Abstract
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.
Key Words
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Article info
Publication history
Published online: November 27, 2021
Accepted:
November 15,
2021
Received in revised form:
October 15,
2021
Received:
May 30,
2021
Footnotes
Competing Interests: No competing interests.
Funding Statement: This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Ethics Statement: Ethical approvement was not required because the patient was managed and treated as per standard institutional protocol. Written and informed consent was obtained from the parents prior to submission of images and information about the patient.
Identification
Copyright
© 2021 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.
