Obstructed hemivagina and ipsilateral renal anomaly is defined by uterine didelphys, unilateral obstructed hemivagina and ipsilateral renal anomaly. Initially reported in 1922, this triad came to be known as Herlyn-Werner-Wunderlich Syndrome beginning in the 1970s1,2. In 2007, the acronym OHVIRA, was suggested by Laufer and colleagues to provide a common basis for describing the syndrome and allowed for inclusion of other uterine and renal anomalies3. Uterus didelphys is the most common finding, however bicornuate and septate uteri have also been described. Ipsilateral agenesis is the most common renal anomaly, although cases with normal renal anatomy and other malformations such as multi-cystic dysplastic kidney, renal duplication, and ectopic ureter have also been reported. OHVIRA occurs due to anomalous development of the reproductive tract, specifically involving the Mullerian ducts. The etiology of OHVIRA is multifactorial with both genetic and environmental factors, however no singular gene mutation has been identified4. The exact incidence of OHVIRA is unknown, however it is now better understood given improved access to gynecologic care and imaging techniques3.

The typical clinical presentation involves an adolescent female who presents a few years after menarche with severe cyclic dysmenorrhea3. Physical examination usually reveals a vaginal bulge on the obstructed side due to entrapped menstrual contents in the obstructed hemivagina4. Rare cases have been diagnosed in the neonatal period while other cases may be diagnosed in early childhood secondary to concurrent kidney concerns. Diagnosis is often delayed due to normal menstruation through the unobstructed side or a microperforation and unawareness of common gynecologic complaints associated with OHVIRA5. Pelvic magnetic resonance imaging (MRI) has become the gold standard for diagnosis and preoperative planning given the more detailed evaluation of the reproductive and urologic systems. Specific characteristics including septal thickness, septum positioning within the vagina, and volume distension of the vagina, cervix, and uterus collectively impact surgical management and procedural success. Prompt and accurate diagnosis of OHVIRA is critical for symptom relief and prevention of complications including infection, endometriosis, and pelvic adhesions. Definitive treatment involves surgical resection of the vaginal septum to drain the hematocolpos and achieve continuity of the vagina3. Although OHVIRA is a rare disorder with few retrospective studies, the understanding of this anatomic disorder has broadened with years of surgical experience. Our study aim was to review the experience of a single tertiary center among adolescent females with OHVIRA who underwent surgical reconstruction and assess outcomes as they relate to preoperative planning based on MRI findings.