Bladder exstrophy is a rare condition in which the bladder does not form properly and is flattened and exposed on the abdominal wall. The condition is more common in males (3:1 male to female ratio), and is associated with epispadias, a condition in which the urethra is opened dorsally as a plate.
In males, the penis is short and broad with upward curvature (dorsal chordee). In females, the clitoris is separated into 2 segments (bifid) associated with divergent labia and an anteriorly displaced vagina.
Bladder exstrophy is a condition that requires a staged reconstructive approach by an experienced surgeon in order to maximize the child’s chances of achieving urinary continence and the ability to urinate spontaneously. The initial stage of reconstruction is performed in the newborn period and includes closure of the bladder plate and reapproximation of the pubic bones. The epispadias repair is usually performed at 6 to 9 months of age, although it is sometimes performed in conjunction with the bladder closure at birth, and the continence procedure (bladder neck repair) is performed at approximately 5 years of age if the bladder is big enough and the child is willing to participate in a voiding program.
Exstrophy patients require lifelong follow-up due to the complexity of their surgical reconstruction and the medical issues they are prone to develop throughout life.