2020 Update to classification of chronic venous disorders

The updated CEAP classification remains a discriminative instrument designed to describe the signs and symptomatic status of each limb of a patient with CVD at ... Servier–Phlebolymphology Phlebolymphologyisaninternationalscientificjournalentirelydevotedtovenousandlymphaticdiseases Home Editorialboard Currentissue Archives Issuesbytopic Specialissues Previousissues VEINews Events RCTs/Operativetreatments RCTsInstructions RCTsClassification Searchbyauthors PREVAIT Searchbytopics Searchbyauthors Home 2020Updatetoclassificationofchronicvenousdisorders Downloadthisissue Backtosummary FedorLURIE,MD,PhD JobstVascularInstituteandtheDivision ofVascularSurgeryattheUniversity ofMichigan,AnnArbor, Michigan,USA. Abstract In2017,theAmericanVenousForum(AVF)createdataskforcetodetermineiftheCEAPclassificationneededarevision.Anextensiveliteraturereviewledthetaskforcetoconcludethattherewassufficientevidencetoupdateittoalignwiththenewestknowledgeofchronicvenousdisorder(CVD)andtoclarifyterminology.UsingthemodifiedDelphimethodology,theAVFtaskforceconcludedits2-yearprojectbypublishingtheCEAP2020update,whichalsobecameareportingstandardforstudiesofpatientswithCVD.TheupdatedCEAPclassificationremainsadiscriminativeinstrumentdesignedtodescribethesignsandsymptomaticstatusofeachlimbofapatientwithCVDataspecifictimepoint.TheCEAP2020updateaddedasubclassC4cforcoronaphlebectatica.ThismodificationreflectscurrentunderstandingthatcoronaphlebectaticahasasimilarnaturalhistorytotheC4aandC4bsubclasses.Anotherupdateforthe“C”componentisamodifier“r”describingrecurrentvaricoseveins(C2r)orrecurrentvenousulcer(C6r).Theupdateforthe“E”componentofCEAPincludescreationoftwosubclassesforsecondaryCVD(Es)asfollows:(i)Esi–intravenouscauses;and(ii)Ese–extravenouscauses.Finally,thenumberingofthevenoussegmentsinthe“A”componentoftheCEAPisreplacedbycommonlyusedanatomicalabbreviations. Introduction Classificationsofdiseasesandpathologicalconditionshaveaverylonghistory.Perhapsthefirstpracticalclassificationwasdevelopedin1662byJohnGrauntwhopublishedanindexofcausesofmortality.Acenturylaterin1768,FrançoisBoissierdeLacroixdevelopedasystematicclassificationofallknowndiseasesatthattime.Aroundthesametime,in1780,WilliamCullenpublishedtheclassificationofdiseasethatbecamewidelyusedbyclinicians,especiallyintheUnitedKingdom.Asmultipleclassificationsbegantoemerge,theneedforaunifiedsingleclassificationbecameapparent.WilliamFarrwrotein1839,“theadvantagesofauniformnomenclature,howeverimperfect,aresoobviousasweightsandmeasuresinthephysicalsciences.Itshouldbesettledwithoutdelayandkeptwithoutchange.”Thisneedwasaddressedin1855attheInternationalStatisticalCongressinParis,whereMarkD’EspineandWilliamFarrestablishedthefirstinternationalclassificationofdiseases–acompromisebetweenFarr’sphenotypicalapproachandd’Espine’spathologicalapproachtoclassification. Thisinternationalclassificationofdiseasesisanexampleofadescriptiveclassificationthatdefinesdistinctdiseasesandconditionsforpublichealthandstatisticalpurposes.Clinicalclassificationissimilartodescriptiveclassificationinthatitdefinesdistinctdiseasesorconditions,butperhapsitsgreaterpurposeistostandardizecommunicationamongpractitionersandclinicalresearchers.Asadescriptivetool,clinicalclassificationdefinesdiseasesbasedontheirphenotypicalmanifestations,suchassymptomsandsigns.However,toaddresstheneedsofclinicalpractice,thesediseasedefinitionsshouldbeconnectedtotreatmentoptions.Evolvingknowledgeofthepathologicalmechanismsofdiseasesdoesnotjustifyachangeinaclinicalclassificationuntilthetreatmentoptionstargetingspecificmechanismsbecomeavailable.Oncologicclassificationsexemplifyatransitionfromempiricalphenotypicalclinicalclassificationtomolecularclassificationsofcancerthatarebasedonbothanunderstandingofpathologicalmechanismsandtheavailabilityoftherapeuticstargetingthesemechanisms. CEAPclassificationofCVD ThecurrentunderstandingofCVDsincludesknowledgeofkeypathologicalmechanisms,suchasrefluxandobstruction,thatcanbetargetedbyinterventionsinsomeanatomicallocations.ItalsoincludesempiricalknowledgethatsomeoftheCVDphenotypeshaveasimilarnaturalhistoryandimpactonapatient’squalityoflife.However,thebiologicalandpathologicalbasisforthesephenotypesispoorlyunderstood.Thiscomplexsituationhasrequiredadifferentclassification.Firstintroducedin1996,theClinical-Etiological-Anatomical-Pathophysiological(CEAP)classificationaddressedthecomplexityofCVDbyincorporatingfourdifferenttaxonomicalapproaches.Theclinicalclass“C”isadescriptionofsignsandthesymptomaticstatusofalowerextremity(LE).TheseclinicalclassesarebasedonthemostfrequentlyseenmanifestationsofCVDthatalsohaveasimilarnaturalhistory.The“E”(etiology)oftheCEAPreflectsthecurrentunderstandingofwhatcausesthesignsandsymptomsinanaffectedLE.The“A”ofCEAPdescribeswhichanatomicalsegmentsoftheLEvenoussystemsareaffected.Finally,the“P”(pathophysiology)describesidentifiedhemodynamicabnormalitiesintheaffectedanatomicalsegments.BecauseofthecomplexityassociatedwithCVD,anindividualcomponentoftheCEAPclassificationalonecannotprovideanappropriateclinicaldescriptionofanaffectedLE,butacombinationofthecomponentsgivestheclinicianamorecompleteunderstandingofeachpatient’sdiseaseandguidesthesubsequentclinicalmanagement. EvolvingCEAPclassification:2004revision TheintroductionandwideuseoftheCEAPclassificationhasmadeitpossibletoconductlargeepidemiologicalstudiesanddevelopasetofclinicalpracticeguidelines.1-4GlobaluseofthisuniformclassificationhasledtomultiplecomparablestudiesthathaveprovidednewevidenceandimprovedourunderstandingofCVD.Asnewknowledgehasdeveloped,theclassificationitselfhasrequiredrevisionsandupdates.Thus,significantrevisionoftheCEAPwasdonein2004.5Althoughthatrevisionsubstantiallyimprovedtheclassification,thetransitiontoanewversionofCEAPtookseveralyears.Studiesthatwereinitiatedbeforetherevisioncontinuedtoreporttheirfindingsusingthepreviousversion,whereassomepublicationswereutilizingtherevisedclassification.TheexperiencesuggestedthatfuturerevisionsofCEAPshouldbebackwardcompatible,sotherevisedversionoftheCEAPmayaddmorespecificsubcategoriesbutleavethepreviouscategoriesunchanged. EvolvingCEAPclassification:2020update In2017,theAmericanVenousForum(AVF)createdataskforcetodetermineiftheCEAPneededfurtherrevision.AnextensiveliteraturereviewledthetaskforcetoconcludethattherewassufficientevidencetoupdateittoalignwiththenewestknowledgeofCVDandtoclarifyterminology.Thetaskforcewasextendedtoincludefourgroups,eachgrouptofocusononeofthefourcomponentsoftheCEAP.TheadvisorygroupofexpertswhoparticipatedinthecreationandpreviousrevisionofCEAPwasassembledtoensurecontinuityoftheprocess(TableI).RealizingthatrevisionoftheCEAPisessentiallyaconsensusprocess,themodifiedDelphimethodologywasused.6Duringa2-yearprocesswithmultiplediscussions,severalproposedchangeswererejectedbecausetheyeitherlackedsupportiveevidence,violatedoneofthepredefinedrevisioncriteria,oraffectedpracticalityofusingtheCEAP.TheserejectedproposalsaredescribedintheCEAP2020publication.7 TableI.TheCEAP(clinical,etiological,anatomical,andpathophysiologicalclassification)TaskForceoftheAmericanVenousForum. TheupdatedCEAPclassificationremainsadiscriminativeinstrumentdesignedtodescribethesignsandsymptomaticstatusofeachlimbofapatientwithCVDataspecifictimepoint.ManifestationofCVDchangessignificantlyovertime,sothesamepatientmayhaveadifferentCEAPdescriptionatdifferenttimepoints.Theinterpretationofsuchchangesisbeyondtheabilityofdiscriminatoryinstruments,andtheCEAPcannotandshouldnotbeusedtointerpretthesechangesasimprovementordeterioration.Thesetermsrequireevaluatoryinstrumentscapableofmeasuringthediseaseseverityanditschangeovertimeorasaresultofanintervention.TheVenousClinicalSeverityScore(VCSS)isanexampleofsuchaninstrument. AllfourcomponentsoftheCEAPshouldbetreatedasnominalvariables.Thisincludestheclinicalclass“C”anditssubclasses.ItisnotappropriatetostatethatapatientwithamanifestationofCVDclassifiedasC4hasamoresevereconditionthanapatientclassifiedasC2.ThisalsoappliestothesubclassesoftheCEAP.TheCEAP2020updateaddedasubclassC4cforcoronaphlebectatica.ThismodificationreflectscurrentunderstandingthatcoronaphlebectaticahasasimilarnaturalhistorytotheC4aandC4bsubclasses.Itwasassignedto“c”subclassofC4inordertopreservethepreviousversionofCEAP,sotheC4aandC4bsubclassesremainunchanged.Thisorderofsubclassesreflectsneithertheseverityofdiseasenoradifferentprognosis.Anotherupdateforthe“C”componentisamodifier“r”describingrecurrentvaricoseveins(C2r)orrecurrentvenousulcer(C6r). Theupdateforthe“E”componentofCEAPincludescreationoftwosubclassesforsecondaryCVD(Es).TheCEAP2020separatesintravenousandextravenouscausesoftheEs.Intravenouscausesareconditionsthatarecausedbyvenouswallorvalvedamage.IntravenoussubclassEsiincludesvenouswalland/orvalvedamagecausedbydeep-veinthrombosis(DVT),primaryintravenoussarcoma,orotherintravenouslesions.Extravenouscausesarepathologicalconditionsthataffectvenoushemodynamicslocallyorsystematicallybutarenotlocatedinthevenouswallorvenouslumen.TheextravenoussubclassoftheEsincludesCVDcausedbycongestiveheartfailure,externalveincompression,perivenousfibrosis,musclepumpdysfunction(paraplegia,arthritis,chronicimmobility,frozenankle,orseveresedentarystate),andobesity. CEAP:classificationofCVD,notsyndromes TheCEAPisaclassificationofCVDs,notsyndromes.ThedifferencebecomesclearwhencomparingtheCEAPdefinitionofthesecondaryetiologyofCVDandthedefinitionofthepost-thromboticsyndrome(PTS).AcuteDVTcandamagevenousvalvescausingreflux–whichwillbeclassifiedasEsi;Ad;PrbytheCEAP–orcauseanobstructiontovenousflowbyintravenousorganizedthrombusorsynechia–whichwillbeclassifiedasEsi;Ad;Po.EachofthesedescriptionsarespecifictothesequelaeoftheDVT.Incontrast,thedefinitionofthePTSisbasedonacombinationofsymptomsandsignsthatarenotspecific,andinmorethan50%ofpatients,arenotrelatedtothesequelaeofDVTbutarecausedbypreexistingprimaryCVD.8,9ThismeansthatstudiesthatusethePTSasanoutcome,suchastheSOX(CompressionStockingstoPreventPost-ThromboticSyndrome)andATTRACT(AcuteVenousThrombosis:ThrombusRemovalwithAdjunctiveCatheter-DirectedThrombolysis)trials,aresubjecttosignificantmisclassificationbias. UseofCEAP2020 Aswiththepreviousversions,CEAP2020canbeusedintwodifferentways.TheabbreviatedCEAPliststhehighestclinicalclasswiththesymptomaticstatus(“s”forsymptomatic,“a”forasymptomatic).Thisisfollowedbythedescriptionofetiology(congenital,primary,orsecondary),anatomy(superficial,deep,perforators,ortheircombination),andpathology(reflux,obstruction,ortheircombination).Suchdescriptionsprovideminimuminformationaboutthepatientbutstillmaybesufficientforsomepurposes.ThecompleteCEAPprovidesmorespecificinformationthatisfrequentlysufficientforclinicalmanagementdecision. Forexample,twopatients(Figure1A,B)withahealedulcerintheleftlegcanbedescribedasLLE(leftLE):C5s;Es;Ad;PobytheabbreviatedCEAP.Suchdescriptionindicatesthatbothpatientshaveahealedulcer,aresymptomatic,andhavesecondaryvenousdiseasecausedbyobstructioninthedeepveins.However,thecompleteCEAPdescriptionofthesepatientsmaybeverydifferent.ThefirstpatientisdescribedasLLE:C3,5s;Ese;Ad;PoCIV.Thispatienthasedemaandahealedulcercausedbyextravenousobstructionoftheleftcommoniliacvein(May-ThurnerSyndrome)andrequiresawork-upforpossibleiliacveinangioplastyandstenting.ThesecondpatientisdescribedasLLE:C4b,5s;Esi;Ad;PoFV,POPV.Hehaslipodermatosclerosisandpost-thromboticobstructionoftheleftfemoralandpoplitealveinsandisunlikelytobetreatedsurgically.AcompleteCEAPprovidesalltheinformationthatotherwisewouldbemissed. Figure1.Leftlowerextremities(LLE)oftwopatientswhocanbedescribedasLLE:C5s;Es;Ad;Po.Thecompleteclinical,etiological,anatomical,andpathophysiological(CEAP)classificationforpatientAisLLE:C3,5s;Ese;Ad;PoCIV(edemaandahealedulcercausedbyextravenousobstructionoftheleftcommoniliacvein;May-ThurnerSyndrome).ThecompleteCEAPclassificationforpatientBisLLE:C4b,5s;Esi;Ad;PoFV,PoPV(lipodermatosclerosisandpost-thromboticobstructionoftheleftfemoralandpoplitealveins). Limitations Aswithanyotherinstrument,theCEAPhasanumberoflimitations.FuturerevisionsandupdatesontheCEAPclassificationmayincludesomeoftheproposedmodificationsthathavebeenrejectedbythetaskforce.Itmaybeconsidered,forexample,thatsomeoftheCEAPclassesshouldincludesubcategoriesforthecomplications.Asufficientlevelofevidenceisrequiredforsuchrevisions,includingestablishingtheincidenceofsuchcomplicationsineachofthespecificCEAPclassesandhowtheychangethenaturalhistoryoftheCVD. Conclusions Althoughanimperfectinstrument,theCEAPhasproventobeanessentialtoolforpractitionersandclinicalresearchers.Itsworldwideutilizationsince1996hascontributedtosubstantialprogressinourunderstandingofCVDanddevelopmentofnewtreatmentoptions.Ultimatelyithasledtoimprovedoutcomesinthemanagementofpatientswithvenousdisorders.CEAP2020istheevidence-basedupdateoftheCEAPclassificationthatreflectstheprogressofthefieldofphlebologyduringthelasttwodecades. REFERENCES 1.WronaM,JöckelKH,PannierF,BockE,HoffmannB,RabeE.Associationofvenousdisorderswithlegsymptoms:resultsfromtheBonnVeinStudy1.EurJVascEndovascSurg.2015;50(3):360-367. 2.RobertsonL,LeeAJ,EvansCJ,etal.IncidenceofchronicvenousdiseaseintheEdinburghVeinStudy.JVascSurgVenousLymphatDisord.2013;1(1):59-67. 3.GloviczkiP,ComerotaAJ,DalsingMC,etal;SocietyforVascularSurgery;AmericanVenousForum.Thecareofpatientswithvaricoseveinsandassociatedchronicvenousdiseases:clinicalpracticeguidelinesoftheSocietyforVascularSurgeryandtheAmericanVenousForum.JVascSurg.2011;53(5suppl):2S-48S. 4.O’DonnellTFJr,PassmanMA,MarstonWA,etal;SocietyforVascularSurgery;AmericanVenousForum.Managementofvenouslegulcers:clinicalpracticeguidelinesoftheSocietyforVascularSurgeryandtheAmericanVenousForum.JVascSurg.2014;60(2suppl):3S-59S. 5.EklöfB,RutherfordRB,BerganJJ,etal;AmericanVenousForumInternationalAdHocCommitteeforRevisionoftheCEAPClassification.RevisionoftheCEAPclassificationforchronicvenousdisorders:consensusstatement.JVascSurg.2004;40(6):1248-1252. 6.DalkeyN,HelmerO.AnexperimentalapplicationoftheDelphimethodtotheuseofexperts.ManagSci.1963;9(3):458-467. 7.LurieF,PassmanM,MeisnerM,etal.The2020updateoftheCEAPclassificationsystemandreportingstandards.JVascSurgVenousLymphatDisord.2020;8(3):342-352. 8.GalanaudJP,HolcroftCA,RodgerMA,etal.ComparisonoftheVillaltapost-thromboticsyndromescoreintheipsilateralvs.contralaterallegafterafirstunprovokeddeepveinthrombosis.JThrombHaemost.2012;10(6):1036-1042. 9.NingJ,MaW,FishJ,TrihnF,LurieF.BiasesofVillaltascaleinclassifyingpost-thromboticsyndromeinpatientswithpre-existingchronicvenousdisease.JVascSurgVenousLymphatDisord.2020;21:S2213-S2333.hypertensionandtheinflammatorycascade:majormanifestationsandtriggermechanisms.Angiology.2005;56:S3-S10. 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