Total surgical reconstruction for patients with abdominal muscular deficiency ("prune-belly") syndrome

Abstract

In the past decade, seven children with the prune-belly syndrome have been seen. Their management has consisted of immediate high tubeless urinary diversion, usually pyelostomy. Thereafter, total reconstruction has been carried out, preferably at one stage. This consists of (1) bilateral shortening, tapering and reimplantation of the ureters, (2) reduction cystoplasty, (3) bilateral orchiopexy, and (4) excision of that part of the abdominal wall that is most redundant and least endowed with musculature. This early effort at mechanical reconstruction has led to gratifying progress in six of the seven children.