Objectives: Updated review about topographic anatomy, clinical and radiologic diagnosis, and management of the circumcaval ureter. Methods: We used Ovid and PubMed (updated January 2006) to conduct a literature electronic search on MEDLINE which included peer-reviewed articles. Moreover, we analysed previous publications regarding both "retrocaval" and "circumcaval" ureter. Results: Circumcaval ureter predominantly involves the right ureter; its exact prevalence is still unknown. The anomaly occurs 2.8 times more commonly in males than females clinically as well. Although the lesion is congenital, symptoms usually present in the third to fourth decade of life; the patients mostly present with right flank pain and discomfort. Intravenous urogram and retrograde urography were commonly used to diagnose a circumcaval ureter. A spiral computed tomography scan and, more recently, Magnetic Resonance Imaging, have been suggested to differentially diagnose a circumcaval ureter as methods of choice. The open ureteroureterostomy remained the gold standard surgical approach to treat the circumcaval ureter for many years; however, minimally invasive laparoscopic procedures have almost replaced open surgery. Conclusions: The circumcaval ureter is a rare congenital anomaly usually associated with upper urinary tract hydronephrosis. Spiral computed tomography scan and Magnetic Resonance Imaging have been recently considered the tool of choice for the diagnosis of inferior vena caval abnormalities. Comparisons among historical reports regarding open surgery and laparoscopic transposition and reanastomosis of a circumcaval ureter have clearly shown the advantages of minimally invasive approaches.
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