OHVIRA Syndrome: A Rare Congenital Anomaly Characterized by Uterine Didelphys with Obstructed Hemivagina and Ipsilateral Renal Anomaly

Abstract

The objective of this case report is to detail and discuss a rare case of OHVIRA or Herlyn-Werner-Wunderlich syndrome, a rare complex congenital Mullerian duct anomaly characterized by uterine anomaly with obstructed hemivagina and ipsilateral renal anomaly. We present this case of 16 year female who presented with cyclical lower abdominal pain, after 3 years of attaining menarche. Initial ultrasonography followed by computed tomography in this patient revealed classic triad of findings. The patient had absent right kidney with compensatory hypertrophy of left kidney and uterine didelphys configuration with right sided hematometrocolpos suggesting obstructed hemivagina. She underwent vaginal septostomy following which there was resolution of her symptoms.  Our case is noteworthy since the diagnosis was made earlier in the disease, before extensive obstruction and hematometrocolpos developed.