078 A rare case of Mayer-Rokitansky-K üster-Hauser syndrome associated with bilateral ovarian agenesis in a normal karyotypic female

Objective: Mayer-Rokitansky-K üster-Hauser (MRKH) syndrome is the second more common cause of primary amenorrhea resulting from congenital absence of the vagina and agenesis or hypoplasia of the Mullerian (paramesonephric) ducts. Patients with MRKH syndrome have normal functioning ovaries with normal 46, XX karyotype and underg o puberty. On the contrary bilateral gonadal agenesis is a rare cause of hypergonadotropic hypogonadism. Combination of these two pathologic entities is sporadically reported in the literature.
Source: European Journal of Obstetrics, Gynecology, and Reproductive Biology - Category: OBGYN Authors: Source Type: research