椎管内肠源性囊肿

1CASEDISCUSSION

精选ppt2Vocabularyhypoalgesia[haɪpəʊæl‘dʒi:sɪə]痛觉减退Hypoesthesia[haɪpəʊs‘θi:zɪə]感觉迟钝Myodynamia[maɪɒdaɪ‘næmjə]肌力analsphincter[ˈsfɪŋktə]肛门括约肌congenitaldysplasia[disˈpleiziə]先天性发育不良spinabifida[ˌspaɪnəˈbɪfɪdə]脊柱裂精选ppt3CASE1Patient:female,34yearsoldWithwaist

pain

andtherightlowerextremity

radicularpainfor6months.Therewasnofever,noheadache,nosurgery,nohistoryoftraumaExamination:hypoesthesiaatthefrontofthethigh,BilateralLowerlimbsmyodynamiaIV,muscletension(-),BilateralBabinskisign(-)Laboratorytests(-)精选ppt4CT精选ppt5T1WIT2WISTIR精选ppt6CE-T1WI精选ppt7CASE2Patient:male,32yearsoldNeckandshoulder

and

bilateralupperlimbs

painwith

limbsnumbnessandweakness

formorethan6years,Thesymptomswereaggravating

withlimitedmobility

for10days.Therewasnofever,noheadache,nosurgery,nohistoryoftraumaExamination:passiveposture,neckmovementwasrestricted.doubleupperlimbsapparenthypoalgesia,bilateralupperlimbsmyodynamiaII;theleftlowerlimbmyodynamiaI,therightlowerlimbmyodynamiaII.muscletension(-).BilateralHoffmannsign(+),Babinskisyndrome(+),Rectaltouch:theanalsphinctercontractionweakness,analreflex(-)Laboratorytests(-)精选ppt8CT精选ppt9T1WIT2WICE-T1精选ppt10T2WICE-T1WI精选ppt11PLEASEGIVEYOUROPINION

精选ppt12手术记录case1精选ppt13手术记录case2精选ppt14Intraspinalneurentericcysts神经肠囊肿上皮细胞的显微照片。（a）囊肿壁：显示单层柱状和立方上皮细胞贴附由Ⅳ型胶原组成的基底层（H和E，×200）（b）柱状上皮、立方上皮和分泌粘液的杯状细胞，高倍镜（H和E，×1000）（C）假复层纤毛柱状上皮的肠源性囊肿壁高倍镜（H和E，×1000）精选ppt15NeurentericcystsMRIofthoracolumbarjunctionwithintradural/extramedullaryneurentericcystatT11.(a)MidsagittalMRIdemonstratesisointenselesion(arrow)onT1-weightedand(b)hyperintensesignal(arrow)onT2-weighted精选ppt16CauseItspathogenesis

isnotclear.

Itgeneralconsidered

asacongenital

diseaserelatedwithendodermdevelopmentdisorders.

Duringembryonicdevelopmentperiod,partoftheendodermaltissueswasabnormal

posteriordisplacementandembedded

intheneuraltube.Heterotopicrestsofepitheliumofgastrointestinaltissueleadtoeventualformationofcompressivecysticlesionsofthepediatricandadultspine.精选ppt17PathologicalmechanismOnly

containingendodermalcomponents,

similar

withthedigestivetract

epithelium.

Form

thecystwallepithelialcells

canbesinglelayer

flat,

cubic,

columnarepithelium,pseudostratifiedcolumnarepithelium

to

stratifiedsquamousepithelium,alsocanhave

mucousgland,

serousgland,

muscle

andfat.Sometimescontainciliaand

mucussecreting

gobletcells.精选ppt18Epidemiologyaccountfor0.7-1.3%ofspinalaxistumors.Mostlyoccursinchildrenandadolescents11days---40yearsMalepredilection:proportionofmentowomenas(2-3):1mostoccuredinthe

subduralextramedullary90%,afewinthe

epidural

orintramedullary.

neckorupper

thoracicweremostinvolved,

lumbarandsacral

rare.

Oftenaccompaniedwith

congenitalspinedeformities(likespina

bifida)精选ppt19ImagingfindingsMRIshowedaroundor

ovalcyst

lesion,

withsmoothedges,thelongaxisdirectionthesameasthespinalcord.butalsocanbe

irregularinshape.

Cyst

resultingin

significant

compressionofthespinalcord,cyst

wallisthinand

uniform.T1WI

shows

mainly

low/equal/slightlyhigherthanCSFT2WI

showshigh/iso

signal

toCSF,

usuallyhomogeniousSignal

strengthdependsonthe

cyst

contents,

more

protein

constituentsor

intracystichemorrhage,

T1WIshowedhighsignal

or

lowT2WIsignal"Spinalcord

embedded

syndrome"Noenhancement精选ppt20DiagnosisThefollowing

characteristics

shouldbehighlysuspected:

(1)inmaleadolescents;(2)partialnerverootpainorspinalcordcompression

symptomsasthemainclinicalmanifestations,remission

andexacerbationofalternating;(3)theMRIdisplaycysticlesionsinthecervicalorthoracic

intraspinalsubduralextramedullary,cystsignalsimilar

withCSF,withthincystwall,noenhancementeffect;with

"Spinalcord

embedded

syndrome"(4)X-rayplainradiographyandCTdisplayothercongenitalmalformations精选ppt21TreatmentSurgicaltreatment,particularlytotalresectionwithrarerecurrencepartialresection’srecurrencerateashighas37%JesseJ.Savageetal.Neurentericcystsofthespine.JCraniovertebrJunctionSpine.2010Jan-Jun;1(1):58–63.精选ppt22Differentialdiagnosis(1)蛛网膜囊肿:arachnoid

cyst:(2)皮样和表皮样囊肿:dermoidandepidermoidcyst(3)囊性神经鞘瘤:cysticschwannoma(4)血管母细胞瘤:hemangioblastoma:(5)脂肪瘤:lipoma精选ppt23蛛网膜囊肿蛛网膜囊肿:青年人多见,一般位于脊髓背侧髓外硬膜下或硬膜外,信号强度在各序列上均与脑脊液一致,无强化。一般不合并其他先天性畸形。精选ppt24表皮样囊肿M/35years:epidermoidcyst多位于腰骶部髓外硬膜下,信号多不均匀,大多数无强化,精选ppt25皮样囊肿F/35y:dermoidcyst精选ppt26囊性神经鞘瘤囊性神经鞘瘤:一般沿神经根走