颅脑先天畸形影像诊断培训课件

1、颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:08.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0505.htmTitle: PowerPoint Presentation Size: 6165 Last Modified: Thu, 15 Jun 2006 02:07:48 GMT学习要点本节应了解、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2颅脑先天畸形影像诊断WebZIP 03

2、0 offline browser (Unregistered) on 09/13/07 10:15:08.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0506.htmTitle: 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 3颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:0

3、9.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0519.htmTitle: PowerPoint Presentation Size: 3628 Last Modified: Thu, 15 Jun 2006 02:07:58 GMT颅脑先天性畸形分类4颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0508.htmTitle: PowerP

4、oint Presentation Size: 2851 Last Modified: Thu, 15 Jun 2006 02:07:48 GMT一、头颅先天性畸形5颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0509.htmTitle: PowerPoint Presentation Size: 4811 Last Modified: Thu, 15 Jun 2006 02:07:48 GMT

5、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6颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0510.htmTitle: 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，头颅左

6、右径生长受限，前后径生长显著 舟状头变形舟状头变形7颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0514.htmTitle: 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8颅

7、脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0515.htmTitle: PowerPoint 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9颅脑

8、先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0518.htmTitle: PowerPoint Presentation Size: 2855 Last Modified: Thu, 15 Jun 2006 02:07:58 GMT二、脑先天性发育异常10颅脑先天畸形影像诊断先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：

9、头颅迅速增大、面部比例失调， 智力低下，多在一岁内死亡11颅脑先天畸形影像诊断影像学表现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12颅脑先天畸形影像诊断先天性脑积水13颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide05

10、33.htmTitle: PowerPoint Presentation 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14颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/sli

11、de0535.htmTitle: 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，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15颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0536.htmTitle: Powe

12、rPoint Presentation Size: 4915 Last Modified: Thu, 15 Jun 2006 02:08:10 GMTDandy-Walker畸形(图)16颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0538.htmTitle: PowerPoint Presentation Size: 8529 Last Modified: Thu, 15 Jun 2006 0

13、2:08:12 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17颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0539.htmTitle: PowerPoint Presentation Size: 9369 Last Modified: Thu, 15 Jun 2006 02:08:12 G

14、MT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、沟缺如 脑室扩大, 侧裂池、蛛网膜 下腔增宽18颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0541.htmTitle: PowerPoint Presentation Size: 4412 Last Modified: Thu, 15 Jun 2006 02:08:12 GMT无脑回畸形（图）19颅脑先天畸

15、形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0551.htmTitle: PowerPoint Presentation Size: 9206 Last Modified: Thu, 15 Jun 2006 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（s

16、chizencephaly）20颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0555.htmTitle: PowerPoint Presentation Size: 4677 Last Modified: Thu, 15 Jun 2006 02:08:22 GMT开放型脑裂畸形 (图)21颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on

17、 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0558.htmTitle: 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（holoprosencepha

18、ly）22颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0560.htmTitle: PowerPoint Presentation Size: 4410 Last Modified: Thu, 15 Jun 2006 02:08:26 GMT前脑无裂畸形（图）23颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:

19、15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0561.htmTitle: PowerPoint Presentation Size: 5290 Last Modified: Thu, 15 Jun 2006 02:08:26 GMT前脑无裂畸形（图）24颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0562.htmTitle: P

20、owerPoint Presentation Size: 3840 Last Modified: Thu, 15 Jun 2006 02:08:28 GMT前脑无裂畸形（图）25颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0521.htmTitle: PowerPoint Presentation Size: 10453 Last Modified: Thu, 15 Jun 2006 02:07

21、: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 囊性脑膜脑膨出临床颅盖组 膨出软组织肿块颅底组 眶、鼻、咽肿块26颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0522.htmTitle: PowerPoint Presentation Size: 5654 Last Modified: Thu, 15 Jun 2006 0

22、2:08:00 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27颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0526.htmTitle: PowerPoint Presentation Size: 4407 Last Modified: Thu, 15 Jun 2006 02:08:04 GMT枕部脑膜脑

23、膨出（图）28颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0527.htmTitle: 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、癫痫胼胝体

24、发育异常（ dysgenesis of the corpus callosum ）29颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0528.htmTitle: 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，

25、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30颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0569.htmTitle: 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

26、膜内囊肿，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）32颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0570.htmTitle: PowerPoint Presentation Size: 8311 Last Modified: Thu, 15 Jun 2006 02

27、: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，大枕大池，脑穿通畸形囊肿影像学标表现及鉴别诊断33颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0574.htmTitle: PowerPoint Presentation Size: 5420 Last Modified: Thu, 1

28、5 Jun 2006 02:08:38 GMT蛛网膜囊肿(图)34颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0578.htmTitle: PowerPoint Presentation Size: 3580 Last Modified: Thu, 15 Jun 2006 02:08:42 GMT三、神经皮肤综合征（neurocutaneous syndromes）35颅脑先天畸形影像诊断Web

29、ZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0580.htmTitle: 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）36颅脑先天畸形影像诊断WebZIP 030 offlin

30、e browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0584.htmTitle: 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37颅脑先天畸形影像诊断WebZIP 030 offline brows

31、er (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0589.htmTitle: PowerPoint Presentation Size: 4676 Last Modified: Thu, 15 Jun 2006 02:08:48 GMT神经纤维瘤病-II型（图）38颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yx

32、x/doc/2-9.files/slide0590.htmTitle: 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）39颅脑先天畸形影像诊断WebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0591.htmTitle: PowerPoint Presentation Size: 6109 Last Modified: Thu, 15 Jun 2006 02:08:50 G