Introduction: Uterus didelphys with obstructed hemivagina associated with ipsilateral renal agenesis (OHVIRA syndrome) is a rare female urogenital malformation and delay in its diagnosis could lead to several complications.
Case presentation: A 21-year-old virgin woman was admitted to the emergency department (ED) with severe abdominal pain, without fever and vaginal discharge. She reported a history of cyclic abdominopelvic pain and dysmenorrhea for 5 years. The primary diagnosis (OHVIRA syndrome) was made using ultrasonography, spiral computed tomography (CT) and magnetic resonance imaging (MRI). In addition, laparoscopy was performed to confirm diagnosis and drain hematosalpinx. Then, hysteroscopy was carried out for septum resection and catheter insertion. At one-month follow-up the ultrasonography showed normal left hemicavity of uterus associated with significant decrease in dysmenorrhea.
Conclusion: Being aware of OHVIRA syndrome and clinical suspicion of this rare anomaly are essential for making a timely diagnosis, preventing complications, relieving symptoms, and preserving future fertility.
Complete septate uterus with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) in a young woman—a rare variant of Herlyn–Werner–Wunderlich syndrome
