小儿两性畸形的外科干预

目的探讨两性畸形治疗中外科干预的相关问题。方法回顾1993年1月～2006年6月收治的26例两性畸形患者的病史及随访资料。26例中女性假两性畸形(FPH)4例,真两性畸形(TH)5例,男性假两性畸形者(MPH)16例,性腺发育不良伴苗勒氏管持续综合征1例。护养性别女性18例,男性8例。根据不同类型的两性畸形,26例患儿分别进行外科手术纠正外生殖器畸形。结果4例FPH患儿外生殖器保持女性外观,患儿与家长均满意;TH和MPH患者中术后男性者,均认可自己的性别,已完成外生殖器整形患儿的睾丸或阴茎不同程度小于同龄儿;2例MPH术后女性者患儿乳房发育较差。1例性腺发育不良伴苗勒氏管持续综合征患儿性别改为男性,随访发现左侧性腺为精原细胞瘤,行放疗及手术治疗。随访发现女性患儿的性格男性化,部分男性患儿的性格女性化。结论对于两性畸形患者的性别选择要综合考虑各方面因素,而外科干预必须在护养性别确定后进行,且应根据不同类型的畸形选择适宜的手术方式。

Surgical treatment of hermaphroditism in children

Objective To explore the related issues of surgical treatment of hermaphroditism in children. Methods From January 1993 to June 2006,the medical records and data of follow-up of 26 patients with hermaphroditism were retrospectively analysed.Among the 26 patients,4 were female pseudohermaphroditism(FPH), 5 true pseudohermaphroditism(TH),16 male pseudohermaphroditism(MPH)and the other one partial gonadal dysgenesis with persistent Mullerian duct syndrome.Eighteen patients were reared as female and 8 male.These 26 patients were performed surgeries of external genital malformation correction respectively according to the types of pseudohermaphroditism.Results Four patients of FPH maintained the appearance of female external genitalia,and both the patients and parents were satisfied with the results.All those maintained the male sex in TH and MPH well accepted their gender,while the testiculus and penis of those with external genital reconstruction were smaller than the counterparts.The mammoplasia of the two female patients after operation in MPH was less satisfactory.The patient with partial gonadal dysgenesis with persistent Mullerian duct syndrome was changed to male gender,and was found spermocytoma in the left genitical gland.Radiotherapy and operation were performed later.It was observed in the follow-up that those female patients tended to have the male characters,while the opposite was found in some of those male patients.Conclusion It is important to have a comprehensive consideration of the gender selection for those with hermaphroditism.The surgical intervention shall be performed after gender determination,and a proper surgical treatment may be based on the type of hermaphroditism.