Prune belly syndrome

Abstract

Prune belly syndrome is characterized by three cardinal features: bilateral undescended testicles, dilated urinary tract, and deficient abdominal wall musculature. These manifestations place patients with prune belly syndrome at risk for testicular malignancy, infertility, urinary tract infections, and renal failure. In addition, extra-genitourinary manifestations may also affect the gastrointestinal, orthopedic, and cardiopulmonary organ systems. The syndrome affects males in 95% of cases with up to 29% of patients dying during the peri-natal period, the majority from complications of pulmonary hypoplasia. The complexity of these patients requires a thorough understanding of their pathophysiology by pediatric surgeons and pediatric urologists alike.