Clinical features and surgical options of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome: A systematic review and a meta‐analysis of prevalence

Abstract Background OHVIRA syndrome, a urogenital malformation, lacks standardized management. Narrative reviews exist, but there is no a comprehensive meta‐analysis. Objectives The aim of this first systematic review and meta‐analysis is to evaluate the current literature and inform management strategies. Search Strategy We searched Scopus, Medline, Embase and Web of Science, up to March 2024. Selection Criteria Case series, case reviews, and longitudinal studies on patients with OHVIRA syndrome. Data Collection and Analysis Data were extracted and meta‐analyzed using R software. Main Results In all, 35 studies (1988–2022) with 526 patients were included. Average symptom onset was 14.45 years, and diagnosis at 16.36 years suggests potential delays. The most common symptoms were abdominal pain (67%, 95% CI 54–77, I 2 = 66%) and dysmenorrhea (64%, 95% CI 55–72, I 2 = 46%). Ultrasound (86%, 95% CI 76–92, I 2 = 58%) and pelvic magnetic resonance imaging (61%, 95% CI 46–74, I 2 = 72%) were primary imaging modalities. Hematocolpos (55%, 95% CI 42–67, I 2 = 53%) and hematometra (53%, 95% CI 37–69, I 2 = 70%) were frequent findings. Pelvic endometriosis, a major long‐term complication, affected 20% of patients. Vaginal septum resection (83%, 95% CI 75–89, I 2 = 48%) was the most common surgical treatment, often accompanied by laparoscopic endometriosis excision. Hysteroscopic septum resection emerged as a minimally invasive option (78%, 95% CI 46–93, I 2 = 54%), with high success rate. Conclusions Early diagnosis and surgery are crucial to prevent complications. Vaginal septum resection remains the gold standard, while hysteroscopy offers a promising minimally invasive alternative.