Abstract

Prune Belly syndrome (PBS) is an extremely rare anatomo-radiological syndrome that combines aplasia of the muscles of the anterior abdominal wall, dilatations of the urinary tract and testicular malformations, thus forming the classic triad of the syndrome. Up to 75% of patients with PBS have pulmonary, skeletal, cardiac, and gastrointestinal malformations. We report the case of a full-term male infant with no particular pathological history admitted for etiological work-up of bilateral ureterohydronephrosis diagnosed antenatally with a distended, prune-shaped abdomen with bilateral testicular ectopia on clinical examination. The outcome can be very variable, ranging from stillbirth due to major renal and respiratory dysplasia to a practically normal child, all of which explains the great diversity of opinions on the attitude to adopt when faced with this syndrome.