Bombard David S, Mousa Shaker A
Pharmaceutical Research Institute, Albany College of Pharmacy and Health Sciences , Rensselaer, NY , USA.
Gynecol Endocrinol. 2014 Sep;30(9):618-23. doi: 10.3109/09513590.2014.927855. Epub 2014 Jun 20.
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a disorder in women that presents as Mullerian agenesis. These patients present internal genitalia abnormalities that include the absence of a uterus and the upper two-thirds of the vagina. In this review, current diagnostic methods, accompanying complications (congenital and psychological) and non-surgical and surgical treatments are summarized. Ultrasound and MRI have been the most documented options in MRKH syndrome diagnosis. Many women with MRKH syndrome have renal, skeletal, hearing or cardiac congenital anomalies and increased levels of psychological distress. Non-surgical interventions can be used to create a sexually functional neovagina through vaginal dilation, and surgical interventions provide alternate methods of creating a neovagina. Additionally, vaginal tissue engineering and gene therapy might provide more effective approaches in solving MRKH syndrome.
迈耶-罗基坦斯基-库斯特-豪泽（MRKH）综合征是一种女性疾病，表现为苗勒氏管发育不全。这些患者存在内生殖器异常，包括子宫缺如和阴道上三分之二缺如。在本综述中，总结了当前的诊断方法、伴随的并发症（先天性和心理性）以及非手术和手术治疗方法。超声和磁共振成像（MRI）是MRKH综合征诊断中记录最多的选择。许多患有MRKH综合征的女性有肾脏、骨骼、听力或心脏先天性异常，且心理困扰水平升高。非手术干预可通过阴道扩张来创建具有性功能的新阴道，手术干预则提供了创建新阴道的替代方法。此外，阴道组织工程和基因治疗可能为解决MRKH综合征提供更有效的方法。
