颅脑和脊髓先天性疾病.pptxVIP

第1页/共47页颅脑和脊髓先天性疾病第2页/共47页abstract 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 . 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 chronic第3页/共47页abstractintracranial 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. All malformation have each special symptom, with the help of x-ray、CT、MIR et al.We can get the right diagnosis easily. Commonly, the operation that rectify the malformation is the key of the therapy.The more early you operate, the better the patient will get. 第4页/共47页vocabularyCongenital adj先天的Hydrocephalus n脑积水Obstructive adj梗阻性的Cranium bifidum n颅裂Spina bifida n脊柱裂 Meningocele n脊膜膨出Encephalocele n脑膨出Cystic adj囊状的第5页/共47页vocabularyMyelomeningocele n髓脊膜膨出Myelocele n脊髓膨出Craniostenosis n狭颅症Turricephaly n塔状头Basilar adj 颅底的Invagination n套入内部第6页/共47页颅脑和脊髓先天畸形craniocerebral and spinal congenital malformation第一节 先天性脑积水 congenital hydrocephalus第二节 颅裂和脊柱裂 cranium bifidum and spina bifida第三节 狭 颅 症 craniostenosis第四节 颅底陷入症 basilar invagination第7页/共47页第一节 先天性脑积水congenital hydrocephalus 婴儿脑积水：婴幼儿期脑脊液循环通路受阻， 吸收障碍或分泌过多使脑脊液 积聚脑室系统和蛛网膜下腔，致 脑室或蛛网膜下腔扩大。形成 头颅扩大、颅内压增高、 脑功 能障碍。成人脑积水：发病机制相同，但无头颅扩大。第8页/共47页分 类非交通性脑积水：梗阻部位----脑室狭窄处Obstructive hyedrocephalus交通性脑积水：梗阻部位---基 底 池Communication hydrocephalus 不能达大脑表面第9页/共47页婴儿脑积水病因1、产伤颅内出血、感染、炎症2、先天畸形：中脑导水管狭窄，中孔、 侧孔闭锁，阿--齐氏畸形3、病因不明第10页/共47页临床表现clinic presentation1、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-