Herlyn-Werner-Wunderlich associated hematometra mimicking endometrioid neoformation: A rare case report

In this case report we described a a case of ultra-rare Mullerian anomaly associated with renal agenesia in a syndromic framework of Herlyn-Werner-Wunderlich syndrome in a 15 years old patient who complained dysmenorrhea and abdominal pain. Abdominal MRI showed a 18 cm hourglass neoformation with dysomogeneous content, suspected for endometrioma. However, transrectal ultrasound showed the presence of didelphys uterus, a single vagina and two cervices. After a pre-operatory echo-guided operative hysteroscopy, that was unable to drain the hematometra because of the presence of a deep fibrotic septum, a mini-Pfannensteil abdominal laparotomy was performed. The hematometra was drained by hysterotomy and the septum was excised trought the cervix to create a continuity with the vagina. The post-operative period was uneventful, and the patient is now under regular follow-up. We underline the need of multidisciplinary counselling for those complex cases where the genetician, gynecologist, urologist should work together for the need and expectations of the patients.