Prune Belly Syndrome in Nigeria – A report of two cases and review of literature

Abstract

Background: Prune belly Syndrome is a rare congenital disorder made up of a myriad of anomalies with a typical triad of absence of  anterior abdominal wall muscles, urinary tract anomalies and bi-lateral cryptorchidism. Incidence is stated to be 1 in 30,000- 40,000 live births. The syndrome classically occurs in males. It is a cause of great morbidity with chronic renal failure and End stage renal disease (ESRD) occurring in 25-30% of patients and mortality of about 60%. Our neonatal unit managed 2 cases of this rare syndrome within a one month period.

Objective: This report aimed to highlight the presentation, investigation and management of the cases and discuss the aetio- pathogenesis, management strategies, and prognostic factors of the condition.

Case report: We report two term male neonates who presented in the first 24 hrs of life with laxity of abdominal wall muscles,  cryptorchidism and varied urinary tract abnormalities which included bilateral hydronephrosis (with multiple communicating cysts),
hydroureter, severe vesicoureteric reflux and bladder outlet obstruction. Treatment was with urinary drainage and prophylactic antibiotics. One of them developed UTI and renal failure which ultimately led to demise, while the other discharged against medical advice.

Conclusion: Prune belly syndrome often has a poor prognosis with high mortality. Though management is controversial and often  involves complex surgical intervention not readily available in resource-poor settings such as ours, early and coordinated multidisciplinary management with adequate patient education would improve survival and quality of life.

Keywords: Congenital disorder, Nigeria, Prune belly syndrome.