Herlyn-Werner-Wunderlich syndrome

Case contributed by Dr Dalia Ibrahim

Presentation

Left pelvic pain.

Patient Data

Age: 20 years
Gender: Female

Complete duplication of uterine horns as well as duplication of the cervix and vagina. 

 The right hemivagina appears collapsed.

The left hemivagina appears distended by fluid like density; likely obstructed left hemivagina, secondary to vaginal septum.

Absent left kidney (at the same side of vaginal obstruction)

Annotated image

Complete duplication of uterine horns as well as duplication of the cervix and vagina. 

 The right hemivagina appears collapsed.

The left hemivagina appears distended by fluid like density; likely obstructed left hemivagina, secondary to vaginal septum.

Absent left kidney (at the same side of vaginal obstruction).

Case Discussion

Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Müllerian ducts and Wolffian structures,

It is characterized by the triad of:

  • Duplication of the uterus, cervix and vagina
  • Unilateral hematocolpos or hematometrocolpos
  • Absent kidney on the same side as the uterovaginal obstruction
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Case information

rID: 29052
Published: 29th Apr 2014
Last edited: 6th Jan 2021
System: Gynaecology
Inclusion in quiz mode: Included