| Mayer-Rokitansky-Küster-Hauser증후군 1예 |
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김승기1, 김성엽2 |
1제주대학교 의과대학 의학과
2제주대학교 의과대학 산부인과학교실 |
| A Case of Mayer-Rokitansky-Küster-Hauser Syndrome |
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Seung Ki Kim1, Sung Yob Kim2 |
1Department of Medicine, Cheju National University, Jeju 690-756, Korea
2Department of Obstetrics and Gynecology College of Medicine, Cheju National University, Jeju 690-756, Korea |
Correspondence:
Sung Yob Kim, Email: mdkim66@cheju.ac.kr
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| Abstract |
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The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in women showing normal development of secondary sexual characteristics and a normal 46, XX karyotype. It affects at least 1 out of 4500 women. MRKH may be isolated (type I) but it is more frequently associated with renal, vertebral, and, to a lesser extent, auditory and cardiac defects (MRKH type II or MURCS association). The first sign of MRKH syndrome is a primary amenorrhea in young women. It is usually with normal development of secondary sexual characteristics and normal external genitalia, with normal and functional ovaries, and karyotype 46, XX without visible chromosomal anomaly. The phenotypic manifestations of MRKH syndrome overlap with various other syndromes. Treatment of vaginal aplasia is creation of a neovagina to allow sexual intercourse. Psychological distress is very important in young women with MRKH, so counseling before and throughout the treatment is essential.
A case of Mayer-Rokitansky-Küster-Hauser syndrome is reviewed with brief literature. |
| Key Words:
Mayer-Rokitansky-Küster-Hauser |
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