Herlyn-Werner-Wunderlich syndrome

Case contributed by Dr Karwan T. Khoshnaw

Presentation

Primary infertility since 7 years

Patient Data

Age: 35
Gender: Female

Evidence of a two completely separate uteri with widely divergent apices associated with two separate cervices and two vaginas, in each uterus normal uterine zonal anatomy is preserved, also there is a markedly distended blood filled right vagina which shows hypointense signal centrally surrounded by a peripheral hyperintense signal on T1WIs and T1 FAT SAT and hyperintense on T2WIs and compressing the left vagina, the picture goes with uterus didelphys (class III müllerian duct anomaly) associated with grossly distended right vagina (Hematocolpos) likely secondary to transverse vaginal septum.

Right renal agenesis as seen in the images and was confirmed previously.

The above findings highly in favor of  Herlyn-Werner-Wunderlich syndrome.

A well-defined round shaped unilocular cystic lesion is seen in the right adnexa measuring 25*23 mm in maximal transverse diameter. It shows fluid signal (hypointense in T1 and high signal in T2 weighted images) and it has a thin wall with no evidence of fatty tissue, no internal septations or mural nodule could be seen, the finding in favor of a simple ovarian cyst.

Clear left adnexa.

Case Discussion

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).

The Herlyn–Werner–Wunderlich syndrome is characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain during menses secondary to hematocolpos. Awareness is necessary in order to diagnose and treat this disorder properly before complications occur.

This syndrome was described for the first time in 1922 and was suspected in a young woman with regular menstruation and gradually increasing pelvic pain and a pelvic mass after menarche. The true incidence of this anomaly is unknown, but it has been reported between 0.1% and 3.8%.The exact cause and pathogenesis are uncertain; however, the diagnosis and treatment at an early stage can relieve acute symptoms and preserve normal fertility.

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Case information

rID: 57380
Published: 21st Dec 2017
Last edited: 16th Jul 2018
Inclusion in quiz mode: Included

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