颅脑先天畸形影像诊断

第九节

颅脑先天畸形及发育异常精选课件WebZIP030

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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精选课件WebZIP030

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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精选课件WebZIP030

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颅脑先天性畸形分类精选课件WebZIP030

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一、头颅先天性畸形精选课件狭颅症（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、小头畸形等精选课件WebZIP030

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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精选课件WebZIP030

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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精选课件WebZIP030

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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为异常精选课件WebZIP030

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二、脑先天性发育异常精选课件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，多在一岁内死亡精选课件影像学表现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精选课件先天性脑积水精选课件WebZIP030

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又称丹-瓦氏畸形Dandy-Walkermalformation

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、侧孔先天性闭塞精选课件WebZIP030

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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精选课件WebZIP030

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Dandy-Walker畸形(图)精选课件WebZIP030

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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2～6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精选课件WebZIP030

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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、蛛网膜下腔增宽精选课件WebZIP030

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无脑回畸形（图）精选课件WebZIP030

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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）精选课件WebZIP030

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开放型脑裂畸形(图)精选课件WebZIP030

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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）精选课件WebZIP030

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前脑无裂畸形（图）精选课件WebZIP030

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前脑无裂畸形（图）精选课件WebZIP030

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前脑无裂畸形（图）精选课件WebZIP030

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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

Ⅰ型脑膜膨出 Ⅱ型脑膜脑膨出

Ⅲ型脑室膨出 Ⅳ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、咽肿块精选课件WebZIP030

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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、扩张精选课件WebZIP030

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枕部脑膜脑膨出（图）精选课件WebZIP030

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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、癫痫胼胝体发育异常

（dysgenesisofthecorpuscallosum）精选课件WebZIP030

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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 影像学表现精选课件WebZIP030

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胼胝体完全性缺如(图）精选课件WebZIP030

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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

（arachnoidcyst）精选课件WebZIP030

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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精选课件WebZIP030

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蛛网膜囊肿(图)精选课件WebZIP030

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三、神经皮肤综合征

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一组遗传性斑痣性错构瘤病

分两型： 神经纤维瘤病Ⅰ型（NF-l）

神经纤维瘤病Ⅱ型（NF-2）神经纤维瘤病

（neurofibromatosis）精选课件WebZIP030

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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

影像学表现精选课件WebZIP030

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神经纤维瘤病-II型（图）精选课件WebZIP030

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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

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