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Overviewofdysphagiainadults
OfficialreprintfromUpToDate
www.uptodate.com2014UpToDate
Overviewofdysphagiainadults
Author
RonnieFass,MD
SectionEditor
MarkFeldman,MD,MACP,
AGAF,FACG
DeputyEditor
ShilpaGrover,MD,MPH
Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Nov2014.|Thistopiclastupdated:Jan08,2014.
INTRODUCTIONDysphagiasuggeststhepresenceofanorganicabnormalityinthepassageofsolidsor
liquidsfromtheoralcavitytothestomach.Patients'complaintsrangefromtheinabilitytoinitiateaswallowtothe
sensationofsolidsorliquidsbeinghinderedduringtheirpassagethroughtheesophagusintothestomach.
Thistopicwillreviewtheinitialevaluationofpatientswithdysphagiaanddiagnostictestinginpatientswith
esophagealdysphagia.Thepathogenesis,diagnosis,andevaluationofpatientswithoropharyngealdysphagiaare
discussedseparately.OurrecommendationsarelargelyconsistentwiththeAmericanGastroenterological
Associationguidelines[1,2].(See"Oropharyngealdysphagia:Etiologyandpathogenesis"and"Oropharyngeal
dysphagia:Clinicalfeatures,diagnosis,andmanagement".)
DEFINITIONS
Dysphagiaisdefinedasasubjectivesensationofdifficultyorabnormalityofswallowing.
Odynophagiaisdefinedaspainwithswallowing.
Globussensationisdefinedasapersistentorintermittentnonpainfulsensationofalumporforeignbodyin
thethroatwiththeoccurrenceofthesensationbetweenmealsandtheabsenceofdysphagia,odynophagia,
anesophagealmotilitydisorder,orgastroesophagealrefluxasthecauseofsymptoms.Thesecriteriamust
befulfilledforthelastthreemonthswithsymptomonsetatleastsixmonthsbeforeadiagnosisofglobus
sensationcanbemade[3].(See"Globussensation".)
Dysphagiacanbeclassifiedasfollows:
OropharyngealdysphagiaOropharyngealortransferdysphagiaischaracterizedbydifficultyinitiatinga
swallow.Swallowingmaybeaccompaniedbycoughing,choking,nasopharyngealregurgitation,aspiration,
andasensationofresidualfoodremaininginthepharynx.(See"Oropharyngealdysphagia:Etiologyand
pathogenesis",sectionon'Etiologyandpathogenesis'and"Oropharyngealdysphagia:Clinicalfeatures,
diagnosis,andmanagement".)
EsophagealdysphagiaEsophagealdysphagiaischaracterizedbydifficultyswallowingseveralseconds
afterinitiatingaswallowandasensationoffoodgettingstuckintheesophagus.
EVALUATIONDysphagiaisanalarmsymptomthatwarrantsimmediateevaluationtodefinetheexactcause
andinitiateappropriatetherapy.Dysphagiainolderadultsubjectsshouldnotbeattributedtonormalaging.Aging
alonecausesmildesophagealmotilityabnormalities,whicharerarelysymptomatic[4].
HistoryThefirststepinevaluatingpatientswithdysphagiaistotrytodeterminebycarefulquestioningifthe
symptomsareduetooropharyngealoresophagealdysphagia(table1)[5].Patientswithoropharyngealdysphagia
havedifficultyinitiatingaswallowandoftenpointtowardthecervicalregionwhenaskedtoidentifythesiteoftheir
symptoms.Oraldysfunctioncanleadtodrooling,foodspillage,sialorrhea,piecemealswallows,anddysarthria.
Pharyngealdysfunctioncanleadtocoughingorchokingduringfoodconsumption,anddysphonia.Incontrast,
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patientswithesophagealdysphagiahavedifficultyswallowingseveralsecondsafterinitiatingaswallowanda
sensationoffoodgettingstuckinthesuprasternalnotchorbehindthesternum.Whileretrosternaldysphagia
usuallycorrespondswiththelocationofthelesion,suprasternaldysphagiaiscommonlyreferredfrombelow[6].
Theevaluationofpatientswithoropharyngealdysphagiatodeterminetheetiologyisdiscussedindetail
separately.(See'Definitions'aboveand"Oropharyngealdysphagia:Clinicalfeatures,diagnosis,and
management",sectionon'Definitions'and"Oropharyngealdysphagia:Clinicalfeatures,diagnosis,and
management",sectionon'Determiningtheetiology'.)
Inpatientswithesophagealdysphagia,acriticalcomponentofthemedicalhistoryisdeterminingthetypesoffood
thatproducesymptoms(solids,liquids,orboth)andthetemporalprogressionofsymptoms(algorithm1).
Dysphagiatobothsolidsandliquidsfromtheonsetofsymptomsisprobablyduetoamotilitydisorderofthe
esophagus.Incontrast,dysphagiaforsolidsthatlaterprogressestoinvolveliquidsismorelikelytoreflect
mechanicalobstruction[7].
Progressivedysphagia,beginningwithdysphagiatosolidsfollowedbydysphagiatoliquids,isusuallycausedby
cancerorapepticstricture.Symptomsofpepticstricturesareusuallyinsidiousandgraduallyprogressive,
whereasthoseduetoamalignancyprogressmorerapidly[8].Symptomscorrespondtothecaliberofthestricture
dysphagiatosolidsisusuallypresentwhentheesophageallumenisnarrowedto13mmorless.Intermittent
dysphagiaismostoftenrelatedtoaloweresophagealringorweb.Patientswithmotilitydisordersmayalso
exhibitprogressivedysphagia(usuallythosewithachalasiaorscleroderma)ormayexhibit
intermittent/nonprogressivedysphagia(usuallythosewithhypertensiveloweresophagealsphincter,diffuse
esophagealspasm,ornonspecificmotilitydisorders).
Associatedsymptomsorfindingssuchasheartburn,weightloss,hematemesis,coffeegroundemesis,anemia,
regurgitationoffoodparticles,andrespiratorysymptomscanfurtherhelptonarrowthedifferentialdiagnosis
(algorithm2).Asanexample,chronicheartburninapatientwithdysphagiamaybeacluetothepresenceof
complicationsofgastroesophagealrefluxdisease,suchaserosiveesophagitis,pepticstricture,and
adenocarcinomaoftheesophagus.However,theabsenceofheartburndoesnotruleoutrefluxrelated
complications,sinceapproximatelyonefourthofpatientswithpepticstrictureandatleastonethirdofthosewith
adenocarcinomaoftheesophagushavenohistoryofheartburn[9,10].Furthermore,morethan40percentof
patientswithachalasiacomplainofretrosternalburningconsistentwithheartburn[11].(See"Complicationsof
gastroesophagealrefluxinadults"and"Clinicalmanifestationsanddiagnosisofachalasia",sectionon'Clinical
manifestations'.)
DiagnostictestingDiagnostictestingtodeterminetheetiologyofesophagealdysphagiashouldbebasedupon
themedicalhistory(algorithm2).Diagnostictestinginpatientswithoropharyngealdysphagiaisdiscussedindetail
separately.(See"Oropharyngealdysphagia:Clinicalfeatures,diagnosis,andmanagement",sectionon
'Determiningtheetiology'.)
UpperendoscopyPatientswithesophagealdysphagiashouldbereferredforanupperendoscopy[12].In
additiontoitsdiagnosticvalue,endoscopyoffersanopportunitytoobtaintissuesamplestodeterminethe
etiology,andtoperformatherapeuticintervention(eg,dilationofanesophagealring).(See"Esophagealringsand
webs",sectionon'Treatment'.)
BariumswallowWeperformabariumswallowinthefollowingpatients:
Astheinitialtest(priortoupperendoscopy)inpatientswithahistoryorclinicalfeaturessuggestiveofa
proximalesophageallesion(eg,surgeryforlaryngealoresophagealcancer,Zenker'sdiverticulum,or
radiationtherapy),aknowncomplex(tortuous)stricture(eg,priorcausticinjuryorradiationtherapy)[2].In
thesepatients,intubationoftheproximalesophagusduringendoscopyisdonerelativelyblindly,thereby
riskingperforationduetoupperesophagealpathology.However,itisimportanttonotethatperforminga
bariumswallowpriortoanupperendoscopyinsuchpatientshasnotbeendemonstratedtodecreasetherate
ofendoscopiccomplicationsorimproveoutcomes[1].
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Followinganegativeupperendoscopyinpatientsinwhomamechanicalobstructionissuspected,aslower
esophagealringsorextrinsicesophagealcompressioncanbemissedbyanupperendoscopy[13].
Patientsshouldbeinstructedtodrinkbariumintheproneobliquepositionmaximaldistensionofthe
esophagogastricjunctionisachievedbyhavingthepatientswallowbariumrapidlyinassociationwithavarietyof
respiratorymaneuvers[14].Inaddition,askingthepatienttoswallow13mmbariumtabletsorasolidbolus,such
asamarshmalloworbread,maybehelpfulfordemonstratingsubtlelesionsinpatientswithpersistentor
intermittentsolidfooddysphagia[15,16].
MotilitytestingMotilitytestingshouldbeperformedinpatientswithdysphagiainwhomupperendoscopyis
unrevealingand/oranesophagealmotilitydisorderissuspected.Althoughcertainmotilitydisorders(eg,achalasia)
canbestronglysuspectedbasedupontheircharacteristicradiographicappearancewheninadvancedstages
(image1),confirmationwithamotilitystudyisrequiredtoestablishthediagnosis[2,13,17,18].Anonspecific
motilitydisorderorachalasiacanbedetectedinupto50percentofpatientswithnonstructuraldysphagia[19,20].
(See"Motilitytesting:Whendoesithelp?"and"Clinicalmanifestationsanddiagnosisofachalasia"and"Clinical
manifestationsanddiagnosisofachalasia",sectionon'Evaluation'.)
DIFFERENTIALDIAGNOSISOFESOPHAGEALDYSPHAGIAEsophagealdysphagiaariseswithinthebody
oftheesophagus,theloweresophagealsphincter,orcardia.Alargenumberofconditionsareassociatedwith
esophagealdysphagia,themostcommonofwhichwillbereviewedhere(table2).Thedifferentialdiagnosisof
oropharyngealdysphagiaisdiscussedindetail,separately(table3).(See"Oropharyngealdysphagia:Clinical
features,diagnosis,andmanagement",sectionon'Determiningtheetiology'.)
Intraluminalcauses
FoodimpactionFoodimpactionisbyfarthemostcommoncauseforacutedysphagiainadults.The
estimatedannualincidenceis13.0per100,000andwithahigherincidenceinmalesascomparedwithfemales
(1.7:1)[21].Theincidenceincreaseswithage,especiallyaftertheseventhdecade.Patientsusuallydevelop
symptomsafteringestingmeat(mostcommonlybeef,chicken,andturkey),whichcompletelyobstructsthe
esophageallumen,resultinginexpectorationofsaliva[22].Thefoodboluscanberemovedusinggraspingdevices
(eitherenblocorpiecemeal,dependingupontheconsistencyofthebolus),oritcanbegentlypushedintothe
stomachusinganendoscope[21,23].Endoscopicmanagementoffoodimpactionisdiscussedindetail
separately.(See"Ingestedforeignbodiesandfoodimpactionsinadults",sectionon'Foodbolus'.)
Intrinsiccauses
EsophagealstricturePepticstrictureisacomplicationofacidreflux,whichoccursinapproximately10
percentofpatientswithgastroesophagealrefluxdisease(GERD)whoseekmedicalattention[24,25].The
developmentofpepticstricturesamongpatientswithrefluxhasbeenassociatedwitholderage,malegender,and
longerdurationofrefluxsymptoms[26].InadditiontoGERD,pepticstrictureshavebeenobservedinanumberof
otherconditionsthatleadtoincreasedesophagealacidexposure(eg,systemicsclerosis,ZollingerEllison
syndrome,nasogastrictubeplacement,andHellermyotomyforachalasia).
Patientswithavarietyofotherdisorders,suchasinfectiousesophagitis,postsurgicalresectionforesophagealor
laryngealcancer,causticingestion,pillesophagitis,andradiationexposure,maydevelopnarrowingofthe
esophagusthatissimilartoapepticstricturedespiteitsnonpepticorigin.
EosinophilicesophagitisUpto15percentofpatientsbeingevaluatedfordysphagiawithendoscopyare
foundtohaveeosinophilicesophagitis[2729].Adultsandteenagersfrequentlypresentwithdysphagiaandfood
impactions[27].Anumberofendoscopicfindingshavebeenassociatedwitheosinophilicesophagitisincluding
stackedcircularrings,strictures,linearfurrows,whitepapulesandasmallcaliberesophagus.Individual
endoscopicfeaturessuggestiveofeosinophilicesophagitishavelowsensitivityrangingfrom15to48percentbut
highspecificityrangingfrom90to95percent[30].Thediagnosisofeosinophilicesophagitisisestablishedby
upperendoscopyandesophagealbiopsywhichdemonstratesanincreasednumberofeosinophils(>15perhigh
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powerfield).(See"Clinicalmanifestationsanddiagnosisofeosinophilicesophagitis",sectionon'Endoscopy'.)
EsophagealwebsandringsEsophagealwebsandringscanpartiallyorcompletelycompromisethe
esophageallumen[31].Theycanbesolitaryormultiple.
Anesophagealwebisathinmucosalfoldthatprotrudesintotheesophageallumenandiscoveredwith
squamousepithelium.Websmostcommonlyoccuranteriorlyinthecervicalesophagus,causingfocal
narrowinginthepostcricoidarea(image2).
Esophagealringsaretypicallymucosalstructuresbutinrarecasesaremuscular.Ringsarefoundatthe
gastroesophagealjunction,aresmooth,thin(<4mminaxiallength),andcoveredwithsquamousmucosa
aboveandcolumnarepitheliumbelow(picture1andimage3)[32].
Patientswithesophagealringsandwebshaveintermittentdysphagiaforsolids.Esophagealringshavebeen
describedinassociationwithirondeficiency(thePlummerVinsonorPatersonKellysyndrome)inwhichcase
anemia,koilonychia,orothermanifestationsofirondeficiencymaybepresent(image4)[33].
Anesophagealweb/ringisdiagnosedonbariumswallowandupperendoscopyandappearsasafocal,thick
constrictionofvariableluminaldiameter[34].Ringsareusuallyfoundatorafewcentimetersabovethe
squamocolumnarjunction.Endoscopyislesssensitivethanthebariumesophagramindetectingesophagealrings
andaringmaybemissedunlesstheloweresophagusiswidelydistended[35].Thecaliberofamuscularring
changesduringperistalsis,distinguishingitfromapepticstrictureormucosalring.(See"Esophagealringsand
webs",sectionon'Clinicalpresentationanddiagnosis'.)
CarcinomaCanceroftheesophagusorgastriccardiaisassociatedwithrapidlyprogressivedysphagia,
initiallyforsolidsandlaterforliquids.Inaddition,patientsmayhavechestpain,odynophagia,anemia,anorexia,
andsignificantweightloss.
Anachalasialikesyndrome(pseudoachalasia)hasalsobeendescribedinpatientswithadenocarcinomaofthe
cardiaduetomicroscopicinfiltrationofthemyentericplexusorthevagusnerve[36].Certainfeaturesincreasethe
likelihoodthatthepatienthaspseudoachalasiaduetomalignancy[37].Theseincludeshortdurationofsymptoms
(lessthansixmonths),presentationafterage60,excessiveweightlossinrelationtothedurationofsymptoms,
anddifficultpassageoftheendoscopethroughthegastroesophagealjunction.Insuchcases,endoscopic
ultrasonographywithfineneedleaspiration(EUSFNA)shouldbeperformedtodiagnoseanunderlyingmalignancy.
(See"Clinicalmanifestationsanddiagnosisofachalasia"and"Epidemiology,pathobiology,andclinical
manifestationsofesophagealcancer".)
RadiationinjuryPatientsundergoingradiationtherapyforthoracicorheadandnecktumorsareatriskfor
developingesophagitisandesophagealstrictures.Intheacutesetting,patientsmaydevelopesophagitisresulting
indysphagiaandodynophagia.Insomepatients,chronicischemiaandfibrosisleadtochronicradiation
esophagitis,whichmaypresentasesophagealulcerationsorstricturesintheproximalesophagus[38].Although
controversial,anotherpotentialcauseofdysphagiainpatientswhohavereceivedthoracicradiationisamotility
disorder[39,40].(See"Gastrointestinaltoxicityofradiationtherapy",sectionon'Esophagitis'.)
LymphocyticesophagitisLymphocyticesophagitisischaracterizedbythepresenceofadense
peripapillarylymphocyticinfiltrateandperipapillaryspongiosisinvolvingthelowertwothirdsoftheesophageal
epitheliumandtheabsenceofsignificantneutrophilicoreosinophilicinfiltrates[41].Whilelymphocyticesophagitis
isbeingincreasinglyrecognizedonhistopathologyinadultsandhasbeenassociatedwithdysphagia,itisunclear
ifitisadistinctclinicalentityanditsetiologyisunknown[4244].
Inoneretrospectivestudyof129,252adultswhohadundergoneanupperendoscopy,0.1percenthadlymphocytic
esophagitisonbiopsy[42].Ascomparedwithpatientswithnormalesophagealbiopsies,patientswithlymphocytic
esophagitisweresignificantlymorelikelytobeolder(63versus55years)andtohavepresentedwithdysphagia
(53versus33percent),andweresignificantlylesslikelytohaveGERD(19versus38percent).
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InfectiousesophagitisPatientswithinfectiousesophagitis,especiallyduetoherpessimplexvirus(HSV),
usuallypresentwithodynophagiaand/ordysphagia[45,46].Othercausesofinfectiousesophagitisinclude
cytomegalovirus(CMV)andCandidaspecies.AlthoughCandidaspeciesarethemostcommonfungalcauseof
esophagitis,otherfungalinfectionsincludingcryptococcosis,histoplasmosis,blastomycosis,andaspergillosis
haverarelybeendescribed[47].Otherpathogens,suchasmycobacteriaandnocardia,occasionallycause
esophagitis[47,48].(See"Herpessimplexvirusinfectionoftheesophagus",sectionon'Clinicalmanifestations'
and"Herpessimplexvirusinfectionoftheesophagus",sectionon'Diagnosis'and"Clinicalmanifestationsof
oropharyngealandesophagealcandidiasis",sectionon'Esophagealcandidiasis'.)
Extrinsiccauses
CardiovascularabnormalitiesAnumberofvascularanomaliescancausedysphagiabycompressingthe
esophagus("dysphagialusoria")butarerare[49].Someoftheaberrantvesselsformcompleterings,whileothers
formincompleteringsaroundtheesophagus[50].(See"Vascularrings".)
Completevascularringanomaliesincludeadoubleaorticarch,rightaorticarchwithretroesophagealleft
subclavianarteryandleftligamentumarteriosum,andrightaorticarchwithmirrorimagebranchingandleft
ligamentumarteriosum[50].Dysphagialusoriaisrare.Extrinsiccompressionoftheesophagusmaybenoted
onbariumswallow,andthediagnosiscanbeestablishedbyendoscopicultrasonographyorCTscan[49].
Incompletevascularringanomaliesincluderetroesophagealrightaberrantsubclavianarteryandanomalous
leftpulmonaryartery[50].
Inolderadults,severeatherosclerosisoralargeaneurysmofthethoracicaortacanresultinimpingementon
theesophagusandproducedysphagia("dysphagiaaortica").
Whensymptomsareintractable,surgicalinterventionshouldbeconsidered.Whenduetocongenitalcauses,
symptomsusuallydevelopduringchildhood,buttheymayalsodevelopinadults.
Mostsubjectswithanaberrantsubclavianarteryaresymptomfreethroughouttheirlives[51].However,coughing,
dysphagia,thoracicpain,orevenHorner'ssyndromemaydevelopatanolderage[52].Ininfants,thereisan
increaseinpulmonaryinfectionsandrespiratoryabnormalities.
Enlargementoftheleftatriummaycausedysphagiainpatientswithmitralvalvedisease[53].Thisisdueto
extrinsiccompressionbytheenlargedatrium,resultinginpartialluminalobstructionatthemidtolowerthird
portionoftheesophagus[54].
Motilitydisorders
AchalasiaPrimaryachalasiaisadiseaseofunknownetiologyinwhichthereisalossofperistalsisinthe
distalesophagusandafailureofloweresophagealsphincter(LES)relaxationwithswallowing(image1andimage
5).(See"Pathophysiologyandetiologyofachalasia".)
Achalasiaisanuncommondisorderthatcanoccuratanyage,butisusuallydiagnosedinpatientsbetween25
and60years.Menandwomenareaffectedwithequalfrequency.Progressivelyworseningdysphagiaforsolids
(91percent)andliquids(85percent)andregurgitationofblandundigestedfoodorsalivaarethemostfrequent
symptomsinpatientswithachalasia.Othersymptomsincludechestpain,heartburn,anddifficultybelching.
Findingsonbariumesophagramthataresuggestiveofachalasiaincludeadilatedesophagusthatterminatesina
beaklikenarrowing,aperistalsis,andpooremptyingofbariumfromtheesophagus.However,bariumesophagram
maybefalselynegativeinonethirdofpatients[55].Manometryisrequiredtoestablishthediagnosisofachalasia.
AperistalsisinthedistaltwothirdsoftheesophagusandincompleteLESrelaxationonconventionalmanometry
arecharacteristicofachalasia.(See"Clinicalmanifestationsanddiagnosisofachalasia",sectionon'Evaluation'
and"Clinicalmanifestationsanddiagnosisofachalasia",sectionon'Diagnosis'.)
SpasticmotilitydisordersDiffuseesophagealspasm(DES),nutcrackeresophagus,hypertensivelower
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esophagealsphincter,andineffectiveesophagealmotilitycancauseintermittentnonprogressivedysphagiato
solidsandliquids.Patientsmayalsoreportassociatedchestpain[56].Thebariumradiographicpictureincludesa
broadspectrumofseverenonperistalticcontractions,whichmayproducestrikingabnormalitiesinthebarium
column.Thesefindingshaveresultedindescriptionssuchas"rosarybead"or"corkscrew"esophagus(image6
andimage7).However,radiographicstudiesmaybeentirelynormalamongpatientswithDESorbeabnormalin
patientswithnormalmotilityasaresult,thesetestsareneithersensitivenorspecific.Manometryisrequiredto
establishthediagnosisofaspasticesophagealmotilitydisorder.Thespecificmanometriccriteriatodiagnose
DES,nutcrackeresophagus,andhypertensiveLESarediscussedindetail,separately.(See"Distalesophageal
spasm,nutcrackeresophagus,andhypertensiveloweresophagealsphincter",sectionon'Manometry'.)
Ineffectiveesophagealmotilitydisorder(IEMD)isdefinedonmanometrybyatleast30percentofthedistal
esophagealamplitudecontractionsbelow30mmHg.Inonestudy,approximately30percentofsubjectswith
IEMDreporteddysphagia.However,studiesusingesophagealintraluminalimpedancetestinghaveshownthatup
to68percentofliquidand59percentofviscousswallowsinsuchpatientsshowednormalbolustransit[57].
Overallonethirdofpatientshadnormalbolustransit,suggestingthatthemanometricdiagnosisofIEMDdoesnot
alwayscorrelatewiththeeffectivenessofesophagealfunction.
Systemicsclerosis(scleroderma)Patientswithsystemicsclerosisoftenhaveahistoryofheartburnand
progressivedysphagiatobothsolidsandliquidssecondarytotheunderlyingmotilityabnormalityorthepresence
ofpepticstricture,whichoccursinupto50percentofthesepatients[58].Thediagnosisofsystemicsclerosisis
suggestedbythepresenceofskinthickeningandhardening(sclerosis)thatisnotconfinedtoonearea(ie,not
localizedscleroderma).Thediagnosisissupportedbythepresenceofextracutaneousfeaturesandcharacteristic
serumautoantibodies.(See'Esophagealstricture'aboveand"Diagnosisanddifferentialdiagnosisofsystemic
sclerosis(scleroderma)inadults",sectionon'Evaluationforsuspectedsystemicsclerosis'.)
Esophagealinvolvementispresentinupto90percentofpatientswithsystemicsclerosis(table4)[5961].
Sclerodermaprimarilyinvolvesthesmoothmusclelayerofthegutwall,resultinginatrophyandsclerosisofthe
distaltwothirdsoftheesophagus[59].Asaresult,themostcommonmotilityabnormalitiesobservedinthedistal
twothirdsoftheesophagusareaperistalsisorlowamplitudecontractions,andloworabsentloweresophageal
sphincterpressure[62].Theproximalesophagus(striatedmuscle)issparedandexhibitsnormalmotility.(See
"Gastrointestinalmanifestationsofsystemicsclerosis(scleroderma)".)
Sjgren'ssyndromeApproximatelythreequartersofpatientswithSjgren'ssyndromehaveassociated
dysphagia[63,64].Defectiveperistalsishasbeendemonstratedinonethirdormoreofpatientswithprimary
Sjgrenssyndrome[65,66].Xerostomiaappearstoexacerbateswallowingdiscomfortbutdoesnotappearto
correlatewithdysphagia[63].ThediagnosisofSjgrenssyndromeisdiscussedseparately.(See"Clinical
manifestationsofSjgren'ssyndrome:Exocrineglanddisease"and"ClinicalmanifestationsofSjgren's
syndrome:Extraglandulardisease",sectionon'Gastrointestinaltract'and"Diagnosisandclassificationof
Sjgren'ssyndrome",sectionon'Diagnosis'.)
FunctionaldysphagiaAccordingtotheRomeIIIcriteria,functionaldysphagiaisdefinedasasenseofsolidor
liquidfoodlodgingorpassingabnormallythroughtheesophagus,absenceofevidencethatGERDisthecauseof
thesymptoms,andabsenceofamotilitydisorder.Allcriteriamustbefulfilledforthepastthreemonthswith
symptomonsetatleastsixmonthspriortothediagnosis.[3,67,68].
Symptomsofdysphagiamaybeintermittentorpresentaftereachmeal.Patientsshouldbereassuredand
instructedtoavoidprecipitatingfactorsandchewwell.Inourexperience,symptomsmayimprovewithtime.In
patientswithseveresymptomsdespitethesemeasures,treatmentwithacalciumchannelblocker,anticholinergic
agent,antidepressant,anxiolytic,orsmoothmusclerelaxantshouldbeconsidered.Althoughempiricesophageal
dilationwitha50to54FMaloneydilatorhasbeendemonstratedtoimprovesymptomsinpatientswithfunctional
dysphagiainatleastonerandomizedtrial,conflictingresultshavealsobeenreported[69,70].
INFORMATIONFORPATIENTSUpToDateofferstwotypesofpatienteducationmaterials,"TheBasics"and
"BeyondtheBasics."TheBasicspatienteducationpiecesarewritteninplainlanguage,atthe5thto6thgrade
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readinglevel,andtheyanswerthefourorfivekeyquestionsapatientmighthaveaboutagivencondition.These
articlesarebestforpatientswhowantageneraloverviewandwhoprefershort,easytoreadmaterials.Beyond
theBasicspatienteducationpiecesarelonger,moresophisticated,andmoredetailed.Thesearticlesarewritten
atthe10thto12thgradereadinglevelandarebestforpatientswhowantindepthinformationandarecomfortable
withsomemedicaljargon.
Herearethepatienteducationarticlesthatarerelevanttothistopic.Weencourageyoutoprintoremailthese
topicstoyourpatients.(Youcanalsolocatepatienteducationarticlesonavarietyofsubjectsbysearchingon
"patientinfo"andthekeyword(s)ofinterest.)
Basicstopics(see"Patientinformation:Dysphagia(TheBasics)"and"Patientinformation:Upperendoscopy
(TheBasics)"and"Patientinformation:Esophagealstricture(TheBasics)")
BeyondtheBasicstopics(see"Patientinformation:Upperendoscopy(BeyondtheBasics)")
SUMMARYANDRECOMMENDATIONS
Dysphagiaisasubjectivesensationofdifficultyorabnormalityofswallowing.Odynophagiaisdefinedas
painwithswallowing.(See'Definitions'above.)
Dysphagiacanbeclassifiedasoropharyngealdysphagiaoresophagealdysphagia.Oropharyngealortransfer
dysphagiaischaracterizedbydifficultyinitiatingaswallow.Swallowingmaybeaccompaniedbycoughing,
choking,nasopharyngealregurgitation,aspiration,andasensationofresidualfoodremaininginthepharynx.
Esophagealdysphagiaischaracterizedbydifficultyswallowingseveralsecondsafterinitiatingaswallowand
asensationoffoodgettingstuckintheesophagus.(See'Definitions'above.)
Dysphagia(algorithm1andalgorithm2)warrantsimmediateevaluationtodefinetheexactcauseandinitiate
appropriatetherapy
Thefirststepinevaluatingpatientswithdysphagiaistodetermineifthesymptomsareduetooropharyngeal
oresophagealdysphagiabyhistory(table1).Furtherevaluationtodeterminetheetiologyoforopharyngeal
dysphagiaisdiscussedseparately.(See'History'aboveand"Oropharyngealdysphagia:Clinicalfeatures,
diagnosis,andmanagement",sectionon'Determiningtheetiology'.)
Esophagealdysphagiamaybeduetointraluminalcauses,intrinsicesophagealcauses,extrinsic
compressionoftheesophagusorduetoanunderlyingesophagealmotilitydisorder(table2).Diagnostic
testingforesophagealdysphagiashouldbebasedonthehistory(algorithm2).(See'Differentialdiagnosisof
esophagealdysphagia'above.)
Functionaldysphagiaisdefinedasasenseofsolidorliquidfoodlodgingorpassingabnormallythroughthe
esophagus,absenceofevidencethatgastroesophagealrefluxdisease(GERD)isthecauseofthe
symptoms,andabsenceofamotilitydisorder.Allcriteriamustbefulfilledforthepastthreemonthswith
symptomonsetatleastsixmonthspriortothediagnosis.(See'Functionaldysphagia'above.)
UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.
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52. JanssenM,BaggenMG,VeenHF,etal.Dysphagialusoria:clinicalaspects,manometricfindings,
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53. CappellMS.Manometricfindingsindysphagiasecondarytoleftatrialdilatation.Giant,cyclicmidesophageal
pressurewavesoccurringwitheveryheartbeat.DigDisSci199136:693.
54. GotsmanI,MogleP,ShapiraMY.Anunusualcauseofdysphagia.PostgradMedJ199975:629.
55. HowardPJ,MaherL,PrydeA,etal.Fiveyearprospectivestudyoftheincidence,clinicalfeatures,and
diagnosisofachalasiainEdinburgh.Gut199233:1011.
56. ClouseRE.Spasticdisordersoftheesophagus.Gastroenterologist19975:112.
57. TutuianR,CastellDO.Clarificationoftheesophagealfunctiondefectinpatientswithmanometric
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motilityandacidrefluxin36patients.AmJGastroenterol199388:870.
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syndrome.JRheumatol19763:63.
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69. ColonVJ,YoungMA,RamirezFC.Theshortandlongtermefficacyofempiricalesophagealdilationin
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Topic2241Version12.0
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GRAPHICS
Questionstoaskpatientswithdysphagia
Doyouhaveproblemsinitiatingaswallowordoyoufeelfoodgettingstuckafewsecondsafter
swallowing?(Helpsdistinguishoropharyngealfromesophagealdysphagia.)
Doyoucoughorchokeorisfoodcomingbackthroughyournoseafterswallowing?(Coughing,
choking,ornasalregurgitationsuggestsaspirationandoropharyngealdysphagia.)
Doyouhaveproblemswallowingsolids,liquids,orboth?(Liquids,notsolids,suggestsamotility
disordersolidsprogressingtoliquidssuggestsabenignormalignantobstruction.)
Howlonghaveyouhadproblemsswallowingandhaveyoursymptomsprogressed,remained
stable,oraretheyintermittent?(Rapidlyprogressivedysphagiaisconcerningformalignancy.)
Couldyoupointtowhereyoufeelfoodisgettingstuck?(Abilitytolocalizesourceofdysphagiais
unreliablebestwithoropharyngealdysphagia.)
Doyouhaveothersymptomssuchaslossofappetite,weightloss,nausea,vomiting,regurgitation
offoodparticles,heartburn,vomitingfreshoroldblood,painduringswallowing,orchestpain?
Doyouhavemedicalproblemssuchasdiabetesmellitus,scleroderma,Sjgren'ssyndrome,overlap
syndrome,AIDS,neuromusculardisorders(stroke,Parkinson's,myastheniagravis,muscular
dystrophy,multiplesclerosis),cancer,Chagas'diseaseorothers?
Haveyouhadsurgeryonyourlarynx,esophagus,stomach,orspine?
Haveyoureceivedradiationtherapyinthepast?
Whatmedicationsareyouusingnow(askspecificallyaboutpotassiumchloride,alendronate,
ferroussulfate,quinidine,ascorbicacid,tetracycline,aspirinandNSAIDs)?(Pillesophagitiscan
causedysphagia.)
AIDs:acquiredimmunedeficiencysyndromeNSAIDs:nonsteroidalantiinflammatorydrugs.
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Diagnosisofdysphagia
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Approachtothepatientwithesophagealdysphagia
GERD:gastroesophagealrefluxdisease.
*Performingabariumswallowpriortoanupperendoscopyiscontroversial.
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Achalasia
Bariumswallowina62yearoldmandemonstratesadilated,barium
filledesophaguswitharegionofpersistentnarrowing(arrow)atthe
gastroesophagealjunction,producingthesocalledbird'sbeak
appearance.Achalasiawasconfirmedwithmanometryandthepatient
underwentsuccessfuldilationoftheesophagus.
CourtesyofJonathanKruskal,MD.
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Causesofesophagealdysphagia
Mechanicallesions
Intrinsic
Benigntumors
Causticesophagitis/stricture
Diverticula
Malignancy
Pepticstricture
Eosinophilicesophagitis
Infectiousesophagitis
Pillesophagitis
Postsurgery(laryngeal,esophageal,gastric)
Radiationesophagitis/stricture
Ringsandwebs
Lymphocyticesophagitis
Extrinsic
Aberrantsubclavianartery
Cervicalosteophytes
Enlargedaorta
Enlargedleftatrium
Mediastinalmass(lymphadenopathy,lungcancer,etc)
Postsurgery(laryngeal,spinal)
Motilitydisorders
Achalasia
Chagas'disease
Primarymotilitydisorders
Secondarymotilitydisorders
Functional
Functionaldysphagia
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Representativecausesoforopharyngealdysphagia
Iatrogenic
Medicationsideeffects(chemotherapy,
neuroleptics,etc)
Postsurgicalmuscularorneurogenic
Radiation
Corrosive(pillinjury,intentional)
Infectious
Mucositis(herpes,cytomegalovirus,
Candida,etc)
Diptheria
Botulism
Lymedisease
Syphilis
Metabolic
Amyloidosis
Cushing'ssyndrome
Thyrotoxicosis
Wilsondisease
Myopathic
Connectivetissuedisease(overlap
syndrome)
Dermatomyositis
Myastheniagravis
Neurological
Brainstemtumors
Headtrauma
Stroke
Cerebralpalsy
GuillainBarrsyndrome
Huntingtondisease
Multiplesclerosis
Polio
Postpoliosyndrome
Tardivedyskinesia
Metabolicencephalopathies
Amyotrophiclateralsclerosis
Parkinsondisease
Dementia
Structural
Cricopharyngealbar
Zenker'sdiverticulum
Cervicalwebs
Oropharyngealtumors
Osteophytesandskeletalabnormalities
Congenital(cleftpalate,diverticula,
pouches,etc)
Myotonicdystrophy
Oculopharyngealdystrophy
Polymyositis
Sarcoidosis
Paraneoplasticsyndromes
Adaptedfrom:CookIJ,KahrilasPJ.AGA:Technicalreview:Managementoforopharyngealdysphagia.
Gastroenterology1999116:455.
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Esophagealwebonbariumswallow
Thismodifiedbariumswallow,obtainedina45yearoldmanwith
dysphagia,demonstratesanasymmetricesophagealwebarisingfrom
therightsideoftheupperesophagus(arrow).
CourtesyofJonathanKruskal,MD,PhD.
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Esophageal(Schatzki)ring
Endoscopicviewofanesophageal(Schatzki)ring,whichoftencannot
bewellvisualizedunlesstheloweresophagusiswidelydistended.
Theringappearsasathinmembranewithaconcentricsmooth
contourthatprojectsintothelumen.
CourtesyofJamesBMcGee,MD.
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Esophageal(Schatzki)ringseenonbarium
swallow
Esophageal(Schatzki)ringatthegastroesophagealjunction
visualizedonabariumswallow.
CourtesyofPeterJKahrilas,MD.
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BariumswallowinapatientwithPlummerVinson
syndrome
Thisbariumswallowstudyobtainedina53yearoldfemalewith
dysphagiaandanemiademonstratesanupperesophagealweb(black
arrow)immediatelyaboveatightstrictureoftheesophagus(white
arrow).
CourtesyofJonathanKruskal,MD,PhD.
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Achalasia
Bariumswallowshowingadilatedesophagusandbird'sbeak
appearancetypicalofachalasia.Retainedfoodisalsovisible.
CourtesyofRamDickman,MD.
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Diffuseesophagealspasm
Thisbariumswallowinanoldermanwithnoncardiogenicchestpain
showsmultipleareasofspasm(arrows)throughoutthelengthofthe
esophagus.Thisspasmwasaccentuatedbystasiswithinthe
esophageallumenandesophagitis.
CourtesyofJonathanKruskal,MD,PhD.
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Corkscrewesophagus
Esophagramperformedina72yearoldmanwithintractable
retrosternalpainandrefluxshowsmarkedspasmthroughoutthe
lengthoftheesophagus,whichproducesacorkscrewlikeappearance.
CourtesyofJonathanKruskal,MD,PhD.
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Manifestationsofgutinvolvementinsystemicsclerosis
Site
Mouth
Esophagus
Stomach
Disorder
Symptom
Investigation
Tightskin,smalloral
aperture
Cosmetic,feeding
difficulty
None
Dentalcaries
Toothache
Dentalradiograph
Siccasyndrome
Drymouth
Salivaryglandbiopsy
Hypomotility
Dysphagia
Manometry
Refluxesophagitis
Heartburn/dysphagia
Endoscopy/24hourpH
study
Stricture
Dysphagia
Bariumswallow,
endoscopy
Gastroparesis
Anorexia
Scintigram
Nausea/vomiting
Earlysatiety
Smallbowel
NSAIDrelatedulcer
Dyspepsia
Endoscopy
Hypomotility
Weightloss
Bariumfollowthrough
Stasis
Postprandialbloating
14Cglycocholateor
hydrogenbreathtest
Bacterialovergrowth
Malabsorption
Jejunalaspiration
Steatorrhea
Nausea,cramps
Largebowel
Anus
Pseudoobstruction
Abdominalpain
distension
NSAIDulceration
Bloodydiarrhea
Pneumatosis
intestinalis
Benign
pneumoperitoneum
Plainabdominal
radiograph
Hypomotility
Alternating
constipationand
diarrhea
Bariumenema
Colonicpseudo
diverticula
Rareperforation
Bariumenema
Pseudoobstruction
Abdominalpain,
Plainabdominal
distension
radiograph
Fecalincontinence
Rectalmanometry
Sphincterinvolvement
Plainabdominal
radiograph
NSAID:nonsteroidalantiinflammatorydrug.
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Disclosures
Disclosures:RonnieFass,MDGrant/Research/ClinicalTrialSupport:Ironwood[GERD(none)]Mederi
Therapeutics[GERD(Stretta)].Speaker'sBureau:AstraZeneca[GERD(omeprazole)]Takeda[GERD
(esomeprazole)]MederiTherapeutics[GERD(Stretta)].Consultant/AdvisoryBoards:GlaxoSmithKline
[GERD(none)]Vecta[GERD(none)]ReckittBenckiser[GERD(gaviscone)].MarkFeldman,MD,
MACP,AGAF,FACGNothingtodisclose.ShilpaGrover,MD,MPHEmployeeofUpToDate,Inc.
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseare
addressedbyvettingthroughamultilevelreviewprocess,andthroughrequirementsforreferencestobe
providedtosupportthecontent.Appropriatelyreferencedcontentisrequiredofallauthorsandmust
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