The Prune Belly Syndrome: A Comprehensive Approach to Management

Abstract

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.

Prune belly syndrome

“…Fallat et al reported on 15 patients who had undergone early urinary reconstruction, consisting of ureteric tapering, reimplantation and reduction cystoplasty. In all patients, there were no changes in pre-and postoperative serum creatinine levels (Level 3) [39]. Bukowski et al examined the reduction cystoplasty element of their urinary reconstructions.…”

“…This is likely due to the poor peristalsis and inherent muscular deficiencies present in the PBS ureter and bladder. Vesico-ureteric junction (VUJ) obstruction requiring re-operation in 40% of patients and failure of resolution of VUR post-reimplant in 30% of patients was reported in one series [39].…”

“…Bukowski et al examined the reduction cystoplasty element of their urinary reconstructions. In their cohort of 11 patients, there was some improvement in voiding and reduction in number and severity of urinary tract infections, but long-term review There is a high complication rate associated with surgery in PBS patients [39,51]. This is likely due to the poor peristalsis and inherent muscular deficiencies present in the PBS ureter and bladder.…”

“…The most common is prematurity with 43% of infants born preterm. Cardiovascular and pulmonary problems are also common (Table 1) [7,39,40].…”

“…It appears as if early aggressive surgery on the basis of urinary tract dilatation alone without evidence of compromised renal function in PBS appears unwarranted given the reported success of the conservative approach coupled with the high complication rate of surgery associated with PBS patients (Level 3) [39,[47][48][49]51].…”