Mayer-Rokitansky-Kuster-Hauser syndrome – An unusual case of primary amenorrhea
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Date
2022-02
Journal Title
Journal ISSN
Volume Title
Publisher
Mansa STM Publishers
Abstract
The onset of menstruation is a significant milestone of sexual maturation in a girl child. Although there are numerous causes of primary amenorrhea, Mayer-Rokitansky-Küster-Hauser syndrome, also known as Müllerian aplasia, is one of the extremely rare causes of primary amenorrhea which is usually picked up by suitable imaging techniques like magnetic resonance imaging. Affected females have a normal female hormonal function and a normal karyotype but non-functional vagina and uterus which make it an interesting entity. Although specific treatment is not known for this entity except for vaginoplasty and creation of a neovagina for sexual gratification, the clinical and diagnostic workup of these patients has evolved in recent years and infertility treatment and child-bearing through in vitrofertilization have become a part of the long-term treatment plan for these females.
Description
Keywords
Mayer-Rokitansky-Küster-Hauser, Mullerian aplasia, Neovagina, Vaginal agenesis
Citation
Dixit Anushree, Aggarwal Kailash Chander, Agrawal Alka. Mayer-Rokitansky-Kuster-Hauser syndrome – An unusual case of primary amenorrhea. Indian Journal of Case Reports. 2022 Feb; 8(2): 43-45