Circumcaval Ureter with Vesico Ureteral Reflux: The First Association in Literature

A circumcaval ureter is a rare congenital anomaly in which the ureter passes behind, and is compressed by, the inferior vena cava. Its etiology is assumed to be abnormal embryologic development of the inferior vena cava as a result of atrophy failure of the right subcardinal vein in the lumbar portion. A circumcaval ureter is also termed a retrocaval ureter. The right supracardinal system fails to develop, whereas the right posterior cardinal vein persists. With one reported exception, the anomaly always occurs on the right side. Patients with this anomaly may develop partial right ureteral obstruction or recurrent urinary tract infections. Therapeutic options include surgical relocation of the ureter anterior to the cava. A 14-year-old female patient came with complaints of fever, intermittent colic and dysuria 4 years ago. A right ureteric fourth-grade VUR and circumcaval ureter were established. An anomaly in which both of these are together could not be found in literature. If after the VUR treatment he has progressive abdomen pain and advancing hydronephrosis, a circumcaval ureter as an additive anomaly must not be forgotten. For that reason, in a patient having a urinary system anomaly, a likely extra anomaly should be searched.