Prune-Belly Syndrome: A Case Report from Rwanda

Abstract

Background: Prune-Belly syndrome, Eagle-Barret syndrome and triad syndrome, all refer to congenital anomalies involving abdominal musculature, urinary tract and testicles. The syndrome consists of a triad of abdominal muscle aplasia, massive ureteral and bladder dilatation and cryptorchidism. Kidneys are often affected by secondary hydronephrosis or by polycystic dysplasia. The full manifestation of the syndrome occurs almost exclusively in boys. Available reports on the epidemiology and outcome show a high perinatal mortality due to related prematurity and associated pulmonary complications. The management of a prune-belly patient has been controversial. However nowadays, the tendency is to assist primarily prune – belly neonates in respiratory failure, and to limit radical urologic interventions. Case Report: We report hereby a full term prune-belly neonate who succumbed from this condition before being adequately investigated. Our purpose is to call clinicians’ attention to early recognition, investigation and management of the syndrome. Even though infants with a full-blown syndrome have a poor prognosis for long term survival, all patients need careful evaluation and individualized management according to the spectrum of the syndrome. In this paper, the morphogenesis and the developmental biology of the abdominal wall will be also recalled, and literature reviewed.