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Mayer-Rokitansky-Küster-Hauser综合征的综合管理:一例报告

Comprehensive management of Mayer-Rokitansky-Küster-Hauser syndrome management: A case report.

作者信息

Mishra Sanskar, Sapkale Bhagyesh, Singh Shreya, Jha Asha, Chaudhari Kamlesh

机构信息

Faculty of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, India.

Department of Pharmacology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, India.

出版信息

Narra J. 2024 Aug;4(2):e755. doi: 10.52225/narra.v4i2.755. Epub 2024 Jul 9.

Abstract

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also known as Müllerian aplasia, is a congenital condition characterized by uterine and upper vaginal aplasia. It affects females with a normal female karyotype and typical secondary sex characteristics. The aim of this case report was to highlight the multidisciplinary management approach for MRKH syndrome, focusing on tailored interventions to address physical and psychological challenges and improve reproductive prospects. A 26-year-old married female presented to Acharya Vinoba Bhave Rural Hospital (AVBRH), Sawangi, Wardha, India, in January 2023 with primary amenorrhea. Physical examination revealed a blind vagina and a hypoplastic uterus, indicative of MRKH syndrome. Further evaluation, including pelvic magnetic resonance imaging (MRI), confirmed Müllerian duct abnormalities and bilateral ovarian anomalies. The absence of a functional vagina significantly impacted the patient's quality of life, leading to difficulties with sexual intercourse and emotional distress related to infertility. A collaborative approach involving a gynecologist and a psychiatrist at AVBRH was initiated to address these challenges. The patient underwent vaginoplasty to create a neovagina, enhancing the sexual function and intimate relationship with the spouse. However, due to the hypoplastic uterus, achieving motherhood through traditional means was not possible. Therefore, assisted reproductive techniques, in particular surrogacy, were explored. Normal, functional ovaries were harvested from the patient for use in surrogacy procedures. This comprehensive management strategy exemplifies the challenges associated with MRKH syndrome and underscores the importance of tailored interventions and long-term follow-up. The case highlights the significance of collaborative care in improving the quality of life and reproductive prospects for individuals with MRKH syndrome.

摘要

迈耶-罗基坦斯基-库斯特-豪泽(MRKH)综合征,也称为苗勒管发育不全,是一种先天性疾病,其特征为子宫和阴道上段发育不全。它影响具有正常女性核型和典型第二性征的女性。本病例报告的目的是强调MRKH综合征的多学科管理方法,重点是针对身体和心理挑战进行定制干预,以改善生殖前景。一名26岁已婚女性于2023年1月前往印度瓦尔达县萨万吉的阿查里亚·维诺巴·巴韦农村医院(AVBRH),主诉原发性闭经。体格检查发现阴道盲端和子宫发育不全,提示MRKH综合征。进一步评估,包括盆腔磁共振成像(MRI),证实了苗勒管异常和双侧卵巢异常。功能性阴道的缺失严重影响了患者的生活质量,导致性交困难以及与不孕相关的情绪困扰。AVBRH启动了由妇科医生和精神科医生参与的协作方法来应对这些挑战。患者接受了阴道成形术以创建新阴道,增强了性功能以及与配偶的亲密关系。然而,由于子宫发育不全,通过传统方式实现生育是不可能的。因此,探索了辅助生殖技术,特别是代孕。从患者体内获取正常、功能正常的卵巢用于代孕程序。这种综合管理策略体现了与MRKH综合征相关的挑战,并强调了定制干预和长期随访的重要性。该病例突出了协作护理对于改善MRKH综合征患者生活质量和生殖前景的重要性。

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