顱腦先天畸形影像診療培訓(xùn)課件

1、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學(xué)習(xí)要點本節(jié)應(yīng)了解、熟悉和掌握的知識點了解顱腦先天發(fā)育異常的分類 熟悉常見顱腦發(fā)育異常的影像學(xué)表現(xiàn) 神經(jīng)皮膚綜合征的影像學(xué)表現(xiàn)1顱腦先天畸形影像診療10/9/2022WebZIP 030

2、 offline browWebZIP 030 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學(xué)習(xí)難點本節(jié)學(xué)習(xí)中的難點腦先天性發(fā)育異常的分類和影像學(xué)表現(xiàn) 2顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP

3、030 offline browser (Unregistered) on 09/13/07 10:15:09.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顱腦先天性畸形分類3顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/

4、13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0508.htmTitle: PowerPoint Presentation Size: 2851 Last Modified: Thu, 15 Jun 2006 02:07:48 GMT一、頭顱先天性畸形4顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g

5、/yxx/doc/2-9.files/slide0509.htmTitle: PowerPoint Presentation Size: 4811 Last Modified: Thu, 15 Jun 2006 02:07:48 GMT狹顱癥（ craniostenosis ）顱縫提早骨化、閉合，致頭顱畸形，包括舟狀 頭畸形、短頭畸形、尖頭畸形、偏頭畸形、小 頭畸形等5顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address:

6、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矢狀縫及頂顳縫提早閉合，頭顱左右徑生長受限，前后徑生長顯著 舟狀頭變形舟狀頭變形6顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/con

7、tents/g/yxx/doc/2-9.files/slide0514.htmTitle: PowerPoint Presentation Size: 5582 Last Modified: Thu, 15 Jun 2006 02:07:56 GMT顱底陷入顱底陷入枕骨大孔周圍骨質(zhì)，枕骨基底部、髁部、鱗部向顱腔內(nèi)陷入扁平顱底前、中、后顱窩失去正常的階梯狀關(guān)系7顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jp

8、k/contents/g/yxx/doc/2-9.files/slide0515.htmTitle: PowerPoint Presentation Size: 9979 Last Modified: Thu, 15 Jun 2006 02:07:56 GMT測量標(biāo)準(zhǔn)Chamberlain：硬腭后緣與枕骨大孔后緣連線， 齒狀突高于此線3mm為異常McGregor： 硬腭后緣與枕骨最低點連線， 齒狀突高于此線6mm為異常8顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 0

9、9/13/07 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、腦先天性發(fā)育異常9顱腦先天畸形影像診療10/9/2022WebZIP 030 offline brow先天性腦積水先天性腦積水：又稱積水性無腦畸形病因：病因不明，可能與胚胎期頸內(nèi)動脈發(fā)育 不良、母體感染、放射損傷有關(guān)臨床與病理：頭顱迅速增大、面部比例失調(diào)， 智力低下，多在一

10、歲內(nèi)死亡10顱腦先天畸形影像診療10/9/2022先天性腦積水先天性腦積水：又稱積水性無腦畸形10顱腦先天畸形影像學(xué)表現(xiàn)X線平片：頭顱呈球形增大，囟門大、顱縫寬、顱壁薄，顱面骨比例失調(diào)CT表現(xiàn)：額、頂、顳葉腦實質(zhì)幾乎消失，部分枕葉、基底神經(jīng)核、丘腦保存，小腦、腦干、四腦室一般正常MRI表現(xiàn)：幕上為水樣信號，大腦鐮、基底節(jié)、小腦及腦干結(jié)構(gòu)正常11顱腦先天畸形影像診療10/9/2022影像學(xué)表現(xiàn)X線平片：頭顱呈球形增大，囟門大、顱縫寬、顱壁薄，先天性腦積水12顱腦先天畸形影像診療10/9/2022先天性腦積水12顱腦先天畸形影像診療10/3/2022WebZIP 030 offline brows

11、er (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0533.htmTitle: PowerPoint Presentation Size: 6666 Last Modified: Thu, 15 Jun 2006 02:08:08 GMT 又稱 丹-瓦氏畸形 Dandy-Walker malformation 小腦半球及蚓部發(fā)育不良、四腦室中孔和側(cè)孔閉 鎖致四腦室擴張和梗阻性腦積水四腦室中、側(cè)孔先天性閉塞13顱腦先天畸形影像診療10/9/2022WebZIP 030

12、offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0535.htmTitle: PowerPoint Presentation Size: 6644 Last Modified: Thu, 15 Jun 2006 02:08:10 GMT后顱凹擴大；巨大囊腫；小腦下蚓缺如，上蚓移位；小腦半球發(fā)育不良；腦干受壓，幕上腦積水鑒別：蛛網(wǎng)膜囊腫、大枕大池影像學(xué)表現(xiàn)14顱腦先天畸形影像診療10/9/2022

13、WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/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(圖)15顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 03

14、0 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 02:08:12 GMT 概述：前腦無裂畸形、無腦回、多小腦回、灰質(zhì)異 位、腦裂畸形及腦溝回移位等，胚胎26月 病因不明：血管性，感染性，化學(xué)性腦裂、腦溝和腦回發(fā)育畸形16顱腦先天畸形影像診療10/9/2022WebZIP 03

15、0 offline browWebZIP 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 GMT無腦回畸形（agyria）無腦回畸形：多見頂枕葉CT、MR表現(xiàn): 腦表面光滑，腦回、溝缺如 腦室擴大, 側(cè)裂池、蛛網(wǎng)膜 下腔增寬17顱腦先天畸形影像診療10/

16、9/2022WebZIP 030 offline browWebZIP 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無腦回畸形（圖）18顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 o

17、ffline 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 臨床：難治性癲癇，運動障礙，智力低下，發(fā) 育遲緩 影像學(xué)表現(xiàn)： 中央前、后回附近表面延伸至室管膜下 裂隙（P-E縫） 周圍皮質(zhì)不規(guī)則增厚腦裂畸形（schizencephaly）19顱

18、腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 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開放型腦裂畸形 (圖)20顱腦先天畸形影像診療10/9/2022WebZIP 030 offline b

19、rowWebZIP 030 offline browser (Unregistered) on 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，可能與染色體異常及基因突變有關(guān) 病理：無腦葉、半腦葉、單腦葉型 影像學(xué)表現(xiàn)： 無腦葉 單腦室，少量腦組織，無中線結(jié)構(gòu)并發(fā) 顱面畸形 半腦葉 三腦室中線結(jié)構(gòu)缺如，或有較

20、小大腦鐮， 縱裂淺 單腦葉 透明隔缺如，半球間裂淺前腦無裂畸形（holoprosencephaly）21顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 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 GM

21、T前腦無裂畸形（圖）22顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.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前腦無裂畸形（圖）23顱腦先天畸形影像診療10/9/2022WebZIP 03

22、0 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0562.htmTitle: PowerPoint Presentation Size: 3840 Last Modified: Thu, 15 Jun 2006 02:08:28 GMT前腦無裂畸形（圖）24顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser

23、 (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 02:07:58 GMT腦膜膨出和腦膜腦膨出顱內(nèi)結(jié)構(gòu)經(jīng)顱骨缺損疝出顱外病理 顯性顱裂畸形型 腦膜膨出型 腦膜腦膨出 型 腦室膨出型 囊性腦膜腦膨出臨床顱蓋組 膨出軟組織腫塊顱底組 眶、鼻、咽腫塊25顱腦先天畸形影像診療10/9/2022WebZIP 030 o

24、ffline browWebZIP 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 02:08:00 GMT影像學(xué)表現(xiàn)CT：顱骨缺損，腦脊液密度膨出疝囊；軟組織密 度疝囊MR：腦脊液信號膨出物、腦實質(zhì)信號膨出物，顱 內(nèi)腦室牽拉、變形、擴張26顱腦先天畸形影像診療10/9/

25、2022WebZIP 030 offline browWebZIP 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枕部腦膜腦膨出（圖）27顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 o

26、ffline 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：胚胎早期損傷嚴(yán)重，致完全缺如；胎晚期損 傷輕，致部分缺如 臨床：無癥狀，輕者視覺、交叉觸覺障礙，重者智 力低下、癲癇胼胝體發(fā)育異常（ dysgenesis of the corpu

27、s callosum ）28顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 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：壓部常見，壓部帶狀結(jié)構(gòu)消失，體變薄、 中斷完全缺如：側(cè)腦室擴大、

28、分離，前角小、后角大，三腦室上升，介側(cè)腦室間，頂緊鄰縱裂 池影像學(xué)表現(xiàn)29顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:09.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0529.htmTitle: PowerPoint Presentation Size: 4678 Last Modified: Thu, 15 Jun 2006 02:08:06 GMT胼胝體完全性缺如(圖）30顱

29、腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 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 腦脊液在腦外異常的局限性積聚 病因 ：先天性 先天發(fā)育 后天性 外傷、感染、手術(shù)等 病理： 蛛網(wǎng)膜內(nèi)囊

30、腫，多不與蛛網(wǎng)膜下腔部交通 蛛網(wǎng)膜下囊腫，多與蛛網(wǎng)膜下腔部相通 相鄰顱骨及腦組織呈受壓改變 臨床： 部分無癥狀，部分有輕癱、癲癇發(fā)作等蛛網(wǎng)膜囊腫（arachnoid cyst）31顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 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

31、Modified: Thu, 15 Jun 2006 02:08:34 GMT 影像表現(xiàn)：CT、MRI 邊緣光滑、腦脊液密度/信號區(qū)，無強化， 占位輕，顱骨變薄 位 置 外側(cè)裂池、大腦凸面、枕大池多見 鑒別診斷：膽脂瘤，大枕大池，腦穿通畸形囊腫影像學(xué)標(biāo)表現(xiàn)及鑒別診斷32顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0574.htmTi

32、tle: PowerPoint Presentation Size: 5420 Last Modified: Thu, 15 Jun 2006 02:08:38 GMT蛛網(wǎng)膜囊腫(圖)33顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 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 L

33、ast Modified: Thu, 15 Jun 2006 02:08:42 GMT三、神經(jīng)皮膚綜合征（neurocutaneous syndromes）34顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 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:

34、Thu, 15 Jun 2006 02:08:42 GMT一組遺傳性斑痣性錯構(gòu)瘤病分兩型：神經(jīng)纖維瘤病型（NF-l）神經(jīng)纖維瘤病型（NF-2）神經(jīng)纖維瘤?。╪eurofibromatosis）35顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0584.htmTitle: PowerPoint Presentation Size: 92

35、35 Last Modified: Thu, 15 Jun 2006 02:08:46 GMT NF-1型 NF-2型 CNS病變 顱內(nèi)神經(jīng)腫瘤膠質(zhì)瘤 單、多發(fā)腦脊膜瘤叢狀神經(jīng)纖維瘤 脊神經(jīng)多發(fā)神經(jīng)鞘瘤其它 髓內(nèi)室管膜瘤、星形細(xì)胞瘤影像學(xué)表現(xiàn)36顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0589.htmTitle: PowerPo

36、int Presentation Size: 4676 Last Modified: Thu, 15 Jun 2006 02:08:48 GMT神經(jīng)纖維瘤病-II型（圖）37顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 030 offline browser (Unregistered) on 09/13/07 10:15:10.Address: 33/jpk/contents/g/yxx/doc/2-9.files/slide0590.htmTitle: PowerPoint Presentation Size: 9739 Last Mod

37、ified: Thu, 15 Jun 2006 02:08:50 GMT 病因：常染色體顯性遺傳，外顯率高 臨床：皮脂腺瘤、癲癇、智力低下結(jié)節(jié)性硬化（tuberous sclerosis）38顱腦先天畸形影像診療10/9/2022WebZIP 030 offline browWebZIP 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