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AVMATLAFORTHECNSCEREBRALARTERIOVENOUSMALFORMATIONSINCIDENCEN052ATAUTOPSYNSLIGHTMALEPREPONDERANCE109TO194NCONGENITALLESIONSALTHOUGHRARELYFAMILIALEMBRYOLOGYNFIRSTHALFOFTHIRDWEEKOFGESTATIONEPIBLASTICCELLSMIGRATETOFORMMESODERMMESODERMALCELLSDIFFERENTIATETOARTERIALANDVENOUSVESSELSONTHESURFACEOFTHEEMBRYONICNERVOUSSYSTEMEMBRYOLOGYNFIRSTHALFOFTHIRDWEEKOFGESTATIONEPIBLASTICCELLSMIGRATETOFORMMESODERMMESODERMALCELLSDIFFERENTAITETOARTERIALANDVENOUSVESSELSONTHESURFACEOFTHEEMBRYONICNERVOUSSYSTEMNSEVENTHGESTATIONALWEEKVESSELSSPROUTBRANCHESPENETRATEDEVELOPINGBRAINREACHTHEGRAYWHITEINTERFACE,EITHERLOOPBACKTOPIALSURFACEORTRAVERSEENTIRENEURALTUBE,THUSEPICEREBRALTRANSCEREBRALCIRCNEVENTUALLYCONNECTARTERIALANDVENOUSSYSTEMSBYAROUNDTHETWELFTHWEEKPATHOLOGYPATHOPHYSIOLOGYNABSENCEOFNORMALCAPILLARYSYSTEMPATHOLOGYPATHOPHYSIOLOGYNABSENCEOFNORMALCAPILLARYSYSTEMNUSUALFUNCTIONDISPLACEDPATHOLOGYPATHOPHYSIOLOGYNABSENCEOFNORMALCAPILLARYSYSTEMNUSUALFUNCTIONDISPLACEDNASYMPTOMATICATBIRTHPATHOLOGYPATHOPHYSIOLOGYNABSENCEOFNORMALCAPILLARYSYSTEMNUSUALFUNCTIONDISPLACEDNASYMPTOMATICATBIRTHNVESSELSCHANGEWITHTIMENMAYDEVELOPANEURYSMSPATHOLOGYPATHOPHYSIOLOGYNABSENCEOFNORMALNPARENCHYMALCAPILLARYSYSTEMCHANGESWITHINANDNUSUALFUNCTIONAROUNDTHELESIONDISPLACEDNASYMPTOMATICATBIRTHNVESSELSCHANGEWITHTIMENMAYDEVELOPANEURYSMSPATHOLOGYPATHOPHYSIOLOGYNABSENCEOFNORMALNPARENCHYMALCAPILLARYSYSTEMCHANGESWITHINANDNUSUALFUNCTIONAROUNDTHELESIONNSITEFREQUENCYISDISPLACEDNASYMPTOMATICATPROPORTIONALTOBRAINBIRTHVOLUMENVESSELSCHANGEWITHTIMENMAYDEVELOPANEURYSMSCLINICALPRESENTATIONN95HAVESYMPTOMSBYAGEOF70YEARSCLINICALPRESENTATIONN95HAVESYMPTOMSBYAGEOF70YEARSNPEAKPRESENTATIONSECONDTOFOURTHDECADECLINICALPRESENTATIONN95HAVESYMPTOMSBYAGEOF70YEARSNPEAKPRESENTATIONSECONDTOFOURTHDECADEHIGHOUTPUTFAILURE,NEONATE,VEINOFGALENHYDROCEPHALUS,FIRSTDECADEHEADACHE,HEMORRHAGE,SEIZURES,2ND3RDCLINICALPRESENTATIONNFACTORSCONTRIBUTINGTOSYMPTOMSVESSELWALLS,FLOWANDPRESSURESCLINICALPRESENTATIONNFACTORSCONTRIBUTINGTOSYMPTOMSVESSELWALLS,FLOWANDPRESSURESENLARGEMENTANDENCROACHMENTCLINICALPRESENTATIONNFACTORSCONTRIBUTINGTOSYMPTOMSVESSELWALLS,FLOWANDPRESSURESENLARGEMENTANDENCROACHMENTDURALSINUSESCLINICALPRESENTATIONNFACTORSCONTRIBUTINGTOSYMPTOMSVESSELWALLS,FLOWANDPRESSURESENLARGEMENTANDENCROACHMENTDURALSINUSESISCHAEMIACLINICALPRESENTATIONNFACTORSCONTRIBUTINGTOSYMPTOMSVESSELWALLS,FLOWANDPRESSURESENLARGEMENTANDENCROACHMENTDURALSINUSESISCHAEMIACARDIACOUTPUTCLINICALPRESENTATIONDEFICITS10HEADACHES10HEMORRHAGE50SEIZURES30HEMORRHAGENAVMNANEURYSMRUPTURENOTARUPTURERELATEDTOFUNCTIONOFSIZEANEURYSMSIZEHEMORRHAGENAVMNANEURYSMRUPTURENOTARUPTURERELATEDTOFUNCTIONOFSIZEANEURYSMSIZENOMARKEDINCREASEINCREASEWITHWITHEXERCISE,TRAUMAEXERCISE,PREGNANCY,TRAUMAENDPREGNANCYHEMORRHAGENAVMNANEURYSMRUPTURENOTARUPTURERELATEDTOFUNCTIONOFSIZEANEURYSMSIZENOMARKEDINCREASEINCREASEWITHWITHEXERCISE,TRAUMAEXERCISE,PREGNANCY,TRAUMAENDPREGNANCYARTERIOVENOUS,ARTERIAL,THEREFORETHEREFORELESSMORESEVERESEVEREHEMORRHAGENAVMNANEURYSMRUPTURENOTARUPTURERELATEDTOFUNCTIONOFSIZEANEURYSMSIZENOMARKEDINCREASEINCREASEWITHWITHEXERCISE,TRAUMAEXERCISE,PREGNANCY,TRAUMAENDPREGNANCYARTERIOVENOUS,ARTERIAL,THEREFORETHEREFORELESSMORESEVEREMORTALITY3050SEVEREMORTALITY6TO136HEMORRHAGENAVMNANEURYSMRUPTURENOTARUPTURERELATEDTOFUNCTIONOFSIZEANEURYSMSIZENOMARKEDINCREASEINCREASEWITHWITHEXERCISE,TRAUMAEXERCISE,PREGNANCY,TRAUMAENDPREGNANCYARTERIOVENOUS,ARTERIAL,THEREFORETHEREFORELESSMORESEVEREMORTALITY3050SEVEREMORTALITY6TO136HIGHERREBLEEDLOWERREBLEEDMORTALITYRATE13MORTALITYRATE1HEMORRHAGENAVMNANEURYSMRUPTURENOTARUPTURERELATEDTOFUNCTIONOFSIZEANEURYSMSIZENOMARKEDINCREASEINCREASEWITHWITHEXERCISE,TRAUMAEXERCISE,PREGNANCY,TRAUMAENDPREGNANCYARTERIOVENOUS,ARTERIAL,THEREFORETHEREFORELESSMORESEVEREMORTALITY3050SEVEREMORTALITY6TO136HIGHERREBLEEDLOWERREBLEEDMORTALITYRATE13MORTALITYRATE1VASOSPASMCOMMONVASOSPASMRAREHEMORRHAGEAVMNNONETHELESS,RISKOFMAJOR,INCAPACITATING,ORFATALHEMORRHAGEINUNTREATEDLESIONIS40TO50HEMORRHAGEAVMNNONETHELESS,RISKOFMAJOR,INCAPACITATING,ORFATALHEMORRHAGEINUNTREATEDLESIONIS40TO50NYEARLYRISKOFINITIALHEMORRHAGE3NREBLEEDINFIRSTSUBSEQUENTYEAR618,REDUCINGTO3AGAINTHEREAFTERNPEDIATRICPROGNOSISWORSETHANADULTCRITERIASCORESIZEOFNIDUSSMALL3CM1SPETZLERMEDIUM36CM2MARTINLARGE6CM3GRADINGELOQUENCEOFADJACENTBRAINSYSTEMNO0YES1DEEPVASCULARCOMPONENTNO0YES1TREATMENTOPTIONSHSURGICALRESECTIONTREATMENTOPTIONSHSURGICALRESECTIONHENDOVASCULAREMBOLISATIONTREATMENTOPTIONSHSURGICALRESECTIONHENDOVASCULAREMBOLISATIONHSTEREOTATICRADIOSURGERYTREATMENTOPTIONSHSURGICALRESECTIONHENDOVASCULAREMBOLISATIONHSTEREOTATICRADIOSURGERYHMULTIMODALTHERAPYTREATMENTOPTIONSHSURGICALRESECTIONHENDOVASCULAREMBOLISATIONHSTEREOTATICRADIOSURGERYHMULTIMODALTHERAPYHCONSERVATIVEMANAGEMENTNORMALPERFUSIONPRESSUREBREAKTHROUGHTHEORYRFSPETZLERETALNORMALPERFUSIONPRESSUREBREAKTHROUGHTHEORYLOSSOFAUTOREGULATIONANDCARBONDIOXIDEREACTIVITYINPRESENCEOFLARGEARTERIOVENOUSMALFORMATIONNORMALPERFUSIONPRESSUREBREAKTHROUGHTHEORYLOSSOFAUTOREGULATIONANDCARBONDIOXIDEREACTIVITYINPRESENCEOFLARGEARTERIOVENOUSMALFORMATIONNORMALHEMISPHERICVESSELSARECHRONICALLYMAXIMALLYDILATEDTOATTEMPTTODIVERTFLOWFROMTHEAVMNORMALPERFUSIONPRESSUREBREAKTHROUGHTHEORYLOSSOFAUTOREGULATIONANDCARBONDIOXIDEREACTIVITYINPRESENCEOFLARGEARTERIOVENOUSMALFORMATIONNORMALHEMISPHERICVESSELSARECHRONICALLYMAXIMALLYDILATEDTOATTEMPTTODIVERTFLOWFROMTHEAVMOBLITERATIONOFTHEAVMDIVERTSALLFLOWTOTHESEMAXIMALLYDILATEDVESSELSWHICHHAVELOSTTHEIRNORMALCONTROLMECHANISMSNORMALPERFUSIONPRESSUREBREAKTHROUGHTHEORYLOSSOFAUTOREGULATIONANDCARBONDIOXIDEREACTIVITYINPRESENCEOFLARGEARTERIOVENOUSMALFORMATION
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