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

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, I2 = 66%) and dysmenorrhea (64%, 95% CI 55-72, I2 = 46%). Ultrasound (86%, 95% CI 76-92, I2 = 58%) and pelvic magnetic resonance imaging (61%, 95% CI 46-74, I2 = 72%) were primary imaging modalities. Hematocolpos (55%, 95% CI 42-67, I2 = 53%) and hematometra (53%, 95% CI 37-69, I2 = 70%) were frequent findings. Pelvic endometriosis, a major long-term complication, affected 20% of patients. Vaginal septum resection (83%, 95% CI 75-89, I2 = 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, I2 = 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.