Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA syndrome) with a multiple renal artery and essential hypertension

Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a syndrome characterized by double uterus, blind hemivagina and ipsilateral renal agenesis. In addition, multiple renal arteries are characterized by the presence of more than one renal artery in one of the kidneys and are often combined with essential hypertension. We report the case of an 11-year- and 6-months-old female. At the age of 8 years, she was indicated unilateral left renal agenesis and uterine hypoplasia. At the age of 9 years and 8 months, she developed headache and blood pressure of 154/82 mmHg, and was admitted to hospital for further evaluation and medical treatment. Antihypertensive treatment was carried out and completed in 4 months, but her blood pressure increased again one year later. Abdominal MRI revealed unilateral left renal agenesis, double uterus and left hemivaginal obstruction, therefore diagnosed as OHVIRA syndrome. In addition, there were two right renal arteries, suggesting right multiple renal artery. In females, unilateral renal agenesis requires further evaluation because of its high rate of association with uterovaginal abnormalities. Further, in this study, hypertension may have developed as a result of a congenital solitary kidney with multiple renal artery.