摘要
目的探讨雄激素不敏感综合征的 MRI 表现及临床特征。资料与方法回顾性分析经腹腔镜手术和临床综合诊断的 10 例雄激素不敏感综合征患者的 MRI 特征和临床资料。结果 10 例患者均经腹腔镜手术和染色体核型检查,并结合临床最终诊断为雄激素不敏感综合征,且均为完全型。10 例均为社会女性表现,染色体核型均为 46,XY。MRI 表现为双侧腹股沟区椭圆形、条索状、结节状异常信号影,边界清,T1WI 呈等信号,T2WI 及 T2WI 压脂序列呈稍低及稍高信号,DWI 序列扩散受限。患者乳房发育正常,无月经来潮,术中均未见子宫及附件。性腺位于盆腔、腹股沟区。所有患者均行腹腔镜下睾丸切除术,其中 2 例同时行腹股沟疝修补术。术后性腺病理结果为睾丸组织。结论雄激素不敏感综合征的临床诊断需结合症状、体征、性激素水平、影像学资料及染色体核型等综合考虑。MRI 可以更好地显示性腺及内生殖器的位置及发育情况,帮助临床评估疾病类型。
Purpose To investigate the MRI manifestations and clinical features of androgen insensitivity syndrome. Materials and Methods The MRI features and clinical data of 10 patients with androgen insensitivity syndrome undergoing laparoscopic surgery and clinical comprehensive diagnosis were retrospectively analyzed. Results All the 10 patients were diagnosed with androgen insensitivity syndrome by laparoscopic surgery, chromosome karyotype examination and clinical diagnosis, and all of them were of complete type. All the patients had social female manifestations with chromosome karyotypes of 46, XY. MRI showed bilateral inguinal region oval, cord-shaped and nodular abnormal signal shadows, with clear boundaries, equisignals on T1WI, slightly lower and slightly higher signals on T2WI and T2WI, and restricted diffusion on DWI sequence. With normal breast development, the patients had no menses, and no uterus and accessories were found during the operation. Gonads were located in the pelvic and inguinal regions. All patients underwent laparoscopic orchiectomy, and inguinal herniorrhaphy was performed in 2 patients. Postoperative gonadal pathology revealed testicular tissue. Conclusion The clinical diagnosis of androgen insensitivity syndrome should be considered comprehensively in combination with symptoms and signs, sex hormone level, imaging data and chromosome karyotype. MRI can better display the location and development of gonads and internal genitalia, and help clinical evaluation of the disease type.
作者
徐生芳
钱吉芳
张金龙
岳松虹
杨磊
朱大林
杨来虎
XU Shengfang;QIAN Jifang;ZHANG Jinlong;YUE Songhong;YANG Lei;ZHU Dalin;YANG Laihu(The 940th Hospital of the PLA Logistics Support Force,Lanzhou 730050,China)
出处
《中国医学影像学杂志》
CSCD
北大核心
2019年第6期460-463,共4页
Chinese Journal of Medical Imaging
基金
兰州市科技计划项目(2018-3-53)
关键词
雄激素迟钝综合征
磁共振成像
诊断
鉴别
Androgen-insensitivity syndrome
Magnetic resonance imaging
Diagnosis,differential
作者简介
通讯作者:张金龙,395394991@qq.com.
