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.