[Clinical characteristics and complications in patients with OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) syndrome. Our experience].

INTRODUCTION: Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) syndrome is a rare variant of Müllerian anomalies. Delay in diagnosis and treatment can produce serious complications in patient's life and fertility. The aim of this study is to present our experience in clinical presentation, diagnosis and complications of patients with OHVIRA syndrome in a 20-year period.

PATIENTS AND METHODS: Retrospective analysis of 9 women under 25 years old with OHVIRA syndrome between 1997 and 2017. Analysis included clinical characteristics, demographic, diagnostic methods, follow-up, treatment and complications.

RESULTS: A total of 9 patients were studied, 5 diagnosed in prepuberal age and 4 postpuberal. 55% had prenatal diagnosis of renal agenesis and in 3 was incidentally diagnosed. Follow up period for single kidney to diagnosis was 3.6 years (range: 0-13,4 years). Right side was affected in 55%. Symptoms were dysmenorrea (44%), recurrent abdominal pain (22%), urinary dysfunction (22%). Complications were present in postpuberal patients and included hydrometrocolpos (22%) and pyocolpos (11%) requiring septostomy or septum resection. Surgical removal of the septum was performed in 44%). Prepuberal patients were asymptomatic.

CONCLUSIONS: OHVIRA syndrome is a rare anomaly in the development of Müllerian ducts with variable clinical presentation. High suspect in patients with single kidney is necessary to avoid potential complications by providing surgical treatment.