Mayer-Rokitansky-Hauser syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Rokitansky sequence

Overview

Amenorrhea can be secondary to abnormalities in the development of the genital tract. Mayer-Rokitansky-Hauser syndrome is one such abnormality in the development of the genital tract which is characterized by an absent uterus and a foreshortened vagina. The ovaries function normally and produce estradiol (E2). As a consequence, the breasts and pubic hair are normal. Mayer-Rokitansky-Hauser syndrome is responsible for 15% of primary amenorrhea cases. Turner syndrome is the most common cause of primary amenorrhea.

Acknowledgements

The content on this page was first contributed by: C. Michael Gibson, M.S., M.D.

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