《颅脑先天畸形》PPT课件.ppt

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1、offline browser(Unregistered)on 09/13/07 10:15:08.Address:Title:PowerPoint Presentation Size:3145 Last Modified:Thu,15 Jun 2006 02:07:46 GMT,第九节 颅脑先天畸形及发育异常,offline browser(Unregistered)on 09/13/07 10:15:08.Address:Title:PowerPoint Presentation Size:6165 Last Modified:Thu,15 Jun 2006 02:07:48 GMT,学习

2、要点,本节应了解、熟悉和掌握的知识点了解颅脑先天发育异常的分类 熟悉常见颅脑发育异常的影像学表现 神经皮肤综合征的影像学表现,offline browser(Unregistered)on 09/13/07 10:15:08.Address:Title:PowerPoint Presentation Size:5554 Last Modified:Thu,15 Jun 2006 02:07:48 GMT,学习难点,本节学习中的难点脑先天性发育异常的分类和影像学表现,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:P

3、owerPoint Presentation Size:3628 Last Modified:Thu,15 Jun 2006 02:07:58 GMT,颅脑先天性畸形分类,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:2851 Last Modified:Thu,15 Jun 2006 02:07:48 GMT,一、头颅先天性畸形,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title

4、:PowerPoint Presentation Size:4811 Last Modified:Thu,15 Jun 2006 02:07:48 GMT,狭颅症（craniostenosis）,颅缝提早骨化、闭合，致头颅畸形，包括舟状 头畸形、短头畸形、尖头畸形、偏头畸形、小 头畸形等,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:5317 Last Modified:Thu,15 Jun 2006 02:07:50 GMT,矢状缝及顶颞缝提早闭合，头颅

5、左右径生长受限，前后径生长显著 舟状头变形,舟状头变形,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:5582 Last Modified:Thu,15 Jun 2006 02:07:56 GMT,颅底陷入,颅底陷入枕骨大孔周围骨质，枕骨基底部、髁部、鳞部向颅腔内陷入扁平颅底前、中、后颅窝失去正常的阶梯状关系,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint

6、 Presentation Size:9979 Last Modified:Thu,15 Jun 2006 02:07:56 GMT,测量标准,Chamberlain：硬腭后缘与枕骨大孔后缘连线，齿状突高于此线3mm为异常McGregor：硬腭后缘与枕骨最低点连线，齿状突高于此线6mm为异常,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:2855 Last Modified:Thu,15 Jun 2006 02:07:58 GMT,二、脑先天性发育异常,先

7、天性脑积水,先天性脑积水：又称积水性无脑畸形病因：病因不明，可能与胚胎期颈内动脉发育 不良、母体感染、放射损伤有关临床与病理：头颅迅速增大、面部比例失调，智力低下，多在一岁内死亡,影像学表现,X线平片：头颅呈球形增大，囟门大、颅缝宽、颅壁薄，颅面骨比例失调CT表现：额、顶、颞叶脑实质几乎消失，部分枕叶、基底神经核、丘脑保存，小脑、脑干、四脑室一般正常MRI表现：幕上为水样信号，大脑镰、基底节、小脑及脑干结构正常,先天性脑积水,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentati

8、on Size:6666 Last Modified:Thu,15 Jun 2006 02:08:08 GMT,又称 丹-瓦氏畸形 Dandy-Walker malformation 小脑半球及蚓部发育不良、四脑室中孔和侧孔闭 锁致四脑室扩张和梗阻性脑积水,四脑室中、侧孔先天性闭塞,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:6644 Last Modified:Thu,15 Jun 2006 02:08:10 GMT,后颅凹扩大；巨大囊肿；小脑下蚓缺如

9、，上蚓移位；小脑半球发育不良；脑干受压，幕上脑积水鉴别：蛛网膜囊肿、大枕大池,影像学表现,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:4915 Last Modified:Thu,15 Jun 2006 02:08:10 GMT,Dandy-Walker畸形(图),offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:852

10、9 Last Modified:Thu,15 Jun 2006 02:08:12 GMT,概述：前脑无裂畸形、无脑回、多小脑回、灰质异 位、脑裂畸形及脑沟回移位等，胚胎26月 病因不明：血管性，感染性，化学性,脑裂、脑沟和脑回发育畸形,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:9369 Last Modified:Thu,15 Jun 2006 02:08:12 GMT,无脑回畸形（agyria）,无脑回畸形：多见顶枕叶CT、MR表现:脑表面光滑，脑回

11、、沟缺如 脑室扩大,侧裂池、蛛网膜 下腔增宽,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:4412 Last Modified:Thu,15 Jun 2006 02:08:12 GMT,无脑回畸形（图）,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:9206 Last Modified:Thu,15 Jun 2006

12、 02:08:20 GMT,病理：融合型、非融合型 临床：难治性癫痫，运动障碍，智力低下，发 育迟缓 影像学表现：中央前、后回附近表面延伸至室管膜下 裂隙（P-E缝）周围皮质不规则增厚,脑裂畸形（schizencephaly）,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:4677 Last Modified:Thu,15 Jun 2006 02:08:22 GMT,开放型脑裂畸形(图),offline browser(Unregistered)on 09

13、/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:9096 Last Modified:Thu,15 Jun 2006 02:08:24 GMT,病因：不清，可能与染色体异常及基因突变有关 病理：无脑叶、半脑叶、单脑叶型 影像学表现：无脑叶 单脑室，少量脑组织，无中线结构并发 颅面畸形 半脑叶 三脑室中线结构缺如，或有较小大脑镰，纵裂浅 单脑叶 透明隔缺如，半球间裂浅,前脑无裂畸形（holoprosencephaly）,offline browser(Unregistered)on 09/13/07 10:15:09.Addr

14、ess:Title:PowerPoint Presentation Size:4410 Last Modified:Thu,15 Jun 2006 02:08:26 GMT,前脑无裂畸形（图）,offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:5290 Last Modified:Thu,15 Jun 2006 02:08:26 GMT,前脑无裂畸形（图）,offline browser(Unregistered)on 09/13/07 10:15:10.Ad

15、dress:Title:PowerPoint Presentation Size:3840 Last Modified:Thu,15 Jun 2006 02:08:28 GMT,前脑无裂畸形（图）,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:10453 Last Modified:Thu,15 Jun 2006 02:07:58 GMT,脑膜膨出和脑膜脑膨出,颅内结构经颅骨缺损疝出颅外病理 显性颅裂畸形型 脑膜膨出型 脑膜脑膨出 型 脑室膨出型 囊性脑膜

16、脑膨出临床颅盖组 膨出软组织肿块颅底组 眶、鼻、咽肿块,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:5654 Last Modified:Thu,15 Jun 2006 02:08:00 GMT,影像学表现,CT：颅骨缺损，脑脊液密度膨出疝囊；软组织密 度疝囊MR：脑脊液信号膨出物、脑实质信号膨出物，颅 内脑室牵拉、变形、扩张,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:

17、PowerPoint Presentation Size:4407 Last Modified:Thu,15 Jun 2006 02:08:04 GMT,枕部脑膜脑膨出（图）,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:9793 Last Modified:Thu,15 Jun 2006 02:08:04 GMT,病因：先天性，后天性 病理：胚胎早期损伤严重，致完全缺如；胎晚期损 伤轻，致部分缺如 临床：无症状，轻者视觉、交叉触觉障碍，重者智 力低下、癫

18、痫,胼胝体发育异常（dysgenesis of the corpus callosum）,offline browser(Unregistered)on 09/13/07 10:15:09.Address:Title:PowerPoint Presentation Size:9292 Last Modified:Thu,15 Jun 2006 02:08:06 GMT,部分缺如：压部常见，压部带状结构消失，体变薄、中断完全缺如：侧脑室扩大、分离，前角小、后角大，三脑室上升，介侧脑室间，顶紧邻纵裂 池,影像学表现,offline browser(Unregistered)on 09/13/07

19、10:15:09.Address:Title:PowerPoint Presentation Size:4678 Last Modified:Thu,15 Jun 2006 02:08:06 GMT,胼胝体完全性缺如(图）,offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:9096 Last Modified:Thu,15 Jun 2006 02:08:34 GMT,脑脊液在脑外异常的局限性积聚 病因：先天性 先天发育 后天性 外伤、感染、手术等 病理：蛛网膜

20、内囊肿，多不与蛛网膜下腔部交通 蛛网膜下囊肿，多与蛛网膜下腔部相通 相邻颅骨及脑组织呈受压改变 临床：部分无症状，部分有轻瘫、癫痫发作等,蛛网膜囊肿（arachnoid cyst）,offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:8311 Last Modified:Thu,15 Jun 2006 02:08:34 GMT,影像表现：CT、MRI 边缘光滑、脑脊液密度/信号区，无强化，占位轻，颅骨变薄 位 置 外侧裂池、大脑凸面、枕大池多见 鉴别诊断：胆脂瘤

21、，大枕大池，脑穿通畸形囊肿,影像学标表现及鉴别诊断,offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:5420 Last Modified:Thu,15 Jun 2006 02:08:38 GMT,蛛网膜囊肿(图),offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:3580 Last Modified:Thu,15 Jun 2

22、006 02:08:42 GMT,三、神经皮肤综合征（neurocutaneous syndromes）,offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:6509 Last Modified:Thu,15 Jun 2006 02:08:42 GMT,一组遗传性斑痣性错构瘤病分两型：神经纤维瘤病型（NF-l）神经纤维瘤病型（NF-2）,神经纤维瘤病（neurofibromatosis）,offline browser(Unregistered)on 09/13

23、/07 10:15:10.Address:Title:PowerPoint Presentation Size:9235 Last Modified:Thu,15 Jun 2006 02:08:46 GMT,NF-1型 NF-2型 CNS病变 颅内神经肿瘤胶质瘤 单、多发脑脊膜瘤丛状神经纤维瘤 脊神经多发神经鞘瘤其它 髓内室管膜瘤、星形细胞瘤,影像学表现,offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:4676 Last Modified:Thu,15 Ju

24、n 2006 02:08:48 GMT,神经纤维瘤病-II型（图）,offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:9739 Last Modified:Thu,15 Jun 2006 02:08:50 GMT,病因：常染色体显性遗传，外显率高 临床：皮脂腺瘤、癫痫、智力低下,结节性硬化（tuberous sclerosis）,offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:Po

25、werPoint Presentation Size:6109 Last Modified:Thu,15 Jun 2006 02:08:50 GMT,灰、白质、室管膜下，异常结节信号、密度区、可钙化室间孔室管膜下巨细胞星形细胞瘤,影像学表现,offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:5156 Last Modified:Thu,15 Jun 2006 02:08:50 GMT,结节性硬化（图）,offline browser(Unregistered)

26、on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:3879 Last Modified:Thu,15 Jun 2006 02:08:52 GMT,结节性硬化（图）,offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:8058 Last Modified:Thu,15 Jun 2006 02:08:54 GMT,病理：软脑膜毛细血管-静脉畸形,颜面三叉 神经分布区血管瘤,眼脉络膜血管畸形临床:面三叉神经分布区血管瘤、癫痫、智力低下,脑颜面血管瘤病（Sturge-Weber syndrome）,offline browser(Unregistered)on 09/13/07 10:15:10.Address:Title:PowerPoint Presentation Size:6149 Last Modified:Thu,15 Jun 2006 02:08:56 GMT,顶枕叶脑回样、轨道状钙化；脑萎缩；脉络丛、引流静脉明显且强化,影像学表现,