OHVIRA Syndrome: A case series of four Patients with Insights into Diagnosis and Treatment Strategies

Abstract

Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome, identified in 1922, results from abnormal embryological development of the Müllerian (paramesonephric) and Wolffian (mesonephric) ducts. The syndrome typically presents in postpubertal females with cyclical lower abdominal pain and abnormal vaginal discharge. While the optimal timing and method of surgical treatment remain unclear, vaginal septum resection is commonly performed. This paper presents four previously asymptomatic patients who developed acute abdomen after menarche and were treated with hysteroscopic resection of the vaginal septum. We retrospectively reviewed four postmenarchal female patients diagnosed with OHVIRA syndrome and treated surgically at our institution between 2023 and 2025 Case 1 & 2: Two patients (14 and 15 years old) presented with acute abdominal pain, dysmenorrhea, and pelvic fullness after menarche. Imaging revealed left renal agenesis and hemorrhagic fluid in the left vagina. They underwent hymenotomy and left transverse vaginal septum resection under general anesthesia, draining 400–450 mL of coagulated blood. Follow-up imaging showed normal uterine morphology. Case 3: An 11-year-old patient with severe dysmenorrhea and a tense mass in the vagina was diagnosed with a didelphic uterus and hematometra. She underwent septum incision under anesthesia, with the drainage of a significant amount of hemorrhagic material. Follow-up showed regular menstruation. Case 4: A 16-year-old girl presented with severe cyclical pelvic pain and was diagnosed with OHVIRA syndrome (Herlyn-Werner-Wunderlich) based on imaging that revealed a didelphic uterus, obstructed right hemivagina with hematocolpos, and right renal agenesis. She underwent successful surgical resection of the vaginal septum, with resolution of symptoms and favorable postoperative recovery. Four female patients aged 11 to 16 years with OHVIRA syndrome presented mainly with progressive dysmenorrhea, pelvic pain, and vaginal fullness after menarche. Imaging confirmed uterus didelphys, obstructed hemivagina, hematocolpos, and ipsilateral renal agenesis in all cases. Surgical management via vaginal incision or resection of the vaginal septum successfully drained accumulated blood and relieved symptoms. Postoperative follow-up showed resolution of hematocolpos, normal uterine morphology, and no major complications or recurrence. All patients reported significant symptom improvement, with regular menstruation restored in the longer-term follow-up. The triad of obstructed hemi-vagina, uterus didelphys, and ipsilateral renal anomaly constitutes OHVIRA syndrome. The incidence of unilateral renal agenesis is 1 in 1000 live births, and the condition affects 30-70% of patients with Müllerian anomalies. There is a growing understanding that renal dysplasia, rather than agenesis, may be more common in OHVIRA. Patients present after menarche with progressive dysmenorrhea, abdominal pain, and vaginal masses. Early diagnosis through a multi-modal approach involving clinical history, imaging studies, and hysteroscopy or laparoscopy is critical. Ultrasound and MRI are essential tools for diagnosis, with MRI offering detailed uterine morphology and vaginal continuity. The recommended surgical intervention involves resection of the vaginal septum, ideally before hematometrocolpos develops. After surgery, regular long-term follow-up is essential to monitor both renal and gynecological outcomes. In cases of preserved renal function, periodic renal assessments should be conducted. Additionally, the presence of vaginal stenosis and endometriosis should be regularly evaluated, as patients with OHVIRA have an increased risk of retrograde menstruation and endometriosis. Postoperative complications may include adhesions, vaginal wall tearing, and re-obstruction. Early diagnosis of OHVIRA syndrome relies primarily on a high index of clinical suspicion in menarchal-age females presenting with cyclical pelvic pain, dysmenorrhea, or a pelvic mass, supported by targeted radiological evaluation including pelvic ultrasound and MRI. Early surgical intervention, such as hysteroscopic vaginal septum incision, was effective in all cases in preventing re-obstruction and minimizing complications. Regular follow-up is critical for monitoring renal function and gynecological outcomes; conclusions regarding long-term fertility preservation await data from extended follow-up.