A rare case of OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) syndrome: Case report

Abstract

Obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA) also known as Herlyn-Werner-Wunderlich syndrome is a rare type of Müllerian duct anomaly with concomitant mesonephric duct anomalies. It is characterized by uterus didelphys, obstructed hemi-vagina, and commonly renal agenesis however; other types of mesonephric duct anomalies such as duplicated kidneys, dysplastic kidneys, rectovesical bands, or crossed fused ectopia have also been reported. We present a case report of a 21-year-old female patient experiencing new-onset vaginal bleeding and pelvic pain over the past month. Subsequent multi-sequence MRI revealed characteristic features indicative of OHVIRA syndrome. Clinical manifestations typically encompass pelvic pain and menstrual abnormalities but mainly depend upon the presence of communication between the hemi-uterus. This article aims to discuss the clinical presentation, types, and diagnostic utility of ultrasound and MRI in OHVIRA syndrome. Additionally, we discuss the possible management plans.

Keywords

• Obstructed hemivagina and ipsilateral renal anomaly syndrome
• OHVIRA
• Müllerian duct anomaly (MDA)
• Mesonephric duct anomaly
• Herlyn-Werner-Wunderlich syndrome