Atypical presentation of OHVIRA Syndrome in an adolescent with chronic pelvic pain: A case report

Introduction: Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome, also known as Herlyn–Werner–Wunderlich syndrome, is a rare congenital Müllerian duct anomaly. It typically presents after menarche with cyclic pelvic pain secondary to menstrual blood retention.
Case Presentation: We report the case of a 15-year-old adolescent girl presenting with chronic pelvic pain in the absence of menstrual or urinary symptoms. Imaging revealed uterus didelphys associated with an obstructed right hemivagina complicated by hematometra and hematocolpos, along with an ipsilateral ectopic pelvic kidney. Surgical excision of the vaginal septum with marsupialization was performed, resulting in complete resolution of symptoms.
Conclusion: OHVIRA syndrome should be considered in adolescents presenting with chronic pelvic pain, even in the absence of menstrual abnormalities. Magnetic resonance imaging (MRI) plays a pivotal role in diagnosis, and early surgical management provides symptom relief while preserving reproductive potential.