Download MBBS Urology Presentations 6 Congenital Anomalies of Penis Lecture Notes

Hypospadias, Epispadias and

Posterior urethral valves

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Department of Urology

Hypospadias

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? Any condition in which the meatus

occurs on the undersurface of the

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penis

? Usually 3 features

? ventral meatus

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? ventral curvature (chordee)

? Dorsal "hood"; deficient

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foreskin ventral y


Classification

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Embryology

? Genital tubercle fuses in

midline

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? Mesodermal folds

create the urethral and

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genital folds

? coalesce in midline as

phal us elongates

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? Distal glans channel

tunnels to proximal

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urethra as solid core

then undergoes

canalization

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Embryology

? Prepuce forms as ridge of

skin from corona

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? Hypospadias

? Failure of ventral aspect to

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form

? Dorsal hood

? Chordee

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? Differential growth

between normally

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developed dorsal tissue and

underdeveloped ventral

corporal tissue

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? Fibrous tissue distal to

hypospadiac meatus

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Incidence

? 1:300 live male births
? 6000 boys each year in the US
? Some genetic component

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? 8% of patients have father with hypospadias
? 14% of patients have male siblings with hypospadias
? If child with hypospadias, risk to next child

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? 12% risk with negative family history
? 19% if cousin or uncle with hypospadias
? 26% if father or sibling

? More common in Caucasians (Jews and Italians)

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? Higher incidence in monozygotic twins (8.5x)
Associated Anomalies

? Undescended testes 9% and inguinal

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hernia 9%

? Upper tract anomalies rare (1-3%)
? Utriculus masculinus

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? 10 to 15% in perineal or penoscrotal

hypospadias

? Incomplete mullerian duct regression

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Associated Anomalies

? Rule out intersex, especially with

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cryptorchidism

? Adrenogenital syndrome
? Mixed gonadal dysgenesis
? Incomplete pseudohermaphroditism

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? True hermaphrotidism
Associated Anomalies

? hypospadias and cryptorchidism

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? high index of suspicion for an intersex state

? Walsh reported the incidence of intersexuality in

children with cryptorchidism, hypospadias, and

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otherwise nonambiguous genitalia to be 27%

? nonpalpable testis were at least threefold more likely to

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have an intersex condition than those with a palpable

undescended testis (50% versus 15% )

Further Evaluation

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? Only with severe hypospadias and sexual ambiguity

? Includes testicular abnormalities
? Up to 25% of these patients have enlarged utricles or other

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female structures

? The incidence of abnormalities with other forms of

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hypospadias approximates that of the general

population

? Therefore no further evaluation is indicated

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Treatment

? Meatoplasty and glanuloplasty

? Multiple techniques

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? Orthoplasty

? Utilize artificial erection
? Release urethra from fibrous tissue

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? Plicate dorsal tunica albuguinea
? Ventral graft if needed

Treatment

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? Urethroplasty

? Onlay vascularized flap

? Tubularized flap

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? Free graft

? Skin cover

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? Mobilized dorsal prepuce and penile skin

? Double faced island flap

? Scrotoplasty

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Hypospadias Repair

? Over 150 operations have be described

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? Distal hypospadias

? Tubulization of the incised urethral plate (Snodgrass)
? Meatal advancement (MAGPI)
? Meatal-based flaps (Mathieu)

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? Proximal hypospadias

? Onlay grafts
? Vascularized inner preputial transfer flaps (Duckett)

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? Free grafts (skin, buccal mucosa)

MAGPI

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Mathieu

Island

Onlay

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Buccal Mucosal Graft

Epispadias

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Posterior Urethral Valves

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Posterior Urethral Valves (PUV)

? Congenital Proximal Urethral Obstruction
? Abnormal congenital mucosal folds in the

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prostatic urethra that look like a thin

membrane that impairs bladder drainage

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PUV Defined

? Type I

? Obstructing membrane that extends distally from each

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side of the verumontanum towards the membranous

urethra where they fuse anteriorly

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? Type II

? Described as folds extending cephalad from the

verumontanum to the bladder neck

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? Type II

? Represent a diaphragm or ring-like membrane with a

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central aperture just distal to the verumontanum

? Thought to represent incompelte dissolution of the

urogenital membrane

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Type I PUV

? Obstructing membrane radiating

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distally from the posterior edge

of the verumontanum to the

membranous urethra

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? During voiding, the fused anterior

portion bulges into the urethra

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with a narrow posterior opening

? Possibly due to anomalous

insertion of the mesonephric

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ducts into the primitive fetal

cloaca

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Type I PUV

Type I I PUV

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? Represent incomplete

dissolution of the UG

membrane

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? Distal to the

verumontanum at the

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membranous urethra

? Ring-like with a central

opening, "wind sock

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valve"
Incidence

? Males only

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? 1:5000 ? 8000 male births
? Type I > 95%
? Type II - 5%
? Children with Type II PUVs have a worse

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prognosis as a group

? 50% of patients with PUV will have

vesicoureteral reflux

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? 50% unilateral, 50% bilateral

Clinical Presentation

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? Varies by degree of obstruction

? Symptoms vary by age of presentation

? Antenatal

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? Bilateral hydronephrosis
? Distended and thickened bladder
? Dilated prostatic urethra
? Oligohydramnios - accounts for co-presentation of

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pulmonary hypoplasia.
Clinical Presentation

? Newborn

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? Palpable abdominal mass

? Distended bladder, hydronephrotic kidney

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? Bladder may feel like a small walnut in the suprapubic area

? Ascites

? 40% of time due to obstructive uropathy

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? History of Oligohydramnios

? Respiratory distress from pulmonary hypoplasia

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? Severity often does not correlate with degree obstruction

? Primary cause of death in newborns

Clinical Presentation

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? Early Infancy

? Dribbling / poor urinary stream
? Urosepsis

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? Dehydration
? Electrolyte abnormalities
? Uremia
? Failure to thrive; due to renal insufficiency

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? Toddlers

? Better renal function (less obstruction)
? Febrile UTI
? Voiding dysfunction ? incontinence

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? Daytime incontinence may be the only symptom in boys with

less severe obstruction
Initial Management

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? Bladder Drainage

? A 5 or 8 Fr pediatric feeding tube is ideal

? A Foley catheter should not be used, due to the tendency

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of the balloon to occlude the ureteral orifice and cause a

bladder spasm.

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? Secondary obstruction

? Broad spectrum antibiotic coverage

? Metabolic panel

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? Assess renal function and metabolic abnormalities

? Acidosis, hyperkalemia common problems

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Radiologic Evaluation of the Lower Tract

? VCUG

? Mandatory for all PUV evaluations

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? Showing a dilated prostatic urethra, valve leaflets,

detrusor hypertrophy, bladder diverticula, bladder

neck hypertrophy, and narrow penile urethra

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stream, as well as possible incomplete emptying


Radiologic Evaluation of the Lower Tract

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? U/S

? Examining the prostatic urethra for characteristic

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dilation and thickening of the bladder wall

VCUG

? dilated prostatic urethra

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? valve leaflets
? detrusor hypertrophy
? cellules or bladder

diverticula

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? bladder neck hypertrophy
? narrow penile urethra

stream

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? possible incomplete

emptying
Radiologic Studies- Upper Tract

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? Renal Ultrasound

? Examination for bilateral hydronephrosis and signs

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of lower tract obstructive process

? Renal Scan

? Assesses the function of the kidneys

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Management

? Transurethral Valve Ablation

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? Incise at 4, 8 & 12 o'clock positions via Pediatric

resectoscope

? Avoid urethral sphincter

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? Catheter drainage for 1-2 days
? VCUG at 2 months to ensure destruction of valves
? Regular U/S to evaluate resolution of hydronephrosis
Management

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? Transurethral Valve Ablation

? Alternatively, 8F cystoscope with a Bugbee

electrode adjacent

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? Insulated crochet hook ("Whitaker hook")

? When urethra too small to accommodate

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cystoscope/Bugbee

Vesicoureteral Reflux

? Present in 33 - 50%

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? Usually Secondary

? High intravesical pressures

? 33% resolve spontaneously when obstruction

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treated

? 33% do well on prophylactic antibiotics
Vesical Dysfunction

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? 50% have abnormal bladder function
? Presents as incontinence

? Not due to sphincter dysfunction or damage

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? Primary myogenic failure
? Uninhibited contractions
? May lead to progressive renal deterioration

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Favorable Prognostic Factors

? Creatinine falling below 1.0 one month after

treatment initiated

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? Absence of VUR
? Preservation of the corticomedullary junction

of the kidneys by renal U/S

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? Radiologic evidence of a "pop-off" valve