Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Spectrum of Clinical Presentations, Diagnostic Challenges, and Surgical Management

Background: Müllerian anomalies are congenital abnormalities of the female reproductive tract that may present with a wide range of clinical manifestations, and some forms may remain undiagnosed until adolescence or until complications develop. This study aimed to evaluate the clinical presentations, radiological findings, diagnostic challenges, and surgical management of patients with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome treated at a tertiary referral center. Methods: The study included patients diagnosed with OHVIRA syndrome from July 17, 2019, to October 1, 2024. Following ethics committee approval, cases were categorized into four different scenarios. Scenario 1: suspected OHVIRA syndrome during infancy; Scenario 2: obstructed hemivagina with hematocolpos at puberty; Scenario 3: spontaneous fistulization of hematocolpos; and Scenario 4: pregnancy following surgical management of OHVIRA syndrome. The diagnostic approach and follow-up processes for patients in each clinical scenario were summarized. Results: Six patients were categorized into four distinct clinical scenarios according to their clinical presentations. Five patients had confirmed OHVIRA syndrome, while one patient had suspected OHVIRA syndrome based on the presence of ipsilateral renal agenesis and a cystic vaginal lesion. Clinical presentations ranged from suspected OHVIRA syndrome presenting with mucocolpos during infancy to hematocolpos at puberty, spontaneous fistulization, and successful pregnancy following previous vaginal septum resection. Pelvic ultrasonography (USG) and magnetic resonance imaging (MRI) accurately identified uterus didelphys, obstructed hemivagina and hematocolpos. We present a case of mucocolpos and a case describing the pregnancy outcome following surgery for hematocolpos. Three patients underwent vaginoscopic hematocolpos drainage, one patient was diagnosed following spontaneous fistulization of hematocolpos, and five patients had favorable clinical outcomes without recurrent obstruction during follow-up. The remaining patient, who was followed during early childhood for mucocolpos, is awaiting menarche. Conclusions: OHVIRA syndrome encompasses a broad spectrum of age-dependent clinical presentations, which can pose significant diagnostic challenges. Early recognition and individualized management based on clinical and anatomical findings are essential, with USG and MRI playing key roles in establishing the diagnosis.