表现为睾丸横过异位的苗勒管综合征二例报告

Persistent müllerian duct syndrome with transverse testicular ectopia (report of 2 cases)

目的 探讨苗勒管保留综合征的诊断、处理和预后。 方法 报告 2例睾丸横过异位型苗勒管综合征 ,分别伴无精子症和睾丸精原细胞瘤。行子宫体大部切除 ,睾丸精索松解固定及睾丸肿瘤切除。 结果 2例随访 2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遗传性男性假两性畸型 ,治疗应力求保护睾丸的血供和生殖功能并密切随访。

Objective To study the diagnosis,management and prognosis of persistent müllerian duct syndrome (PMDS). Methods 2 cases of PMDS associated with transverse testicular ectopia were reported.Clinically there was azoospermia or testis tumor.Corporeal hysterectomy and orchidopexy were performed and the testis tumor was excised. Results Vascular supply and hormonal function of the testis were normal in both 2 patients after 2 year's follow up. Conclusions PMDS is an inherited male pseudohermaphroditism. Every effort should be made to preserve the testis and its fertility function when surgical management is needed.The possible development of testis tumor should be kept in mind and closely observed.