Genitourinary defect

Extrophy of bladder with epispadias with left undescended testis. The incidence of bladder extrophy is 1 in 35,000 to 40,000 births. The male female ratio is 2:1. The severity of the anomaly ranges from small fistula to complete extrophy of the cloaca exposing entire hind gut and the bladder. In classical cases the bladder protrudes from the abdominal wall and its mucosa is exposed. Generally it is associated with undescended testes and epispadias with shallow scrotum. In females, epispadias is present with separation of two sides of clitoris with wide separation of labia. The anus is displaced anteriorly and there may be rectal prolapse. Management of the bladder extrophy should start at birth. Bladder should be covered with plastic wrap to keep the mucosa moist. Application of gauze or petroleum gauzes should be avoided. Prompt closure of the extrophy is the preferred treatment and if the bladder closure is performed during the first 48 hours of life there is sufficient mobility of the pubic rami to allow approximation of the pubic symphysis. The initial operation is the closure of the bladder, closure of the abdominal wall and, in the male, elongation of the urethral plate and penis. In the male the second stage is epispadias repair which is usually performed between the age of 1 to 2 years. The final stage of reconstruction involves creation of sphincter muscle for bladder control and correction of the vesicoureteral reflux.

E-published: August 2011 Vol 8 Issue 8 Art No 53

References :

Behrman RE, Kliegman R, Jenson HB. Nelson Textbook of Pediatrics. 16th ed. Philadelphia. Saunders. 2000.