OHVIRA syndrome presenting with acute abdomen: a case report and review of the literature

Clin Imaging. 2014 May-Jun;38(3):357-9. doi: 10.1016/j.clinimag.2013.12.011. Epub 2013 Dec 21.

Abstract

Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) or Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital urogenital anomaly. A 13-year-old female presented with acute abdominal pain and dysmenorrhea. Ultrasonography and magnetic resonance imaging showed uterus didelphys, hematometrocolpos, obstructed hemivagina, and right renal agenesis. Hemivaginal septal resection and anastomosis between the obstructed hemivagina and the normal vagina was tried, but it was not possible. Unilateral hysterectomy was performed. HWW syndrome may present with acute abdomen and is usually treated with vaginal septum resection and drainage of the hematometrocolpos.

Keywords: Mullerian anomaly; OHVIRA syndrome; Obstructed hemivagina; Unilateral hysterectomy; Uterus didelphys; Vaginoplasty.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Abdomen, Acute / diagnosis*
  • Abdominal Pain / diagnosis*
  • Abnormalities, Multiple / diagnosis*
  • Adolescent
  • Congenital Abnormalities / diagnosis*
  • Female
  • Humans
  • Hysterectomy
  • Kidney / abnormalities*
  • Kidney Diseases / congenital*
  • Kidney Diseases / diagnosis
  • Magnetic Resonance Imaging
  • Syndrome
  • Urogenital Abnormalities / diagnosis*
  • Urogenital Abnormalities / surgery
  • Uterus / abnormalities*
  • Uterus / surgery
  • Vagina / abnormalities*
  • Vagina / surgery

Supplementary concepts

  • Hereditary renal agenesis
  • Uterine Anomalies