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首页> 外文期刊>Case Reports in Obstetrics and Gynecology >Primary Amenorrhea, Aplastic Uterus, and a Functional Vagina: A Rare Case of Mayer-Rokitansky-Küster-Hauser Syndrome
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Primary Amenorrhea, Aplastic Uterus, and a Functional Vagina: A Rare Case of Mayer-Rokitansky-Küster-Hauser Syndrome

机译：原发性闭经，aplastic子宫和功能性阴道：罕见的mayer-rokitansky-küster-hauser综合征

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Background. Müllerian agenesis, also known as Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS), a failure of female urogenital development, typically results in a completely stenotic or rudimentary dimple vagina, both of which are generally nonfunctional in adulthood without mechanical dilation or surgical reconstruction. Case. A 20-year-old Tanner stage V heterosexual woman with normal sexual function since coitarche presented with a chief complaint of primary amenorrhea. She was found to have aplastic uterine buds, absent endometrium/cervix, normal ovaries, and an unusually well-developed lower vagina, a rare presentation of MRKHS. We discuss mechanisms by which the anomaly may have arisen. Summary & Conclusion. This case thus expands the clinical presentation of MRKHS to include a normal appearing vagina with intact sexual function from first sexual encounter, raising interesting questions about the basic underlying embryology.
机译：背景。 Müllerian患者，又称Mayer-Rokitansky-Küster-hauser综合征（MRKHS），雌性泌尿生殖发育的失败，通常导致完全狭窄或基本的凹坑阴道，这两者在成年时通常是无功能的，没有机械扩张或手术重建。案件。自体专业专业专业人员患有原发性闭经的专业技术以来，一名20岁的坦纳阶段v异性恋女性。她被发现具有增生性子宫芽，缺乏子宫内膜/子宫颈，正常卵巢，以及一个异常发达的下阴道不良，罕见的MRKHS呈现。我们讨论了可能出现异常的机制。摘要与结论。因此，这种情况扩大了MRKHs的临床介绍，包括正常出现的阴道，从第一次性遭遇，提高了关于基本潜在胚胎学的有趣问题。

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