EC Gynaecology

A 13-year-old girl was diagnosed with OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly). In the antenatal period, a multicystic dysplasia of the ectopic pelvic right kidney was diagnosed. During puberty, she consulted for dysmenorrhea and the exams concluded to a didelphys uterus with obstructed hemivagina. The OHVIRA syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is rare. Urinary and genital malformations are often associated because of their close relation during embryogenesis. The symptoms are unspecific. Depending on the subtype of the obstructed hemivagina, the symptoms and the age of onset can vary greatly, making the diagnosis challenging. However, it is vital to correctly diagnose this malformation and treat it to avoid complications such as pelvic inflammatory disease (PID), endometriosis, and infertility. The treatment consists of surgery: ideally a surgical resection of the vaginal septum in one step.

It is important to put in place a protocol to screen Mullerian anomalies when renal anomalies are diagnosed because of the high association between the two, and the risk of complication if left untreated.

 Keywords: OHVIRA Syndrome; Mullerian Duct Anomalies; Early Detection; Renal Anomalies

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