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 yClassification
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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 corethen 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 andunderdeveloped 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 penoscrotalhypospadias
? 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 hermaphrotidismAssociated 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 palpableundescended 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 generalpopulation
? 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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MathieuIsland
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 thinmembrane 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 edgeof 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 incompletedissolution 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 withless 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 wallVCUG
? 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 Pediatricresectoscope
? 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/BugbeeVesicoureteral 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