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ControversiesintheNeurosurgicalManagementofCerebellar
HemorrhageandInfarction
ArunPaulAmar,M.D.
NeurosurgFocus.201232(4):e1

AbstractandIntroduction
Abstract

Evidencebasedguidelinesforthemanagementofhemorrhagicandischemiccerebellarstrokearesparse,andmostavailable
datacomefromClassIIIstudies.Asaresult,opinionsandpracticesregardingthenatureandroleofneurosurgicalintervention
varywidely.Acomprehensiveliteraturereviewwasconductedtoadjudicateseveralcontentiousissues,suchasthedifferencein
themanagementofcerebellarhemorrhageversusinfarction,criteriaforimagingtoexcludeanunderlyingstructurallesion,the
valueofMRIforpatientselection,theroleofexternalventriculardrainage,theindicationsforoperativemanagement,thetiming
ofsurgicalintervention,andvariousoptionsofsurgicaltechnique,amongothers.Treatmentalgorithmsproposedinseveral
differentstudiesarecomparedandcontrasted.Thisanalysisisconcludedbyasummaryoftherecommendationsfromthe
AmericanStrokeAssociation,whichadvisesthatpatientswithcerebellarhemorrhagewhoexperienceneurologicaldeterioration
orwhohavebrainstemcompressionand/orhydrocephalusduetoventricularobstructionshouldundergosurgicalevacuationof
thehemorrhageassoonaspossible,andthatinitialtreatmentofsuchpatientswithventriculardrainagealoneratherthan
surgicalremovalofthehemorrhageisnotrecommended.
Introduction

ThemanagementofcerebellarhemorrhagehasenduredcontroversyeversinceSirCharlesBallancereportedthefirstsuccessful
surgicalevacuationin1906. [2]Institutionalandindividualvariationsaboundinthemanagementofcerebellarhemorrhage.Toa
degree,thisdebateisduetotherelativelyflimsyqualityofthemedicalliteratureinsupportofdifferentpractices.Forinstance,in
advocatingagainstexternalventriculardrainagealoneinthetreatmentofpatientswithcerebellarhemorrhagewhoare
deterioratingneurologicallyorwhohavebrainstemcompressionand/orhydrocephalusfromventricularobstruction,theAmerican
StrokeAssociationreliesonevidenceratedasLevelC,theweakestcategoryineffectatthetime(consensusopinionofexperts,
casestudies,orstandardofcare). [24]Similarly,theirpriorrecommendationthatangiographyisnotrequiredforolder,
hypertensivepatientswithcerebellarhemorrhageinwhomCTfindingsdonotsuggestastructurallesionisbaseduponLevelV
evidence,theweakestcategoryineffectatthetime(datafromanecdotalcaseseriesonly). [4]
SeveralrandomizedtrialscomparingearlysurgerywithinitialconservativemanagementforICHhavebeenconducted,including
therecentSurgicalTrialinIntracerebralHemorrhage(STICH). [23]Overall,thesestudieshavelargelyshownnobenefitto
surgery,althoughposthocsubgroupanalysisrevealssomeexceptions.PatientswithcerebellarICHhavebeenexcludedfromall
theserandomizedtrials,becauseclinicalequipoisewasnotbelievedtobepresent. [24]Asoneexpertcommentedaboutthe
relatedconditionofcerebellarinfarction,"theresultsofsurgeryhavebeensoconsistentlyfavorableinpatientswhoclearlywere
progressivelydeterioratingthatitseemsfairtosaythatthisisonesurgicalindicationthatdoesnotneedthescrutinyofa
randomizedstudy." [12]
Asaresultofthesebiases,dataprincipallyconsistofuncontrolled,singleinstitutionretrospectivecaseseries(ClassIII
evidence).Collectively,however,thesereportssuggestthatthebenefitofsurgeryisnotsostraightforward.Donaueretal. [7]
reviewed21papersfrom1958to1993andperformedametaanalysiscomparingmedicalversusoperativetreatmentof
cerebellarICH.Inthecohortof357patientswhounderwentsurgery,themortalityratewas49%,whilethatinthe269patients
treatedconservativelywas50%.Similarly,HankeyandHon[10]reviewed8priorseriesofsurgeryforinfratentorialhemorrhage
comprisingatotalof405patients.Onestudysuggestedoverallbenefit,while2studiesreportedbenefitonlyincertainsubgroups
(consciousordrowsybutdeterioratingpatients),andtheremaining5studieswereeitherinconclusiveorshowednobenefitof
surgery.
Inanefforttoprovidemoreconcretenesstothisissueandtoelucidaterelatedconceptsinthemanagementofcerebellar
hemorrhageandinfarction,thisarticlereviewsrelevantstudiesfromthepastcentury.Itbeginswithanoverviewofthe
pathogenesisandnaturalhistory,whichformthefoundationandrationaleforalltreatment.Next,9separateareasofcontroversy
areexploredindetail.ThereviewconcludeswithasummaryoftherecommendationsfromtheAmericanStrokeAssociation,
whosepositionstatementshaveevolvedconsiderablyfromtheirfirstpublicationin1999to2010. [3,4,24]

PathophysiologyandNaturalHistory
Spontaneouscerebellarhematomasrepresentapproximately10%15%ofallICH. [11,18,35]Aswithcerebellarinfarction,
cerebellarhemorrhageoccursmostfrequentlyinthe5ththroughthe8thdecadesoflifeandwithgreaterfrequencyinmalesthan
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infemales. [11,18]Between60%and90%ofallspontaneouscerebellarhemorrhagesoccurinhypertensivepatients.Vascular
malformations,coagulopathies(includingtheuseofanticoagulants),neoplasms,aneurysms,cerebralamyloidangiopathy,and
traumaaccountfortheremainder. [11,17]Inyoungerpatients,underlyingstructuralconditionsaretheprevailingcauses.
Cerebellarinfarctions,ontheotherhand,mayresultfromcardiacemboli,traumaticinjurytothevertebralarteries,andother
causes. [11,14,26]Themajorityofpatientsalsohavehypertension. [13,26]Theinfarctionmostfrequentlyoccursinthevascular
distributionoftheposteriorinferiorcerebellarartery,buttheanteriorinferiorcerebellararteryand/orsuperiorcerebellarartery
territoriescanalsobeinvolved. [14,26]Cerebellarinfarctionsareapproximatelytwothirdsascommonascerebellarhemorrhage.
[11]

Inhypertensivepatients,cerebellarhematomaisbelievedtoresultfromruptureofmicroaneurysms,asfirstproposedbyCharcot
andBouchard,andrecentlyconfirmed. [31]Typically,thesehemorrhagesbeginintheareaofthedentatenucleusandthen
spreadthroughouttheipsilateralhemisphere. [15]Theymayalsoextendacrossthevermistothecontralateralside.Althoughthey
commonlyspreadintothecerebellarpedunclesorruptureintothefourthventricle,onlyrarelydotheydirectlyinvolvethe
brainstem. [11]Dizziness,headache,nausea,vomiting,lossofbalance,anddifficultywalkingarethemostcommonpresenting
symptomsofbothcerebellarhemorrhageandinfarction. [1,11,14,26,30]
Clinicaldeteriorationbefallsupto50%ofpatientswithcerebellarICH. [25]Initsmildform,deteriorationmanifestsasirritability,
confusion,orsomnolence,whilethemoresevereformpresentsascoma,stupor,posturing,andhemodynamicorrespiratory
instabilityduetolossofbrainstemregulation. [13]Thepeakincidenceofdeteriorationis3daysafteronset,althoughitmayoccur
withinhoursorevenweekslater.Whendeteriorationoccurs,mortalityhasbeenreportedtobehigh(25%100%),regardlessof
treatment. [1,7,17,18,30]Deteriorationcanoccurunpredictably,eveninpatientswhoappeartohavereachedaclinicalplateau.
[11,17]Ottetal. [25]reportedthat50%ofpatientswhoremainedalertandrelativelystablefor2daysdegeneratedintocomaover
thecourseofthenextseveraldays,andadisconcerting25%ofpatientswhoremainedawakefor7dayssubsequently
deteriorated.
Thecausesofdeteriorationareproteanandincludeincreasedmasseffectfromsurroundingedemaorexpansionofthe
hematomafromrepeatbleeding.Eithermechanismcancausedirectbrainstemcompression,whichleadstoupwardsherniation
throughthetentorialincisuraordownwardtonsillarherniationthroughtheforamenmagnum.Obstructivehydrocephalus,caused
byintraventricularextensionofthehemorrhageorbycompressionofthefourthventricle,isanothermechanismofclinical
decline.
Incerebellarinfarction,brainswellingresultsfrombothcytotoxicandvasogenicedema.Initially,brainischemiadisruptscell
membraneintegrity,whichcausestheaccumulationofintracellularfluid.Later,vasogenicedemaresultsfromthediffusionof
proteinboundfluidacrossadamagedbloodbrainbarrier. [5,19]Withprogressivemasseffectcausedbytheinfarctand
surroundingedema,brainstemcompressionand/orfourthventriclecompressioncanresult.Therangeoftimethatcanelapse
betweensymptomonsetandfurtherneurologicaldeteriorationistypically17days,withamedianandmodeof3days. [5,13,14]
However,thelikelihoodofdeteriorationhasbeenreportedtobelowerincerebellarinfarct(7%32%)thaninhemorrhage. [1,14]
Whereashydrocephalusandbrainstemcompressioncanbothcausedecreasedlevelofconsciousness,thelatterisallegedto
haveassociatedfocalneurologicalsigns. [11,13,29]Earlycompressionofthedorsalponsresultsinipsilateralsixthnerveparesisof
voluntarylateralgazethatcanbeovercomewithcaloricstimulation.Later,asthecompressionprogresses,conjugategaze
paresisthatisunresponsivetocaloricstimulationoccursfrompressureuponthehorizontalgazecenter.Atthisstage,ipsilateral
peripheraltypefacialparesisisusuallypresentduetoconcomitantcompressionofthefacialcolliculus.Babinskisigns,Horner
syndrome,andhemiparesisarealllatesignsofbrainstemcompression. [11,13,29]Recognitionofthesefindings,alongwith
neuroimaging,canhelpdistinguishbetweenalteredlevelofconsciousnessduetohydrocephalusversusthatduetodirect
brainstemcompression.Appropriatetherapy(forexample,ventriculardrainageversussurgicaldecompression)canthenbe
targetedtotheunderlyingmechanism.
ThetenetsofmedicalmanagementofcerebellarhemorrhagearesimilartothoseofsupratentorialICH. [24]Patientsaregenerally
monitoredinacriticalcaresetting,withfrequentneurologicalassessment.Thosewithseverecoagulationfactordeficiencyor
thrombocytopeniashouldreceivetransfusionofappropriatebloodproductstocorrectthedisorder.Patientswhosehemorrhageis
causedbyoralanticoagulationtherapyshouldreceiveintravenousvitaminKaswellastherapytoreplacethevitaminK
dependentfactors.Prothrombincomplexconcentrateshavenotbeenproventoimproveoutcomecomparedwithfreshfrozen
plasma,butmayhavefewercomplications. [24]RecombinantfactorVIIaisnotroutinelyrecommendedasthesoleagentfor
reversaloforalanticoagulationtherapy. [24]Allpatientsshouldundergointermittentpneumaticcompressionforpreventionof
venousthromboembolisminadditiontoelasticstockings.Afterdocumentationofcessationofbleeding,lowdosesubcutaneous
heparinformulationsmaybeconsideredaswell. [24]Glucoseshouldbemonitoredclosely,andnormoglycemicaisrecommended.
Themanagementofbloodpressureremainsdisputed,withoutclearguidelinesortargetparameters,butinpatientspresenting
withsystolicbloodpressureof150to220mmHg,acuteloweringto140mmHgisprobablysafe. [24]Therapeuticcoolinghasnot
beenadequatelystudiedincerebellarICH,althoughmostpractitionersfavoravoidanceofhyperthermia.
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HemorrhagicversusIschemicCerebellarStroke

SincethefirstreportsofdecompressivesurgeryperformedbyFairburnandOliver[8]andbyLindgren, [20]bothin1956,the
potentialvalueofsuboccipitalcraniectomyandresectionofnecrotictissueincerebellarinfarctionhasbeenrecognized.However,
cerebellarhemorrhageandinfarctionaredistinctentities,whichcallsintoquestionwhetherthesamemanagementprinciples
shouldapplytoeach.
Mathewetal. [21]comparedtheneurosurgicalmanagementof48patientswithcerebellarICHtothatof71patientswith
cerebellarinfarction.Theyfoundthatpatientswithhematomaweremorelikelytobeinacomaandmorelikelytohave
brainstemcompressionuponpresentationthanthosewithinfarction.Thisexplainswhy75%oftheirpatientswithICHrequired
surgery,whileitwasnecessaryinonly24%forinfarction.
Inbothcerebellarhemorrhageandinfarction,perilesionaledemacanaggravatethespaceoccupyingeffectwithintheconfinesof
theposteriorfossa.However,aconditionuniquetoICHisthetoxiceffectsofbloodproductsandassociatedinflammation,which
mightprovideimpetusforitsremovalregardlessofthemechanicalcompressionofadjacenttissue.Furthermore,cerebellar
hemorrhagemayextendintotheventriclesystem,thusprovidinganadditionalmechanismofhydrocephalusbesidesfourth
ventricleeffacement.Conversely,cerebellarICHonlyrarelyextendsdirectlyintothebrainstem. [17]
Bycomparison,cerebellarinfarctiondoesnotleadtointraventricularhemorrhageandisthuslesslikelytocausehydrocephalus
thancerebellarhematoma.InthepatientseriesofAueretal., [1]occlusivehydrocephalusdevelopedin75%ofpatientswith
cerebellarhemorrhagebutonly23%ofthosewithcerebellarinfarction.PatientswithcerebellarICHalsohadahigherincidence
ofhydrocephalusthanthosewithcerebellarinfarctintheseriesbyMathewetal. [21]However,cerebellarinfarctionismorelikely
todirectlyinvolvethebrainstemthancerebellarICHduetosharedvascularterritorythisoccurredin2of40patientsintheseries
ofAueretal. [1]
Emergingdatasuggestthatinsomecircumstances,theareaofrestricteddiffusionapparentonMRI,oncebelievedtorepresent
permanentdamage,maybereversible. [16]Therefore,itisconceivablethatresectionofthispresumednecrotictissuein
cerebellarinfarctionmayactuallycompromiserecovery.
Inlightoftheseconsiderations,apolicythatlimitstheextentofresectionofapparentnecrotictissuetothemininumneededto
achieveadequatedecompressionappearsreasonable,althoughthedatainsupportofthispracticearenotrobust.
CriteriaforCTAngiogramorCatheterAngiogram

Althoughmostcasesofspontaneouscerebellarhemorrhagearetheresultofhypertension,somearecausedbyunderlying
lesions.InKobayashietal.'sseriesof110patients, [18]forexample,5hemorrhagesresultedfromacerebellarAVM,2resulted
fromacerebellartumor,andtheremaining103werebelievedtobecausedbyhypertensiononthebasisofpriorhistoryand/or
negativeangiographicstudies.
Eveninthepresenceofpreexistinghypertension,however,asmanyas36%ofallICHcasesareassociatedwithsecondary
causes. [35]Theindications,nature,anddiagnosticaccuracyofimagingforanunderlyingstructurallesioninspontaneous
cerebellarhemorrhageremainscontroversial.Thepresenceofsubarachnoidblood,calcification,prominentvascularstructures,or
edemaoutofproportiontothesizeandageofthehemorrhagemightsuggestthepresenceofanunderlyinglesion.Similarly,a
hemorrhagethathasanunusual(geographicornoncircular)shapeorislocatedinanunusuallocation,suchasanepicenter
remotefromthedentatenucleus,mightpromptfurtherstudy(Fig.1).However,featuresofCTinisolationhadasensitivityof
only77%andspecificityofonly84%in1study. [9]Clinicalfeaturessuchasageandhistoryofpreexistinghypertensionalsoaffect
thedecisiontopursueadvancedimaging.

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Figure1.

Imagesobtainedina77yearoldwomanwithamedicalhistorysignificantforhypertension,heartmurmur,andCrohndisease
requiringlargedosesofaspirin.Sheawokewithheadache,dizziness,andincoordinationoftherightarm.A:Unenhancedaxial
CTscansofherbrainrevealacutehemorrhageoftherightcerebellumextendingtothetentorialsurfaceandacrossthevermisto
thelefthemisphere.B:Althoughshehadmultipleriskfactorsforspontaneousintracerebralhemorrhage(suchashypertension,
possiblecardiacembolismrelatedtothecauseofherheartmurmur,aspirinuse,andpossibleagerelatedamyloidangiopathy),
theunusuallocationofthehemorrhageandquestionableprominenceofvesselsneartheveinofGalenpromptedfurther
imaging.ThisfrontalviewofaleftvertebralcatheterangiogramrevealsanAVMofthecerebellarvermissuppliedprimarilyby
branchesofthebilateralsuperiorcerebellararteries.Highriskfeaturesincludefeedingarteryaneurysmsbilaterallyandvenous
outflowrestrictionofthedrainingveincoursingtothestraightsinus.C:Thepatientunderwentaseriesofstagedliquidadhesive
embolizationsessionstoprotectagainstrehemorrhage.Thisfrontalviewofleftvertebralcatheterangiographyafterembolization
revealseliminationofthefeedingarteryaneurysmsandsignificantlyreducedflowthroughthenidus.
Halpinetal. [9]performedaprospectiveevaluationofcathetercerebralangiographyintheworkupof102patientswith
spontaneouscerebralhematoma.Bothsupratentorialandcerebellarhemorrhageswereincludedintheanalysis.Overall,an
aneurysmorAVMwasthecauseofthehemorrhagein12.8%ofhypertensivepatientsandin18.2%ofthosewithposteriorfossa
hemorrhage.Theauthorsprospectivelystratifiedthepatientsinto2groups:thosesuspectedtohaveahighlikelihoodofan
underlyingstructurallesionbasedonCTfeatures(Group1)andthosewithoutsuchfindings(Group2).Catheterangiographywas
positiveforAVMoraneurysmin84%ofthehighsuspicioncohortand24%inthelowsuspicionone.
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Inanotherstudy,Zhuetal. [35]reviewed206consecutivepatientswithspontaneousICHtodeterminethediagnosticyieldof
cerebralangiography.Bothsupraandinfratentorialhemorrhageswereincluded.Patientsinwhomseverecoagulopathy
accountedfortheICH,thosewithbleedingintotumor,orthosewithpredominantsubarachnoidhemorrhagewereexcluded.
Overall,theangiographicyieldwassignificantlyhigherinpatientslessthanthemedianageof45yearsandthosewithoutprior
hypertension.In15patientswithposteriorfossahemorrhage,5(33%)werefoundtohaveanunderlyingAVM.All5were
normotensive,andtheoldestpatientinthisgroupwas39.Another6patientshadpreexistinghypertension,theyoungestof
whomwas48.Noneofthose6patientshadpositiveangiography.Theauthorsamalgamatedhemorrhagesintheputamen,
thalamus,orposteriorfossaintoasinglegroupforanalysis.Inthiscollective,theangiographicyieldinpatientswithyoungerage
andwithoutpreexistinghypertensionwas48%,whileinhypertensivepatientstheyieldwas0%.Theyconcludedthatdiagnostic
angiographyshouldnotberoutinelyperformedinpatientswithcerebellarhemorrhageover45yearsoldwithpreexisting
hypertension.
AlthoughCTangiographyandcatheterangiographyarepotentiallyusefulintheworkupofspontaneouscerebellarICH,neitheris
completelyreliable.Insomecases,compressionofadjacentvesselsbythehematomacangivethefalseappearanceofa
vascularmalformation,thusreducingthespecificityofthesetests(Fig.2).Conversely,themasseffectcanconcealanunderlying
lesion,thusreducingthesensitivityofvascularstudiesperformedacutely.IntheseriesofHalpinetal., [9]forexample,followup
angiographyat3monthsshowedanAVMin1of7patientsinthehighsuspiciongroup,eventhoughtheoriginalstudyresults
werenormal.Thus,whenclinicallywarranted,vascularstudiesshouldberepeatedinadelayedfashioneveniftheinitialworkup
isnegative.

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Figure2.

Imagesobtainedina43yearoldmanwithnopriormedicalhistory,whoawokewithheadache,nausea,anddizziness,and
whosebloodpressurewas109/54mmHg.A:AxialCTscanswithoutcontrastdemonstratealargehemorrhageoftheright
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cerebellarhemisphere.B:Becauseofthepatient'syoungageandlackofhypertension,ahighsuspicionforanunderlying
structurallesionledtotheperformanceofaCTangiogram,whichdemonstratesanabundantlyprominentcollectionofvessels
suggestingapossibleAVM.Inretrospect,however,thisfindingwasfoundtomerelyrepresentcompressionofnormalvesselsby
themasseffectofthebleed.C:AxialCTscanaftersurgicalevacuationofthehemorrhagerevealsnofurthermasseffect.D:
FrontalviewofavertebralarterycatheterangiogramconfirmstheabsenceofanunderlyingAVM.E:Lateralviewofavertebral
arterycatheterangiogramconfirmstheabsenceofanunderlyingAVM.
RoleofExternalVentricularDrainage

Theindicationsforventriculardrainageincerebellarhemorrhageandinfarctionremaincontested.Manyproposethat
hydrocephalusresultingfromfourthventricleobstructionshouldbetreatedwithsurgicaldecompressionratherthanCSF
diversion. [5,17,33]Amongthisgroup,someadvocateforpreorintraoperativeplacementofaventricularcatheter,incasethe
decompressionfailstoachievereconstitutionofCSFpathways,whileothersrenounceventriculardrainagealtogether.One
argumentinfavorofthisapproachisthatdecompressivesurgerycanshortenthedurationofCSFdiversionandreducetheneed
forapermanentshunt. [5]Inmanycases,thecathetercanberemovedwithin72hoursaftersurgery. [5]IntheseriesofMathewet
al., [21]nopatientrequiredexternalventriculardrainageordelayedshuntplacementafterinitialtreatmentwithcraniectomy.
Conversely,othersarguethattheeffectsofhydrocephaluscanbefalselyinterpretedasresultingfrombrainstemcompression.
TheyadvocateliberaluseofCTimagingtodistinguishbetweenthetwoandinstitutionofventriculardrainageifhydrocephalusis
presentandthereisanyclinicalsignofelevatedintracranialpressure. [7,17,18,26,29]Inthisalgorithm,decompressionofthe
brainstemfollowsonlyifthepatientdoesnotimprovewithreliefofthehydrocephalus.
Thethresholdofdrainageisanotherdebatedissue,duetotheriskofupwardherniationcausedbyalteringthepressuregradient
acrossthetentorium.Thisphenomenonispurportedtohaveoccurredin2of30patientsintheseriesofvanLoonetal., [30]
althoughothersbelievethatconcernforthiseventisoverstated. [4,26,29]Conventionalneurosurgicaltrainingadvisesdrainageat
nolessthan1520mmHgabovethelevelofthethirdventricle. [7]
ThesedebatesbeganinthepreCTera,whenattributionoftheunderlyingcauseofclinicaldeterioration(hydrocephalusvs
brainstemcompression)waslefttoclinicalexaminationalone.In1960,McKissocketal. [22]reported34casesofcerebellar
hemorrhage.Ninepatientsweretreatedwithventriculardrainage,andallofthemdied.Insomeofthesepatients,sudden
declineafterventriculardeteriorationwashypothesizedtobeduetoupwardherniation,althoughthiswasnotconfirmed.
Conversely,ofthe14patientstreatedwithcraniectomyandhematomaresection,9survived.
Inmorerecentseries,however,thepotentialvalueofventriculardrainagehasbeenaffirmed.In2003,Racoetal. [26]reviewed
44patientswithcerebellarinfarction.Of17patientswhodeterioratedclinicallyandrequiredintervention,13patientswith
hydrocephalusunderwenttreatmentwithventriculardrainage,whiletheremaining4withouthydrocephalusunderwent
craniectomy.Ofthe13initiallymanagedusingCSFdiversion,5requiredsubsequentcraniectomy,while8wereabletobe
treatedwithventriculardrainagealone.Similarly,intheseriesofHornigetal., [13]of10patientswithcerebellarinfarctionand
clinicaldeteriorationinitiallytreatedwithventriculostomy,only4requiredsecondarycraniectomybecauseofcontinueddecline.
Outcomeconcerningmortalityandfunctionalstatuswasnotdifferentwhenresultsofexternalventriculardrainageand
suboccipitalcraniectomywerecomparedinthisstudy. [13]IntheseriesofvanLoonetal., [30]secondarycraniectomywas
necessaryinonly6(20%)of30patientswithcerebellarICH,whileinotherseriesthepercentageofpatientsrequiring
craniectomybecauseofdeteriorationorfailuretoimproveafterventriculardrainagerangesfrom25%to80%. [1,13,16,30]Mathew
etal. [21]foundthatmorethanhalfoftheirpatientswithcerebellarICHwhowereinitiallytreatedwithventriculardrainage
subsequentlyrequiredcraniectomy,incontrasttoonly2(18%)of11patientswithcerebellarinfarction.
Insummary,whilesomepatientswithcerebellarICHandstrokewhodeteriorateneurologicallyanddevelophydrocephalushave
beensuccessfullymanagedwithexternalventriculardrainagealone,othersstillrequiresurgery.Asdiscussedbelow,the
AmericanStrokeAssociationfavorsoperativeresectionoftheICHinthisscenario.
IndicationsforCraniotomy

InthemanagementofcerebellarICHandinfarction,theindicationsforoperativeinterventionremainthesupremecontroversy.
Someauthorsinvokeasizethreshold,typically3or4cm,abovewhichtheyrecommendsurgicalevacuationofthehemorrhage
regardlessofclinicalstatus.Othersusethecriteriaofradiographicevidenceofbrainstemcompressionorcisternaleffacement,
whichaccountsforsurroundingedemainadditiontothesizeoftheICHorinfarctindeterminingoverallmasseffect.For
instance,Tanedaetal. [28]reported75casesofspontaneouscerebellarICHandclassifiedtheappearanceofthequadrigeminal
cisterninto3groups:GradeI(normal),GradeII(compressed),andGradeIII(absent).Goodoutcomeswerereportedin88%,
69%,and0%ofGradeI,II,andIIIcases,respectively.However,theynotedthatthesizeofthehematomawasunrelatedtothe
degreeofcisternalcompression,pointingouttheinfluenceofedemaorhydrocephalusonoverallmasseffect.Thepredictive
valueofquadrigeminalcisterncompressionwasconfirmedintheseriesofvanLoonetal., [30]andpatientswithtotallyobliterated
cisternshadpooroutcomesregardlessoftreatment.
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Stillothersdiscounttheseradiographicfeaturesandemphasizetheneurologicalexamination,includinglevelofconsciousness
andbrainstemreflexes,indeterminingcriteriaforsurgery.Kobayashietal. [18]performedaretrospectivereviewof52patients
withhypertensivecerebellarICH.Onthebasisofthisanalysis,theyproposednewcriteriaforinterventionthatwereprospectively
appliedtothenext49patientsforvalidationandconfirmation.PatientswithGCSscoresof14or15andwithhematomasizes
lessthan4cminmaximaldiameterweretreatedconservatively,whilepatientswithGCSscoresof13orlessatadmissionor
withahematomameasuringgreaterthan4cmunderwentsurgicalevacuation.Forpatientswithflaccidtetraplegiaandabsent
brainstemreflexes,intensivetherapywasnotrendered.
Kirollosetal. [17]developedadifferentprotocol,basedoncompressionofthefourthventricleasameasureofmasseffect,which
theyappliedprospectivelyinthemanagementof50consecutivepatientswithcerebellarICH.Theappearanceofthefourth
ventriclewasdividedinto3groups:GradeI(normalsizeandconfiguration),GradeII(partiallycompressedandshifted),and
GradeIII(completelyobliterated).TheICHwasevacuatedforallpatientswithGradeIIIcompressionandforpatientswithGrade
IIcompressionwhentheGCSscoredeterioratedintheabsenceofuntreatedhydrocephalus.PatientswithGradeIorII
compressionwereinitiallytreatedusingonlyventriculardrainageiftheydevelopedhydrocephalusandclinicaldeterioration.
StableGradeIandIIpatientsweremanagedconservatively.Acutedeteriorationtocomatosestateoccurredin6(43%)ofthe14
patientswithGradeIIIcompressionwhowereconsciousatpresentationnoneofthemexperiencedgoodoutcomes.However,15
(60%)of25patientswithhematomasgreaterthan3cmandGradeIorIIcompressiondidnotrequireclotevacuation.
Insummary,clinicalconsiderationsshouldcomplementradiographicappearanceinthemanagementalgorithm,anddecisionsfor
surgicalinterventionshouldrarelybemadeonthebasisofimagingfindingsalone.
TimingofSurgicalIntervention

Evidenceconsistentlyshowsthatpostoperativeoutcomesgenerallycorrelatewithpreoperativestatus. [6,17]Forinstance,inthe
seriesofOttetal., [25]themortalityratewas17%forpatientswhowereconsciousatthetimeofsurgeryand75%forthosewho
wereunconscious.Similarly,intheseriesofDonaueretal., [7]patientspresentingwithGCSscoreslessthan6hada60%
mortalityrateandKarnofskyOutcomeIndextotalof26,whilethosewithGCSscoresgreaterthan10hadonlya20%mortality
rateandaKarnofskyOutcomeIndextotalof66.IntheseriesofKobayashietal., [18]ofthe5patientswithGCSscoresof4,3
diedand2remainedvegetativedespitesurgery.IntheseriesofvanLoonetal., [30]patientswithtotalobliterationofthe
quadrigeminalcisternhadapooroutcomeirrespectiveoftreatment.
Furthermore,manypatientswhoexperienceclinicaldeteriorationimprovesignificantlyaftersurgery.Somehaveevenbeen
restoredtofunctionalcapacity.Onthisbasis,itisnaturaltoquestionwhetherpatientswhoremaindependentaftersurgerywould
havefaredbetterifinterventionhadbeenperformedearlierintheircourse. [12,19]
Forthesereasons,manyrecommendsurgerypriortoclinicaldeterioration. [12]Yoshidaetal. [34]emphasizetheimportanceof
surgicaltherapyevenforalertpatientsifthehematomaislargerthan3cmtoavoiddelayeddeterioration.Similarly,intheseries
ofKirollosetal., [17]nopatientwithGradeIIIcompressionofthefourthventriclewhowasconsciousattheoutsetbutthen
developedaGCSscorelessthan8experiencedagoodoutcome.Theseauthorsthusadvocateaggressiveearlysurgical
evacuationofthehematomaforallGradeIIIpatients,regardlessofinitialexaminationresults,beforedeteriorationoccurs.
Conversely,Dammannetal. [6]reviewedtheirseriesof57patientswhounderwentsurgicalevacuationofspontaneouscerebellar
ICH.Theinitialneurologicalconditionprovedtobehighlypredictiveofoutcome.Basedontheexcellentresultsinpatientswith
goodinitialclinicalconditionwhounderwentsurgeryduetosecondarydeterioration,thisgroupadvisesagainstthepreventive
evacuationofcerebellarhemorrhage.Similarly,intheGermanAustrianCerebellarInfarctionStudy,84patientswithmassive
cerebellarinfarctionwereprospectivelyobservedafterassignmentto1of3groups:craniotomyandevacuation,ventriculostomy,
ormedicaltherapyalone.Treatmentwaslefttothediscretionoftheproviderinthisunrandomizedtrial.Inalogisticregression
model,therewasfoundtobenobenefittoperformingsurgeryinpatientswhohadnotyetdeterioratedtocoma. [14]
DespitethefocusonearlyevacuationofcerebellarICH,evendelayedremovalmighthavebenefit.Aueretal. [1]reported2
patientswithcerebellarhemorrhageandfocalsignsofaposteriorfossalesion.Becausetheyhadnoimpairmentof
consciousness,theywereinitiallymanagedconservatively.Becausetheirsymptomshadnotshownatendencytoimprove,
however,theyunderwentevacuationofthehematomaperformedonDays23and54,respectively.Bothpatientsthenrecovered
fullywithoutneurologicaldeficit.
WhatConstitutesFutility?

Asstated,evidenceconsistentlyshowsthatpostoperativeoutcomesgenerallycorrelatewithpreoperativestatus.However,there
arenumerousanecdotalaccountsofgoodpostoperativeoutcomeamongcomatosepatients. [11,12]Insomecases,evenpatients
withfixedanddilatedpupilsorotherabsentbrainstemreflexeshaverecovered. [32]IntheseriesofHornigetal., [13]38%of
comatosepatientsachievedagoodrecovery(nondisabledathospitaldischarge)afterdecompressivesurgery.IntheGerman
AustrianCerebellarInfarctionStudy,halfofallpatientswhodeterioratedintocomaandweretreatedwithventriculardrainageor
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decompressivecraniotomyexperiencedameaningfulrecovery(modifiedRankinscoreof2orless). [14]Similarly,Kobayashietal.
[18]reported2patientswithrupturedcerebellarAVMswhohadflaccidtetraplegiaandapneaatadmission.Afteremergency
surgery,bothrecoveredtoenjoy"ausefullife." [18]
Furthermore,pathoanatomicalstudiesrevealsurprisinglyfewstructuralchangesduetobrainstemcompressioninpatientswith
fatalspaceoccupyingcerebellarinfarcts. [27]Similarly,thereisnointrinsicdamagetosupratentorialtelencephalicstructuresin
cerebellarICHandinfarct,whichsuggeststhepossibilityoffullintellectualandcognitiverecoveryinsomecases. [7,12]Inlightof
theseconsiderations,itisreasonabletoquestionwhetheranypatient'sconditionis"toopoor"toforegosurgicalinterventionand
whatconstitutesfutiletreatment.Fromapracticalstandpoint,surgerymightbeconsidered,evenifthesituationappears
"hopeless."
ValueofPreoperativeMRI

Yanakaetal. [33]studiedtheprognosticvalueofpostoperativeMRIin31patients,allwithGCSscoresof8orless,who
underwentsurgicalevacuationofcerebellarICH.Thepatientsweredividedinto2groupsbasedonoutcome.Goodrecoveryor
onlymoderatedisabilitywasachievedin8patients,whiletheremaining23diedorbecameseverelydisabled/vegetative.There
werenosignificantdifferencesbetweenthe2groupsinpreoperativeCTfindingssuchashematomasize,presenceof
hydrocephalus,fourthventricularcompression,orobliterationoftheperimesencephaliccistern.However,theincidenceofhigh
signalintensityintheponsandmidbrainonT2weightedMRI,indicatingbrainstemdamage,wassignificantlyhigherinthepoor
outcomegroup.TheseintriguingresultsraisethequestionofwhetherpreoperativeMRIcanbeusedasapredictivetoolto
screenpatientsforbrainsteminjury,thusimprovingpatientselectionforaggressivetherapy.However,nostudyhasyet
addressedthisissue,possiblybecauseoflogisticalimpedimentstoperformingMRIscansacutelyincriticallyillpatients.The
absenceofbrainsteminjury,confirmedbypreoperativeMRI,mightprovideimpetusforsurgicalinterventioninpatientswho
otherwisemighthavebeenconsidered"hopeless."
TechnicalAspectsofSurgery

Numeroustechnicalconsiderationsintheoperativemanagementofcerebellarhemorrhageandinfarctionremainintherealmof
individualpreference.Theseincludethesizeofthesuboccipitalboneremovedandwhethertofixatetheboneflap(craniotomy)
orfloatitorabandonit(craniectomy)attheendoftheprocedure.Otheradjunctssuchastheremovalofthearchofthefirst
cervicalvertebraremainoptional.IntheGermanAustrianCerebellarInfarctionStudy,forinstance,decompressivesurgery
consistedofalargecraniotomy,duraplasty,andresectionoftheposterioratlasarchiftonsillarherniationwasapparent,but
resectionofnecrotictissuewasnotmandatory. [14]Inotherseries,however,craniectomywithresectionoftheinfarctedtissue
wasapplied,includingpossibleresectionofcerebellartonsils. [13,25]Oneriskoftoolargeacraniectomyissubsequentsaggingof
thecerebellarhemispheres.Conversely,aboneflapthatistoosmallandthenreplacedmayfailtoachieveadequate
decompression(Fig.3).Becausethedegreeofmasseffectisdifferentineachpatient,intraoperativejudgementmustbe
exercisedindeterminingtheextentofboneremovalnecessarytoachievedecompression,andnorigidguidelinescanbeoffered
aboutaprespecifiedsizethreshold.

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Figure3.

Imagesobtainedina56yearoldwomanwithmitralregurgitation,whounderwentcardiacsurgeryanddevelopeddecreased
consciousness1daylater.A:AxialCTscansofthebrainwithoutcontrastdemonstratealargeinfarctionoftherightcerebellar
hemispherewithmasseffectandhydrocephalus.B:Duetoprogressiveobtundation,thepatientunderwentsuboccipital
craniotomyandpartialremovalofinfarctedtissue.Theboneflapwasreplacedandleftfloating.ThesepostoperativeaxialCT
scansrevealpersistentmasseffect.C:PostoperativediffusionweightedaxialMRimagesdemonstratepersistent,widespread
infarctedtissueandmasseffect.D:MagneticresonanceimagingperformedonpostoperativeDay3.AxialFLAIRsequence
(upperleft)showspersistentedemaandmasseffect.Axialgradientechosequence(uppercenterandright)showshemorrhagic
transformation.SagittalT1weightedimages(lowerrow)showascendingtranstentorialherniationandtonsillarherniationthrough
theforamenmagnum.Thepatientremainedsymptomaticforthenext2weeks,suggestingthatthebonedecompressionand/or
removalofinfarctedtissuewasinsufficient.
RoleofOtherInterventions

Inlieuofsuboccipitalcraniectomyandevacuationofthehemorrhage,severalothersurgicalapproacheshavebeenproposedfor
themanagementofcerebellarICH,includingstereotacticaspiration,endoscopicburholeevacuation,andlocalinfusionofa
thrombolyticagentsuchastissueplasminogenactivator. [1]Dataregardingthesafetyandefficacyoftheseproceduresare
lacking,andtheyarecurrentlynotconsideredmainstreamtherapy.

AmericanStrokeAssociationGuidelines
RecognizingthatthemanagementofICHbyneurologistsandneurosurgeonsthroughouttheworldvariesgreatly,theStroke
CounciloftheAmericanHeartAssociationformedataskforcetodeveloppracticeguidelinesandtosuggestareaswherefurther
researchwasneeded.In1999,thefirstguidelineswerepublished,althoughtheauthorsacknowledgedthatthestrengthoftheir
recommendationswaslimitedbythequalityofthemedicalliterature,whichconsistsmoreofanecdotalcaseseriesthanwell
designedclinicaltrials. [4]Sincethen,theguidelineshaveundergoneupdatesin2007and2010. [3,24]Besidestheevolutionofthe
positionstatements,theseupdatesreclassifiedthelevelofcertaintyofthetreatmenteffectandrecategorizedtheclassof
evidencefromwhichtheyarederived.
RegardingtheindicationsforvascularimagingtosearchforanunderlyingstructuralcauseoftheICH,the1999Councilwrote,
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"Angiographyshouldbeconsideredforallpatientswithoutaclearcauseofhemorrhagewhoaresurgicalcandidates,particularly
young,normotensivepatientswhoareclinicallystable(levelofevidenceV,gradeCrecommendation)." [4]Additionally,they
wrote,"Angiographyisnotrequiredforolderhypertensivepatientswhohaveahemorrhageinthebasalganglia,thalamus,
cerebellum,orbrainstemandinwhomCTfindingsdonotsuggestastructurallesion.(levelofevidenceV,gradeC
recommendation)." 4Underthedefinitionsineffectatthattime,theseweretheweakestpossiblerecommendationsandbasedon
thelowestqualitydata.In2010,theguidelinesstatethat,"CTangiography,CTvenography,contrastenhancedCT,contrast
enhancedMRI,magneticresonanceangiography,andmagneticresonancevenographycanbeusefultoevaluateforunderlying
structurallesions,includingvascularmalformationsandtumorswhenthereisclinicalorradiologicalsuspicion(ClassIIaLevelof
Evidence:B)." [24]
RegardingtheindicationsforsurgicalremovalofICH,the1999councilwrote: [4]
Patientswithcerebellarhemorrhage>3cmwhoareneurologicallydeterioratingorwhohavebrainstemcompressionand
hydrocephalusfromventricularobstructionshouldhavesurgicalremovalofthehemorrhageassoonaspossible(levelsof
evidenceIIIthroughV,gradeCrecommendation)Stereotacticaspirationmaybeassociatedwithbetteroutcomesthan
standardcraniotomyformoderatesizedcerebellarhemorrhages,butthishypothesishasyettobetestedinarandomized
study(norecommendation).
In2007,therewasnochangetothisrecommendation,althoughtheauthorsrevisedthecategorizationofitsstrengthas"Class
1,LevelofEvidenceB,"whichisanintermediategrade. [3]In2010,thequalificationofthe3cmsizethresholdwasabandoned,
andanewrecommendationconcerningventriculardrainagewasoffered: [24]
Patientswithcerebellarhemorrhagewhoaredeterioratingneurologicallyorwhohavebrainstemcompressionand/or
hydrocephalusfromventricularobstructionshouldundergosurgicalremovalofthehemorrhageassoonaspossible(Class1
LevelofEvidence:B).(Revisedfromthepreviousguideline).Initialtreatmentofthesepatientswithventriculardrainage
aloneratherthansurgicalevacuationisnotrecommended(ClassIIILevelofEvidence:C).(Newrecommendation).

Conclusions
Themanagementofcerebellarhemorrhagicandischemicstrokeiscontroversial.Issuessuchasthedifferenceinthetreatment
algorithmofcerebellarICHversusinfarction,criteriaforimagingtoexcludeanunderlyingstructurallesion,thevalueofMRIfor
patientselection,theroleofexternalventriculardrainage,theindicationsforoperativemanagement,thetimingofsurgical
intervention,andvariousoptionsofsurgicaltechniqueremainunresolved.Professionalsocietyguidelinesfortheseconsiderations
aresparseandbasedonrelativelypoorqualitydata.Nonetheless,thepotentialvalueofneurosurgicalinterventionremainswell
established.
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Abbreviationsusedinthispaper
AVM=arteriovenousmalformationGCS=GlascowComaScaleICH=intracerebralhemorrhage.
NeurosurgFocus.201232(4):e12012AmericanAssociationofNeurologicalSurgeons

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