Fertility, Pregnancy, and Psychological Burden in OHVIRA Syndrome: Clinical Case Study and Review of the Literature

Introduction: Obstructed HemiVagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome, also known as Herlyn–Werner–Wunderlich syndrome, is a rare congenital Müllerian duct anomaly, characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Symptoms typically appear shortly after menarche and include dysmenorrhea and pelvic pain. The psychological burden associated with fertility and reproductive outcomes in women with OHVIRA syndrome remains poorly investigated. Materials and methods: A 30-year-old primigravida with left renal agenesis and a history of vaginal abscess, dysmenorrhea, and chronic pelvic pain received a delayed OHVIRA syndrome diagnosis. The patient had previously been informed that spontaneous conception and an uncomplicated pregnancy were highly unlikely because of her congenital gynecological condition, resulting in significant fertility-related anxiety and psychological distress. Under careful supervision and counseling, she conceived successfully, and the pregnancy progressed without complications; an elective cesarean section was performed at term. A literature search using the PubMed and Embase databases was conducted between November 2025 to April 2026 to identify studies reporting reproductive outcomes and psychological aspects in patients diagnosed with OHVIRA syndrome and other Müllerian anomalies. Results: Evidence-based counseling contributed to improvement of quality of life and reduction of pregnancy-related anxiety of the reported patient with OHVIRA syndrome. A limited number of studies discuss the mental burden and fertility-related anxiety of patients with OHVIRA syndrome and other Müllerian anomalies. Conclusions: Spontaneous conception and uncomplicated pregnancy are possible for women with OHVIRA syndrome. The psychological burden associated with congenital gynecological conditions remains under-recognized and requires further investigation. Comprehensive counseling and interdisciplinary care are essential to improve reproductive education, mental health support, and pregnancy outcomes in patients with congenital gynecological anomalies.