Download MBBS Urology Presentations 6 Congenital Anomalies of Penis Lecture Notes

Download MBBS (Bachelor of Medicine, Bachelor of Surgery) 1st Year, 2nd Year, 3rd Year and Final year Urology 6 Congenital Anomalies of Penis PPT-Powerpoint Presentations and lecture notes

Hypospadias, Epispadias and

Posterior urethral valves

Department of Urology


? Any condition in which the meatus

occurs on the undersurface of the


? Usually 3 features

? ventral meatus

? ventral curvature (chordee)

? Dorsal "hood"; deficient

foreskin ventral y



? Genital tubercle fuses in


? 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


? Prepuce forms as ridge of

skin from corona

? Hypospadias

? Failure of ventral aspect to


? Dorsal hood

? Chordee

? Differential growth

between normally

developed dorsal tissue and

underdeveloped ventral

corporal tissue

? Fibrous tissue distal to

hypospadiac meatus


? 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


? Incomplete mullerian duct regression

Associated Anomalies

? Rule out intersex, especially with


? 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


? Therefore no further evaluation is indicated

? Meatoplasty and glanuloplasty

? Multiple techniques

? Orthoplasty

? Utilize artificial erection
? Release urethra from fibrous tissue
? Plicate dorsal tunica albuguinea
? Ventral graft if needed


? 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)





Buccal Mucosal Graft


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


Type I PUV

Type I I PUV

? Represent incomplete

dissolution of the UG


? Distal to the

verumontanum at the

membranous urethra

? Ring-like with a central

opening, "wind sock


? 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


? 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


? dilated prostatic urethra
? valve leaflets
? detrusor hypertrophy
? cellules or bladder


? bladder neck hypertrophy
? narrow penile urethra


? possible incomplete

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


? Transurethral Valve Ablation

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


? 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

? Transurethral Valve Ablation

? Alternatively, 8F cystoscope with a Bugbee

electrode adjacent

? Insulated crochet hook ("Whitaker hook")

? When urethra too small to accommodate


Vesicoureteral Reflux

? Present in 33 - 50%
? Usually Secondary

? High intravesical pressures

? 33% resolve spontaneously when obstruction


? 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