真两性畸形2例报告

True herm aphrodites (Reportof 2 cases)

目的：总结对真两性畸形的诊治经验。方法：回顾性分析２ 例本病患者的临床资料。结果：对１ 例社会性别为女性，染色体为４６，ＸＸ，内分泌检查证明卵巢有功能者行阴茎海绵体及双侧隐睾切除，阴蒂阴道成形术，术后小剂量雌激素治疗１４ 个月。随访２ 年，有规则月经，第二性征向女性方向发展，内分泌功能基本正常。另１ 例社会性别为男性，染色体为４６，ＸＸ／４６，ＸＹ，男性性征明显，内分泌功能为男性者，行隐睾下降固定及Ⅱ期尿道成形术，并切除发育不全的输卵管及卵巢。术后生长发育与同龄男孩无差异，阴茎和睾丸大小及形态基本正常。结论：治疗本病应根据患者性腺功能情况和外生殖器矫形的可能性确定性别而行相应的外生殖器矫形术，切除与确定性别相抵触的性腺。

Purpose:To explore the diagnosis and treatm entofture herm aphrodites.Methods: Tw o cases w ith true herm aphroditism are described.Results:The first patient social sex fem ale, w ith of46,XXw as changed to a fem ale by rem oving ofundescended testes and fem inizing genito- plasty.Developm ent ofsecondary sexualcharacieristics in this patients tends to fem ale during the tw o years follow -up period.The second patientw ith socialsex m ale hasa karyotype of46,XX/46, XY.The fallopian tubes and ovaries w ere rem oved.Urethroplasty w as perform ed and Billateral cryptorchidism s w ere placed in the scrotum .Sex developm ent of the patients sim ilar to the boys w ith the sam e age.Conclusions:As far as the treatm entofture herm aphroditism is concerned,the externalgenitals should be changed according to the gonads and anatom ic findings.Contradictory gonadaland intem alductalstructures should be rem oved.Sex horm ons m ay be given follow ing the operation.