Severe Neonatal Prune Belly Syndrome Complicated by Terminal Obstructive Renal Failure: A Case Report from CHME Nouakchott, Mauritania
Abstract
Alya Tatah, Mah Atigh, Moussa Taher, Mariam Sidatt, Vayza Elbechir and Houda Rabani
Prune Belly Syndrome is a rare congenital anomaly characterized by the triad of abdominal wall muscle deficiency, severe urinary tract abnormalities, and bilateral cryptorchidism. Prognosis mainly depends on the severity of renal involvement. We report the case of a male full-term neonate born to consanguineous parents who presented with a severe form of Prune Belly Syndrome revealed by marked bilateral ureterohydronephrosis associated with near-complete destruction of the right kidney, progressive renal failure, and post-obstructive diuresis complicated by refractory severe hyponatremia. Despite multidisciplinary management and urinary drainage, the clinical outcome was fatal. This case highlights the severity of neonatal forms of Prune Belly Syndrome and emphasizes the importance of early antenatal diagnosis and specialized multidisciplinary management.