m acula) and retinal hem orrhages; Fin d in gs: peripheral pigm entary retinopathy, pigm ent
optic atrophy and pigm entary changes occur later deposition in eyelids, cornea, and lens (Fig. 11-75)

To xic d o se: seen after injecting 0.1 m g of gentam icin (also ERG: depressed a and b-waves
described after diffusion through cataract wound from Progression of retinopathy can occur after cessation of
subconjunctival injection) m edication

Ch lo r p ro m a zin e (Th o ra zin e )

FA: sharp zones of capillary nonperfusion corresponding to
the areas of ischem ic retina To xic d o se: 1200–2400 m g/day for at least 12 m onths
No effective treatm ent with poor visual prognosis
Fin d in gs: pigm ent deposition in eyelids, cornea, lens, and
Ch lo r o q u in e (Ar a le n )/ Hyd ro xyc h lo ro q u in e retina
(P la q u e n il)
Ch lo r a m p h e n ic o l
Binds to m elanin in RPE; ganglion cells are also directly
affected Atrophy of m aculopapillar bundle

VF: cecocentral scotom a

To xic d o se: > 2.3 m g/kg real weight for chloroquine,
> 5.0 m g/kg real weight for hydroxychloroquine Daily dosage
is m ost im portant risk factor for retinal toxicity. Other risk Qu in in e
factors for hydroxychloroquine toxicity: duration of use (> 5 Fin d in gs: fine RPE m ottling, retinal vascular attenuation,
years with no other risk factors), renal disease, concom itant disc pallor
tam oxifen use (5x increase risk), liver disease, older age, and Toxicity causes blurred vision, visual field loss,
retinal/m acular disease. Toxicity in 7.5% of long-term users; photophobia, som etim es transient blindness; patient
risk of toxicity at recom m ended doses (< 5.0 m g/kg/day) is m ay becom e com atose
< 1% up to 5 years, < 2% up to 10 years, and $ 20% after 20 Acute toxicity (single dose of 4 g): blurred vision,
years. nyctalopia, nausea, loss of hearing, drowsiness, dilated
pupils, retinal opacification with cherry red spot, dilated
Fin d in gs: early, m ild m ottling of perifoveal RPE with retinal vessels; after acute phase resolves, vision can
decreased foveal reflex; progresses to bull’s-eye m aculopathy; im prove
peripheral pigm entary retinopathy, eyelash whitening, and
cornea vortex keratopathy m ay develop

DDx o f b u ll’s-eye m acu lo p ath y: cone dystrophy, AMD,

Stargardt disease, fundus flavim aculatus, Spielm eyer-Vogt
disease, albinism , fenestrated sheen m acular dystrophy, central
areolar choroidal dystrophy, benign concentric annular
m acular dystrophy, clofazim ine toxicity, fucosidosis

VF: decreased; paracentral scotom as

ERG: enlarged a-wave, depressed b-wave

EOG: depressed

OCT: ring of outer retinal thinning (UFO sign)

FAF: bull's eye pattern is often seen Fig ure 11-75. Thioridazine retinopathy associated with chronic use (nummular
retinopathy). (From Weinberg DV, D’Amico DJ : Retinal toxicity of systemic drugs.
Progression of retinopathy can occur after cessation of In: Albert DM, J akobiec FA (eds ): Principles and practice of ophthalmology.
m edication; vision loss rarely recovers Philadelphia, 1994, WB Saunders.)

349
CHAPTER 11 • Posterior Segm ent

Ta m o xife n Me t h o xyflu ra n e
To xic d o se: > 30 m g/day Crystalline retinopathy due to oxalate crystals
Methoxyflurane is m etabolized to oxalate, which binds with
Sym p to m s: asym ptom atic calcium to form insoluble calcium oxalate salt
Crystals are perm anent
Fin d in gs: fleck-like crystalline retinopathy with refractile
retinal deposits, typically in ring around m acula; m ay develop
pigm entary retinal changes and m ild CME (Fig. 11-76) Ta lc
Particles deposit in blood vessels of IV drug users; appear as
FA: m acular edem a tiny crystals
Talc deposits in lungs; with prolonged abuse pulm onary AV
Ca n t h a xa n t h in e shunts develop and talc passes into system ic circulation
To xic d o se: > 35 g (cum ulative) of this oral tanning agent Talc em boli can cause arteriolar occlusions, resulting in
ischem ic m aculopathy, retinal NV, and VH
Sym p to m s: asym ptom atic, or m ild m etam orphopsia
Tr eatm en t: consider PRP for neovascularization
Fin d in gs: refractile yellow deposits in a ring around the
fovea; “gold-dust” retinopathy (Fig. 11-77)
Nic o t in ic a c id m a c u lo p a t h y
Atypical nonleaking CME due to intracellular edem a of
Mu€ ller’s cells

Sym p to m s: blurred vision with m etam orphopsia

FA: CME does not leak

Dig o xin
Direct effect on cones; causes visual disturbance with
m inim al fundus changes

Sym p to m s: xanthopsia (yellow vision), blurred vision

Fin d in gs: poor color vision, paracentral scotom as, and

reduced vision with increased background light

Fig ure 11-76. Severe tamoxifen retinopathy. (From McKeown CA, Swartz M,
Blom J , et al: Tamoxifen retinopathy. Br J Ophthalmol 65:177–179, 1981.)
Erg o t a m in e
Vasoconstriction of retinal vessels, CME, CVO, papillitis, optic
disc pallor; orthostatic hypotension, postpartum hem orrhages

Ora l c o n t ra c e p t ive s
Associated with throm boem bolic disease
May develop CME, retinal hem orrhages, vascular occlusions

Va n c o m yc in
Intraocular/intracam eral (1 m g/0.1 m L) rarely associated with
hem orrhagic occlusive retinal vasculitis (HORV); probably
im m une hypersensitivity reaction (Type III) rather than direct
toxicity.
Delayed onset (1–14 days) of severe vision loss, m ild anterior
Fig ure 11-77. Canthaxanthine retinopathy. (From Weinberg DV, D’Amico DJ :
cham ber and vitreous reaction, large patches of intraretinal
Retinal toxicity of systemic drugs. In: Albert DM, J akobiec FA (eds): Principles and hem orrhages in sectors of retinal vascular occlusion; often
practice of ophthalmology. Philadelphia, 1994, WB Saunders.) develop NVG

350
 Disorders

In t e rfe ro n Me r id io n a l c o m p le x
Presum ed to be due to im m une com plex deposition in retinal Meridional fold extending to posterior aspect of a ciliary
vasculature followed by white blood cell infiltration with process
eventual vascular closure
Retinal break m ay occur
Fin d in gs: cotton wool spots, hem orrhages, m acular edem a, Norm al variation of anatom y at ora serrata
capillary nonperfusion, vascular occlusions
Vit re o re t in a l t u ft
May exacerbate autoim m une thyroiditis and polyarthropathy
Sm all internal projection of retinal tissue
S ild e n a fil (Via g ra )/ t a d a la fil (Cia lis )/ va rd e n a fil No n cystic r etin al tu ft: short, thin (base < 0.1 m m )
(Le vit ra ) projection of fibroglial tissue; can break off, leaving fragm ents
May cause transient blue hue to vision 1–2 hours after in vitreous; usually inferonasal; not associated with retinal
ingestion, possibly by changing the transduction cascade in break; not present at birth
photoreceptors

ERG: m ildly reduced photopic and scotopic b-wave

am plitudes and less than 10% decrease in photopic a- and
b-wave im plicit tim es during acute episode, reverts back to
norm al over tim e
For Viagra, occurs in 3% of individuals taking a dose of
25–50 mg, 11% of patients taking 100 m g dose, and in 50%
taking > 100 m g; no perm anent effects seen

In h e rit e d Re t in a l Dis e a s e s
(See Chapter 5, Pediatrics/Strabism us)

Me t a b o lic Dis e a s e s
(See Chapter 5, Pediatrics/Strabism us)

Vit re o re t in a l Dis o rd e rs
(See Chapter 5, Pediatrics/Strabism us)

De g e n e ra t io n s
(Table 11-5)

Ora l b a y
Oval island of pars plana epithelium im m ediately posterior to Fig ure 11-78. Enclos ed oral bays. (From Tasman WS: Peripheral retinal lesions.
ora serrata (Fig. 11-78); retinal break m ay occur In: Yanoff M, Duker J S (eds ): Ophthalmology. London, 1999, Mosby.)

Me rid io n a l fo ld
Elevated fold of retina in upper nasal quadrant
20% prevalence
Retinal break m ay occur (Fig. 11-79)

Ta b le 11-5. Classification of select retinal degenerations

Be nig n P re d is p o s ing to RD
White without pressure Lattice degeneration
Pigment clumping Snail track degeneration
Diffuse chorioretinal atrophy Zonuloretinal traction tufts
Peripheral microcystoid changes Snowflake degeneration
Pavingstone degeneration
Oral pigmentary degeneration Fig ure 11-79. Meridional fold with a small break at the base of
Degenerative “senile” retinoschisis the fold. (From Tasman WS: Peripheral retinal lesions. In: Yanoff M, Duker J S
(eds): Ophthalmology. London, 1999, Mosby.)

351
CHAPTER 11 • Posterior Segm ent

Cystic r etin al tu ft: chalky white, nodular projection (base Wh ite with p r essu r e: visible only with scleral depression
0.1–1.0 m m ) of fibroglial tissue; usually nasal; m ay have
pigm ent at base; increased vitreous adhesion predisposes to Wh ite with o u t p r essu r e: visible without scleral
tractional tears from PVD; present at birth; occurs in 5% of depression
population; < 1% risk of RD, therefore no prophylactic
treatm ent; associated with 10% of RDs; second to lattice
degeneration as visible peripheral retinal lesion associated P e rip h e ra l m ic ro c ys t o id d e g e n e ra t io n
with RD Typ ical / “sen ile” (Blessig-Iwanoff cysts): outer plexiform
layer; bubble-like appearance behind ora serrata; lined by
Zo n u lar tr actio n tu ft: thin strand of fibroglial tissue Mu€ ller’s cells; m ay coalesce and progress to typical
extending from peripheral retina anteriorly to a zonule; usually degenerative (“senile”) retinoschisis
nasal; present at birth; break occurs in 2–10%
Reticu lar : nerve fiber layer; linear or reticular pattern,
P a rs p la n a c ys t corresponding to retinal vessels; finely stippled internal
surface; continuous with and posterior to typical type
Clear cystoid space between pigm ented and nonpigm ented (therefore, does not reach the ora); m ay progress to reticular
ciliary epithelium of pars plana; filled with degenerative retinoschisis
m ucopolysaccharides (hyaluronic acid) (Fig. 11-80)
Not a true cyst (not lined with epithelium ) De g e n e ra t ive (in vo lu tio n a l) “s e n ile ” re t in o s c h is is
Occurs in 17% of autopsied eyes Occurs in 7–31% of individuals > 40 years old, 50–80%
Analagous to detachm ent of sensory retina from underlying bilateral, often sym m etric; 70% inferotem poral, 25%
RPE; caused by traction by vitreous base and zonules; no superotem poral
increased risk of RD Usually asym ptom atic and nonprogressive; hyperopia in 70%
Stains with Alcian blue
Fin d in gs: splits or cysts within neurosensory retinal layers;
Also seen in conditions with form ation of abnorm al proteins typical peripheral cystoid degeneration is precursor
(m ultiple m yelom a) Inner wall: sm ooth, dom e-shaped surface; m ay be
m arkedly elevated in reticular type; sheathed retinal
Ora s e rra t a p e a rl vessels; holes uncom m on (sm all if present)
Glistening opacity over oral tooth Outer wall: pock-m arked on scleral depression; holes (16–
23%) are larger and well delineated, m ore com m on in
Increased prevalence with age reticular type, have rolled m argins (Fig. 11-81)
Resem bles drusen Typical: splitting of OPL; bilateral; elderly patients; 1% of
adult population; follows coalescence of cavities of
Wh it e w it h / w it h o u t p re s s u r e m icrocystoid degeneration; beaten m etal appearance;
white spots (Mu€ ller’s cell rem nants [Gunn’s dots]) in
Geographic areas of peripheral retinal whitening stippled pattern along edge appear as glistening dots or
More com m on in young individuals, African Am ericans, and snowflakes; inner layer holes can occur; no risk of RD
m yopes

Fig ure 11-80. Pars plana cysts. (Courtesy of Dr. Ralph Eagle. From Tasman WS: Fig ure 11-81. Degenerative retinoschisis demonstrating multiple outer layer
Peripheral retinal lesions. In: Yanoff M, Duker J S (eds): Ophthalmology. London, breaks. (From Tasman WS: Peripheral retinal lesions. In: Yanoff M, Duker J S
1999, Mosby.) (eds): Ophthalmology. London, 1999, Mosby.)

352
 Disorders

unless outer layer hole is also present; intact outer retinal La t t ic e d e g e n e ra t io n

layer whitens / blanches with scleral depression
Occurs in 7% of population; m ore com m on in m yopic eyes; no
Reticular: splitting of NFL; 41% bilateral; 18% of adult
sex predilection
population; always posterior to peripheral cystoid; fine
delicately stippled surface Often bilateral; usually superotem poral
Present in 20–35% of eyes with RD; 0.5% risk that patient with
Differ en tiate fr o m RD: no underlying RPE degeneration,
lattice will develop an RD
no tobacco dust; absolute scotom a (relative scotom a in RD);
laser treatm ent blanches underlying RPE (RD will not blanch);
no shifting fluid; dom e shaped Fin d in gs: peripheral circum ferential cigar-shaped atrophic
retinal patches; criss-crossing pattern of sclerosed vessels (fine
Path o lo gy: cavity contains hyaluronic acid, Mu€ ller’s cell white lines) within lesion in 12%; superficial white dots in
rem nants on inner and outer surfaces of cavity, fibrous 80% (ILM and inner retina); pigm entary disturbance in 82%;
thickening of vessel walls with patent lum en firm adhesions of vitreous at m argins; clear pockets of vitreous
fluid over central thin portion; retinal breaks (round or
Tr eatm en t: laser barrier, treat tears and RD atrophic holes) in 18%, horseshoe tear in 1.4% at posterior or
lateral edge due to severe vitreous traction (Fig. 11-83)
Co m p licatio n s: RD (from outer layer holes; slowly
progressive, unless both outer and inner holes), progression Path o lo gy: discontinuity of ILM, overlying pocket of liquid
(toward posterior pole; rare through fovea), hem orrhage (into vitreous, condensation and adherence of vitreous at m argin of
vitreous or schisis cavity) lesion, focal area of retinal thinning with loss of inner retinal
layers, m elanin-laden m acrophages, fibrous thickening of
retinal vessel walls
P a vin g s t o n e / c o b b le s t o n e d e g e n e r a t io n
Usually inferior; 33% bilateral Tr eatm en t: no proof that prophylactic treatm ent prevents
Occlusion of choriocapillaris causes loss of outer retinal layers RD; consider treatm ent of fellow eye if history of RD from lattice
and RPE
De t a c h m e n t s
Increased incidence with age and m yopia (27% in those
> 20 years old) Rh e g m a t o g e n o u s re t in a l d e t a c h m e n t (RD)
No sym ptom s or com plications Etio lo gy: retinal break allows liquid vitreous access to
subretinal space (Figs.11-84 and 11-85)
Fin d in gs: yellow-white spots ½–2 DD in size adjacent to ora;
1 in 10,000/year; retinal break can be found in 97%
traverse retinal and choroidal vessels; m ay have irregular black
pigm entation at m argins Most tears (70%) are located superiorly between 10 and 2
o’clock positions
Path o lo gy: degeneration of choroid and retina; loss of outer
retinal layers; RPE absent; firm adhesion between retina and Typ es o f b r eaks:
Bruch’s m em brane / choroid (no predisposition to RD) Horseshoe tear
(Fig. 11-82)

Fig ure 11-82. Pavingstone degeneration. (Courtesy of Dr. Ralph Eagle. From Fig ure 11-83. Lattice degeneration showing the typical white lines. (From
Tasman WS: Peripheral retinal lesions. In: Yanoff M, Duker J S (eds): Tasman WS: Peripheral retinal lesions. In: Yanoff M, Duker J S (eds ):
Ophthalmology. London, 1999, Mosby.) Ophthalmology. London, 1999, Mosby.)

353
CHAPTER 11 • Posterior Segm ent

Vitre ous ge l Vitre ous ge l

Fre e ope rculum

Ante rior fla p

Re tina l te a r
with pe rs is te nt Re tina l hole
vitre ore tina l
tra ction

Re tina Re tina
Fig ure 11-84. Retinal breaks demonstrating horseshoe tear and operculated hole. From Wilkinson CP: Rhegmatogenous retinal ophthalmology. In: Yanoff M, Duker J S
(eds): Ophthalmology. London, 1999, Mosby.)

S ite of a bnorma l S ubre tina l s pa ce Dire ction of liquid After blunt traum a, dialysis is m ost com m on form of tear,
vitre ore tina l pa s s ing into the followed by giant retinal tear, flap tear, and tear around lattice
a dhe s ion s ubre tina l s pa ce degeneration
P re s s ure e xe rte d Liquid vitre ous
by de ta che d pos te rior to
vitre ous ge l vitre ous ge l Sym p to m s: flashes and floaters in 50%

Fin d in gs: retinal break; detached retina is opaque,

corrugated, and undulates; tobacco dust (pigm ent in vitreous);
decreased IOP; VH; nonshifting subretinal fluid
Long-standing RD: thin retina, sm all breaks or dialysis,
dem arcation lines, underlying RPE atrophy, subretinal
precipitates, m acrocysts; m ay have increased IOP, PVR

DDx: exudative or traction RD

Vitre ous ge l

Fig ure 11-85. Classic pathogenesis of rhegmatogenous retinal Tr eatm en t:

detachment. (From Wilkinson CP: Rhegmatogenous retinal ophthalmology. In: Pneumatic retinopexy: ideal if RD caused by single break in
Yanoff M, Duker J S (eds): Ophthalmology. London, 1999, Mosby.) superior 8 clock hours or m ultiple breaks within 1–2
clock hours; phakic patients tend to do better than
Atrophic hole: m yopia, increasing age aphakic patients
Dialysis: splitting of vitreous base (usually Injection of inert gas or sterile air into vitreous cavity; strict
inferotem porally, second m ost com m on site is positioning of patient’s head allows gas bubble to
superonasal); traum atic or idiopathic contact retinal break and form barrier (RPE pum ps
Giant tear: greater than 3 clock hours or 90°; traum a subretinal fluid into choroid, allowing retina to
(! 90%), m yopia; 50% risk of RD in fellow eye reattach), break is sealed with cryo at tim e of gas injection
Operculated hole: fragm ent of retinal tissue found in or sealed with laser once subretinal fluid has resorbed
overlying vitreous INTRAOCULAR GASES (in order of decreasing tim e gas
rem ains in eye): perfluoropropane (C3 F8 ),
Risk facto r s: age, history of RD in fellow eye (15%), high perfluoroethane (C2 F6 ), sulfur hexafluoride (SF6 ), air
m yopia / axial length (7%), fam ily history, lattice degeneration, Surgery: scleral buckle and / or vitrectom y, with laser
traum a, cataract surgery (1% after ICCE; 0.1% after ECCE with therapy or cryotherapy
intact posterior capsule), diabetes, Nd :YAG laser posterior GOALS: Identify all retinal breaks
capsulotom y Close all retinal breaks

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 Disorders

Mus cle
a tta chme nt

S uture s

Fig ure 11-87. Star fold from proliferative vitreoretinopathy. (From Aylward GW:
Fig ure 11-86. Suture placement for both tire and meridional Proliferative vitreoretinopathy. In: Yanoff M, Duker J S (eds): Ophthalmology.
elements. (From Williams GA: Scleral buckling surgery. In: Yanoff M, Duker J S London, 1999, Mosby.)
(eds): Ophthalmology, 2nd edn. St Louis 1999, Mosby.)
Risk o f PVR: following ocular perforation ¼ 43%, following
rupture ¼ 21%, following penetration ¼ 15%; associated with
Place scleral buckle to support all retinal breaks and intraocular foreign body in 11%
bring retina and choroid into contact; retinal tears Most com m on reason for failure of retinal reattachm ent
should be flat on buckle (Fig. 11-86) surgery; usually occurs 4–6 weeks after initial repair
Reduce vitreous traction on retinal breaks
Complications of cryotherapy: proliferative Classificatio n system : 3 grades (A–C) in order of
vitreoretinopathy (PVR), uveitis, CME, intraocular increasing severity
hem orrhage, chorioretinal necrosis
Complications of scleral buckle surgery: ischem ia (anterior
B-scan u ltr aso u n d : detached retina with triangle sign
or posterior segm ents), infection, perforation,
(transvitreal m em brane connecting the 2 sides)
strabism us, erosion or extrusion of explant, change in
refractive error (induced m yopia from increased axial
length), m acular pucker, cataract, glaucom a, new retinal Tr eatm en t: vitreoretinal surgery is successful in
tears (1.6%), PVR (4%), failure (5–10%) approxim ately 70% of cases, leading to anatom ic success
Complications of SRF drainage: hem orrhage, retinal
Exu d a t ive RD
tear, retinal incarceration, hypotony, vitreous loss,
infection Etio lo gy: uveitis (VKH syndrom e, sym pathetic ophthalm ia,
Silicone oil: specific gravity less than water; surface pars planitis, viral retinitis), tum ors, glom erulonephritis,
tension less than that of all gases; buoyant when hypertension, eclam psia / pre-eclam psia, hypothyroidism ,
placed in eye; therefore, m ake iridectom y Coats disease, nanophthalm os, scleritis, CSR (Fig. 11-88)
inferiorly; com plications: cataract (100%), band
keratopathy (24%), glaucom a (19%), corneal Fin d in gs: shifting fluid, sm ooth retinal surface, no retinal
decom pensation (8%) break, retina behind the lens (pathognom onic)

Pr o gn o sis: scleral buckle has 91% success rate; Tra c t io n RD

Final vision depends on m acular involvem ent: worse Etio lo gy: penetrating traum a (PVR), proliferative
prognosis if m acular detached; tim ing of m acular retinopathies, PHPV, toxoplasm osis, vitreous degenerations
detachm ent is also im portant: if < 1 week, 75% recover
vision > 20/70; if > 1 week, 50% > 20/70 Fin d in gs: taut retinal surface, im m obile, concave shape, no
retinal break; does not extend to the ora serrata
Co m p licatio n s: PVR
Path o lo gy: subretinal fluid, photoreceptor degeneration,
P ro life r a t ive vit re o re t in o p a t h y (P VR) cystoid degeneration, glial m em brane form ation
Retinal break allows cells (RPE, glial, m yofibroblasts) to
Fo ld s o f n e u ro s e n s o ry re t in a
proliferate on inner and outer surfaces of retina along scaffold
of detached vitreous (Fig. 11-87) Due to epiretinal m em brane or rhegm atogenous RD
Mem branes contract, causing fixed folds and tractional RD Not visible on FA; can be seen on OCT

355
CHAPTER 11 • Posterior Segm ent

Abnorma l re tina l ve s s e ls
Choroida l tumor with lipid-rich e xuda te
Coa ts
Re tina dis e a s e

Re tina
S ubre tina l fluid

Choroid

Re tina l pigme nt Choroid

e pithe lium (RP E) Choroida l e ffus ion
da ma ge Re tina Choroid
Arte ria l-ve nous
fis tula

Ce ntra l s e rous choriore tinopa thy S cle ritis choroiditis

Re tina
Re tina
Re ve rs a l of
ionic pump

Inflow of fluid RP E
Choroid Choroida l infla mma tion

Fig ure 11-88. Pathologic processes that result in serous detachment of the neural retina. (From Anand R: Serous detachment of the neural retina. In: Yanoff M, Duker J S
(eds): Ophthalmology, 2nd edn. St Louis 1999, Mosby.)

Cho ro id a l Ab no rm a litie s Etio lo gy: hypotony, uveitis, idiopathic, urem ia,

nanophthalmos (thickened sclera im pedes vortex venous
drainage), intraocular surgery (rapid change in IOP shears
Ch o ro id a l fo ld s
choroidal perforating arteries), intense PRP
Results from shrinkage of inner choroid or Bruch’s m em brane,
causing undulations of overlying RPE and outer retina
Hem orrhage is caused by rupture of sm all vessels with sudden
decrease of IOP (opening globe for surgery); m ay be expulsive
with loss of intraocular contents through wound
Etio lo gy: (m nem onic THIN RPE): Tum ors, Hypotony,
Inflam m ation / Idiopathic, Neovascular m em brane,
Retrobulbar m ass, Papilledem a, Extraocular hardware Fin d in gs: dark dom e-shaped appearance (Fig. 11-89)

Fin d in gs: yellow elevated crests alternating with darker B-scan u ltr aso u n d : echogenic convexity attached at scleral
bands between crests spur and vortex veins (not at optic nerve)

FA: light bands / crests contain thinner RPE, producing Tr eatm en t: treat underlying disorder; consider partial-
prom inent choroidal fluorescence; dark bands / troughs thickness scleral windows near vortex vein exit sites in 3 or 4
contain com pressed RPE, producing relative hypofluorescence quadrants; if it occurs during surgery, im m ediately close globe
and give m annitol IV; m ay require drainage (sclerotom ies)

Ch o ro id a l d e t a c h m e n t
Ch o ro id a l is c h e m ia
Due to fluid (choroidal effusion) or blood (choroidal
hem orrhage) in suprachoroidal space May occur with hypertensive retinopathy

356
 Disorders

A B

C
Fig ure 11-89. A–C, Hemorrhagic choroidal detachment viewed ophthalmoscopically. (From Kapusta MA, Lopez PF: Choroidal hemorrage. In: Yanoff M, Duker J S (eds):
Ophthalmology. London, 1999, Mosby.)

Elsch n ig’s sp o t: zone of nonperfusion of choriocapillaris, Tum o rs

which appears as atrophic area of RPE overlying infarcted
choroidal lobule
Be n ig n Tu m o r s

Siegr ist str eak: reactive RPE hyperplasia along sclerosed Ch o ro id a l n e vu s

choroidal vessel
Neoplasm of choroidal m elanocytes; m ost com m on prim ary
intraocular tum or
P o lyp o id a l c h o ro id a l va s c u lo p a t h y (P C V)/
Can also arise within iris, ciliary body, or optic disc
P o s t e rio r u ve a l b le e d in g s yn d ro m e (P UBS )
Usually unilateral and unifocal; increased frequency with age;
Branching inner choroidal vessels external to the prevalence of 6.5% in US population > 49 years old
choriocapillaris with polyp-like term inal dilations; classified as
type 1 CNV; PCV is idiopathic and peripapillary
More com m on in African Am ericans and Asians Mu ltifo cal n evi: associated with neurofibrom atosis
Risk factors include hypertension, sm oking and genetic
Fin d in gs: flat slate-gray to dark brown choroidal lesion
m utations in ARMS2, HTRA1, Y402H, C2, and CFH genes
with ill-defined m argins; surface drusen com m on;
May leak fluid or bleed causing serous and hem orrhagic RPE subretinal fluid and orange clum ps of pigm ent rare
detachm ents (often notched); best seen with ICG angiography (Fig. 11-90)
Treatm ent is with anti-VEGF and/or verteporfin PDT; rates of
com plete polyp closure: aflibercept ¼ PDT+ ranibizum ab
Risk factor s fo r m align an t tr an sfor mation :
> ranibizum ab alone (EVEREST 1 and 2, PLANET studies)
m nem onic TFSOM (To Find Sm all Ocular Melanom a)
Thickness (>2 m m )
Fluid (subretinal)
Mis c e lla n e o u s
Sym ptom s (visual sym ptom s, often flashes or floaters)
Albinism , Aicardi’s syndrom e, color blindness (see Chapter 5, Orange pigm ent (lipofuscin)
Pediatrics/Strabism us) Margin (juxtapapillary location, 3 m m from optic nerve)

357
CHAPTER 11 • Posterior Segm ent

Fig ure 11-91. Hemangioma of choroid. (From Yanoff M, Fine BS: Ocular
Fig ure 11-90. Choroidal nevus showing drusen overlying a heavily pigmented pathology, ed 5, St Louis, 2002, Mosby.)
choroidal nevus composed almost completely of plump polyhedral
nevus cells. (Modified From Naumann G, et al: Arch Ophthalmol 76:784, 1966.
From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.)

Path o lo gy: benign spindle cells with pigm ent

DDx: CHRPE, m elanom a, m elanocytom a

FA: hyperfluorescence

Ch o ro id a l c a ve rn o u s h e m a n g io m a
Congenital, unilateral vascular tum or
A
Typ es:
Diffuse: associated with Sturge-Weber syndrom e
Circumscribed: not associated with system ic disease

Sym p to m s: blurred vision, m icropsia, m etam orphopsia;

vision loss due to cystic m acular retinal degeneration or
adjacent exudative RD

Fin d in gs:
Diffuse type: generalized red-orange choroidal thickening
(“tom ato catsup” fundus); m ay have elevated IOP
Circumscribed type: red-orange dom e-shaped choroidal B
m ass within 2 DD of optic disc or fovea

Path o lo gy: lakes of erythrocytes separated by thin, fibrous

septa; large-caliber vascular channels lined by m ature
endothelium . Circum scribed type has sharply dem arcated
borders and com presses surrounding m elanocytes and
choroidal lam ellae (seen clinically as ring of
hyperpigm entation at periphery of lesion and on FA as ring of
blockage of underlying choroidal fluorescence) (Fig. 11-91)

FA: early hyperfluorescent filling of lesion at sam e tim e as

retinal vessels, and late leakage / staining of entire lesion C
Fig ure 11-92. A–C, Indocyanine green angiography of circumscribed choroidal
ICG: early hyperfluorescent filling of intralesional vessels hemangioma. (From Augsburger J J, Anand R, Sanborn GE: Choroidal
(Fig. 11-92) hemangiomas. In: YanoffM, DukerJ S (eds): Ophthalmology. London, 1999, Mosby.)

358
 Disorders

A
Fig ure 11-93. B-scan ultrasonography of circumscribed choroidal
hemangioma. (From Augsburger J J , Anand R, Sanborn GE: Choroidal
hemangiomas. In: Yanoff M, Duker J S (eds): Ophthalmology. London, 1999,
Mosby.)

Ultr aso u n d : A-scan shows high initial spike with high

internal reflectivity; B-scan shows sonoreflective tissue, often
with choroidal thickening (Fig. 11-93)

Tr eatm en t: laser therapy, photodynam ic therapy, or

low-dose external beam XRT for exudative RD

Ch o ro id a l o s t e o m a
Tum or com posed of bone B
Fig ure 11-94. B-scan ultrasonography of choroidal osteoma. A, At 77 dB the
Sporadic, unifocal, bilateral in 20%
osteoma appears as an intensely white plate in the posterior eye wall. B, At 55 dB
Arises in late childhood to early adulthood; m ore com m on in the lesion persists but most of the normal tissues are no longer evident. (From
Yanoff M, Duker J S (eds): Ophthalmology. London, 1999, Mosby.)
wom en (90%)

Sym p to m s: blurred vision, central scotom a capillary hem angiom as [40–60%], cystic cerebellar
hem angioblastom a [60%, m ost com m on cause of death],
Fin d in gs: yellow-white to pale orange circum papillary renal cell carcinom a, pheochrom ocytom a, liver, pancreas, and
lesion with well-defined pseudopod-like m argins; CNV and epididym is cysts ¼ chrom osom e 3p26-p25; m utation in the
RPE disruption can cause progressive visual im pairm ent VHL tum or suppressor gene)
Arises in older children and young adults
Path o lo gy: plaque of m ature bone involving full-thickness
choroid, usually sparing RPE
Sym p to m s: blurred vision, visual field loss
FA: patchy early hyperfluorescence and late staining of lesion
Fin d in gs: red globular m ass with prom inent dilated
tortuous afferent and efferent retinal blood vessels, exudative
ICG: hypofluorescence of lesion with hyperfluorescent
RD m ay develop
intralesional vessels, late leakage of lesion

B-scan u ltr aso u n d : highly reflective plate-like lesion with FA: feeding arteriole fills rapidly; dye leaks into vitreous and
orbital shadowing beyond lesion (Fig. 11-94) subretinal space

CT scan : plate-like thickening of eye wall Tr eatm en t: slowly progressive if untreated; can treat with
laser, cryotherapy, or photodynam ic therapy (PDT)
Tr eatm en t: for CNV if present
Re t in a l c a ve rn o u s h e m a n g io m a
Re t in a l c a p illa r y h e m a n g io m a
Vascular tum or; probably congenital; occasionally associated
Vascular tum or (hem angioblastom a); AD, sporadic, or with sim ilar CNS or skin vascular lesions
associated with von Hippel-Lindau disease (bilateral retinal Usually unilateral, unifocal; rarely progressive

359
CHAPTER 11 • Posterior Segm ent

Fin d in gs: cluster of dark red intraretinal vascular sacs Co m b in e d h a m a rt o m a o f t h e re t in a a n d RP E

(“bunch of grapes” appearance), typically associated with an
Tum or com posed of neurosensory retina and RPE; probably
anom alous retinal vein; associated overlying retinal gliosis; no
congenital; associated with neurofibrom atosis type 2
intraretinal exudates or detachm ent; m ay have VH
Usually unilateral, unifocal; m inim al progression

FA: fluid levels within the vascular sacs

Sym p to m s: blurred vision

Tr eatm en t: vitrectom y for nonclearing VH Fin d in gs: gray, juxtapapillary lesion with surface gliosis and
prom inent intralesional retinal vascular tortuosity; am blyopia
Re t in a l a s t ro c yt o m a (As t ro c yt ic Ha m a rt o m a ) (Fig. 11-95)

Gliom a arising from retinal glial cells

Path o lo gy: cords of proliferated pigm ent epithelium with
Occurs in older children and young adults; can be unilateral or excess blood vessels and glial tissue
bilateral, unifocal or m ultifocal; usually stable
Rarely associated with tuberous sclerosis (m ultifocal, bilateral) Tr eatm en t: none
and NF
Ad e n o m a a n d a d e n o c a rc in o m a
Sym p to m s: blurred vision, visual field loss, or Tum or com posed of RPE cells, rarely arise from nonpigm ented
asym ptom atic ciliary epithelium ; seldom undergoes m alignant
transform ation
Fin d in gs: 1 or m ore yellow-white m asses that obscure
retinal vessels; occasionally exudative RD; calcification in som e Fin d in gs: elevated dark black lesion, m ay resem ble deeply
larger lesions; m ay have glistening, m ulberry appearance or pigm ented m elanom a; m ay have iridocyclitis, secondary
softer, fluffy appearance cataract, subluxated lens

A B

C D
Fig ure 11-95. A–D, Combined hamartoma of neural retina and retinal pigment epithelium. (C and D, Courtesy Dr. E Howes. From Augsburger J J , Meyers
S: Combined harartoma of retina. In: Yanoff M, Duker J S (eds): Ophthalmology. London, 1999, Mosby.)

360
 Disorders

Path o lo gy: m assive proliferation of RPE cells

Tr eatm en t: excision
Ra c e m o s e h e m a n g io m a
Congenital AV m alform ation
Associated with Wyburn-Mason syndrom e when associated
with AVM of m idbrain
No exudate or leakage

Ma lig n a n t Tu m o rs
Re t in o b la s t o m a (RB) Fig ure 11-97. Callender classification. Spindle A cells, identified by a dark
stripe parallel to the long axis of the nucleus, are seen in longitudinal section.
(See Chapter 5, Pediatrics/Strabism us) They are identified in transverse cross section by the infolding of the nuclear
membrane that causes the dark stripe. (From Yanoff M, Fine BS: Ocular
Ch o ro id a l m a lig n a n t m e la n o m a pathology, ed 5, St Louis, 2002, Mosby.)

Malignant neoplasm of uveal m elanocytes; unilateral, unifocal;

85% of ocular m elanom as arise in choroid, less com m on in CB
(10%) or iris (5%)
Most com m on prim ary intraocular m alignant tum or in
Caucasian adults (1 in 2000)
Uncom m on before age 40 years

Risk facto r s: ocular m elanocytosis, sunlight exposure, uveal

nevi, race (Caucasian; only 1–2% in African Am ericans or
Asians), cigarette sm oking, neurofibrom atosis, dysplastic
nevus syndrom e, bilateral diffuse uveal m elanocytic
proliferation (BDUMP) syndrom e

Sym p to m s: blurred vision, visual field loss, flashes and

floaters
Fig ure 11-98. Callender classification. Histologic section of spindle
Fin d in gs: brown, dom ed-shaped or m ushroom -shaped B cells. (From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis, 2002, Mosby.)
tum or; orange pigm ent clum ps (accum ulation of lipofuscin in
RPE), adjacent exudative RD, sentinel vessel (dilated tortuous
episcleral vessel); secondary glaucom a; m ay be am elanotic and
appear as a pale m ass

Path o lo gy: Callender classification (Figs. 11-96 to 11-99)

Spindle A cells: slender, cigar-shaped nucleus with finely
dispersed chrom atin; low nuclear-to-cytoplasm ic ratio;
absent or inconspicuous nucleolus; no m itotic figures;

Fig ure 11-99. Callender classification. Histologic section of

epithelioid cells. (From Yanoff M, Fine BS: Ocular pathology, ed 5, St Louis,
2002, Mosby.)

tum ors com posed exclusively of spindle A cells are

considered benign nevi
Spindle B cells: oval (larger) nucleus with coarser chrom atin
and prom inent nucleolus; m itotic figures; tum ors with
spindle A and B cells are called spindle cell m elanom as.
Fig ure 11-96. Uveal mushroom melanoma. (From Yanoff M, Fine BS: Ocular Spindle A and B cells grow as a syncytium with indistinct
pathology, ed 5, St Louis, 2002, Mosby.) cytoplasm ic borders

361
CHAPTER 11 • Posterior Segm ent

Epithelioid cells: polyhedral with abundant cytoplasm ;

poorly cohesive; distinct borders; m ost m alignant; large,
round-to-oval nucleus with peripheral m argination of
chrom atin; prom inent eosinophilic or purple nucleolus
(epithelioid cells “look back at you”); worst prognosis
Mixed cell melanoma: com posed of spindle and
epithelioid cells
Gross exam ination requires identification of vortex veins to
rule out tum or extension
Tr an sillu m in atio n : m ost m elanom as cast shadow
Ultr aso u n d : A-scan shows low internal reflectivity with
characteristic reduction in am plitude from front to back, high-
am plitude spikes consistent with break in Bruch’s m em brane;
B-scan shows solid tum or with m ushroom shape or biconvex
shape (Fig. 11-100)
FA: “double” circulation (choroidal and retinal vessels), m ass
is hypofluorescent early, pinpoint leakage late (Fig. 11-101)
A
OCT: subretinal fluid
FAF: orange pigm ent (lipofuscin)
MRI: bright on T1; dark on T2

Tr eatm en t:
Enucleation: large tum or, optic nerve invasion, painful eye
with poor visual potential

B
Fig ure 11-101. A, B, Fluores cein angiograms of mushroom-shaped choroidal
melanoma. (From Augsburger J J , Damato BE, Bornfield N: Uveal melanoma. In:
A Yanoff M, Duker J S (eds): Ophthalmology. London, 1999, Mosby.)

Plaque brachytherapy: m ost eyes with m edium -sized

tum ors with visual potential, or failure of laser
HIGH ENERGY: cobalt-60, iridium -192
LOW ENERGY: iodine-125 (sphere of radiation),
palladium -106 (25% less energy than I-125),
ruthenium -106 (β-particles, travel only 5 m m ;
therefore, use for sm all tum ors)
Charged particle radiation: proton beam (cylinder of
radiation); large anterior segm ent dose, low m acular
dose (reverse of plaque)
Resection: m ost irido and iridociliary tum ors; som e ciliary
body and choroidal tum ors
Transpupillary thermotherapy: sm all tum ors
B Zimmerman hypothesis: m anipulation of tum or during
Fig ure 11-100. A, B, Ultras onography of posterior uveal melanoma. (From enucleation causes m etastasis and increases m ortality;
Augsburger J J , Damato BE, Bornfield N: Uveal melanoma. In: Yanoff M, Duker J S not true. Derived from plotting of life table data,
(eds): Ophthalmology. London, 1999, Mosby.) which showed Bell-shaped curve for death rate;

362
 Disorders

specifically, m ortality rate increased to 8% following

enucleation (vs 1% per year in patients with untreated MAJ OR CLINICAL STUDY
tum ors)
Complications of treatment: radiation retinopathy (if
severe, treat with PRP to prevent NVG); cataracts
Co lla b o ra tive Oc ula r Me la no m a
(PSC in 42% within 3 years of proton beam therapy); Stud y (COMS)
dry eye
Collaborative Ocular Melanoma Study (COMS): Ob je c t ive : To Evaluate Treatm ent Options For
SMALL TUMOR TRIAL (apical height ¼ 1.5–2.4 m m and Choroidal Malignant Melanom as
basal diam eter ¼ 5–16 m m ): ⅓ grew; 5-year tum or
m ortality¼ 1%
Me t h o d s :
MEDIUM TUMOR TRIAL (apical height ¼ 2.5–10 m m and
basal diam eter 16 m m ): survival rates sim ilar Small tumor study (not large enough for COMS trial)
between enucleation and iodine-125 plaque (apical height ¼ 1.5–2.4 m m and basal diam eter 5–
brachytherapy; I-125 brachytherapy has a high risk of 16 m m ): not random ized; observation vs treatm ent at
substantial visual loss (up to 49%); 5-year tum or discretion of investigator
m ortality 10% Medium tumor trial (apical height ¼ 2.5–10 m m
LARGE TUMOR TRIAL (apical height > 10 m m and basal and basal diam eter 16 m m ): random ized to
diam eter > 16 m m ; without m ets): pre-enucleation enucleation vs radiation (I-125 plaque
radiation does not change the survival rate in patients brachytherapy)
with large choroidal m elanom as with or without Large tumor trial (apical height > 10 m m and basal
m etastases over enucleation alone; 5-year tum or diam eter > 16 m m ; no m ets): random ized
m ortality 27% enucleation alone vs 20 Gy external beam radiation
Updated definition of large tum or is: apical height for 5 days and then enucleation (PERT)
> 2 m m and basal diam eter > 16 m m , or apical Primary outcom e was survival; secondary outcom es
height > 10 m m regardless of basal diam eter, or included tum or growth, visual function, tim e to
apical height > 8 m m and less than 2 m m from m etastasis, and quality of life
optic disc
Re s u lt s :
Pr o gn o sis:
Factors: Small tumor study: 21% grew by 2 years and 31% by
CELL TYPE: epithelioid has worst prognosis (5-year
5 years; 6 deaths were due to m etastatic m elanom a;
survival < 30%); presence of epithelioid cells is 5-year all-cause m ortality was 6%, and 8-year was
14.9%; risk factors for growth included initial tum or
im portant in survivability; 5-year survival rate for
spindle A cell is 95%, 15-year survival rate for m ixed thickness and diam eter, presence of orange pigm ent,
tum or is 37%, 15-year survival for pure spindle cell absence of drusen, and absence of adjacent RPE
m elanoma with no epithelioid cells is 72% changes
SIZE: larger and diffuse tum ors have a worse prognosis
Medium tumor trial: after 3 years, 43–49% of I-125–
LOCATION
treated eyes had poor vision outcom es (loss of ! 6
VARIABILITY IN NUCLEOLAR SIZE
lin es of visual acuity or VA of 20/200 or worse),
and this was strongly associated with larger tum or
PRESENCE OF INTRATUMOR VASCULAR NETWORKS
PRESENCE OF NECROSIS
apical height and sm aller distance from the foveal
METALLOTHIONEIN LEVELS: m ay indicate m etastatic
avascular zone; other risk factors for poor visual
outcom e included diabetes, tum or-related retinal
potential and poor survival
GENETICS: m onosom y 3 associated with higher death
detachm ent, and tum ors that were not dom e-
rates; gene expression profiling can also predict shaped; estim ated 5-year survival rates were 81%
m elanoma-related m ortality for the enucleation group and 82% for the I-
Approxim ately 50% with large tum ors have m etastasis 125 group
within 5 years Large tumor trial: 1003 patients enrolled. Estim ated
Mean survival after m etastasis is 9 m onths 5-year survival rates were 57% for enucleation alone
and 62% for radiation followed by enucleation, and
for patients with m etastases at the tim e of death, the
Metastasis: hem atogenous spread via vortex veins; risks 5-year survival was 72% vs 74%; prognostic risk
include elevation (> 2 m m ), proxim ity to optic nerve, factors were age and basal diam eter
visual sym ptom s, growth; 92% of m etastases are to liver, also
skin, lungs, other organs; local extension into optic nerve Co n c lu s io n s :
and brain
Metastatic workup: abdom inal exam ination Small tumor study: otherwise healthy patients with sm all
(hepatosplenom egaly), liver function tests (LFTs), CXR; choroidal m elanom as have a low risk of death within
2% have evidence of m etastasis at diagnosis; if any 5 years
abnorm al then liver / abdom en / chest CT and MRI

363
CHAPTER 11 • Posterior Segm ent

Medium tumor trial: there is a high risk of substantial

visual loss from I-125 brachytherapy (up to 49%);
I-125 brachytherapy does not change the survival rate
(for up to 12 years) in patients with m edium
choroidal m elanom as
Large tumor trial: pre-enucleation radiation does not
change the survival rate in patients with large
choroidal m elanom as with or without m etastases

P rim a r y in t ra o c u la r lym p h o m a
(Re t ic u lu m c e ll s a rc o m a )
Non-Hodgkin’s B-cell lym phom a, large cell type
Fig ure 11-103. Large neoplastic cells in a vitreous specimen. (From Burnier MN,
Affects older (m ean age 70) and im m unocom prom ised Blanco G: Intraocular lymphoma. In: Yanoff M, Duker J S (eds): Ophthalmology.
patients; usually bilateral and m ultifocal London, 1999, Mosby.)

No system ic involvem ent outside of CNS

FA: leopard spots
Increased risk of developm ent of concurrent prim ary CNS
lym phom a
Tr eatm en t: chem otherapy or XRT
Sym p to m s (precede CNS signs in 80%): blurred vision,
floaters; dem entia m ay occur Pr o gn o sis: poor, m ortality often within 2 years (m ean
survival is 22 m onths)
Fin d in gs: cream y white, diffuse sub-RPE and vitreous
infiltrates; AC reaction, hypopyon, vitritis, secondary
glaucom a; m ay have m ultifocal atrophic or punched-out Re a c t ive lym p h o id h yp e r p la s ia o f t h e c h o ro id
lesions, and exudative RD; choroidal form m anifests with
benign counterpart
choroidal nodules or detachm ents of RPE and is associated
with system ic lym phom a (Fig. 11-102)
Fin d in gs: diffuse or m ultiple yellow choroidal lesions;
Diagn o sis: MRI, LP (positive in 25%), vitreous biopsy (low overlying RD m ay occur; often sim ilar infiltrates in conjunctiva
yield; atypical lym phocytes with prom inent nucleoli, m itoses; and orbit (check conjunctiva for salm on patch)
cellular necrosis) (Fig. 11-103)
Associated with system ic lym phom a (vs retinovitreous
lym phom a, which is associated with CNS lesions)

Path o lo gy: m ass of benign lym phocytes, plasm a cells, and

Dutcher bodies

Tr eatm en t: low-dose XRT

In t ra o c u la r le u ke m ia
(See Chapter 5, Pediatrics/Strabism us)

Me t a s t a s e s
Most com m on intraocular m alignancy (including cases
evident only at autopsy)
Most com m only breast carcinom a in wom en (m ost have
previous m astectom y), lung carcinom a in m en (often occult)
Bilateral and m ultifocal in 20%; 25% have no previous history
of cancer
Fig ure 11-102. Fundus view of yellow-white hemorrhagic retinal
infiltrates. (From Burnier MN, Blanco G: Intraocular lymphoma. In: Yanoff M, 93% to choroid; 2% to ciliary body; 5% to iris; can also
Duker J S (eds): Ophthalmology. London, 1999, Mosby.) m etastasize to optic nerve; retina is rare

364
 Laser Treatm ent

Sym p to m s: blurred vision, visual field defects, flashes and Ca rc in o m a -a s s o c ia t e d re t in o p a t h y (CAR)

floaters
Rare, paraneoplastic syndrom e (m ainly sm all cell lung
carcinom a, breast cancer, and gynecologic cancer; also reported
Fin d in gs: 1 or m ore golden-yellow, thin, dom e-shaped in other types of lung cancer, colon cancer, m ixed Müllerian
choroidal tum ors or cream y yellow am elanotic tum ors with tum or, skin squam ous cancer, kidney cancer, pancreatic,
placoid or num m ular configuration; m ay produce extensive lym phom a, basal cell tum or, and prostate cancer); loss of
exudative RD; usually m etastasize to m acula (richest blood photoreceptors without inflam m ation
supply)

Fin d in gs: severe decreased vision, visual field defects,

DDx o f am elan o tic ch o r o id al m ass: am elanotic nyctalopia, APD if asym m etric (but usually bilateral) that
m elanom a, old subretinal hem orrhage, choroidal osteom a, clinically appears norm al in early cases, but m ay later develop
granulom a, posterior scleritis uveitis, chorioretinal atrophy, retinal pigm ent degeneration,
narrowed retinal vessels, optic atrophy, and vitreous cells;
FA: early hyperfluorescence with late staining of lesions antibodies to retinal proteins including recoverin, carbonic
anhydrase II, transducin B, α-enolase, TULP1 [Tubby-like
Protein 1], PNR photoreceptor cell-specific nuclear receptor,
Ultr aso u n d : A-scan shows m edium to high internal heat shock cognate protein HSC 70, arrestin, glyceraldehyde
reflectivity; B-scan is sonoreflective (Fig. 11-104)
3-phosphate dehydrogenase have been described

Tr eatm en t: chem otherapy, external beam XRT, or

com bination ERG: extinguished

FAF: parafoveal ring of enhanced autofluorescence with

norm al autofluorescence within the ring and
hypoautofluorescence outside the ring

Tr eatm en t: system atic im m unosuppression (high-dose

steroid with m ethylprednisolone and prednisone, cyclosporin,
azathioprine, alem tuzum ab), intravenous im m unoglobulin,
plasm apheresis, and com bination of these treatm ents have
been tried; find and treat prim ary cancer

Pr o gn o sis: poor

Bila t e ra l d iffu s e u ve a l m e la n o c yt ic p ro life ra t io n

s yn d ro m e (BDUMP S )
Paraneoplastic syndrom e (ovarian, uterine, lung cancer m ost
A com m on)
Diffuse uveal thickening

Fin d in gs: orange or pigm ented fundus spots, iris thickening

and pigm entation

ERG: m arkedly reduced

FA: orange spots hyperfluoresce

Tr eatm en t: none

B LASER TREATMENT
Fig ure 11-104. Ultrasonography of metastatic carcinoma to choroid. A, A-scan.
B, B-scan. (From Augsburger J J , Guthoff R: Metastatic cancer to the eye. In: Therm al burn (photocoagulation) of inner retina will occur
Yanoff M, Duker J S (eds): Ophthalmology. London, 1999, Mosby.) with argon blue-green or xenon arc

365
CHAPTER 11 • Posterior Segm ent

Retin al str u ctu r es th at ab so r b laser : Fo c a l la s e r

Melanin: prim ary site of light absorption and heat
In d icatio n : treatm ent of m acular edem a (CSME, BVO, JXT)
em ission; absorbs argon blue-green, argon green,
and krypton red
Hemoglobin: absorbs argon blue-green, argon green; Par am eter s: 50–100 µm spot size, 0.1 s duration; burns that
does not absorb krypton (used to penetrate hem orrhage) slightly blanch the RPE, should be barely visible im m ediately
Xanthophyll: absorbs blue wavelengths after treatm ent, grid pattern 1 burn-width apart, in areas of
diffuse edem a; focal treatm ent of leaking vessels and
m icroaneurysm s; whiten / darken m icroaneurysm s and / or
Wavelen gth s: cause m ild depigm entation of RPE; do not treat over
Blue-green (488 and 514 nm ): blue light is absorbed by hem orrhage; identify leaking m icroaneurysm s by FA;
cornea and lens, heavily absorbed by nuclear sclerotic subthreshold m icropulse laser with 810nm divides laser pulse
cataracts and absorbed by xanthophyll pigm ent into short, repetitive pulses that persist for 0.1 seconds to 0.5
Green (514 nm ): absorbed by blood, m elanin; preferred seconds (“on” tim e is duration of each m icropulse (typically
for treatm ent of CNV in cases in which RPE has little 100µs to 300µs) and “off” tim e (1,700µs to 1,900µs) is interval
pigm ent between successive m icropulses) has also been used
Yellow (577 nm ): m ay be better for treatm ent of successfully
m icroaneurysm s; be careful of blood vessels
Decreases risk of m oderate visual loss over 3 years by 50%
Red (647 nm ): penetrates cataracts better; not absorbed by
in CSME; DRCR Protocol I 5-year results suggest that focal
blood; causes less inner retinal dam age; absorbed by
laser treatm ent at initiation of intravitreal ranibizum ab
m elanin; poorly absorbed by xanthophyll (safer near
injections is no better than deferring laser treatm ent for ! 24
fovea); passes through m ild vitreous hem orrhage and
weeks in eyes with center involving DME with vision
nuclear sclerotic cataracts better than argon
im pairm ent; thus, focal laser is second line therapy for center
Infrared (810 nm ): has sim ilar characteristics as red, but
involving edem a
with deeper penetration
Xenon arc: polychrom atic white light; not as precisely Retreat at 3 m onths if CSME still present, or treatable areas seen
focused as m onochrom atic light; em its considerable on repeat FA
am ount of blue light, which can be harm ful to retina
and lens
Nd:YAG (532 nm ): pattern scanning laser (PASCAL);
causes less dam age REVIEW QUESTIONS (Answers start on page 391)

1. Which substance does not cause crystalline deposits in the

P a n re t in a l p h o t o c o a g u la t io n (P RP ) retina?
a. thioridazine
In d icatio n s: treatment of NV and peripheral nonperfusion, b. canthaxanthine
most com monly in retinal vascular diseases (PDR, CVO, BVO, c. tam oxifen
sickle cell, radiation, and hypertensive retinopathy); DRCR d. talc
Protocol S reported that intravitreal ranibizumab injections for 2. The finding m ost predictive of visual acuity in a patient
PDR was associated with superior vision over the course of 2 with PDR is
years, reduced the incidence of center involving DME (9% versus a. m icroaneurysm s
28%), less peripheral visual field loss, fewer vitrectom ies (4% b. m acular edem a
versus 15%), and no major ocular or systemic safety differences c. cotton wool spots
except for one case of endophthalm itis versus prompt PRP d. intraretinal m icrovascular abnorm alities
3. The MPS showed the best prognosis for laser treatm ent of
Par am eter s: 500 µm spot size, 0.1-0.5 s duration, CNV in which disorder?
1200–2000 spots; moderate white burn, 1 burn-width apart, a. AMD
2 DD from fovea and 1 DD from optic nerve; 2 or m ore sessions b. m yopia
c. idiopathic
Ad ver se effects: decreased acuity (worsened by 1 line in d. POHS
11%; worsened by 2 lines in 3%), constricted visual field, loss 4. Which of the following is not a feature of Stickler’s syndrome?
of color vision in 10% a. Pierre-Robin m alform ation
Retreat at 4 weeks if NVD fails to regress or develops, new NVE b. autosom al dom inant inheritance
develops, iris or angle NV develops, further regression of NV c. retinoschisis
desired d. m arfinoid habitus
5. Sites at which the uvea is attached to the sclera include all
Co m p licatio n s: choroidal effusion, exudative RD, of the following except
perm anent m ydriasis and im pairm ent of accom m odation a. vortex veins
(ciliary nerve dam age), vitreous hem orrhage, lens or corneal b. optic nerve
dam age, foveal or optic nerve dam age, CME, CNV, retinal c. scleral spur
vascular occlusion, angle closure d. ora serrata

366
 Review Questions

6. A patient with m ultifocal choroiditis and retinal vasculitis c. decreased cGMP, closed Na channel
is m ost likely to have d. decreased cGMP, open Na channel
a. Vogt-Koyanagi-Harada disease 16. Prognostic factors for choroidal m elanom a include all of
b. tuberculosis the following except
c. cat-scratch disease sarcoidosis a. size
7. All 3 types of retinal hem orrhage (preretinal, intraretinal, b. location
and subretinal) m ay occur sim ultaneously in all of the c. cell type
following conditions except d. pigm entation
a. m acroaneurysm 17. Which of the following does not involve the outer
b. AMD plexiform layer of the retina?
c. diabetes a. degenerative retinoschisis
d. capillary hem angiom a b. cystoid m acular edem a
8. Characteristics of choroidal m elanom a include all of the c. Henle’s fiber layer
following except d. diabetic m icroaneurysm
a. choroidal excavation 18. The best test for distinguishing between a subretinal
b. high internal reflectivity hem orrhage and a choroidal m elanom a is
c. double circulation a. FA
d. orbital shadowing b. B-scan
9. Which of the following statem ents regarding the ETDRS c. A-scan
is false? d. ERG
a. The ETDRS concluded that PRP reduces the risk of 19. Which of the following is the least radiosensitive lesion?
severe visual loss in patients with high-risk PDR a. m etastases
b. The ETDRS identified the risk factors for developm ent b. lym phom a
of PDR c. RB
c. The ETDRS defined CSME d. m elanom a
d. The ETDRS showed that aspirin does not affect disease 20. Which of the following is the least com m on com plication
progression of PRP?
10. The DRVS found that early vitrectom y for vitreous a. iritis
hem orrhage was helpful in b. increased IOP
a. type 1 diabetic patients c. retinal detachm ent
b. type 2 diabetic patients d. narrow angle
c. type 1 and 2 diabetic patients 21. A cluster of pigmented lesions is seen in the peripheral
d. none of the above retina of a patient’s left eye during routine ophthalm oscopy.
11. Which of the following treatm ents is a CVOS Which of the following tests would be most helpful in
recom m endation? detecting an associated hereditary disorder?
a. focal laser for m acular edem a of > 3 to 6 m onths’ a. EKG
duration with VA < 20/40 b. colonoscopy
b. PRP for > 10 disc areas of nonperfusion c. brain MRI
c. PRP for iris or angle neovascularization d. chest CT scan
d. m acular grid photocoagulation for CME 22. Which of the following is not associated with a typical
12. Which peripheral retinal lesion has the greatest risk of a angiographic appearance of CME?
retinal detachm ent? a. nicotinic acid
a. cystic retinal tuft b. epinephrine
b. aym ptom atic retinal hole c. Irvine-Gass syndrom e
c. senile retinoschisis d. pars planitis
d. lattice degeneration 23. The least likely finding in a patient with a chronic
13. The intraocular structure m ost com m only affected by detachm ent of the inferotem poral retina is
leukem ia is a. retinal thinning
a. iris b. m acrocysts
b. choroid c. fixed folds
c. retina d. RPE atrophy
d. optic nerve 24. In a patient with AMD, which type of drusen is m ost
14. Which is not a function of the RPE? associated with developm ent of CNV?
a. conversion of vitam in A alcohol to aldehyde a. soft
b. concentration of taurine b. basal lam inar
c. inactivation of toxic products of oxygen c. hard
m etabolism d. calcific
d. phagocytosis of rod outer segm ents 25. The ERG oscillatory potential is caused by which cell type?
15. Which of the following best describes the cellular reaction a. Mu€ ller’s
when light strikes a photoreceptor? b. am acrine
a. increased cGMP, closed Na channel c. bipolar
b. increased cGMP, open Na channel d. RPE

367
CHAPTER 11 • Posterior Segm ent

26. Chloroquine retinopathy 36. Focal laser treatm ent is indicated for diabetic m acular
a. is reversible with discontinuation of the m edication edem a when there is
b. can be associated with other CNS reactions a. hard exudates with retinal thickening 1000 µm from
c. is best diagnosed with red-free photographs the center of the fovea
d. is m ore likely with higher cum ulative doses, rather than b. neovascularization elsewhere greater than ⅓ disc area
higher daily doses in size
27. Proven system ic control for diabetic retinopathy includes c. ischem ia within 250 µm of the center of the fovea
a. lowering serum cholesterol d. retinal edem a within 500 µm of the center of
b. aspirin use the fovea
c. ACE inhibitors 37. Reduced IOP would be m ost unexpected in a patient with
d. blood pressure control a. choroidal effusion
28. Retinal artery m acroaneursysm s b. choroidal hem orrhage
a. are usually located in the m acula c. serous retinal detachm ent
b. produce m ultilayered hem orrhages d. rhegm atogenous retinal detachm ent
c. are best seen on ICG angiography 38. Which of the following is the correct indication for
d. should be treated with focal laser photocoagulation to treating m acular edem a from a branch retinal vein
prevent bleeding occlusion?
29. The Branch Vein Occlusion Study reported that a. ! 5 DD of capillary nonperfusion
a. aspirin prevented recurrent episodes b. thickening within 500 µm of the fovea
b. focal laser photocoagulation decreased visual loss c. exudates in the fovea
c. panretinal photocoagulation should be applied in d. vision worse than 20/40 for greater than 3 m onths
ischem ic patients 39. Which test is best for distinguishing a choroidal
d. photocoagulation should be delayed in the presence of m elanom a from a choroidal hem angiom a?
extensive m acular ischem ia a. transillum ination
30. True statem ents about ocular photodynam ic therapy b. red free photo
include all of the following except c. ultrasound
a. requires ligh t precautions for 48 hours after d. CT scan
treatm en t 40. The etiology of vitreous hemorrhage in Terson’s syndrome is
b. is indicated for occult with no classic CNV lesions a. intracranial hypertension
c. often requires retreatm ent every 6 weeks b. hypercoagulability
d. stabilizes but rarely im proves vision c. em bolus
31. Cystoid m acular edem a d. neovascularization
a. does not leak fluorescein when associated with 41. The m ost com m on site of m etastasis for a choroidal
nicotinic acid m elanom a is
b. responds to nonsteroidal anti-inflam m atory a. brain
drops b. liver
c. is associated with beta-blockers c. lungs
d. has a petalloid appearance on fluorescein d. skin
angiography 42. Which of the following is least characteristic of Eales’
32. PDR is m ost likely to develop if which one of the following disease?
findings is present on fundus exam ? a. vascular sheathing
a. exudates b. vitreous hem orrhage
b. hem orrhages c. neovascularization
c. m icroaneurysm s d. m acular edem a
d. venous beading 43. Crystalline retinopathy is not associated with
33. Which of the following is the biggest risk factor a. tam oxifen
for AMD? b. m ethoxyflurane
a. sm oking c. thioridazine
b. elevated serum cholesterol level d. canthaxanthine
c. dark iris color 44. Com bined ham artom a of the retina and RPE has been
d. m yopia associated with all of the following except
34. The m ost im portant visual prognostic factor for a a. neurofibrom atosis
rhegm atogenous RD is b. tuberous sclerosis
a. size of the break c. Gorlin syndrom e
b. extent of the detachm ent d. Gardner syndrom e
c. m acular involvem ent 45. All of the following statem ents regarding uveal m etastases
d. type of surgery are true except
35. The earliest sign of a m acular hole is a. m ost com m on prim ary sites are breast and lung
a. RPE atrophy in the fovea b. m ost com m on ocular location is anterior choroid
b. vitreous detachm ent at the fovea c. often have an exudative RD
c. yellow spot in the fovea d. fluorescein angiography usually dem onstrates early
d. partial-thickness eccentric hole in the fovea hyperfluorescence

368
 Suggested Readings

46. The m ost worrisom e sign in a patient with an acute

posterior vitreous detachm ent is SUGGESTED READINGS
a. pigm ent in the vitreous
b. m ultiple vitreous floaters Agarwal, A. (2012). Gass’ Atlas of Macular Diseases (5th ed.).
c. lattice degeneration Philadelphia: Saunders.
d. Weiss ring Basic and Clinical Sciences Course. (2016). Section 12: Retina and
47. The m ost likely diagnosis in a patient with choroidal Vitreous. San Francisco: AAO.
neovascular m em brane in the m acula, peripapillary Byrne, S. F., & Green, R. L. (1992). Ultrasound of the Eye and Orbit (2nd
ed.). St Louis: Mosby.
atrophy and punched-out chorioretinal scars is
Freund, K. B., Sarraf, D., & Mieler, W. F. (2016). The retinal atlas
a. toxocariasis (2nd ed.). Philadelphia: Saunders.
b. toxoplasm osis Guyer, D. R., Yannuzzi, L. A., Chang, A., et al. (1999). Retina-Vitreous-
c. presum ed ocular histoplasm osis syndrom e Macula. Philadelphia: WB Saunders.
d. syphilis Ryan, S. J., & Schachat, A. P. (2012). Retina (5th ed.). Philadelphia:
48. The m ost com m on com plication of an epiretinal Saunders.
m em brane is Schum an, J. S., Puliafito, C. A., Fujim oto, J. G., & Duker, J. S. (2012).
a. m acular hole Optical Coherence Tomography of Ocular Diseases (3rd ed). Thorofare,
b. cystoid m acular edem a NJ: SLACK.
c. retinal detachm ent Shields, J. A., & Shields, C. L. (2007). Intraocular tumors: A text and atlas
(2nd ed.). Philadelphia: Lippincott William s & Wilkins.
d. choroidal neovascular m em brane
Wilkinson, C. P., & Rice, T. A. (1997). Michels’s retinal detachment
49. The m ost helpful test to obtain in a patient with a branch (2nd ed.). St Louis: Mosby.
retinal artery occlusion is
a. EKG
b. ERG
c. OCT
d. carotid ultrasound
50. Angioid streaks are associated with all of the following
system ic conditions except
a. Ehlers-Danlos syndrom e
b. Paget’s disease
c. Syphilis
d. Pseudoxanthom a elasticum

369
This pa ge inte ntiona lly le ft bla nk
 Answers to Questions

CHAPTER 1 O PTICS d. 27 m m —20/60 letter subtends 5 m inutes at 60 ft

(18 m ); therefore, size of letter ¼ 18 (tan 5 0) ¼ 18
1. Prince’s rule is helpful in determ ining all of the following (0.0015) ¼ 0.027 m or 27 m m
except 9. A Galilean telescope with a +5 D objective and a À20 D
d. accom m odative convergence eyepiece produces an im age with what m agnification
2. A m yope who pushes his spectacles closer to his face and and direction?
tilts them is a. 4 Â, erect—m agnification ¼ À(À 20)/5 ¼ +4, m eaning
d. increasing effectivity, increasing cylinder 4 Â m agnification and erect im age

3. The Prentice position refers to 10. An object is placed 33 cm in front of an eye. The im age
a. glass prism perpendicular to visual axis form ed by reflection from the front surface of the cornea
(radius of curvature equals 8 m m ) is located
4. The purpose of Q-switching a laser is to b. 4 m m behind cornea—reflecting power of the cornea
b. decrease energy, increase power is À2/0.008 ¼ À 250 D; thus, the focal point is
5. A 50-year-old wom an with aphakic glasses wants a new 1/À250 ¼ À 4 m m , or 4 m m behind the cornea
pair of spectacles to use when applying m ake-up. How 11. A convex m irror produces what type of im age?
m uch power should be added to her distance correction d. virtual, erect, m inified—rem em ber m nem onic VErMin
so that she can focus while sitting 50 cm in front of her
m irror? 12. In general, the m ost bothersom e problem associated with
c. +1.00 D—with a plane m irror, the im age is located as bifocals is
far behind the m irror as the object is in front of the a. im age jum p—therefore, choose bifocal segm ent type to
m irror; therefore, the distance between her eye and m inim ize jum p; flat-top segm ents m inim ize im age
the im age of her eye is 100 cm ¼ 1 m , and this jum p because the optical center is near the top; this
requires +1.00 D of extra m agnification also reduces im age displacem ent in m yopes

6. How far from a plano m irror m ust a 6-ft-tall m an stand to 13. A refraction with a stenopeic slit gives the following
see his whole body? m easurem ents: +1.00 at 90 degrees and À2.00 at 180
b. 3 ft—to view one’s entire body, a plane m irror need be degrees. The corresponding spectacle prescription is
only half one’s height c. À2.00 + 3.00 Â 180—this is the correct spherocylindrical
notation for a power cross diagram with +1.00 along the
7. A 33-year-old wom an with a refraction of À9.00 90 degrees m eridian and À2.00 along the 180 degree
+ 3.00 Â 90 OD at vertex distance 10 m m and m eridian
keratom etry readings of 46.00@90/43.00@180 is fit for
a rigid gas-perm eable (RGP) contact lens 1 D steeper 14. A point source of light is placed 1/3 of a m eter to the left of
than flattest K. What power lens is required? a +7 D lens. Where will its im age com e to focus?
c. À7.00 D—step 1: change refraction to m inus cylinder a. 25 cm to the right of the lens—the light has vergence of
form (because the tear lake negates the m inus À3 D and encounters a +7 D lens; thus, the exiting light
cylinder) and use this sphere (À6.00); step 2: adjust to has vergence of +4 D, and the im age will com e to focus
zero vertex distance (À6 + 0.01[À6]2 ¼ À 5.64); step 3: 25 cm to the right of the lens
adjust for tear lens (SAM-FAP rule) (add
15. What is the equivalent sphere of the following cross
À1.00 ¼ À 6.64)
cylinder: À3.00 Â 180 com bined with +0.50 Â 90?
8. What is the size of a 20/60 letter on a standard b. À1.25—the cross cylinder has a spherocylindrical
20-ft Snellen ch art (tangent of 1 m in ute of notation of À3.00 + 3.50 Â 90; so the spherical
arc ¼ 0.0003)? equivalent is À3.00 + (3.50/2) ¼ À 1.25

371
Answers to Questions

16. What is the size of a letter on a standard 20-ft Snellen chart 27. What is the appropriate correction in the intra-ocular lens
if it form s an im age of 0.5 m m on a patient’s retina? (IOL) power if the A constant for the lens to be im planted
d. 18 cm —using the reduced schem atic eye and sim ilar is changed from 117 to 118?
triangles, 0.5/17 m m ¼ object size/6 m ; solving for b. increase IOL power by 1.0 D—the change in A constant
object size yields 18 cm is equivalent to the change in IOL power
17. The im age of a distant object is largest in which patient? 28. An intra-ocular lens (IOL) labeled with a power of +20 D
b. hyperope with spectacles has a refractive index of 1.5. If this lens were rem oved from
the package and m easured with a lensom eter, what power
18. What type of im age is produced if an object is placed in
would be found?
front of a convex lens within its focal length?
d. +59 D—using the form ula for calculating the power of
b. erect and virtual
a thin lens im m ersed in fluid (D air/D aqueous) ¼
19. What is the correct glasses prescription if retinoscopy (n iol À n air)/(n iol À n aqueous), D air ¼ 20 ([1.5 À 1.0]/
perform ed at 50 cm shows neutralization with a plano lens? [1.5 À 1.33]) ¼ +58.8 D
a. À2.00—rem em ber to subtract for working distance
29. The spherical equivalent of a 0.50 D cross cylinder is
(1/0.5 m ¼ 2 D); thus, a plano lens m inus 2 D yields
a. plano—by definition, all cross cylinders have a
a À2.00 D glasses prescription
spherical equivalent of zero; this is evident by writing
20. An anisom etropic patient experiences difficulty while the cross cylinder in spherocylindrical notation
reading with bifocals. Which of the following is not exam ple: +0.50 À 1.00 Â 90
helpful for reducing the induced phoria? 30. To m inim ize im age displacem ent in a hyperope, the best
c. progressive lenses—this problem is due to the prism atic type of bifocal segm ent style is
effect of the underlying lens and will occur with all c. round top—to m inim ize im age displacem ent, the
bifocal styles, including progressive lenses prism atic effects of the bifocal segm ent and the
21. A Geneva lens clock is used to m easure what? distance lens should be in opposite directions; a
d. base curve round top acts like a base-down prism and the
underlying hyperopic lens acts like a base-up prism
22. What is the induced prism when a 67-year-old wom an
reads 10 m m below the upper segm ent optical center of 31. The logMAR equivalent to 20/40 Snellen acuity is
her bifocals, which m easure +2.50 + 1.00 Â 90 OD and b. 0.3
À1.50 + 1.50 Â 180 OS add +2.50 OU? 32. A patient who is pseudophakic in one eye and phakic in
c. 2.5 prism diopters—using Prentice’s rule, the induced the other eye will have what am ount of aniseikonia?
prism is (+2.50 Â 1) OD and ([À1.50 + 1.50] Â 1) OS b. 2.5%
(cylinder power acts in the vertical m eridian, so it
m ust be used in the calculation); the total is +2.5 PD 33. A patient with 20/80 vision is seen for a low vision
BU OD; the effect is due to the underlying lens; the evaluation. What add power should be prescribed so the
power of the bifocal segm ent can be ignored because patient does not have to use accom m odation to read the
it is the sam e for both lenses (5 PD BU OD and 2.5 newspaper?
PD BU OS¼ 2.5 PD BU OD) b. +4—the add power is calculated from the inverse of the
distance Snellen acuity: 80/20 ¼ 4
23. The optim al size of a pinhole for m easuring pinhole visual
acuity is approxim ately 34. The spherical equivalent of a À2.00 + 1.50 Â 90 lens is
c. 1.25 m m —lim ited by diffraction if sm aller than c. À1.25—the spherical equivalent is obtained by adding
1.2 m m half the cylinder power to the sphere: À2.00
+ 0.75 ¼ À 1.25
24. A person looking at an object 5 m away through a 10 Δ
prism placed base-in over the right eye would see the 35. After extracapsular cataract extraction a patient is found to
im age displaced have 2 D of with-the-rule astigm atism and a tight suture
b. 50 cm to the right—a 10 Δ prism displaces light 10 cm/1 m across the wound at 12 o’clock. Corneal topography is
or 50 cm at 5 m, and the image is displaced to the apex obtained and the placido disc im age shows an oval
pattern with the m ires closest together at
25. Calculate the soft contact lens power for a 40-year-old a. 12 o’clock with the short axis at 90 degrees—WTR
hyperope who wears +14.00 D glasses at a vertex astigm atism refers to steep m eridian (short axis) at 90
distance of 11 m m . degrees (12 o’clock) which corresponds to the tight
b. +16.00 D—using the sim plified form ula, new suture. The pattern of projected placido rings is oval
power ¼ 14 + 0.011 (14) 2 ¼ 16.156 in astigm atism with the lines closest together in the
steepest m eridian (short axis)
26. After cataract surgery, a patient’s refraction is À0.75
+ 1.75 Â 10, in what m eridian should a suture be cut to 36. A 57-year-old wom an has a 0.25 m m m acular hole in her
reduce the astigm atism ? left eye. The size of the corresponding scotom a on a
d. 10 degrees—the steep m eridian corresponds to the axis tangent screen at 1 m is approxim ately
of the plus cylinder (plus cylinder acts to steepen the b. 1.5 cm —using sim ilar triangles and the m odel eye with
flat m eridian, which is 90 degrees away from the a nodal point of 17 m m , the resulting equation is: 0.25/
cylinder axis) 17 ¼ x/1000

372
 Answers to Questions

37. During retinoscopy, when neutralization is reached, the a. latent hyperopia—a cycloplegic refraction is necessary
light reflex is to uncover the full am ount of hyperopia, and a
d. widest and fastest—at neutralization, the retinoscopic glasses prescription should be determ ined from a
reflex is fastest, widest, and brightest subsequent m anifest refraction pushing plus and
using trial fram es to allow the patient to adapt.
38. A patient undergoing fogged refraction with an astigm atic
dial sees the 9 to 3 o’clock line clearer than all the others. At 48. In 1 year, a diabetic 60-year-old wom an has a change in
what axis should this patient’s m inus cylinder correcting refraction from À5.00 to À6.50 OU, which im proves her
lens be placed? vision to 20/20 OU. What is the m ost likely cause of her
c. 90 degrees—with an astigm atic dial, the axis of the refractive change?
m inus cylinder correcting lens is found by c. increasing nuclear sclerosis—nuclear sclerosis of the
m ultiplying the lower num ber of the clearest line by crystalline lens is the m ost com m on cause of a
30 (ie, for the 9 to 3 o’clock line: 3 Â 30 ¼ 90) m yopic shift in adults
39. Myopia is associated with all of the following conditions 49. The m ost com m on cause of m onocular diplopia is
except a. uncorrected astigm atism
a. nanophthalm os—nanophthalm os is associated with
50. A patient with a prescription of À5.50 sphere OD
hyperopia.
and À1.00 sphere O S is 20/ 20 in each eye but glasses
40. What is the ratio of the m agnification from a direct cause headache and double vision. The m ost likely
ophthalm oscope to the m agnification from an indirect reason is
ophthalm oscope with a 20 D lens at a distance of 25 cm d. aniseikonia—anisom etropia of> 3D results in an
if the patient and exam iner are both em m etropic? im age size discrepancy (aniseikonia) that becom es
c. 5 :1—the m agnification for the direct is 60/4 ¼ 15 and sym ptom atic
the m agnification for the indirect is 60/20 ¼ 3, so the
51. A hyperopic refraction is m ost likely caused by
ratio of the m agnifications is 15 :3 ¼ 5 :1
c. flat cornea and short axial length
41. A patient with anisom etropia wears glasses with a
52. How m uch accom m odation is needed for a patient with a
prescription of +5.00 OD and +1.25 OS. Which of the
distance correction of +1.50 to read without correction at
following actions will not reduce the am ount of
40 cm ?
aniseikonia?
c. +4.00—the am ount of accom m odation required ¼
b. decrease center thickness of left lens—this will have the
(100 cm /40 cm ) +1.50 ¼ 2.50 + 1.50 ¼ 4.00
opposite effect (increase the anisom etropia)
53. The lim bal relaxing incision for a patient with a refraction
42. The principal m easurem ent determ ined by a Prince’s rule
of +0.75-1.50x180 and no lenticular astigm atism should
and +3 D lens in front of the patient’s eye is the
be placed at
b. am plitude of accom m odation—the Prince’s rule is
b. 90 degrees—LRI for astigm atism correction is centered
prim arily designed to m easure the am plitude of
on the steep m eridian
accom m odation
54. The denom inator in Snellen visual acuity notation 20/60
43. The 10 Â eyepiece of the slit lam p biom icroscope is represents
essentially a sim ple m agnifier. Using the standard d. the distance at which the letter subtends the standard
reference distance of 25 cm , what is the dioptric power visual angle
of the 10 Â eyepiece?
d. +40 D—m agnification ¼ D/4, so 10 ¼ D/4 and D ¼ 40 55. A 26-year-old-wom an with a history of m yopic LASIK
com plains of blurriness OD. Her uncorrected visual
44. When refracting an astigm atic patient with a Lancaster dial, acuity is 20/20 but corneal topography shows a
the exam iner should place the decentered ablation. Which aberration is m ost likely to
d. entire conoid of Sturm in front of the retina—the be found on wavefront analysis?
patient m ust be fogged so that the entire conoid of c. third order—com a is m ost likely to be the etiology of
Sturm is in front of the retina her blurred vision
45. To increase the m agnification of the im age during indirect 56. If a Snellen chart is not present, then which of the
ophthalm oscopy, the exam iner should following tests is best for evaluating visual acuity?
a. m ove closer to the condensing lens—this causes the a. contrast sensitivity
im age to subtend a larger angle on the exam iner’s
retina thereby increasing the m agnification 57. A cycloplegic refraction would be m ost helpful for a
a. 10-year-old who has headaches—cycloplegia should be
46. A patient with which of the following refractive errors is used to prevent accom m odation and reveal the true
m ost likely to develop am blyopia? refractive error of the eyes
c. +5.00 OD, +5.00 OS
58. The m ost likely cause of a refractive surprise after
47. A 23-year-old m an reports blurry vision at near and uncom plicated cataract surgery is
wears +1.25 D reading glasses to see clearly. Which d. wrong keratom etry readings—this is the correct choice
of the following is m ost likely to be found on for the options given, but wrong axial length is usually
exam ination? the m ost com m on cause

373
Answers to Questions

59. Decreased color discrim ination is m ost likely to be caused 15. Hydroxychloroquine toxicity depends m ost on
by which type of cataract? b. cum ulative dose
a. nuclear sclerotic
16. Calculate the am ount of cocaine in 2 m L of a 4% solution.
60. A 3-piece acrylic intra-ocular lens (IOL) intended for the d. 80 m g—a 1% solution indicates 1 g/100 m L; therefore,
capsular bag is placed in the sulcus without optic a 4% solution is 4000 m g/100 m L¼ 40 m g/m L
capture through the capsulotom y. This patient is m ost
likely to experience what type of refractive error? 17. NSAIDs block the form ation of all of the following
d. m yopia—the power of an IOL placed in the sulcus substances except
instead of the capsular bag should be reduced by 1 D b. leukotrienes—NSAIDs block the conversion of
to achieve the sam e refractive outcom e arachidonic acid by cyclooxygenase into
endoperoxides (thus inhibiting the form ation of
prostaglandins, throm boxane, and prostacyclin);
leukotrienes are form ed from arachidonic acid by
CHAPTER 2 P HARMACOLOGY
lipoxygenase; steroids block the form ation of
arachidonic acid (and therefore, all subsequent end
1. Which antibiotic results in the highest intravitreal
products) by inhibiting phospholipase A2
concentration when adm inistered orally?
a. ciprofloxacin 18. System ic effects of steroids m ay include all of the following
except
2. Which anesthetic agent would m ost interfere with an
d. renal tubular acidosis
intraocular gas bubble?
d. nitrous oxide 19. Which drug does not produce decreased tear production?
a. pilocarpine
3. Which of the following is not an adverse effect of CAIs?
c. iris cysts 20. Natam ycin is a
c. polyene
4. Which β-blocker has the least effect on β2 receptors?
b. betaxolol—this is a cardioselective β1 -blocker 21. Which glaucom a m edicine does not decrease aqueous
production?
5. Which drug has the least effect on uveoscleral outflow? b. pilocarpine—m iotics increase aqueous outflow
d. Trusopt—this is a CAI that decreases aqueous
production; Xalatan and atropine increase and 22. β-blockers m ay cause all of the following except
pilocarpine decreases uveoscleral outflow. a. constipation

6. Which enzym e is inhibited by steroids? 23. Idoxuridine m ay cause all of the following except
b. phospholipase A2 c. corneal hypesthesia

7. Which of the following steroid form ulations has the best 24. Which of the following antifungal agents has the broadest
corneal penetrability? spectrum against yeast-like fungi?
a. prednisolone acetate—order of corneal penetrability is d. am photericin
acetate > alcohol > phosphate 25. All of the following m edications are com bination
8. Adverse effects of foscarnet include all of the following antihistam ine and m ast cell stabilizers except
except a. Alom ide—is only a m ast cell stabilizer
b. infertility 26. The antidote for atropine toxicity is
9. Which glaucom a m edication is not effective when the IOP b. physostigm ine
is > 60 m m Hg? 27. Which of the following agents is contraindicated for
c. pilocarpine—because of iris ischem ia, which occurs at ruptured globe repair?
IOP > 40 m m Hg d. succinylcholine—this nicotinic receptor antagonist causes
10. Which m edicine is not associated with OCP-like muscle contraction and is therefore contraindicated in
conjunctival shrinkage? ruptured globe repair since EOM contraction could
d. tim optic result in extrusion of intraocular contents

11. Which β-blocker is β1 -selective? 28. The duration of action of one drop of proparacaine is
c. betaxolol b. 20 m inutes—the duration of proparacaine is
10–30 m inutes
12. The m ost appropriate treatm ent for neurosyphilis is
a. penicillin G—IVpenicillin is used to treat neurosyphilis 29. Which of the following m edications is not com m ercially
available as a topical form ulation?
13. The correct m echanism of action of botulinum toxin is d. vancom ycin—m ust be com pounded, the others are
a. prevents release of acetylcholine available as Zirgan, Azasite, and Restasis, respectively

14. Fluoroquinolones are least effective against 30. All of the following are com plications of CAIs except
c. anaerobic cocci c. m etabolic alkalosis—CAIs cause m etabolic acidosis

374
 Answers to Questions

31. Topirim ate is associated with c. alpha-1 adrenergic antagonist—these oral m edications
d. angle-closure glaucom a without pupillary block (ie, tam sulosin [Flom ax]) are associated with
intraoperative floppy iris syndrom e (IFIS) and
32. A patient with ocular hypertension and an allergy to cataract surgery com plications
sulfonam ides should not be treated with
b. dorzolam ide— CAIs are sulfa drugs and are 45. A patient suddenly stops breathing after adm inistration
contraindicated in patients with a sulfa allergy of a peribulbar injection of anesthetic. The m ost likely
reason is
33. Infectious keratitis due to Candida albicans is best treated d. injection into the optic nerve sheath—anesthetic
with topical injected into the optic nerve sheath m ay result in
a. am photericin B central anesthesia with respiratory arrest
34. Which of the following oral agents should be used to treat
a patient with ocular cicatricial pem phigoid?
c. cyclophospham ide—this cytotoxic agent is used to treat CHAPTER 3 EMBRYOLOGY/P ATHOLOGY
patients with OCP
1. Which stain is the m ost helpful in the diagnosis of
35. The glaucom a m edication contraindicated in infants is sebaceous gland carcinom a?
b. brim onidine—selective alpha-agonists can cause death c. Oil-red-O, which stains lipid
in infants
2. Pagetoid spread is m ost com m only associated with
36. Which system ic antibiotic is used to treat chlam ydia c. sebaceous gland carcinoma—this is invasion of intact
during pregnancy? epithelium by nests of cells and is characteristic of
d. erythrom ycin—erythrom ycins are the preferred sebaceous gland carcinoma; pagetoid spread can also
antibiotic for chlam ydia infection and are safe during occur in the rare superficial spreading form of melanoma
pregnancy that occurs on unexposed skin areas (back, legs)

37. The local anesthetic with the longest duration of action is 3. A m elanom a occurring in which of the following locations
c. bupivacaine has the best prognosis?
a. iris—often can be com pletely excised
38. A 33-year-old m an has had follicular conjunctivitis with a
watery discharge for 5 weeks. Elem entary bodies are 4. Calcification in retinoblastom a is due to
present on a conjunctival sm ear, therefore, the m ost a. RPE m etaplasia
appropriate treatm ent is 5. The type of organism that causes Lym e disease is a
a. oral azithrom ycin—the patient has chlam ydial b. spirochete
conjunctivitis and requires oral antibiotics
6. Characteristics of ghost cells include all of the following
39. The m ost appropriate treatm ent for Fusarium keratitis is except
topical d. biconcave
b. pim aricin—this antifungal, also known as natam ycin,
is used to treat fungal keratitis 7. A gland of Moll is best categorized as
b. apocrine
40. All of the following are associated with vitam in A toxicity
except 8. Which of the following is not a Gram -positive rod?
d. band keratopathy c. Serratia—this bacterium is Gram -negative

41. Which of the following is a serious adverse effect of a long- 9. Trantas’ dots are com posed of what cell type?
acting cylcoplegic agent? c. eosinophil
c. urinary retention—cycloplegic agents are anticholinergic 10. Types of collagen that can be found in the cornea include
drugs (m uscarinic antagonists) and long-acting ones all of the following except
can have serious systemic effects including urinary b. II—is found in vitreous; type I is found in norm al
retention, particularly in elderly patients coneal strom a, III in strom al wound healing, and IV
42. An oral NSAID should not be used in a patient with in basem ent m em branes
a. renal insufficiency—NSAIDs can cause acute renal 11. Lens nuclei are retained in all of the following conditions
failure in healthy individuals and should not be except
adm in istered to patien ts with ch ron ic kid n ey d. Alport’s syndrom e
disease
12. Vogt-Koyanagi-Harada syndrom e is best described by
43. Ocular rosacea is best treated with which of the following which type of hypersensitivity reaction?
oral m edications? d. IV
c. doxycycline
13. Lacy vacuolization of the iris pigm ent epithelium occurs in
44. A patient taking which of the following m edications is at which disease?
increased risk for com plications at the tim e of cataract b. diabetes—lacy vacuolization is a pathologic finding of
surgery? glycogen-filled cysts in the iris pigm ent epithelium

375
Answers to Questions

14. Antoni A and B cells occur in which tum or? 29. Subepithelial infiltrates in the cornea from epidem ic
a. neurilem m om a (schwannom a) keratoconjunctivitis are thought to be
c. m acrophages containing adenoviral particles
15. Which tum or is classically described as having a ‘Swiss
cheese’ appearance? 30. Which is the correct order of solutions for perform ing a
b. adenoid cystic carcinom a Gram stain?
d. crystal violet stain, iodine solution, ethanol, safranin
16. Which iris nodule is correctly paired with its pathology
b. Lisch nodule, neural crest ham artom a—this is correct. 31. The iris sphincter is derived from what em bryologic tissue?
JXG nodules are com posed of histiocytes and Touton c. neural ectoderm
giant cells, Koeppe nodules are collections of
32. Blepharitis is m ost com m only associated with
inflam m atory cells, and Brushfield spots are strom al
b. Staphylococcus aureus
hyperplasia.
33. Which type of radiation causes lens capsule scrolling?
17. Which of the following statem ents is true concerning
a. infrared—this nonionizing radiation causes true
im m unoglobulin
exfoliation of the crystalline lens capsule, also known
a. IgG crosses the placenta
as glassblowers’ cataract
18. A retinal detachm ent caused by fixation artifact can be
34. Which of the following m easures is m ost likely to reduce
differentiated from a true retinal detachm ent by all of
the risk of postoperative endophthalm itis following
the following except
cataract surgery?
a. a fold at the ora serrata—is a fixation artifact found in
a. preoperative povidone-iodine drops—when applied to
newborn eyes called Lange’s fold. It is not related to an
the conjunctiva as part of the preoperative prep,
artifactual or true retinal detachm ent.
povidone-iodine has been docum ented to reduce the
19. Which of the following epithelial changes in the eyelid rate of postop endophthalm itis
refers to thickening of the squam ous cell layer
35. The m ost com m on causitive organism of canaliculitis is
b. acanthosis
c. Actinomyces israelii
20. Intraocular hem orrhage m ay cause all of the following
sequelae except
c. asteroid hyalosis—is calcium soaps suspended in the CHAPTER 4 NEURO-O PHTHALMOLOGY
vitreous and is unrelated to hem orrhage.
1. The visual field defect most characteristic of optic neuritis is
21. Intraocular calcification m ay occur in all of the following
b. central—all of these m ay occur in optic neuritis, but a
except
central scotom a is m ost characteristic
b. m edulloepitheliom a—this tum or m ay contain cartilage.
2. Which cranial nerve is m ost prone to injury in the
22. The histopathology of which tum or is classically described
cavernous sinus?
as a storiform pattern of tum or cells
d. 6—travels in m iddle of sinus and is not protected by
d. fibrous histiocytom a
lateral wall as are CN 3, 4, and 5
23. Which of the following findings is a histologic fixation
3. Which of the following agents is least toxic to the optic
artifact
nerve?
a. Lange’s fold—is a fold at the ora serrata that is a fixation
b. dapsone
artifact in newborn eyes.
4. Seesaw nystagm us is produced by a lesion located in which
24. The corneal strom a is com posed of
area?
b. neural crest cells
c. suprasellar—also associated with bitemporal hemianopia;
25. Neisseria is best cultured with which m edia chiasmal gliomas can cause spasmus nutans—like eye
d. chocolate agar movements in children; lesions in the posterior fossa
cause dissociated nystagmus, and those in the
26. Which of the following stains is used to detect am yloid cervicomedullary junction cause downbeat nystagmus
c. crystal violet—stains used to detect am yloid are Congo
red, crystal violet, and thioflavin T 5. What is the location of a lesion that causes an ipsilateral
Horner’s syndrom e and a contralateral CN 4 palsy?
27. HLA-B7 is associated with a. m idbrain—this is due to a nuclear/fascicular lesion at
b. presum ed ocular histoplasm osis syndrom e—POHS is the level of the m idbrain
associated with HLA-B7; the other associations are
Behçet’s disease with B51, iridocyclitis with B27, and 6. The least useful test for functional visual loss is
sym pathetic ophthalm ia with DR4 c. HVF—the other tests can com m only be used to trick the
patient
28. Which of the following conjunctival lesions should be sent
to the pathology lab as a fresh unfixed tissue specim ent? 7. Optociliary shunt vessels m ay occur in all of the following
a. lym phom a—fresh tissue is required for conditions except
im m unohistochem ical staining d. ischem ic optic neuropathy

376
 Answers to Questions

8. Which is not a sym ptom of pseudotum or cerebri? 20. A lesion in the pons causes
b. entoptic phenom ena b. m iosis
9. A 63-year-old wom an reports sudden onset of jagged 21. Which of the following syndrom es is characterized by
lines in the right peripheral vision. She has experienced abduction deficit and contralateral hem iplegia?
three episodes in the past m onth, which lasted c. Millard-Gubler—Foville’s and Gradenigo’s involve CN 6
approxim ately 10 to 20 m inutes. She denies headaches but do not cause hemiparesis; Weber’s does cause
and any history or fam ily history of m igraines. The m ost hemiparesis but involves CN 3, not CN 6
likely diagnosis is
c. m igraine variant—this is a characteristic visual 22. All of the following are features of progressive
disturbance that occurs in acephalgic m igraines and is supranuclear palsy except
called a fortification phenom enon; the other b. loss of oculovestibular reflex
com m on visual alteration is a scintillating scotom a 23. Pituitary apoplexy is characterized by all of the following
(appears as flickering colored lights that grow in the
except
visual field) a. nystagm us
10. A 60-year-old m an with optic disc swelling in the right eye
24. Which of the following is m ost likely to produce a
and left optic atrophy m ost likely has
junctional scotom a?
a. ischem ic optic neuropathy—these findings are
d. m eningiom a—a junctional scotom a is caused by a
consistent with Foster-Kennedy syndrom e; however,
lesion at the junction of the optic nerve and chiasm ;
the m ost com m on cause is pseudo-Foster-Kennedy
this is m ost com m only due to a m eningiom a
syndrom e due to ION
25. All of the following are characteristics of an optic tract
11. Which of the following findings m ay not be present in a
lesion except
patient with an INO?
b. decreased vision—visual acuity is not affected
c. absent convergence—this occurs only in an anterior
INO, not in a posterior INO (convergence is 26. The saccade system does not involve the
preserved); thus if the patient has a posterior INO, a. occipital m otor area
then absent convergence will not be found; the other
three findings occur in both anterior and posterior 27. A 22-year-old m an sustains traum a resulting in a
form s of INO transected left optic nerve. Which of the following is true
regarding the right pupil?
12. A paradoxical pupillary reaction is not found in which c. it is equal in size to the left pupil—because of the intact
condition? consensual response in the left eye
b. albinism —foveal hypoplasia occurs but pupillary
reactivity is norm al; paradoxical pupillary response is 28. Characteristics of spasm us nutans include all of the
found in CSNB, achrom atopsia, Leber’s congenital following except
am aurosis, optic atrophy, and optic nerve hypoplasia d. signs present during sleep—spasm us nutans disappears
during sleep
13. Inheritance of Leber’s optic neuropathy is
c. m itochondrial DNA 29. A congenital CN 4 palsy can be distinguished from an
acquired palsy by
14. An OKN strip m oved to the left stim ulates what part of the a. vertical fusional am plitude > 10 prism diopters
brain?
a. right frontal, left occipital 30. Characteristics of a diabetic CN 3 palsy m ay include all of
the following except
15. The sm ooth pursuit system does not involve the
d. aberrant regeneration—this does not occur with
d. frontal m otor area—this area is involved with fast eye
vasculopathic causes of CN 3 palsy, only with
m ovem ents
com pression (aneurysm , tum or) or traum a
16. Dorsal m idbrain syndrom e is not associated with
31. A CN 3 lesion m ay cause all of the following except
a. absent convergence—convergence is present and results
a. contralateral ptosis—depending on location of the
in convergence-retraction nystagm us
lesion, the ptosis m ay be ipsilateral (com plete or
17. The location of Horner’s syndrom e is best differentiated by superior division CN 3 paresis), bilateral (nuclear), or
which drug? no ptosis present (inferior division CN 3 palsy)
b. paredrine—distinguishes between preganglionic and
32. Optic nerve drusen is associated with all of the following
postganglionic lesions
except
18. The blood supply to the prelam inar optic nerve is c. CME
c. short posterior ciliary arteries
33. A lesion causing lim ited upgaze with an intact Bell’s
19. Optic nerve hypoplasia is associated with all of the phenom enon is located where?
following except a. supranuclear—if Bell’s phenom enon is intact, then the
d. spasm us nutans lesion m ust be supranuclear

377
Answers to Questions

34. An acute subarachnoid hem orrhage due to a ruptured 48. All of the following findings can occur in optic neuritis
aneurysm m ay produce all of the following except except
b. orbital hem orrhage—does not result from a d. m etam orphopsia
subarachnoid hem orrhage, but vitreous hem orrhage
(Terson’s syndrom e), ptosis, and an efferent pupillary 49. Which of the following findings is not associated with an
defect (CN 3 palsy) can occur acoustic neurom a
b. light-near dissociation
35. The length of the canalicular portion of the optic nerve is
approxim ately 50. A superior oblique m uscle palsy is m ost com m only
b. 10 m m caused by
d. traum a—the m ost com m on etiology of a CN 4 palsy
36. Findings in ocular m otor apraxia include all of the
following except 51. A 29-year-old obese wom an with headaches, papilledem a,
b. abnorm al pursuits and a norm al head CT scan is diagnosed with idiopathic
intracranial hypertension. All of the following findings
37. Which of the following statem ents is true regarding the
are consistent with her diagnosis except
optic chiasm ?
b. hom onym ous hem ianopia
c. 53% of nasal retinal fibers cross to the contralateral
optic tract 52. Transection of the left optic nerve adjacent to the chiasm
38. Which of the following statem ents is false regarding the results in
lateral geniculate body (LGB)? a. visual field defect in the right eye—junctional scotom a
d. P cells are im portant for m otion detection—this is false, due to crossing fibers in knee of von Willebrand
P cells are involved with fine spatial resolution and 53. The Am sler grid tests how m any degrees of central vision?
color vision. M cells are im portant for m otion
b. 10
detection, stereoacuity, and contrast
54. Aberrant regeneration of CN 3 m ay cause all of the
39. A patient with a hom onym ous hem ianopia is found to
following except
have an asym m etric OKN response. The location of the
c. m onocular dam pening of the OKN response
lesion is
a. parietal lobe—this is Cogan’s dictum : for 55. A 42-year-old wom an adm itted to the hospital with severe
hom onym ous hem ianopia, asym m etric OKN headache and neck stiffness suddenly becom es disoriented
indicates parietal lobe lesion and sym m etric OKN and vom its. On exam ination her left pupil is dilated and
indicates occipital lobe lesion does not react to light. She m ost likely has
40. The only intact eye movement in one-and-a-half syndrome is d. subarachnoid hem orrhage—this scenario represents a
c. abduction of contralateral eye CN 3 palsy due to a ruptured aneurysm (posterior
com m unicating artery)
41. A pineal tum or is m ost likely to cause
d. Parinaud’s syndrom e 56. Ahealthy 19-year-old wom an presents with gradual loss of
vision OD and pain when looking side to side. Her past
42. Metastatic neuroblastom a is m ost likely to be associated m edical history and review of system s are negative.
with Exam shows visual acuity of 20/50, reduced color vision,
a. opsoclonus a relative afferent pupillary defect, and a norm al
43. Which of the following statem ents regarding pupillary appearing optic nerve OD. The m ost im portant test to
innervation is true? obtain is
d. sym pathetic innervation of the iris dilator involves c. MRI—patients with optic neuritis without a diagnosis
three neurons and the ciliospinal center of Budge of MS should have an MRI of the head and orbits to
detect dem yelinating lesions/plaques in the
44. The m ost im portant test to order in a patient with chronic periventricular white m atter
progressive external ophthalm oplegia is
b. EKG—to rule out heart block from Kearns-Sayre 57. A 68-year old diabetic patient reports double vision.
syndrom e Exam is norm al except for a right abducens palsy.
Further questioning reveals recent weight loss and
45. Pseudotum or cerebri is m ost likely to cause a palsy of scalp and jaw pain. Which of the following tests is m ost
which cranial nerve? useful?
d. 6 b. C-reactive protein—giant cell arteritis (GCA) can
46. CT scan of a patient with visual loss shows a railroad-track present with an isolated cranial nerve palsy
sign. The m ost likely diagnosis is
58. A patient is found to have anisocoria, which is greater in a
c. optic nerve m eningiom a
dim room . The m ost likely etiology is
47. The m ost likely etiology of hom onym ous hem ianopia b. Horner’s syndrom e—anisocoria is accentuated in dim
with m acular sparing is light when the abnorm al pupil is m iotic (and
a. vascular—the m ost com m on cause of occipital lobe conversely in bright light when the abnorm al pupil is
lesions m ydriatic)

378
 Answers to Questions

59. A m iddle-aged m an relates a history of double vision and 12. In the treatm ent of a superior oblique palsy, Knapp
hearing loss from his left ear. On exam he has a left recom m ended all of the following except
esotropia and facial palsy. The m ost likely location of d. resection of the contralateral SR—the contralateral SR
his lesion is should be weakened with a recession, not
d. cerebellopontine angle—usually a m eningiom a or strengthened with a resection
acoustic neurom a that causes pseudo-Gradenigo’s
13. The best results of cryotherapy for ROP occur for treatm ent
syndrom e
of disease in which location?
60. A risk factor for nonarteritic anterior ischem ic optic b. anterior zone 2
neuropathy is
b. hypertension 14. The least com m on finding of congenital ET is
c. am blyopia
15. The contralateral antagonist of the right superior rectus
CHAPTER 5 P EDIATRICS /S TRABISMUS a. passes under another m uscle—the contralateral
antagonist of the right superior rectus is the left
1. The approxim ate age of onset for accom m odative ET is superior oblique (the antagonist [left superior
closest to oblique (LSO)] of the yoke m uscle [left inferior
b. 3 years old oblique (LIO)] of the paretic m uscle [right superior
rectus (RSR)]); the superior oblique (SO) passes
2. A 15-year-old girl with strabism us is exam ined, and the under the superior rectus (SR), is an incyclotorter
following m easurem ents are recorded: distance deviation (therefore causes excyclotorsion when paretic),
of 10 prism diopters, near deviation of 35 prism abducts the eye, and is innervated by CN 4
diopters at 20 cm , and interpupillary distance of 60 m m .
Her AC/A ratio is 16. With respect to Panum ’s area, physiologic diplopia occurs
a. 11 :1—to calculate the AC/A ratio with this at what point?
inform ation, use the heterophoria m ethod: IPD + c. in front of Panum ’s area—physiologic diplopia occurs
[(N À D)/Diopt] ¼ 6 + [(35 À 10)/5] ¼ 11 (rem em ber to in front of and behind Panum ’s area; within Panum ’s
convert IPD to cm , and 20 cm ¼ 5 D) area binocular vision occurs with fusion and
stereopsis, on the horopter only fusion
3. Duane’s syndrom e is thought to be due to a
developm ental abnorm ality of 17. The best treatm ent of an A pattern ET with m uscle
d. abducens nucleus transposition is
d. LRresection with downward transposition—appropriate
4. The m ost helpful test in a patient with aniridia is surgery for the ET is LR resection or MR recession; to fix
b. abdom inal ultrasound—to rule out Wilm s’ tum or in the A pattern, the LRs are m oved toward the empty
sporadic cases space of the pattern (down for ET) or the MRs are
m oved toward the apex of the pattern (up for ET)
5. The best test for an infant with a norm al fundus and
searching eye m ovem ents is 18. A superior rectus Faden suture is used for the treatm ent of
a. VER—can be used to determ ine acuity which condition?
b. dissociated vertical deviation
6. The m ost com m on congenital infection is
c. CMV 19. Which m edication should be adm inistered to a child who
develops trism us under general anesthesia?
7. ARC is m ost likely to develop in a child with
d. dantrolene—because trism us is a sign of m alignant
a. congenital esotropia
hypertherm ia
8. Which of the following m ost accurately reflects what a
20. Congenital superior oblique palsy is characterized by all of
patient with harm onious ARC reports when the angle of
the following except
anom aly is equal to the objective angle?
d. < 10 D of vertical vergence am plitudes—usually, these
c. sim ultaneous m acular perception
am plitudes are > 10 D
9. The inferior oblique m uscle is weakened m ost by which
21. Which of the following statem ents regarding
procedure?
m onofixation syndrom e is false?
d. anteriorization
c. Fusional vergence am plitudes are absent
10. The test that gives the best dissociation is
22. Iridocyclitis is m ost com m only associated with which
b. Worth 4 Dot form of JRA?
11. The 3-step test shows a left hypertropia in prim ary position d. pauciarticular
that worsens on right gaze and with left head tilt. The best 23. Congenital rubella is m ost com m only associated with
surgical procedure is a. retinal pigm ent epitheliopathy
d. LIO weakening—this is a LSO palsy, so possible
treatm ents include LIO weakening, RIR recession, 24. The m ost com m on cause of proptosis in a child is
LSO tuck, and LIR resection b. orbital cellulitis

379
Answers to Questions

25. Which form of rhabdomyosarcoma has the worst prognosis? 40. Hearing loss is not found in
c. alveolar—is the m ost m alignant and has the worst b. Refsum ’s disease—this is a form of retinitis pigm entosa
prognosis; em bryonal is the m ost com m on, botryoid (RP) without hearing loss
is a subtype of em bryonal, and pleom orphic is the
rarest and has the best prognosis 41. Pheochrom ocytom a m ay occur in all of the following
phakom atoses except
26. Which of the following conditions is the least com m on a. Louis-Bar syndrom e
cause of childhood proptosis?
a. cavernous hem angiom a—this is the m ost com m on 42. Maternal ingestion of LSD is m ost likely to result in which
benign orbital tum or of adults and occurs m ost congenital optic nerve disorder
com m only in m iddle-aged wom en c. hypoplasia—optic nerve hypoplasia is associated with
m aternal ingestion of alcohol, LSD, quinine, and
27. A child with retinoblastom a is born to healthy parents dilantin
with no fam ily history of RB. The chance of RB
occurring in a second child is approxim ately 43. A patient with strabism us wearing À6 D glasses is
a. 5% m easured with prism and cover test. Com pared to the
actual am ount of deviation, the m easurem ent would find
28. The best chronologic age to exam ine a baby for ROP is b. m ore esotropia and m ore exotropia—rem em ber,
c. 36 weeks m inus m easures m ore
29. All of the following are associated with trisom y 13 except 44. Prism glasses are least helpful for treating
c. epiblepharon c. sensory esotropia
30. Paradoxical pupillary response does not occur in 45. A 4-year-old boy has bilateral lateral rectus recessions for
d. albinism exotropia. Two days after surgery he has an esotropia
31. An infant with bilateral cataracts is diagnosed with m easuring 50 Δ . The m ost appropriate treatm ent is
galactosem ia. Which enzym e is m ost likely to be defective? d. surgery—this indicates a slipped m uscle which requires
b. galactose-1-P-uridyl transferase surgical repair

32. All of the following are associated with optic nerve drusen 46. The m ost com mon cause of a vitreous hem orrhage in a
except child is
c. increased risk of intracranial tum ors b. shaken baby syndrom e—traum a is the m ost com m on
etiology followed by regressed ROP (which is the
33. The etiology of torticollis and interm ittent, fine, rapid, m ost com m on cause of a spontaneous vitreous
pendular nystagm us of the right eye in a 10-m onth-old hem orrhage)
baby is m ost likely
d. none ofthe above—this infant has spasmus nutans, which 47. A 5-year-old girl with 20/20 vision OD and 20/50 vision
is usually benign and rarely due to optic nerve glioma OS is diagnosed with an anterior polar cataract OS. The
m ost appropriate treatm ent is
34. The m ost com m on m alignant tum or of the orbit in a a. start occlusion therapy
6-year-old boy is
b. rhabdom yosarcom a 48. Chronic iritis in a child is m ost com m only caused by
a. JRA
35. Retinitis pigm entosa and deafness occur in all of the
following disorders except 49. All are features of ataxia-telangiectasia except
c. Refsum ’s disease—there is no deafness in this retinitis b. thym ic hyperplasia—the thym us is hypoplastic
pigm entosa variant
50. All of the following vitreoretinal disorders are inherited in
36. α-galactosidase A deficiency is associated with an autosom al dom inant pattern except
a. cornea verticillata—this is the enzym e defect in Fabry’s d. Goldm ann-Favre disease—this is autosom al recessive
disease
51. The m ost com m on location for an iris colobom a is
37. Congenital cataracts and glaucom a m ay occur in all of the d. inferonasal
following disorders except
52. Von Hippel-Lindau disease has been m apped to which
b. Alport’s syndrom e—glaucom a is not a finding in
chrom osom e?
Alport’s syndrom e
a. 3
38. RPE degeneration and optic atrophy are found in all of the
53. Which X-linked disorder is not associated with an ocular
following m ucopolysaccharidoses except
abnorm ality in the fem ale carrier?
d. MPS type IV—findings in MPS type IV include corneal
c. juvenile retinoschisis—fem ale carriers have norm al
clouding and optic atrophy, not RPE degeneration
fundus; in the other three disorders, fem ale carriers
39. Which vitam in is not deficient in a patient with have retinal changes: m idperipheral pigm ent clusters
abetalipoproteinem ia (Bassen-Kornzweig syndrom e)? and m ottling in m acula in albinism , equatorial
b. C—the fat soluble vitam ins A, D, E, and K cannot be pigm ent m ottling in choroiderem ia, golden reflex in
absorbed in abetalipoproteinem ia posterior pole in retinitis pigm entosa

380
 Answers to Questions

54. Which tum or is not associated with von Hippel-Lindau 3. Which procedure is the best treatm ent option for the repair
disease? of a large upper eyelid defect?
a. hepatocellular carcinom a a. Cutler-Beard—is a lid-sharing procedure for repair of
large upper eyelid defects, Bick is a horizontal lid-
55. The m ost useful diagnostic test in an infant with an oil-
shortening procedure, Hughes is a lid-sharing
droplet cataract is
procedure for repair of large lower eyelid defects, and
d. urine-reducing substances—to check for galactosem ia
Fasanella-Servat is a tarsoconjunctival resection for
56. Genetics of aniridia are best sum m arized as ptosis repair
d. ¼ sporadic, ¾ AD
4. The extraocular m uscle with the largest arc of contact is the
57. A pigm entary retinopathy occurs in which m esoderm al b. IO—which is 15 m m ; next is LR at 12 m m , then SO at
dysgenesis syndrom e? 7–8 m m , and MR at 7 m m
b. Alagille’s syndrom e
5. The risk of system ic involvem ent is highest for an ocular
58. Which of the following laboratory tests is m ost com m only lym phoid tum or in which location?
found in JRA-related iritis?
b. eyelid—67% have system ic involvem ent; for orbit, it is
c. RF–, ANA+ —which occurs in early-onset 35% and for conjunctiva, 20%
pauciarticular JRA
59. The size of an esodeviation is m easured with 6. The rectus m uscle with the shortest tendon of insertion
c. Alternate prism and cover test is the
c. MR—the IO has the shortest tendon (1 m m ), but of the
60. Toxoplasm osis is m ost likely to be acquired from rectus m uscles, the MR has the shortest tendon at
b. undercooked m eat 4.5 m m ; the others are SR¼ 6 m m , IR¼ 7 m m , and
61. A 10-day-old infant develops an acute, papillary LR¼ 7 m m
conjunctivitis with m ucoid discharge. Which of the 7. Which of the following bones does not m ake up the
following is the m ost likely cause? m edial orbital wall?
c. Chlam ydia d. palatin e—is part of th e orbital floor; th e m edial wall
62. An infant is brought to the ER after a fall. There is a bruise is com posed of th e oth er th ree bon es an d th e
on the forehead and num erous retinal hem orrhages. There sph en oid
are also bruises on the back. An X-ray shows previous rib
8. Which of the following clinical features is least com m only
fractures. The m ost likely diagnosis is
associated with a tripod fracture?
d. non-accidental traum a—fundus hem orrhages at
a. restriction of the inferior rectus—tripod fractures
m ultiple layers in an infant is m ost com m only due to
include disruption of the orbital floor, but
shaken baby syndrom e, and other signs of abuse
entrapm ent of ocular tissues is rare and is usually
should be looked for
associated with large floor fractures (blow-out
63. A child undergoes uncom plicated cataract surgery with fracture); the other three findings are m uch m ore
phacoem ulsification and insertion of an acrylic posterior com m on in a tripod fracture
cham ber intraocular lens. What is the m ost likely
com plication to develop in the future? 9. A carotid-cavernous fistula is com m only differentiated
a. capsular opacification from a dural-sinus fistula by all of the following
characteristics except
64. On a routine eye exam , a 5-year-old girl is found to have a. proptosis—this can occur in both types of AV fistula;
m ild iritis in both eyes. What is the m ost helpful test to (a bruit can too, but rarely); the other signs are seen
order? with CC fistulas
a. ANA—ANA and RF are the m ost helpful test to
diagnose JRA 10. Basal cell carcinom a is least likely to occur at which site?
d. lateral canthus—the order (in decreasing frequency) is
65. The m ost com m on color vision defect is as follows: lower lid > m edial canthus > upper
c. deuteranom aly—approxim ately 5% of m en have this lid > lateral canthus
X-linked recessive congenital color deficiency
11. All of the following are sites of attachm ent of the lim bs of
the m edial canthal tendon except
CHAPTER 6 O RBIT/LIDS /ADNEXA c. orbital process of the frontal bone

1. Which organism is m ost com m only associated with 12. Which m uscle is m ost com m only responsible for vertical
angular blepharitis? diplopia after 4-lid blepharoplasty?
b. Moraxella b. inferior oblique—because it lies below the inferior rectus
and is encircled by the capsulopalpebral fascia
2. Sequelae of a CN 7 palsy m ay include all of the following
except 13. Congenital and involutional ptosis can be distinguished
b. ptosis—CN 7 palsy causes inability to close the lid and by all of the following except
exposure keratopathy; CN 3 palsy causes ptosis c. width of palprebal fissure

381
Answers to Questions

14. Congenital obstruction of the lacrim al drainage system 27. Which of the following factors is least likely to contribute
usually occurs at the to the developm ent of entropion?
d. valve of Hasner a. preseptal orbicularis override—this can occur but is less
com m on than the other factors
15. What is the correct order of structures that would be
encountered when the upper eyelid is penetrated 14 m m 28. A 24-year-old wom an presents after blunt trauma to the left
above the lid m argin? orbit with enophthalm os and restriction of upgaze. Which
a. preseptal orbicularis m uscle, orbital septum , levator plain film radiographic view would be m ost helpful?
aponeurosis, Mu€ ller’s m uscle c. Waters view—gives best view of orbital floor
16. What is the best treatm ent option for a child who develops 29. All of the following m ay cause enophthalm os except
recurrent proptosis after upper respiratory infections? b. lym phom a—m ay cause proptosis but not
a. observation—this scenario is com m on with an orbital enophthalm os; breast cancer can cause either, and
lym phangiom a, and spontaneous regression often phthisis and floor fractures m ay cause enophthalm os
occurs
30. All of the following nerves pass through the superior
17. All of the following are features of m ucorm ycosis except orbital fissure except
b. ipsilateral CN 7 palsy—m ucor m ay cause an orbital c. CN V2 —passes through the inferior orbital fissure
apex syndrom e, but CN 7 is not involved
31. Blepharospasm is associated with
18. All of the following are associated with blepharophim osis d. Parkinson’s disease
except
32. The anatom ic boundaries of the superior orbital fissure are
c. AR inheritance—blepharophim osis m ay be part of an
b. the greater and lesser wings of the sphenoid
AD syndrom e (chrom osom e 3q), as well as trisom y
18; findings include blepharophim osis, ptosis, 33. Which of the following is m ost likely to exacerbate the
telecanthus, ectropion, and epicanthus inversus sym ptom s of thyroid-related ophthalm opathy
b. cigarettes
19. Which of the following is the m ost im portant test to
perform in a patient with a capillary hem angiom a? 34. a 44-year-old wom an develops a left lower eyelid
d. bleeding tim e—to look for Kassabach-Merritt ectropion following a severe facial burn. The m ost
syndrom e (consum ptive coagulopathy) appropriate procedure includes
a. horizontal tightening—and revision of the cicatrix is
20. For entropion repair, the lateral tarsal strip is sutured
performed for cicatricial ectropion repair, and a
c. above and anterior to the rim
vertical lengthening procedure with full-thickness
21. Staged surgery for a patient with severe thyroid-related graft may also be required
ophthalm opathy is best done in what order
35. All of the following are m ethods of treating spastic
a. decom pression, strabism us, lid repair—because
entropion except
decom pression m ay affect ocular alignm ent and lid
c. Wies m arginal rotation—is used to treat involutional
position, and strabism us surgery m ay affect lid
entropion
position
36. The m ost com m on com plication of a hydroxyapatite
22. Which of the following best explains why when a ptotic lid
orbital im plant is
is lifted, the contralateral lid falls?
d. conjunctival erosion
d. Hering’s law—equal and sim ultaneous innervation to
synergistic m uscles; thus, lifting a ptotic lid decreases 37. Which collagen vascular disease is associated with
the innervation to the levator bilaterally so the m alignancy?
contralateral lid will fall slightly a. derm atom yositis
23. Which study is m ost helpful in the evaluation of a patient 38. Oral antibiotics are indicated for
with opsoclonus? b. dacryocystitis—dacryocystitis is treated with topical
b. MRI—to rule out neuroblastom a or visceral and system ic antibiotics, while dacryoadenitis m ay
carcin om a som etim es require system ic antibiotics.
24. What is the m ost appropriate treatm ent for a benign m ixed 39. The levator m uscle inserts onto all of the following
tum or of the lacrim al gland? structures except
b. excision—m ust excise com pletely en bloc to prevent d. trochlea
recurrence and m alignant transform ation
40. When perform ing a DCR, the osteum is created at the level
25. What is the m ost appropriate treatm ent for a biopsy- of the
positive basal cell carcinom a of the lower eyelid? b. m iddle turbinate
d. excision with frozen section control of the m argins
41. An adult with a com plete nasolacrim al duct obstruction
26. Which of the following CT enhancing lesions has a and patent puncta and canaliculi is best treated with
pathognom onic appearance? which procedure?
d. m eningiom a—produces characteristic railroad track d. dacryocystorhinostomy—acquired NLDO is treated
sign with DCR

382
 Answers to Questions

42. The m ost effective procedure for involutional ectropion is 4. Which disease has never been transmitted by a corneal graft?
a. lateral tarsal strip a. CMV—the others have been transm itted in hum ans or
experim entally in anim als
43. A patient presents with follicular conjunctivitis and a
cluster of um bilicated papules are noted near the 5. Which corneal dystrophy does not recur in a corneal graft?
eyelashes of the left eye. The m ost effective treatm ent for d. PPCD
this condition is
c. cryotherapy—m olluscum contagiosum is best treated 6. A conjunctival m ap biopsy is typically used for which
with excision, cryotherapy, or incision and curettage m alignancy?
c. sebaceous gland carcinom a
44. The m ost com m on cause of unilateral proptosis in a
m iddle-aged wom an is 7. All of the following m ay cause follicular conjunctivitis
b. thyroid-related orbitopathy—this is the m ost com m on except
cause of proptosis in adults (unilateral and bilateral) b. Neisseria
8. Which of the following tests is least helpful in determ ining
45. The m ost com m on cause of involutional entropion of the
the etiology of enlarged corneal nerves?
lower eyelid is
a. EKG—the others are all helpful in detecting disorders
b. laxity and retractor disinsertion
that are associated with enlarged corneal nerves:
46. An elderly wom an with chronic unilateral blepharitis, calcitonin for m edullary thyroid carcinom a (MEN
thickening of the left upper eyelid, and subm andibular 2b), urinary vitreom acular adhesion (VMA) for
lym phadenopathy is m ost likely to have pheochrom ocytom a (MEN 2b), acid-fast stain for
d. sebaceous gland carcinom a—this m alignant tum or can atypical Mycobacteria (leprosy)
m asquerade as chronic blepharitis
9. Corneal filam ents are least likely to be present in which
47. A 56-year-old diabetic wom an presents with pain, condition?
swelling, and redness of the left upper eyelid. Orbital b. Thygeson’s SPK
involvem ent is m ost likely if she also has
10. Which of the following is not an appropriate treatm ent for
d. pain with eye m ovem ent—this is a sign of orbital
SLK?
involvem ent of the infection; ptosis m ay occur in
c. silver nitrate stick—m ay cause globe perforation;
both preseptal and orbital cellulitus
therefore, use only silver nitrate solution
48. A72-year-old m an has bilateral ptosis and levator function
11. In what level of the cornea does a Kayser-Fleischer ring occur?
m easuring 14 m m OU. The m ost likely diagnosis is
d. Descem et’s m em brane
c. levator aponeurotic dehiscence—this is the m ost
com m on cause of ptosis with good levator function 12. Cornea verticillata—like changes are associated with all of
in elderly individuals the following except
b. haloperidol
49. The sensory nerve m ost likely to be affected by an orbital
fracture is 13. The least com m on location for a nevus is
c. infraorbital—which results in infraorbital hypesthesia a. bulbar conjunctiva
after orbital floor and zygom atic fractures
14. All of the following ions m ove across the corneal
50. Which of the following findings is m ost helpful for m aking endothelium by both active transport and passive
the diagnosis in a patient with suspected thyroid-related diffusion except
orbitopathy a. ClÀ —moves across endothelium only by passive diffusion
a. abnorm al forced ductions—TRO causes restrictive
strabism us 15. Which organism is associated with crystalline keratopathy?
d. S. viridans
16. Which of the following conditions is associated with the
CHAPTER 7 C ORNEA/EXTERNAL DISEASE best 5-year prognosis for a corneal graft?
b. Fuchs dystrophy
1. Which is the least desirable m ethod for corneal graft
storage? 17. The best strategy for loosening a tight contact lens is to
b. glycerin—does not preserve endothelial cells and can be c. decrease the diam eter—decreasing the curvature will
used only for lamellar or patch grafts; moist chamber at also loosen a tight lens but not as well; increasing the
4°C preserves tissue for 48 hours, Optisol for up to diameter or curvature will tighten a lens
10 days, and cryopreservation potentially for years
18. The type of contact lens that causes the least endothelial
2. Presently in the United States, phlyctenule is m ost pleomorphism is
com m only associated with a. soft daily wear
c. Staphylococcus
19. Which of the following conditions is associated with the
3. Which blood test is m ost helpful in the evaluation of a worst prognosis for a corneal graft?
patient with Schnyder corneal dystrophy? c. Reis-Bucklers dystrophy—this dystrophy com m only
d. cholesterol—m ay be elevated recurs in the graft

383
Answers to Questions

20. Which is not a treatm ent of acute hydrops? 36. PTK would be m ost appropriate for treating which of the
d. corneal transplant—eventually, this m ay be an option, following corneal disorders?
but short-term treatm ent is m edical only a. superficial granular dystrophy
21. Which organism cannot penetrate intact corneal 37. A 62-year-old wom an with keratoconjunctivitis sicca is
epithelium ? m ost likely to dem onstrate corneal staining in which
c. P. aeruginosa—cannot penetrate intact corneal location?
epithelium ; the others and Listeria can b. m iddle third (interpalpebral)—due to exposure
between the eyelids
22. Which of the following m edications would be the
best choice in the treatm ent of m icrosporidial 38. Which of the following findings is most comm only
keratoconjunctivitis? associated with SLK?
a. fum agillin a. filam ents—found in 50%
23. All of the following agents are used in the treatm ent of 39. Which lab test is m ost helpful to obtain in a 38-year-old
Acanthamoeba keratitis except m an with herpes zoster ophthalm icus?
b. natam ycin d. HIV test—herpes zoster is rare in healthy individuals
24. Goblet cells are m ost abundant in which location? younger than 40 years old and m ay indicate
a. fornix im m unosuppression

25. Thygeson’s superficial punctate keratopathy is best treated 40. A patient with conjunctival intraepithelial neoplasia is
with topical m ost likely to have
c. loteprednol—steroids are the best treatm ent d. HPV—this virus is associated with CIN, and patient
who have HIV are also m ore likely to develop CIN
26. EKC is typically contagious for how m any days?
d. 14 days 41. Which of the following disorders is m ost likely to be found
in a patient suffering from sleep apnea?
27. A shield ulcer is associated with c. follicular conjunctivitis—secondary to floppy eyelid
c. VKC syndrom e

28. Which of the following is m ost likely to be associated with 42. A patient with graft-vs-host disease is m ost likely to have
m elanom a of the uvea? which eye finding?
c. Nevus of Ota—increased risk of uveal m elanom as b. sym blepharon—due to cicatrizing conjunctivitis
in Caucasians. This is rarer for congenital m elanosis
43. Topical corticosteroids should not be used in a patient
oculi (ocular m elanocytosis), and acquired m elanosis
with which form of herpes sim plex keratitis?
oculi (PAM) is not associated with uveal m elanom a.
a. epithelial
29. Which of the following is not associated with N. gonorrheae
44. What is the m ost appropriate m anagem ent for a patient
conjunctivitis?
who reports recurrent foreign body sensation when
a. pseudom em brane—gonococcal (GC) conjunctivitis
waking up but on exam does not have a discrete
causes a true m em brane
epithelial defect?
30. Even spreading of the tear film depends most on which factor? b. lubrication—initial m anagem ent of recurrent erosion
c. m ucin syndrom e is with lubrication/Muro 128
31. A neurotrophic ulcer should not be treated with 45. A 24-year-old swim m er reports a painful, red left eye and
b. antiviral—this can cause m ore toxicity blurry vision after sleeping with her contact lens for several
days. Exam ination shows a corneal ulcer with edem a and a
32. Which layer of the cornea can regenerate?
hypopyon. The m ost likely diagnosis is
c. Descem et’s m em brane—can regenerate if endothelium
c. Acantham oeba—this organism is associated with
is intact
corneal infections in contact lens wearers who clean
33. The m ost appropriate treatm ent for a patient with their lenses with hom em ade saline and swim or hot
sclerom alacia is tub with their contacts in
d. oral im m unosuppressive agent—sclerom alacia is
46. The best test to m easure basal tear secretion is
caused by severe rheum atoid arthritis
c. Schirm er’s test with anesthesia—m easures basal tear
34. The HEDS recom m endation for treating strom al secretion, Schirm er’s without anesthesia m easures
(disciform ) keratitis is reflex and basal tear secretion, and tear breakup tim e
b. topical steroid and topical antiviral—oral acyclovir was is used to evaluate the stability of the tear film . The
not found to be useful dye disappearance and Jones tests help to identify
obstruction of the lacrim al drainage system .
35. Feathery edges and a satellite infiltrate are m ost
characteristic of a corneal ulcer caused by 47. Adenoviral m em branous conjunctivitis is m ost likely to
d. Fusarium —these characteristics are associated with cause which of the following?
fungal ulcers a. sym blepharon

384
 Answers to Questions

48. After a corneal alkali burn, which of the following signs is 5. The m ost com m on organism causing endopthalm itis
associated with the worst prognosis? following cataract surgery is
d. com plete lim bal blanching—this is a sign of lim bal d. S. epidermidis
ischem ia, which is associated with m ore severe injury.
The larger the area of lim bal involvem ent, the worse 6. MEWDS can be differentiated from APMPPE by
the prognosis b. fem ale predilection

49. The m ost likely cause of a corneal keratom etry 7. All of the following disorders are correctly paired with their
m easurem ent 2.5 D steeper inferiorly than superiorly at HLA associations except
the 3 m m zone is a. Presum ed ocular histoplasm osis syndrom e (POHS),
c. keratoconus—one of the corneal topography B9—POHS is associated with HLA-B7, DR2
param eters classically used to diagnose keratoconus is 8. Decreased vision in a patient with interm ediate uveitis is
an I-S value (difference between average inferior and m ost likely due to
superior corneal powers 3 m m from the center of the b. m acular edem a
cornea) > 1.4 D
9. A 71-year-old wom an with a 6-m onth history of fatigue,
50. The nasociliary branch of the trigem inal nerve innervates anorexia, and 10-pound weight loss is found to have
the tip of the nose and the left-sided weakness, visual acuity of 20/80 OD and
d. cornea—this is the reason that ocular involvem ent 20/60 OS, and vitreous cells. The m ost helpful workup is
should be suspected when a herpes zoster lesion is a. LP and vitrectom y—to rule out CNS lym phom a
present on the nose (Hutchinson’s sign)
10. The m ost com m on organism s causing endophthalm itis
51. The m ost com m on com plication of pterygium surgery is
following traum a is
d. recurrence
b. Bacillus species and S. epidermidis
52. The refractive effect of Intacs is titrated by the
11. All of the following are features com m on to both
c. thickness of the ring segm ents
sym pathetic ophthalm ia and Vogt-Koyanagi-Harada
53. At the 4-week postop exam of a patient who underwent syndrom e except
uncom plicated PRK for m oderate m yopia, the m anifest c. pathology localized to choroid—retina is also involved
refraction is À0.25 D OD and À1.25 D OS. Slit lam p in VKH
exam shows m oderate anterior strom al haze OS. How
12. Which disorder is m ore com m on in m ales?
would you m anage this patient?
b. uveal effusion syndrom e
b. increase topical steroids—this is the initial management
of corneal haze in the early postop period after PRK 13. EVS findings include all of the following except
54. A buttonhole flap is m ost likely to occur with a b. intravitreal corticosteroids were helpful—intravitreal
m icrokeratom e if the patient has steroids were not evaluated in the EVS
c. keratom etry > 47 D—a steep cornea is a risk factor for a 14. Which of the following is not characteristic of MEWDS?
buttonhole flap b. bilaterality
55. The best surgical option for a patient with Fuchs 15. The m ost com m on cause of posterior uveitis is
dystrophy, corneal edem a, and a visually significant d. toxoplasm osis
cataract is
d. phacoem ulsification, IOL, and endothelial keratoplasty 16. All of the following are causes of HLA-B27-associated
uveitis except
d. psoriasis—is not associated with uveitis, but psoriatic
arthritis is
CHAPTER 8 UVEITIS 17. Which of the following is not part of the classic triad of
findings in Reiter’s syndrom e?
1. The m ost effective antibiotic for the treatm ent of P. acnes
a. iritis—iritis occurs but the classic triad of Reiter’s is
endophthalm itis is
arthritis, conjunctivitis, and urethritis
c. vancom ycin
18. Phacoantigenic endophthalm itis is characterized by which
2. For the diagnosis of granulom atous inflam m ation, which
pattern of granulom atous inflam m ation?
cell type m ust be present?
a. zonal
d. epithelioid histiocyte
19. A 35-year-old m an with decreased vision OD is found to
3. All of the following are true concerning sarcoidosis except
have optic nerve edem a and a m acular star. The
a. Touton giant cells are com m on—sarcoidosis is
causative organism m ost likely is
characterized by Langhans’ giant cells
b. Bartonella henselae—which causes cat-scratch disease
4. Which of the following is not characteristic of Fuchs’ and a neuroretinitis; O. volvulus causes onchocerciasis,
heterochrom ic iridocyclitis? T. pallidum causes syphilis, and B. burgdorferi causes
c. posterior synechiae Lym e disease

385
Answers to Questions

20. A person living in which area of the US would be m ost 37. A patient with APMPPE is m ost likely to have
likely to develop POHS? c. viral prodrom e
c. Midwest—in the US, histoplasm osis is m ost prevalent
38. A patient with a m ild anterior cham ber reaction, increased
in the Ohio River valley and Eastern states
intraocular pressure, and iris heterochrom ia is m ost likely
21. All of the following are true of birdshot choroidopathy to also exhibit which other finding?
except d. fine vessels in the angle—occur in Fuchs’
a. m ore com m on in m ales—birdshot choroidopathy is heterochrom ic iridocyclitis
m ore com m on in fem ales
39. Which of the following cell types is found in both
22. Which of the following is least com m only associated with granulom atous and nongranulom atous keratic
Treponema pallidum infection? precipitates?
d. glaucom a—is not associated with ocular involvem ent c. lym phocytes
of syphilis
40. All of the following are m asquerade syndrom es except
23. The HLA association for pars planitis with m ultiple a. vitreous hem orrhage—this m ay occur in uveitis
sclerosis is (eg, VKH, POHS) but is not a condition that presents
d. DR15 as uveitis
24. Retinal S antigen is found in 41. Afalse-positive VDRLtest is least likely to occur in a patient
c. photoreceptors—retinal S antigen is found in outer with
segm ents of receptor cells a. Wegener’s granulom atosis
25. Features of Harada’s disease include all of the following 42. A 54-year-old m an with chronic recurrent uveitis OS
except controlled with topical steroids has developed a visually
b. deafness—only eye findings are seen in Harada’s significant cataract. What is the m ost appropriate treatment?
disease d. delay cataract surgery until the eye has been quiet for at
least 3 m onths
26. Larva cause all of the following infections except
d. cat-scratch disease—is caused by the bacteria Bartonella 43. All of the following are risk factors for traum atic
henselae endophthalm itis except
d. double penetrating injury
27. Which of the following signs of pars planitis is m ost
associated with m ultiple sclerosis 44. A pseudophakic patient develops granulom atous
c. periphlebitis inflam m ation 8 m onths after cataract surgery, and a
white plaque is present on the posterior capsule. The
28. CSF abnorm alities are associated with all of the following
organism m ost likely to be causing this condition is
disorders except
c. P. acnes—an anaerobic, Gram -positive rod, is the m ost
b. ocular sarcoidosis
com m on organism causing delayed endopthalm itis
29. All of the following can present as uveitis except following cataract surgery
b. choroidal hem angiom a
45. A 28-year-old wom an acutely develops reduced vision,
30. Which of the following is not associated with pain, redness, and floaters OS. Exam shows a m ild iritis
inflam m atory bowel disease? with granulom atous KP and discrete patches of
c. interstitial keratitis peripheral necrotizing retinitis. The m ost appropriate
m anagem ent is to begin treatm ent with
31. Anterior vitreous cells are least likely to be found in a. acyclovir—antiviral m edication, steroids, and aspirin
a. retinitis pigm entosa are used to treat acute retinal necrosis
32. Gastrointestinal disorders associated with uveitis include
all of the following except
c. diverticulitis
CHAPTER 9 G LAUCOMA
33. All of the following m ay occur in ocular sarcoidosis except
d. low serum gam m a globulin—serum gam m a globulin 1. What is the m ost appropriate initial treatm ent of pupillary
level is elevated block in a patient with m icrospherophakia?
34. The choroid is the prim ary location of the pathologic d. cyclopentolate—which helps pull the anteriorly
process in displaced lens back to its norm al position
d. VKH syndrom e 2. Which of the following statem ents is true? Uveoscleral
35. Which of the following is least likely to be found in a outflow is
patient with sym pathetic ophthalm ia c. increased by atropine—cycloplegics and prostaglandin
b. granulom atous nodules in the retina—nodules occur analogues (Xalatan, Lum igan, Travatan) increase
in the choroid (and m iotics decrease) uveoscleral outflow;
fluorophotom etry m easures the rate of aqueous
36. Band keratopathy is least likely to occur in a patient with form ation; uveoscleral outflow accounts for 15% to
c. Behçet’s disease 20% of total outflow and is independent of pressure

386
 Answers to Questions

3. Risk factors for angle-closure glaucom a include all of the 15. Which of the following is least likely to cause increased
following except IOP 2 days postoperatively?
b. m yopia—this is a risk factor for open-angle glaucom a, d. steroid drops—steroid response usually takes at least
not angle-closure glaucom a 7–10 days to occur
4. Which of the following would cause the greatest elevation 16. Treatm ent of m alignant glaucom a m ay include all of the
in IOP? following except
a. blinking—IOP is lowered by decreased blood cortisol b. pilocarpine—m iotics are contraindicated in m alignant
levels (reduces aqueous form ation) and elevation of glaucom a because the aim of m edical therapy is to pull
the head (changing from supine to sitting position); the lens/iris diaphragm posteriorly (cycloplegics)
darkening the room m ay increase IOP in patients
with narrow angles 17. The m ost com m on organism associated with bleb-related
endophthalm itis is
5. The m ost likely cause of a large filtering bleb and a shallow a. Streptococcus species—are m ost com m on ($ 50%)
cham ber is followed by coagulase-negative Staph and then H. flu
d. overfiltration—which causes both findings; a leak would
cause a flat or low bleb; pupillary block and aqueous 18. Which visual field defect is least characteristic of
m isdirection would not change the size of the blebs glaucom a?
c. central scotom a—central vision is not affected until late
6. A change in Goldm ann visual field stim ulus from I4e to in the disease
II4e is equivalent to
a. 1 log—the Rom an num eral indicates the size of the test 19. The type of tonom eter m ost greatly affected by scleral
object in m m 2 (0 ¼ 1/16, I ¼ 1/4, II ¼ 1, III ¼ 4, IV¼ 16, rigidity is
V¼ 64) and is a logarithm ic scale; therefore, changing c. Schiøtz—low scleral rigidity yields falsely low readings
from size I to II is 1 log unit (ie., high m yopia, thyroid disease, previous ocular
surgery, m iotic therapy), and high scleral rigidity
7. An Am sler grid held at 33 cm m easures approxim ately how yields falsely high readings (ie, high hyperopia,
m any degrees of central vision? vasoconstrictor therapy)
b. 10
20. As com pared with plasm a, aqueous has a higher
8. The m ost decreased sensitivity in an arcuate scotom a
concentration of
occurs in which quadrant?
c. ascorbate—has a concentration 15 Â higher in aqueous
c. superotem poral
than in plasm a, whereas the concentrations of sodium ,
9. The best gonioscopy lens for distinguishing appositional calcium , and protein are lower in aqueous
from synechial angle closure is
21. The rate of aqueous production per m inute is
b. Zeiss—this is the only lens of the four that can be used
approxim ately
for indentation gonioscopy (indenting the cornea
b. 2.6 µL—1% of aqueous turned over per m inute, 100%
forces aqueous into the angle pushing the iris
turnover in 100 m in; AC volum e ¼ 200 µL, PC
backward and allowing one to view the angle)
volum e ¼ 60 µL, total volum e ¼ 260 µL
10. Which is not a risk factor for POAG?
d. CRAO 22. The location of the greatest resistance to aqueous outflow is
c. juxtacanalicular connective tissue
11. ALTwould be m ost effective in a patient with which type of
glaucom a? 23. Facility of aqueous outflow is best m easured by
c. pigm entary—patients with pigm entary glaucom a often a. tonography—m anom etry m easures episcleral venous
respond well to ALT, whereas those with inflam m atory, pressure, tonom etry m easures intraocular pressure,
congenital, and aphakic glaucom a do not fluorophotom etry m easures rate of aqueous
form ation (via decreasing fluorescein concentration)
12. In which direction should a patient look to aid the
exam iner’s view of the angle during Zeiss gonioscopy? 24. A patient recently had an acute angle closure attack in the
b. toward the m irror—this allows for a better view of the right eye. What is the m ost appropriate treatm ent for her
portion of the angle being inspected. Alternatively tilt left eye?
the lens towards the angle being inspected (away b. laser peripheral iridotom y—iridotom ies m ust be
from m irror). perform ed in both eyes

13. The best param eter for determ ining the unreliability of a 25. The m ost likely gonioscopic finding in a patient with
Hum phrey visual field is glaucom a and radial m idperipheral spoke-like iris
a. fixation losses transillum ination defects is
a. concave peripheral iris—this is the characteristic iris
14. Which of the following does not cause angle-closure configuration in pigm ent dispersion syndrom e and
glaucom a? pigm entary glaucom a
c. RD—does not cause angle closure; the others do: PHPV
and choroidal effusion by a posterior pushing 26. A 60-year-old m yope with early cataracts and enlarged
m echanism and ICE syndrome by an anterior pulling cup-to-disc ratios of 0.6 OU is found to have an
m echanism abnorm al Hum phrey visual field test OS. He has no

387
Answers to Questions

other risk factors for glaucom a. What is the m ost 37. Which optic disc finding is m ost likely to indicate
appropriate next step for this patient? progression of glaucom a?
a. repeat visual fields—any initial abnorm al glaucom a d. splinter hem orrhage—a disc hem orrhage indicates
field test or interval change in field should be glaucom atous dam age and is often followed by
confirm ed by repeating the test notching

27. Bilateral scattered PAS in an elderly hyperope with no past 38. The m ost likely risk factor for phacom orphic glaucom a in a
ocular history is m ost likely due to patient with brunescent cataracts and narrow angles is
c. chronic angle-closure glaucom a d. hyperopia—hyperopic eyes tend to be sm aller with
shorter anterior cham ber depths, which is further
28. According to the CIGTS 5-year results, initial treatm ent of shortened with the thickening of the lens from
POAG with which two m ethods had sim ilar visual field nuclear sclerosis
outcom es?
b. m edicine or trabeculectom y 39. A patient with an anterior cham ber IOL presents with
ciliary block. Exam shows a patent iridectom y. Which of
29. Which of the following m edications should not be used the following is the m ost appropriate treatm ent option?
to treat a patient with HSV keratouveitis and elevated a. topical cycloplegic—to relax the ciliary m uscle and
IOP? m ove the lens and iris posteriorly
a. pilocarpine—m iotics can exacerbate the inflam m ation
and increase the risk of developing posterior synechiae 40. A m echanic presents one week after an eye injury with
decreased vision, redness, and eye pain. Exam reveals a
30. A patient undergoes m ultiple subconjunctival injections self-sealing corneal laceration, elevated IOP, and white
of 5-FU after glaucom a filtration surgery. The m ost fluffy m aterial in the anterior cham ber. The m ost likely
com m on reason for discontinuing these injections is if diagnosis is
the patient develops toxicity of which tissue? b. lens-particle glaucom a
c. cornea—corneal epithelial toxicity (ie, superficial
punctate keratitis, epithelial defects, ulcers) is the
m ost com m on com plication of subconjunctival 5-FU
injections CHAPTER 10 ANTERIOR S EGMENT
31. A patient with retinoblastom a develops glaucom a. The
1. The m ost helpful test for evaluating m acular function in a
m ost com m on m echanism is patient with advanced cataract is
c. neovascular—NVG (due to retinal ischem ia) is the b. 2-light separation—the others test gross function
cause of $ 75% of RB-associated glaucom a cases; the
other m echanism is secondary angle closure due to 2. Ectopia lentis is least likely to be associated with
anterior displacem ent of the lens-iris diaphragm a. cleft palate—is not associated with lens subluxation;
the other abnorm alities are pectus excavatum in
32. Glaucom a due to elevated episcleral venous pressure Marfan’s syndrom e, short stature in Weill-Marchesani
occurs in all of the following except syndrom e, and m ental retardation in hom ocystinuria,
b. hyphem a—glaucom a is caused by red blood cells sulfite oxidase deficiency, and hyperlysinem ia
clogging the trabecular m eshwork
3. Anterior segm ent signs of ciliary body m elanom a include
33. The earliest color deficit in glaucom a is loss of all of the following except
d. blue-yellow axis—this is the rationale for using short- a. corneal edem a—does not occur; however, ciliary body
wavelength autom ated perim etry (SWAP) visual field m elanom a can cause increased IOP from angle closure
testing for early glaucom a (posterior pushing m echanism ), and astigm atism and
34. Blood in Schlem m ’s canal is not associated with cataract from m echanical effects on the crystalline lens
a. Fuchs’ heterochrom ic iridocyclitis—there is no blood 4. Which of the following is least characteristic of ICE
in Schlem m ’s canal, but fine-angle neovascularization syndrom e?
occurs and m ay cause spontaneous hyphem a b. increased IOP
(Am sler sign)
5. A stellate anterior subcapsular cataract is m ost likely to be
35. According to the OHTS conclusions, a predictive factor for found in a patient with
developing POAG is a central corneal thickness of less than d. electrical injury—the other entities cause different types
or equal to how m any m icrons? of cataract: atopic derm atitis (ASC), m yotonic
b. 555 dystrophy (Christm as tree cataract), Fabry’s disease
(spoke-like cataract)
36. Which index on Hum phrey visual field testing is m ost
helpful for determ ining progression of visual field loss? 6. Which of the following does not occur in siderosis bulbi?
c. pattern standard deviation —a m easure of the change in c. sunflower cataract—is seen in conditions with
shape of the field from the expected shape for a abnorm al copper (not iron) deposition (ie, Wilson’s
norm al field disease and chalcosis)

388
 Answers to Questions

7. A patient has a history of increased IOP with exercise; 18. After finishing phacoem ulsification on a dense cataract,
which finding is associated with this condition? the surgeon notes whitening of the clear corneal
a. Krukenberg spindle—these are both signs of pigm ent incision. The m ost likely cause is
dispersion syndrom e a. tight incision—causes reduced irrigation and
insufficient cooling of the phaco needle resulting in a
8. Separation between the longitudinal and circum ferential
wound burn
fibers of the ciliary m uscle is called
b. angle recession—this is the definition; iridoschisis is 19. Nuclear brunescence increases with higher concentrations
separation of the iris surface, iridodialysis is separation of which lens protein?
of the iris root from its insertion, and cyclodialysis is d. m ain intrinsic polypeptide
separation of the ciliary body from the scleral spur
20. Lens fibers contain nuclei in all of the following conditions
9. Characteristics of pigm ent dispersion syndrom e include all except
of the following except b. syphilis
c. phacodenesis
21. The m ost likely cause of an intraoperative com plication
10. A sickle cell patient with a hyphem a develops increased during cataract surgery in a patient with
IOP; which of the following treatm ent choices is best? pseudoexfoliation syndrom e is
d. timoptic—is the safest m edication because it does c. weak zonules
not affect sickling; miotics destabilize the blood-
aqueous barrier, CAIs decrease aqueous pH (leading to 22. The m ajority of glucose m etabolism in the lens is by
sickling), and hyperosmotics cause hemoconcentration a. glycolysis
and sickling 23. The m ost appropriate system ic treatm ent for a patient with
a sunflower cataract is
11. Of the following causes of iris heterochrom ia, the involved
b. penicillam ine—to reduce serum copper levels
iris is hyperchrom ic in
a. ICE syndrom e—pigm ented iris nodules (pseudonevi) 24. Which of the following is the least likely cause of decreased
can occur; the involved iris is hypochrom ic in the vision 2 years after cataract surgery?
other three conditions d. cystoid m acular edem a—occurs in the first 2 m onths
after surgery
12. Which of the following iris lesions is a true tum or?
c. Lisch nodule—is an iris ham artom a associated with 25. Which type of cataract is m ost closely associated with UV-B
neurofibrom atosis; Kunkm ann-Wolffian body is exposure?
com posed of norm al iris strom a, Koeppe nodule is b. cortical
com posed of inflam m atory cells, and nodules in JXG
26. Which of the following strategies is least likely to be effective
are granulom atous infiltrates
for treating IFIS?
13. At which location is the lens capsule thinnest? c. m anual pupil stretching—the iris is floppy and atonic
b. posterior capsule so stretching the pupil will not im prove IFIS.
However, this strategy is helpful in cases of a rigid or
14. Which of the following is not associated with sunset fibrotic pupil such as in pseudoexfoliation syndrom e
syndrom e? or chronic uveitis
d. hyphem a—is not associated with inferior decentration
of an IOL; polyopia can occur if the edge of the lens is 27. Ocular m elanom a is least likely to occur in which portion
within the pupil; asym m etric haptic placem ent and of the uvea?
weak zonules (which occur in pseudoexfoliation) are b. iris—this is the least likely site of developing a uveal
risk factors m elanom a; conjunctival m elanom a is the rarest
ocular m elanom a (followed by eyelid m elanom a,
15. Lens epithelial cells differentiate into lens fibers then uveal m elanom a) and only accounts for 2% of
a. anterior to the equator ocular m alignancies
16. Light of which wavelength is absorbed greatest by a dense 28. A patient reports acute pain while ham m ering m etal and
nuclear sclerotic cataract? presents with 20/20 vision, subconjunctival hem orrhage,
d. blue—this is why patients notice that blues and purples and sm all hyphem a OD. The m ost appropriate test to
are especially vibrant after cataract surgery obtain is
d. CT—this patient has a presum ed m etallic intraocular
17. A patient with background diabetic retinopathy and foreign body
clinically significant m acular edem a desires cataract
surgery. The m ost appropriate m anagem ent is 29. A patient with a traum atic cataract requires cataract
b. focal laser treatm ent then cataract surgery—it is surgery. Which of the following is m ost helpful?
im portant to treat and stabilize the preexisting a. laser interferom eter—tests m acular potential in
m acular edem a prior to cataract surgery, which can patients with m edia opacities and would be the m ost
exacerbate it helpful for evaluating potential visual acuity

389
Answers to Questions

30. Pseudoexfoliation syndrom e and pigm ent dispersion 40. In which situation does optical biom etry have the largest
syndrom e share which of the following findings? advantage over ultrasound biom etry in determ ining an
b. pigm ented trabecular m eshwork—increased pigm ent accurate m easurem ent?
on the angle structures is com m on to both syndrom es c. high m yopia—m ay have posterior staphylom a, which
is m ore accurately m easured with optical biom etry;
31. The m ost likely cause of m onocular diplopia after blunt ultrasound biom etry has an advantage in patients
traum a is with dense posterior capsular and m ature cataracts
c. lens subluxation
41. During phacoem ulsification a m ilky substance (lens m ilk
32. Which of the following tests is m ost helpful in a patient
or lens dust) appears. What is the m ost appropriate next
with a Morgagnian cataract?
step?
b. HbA1C—diabetics are m ore likely to develop cortical
a. check for tight incision—lens m ilk/dust is a sign of not
(and posterior subcapsular) cataracts, and the risk of
enough irrigation and if not corrected a wound burn
cataract form ation increases with elevated blood
m ay rapidly occur, so the first step should be to check
sugar levels
if the incision is too tight thereby com pressing the
33. A truck driver desires the best range of vision after cataract sleeve or the phaco tip is obstructed
surgery. Which IOL strategy is m ost appropriate?
42. Which of the following is the m ost likely com plication of
c. m ini-m onovision with accom m odating lens—
Nd:YAG laser posterior capsulotom y?
Crystalens with m ini-m onovision will achieve the
c. retinal detachm ent
best range of vision with the least risk of glare;
m ultifocal lenses should be used with caution in 43. A patient with vitreous to the wound is m ost at risk for
professional drivers because of nighttim e glare which postoperative com plication?
d. cystoid m acular edem a
34. One week after cataract surgery, a patient has increased
anterior cham ber cells and flare, IOP of 36 m m Hg, and 44. On postop day 1 after cataract surgery, exam shows wound
a nuclear fragm ent in the inferior angle. The m ost gape and a flat anterior cham ber. The m ost appropriate
appropriate treatm ent option is treatm ent is
d. rem ove the retained fragm ent—retained nuclear c. suture wound—a significant wound leak requires
m aterial in the anterior cham ber are poorly tolerated sutures or ocular sealant
(even sm all fragm ents) and m ust be rem oved if
corneal edem a, iritis, elevated IOP or CME develops 45. A silicone IOL should not be used in a patient with
b. proliferative diabetic retinopathy—a patient who m ay
35. Which of the following tools is m ost helpful when require silicone oil for retinal detachm ent repair
perform ing surgery on a patient with a m ature cataract? should not have a silicone IOL because silicone oil
a. Trypan blue—capsular stain to aid in the creation of the can adhere to the IOL
capsulorhexis
46. A patient with a posterior subcapsular cataract desires
36. Glaukom flecken is caused by surgery. What is the m ost appropriate reason for
d. ischem ia—elevated IOP from angle-closure causes perform ing cataract surgery?
ischem ia and necrosis of lens epithelial cells, a. patient com plains of glare and difficulty reading—
appearing as central, subcapsular, white dots and flecks indication for cataract surgery is subjective and
37. Which of the following sym ptom s is least associated with depends on patient sym ptom s
posterior subcapsular cataracts? 47. An anterior subcapsular cataract is m ost likely to develop
b. poor blue discrim ination—this is a characteristic of in a patient with which skin condition?
nuclear sclerosis d. atopic derm atitis
38. A piggyback IOL is m ost likely to be necessary in which of 48. Reduced color discrim ination is m ost likely caused by
the following conditions? which type of cataract?
c. high hyperopia—a patient with high hyperopia is m ost c. nuclear sclerotic
likely to require a prim ary piggyback lens (needs IOL
power > +34 D) or secondary piggyback lens (due to 49. Which of the following is m ost likely to occur following
refractive surprise) cataract surgery in a patient with previous m yopic LASIK?
b. hyperopia—secondary to incorrect keratom etry
39. A 77-year-old wom an with cataracts and m acular readings (true curvature is flatter than m easured)
degeneration is interested in cataract surgery. The m ost
helpful test for evaluating this patient is 50. A patient with no visual com plaints is noted to have a
d. potential acuity m eter— the m ost useful test for norm al eye exam except for chronic pupillary capture of
evaluating the benefit of cataract surgery in this an IOL. What is the m ost appropriate m anagem ent?
patient is the PAM, which projects im age of letter a. observation—if the patient is asym ptom atic and
chart onto retina to test m acular potential in patients there are no ocular sequelae, then patient should
with m edia opacities be observed.

390
 Answers to Questions

51. One day after uncom plicated cataract surgery with 59. A 32-year-old m an presents 1 week after blunt traum a
phacoem ulsification, the exam shows counting fingers with 20/20 visual acuity and a dilated, unresponsive
vision, 3 + corneal edem a with Descem et’s folds, and pupil OD. The m ost appropriate next step is
severe anterior cham ber reaction with fibrinous m aterial. a. careful slit lam p exam of iris—traum atic m ydriasis
The m ost likely diagnosis is caused by sphincter tears is the m ost likely
c. toxic anterior segm ent syndrom e—a form of sterile diagnosis so a careful slit lam p exam should be
endophthalm itis perform ed first

52. A patient with high hyperopia is at increased risk for which 60. Which of the following IOL design is m ost appropriate for
of the following com plications of cataract surgery? placem ent in the sulcus?
d. choroidal effusion—short axial length increases the d. 13.5 m m polym ethylm ethacrylate—the best IOL
risk of choroidal effusion/hem orrhage from cataract design for the ciliary sulcus is a 3-piece foldable lens
surgery or a 1-piece PMMA lens that is posteriorly vaulted
with an overall length of 13.5 m m and optic
53. The largest risk for capsule rupture during hydrodissection diam eter of at least 6 m m
occurs with
c. posterior polar cataract—hydrodissection should not
be perform ed in the presence of a posterior polar CHAPTER 11 P OSTERIOR S EGMENT
cataract because of the high risk of a posterior
capsular defect, instead, only hydrodelineation 1. Which substance does not cause crystalline deposits in the
should be perform ed. Mature cataract increases the retina?
risk of an anterior capsular tear during capsulorhexis, a. thioridazine
and pseudoexfoliation syndrom e and sm all pupil are
risk factors for capsular rupture during 2. The finding m ost predictive of visual acuity in a patient
phacoem ulsification and I/A with PDR is
b. m acular edem a
54. After hydrodissection you notice capsular block. The next
step is to 3. The MPS showed the best prognosis for laser treatm ent of
d. decom press the nucleus and lift the anterior capsule— CNV in which disorder?
this technique is used to reverse capsular block d. POHS—The MPS studied laser treatm ent of CNV in
patients with AMD, POHS, and idiopathic
55. Which of the following m easures is m ost likely to reduce m em branes; POHS patients had the best response to
the risk of post-occlusion surge during laser treatm ent; patients with CNV secondary to
phacoem ulsification? m yopia and angioid streaks were not studied
b. reduce m axim um vacuum setting
4. Which of the following is not a feature of Stickler’s
56. Which of the following IOL designs is m ost likely to be syndrom e?
associated with a com plication of laser posterior c. retinoschisis
capsulotom y? 5. Sites at which the uvea is attached to the sclera include all
c. silicone plate haptic—this type of IOL has been of the following except
associated with posterior dislocation after laser d. ora serrata
capsulotom y, particularly if perform ed shortly after
cataract surgery 6. A patient with m ultifocal choroiditis and retinal vasculitis
is m ost likely to have
57. A patient with previous RK surgery undergoes d. sarcoidosis
uncom plicated phacoem ulsification. Two weeks after the
cataract surgery, the patient states that her vision is 7. All 3 types of retinal hem orrhage (preretinal, intraretinal,
blurry and unchanged. Her refraction is +2.00 + 0.50x30, and subretinal) m ay occur sim ultaneously in all of the
which im proves her vision to 20/20. At this point, the following conditions except
m ost appropriate m anagem ent option is c. diabetes—diabetic retinopathy can cause preretinal and
a. observation—refraction m ay take over 1 m onth to intraretinal hem orrhages but does not cause subretinal
stabilize after cataract surgery in patients with hem orrhage
previous corneal refractive surgery, especially post RK 8. Characteristics of choroidal m elanom a include all of the
where hyperopic refraction is com m on due to following except
corneal edem a b. high internal reflectivity
58. What is the m ost appropriate treatm ent for a patient 9. Which of the following statem ents regarding the ETDRS is
with a hyphem a and persistent elevated IOP for 1 week false?
despite m axim al m edical therapy? a. The ETDRS concluded that PRP reduces the risk of
d. anterior cham ber washout—uncontrolled increased severe visual loss in patients with high-risk PDR—this
IOP is an indication for AC washout for hyphem a conclusion is from the DRS

391
Answers to Questions

10. The DRVS found that early vitrectom y for vitreous 25. The ERG oscillatory potential is caused by which cell type
hem orrhage was helpful in b. am acrine
a. type 1 diabetic patients
26. Chloroquine retinopathy
11. Which of the following treatm ents is a CVOS b. can be associated with other CNS reactions—including
recom m endation? tinnitus
c. PRP for iris or angle neovascularization
27. Proven system ic control for diabetic retinopathy include
12. Which peripheral retinal lesion has the m ost risk of a d. blood pressure control—shown in the United Kingdom
retinal detachm ent? Prospective Diabetes Study
d. lattice degeneration
28. Retinal artery m acroaneursysm s
13. The intraocular structure m ost com m only affected by b. produce m ultilayered hem orrhages
leukem ia is
29. The Branch Vein Occlusion Study reported that
d. optic nerve
b. focal laser photocoagulation decreased visual loss—
14. Which is not a function of the RPE? especially when vision was worse than 20/40
b. concentration of taurine
30. True statem ents about ocular photodynam ic therapy
15. Which of the following best describes the cellular reaction include all of the following except
when light strikes a photoreceptor? c. often requires retreatm ent every 6 weeks—actually it is
c. decreased cGMP, closed Na channel every 3 m onths, early retreatm ent was not shown to be
beneficial
16. Prognostic factors for choroidal m elanom a include all of
the following except 31. Cystoid m acular edem a
d. pigm entation a. does not leak fluorescein when associated with
nicotinic acid
17. Which of the following does not involve the outer
plexiform layer of the retina? 32. PDR is m ost likely to develop if which one of the following
d. diabetic m icroaneurysm findings is present on fundus exam ?
d. venous beading
18. The best test for distinguishing between a subretinal
hem orrhage and a choroidal m elanom a is 33. Which of the following is the biggest risk factor for AMD?
a. FA—blockage occurs from the hem orrhage, and tum or a. sm oking
circulation is visible in the m elanom a
34. The m ost im portant visual prognostic factor for a
19. Which of the following is the least radiosensitive lesion? rhegm atogenous RD is
d. m elanom a c. m acular involvem ent—m acula-on has a better
prognosis than m acula-off
20. Which of the following is not a com plication of PRP?
c. retinal detachm ent 35. The earliest sign of a m acular hole is
c. yellow spot in the fovea—stage 1a hole
21. A cluster of pigm ented lesions is seen in the peripheral
retina of a patient’s left eye during routine 36. Focal laser treatm ent is indicated for diabetic m acular
ophthalm oscopy. Which of the following tests would edem a when there is
be m ost helpful in detecting an associated hereditary d. retinal edem a within 500 µm of the center of the fovea
disorder?
37. Reduced IOP would be m ost unexpected in a patient with
b. colonoscopy—check for polyps (fam ilial adenom atous
b. choroidal hem orrhage—choroidal hem orrhage causes
polyposis, Gardner’s disease)
increased IOP
22. Which of the following is not associated with a typical 38. Which of the following is the correct indication for treating
angiographic appearance of CME? m acular edem a from a branch retinal vein occlusion?
a. nicotinic acid—this m aculopathy clinically appears like d. vision worse than 20/40 for greater than 3 m onths
CME, but there is no fluorescein filling of the cysts;
other entities that fall into this category are juvenile 39. Which test is best for distinguishing a choroidal m elanom a
retinoschisis, Goldm ann-Favre disease, and som e from a choroidal hem angiom a?
types of RP c. ultrasound

23. The least likely finding in a patient with a chronic 40. The etiology of vitreous hem orrhage in Terson’s
detachm ent of the inferotem poral retina is syndrom e is
c. fixed folds—m ay occur from PVR, whereas the other a. intracranial hypertension—from a subarachnoid or
three findings are com m on with chronic RD subdural hem orrhage

24. In a patient with AMD, which type of drusen is m ost 41. The m ost com m on site of m etastasis for a choroidal
associated with developm ent of CNV? m elanom a is
a. soft b. liver—92% of m etastases are to liver

392
 Answers to Questions

42. Which of the following is least characteristic of Eales 47. The m ost likely diagnosis in a patient with choroidal
disease? neovascular m em brane in the m acula, peipapillary
d. m acular edem a atrophy and punched-out chorioretinal scars is
c. presum ed ocular histoplasm osis syndrom e
43. Crystalline retinopathy is not associated with
c. thioridazine—causes a pigm entary retinopathy 48. The m ost com m on com plication of an epiretinal
m em brane is
44. Com bined ham artom a of the retina and RPE has been
b. cystoid m acular edem a—ERM m ost com m only causes
associated with all of the following except
chronic CME
d. Gardner syndrom e
49. The m ost helpful test to obtain in a patient with a branch
45. All of the following statem ents regarding uveal m etastases
retinal artery occlusion is
are true except
d. carotid ultrasound—90% of BRAO is caused by em boli
b. m ost com m on ocular location is anterior choroid—
so patients should have a com plete cardiovascular
usually m etastasize to m acula because of richest
workup
blood supply
50. Angioid streaks are associated with all of the following
46. The m ost worrisom e sign in a patient with an acute
system ic conditions except
posterior vitreous detachm ent is
c. Syphilis
a. pigm ent in the vitreous—also known as tobacco dust is
associated with a retinal tear

393
This pa ge inte ntiona lly le ft bla nk
 Additional Readings

Albert, W. M., & Jakobiec, F. A. (2008). Principles and practice of Spaeth, G. L., Danesh-Meyer, H., Goldberg, I., & Kam pik, A.
ophthalmology (3rd ed.). Philadelphia: WB Saunders. (2011). Ophthalmic surgery principles and practice (4th ed.).
Kaiser, P. K., Friedm an, N. J., & Pineda, R., II (2014). The Philadelphia: Saunders.
massachusetts eye & ear infirmary’s illustrated manual of Spalton, D. J., Hitchings, R. A., & Hunter, P. A. (2004). Atlas of
ophthalmology (4th ed.). Philadelphia: Saunders. clinical ophthalmology (3rd ed.). St. Louis: Mosby.
Kanski, J. J. (2015). Clinical ophthalmology (8th ed.). Tabbara, K. F., Abu El-Asrar, A. M., & Khairallah, M. (2014).
Philadelphia: Saunders. Ocular infections. New York: Springer.
Levin, L. A., Nilsson, S. F. E., Ver Hoeve, J., et al. (2011). Adler’s Tasm an, W., & Jaeger, E. A. (2013). Duane’s ophthalmology.
physiology of the eye (11th ed.). Philadelphia: Saunders. Philadelphia: Lippincott William s & Wilkins.
Mannis, M. J., MacSai, M. S., & Huntley, A. C. (1996). Eye and Vaughan, D., & Asbury, T. (2011). General ophthalmology (18th
skin disease. Philadelphia: Lippincott-Raven. ed.). Colum bus, OH: McGraw-Hill.
Pepose, J. S., Holland, G. N., & Wilhelm us, K. R. (1996). Ocular Weingeist, T. A., & Gold, D. H. (2001). Color atlas of the eye in
infection and immunity. St. Louis: Mosby. systemic disease. Philadelphia: Lippincott William s & Wilkins.
Roy, F. H. (2002). Ocular differential diagnosis (7th ed.). Yanoff, M., & Duker, J. S. (2013). Ophthalmology (4th ed.).
Philadelphia: Lippincott William s & Wilkins. Philadelphia: Saunders.

395
This pa ge inte ntiona lly le ft bla nk
 Index

Note: Page num bers followed by f indicate figures, t indicate tables, and b indicate boxes.

A Acute postoperative endophthalm itis, 252 Albendazole, 30

Acute retinal necrosis (ARN), posterior uveitis and, Albinism , 129
A and V patterns, 153–154, 153f 245, 245f Alcian blue, 40
Abducens nerve (CN6), 63f, 70f, 162f Acute retinal pigm ent epitheliitis, 251, 251f Alexia, 80
palsy, 66–67, 67f Acute unilateral follicular conjunctivitis, 216 with agraphia, 80
Aberrant regeneration, oculom otor nerve palsy, 64 Acute zonal occult outer retinopathy (AZOOR), 251 without agraphia, 80
Ablepharon, 98 Acyclovir, 30 Alizarin red stain, 40
Abney effect, color vision, 11 clinical study of, 217–218b Alkali burns, 45
Abnorm al doll’s head, 58 Adenocarcinom a of cornea, 209
Abnorm al light response, 72 of ciliary body, 288 Alkaptonuria, 214
Abnorm al near response, 72 RPE, 360–361 Allergy
Abnorm al retinal correspondence (ARC), 143 Adenoid cystic carcinom a, of orbit, 179, 179f antihypertensive drugs and, 25
Abrasion Adenom a in conjunctival disorders, 201–202
in contact lens-related problem s, 227 of ciliary body, 288 Alpha crystallins, of lens, 289
of cornea, 209 RPE, 360–361 Alpha-chym otrypsin induced secondary open-angle
Absorption, of light, 2 Adenovirus, conjunctivitis and, 203 glaucom a, 271
AC/A ratio, 16 Adie’s syndrom e, 73 Alphagan P (brim onidine), 24
Acantham oeba, corneal infections and, 220 Adie’s tonic pupil, 72–73 Alport’s syndrom e, 108, 126
Acantholysis, 43 Adipose tissue, 165 Alstrom ’s disease, 125
Acanthosis, 43 Adnexa, 161–196 ALT. See Argon laser trabeculoplasty (ALT)
Accom m odation anatom y of, 161–170 Altitudinal defect, 54
am plitude of, 10 Adrenergic drugs, 24 Alveolar rhabdom yosarcom a, 95
of lens, 289 sym patholytics, 24 Am acrine cell, 303f
telescopes, 8, 16 sym pathom im etics, 24 Am aurosis fugax, 83
theories of, 11 Adrenergic receptors, 23 Am blyopia, 144
Accom m odative dem and, contact lenses, 12–13 Adult nonbanded layer, in Descem et’s m em brane, Am byloscope, for ARC, 143
ACh receptor antibody, ophthalm oplegia diagnosis, 198 Am etropia, correction of, 10
74 Adults, prescription of glasses, 12 Am ikacin, retinal toxicity, 349
Achrom atism , 130 Advanced Glaucom a Intervention Study (AGIS), Am inocaproic acid, 30
Achrom atopsia 277–279b Am inoglycosides, 29
cerebral, 81 Aflibercept, 322 retinal toxicity, 349
congenital, 121 Aflibercept for Age-Related Macular Degeneration Am iodarone
electroretinogram , 308 Study (VIEW 1/2), 326 ocular toxicity and, 32
Acid burns, 45, 209 Afterim age test, for ARC, 143 toxic cataracts and, 290
Acid fast stain, 39 Afterim ages, color vision, 11 Am orphous corneal dystrophy, posterior, 225
Acne rosacea, of eyelids, 181 "Against-the-rule," astigm atism and, 10 Am photericin B, 30
Acquired astigm atism , 10 Age-Related Eye Disease Study (AREDS), 325 Am plitude of accom m odation, 10
Acquired cataracts, 289–291 Age-Related Eye Disease Study 2 (AREDS2), 325 Am sler grid, 54
Acquired hyperopia, 10 Age-related m acular degeneration (ARMD, AMD), Am yloidosis
Acquired m yopia, 10 319–327 of conjunctiva, 200
Acquired nystagm us, 61–62 clinical studies, 323–327b of eyelids, 191
Acquired sebaceous gland hyperplasia, of eyelids, form s, 319, 319–321f prim ary, vitreous, 314
185 prognosis, 323–327 Anaesthetics, parenteral, 22
Acquired syphilis, 256, 256f treatm ent, 322 Anaplasia, eyelids, 43
Acquired tearing, nasolacrim al system disorders and, Aging, changes, 44 Aneurysm s, chiasm com pression, 79
191 AGIS. See Advanced Glaucom a Intervention Study Angioid streaks, 327–328, 328f
Actinic keratopathy, 213 (AGIS) Angiom atosis retinae, 135
Actinic keratosis, of eyelids, 186–187, 186f Agonist, m otor testing, 144, 144t Angle, em bryology of, 37
Acute bacterial conjunctivitis, 203 α2 -agonists, 24 Angle abnorm alities, 263
Acute follicular conjunctivitis, 203 Aicardi’s syndrom e, 129 Angle Kappa, m otor testing, 145, 145f
Acute inflam m ation, 42 AION. See Anterior ischem ic optic neuropathy Angle of m inim um deviation, prism s, 3
Acute posterior m ultifocal placoid pigm ent (AION) Angle recession, 45, 263, 284
epitheliopathy (APMPPE), 248, 248–249f, Air puff tonom eter, 264 Angle traum a, 284
248t Alagille’s syndrom e, 103 Angular artery, 169f

397
Index

Angular m agnification, 7, 16 Arden ratio, electro-oculogram , 308 Benign caruncle tum ors, 208–209
Angular vein, 169f Area of Martegiani, 302f Benign epithelial tum ors, of eyelids, 184
Aniridia, 105 em bryology of, 37 Benign essential blepharospasm , facial nerve palsy,
Aniseikonia, 7 Argon laser, cataracts and, 290 70
Anisocoria, 72 Argon laser trabeculoplasty (ALT), 275–276, 275f Benign hereditary intraepithelial dyskeratosis
sim ple, 73 Argyll-Robertson pupil, 73 (BHID), 208
Anisom etropia, 7 Argyrosis, 214 Benign lesions, pediatrics, 93–95
Ankyloblepharon, 98–99 Arteriovenous m alform ation (AVM), 84–85 Benign m ixed tum or, of orbit, 179, 179f
Ankylosin g spondylitis, 241 Artifactual RD, 41, 41f Benign reactive lym phoid hyperplasia, of
Annulus of Zinn, 165 ASC. See Anterior subcapsular cataracts (ASC) orbit, 177
Anom aloscope, 130 A-scan ultrasound, 310, 310f Benoxinate, 22
Anom alous trichrom atism , color blindness, 130 Aspergillosis, of orbit, 173 Bepotastine, 27
Anom ia, cortical lesions, 80 Asteroid hyalosis, 314 Berger’s space, 37, 302f
Anophthalm os, 92 ultrasound, 311 Bergm eister’s papillae, 36
Antagonist, m otor testing, 144 Asthenopia, conditions cause of, 11 Berlin nodules, 287
Anterior capsular contraction syndrom e, 297 Astigm atic keratotom y, 231–232 in anterior uveitis, 240
Anterior cham ber, inflam m ation in, 43 Astigm atism , 10 Berlin’s edem a, 315, 315f
Anterior cham ber (AC) angle, 283–288 acquired, 10 Best disease, 119–120, 120f
Anterior chiasm al syndrom e, 55 against-the-rule, 10 Beta crystallins, of lens, 289
Anterior clinoid, 163f classification of, 10, 10t Bevacizum ab (Avastin), clinical trials, 322
Anterior com m unicating artery, 51f of oblique incidence, 7 Bevacizum ab Elim inates the Angiogenic Threat of
Anterior ethm oidal artery, 163f with-the-rule, 10 Retinopathy of Prem aturity (BEAT-ROP)
Anterior facial vein, 169f Astrocytes, retina, 304 study, 116–117b
Anterior ischem ic optic neuropathy (AION), 76–77, Astrocytic ham artom a, 360 Bezold-Brucke phenom enon, 11
77f Astrocytom a BHID. See Benign hereditary intraepithelial
Anterior lim bal border, 284f grade I, 94–95 dyskeratosis (BHID)
Anterior segm ent, 283–300 low-grade, optic nerve gliom a, 78 Bielschowsky’s phenom enon, 151
disorders of, 283–288 Astronom ical telescope (Keplerian), 7, 7f Bietti’s crystalline retinopathy, 125, 126f
Anterior segm ent dysgenesis, 103 Ataxia-telangiectasia, 136 Bietti’s hyaline degeneration, 213
Anterior segm ent ischem ia, in strabism us surgery, Atopic derm atitis, cataracts and, 290 Bifocal add, prescription of glasses, 12
156 Atopic keratoconjunctivitis (AKC), 202 Bifocal glasses, prism atic effect of, 4
Anterior subcapsular cataracts (ASC), 290 Atresia of lacrim al puncta, 100 Bifocal segm ent, prism atic effect of, 4
Anterior uveitis, 239–243, 240f Atrophia bulbi, 173 Biguanides, 30
pediatrics, 110–111 Atrophic hole, retinal detachm ent, 354 Bilateral congruous central islands with vertical
Antiam oebics drug, 30 Atropine, 23 step, 56
Antibiotics, 28–29 Atypical lym phoid hyperplasia, of orbit, 177 Bilateral diffuse uveal m elanocytic proliferation
anti-tuberculosis agents of, 29 Atypical m ultifocal bilateral variant, of syndrom e (BDUMPS), 365
cell wall synthesis inhibitors of, 28 CHRPE, 131 Bilateral hom onym ous altitudinal defects, 56
interm ediary m etabolism inhibitors of, 28 Auditory clicks, 76 Bim atoprost (Lum igan), 25
ocular toxicity and, 31 AV fistula, 175 BIMVAT. See Binocular integrated m ulticolored
protein synthesis inhibitors of, 29 Avastin (Bevacizum ab), clinical trials, 322 vision assessm ent test (BIMVAT)
Anticholinergic drugs, ocular toxicity and, 31 AVM. See Arteriovenous m alform ation (AVM) Binasal defect, 55
Antifungal drugs, 30 Avulsion, vitreous base, 316 Binocular balance, prescription of glasses, 12
antim etabolites, 30 Axenfeld’s anom aly, 103 Binocular integrated m ulticolored vision assessm ent
azoles, 30 Axial hyperopia, 9 test (BIMVAT), 83
polyenes, 30 Axial m agnification, 16 Binocular vision, sensory testing in, 141, 141f, 142t
Antihelm intics drugs, 30 Axial m yopia, 9 Binocularity, types of, 141
Antihistam ines, 27 Axial versus refractive, 9 Bioavailability, 21
ocular toxicity and, 31 Azathioprine, 27 Biom icroscope, slit-lam p, 14, 14f
Anti-infective drugs, 28–30 Azelastine, 27 Bipolar cell, 303f
Antim alarial drugs, ocular toxicity and, 31 Azoles, 30 Birdshot choroidopathy (vitiliginous chorioretinitis),
Antim etabolites, trabeculectom y, 276–277 AZOOR. See Acute zonal occult outer retinopathy 248t, 250, 250f
Anti-Vascular Endothelial Growth Factor (VEGF) (AZOOR) Blepharitis, 180–181
Antibody for the Treatm ent of Predom inantly Blepharochalasis, 182
Classic Choroidal Neovascularization (CNV) Blepharophim osis, 99
in Age-Related Macular Degeneration B Blepharoptosis
(ANCHOR) trial, 322, 326 acquired, 182–183
Anti-VEGF agents, diabetic retinopathy therapy, 333 Bacitracin, 29 congenital, 99
Antiviral drugs, 29–30 Bacterial infections, of orbit, 173 Blepharospasm , 182
Anton’s syndrom e, 56 Bagolini lenses, for ARC, 143, 144f benign essential, 70
Apert’s syndrom e, 98 Ballasted lens, contact, 12 Blind spot, 54
Apertures, 161 Band keratopathy, 213 Blindness
Aphakia, congenital, 107 uveitis and, 257 cerebral, 85
Aphakic spectacles, 12 Barbiturates, ocular toxicity and, 31 cortical, 56, 85
Aplasia, 136 Baring of blind spot, 54 Bloch-Sulzberger syndrom e, 136, 136f
APMPPE. See Acute posterior m ultifocal placoid Basal cell carcinom a (BCC), of eyelids, 187, 188f β-blockers, 24
pigm ent epitheliopathy (APMPPE) Basal lam ina, of cornea, 198 Blood agar, 40
Applanation tonom etry, 15 Base curve m atching, prescription of glasses, 12 Blood-aqueous barrier, 261–262
Apraclonidine (Iopidine), 24 Basic lens form ula, vergence, 4 Blood-retinal barrier, 304
Aqueous deficiency, of cornea, 210 Basilar artery, 51f, 63f Blot hem orrhages, 304
Aqueous hum or Basilar layer, of eyelid skin, 166 Blue cone m onochrom atism , 121
com position of, 261 Bassen-Kornzweig syndrom e, 124, 154 Blue field entopic test, Purkinje vascular phenom ena,
functions of, 261 BDUMPS. See Bilateral diffuse uveal m elanocytic 54
production of, 261 proliferation syndrom e (BDUMPS) Blue sclera, 230
Aqueous m isdirection syndrom e, 274 Behçet’s disease, panuveitis and, 254 Blue-yellow disorders, color blindness, 130
Arachnoidal cells, 304 Behçet’s syndrom e, panuveitis and, 254f, 254b Blunt traum a, 44–45
Aralen, 349 Behr’s syndrom e, 138 Boat-shaped hem orrhages, 304
ARC. See Abnorm al retinal correspondence (ARC) Bell’s palsy Bodian stain, 40
Arcuate scotom a, 54 facial nerve palsy, 69 Botox, 182
Arcus juvenilis, 213 uveitis and, 257 Botryoid rhabdom yosarcom a, 95
Arcus m arginalis, 165 Bell’s phenom enon, 58 Botulinum toxin, 31
Arcus senilis, 213 Benedikt’s syndrom e, 64 in strabism us surgery, 156

398
 Index

Bouin’s fixative, 40 Cataract surgery (Continued) Chicken tracks, 107

Bourneville’s disease, 135–136 piggyback IOL, 293–294 Chickenpox, in cornea, 219
Bowm an ’s m em brane, 198, 284f viscoelastic device, 294 Childhood glaucom a, 109, 109f
Brain stem lesion, facial nerve palsy, 69 Cataracts, 289 Childhood nystagm us, 61
Brain tum ors, 81, 81t acquired, 289–291 Children, prescription of glasses, 12, 12t
Branch retinal artery occlusion (BRAO), 345 anterior subcapsular, 290 Chlamydia, ophthalm ia neonatorum , 101
Branch retinal vein occlusion (BVO), 340–341, 341f congenital, 107–109, 289 Chlam ydial infections, conjunctivitis and, 204
Branch Vein Occlusion Study (BVO S), 340–343 types of, 107–108 Chloram bucil, 27
BRAO. See Branch retinal artery occlusion (BRAO) cortical, 289, 289f Chloram phenicol, 29
BRAVO study, 341, 343–344 hyperm ature, 289 retinal toxicity, 349
Breaks, in Descem et’s m em brane, 198, 198f m ature, 289 Chlorom a, 97
Breast carcinom a, m etastases, of orbit, 180 Morgagnian, 289, 289f Chloroquine (Aralen), retinal toxicity, 349
Brim onidine, 24–25 nuclear sclerosis and, 290 Chlorprom azine (Thorazine), retinal toxicity, 349
Brinzolam ide-brim onidine (Sim brinza), 25 posterior subcapsular, 290 Chocolate agar, 40
Brown-McLean syndrom e, 226 toxic, 290 Cholesteatom a, erosive, 70
Brown’s syndrom e, 153 traum atic, 290 Cholinergic drugs, 23
Bruch’s m em brane, 263f, 304 Catechol-O-m ethyltransferase, 23 direct-acting agonists of, 23
Bruns’ nystagm us, 61 Cat-scratch disease, 247 indirect-acting agonists of, 23
Brushfield’s spots, 106 Cavernous hem angiom a m uscarin ic antagonists of, 23
B-scan ultrasound, 310, 311f choroidal, 358–359, 358–359f nicotinic antagonists of, 23
Bulbar conjunctiva, 197 of conjunctiva, 207–208 Cholinergic receptors, parasym pathetic nervous
Bullous keratopathy, 226 of eyelids, 190 system , 23
“Bull’s-eye” dystrophy, 121f of orbit, 176, 176f Choriocapillaris, 305, 305f
Buphthalm os, 92 Cavernous sinus syndrom e, 64–65, 65f Chorioretin opathy, 318–319, 318–319f
Burkitt’s lym phom a, 97 abducens nerve palsy, 66, 67f Choristom as
Burns, 209–210 Cavernous sinus throm bosis, 85 of conjunctiva, 205
Busacca nodules, 287 CCDF. See Central cloudy dystrophy of Francois orbit, 175–176
in anterior uveitis, 240, 240f (CCDF) Choroid, 302f
Busulfan, toxic cataracts and, 290 Cecocentral scotom a, 54 anatom y, 304–305
Butterfly-shaped dystrophy, 121 Cellophane m aculopathy, 317 em bryology of, 37
Buttonhole, in laser in situ keratom ileusis, 233 Cellulitis, preseptal, 92 reactive lym phoid hyperplasia and, 364
BVO. See Branch retinal vein occlusion (BVO) Center thickness, glasses m agnification, 7 rupture of, 45
BVOS. See Branch Vein O cclusion Study (BVOS) Central anesthesia, com plications of cataract surgery, Choroidal abnorm alities, 356–357
295 Choroidal cavernous hem angiom a, 358–359,
Central areolar choroidal dystrophy, 125, 125f 358–359f
C Central artery, 51f Choroidal detachm ent, 356, 357f
Central cloudy dystrophy of Francois (CCDF), ultrasound, 311t
CAIs. See Carbonic anhydrase inhibitors (CAIs) 225 Choroidal folds, 356
Calcarine fissure, 50f Central hom onym ous hem ianopia, 80 Choroidal hem angiom a, ultrasound, 311t
“Calcifying epitheliom a of Malherbe”, 186 Central nervous system (CNS) involvem ent, uveitis Choroidal hem orrhages, ultrasound, 311t
Calcofluor white stain, 39 and, 257 Choroidal ischem ia, 356–357
Caldwell view, of radiographs, 171 Central ray, m irrors and, 8 Choroidal m alignant m elanom a, 361–364,
Calorics, com a, 75 Central retinal artery, 161–163, 163f, 263f 361–362f
Canal of Hannover, 302f Central retinal artery occlusion (CRAO), 345–346, Choroidal m ass, ultrasound, 312f
Canalicular obstruction, nasolacrim al system 346f Choroidal neovascular m em brane (CNV), disorders,
disorders, 191 electroretinogram and, 307 327
Canaliculi, in nasolacrim al system , 170 Central retinal vein, 164, 164f Choroidal nevus, 357–358, 358f
Canaliculitis, 191–193 Central retinal vein occlusion (CVO), 341–342, ultrasound, 311t
Candidiasis, posterior uveitis and, 246 341–342f Choroidal osteom a, 359, 359f
Canthaxanthine, 350, 350f Central scotom a, 54 Choroidal rupture, 315, 315f
Canthom eatal line, of radiographs, 171 Central serous retinopathy (CSR), 318–319, Choroiderem ia, 127, 127f
Capillary hem angiom a, 93, 93f 318–319f Choroidopathy, 331
Capsular block syndrom e, com plications of cataract Central vein, 51f birdshot, 248t, 250, 250f
surgery, 297 Central Vein Occlusion Study (CVOS), 342, 344 Chrom atic effects, prism s and, 4
Capsular phim osis, 297 Cephalosporins, 28 Chrom atic lens, aberrations of, 7
Capsule, of lens, 288 Cerebellopontine angle tum ors, 81 Chronic angle closure, prim ary angle-closure
Capsulopalpebral fascia, 168 Cerebral achrom atopsia, cortical lesions, 81 glaucom a, 273
CAR. See Carcinom a-associated retinopath y (CAR) Cerebral aneurysm , 84 Chronic bacterial conjunctivitis, 203–204
Carbachol, 24 Cerebral blindness, 85 Chronic follicular conjunctivitis, 203
Carbapenem s, 28 Cerebral venous throm bosis, 85–86 Chronic inflam m ation, 43
Carbon dioxide, blood agar in 5% to 10%, 40 Chalcosis Chronic progressive external ophthalm oplegia
Carbonic anhydrase inhibitors (CAIs), 25 of cornea, 214 (CPEO), 73, 125, 154–155
Carcinom a in situ electroretinogram , 308 CHRPE. See Congenital hypertrophy of the retinal
of conjunctiva, 205, 205f intraocular foreign bodies and, 285 pigm ent epithelium (CHRPE)
eyelids, 43 secondary open-angle glaucom a, 272 Chrysiasis, 214
Carcinom a-associated retinopathy (CAR), 365 Chalcosis lentis, cataracts and, 290 CHSD. See Congenital hereditary strom al dystrophy
Cardinal points, of lenses, 5 Chandler’s syndrom e, 286 (CHSD)
Cardinal position s, m otor testing, 144, 144f Charged particle radiation, choroidal m alignant Cialis (Tadalafil), 351
Cardioselective β-blockers, 24 m elanom a, 362 Cicatricial ectropion, 183
Carm ustine, ocular toxicity and, 32 Check ligam ents, 139 Cicatricial entropion, 184
Carotid artery dissection, 85 Checkerboard field, 56 Cidofovir, for cytom egalovirus (CMV) infections,
Carotid ultrasound, for corneal arcus, 213 CHED. See Congenital hereditary endothelial 244–245
Carotidynia, 83 dystrophy (CHED) CIGTS. See Collaborative Initial Glaucom a Treatm ent
Carpenter’s syndrom e, 98 Chem ical burn, traum atic, secondary open-angle Study (CIGTS)
Caruncle, 197 glaucom a, 272 Ciliary body (CB)
Caruncle tum ors, of conjunctiva, 208 Chem ical injury, 45 anatom y/physiology of, 261–262, 262f
Cataract surgery, 292–295 Chem osis, of conjunctiva, 200 angle structures, 262–263, 262f
com plications of, 295–297 Cherry red spot, differential diagnosis, 346 em bryology of, 38
contact A scan, 293, 293f Chiasm , 49–50 functions of, 261–262
IOL calculations, 292–293 anatom y, 78–79 inflam m ation in, 43
keratom etry, 293 com pression of, 78–79 outflow pathways, 262
phacodynam ics, 294–295 Chiasm atic groove, 163f tum ors and, 288

399
Index

Ciliary body leiom yom a, 288 Confocal m icroscopy, for cornea, 199 Constricted field, 55
Ciliary epithelium , aging changes in, 44 Confocal scanning laser ophthalm oscopy (CSLO), Constructive interferen ce, 1
Ciliary ganglia, 52, 63f, 68f 266, 267f Contact A scan, cataract surgery and, 293, 293f
Ciliary m uscle, 261 Congenital achrom atopsia, 121 Contact derm atitis, of eyelids, 181
Ciliary processes, 261 Congenital anom alies, pediatrics, 92 Contact lenses (CL), 12–13
Ciliary spasm , 150 Congenital aphakia, 107 Contact lens-related problem s, 227
Ciliary vessels, 261 Congenital blepharoptosis, 99 Contact tim e, bioavailability, 21
Ciliolenticular block, 274 Congenital cataracts, 107–109 Contrast sensitivity, 9
Ciliospin al center of Budge, 52 types of, 107–108 tests for, 54
Ciliovitreal block, 274 Congenital cloudy cornea, differential diagnosis of, Contusion, cataracts and, 290
CIN. See Conjunctival intraepithelial neoplasia (CIN) 103b Convergence, vergence, 4
Circle of least confusion, spherocylindrical lens Congenital corneal staphylom a, 102 Convergence insufficiency, 151
and, 6 Congenital dichrom atism , 130 Convergence paralysis, secondary to intracranial
Circle of Zinn-Haller, 51f Congenital ectropion, 99 lesion, 151
City university test, 130 Congenital entropion, 99, 184 Convergence spasm , 74
Claude’s syndrom e, 64 Congenital fibrosis of inferior rectus, 154 Convergence-retraction nystagm us, 61
Clefts, in corneal strom a, 41 Congenital fibrosis syndrom e, 154 Convex m irrors, 8
Clindam ycin, 29 types of, 154 Coom bs and Gell classification, 41
Clinically significant m acular edem a (CSME), 332 Congenital hereditary endothelial dystrophy COPERNICUS, 342, 345
Clivus, 163f (CHED), 104, 226 Copper
Cloquet’s canal, 302f Congenital hereditary strom al dystrophy (CHSD), cataracts and, 290
em bryology of, 37 104 intraocular foreign body and, 45, 285
Clotrim azole, 30 Congenital Horner’s syndrom e, 99 Corectopia, 106, 286
Cluster headache, 83 Congenital hypertrophy of the retinal pigm ent Cornea, 197–238
CME. See Cystoid m acular edem a (CME) epithelium (CHRPE), 130–131, 130–131f, aging changes in, 44
CME (Irvine-Gass syndrom e), com plications of 131b anatom y/physiology of, 197–199, 198f
cataract surgery, 297 Congenital iris ectropion, 105 basal lam ina of, 198
CNTGS. See Collaborative Norm al Tension Congenital iris hypoplasia, 105 Bowm an’s m em brane of, 198
Glaucom a Study (CNTGS) Congenital m iosis, 106 conjunctival disorders of, 199–209
Coats disease, 117–118, 118f Congenital m ydriasis, 106 Descem et’s m em brane of, 198
Coat’s white ring, in cornea, 214 Congenital nystagm us, 147 em bryology of, 37
Cobblestone degeneration, 353, 353f Congenital ocular m elanosis, of conjunctiva, 207 endothelium of, 198–199
Cocaine, 22 Congenital rubella, electroretinogram , 308 epithelium of, 197–198
Cockayne’s syndrom e, 125 Congenital rubella syndrom e, 121–122, 122f innervation of, 199
Cogan-Reese syndrom e, 287 Congenital sebaceous gland hyperplasia, of eyelids, scleral disorders of, 229–230
Cogan’s dictum , 79 185 stains of, 199
Cogan’s senile scleral plaque, 230 Congenital stationary night blindness (CSNB), 126, strom a of, 198
Cogan’s syndrom e, 212 126f surgery for, 230–235
Coherence, of light, 1 electroretin ogram , 307f wound healing in, 44
Colchicine, 28 Congenital strom al corneal dystrophy, 225 Cornea farinata, 213
Collaborative Initial Glaucom a Treatm ent Study Congenital syphilis, 256 Cornea guttata, 226
(CIGTS), 278 Congenital tarsal kink, 99 Cornea plana, 102–103
Collaborative Norm al Tension Glaucom a Study Congenital tearing, nasolacrim al system disorders Cornea verticillata, 214
(CNTGS), 279 and, 191 Corneal arcus, 213
Collaborative Ocular Melanom a Study (COMS), Congenital tum ors, of conjunctiva, 205 Corneal blood staining, 44, 283
363, 363–364b Congenital unilateral fibrosis, 154 Corneal contact lens, 13f
Collagen, 44b Congo red, 40 Corneal disorders, 209–229
Collagen lam ellae, in corn eal strom a, 198 Conjunctiva burns in, 209–210
Colloidal iron stain, 40 anatom y, 197 degenerations in, 212–213
Colobom a, 99, 105, 112–113, 137 palpebral, 197 depositions in, 213–214
em bryology of, 35 Conjunctival derm oid cyst, 104 dystrophies in, 221–225
lens, 107 Conjunctival disorders, 199–209 ectasias in, 220–221
Color perception, 11 allergy in, 201–202 inflam m ation in, 43, 212
Color vision, 11 conjunctival telangiectasia in, 201 m iscellaneous, 226–229
tests for, 52–54 degenerations in, 200–201 ocular surface disease in, 210–211
Colorblindness, 129–130, 129t deposits in, 201 pediatrics, 102–105
Com a inflam m ation in, 199–200 traum a in, 209
eye m ovem ents in, 74–75 pediatrics, 100–102 ulcers in, 215–220
lens aberrations, 6 tum ors in, 204–209 Corneal dystrophy of Bowm an’s m em brane
Com bigan (brim onidine), 25 Conjunctival inclusion cyst, in strabism us surgery, type I, 223
Com bined ham artom a of the retina and RPE, 360, 156 type II, 223
360f Conjunctival intraepithelial neoplasia (CIN), 205 Corneal ectasia, in laser in situ keratom ileusis, 234
Com itant deviations, 148 Conjunctival scarring, in strabism us surgery, 156 Corneal edem a
Com m otio retinae (Berlin’s edem a), 45, 315, 315f Conjunctival telangiectasia, in conjunctival com plications of cataract surgery, 296
Com parison of AMD Treatm ent Trials (CATT), disorders, 201 epithelial, 198
326–327 Conjunctivitis Corneal filam ents, 198
Com plete rod m onochrom atism , 121 acute bacterial, 203 Corneal light reflex, in deviations, 145
Com plex choristom a, 102 acute follicular, 203 Corneal m elting, 215
Com poun d nevus allergic, 201 com plications of cataract surgery, 296
of conjunctiva, 206 bacterial infections and, 203 Corneal opacity, other causes of, 104
of eyelids, 186, 187f chronic follicular, 203 Corneal strom a, clefts in, 41
Com pressive optic neuropathy, 77 inclusion, 204 Corneal transplant
COMS. See Collaborative Ocular Melanom a Study infectious, 202–204 failure, 227
(COMS) pediatrics, 101–102 in traum atic, secondary open-angle glaucom a, 272
Concave m irrors, 8 Kawasaki’s disease, 102 Corneal ulceration, com plications of cataract surgery,
Concentration, bioavailability, 22 ligneous, 101–102 296
Concretions, of conjunctiva, 200 ophthalm ia neonatorum , 101 Corneal warpage, in contact lens-related problem s,
Conductive keratoplasty, 234–235 other infections in, 101 227
Cone cell, 303f vernal keratoconjunctivitis, 101 Corneoscleral m eshwork, 262
degeneration, 307f with system ic diseases, 202 Corresponding retinal points, 143
dystrophy, 308 viral infections and, 203 Corrugator m uscle, 167
Cone m onochrom atism , 130 Conjunctivochalasis, 200 Cortical blindness, 56, 85
Cone-rod degeneration, electroretinogram , 307f Conoid of Sturm , spherocylindrical lens and, 6, 6f Cortical cataracts, 289, 289f

400
 Index

Cortical lesions, 80–81 Degenerations Diuretics, ocular toxicity and, 32

Corticospinal tract, 63f in conjunctival disorders, 200–201 Divergence, vergence, 4
Corticosteroid-induced secondary open-angle in corneal disorders, 212–213 Doll’s head
glaucom a, 272 retinal abnorm alities, 351, 351t abnorm al, 58
Corticosteroids Degenerative senile retinoschisis, 352–353, 352f intact, 58
ocular toxicity and, 32 Delayed postoperative endophthalm itis, 252 testing, 59
toxic cataracts and, 290 Delle Donder’s table, accom m odation, 11, 11t
Cospot (dorzolam ide-tim olol), 25 in cornea, 226 Dorsal m idbrain syndrom e, 60
Cotton wool spot, 328 in strabism us surgery, 156 Dorsal nasal artery, 163f
Cowden’s disease, 186 Dendritic ulcer, 216 Dorzolam ide-tim olol (Cospot), 25
CPEO. See Chronic progressive external Dendritiform epithelial plaques, 219 Dot hem orrhages, 304
ophthalm oplegia (CPEO) Denervation, extirpation and, 155 Double elevator palsy, 153
Cranial nerve palsies, 62–71, 63f Dengue fever, 173 Double Maddox rod test, 146–147
Craniofacial cleft syndrom es, 97 Deposits, in conjunctival disorders, 201 Downbeat nystagm us, 61
Craniofacial disorders, pediatrics, 97–98 Derm atochalasis, 182 Down’s syndrom e, 108
Craniofacial dysostosis, 98 Derm oid cyst, 93, 104 Doyne’s honeycom b dystrophy, 120
Craniopharyngiom a, 79 of eyelids, 185 DR. See Diabetic retinopathy (DR)
Craniosynostoses, 98 Derm olipom a, of conjunctiva, 104 Drainage im plants, in glaucom a, treatm ent of, 277
CRAO. See Central retinal artery occlusion Descem etocele, 226 DRS. See Diabetic Retinopathy Study (DRS)
(CRAO) Descem et’s m em brane, 198, 284f Drug-induced eye m ovem ent disorders, 59
Critical angle, refraction and, 2 detachm ent of, 296 Drusen, 320
Crocodile shagreen, 213 rupture of, 45 size classification, 321
Crocodile tears, 70 Descem et’s tear/rupture, 104 DRVS. See Diabetic Retinopathy Vitrectom y Study
Crom olyn, 27 Destructive interference, 1 (DRVS)
Crouzon’s syndrom e, 98 Deuteranom aly, color blindness, 130 Dry eye, in laser in situ keratom ileusis, 234
CRUISE study, 342, 344–345 Deuteranopia, 130 DTD. See Dissociated torsional deviation (DTD)
Cryotherapy, com plications, 355 DHD. See Dissociated horizontal deviation (DHD) Duane’s retraction syndrom e, 154
Cryotherapy for ROP study (Cryo-ROP), Diabetes, cataracts and, 290 Ductions, m otor testing, 144
116–117b Diabetes Control and Com plications Trial (DCCT), Duochrom e test, prism s, 4
Cryptophthalm os, 92 333, 335–336 Dural sinus throm bosis, 85–86
Crystal violet, 40 Diabetic cataract, 332 Dural-sinus fistula, 175
Crystalline keratopathy, 216 Diabetic iridopathy, 332 DUSN. See Diffuse unilateral subacute neuroretinitis
CSLO. See Confocal scanning laser ophthalm oscopy Diabetic retinopathy (DR), 331–339, 331–333f (DUSN)
(CSLO) clinical studies, 334–339b DVD. See Dissociated vertical deviation (DVD)
CSME. See Clinically significant m acular edem a clinically significant m acular edem a and, 332 Dye disappearance test, 191
(CSME) diabetic cataract and, 332 Dyschrom atopsias, com parison of, 129t
CSNB. See Congenital stationary night blindness diabetic iridopathy and, 332 Dyscoria, 106
(CSNB) isolated cranial nerve palsies and, 332 Dyskeratosis, 43
CSR. See Central serous retinopathy (CSR) papillitis and, 332 Dyslexia, cortical lesions, 80
CT scan, 171, 171t treatm ent, 333 Dyslipoproteinem ias, 214
Curvature of field, 7 Diabetic Retinopathy Clinical Research Network Dysplasia, 43
CVOS. See Central Vein Occlusion Study (CVOS) (DRCR.net): Major Protocols Only, 337–339 Dystrophies
Cyclic oculom otor palsy, 64 Diabetic Retinopathy Study (DRS), 333–334 in cornea, 221–225
Cyclocryotherapy, 277 Diabetic Retinopathy Vitrectom y Study (DRVS), 333, epithelial and subepithelial, 221, 222f
Cyclodialysis, 45, 284f 335 strom al, 224
Cyclodialysis cleft, 263 Dialysis
Cyclophospham ide, 27 retinal detachm ent and, 354
Cyclophotocoagulation , 277 retinal tear and, 316 E
Cyclosporine, 27 Diam idines, 30
Cylinder transposition, lenses and, 6 Dichrom atism , congenital, 130 Eales’ disease, 340
Cylinders, lenses of, 6 Diethylcarbam azine, 30 Early Manifest Glaucom a Trial (EMGT), 278–279
Cyst Diffraction, of light, 1 Early receptor potential (ERP), electroretinogram ,
of eyelids, 185 Diffuse progressive retinal disease, electroretinogram 306
inclusion, 205 and, 308 Early Treatm ent Diabetic Retinopathy Study
pars plana, 352, 352f Diffuse scleritis, 229 (ETDRS), 333–335
sim ple, 205 Diffuse unilateral subacute neuroretinitis (DUSN), chart, 9
Cystic lesions, of iris, 287–288 348 Early Treatm ent for Retinopathy of Prem aturity
Cystic retinal tuft, 352 posterior uveitis and, 247 Random ized Trial (ETROP), 116–117b
Cystic tum ors, of conjunctiva, 205 Digoxin, toxicity and, 32, 350 Eaton-Lam bert syndrom e, 74
Cysticercosis Diktyom a, 106–107 EBV. See Epstein-Barr virus (EBV)
of orbit, 173 Dilantin, toxicity and, 31 Echothiophate, 24–25
posterior uveitis and, 246, 246f Dim ensions, 161 Ectasias, 220–221
Cystinosis, 122–123 Dim orphic fungi infections, 219 Ectopia lentis, 107
Cystoid m acular edem a (CME), 329, 329–330f Diopter, vergence, 4 lens capsule abnorm alities and, 291
Cytom egalovirus (CMV), posterior uveitis and, Dipivefrin, 24 Ectopic lacrim al gland, 102
244–245, 244f Diplopia, 58 Ectropion, 183
Cytotoxic therapy, for posterior uveitis, 244 sensory adaptations to, 142–143, 142–144f cicatricial, 183
in strabism us surgery, 156 congenital, 99
Direct carotid-cavernous sinus fistula, 175 involutional, 183
D Direct ophthalm oscope, 14, 14f m echanical, 183
Disciform keratitis, 217 paralytic, 183
Dacryocystitis, 193 Disciform lesion, ultrasound, 311t Eczem a, of conjunctiva, 202
Dacryocystocele, 100 Discoloration, of sclera, 230 Edem a
Dacryocystogram , 191 Disconnection syndrom e, retrochiasm al disorders, in contact lens-related problem s, 227
Dacryocystorhinostom y (DCR), 193 80 vascular disease, 328
Dacryoscintogram , 191 Dislocation, lens capsule abnorm alities, 291 Edinger-Westphal nucleus, 63f
Dark focus, night m yopia, 10 Dissociated horizontal deviation (DHD), 151–152 Egger’s line, 302f
Dark hem orrhage, 304 Dissociated nystagm us, 61 Ehlers-Danlos syndrom e
DCCT. See Diabetes Control and Com plications Trial Dissociated torsional deviation (DTD), 151–152 angioid streaks, 328
(DCCT) Dissociated vertical deviation (DVD), 151–152 ectopia lentis and, 292
DCR. See Dacryocystorhinostom y (DCR) Distance devices, low-vision aids and, 13 8-ball hyphem a, 283
De Morsier’s syndrom e, 137 Distichiasis, 99 EKC. See Epidem ic keratoconjunctivitis (EKC)
Deep filiform dystrophy, 225 Distortion, lens aberration s of, 7 Elastosis, 43, 44f

401
Index

Electrom agnetic spectrum , 2f Epithelial ingrowth Eyelid (Continued)

Electro-oculogram (EOG), 308, 309f in laser in situ keratom ileusis, 234 epithelial changes in, 43
Electroretinogram (ERG), 305–308 as wound com plication, 44 glands of, 170, 170t
com ponents, 306, 306f Epithelial keratitis, 216–217 innervation of, 169
disease states, 307–308, 307f, 308t Epithelial lacrim al gland tum ors, 179 lym phatic drainage of, 169
early receptor potential, 306, 306f Epithelial recurrent erosion dystrophies, 222, 222f m argin of, 169
indications, 307 Epithelial slough, in laser in situ keratom ileusis, 233 retraction of, 183
photopic (light adapted), 306–307 Epithelial-strom al (TGFBI) dystrophies, 223 tum ors of, 184–191
scotopic (dark adapted), 306–307f, 307 Epithelioid histiocyte, 42 vascular supply of, 169, 169f
Elevated episcleral venous pressure, traum atic, Epithelium Eyelid disorders, 180–191
secondary open-angle glaucom a, 272 of cornea, 197–198 infections and, 181–182
Elschnig’s spot, 357 of lens, 288 inflam m ation and, 180–181
Em bryology, 35–48 Epstein-Barr virus (EBV), 220 m alposition and, 182–184
angle, 37 ERG. See Electroretinogram (ERG) traum a and, 180
choroid, 37 Ergotam ine, retinal toxicity, 350
ciliary body, 38 Erosive cholesteatom a, 70
cornea, 37 Erythem a m ultiform e, of cornea, 211 F
em bryonic fissure, 35 Erythem a nodosum , uveitis and, 258
em bryonic plate, 35, 36f Esodeviations, 148 FA. See Fluorescein angiogram (FA)
eyelids, 38 Esophoria, 148 Fabry’s disease, 108, 122
form ation of eye, 35 Esotropia, 148, 148b Facial artery, 169f
hyaloid artery, 35–36, 36f accom m odative, 149–150 Facial colliculus, 163f
iris, 37–38 types of, 149t Facial hem angiom a, 135
lens, 37, 38f acquired, nonaccom m odative, 150 Facial m yokym ia, 70
nasolacrim al system , 38 congenital, 148–149 Facial nerve (CN 7) palsy, 67–70, 69f
optic cup, 35, 36f interm ittent, 148 Fam ilial drusen, 120
optic pit, 35 Essential iris atrophy, 286 Fam ilial exudative vitreoretinopathy (FEVR), 117, 117f
optic vesicle, 35 ETDRS. See Early Treatm ent Diabetic Retinopathy Fam ily history, pediatrics, 91
prim itive epithelial papillae, 36 Study (ETDRS) Far point, 10
retina, 37 Etham butol, 29 range of accom m odation of, 10
sclera, 37 Ethm oid bone, 162f Farnsworth-Munsell 100 hue test, 130
tim eline of, 39f Ethm oid sinus, 165 Farnsworth’s panel D-15, 130
tissues and their com ponents, 38 Ethm oidal foram ina, 161 Fat adherence syndrom e, in strabism us surgery, 156
vitreous, 36–37, 37f Ethyl alcohol, for tissue fixation, 40 Fat em boli syndrom e, 317
Em bryonal rhabdom yosarcom a, 95 ETROP. See Early Treatm ent for Retinopathy of Fat pads, preapon eurotic, 165
Em bryonic fissure, 35 Prem aturity Random ized Trial (ETROP) Ferry’s line, 214
Em bryonic plate, 35, 36f Euryblepharon, 100 Fetal alcohol syndrom e, 98
Em bryotoxin, posterior, 199 Evisceration, 193 Fetal banded layer, in Descem et’s m em brane, 198
Em edastine, 27 EVS. See Endophthalm itis Vitrectom y Study (EVS) FEVR. See Fam ilial exudative vitreoretinopathy
EMGT. See Early Manifest Glaucom a Trial (EMGT) Ewing’s sarcom a, 96–97 (FEVR)
Encephalotrigem inal angiom atosis, 135 Exenteration, 193 Fibrom a, ossifying, 178
Endogenous deposits, of conjunctiva, 201 Exodeviations, 148 Fibro-osseous tum ors, 178
Endogenous endophthalm itis, 252 Exogenous deposits, of conjunctiva, 201 Fibrosis
Endophthalm itis, 252–253 Exophoria, 148 congenital unilateral, 154
phacoanaph ylactic, 242–243 Exophthalm om etry m easurem en ts, 162t generalized, 154
Endophthalm itis Vitrectom y Study (EVS), Exophytic retinoblastom a, 132 Fibrosis syndrom e, congenital, 154
252–253b Exotropia, 148 Fibrous downgrowth, as wound com plication, 44
Endophytic retinoblastom a, 132 congenital, 151 Fibrous dysplasia, 94
Endothelial dystrophies, 225 consecutive, 151 Fibrous histiocytom a
Endothelial keratoplasty, 231 interm ittent, 148, 150 of conjunctiva, 208
Endotheliitis, 217, 219 sensory, 151 of orbit, 178, 178f
Endothelium , of cornea, 198–199 Exposure keratopathy, 226–227 Fibrous ingrowth, 228
Entropion, 183–184 Expulsive suprachoroidal hem orrhage, secondary angle-closure glaucom a, 274
cicatricial, 184 com plications of cataract surgery, 296 Filam entary keratitis, 212
congenital, 99, 184 External lim iting m em brane, 301, 303f Finnish fam ilial am yloidosis, 223
involutional, 184 Extirpation , denervation and, 155 Fish eye disease, 214
spastic, 184 Extraocular m uscles, 139–140, 140t, 140–141f Fit, evaluating, contact lenses and, 13, 13f
Enucleation, 193 Exudative RD, 355, 356f Fitting rigid CL, 13
choroidal m alignant m elanom a, 362 Eye(s) Flam e hem orrhages, 304
EOG. See Electro-oculogram (EOG) accom m odation of, 10–11 Flap dislocation, in laser in situ keratom ileusis, 233
Eosinophil suppressor, m ast cell stabilizer plus, 27 em bryology of, 35–48 Flap striae, in laser in situ keratom ileusis, 233
Eosinophilic granulom a, 97 m odel eye, 8 Fleck corneal dystrophy, 225
Eosinophils, 42 as optical system , 8–11 Fleischer ring, 214
Epiblepharon, 99 refractive error of, 9–10, 9f Fleurettes, retinoblastom a, 132
Epibulbar osseous choristom a, 102 schem atic, 8f Flexner-Wintersteiner rosette, retinoblastom a, 132,
Epicanthus, 99–100 vision m easurem ents of, 8–9 132f
inversus, 100 Eye m ovem ent disorders, 58–61 Floppy eyelid syndrom e, 184, 204
palpebralis, 100 central disorders (supranuclear), 58, 58f Fluconazole, 30
supraciliaris, 100 horizontal gaze palsies, 58–60 Flucytosine, 30
tarsalis, 100 vertical gaze abnorm alities, 60–61 Fluorescein, for cornea, 199
Epidem ic keratoconjunctivitis (EKC), 203 Eye m ovem ents Fluorescein angiogram (FA), 312–314, 313f
Epidem iology of Diabetes Interventions and in com a, 74–75 Fluorescein dye, 31
Com plications (EDIC) Trial, 336 horizontal gaze center, 56, 56–57f Fluoroquinolones, 29
Epiderm oid cyst, 93 nonoptic reflex system , 57–58, 57f 5-Fluorouracil, trabeculectom y, 276
Epikeratoph akia, 231, 232f position m aintenance system , 56 Focal laser, 366
Epikeratoplasty, 231, 232f saccadic system , 56 Focal length
Epinastine, 27 sm ooth pursuit system , 56 of lenses, 5
Epinephrine, 24 supranuclear control, 56–58 m irrors and, 8
Epiretinal m em brane, 317 vergence system , 56 Focal points, of lenses, 5
Episcleritis, 229 Eyelid Focal thickening, in Descem et’s m em brane, 198, 199f
Epithelial downgrowth anatom y of, 166–170 Follicles, inflam m ation of, 199
of cornea, 227–228, 227f differential diagnosis of com m on disorders of, 192t Fontana-Masson stain, 40
secondary angle-closure glaucom a, 274 em bryology of, 38 Foram en lacerum , 161, 163f

402
 Index

Foram en m agnum , 163f Glaucom a Laser Trial (GLT), 279 Hassall-Henle bodies, 213
Foram en ovale, 68f, 161, 163f Glaucom a m edications, 24–25 Headaches, 81–83, 82f
Foram en rotundum , 68f, 161, 163f Glaucom as, 261–282 HEDS. See Herpetic Eye Disease Study (HEDS)
Foreign body childhood, 267 Heerfordt’s syndrom e, 254
in cornea, 209 disorders of, 267–275 Heidelberg retinal tom ograph (HRT), 310
intraorbital, 172 hem olytic, 270 Helm holtz theory, of accom m odation, 11
Foreign body granulom a, in strabism us surgery, 156 lytic, 270 Hem angiom as
Form alin, for tissue fixation, 40 m alignant, 274 cavernous See Cavernous hem angiom a
Foscarnet, 30 m elanocytom alytic, 270 choroidal, 311t
for cytom egalovirus (CMV) infections, 244 m elanom alytic, 270 choroidal cavernous, 358–359, 358–359f
Foster-Kennedy syndrom e, 81 norm al tension glaucom a (NTG), 275 Hem angiopericytom a, of orbit, 176
Fovea, 303, 303f pathology of, 267, 269f Hem atoxylin and eosin (H&E) stain, 40
Foveal diam eter, 303f pediatrics, 109–110 Hem ianopia, pseudobitem poral, 55
Foveola, 304 childhood, 109, 109f Hem ifacial m icrosom ia, 98
Foveom acular vitelliform dystrophy, adult onset, 121 prim ary congenital, 109–110 Hem ifacial spasm , 70
Foville’s syndrom e, 66 phacolytic, 270 Hem iretinal vein occlusion (HRVO), 342–345
Francois-Neetens corneal dystrophy, 225 prim ary angle-closure glaucom a. See Prim ary Hem oglobin, laser absorption and, 366
Freckle angle-closure glaucom a Hem olytic glaucom as, secondary open-angle
of conjunctiva, 206 prim ary open-angle glaucom a. See Prim ary open- glaucom a and, 270
of iris, 287 angle glaucom a (POAG) Hem orrhages
Free cap, in laser in situ keratom ileusis, 233 secondary angle-closure glaucom a. See Secondary blot or dot, 304
Fresnel prism s, 3 angle-closure glaucom a flam e or splinter, 304
Front surface curvature, glasses m agnification of, 7 secondary open-angle glaucom a. See Secondary retina, 304
Frontal bone, 162f open-angle glaucom a subconjunctival, 203
Frontal nerve, 162f, 164 testing for, 263–266 vascular disease and, 328
Frontal sinus, 165 treatm ent, 275–279 as wound com plication, 44
Frontalis m uscle, 167 laser, 275–276 Hem osiderosis bulbi, 44
Frontoparietal lesion, 59 surgery, 276–279 Hepatolenticular degeneration, 214
Frontosphenoidal foram ina, 161 uveitic, 241 Hereditary arthro-ophthalm opathy, 128
Frosted branch angiitis, 251, 251f Glaucom atocyclitic crisis Hereditary optic neuropathy, 138
Fuchs’ adenom a, 288 in anterior uveitis, 242 Hering’s law, 144–145
Fuchs endothelial corneal dystrophy, 225 secondary open-angle glaucom a and, 272 Herpes infection, of eyelid, 182
Fuchs’ heterochrom ic iridocyclitis Glaukom flecken, 290 Herpes sim plex, corneal ulcers and, 216–218
in anterior uveitis, 242, 242f Glial cells, 52 Herpes sim plex virus (HSV), 105
secondary open-angle glaucom a, 272 Gliom a, chiasm com pression, 79 of eyelids, 182
surgery for, 258 Global Evaluation of Im plantable Dexam ethasone in ophthalm ia neonatorum , 101
Fuchs’ superficial m arginal keratitis, 215 Retinal Vein Occlusion with Macular Edem a Herpes zoster infections, posterior uveitis and, 246
Fum agillin, 29 (GENEVA) study, 341–342 Herpes zoster ophthalm icus (HZO), 70–71, 218–219
Fundus, 303f Glossoph aryngeal neuralgia, 83 Herpetic epithelial keratitis, 217t
Fundus albipunctatus, 126, 126f GLT. See Glaucom a Laser Trial (GLT) Herpetic Eye Disease Study (HEDS), 217, 217–218b
Fundus flavim aculatus, 119 Glutaraldehyde, for tissue fixation, 40 Hertel exophthalm om etry m easurem ents, 162t
Fundus pulverulentus, 121 Glycerin, 25 Heterophoria m ethod, 16
Fungal infections, of orbit, 173 Glycosam inoglycans, in corneal strom a, 198 Hirschberg’s m ethod, in deviations, 146, 146f
Fungi, cornea and, 219–220 Goblet cells, 170t Histam ine H 1 -blockers, 27
Furrow degeneration, of cornea, 213 Gold, ocular toxicity, 31 m ast cell stabilizers, 27
Fusion, types of binocularity, 141 Goldenhar’s syndrom e, 97 Histiocyte, epithelioid, 42
Goldm ann equation, for intraocular pressure, Histiocytom a, fibrous, of orbit, 178, 178f
263–264 Histiocytosis X, 97
G Goldm ann tonom eter, 264 HIV retinopathy, 348
Goldm ann visual field, testing for, 265 Hom er-Wright rosette, retinoblastom a, 132, 132f
Galactosem ia, 108 Goldm ann-Favre disease, 128 Hom ocystinuria (AR), ectopia lentis and, 291–292
Galilean telescope, 7–8, 8f Gom ori’s m ethenam ine silver stain, 39 Hom onym ous hem ianopia with m acular
GALILEO, 345 Gonioscopy, 264–265 sparing, 56
Ganciclovir, 30 Goniosynechialysis, in glaucom a, treatm ent Honeycom b-shaped corneal dystrophy, 223
for cytom egalovirus (CMV) infections, 244 of, 277 Hordeolum , 180, 181f
Ganglion cell layer, 301, 303f GPC. See Giant papillary conjunctivitis (GPC) Horizontal deviations, 148–151
Ganglion cell necrosis, prim ary open-angle Grade I astrocytom a, 94–95 Horizontal gaze center, 52, 53f
glaucom a, 268 Gradenigo’s syndrom e, 66 eye m ovem ents, 56, 56–57f
Gass classification, m acular diseases and, 317, 317f Graft-versus-host disease (GVHD), 228 Horner’s m uscle, 167
Gasserian ganglion, 63f, 70f Gram stain, 39 Horner’s syndrom e, 72
Gaze-evoked nystagm us, 61 Granular cell layer, of eyelid skin, 166 congenital, 99
Gelatinous drop-like corneal dystrophy, 222–223 Granular corneal dystrophy type 1, 223–224, 224f Horopter, 141
General anaesthetics, 22 Granulocytic sarcom a, 97 Horseshoe tear, retinal detachm ent and, 353
Generalized fibrosis, 154 Granulom atous anterior uveitis, 240 HRT. See Heidelberg retinal tom ograph (HRT)
Geniculate ganglia, 52 Granulom atous arteritis, HSV infections and, 219 HRVO. See Hem iretinal vein occlusion (HRVO)
Geniculo-calcarine radiation, 50f Graves’ disease, 95 HSV. See Herpes sim plex virus (HSV)
Genital ulcers, uveitis and, 258 Gray line, of eyelid m argin, 169 Hudson-St€a hli line, 214
Genitourinary involvem ent, uveitis and, 257 Groenouw corneal dystrophy type 1, 223–224, 224f Hue, color vision and, 11
Gentam icin, retinal toxicity and, 349–350 Gruber’s ligam ent, 163f Hum an leukocyte antigen (HLA) system ,
Geographic ulcer, 216 Guillain-Barre syndrom e, 70 41–42, 41t
Gerstm ann’s syndrom e, 80 GVHD. See Graft-versus-host disease (GVHD) Hum prey visual fields, testing for, 265
Ghost cell, secondary open-angle glaucom a, 270 Gyrate atrophy, 127, 127f Huntington’s chorea, 59
Giant cells, 42 Hutchinson’s m alignant freckle, 189
Giant hairy nevus, of face and scalp, 187 Hutchinson’s pupil, 73
Giant papillary conjunctivitis (GPC), 201 H Hutchinson’s rule, 71
in contact lens-related problem s, 227 Hutchinson’s triad, 105
Giant tear, retinal detachm ent and, 354 Hallerm ann-Streiff syndrom e, 98, 108 Hyaloid artery, em bryology of, 35–36, 36f
Giem sa stain, 39 Ham artom a Hyaloid system , persistence of, 137
Glands, of eyelid, 170, 170t of conjunctiva, 204 Hydrocystom a, eyelids, 185
Glare/halos, in laser in situ keratom ileusis, 234 of orbit, 175 Hydrops, in keratoconus, 220
Glasses Hand-Sch u€ ller-Christian disease, 97 Hydroxychloroquine (plaquenil), 349
prescribing, 11–12 Harada-Ito procedure, 155 Hyperacute bacterial conjunctivitis, 203
size of im age through, 7 Harada’s disease, 254 Hypercoagulable states, 347

403
Index

Hypergam m aglobulinem ia, in cornea, 214 Infections, 46t Intraocular lenses (IOL), 13–14
Hyperhom ocystinem ia, ectopia lentis and, 291 intrauterine, 108 calculations, cataract surgery, 292–293
Hyperkeratosis, eyelids, 43 in pediatrics, 92, 105 decentration, com plications of cataract surgery,
Hyperlipoproteinem ia, in cornea, 214 in strabism us surgery, 156 296–297
Hyperlysinem ia, ectopia lentis and, 292 Infectious keratitis, in laser in situ keratom ileusis, power, 16
Hyperm ature cataracts, 289 234 Intraocular pressure, testing for, 263–264
Hyperopes, contact lenses and, 12 Infectious ulcer, 215 Intraocular tum ors, secondary angle-closure
Hyperopia Inferior colliculus, 63f glaucom a and, 274
acquired, 10 Inferior m arginal arterial arcade, 169f Intraoperative floppy iris syndrom e (IFIS), 295–296
piggyback IOL and, 293 Inferior oblique m uscle, 63f, 140 Intraorbital foreign bodies, 172
range of accom m odation for, 10 Inferior oblique overaction (IOOA), 152 Intrastrom al corneal ring segm ents (Intacs), 234,
Hyperosm otic agents, 25 Inferior oblique palsy, 152 235f
Hyperplasia, retinal pigm ented epithelium and, 304 Inferior ophthalm ic vein, 162f, 164, 164f Intrauterine infections, 108
Hypersensitivity reaction, 41 Inferior orbital fissure, 161, 162f Intravitreal Aflibercept for Diabetic Macular Edem a
type I, of conjunctiva, 201 Inferior peripheral venous arcade, 169f (VIVID/VISTA), 339
Hypertelorism , 98 Inferior rectus m uscle, 63f, 139 Intravitreal aflibercept for m acular edem a
Hypertensive retinopathy, 331 Inferior rectus palsy, 152 following branch retinal vein occlusion
Hypertherm ia, m alignant, 156 Inferior tarsal m uscle, 169 (VIBRANT), 344
Hypertrophy, retinal pigm ented epithelium and, 304 Infiltrates, in contact lens-related problem s, 227 Involution al ectropion, 183
Hyphem a, 283–284 Infiltrative optic neuropathy, 77 Involution al senile retinoschisis, 352–353, 352f
com plications of cataract surgery, 296 Inflam m ation, 26, 42–43 IOI. See Idiopathic orbital inflam m ation (IOI)
secondary open-angle glaucom a and, 270 acute, 42 IOL. See Intraocular lenses (IOL)
sickle cell and, 283–284 chronic, 43 IONDT. See Ischem ic Optic Neuropathy
uveitis and, 257 inflam m atory cells, types of, 42 see also specific Decom pression Trial (IONDT)
Hypocalcem ia, 108 cells Ionizing radiation
Hypoglycem ia, during pregnancy, 108 sequelae of, 43 cataracts and, 290
Hypoplasia, 137 types of, 42–43 ocular injury and, 46
Hypopyon , in anterior uveitis, 240 Inflam m atory bowel disease, in anterior Iopidine (apraclonidin e), 24
Hypoten sive m edications, ocular, 24–25 uveitis, 242 Iridocorneal endothelial (ICE) syndrom e, 286
carbonic anhydrase inhibitors, 25 Inflam m atory pathway, antiinflam m atory drugs of, Iridodialysis, 45, 263, 284
com binations of, 25 25, 25f Iridoplasty, glaucom a, treatm ent, 276
hyperosm otic agents of, 25 Inflam m atory ulceration, 219 Iridoschisis, 286
m iotics of, 24–25 Infraorbital foram en, 162f Iris
prostaglandin analogues/prostanoids of, 25 Infraorbital nerve, 68f anatom y of, 283–288
HZO. See Herpes zoster ophthalm icus (HZO) Inherited retinal diseases, pediatrics, 119–121 dilator, 71
Inhibit VEGF in Age-related choroidal em bryology of, 37–38
Neovascularisation Study (IVAN), alternative sphincter, 71
I treatm ents to, 327 traum a and, 284, 285f
Inhibitory quotient (IQ), 21 tum ors, 287
ICE syndrom e, secondary angle-closure glaucom a Inner lim iting m em brane of Elschnig, em bryology of, wound healing in, 44
and, 274 36 Iris atrophy, uveitis and, 257
ICSC. See Idiopathic central serous choroidopathy Inner nuclear layer, neurosensory retina and, 301 Iris disorders, pediatrics, 105–107
(ICSC) Inner plexiform layer, 301 Iris ectropion, congenital, 105
Ictal hallucinations, 84 Innervation Iris heterochrom ia, 286
Idiopathic central serous choroidopathy (ICSC), of cornea, 199 in anterior uveitis, 242
318–319, 318–319f of eye, 164, 165f Iris hypoplasia, congenital, 105
Idiopathic intracranial hypertension (IIH; of eyelid, 169 Iris nevus syndrom e, 287
pseudotum or cerebri), 75–76 INO. See Internuclear ophthalm oplegia (INO) Iris nodules, 286
Idiopathic orbital inflam m ation (IOI), 95, Intacs (intrastrom al corneal ring segm ents), 234, in anterior uveitis, 240
173–174 235f uveitis and, 257
Idiopathic uveal effusion syndrom e, 251–252 Intact doll’s head, 58 Iris pigm ent epithelium , tum ors of, 287
Idoxuridin e, 29 Intercellular junctions, of retina, 304 Iris prolapse, com plications of cataract surgery, 295
IFIS. See Intraoperative floppy iris syndrom e (IFIS) Interference, 1 Iris sphincter tear, 284
Illum ination, of light, 2 Interferon Iron, intraocular foreign body and, 285
ILM. See Internal lim iting m em brane (ILM) ocular toxicity and, 32 Iron lines, in cornea, 214
Im age displacem ent, bifocal glasses of, 4 retinal toxicity and, 351 Irregular ablation, in laser in
Im age jum p, bifocal glasses of, 4 Interm ediate uveitis, 243 situ keratom ileusis, 234
Im ages pediatrics, 111 Irvine-Gass syndrom e, 297
m irrors and, 8 Interm ittent angle closure glaucom a, 273 Ischem ia, acute angle-closure glaucom a and, 273
vergence of, 4–5 Interm uscular septum , 139 Ischem ic Optic Neuropathy Decom pression Trial
Im aging, 171–172 Internal auditory m eatus, 163f (IONDT), 77
of retina, 309–314 Internal carotid artery, 51f, 63f, 68f, 70f Isolated CN 4 palsy, 65, 65f
Im idazoles, 30 Internal lim iting m em brane (ILM), 301 Isolated CN 6 palsy, 66–67, 67f
Im m unoglobulin A (IgA), 41 Internuclear ophthalm oplegia (INO), 59, 59f Isolated cranial nerve palsies, diabetic retinopathy
Im m unoglobulin D (IgD), 41 Interstitial keratitis, 212 and, 332
Im m unoglobulin E (IgE), 41 in corneal opacity, 104 Isoniazid, 29
Im m unoglobulin G (IgG), 41 Interstitial nephritis, in anterior uveitis, 243 Isosorbide, 25
Im m unoglobulin M (IgM), 41 Interval of Sturm , spherocylindrical lens and, 6 Isotretinoin , 32
Im m unoglobulins, 41 Intracorn eal inlays, 234 Itraconazole, 30
Im m unosuppressive agents Intracranial lesion, convergence paralysis secondary Iverm ectin, 30
cytostatic antiinflam m atories of, 27 to, 151
cytotoxic alkylating agents of, 27 Intracytoplasm ic basophilic inclusions, 40
cytotoxic antim etabolites of, 27 Intraderm al nevus, of eyelids, 186 J
im m unom odulator of, 27 Intranuclear eosinophilic inclusions, 40
oncolytic agents, 28 Intraocular calcification Jansen syndrom e, 128
Inclusion conjunctivitis, 204 differential diagnostics of, 44b Jaundice, uveitis and, 257
Inclusion cysts, 205 ultrasound, 311 Jones I test, 191
Incom itant deviations, 148 Intraocular cartilage, differential diagnostics of, 44b Jones II test, 191
Incontinentia pigm enti, 136, 136f Intraocular drugs, 22 JRA. See Juvenile rheum atoid arthritis (JRA)
Index of refraction, 1 Intraocular foreign body, 45, 284–286 Junctional nevus
Indirect ophthalm oscope, 14, 14f ultrasound, 311 of conjunctiva, 206
Indocyanine green (ICG), 314, 314f uveitis and, 257 of eyelids, 186
dye, 31 Intraocular gases, 354 Juvenile retinoschisis, 127–128

404
 Index

Juvenile rheum atoid arthritis (JRA), 110, 110t Lacrim al sac Leprosy
in anterior uveitis, 242 obstruction of, 191 of eyelids, 182
surgery for, 258 tum ors of, 193 posterior uveitis and, 246–247
Juvenile xanthogranulom a (JXG), 106 Lacrim al vein, 164f Lesions
uveitis and, 257 β-Lactam s, 28 CT and MRI characteristics of, 171t
Juxtacanalicular tissue, 262 Lam ellar keratoplasty, 231 disciform , ultrasound, 311t
JXG. See Juvenile xanthogranulom a (JXG) Lange’s fold, 41 frontoparietal, 59
LASEK. See Laser-assisted epithelial keratom ileusis parieto-occipital, 59
(LASEK) precancerous, eyelids, 186
K Laser, 2, 365–366 ultrasound characteristics of, 171t
Laser in situ keratom ileusis (LASIK), 233–234 Letterer-Siwe disease, 97
Kandori’s flecked retina, 126 Laser inferom eter, 9 Leukem ia, 133, 134f
Kaposi’s sarcom a Laser iridotom y, in glaucom a, treatm ent of, 276 of iris, 287
of conjunctiva, 207, 208f Laser-assisted epithelial keratom ileusis (LASEK), 234 uveitis and, 257
of eyelids, 190 Lash line, of eyelid m argin, 169 Levator palpebrae, 168, 168f
Kassabach-Merritt syndrom e, 93 LASIK. See Laser in situ keratom ileusis (LASIK) Levator palpebrae superioris m uscle, 63f
Kawasaki’s disease, 102 Latanoprost (Xalatan), 25 Levitra (Vardenafil), 351
in anterior uveitis, 242 Latent nystagm us, 147 Levocabastine, 27
Kayser-Fleischer ring, 214 Lateral canthal tendon, 169 LHON. See Leber’s hereditary optic neuropathy
Kearns-Sayre syndrom e, 73, 154 Lateral collateral vein, 164f (LHON)
Keith-Wagener-Barker classification , hypertensive Lateral geniculate body, 51–52, 52f Lid avulsion, 180
retinopath y and, 331 Lateral palpebral artery, 169f Lid disorders, pediatrics, 98–100
Keplerian telescopes, 7 Lateral palpebral vein, 169f Lid laceration, 180
Keratan sulfate, in corneal strom a, 198 Lateral posterior ciliary artery, 163f Lid surgery, thyroid-related ophthalm opathy, 175
Keratin layer, of eyelid skin, 166 Lateral rectus m uscle, 63f, 139 Lid-gaze dyskinesis, oculom otor nerve palsy, 64
Keratinoid degeneration, 213 Lateral sinus throm bosis, 85 Lids, 161–196
Keratitic precipitates, in anterior uveitis, 240, 240f Lateral view, of radiographs, 172 anatom y of, 161–170
Keratitis Lattice corneal dystrophy type 1, 223, 223f Light
filam entary, 212 Lattice degeneration, 353, 353f absorption of, 2
interstitial, 104, 212 Law of reflection, 8 coherence of, 1
non-necrotizing, 217 Lawrence-Moon-Bardet-Biedl syndrom e, 124, 125f diffraction of, 1
peripheral ulcerative, 215 LCAT deficiency, 214 illum ination of, 2
Keratoacanthom a, of eyelids, 188, 189f Leber’s congenital am aurosis, 124 index of refraction, 1
Keratoconjunctivitis electroretinogram , 308 interferen ce of, 1
epidem ic, 203 Leber’s hereditary optic neuropathy (LHON), 138 lum inance of, 2
toxic, 202 Leber’s idiopathic stellate neuroretinitis (LISN), polarization of, 1
Keratoconjunctivitis sicca, 210 348 properties of, 1–2
Keratoconus, 220–221 Leber’s m iliary aneurysm s, 117–118, 118f reflection of, 2
Keratoglobus, 221 LeFort, orbital fractures, 172 scattering of, 1
Keratom eter, 15, 15f Legal blindness, 9 speed of, 1
Keratom etry Leiom yom a, of iris, 287 transm ission of, 2
cataract surgery and, 293 Lens capsule abnorm alities, 291 Light response, abnorm al, 72
for cornea, 199 ectopia lentis and, 291 Light-near dissociation, 71–72
Keratom ileusis, 231, 232f posterior capsular opacification and, 291 Ligneous conjunctivitis, 101–102
Keratopathy pseudoexfoliation syndrom e and, 291 Lim bal stem cell deficiency, 228
band, 213 rupture, 45 Lim bus, 199
Labrador, 213 true exfoliation and, 291 anatom y of, 283, 284f
Thygeson’s superficial punctate, 212 Lens colobom a, 107 Linear m agnification, 16
vortex, 214 Lens disorders, pediatrics, 107–109 Lipid, vascular disease, 328
Keratophakia, 231 Lens gradient m ethod, 16 Lipid deficiency, of cornea, 211
Kernicterus, 61 Lens power, 15 Lipid droplet degeneration, 213
Kestenbaum procedure, 155 Lenses, 5, 5f, 288–297 Lipid keratopathy, of cornea, 214
Kestenbaum ’s rule aberration s and, 6–7 Lipoderm oid, 93
low-vision aids of, 13 anatom y/physiology of, 288–289, 288f Lisch nodules, 106, 287
prescription of glasses, 12 cardinal points of, 5 LISN. See Leber’s idiopathic stellate neuroretinitis
Ketoconazole, 30 capsule, 288 (LISN)
Ketotifen, 27 com bining cylinders at oblique axis, 6 Lissam ine green, for cornea, 199
Kissing nevus, of eyelids, 187 cylinder transposition and, 6 Lithiasis, of conjunctiva, 200
Kjer’s syndrom e, 138 disorders of, 289–292 Liver enlargem ent, uveitis and, 257
Klippel-Trenaunay-Weber syndrom e, 135 effectivity, 5 Lockwood’s ligam ent, 139
Knapp’s rule, 7 em bryology of, 37, 38f Lockwood’s suspensory ligam ent, 168
Knee of von Willebrand, chiasm , 49 epithelium , 288 Lodoxam ide, 27
Koeppe nodules, 287 focal length of, 5 Loeffler’s agar, 40
in anterior uveitis, 240 focal points of, 5 Lofgren’s syndrom e, 254
KOH stain, 39 inflam m ation in, 43 Longitudinal relaxation tim e (T1), 171
Kollner’s rule, 130 m eniscus, 5 Louis-Bar syndrom e, 136
Koniocellular neurons (K cells), 52 nodal point of, 5 L€
o wenstein-Jensen agar, 40
Krabbe’s disease, 122 power cross diagram and, 6, 6f Lower eyelid
Krause lacrim al gland, 170t power of fat pads, 165
Krill’s disease, 251, 251f cylinder at oblique axis, 6 retractors of, 168–169, 169f
Krukenberg’s spindle, 214 vertex distance, 16 Lowe’s syndrom e, 108
prism atic effect of, 3, 3f Low-grade astrocytom a, optic nerve gliom a, 78
pure cylindrical, 6 Low-vision aids, 13
L spherocylindrical, 6 Lum igan (bim atoprost), 25
sutures, 288 Lum inance, of light, 2
Labrador keratopathy, 213
tunica vasculosa lentis and, 288 Lum inosity, color vision, 11
Laceration, in corn ea, 209
vergence and, 4 Lum inosity curves, 305, 306f
Lacrim al bone, 162f
zonules, 288 color vision, 11
Lacrim al disorders, pediatrics, 100
Lensom eter, 15, 15f Lung carcinom a, m etastases, of orbit, 180
Lacrim al fistula, 100
Lens-particle glaucom a, 271 Luxol fast blue stain, 40
Lacrim al gland, 165–166, 170t
Lenticonus, 107 Lym e disease, in anterior uveitis, 242
Lacrim al nerve, 162f, 164
Lentiglobus, 107 Lym phadenopathy, preauricular, conjunctivitis
Lacrim al pum p, 170
Lentigo m aligna m elanom a, of eyelids, 187, 189 with, 203
Lacrim al puncta, atresia of, 100

405
Index

Lym phangiom a, 94, 94f Medial orbital vein, 164f Mikulicz’s syndrom e, 210
of conjunctiva, 208 Medial palpebral artery, 169f panuveitis and, 254
Lym phocytes, 42 Medial palpebral veins, 169f Millard-Gubler syndrom e, 66
Lym phoid tum ors Medial posterior ciliary artery, 163f Minim ally Classic/Occult Trial of the Anti-VEGF
of conjunctiva, 208 Medial rectus m uscle, 63f, 139 Antibody Ranibizum ab in the Treatm ent of
of orbit, 177 Medial wall, fractures of, 172 Neovascularization AMD (MARINA) trial,
Lym phom a, of lacrim al sac, 193 Median facial cleft syndrom e, 98 322, 326
Lytic glaucom as, secondary open-angle glaucom a Medulla, 63f, 68f Minim um discrim inable, vision m easurem ents and,
and, 270 Medulloepitheliom a, 106–107 8
Megalocornea, 103 Minim um separable, vision m easurem ents and, 8
Megalopapilla, 137 Minim um visible, vision m easurem ents and, 8
M Meibom ian gland, 170t Minus cylinder grinding, prescription of glasses, 12
Meibom ian gland orifices, of eyelid m argin, 169 Miosis, 72
Mackay-Marg tonom eter, 264 Meige’s syndrom e, 182 congenital, 106
Macrolides, 29 Melanin, laser absorption and, 366 Miotics, 24–25
Macrophages, 42, 42f Melanocytic tum ors, of conjunctiva, 206 of hypotensive m edications, ocular, 24–25
Macula adherens, 304 Melanocytom a, 138 toxic cataracts and, 290
Macular corneal dystrophy, 224, 224f of iris, 287 Mirrors, 8
Macular diseases, 317 Melanocytom alytic glaucom as, secondary open- Miscellaneous retinal disorders, pediatrics, 129–130
Gass classification, 317, 317f angle glaucom a and, 270 Mitochon drial diseases, 138–139, 139f
Macular hole, 317–318 Melanom a Mitom ycin-C (MMC), trabeculectom y, 276
Macular Photocoagulation Study (MPS), 322–324 ciliary body and, 288 Mittendorf’s dot, 107
extrafoveal study, 323 ultrasound, 311t em bryology of, 36
juxtafoveal study, 323–324 Melanom a-associated retinopathy (MAR), 365 M€
o bius’ syndrom e, 58, 66, 154
Macular pucker, 317 Melanom alytic glaucom as, secondary open-angle Model eye, 8
Macular telangiectasia (MacTel), 330 glaucom a and, 270 Modified Krim sky’s m ethod, in deviations,
Maculopathy, cellophane and, 317 Melanotic freckle of Hutchinson, 187 146, 146f
Madarosis, 184 Melkersson-Rosenthal syndrom e, 70 Molds, cornea and, 219
Magnetic resonance im aging, 171, 171t Mellaril, 349, 349f Moll gland, 170t
Magnification, 7–8 MEN. See Multiple endocrine neoplasia (MEN) Molluscum contagiosum
angular, 7 Meningiom a, 177 conjunctivitis and, 203
axial, 7 chiasm com pression, 79 of eyelid, 181–182, 181f
glasses, 7 Meningoencephalitis, HSV infections and, 219 Monoam ine oxidase (MAO), sym pathetic nervous
transverse, 7 Meniscus lenses, 5 system , 23
Magnocellular neurons (M cells), of lateral geniculate Mercurial lentis, cataracts and, 290 Monobactam s, 28
body, 52 Meretoja syndrom e, 223 Monochrom atism , 130
Magnocellular nevus, of optic disc, 138 Meridional com plex degeneration, 351 cone, 130
Malignant caruncle tum ors, 209 Meridional fold degeneration, 351, 351f rod, 130
Malignant epithelial tum ors, 187 Merkel cell tum or, of eyelids, 190 Monocular cover-uncover test, in deviations, 145,
Malignant glaucom a, 274 Mesenchym e, 38, 39f 145f
Malignant hypertherm ia, 156 Mesoderm , 38 Monocular elevation deficiency, 153
anesthetics and, 22 Mesoderm al dysgenesis syndrom es, 103 Monocular tem poral crescent defect, 56
Malignant m elanom a (MM) Metabolic disorders, pediatrics, 104, 112, Monofixation syndrom e, 142–143
of conjunctiva, 207, 207f 113t, 114b Mooren’s ulcer, 215
of eyelids, 189–190, 189f Metaplasia, retinal pigm ented epithelium and, 304 Morgagnian cataracts, 289, 289f
of iris, 287 Metastases Morning glory disc, 137
uveitis and, 257 brain tum ors, 81 Morpheaform basal cell carcinom a, 187, 188f
Malignant m ixed tum or, of orbit, 179 of iris, 287 Motor testing, 144–147, 144t
Malignant neoplasm s, pediatrics, 95–97 posterior segm ent, 364–365, 365f Movat’s pentachrom e stain, 40
Malignant optic nerve gliom a, 78 ultrasound, 311t, 312f MPS. See Macular Photocoagulation Study (MPS)
Mandibular division (V3 ), 63f Metastatic tum ors Mucicarm ine stain, 40
Mandibular nerve (V3 ), 68f, 70f of conjunctiva, 208 Mucin deficiency, of cornea, 210
Mandibulofacial dysostosis, 97 of orbit, 180 Mucocutaneous disorders, conjunctivitis and, 202
Manifest latent nystagm us, 149 Methotrexate, 27 Mucocutaneous lym ph node syndrom e, 102
Mannitol, 25 Methoxyflurane, retinal toxicity and, 350 Mucoepiderm oid carcinom a, of conjunctiva, 206
Mannosidosis, 108 MEWDS. See Multiple evanescent white dot Mucopolysaccharidoses, 122, 214
MAR. See Melanom a-associated retinopath y syndrom e (MEWDS) Mucorm ycosis, orbit, 173
(MAR) Meyer’s loop, 50f Muir-Torre syndrom e, 185, 189
Marcus-Gun n jaw winking, 70 Miconazole, 30 Mu€ ller’s cells, 304
Marcus-Gun n pupil, 71 Microaneurysm , vascular disease and, 328 M€
u ller’s fiber, 303f
Marfan’s syndrom e, ectopia lentis and, 291 Microbial keratitis, 215 Mu€ ller’s m uscle, 168, 168f
Marginal keratolysis, 215 Microbial studies, 39–40 Multifocal choroiditis, 248t, 250, 250f
Marginal ulcer, 216 culture m edia, 40 see also specific culture m edia Multifocal ERG (m fERG), 308
Masson trichrom e stain, 40 cytology, 40 Multifocal nevi, 357, 358f
Mast cell stabilizer, 27 stains, 39 see also specific stains Multiple cranial nerve palsies, 70–71
H 1 -blockers plus, 27 tissue stains, 39 Multiple endocrine neoplasia (MEN), 228–229
plus eosinophil suppressor, 27 Microbleph aron, 100 Multiple evanescent white dot syndrom e (MEWDS),
Mast cells, 42 Microcornea, 103 248t, 249, 249f
Match base curves, prescription of glasses, 12 Microglia, of retina, 304 electroretinogram , 308
Maternal inherited diabetes and deafness (MIDD), Microphakia, 107 Multiple sclerosis
120 Microphthalm os, 92 in pars planitis, 243
Matrix m etalloproteinases (MMP), in corneal strom a, with cyst, 35, 92 uveitis and, 257
198 Microspherophakia, 107 Muro, 25
Mature cataracts, 289 Microsporidia, cornea and, 220 Muscarinic cholinergic receptors, 23
Maxillary bone, 162f Midbrain, 63f Myasthenia gravis, ophthalm oplegia, 73–74
Maxillary nerve (V2 ), 63f, 68f, 70f, 162f, 164 MIDD. See Maternal inherited diabetes and deafness Mycosis fungoides, eyelids, 191
Maxillary sinus, 165 (MIDD) Mydriasis, 72
Measles, 122 Middle lim iting m em brane com plications of cataract surgery, 297
conjunctivitis and, 203 neurosensory retina and, 301 congenital, 106
Mebendazole, 30 of retina, 303f Myectom y, 155
Mechanical ectropion, 183 Midline positions, m otor testing, 144 Myelinated nerve fibers, 137
Medial collateral vein, 164f Migraine, 81–83 Myelitis, HSV infections and, 219
Medial longitudin al fasciculus, 52 ophthalm oplegic, 64 Myoclonus, ocular, 62

406
 Index

Myokym ia Newcastle’s disease, 203 Ocular coherence tom ography (OCT), 15, 317
facial, 70 Nicotinic acid m aculopathy, 350 Ocular dipping, 62
superior oblique, 74 Nicotinic cholinergic receptors, 23 Ocular Hypertension Treatm ent Study (OHTS), 278
Myopia Niem ann-Pick’s disease, 122 Ocular injuries, 44–46
acquired, 10 Night m yopia, 10 Ocular ischem ic syndrom e, 346–347, 347f
night, 10 NLDO. See Nasolacrim al duct obstruction (NLDO) Ocular m elanocytosis, 100
pathologic, 328 Nodal point, of lenses, 5 Ocular m otor apraxia, 58–59
piggyback IOL and, 293–294 Nodular basal cell carcinom a, 187 Ocular m uscle disorders, 73–74
range of accom m odation for, 10 Nodular m elanom a, of eyelids, 190 prim ary overaction syndrom es, 74
Myotom y, 155 Nodular scleritis, 229 Ocular m yoclonus, 62
Myotonic dystrophy Non β-Lactam s, 28 Ocular neurom yotonia, 74
cataracts and, 290 Nonarteritic anterior ischem ic optic neuropathy Ocular pharm acology, 21
ophthalm oplegia, 73 (NAION), 77 Ocular surface disease, of cornea, 210–211
Noncystic retinal tuft, 351 Oculoauriculovertebral dysplasia, 97
Nongranulom atous anterior uveitis, 239–240 Oculocardiac reflex, in strabism us surgery, 156
N Noninfectious neuroretinitis, 348 Oculogyric crisis, 74
Nonionizing radiation Oculom otor nerve (CN 3), 63f, 70f, 162f
NAION. See Nonarteritic anterior ischem ic optic cataracts and, 290 palsy, 62–65, 64f
neuropathy (NAION) ocular injury and, 46 Oculom otor nucleus, 63f
Nanophthalm os, 92 Non-necrotizing keratitis, 217 Oculopharyngeal dystrophy, 154
secondary angle-closure glaucom a and, 274 Non-nutrient agar with E. coli overgrowth , 40 ophthalm oplegia, 73
Naphazoline hydrochloride, 27 Nonoptic reflex system s, eye m ovem ents, Oguchi’s disease, 126, 126f
Narcotics, ocular toxicity and, 32 57–58, 57f OHTS. See Ocular Hypertension Treatm ent Study
Nasal bone, 162f Non-penetrating filtration surgery, in glaucom a, (OHTS)
Nasociliary nerve, 162f, 164 treatm ent of, 277 Oil red O stain, 40
Nasofrontal vein, 169f Nonpolarized light, 1 Ointm ents, topical drugs, 22
Nasolacrim al canal, 161 Nonprogressive retinal disease, electroretinogram , Oligodendrocytes, of retina, 304
Nasolacrim al duct, 170 308 Olivopon tocerebellar atrophy, 61, 125
obstruction of, 191 Nonsteroidal anti-inflam m atory drugs (NSAIDs), 26 Olopatadine, 27
Nasolacrim al duct obstruction (NLDO), 100 ocular toxicity and, 32 Onchocerciasis (river blindness), 247, 247f
Nasolacrim al sac, 170 Nonteratoid m edulloepitheliom a, 106 Oncocytom a, of conjunctiva, 209
Nasolacrim al system , 170, 170f Norm al tension glaucom a (NTG), 275 One-and-a-half syndrom e, 59–60, 60f
disorders of, 191–193 Norrie disease, 118 Operculated hole, retinal detachm ent and, 354
em bryology of, 38 North Carolina m acular dystrophy, 120, 120f Ophthalm ia neonatorum , 101
infections of, 191–193 Nothnagel’s syndrom e, 64 Ophthalm ic artery, 51f, 161–164, 162f
obstructions of, 191 NSAIDs. See Nonsteroidal anti-inflam m atory drugs occlusion , 346
Natam ycin , 30 (NSAIDs) Ophthalm ic division (V1 ), 63f
Nd:YAG laser, 292 NTG. See Norm al tension glaucom a (NTG) Ophthalm ic instrum ents, 14–15
Near devices, low-vision aids and, 13 Nuclear CN 3 palsy, 62–63, 64f Ophthalm ic nerve, 70f
Near point, range of accom m odation, 10 Nuclear sclerosis, cataracts and, 290 Ophthalm ic nerve (V1 ), 68f
Near response, abnorm al, 72 Nuclear/ fascicular syndrom e, 65, 65f Ophthalm ic vein, 51f
Necrobiotic xanthogranulom a, of eyelids, 191 Nutritional optic neuropathy, 77–78 Ophthalm ic viscosurgical device (OVD), cataract
Necrotizing keratitis, 217 Nystagm us, 61–62, 147–148 surgery and, 294
Necrotizing scleritis, 229 acquired, 61–62 Ophthalm om yiasis, 247
Nedocrom il, 27 Bruns’, 61 Ophthalm oplegia, 73–74
Neisseria gonorrheae, ophthalm ia neonatorum , 101 childhood, 61 com a, 75
Neovascular glaucom a (NVG), secondary angle- com a, 75 static, 73
closure glaucom a and, 273 congenital, 147 Ophthalm oplegic m igraine, oculom otor nerve palsy,
Neovascularization convergence-retraction, 61 64
in contact lens-related problem s, 227 dissociated, 61 Ophthalm oscope
peripheral, 340 downbeat, 61 direct, 14, 14f
vascular disease, 329 gaze-evoked, 61 indirect, 14, 14f
Nerve fiber layer (NFL), 301 optokinetic, 54 Opsoclonus, 62
Neural crest cells, 38 periodic alternating, 62 Optic atrophy, 78
Neural ectoderm , 38 physiologic, 61 Optic canal, 161, 162f
Neuralgia, glossopharyngeal, 83 seesaw, 62 Optic cup, em bryology, 35, 36f
Neurilem m om a (schwannom a) sensory, 147 Optic foram en, 163f
of eyelids, 190 spasm us nutans in, 147–148 Optic nerve (ON), 63f, 70f, 162f
of orbit, 176, 176–177f upbeat, 62 anatom y of, 49, 51f, 161, 263
Neuroblastom a, 96, 96f vestibular, 62 blood flow m easurem ent of, 266, 269f
Neurofibrom a, 94, 95f voluntary, 62 disorders, pediatrics, 136–139
of eyelids, 190 Nystagm us blockage syndrom e, 149 drusen, 137–138
Neurofibrom atosis, 134–135 gliom a, 94–95
type 2, 290 vascular supply of, 263f
types of, 134–135 O Optic nerve disorders, 75–78
Neurogenic tum ors, 190 gliom a, 78, 78f
Neuroglial cells, of retina, 304 Objects hereditary, 78
Neurologic visual field defects, 55–56 m irrors of, 8 m eningiom a, 78, 78f
Neurom yotonia, ocular, 74 vergence of, 4–5 neuropathies, 76–78
Neuronal ceroid lipofuscin osis, 125 Oblique axis, lenses of, 6 swelling, 75
Neuro-ophthalm ology, 49–90 Oblique views, of radiographs, 172 tum ors, 78
Neuroretinitis, 348 Occipital lobe, 50f Optic nerve head (ONH) analyzers, 266, 266t
Neurotransm itter retrochiasm al disorders, 80 Optic nerve sheath, 51f
parasym pathetic nervous system and, 23 visual field defects, 56 Optic nerve sheath m eningiom a, 177
sym pathetic nervous system and, 23 Ochre m em brane, 44 Optic neuritis, 76
Neurotrophic keratitis, 226 Ochronosis, 214, 230 Optic Neuritis Treatm ent Trial (ONTT), 76b
Neurotrophic keratopathy, 217 OCT. See Ocular coherence tom ography (OCT); Optic neuropathy, 331
Neutrophils, 42 Optical coherence tom ography (OCT) infiltrative, 77
Nevoxanthoendotheliom a, 106 Ocular alignm ent, 148–154 Optic pit, 137
Nevus horizontal deviations in, 148–151 em bryology, 35
of conjunctiva, 206 vertical deviations in, 151–154 Optic radiation, 52
of eyelids, 186 Ocular bobbing, 62 Optic tract, 50–51, 51f
of iris, 287 Ocular cicatricial pem phigoid, 211 Optic vesicle, em bryology, 35

407
Index

Optical coherence tom ography (OCT), 266, 268f, Papillom atosis, eyelids, 43 Peripapillary staphylom a, 137
309, 309f Paraffin, for tissue fixation, 40 Peripheral anterior synechia (PAS), 263
Optics, 1–20 Parakeratosis, 43 Peripheral arterial arcade, 169f
equations in, 15–16 Parallel rays, vergence and, 4 Peripheral CN 7 lesion, facial nerve palsy, 69–70
Optokinetic nystagm us (OKN), 54 Paralytic ectropion, 183 Peripheral corneal ulcers, 215
Ora serrata, 302f Paraneoplastic syndrom e, associated with Peripheral m icrocystoid degeneration, 352
Ora serrata pearl, 352 neuroblastom a, 96 Peripheral neovascularization, 340
Oral bay degeneration, 351, 351f Parasitic infections, of orbit, 173 Peripheral retina, 302, 303f
Oral contraceptives, toxicity, 32, 350 Parasym pathetic nervous system , 23 Peripheral ulcerative keratitis, 215
Oral drugs, carbonic anhydrase inhibitors, 25 Parenteral anaesthetics, 22 Perkins tonom eter, 264
Oral tear, of retina, 316 Parietal lobe Persistent fetal vasculature (PFV), 112
Oral ulcers, uveitis and, 258 retrochiasm al disorders, 80 Persistent hyperplastic prim ary vitreous (PHPV), 112
Orbicularis oculi, 166–167, 167f visual field defects, 56 secondary angle-closure glaucom a and, 274
Orbit, 161–196 Parieto-occipital lesion, 59 Persistent pupillary m em brane, 106
anatom y of, 161–170 Parinaud’s oculoglandular syndrom e, 204 em bryology of, 36
differential diagnosis of com m on disorders of, Parinaud’s syndrom e, 60 Peter’s anom aly, 103
192t Parkinson ’s disease, 59 Petroclinoid, 163f
osteology of, 162t Parks-Bielschowsky three-step test, in deviations, Petroclinoid ligam ent, 68f
Orbital apex, fractures of, 172 146–147, 147f Petrous apex syndrom e, 66, 67f
Orbital cellulitis, 92 Pars plana, 302f Pfeiffer’s syndrom e, 98
Orbital contusion, 172 aging changes in, 44 pH, bioavailability of, 21
Orbital decom pression, thyroid-related cyst, 352, 352f Phacoanaph ylactic endophthalm itis, 272
ophthalm opathy, 174 Pars planitis in anterior uveitis, 242–243
Orbital disorders, 172–180 in interm ediate uveitis, 243, 243f Phacoantigenic uveitis, 272
degeneration and, 173 surgery for, 258 Phacodynam ics, cataract surgery and, 294–295
infections and, 173 Pars plicata, 302f Phacolytic glaucom a, secondary open-angle
inflam m ation and, 173–175 aging changes in, 44 glaucom a and, 270
traum a and, 172 anatom y of, 261 Phakom atoses, pediatrics, 134–136, 134t
tum ors and, 175–180, 175t Partial cone degeneration, electroretinogram , 307f Pharm acodynam ics, 21
vascular abnorm alities and, 175 Parvocellular neurons (P cells), of lateral geniculate Pharm acokinetics, 21
Orbital floor (blow-out), fractures of, 155, 172 body, 52 Pharm acology, 21–34
Orbital foram en, 161 PAS. See Peripheral anterior synechia (PAS) adm inistration routes of, 21–22
Orbital fractures, 172 Pathologic m yopia, 328 anesthetics of, 22
Orbital (retrobulbar) hem orrhage, 172 Pattern dystrophies, 121 antiallergy m edications of, 27
Orbital lym phom a, 177, 178f Pattern electroretinogram (PERG), 308, 308f antiinflam m atory drugs of, 25–26
Orbital m yositis, 174 Pavingstone degeneration, 353, 353f autonom ic system of, 23–24
Orbital pseudotum or, 95, 173–174 Pediatric eye exam ination, 140–141 concentration of, 22
Orbital roof, fractures of, 172 Pediatrics, 91–139 im m unosuppressive agents of, 27–28
Orbital septum , anatom y of, 167–168, 167f anatom y of, 91, 92t m iscellaneous of, 30–31
Orbital surgery, 193 anterior uveitis in, 110–111 ocular, 21
Orbital syndrom e, 64–65, 65f benign lesions in, 93–95 ocular toxicology and, 31–32, 31t
abducens nerve palsy, 66–67, 67f congenital anom alies in, 92 Pharyngoconjunctival fever (PCF), 203
Organic m atter, intraocular foreign body, 45 conjunctival disorders of, 100–102 Pheniram ine m aleate, 27
Orientation of globe, for tissue fixation, 40 conjunctivitis in, 101–102 Phenothiazines
Orthophoria, 148 corneal disorders of, 102–105 ocular toxicity and, 31
Oscillopsia, 62 craniofacial disorders of, 97–98 toxic cataracts and, 290
Osm otics, bioavailability of, 21 syndrom es, 97–98 Phim osis, capsular, 297
Osteom a glaucom a of, 109–110, 109f Phosphenes, 76
choroidal, 359 history, 91–92 Phosphodiesterase 5 inhibitors, ocular toxicity
orbit, 178 infections in, 92, 105, 121–122 and, 32
Outer nuclear layer, neurosen sory retina, 301 inherited retinal diseases in, 119–121 Photopic (light adapted) electroretinogram , 306–307
Outer plexiform layer, neurosensory retina, 301 interm ediate uveitis in, 111 Photoreceptor layer, 302, 303f
Overactivity, facial nerve palsy, 70 iris disorders of, 105–107 Photorefractive keratectom y (PRK), 233
Overall diam eter, contact lenses and, 12 lacrim al disorders of, 100 Photostress recovery test, 54
Oxygen transm ission, contact lenses and, 12 lens disorders of, 107–109 Phototoxicity, com plications of cataract surgery, 296
lid disorders of, 98–100 PHPV. See Persistent hyperplastic prim ary vitreous
m alignant neoplasm s in, 95–97 (PHPV)
P m etabolic disorders of, 104, 112, 113t, 114b Phthiriasis palpebrum /pediculosis, 182
m iscellaneous retinal disorders of, 129–130 Phthisis, 43
Pachym etry, 199 optic nerve disorders of, 136–139 Phthisis bulbi, 45, 173
Paget’s disease, 328 orbital disorders of, 92–97 Physiologic nystagm us, 61
Palinopsia, 84 phakom atoses in, 134–136 Pial vasculature, 51f
Palpebral conjunctiva, 197 physiology of, 91–92 PIC. See Punctate inner choroidopathy (PIC)
PAM. See Potential acuity m eter (PAM) posterior uveitis in, 111–112 Pierre Robin sequence, 98
PAN. See Periodic alternating nystagm us (PAN) retinal disorders of, 112–133 Piggyback intraocular lenses (IOL), cataract surgery
Pannus, in cornea, 212 retinal tum ors in, 130–133 and, 293–294
Panretinal photocoagulation (PRP), 366 tapetoretinal degeneration in, 123–127 Pigm ent dispersion syndrom e, 286
Panum ’s fusional space, 141 tum ors in, 102 Pigm entary glaucom a (PG), 270–271, 271f, 275f
Panuveitis, 253–256 uveitis of, 110–112 Pilocarpine, 24
Behçet’s disease in, 254, 254f, 254b vitreoretinal degenerations in, 128–129 Pilom atrixom a, of eyelids, 186
Heerfordt’s syndrom e in, 254 vitreoretinal dystrophies in, 127–128 Pingueculitis, 201
Lofgren’s syndrom e in, 254 Pedunculated squam ous papillom a, 205 Pingueculum , of conjunctiva, 200–201
Mikulicz’s syndrom e in, 254 Pegaptanib (Macugen), clinical trials, 322 Pituitary apoplexy, chiasm com pression, 79
sarcoidosis in, 253–254, 253b, 253f Pellucid m arginal degeneration, 221 Pituitary gland, 70f
sym pathetic ophthalm ia in, 255–256, 255–256f Pem irolast, 27 tum ors, 79
syphilis in, 256 Penetrating keratoplasty, 230–231 Plano, m irrors of, 8
tuberculosis in, 256 Penicillins (PCN), 28 Plant sap, 214
Vogt-Koyanagi-Harada syndrom e (VKH) in, PERG. See Pattern electroretin ogram (PERG) Planum sphenoidale, 163f
254–255, 255f Peribulbar drugs, 22 Plaque brachytherapy, choroidal m alignant
Papillae, inflam m ation of, 199–200 Periodic acid-Schiff (PAS) stain, 39–40 m elanom a and, 362
Papilledem a, 75 Periodic alternating nystagm us (PAN), 62 Plaquenil, 349
Papillitis, diabetic retinopathy, 332 Periorbita, 165 Plasm a cells, 42
Papillom a, of eyelids, 182 Peripapillary loop, em bryology of, 36 Plasm acytom a, 177–178

408
 Index

Plateau iris, prim ary angle-closure glaucom a and, Preoral tear, retina, 316 Puncta, 170
273 Presbyopia, 10 Punctal obstruction, nasolacrim al system disorders,
Pleom orphic adenocarcinom a, of orbit, 179 prem ature, 11 191
Pleom orphic adenom a, of orbit, 179, 179f Preseptal cellulitis, 92 Punctate epithelial keratitis, 218
Pleom orphic rhabdom yosarcom a, 95 Presum ed ocular histoplasm osis syndrom e (POHS), Punctate inner choroidopathy (PIC), 248t, 250–251,
Pleom orphism , of endothelium , of cornea, 199 327, 328f 251f
Plica sem ilunaris, 197 posterior uveitis and, 244 Pupil-gaze dyskinesis, oculom otor nerve palsy, 64
Pneum atic retinopexy, rhegm atogenous retinal Prickle cell layer, of eyelid skin, 166 Pupillary block, m echanism of, prim ary angle-
detachm ent and, 354 Prim ary acquired m elanosis, of conjunctiva, closure glaucom a and, 272
Pneumocystis choroiditis, 246, 246f 206–207, 207f Pupillary capture, com plications of cataract surgery,
POAG. See Prim ary open-angle glaucom a (POAG) Prim ary am yloidosis, vitreous, 314 296
POHS. See Presum ed ocular histoplasm osis Prim ary angle-closure glaucom a, 272–273 Pupillary light reflex, pathway of, 71
syndrom e (POHS) acute angle, 272–273 Pupils, 71–73
Polarization , of light, 1 chronic, 273 disorders of, 71–73
Polarized light, 1 epidem iology, 272 innervation of, 71, 71f
Polarizing filters, for tissue stain, 40 interm ittent, 273 Pupil-sparing isolated CN 3 palsy, 64
Poliosis, 184 plateau iris, 273 Pure cylindrical lens, 6
Polyarteritis nodosa Prim ary congenital glaucom a, 109–110 Purkinje shift, night m yopia of, 10
corneal ulcers and, 215 Prim ary Herpes sim plex virus epithelial keratitis, 216 Purkinje vascular phenom ena, 54
in sclera, 230 Prim ary intraocular lym phom a, 364, 364f Purkinje-Sanson im ages, 8
Polyenes, 30 uveitis and, 257 Purtscher’s retinopathy, 316, 316f
Polym egathism , of endothelium , of cornea, 199 Prim ary iris cysts, 106 PVD. See Posterior vitreous detachm ent (PVD)
Polym orphic am yloid degeneration, 213 Prim ary open-angle glaucom a (POAG), 267–270 PXE. See Pseudoxanth om a elasticum (PXE)
Polym orphous corneal dystrophy, posterior, Prim ary visual cortex, 52 PXG. See Pseudoexfoliation glaucom a (PXG)
225–226 Prim ary vitreous, em bryology of, 36 PXS. See Pseudoexfoliation syndrom e (PXS)
Polym yxin B, 28 Prim itive epithelial papillae, em bryology of, 36 Pyogenic granulom a, of conjunctiva, 207
Pons, 63f, 68f Prince rule, am plitude of accom m odation, 12 Pyrantel pam oate, 30
Pontine lesion, 59 Prism adaptation test, 150 Pyrazinam ide, 29
PORN. See Progressive outer retinal necrosis (PORN) Prism diopter, 3
Position m aintenance system , eye m ovem ents, 56 Prism atic power, 15
Posner-Schlossm an syndrom e, 242 Prism s, 3–4, 3f Q
Posterior capsular opacification (PCO), lens capsule vector addition of, 4, 4f
abnorm alities and, 291 Procerus m uscle, 167 Quinine, toxicity, 31, 349
Posterior capsulotom y, Nd:YAG laser, 292 Prodrug, 22
Posterior cerebral artery, 63f Progressive cone dystrophy, 121, 121f
aneurysm , 64 Progressive ophthalm oplegia, 73 R
Posterior ciliary arteries, 164 Progressive outer retinal necrosis (PORN), 245, 246f
Racem ose hem angiom a, 361
Posterior com m unicating artery, 51f, 63f Progressive supranuclear palsy, 60
Racem ose hem angiom atosis, 136
Posterior em bryotoxin, of endothelium , of cornea, Proliferative vitreoretinopathy, 355, 355f
Racial m elanosis, of conjunctiva, 206
199 Proparacaine, 22
Radial keratotom y, 232–233
Posterior fixation suture, 155 Propionibacterium acnes, 247–248
Radiation
Posterior fossa tum ors, 81 Proptosis, 161
cornea and, 209–210
Posterior keratoconus, 103 Prosopagnosia, cortical lesions, 80
ocular injury and, 46
Posterior lim bal border, 284f Prostaglandin analogues/prostanoids, hypotensive
Radiation retinopathy, 347
Posterior polym orphous corneal dystrophy (PPCD), m edications, 25
Radiographs, 171–172
225–226 Prostate carcinom a, m etastases, of orbit, 180
Radius of curvature, contact lenses, 12
Posterior polym orphous m acular dystrophy Protanom aly, color blindness, 130
RAM. See Retinal artery m acroaneurysm (RAM)
(PPMD), secondary angle-closure glaucom a Protanopia, 130
Ram say-Hunt syndrom e, 70, 219
and, 274 Protocol S, 338
Random dot stereogram s, types of binocularity, 141
Posterior polypoidal choroidal vasculopathy, 357 Protocol T, 338–339
Range of accom m odation, 10
Posterior segm ent, 301–370 Provocative tests, for prim ary angle-closure
far point, 10
anatom y, 301–305 glaucom a, 273
near point, 10
electrophysiology, 305–308 PRP. See Panretinal photocoagulation (PRP)
Ranibizum ab, clinical trials, 322, 326, 333, 336–337,
physiology, 305 Prussian blue stain, 40
342, 344
Posterior segm ent disorders, 314–365 PSC. See Posterior subcapsular cataracts (PSC)
RAPD. See Relative afferent pupillary defect
tum ors and, 357–365 Pseudoalexia, 80
(RAPD)
vascular disease and, 328 Pseudobitem poral hem ianopia, 55
Ray tracing, 5, 5f
Posterior subcapsular cataracts (PSC), 290 Pseudo-CN 6 palsy, 67
Raym ond’s syndrom e, 66
Posterior uveal bleeding syndrom e (PUBS), 357 Pseudodendritic keratitis, 218
RB. See Retinoblastom a (RB)
Posterior uveitis, 243–252 Pseudoepitheliom atous hyperplasia
Reactive lym phoid hyperplasia, choroid, 364
pediatrics, 111–112 of conjunctiva, 208
Recurrent erosion, of cornea, 209
Posterior vitreous detachm ent (PVD), 314 eyelids, 43
Red glass test, for ARC, 143
ultrasound, 311t Pseudoesotropia, 148
Red lens phenom enon, 151
Postherpetic neuralgia, HSV infections and, 219 Pseudoexfoliation glaucom a (PXG), 271, 271f
Red nucleus, 63f
Potential acuity m eter (PAM), 54 Pseudoexfoliation syndrom e (PXS), 291
Red-green disorders, color blindness, 130
Power calculation, contact lenses, 13, 13f Pseudoexotropia, 148
Reduced schem atic eye, 16
Power cross diagram , lenses of, 6, 6f Pseudo-Foster-Kennedy syndrom e, 77, 81
Reflecting power
Power factors, glasses m agnification of, 7 Pseudogaze palsies, 59
m irrors of, 8
PPCD. See Posterior polym orphous corneal Pseudo-Gradenigo’s syndrom e, 66
spherical m irror, 16
dystrophy (PPCD) Pseudoinflam m atory m acular dystrophy, 121
Reflection, of light, 2
Praziquantel, 30 Pseudoisochrom atic plates, 130
Refracting power, of spherical surface, 16
Preaponeurotic fat pads, 165 Pseudoretinitis pigm entosa, 124
Refraction, 2–3
Preauricular lym phadenopathy, with conjunctivitis, Pseudorosettes, retinoblastom a, 132
index of, 1
203 Pseudotum or cerebri, 75–76
Refractive error, 9–10, 9f
Preauricular nodes, 169 Pseudoxan thom a elasticum (PXE), 328
Refractive hyperopia, 9
Precancerous lesions, of eyelids, 186 Psoriatic arthritis, in anterior uveitis, 242
Refractive index, lens, 289
Precorneal tear film , 197 Pterygium , of conjunctiva, 201
Refractive m yopia, 9
Pre-Descem et corneal dystrophy, 225 Pterygoid nerve, 162f
Refsum ’s disease, 124, 154
Preeclam psia, 81 Pterygopalatine ganglion nerve, 162f
Reis-Bucklers dystrophy, 223
Pregnancy, visual disorders, 81 Ptosis, com plications of cataract surgery, 297
Reiter’s syndrom e, 204
Prem ature presbyopia, 11 PUBS. See Posterior uveal bleeding syndrom e (PUBS)
in anterior uveitis, 241–242
Prentice’s rule, 15 Pulfrich phenom enon, 76
Relapsing polychondritis, in sclera, 230
prism s and, 3–4 Pulm onary involvem ent, uveitis and, 258
Relative afferent pupillary defect (RAPD), 50, 71

409
Index

Relaxation tim es, in MRI, 171 Retrochiasm al disorders, 55f, 79–80 Scotom a
Release hallucinations, 84 Retrolental fibroplasia, 114–117 arcuate, 54
Rescula (unoprostone isopropyl), 25 Rhabdom yosarcom a, 95–96, 96f cecocentral, 54
Resection, choroidal m alignant m elanom a types of, 95 central, 54
and, 362 Rhegm atogenous retinal detachm ent (RD), 353–355, ring, 55
Residual strabism us, 156 354f scintillating, 83
RESTORE/RESOLVE, 337 com plications of cataract surgery, 297 Scotopic (dark adapted) electroretinogram ,
Reticulum cell sarcom a, uveitis and, 257 Rheum atoid arthritis, corneal ulcer and, 215 306–307f, 307
Retina, 302f Richner-Hanh art syndrom e, 214 Sebaceous gland carcinom a, of eyelids, 189, 189f
aging changes in, 44 Riddoch phenom enon, 56 Sebaceous gland tum ors, 185
anatom y, 301–304, 302f RIDE, 336–337 Seborrheic keratosis, of eyelids, 184, 185f
arachnoidal cells, 304 Rieger’s anom aly, 103 Secondary acquired conjunctival m elanosis, 207
Bruch’s m em brane, 304 Rieger’s syndrom e, 103 Secondary angle-closure glaucom a, 273–275
em bryology of, 37 Rifam pin, 29 epithelial downgrowth and, 274
fovea, 303, 303f Rigid contact lenses, 13 fibrous ingrowth and, 274
foveola, 303f, 304 Riley-Day syndrom e, in corneal opacity, 104 ICE syndrom e and, 274
im aging, 309–314 Ring scotom a, 55 intraocular tum ors and, 274
intercellular junctions, 304 RISE, 337 m echanism /etiology of, 273
laser treatm ent, 365–366 Risley prism , 3 nanophthalm os and, 274
m acula, 302–303 River blindness, posterior uveitis and, 247 NVG and, 274–275
m icroglia, 304 Rod cell, retina, 303f PHPV and, 274
M€ u ller’s cells, 304 Rod m onochrom atism , 130 PPMD and, 274
neuroglial cells, 304 Rod photoreceptor m em branes, 305 ROP and, 274
neurosensory retina, 301–302 Rod-cone degeneration, electroretinogram , 307f Secondary open-angle glaucom a, 270–272
oligodendrocytes, 304 Rose Bengal, for cornea, 199 alpha-chym otrypsin induced, 271
peripheral retina, 302, 303f Roth spot, vascular disease, 329 chalcosis and, 272
pigm ented epithelium . See Retinal pigm ented RPE. See Retinal pigm ented epithelium (RPE) corticosteroid-induced, 272
epithelium (RPE) RTA. See Retinal thickness analyzer (RTA) Fuchs’ heterochrom ic iridocyclitis and, 272
Schwann cells, 304 Rubella, congenital, electroretin ogram , 308 ghost cell, 270
wound healing in, 44 Rubella syndrom e, congenital, 121–122, 122f glaucom atocyclitic crisis and, 272
Retinal abnorm alities, 315–355 Rubeosis iridis, 286 hyphem a and, 270
astrocytom a and, 360 JRA-associated uveitis and, 272
capillary hem angiom a and, 359 lens-particle glaucom a and, 271
cavernous hem angiom a and, 359–360 S lytic glaucom as and, 270
degenerations and, 351, 351t pigm entary glaucom a and, 270–271, 275f
hem orrhages and, 304 S-100 protein stain, 40 pseudoexfoliation glaucom a and, 271, 271f
m icrovascular disease and, electroretinogram , 308 Sabouraud’s agar, 40 Schwartz-Matsuo syndrom e and, 271
sclopetaria and, 315, 316f Saccades, 56 siderosis and, 272
traum a and, 315 Saccadic system , eye m ovem ents, 56 traum atic, 272
vasculitis and, 329 Saccadom ania, 62 tum or cells and, 270
Retinal artery m acroaneurysm (RAM), 330–331, 330f Sagittal depth/apical height, contact, 12 uveitic, 272
Retinal astrocytom a, 360 Salzm ann’s nodular degeneration, 213 uveitis-glaucom a-hyphem a and, 272
Retinal cavernous hem angiom a, 136 Sandhoff’s disease, 122 Secondary vitreous, em bryology of, 36
Retinal correspondence, 143 Sarcoidosis, 70 Seesaw nystagm us, 62
abnorm al, 143 of eyelids, 191 Selective laser trabeculoplasty (SLT), 276
norm al, 143 facial nerve palsy, 70 Sensory nystagm us, 147
Retinal detachm ent, 353 of orbit, 175 Sensory phenom ena, associated with strabism us, 142
electroretin ogram , 308 panuveitis and, 253–254, 253b, 253f Sensory testing, 141–143
ultrasound, 311t Saturation, color vision, 11 Serpiginous choroidopathy, 248t, 249–250, 250f
Retinal dialysis, 45 Scanning laser ophthalm oscope (SLO), 310 Sessile squam ous papillom a, 205
Retinal disorders Scanning laser polarim etry (SLP), 266, 268f Setons, in glaucom a, treatm ent of, 277
dysplasia, 37 Scattering, of light, 1 Shaken baby syndrom e, 118
inflam m ation, 43 SCC. See Squam ous cell carcinom a (SCC) Shape factors, glasses m agnification of, 7
pediatrics, 112–133 Schaffer gonioscopy, 264, 264f Sheehan’s syndrom e, 79
Retinal pigm ent epithelial detachm ent (PED), 319 Scheie classification, hypertensive retinopathy, 331 Sherrington’s law, 145
Retinal pigm ented epithelium (RPE), 304 Scheie gonioscopy, 264 Short posterior ciliary artery, 51f
Retinal S-antigen, in uveitis, 239 Schiøtz tonom eter, 264 Sickle cell
Retinal thickness analyzer (RTA), 310 Schirm er’s test, for cornea, 199 angioid streaks, 328
Retinal tum ors, pediatrics, 130–133 Schlem m ’s canal, 262f, 263 hyphem a and, 283–284
Retinal vein occlusion, clinical studies, 342–345b Schnabel’s cavernous optic atrophy, 267, 270f retinopathy, 339–340, 339–340f
Retinitis pigm entosa, 123, 123f Schnyder corneal dystrophy, 224–225 secondary open-angle glaucom a and, 270
electroretin ogram , 307 Schwalbe’s line, 199, 262, 262f Siderosis
uveitis and, 257 Schwalbe’s ring, 199 in cornea, 214
Retinoblastom a (RB), 131–133, 131t, 132–133f Schwann cells, retina, 304 secondary open-angle glaucom a and, 272
types of, 132 Schwannom a, of ciliary body, 288 Siderosis lentis, cataracts and, 290
uveitis and, 257 Schwartz-Matsuo syndrom e, 271 Siegrist streak, choroidal ischem ia, 357
Retinocytom a, 133 Scintillating scotom a, 83 Sildenafil (Viagra), 351
Retinom a, 133 Sclera, 199, 302f Sim brinza (brinzolam ide-brim onidin e), 25
Retinopathy aging changes in, 44 Sim ple anisocoria, 73
diabetic. See Diabetic retinopathy em bryology of, 37 Sim ple m agnifier, 16
hypertensive, 331 rupture of, 44–45 Sim ultaneous perception, types of binocularity, 141
Purtscher’s, 316, 316f Scleral buckle surgery, com plications of, 355f Sinus carcinom a, of orbit, 180
Valsalva, 317 com plications, 355 Sinus m ucocele, of orbit, 179–180
whiplash, 317 Scleral disorders, 229–230 Sinus tum ors, of orbit, 179
Retinopathy of prem aturity (ROP), 114–117, Scleral icterus, 230 Sinuses, 165, 166f
114–115f Scleral perforation, in strabism us surgery, 156 Sipple-Gorlin syndrom e, 228–229
secondary angle-closure glaucom a and, 273 Scleral spur, 262f, 263 Sipple’s syndrom e, 228
Retinoscope, 14 Scleral thickening, ultrasound, 312f Sjo€ gren syndrom e, 210
Retrobulbar drugs, 22 Scleritis, 229–230 Sjo€ gren’s reticular dystrophy, 121
Retrobulbar hem orrhage, 172 Sclerocornea, 103–104 Skew deviation, 60–61
com plications of cataract surgery, 295–297 Scleroderm a, corneal ulcers and, 215 Skin, of eyelid, 166
Retrobulbar optic neuropathy, 77 Sclerosin g orbital pseudotum or, 174 Skin rash, uveitis and, 257
retrochiasm al disorders, 55f Sclopeteria, 45 SLK. See Superior lim bic keratocon junctivitis (SLK)

410
 Index

SLO. See Scanning laser ophthalm oscope (SLO) Strom a, 305 Takayasu’s disease, 230
SLP. See Scanning laser polarim etry (SLP) of cornea, 198 Talc
SLT. See Selective laser trabeculoplasty (SLT) Strom al dystrophies, 224 ocular toxicity and, 32
Sm ooth pursuit system , eye m ovem ents, 56 Strom al keratitis, 217 retinal toxicity and, 350
Snellen acuity, 9, 9f Sturge-Weber syndrom e, 135 Tam oxifen
Snell’s law, 2, 3f, 15 Subacute sclerosing panencephalitis, 122 ocular toxicity and, 32
Snowballs, in interm ediate uveitis, 243 Subarachnoid space, 51f retinal toxicity and, 350, 350f
Snowbank, in interm ediate uveitis, 243 Subarachnoid space syndrom e, 65, 65f Tangent screen, visual field, testing for, 265
Snowflake degeneration, 128–129 abducens nerve palsy, 66, 67f Tangier’s disease, 214
SO tendon sheath syndrom e, 153 Subconjunctival drugs, 22 Tapetoretin al degeneration, pediatrics, 123–127
Soem m ering’s ring, cataracts and, 290 Subconjunctival fascia, 139, 139f Tarsal kink, congenital, 99
Soft tissues, of eye, 165–166, 166f Subconjunctival hem orrhage, in infectious Tarsus, 169
Solar retinopathy, 318, 318f conjunctivitis, 203 Tay-Sachs disease, 122
Sound attenuation, ultrasound, 311 Subepithelial infiltrates, in infectious conjunctivitis, Tegm ental lesion, 59
Spaeth gonioscopy, 264 202 Telecanthus, 100
Spasm Subepithelial nevus, of conjunctiva, 206, 206f Telescopes, 13, 16
hem ifacial, 70 Subfrontal m ass lesion , 79 m agnification of, 7–8
of near synkinetic reflex, 150 Subluxation, lens capsule abnorm alities total accom m odation through, 16
Spasm us nutans, 147–148 and, 291 Tem poral lobe
Spastic entropion, 184 Subm andibular nodes, 169 retrochiasm al disorders, 80
Spectinom ycin, 29 Subm ental vertex view, of radiographs, 172 visual field defects, 56
Spectral-dom ain optical coherence tom ography Subretinal invasion, in ophthalm om yiasis, 247 Tem porom andibular joint (TMJ) syndrom e, 83
(SDOCT), 309 Sub-Tenon’s drugs, 22 Tenectom y, 155
Specular m icroscopy, for cornea, 199 Sudan black stain, 40 Tenon’s capsule, 139
Speed, of light, 1 Sudiforous cyst, of eyelids, 185 Tenotom y, 155
Sphenoid bone, 162f Sulfite oxidase deficiency (AR), ectopia lentis and, Tensilon test, 74
Sphenoid ridge, 163f 292 Tension headache, 83
Sphenoid sinus, 165 Sulfonam ides, 28 Teratoid m edulloepitheliom a, 106
Sphenoid wing m eningiom a, 78, 177 Sunrise syndrom e, 297 Teratom a, 93
Sphenopalatine ganglia, 52 Sunset syndrom e, 297 Terrien’s m arginal degeneration, 215
Spherical aberrations, lens and, 6, 7f Superficial spreading m elanom a, of eyelids, 189–190 Terson’s syndrom e, 316, 316f
Spherical equivalent, 16 Superior cerebellar artery, 63f Tertiary vitreous, em bryology of, 37
spherocylindrical lens and, 6 Superior cervical ganglia, 52 Tetracaine, 22
Spherical m irror, reflecting power, 16 Superior colliculus, 50–51, 50f, 63f, 163f Tetracyclines, 29
Spherical surface, refracting power, 16 Superior lim bic keratocon junctivitis (SLK), 202 The Standard Care vs Corticosteroid for Retinal Vein
Spherocylindrical lens, 6 in contact lens-related problem s, 227 Occlusion (SCORE) Study, 343
Spheroidal degeneration, of cornea, 213 Superior m arginal arterial arcade, 169f Therapeutic index, 21
Sphingolipidoses, 122, 214 Superior oblique m uscle, 63f, 139 Thiabendazole, 30
Spielm eyer-Vogt-Batten-Mayou syndrom e, 124–125 Superior oblique m yokym ia, 74 Thiel-Behnke corneal dystrophy, 223
Spindle cell nevus, of eyelids, 187 Superior oblique overaction, 152 Thin lens, power of, 16
Spiral of Tillaux, 139, 141f Superior oblique palsy, 152 Thioglycolate agar, 40
Spiraling, of VF, 54 Superior ophthalm ic vein, 162f, 164, 164f Thioridazine (Mellaril), retinal toxicity and, 349,
Splinter hem orrh ages, 304 Superior orbital fissure, 68f, 161, 162f 349f
Squam ous cell carcinom a (SCC) Superior palpebral artery, 169f 13q Deletion syndrom e, 133
of conjunctiva, 205–206, 206f Superior palpebral vein, 169f Thorazine, retinal toxicity and, 349
of eyelids, 43, 187–188, 188f Superior peripheral venous arcade, 169f 3 and 9 o’clock staining, in contact lens-related
of lacrim al sac, 193 Superior rectus m uscle, 63f, 139 problem s, 227
Squam ous papillom a Superior sagittal sinus (SSS) throm bosis, 85–86 Thygeson’s superficial punctate keratopathy, 212
of conjunctiva, 205, 205f Supernum erary puncta, 100 Thym oxam ine test, acute angle-closure glaucom a
of eyelids, 184, 185f Suprachoroidal space, 305 and, 273
of lacrim al sac, 193 Supranuclear control, eye m ovem ents, 56–58 Thyroid-related ophthalm opathy, 174–175, 174f
Squam ous tum ors, of conjunctiva, 205 Supranuclear palsy, facial nerve palsy, 69 Tic douloureux, 67
Stains, of cornea, 199 Supraorbital artery, 163f, 169f Tilted disc, 137
Staph m arginal ulcer, 215 Supraorbital foram en, 162f Tissue fixation, 40–41
Staphylococcal disease, of conjunctiva, 204 Supraorbital vein, 169f artifacts, 41, 41f
Stargardt disease, 119, 119f Supratrochlear artery, 169f Tissue stains, 40
Static ophthalm oplegia, 73 Surface ectoderm , 38 Tissues, em bryonic, 38
Stationary cone disorders, 121 Surfactants, bioavailability of, 21 TM. See Trabecular m eshwork (TM)
Steel, intraocular foreign body, 45 Surgical iridectom y, 277–279 Tobram ycin, retinal toxicity and, 349
Steele-Richardson-Olszewski syndrom e, 60 Sutures, of lens, 288 Tonom etry
Stereoacuity, 83 Sweat gland, of eyelid, 170t applanation, 264
Stereopsis, types of binocularity, 141 Sweat gland tum ors, of eyelids, 185 in intraocular pressure, testing of, 264
Steroids, 26, 26t Sym blepharon, 200 Topical drugs, 21–22
diabetic retinopathy therapy, 333 Sym pathetic nervous system , 23, 23t anaesthetics, 22
Stevens-Johnson syndrom e, 211 Sym pathetic ophthalm ia, panuveitis and, 255–256, carbonic anhydrase inhibitors, 25
Stickler’s syndrom e, 128, 128f 255–256f Topography, for cornea, 199
Stocker’s line, 214 Sym pathetic plexus, 70f Toric lens, contact lenses and, 12
Strabism us, 139–156 Synchisis scintillans (cholesterol bulbi), 44, 314 Total internal reflection, 2–3
am blyopia in, 144 Synergist, m otor testing, 144 Toxic Anterior Segm ent Syndrom e (TASS),
anatom y of, 139–147 Synkinetic reflex, spasm of near, 150 com plications of cataract surgery, 296
com plications of cataract surgery, 295 Syphilis, pediatrics, 105 Toxic cataracts, 290
extraocular m uscles, 139–140, 140t, 140–141f Syringom a, of eyelids, 185 Toxic keratoconjunctivitis, 202
m otor testing in, 144–147 System ic disease, cataracts and, 290–291 Toxic optic neuropathy, 77–78
pediatric eye exam ination in, 140–141 System ic drugs, 22 Toxic retinopathies, 349
physiology of, 139–147 System ic lupus erythem atosus, corneal ulcers and, Toxocariasis, 112, 112t, 112f
sensory testing in, 141–143 215 posterior uveitis and, 244
special form s of, 154–155 System ic lym phom a, of orbit, 178 Toxoplasm osis, 111–112, 111f
subconjunctival fascia in, 139, 139f posterior uveitis and, 244
surgery, 155–156 Trabecular m eshwork (TM), 262–263, 262f
com plications of, 156 T Trabeculectom y, in glaucom a, treatm ent of,
Strabism us fixus, 154 276–277, 276t
Strabism us surgery, thyroid-related ophthalm opathy, Tadalafil (Cialis), 351 Trachom a, 204
174–175 Tafluprost (Zioptan), 25 Trachom a inclusion conjunctivitis (TRIC), 204

411
Index

Traction RD, 355 Ultrasound, 171, 171t, 310–311 Vergence (Continued)

Transm ission, of light, 2 A-scan, 310, 310f im ages of, 4–5
Transpupillary therm otherapy, choroidal m alignant B-scan, 310, 311f lens of, 4
m elanom a and, 362 choroidal cavernous hem angiom a, 359, 359f m otor testing, 144
Transverse m agnification, 7 internal structure, 311 objects of, 4–5
Transverse relaxation tim e (T2), 171 sound attenuation , 311 parallel rays of, 4
Traum a specific lesions, 311, 311t, 311–312f Vergence system , eye m ovem ents, 56
anterior cham ber, disorders of, 283 vascularity, 311 Verhoeff Van Gieson stain, 40
blunt, 44–45 Uncal herniation, 64 Vernal keratocon junctivitis (VKC), 101, 202
cataracts and, 290 Underlying lens, prism atic effect of, 4 Vernier acuity, 9
in corneal, 209 United Kingdom Prospective Diabetes Study Verruca vulgaris (papillom a), of eyelids, 182
eyelid disorders, 180 (UKPDS), 333, 336 Versions, m otor testing, 144
facial nerve palsy, 69 United Kingdom Prospective Diabetes Study - Vertebrobasilar dissection, 85
m acular hole and, 317–318 Hypertension in Diabetes Study Vertebrobasilar insufficiency (VBI), 85
m ydriasis and, 284 (UKPDS-HDS), 336 Verteporfin in Photodynam ic Therapy (VIP)
optic neuropathy, 78 Unoproston e isopropyl (Rescula), 25 trial, 322
orbital disorders, 172 Upbeat nystagm us, 62 Verteporfin in Photodynam ic Therapy-Pathologic
penetrating, 45 Upper eyelid Myopia (VIP-PM) trial, 324–325
retinal tear and, 315–316 fat pads, 165 Vertex distance conversion, lenses and, 5
sequelae of, 45 lam ellae of, 166, 167f Vertical deviations, pediatrics, 151–154
Travatan (travoprost), 25 retractors of, 168, 168f Vertical gaze center, 52, 53f
Travoprost (Travatan), 25 Urea, 25 Vertical retraction syndrom e, 154
Treacher-Collins syndrom e, 97 Usher’s syndrom e, 124 Vesicles, HSV infections and, 216
Treatm ent of Age-Related Macular Degeneration with Uveal m eshwork, 262 Vesicular blepharitis, 216
Photodynam ic Therapy (TAP) trial, 322, 324 Uveal tract, blunt traum a and, 45 Vestibular nystagm us, 62
Triazoles, 30 Uveitic glaucom a, in anterior uveitis, 241 Vestibulo-ocular reflex (VOR), 56
TRIC. See Trachom a inclusion conjunctivitis (TRIC) Uveitic secondary open-angle glaucom a, 272 VH. See Vitreous hem orrhage (VH)
Trichiasis, 184 Uveitis, 239–260 Viagra (sildenafil), 351
Trichinosis, of orbit, 173 anterior, 239–243 Vidarabin e, 30
Trichofolliculom a, of eyelids, 186 classification of, 239 VIEW 1 & 2 trials, 322
Tricholem m om a, of eyelids, 186 differential diagnosis of, 257–258 Viral infections
Tricyclic antidepressants, ocular toxicity and, 31 endophthalm itis in, 252–253 conjunctivitis and, 203
Trifluorothym idine, 30 infections and, 244–248 of orbit, 173
Trigem inal nerve (CN 5), 63f, 68f, 70f inflam m ations and, 248–252, 248t Viscoelastic device, cataract surgery and, 294
palsy, 67, 68f interm ediate, 243 Viscosity, bioavailability of, 21
Trigem inal neuralgia, 67 m asquerade syndrom es of, 257 Visual acuity
Trilateral retinoblastom a, 132 panuveitis in, 253–256 children, testing and, 9
Trim ethoprim , 28 pathophysiology of, 239 near acuity m easurem ents, 9
Trisom y 13, intraocular cartilage and, 45f pediatrics, 110–112 pupil size, 9
Tritanom aly, color blindness, 130 anterior, 110–111 Visual agnosia, cortical lesions, 80
Tritanopia, 130 interm ediate, 111 Visual confusion, sensory adaptations to, 142–143,
Trochlear nerve (CN 4), 63f, 70f, 162f posterior, 111–112 142–144f
palsy, 65–66, 65f posterior, 243–252 Visual disturbances, 83–84
True exfoliation, lens capsule abnorm alities surgery and, 258 functional visual loss, 83
and, 291 Uveitis-glaucom a-hyphem a, secondary open-angle transient visual loss, 83–84
Tscherning-Schachar theory, of accom m odation, 11 glaucom a and, 272 Visual extinction, cortical lesions, 80
Tuberculosis, panuveitis and, 256 Uveitis-glaucom a-hyphem a (UGH) syndrom e, Visual field defects, 54–56, 55f, 265–266, 266f
Tuberculum sellae, 163f com plications of cataract surgery, 296 localizing, 55
Tuberculum sellae m eningiom a, 79 Uveoscleral outflow, 262 neurologic, 55–56
Tuberous sclerosis, 135–136 prim ary open-angle glaucom a and, 267–270
Tum or cells, secondary open-angle glaucom a and, types of, 54–55
270 V Visual fields, testing for, 265–266, 265f
Tum ors, 46b Visual hallucinations, 84
brain, 81, 81t Valsalva retinopathy, 317 Visual neglect, cortical lesions, 80
caruncle, 208 Vancom ycin, 29 Visual obscuration, 83
cerebellopontine angle tum ors, 81 Vardenafil (Levitra), 351 Visual pathway
of ciliary body, 288 Varicella zoster virus (VZV), of eyelids, 182 anatom y of, 49–52, 50f
congenital, 205 Varix, 94 physiology of, 52–58
of conjunctiva, 204–209 Vascular diseases, posterior segm ent disorders and, Visual pigm ents, 305, 305t
cystic, 205 328 Visual testing, 52–54
of eyelid, 184–191 Vascular supply Visually evoked cortical potentials (VEP), 54
facial nerve palsy, 70 of eye, 161–164, 163–165f Visually evoked cortical responses (VER),
of hair follicle origin, 185 of eyelid, 169, 169f 54, 54f
intraocular, secondary angle-closure glaucom a Vascular tortuosity, 329 Vitelliform dystrophy, 119–120, 120f
and, 274 Vascular tum ors Vitiliginous chorioretin itis, 248t, 250, 250f
of iris, 287 of conjunctiva, 207 Vitiligo, 184
of iris pigm ent epithelium , 287 of eyelids, 190 Vitrectom y
of lacrim al sac, 193 Vascularity, ultrasound, 311 indications for, 333
m elanocytic, 206 Vasculature in traum atic, secondary open-angle glaucom a
optic nerve, 78 chiasm , 50, 51f and, 272
orbital disorders and, 175–180, 175t lateral geniculate body, 52 Vitreolysis, Nd:YAG laser and, 292
pediatrics, 102 optic nerve, 49, 51f Vitreoretinal degenerations, pediatrics,
pituitary gland, 79 VBI. See Vertebrobasilar insufficiency (VBI) 128–129
posterior fossa, 81 VEGF Inhibition Study in Ocular Vitreoretinal dystrophies, pediatrics, 127–128
posterior segm ent disorders and, 357–365 Neovascularization trial (VISION), 322, Vitreoretinal junctions, 301
Tunica vasculosa lentis, 288 325–326 Vitreoretinal tuft, 351–352
Tyrosinem ia type II, 214 Venous throm bosis, cerebral, 85–86 Vitreoretinopathy, proliferative, 355, 355f
Vergence, 4–7 Vitreous, 261
basic lens form ula, 4 abnorm alities, 314–315
U convergence of, 4 anatom y, 301, 302f
diopter of, 4 em bryology of, 36–37, 37f
Uhthoff’s sym ptom , 76, 83 divergence of, 4 vitreoretinal junctions, 301
Ultrasonography, 15 form ula of, 15 vitreous base, 301

412
 Index

Vitreous base, 301 Werner’s syndrom e, 228, 290 X-linked foveal retinoschisis, electroretin ogram ,
avulsion, 316 Whiplash retinopathy, 317 308
Vitreous hem orrhage (VH), 315 Whipple’s disease, 61 X-linked incom plete blue cone m onochrom at,
uveitis and, 257 posterior uveitis and, 247 electroretin ogram , 307f
Vitritis, 243–244 White lim bal girdle of Vogt, 213 X-linked retinoschisis, electroretinogram , 307f
VKC. See Vernal keratoconjunctivitis (VKC) Whitnall’s ligam ent, 168, 168f
Vogt-Koyanagi-Harada syndrom e (VKH), panuveitis Wieger’s ligam ent, 302f
and, 254–255, 255f Wilder stain, 40 Y
Voluntary nystagm us, 62 Wilson’s disease, 214
Von Hippel-Lindau disease, 135 cataracts and, 290 Yeast, cornea and, 219
von Kossa stain, 40 "With-the-rule," astigm atism and, 10 Yellow foveal spot, 321
von Recklinghausen’s syndrom e, 134–135 Wolfram syndrom e, 138 Yoke m uscles, m otor testing, 144
VOR. See Vestibulo-ocular reflex (VOR) Wolfring lacrim al gland, 170t
Vortex keratopath y, 214 Wounds, 44
Vortex vein, 164, 164f burns, com plications of cataract surgery, 295 Z
Vossius ring, 45 com plications of, 44
Zeis gland, 170t
cataracts and, 290 healing of, 44
Zim m erm an hypothesis, 362–363
Wyburn-Mason syndrom e, 136
Zioptan (tafluprost ), 25
Zonula adherens, 304
W Zonular traction tuft, 352
X Zonules, of lens, 288
Waardenburg-Jonkers corneal dystrophy, 223
Waardenburg’s syndrom e, 98 Xalatan (latanoprost), 25 Zoster sine herpetae, 219
Wagner syndrom e, 128 Xanthelasm a, of eyelids, 190–191 Zygom atic bone, 162f
Waldenstro€ m ’s m acroglobulinem ia, 178 Xanthogranulom a, necrobiotic, of eyelids, 191 Zygom atic (tripod) fractures, 172
Waters view, of radiographs, 171 Xanthophyll, laser absorption and, 366 Zygom aticofacial artery, 163f
Watze-Allen sign, 317 XECD. See X-linked endothelial corneal dystrophy Zygom aticofacial canals, 161
Weber’s syndrom e, 64 (XECD) Zygom aticofacial foram en, 162f
Wegener’s granulom atosis, 175 Xeroderm a pigm entosa, of eyelids, 187 Zygom aticotem poral artery, 163f
corneal ulcers and, 215 Xerophthalm ia, 210–211 Zygom aticotem poral canals, 161
in sclera, 230 X-linked endothelial corneal dystrophy (XECD), 104, Zygom aticotem poral foram en, 162f
Weill-Marchesani syndrom e, 292 226

413
This pa ge inte ntiona lly le ft bla nk