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Herlyn-Werner-Wunderlich syndrome with tubo-ovarian endometriosis

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Pelvic pain with a palpable pelvic mass.

Patient Data

Age: 13 years
Gender: Female

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:

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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