Vascular disorders

Dr. Eri Siti Khoeriyah , SpPK, MKes

Abnormal bleeding may result from:
1- Vascular disorders
2- Thrombocytopenia (low platelets)
3- Platelet function defects
4- Defective coagulation
Vascular bleeding disorders:

Are heterogeneous group of conditions characterized by

easy bruising & spontaneous bleeding from small vessels

The underlying abnormalities are either in the vessels

themselves or in the perivascular connective tissue

VASCULAR DISORDERS THAT CAN CAUSE
BLEEDING

• Inherited
• Acquired
• Infiltrative disease
• Vasculitis only)
1. Inherited vascular disorders
Hereditary haemorrhagic telangiectasia
 autosomal dominant trait
 dilated microvascular swellings which appear
during childhood and become more numerous in
adult life
 These telangiectasia develop in the skin, mucous
membranes and internal organs. Pulmonary and
cerebral arteriovenous malformations are seen in a
minority of cases. Recurrent epistaxes are
frequent and recurrent gastrointestinal tract
haemorrhage.
Connective tissue disorders

 Ehlers-Danlos syndrome there are hereditary

collagen abnormalities with purpura resulting from
defective platelet aggregation, hyperextensibility
of joints and hyperelastic skin
 Pseudoxanthoma elasticum is associated with
arterial haemorrhage and thrombosis.
 Mild cases may present with superficial bruising
and purpura following minor trauma
Giant cavernous haemangioma

 congenital malformations occasionally

cause chronic activation of coagulation
leading to laboratory features of DIC
and in some cases thrombocytopenia.
 2- Acquired vascular defect:
 Simple easy bruising is common in
healthy women especially of
childbearing age.
 Senile purpura due to atrophy of
supporting tissues of cutaneous blood
vessels.
 Purpura associated with infections:
many bacteria, viral or rickettsial
infections may cause purpura from
vascular damage by microorganism or as
a result of immune complex formation
*Scurvy: In vitamin C deficiency, defective collagen may
occure may cause perifollicular petechiae, bruising &
mucosal hemorrhage.
*Steroid purpura associated with long standing steroid
therapy or Cushing syndrome cased by defective
vascular supportive tissue.
*Other: e.g. auto erythrocyte sensitization, DNA
sensitivity,& fat embolism.
The Henoch –Sconlein syndrome

 an immune complex (type III) hypersensitivity reaction

usually found in children often following acute infection

 The characteristic purpuric rash accompanied by localized

edema & itching is often most prominent on the buttocks
&extensor surfaces of lower legs & elbows
 Painful joint swelling, haematuria & abdominal pain
 usually self limiting but occasionally patients develop renal
failure.
b a
 Arterial and Venous Disease

ARTERIAL DISEASE
 Also known as Peripheral Vascular Disease (PVD) or
Peripheral Arterial Disease (PAD), lower extremity
occlusive disease
 = progressive narrowing or occlusion of lower extremity
arteries resulting in decreased blood flow to limbs,
thereby decreasing the amount of oxygen and nutrients
delivered to tissues
Causes of PAD

1. Atherosclerosis 2. Others
 Most common etiology  Aneurysms (hereditary or acquired)
 Closely associated with  Trauma / Radiation
coronary artery disease (CAD),  Infection
cerebrovascular diseased
 Functional spasms (eg, Raynoud
(CVD), AAA, renal artery syndrome/dz)
stenosis, mesenteric ischemia,
and their risk factors  Vasculitis (eg, Buerger’s disease, aka
thromboangitis obliterans)
 Anatomic abnormalities (eg, popliteal
entrapment syndrome in young pts)

http://www.healthfixdaily.com/?p=556
Major Risk Factors for PAD

 Diabetes mellitus
 Current smoking
 Hypercholesterolemia
 Age > 75 yrs

* based on U.S. NHANES and Framingham Heart Study data

Clinical Presentation of PAD

 Progressive or acute
 Varying levels of severity, including life- or limb-threatening
 Asymptomatic: 20-50%
 Atypical leg pain: 40-50%
 Classic claudication symptoms: 10-35%
 Critical limb ischemia (or limb-threatening ischemia): 1-2%
 Neurologic or abdominal symptoms
Mild Intermittent Claudication

 Classic / typical
Intermittent cramping pain or discomfortoccurs consistently and reproducibly

with exertion, causing to stop walking, and is relieved by rest

 Atypical :

 Similar to classic, but not severe enough to cause pt to stop walking or

may not be relieved with rest

 Pain beginning at rest

Critical Limb Ischemia
 Ischemic rest pain
 Represents more severe decrease in limb perfusion; can lead to ischemic
neuropathic pain (described as burning or throbbing pain)
 Pain at rest, typically at night and involving foot and toes. Often
paradoxically relieved w/ hanging feet over edge of bed or walking
around
 Ischemic ulcers and/or gangrene
 Acute limb ischemia
 Rapid or sudden decrease in limb perfusion that treathens tissue / limb
viability. A vascular emergency!
 5 (or 6) P’s: PULSELESSNESS, PAIN, PALLOR, (PERISHING COLD or
POIKYLOTHERMIA), PARESTHESIAS, PARALYSIS
 High risk of limb amputation(25%) and CV mortality (25%) at 1 year
Acute limb ischemia

Images courtesy of Mr. Nikolas Kosanovic M.D, General Surgery, Rural Clinical School, University of Melbourne
Other associated signs of PVD
 Skin changes  Diminished distal pulses
 Thin, brittle, and shiny  Toenail changes
 Cool temperature (thickened and opaque)
 Dusky erythema  Impotence
 Hair loss  Weakness / decreased
 Ulcers or non-healing mobility / muscle
wounds over pressure atrophy
points
 Pallor when legs
elevated

Image courtesy of Mr. Nikolas Kosanovic M.D, General Surgery, Rural Clinical School, University of Melbourne
Arterial Ulcers

 Characteristics
 Often located on toes or
pressure points
 Pale or cyanotic appearance
with irregular margins
 Painful, sometimes severe,
often at night
 Surrounding skin shiny

Images courtesy of http://www.vascularcarecentre.com/i-have-leg-ulcer-what-should.php and http://www.medschoolforums.com/showthread.php/review-leg-ulcers-215.html

Evaluation of PAD
 History
 Physical examination
 Diagnostic testing
 Ankle-brachial index (ABI)
 Segmental Doppler pressures
 Duplex ultrasound imaging
 Contrast Angiography
 CT/MRI Angiography
Buerger’s test

 Patient is lying supine

 Raise the foot and leg at 45 degrees
or less
 Watch the leg becoming pale
 Ask patient to sit up and hang the leg
of the end of bed
 If leg becomes blue then red it’s a
positive test (hyperactive
hyperemia)

Image courtesy of Mr. Nikolas Kosanovic M.D, General Surgery, Rural Clinical School, University of Melbourne
Medical Treatment of PAD
 Stop Smoking
 Aspirin , clopidogrel
 Treat underlying disease: hypercholesterolemia, DM
 ACE inhibitor for CV risk reduction
 intermittent claudication: cilostazol
Surgical Treatment of PAD

 Indications for surgical intervention:

 ischemic rest pain, tissue loss, frank gangrene
 Peripheral atheroembolisation
 not responsive to medical therapy
 For interm. claudication, revascularization with:
 Endovascular revascularization / percutaneous balloon
angioplasty
 Stents (usually reservered for aortoiliac lesions)
 For critical limb ischemia
 Bypass grafts
 Amputation
Venous disease

 chronic dilation or reflux of veins in the lower

extremity
 Independent of peripheral arterial disease, but can be
just as debilitating.
 Venous Anatomy
 Superficial, deep, and perforating / communicating veins
 One-way valves prevent pooling of blood in legs
 Muscle contractions help facilitate blood return to heart
 Flows superficial deep veins

Images courtesy of http://emirateshospital.ae/ar/index.php?option=com_content&view=article&id=136:varicocele&catid=7:urology&Itemid=14 and

http://www.arkansasvein.com/varicoseveins.html
Lower Extremity Venous Anatomy
Superficial Veins Deep Veins

Images courtesy of Up-To-Date Overview and management of lower extremity chronic venous disease and
http://www.health.com/health/library/mdp/0,,zm2346,00.html
Chronic Venous Disease / Insufficiency

 Chronic Venous Disease = arise from

venous valve incompetence resulting in
retrograde blood venous blood flow,
lasting for an abnormal duration and
with associated signs / symptoms
 Chronic Venous Insufficiency =
represents more advanced state of
above, including edema, skin changes,
and ulcerations

http://advancedvenoussolutions.com/why/insufficiency.ht
ml
Clinical Signs of Venous Disease
Symptoms Signs
 Limb discomfort (tiredness,  Evidence of dilated veins,
heaviness) including telangiectasias,
reticular veins, and varicose veins
 LE pain (generalized achiness or
localized) or swelling  Leg oedema
 Worse w/ standing  Can be unilateral in early stages

 Improves w/ elevation or walking  Often localized only to legs/feet

 Paresthesias (tingling, burning)  Skin changes

 Tightness in legs  Ulcers
 Skin irritation/itching or  Haemorrhaging
discoloration / redness  Superficial thrombophlebitis
 Bleeding
 Muscle cramps
 Generalized fatigue
Oedema

Mild Moderate to severe

Reticular veins are dilated bluish Significant pitting oedema of lower leg
subdermal veins, one to three with skin changes consistent with chronic
millimeters in diameter. The deeper blue venous stasis, including stasis dermatitis
reticular veins contrast the bright and lipodermatosclerosis.
red,fine telangiectasias. Mild ankle
oedema in this patient is evident at
medial ankle below the malleolus.
Images courtesy of Up-To-Date Clinical evaluation of lower extremity chronic venous disease and
http://ookaboo.com/o/pictures/topic/12340870/Edema
Differential Dx for LE oedema
 Increased capillary hydrostatic  Increased capillary permeability
pressure  Trauma
 CHF, including cor pulmonale
 Burns
 Renal disease
 Inflammation
 Medication effect causing Na
 Sepsis
retention (Ca-channel blockers)
 Allergic reactions
 Pregnancy
 ARDS (Adult Respiratory Distress
 Localized venous obstruction (eg,
Syndrome)
thrombus, valve incompetence)
 Diabetes mellitus
 Cirrhosis or hepatic venous
obstruction  Malignant ascites
 Acute Pulm Oedema  Iatrogenic, IL-2 therapy
 Hyopalbuminemia (ie, decreased  Lymphatic obstruction (or increased
oncotic pressure) interstitial oncotic pressure)
 Nephrotic syndrome  Lymph node dissection
 Protein-losing enteropathy  LN enlargement due to malignancy
 Liver disease  Hypothyroidism
 Malnutrition  Malignant ascites
Chronic Skin Changes

 Leathery, thick skin at ankle or leg

due to irritation / scratching, but
unerlying skin is thin and fragile and
can easily ulcerate
 Stasis dermatitis = Hyper-
pigmentation from hemosiderin
deposition creating a reddish-brown
appearance with diffuse or spotty
pigmentation
 Lipodermatosclerosis
 Pale or white lesions
 Autoeczematous reaction

Image courtesy of Up-To-Date Clinical evaluation of lower extremity chronic venous diseases
Stasis Dermatitis
 Aka “congestion or stasis
eczema”
 Hyper-pigmentation from
hemosiderin deposition creating a
reddish-brown appearance with
scaliness in diffuse or spotty
pattern
 An inflammatory process that
presents insidiously w/ itching,
skin discoloration, scale, thin skin
of one or both legs
 Commonly effects medial
malleolus region first

Image courtesy of Up-To-Date Clinical evaluation of lower extremity chronic venous diseases
Lipodermatosclerosis

 Localized chronic inflammation

(panniculitis) leading to fiborsis of
the skin and subcutaneous tissues
of the lower leg
 Skin appears hyper-pigmented,
red, scaly, and hard or indurated
 Can involve most of the leg
circumferentially
 “Inverted champagne bottle
shape”

Images courtesy of Up-To-Date Clinical evaluation of lower extremity chronic venous diseases, Mr. Nikolas Kosanovic M.D, General Surgery, Rural Clinical School, University of Melbourne, and
http://dermnetnz.org/doctors/wound-healing/leg-ulcers.html
Atrophie blanche

 Localized, usually circular

atrophic, pale to whitish skin areas
surrounded by dilated capillaries
(telangiectasias) and
hyperpigmentation
 Most often seen on the medial
distal leg near the malleolus, or
can occur within
lipodermatosclerotic skin, where
they correspond to points of
avascular fibrosis

Image courtesy of Up-To-Date Clinical evaluation of lower extremity chronic venous diseases
Autoeczematous reaction
 Eczematous, dry, scaly rash can cause difficult-to-control
pruritis. Can lead to many excoriations from scratching,
that can be a source of infection.
 Rashes mimicking the dermatitis on the legs can appear as
eczematous patches on other body sites, or can present as
a generalized body rash, an auto-eczematous or "id"
reaction

Up-To-Date. Classification of lower extremity chronic venous disorders.

Varicose Veins
 Dilated, elongated, tortuous
subcutaneous veins ≥3 mm in
diameter
 Incompetent venous valves

Images courtesy of Up-To-Date Overview and management of lower extremity chronic venous disease and
http://en.wikipedia.org/wiki/Varicose_veins
 Varicose Veins
 Prevalence
 Generally F > M, 10-30% ,increases with age
 Risk Factors:
 Age, female, obesity, lifestyle, prolonged standing,
smoking, trauma to LE, prior venous thrombus (deep
or superficial), pregnancy / high estrogen states, lax
ligaments (flat feet, hernias), AV fistulas
Varicose Veins

Primary Secondary
 Varicose veins caused by venous  Varicose veins caused by venous
insufficiency due to venous wall insufficiency due to venous
weakness damage from other etiology
 Example: due to age or pregnancy  Example: deep vein thrombosis or
leg injury
Clinical Signs of Varicose Veins

 Similar to venous insufficiency

 Dilated superficial vessels
 Itching
 Pain
 Bleeding – can be quite significant
 Skin changes
 Tired, achy, heavy legs
Clinical Evaluation for Venous Insufficiency and
Varicose Veins

 Inspection
 Palpation
 Neurologic
 Try to localize incompetent vein
Trendelenburg Test

 Ask the patient to lie down

 Elevate leg to empty engorged veins
 Press on the saphenofemoral junction
 Locate the femoral artery
 The saphenofemoral junction is
medial and 2cm below it
 Stand patient up and watch for
varicose veins
 If varicose veins are controlled the
saphenofemoral junction is
incompetent
 If not, there is an incompetent
perforator below

http://primumn0nn0cere.wordpress.com/2010/07/29/trendelenburg-test/
Tourniquet test
 Same principle as Trendelenburg
test
 Lie patient supine
 Elevate leg to empty engorged
veins
 Apply tourniquet to thigh, lower
thigh, then below knee and stand
patient up
 If varicose veins are controlled,
incompetence is above the
tourniquet
 If not it’s below tourniquet

http://orthoinfo.aaos.org/topic.cfm?topic=A00534
Complications of Chronic Venous
Insufficiency
 Cellulitis
 Venous ulcers
 Haemorrhage
 Thrombophlebitis

Up-To-Date. Clinical evaluation of lower extremity chronic venous disease. Copyright ©2006 The McGraw-Hill Companies. Access
Medicine.
Diagnostic Testing
 Clinical
 Duplex Ultrasound
 Preferred method (accurate, non-invasive,
reproducible, inexpensive)
 Assesses deep and superficial veins for presence and
direction of blood flow (therefore assesses for reflux
and obstruction)
 Descending venography
 Rarely used (uses contrast, expensive, invasive,
uncomfortable, risk of phlebitis)
Medical Management
 Avoid long periods of standing or sitting
 Exercise
 Weight loss
 Compression Stockings
 Leg elevation
 Medications: Aspirin, Pentoxifylline, Stanozolol (anabolic
steroid that stimulates fibrionlysis and may reduce lipodermatosclerosis)

No role for diuretics for edema purely due to CVI

 Skin care : cleansing, emollients, barrier creams
(petroleum, zinc oxide), topical steroids
Compression Stockings
Class Uses Pressure at Ankle
(mmHg)

I Mild venous insufficiency or varicose 14-17

veins

II Treatment and Prevention of Venous 18-24

Ulcer Recurrence

III Treatment of severe venous 25-35

hypertension and ulcer prevention in
large diameter calves
Compression Stocking Application

Up-To-Date. Medical management of lower extremity chronic venous disease.

Indications for Invasive Treatment
 Not responsive to medical therapy
 Large or multiple ulcers
 Perforator or saphenofemoral reflux

Contraindications:
 DVT

 Severe oedema
 Infection
Invasive Therapy for Varicose Veins

 Non-Surgical
 Sclerotherapy (chemical ablation)
 ThermalAblation (superficial surface lasers or
endovenous light/radiofrequency)
 Surgical
 Ligation and Stripping (mechanical ablation)
 Microincision and phlebectomy (mechanical ablation)
 Vein bypass
Venous Ulcers
 Characteristics
 Exquisitely tender
 Shallow
 Exudative w/ granulomatous red
base
 Irregular borders, but well
demarcated
 Usually located on distal leg over
medial aspect
 Multiple or single
 Can extend around circumference
of leg if not treated
 Surrounding skin
hyperpigmentation

Up-To-Date. Clinical evaluation of lower extremity chronic venous disease.

Medical Treatment of
Chronic Venous Ulcers
 Wound management
 Compression stockings
 Pentoxyfylline
 EMLA cream (lidocaine 2.5% and
prilocaine 2.5%)

Images courtesy of Mr. Nikolas Kosanovic M.D, General Surgery, Rural Clinical School, University of Melbourne
Surgical Management:

 Surgical debridement
 Skin grafting w/ bilayer artificial skin plus
compression bandages
 Venous surgery

Images courtesy of Mr. Nikolas Kosanovic M.D, General Surgery, Rural Clinical School, University of Melbourne
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