Presentation
Primary infertility since 7 years
Patient Data
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 (Haematocolpos) likely secondary to transverse vaginal septum.
Right renal agenesis as seen in the images and was confirmed previously.
The above findings highly in favour 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 favour of a simple ovarian cyst.
Clear left adnexa.
Case Discussion
Herlyn-Werner-Wunderlich (HWW) syndrome, is a rare anomaly characterised 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 characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain during menses secondary to haematocolpos. 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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