Late diagnosis of OHVIRA syndrome — case series and literature review

Abstract

OBJECTIVES: The aim of this study was to present three adult cases of obstructed hemivagina, and ipsilateral renal anomaly (OHVIRA) syndrome diagnosed in different gynecological centers in Poland. The clinical presentations, diagnostic imaging, surgical treatment, and outcomes were analyzed to highlight the diagnostic challenges and variability in clinical course. MATERIAL AND METHODS: This retrospective case series included three female patients aged 19,20 and 30 diagnosed in adulthood with OHVIRA syndrome. RESULTS: All three patients were diagnosed with uterus didelphys. Two had confirmed congenital right renal agenesis, while one had undergone nephrectomy in infancy. One patient presented with severe dysmenorrhea and purulent content in the right hemivagina, initially suspected to be hematocolpos. Another patient, with a history of adolescent hematocolpos, was diagnosed postpartum during assessment following cesarean section. The third case was identified incidentally during nephrological imaging. The patient had undergone the right nephrectomy in infancy. All patients underwent resection of the vaginal septum and drainage of hematocolpos. CONCLUSIONS: These cases show the variable clinical presentation of OHVIRA syndrome and emphasize the importance of a detailed history and imaging evaluation. Early diagnosis remains essential to prevent complications and provide treatment.