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KMID : 0882420070720000266
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Korean Journal of Medicine
2007 Volume.72 No. 0 p.266 ~ p.270
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A case of Mayer-Rokitansky-Kuster-Hauser syndrome
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Kim Ji-Eun
Lee Seung-Geun
Kim Bo-Hyun
Park Jeong-Rae
Kang Yang-Ho
Son Suk-Man
Kim Yong-Gi
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Abstract
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Mayer-Rokitansky-Kuster-Hauser syndrome (Mllerian agenesis) is a rare malformative anomaly (1/5000 women) that is associated with the congenital absence of the upper vagina and uterus with normal ovaries. We report a case of a 15-year-old woman who presented with primary amenorrhea and the absence of secondary sexual development. A hormone assay revealed hypogonadotrophic hypogonadism. The karyotype was normal, 46XX. Internal genitalia could not be identified on a pelvic ultrasound. There were no other morphological malformations. Hormone substitution therapy remains the only therapeutic option. Hormone substitution is aimed at triggering the development of secondary sexual haracteristics and preventing osteoporosis. The problem of infertility remains unsolved.(Korean J Med 72:S266-S270, 2007)
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KEYWORD
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Mayer-Rokitansky-Kuster-Hauser syndrome, Primary amenorrhea, secondary sexual development
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