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Mayer-Rokitansky-Küster-Hauser syndrome

Case contributed by Ahmed Abdrabou
Diagnosis almost certain

Presentation

Primary amenorrhea

Patient Data

Age: 17 years
Gender: Female

The uterus and vagina couldn't be seen while the ovaries are well developed and show multiple follicles consistent with mullerian duct agenesis.

Case Discussion

The case shows compete agenesis of the uterus, cervix and vagina which is consistent with Class I mullerian duct agenesis or Mayer Rokitanksy Kuster Hauser syndrome.

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