OHVIRA-Syndrom

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

Clinical presentation

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

Pathology

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  but duplicated kidneys, dysplastic kidneys, rectovesical bands or crossed fused ectopia may also be seen.

Radiographic features

Irrespective of imaging modality, the typical findings are:

Treatment and prognosis

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 .

Differential diagnosis

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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