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Primary(spontaneous)upperextremitydeepveinthrombosis

OfficialreprintfromUpToDate
www.uptodate.com2016UpToDate

Primary(spontaneous)upperextremitydeepveinthrombosis
Author
KaoruGoshima,MD

SectionEditors
JohnFEidt,MD
JosephLMills,Sr,MD

DeputyEditor
KathrynACollins,MD,PhD,FACS

Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Feb2016.|Thistopiclastupdated:Feb18,2016.
INTRODUCTIONPrimary,"spontaneous"upperextremitydeepveinthrombosisisrareandisdefinedas
thrombosisofthedeepveinsdrainingtheupperextremityduetoanatomicabnormalitiesofthethoracicoutlet
causingaxillosubclaviancompressionandsubsequentthrombosis.Thesyndromeisappropriatelytermedvenous
thoracicoutletsyndrome,butisalsoreferredtoasPagetSchroettersyndrome,andalternativelyaseffort
thrombosis[1].Ittypicallypresentsinyoung,otherwisehealthyindividualsassudden,severeupperextremity
painandswellingfollowingvigorousupperextremityactivity.Anaggressivetreatmentapproachthatincludes
anticoagulation,catheterdirectedthrombolysisandthoracicoutletdecompressionisaimedatrelievingacute
symptoms,andminimizingcomplicationsincludingrecurrentthromboembolismandpostthromboticsyndrome.
Theepidemiology,riskfactors,pathophysiology,clinicalfeatures,diagnosisandtreatmentofprimary
(spontaneous)upperextremityvenousthrombosiswillbereviewedhere.Catheterinducedupperextremityvenous
thrombosisandlowerextremitydeepveinthrombosisarediscussedelsewhere.(See"Catheterrelatedupper
extremityvenousthrombosis"and"Approachtothediagnosisandtherapyoflowerextremitydeepvein
thrombosis".)
UPPEREXTREMITYANATOMYTheupperextremityveinsaredividedintothesuperficialanddeepvenous
systems(figure1).
SuperficialveinsThemainsuperficialveinsoftheupperextremityincludethecephalic,basilic,median
cubital,andaccessorycephalicveins(figure1).Thebasilicveinisacommonaccesssiteforperformingdigital
subtractionvenography.
DeepveinsThedeepveinsoftheupperextremityincludethepairedulnar,radialandinterosseousveinsinthe
forearm,pairedbrachialveinsoftheupperarm,andaxillaryvein.Theaxillaryveinbecomesthesubclavianveinat
thelowerborderoftheteresmajormuscle(figure2).
ThoracicoutletanatomyThethoracicoutletisboundedbythebonystructuresofthespinalcolumn,firstribs,
andsternum(figure3A).Compressionofthevenousstructuresthattraversethethoracicoutletoccursintwo
distinctspaces:thescalenetriangleandthecostoclavicularspace.
ScalenetriangleTheanteriorborderofthescalenetriangleisformedbytheanteriorscalenemuscle,which
originatesfromthetransverseprocessesofthethirdthroughsixthcervicalvertebrae(C3C6)andinsertson
theinnerbordersandsuperiorsurfacesofthefirstrib.Theposteriorwallofthescalenetriangleisformedby
themiddlescalenemuscle,whicharisesfromthetransverseprocessesofthesecondthroughseventh
cervicalvertebrae(C2C7)andinsertsbroadlyontotheposterioraspectsofthefirstrib.Thesuperiorborder
ofthefirstribformsthebaseofthescalenetriangle.Thetrunksofthebrachialplexusandthesubclavian
arterypassbetweentheanteriorandmiddlescalenemuscles,whilethesubclavianveincourses
anteromedialtothescalenetriangle(figure3B).
CostoclavicularspaceThecostoclavicularspacecomprisestheareabetweenthefirstribandtheclavicle.
Thebrachialplexus,subclavianarteryandsubclavianveinpassthroughthisspace.Thesubclavianveinis
mostlikelytobecompressedatthissite.
PATHOGENESISPrimaryupperextremitydeepveinthrombosisisdefinedasthrombosisofthedeepveins
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drainingtheupperextremityduetoanunderlyinganatomicanomalyatthethoracicoutletcausingcompressionor
repetitiveinjurytotheunderlyingaxillosubclavianvein[25].Primaryupperextremitydeepveinthrombosisisa
manifestationofvenousthoracicoutletsyndrome(vTOS).(See"Overviewofthoracicoutletsyndromes",section
on'VenousTOS'.)
Thrombosisoftheveinsdrainingtheupperextremitywasoriginallypostulatedtobethecauseofacutearmpain
andswellingbyPaget[6],andlaterVonSchroetterrelatedtheclinicalsyndromespecificallytotheaxillaryand
subclavianveins[7].ThisclinicalentitywasreferredtoasPagetSchroettersyndrome[8].Inthemid20thcentury,
thetermeffortthrombosiswascoined[9],duetothefactthatthesyndromeoftenoccurredinphysicallyactive
individualsafterunusuallystrenuoususeofthearmandshoulder[1012].Theterm"spontaneous"upperextremity
venousthrombosishasalsobeenusedhighlightingtheoftendramaticpresentationinanotherwisehealthy,young
individual.Forthepurposesofourdiscussion,wewillrefertothesyndromeasprimaryupperextremitydeepvein
thrombosistodistinguishitfromsecondarycauses,whichareassociatedwithincitingfactorssuchasindwelling
cathetersorprothromboticstates.(See"Catheterrelatedupperextremityvenousthrombosis".)
Anatomicabnormalitiesofthethoracicoutletthatresultincompressionoftheveincanbecongenitaloracquired.
Congenitalanomaliesconsistofcervicalribs,supernumerarymuscles,abnormaltendoninsertionsorabnormal
muscularortendinousbands[13,14].Acquiredabnormalitiesincludebonyovergrowthduetobonyfracture(eg,
clavicle,firstrib)[1518],orhypertrophyofanteriorscalenemuscleorsubclaviusmuscles,oftenrelatedto
repetitivelifting.Theabnormalitiesofthethoracicoutletareoftenbilateral,andbilateralprimaryupperextremity
deepveinthrombosishasbeenreported[19,20].Anatomicabnormalitiesnarrowthescalenetriangle,ormore
commonlythecostoclavicularspace,predisposingtheveintocompressionbetweenthefirstribandmuscleor
tendon(figure3AB),orbetweenanomaloustendoninsertions.Lesscommonly,compressionoftheveinbetween
theclavicleandacervicalribcanoccur,andpartialocclusionoftheveinbyacongenitalwebhasalsobeen
reported[13,14].(See'Thoracicoutletanatomy'above.)
Undersomecircumstances,itappearsthatananatomicabnormalityisnotnecessarytoproduceinjurytothevein.
Extremesinrangeofmotionoftheupperextremitycanleadtomovementoftheclaviclerelativetothefirstrib
sufficienttocausevenouscompression.Repetitiveoverheadarmmovementsorhyperabductionandexternal
rotationoftheshoulderaremostoftenimplicated[2123].Repetitiveinjurycausesperivenousfibrosis,which
eventuallyleadstothrombosis.Itisimportanttorecognizethatthepatientoftenpresentswithacuteonsetof
symptomsrelatedtothethrombosis,buttheunderliningproblemmaybeachronicrepetitiveinjurythathad
narrowedthevein.
EPIDEMIOLOGYANDRISKFACTORSUpperextremitydeepveinthrombosis(allcauses)represents1to4
percentofallcasesofdeepveinthrombosis[24].Primaryupperextremitydeepveinthrombosisisrarewithan
estimatedannualincidenceof1to2casesper100,000population[10,24].Themajorityofcasesofupper
extremitydeepveinthrombosisaresecondaryandrelatedtocentralvenouscannulation(eg,centralline,
pacemaker)orprothromboticstates(eg,thrombophilia,malignancy)[1,10,25,26](See"Catheterrelatedupper
extremityvenousthrombosis".)
Between60and80percentofpatientswithprimaryupperextremitydeepveinthrombosisreportahistoryof
exerciseorstrenuousactivityinvolvingusuallythedominantupperextremitypriortotheonsetofsymptoms.
Strenuousactivitiesincludeweightlifting,rowing,oractivitiesinvolvingrepetitiveoverheadarmmovements,
particularlyhyperabduction,suchaspitching[1,27,28].Theaverageageatpresentationisintheearlythirtiesand
themaletofemaleratiois2:1[1].Apredominanceofrighthandedindividualsmayexplainwhytheright
axillosubclavianveinismorecommonlyaffected.(See'Pathogenesis'above.)
RiskfactorsRiskfactorsforprimaryupperextremitydeepveinthrombosisincludethefollowing[1,10,2530]:
Youngerage
Athleticmuscularmale
Strenuousupperextremityactivity
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Repetitiveoverarmhyperabduction
Anatomicabnormalitiesofthethoracicoutlet(congenital,acquired)
Thrombophilia
CLINICALPRESENTATIONSPrimaryupperextremitydeepveinthrombosiscanpresentacutelywith
symptomsandsignsofupperextremitydeepveinthrombosisorpulmonaryembolism,orwithchronicor
intermittentsymptoms.
AcuteupperextremitydeepveinthrombosisAcutepresentationsareduetosuddenthrombosisofthe
axillosubclavianvein.
Theclassicpresentationisthatofayoung,athleticmalepresentingwithacuteonsetofupperextremitypainand
swellinginthedominantarmfollowingaparticularlystrenuousactivity[1,27,28].Strenuoususeofthearmpriorto
theonsetofextremityswellingorpainisrecalledin40to80percentofpatients,andsymptomsaregenerally
noticedwithin24hoursofthestrenuousactivity[10,11,23,31].Themajorityofpatients(70to80percent)manifest
withvariabledegreesofneck,shoulder,oraxillarydiscomfort,armheavinessandpainassociatedwithcomplaints
ofupperextremityswelling[32,33].Swellingandpaintypicallyimprovewithrestandelevationofthearmtothe
leveloftheheart,whereaselevationoftheextremityoverheadmayaggravatethesymptoms[34].
Physicalexaminationgenerallyrevealsedemaoftheaffectedextremity,oftenaccompaniedbycyanosisofthe
handandfingers.Thepatientmayalsohavealowgradefever.Apalpablevenouscord(superficial
thrombophlebitis)maybeapparentinassociatedsuperficialveins(eg,proximalcephalicvein).Dilated
subcutaneouscollateralveins,alsoknownasUrschelssign,maybenoticeableovertheupperchestandproximal
upperextremity,particularlyinthosewithanunderlyingchronicvenousstenosis[11,27,32].
Theupperextremityarterialvascularexaminationshouldbenormal.Reducedarterialbloodflowduetovenous
congestion(phlegmasiaceruleadolens)israreinthelowerextremityandevenmoresointheupperextremity
[35,36].However,ifpresent,itrepresentsanemergencyandindicatestheneedforemergenttreatment.(See
'Thrombolytictherapy'below.)
Coexistentsignsrelatedtobrachialplexuscompression(ie,neurogenicthoracicoutletsyndrome)maybepresent,
manifestingasparaesthesiasorpainintheulnarnervedistribution,tendernessoverthesupraclavicularfossa,and
wastingoftheintrinsichandmuscles.(See"Overviewofthoracicoutletsyndromes",sectionon'Clinical
evaluation'.)
AcutepulmonaryembolismInadditiontoupperextremityswellingandpain,upperextremitydeepvein
thrombosiscaninitiallypresentassymptomaticorasymptomaticpulmonaryembolism[12,28,33,3741].The
clinicalfeatures,diagnosisandtreatmentofpulmonaryembolismarediscussedindetailelsewhere.(See
"Overviewofacutepulmonaryembolisminadults".)
ChronicorintermittentsymptomsInpatientswithpartialthrombosisorchronicvenousstenosisdueto
repetitiveinjurythatcausesactivityrelatedobstruction,symptomsmaybeintermittentandlesssevere.Ifvenous
occlusiondevelopsoveraprotractedperiodoftime,edemaorpainmaybeminimal,andincreasedvenous
collateralflowoverthechest(Urschelssign)maybetheonlyclinicalsignthatisapparent[11,27,32,42].
DIAGNOSISAdiagnosisofupperextremityvenousoutflowobstruction(ie,deepveinthrombosisorvenous
stenosis)maybesuspectedbasedupontheclinicalpresentation,butshouldbeconfirmedwithimaging,typically
initiallyusingultrasound.Ddimerisusefulforexcludingthrombosisasanetiology,butwillnotexcludevenous
stenosiswithoutthrombosisasasourceofsymptoms.Onceadiagnosisofvenousoutflowobstructionis
established,aprimaryetiologyshouldbesoughttoidentifytheunderlyinganatomicabnormalitythatisthesource
oftheobstruction.Weobtainaplainchestradiographonallpatientstoidentifyanyobviousbonyabnormalities
however,moreadvancedimagingmaybeneededtodemonstrateabnormalmuscularattachments.Itisimportant
toassesscontralaterallimbinvolvementbecauseabouthalfofpatientswillhavesomedegreeofcontralateral
venousobstruction,evenintheabsenceofsymptoms.(See'Pathogenesis'above.)
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DdimerPlasmaDdimer,whichisadegradationproductofcrosslinkedfibrin,maybeelevatedinpatients
withupperextremitydeepveinthrombosis,asinthosewithotherlowerextremitydeepveinthrombosisor
pulmonaryembolism.However,althoughaplasmaDdimer>500g/Lissensitiveforthrombosis,andhasahigh
negativepredictivevalue,itisnotspecificfortheanatomiclocationofthethrombosis,andwillnotexcludevein
compression/stenosisasasourceforsymptoms[43].
VenousoutflowobstructionBmodeultrasound,colorDopplerultrasound,andduplexultrasoundhavebeen
usedextensivelyinthediagnosisofdeepveinobstruction.NoncompressibilityoftheveinonBmodeultrasound
withorwithoutvisibleintraluminalthrombusisthemajorcriterionforthediagnosisofvenousthrombosis.Weuse
duplexultrasoundastheinitialtestfordiagnosingupperextremityvenousoutflowobstructionbecauseitis
noninvasive,inexpensive,andinobservationalstudies,hasanacceptablesensitivityandspecificityforthe
diagnosisofupperextremitydeepveinthrombosis[33,4451].Asystematicreviewevaluated17studies,
concludingthatcompressionultrasonographyisanacceptablealternativetostandardcontrastvenography[44].
Thesummaryestimatesofthesensitivityofcompression,Dopplerultrasound,andDopplerultrasoundwith
compressionwere97,84,and81percent,respectively,andspecificitieswere96,94,and93percent,
respectively.Disadvantagesofultrasoundarethatitistechniciandependent,andthatnonocclusivemural
thrombusandthrombusintheproximalsubclavianorinnominateveinsmaynotbeadequatelyseenasaresultof
acousticshadowingbytheoverlyingclavicleandsternum[50,52,53].However,proximalsubclavianvein
obstructionislesstypicalofprimarycausesofupperextremityvenousoutflowobstruction,whichtendtoaffect
themidtodistalsubclavian/proximalaxillaryveinatthethoracicoutlet.Whenacousticshadowingisaproblem,
venousthrombusorstenosismoreproximaltotheplacementoftheultrasoundprobecanbeinferredfrom
abnormalrespiratoryvariation,abnormalaugmentation,andabnormalDopplerflow.
Althoughstandardcatheterbased(digitalsubtraction)venographyprovidesthebestdefinitionofabnormalvenous
anatomyandisthestandardwithwhichothermodalitiesarecompared[44,54],itisgenerallynotneededto
establishadiagnosisofupperextremitydeepveinthrombosis.Venographyrequirescannulationofaperipheral
veinoftheaffectedupperextremity,whichcanbechallenginginthefaceofsignificantextremityedema,andthe
studyrequiresasubstantialintravenouscontrastload.Assuch,catheterbasedvenographyisgenerallyreserved
forsituationswherenoninvasivestudiesareequivocal,butclinicalsuspicionremainshighforaprimarycauseof
venousoutletobstruction[1].Forpatientswithintermittentorchronicsymptoms,extrinsiccompressionofthevein
canbedemonstratedduringcatheterbasedvenographybyperformingdynamicstudiesthatplacethearmin
variouspositionsduringthestudy.Thevenogrammaybenormalatrestbutabnormal(varyingdegreesofextrinsic
compressionwithnewvenouscollaterals)witharmabductionhowever,veincompressionwitharmabduction
canbeanormalvariant[55]Bonyabnormalitiesmayalsobeseenwithfluoroscopicimagingduringcatheterbased
venography,butabnormalfibrousbandsormuscleinsertionswillnot.
Lessinvasivemethodsofvenographyincludecomputedtomographic(CT)andmagneticresonance(MR)
venography[53,5658].Thesemodalitiesarenottypicallyusedtoestablishadiagnosisofupperextremityvenous
outflowobstruction.Rather,thesestudiesaremoreusefulforidentifyinganatomicabnormalitiesandother
secondarycausesfordeepveinthrombosis(eg,tumor).(See'Anatomicabnormalitiesofthethoracicoutlet'
below.)
CTvenographycanbeusedtoconfirmorexcludecentralveinthrombushowever,likecatheterbased
venography,substantialcontrastloadsarerequired.CTvenographyhasnotbeenstudiedsufficientlyto
determineitssensitivityandspecificity.Asmallstudyof18patientscomparedCTvenographyanddigital
subtractionvenographyfortheirabilitytodiscriminatetheseverityandextentofvenousobstruction,the
causeofupperextremitydeepveinthrombosis,andimplicationsfortheplanningoftreatment[58].CT
venographywasfelttoprovidemoreinformationthandigitalsubtractionvenography,andinhalfofthe
patients,thefindingsofCTvenographychangedthetreatmentplan.
ContrastenhancedMRimagingisveryspecificinitsabilitytoimagesubclavianveinthrombosis,butits
sensitivityforthrombosisistoolowtobeausefulscreeningmodality[53].However,withproperprotocol
anddynamicsequencing,contrastenhancedthreedimensional(3D)MRangiographyisanexcellent
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noninvasivealternativetovenogram[59].Onemustrememberthatanatomiccompressionofvascular
structuresinthoracicoutletiscommonandpositivefindingsandthepresenceofpathologicalfindingsmust
beclinicallycorrelated.
AnatomicabnormalitiesofthethoracicoutletOnceadiagnosisofupperextremityvenousoutflow
obstructionisestablished,furtherimagingshouldbeperformedtoidentifyaprimarycauseforthrombosis,suchas
cervicalribs,supernumeraryribs,abnormalbandsorabnormalmuscleinsertions.Foranypatientsuspectedof
havingaprimarycauseforupperextremitydeepveinthrombosisorstenosis,weobtainaplainchestfilmto
identifyanybonyabnormalities[60].Ideally,theanatomicabnormalityshouldbeidentifiedpriortothoracicoutlet
decompressionhowever,thisisnotalwayspossible.Attimes,theanatomicabnormalitymaynotbeapparent
untilthetimeofsurgicalexploration.(See'Thoracicoutletdecompression'below.)
Althoughcomputedtomography(CT)andmagneticresonance(MR)imagingarelessappropriateinitialstudiesfor
screeningpatientssuspectedofhavingupperextremitydeepveinthrombosis,thesestudiesprovidemore
anatomicdetailandshowtherelationshipofvenousstructurestothesurroundingboneandmuscle.CTandMRI
alsoallowtheassessmentofcentralvenousstenosisorocclusion,whichcanbemissedbyultrasounddueto
acousticshadowingfromoverlyingbonystructures.Inaddition,lessobviousbonyabnormalitiescanbeseenon
thesestudies,andattimes,venouscompressionrelatedtobonyormuscularabnormalitiescanalsobeseen.
Intheabsenceofanobviousbonyanatomicabnormality,aprimarycauseforthethrombosisorvenousstenosis
canbepresumedintheyoung,otherwisehealthy,activeindividualwithaclassicpresentationwhodoesnothave
ahistoryofcentralvenousinstrumentationorothermedicalproblemsassociatedwithsecondaryetiologiesfor
venousoutflowobstruction.Thespecificabnormalitymaynotbedetermineduntilthetimeofsurgicalexploration.
(See'Approachtotreatment'below.)
DIFFERENTIALDIAGNOSISThedifferentialdiagnosisforupperextremityedemanotrelatedtoprimaryupper
extremitydeepveinthrombosisincludesedemarelatedtootheretiologies,secondarycausesofvenous
thrombosis,andlymphedema.
Primaryupperextremitydeepveinthrombosiscanbedistinguishedfromsecondarycausesbytheabsenceof
venousinstrumentation,ayoung,otherwisehealthypatientdemographic,andamoretypicallysuddenonsetof
symptoms.Theclinicalfeaturesofupperextremitydeepveinthrombosisareotherwisesimilarandincludeupper
extremityedemaandpain,andcyanosisoftheskinduetovenouscongestion.Upperextremitydeepvein
thrombosisthatoccursintheabsenceofinstrumentationandwithnoidentifiableanatomicabnormalitiesorother
riskfactorsforvenousthrombosis(eg,oralcontraceptives)raisesaconcernofoccultmalignancy.Upto25
percentofpatientswillbediagnosedwithinoneyearofavenousthromboembolicevent[33,61].Ifaprimarycause
forupperextremitydeepveinthrombosisisnotimmediatelyapparentonimagingstudies,thepatienthasno
historyofinstrumentation,andthepatienthasnoneoftheriskfactorslistedaboveforprimaryupperextremity
deepvenousthrombosis,wesuggestamoreformallaboratoryevaluationtoruleoutsecondarycausesforupper
extremitydeepveinthrombosis,includingcoagulationstudies,whichshouldbedrawnpriortotheinitiationof
anticoagulation.(See"Screeningforinheritedthrombophiliainasymptomaticindividuals"and"Riskandprevention
ofvenousthromboembolisminadultswithcancer".)
Patientswithvenousthrombosisduetocompressionofstructuresofthethoracicoutletmayalsohavesymptoms
attributabletothearterialorneurologicstructuresthatpassthroughthisspace.Distinguishingbetween
neurogenic,arterial,andvenousthoracicoutletsyndromeisdiscussedelsewhere.(See"Overviewofthoracic
outletsyndromes",sectionon'Clinicalevaluation'.)
Therearemanycausesofextremityedemathatarenotrelatedtovenousobstruction.Themedicalhistorywill
usuallygiveaclueastothepotentialetiologyforedema(eg,historyofheartfailure).Althoughsystemicetiologies
typicallypresentwithbilateralextremityedema,thisfeatureisnothelpfulgiventhatanatomicabnormalitiesofthe
thoracicoutletarecommonandpatientswithprimaryupperextremitydeepveinthrombosiscanpresentwith
bilateralsymptoms.Routinelaboratorystudiestypicallyimportantintheevaluationofpatientswithextremity
edemaincludeacompletebloodcount,electrolytes,andliverfunctiontests.Thesestudiesmaypointtoan
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alternativeetiologyforupperextremityedema.Thegeneralapproachtothepatientwithedemaisdiscussedin
detailelsewhere.(See"Pathophysiologyandetiologyofedemainadults"and"Clinicalmanifestationsand
diagnosisofedemainadults".)
Upperextremityarmswellingcanbeduetolymphedemahowever,swellingfromacutevenousthrombosishasa
moreabruptonsetandanantecedentriskfactorsuchasprioraxillarylymphnodedissectionislacking.(See
"Clinicalfeaturesanddiagnosisofperipherallymphedema".)
APPROACHTOTREATMENTThegoalsoftreatmentofprimaryupperextremitydeepveinthrombosisare
relievingsymptomsrelatedtovenousobstruction,preventingcomplicationsofdeepveinthrombosis,and
preventingrecurrentthrombosis[62].Treatmentoptionsincludeanticoagulation,thrombolysis,andsurgical
decompressionofthethoracicoutlet.Notreatmentorcombinationoftreatmentshasbeenrigorouslyevaluatedfor
thetreatmentofupperextremitydeepveinthrombosis.Asaresult,recommendationsarebaseduponavailable
retrospectivestudiesandindirectevidenceprovidedfromtheexperiencewithdeepveinthrombosisofthelower
extremity[1].
Ourapproachtotreatmentisasfollows:
WeagreewithguidelinesfromtheAmericanCollegeofChestPhysiciansthatrecommendanticoagulation
foraminimumofthreemonthsforallpatientsidentifiedwithupperextremitydeepveinthrombosis[63].(See
'Anticoagulation'below.)
Forpatientswithprimaryupperextremityaxillosubclaviandeepveinthrombosiswithsuddenonset,
moderatetosevereupperextremitysymptomsoflessthantwoweeksduration,wesuggestthrombolysisto
eliminatethrombustotheextentthatispossible.Lysisislesseffectivewhensymptomshavebeenpresent
formorethantwoweeks.(See'Thrombolytictherapy'below.)
Forgoodrisksurgicalpatientsidentifiedwithanatomicabnormalitiesofthethoracicoutletcausing
symptomaticvenouscompression,wesuggestthoracicoutletdecompression.Thespecificprocedureis
targetedtothetypeofabnormalityidentified.Forthoseinwhomaspecificabnormalityhasnotbeen
identified,weperformfirstribresection,providedthatasecondarycauseofupperextremityvenous
thrombosisisnotpresent.
Anticoagulationalone(nothrombolysis)withorwithoutthoracicoutletdecompressionmaybeappropriatefor
patientswithmildsymptoms,intermittentsymptoms,andthosewhopresentinadelayedmanner(>2
weeks).Thenaturalhistoryofthesepatientsisunclear.(See'Symptomaticcare'belowand'Anticoagulation'
below.)
RationaleforaggressivetreatmentAnaggressiveapproachthatincludesacombinationofthrombolysisand
thoracicoutletdecompressionwithorwithoutvenoplasty(percutaneous,open)appearstoimprovelongterm
outcomesinpatientswithprimaryupperextremitydeepveinthrombosis,particularlythosewithacute,moderate
toseveresymptoms[6485].Withanaggressiveapproach,successratesforreestablishingsubclavianvein
patencyarenearly100percentprovidedthatthrombolysisisperformedwithintwoweeksoftheonsetof
symptoms[8688].Althoughearlyinterventionisadvocated,patientswithprimaryupperextremitydeepvein
thrombosiswhopresentlaterthantwoweeksmayalsobenefitfromthoracicoutletdecompression(no
thrombolysis)giventhehighratesofrecurrentthrombosisandlongtermmorbidityassociatedwithanticoagulation
alone[3,22,89,90].
Inaworldwideclinicalseriesof606patientswithprimaryupperextremitydeepveinthrombosis,early
thrombolysisandfirstribresectionprovidedthebestoutcome,with95percentofthesurgicalcohortexperiencing
anexcellentclinicaloutcomecomparedwith29percenttreatedconservatively,whichconsistedofanticoagulation,
armelevation,andupperextremitycompression[27].Residualvenousobstructionwaspresentin78percentof
patients.Theclinicaloutcomesassociatedwithanticoagulationalonewereevaluatedinalaterseriesof54
patients,nearlyallofwhomweretreatedwithwarfarin[21].Afterameanfollowupoffiveyears,22percenthad
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persistentseverevenousoutflowobstructiononfollowupultrasound.About50percentofthepatientswere
asymptomatic,but13percenthadsevereordisablingsymptoms.Subsequentpulmonaryembolismwas
documentedin26percentandwassymptomaticinonethirdofthepatients.Bycomparison,amongpatients
treatedwiththrombolysis(withoutthoracicoutletdecompression),76percentwereasymptomaticafteramean
followupof55months[21,86].Inotherretrospectivereviews,persistentsymptomsanddisabilityoccurredin41
to91percentofpatientstreatedconservatively[1,27].
Firstribresectionwithoutpreoperativethrombolysishasbeenproposedforthemanagementofprimarysubacute
venousthrombosis.Intheretrospectivereview,45of110patientsunderwentpreoperativethrombolysisaloneor
thrombolysisandballoonvenoplastypriortothoracicoutletdecompression.Theremaining65patientswere
treatedwithanticoagulationalonepriortothoracicoutletdecompression.Upto80percentofoccluded
axillosubclavianveinsrecanalizedduringthefollowupperiodintheanticoagulationgroup,andtheoverallratesof
venouspatencyweresimilarbetweenthegroups[89].
RecommendationsofothersOurrecommendationsareingeneralagreementwiththeguidelinesfromthe
AmericanCollegeofChestPhysicians(ACCP)however,theACCPsuggestsanticoagulanttherapyaloneover
thrombolysisforpatientswithacuteupperextremitydeepveinthrombosisthatinvolvestheaxillaryormore
proximalveins[63].Theyfurtherstatethatpatientsarelikelytochoosethrombolytictherapyoveranticoagulation
aloneiftheyaremorelikelytobenefitfromthrombolysis,haveaccesstocatheterbasedtherapy,attachahigh
valuetothepreventionofpostthromboticsyndrome,andattachalowervaluetotheinitialcomplexity,cost,and
riskofbleedingwiththrombolytictherapy.Thisrecommendationdoesnotdistinguishbetweenprimaryand
secondarycausesofupperextremitydeepveinthrombosisdirectlyhowever,giventhatpatientswithprimary
upperextremitydeepveinthrombosisaremorelikelytobenefitfromthrombolysiscomparedwithpatientswith
secondarycausesofdeepveinthrombosis,wesupportamoreaggressivetreatmentstrategy.(See'Rationalefor
aggressivetreatment'above.)
INITIALMANAGEMENTPatientswhoarediagnosedwithprimaryupperextremitydeepveinthrombosisare
initiallymanagedwithmeasurestoimprovetheircomfortandareanticoagulated.Anticoagulationhelpstomaintain
patencyofcollateralveinsandreducespropagationofthrombus.Anticoagulanttherapywithheparinorwarfarinis
alsoeffectiveinpreventingpulmonaryembolismwithlowerextremityvenousthrombosis,andbyextrapolation,
mayalsopreventembolismfromupperextremitydeepveinthrombosis[28,63].Thedecisiontoproceedwith
thrombolysisorthoracicoutletdecompressionisbaseduponsymptomseverityandthetypeofassociated
anatomicabnormality.(See'Approachtotreatment'above.)
SymptomaticcareSymptomaticcareofphlebiticsymptomsrelatedtoupperextremitydeepveinthrombosis
includesupperextremityelevation,andnonsteroidalantiinflammatorydrugs(NSAIDs)forpainmanagement.
Armelevationshouldhelpreduceupperextremityswelling.Graduatedcompressionstockingshavebeenshownto
reducetherateofpostthromboticsyndromeinpatientswithlowerextremitydeepveinthrombosis,andmayalso
bebeneficialinpatientswithupperextremitydeepveinthrombosis[33].However,compressionislikely
unnecessaryintreatedpatientsforwhomthelesioniscorrectedandedemahasresolved.
AnticoagulationWeagreewithguidelinesfromtheAmericanCollegeofChestPhysiciansthatrecommend
parenteralanticoagulation(eg,lowmolecularweightheparin,fondaparinux,intravenousunfractionatedheparin,
subcutaneousunfractionatedheparin)forallpatientswithaxillosubclavianveinthrombosis[63].Inourpractice,we
beginparenteralanticoagulationonceadiagnosisofdeepveinthrombosisismadebutafteranynecessary
laboratoryteststoevaluateforhypercoagulablestateshavebeenobtained.
Thechoiceofinitialparenteralagentforanticoagulationinpatientswithprimaryupperextremitydeepvein
thrombosisdependsupontheneedforfurthertreatmentintheformofthrombolysisorthoracicoutlet
decompression.Forpatientswithmild,intermittent,orchronicsymptomswhowillbemanagedonanoutpatient
basis,lowmolecularweightheparin(LMWH)orfondaparinuxcanbeinitiatedtobridgetolongtermtherapyina
similarfashionasthosewithlowerextremitydeepveinthrombosis[63,9193].(See"Overviewofthetreatmentof
lowerextremitydeepveinthrombosis(DVT)".)
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Whenthrombolysisisanticipated,weadministerunfractionatedheparin,andmaintaintherapeuticlevels(aPTT1.5
to2.5timescontrol)untilthrombolysisisinitiated.Duringthrombolysis,thedoseofheparinshouldbeloweredto
minimizebleedingcomplications,butoncethrombolysisiscompleted,fullanticoagulationcanberesumed[94,95].
Similarly,thedoseofheparinshouldbeloweredaroundthetimeofsurgicalintervention.Onceanynecessary
interventionsarecompleted,bridginganticoagulationcanbeusetotransitiontolongtermtherapyinanticipationof
discharge.(See"Perioperativemanagementofpatientsreceivinganticoagulants",sectionon'Bridging
anticoagulation'.)
AdmissionandreferralManypatientswithacute,severesymptomsrelatedtoprimaryupperextremitydeep
veinthrombosiswillrequireadmissiontomanagesymptomsandinanticipationofthrombolytictherapyand/or
surgicaldecompression.
Thereisno"onesizefitsall"approachtothetreatmentofprimaryupperextremitydeepveinthrombosis.
Individualizedcarerequiresateamofspecialistswithsufficientexperienceandreadilyavailableresourcesand
ancillarypersonnel.Suchteamsarerareoutsidetertiaryreferralcenters,andthus,referraltoavascularcenter
withsuchateamisappropriate[69,96].
Initialoutpatientanticoagulationandoutpatientreferralforpossiblefurthertreatmentmaybeappropriatefor
patientswith:
Minimalsymptoms
Delayedpresentationmorethantwoweeksaftertheonsetofsymptoms
Intermittentsymptomsduetovenousobstructionwithoutthrombosis
THROMBOLYTICTHERAPYTheaimofthrombolytictherapyispromptdissolutionofthrombustominimize
inflammationandendothelialinjuryandtorestoreveinpatency,whichreducesextremityedemaandassociated
symptoms[28,85,97,98].Thrombolytictherapyappearstohavethemostbenefitforpatientswhopresentwith
acute,moderatetoseveresymptomsrelatedtosuddenaxillosubclavianthrombosis.Inourexperience,thrombi
thathavebeensymptomaticforuptotwoweekshaveareasonablechanceoflysiswithcatheterdirectedinfusion
ofalteplasedirectedintothethrombus.Thrombolysisrestoresveinpatencyin64to84percentofpatients,with
betterpatencyratesassociatedwithearlierinitiationoflytictherapy[85].However,evenaftersuccessfullysis
thatrestoresveinpatency,uptoonethirdofpatientswillreocclude[1,85].(See'Rationaleforaggressive
treatment'above.)
Thegeneralcontraindicationstothrombolytictherapyaregiveninthetable(table1).Wedonotperform
thrombolysisinpatientswithonlypartialthrombosis,orthosewithmildacuteorchronic,intermittentsymptoms.
Symptomaticpatientswhopresentmorethantwoweeksaftertheonsetofsymptomsarelesslikelytobenefit
fromthrombolysisduetotheorganizednatureoftheclotandinflammatorychangesinthevein.
Observationalstudieshaveshownthatcatheterdirectedpharmacologicthrombolysis,whichinvolvesembedding
aninfusioncatheterintotheaxillosubclavianveinthrombus,achieveshigherratesofclotdissolutioncompared
withsystemicinfusionandrequiresanoveralllowerdoseandshorterdurationoflysis,whichreducesbleeding
complications[28,85,98].
Catheterdirectedpharmacologicupperextremitythrombolysisisperformedinthefollowingmanner:
Thebasilicveinoftheaffectedlimbisaccessedusingultrasoundguidance.
Aguidewireandcatheterareusedtotraversethethrombosedaxillosubclavianvein.
Amultisideholeinfusioncatheterisembeddedwithinthethrombusandinfusionofalteplaseinitiatedat0.01
mg/kg/hour[85,99].Alternatively,urokinase(notavailableintheUnitedStates)canbeused[100].
Thecatheterissuturedintoposition,andthepatienttransferredtoamonitoredsettingtomonitorforany
bleedingcomplications.Theprogressofthrombolysisisevaluatedbyperformingvenographyat12hours,
andagainat24hoursifnecessary.Atotaldoseof20to25mgalteplasecanbesafelyusedinmostcases.
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Forpatientsundergoingcatheterdirectedorsystemicthrombolysis,aspirinshouldbeadministeredalongwith
unfractionatedheparinduringinfusionofthethrombolyticagenttocounteractplateletactivationand
potentiallyincreasedthrombogenicitythatmaybeinducedwithlytictherapy[33].
Mechanicalthrombolysis(eg,AngioJet,EKOScatheter)isoftenusedincombinationwithpharmacologic
thrombolysis[101].Therearelimiteddatainvolvingtheuseofthesedevicestotreatupperextremitythrombosis.
However,baseduponavailableresultsforlowerextremitydeepveinthrombosis,thesetreatmentsmaybeuseful
inupperextremitythrombosistorapidlyextractalargeburdenofthrombusandreducetheoveralldoseand
durationoflytictherapy[102].
Oncevenouspatencyhasbeenreestablishedwithcatheterdirectedthrombolysis,itisimportanttoevaluatethe
residualaxillosubclavianveinforpersistentcompressionorstenosis.Occasionally,adjunctivepercutaneous
transluminalangioplasty(PTA)followingsuccessfulthrombolysisisneededtoopentheveinsufficientlysothat
anticoagulanttreatmentcanmaintainpatencyuntilthoracicoutletdecompressioncanbeperformed[1].(See
'Venoplasty'belowand'Thoracicoutletdecompression'below.)
THORACICOUTLETDECOMPRESSIONForpatientswithprimaryupperextremityaxillosubclaviandeep
veinthrombosis,andselectedpatientswithaxillosubclavianvenouscompression/stenosis,observationalstudies
supportsurgicaldecompressionofthethoracicoutlet,whichprovidesthelowerratesofrecurrent(orfuture)
thrombosis,andreducedlongtermmorbiditycomparedwithmoreconservativemanagement[1,27].(See
'Rationaleforaggressivetreatment'above.)
Thoracicoutletdecompression,whichmayincludeanyoneofacombinationoftheproceduresdiscussedbelow,
isindicatedforgoodrisksurgicalpatientswithanyofthefollowingpresentations[1,27,64,103,104]:
Patientswhopresentwithacute,moderatetoseveresymptomsduetoprimaryupperextremity
axillosubclavianthrombosis,followingthrombolysis.
Symptomaticpatients(intermittentorrecurrent)withathoracicoutletanatomicabnormalitycausingvenous
compression/stenosis.Objectiveevidenceofvenousthrombosismayormaynotbepresent.
Thoracicoutletdecompressionmaybeindicatedinpatientswithsymptomaticsubacute(>2weeks)or
symptomaticchronicthrombosis[89].
Followingsuccessfulthrombolysis,somecliniciansfavoranticoagulationforonetothreemonthstoallow
endothelialhealingandresolutionofacuteinflammationbeforethoracicoutletdecompression[64].However,given
theriskofrethrombosis,weagreewiththemajorityofsurgeonsinadvocatingsurgicaldecompressionduringthe
samehospitalizationasthrombolytictherapy[1,3,96].Regardlessofthetimingofsurgicaldecompression,
anticoagulationismaintaineduntilthesurgerycanbeperformed.(See'Anticoagulation'above.)
Forpatientswithresidualthrombusfollowingthrombolysisorthosewithchronicaxillosubclavianthrombosis,the
axillosubclavianveincanbereconstructedconcurrentwiththoracicoutletdecompressionoratalatertimeusing
openorendovasculartechniques,ifneeded.Inaseriesofpatientsundergoingfirstribresectionforchronic
symptomsrelatedtoprimarysubclavianveinthrombosis,subclavianveinthrombusresolvedin14of16patients
treatedwithongoinganticoagulation[105].(See'Venoplasty'below.)
Theaimofthoracicoutletdecompressionistoprovidemorespacethroughwhichtheneurovascularstructuresof
theupperextremitycanpass.Thoracicoutletdecompressionmayincludeanyoneoracombinationofthe
followingprocedures,thechoiceofwhichwilldependuponthespecificanatomicabnormalityidentified[22,105].
(See"Overviewofthoracicoutletsyndromes",sectionon'Pathogenesis'.)

Firstribresection
Cervicalribresection(lesscommonforvenousthoracicoutletsyndrome)
Divisionofanomalousbands
Divisionofanomalousmusculotendinousinsertions

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Scalenectomy
Forpatientsidentifiedwithacervicalrib,cervicalribresectionmaybeallthatisrequired.However,whena
cervicalribisnotpresent,firstribresectioniscombinedwithdissectionanddivisionofstructuresidentifiedatthe
timeofsurgicalexplorationaspotentiallycausingcompressionontheneurovascularstructuresofthethoracic
outlet,whetherornotthesewereidentifiedasaspecificsourceofcompressiononpriorimagingstudies.
Threesurgicalapproachesareusedforthoracicoutletdecompression:thetransaxillary,supraclavicular,and
infraclavicularapproaches,eachwithadvantagesanddisadvantages.Therearenotrialscomparingsurgical
approachesfordecompression,andthereremainsnoexpertconsensusastowhichsurgicalapproachormethod
oftreatingtheinjuredvenoussegmentisbetter.Assuch,thesurgicalapproachdependslargelyuponthetypeof
anatomicabnormalitiesidentifiedandsurgeonpreference.Theseapproachesandthecomplicationsassociated
withthemarediscussedseparately.(See"Overviewofthoracicoutletsyndromes",sectionon'Thoracicoutlet
decompression'.)
Complicationsofthoracicoutletdecompressionincludehemopneumothorax,longthoracicnerveinjury,incomplete
ribresectionthatcanleadtorecurrentsymptoms,brachialplexusinjury,arterialinjury,lymphaticleak,and
surgicalsiteinfection.
VENOPLASTYAlthoughthereisuniformagreementthatthoracicoutletdecompressionshouldbeperformed
earlyinpatientswithacuteaxillosubclavianthrombosis[1],debateexistsoverthemanagementofthevein.
Concurrentwiththoracicoutletdecompression,somefeelthatvenolysisorvenoplasty(percutaneoustransluminal
oropen)issufficientwhileothersadvocateveinrepair(patchvenoplasty,interpositionveingraft,veinbypass,
jugularturndown)[27,80,106].Thereareinsufficientdatatosupportoneapproachoveranother.
Occasionally,percutaneoustransluminalangioplasty(PTA)isneededtokeeptheveinopenfollowingthrombolysis
untilthoracicoutletdecompressioncanbeaccomplished[1].However,priortothoracicoutletdecompression,
stentingshouldbeavoided[42,86,107109].Shouldermovementssubjectthestenttorepetitivecompressionthat
canleadtostentfracture.Inonesmallobservationalstudy,stentusewasfoundtobeanindependentriskfactor
forupperextremityrethrombosis[86].Oncethoracicoutletdecompressionhasbeenperformed,whetherornota
veinpatchrepairorbypasswasperformed,residualorrecurrentstenosismaywarrantPTAandpossiblystenting,
withacceptableclinicaloutcomes[1,22].
FOLLOWUPWeobtainaDuplexultrasoundinthepostsurgicalperiodtoconfirmpatencyofaxillosubclavian
vein.Ifthepatientexperiencesrecurrentsymptoms,weagreewithothersinadvocatinganimmediatevenogram
toevaluatethepatencyoftherepairedvenoussegment[105].
DurationofanticoagulationInpatientswithprimaryupperextremitydeepveinthrombosis,anticoagulation
shouldbecontinuedforaminimumofthreemonthsfollowingtheinitialthromboticevent,withalongerdurationof
therapyindicatedforthosewhohavehadarecurrentevent[63,110].Wemaintainanticoagulationregardlessof
whetherintervention(thrombolysis,thoracicoutletdecompression)wasperformed.
PERIOPERATIVEMORBIDITYANDMORTALITYMortalityrelatedtoprimaryupperextremitydeepvein
thrombosisisoveralllow,duetotherelativelyyoungpopulationofpatientswhoaretypicallyaffected.By
comparison,mortalityrelatedtosecondarycausesofupperextremitydeepveinthrombosisishigher,rangingfrom
15to50percentreflectingunderlyingcomorbiditiessuchasmalignancy,renalfailure,andmultiorganfailure[12].
RecurrentthromboembolismRecurrentupperextremitydeepveinthrombosis(allcauses)occursin2to8
percentofpatientsfollowingtreatment[28,111,112].Theseratesaresignificantlylowerthanrecurrenceratesfor
lowerextremitydeepveinthrombosis,whichinonelongtermstudywas30percentateightyears[113].
Ahypercoagulablestateneedstobeconsideredinpatientswhodeveloprecurrentthromboembolism[12].Higher
ratesofthrombophiliahavebeenreportedinpatientswithrecurrentupperextremitydeepveinthrombosis[30,114
117].Inonestudy,90percentofpostoperativecomplicationswereassociatedwithsomeformofthrombophilia
[115].Theevaluationofthrombophiliaisdiscussedelsewhere.(See"Evaluatingpatientswithestablishedvenous
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thromboembolismforacquiredandinheritedriskfactors".)
PostthromboticsyndromePostthromboticsyndromereferstothedevelopmentofsymptomsorsignsof
chronicvenousinsufficiencyrelatedtoapriordeepveinthrombosis.Postthromboticsyndromeisdiscussedin
detailelsewhere.(See"Postthrombotic(postphlebitic)syndrome".)
Qualityoflifeisreducedinpatientswithpostthromboticsyndrome,particularlyifthedominantarmisaffected.
Severeupperextremitysymptomswithskinulcerationarerare,butpostthromboticsyndromeaffectingtheupper
extremitycanresultinoccupationaldisabilityinpatientswhosejobrequiresmanuallabor.Eveninpatientswhose
occupationdoesnotinvolvethevigoroususeofthearms,symptomsofpostthromboticsyndromecanlimitother
activitiesandadverselyimpactqualityoflife.
Theincidenceofpostthromboticsyndromefollowingupperextremitydeepveinthrombosis(allcauses)ranges
from7to44percentbutappearstobemoreprevalentfollowingprimarycomparedwithsecondaryetiologies
[12,28].Sinceprimaryupperextremitydeepveinthrombosisgenerallyaffectsyoung,otherwisehealthyindividuals
withanactivelifestyleandlonglifeexpectancy,oneoftheaimsofearlyaggressivetreatmentisminimizing
symptomsofpostthromboticsyndrome.(See'Rationaleforaggressivetreatment'above.)
Inpatientswithprimaryupperextremitydeepveinthrombosis,upto53percentofpatientstreatedwith
anticoagulationalonehistoricallydevelopedpostthromboticsyndromeat5years[27,103].Withaggressive
therapythatincludesanticoagulation,thrombolysis,andthoracicoutletdecompression,theincidenceofresidual
symptomsrangesfrom12to25percent[1,27].Theriskofdevelopingpostthromboticsyndromemaybegreaterin
patientswhohaveresidualveinobstruction.However,inonestudy,noassociationbetweenultrasoundfindings
andthedevelopmentofpostthromboticsyndromewasfound[118].
SUMMARYANDRECOMMENDATIONS
Primary,"spontaneous"upperextremitydeepveinthrombosisisestimatedtorepresentbetween1and4
percentofallcasesofupperextremitydeepveinthrombosis,withsecondarycausesofthrombosisrelatedto
centralveincannulation(eg,centralline,pacemaker)orprothromboticstates(eg,thrombophilia,malignancy)
muchmorecommon.(See'Introduction'above.)
Primaryupperextremitydeepveinthrombosisisdefinedasthrombosisofthedeepveinsdrainingtheupper
extremity(axillary,subclavian)duetoanunderlyinganatomicanomalyatthethoracicoutletcausing
compressionorrepetitivevenousinjury.Thesyndromeisappropriatelytermedvenousthoracicoutlet
syndrome,butisalsoreferredtoasPagetSchroettersyndromeandeffortthrombosis.Anatomic
abnormalitiescanbecongenitaloracquired.Congenitalanomaliesconsistofcervicalribs,supernumerary
muscles,abnormaltendoninsertions,orabnormalmuscularortendinousbands.Acquiredabnormalities
includebonyovergrowthduetobonyfracture(usuallyoftheclavicle),orhypertrophyofanteriorscalene
muscleorsubclaviusmuscles,oftenrelatedtorepetitivelifting.(See'Pathogenesis'above.)
Primaryupperextremitydeepveinthrombosistypicallypresentsinyoung,otherwisehealthyindividualsas
sudden,severearmswelling.Althoughmorecommonlyassociatedwithlowerextremitydeepvein
thrombosis,pulmonaryembolismfromupperextremitydeepveinthrombosis(primaryandsecondary)occurs
in4to10percentofpatients.Theclinicianshouldhaveahighindexofsuspicionforthisdisorderwhena
youngpatientwithnoincitingfactorspresentswithsignsofupperextremityvenousthrombosis,or
pulmonaryembolismintheabsenceoflowerextremitysymptoms.(See'Clinicalpresentations'above.)
Adiagnosisofupperextremityvenousoutflowobstruction(ie,deepveinthrombosisorvenousstenosis)may
besuspectedbasedupontheclinicalpresentation,butshouldbeconfirmedwithimaging,typicallyinitially
usingultrasound.Ddimerisusefulforexcludingthrombosisasanetiology,butwillnotexcludevein
compression/stenosiswithoutthrombosisasasourceofsymptoms.Onceadiagnosisofvenousoutflow
obstructionisestablished,aprimaryetiologyshouldbesoughttoidentifytheunderlyinganatomic
abnormalitythatisthesourceoftheobstruction.Weobtainaplainchestradiographonallpatientstoidentify
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anyobviousbonyabnormalities.Moreadvancedimagingmaybenecessarytodemonstrateabnormal
muscularattachmentsordynamicvenouscompression.(See'Diagnosis'above.)
Treatmentofprimaryupperextremitydeepveinthrombosisisaimedatpreventingpulmonaryembolism,
recurrentvenousthrombosisandpostthromboticsyndrome.Forpatientswithacutelysymptomaticprimary
upperextremitydeepveinthrombosis,werecommendanticoagulationovernosuchtherapy(Grade1B).
Anticoagulationhelpstomaintainpatencyofcollateralveins,reducespropagationofthrombus,andis
effectiveforpreventingpulmonaryembolism.Weprefertouseunfractionatedheparintofacilitaterapiddose
adjustmentinpatientswhowillundergootherinterventionssuchasthrombolysisandthoracicoutlet
decompression.
Forpatientswithmoderatetosevereacutesymptomswhoarediagnosedwithprimaryupperextremitydeep
veinthrombosis,wesuggestthrombolysisoveranticoagulationalone(Grade2C).Followingthrombolysis,
wesuggestthoracicoutletdecompression,ratherthannodecompression(Grade2C).Thisapproach
decreasestheriskforrecurrentthrombosisandpostthromboticsyndrome.Anticoagulationalonemaybe
adequateforminimallysymptomaticorintermittentlysymptomaticpatients.Forthosewhopresentina
delayedmanner(>2weeksfromtheonsetofsymptoms),decompressionofthoracicoutletinadditionto
anticoagulationmaybebeneficial.(See'Approachtotreatment'aboveand'Rationaleforaggressive
treatment'above.)
WeagreewithAmericanCollegeofChestPhysicianswhorecommendaminimumofthreemonthsof
anticoagulationfollowinganinitialthromboticevent.Wemaintainanticoagulationforaminimumofthree
monthsregardlessofwhetherintervention(thrombolysis,thoracicoutletdecompression)wasperformed.
(See'Approachtotreatment'above.)
UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.
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GRAPHICS
Anteriorviewsuperficialveinsoftheupper
extremity

Thecephalicveinoriginatesattheradialaspectofthewrist
traversingtheradialborderoftheforearm.Itreceivestributaries
fromboththeventralanddorsalsurfaces.Attheantecubitalfossa
itprovidesatributarytothemediancubitalvein.Intheupperarm,
ittravelsinthegroovebetweenthepectoralismajoranddeltoid
muscles.Itpiercesthecoracoclavicularfasciaand,crossingthe
axillaryartery,endsintheaxillaryveinjustbelowtheclavicle.
Sometimesitcommunicateswiththeexternaljugularveinbya
branchwhichascendsanteriortotheclavicle.
Thebasilicveinoriginatesintheulnaraspectofthewrist
traversingtheulnarsideoftheforearmtotheantecubitalfossa
whereitisjoinedbythemediancubitalvein.Itascendsinthe
groovebetweenthebicepsbrachiiandpronatorteres,crossesthe
brachialarteryattheelbowandcontinuescephaladalongthe
medialborderofthebicepsbrachii.Itperforatesthedeepfasciaof
theupperarmandjoinsthebrachialvein.
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Deepveinsoftheupperextremity

Thedeepveinsoftheupperextremityincludethepairedulnar,radial
andinterosseousveinsintheforearm,pairedbrachialveinsoftheupper
arm,andaxillaryvein.Theaxillaryveinoriginatesatthelowerborderof
theteresmajormuscleincontinuitywiththebrachialveins.Thebasilic
andcephalicveins,whicharesuperficialveins,contributetotheaxillary
vein,thoughmanyanatomicvariationsoccur.Afterpassingtheouter
marginofthefirstrib,theaxillaryveincontinuesasthesubclavianvein.

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Anatomyofthethoracicoutlet

Thethoracicoutletreferstotheconfinedspacebetweentheclavicleand
firstrib.Structuresthatpassthroughthisregionincludethenervesofthe
brachialplexus,thesubclavianarteryandsubclavianvein.
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Cervicalribcausingthoracicoutletsyndrome

Thoracicoutletsyndromesareduetocompressionofanyofthestructures
(brachialplexus,subclavianartery,subclavianvein)thattraversethe
confinedspacebetweentheclavicleandfirstrib.Anatomicabnormaliaties
suchasacervicalrib(shown),musculotendinousabnormalitiesorscarring
cancontributetothepathology.
Graphic50430Version5.0

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Contraindicationstofibrinolytictherapyfordeepvenousthrombosis
oracutepulmonaryembolism
Absolutecontraindications
Priorintracranialhemorrhage
Knownstructuralcerebralvascularlesion
Knownmalignantintracranialneoplasm
Ischemicstrokewithinthreemonths(excludingstrokewithinthreehours*)
Suspectedaorticdissection
Activebleedingorbleedingdiathesis(excludingmenses)
Significantclosedheadtraumaorfacialtraumawithinthreemonths

Relativecontraindications
Historyofchronic,severe,poorlycontrolledhypertension
Severeuncontrolledhypertensiononpresentation(SBP>180mmHgorDBP>110mmHg)
Historyofischemicstrokemorethanthreemonthsprior
Traumaticorprolonged(>10minute)CPRormajorsurgerylessthanthreeweeks
Recent(withintwotofourweeks)internalbleeding
Noncompressiblevascularpunctures
Recentinvasiveprocedure
Forstreptokinase/anistreplasePriorexposure(morethanfivedaysago)orpriorallergic
reactiontotheseagents
Pregnancy
Activepepticulcer
Pericarditisorpericardialfluid
Currentuseofanticoagulant(eg,warfarinsodium)thathasproducedanelevatedinternational
normalizedratio(INR)>1.7orprothrombintime(PT)>15seconds
Age>75years
Diabeticretinopathy
SBP:systolicbloodpressureDBP:diastolicbloodpressureCPR:cardiopulmonaryresuscitation.
*TheAmericanCollegeofCardiologysuggeststhatselectpatientswithstrokemaybenefitfrom
thrombolytictherapywithin4.5hoursoftheonsetofsymptoms.
ReproducedwithpermissionfromtheAmericanCollegeofChestPhysicians.KearonC,AklEA,Comerota
AJ,etal.AntithrombotictherapyforVTEdisease:AntithromboticTherapyandPreventionofThrombosis,
9thed:AmericanCollegeofChestPhysiciansEvidenceBasedClinicalPracticeGuidelines.Chest2012
141:e419S.Copyright2012.
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Disclosures
Disclosures:KaoruGoshima,MDNothingtodisclose.JohnFEidt,MDNothingtodisclose.JosephLMills,Sr,MD
Grant/Research/ClinicalTrialSupport:NIHInstituteofAging(abdominalaorticaneurysmstudy).Consultant/AdvisoryBoards:AnGes
(criticallimbischemia)CescaTherapeutics(criticallimbischemia).Speaker:Gore(bypasssummit[Polytetrafluoroethylene]).Other
financialinterests:Elsevier(vascularsurgerytextbooks).KathrynACollins,MD,PhD,FACSNothingtodisclose.
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseareaddressedbyvettingthrougha
multilevelreviewprocess,andthroughrequirementsforreferencestobeprovidedtosupportthecontent.Appropriatelyreferenced
contentisrequiredofallauthorsandmustconformtoUpToDatestandardsofevidence.
Conflictofinterestpolicy

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