Herlyn-Werner-Wunderlich syndrome with tubo-ovarian endometriosis
Diagnosis almost certain
Presentation
Pelvic pain with a palpable pelvic mass.
Patient Data
Age: 13 years
Gender: Female
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The MRI sequences demonstrate:
- duplication of the uterus, cervix and vagina
- distended fluid-filled right hemivagina in keeping with hematocolpos, compressing and displacing the left hemivagina
- right renal agenesis well-demonstrated on coronal FIESTA sequence
- Enlarged right ovary with a few cysts of high signal on T1, T1 fat sat with T2 shading as well as an ipsilateral tubular cystic structure eliciting the same signal suggestive of a tubo-ovarian endometriosis
Case Discussion
MRI features most consistent with Herlyn-Werner-Wunderlich syndrome which is characterized by:
- duplication of the uterus, cervix and vagina
- unilateral hematocolpos or hematometrocolpos
- congenital renal anomalies on the same side as the uterovaginal obstruction (most likely Renal agenesis, and less likely multicystic dysplastic kidney and renal duplication)
Around 20% of patients with Herlyn-Werner-Wunderlich syndrome may develop pelvic endometriosis, usually ipsilateral to the hematocolpos or hematometrocolpos 1.
Additional contributor: A. Ramdani, MD, CIM Aurès, Batna, Algeria
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