EPISPADIAS

Epispadias
In epispadias, the urethra opens onto the dorsal surface of the penis, anywhere from the glans, penile shaft, or, most commonly, the penopubic region. An incomplete urethral sphincter mechanism results in a high risk of incontinence. Epispadias is also associated with dorsal chordee (causing an upward curvature of the penis), with incomplete foreskin dorsally. Epispadias is part of the exstrophy epispadias complex (which also includes bladder exstrophy and cloacal exstrophy).

Associated anomalies
Diastasis of the symphysis pubis results in splaying of the corpora cavernosa and shortening of the penile shaft. Females have a bifid clitoris, poorly developed labia, and demonstrate a spectrum of urethral deformities ranging from a patulous urethral orifice to a urethral cleft affecting the entire length of the urethra and sphincter. There is a 40% risk of vesicoureteric reflux (VUR).

Incidence
Affects 1 in 117,000 males. Rarely seen in females (male:female ratio is 5:1).

Management
Urethroplasty with functional and cosmetic reconstruction of the external genitalia (penile lengthening and correction of chordee) at 6 12 months. The modified Cantwell Ransley technique is commonly used in males. It describes mobilizing the urethra to the ventral aspect of the penis, with advancement of the urethral meatus onto the glans with a reverse MAGPI (meatal advancement-glanduloplasty). The corporal bodies are separated and rotated medially above the urethra and re-approximated. From age 4 5 years, when children can be toilet trained, bladder neck reconstruction can be performed (Youngs Dees Leadbetter procedure). This achieves continence, and any bladder residuals may then be emptied by urethral catheterization. If this surgery fails, insertion of artificial urinary sphincters or collagen injections of the sphincter may be tried.