Obstructed hemivagina and ipsilateral renal anomaly syndrome: a case series of 4 patients with insights into diagnosis and treatment strategies.

Obstructed hemivagina and ipsilateral renal anomaly syndrome was first identified by Purslow in 1922 as a result of abnormal embryologic development of the Müllerian (paramesonephric) and Wolffian (mesonephric) ducts. The syndrome typically presents with cyclical lower abdominal pain and abnormal vaginal discharge in postpubertal females. Although the optimal timing and method of surgical treatment remain unclear, resection of the vaginal septum is commonly performed. This article presents 4 previously asymptomatic patients who developed an acute abdomen after menarche and were treated with hysteroscopic resection of the vaginal septum. This study retrospectively reviewed 4 postmenarchal female patients diagnosed with obstructed hemivagina and ipsilateral renal anomaly syndrome and treated surgically at our institution between 2023 and 2025. Cases 1 and 2 were 2 female patients (aged 14 and 15 years, respectively) who presented with acute abdominal pain, dysmenorrhea, and pelvic fullness after menarche. Imaging revealed left renal agenesis and hemorrhagic fluid in the left vagina. The patients underwent hymenotomy and left transverse vaginal septum resection under general anesthesia, draining 400 to 450 mL of coagulated blood. Follow-up imaging revealed a normal uterine morphology. Case 3 was an 11-year-old female patient with severe dysmenorrhea and a tense mass in the vagina who was diagnosed with a didelphic uterus and hematometra. The patient underwent a septum incision under anesthesia, with a significant amount of hemorrhagic material drained. Follow-up showed regular menstruation. Case 4 was a 16-year-old female patient who presented with severe cyclical pelvic pain and was diagnosed with obstructed hemivagina and ipsilateral renal anomaly syndrome (Herlyn-Werner-Wunderlich syndrome) based on imaging findings that revealed a didelphic uterus, an obstructed right hemivagina with hematocolpos, and a right renal agenesis. The patient underwent a successful surgical resection of the vaginal septum, which resulted in the resolution of her symptoms and a favorable postoperative recovery. Of note, 4 female patients aged 11 to 16 years with obstructed hemivagina and ipsilateral renal anomaly syndrome presented with progressive dysmenorrhea, pelvic pain, and vaginal fullness after menarche. Imaging confirmed a didelphys uterus, an obstructed hemivagina, a hematocolpos, and an ipsilateral renal agenesis in all cases. Surgical management via vaginal incision or resection of the vaginal septum successfully drained the accumulated blood and relieved symptoms. Postoperative follow-up showed resolution of hematocolpos, normal uterine morphology, and no major complication or recurrence. All patients reported a significant improvement in symptoms, with regular menstruation restored in the longer-term follow-up. The triad of obstructed hemivagina, uterus didelphys, and ipsilateral renal anomaly constitutes obstructed hemivagina and ipsilateral renal anomaly syndrome. The incidence of unilateral renal agenesis is 1 in 1000 live births, and the condition affects 30% to 70% of patients with Müllerian anomalies. There is a growing understanding that renal dysplasia may be more common in obstructed hemivagina and ipsilateral renal anomaly than in agenesis. After menarche, patients present with progressive dysmenorrhea, abdominal pain, and vaginal masses. Early diagnosis through a multimodal approach involving clinical history, imaging studies, and hysteroscopy or laparoscopy is crucial. Ultrasound and magnetic resonance imaging are essential tools for diagnosis, with magnetic resonance imaging offering detailed uterine morphology and information on vaginal continuity. The recommended surgical intervention involves resection of the vaginal septum, ideally before hematometrocolpos develops. Regular long-term follow-up after surgery is essential to monitor both renal and gynecologic outcomes. In cases where renal function is preserved, periodic renal assessments should be performed. In addition, the presence of vaginal stenosis and endometriosis should be regularly evaluated, as patients with obstructed hemivagina and ipsilateral renal anomaly are at an increased risk of retrograde menstruation and endometriosis. Postoperative complications may include adhesions, vaginal wall tearing, and reobstruction. Early diagnosis of obstructed hemivagina and ipsilateral renal anomaly 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 magnetic resonance imaging. Early surgical intervention, such as hysteroscopic vaginal septum incision, is effective in preventing reobstruction and minimizing complications in all cases. Regular follow-up is crucial to monitor renal function and gynecologic outcomes. The conclusions regarding long-term fertility preservation await data from extended follow-up.