Obstructed Hemi-vagina with Ipsilateral Renal Agenesis Syndrome: A Case Report

The Obstructed Hemi-vagina with Ipsilateral Renal Agenesis (OHVIRA) syndrome, or Herlyn-Werner-Wunderlich (HWW) syndrome, is a rare congenital disorder characterised by abnormalities of the Müllerian ducts and ipsilateral renal agenesis. Hereby, the authors present a case of a 15-year-old female who presented with lower abdominal pain and irregular menstrual cycles for one year. On complete evaluation of the pelvis using ultrasound, Computed Tomography (CT) and Magnetic Resonance Imaging (MRI), findings of right renal agenesis with compensatory left renal hypertrophy, Bicorporeal septate uterus with bicollis (uterine duplication anomaly), Complete obstruction of the right hemi-vagina by a transverse vaginal septum, and haematocolpos due to retained menstrual blood were seen, which are diagnostic of OHVIRA syndrome with right hemi-vaginal obstruction and ipsilateral renal agenesis, for which the patient underwent diagnostic hysterolaparoscopy with vaginal septal resection. The collection was drained from the right hemi-vagina and contained thick, blood-stained, purulent material, with E. coli growth on culture. The case shows the significance of early diagnosis of the syndrome in an adolescent female patient. If corrected at the right time using surgical techniques, other complications are avoided, such as secondary infections, chronic pelvic inflammatory disease, endometriosis, and infertility