颅脑先天畸形影像诊断.ppt

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1、WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:08.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0504.htmTitle: 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 WebZIP 7.0.3.1030 offline browser (Unregistered) on

2、09/13/07 10:15:08.Address: http:/202.113.53.133/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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/1

3、3/07 10:15:08.Address: http:/202.113.53.133/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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.5

4、3.133/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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0508.htmTitle:

5、 PowerPoint Presentation Size: 2851 Last Modified: Thu, 15 Jun 2006 02:07:48 GMT一、头颅先天性畸形 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0509.htmTitle: PowerPoint Presentation Size: 4811 Last Modified: Thu, 15

6、 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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0510.htmTitle: PowerPoint Presentation Size: 5317 Last Modified: Thu, 15

7、 Jun 2006 02:07:50 GMT矢 状 缝 及 顶 颞 缝 提 早 闭 合 ， 头 颅 左 右 径 生 长受 限 ， 前 后 径 生 长 显 著 舟 状 头 变 形舟状头变形 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0514.htmTitle: PowerPoint Presentation Size: 5582 Last Modified: Thu

8、, 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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0515.htmTitle: PowerPoint Presentation Size: 9979 Last Modified: Thu, 15 Jun 200

9、6 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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0518.htmTitle: PowerPoint Presentation Size: 2855 Last Modified:

10、 Thu, 15 Jun 2006 02:07:58 GMT二、脑先天性发育异常 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：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 WebZIP 7.0.3.1030 offline browser (Unregi

11、stered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0533.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 WebZIP 7.0.3.1030 offli

12、ne browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0535.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 WebZIP 7.0.3.1030 offline brow

13、ser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0536.htmTitle: PowerPoint Presentation Size: 4915 Last Modified: Thu, 15 Jun 2006 02:08:10 GMTDandy-Walker畸形(图) WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: h

14、ttp:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0538.htmTitle: PowerPoint Presentation Size: 8529 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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Addre

15、ss: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0539.htmTitle: 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、沟缺如 脑室扩大, 侧裂池、蛛网膜 下腔增宽 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address

16、: http:/202.113.53.133/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） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/sli

17、de0551.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（schizencephaly） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/j

18、pk/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) WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0558.htmTitle: Powe

19、rPoint 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） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:

20、/202.113.53.133/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） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0561

21、.htmTitle: PowerPoint Presentation Size: 5290 Last Modified: Thu, 15 Jun 2006 02:08:26 GMT前脑无裂畸形（图） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0562.htmTitle: PowerPoint Presentation Size: 3840 Last Modifie

22、d: Thu, 15 Jun 2006 02:08:28 GMT前脑无裂畸形（图） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0521.htmTitle: 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

23、 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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0522.htmTitle: PowerPoint Presentation Size: 5654 Last Modified: Thu, 15 Jun 2006 02:08:00 GMT影像学表现C

24、T：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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0526.htmTitle: PowerPoint Presentation Size: 4407 Last Modified: Thu, 15 Jun 2006 02:08:04 GMT枕部脑膜脑膨出（

25、图） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/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

26、智 力低下、癫痫胼胝体发育异常（ dysgenesis of the corpus callosum ） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/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

27、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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0529.htmTitle: PowerPoint Presentation Size: 4678 Last Modified: Thu, 15 Jun 2006 02:08:06 GMT胼胝体完全性缺如(图

28、） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/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： 蛛网膜内囊肿，多不与蛛网膜下腔部交通

29、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） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0570.htmTitle: PowerPoint Presentation Size: 8311 Last Modified: Thu, 15 Jun 2006 02:08

30、: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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0574.htmTitle: PowerPoint Presentation Size: 5420 Last Modif

31、ied: Thu, 15 Jun 2006 02:08:38 GMT蛛网膜囊肿(图) WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/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

32、syndromes） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/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（神经纤维瘤病型（神经纤维瘤病（neurofibromatosis）

33、 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/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

34、胞瘤影像学表现 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/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） WebZIP 7.0.3.1030 offline browser (Unregistere

35、d) on 09/13/07 10:15:10.Address: http:/202.113.53.133/jpk/contents/g/yxx/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） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/

36、13/07 10:15:10.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0591.htmTitle: PowerPoint 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 WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address:

37、 http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0592.htmTitle: PowerPoint Presentation Size: 5156 Last Modified: Thu, 15 Jun 2006 02:08:50 GMT结节性硬化（图） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slid

38、e0593.htmTitle: PowerPoint Presentation Size: 3879 Last Modified: Thu, 15 Jun 2006 02:08:52 GMT结节性硬化（图） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0596.htmTitle: PowerPoint Presentation Size: 8058 Last Mod

39、ified: 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） WebZIP 7.0.3.1030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: http:/202.113.53.133/jpk/contents/g/yxx/doc/2-9.files/slide0597.htmTitle: 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