Diagnostic Dilemma and Surgical Management of Circumcaval Ureter: A Case Report

Circumcaval ureter (CVU) is an uncommon congenital anomaly, in which the proximal ureter makes a loop posterior to the inferior vena cava (IVC) usually resulting in ureteric obstruction with consequential hydronephrosis and a right nonfunctioning kidney. It is also called a retrocaval or postcaval ureter. CVU is rarely encountered and, hence, may pose a diagnostic dilemma for radiologists and urologists. Clinical presentation occurs in the 3rd and 4th decades of life, manifesting mainly with flank pain. We present our experience in the diagnosis and management of CVU in a Nigerian Centre. A 56-year-old man presented with a 2-year history of recurrent, colicky right flank pain. He had been seen in multiple tertiary hospitals with no definitive diagnosis or treatment. A computerised tomography urogram showed an abnormally dilated proximal ureter with the classical fish-hook appearance and medial displacement, posterior to the IVC. At surgery, via an open, flank, extraperitoneal approach a CVU was found with significant stenosis and prestenotic dilatation. An excision of the stenotic segment with ureteroureterostomy was performed over a 6F D-J stent, and he had a smooth postoperative period. A CVU is extremely rare, and hence, the diagnosis can be missed. If left unattended, CVUs can progress to hydronephrosis and, eventually, a nonfunctioning right kidney. A high index of suspicion and early surgical intervention are key to a successful outcome.