Background: Obstructed Hemi Vagina with Ipsilateral Renal Anomaly (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare congenital Müllerian and Wolffian duct anomaly characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Early recognition is essential to prevent complications related to obstruction and preserve reproductive health.
Case Presentation: We report the case of a 14-year-old girl who presented with acute urinary retention and dull lower abdominal pain of one-day duration. Clinical examination revealed a tense cystic pelvic mass extending up to the umbilicus. Ultrasound and MRI confirmed uterus didelphys with left-sided hematometrocolpos due to an obstructed hemivagina associated with left renal agenesis, consistent with OHVIRA syndrome. Under general anaesthesia, vaginal septal resection and drainage of approximately 350 mL of altered blood were performed. Postoperatively, both cervices were visualized, urinary function normalized, and recovery was uneventful.
Conclusion: This case highlights the importance of considering OHVIRA syndrome in adolescent girls presenting with pelvic masses and urinary or menstrual symptoms. Early diagnosis and prompt surgical intervention result in excellent outcomes and help preserve future reproductive potential.
International Journal of Clinical Obstetrics and Gynaecology
Indexed in Index Copernicus
Research Journals