Epispadias

Excerpt

Epispadias is a rare urogenital malformation characterized by the failure of the urethral tube to tubularize on the dorsal aspect. Unlike in hypospadias, where the meatus is on the ventral aspect, children with epispadias have a wide-open urethral plate on the dorsum. It is commonly seen as a component in the spectrum of bladder exstrophy-epispadias-complex (BEEC). Isolated epispadias constitutes less than 10 percent of the total cases of epispadias. Isolated male epispadias is rare, with an incidence of less than 1 per 100,000 live births.

Males have characteristic anatomic abnormalities, including a short-stubby phallus with a dorsally located meatus, upward-pointing phallus, and ventral hooding of the prepuce. Female epispadias is even rarer with an incidence ranging from at least 1 in 160,000 to 1 in 480,000 live births. Girls have a bifid clitoris, patulous urethral opening, anteriorly placed vaginal opening, and ill-formed or absent mons. Pubic diastasis is seen in both males and females with epispadias. Varying degrees of urinary continence are reported in these patients, and depending on the degree of continence, they can be categorized into continent or incontinent epispadias. The diagnosis of epispadias is clinical and does not require any additional investigations. The most important factor determining postoperative continence in these patients is the availability of a skilled pediatric surgeon/urologist. Long-term follow-up is necessary to address the psychosexual issues during the transition from childhood to adolescence and adulthood.