Journal of South Asian Federation of Obstetrics and Gynaecology

Register      Login

VOLUME 15 , ISSUE 3 ( May-June, 2023 ) > List of Articles

CASE REPORT

OHVIRA Syndrome with a Rare Presentation

Anusha Tanneru, Pranjali Rai, Neetha Poonja

Keywords : Acute urinary retention, OHVIRA syndrome, Renal agenesis, Uterine didelphys

Citation Information : Tanneru A, Rai P, Poonja N. OHVIRA Syndrome with a Rare Presentation. J South Asian Feder Obs Gynae 2023; 15 (3):354-356.

DOI: 10.5005/jp-journals-10006-2244

License: CC BY-NC 4.0

Published Online: 31-07-2023

Copyright Statement:  Copyright © 2023; The Author(s).


Abstract

Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare complex Mullerian anomaly with an incidence of 1:20,000. The commonest presentation is pain in the abdomen and progressive dysmenorrhea. Other rare symptoms could include vaginal discharge, fever, infertility, or acute abdomen. Case description: A 15-year girl presented with acute retention of urine which required catheterization. She had attained menarche 8 months back. On evaluation, the ultrasonography report revealed the presence of uterine didelphys with right side pelvic collection and absent kidney on the same side of the collection. On magnetic resonance imaging (MRI), a right obstructed hemivagina was clearly seen along with other findings suggesting the OHVIRA syndrome. Laparoscopically, a bulge was seen just below the right uterine horn which caused the retention of urine. Septal resection was performed vaginally, following which her symptoms subsided. Clinical significance: Suspect OHVIRA syndrome in adolescent girls when there is a renal anomaly with Mullerian defect. The patient can also present with acute retention of urine because of hematocolpos. Early detection and treatment will help to prevent complications of endometriosis and adverse fertility outcomes.


HTML PDF Share
  1. Hamidi H, Haidary N. Late presentation, MR imaging features and surgical management of Herlyn-Werner-Wunderlich syndrome (classification 2.2): A case report. BMC Women's Health 2018;18:161. DOI: 10.1186/s12905-018-0655-4.
  2. Ugurlucan FG, Dural O, Yasa C, et al. Diagnosis, management and outcome of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome): Is there a correlation between MRI findings and outcome? Clinical Imaging 2020;59:172–178. DOI: 10.1016/j.clinimag.2019.11.013.
  3. Kriplani A, Dalal V, Kachwhawa G, et al. Minimally invasive endoscopic approach for management of OHVIRA syndrome. J Obstet Gynecol India 2019;69(4):350–355. DOI: 10.1007/s13224-019-01240-4.
  4. Tzialidou Palermo I, Von Kaisenberg CS, Garcia Rocha GJ, et al. Diagnostic challenges of hemihematocolpos and dysmenorrhea in adolescents: obstructed hemivagina, didelphys or bicornuate uterus and renal aplasia is a rare female genital malformation. Arch Gynecol Obstet 2012;286:785–791. DOI: 10.1007/s00404-012-2392-5.
  5. Sharma R, Mishra P, Seth S, et al. OHVIRA syndrome—diagnostic dilemmas and review of literature. J South Asian Feder Obst Gynae 2020;12(6):421–426. DOI: 10.5005/jp-journals-10006-1847.
  6. David A, Gudi SN, Shankar R. Herlyn– Werner–Wunderlich syndrome: premenarche. J South Asian Feder Obst Gynae 2017;9(2):207–210. DOI: 10.5005/jp-journals-10006-1496.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.