Cloacal exstrophy--improving the quality of life: the Johns Hopkins experience.

PURPOSE: Exstrophy of the cloaca is a multisystem anomaly involving the gastrointestinal, nervous, musculoskeletal and genitourinary tracts which should be managed with a multidisciplinary approach. Improvement in management has led to survival for the majority of infants, and the focus has shifted to improvement in quality of life. The experience with management of cloacal exstrophy at a large center is evaluated.

MATERIALS AND METHODS: Demographic data as well as functional results of management of the multiple anomalies in 37 patients with cloacal exstrophy were evaluated. Surgical reconstruction was aimed at providing the best functional and cosmetic results.

RESULTS: Average patient age at review was 13.6 years. Most patients (32 of 37) had undergone an initial attempt at bladder closure from birth to greater than 24 months of age. Colostomy was performed when possible and if initial ileostomy was performed, the bowel was augmented later with the hindgut segment. When the hindgut segment was not used for bowel reconstruction, it was preserved for bladder augmentation or genital reconstruction. While urinary continence was achievable in many children, it was usually after augmentation and/or continent diversion.

CONCLUSIONS: Improvements in perinatal management have increased survival in cloacal exstrophy. Therefore, the focus of reconstruction has shifted to reduction in the numbers of incontinent stomas, assistance with ambulation and improved cosmesis. All of these goals are achievable using a multidisciplinary approach to the management of this complex anomaly.