Complex genitourinary anomaly presenting in adolescence: should antenatal kidney abnormalities prompt future screening for Mullerian abnormalities?

Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital syndrome encompassing both Mullerian and mesonephric duct abnormalities. An early adolescent female presented with acute onset lower abdominal pain and sepsis several days after menarche. The accurate diagnosis was only achieved after her second presentation, a diagnostic laparoscopy and both CT and MRI. Her antenatal history was significant for a multicystic dysplastic kidney. As 30% of renal anomalies are associated with a Mullerian anomaly, an opportunity exists antenatally to facilitate screening for this in females with a prenatal diagnosis of a renal abnormality, thereby possibly avoiding the complications of diagnostic delay.