椎管内肠源性囊肿ppt课件.pptx

1 CASEDISCUSSION 2 Vocabulary hypoalgesia ha p l d i s 痛觉减退Hypoesthesia ha p s i z 感觉迟钝Myodynamia ma da n mj 肌力analsphincter sf kt 肛门括约肌congenitaldysplasia dis pleizi 先天性发育不良spinabifida spa n b f d 脊柱裂 3 CASE1 Patient female 34yearsoldWithwaistpainandtherightlowerextremityradicularpainfor6months Therewasnofever noheadache nosurgery nohistoryoftraumaExamination hypoesthesiaatthefrontofthethigh BilateralLowerlimbsmyodynamiaIV muscletension BilateralBabinskisign Laboratorytests 4 CT 5 T1WIT2WISTIR 6 CE T1WI 7 CASE2 Patient male 32yearsoldNeckandshoulderandbilateralupperlimbspainwithlimbsnumbnessandweaknessformorethan6years Thesymptomswereaggravatingwithlimitedmobilityfor10days Therewasnofever noheadache nosurgery nohistoryoftraumaExamination passiveposture neckmovementwasrestricted doubleupperlimbsapparenthypoalgesia bilateralupperlimbsmyodynamiaII theleftlowerlimbmyodynamiaI therightlowerlimbmyodynamiaII muscletension BilateralHoffmannsign Babinskisyndrome Rectaltouch theanalsphinctercontractionweakness analreflex Laboratorytests 8 CT 9 T1WIT2WICE T1 10 T2WICE T1WI 11 PLEASEGIVEYOUROPINION 12 手术记录case1 13 手术记录case2 14 Intraspinalneurentericcysts 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 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 15 Neurentericcysts MRIofthoracolumbarjunctionwithintradural extramedullaryneurentericcystatT11 a MidsagittalMRIdemonstratesisointenselesion arrow onT1 weightedand b hyperintensesignal arrow onT2 weighted 16 Cause Itspathogenesisisnotclear Itgeneralconsideredasacongenitaldiseaserelatedwithendodermdevelopmentdisorders Duringembryonicdevelopmentperiod partoftheendodermaltissueswasabnormalposteriordisplacementandembeddedintheneuraltube Heterotopicrestsofepitheliumofgastrointestinaltissueleadtoeventualformationofcompressivecysticlesionsofthepediatricandadultspine 17 Pathologicalmechanism Onlycontainingendodermalcomponents similarwiththedigestivetractepithelium Formthecystwallepithelialcellscanbesinglelayerflat cubic columnarepithelium pseudostratifiedcolumnarepitheliumtostratifiedsquamousepithelium alsocanhavemucousgland serousgland muscleandfat Sometimescontainciliaandmucussecretinggobletcells 18 Epidemiology accountfor0 7 1 3 ofspinalaxistumors Mostlyoccursinchildrenandadolescents11days 40yearsMalepredilection proportionofmentowomenas 2 3 1mostoccuredinthesubduralextramedullary90 afewintheepiduralorintramedullary neckorupperthoracicweremostinvolved lumbarandsacralrare Oftenaccompaniedwithcongenitalspinedeformities likespinabifida 19 Imagingfindings MRIshowedaroundorovalcystlesion withsmoothedges thelongaxisdirectionthesameasthespinalcord butalsocanbeirregularinshape Cystresultinginsignificantcompressionofthespinalcord cystwallisthinanduniform T1WIshowsmainlylow equal slightlyhigherthanCSFT2WIshowshigh isosignaltoCSF usuallyhomogeniousSignalstrengthdependsonthecystcontents moreproteinconstituentsorintracystichemorrhage T1WIshowedhighsignalorlowT2WIsignal Spinalcordembeddedsyndrome Noenhancement 20 Diagnosis Thefollowingcharacteristicsshouldbehighlysuspected 1 inmaleadolescents 2 partialnerverootpainorspinalcordcompressionsymptomsasthemainclinicalmanifestations remissionandexacerbationofalternating 3 theMRIdisplaycysticlesionsinthecervicalorthoracicintraspinalsubduralextramedullary cystsignalsimilarwithCSF withthincystwall noenhancementeffect with Spinalcordembeddedsyndrome 4 X rayplainradiographyandCTdisplayothercongenitalmalformations 21 Treatment Surgicaltreatment particularlytotalresectionwithrarerecurrencepartialresection srecurrencerateashighas37 JesseJ Savageetal Neurentericcystsofthespine JCraniovertebrJunctionSpine 2010Jan Jun 1 1 58 63 22 Differentialdiagnosis 1 蛛网膜囊肿 arachnoidcyst 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 23 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 24 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 25 皮样囊肿 F 35y dermoidcyst 26 囊性神经鞘瘤 囊性神经鞘瘤 一般沿神经根走行分布 典型