HERLYN-WERNER-WUNDERLICH SYNDROME: A VERY RARE UROGENITAL ANOMALY IN A TEENAGE GIRL


Karaca L., Pirimoglu B., BAYRAKTUTAN Ü., OĞUL H., Oral A., Kantarci M.

JOURNAL OF EMERGENCY MEDICINE, vol.48, no.3, 2015 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 48 Issue: 3
  • Publication Date: 2015
  • Doi Number: 10.1016/j.jemermed.2014.09.064
  • Journal Name: JOURNAL OF EMERGENCY MEDICINE
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Keywords: Herlyn Werner Wunderlich syndrome, hematocolpos, renal agenesis, Mullerian duct anomalies, IPSILATERAL RENAL AGENESIS, BLIND HEMIVAGINA, UTERUS
  • Inonu University Affiliated: No

Abstract

Background: Herlyn-Werner-Wunderlich (HWW) syndrome is an uncommon variant of Mullerian duct anomalies, consisting of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents in a post-pubertal adolescent or adult woman in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. Case Report: We report the case of a 13-year-old girl who presented to the emergency radiology department with sudden onset of severe pain at the right lower quadrant of the abdomen; imaging confirmed the diagnosis of HWW syndrome. Why should an emergency physician be aware of this?: When unilateral renal agenesis and uterus didelphys coexist, the first thing that the physician should remember is to confirm or refute the presence of a blind vagina for diagnosis of HWW syndrome. (C) 2015 Elsevier Inc.