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Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single uterus
1Division of Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynecology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
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.
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