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Hypospadias, Epispadias and
Posterior urethral valves
Department of Urology
Hypospadias
? Any condition in which the meatus
occurs on the undersurface of the
penis
? Usually 3 features
? ventral meatus
? ventral curvature (chordee)
? Dorsal "hood"; deficient
foreskin ventral y
Classification
Embryology
? Genital tubercle fuses in
midline
? Mesodermal folds
create the urethral and
genital folds
? coalesce in midline as
phal us elongates
? Distal glans channel
tunnels to proximal
urethra as solid core
then undergoes
canalization
Embryology
? Prepuce forms as ridge of
skin from corona
? Hypospadias
? Failure of ventral aspect to
form
? Dorsal hood
? Chordee
? Differential growth
between normally
developed dorsal tissue and
underdeveloped ventral
corporal tissue
? Fibrous tissue distal to
hypospadiac meatus
Incidence
? 1:300 live male births
? 6000 boys each year in the US
? Some genetic component
? 8% of patients have father with hypospadias
? 14% of patients have male siblings with hypospadias
? If child with hypospadias, risk to next child
? 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)
? Higher incidence in monozygotic twins (8.5x)
Associated Anomalies
? Undescended testes 9% and inguinal
hernia 9%
? Upper tract anomalies rare (1-3%)
? Utriculus masculinus
? 10 to 15% in perineal or penoscrotal
hypospadias
? Incomplete mullerian duct regression
Associated Anomalies
? Rule out intersex, especially with
cryptorchidism
? Adrenogenital syndrome
? Mixed gonadal dysgenesis
? Incomplete pseudohermaphroditism
? True hermaphrotidism
Associated Anomalies
? hypospadias and cryptorchidism
? high index of suspicion for an intersex state
? Walsh reported the incidence of intersexuality in
children with cryptorchidism, hypospadias, and
otherwise nonambiguous genitalia to be 27%
? nonpalpable testis were at least threefold more likely to
have an intersex condition than those with a palpable
undescended testis (50% versus 15% )
Further Evaluation
? Only with severe hypospadias and sexual ambiguity
? Includes testicular abnormalities
? Up to 25% of these patients have enlarged utricles or other
female structures
? The incidence of abnormalities with other forms of
hypospadias approximates that of the general
population
? Therefore no further evaluation is indicated
Treatment
? Meatoplasty and glanuloplasty
? Multiple techniques
? Orthoplasty
? Utilize artificial erection
? Release urethra from fibrous tissue
? Plicate dorsal tunica albuguinea
? Ventral graft if needed
Treatment
? Urethroplasty
? Onlay vascularized flap
? Tubularized flap
? Free graft
? Skin cover
? Mobilized dorsal prepuce and penile skin
? Double faced island flap
? Scrotoplasty
Hypospadias Repair
? Over 150 operations have be described
? Distal hypospadias
? Tubulization of the incised urethral plate (Snodgrass)
? Meatal advancement (MAGPI)
? Meatal-based flaps (Mathieu)
? Proximal hypospadias
? Onlay grafts
? Vascularized inner preputial transfer flaps (Duckett)
? Free grafts (skin, buccal mucosa)
MAGPI
Mathieu
Island
Onlay
Buccal Mucosal Graft
Epispadias
Posterior Urethral Valves
Posterior Urethral Valves (PUV)
? Congenital Proximal Urethral Obstruction
? Abnormal congenital mucosal folds in the
prostatic urethra that look like a thin
membrane that impairs bladder drainage
PUV Defined
? Type I
? Obstructing membrane that extends distally from each
side of the verumontanum towards the membranous
urethra where they fuse anteriorly
? Type II
? Described as folds extending cephalad from the
verumontanum to the bladder neck
? Type II
? Represent a diaphragm or ring-like membrane with a
central aperture just distal to the verumontanum
? Thought to represent incompelte dissolution of the
urogenital membrane
Type I PUV
? Obstructing membrane radiating
distally from the posterior edge
of the verumontanum to the
membranous urethra
? During voiding, the fused anterior
portion bulges into the urethra
with a narrow posterior opening
? Possibly due to anomalous
insertion of the mesonephric
ducts into the primitive fetal
cloaca
Type I PUV
Type I I PUV
? Represent incomplete
dissolution of the UG
membrane
? Distal to the
verumontanum at the
membranous urethra
? Ring-like with a central
opening, "wind sock
valve"
Incidence
? Males only
? 1:5000 ? 8000 male births
? Type I > 95%
? Type II - 5%
? Children with Type II PUVs have a worse
prognosis as a group
? 50% of patients with PUV will have
vesicoureteral reflux
? 50% unilateral, 50% bilateral
Clinical Presentation
? Varies by degree of obstruction
? Symptoms vary by age of presentation
? Antenatal
? Bilateral hydronephrosis
? Distended and thickened bladder
? Dilated prostatic urethra
? Oligohydramnios - accounts for co-presentation of
pulmonary hypoplasia.
Clinical Presentation
? Newborn
? Palpable abdominal mass
? Distended bladder, hydronephrotic kidney
? Bladder may feel like a small walnut in the suprapubic area
? Ascites
? 40% of time due to obstructive uropathy
? History of Oligohydramnios
? Respiratory distress from pulmonary hypoplasia
? Severity often does not correlate with degree obstruction
? Primary cause of death in newborns
Clinical Presentation
? Early Infancy
? Dribbling / poor urinary stream
? Urosepsis
? Dehydration
? Electrolyte abnormalities
? Uremia
? Failure to thrive; due to renal insufficiency
? Toddlers
? Better renal function (less obstruction)
? Febrile UTI
? Voiding dysfunction ? incontinence
? Daytime incontinence may be the only symptom in boys with
less severe obstruction
Initial Management
? Bladder Drainage
? A 5 or 8 Fr pediatric feeding tube is ideal
? A Foley catheter should not be used, due to the tendency
of the balloon to occlude the ureteral orifice and cause a
bladder spasm.
? Secondary obstruction
? Broad spectrum antibiotic coverage
? Metabolic panel
? Assess renal function and metabolic abnormalities
? Acidosis, hyperkalemia common problems
Radiologic Evaluation of the Lower Tract
? VCUG
? Mandatory for all PUV evaluations
? Showing a dilated prostatic urethra, valve leaflets,
detrusor hypertrophy, bladder diverticula, bladder
neck hypertrophy, and narrow penile urethra
stream, as well as possible incomplete emptying
Radiologic Evaluation of the Lower Tract
? U/S
? Examining the prostatic urethra for characteristic
dilation and thickening of the bladder wall
VCUG
? dilated prostatic urethra
? valve leaflets
? detrusor hypertrophy
? cellules or bladder
diverticula
? bladder neck hypertrophy
? narrow penile urethra
stream
? possible incomplete
emptying
Radiologic Studies- Upper Tract
? Renal Ultrasound
? Examination for bilateral hydronephrosis and signs
of lower tract obstructive process
? Renal Scan
? Assesses the function of the kidneys
Management
? Transurethral Valve Ablation
? Incise at 4, 8 & 12 o'clock positions via Pediatric
resectoscope
? Avoid urethral sphincter
? Catheter drainage for 1-2 days
? VCUG at 2 months to ensure destruction of valves
? Regular U/S to evaluate resolution of hydronephrosis
Management
? Transurethral Valve Ablation
? Alternatively, 8F cystoscope with a Bugbee
electrode adjacent
? Insulated crochet hook ("Whitaker hook")
? When urethra too small to accommodate
cystoscope/Bugbee
Vesicoureteral Reflux
? Present in 33 - 50%
? Usually Secondary
? High intravesical pressures
? 33% resolve spontaneously when obstruction
treated
? 33% do well on prophylactic antibiotics
Vesical Dysfunction
? 50% have abnormal bladder function
? Presents as incontinence
? Not due to sphincter dysfunction or damage
? Primary myogenic failure
? Uninhibited contractions
? May lead to progressive renal deterioration
Favorable Prognostic Factors
? Creatinine falling below 1.0 one month after
treatment initiated
? Absence of VUR
? Preservation of the corticomedullary junction
of the kidneys by renal U/S
? Radiologic evidence of a "pop-off" valve
This post was last modified on 08 April 2022