he Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) syndrome. A case report and review of the updated literature

Abstract

The OHVIRA syndrome, previously known as Herlyn-Werner-Wunderlich syndrome, is a rare female urogenital malformation characterized by the  coexistence of müllerian and renal anomalies, prominently featuring a didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis. Diagnosis is typically established after menarche, with the most common clinical symptoms including nonspecific pelvic pain associated with amenorrhea and palpable abdominopelvic mass secondary to hematocolpos. Frequent complications include chronic pelvic pain, infections, infertility, and endometriosis. In this context, the contribution of diagnostic imaging (ultrasound and magnetic resonance imaging) is essential for precise characterization of these findings. Furthermore, a comprehensive understanding of this syndrome, its multiple manifestations, and classification is crucial for implementing appropriate and timely treatment. We present the case of an 11-year-old female patient, accompanied by an updated review of the literature.