A Case of Prune Belly Syndrome: Radiological Aspects of a Rare Disease

Abstract

Prune Belly Syndrome (PBS) is a rare but morbid entity usually found in male neonates. It is classically defined by a triad of cardinal features that includes deficient abdominal wall musculature, urinary tract malformation and in males, cryptorchidism. Most of the patients have associated pulmonary, cardiac, skeletal and gastrointestinal tract anomalies. Children often require numerous surgical interventions including bilateral orchidopexy as well as individually tailored urinary tract and abdominal wall reconstruction. The prognosis of infants with PBS may be improved by quality antenatal follow-up, to enable the early diagnosis and preparation for prompt surgical intervention. We describe a case of PBS and emphasize the role of imaging, in the process of diagnosis and briefly review the subject.