Turning obstruction into opportunity: successful obstetric outcome in a rare and complex case of OHVIRA syndrome variant

Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn–Werner–Wunderlich syndrome, is a rare Müllerian duct malformation characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Although rare, it bears significant gynaecological and reproductive consequences. We report a case of 24-year-old nulligravida who presented to us with chronic pelvic pain, dysmenorrhea, and persistent foul-smelling vaginal discharge. Magnetic resonance imaging demonstrated uterine didelphys with a distended obstructed left hemivagina and absent left kidney, turning clinical suspicion into reality. She underwent examination under anaesthesia followed by an exploratory laparotomy with drainage of hematopyometra, partial excision of the transverse vaginal septum, left endometrioma drainage, and left tubal delinking. Postoperative recovery was uneventful, with complete resolution of symptoms and restoration of vaginal patency. Ten months later, she conceived spontaneously, with the pregnancy localized to the right uterine horn which was meticulously monitored. At 32 weeks and 6 days of gestation, spontaneous preterm labour ensued, necessitating an emergency lower segment caesarean section. A live male neonate was delivered, and both maternal and neonatal outcomes were favourable. This case highlights the indispensable role of advanced imaging in precise anatomical delineation and emphasizes that timely, individualized surgical correction can restore reproductive potential. Despite increased risks of preterm birth and operative delivery, successful spontaneous conception and live birth are achievable in carefully managed women with OHVIRA syndrome. Early recognition remains crucial to prevent infection, endometriosis, and long-term reproductive morbidity in affected women.