Background: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare congenital anomaly of the female urogenital tract. This report presents the clinical features, diagnostic approach, and management of an adolescent girl with OHVIRA syndrome.
Clinical case: A 14-year-old female was admitted with abdominal pain and irregular menstruation. Ultrasonography and MRI demonstrated uterus didelphys with right-sided hematometrocolpos and ipsilateral renal agenesis. External genital examination revealed an intact hymen with a bluish bulge 2 cm above the hymenal ring. Surgical exploration confirmed an obstructed right hemivagina, which was opened while preserving hymenal integrity. Menstrual blood was drained, and a Foley catheter was placed temporarily to ensure patency. The postoperative course was uneventful, and the patient was discharged on the second day.
Discussion and Conclusions: This case illustrates successful surgical management of OHVIRA syndrome while preserving hymenal integrity. Early diagnosis and imaging are essential to prevent complications such as hematometrocolpos, endometriosis, and infertility.
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