Herlyn-Werner-Wunderlich syndrome

Last revised by Mostafa Elfeky on 18 Aug 2023

Herlyn-Werner-Wunderlich (HWW) syndrome, is a rare anomaly characterized by Müllerian duct anomalies (MDA) associated with mesonephric duct anomalies 3. This entity is also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). It is not to be confused with the Wunderlich syndrome (a spontaneous non-traumatic subcapsular and/or perirenal space hematoma).

Presentation is typically after menarche with pelvic pain due to the obstructed hemivagina. There may be a palpable pelvic mass.

Herlyn-Werner-Wunderlich (HWW) syndrome is characterized by a triad of type III Müllerian duct anomaly (uterus didelphys), obstructed hemivagina and mesonephric duct anomalies. Most often the latter manifests as renal agenesis 3 but duplicated kidneys, dysplastic kidneys, rectovesical bands or crossed fused ectopia may also be seen. 

Irrespective of imaging modality, the typical findings are:

Treatment is surgical, with resection of the septum dividing the two hemivaginas in order to relieve the obstruction. Subsequent successful pregnancy in the obstructed uterus has been reported 5.

When uterovaginal obstruction results in significant dilatation, the imaging differential may include a large adnexal mass such as a cystadenoma or endometrioma.

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