顱腦和脊髓先天畸形課件

匯報(bào)人：xxx20xx-03-14顱腦和脊髓先天畸形ppt課件目錄顱腦和脊髓先天畸形概述顱腦先天畸形脊髓先天畸形顱腦和脊髓先天畸形的臨床表現(xiàn)診斷與鑒別診斷治療及預(yù)后01顱腦和脊髓先天畸形概述顱腦和脊髓先天畸形是指胎兒在母體內(nèi)發(fā)育過(guò)程中，由于遺傳、環(huán)境等因素影響，導(dǎo)致顱腦和脊髓結(jié)構(gòu)發(fā)育異常的一類疾病。定義根據(jù)畸形的發(fā)生部位和性質(zhì)，可分為腦積水、腦膨出、小頭畸形、無(wú)腦兒、脊柱裂等多種類型。分類定義與分類基因突變、染色體異常等遺傳因素是導(dǎo)致顱腦和脊髓先天畸形的重要原因。遺傳因素環(huán)境因素危險(xiǎn)因素孕期感染、藥物暴露、放射線照射等不良環(huán)境因素也可能導(dǎo)致胎兒發(fā)育異常。高齡產(chǎn)婦、孕期營(yíng)養(yǎng)不良、慢性疾病等都可能增加胎兒發(fā)生顱腦和脊髓先天畸形的風(fēng)險(xiǎn)。030201發(fā)病原因及危險(xiǎn)因素以下附贈(zèng)各項(xiàng)管理制度英文版（不需要可刪）急救藥品、器材管理制度:1.Rescuedrugsandequipmentshouldbe"fivefixed"(fixedquantityandvariety,designatedplacement,designatedpersonstorage,regulardisinfectionandsterilization,regularinspectionandmaintenance)and"twotimely"(timelyinspectionandmaintenance,timelyreceiptandsupplementation).Theitemisclearlymarkedandcannotbeusedarbitrarily.2.Thenecessaryrescueequipmentiscomplete,ingoodperformance,andinstandbycondition.3.Therescuedrugsarecomplete,withcleardruglabelsandnodiscoloration,deterioration,expiration,ordamage.Theyshouldbeplacedandusedintheorderofdrugexpirationdates(fromrighttoleft).4.Emergencydrugsanditemsforeachdepartment'srescuevehicleshallbeuniformlyequippedaccordingtorequirements.Specializedemergencydrugsanditemsmustbereviewedandapprovedbythedepartmentdirectortodeterminethetype,quantity,specifications,anddosagetobeequipped.Rescuevehiclesmustbeplacedindesignatedlocationsandmanagedbydesignatedpersonneltoensuresafetyandeaseofuse.5.Afterusingrescuedrugsandequipment,theyshouldbefullyreplenishedwithin24hours.Iftheycannotbereplenishedduetospecialreasons,theyshouldbenotedonthehandoverregistrationformandreportedtotheheadnurseforcoordinationandresolutiontoensuretimelyuseduringpatientrescue.6.Thereisaregistrationbookfortheprovisionofdrugsandequipment.Ensureconsistencybetweenaccountsandmaterials,andhandoverbetweenshifts.7.Managementofsealedrescuevehicles:Beforesealing,theheadnurse(ornurseincharge)andanothernurseshallcountthedrugsandequipmentaccordingtotheregistrationbookofdrugandequipmentequipment,verifytheiraccuracy,andsealthemwithaseal.Twopeopleshallsignandfillinthesealingtime.Nurseschecktheconditionofthesealsoncepershiftandcompletethehandover.Theresponsiblenursescheckonceaweek,andtheheadnurseandresponsiblenursesopenthesealsandinspectthedrugsandequipmentintheambulanceonceamonth,withrecordskept.8.Nonsealedrescuevehiclemanagement:Eachshiftshallcountthedrugsandequipmentaccordingtotheregistrationbookandcompletethehandover.Theresponsiblenurseshallinspectonceaweek,andtheheadnurseshallinspectonceeverytwoweeksandkeeprecords,ensuringthattheaccountsmatchthematerials.護(hù)理文書書寫制度:

1.Nursingstaffstrictlyfollowthelatestrequirementswhenwritingnursingmedicalrecords.2.Thecontentofnursingrecordsshouldbeobjective,truthful,accurate,timely,complete,andstandardized.3.Allnursingdocumentsshouldbewrittenwithablueblackorcarboninkpen.4.AllnursingdocumentsshouldbewritteninArabicnumeralsfordateandtime,withdatesinyears,months,anddays,usinga24-hoursystem,specifictominutes.5.WritingshoulduseChinese,medicalterminology,andcommonlyusedforeignlanguageabbreviations;Completerecorditems;Thetextisneat,thehandwritingisclear,andthelayoutisclean;Accurateexpression,fluentsentences,simpleandconcise:correctformatandpunctuation,notypos.6.Whenerrorsoccurduringthewritingprocess,doublelinethemonthewrongwords,keeptheoriginalrecordclearanddistinguishable,signthemodifier,indicatethemodificationtime,continuetowritethecorrectcontent,anddonotusescraping,sticking,paintingorothermethodstocoveruporremovetheoriginalhandwriting.Eachpageshouldbemodifiednomorethantwotimes,otherwisetheoriginalrecorderwillpromptlycopyagain(exceptformodificationsmadebysuperiors).7.Nursingrecordswrittenbyinternnurses,probationarynurses,orunregisterednursesshouldbereviewedandsignedbynurseswithlegalprofessionalqualificationsinthismedicalinstitution.8.Furthertrainingnursescanonlywritenursingdocumentsafterbeingrecognizedbythemedicalinstitutionreceivingthetrainingfortheirworkability.9.Superiornursingstaffhavetheresponsibilitytoreviewandmodifythewrittenrecordsofsubordinatenursingstaff.Whenmakingmodifications,reddoublelinesshouldbeusedtomarkerrors,writethemodifiedcontent,signandindicatethemodificationtime.10.Temperaturerecords,medicalorders,patientcarerecords,andsurgicalinventoryrecordsshouldbearchivedontime.顱腦和脊髓先天畸形的發(fā)病率因地區(qū)、種族等因素而有所差異。發(fā)病率部分顱腦和脊髓先天畸形在性別分布上存在差異，如腦積水男性患兒多于女性。性別分布顱腦和脊髓先天畸形的預(yù)后因畸形類型和嚴(yán)重程度而異，輕者可能無(wú)明顯癥狀，重者可能導(dǎo)致嚴(yán)重神經(jīng)系統(tǒng)功能障礙甚至死亡。預(yù)后情況流行病學(xué)特點(diǎn)02顱腦先天畸形顱縫早閉定義狹顱癥表現(xiàn)診斷方法治療手段顱縫早閉與狹顱癥顱縫過(guò)早閉合，導(dǎo)致顱骨生長(zhǎng)受限，影響腦發(fā)育。頭顱X線、CT掃描等影像學(xué)檢查。頭顱畸形、顱內(nèi)壓增高、神經(jīng)功能缺損等。手術(shù)矯正為主，輔以藥物治療和康復(fù)訓(xùn)練。腦脊液分泌過(guò)多、吸收障礙或循環(huán)受阻。腦積水成因臨床表現(xiàn)診斷依據(jù)治療措施頭顱增大、前囟擴(kuò)大、顱內(nèi)壓增高等。頭顱B超、CT或MRI等影像學(xué)檢查。手術(shù)治療為主，如腦室-腹腔分流術(shù)等。先天性腦積水硬腦膜膨出、軟腦膜膨出等。腦膨出類型局部腫塊、神經(jīng)功能缺損等。癥狀表現(xiàn)B超、CT或MRI等影像學(xué)檢查。診斷手段手術(shù)切除為主，注意保護(hù)神經(jīng)功能。治療方法先天性腦膨大腦表面缺乏正常腦回結(jié)構(gòu)，表現(xiàn)為智力低下、癲癇等癥狀。無(wú)腦回畸形腦回異常增大，可伴有智力障礙、運(yùn)動(dòng)發(fā)育遲緩等。巨腦回畸形胼胝體部分或完全缺失，導(dǎo)致左右大腦半球信息傳遞受阻，表現(xiàn)為智力障礙、視覺(jué)障礙等。胼胝體發(fā)育不良大腦皮質(zhì)下灰質(zhì)異常聚集，可引發(fā)癲癇、智力障礙等?；屹|(zhì)異位癥其他顱腦先天畸形03脊髓先天畸形脊髓脊膜膨定義脊髓脊膜膨出是一種先天性神經(jīng)系統(tǒng)發(fā)育畸形，由于先天性椎板發(fā)育不全，同時(shí)存在脊髓、脊膜通過(guò)椎板缺損處向椎管外膨出。發(fā)病率全球發(fā)病率約0.05%-0.1%，是新生兒致殘和致死重要原因之一。癥狀與影響膨出的脊膜可壓迫脊髓和神經(jīng)根，導(dǎo)致相應(yīng)的神經(jīng)功能障礙，如下肢癱瘓、大小便失禁等。診斷與治療通過(guò)MRI等影像學(xué)檢查可明確診斷，治療以手術(shù)為主，需切除膨出的脊膜并修補(bǔ)缺損的椎板。脊髓縱裂定義脊髓縱裂是在胚胎時(shí)期，由于脊髓或者椎管發(fā)育畸形，使脊髓分裂為左右兩部分。癥狀與影響臨床上可無(wú)明顯癥狀，但部分患者伴有脊髓栓系綜合征，表現(xiàn)為下肢感覺(jué)、運(yùn)動(dòng)障礙及疼痛，嚴(yán)重者出現(xiàn)下肢癱瘓和大小便功能障礙。發(fā)病率多見(jiàn)于嬰幼兒和少年，偶見(jiàn)于成年人，發(fā)生率很低。診斷與治療通過(guò)MRI等影像學(xué)檢查可明確診斷，治療以手術(shù)為主，需切除分裂的脊髓并解除脊髓栓系。診斷與治療通過(guò)MRI等影像學(xué)檢查可明確診斷，治療以手術(shù)為主，需切除空洞并解除壓迫。定義脊髓空洞癥是脊髓的一種慢性、進(jìn)行性的病變，其病變特點(diǎn)是脊髓內(nèi)形成管狀空腔以及膠質(zhì)增生。病因不十分清楚，可能與先天性發(fā)育異常、脊髓損傷、脊髓腫瘤等因素有關(guān)。癥狀與影響常好發(fā)于頸部脊髓，當(dāng)病變累及延髓時(shí)，則稱為延髓空洞癥?；颊呖赡艹霈F(xiàn)上肢麻木、疼痛、無(wú)力等癥狀，嚴(yán)重者出現(xiàn)肌肉萎縮和截癱。脊髓空洞癥03脊髓拴系綜合征由于脊髓受到異常牽拉導(dǎo)致的神經(jīng)功能障礙，如下肢感覺(jué)運(yùn)動(dòng)障礙、大小便失禁等。01先天性脊柱側(cè)彎由于脊柱發(fā)育異常導(dǎo)致的脊柱向一側(cè)彎曲，可能伴有椎體旋轉(zhuǎn)和肋骨畸形。02脊髓內(nèi)腫瘤脊髓內(nèi)發(fā)生的腫瘤，可能壓迫和損傷脊髓，導(dǎo)致相應(yīng)的神經(jīng)功能障礙。其他脊髓先天畸形04顱腦和脊髓先天畸形的臨床表現(xiàn)腦積水表現(xiàn)為頭顱異常增大，前囟擴(kuò)大且飽滿，顱縫開(kāi)裂等，主要由于腦脊液循環(huán)通路受阻或分泌過(guò)多導(dǎo)致。顏面部畸形如眼距過(guò)寬、鼻梁塌陷、下頜短小等，常與顱腦先天畸形伴發(fā)。腦zu織發(fā)育異常如胼胝體發(fā)育不良、腦穿通畸形、無(wú)腦回畸形等，可導(dǎo)致智力低下、癲癇等神經(jīng)系統(tǒng)癥狀。頭形異常如小頭、尖頭、舟狀頭等，可能由于顱骨骨縫過(guò)早閉合或發(fā)育異常引起。顱腦先天畸形的臨床表現(xiàn)輸入標(biāo)題脊髓脊膜膨出脊柱裂脊髓先天畸形的臨床表現(xiàn)是脊髓先天畸形中最常見(jiàn)的一種，表現(xiàn)為脊柱后側(cè)或側(cè)方的缺損，可伴有脊膜、脊髓和神經(jīng)根的膨出?；颊呖赡艹霈F(xiàn)下肢癱瘓、大小便失禁等癥狀。由于脊髓受到異常牽拉導(dǎo)致，患者可能出現(xiàn)下肢感覺(jué)運(yùn)動(dòng)障礙、大小便失禁等癥狀，嚴(yán)重時(shí)可影響生活自理能力。脊髓內(nèi)出現(xiàn)空腔，導(dǎo)致脊髓功能障礙，患者可能出現(xiàn)上肢或軀干部的疼痛、溫覺(jué)減退等癥狀，嚴(yán)重時(shí)可導(dǎo)致肌肉萎縮和關(guān)節(jié)畸形。表現(xiàn)為腰骶部或頸后部的囊性包塊，常伴有神經(jīng)損害癥狀，如下肢無(wú)力、肌肉萎縮等。脊髓栓系綜合征脊髓空洞癥05診斷與鑒別診斷包括臨床表現(xiàn)、