男性假两性畸形的诊断与治疗初探(附47例报告)

Clinical study of the diagnosis and treatment of male pseudohermaphroditism(reports of 47 cases)

目的提高对男性假两性畸形的诊断水平,总结治疗经验。方法回顾1982年至2005年间我院诊治的47名男性假两性畸形患者,其中最长随访时间23年,并结合文献对其病因、诊断、治疗进行讨论。结果47名患者临床确诊为男性假两性畸形,其中31名患者通过实验室、影像学等检查进一步明确病因学诊断。42名患者接受了手术治疗,24名术后性别为男性,18名为女性。24名男性患者中,第1次手术后恢复良好出院6名,第1次手术后恢复欠佳出院18名；在我院接受2次及2次以上手术共17例次。18名女性患者,均第1次手术后恢复良好出院,其中12名建议婚前再次手术成形。结论早期诊断对提高治疗效果意义重大,病因诊断对治疗有一定的指导意义,治疗应着重于性别选择和性腺处理两个方面,术后性别宜首选女性。

Objective To improve the accuracy of diagnosis and summarize the experience of treatment for male pseudohermaphroditism. Methods To review the 47 cases with the diagnosis of male pseudohermaphroditism from 1982 to 2005. The longest follow-up time was 17 years. Results Etiologic diagnosis had been made for 31 of the 47 patients with the help of laboratory examination, ultrasonic and radiological examination. 42 patients had received operations after which 24 of them went to their result as male and 18 as female. In man＇s cases, only 6 of 24 patients recover completely in hospital after the first operation. The male patients had received further operations 17 persontime in our hospital. While in female cases, all the 18 patients recovered successfully in hospital after the first operation, and 12 of them were recommended to receive plastic operation before the marriage. Conclusion Early accurate diagnosis is very important for the medical treatment and etiologic diagnosis is useful in this course. Emphasis should be placed on the sex choice and gonad treatment. Female is more advisable in the sex choice for the patients.