138例两性畸形的临床诊治分析

Diagnosis and treatment of 138 hermaphroditism cases

目的提高两性畸形的临床诊治水平。方法对138例两性畸形患者的诊断及治疗方法进行总结并结合相关文献分析。结果住院患者138例(年龄2～36岁,平均17岁)社会性别男性42例,女性96例。入院后经体格检查,完善实验室、超声、放射及病理活检等相关检查明确诊断。138例中女性假两性畸形54例,男性假两性畸形66例,真两性畸形10例,纯性腺发育不全2例,无睾丸综合征2例,先天性睾丸发育不全症4例。132例行手术治疗,76例行腹腔镜探查术;术后121例保留女性性别,11例保留男性性别。女性假两性畸形皆保留女性性别;男性假两性畸形59例保留女性性别,4例保留男性性别,3例未行手术治疗;真两性畸形中8例维持女性性别,2例保留男性性别。结论正确及时的性别确定是治疗的关键。病因诊断对治疗有一定指导意义,虽然两性畸形按女性抚养较为适宜,但仍应综合考虑患者及家属的意愿。

Objective To improve the diagnosis and treatment of hermaphroditism. Methods The data of 138 cases of hermaphroditism who were hospitalized in our department between March 2005 and April 2008 were reviewed and and analyzed to summarize our experience in the diagnosis and treatment. Results Among the 138 cases with a age ranging from 2 to 36 （ mean 17 years old）, 42 were socially male and 96 were socially female. With the help of medical examination,, laboratory examination, ultrasonic examination, radiological examination and pathologic diagnosis, 54 patients were diagnosed as female pseudohermaphroditism, 66 as male pseudohermaphroditism, 10 patients as true hermaphroditism, 2 patients as pure gonadal dysgenesis, 2 as testicle degeneration, and the left 4 patients as Klinefelter syndrome. Among the 138 patients, 132 patients were received surgery operation, including 76 of them receiving laparoscopy. After operation, 121 patients were maintained female sex,11 patients were maintained male sex. Female pseudohermaphroditism were all maintained female sex. Fifty-nine patients of male pseudoherInaphroditism were maintained female sex. There were 4 patients being maintained male sex, and 3 patients having not received operation. Of the cases of the true hermaphroditism, 8 patients were maintained female sex and 2 patients were maintained male sex. Conclusion Early accurate diagnosis is very important to the treatment of hermaphroditism. Etiologic diagnosis is useful in the course. Although it is good for the hermaphroditism to maintain female sex, the decision of the patients and the family should be considered.