Pediatr Emerg Med J.  2016 Jun;3(1):32-35. 10.0000/pemj.2016.3.1.32.

A case of Herlyn-Werner-Wunderlich syndrome: a rare, congenital genitourinary anomaly in a 12-year-old girl

• Park JW
• Jung JY

• KMID: 2368373
• DOI: http://doi.org/10.0000/pemj.2016.3.1.32

Abstract

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare, congenital genitourinary anomaly involving the Müllerian and Wolffian structures, and is characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents in adolescent girls in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. Accurate diagnosis and surgical treatment can be delayed for several months or even years. Here, we report a case of a 12-year-old girl who presented to the emergency department with lower abdominal pain and mass that had lasted for 2 weeks. After the confirmation of HWW syndrome with magnetic resonance imaging, hysteroscopic septostomy was carried out as a definitive treatment. When we evaluate adolescent girls with lower abdominal pain and mass, we should consider the possibility of HWW syndrome.

Keyword

Hematocolpos; Hereditary Renal Agenesis; Mullerian Ducts; Uterine Anomalies; Wolffian Ducts

MeSH Terms

Abdominal Pain
Adolescent
Child*
Diagnosis
Emergency Service, Hospital
Female*
Hematocolpos
Humans
Magnetic Resonance Imaging
Mullerian Ducts
Wolffian Ducts