Proximal ureteral atresia in a duplex kidney with preserved renal function: A case report and review of literature

Ureteral atresia is a rare congenital anomaly and is usually associated with renal dysplasia. Distal atresia is more common and other associated urinary anomalies are rare. We report a case of an eight year old boy who presented with left flank pain and vomiting. Ultrasound abdomen and pelvis and renal scintigraphy were suggestive of hydroureteronephrosis with obstructed drainage. He was suspected to have ureteric stricture and taken up for surgery. Cystoscopy and retrograde pyelogram revealed an incomplete duplication with lower moiety ureter ending blindly. Exploration of left renal fossa showed a duplex anomaly with normal upper moiety and hydronephrosis of lower moiety with a ureteric stump. Ureteropelvic anastomosis was done between the dilated renal pelvis and the blind ending ureter. Patient was doing well post operatively with good function in both moieties. Review of literature shows only two previously reported cases of proximal ureteric atresia. Preservation of renal function in an older child has also been reported only once. Ureteral atresia is usually diagnosed intra-operatively and various reconstruction options are available in patients with preserved function.