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AchalasiaTreatment&Management

Author:MarcoEttoreAllaix,MD,PhDChiefEditor:JulianKatz,MDmore...
Updated:Nov06,2014

ApproachConsiderations
TheAmericanCollegeofGastroenterologyreleasednewguidelinesforthediagnosisandmanagementofachalasia
inJuly2013. [4,5]Treatmentrecommendationsareasfollows:
Initialtherapyshouldbeeithergradedpneumaticdilation(PD)orlaparoscopicsurgicalmyotomywitha
partialfundoplicationinpatientsfittoundergosurgery
Proceduresshouldbeperformedinhighvolumecentersofexcellence
Initialtherapychoiceshouldbebasedonpatientage,sex,preference,andlocalinstitutionalexpertise
BotulinumtoxintherapyisrecommendedforpatientsnotsuitedtoPDorsurgery
PharmacologictherapycanbeusedforpatientsnotundergoingPDormyotomyandwhohavefailed
botulinumtoxintherapy(nitratesandcalciumchannelblockersmostcommon)

MedicalCare
Thegoaloftherapyforachalasiaistorelievesymptomsbyeliminatingtheoutflowresistancecausedbythe
hypertensiveandnonrelaxingLES.Oncetheobstructionisrelieved,thefoodboluscantravelthroughthe
aperistalticbodyoftheesophagusbygravity.
CalciumchannelblockersandnitratesareusedtodecreaseLESpressure.Approximately10%ofpatientsbenefit
fromthistreatment.Thistreatmentisusedprimarilyinelderlypatientswhohavecontraindicationstoeither
pneumaticdilatationorsurgery.
Endoscopictreatmentincludesanintrasphinctericinjectionofbotulinumtoxintoblockthereleaseofacetylcholineat
theleveloftheLES,therebyrestoringthebalancebetweenexcitatoryandinhibitoryneurotransmitters. [8]This
treatmenthaslimitedvalue.Only30%ofpatientstreatedendoscopicallystillhavereliefofdysphagia1yearafter
treatment.Mostpatientsneedrepeatedbotulinumtoxininjections,withshortlastingclinicalbenefits.Thistreatment
cancauseaninflammatoryreactionatthelevelofthegastroesophagealjunction,makingasubsequentmyotomy
verydifficult.Comparedwithpneumaticdilation,botulinumtoxininjectionisassociatedwithsignificantlyhigher
symptomrecurrenceratesat12months. [9]Similarly,thistreatmentmodalityislesseffectivethanlaparoscopic
Hellermyotomyat2yearfollowup. [10]Usethistreatmentinelderlypatientswhoarepoorcandidatesfordilatation
orsurgery.
Pneumaticdilatationperformedbyaqualifiedgastroenterologististherecommendedtreatmentinthosesporadic
casesinwhichsurgeryisnotappropriate. [11]Aballoonisinflatedatthelevelofthegastroesophagealjunctionto
blindlyrupturethemusclefiberswhileleavingthemucosaintact.Thesuccessrateis7080%,andtheperforation
rateisapproximately5%.Ifaperforationoccurs,emergencysurgeryisneededtoclosetheperforationandto
performamyotomy.Asmanyas50%ofpatientsmayrequiremorethan1dilatation.Theincidenceofpathologic
gastroesophagealrefluxaftertheprocedureisapproximately30%.
AlaparoscopicHellermyotomyisconsideredbymanytobetheappropriateprimarytreatmentofpatientswith
achalasia(seeSurgicalCare).AHellermyotomyandapartialfundoplicationperformedfromthechest
(thoracoscopic)haveahighincidenceofgastroesophagealreflux. [12]
Peroralendoscopicmyotomy(POEM)hasbeenrecentlyintroducedasanovelapproachtoachalasia. [13]This
procedureisperformedundergeneralanesthesiawithendotrachealintubation.A2cmlongitudinalmucosalincision
ismadeonthemucosalsurfacetocreateamucosalentrytothesubmucosalspace.Ananteriorsubmucosaltunnel
iscreateddownwards,passingthegastroesophagealjunctionandabout3cmintotheproximalstomach.Oncethe
submucosaltunneliscompleted,sectionofthecircularmusclefibersbegins23cmdistaltothemucosalentry,
approximately7cmabovethegastroesophagealjunction.Themyotomyiscontinuedstepbystepdistallyuntilthe
gastricsubmucosaisreached,extendingapproximately23cmdistaltothegastroesophagealjunction.After
identificationandsectionofthecircularmusclefibersoftheloweresophagusandproximalstomach,themucosal
entrysiteisclosedwithhemostaticclips.
SeveralpotentialadvantagesofPOEMcomparedwithlaparoscopicHellermyotomyhavebeenproposed.The
endoscopicapproachshouldtheoreticallyminimizepostoperativepain.Alongermyotomycanbeperformed,
extendingtothemediumthirdoftheesophagus,justbelowtheaorticarch.Aconcomitantantirefluxsurgerymay
notberequiredbecauseoftheselectivesectionofthecircularmusclefiberswithoutanydissectionatthelevelof
thegastroesophagealjunction.Incurrentpractice,fewdataareavailableregardingclinicaloutcomesinsmallseries
ofpatientsoververyshortfollowupperiods.
Basedonthelimitedevidenceavailable,POEMseemstobeapromisingnewprocedure.However,therearesome
concernsaboutthisnewtechnique.Endoscopicmyotomyisaverydemandingprocedure,requiringmajorskills,with
averylonglearningcurve.EventhoughseveralstudieshavereportedsignificantreductionofLESpressureas
demonstratedbymanometry,theLESpressurewasoftenbetween15and20mmHg.Itisknownthatapredictor
oflongtermsuccessisanLESpressurearound10mmHg. [14]Gastroesophagealrefluxisreportedinupto50%of
patientsafterPOEM,replicatingtheresultsobtainedwhenamyotomyalonewasperformedwithoutanantireflux
operation.SurgicalrevisioninpatientswithrecurrentdysphagiaafterPOEMmightbechallenging.Thepresenceof
adhesionsbetweenthesubmucosalandlongitudinalmuscularlayersafterPOEMmightmakethedissectionatthis
levelverydifficult.

SurgicalCare

Becauseofexcellentresults,ashorthospitalstay,andafastrecoverytime,theprimarytreatmentisconsideredby
manytobealaparoscopicHellermyotomyandpartialfundoplication.Intheauthor'sexperienceandinthe
experienceofmanyauthors,thistreatmentprovidesafinebalanceinrelievingsymptomsofdysphagiaby
performingthemyotomyandinpreventinggastroesophagealrefluxbyaddingapartialwrap. [15,16]Aprospective,
randomizedstudyfromVanderbiltUniversityindicatedthatthereissignificantlylessriskofpostoperativereflux
followingaHellermyotomyplusapartialfundoplicationthanthereisafteraHellermyotomyalone. [17]Theauthors
ofthisstudyalsoshowedthatinpatientswithachalasia,addingapartialfundoplicationnotonlyismoreeffectivein
preventingpostoperativerefluxbutalsoismorecosteffectiveatatimehorizonof10years. [18]
Apartialfundoplicationaddedtothemyotomyentailsbetterfunctionalresultswhencomparedwithatotal
fundoplication,withalowerriskofpersistentorrecurrentdysphagia. [19]Recently,amulticenter,randomized
controlledtrialcomparingpartialanterior(Dor)withpartialposterior(Toupet)fundoplicationdidnotfindsignificant
differencesintermsofpostoperativeincidenceofgastroesophagealreflux. [20]Incurrentpractice,apartialanterior
fundoplicationismorefrequentlyperformedsinceitissimplertoperformandcoverstheexposedesophageal
mucosa. [21]
IntheEuropeanAchalasiaTrial,treatmentwithpneumaticdilationorlaparoscopicHellermyotomywassuccessful
inahigherpercentageofpatientswithtypeIIachalasiathaninpatientswiththetypeIorIIIformofthedisease.
AlthoughsuccessrateswerehighforbothapproachesintypeIIachalasia,thesuccessratewassignificantlyhigher
inthegrouptreatedwithpneumaticdilation. [22]However,relapseiscommonafterpneumaticdilation.Forgood
longtermoutcomes,closefollowupandrepeatdilationarerequired. [23]
Minimallyinvasivesurgeryforachalasiaisperformedundergeneralanesthesiawiththeuseof5trocars.A
controlleddivisionofthemusclefibers(myotomy)oftheloweresophagus(5cm)andtheproximalstomach(1.5cm)
iscarriedout,followedbyapartialfundoplicationtopreventreflux.Seetheimagesbelow.

Hellermyotomyextending1.5cmontothegastricwall.

Dorfundoplication,leftrowofsutures(afterdiv
Dorfundoplication,leftrowofsutures(afterdivisionofshortgastricvessels).

CompletedDorfundoplication.

Patientsremainhospitalizedfor2448hoursandreturntoregularactivitiesinabout2weeks.
Theoperationrelievessymptomsin8595%ofpatients,andtheincidenceofpostoperativerefluxisabout20%.

Forpatientsinwhomsurgeryfails,theymaybetreatedwithanendoscopicdilatationfirst.Ifthisfails,asecond
operation(extendingthepreviousmyotomyontotheanteriorgastricwall)canbeattemptedoncethecauseof
failurehasbeenidentifiedwithimagingstudies.Thelastresortistosurgicallyremovetheesophagus(ie,
esophagectomy).
Treatmentoptionsvaryforpatientswithdifferentdegreesofillnessseverity.AstudybyReynosoetalsuggeststhat
amonghospitalizedpatientswithminor/moderateillnessseverity,laparoscopicmyotomyforachalasiashowed
comparableorbetteroutcomesthanesophagealdilation. [24]Formajor/extremeillnessseverity,dilationshoweda
comparableorbetterprofileforhospitalizedpatientswithachalasia.
Esophagectomywasthestandardtreatmentinpatientswithachalasiaandamarkedlydilatedorsigmoidshaped
esophagus,withHellermyotomyconsideredtobeineffectiveinsuchcases.However,inastudybySweetand
colleaguesof113patientswithachalasia,theinvestigatorsreportedthat(1)inmostofthestudy'spatients,even
thosewithachalasiaandadilatedesophagus,alaparoscopicHellermyotomyrelieveddysphagia(2)additional
treatmentwasneededinabout20%ofpatientsand(3)intheend,90%ofpatientshadattainedgoodswallowing
ability.Esophagectomywasnotrequiredinanyofthepatientstomaintainclinicallyadequateswallowing. [25]
Cowgilletalreportedonoutcomesin47patientsmorethan10yearsafterlaparoscopicHellermyotomyfor
achalasia. [26]Theyfoundthatnotablecomplicationswereinfrequentfollowingtheprocedureandthatno
perioperativedeathshadoccurred.Onepatientunderwentasecondmyotomy5yearsafterthefirst,becauseof
symptomrecurrence.Therewere33survivingpatientsatthetimeofthestudytheauthorsreportedthattheother
patientsdiedfromcausesunrelatedtomyotomy.UsingaLikertscaleandaWilcoxonmatchedpairstesttoassess
patientssymptomsbeforeandafterlaparoscopicmyotomy,Cowgilletalfoundsignificantpostsurgerydecreasesin
thefrequencyandseverityscoresfordysphagia,chestpain,vomiting,regurgitation,choking,andheartburn.They
concludedthat"thesymptomsofachalasiaaredurablyamelioratedbylaparoscopicHellermyotomyduringlong
termfollowupevaluation."
Comparedwithpneumaticdilatation,laparoscopicHellermyotomyisassociatedwithbetterresultsintermsof
dysphagiaimprovementandpostoperativegastroesophagealrefluxrates,withasignificantlylowerriskofre
intervention. [27]Whiletheresultsaresimilaratashorttermfollowup, [28]longtermfollowupshowsthatmost
patientsaftersurgeryareasymptomatic,comparedwithonly50%ofpatientsevenaftermultiplepneumatic
dilatations. [29]
SeveralstudieshaveshownbetteroutcomesafterlaparoscopicHellermyotomythanpneumaticdilatationin
patientsyoungerthan40years. [28]Inaddition,previousendoscopictreatment,suchasbotulinumtoxininjectionor
pneumaticdilatation,maycompromisetheclinicaloutcomeoflaparoscopicHellermyotomy.Higherintraoperative
complicationsratesandpoorerlongtermoutcomesafterlaparoscopicHellermyotomyhavebeenreportedinseveral
seriesofpatientspreviouslytreatedwithendoscopictreatments.Thesefindingsmayberelatedtoscartissueatthe
levelofthegastroesophagealjunction,whichmakessurgicaldissectionoftheanatomicplanesmuchmoredifficult.
[30,31]

In2011,Boeckxstaensetal[28]reportedtheresultsofamulticenter,randomizedtrialcomparingpneumatic
dilatation(95patients)tolaparoscopicHellermyotomywithDorfundoplication(106patients)foruntreated
esophagealachalasia.TheperforationrateduringpneumaticdilatationandlaparoscopicHellermyotomywas4%
and12%,respectively.TherapeuticsuccesswasdefinedasadropinEckardtscorebelow3.Thestudyshowed
similarsuccessratesafterlaparoscopicHellermyotomy(90%)andpneumaticdilatation(86%)overa2yearfollow
upperiod.
Inconclusion,whilepneumaticdilatationwasconsideredthemaintreatmentmodalityforpatientswithachalasiain
the1980s,withsurgeryhavingasecondaryroleincaseofdilatationfailure, [32,33]incurrentpracticepneumatic
dilatationshouldbereservedforwhensurgicalexpertiseisnotavailableandforthetreatmentofrecurrent
dysphagiaaftermyotomy.
OnestudyonlyhascomparedinaretrospectivefashionPOEMandlaparoscopicHellermyotomy. [34]Eighteen
patientsundergoingPOEMwerecomparedinanonrandomizedfashionto55patientstreatedbylaparoscopic
Hellermyotomy.Nodifferenceswereobservedintermsoflengthofthemyotomy,complicationrate,andlengthof
hospitalstay.Veressneedledecompressionofthepneumoperitoneumwasrequiredintraoperativelyin7(39%)
patientsundergoingPOEM.Treatmentsuccess(Eckardtscore3)afterPOEMwasachievedin16(89%)patients
atmedian6monthfollowup.SixweeksafterPOEM,routinefollowupmanometryandtimedesophagramshowed
normalizationofesophagogastricjunctionpressuresandcontrastcolumnheights.
OnlylongtermfollowupandprospectivetrialscomparingPOEMwithlaparoscopicHellermyotomyand
fundoplicationwilldeterminetheroleofthisnewtechniqueinthetreatmentofesophagealachalasia.
Medication

ContributorInformationandDisclosures
Author
MarcoEttoreAllaix,MD,PhDResearchFellowinGeneralSurgery,DepartmentofSurgery,Universityof
Chicago,ThePritzkerSchoolofMedicine
MarcoEttoreAllaix,MD,PhDisamemberofthefollowingmedicalsocieties:AmericanCollegeofSurgeons,
SocietyforSurgeryoftheAlimentaryTract
Disclosure:Nothingtodisclose.
Coauthor(s)
MarcoGPatti,MDProfessorofSurgery,Director,CenterforEsophagealDiseases,UniversityofChicago
PritzkerSchoolofMedicine
MarcoGPatti,MDisamemberofthefollowingmedicalsocieties:AmericanAssociationfortheAdvancementof
Science,AmericanSurgicalAssociation,AmericanCollegeofSurgeons,AmericanGastroenterological
Association,AmericanMedicalAssociation,AssociationforAcademicSurgery,PanPacificSurgicalAssociation,
SocietyforSurgeryoftheAlimentaryTract,SocietyofAmericanGastrointestinalandEndoscopicSurgeons,
SouthwesternSurgicalCongress,WesternSurgicalAssociation
Disclosure:Nothingtodisclose.

SpecialtyEditorBoard
FranciscoTalavera,PharmD,PhDAdjunctAssistantProfessor,UniversityofNebraskaMedicalCenterCollege
ofPharmacyEditorinChief,MedscapeDrugReference
Disclosure:ReceivedsalaryfromMedscapeforemployment.for:Medscape.
ChiefEditor
JulianKatz,MDClinicalProfessorofMedicine,DrexelUniversityCollegeofMedicine
JulianKatz,MDisamemberofthefollowingmedicalsocieties:AmericanCollegeofGastroenterology,
AmericanCollegeofPhysicians,AmericanGastroenterologicalAssociation,AmericanGeriatricsSociety,
AmericanMedicalAssociation,AmericanSocietyforGastrointestinalEndoscopy,AmericanSocietyofLaw,
Medicine&Ethics,AmericanTraumaSociety,AssociationofAmericanMedicalColleges,PhysiciansforSocial
Responsibility
Disclosure:Nothingtodisclose.
AdditionalContributors
DavidEricBernstein,MDDirectorofHepatology,NorthShoreUniversityHospitalProfessorofClinical
Medicine,AlbertEinsteinCollegeofMedicine
DavidEricBernstein,MDisamemberofthefollowingmedicalsocieties:AmericanAssociationfortheStudyof
LiverDiseases,AmericanCollegeofGastroenterology,AmericanCollegeofPhysicians,American
GastroenterologicalAssociation,AmericanSocietyforGastrointestinalEndoscopy
Disclosure:Nothingtodisclose.

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