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Uterus didelphys with hematometrocolpos

Case contributed by Ahmed Abdrabou
Diagnosis certain

Presentation

Cyclic lower abdominal pain related to menstruation. The patient started menstruation few months ago.

Patient Data

Age: 14 years
Gender: Female

Duplication of the uterine cavity and cervix with vertical vaginal septum. On the right side there is cystic dilatation of the right vagina, dilatation of the right cervix and right uterine horn with fluid that returns bright signal on T1, T2 and SPIR indicating blood. Moreover, the patient had absent right kidney and compensatory hypertrophy of the left one.

Case Discussion

Uterus didelphys is class III congenital utero-vaginal anomalies as the abnormalities in lateral fusion of the mullerian ducts. The patient also had imperforate hymen on the right side with normal hymen on the left which makes menstruation occurs from the left side and blood accumulate on the right side forming hematometrocolpos.

The presence of uterus didelphys with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) triad is known as Herlyn-Werner-Wunderlich syndrome.

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