摘要
目的
分析睾丸女性化综合征的临床特点，探讨其诊断及处理原则。
方法
回顾性分析1999年1月至2006年6月浙江大学医学院附属妇产科医院收治的27例睾丸女性化综合征患者的临床资料。
结果
1．睾丸女性化综合征患者共27例，其中18例为完全型，9例为不完全型。
2．完全型均表现为女性第二性征，不完全型表现为不同程度的男性化。
3．所有睾丸女性化综合征患者的血睾酮值均高于正常女性最高限，且完全型(19.15±11.9)nmol／L明显高于不完全型(5.5±4.6)nmol／L，有统计学差异。
4．所有患者均行睾丸切除术，其中2例不完全型患者睾丸病理检查示睾丸支持细胞-间质细胞瘤。
结论
1．睾丸女性化综合征患者年幼时若外阴畸形应尽早行外阴整形术，术后定期复查。
2．所有患者均应行睾丸切除术，完全型可选择在青春期后切除，不完全型在青春期前切除较为安全。
3．婚前必要时可行阴道成形术。
Objective
To analyze the clinical characteristics and investigate the diagnosis and treatment principles of testicular feminization syndrome(TFS). Methods
27 cases of TFS admitted to The Affiliated Obstetrical and Gynecological Hospital of Zhejiang University from January of 1999 to June of 2006 were studied retrospectively through analyzing their related literature. Results
1. There were 27 TFS cases in all, in which 18 cases were complete type and 9 cases were incomplete type.
2. The phenotype was complete feminization in complete TFS patients, and more or less virilization in incomplete TFS patients.
3. Serous testosterone(TTE) in TFS patients was higher than that in the normal women, and TTE in complete patients (19.15 nmol/L±11.9nmol/L) was significantly higher than incomplete ones(5. 5nmol/L±4.6nmol/L).
4. All patients underwent testectomy, and two of the incomplete patients had testicle sertoli-leydig cell tumors.
Conclusions
1. TFS patients who have genital malformations should undergo vulvoplasty in the shortest time when young.
2. Because of the possibility of carcinogenesis with the ectopic gonads in TFS, gonads should be removed for all patients. But the time of testectomy is controversial. In general, gonads should be removed after puberty in patients with complete TFS, while in patients with incomplete TFS, gonads should be removed before puberty.
3. Vaginoplasty should be taken before wedding if necessary.
引文
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