JSWMC

Obstructed hemivagina with ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital anomaly involving uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We present a case of a 15-year-old girl who presented with lower abdominal pain and progressive dysmenorrhea since menarche. Transabdominal ultrasonography suggested uterine duplication with left-sided hematocolpos and absent left kidney. MRI confirmed uterus didelphys with complete vaginal and cervical duplication, left-sided hematometra, hematocolpos, and hematosalpinx. A CT scan confirmed left renal agenesis. Surgical management involved left hemihysterectomy, salpingectomy, and resection of the vaginal septum. The patient experienced immediate relief postoperatively and was counseled on long-term reproductive outcomes. This case highlights the importance of early recognition of OHVIRA syndrome in adolescent females with cyclical pelvic pain. Timely imaging, particularly MRI, and appropriate surgical intervention can prevent complications and preserve fertility.