Complex Müllerian Duct Anomaly Presenting as a Rare OHVIRA Variant: Clinical and Surgical Perspectives

Abstract Background Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) syndrome, also referred to as Herlyn–Werner–Wunderlich syndrome, is an uncommon congenital anomaly of the genitourinary tract characterized by uterine malformations, an obstructed hemivagina, and ipsilateral renal agenesis. Atypical variants add further complexity, often posing significant diagnostic and therapeutic challenges. Case Presentation: We report the case of a 14-year-old girl presenting with primary amenorrhea and cyclical abdominal pain. Imaging revealed a bicornuate unicollis uterus with hematometrocolpos, absent left kidney, and a grossly dilated ectopic right ureter. Intraoperatively, the cervix was rudimentary and hypoplastic, precluding fertility preservation. A complete hysterectomy with right ureteric reimplantation was performed using the Politano–Leadbetter technique. Postoperative recovery was uneventful, and the patient experienced complete resolution of symptoms at follow-up. Discussion This case demonstrates an atypical variant of Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) syndrome, characterized by bicornuate uterus, rudimentary cervix, segmental vaginal atresia, unilateral renal agenesis, and ureteric malpositioning. Such complex anomalies underscore the limitations of current classification systems and emphasize the importance of detailed anatomical description. Magnetic resonance imaging remains the diagnostic gold standard, while multidisciplinary planning is crucial for tailoring management to individual patients. When reconstructive options are not feasible, definitive surgical intervention is often required to achieve symptom relief and prevent long-term morbidity. Conclusion Atypical variants of OHVIRA syndrome underscore the importance of integrating embryological understanding, systematic renal screening, and collaborative surgical planning. Early recognition and tailored intervention are critical to achieving symptom relief and preventing long-term morbidity.