Abstract
BACKGROUND: Prune Belly Syndrome (PBS), also known as Eagle-Barrett syndrome, is an uncommon congenital disorder characterized by a combination of abdominal wall hypoplasia, urinary tract malformations, and bilateral cryptorchidism. The condition predominantly affects males and is frequently associated with significant neonatal morbidity and mortality related to renal and pulmonary complications. Congenital urinary fistula formation represents a particularly rare and complex manifestation of this syndrome. CASE PRESENTATION: We report a case of a 2-day-old male neonate presenting to a hospital in a resource-limited setting with respiratory distress and passage of urine via the umbilicus. The patient was born at term via spontaneous vaginal delivery to a mother with no antenatal care. Physical examination revealed a critically ill neonate with a classic "prune-like" abdomen, bilateral cryptorchidism, urine flow from an infected umbilical cord, and associated anomalies including polydactyly and clubfoot. Ultrasonography confirmed bilateral gross hydronephrosis and a urinary fistula communicating with the umbilicus. CONCLUSION: This case highlights a classic presentation of PBS, unusually complicated by a congenital urinary fistula. It underscores the profound challenges in managing this complex syndrome in settings with limited resources, where definitive surgical intervention is unavailable. The report emphasizes the critical importance of prenatal diagnosis and the need for a multidisciplinary approach to improve outcomes, which was not feasible in this context. Ultimately, management was restricted to supportive care and antibiotics, illustrating the dire consequences of diagnostic and therapeutic limitations in such environments.The patient was stabilized and referred to a higher center; however, long-term outcome was not available.