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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.
REFERENCES
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3. GalmicheJP,ClouseRE,BlintA,etal.Functionalesophagealdisorders.Gastroenterology2006
130:1459.
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38. DhirV,VegeSS,MohandasKM,DesaiDC.Dilationofproximalesophagealstricturesfollowingtherapyfor
headandneckcancer:experiencewithSavaryGilliarddilators.JSurgOncol199663:187.
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andadolescents:reportof3casesandreviewoftheliterature.Medicine(Baltimore)201089:204.
46. GnreauT,LortholaryO,BouchaudO,etal.HerpessimplexesophagitisinpatientswithAIDS:reportof34
cases.TheCooperativeStudyGrouponHerpeticEsophagitisinHIVInfection.ClinInfectDis199622:926.
47. SuttonFM,GrahamDY,GoodgameRW.Infectiousesophagitis.GastrointestEndoscClinNAm1994
4:713.
48. ElSeragHB,JohnstonDE.Mycobacteriumaviumcomplexesophagitis.AmJGastroenterol199792:1561.
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GastrointestEndosc200052:670.
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(Eds),Lippincott,Williams&Wilkins,Philadelphia1999.p.33.
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formsofthemalformation.Evaluationof1378observations.VirchowsArchAPatholAnatHistol1978
380:303.
52. JanssenM,BaggenMG,VeenHF,etal.Dysphagialusoria:clinicalaspects,manometricfindings,
diagnosis,andtherapy.AmJGastroenterol200095:1411.
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
ineffectiveesophagealmotility:studiesusingcombinedimpedancemanometry.ClinGastroenterolHepatol
20042:230.
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58. ZamostBJ,HirschbergJ,IppolitiAF,etal.Esophagitisinscleroderma.Prevalenceandriskfactors.
Gastroenterology198792:421.
59. RoseS,YoungMA,ReynoldsJC.Gastrointestinalmanifestationsofscleroderma.GastroenterolClinNorth
Am199827:563.
60. AkessonA,WollheimFA.Organmanifestationsin100patientswithprogressivesystemicsclerosis:a
comparisonbetweentheCRESTsyndromeanddiffusescleroderma.BrJRheumatol198928:281.
61. StampflD,DenunaS,VargaJ,etal.Relationsbetweendysmotilityandacidexposureinscleroderma
(SSc).AmJGastroenterol199085:1226.
62. YarzeJC,VargaJ,StampflD,etal.Esophagealfunctioninsystemicsclerosis:aprospectiveevaluationof
motilityandacidrefluxin36patients.AmJGastroenterol199388:870.
63. AnselminoM,ZaninottoG,CostantiniM,etal.EsophagealmotorfunctioninprimarySjgren'ssyndrome:
correlationwithdysphagiaandxerostomia.DigDisSci199742:113.
64. KjellnG,FranssonSG,LindstrmF,etal.Esophagealfunction,radiography,anddysphagiainSjgren's
syndrome.DigDisSci198631:225.
65. RamirezMataM,PenaAnciraFF,AlarconSegoviaD.AbnormalesophagealmotilityinprimarySjgren's
syndrome.JRheumatol19763:63.
66. PalmaR,FreireA,FreitasJ,etal.EsophagealmotilitydisordersinpatientswithSjgren'ssyndrome.Dig
DisSci199439:758.
67. DrossmanDA.RomeIII:TheFunctionalGastrointestinalDisorder,3rded,DegnonAssociates,Inc,
McLean,VA2006.
68. KahrilasPJ,SmoutAJ.Esophagealdisorders.AmJGastroenterol2010105:747.
69. ColonVJ,YoungMA,RamirezFC.Theshortandlongtermefficacyofempiricalesophagealdilationin
patientswithnonobstructivedysphagia:aprospective,randomizedstudy.AmJGastroenterol200095:910.
70. ScolapioJS,GostoutCJ,SchroederKW,etal.Dysphagiawithoutendoscopicallyevidentdisease:todilate
ornot?AmJGastroenterol200196:327.
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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
conformtoUpToDatestandardsofevidence.
Conflictofinterestpolicy

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