A Rare Case of Obstructed Hemivagina with Ipsilateral Renal Agenesis and Uterine Anomaly in an Adolescent: A Variant of Herlyn-Werner-Wunderlich Syndrome

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn–Werner–Wunderlich syndrome, is a rare congenital Müllerian anomaly typically characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. However, variants involving atypical uterine configurations and vaginal septa may occur, complicating diagnosis and management. We report the case of a 14-year-old adolescent girl who presented with recurrent lower abdominal pain a few months after menarche. Clinical evaluation revealed no palpable mass, but pelvic ultrasound showed the absence of the right kidney and two separate uterine cavities. MRI confirmed the presence of a bicorporeal uterus with two endometrial cavities and two cervical canals, a longitudinal non-obstructing vaginal septum, and a transverse obstructing vaginal septum. The obstruction resulted in hemato-trechalos and bilateral hematometra. The anomaly was classified as U3C2V1 under the ESHRE/ESGE classification. The patient underwent exploratory laparotomy with resection of the transverse vaginal septum and drainage of retained blood. Postoperative recovery was uneventful, and the patient was counselled regarding reproductive implications. This case represents a rare and complex variant of OHVIRA syndrome. Dual vaginal septa are uncommon, and their presence, along with bicorporeal uterus, underscores the diagnostic complexity. MRI proved invaluable in delineating anatomy and guiding management. Literature supports early diagnosis and timely surgical correction to prevent complications like endometriosis and infertility. This case highlights the importance of considering Müllerian anomalies in adolescents with post-menarche cyclical pain. MRI facilitates accurate diagnosis, and early surgical intervention can optimize clinical and reproductive outcomes.