A Rare Association of Obstructed Hemivagina with Ipsilateral Renal Agenesis with Congenital Pouch Colon.

Background: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA), or Herlyn-Werner-Wunderlich syndrome, is a rare Mullerian duct anomaly. Several associations of OHVIRA with other anomalies are being reported.
Case: A 13-year-old girl reported with acute onset abdominal pain. Patient was operated on in the past for type IV congenital pouch colon (all stages complete). She was diagnosed with hematometrocolpos and underwent tube vaginostomy. Further workup was suggestive of OHVIRA, which was subsequently managed with surgical repair.
Conclusion: Delineation of reproductive anomalies in female patients with anorectal malformations is of paramount importance. OHVIRA syndrome should be considered at a high index of suspicion in female patients with a solitary functioning kidney. Nonspecific symptoms in an adolescent female can lead to erroneous judgment, leading to unnecessary investigations, which, if not well managed in time, can be detrimental to fertility.
(Copyright © 2021. Published by Elsevier Inc.)