Recurrent Uterine Leiomyomas in Herlyn-Werner-Wunderlich Syndrome (OHVIRA): A Saudi Arabian Case Report and Review of the Literature

Abstract

Herlyn-Werner-Wunderlich (HWW) syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, is a rare congenital Mullerian anomaly characterized by uterine didelphys, an obstructed hemivagina, and an ipsilateral renal anomaly. It is a rare condition, with some published series estimating an incidence below 1% (approximately 0.1-0.8%), although broader estimates of 0.1-3.8% have also been reported [11]. Surgical treatment during adolescence is directed at relieving vaginal obstruction while preserving reproductive function; however, long-term gynecologic follow-up remains important. We report a 45-year-old nulliparous woman with known HWW/OHVIRA syndrome and right renal agenesis who had previously undergone surgery for the obstructed hemivagina and associated right-sided Mullerian abnormalities. She later developed progressive abnormal uterine bleeding, pelvic pressure, and urinary frequency secondary to recurrent uterine leiomyomas. Pelvic MRI demonstrated multiple intramural and subserosal fibroids, with a dominant lesion measuring 7.2 x 7.5 x 6.8 cm and displacing the endometrial cavity. Her history included two prior myomectomies and seven unsuccessful intrauterine insemination (IUI) attempts. After counseling regarding conservative and definitive treatment options, she elected definitive surgical management. This case highlights the potential for significant adult gynecologic pathology in patients with previously treated HWW/OHVIRA syndrome and emphasizes the role of MRI in preoperative assessment of complex Mullerian anatomy.