Case number 3 : Prune Belly Syndrome aka Eagle Barrett Syndrome

Abstract

HM, male Severe bilateral hydronephrosis was noted at 20 weeks gestation with normal liquor at the time. The baby was delivered by elective C-section at 39 weeks gestation and very little liquor was noted. He was noted to have deficient abdominal wall musculature and cryptorchidism and prune-belly syndrome was suspected. A renal US confirmed bilateral severe hydroureteronephrosis with no evidence of posterior urethral valves or vesico-ureteric reflux on micturating cystourethrogram. A DTPA scan excluded outflow tract obstruction and a DMSA scan showed a non-functioning left kidney. His creatinine was noted to be 228umol/l (normal creatinine at this age 20-30umol/l). An ultrasound of the brain and back revealed no spinal anomalies and an echocardiogram showed a normal heart. Over the first few months of life he was managed conservatively on medications for chronic kidney disease. At the age of 1 year he underwent a left orchidopexy and a right first stage Fowler Stephen’s procedure. The second stage was performed a few months later.