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目的探讨持久苗勒管综合征的诊断、治疗和预后。方法报道2例持久苗勒管综合征的临床诊治资料,结合文献复习讨论该病的病因、临床表现、诊断、治疗及预后。结果2例患者随访半年,患者睾丸血流和性激素测定均正常。结论苗勒管综合征为一种罕见的男性假两性畸型,其特征为具有睾丸发育、表型正常的男性体内存在一组无功能的子宫及输卵管。治疗应力求保护睾丸的血供和生殖功能并定期随访。
Objective To investigate the diagnosis, treatment and prognosis of persistent Mullerian syndrome. Methods The clinical data of 2 cases of persistent Mullerian duct syndrome were reported. The etiology, clinical manifestations, diagnosis, treatment and prognosis of this disease were reviewed and discussed in the literature review. Results Two patients were followed up for six months, testicular blood flow and sex hormone were normal. Conclusions Müllerian duct syndrome is a rare male pseudohermaphroditism characterized by a group of nonfunctional uterus and fallopian tubes in men with normal testicular development and normal phenotype. Treatment should seek to protect the testicular blood supply and reproductive function and regular follow-up.