Sadia Anwar


These are two case reports of females with primary amenorrhea, well developed secondary sexual characteristics and congenital absence of uterus. The incidence of Mayer-Rokitansky-Kuester-Hauser syndrome was not clearly established, but studies indicate a variation of 1/4,000 and 1/5,000 live births of the female sex. The syndrome is characterized by aplasia of the Müllerian duct (uterus and upper two-thirds of the vagina) on a person who has karyotype 46, XX with female phenotype characteristic of primary amenorrhea in adolescence. Treatment is usually delayed until the patient is ready to begin sexual activity.


Rokitansky syndrome; Mullerian duct; Karyotype; Primary amenorrhea.

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