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

Abstract

To describe an unusual case of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single uterus. Case report. Major academic medical center. A 12-year-old girl with OHVIRA syndrome. None. None. 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. 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.