Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single uterus

Objective: To describe an unusual case of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single uterus. Design: Case report. Setting: Major academic medical center. Patient(s): A 12-year-old girl with OHVIRA syndrome. Intervention(s): None. Main Outcome Measure(s): None. Result(s): A 12-year-old girl presented with presumed OHVIRA syndrome and had surgical correction of the obstructed hemivagina. Two years later she presented with increasing pelvic pain and underwent laparoscopy for presumed endometriosis. A single uterus with a broad flat fundus was found as well as endometriosis. Conclusion(s): OHVIRA syndrome is typically associated with a didelphys uterus with two cervices and two vaginas, one of which is obstructed. The obstruction usually occurs on the same side as the renal anomaly. We report a rare congenital anomaly of the female reproductive tract: OHVIRA syndrome with a single uterus. From a fertility standpoint, cases of a single uterus with two cervices are managed differently than two uteri with two cervices. Healthcare providers managing complex reproductive tract anomalies should be aware of this potential variant. (Fertil Steril (R) 2011; 96: e39-41. (C) 2011 by American Society for Reproductive Medicine.)