脑动静脉畸形--大学精ppt课件

CEREBRALARTERIOVENOUSMALFORMATIONS,AVM:aTLAfortheCNS,Incidence,0.52%atautopsySlightmalepreponderance(1.09to1.94)Congenitallesions(althoughrarelyfamilial),Embryology,Firsthalfofthirdweekofgestationepiblasticcellsmigratetoformmesodermmesodermalcellsdifferentiatetoarterialandvenousvesselsonthesurfaceoftheembryonicnervoussystem,Embryology,FirsthalfofthirdweekofgestationepiblasticcellsmigratetoformmesodermmesodermalcellsdifferentaitetoarterialandvenousvesselsonthesurfaceoftheembryonicnervoussystemSeventhgestationalweekvesselssproutbranches&penetratedevelopingbrainreachthegray-whiteinterface,eitherloopbacktopialsurfaceortraverseentireneuraltube,thusepicerebral&transcerebralcircneventuallyconnectarterialandvenoussystemsbyaroundthetwelfthweek,Pathology&Pathophysiology,absenceofnormalcapillarysystem,Pathology&Pathophysiology,absenceofnormalcapillarysystemusualfunctiondisplaced,Pathology&Pathophysiology,absenceofnormalcapillarysystemusualfunctiondisplacedasymptomaticatbirth,Pathology&Pathophysiology,absenceofnormalcapillarysystemusualfunctiondisplacedasymptomaticatbirthvesselschangewithtimemaydevelopaneurysms,parenchymalchangeswithinandaroundthelesion,Pathology&Pathophysiology,absenceofnormalcapillarysystemusualfunctiondisplacedasymptomaticatbirthvesselschangewithtimemaydevelopaneurysms,parenchymalchangeswithinandaroundthelesionsitefrequencyisproportionaltobrainvolume,Pathology&Pathophysiology,absenceofnormalcapillarysystemusualfunctiondisplacedasymptomaticatbirthvesselschangewithtimemaydevelopaneurysms,Clinicalpresentation,95%havesymptomsbyageof70years,Clinicalpresentation,95%havesymptomsbyageof70yearspeakpresentationsecondtofourthdecade,Clinicalpresentation,95%havesymptomsbyageof70yearspeakpresentationsecondtofourthdecadehighoutputfailure,neonate,veinofGalenhydrocephalus,firstdecadeheadache,hemorrhage,seizures,2nd&3rd,Clinicalpresentation,factorscontributingtosymptomsvesselwalls,flowandpressures,Clinicalpresentation,factorscontributingtosymptomsvesselwalls,flowandpressuresenlargementandencroachment,Clinicalpresentation,factorscontributingtosymptomsvesselwalls,flowandpressuresenlargementandencroachmentduralsinuses,Clinicalpresentation,factorscontributingtosymptomsvesselwalls,flowandpressuresenlargementandencroachmentduralsinusesischaemia,Clinicalpresentation,factorscontributingtosymptomsvesselwalls,flowandpressuresenlargementandencroachmentduralsinusesischaemiacardiacoutput,Clinicalpresentation,Hemorrhage,AVMrupturenotafunctionofsize,Aneurysmrupturerelatedtoaneurysmsize,Hemorrhage,AVMrupturenotafunctionofsizenomarkedincreasewithexercise,pregnancy,trauma,Aneurysmrupturerelatedtoaneurysmsizeincreasewithtraumaexercise,endpregnancy,Hemorrhage,AVMrupturenotafunctionofsizenomarkedincreasewithexercise,pregnancy,traumaarteriovenous,thereforelesssevere,Aneurysmrupturerelatedtoaneurysmsizeincreasewithtraumaexercise,endpregnancyarterial,thereforemoresevere,Hemorrhage,AVMrupturenotafunctionofsizenomarkedincreasewithexercise,pregnancy,traumaarteriovenous,thereforelessseveremortality6to13.6%,Aneurysmrupturerelatedtoaneurysmsizeincreasewithtraumaexercise,endpregnancyarterial,thereforemoreseveremortality30-50%,Hemorrhage,AVMrupturenotafunctionofsizenomarkedincreasewithexercise,pregnancy,traumaarteriovenous,thereforelessseveremortality6to13.6%lowerrebleedmortalityrate(1%),Aneurysmrupturerelatedtoaneurysmsizeincreasewithtraumaexercise,endpregnancyarterial,thereforemoreseveremortality30-50%higherrebleedmortalityrate(13%),Hemorrhage,AVMrupturenotafunctionofsizenomarkedincreasewithexercise,pregnancy,traumaarteriovenous,thereforelessseveremortality6to13.6%lowerrebleedmortalityrate(1%)vasospasmrare,Aneurysmrupturerelatedtoaneurysmsizeincreasewithtraumaexercise,endpregnancyarterial,thereforemoreseveremortality30-50%higherrebleedmortalityrate(13%)vasospasmcommon,Hemorrhage-AVM,Nonetheless,riskofmajor,incapacitating,orfatalhemorrhageinuntreatedlesionis40to50%,Hemorrhage-AVM,Nonetheless,riskofmajor,incapacitating,orfatalhemorrhageinuntreatedlesionis40to50%Yearlyriskofinitialhemorrhage3%Rebleedinfirstsubsequentyear6-18%,reducingto3%againthereafterPediatricprognosisworsethanadult,Spetzler&MartinGradingSystem,Criteria,Score,SizeofNidus,Small(6cm),3,EloquenceofAdjacentBrain,No,0,Yes,1,DeepVascularComponent,No,0,Yes,1,TreatmentOptions,SurgicalResection,TreatmentOptions,SurgicalResectionEndovascularEmbolisation,TreatmentOptions,SurgicalResectionEndovascularEmbolisationStereotaticRadiosurgery,TreatmentOptions,SurgicalResectionEndovascularEmbolisationStereotaticRadiosurgeryMultimodalTherapy,TreatmentOptions,SurgicalResectionEndovascularEmbolisationStereotaticRadiosurgeryMultimodalTherapyConservativeManagement,NormalPerfusionPressureBreakthroughTheory,R.F.Spetzleretal,Normalperfusionpressurebreakthroughtheory,Lossofautoregulationandcarbondioxidereactivityinpresenceoflargearteriovenousmalformation.,Normalperfusionpressurebreakthroughtheory,Lossofautoregulationandcarbondioxidereactivityinpresenceoflargearteriovenousmalformation.NormalhemisphericvesselsarechronicallymaximallydilatedtoattempttodivertflowfromtheAVM,Normalperfusionpressurebreakthroughtheory,Lossofautoregulationandcarbondioxidereactivityinpresenceoflargearteriovenousmalformation.NormalhemisphericvesselsarechronicallymaximallydilatedtoattempttodivertflowfromtheAVMObliterationoftheAVMdivertsallflowtothesemaximallydilatedvesselswhichhavelosttheirnormalcontrolmechanisms,Normalperfusionpressurebreakthroughtheory,Lossofautoregulationandcarbondioxidereactivityinpresenceoflargearteriovenousmalformation.NormalhemisphericvesselsarechronicallymaximallydilatedtoattempttodivertflowfromtheAVMObliterationoftheAVMdi