Bladder exstrophy

Abstract

Bladder exstrophy falls on a spectrum spanning a gamut from the relatively benign isolated epispadias to the severe cloacal exstrophy. In regards to bladder exstrophy, the disorder is characterized by a lower midline defect, with the bladder lumen being exposed. In addition to the bladder, there are defects to the genitalia, pelvic bony anatomy, rectum, anus, and abdominal wall. The condition is thought to be an abnormal development of the midline, specifically the cloacal membrane. Following birth, these patients require surgical intervention for functional closure of the bladder. After the closure of the bladder, there are several surgical milestones to recapitulate the normal lower genitourinary tract anatomy. That includes repair of epispadias in males and a continence surgery for both males and females. Throughout the last several decades, treatment success has improved in regard to functional closure. The management of these patients has turned to improving quality of life, especially in terms of patients achieving urinary continence. Advances in modern medicine have rendered this major congenital anomaly compatible with a long life into adulthood. Because most experience is isolated to large centers with experienced exstrophy surgeons, data may not be generalizable. With more collaborative efforts between institutions and centralization in care at high volume centers, questions still unclear in the management of these patients may hopefully be elucidated in the near future.