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Herlyn-Werner-Wunderlich syndrome

Case contributed by Humberto Rodrigo Prochnow Tibau
Diagnosis certain

Presentation

Female with known left renal agenesis, presenting with menstrual cramps and lower abdominal discomfort.

Patient Data

Age: 13 years
Gender: Female

Uterus didelphys noted, with duplication of the cervix and a vagina separated by a vertical septum. The left hemivagina is obstructed resulting in hematocolpos. Right Gardner cyst.

Case Discussion

Herlyn-Werner-Wunderlich syndrome is a rare syndrome caused by a disturbance in the fusion of the Mullerian ducts characterized by uterus didelphys, unilateral obstructed hemivagina (hematocolpos) and ipsilateral renal anomalies. The syndrome is also known as OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly). The patients are usually diagnosed after menarche when they present with lower abdominal pain and occasionally a palpable mass in the pelvis.

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