脑动静脉畸形.ppt

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 normalhemisphericvesselsarechronicallymaximallydilatedtoattempttodivertflowfromtheavmobliterationoftheavmd