A Case of Mayer-Rokitansky-Kster-Hayser Syndrom.

Lee, Kyoung Suk; Kang, Jeong Bae; Kim, Hong Bai; Lee, Keun Yong; Kang, Sung Won

Case Report

Korean Journal of Obstetrics & Gynecology 1997;40(8):1751-1755.
Published online January 1, 2001.

A Case of Mayer-Rokitansky-Kster-Hayser Syndrom.

Kyoung Suk Lee, Jeong Bae Kang, Hong Bai Kim, Keun Yong Lee, Sung Won Kang

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.

Key Words: Mayer-Rokitansky-Kster-Hauser syndrome