Double trouble: diagnostic dilemma in a rare association of proximal ureteric valve with orthotopic ureterocele—case report

Abstract

Abstract Background Association of the ureterocele and proximal ureteral valve is a rare congenital anomaly which can lead to quick detoriation of renal function due to double obstruction. Though ureteral valves have been associated with other ureteral anomalies, this specific association is extremely rare. Case presentation A 7-month-old boy was referred to us for antenatally detected hydronephrosis. Provisional diagnosis of orthotopic ureterocele with pelviureteric junction (PUJ) obstruction was made based on preoperative imaging. Endoscopic incision of ureterocele was done and retrograde pyelography showed tight obstruction at PUJ which necessitated a pyeloplasty. Examination of resected specimen confirmed a ureteral valve. Postoperatively, the patient had good improvement in kidney function. Conclusion To conclude, congenital ureteral valves are a rare cause of ureteric obstruction and its association with ureterocele is rarer still. Identification of an ureterocele should not deter us from searching for an associated pathology like a ureteric valve when imaging shows more severe hydronephrosis suggestive of more proximal obstruction.