The prune belly syndrome: a comprehensive approach to management.

Our approach to management of the prune belly syndrome entails a program of comprehensive early reconstruction, including abdominoplasty, bilateral orchiopexy, reduction cystoplasty, and selective ureteral tailoring and reimplantation. Of 20 patients with the prune belly syndrome treated at our institution during the last 18 years 15 underwent comprehensive reconstruction. Abdominoplasty was performed in 16 patients. Results were evaluated as to cosmetic appearance, functional performance, and preoperative and postoperative electromyography. Immediate good results were obtained in 9 patients. Of 16 patients 6 required more than 1 abdominal wall tailoring and 3 had minor complications that did not compromise the ultimate result. Transabdominal orchiopexy was performed on 32 testes; 28 (88 per cent) are in the scrotum and growing. Of the 4 failures 3 occurred in patients greater than 2 years old at repair. Selective urinary tract reconstruction has been performed; 13 patients underwent tapering and reimplantation of 1 or both ureters. Reduction cystoplasty was done in all 15 patients. Functional assessment of the upper urinary tract by improved radiological techniques and the necessity for long-term antibiotic prophylaxis have led to a more conservative approach in 5 recent patients. Renal function as measured by serum creatinine and/or glomerular filtration rate has remained stable in 12 of 15 patients (92 per cent) undergoing comprehensive reconstruction. Aggressive surgical management of patients with the prune belly syndrome provides improved abdominal wall function and appearance, and offers excellent testicular salvage. Surgical reconstruction of deformed ureters should be selective and decided on longitudinal studies of renal function.