Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up

Objective: To review the experience at two large referral centers with the syndrome of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), and to review the risks, benefits, and complications of surgical management options. Design: Retrospective case series. Setting: Children's Hospital Boston and Brigham and Women's Hospital, Boston, Massachusetts. Patient(s): Twenty-seven cases of obstructed hemivagina over a 12-year period. Main Outcome Measure(S): For each patient who presented symptoms, diagnostic studies, anatomic findings, surgical management, outcomes, and complications were reviewed. Result(S): The mean age of diagnosis was 14 years. Twenty-three patients had ipsilateral renal anomalies, including 20 patients who had renal agenesis. Two had dysplastic ipsilateral kidneys requiring nephrectomy in infancy. Twenty-six patients underwent vaginal reconstruction, and eight of those additionally underwent laparoscopy for clarification of diagnosis. Six required a two-stage vaginoplasty because of incomplete previous resection (n = 1), infection or anatomic distortion (n = 4), or restenosis (n = 2). Vaginal septum adenosis was seen in eight patients. Conclusion(s): Most patients with OHVIRA syndrome can be treated solely with single-stage vaginoplasty. Routine laparoscopy is not essential to management. Vaginal stenosis is a postoperative possibility, and may be associated with vaginal adenosis.