杨朝湘,刘倩,冯长征,韩鹏慧,陈文俊,欧娟婷.胎儿Joubert综合征及相关疾病MRI表现[J].中国医学影像技术,2018,34(2):275~278
胎儿Joubert综合征及相关疾病MRI表现
MRI manifestations of fetal Joubert syndrome and related disorders
投稿时间:2017-10-09  修订日期:2018-01-06
DOI:10.13929/j.1003-3289.201710015
中文关键词:  胎儿  磁共振成像  神经系统  先天性畸形
英文关键词:Fetus  Magnetic resonance imaging  Nervous system  Congenital abnormalities
基金项目:
作者单位E-mail
杨朝湘 广东省妇幼保健院放射科, 广东 广州 510010 yangchaoxiang@sina.com 
刘倩 广东省妇幼保健院产前诊断科, 广东 广州 510010  
冯长征 广东省妇幼保健院放射科, 广东 广州 510010  
韩鹏慧 广东省妇幼保健院放射科, 广东 广州 510010  
陈文俊 广东省妇幼保健院放射科, 广东 广州 510010  
欧娟婷 广东省妇幼保健院放射科, 广东 广州 510010  
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中文摘要:
      目的 探讨产前MRI诊断胎儿Joubert综合征及相关疾病(JSRD)的价值。方法 回顾性分析5胎JSRD胎儿产前MRI资料,并于MRI中脑轴位层面分别测量冠根比、峡池比及第四脑室顶部左右径和前后径比。结果 5胎产前MRI中脑轴位层面均可见"臼齿"征(MTS),小脑蚓部缺失,表现为"狭缝"征;其中3胎伴枕大池增宽。5胎冠根比为0.58~0.90,峡池比0.84~1.00,第四脑室顶部横径和前后径比0.42~0.60。结论 "臼齿征"和"狭缝"征为JSRD特征性MRI表现,有助于产前诊断胎儿JSRD。
英文摘要:
      Objective To explore the diagnostic value of prenatal MRI in fetal Joubert syndrome and related disorders(JSRD). Methods Prenatal MRI data of 5 JSRD fetuses were analyzed retrospectively. The ratio of AP diameters of crown to root of molar tooth sign, the ratio of AP diameters of isthmus to interpeduncular fossa, and the ratio of the transverse to AP diameters of the roof of the fourth ventricle on the axial images of the midbrain were measured. Results Molar tooth signs were observed in 5 fetuses on the axial images of midbrain. The cerebellar vermis disappeared, and cleft sign was observed. Enlarged cisterna magna was observed in 3 fetuses. The ratios of AP diameters of crown to root were 0.58-0.90, of AP diameters of isthmus to interpeduncular fossa were 0.84-1.00, and of transverse to AP diameters of the fourth ventricle roof were 0.42-0.60. Conclusion Molar tooth sign and cleft sign were characteristic findings for JSRD on MRI, therefore being helpful to prenatal diagnosis of JSRD.
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