CLOACAL EXSTROPHY - IMPROVING THE QUALITY-OF-LIFE - THE HOPKINS,JOHNSEXPERIENCE

Purpose: Exstrophy of the cloaca is a multisystem anomaly involving th e 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 th e focus has shifted to improvement in quality of life. The experience with management of cloacal exstrophy at a large center is evaluated, M aterials and Methods: Demographic data as well as functional results o f management of the multiple anomalies in 37 patients with cloacal exs trophy 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 i nitial attempt at bladder closure from birth to greater than 24 months of age. Colostomy was performed when possible and if initial ileostom y was performed, the bowel was augmented later with the hindgut segmen t. When the hindgut segment was not used for bowel reconstruction, it was preserved for bladder augmentation or genital reconstruction. Whil e urinary continence was achievable in many children, it was usually a fter augmentation and/or continent diversion. Conclusions: Improvement s in perinatal management have increased survival in cloacal exstrophy . Therefore, the focus of reconstruction has shifted to reduction in t he numbers of incontinent stomas, assistance with ambulation and impro ved cosmesis. All of these goals are achievable using a multidisciplin ary approach to the management of this complex anomaly.