abstractThecongenitalmalformationofcentralnervoussystemisthecommondiseaseofchildreninneurosurgeryfield.Thischaptermainlyintroducecongenitalhydrocephalus,craniumbifidumandspinabifida,craniostenosandbasilarinvaginationThecausesofthesediseasesareanypartsofthecirculatoryroute,thepdifferent.Whenanobstructionhappensihydrocephalushappens.ThepatientshaveesymptomandsignofchronIcabstract1abstractntracranialpressureThecauseofcranium,spinaandbasilarinvaginationscongenitalheterplasiaButcraniostenosisisbyreasonsofbonesutureclosingsoearlythatthebraincannotdevelopnormally.Allmalformationhaveeachspeciasymptom,withthehelpofx-ray、CT、MIRetal.WecangettherightdiagnosiseasilCommonly,theoperationthatrectifythemalformationisthekeyofthetherapy.Themoreearlyyouoperate,thebetterthepatientwillgetabstract2vocabularyCongenital先天的eHydrocephalusObstructiveeCraniumbifidumeSpinabifidaeMeningoceleanannnn腦積水梗阻性的顱裂脊柱裂脊膜膨出oEncephalocele腦膨出●Cystic囊狀的vocabulary3vocabularyMyelomeningocelen髓脊膜膨出●Myelocelen脊髓膨出eCraniostenosin狹顱癥●Turricephaln塔狀頭eBasilaradj顱底的●Invaginationn套入內(nèi)部vocabulary4顱腦和脊髓先天畸形craniocerebralandspinalcongenitalmalformation第一節(jié)先天性腦積水congenitalhydrocephalus第二節(jié)顱裂和脊柱裂ocraniumbifidumandspinabifida●第三節(jié)狹顱癥craniostenosis第四節(jié)顱底陷入癥basilarinvagination顱腦和脊髓先天畸形5第先天性腦積水congenitalhydrocephalus●嬰兒腦積水:嬰幼兒期腦脊液循環(huán)通路受阻,吸收障礙或分泌過多使腦脊液積聚腦室系統(tǒng)和蛛網(wǎng)膜下腔,致腦室或蛛網(wǎng)膜下腔擴(kuò)大。形成頭顱擴(kuò)大、顱內(nèi)壓增高、腦功能障礙?！癯扇四X積水:發(fā)病機(jī)制相同,但無(wú)頭顱擴(kuò)大第6分類●非交通性腦積水:梗阻部位--腦室狹窄處oObstructivehydrocephalus●交通性腦積水:梗阻部位-基底池oCommunicationhydrocephalus不能達(dá)大腦表面分類7嬰兒腦積水病因●1、產(chǎn)傷顱內(nèi)出血、感染、炎癥2、先天畸形:中腦導(dǎo)水管狹窄,中孔側(cè)孔閉鎖,阿-齊氏畸形病因不明嬰兒腦積水病因8臨床表現(xiàn)clinicpresentation頭圍異常增大,額頂突出,囟門擴(kuò)大隆起顱縫增寬,頭頂扁平,頭發(fā)稀少,頭皮靜脈怒張,面顱小于頭顱顱骨菲薄,叩診破壸音3、眶頂受壓變薄下移,眼球受壓下旋,上部鞏膜外露--落日征4、腦功能障礙:智能低下,瞳孔反射異常眼球運(yùn)動(dòng)障礙,抽搐發(fā)作。臨床表現(xiàn)clinicpresentation9然后,劑。早先天腦積后水落日征菠內(nèi)圖20-1x先天性腦積水的外貌(虛線表示擴(kuò)大的前囟)然后,10顱腦和脊髓先天性疾病課件11顱腦和脊髓先天性疾病課件12顱腦和脊髓先天性疾病課件13顱腦和脊髓先天性疾病課件14顱腦和脊髓先天性疾病課件15顱腦和脊髓先天性疾病課件16顱腦和脊髓先天性疾病課件17顱腦和脊髓先天性疾病課件18顱腦和脊髓先天性疾病課件19顱腦和脊髓先天性疾病課件20顱腦和脊髓先天性疾病課件21顱腦和脊髓先天性疾病課件22顱腦和脊髓先天性疾病課件23顱腦和脊髓先天性疾病課件24顱腦和脊髓先天性疾病課件25顱腦和脊髓先天性疾病課件26顱腦和脊髓先天性疾病課件27顱腦和脊髓先天性疾病課件28顱腦和脊髓先天性疾病課件29顱腦和脊髓先天性疾病課件30顱腦和脊髓先天性疾病課件31顱腦和脊髓先天性疾病課件32顱腦和脊髓先天性疾病課件33顱腦和脊髓先天性疾病課件34顱腦和脊髓先天性疾病課件35顱腦和脊髓先天性疾病課件36顱腦和脊髓先天性疾病課件37顱腦和脊髓先天性疾病課件38顱腦和脊髓先天性疾病課件39顱腦和脊髓先天性疾病課件40顱腦和脊髓先天性疾病課件41顱腦和脊髓先天性疾病課件42顱腦和脊髓先天性疾病課件43顱腦和脊髓先天性疾病課件44顱腦和脊髓先天性疾病課件45顱腦和脊髓先天性疾病課件46abstractThecongenitalmalformationofcentralnervoussystemisthecommondiseaseofchildreninneurosurgeryfield.Thischaptermainlyintroducecongenitalhydrocephalus,craniumbifidumandspinabifida,craniostenosandbasilarinvaginationThecausesofthesediseasesareanypartsofthecirculatoryroute,thepdifferent.Whenanobstructionhappensihydrocephalushappens.ThepatientshaveesymptomandsignofchronIcabstract47abstractntracranialpressureThecauseofcranium,spinaandbasilarinvaginationscongenitalheterplasiaButcraniostenosisisbyreasonsofbonesutureclosingsoearlythatthebraincannotdevelopnormally.Allmalformationhaveeachspeciasymptom,withthehelpofx-ray、CT、MIRetal.WecangettherightdiagnosiseasilCommonly,theoperationthatrectifythemalformationisthekeyofthetherapy.Themoreearlyyouoperate,thebetterthepatientwillgetabstract48vocabularyCongenital先天的eHydrocephalusObstructiveeCraniumbifidumeSpinabifidaeMeningoceleanannnn腦積水梗阻性的顱裂脊柱裂脊膜膨出oEncephalocele腦膨出●Cystic囊狀的vocabulary49vocabularyMyelomeningocelen髓脊膜膨出●Myelocelen脊髓膨出eCraniostenosin狹顱癥●Turricephaln塔狀頭eBasilaradj顱底的●Invaginationn套入內(nèi)部vocabulary50顱腦和脊髓先天畸形craniocerebralandspinalcongenitalmalformation第一節(jié)先天性腦積水congenitalhydrocephalus第二節(jié)顱裂和脊柱裂ocraniumbifidumandspinabifida●第三節(jié)狹顱癥craniostenosis第四節(jié)顱底陷入癥basilarinvagination顱腦和脊髓先天畸形51第先天性腦積水congenitalhydrocephalus●嬰兒腦積水:嬰幼兒期腦脊液循環(huán)通路受阻,吸收障礙或分泌過多使腦脊液積聚腦室系統(tǒng)和蛛網(wǎng)膜下腔,致腦室或蛛網(wǎng)膜下腔擴(kuò)大。形成頭顱擴(kuò)大、顱內(nèi)壓增高、腦功能障礙?！癯扇四X積水:發(fā)病機(jī)制相同,但無(wú)頭顱擴(kuò)大第52分類●非交通性腦積水:梗阻部位--腦室狹窄處oObstructivehydrocephalus●交通性腦積水:梗阻部位-基底池oCommunicationhydrocephalus不能達(dá)大腦表面分類53嬰兒腦積水病因●1、產(chǎn)傷顱內(nèi)出血、感染、炎癥2、先天畸形:中腦導(dǎo)水管狹窄,中孔側(cè)孔閉鎖,阿-齊氏畸形病因不明嬰兒腦積水病因54臨床表現(xiàn)clinicpresentation頭圍異常增大,額頂突出,囟門擴(kuò)大隆起顱縫增寬,頭頂扁平,頭發(fā)稀少,頭皮靜脈怒張,面顱小于頭顱顱骨菲薄,叩診破壸音3、眶頂受壓變薄下移,眼球受壓下旋,上部鞏膜外露--落日征4、腦功能障礙:智能低下,瞳孔反射異常眼球運(yùn)動(dòng)障礙,抽搐發(fā)作。臨床表現(xiàn)clinicpresentation55然后,劑。早先天腦積后水落日征菠內(nèi)圖20-1x先天性腦積水的外貌(虛線表示擴(kuò)大的前囟)然后,56顱腦和脊髓先天性疾病課件57顱腦和脊髓先天性疾病課件58顱腦和脊髓先天性疾病課件59顱腦和脊髓先天性疾病課件60顱腦和脊髓先天性疾病課件61顱腦和脊髓先天性疾病課件62顱腦和脊髓先天性疾病課件63顱腦和脊髓先天性疾病課件64顱腦和脊髓先天性疾病課件65顱腦和脊髓先天性疾病課件66顱腦和脊髓先天性疾病課件67顱腦和脊髓先天性疾病課件68顱腦和脊髓先天性疾病課件69顱腦和脊髓先天性疾病課件70顱腦和脊髓先天性疾病課件71顱腦和脊髓先天性疾病課件72顱腦和脊髓先天性疾病課件73顱腦和脊髓先天性疾病課件74顱腦和脊髓先天性疾病課件75顱腦和脊髓先天性疾病課件