先天性无阴道综合征临床研究进展

Mayer-Rokitansky-Küster-Hauser Syndrome: Recent Clinical Findings

先天性无阴道综合征(Mayer-Rokitansky-Küster-Hauser syndrome,MRKH综合征)表现为苗勒管发育不全(子宫和阴道上2/3发育不全),染色体核型46,XX,有正常女性第二性征,因青春期原发性闭经被临床诊断。MRKH综合征病因仍不明确,临床分类尚未统一,应与雄激素不敏感综合征、阴道闭锁等疾病鉴别。治疗方法主要为阴道成形术,以腹膜阴道成形术(罗湖术式)、乙状结肠阴道成形术等为主流手术。

Mayer-Rokitansky-Küster-Hauser（MRKH） syndrome is characterized by Müllerian duct aplasia（uterus plus upper two-thirds of the vagina） in an XX individual with female phenotype which is clinical diagnosed by primary amenorrhea at adolescence.The etiology of MRKH syndrome still remains unclear,clinical classification is not yet uniform.It should be distinguished from androgen insensitivity syndrome and atresia of vagina and other diseases.Treatment is mainly for the vaginoplasty.The most widely used remedies are vaginoplasty with peritoneum（LuoHu-treatment） or sigmoid.