先天性无阴道合并双子宫始基子宫1例

Congenital Vaginal Flaw Combined with Double Uterine Initiation Uterus: 1 Case

先天性无阴道无子宫综合征(Mayer-Rokitansky-Küster-Hauser, MRKH)是一种副肾管的抑制性畸形,主要表现为具有正常核型的女性先天性子宫、宫颈及阴道上2/3缺失(46, xx),有正常的卵巢及输卵管远端一部分组织。是苗勒管发育异常最常见的表现,临床表现为原发性闭经、腹痛和阴道性交功能不足等。其发病机制尚不明确,发病率较低,目前国内外发病率为1/4500~1/5000。现将我院收治的1例先天性无阴道合并双子宫始基子宫病例进行回顾性分析,结合相关文献报告进行总结,为今后对此类疾病的诊断治疗提供帮助,提高该类疾病患者的幸福指数。

Mayer-Rokitansky-Küster-Hauser (MRKH) is an inhibitory malformation of the accessory renal duct, mainly manifested as congenital 2/3 deletion of the uterus, cervix, and vagina (46, xx) in women with normal karyotypes, with normal ovarian and distal fallopian tube tissues. It is the most com-mon manifestation of Müllerian duct dysplasia, with clinical manifestations such as primary amen-orrhea, abdominal pain, and insufficiency of vaginal intercourse. Its pathogenesis is not clear, the incidence is low, the current incidence at home and abroad is 1/4500~1/5000. A case of congenital vagina flaw combined with double uterine initiation uterus admitted to our hospital is retrospec-tively analyzed, and summarized in combination with relevant literature reports, so as to provide assistance for the diagnosis and treatment of such diseases in the future and improve the happiness index of patients with such diseases.