Herlyn-Werner-Wunderlich syndrome

Case contributed by Mostafa Elfeky
Diagnosis almost certain

Presentation

Progressive pelvic pain since menarche.

Patient Data

Age: 12 years
Gender: Female

Evidence of two separate uterine cavities, two cervices and upper vagina, consistent with uterine didelphys.

The right uterine cavity and cervical canal are filled with T1 and T2 high signal intensity fluid (subacute blood), suggesting right haematometrium secondary to obstructed right hemivagina.

Right hematosalpinx is also seen with T2 dark spot.

Both ovaries are normal.

Right renal agenesis. The left kidney shows physiological hypertrophy.

Case Discussion

Herlyn-Werner-Wunderlich syndrome is a rare type III Mullerian anomaly characterized by uterine didelphys, obstructed hemivagina forming haematometrium and ipsilateral renal agenesis (OHVIRA).

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