脑动静脉畸形--大学课件ppt课件

CEREBRALARTERIOVENOUSMALFORMATIONS AVM aTLAfortheCNS Incidence 0 52 atautopsySlightmalepreponderance 1 09to1 94 Congenitallesions althoughrarelyfamilial Embryology Firsthalfofthirdweekofgestationepiblasticcellsmigratetoformmesodermmesodermalcellsdifferentiatetoarterialandvenousvesselsonthesurfaceoftheembryonicnervoussystem Embryology FirsthalfofthirdweekofgestationepiblasticcellsmigratetoformmesodermmesodermalcellsdifferentaitetoarterialandvenousvesselsonthesurfaceoftheembryonicnervoussystemSeventhgestationalweekvesselssproutbranches penetratedevelopingbrainreachthegray whiteinterface eitherloopbacktopialsurfaceortraverseentireneuraltube thusepicerebral transcerebralcirc neventuallyconnectarterialandvenoussystemsbyaroundthetwelfthweek 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 Yearlyriskofinitialhemorrhage 3 Rebleedinfirstsubsequentyear6 18 reducingto 3 againthereafterPediatricprognosisworsethanadult Spetzler MartinGradingSystem Criteria Score SizeofNidus Small 3cm 1 Medium 3 6cm 2 Large 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 NormalhemisphericvesselsarechronicallymaximallydilatedtoattempttodivertflowfromtheAVMObliterationoftheAVMdivertsallflowtothesemaximallydilatedvesselswh