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HYPOSPADIAS
LUMINA.S M.Sc nursing (child health) DEFINITION
‘’Hypospadias is a developmental anomaly characterized by
a urethral meatus that opens on to the ventral surface of the penis, proximal to the end of the glans’’ (ASHCRAFT) EMBRYOLOGY Normal Phallic development occurs in weeks of 7-14 WOG By 6 WOG ,genital tubercle is formed anterior to the urogenital sinus In the next week, two genital folds form caudal to the tubercle & a urethral plate forms between them The formation of the penile urethra is thus generally completed by the end of 1st trimester CONT…. This sequence probably accounts for the predominance of glanular & coronal hypospadias Mesenchymal tissues dorsal to the developing urethra forms Buck’s fascia, Dartos fascia & Corpus spongiosum It is the tissue that normally surrounds the urethra & communicates with erectile tissue of glans CONT…. Buck’s fascia is the deep layer of fascia that surrounds corpus bodies & invests spongiosum
Superficial to this layer is Dartos fascia which is loose
subcutaneous layer that combines superficial veins & lymphatics
These structures form subsequent to completion to
urethra by medial fusion of outer genital folds, proceeding from proximal to distal aspect of penis CONT…. This development accounts for how a fully formed urethra can have a poorly formed spongiosum with thin overlying skin & ventral tethering , the meatus being located at the tip of the glans Finally ,the prepuce is formed originating at the coronal sulcus It gradually encloses the glans circumferentially Arrested development of the urethra may leave the meatus located anywhere along the ventral surface of penis CONT….
Typically this would lead to foreshortening
of the ventral aspect of the penis distal to meatus & failure of prepuce to form circumferentially HISTORICAL PERSPECTIVES The first description of Hypospadias & its surgical correction was reported in the 1st & 2nd centuries by the Alexandrian surgeons, Heliodorus & Antyllus
In 1874,Theophile Anger reported the successful
repair of a Penoscrotal hypospadias using technique of 1869 in which lateral skin flaps were tubularized to form the neourethra CONT…. Edmond in 1913 was the first to describe the transfer of prepuital skin to the ventral aspect of penis
Improved techniques in preputial & meatal-based
vascularized flaps since the 1970s -1980s have greatly advanced hypospadias repair 1. INCIDENCE Incidence estimated to be between 0.8 & 8.2 per 1000 live births
If minor hypospadias are included ,incidence is 1 in
125 live male births
Familial tendencies have been identified in 8% of pts.
CONT…. 14% of male siblings of an index child have hypospadias & if 2 members of family have hypospadias ,the risk for the disorder is 21%
Cause is still unknown ,but most likely polygenic
because of higher familial incidence 2.CLASSIFICATION 1) ANTERIOR HYPOSPADIAS (71%) Glanular- Meatus situated on the inferior surface of the glans Coronal Anterior Penile
2) MIDDLE HYPOSPADIAS (16%)
Meatus situated in the middle third of the shaft 3)POSTERIOR HYPOSPADIAS (13%)
Meatus situated in the posterior third of the shaft
Penoscrotal - Meatus at base of the shaft in front of the scrotum Scrotal- Meatus behind the scrotum Perineal- Meatus behind genital swellings 3.Etiology A combination of Endocrine, Environmental & Genetic factors determine risk for developing HYPOSPADIAS A defect in the androgen stimulation
It is the ultimate cause of hypospadias
Diminished response to HCG in some pt. with hypospadias CONT… Higher incidence in winter conceptions Weak association between maternal ingestion of progestin like agents with disease No association between oral contraceptives use before /in early pregnancy 5.Associated anomalies Inguinal Hernia & undescended testis ( common) An enlarged prostatic utricle Infection Urinary tract anomalies Associated myelomeningocele & Imperforate Anus Upper/lower tract obstructions/ anomalies ,hematuria & family history of urinary tract abnormalities Inter sex state Elements of Hypospadias anomaly Meatal Dystopia o Urethral meatus may only be slightly displaced in a direction immediately ventral, below a blind dimple at the normal meatal opening on the glans o On the other hand, urethra may be displaced so far back in the perineum (Vaginal hypospadias) o More distal locations are often associated with a stenotic meatus Dermal defects o The skin of the penis is radically changed as a result of disturbance in the formation of urethra o Distal to meatus- skin shows a V-shaped defect(Van Der Meulen) o Lateral- 2 raphes obliquely extend from the edge of urethral delta on the dorsum 1cm apart o Proximal- thin skin o Urethral plate is well developed o Undeveloped act as a tethering fibrous band that bend the penis ventrally in artificial erection Penile Curvature o Caused by deficiency of the normal structure on the ventral side o A strand of CT has been stretched like a cord between meatus & glans giving rise to bowstringing Penoscrotal Transposition & Bifid Scrotum o Penis may be caught between the 2 scrotal halves & become engulfed with fusion of the penoscrotal area o Boundary between penis & scrotum may be formed by 2 oblique raphes that extend from the very proximal meatus to dorsal side of the penis Chordee Without Hypospadias Defect in the course of urethra (congenital urethral fistula) & as a curvature of the penis without Hypospadias DIAGNOSIS Treatment Repair done on Out Patient basis Defect is not ‘’TOO MILD’’ & risk of complications are not ‘’TOO HIGH’’ Chance to make the normal phallus as normal as possible should be offered to all children regardless of severity of the defects Age at repair Most cases repair in 1st year of life Most surgeons prefer to do in 6months-18months of age Anesthetic risk is low, post-operative care is easier for parents than when child is at toddler age group Objectives of repair 1)Complete straightening of penis 2)Placing meatus at the tip of the glans 3)Forming a symmetrical conically shaped glans 4)Constructing a neo-urethra uniform in caliber 5)Completing a satisfactory cosmetic skin coverage 1) STRAIGHTENING
Artificial erection by injection physiologic Saline in
the corpora at the time of operation allows determination of exact degree of curvature Curvature secondary to true fibrous chordee requires division of urethral plate & excision of fibrous tissue down to the tunica albugenia CONT… True deficiency of ventral corporal development can be treated by making a releasing incision in the ventral tunica albugenia
Axial rotation of the penis /penile torsion is managed
by releasing the dartos layer as far as proximal as possible on the penile shaft 2) PLACING THE MEATUS
Placing the meatus at the tip of the glans has not
always been standard in hypospadias repair Risk of complications to be too great Surgical techniques- glans channeling & glans splitting technique are used to reduce complications Meatoplasty with or without dorsal advancement distal urethral mobilization & tubularization /meatal based flaps are most selected methods CONT… Proximal – creating neourethra with local vascularized skin flaps/free grafts allows placement of urethra at the end of penis In glanular channeling/glans splitting accomplishes placement of meatus at the tip of glans 3) Glans Shape Conically shaped glans is the objective of the glansplasty component of repair Approximation of well-developed glans wings to the midline over a neourethra ,in a split glans restores the glans to its normal conical shape 4) Urethral Construction
Formation of neourethra can be accomplished with local skin
flaps, various types of free grafts /vascularized pedicle flaps Avoid flaps too thin to avoid risk of less blood supply
Mobilized vascularized flaps of preputium have more relate
blood supply than do free grafts
A water tight closure of the well vascularized neourethra is
formed with care being taken to make it uniform in caliber & of appropriate size for the age of child Skin flaps in proximal hypospadias Surgical procedures Because of wide variations in anatomic presentations of hypospadias, NO SINGLE URETHROPLASTY is applicable to every case
The versatility & experience to deal with all
variants & options of surgical treatment are KEY successful management Specific techniques 1) ANTERIOR VARIANTS Some glanular variants are amenable to MEATAL ADVANCEMENT & GLANULOPLASTY(MAGPI) A stenotic meatus with good mobility of urethra & a fairly shallow ventral groove –best suited
Meatal based flap repair may be used if chordee is not
present & if mobile well vascularized skin exists proximal to the meatus CONT… Glans approximation procedure is useful when a wide mouthed proximal glanular meatus exists with a very deep groove Pyramid procedure is well suited for the fish mouth type of meatus & gives a good cosmetic result
Tubularized Incised Plate Urethroplasty (TIP) involves a
deep longitudinal incision of the urethral plate in the meatus It allows the lateral skin flaps to be mobilized & closed in midline without tension Tubularized Incised Plate Urethroplasty (TIP) 2) MIDDLE VARIANTS
When no significant chordee is present, TIP repair / Meatal
based flap can sometimes be done Onlay Island Flap Repair- mobilizing an inner preputial flap on its pedicle & rotating it ventrally to lay it on the well,developed ventral urethral plate to complete the tubularization of neourethra lateral to the urethral plate It can be used instead of tubularization pedicle flap which has higher incidence of complications 3) PROXIMAL VARIANTS
1) Staged procedure in which coverage of the ventrile penile
shaft is attained by rotation of dorsal flap to the ventrum with later tubularization to form the neourethra technical perspectives Optical magnification : 2.5power-4.5power is standard Sutures & instruments: 6.0-7.0 size sutures, small single toothed forceps & delicate instruments are preferred a)Bleeding complications b)Infection c) Devitalized skin flaps d)Fistula e)Strictures f) Diverticulum/ureterocele g)Persistent Chordee h)Recurrent multiple complications i) Sexual function THANK YOU
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