Herlyn-Werner-Wunderlich syndrome (OHVIRA)

Presentation

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

Patient Data

Age: 13 years
Gender: Female
Modality: MRI

Uterus didelphys noted, with duplication of the cervix and a vagina separated by a vertical septum. The left hemivagina is obstructed resulting in haematocolpos. You can also see a Gardner cyst on the right, on T2 axial images.

Case Discussion

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

 

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Case Information

rID: 46823
Case created: 18th Jul 2016
Last edited: 26th Oct 2016
Inclusion in quiz mode: Excluded

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