Prune-Belly Syndrome

Abstract

Prune-belly syndrome is a rare congenital disorder characterized by three major features: deficient abdominal wall musculature, urinary tract anomalies, and bilateral cryptorchidism in males. Incidence is estimated between 1/30,000 and 1/50,000 live births and the 3–5% of known cases are females. The exact pathogenesis of prune-belly syndrome is not clearly known as yet. Three major theories are known, but none of them have universal acceptance because none completely explains the whole constellation of findings in the syndrome. No specific gene defect has been identified for PBS. Many authors tried to formulate classification systems, but the considered classification system to date is by Woodard. The main goal of treatment of patients with prune-belly syndrome is to preserve renal function. The prognosis and the postnatal management of these patients, indeed, are closely related to it. The spectrum of therapeutic possibilities range from a “wait and see” approach to immediate or delayed urologic surgery.