Presentation
Right-sided chronic pelvic pain
Patient Data
Age: 30 years
Gender: Female
From the case:
Herlyn-Werner-Wunderlich syndrome
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- uterus didelphys
- complete duplication of uterine horns as well as duplication of the cervix and vagina
- virginal gel within the left hemivagina
- the right hemivagina appears distended by fluid-like density because of obstructed right hemivagina, secondary to the low signal oblique vaginal septum
Multiple myomas infiltrated both horns as followed:
Right horn :
- intramural myoma with subserosal extension measuring about 30 x 34 mm along with anterior wall (FIGO V)
- intramural myoma with subserosal extension measuring about 30 x 32 mm along with posterior wall (FIGO V)
- intramural myoma with subserosal extension measuring about 20 x 23 mm along with uterine fundus (FIGO V)
- a few intramural myomas with a maximum diameter of up to 20 mm (FIGO IV)
Left horn :
- subserosal pedunculated myoma measuring about 43 x 56 mm originated from the posterior wall (FIGO VII)
- subserosal myoma measuring about 18 x 23 mm originated from uterus lower segment posterior wall (FIGO VI)
- three intramural myomas with a maximum diameter up to 13 mm (FIGO IV)
After contrast injection, all myomas show hypo enhancement in comparison with normal uterine parenchyma.
- ptotic right kidney and left kidney with a duplex pelvicalyceal system on the sonogram (not shown)
Case Discussion
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 1 but duplicated kidneys, dysplastic kidneys, rectovesical bands, or crossed fused ectopia may also be seen.
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