Background: Prune belly syndrome is a rare congenital disorder characterized by a triad of deficient abdominal wall muscles, urinary tract anomalies and cryptorchidism. Most of the affected cases are males. This syndrome is associated with a high mortality rate in the peri-natal period due to pulmonary complications. We present the first prune belly syndrome case in Lebanon. Besides, despite the presence of bilateral urinary tract dilation, no pulmonary consequences emerged at birth.

Case Presentation: A case of a term baby boy born to a 30 years old woman was found to have floppy abdominal wall and mild tachypnea so was admitted to the neonatal intensive care unit for investigations and management. Physical examination revealed bilateral undescended testicles. Imaging reported bilateral severe hydronephrosis with ureteral dilation and absence of ventral abdominal musculature. So, a diagnosis of Prune belly syndrome was established. Due to evidence of kidney injury, supra-pubic cystostomy was performed to relieve the obstruction. This lead to improvement in kidney function test. Patient was planned for later repair of abdominal wall and for orchiopexy.

Conclusion: The case was a very interesting experience for the whole team especially due to the absence of clear guidelines due to the rarity of the syndrome. It highlights the importance of shared decision between the neonatologists and the urologists in any unusual disorder. Moreover, it shows the significance of pre-natal imaging and the necessity of searching for associated anomalies in the case of severe hydronephrosis pre-natally.

Keywords: Prune belly syndrome; Abdominal musculature; Bilateral hydronephrosis; Newborn