A Case of Mayer-Rokitansky-Kster-Hayser Syndrom / 대한산부인과학회잡지

ABSTRACT

A perplexing developmental error is 46,XX congenital absence of vagina ( Mayer-Rokitan-sky-Kster-Hauser syndrome, Mullerian dysgenesis, vaginal aplasia ). The Mayer-Rokitansky-Kster-Hauser syndrome refers to the climical entity consisting of primary amenorrhea associated with congenital absence of the vagina, 46,XX karyototype, a rudimentary uterus or complete absense, normal overian function and normal ovulation, normal female breast development, body proprotion and body hair, frequent association of renal, skeletal and other cpngenital anomalies. This syndrome results from agenesis of both Mllerian ducts or from a failure of the Mllerian ducts to estavlish a proper communication with that part of the vagina from the urogenital sinus. A case of mayer-Rokitansky-Kster-Hauser syndrom was reviewed briefly.