颅脑和脊髓先天畸形

颅脑和脊髓先天畸形颅脑和脊髓先天畸形 craniocerebral and spinal congenital malformationcraniocerebral and spinal congenital malformation 制作制作 哈医大一院哈医大一院 神经外科神经外科 abstractl The congenital malformation of central nervous system is the common disease of children in neurosurgery field.This chapter mainly introduce congenital hydrocephalus,cranium bifidum and spina bifida,craniostenosis and basilar invagination.l The causes of these diseases are different.When an obstruction happens in any parts of the circulatory route,the hydrocephalus happens.The patients have the symptom and sign of chronicabstractlintracranial pressure.The cause of cranium,spina and basilar in vagination is congenital heterplasia.But craniostenosis is by reasons of bone suture closing so early that the brain can not develop normally.l All malformation have each special symptom,with the help of x-ray、CT、MIR et al.We can get the right diagnosis easily.l Commonly,the operation that rectify the malformation is the key of the therapy.The more early you operate,the better the patient will get.vocabularylCongenital adj 先天的lHydrocephalus n 脑积水lObstructive adj 梗阻性的lCranium bifidum n 颅裂lSpina bifida n 脊柱裂l Meningocele n 脊膜膨出lEncephalocele n 脑膨出lCystic adj 囊状的vocabularylMyelomeningocele n 髓脊膜膨出lMyelocele n 脊髓膨出lCraniostenosis n 狭颅症lTurricephaly n 塔状头lBasilar adj 颅底的lInvagination n 套入内部颅脑和脊髓先天畸形craniocerebral and spinal congenital malformationl第一节 先天性脑积水l congenital hydrocephalusl第二节 颅裂和脊柱裂l cranium bifidum and spina bifidal第三节 狭 颅 症l craniostenosisl第四节 颅底陷入症l basilar invagination第一节 先天性脑积水congenital hydrocephalus l婴儿脑积水：婴幼儿期脑脊液循环通路受阻，l 吸收障碍或分泌过多使脑脊液l 积聚脑室系统和蛛网膜下腔，致l 脑室或蛛网膜下腔扩大。形成l 头颅扩大、颅内压增高、脑功l 能障碍。l成人脑积水：发病机制相同，但无头颅扩大。分 类l非交通性脑积水：梗阻部位-脑室狭窄处lObstructive hyedrocephalusl交通性脑积水：梗阻部位-基 底 池lCommunication hydrocephalus l l 不能达大脑表面婴儿脑积水病因l1、产伤颅内出血、感染、炎症l2、先天畸形：中脑导水管狭窄，中孔、l 侧孔闭锁，阿-齐氏畸形l3、病因不明临床表现clinic presentationl1、头围异常增大，额顶突出，囟门扩大隆起，颅缝增宽，头顶扁平，头发稀少，头皮静脉怒张，面颅小于头颅。2、颅骨菲薄，叩诊破壶音。3、眶顶受压变薄下移，眼球受压下旋，上部 巩膜外露-落日征。l4、脑功能障碍：智能低下，瞳孔反射异常，眼球运动障碍，抽搐发作。先天脑积水-落日征辅 助 检 查l1、X片：颅腔扩大，颅骨变薄，囟门增大，l 骨缝分离。l2、CT：脑室扩大，皮质变薄。l3、MRI：脑室系统情况、梗阻、肿瘤及位置、l 畸形、等病变。治 疗 therapyl1、解除梗阻手术：畸形、梗阻l2、旁路手术：l （1）Torkildson手术：侧脑室-枕大池l 分流，适用于导水管阻塞。l （2）第三脑室造瘘术：终板打开。不持久。l3、分流术：l 1、腰池-腹腔分流术l 2、脑室-体腔分流术 l （1）脑室-腹腔分流术l （2）脑室-心房分流术l 并发症l1、堵塞：临床症状重现-梗阻l 原因：（1）脑脊液蛋白高l （2）脑室内出血l （3）大网膜粘连阻塞远端l2、感染：l 原因：（1）皮肤破溃l （2）操作污染l （3）分流管消毒不严l l 第二节 颅裂和脊柱裂cranium bifidum and spina bifida分 类 classification颅裂和脊柱裂是胚胎发育障碍 1、隐性：只有颅骨或椎管骨缺损，无内容膨出。2、显性：颅骨或椎管骨缺损+颅 内容或椎管内容膨出。颅裂和脊柱裂好发部位l l 显性颅裂 cranium bifidum apertum一、根据膨出内容分类l1、脑膜膨出meningocele：脑膜+脑脊液l2、脑 膨 出encephalocele：脑膜+脑实质l3、脑膜脑囊状膨cysticMingoencephalocele：l 脑膜+脑实质+部分脑室+脑脊液l4、脑囊状膨出cystic encephalocele：l l 脑膜+部分脑室+脑实质二、临床表现 clinic presentationl1、中线部位肿块：出生就有，逐渐长大，l 多见于鼻根、枕部。l2、脑膜膨出：颅骨缺损小，透光能压缩。l3、脑膜脑膨出：颅骨缺损大，不透光，不l 能压缩，实体感。l4、可合并其他脑畸形。l5、合并智力障碍等神经系统体征。l 三、诊 断 diagnosisl一、临床表现：l二、辅助检查：l 1、X片：颅骨缺损l 2、C T：膨出内容l 3、MRI：优于CT颅裂四、治 疗 therapyl l 关闭缺损，切除膨出肿块，l一岁内安全。l l 显性脊柱裂 spina bifida apertum显性脊柱裂分类l1、脊膜膨出 meringocele:l 脊膜+脑脊液l2、脊髓脊膜膨出myelomeningocele：l 脊髓+脊膜+脑脊液l3、脊髓膨出myelocele：l 脊髓外露二、临床表现 clinic presentationl1、局部表现：肿块l2、脊髓神经受损：下肢瘫，尿便障 l 碍，脊髓栓系综合征三、诊断 diagnosisl一、临床表现：l二、辅助检查：l 1、X片：颅骨缺损l l 2、MRI：脊髓圆椎下移、内容 四、治 疗 therapyl手术时机 ：13个月最好l切开囊壁 游离组织 还纳 切除 l 囊壁 l l 缝合栓系-切断终丝l 第三节 狭颅症 craniostenosisl狭颅症：颅缝早闭、颅缝骨化症l颅腔狭小 脑发育受限l 颅高压l l 脑功能障碍临 床 表 现 clinic presentationl1、头颅畸形：l 所有颅缝-尖头畸形、塔头l 矢 状 缝-舟状头、长头畸形l 两侧冠缝-短头或扁头畸形l 一侧冠缝-斜头畸形临 床 表 现l2、脑功能障碍和颅内压增高：l l 智力低下，精神萎靡，癫l痫，头痛，呕吐，视乳头水肿临 床 表 现l3、眼部症状l 眼眶变浅，突眼，分离性斜视。l4、其他身体畸形l 脊柱裂、唇裂、并指诊 断 diagnosisl1、临床表现：l2、辅助检查：l X片：骨缝早闭，颅高压征象治 疗 therapyl手术治疗：6个月内进行l l 一、切除早闭颅缝l l 二、切除大骨块减压l l第四节 颅底陷入症basilar invaginationl 特 点characteristic枕大孔周围的颅底结构向颅内陷入,齿突高于正常,枕骨大孔前后径缩短,颅后窝狭小,结果：延髓受压，局部神经受牵拉.病 因pathogenyl 先天发育畸形多见l常合并扁平颅底、环枢椎畸形、l小脑扁桃体下疝临 床 表 现 clinic presentationl1、婴幼儿期：颅底、颈椎骨未骨化，组织弹性好，无症状。l2、成年：筋膜、韧带、硬膜、蛛网膜增厚，瘢痕粘 连，损伤，神经血管受损，颈神经、尾组 颅神经、延髓、小脑功能障碍，颅内压增 高。l3、颈粗短，发际低，头颈歪斜缓慢进行性加重。诊 断 diagnosisl1、颅颈交界X片：l 1）Chamberlain线：硬腭后缘枕大孔上 l 缘连线，齿突高于3mm可确诊。l 2）Booggard线：颅前窝底-斜坡夹角，l 115145度，大于145度为扁平颅底l2、MRI：l 显示颈髓受压、下疝畸形、脊髓空洞症等l -有助于制定手术方案颅底陷入症治 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