Exstrophy-Epispadias Complex

Abstract

Bladder exstrophy is a complex congenital birth defect ranging from complete epispadias to complete bladder exstrophy. Though not included in the exstrophy-epispadias complex (EEC) disease spectrum, cloacal exstrophy is a more severe form which includes bowel segments in the defect and has many of the same defects as the EEC. Initial care of these complex patients focuses on the primary repair of the defect, a repair which is the most important of the many operations these children will have in their lifetime. A failure of primary closure is associated with worse outcomes across the board. The most essential goal in treatment of the exstrophy patient is renal protection. Once that can be assured, social continence then becomes the most important goal. Given the complex nature of this disorder, these children should be managed at speciality centers with orthopedic, urologic, and nursing experience with these patients.