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 syndrome (MRKH syndrome) manifested as Müllerian ductal hypoplasia (2/3 of the uterus and vagina hypoplasia), chromosomal karyotype 46, XX, with normal female subjects Sexual signs, due to adolescent primary amenorrhea was clinically diagnosed. The cause of MRKH syndrome is still not clear, the clinical classification has not been uniform, and androgen insensitivity syndrome, vaginal atresia and other diseases identified. The main treatment for vaginoplasty, peritoneal vaginoplasty (Lo Wu operation), sigmoid colon vaginoplasty as the mainstream surgery.