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Surgicalmyotomyforachalasia

OfficialreprintfromUpToDate
www.uptodate.com2016UpToDate

Surgicalmyotomyforachalasia
Authors
BrantKOelschlager,MD
RebeccaPPetersen,MD,MSc

SectionEditor
JosephSFriedberg,MD

DeputyEditor
WenliangChen,MD,PhD

Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Feb2016.|Thistopiclastupdated:Feb06,2015.
INTRODUCTIONHellerdescribedasurgicalapproachforthetreatmentofachalasiain1913[1,2].TheHeller
myotomywithafundoplicationistheoptimalsurgicaltreatmentofachalasia,witheffectivesymptomcontrolin90
to97percentofpatients[3,4].Themusclefibersoftheloweresophagealsphincterareincisedwithoutdisrupting
themucosalliningoftheesophagusandcanbeperformedasalaparoscopicoropenprocedure.Withthe
advancementoflaparoscopicsurgery,theopentechniqueisrarelyused.
Theindicationsforsurgery,surgicaltechnicalinsights,andpostoperativeresultsoftheHellermyotomywillbe
reviewedhere.Thepathophysiologyandetiology,clinicalmanifestationsanddiagnosis,andmedicaltreatmentof
achalasiaarediscussedelsewhere.(See"Overviewofthetreatmentofachalasia"and"Pathophysiologyand
etiologyofachalasia"and"Clinicalmanifestationsanddiagnosisofachalasia"and"Pneumaticdilationand
botulinumtoxininjectionforachalasia".)
PATIENTSELECTIONCRITERIAThekeycomponentforselectingappropriatepatientsforsurgical
managementistodifferentiateachalasiafromothermotilitydisordersandfrompseudoachalasia,malignancy,and
mechanicalobstruction.Thepreoperativeevaluationbythesurgeonincludesahistoryofpatientsymptomsaswell
asareviewofpreviousstudiesandtheresultsofmedicaltherapiestoalleviatesymptoms.Asanexample,
patientswhoareolderthan50years,withsymptomslessthansixmonthsduration,and/orwhohavelostmore
than10pounds(4.5kg)mustbeevaluatedforesophagealcancer.
Pertinentdetailsofthepreoperativeassessmentinclude:

Age
Historyofweightloss
Qualityofsymptoms(dysphagia,regurgitation,chestpain,etc.)
Durationofsymptoms
Physiologicstudiesofesophagealfunction
Radiographicimagingstudies
Resultsofdilatations
Botulinumtoxininjections
Biopsyresults
Previousintraabdominalandintrathoracicsurgery
Comorbidillnesses

Medicaltherapies,whicharenonspecificandhaveinconsistentresultsandsideeffects,havealimitedroleinthe
treatmentofachalasia[5,6].Whilesomecontroversyexistsastowhetherendoscopicorsurgicaltherapyshould
beinitiatedfirst[7],mostspecialistsagreethatitisnolongernecessaryorevenpreferredthatpatientsfirst
undergoatrialofmedicaltherapyordilatation.
Pneumaticdilatationisusedincenterswithsignificantexperiencewithitsuseandforpatientswhoprefertoavoid
surgery,haveundergonemultiplepriorabdominaloperations,orwhowouldbeunabletotoleratethe
pneumoperitoneumrequiredtoperformtheprocedurelaparoscopically(eg,restrictivepulmonarydisease,chronic
heartfailure).However,anetworkmetaanalysisthatincluded16studiesand590patientsfoundthatpatients
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undergoinglaparoscopicHellermyotomyhadsuperiorshortandlongtermefficacycomparedwithpatients
undergoingendoscopicballoondilatation(oddsratio[OR]2.295%,CI1.24.19,p=0.01at12monthsandOR
29.8,34.0224.7,p=0.001at60months)[8].
Peroralendoscopicmyotomyisaninvestigativeprocedureforthemanagementofachalasia.(See"Overviewof
thetreatmentofachalasia",sectionon'Peroralendoscopicmyotomy'.)
REVIEWOFDIAGNOSTICEVALUATIONThediagnosisofachalasiaisbasedupontheclinicalhistory,
bariumswallow,andesophagealmanometry.Esophagealmanometry,endoscopy,andabariumswallowstudyare
essentialtoconfirmingthediagnosisofachalasia,andthestudiesshouldbepersonallyreviewedbythesurgeon
beforeproceedingwithanoperation.(See"Clinicalmanifestationsanddiagnosisofachalasia",sectionon
'Evaluation'.)
Thekeydiagnosticstudiesthatasurgeonmustrevieworperformtoevaluateapatientfortheoperationare
reviewedbelow.
ManometryManometryisthemeasurementofmuscularfunctionoftheesophagusandincludesthree
zones:loweresophagealsphincter(LES),esophagealbody,andupperesophagealsphincter(UES).High
resolutionmanometry(HRM)providesforamoreaccuratecharacterizationofesophagealfunctioncompared
withstandardmanometry(figure1andpicture1)[9].HRMpermitscontinuousrecordingofmotoractivity
alongtheentirelengthoftheesophagusandyieldsamorecompleteanddetailedpictureofesophageal
motility.Manometrysystemsincludeacatheterwithmultiplepressuresensorchannels,pressure
transducers,andarecordingdevicewithacomputerforanalysis.Thetechniqueandtheinterpretationofthe
resultsarediscussedelsewhere.(See"Clinicalmanifestationsanddiagnosisofachalasia",sectionon'High
resolutionmanometry'and"Motilitytesting:Whendoesithelp?",sectionon'Esophagealmanometry'and
"Oropharyngealdysphagia:Clinicalfeatures,diagnosis,andmanagement",sectionon'Manometry'and
"Clinicalmanifestationsanddiagnosisofachalasia",sectionon'Manometry'.)
EndoscopyMostpatientsundergoendoscopytoestablishadiagnosiswellinadvanceofconsiderationfor
surgery.Itisimportantthatthesurgeonreviewtheresultstoexcludeotheretiologiesofthesymptoms.Itis
notnecessarytoroutinelyrepeattheendoscopybeforeeveryoperativeprocedure,butthesurgeonmust
havealowthresholdtorepeatendoscopyifconcomitantoralternativeuppergastrointestinaldiseaseremains
aconsideration.(See"Clinicalmanifestationsanddiagnosisofachalasia",sectionon'Upperendoscopy'.)
RadiographicimagingThebariumswallowisthepreferredinitialstudytodefinetheesophagealanatomy
(image1).Thisstudywillshowtheextentofesophagealdilation,shape,andgastroesophagealjunction
(GEJ)obstruction.AbsenceofobstructionattheGEJ,whichischaracterizedbyacolumnofbariumwithair
fluidlevels,shouldmakeonequestionthediagnosis.RoutineCTscansofthechestandabdomenarenot
necessary,butifpseudoachalasiaissuspectedbasedontheclinicalhistory,aCTscanorendoscopic
ultrasoundshouldbeperformedtoensureaccuracyofdiagnosis.Asigmoidesophagusisacomplicationof
longstandingachalasia.Thesurgicalapproachtoasigmoidesophagusmaybetolimittheprocedureto
myotomywithoutfundoplicationoranesophagectomyforpatientswithpriorattemptsatmyotomyormega
esophagus.
SURGICALMYOTOMYSurgicalmyotomy,inwhichtheloweresophagealsphincter(LES)isweakenedby
incisingthemusclefibers,istheprimaryalternativetopneumaticdilatationforachalasia.Theadvantagesof
surgicalmyotomyarehighinitialsuccessratesand,comparedwithpneumaticdilation,lowerratesofsymptom
recurrence.Themaindisadvantagesofsurgeryarethehighinitialcost,theprotractedrecoveryperiod,andthe
frequentdevelopmentofgastroesophagealrefluxdiseasepostoperatively,especiallyifafundoplicationisnotpart
oftheprocedure[10].
TheprimarygoaloftheoperativetreatmentistorelievethefunctionalobstructionoftheLESwhilepreventing
reflux.Thesurgicalprinciplesinclude:

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Minimaldissectionofthecardia
AdequatedistalmyotomytoreleasethepressureoftheLES
Preventionofpostoperativegastroesophagealreflux
Preventionofscarredclosureofthemyotomysite

Surgicalmyotomywasfirstdescribedin1913byErnestHeller,whoperformedbothanteriorandposterior
incisionsatthegastroesophagealjunction[1].Histechniquewasmodifiedtoincludeonlytheanteriormyotomy.
ThemodifiedHellermyotomyisthemostcommonoperativeprocedureusedtotreatachalasia[11].Theoperation
canbeperformedusinganopenoraminimallyinvasivetechnique,andtheesophaguscanbeapproachedthrough
theabdomenorthorax[7,1218].Withadvancesinminimallyinvasivesurgeryintheearly1990s,the
thoracoscopicapproachandlaterthelaparoscopicapproachbecamepopular[1417,19].Thereareadvantagesand
disadvantagestoeachapproach.Themaindisadvantagesofathoracoscopicapproachcomparedwitha
laparoscopicesophagealmyotomyincludelimitedexposureofthedistalloweresophagealsphincter(LES)and
stomachandthetechnicalchallengesofperformingafundoplication.Withregardstoclinicaloutcomes,the
thoracoscopicapproachisassociatedwithahigherrateofresidualorrecurrentdysphagia,morepostoperative
pain,andalongerhospitalstay[20].Outcomesforthelaparoscopicandopentechniquesarediscussed
elsewhere.(See"Overviewofthetreatmentofachalasia",sectionon'Surgicalmyotomy'.)
LaparoscopictechniqueThefollowingisadescriptionofthekeytechnicalelementsofperforminga
laparoscopicesophagealmyotomy[10,14].Generalissuesrelatedtolaparoscopicabdominalsurgeryare
discussedelsewhere.(See"Abdominalaccesstechniquesusedinlaparoscopicsurgery".)
PatientpositionPatientsareplacedinasupine,splitleg,orlithotomypositionwithpadding,suchasa
surgicalbeanbag.Wepreferthelithotomypositionforoptimalergonomicsandaccesstothehiatus.Thepatientis
positionedinasteepreverseTrendelenburgposition,whichallowsthestomachandotherorganstofallawayfrom
theesophagealhiatus.
AbdominalaccessandportplacementAbdominalaccessisobtainedattheleftupperquadrantjust
inferiortothecostalmarginbyinsertingaVeressneedlefollowedbyplacementofanopticaltrocarafter
establishingpneumoperitoneumaccordingtostandardlaparoscopictechniques.Fouroperativeports(twoforthe
surgeon,onefortheassistant,andoneforthescope)arethenplacedunderdirectvision,andliverretractionis
thenachievedbyanynumberofsuchdevicesonthemarketviaanadditionalportsite(figure2).Otherport
placementscanalsobeused.(See"Abdominalaccesstechniquesusedinlaparoscopicsurgery",sectionon
'Foregutsurgery'.)
MobilizationofthegastricfundusBasedonthesurgeonspreference,theinitialdissectioncanbeginon
therightortheleftsideoftheesophagealhiatus.Thestepstomobilizethegastricfundusinclude:
Dividingtheleftphrenogastricligamentsbydividingtheshortgastricarteries,startingattheinferiorpoleof
thespleentotheexposedleftcrusofthediaphragm
Incisingthegastrohepaticligamentinanavascularplane
PreservingthenerveofLatarjetandavoidinginjurytoanaccessoryorreplacedhepaticartery
Dividingtherightanteriorphrenoesophagealligamentandtheperitoneumoverlyingtheanteriorabdominal
esophagus
Preservingtheanteriorvagusnerve,whichliesimmediatelyposteriortotherightanteriorphrenoesophageal
ligament
Atthispoint,ifaposteriorpartial(Toupet)fundoplicationisperformed,aposterioresophagealwindowis
created.Inperformingthiswindow,theposteriorvagusnerveisidentifiedandprotected.Ifananterior
fundoplication(Dor)techniqueisused,aposterioresophagealwindowisunnecessaryunlessahiatalhernia
and/orandarelativelyshortesophagusisencounteredandthereisaneedforfurthermobilizationtoallow
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moreintraabdominallengthinordertoconstructaproperfundoplication.(See'Fundoplication'belowand
'Additionofafundoplication'below.)
MobilizationofthemediastinalesophagusThedistalportionofthemediastinalesophagusismobilized
toachievesufficientlengthtoperformamyotomyincisionthatdividestheentirelengthoftheLESandpermitsa
tensionfreefundoplication.APenrosedrainmaybeplacedaroundthegastroesophagealjunctiontofacilitate
retractionoftheesophagus,butisnotessential,especiallyifananteriorfundoplicationistobeperformed.
MyotomyItiscriticalwhenperformingthemyotomythatvisualizationandexposureareadequateto
preventinadvertentmucosalinjuries.Thecardioesophagealfatpadandtheanteriorvagusnervemustbecleared
fromtheesophagusandthegastroesophagealjunction.Acontinuousmyotomyisperformedfor6cmonthe
esophagusand3cmontothestomach.
Thefollowingprinciplesareimportantwhenperformingthemyotomy:
Asuitableandstableplatformisuseful.Ourpreferenceisalightedbougiedilator(50Fr),whichilluminates
theesophagusandstretchesthemusclefibersaroundthegastroesophagealjunction,facilitatingtheir
division.Anendoscopemaybeusedinsteadofthelightedbougiedilator.Theanteriorsurfaceofthe
esophagusiscompletelyexposedandslighttensioniscreatedbyretractingcaudallywithaBabcock
retractororsimilarinstrument.
Theincisionmaybestartedoneitherthestomachortheesophagus.Wepreferstartingonthestomach
whilethisisamoredifficultsubmucosalplanetoidentify,wefinditeasiertoproceedinacephaladthan
caudaldirection.
Theuseofelectrocauteryshouldbeavoidedunlesscriticalwhencreatingthemyotomy.Ifbleedingis
encountered,itshouldbecontrolledwithpressureandpatiencesincethermalinjurycanleadtoanunrecognized
perforationoftheesophagus.
Themyotomyisperformedbyindividuallydividingtheesophagealandgastricmusclefibers.
Thelongitudinalmusclesaredividedfirst,whichexposestheunderlyingcircularmuscles.
Divisionofthecircularlayerrevealsabulgingmucosalplanethatshouldappearsmoothandwhite
(picture2).
Themostcriticalandchallengingfactoristocreatea3cmmyotomycaudaltothegastroesophageal
junction,wherethetissueplanebecomeslessreadilyidentifiable.Aninterveningslingofmusclefibers
mayblurthedissection,andthestomachmucosaisthinnerandmorepronetoperforation(figure3).
Theportionofthemyotomyontheesophagusshouldbeapproximately6cminlength.Thus,thetotal
lengthoftheentiremyotomyis9cm.
Endoscopicinspectionofthemucosaandthemyotomyisperformedpriortoproceedingtothenextstepsto
identifyandrepairanymucosalperforations.
FundoplicationIfafundoplicationprocedureisperformed,itistypicallyapartial(eg,ToupetorDor)and
notacircumferential(Nissen)wrap(see'Additionofafundoplication'below).AToupetfundoplicationisa270
degreeposteriorwrapofthefundusaroundtheesophagus,whiletheDorfundoplicationisa180degreeanterior
wrap.
IfaToupet(posterior)fundoplicationisbeingperformed,aretroesophagealwindowisestablishedalongthe
linesofdissection(figure4andpicture3).Thefollowingisabriefdescriptionofthetechnique:
Thefundusismobilizedbydividingtheshortgastricvesselsandallfundalattachmentsstarting
approximatelyattheinferiorpoleofthespleen,approximately10to15cminferiortotheangleofHis.
Theretroesophagealwindowisdevelopedbyfurtherdissectionalongthebaseoftheleftcrus.
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Followingmobilizationofthemediastinalesophagus,thegastroesophagealhiatusisclosedposteriorly
withinterruptedsuturesifalargedefectispresent,butourpreferenceistoleavelaxityofthehiatus.
Theposterioraspectofthegastricfundusispassedthroughtheretroesophagealwindowandsecured
totherightedgeofthemyotomyandrightcrusofthediaphragmwithacoronalsuture.Another
interruptedsutureisplacedtosecuretheposteriorfundustothebaseoftherightcrus.
Theleadingposterioredgeofthefundusissecuredtotherightedgeofthemyotomywithinterrupted
sutures.
Anyredundancyofthefundusisremovedfrombehindtheesophagusandtheproximal,anterioraspect
ofthefundusissecuredtotheleftsideinasimilarfashion.
ADor(anterior)fundoplicationmaybeparticularlyusefultobuttresstherepairofanesophagealperforation
thatmayoccur.Thegreatercurveofthefundusisgraspedandplacedanteriorlytotherightsideofthe
gastroesophagealhiatus.Thefollowingisabriefdescriptionofthetechnique:
Aninnerrowofinterruptedsuturesareusedtosecurethemedialaspectofthefundustotheleftsideof
themyotomy,whichbeginstorecreatetheangleofHis.
Theanteriorfundusisfoldedovertheesophagus,andacoronalsuturetotherightcrusandrightsideof
theesophagusisperformed.
Asecondrowofinterruptedsuturesisplacedtofixtheleadingedgetotherightsideofthemyotomy
(picture4).Afinalsuturefromtheapexofthegastroesophagealhiatustothefunduscompletesthe
fundoplication.
OpentechniqueTherearenodifferencesinsurgicaltechniqueorprincipleswhenperformingamyotomywith
orwithoutafundoplicationbetweenalaparoscopicoropen(laparotomyorthoracotomy)approachexceptforafew
technicalvariationsthatarediscussedbelow.Althoughintheoryanopenapproachmightbemoreappropriatefor
patientswithseverepulmonarydiseasewhowillnottoleratepneumoperitoneum,suchapatientmostlikelywould
notbeconsideredasurgicalcandidateatall.Anotherindicationforanopenapproachmightbelessinexperience
withadvancedlaparoscopyhowever,giventhatthisdiseaseisrare,andalmostalwayscanbemanaged
electively,thereisampletimeforthepatienttobereferredtoacenterwithexperiencedsurgeons[21].
IntraoperativetechnicalrisksThemajorintraoperativerisksincludeanunrecognizedperforationofthe
esophagealorgastricmucosa,divisionorinjurytotheanteriorvagusnerve,andsplenicinjury.
EsophagealorgastricperforationTheriskofanesophagealorgastricperforationduringsurgicalmyotomy
rangesfrom10to16percent[3,22].Mucosalperforationsarerepairedwithfine40and50absorbable
monofilamentsuture.Anadvantageoftheanterior(Dor)fundoplicationisthatitwillbuttresstherepair.
DivisionofvagusnerveUsingcarefuldissectionandattentiontodetail,injurytothevagusnervesisrare.
Theanteriorvagusnerveisatriskofinjuryduringseveraltechnicalstepsoftheoperationincludingthe
initialdissectionoftheesophagus,mobilizingthegastroesophagealfatpad,performingthemyotomy,and
performingthefundoplication[23].Theposteriorvagusnerveislesslikelytobeinjured.Ifaninjurytoonly
theanteriororposteriorvagusnerveoccurs,itisnotrepairedaspostvagotomydiarrhea,bloating,early
satiety,and/ordumpingsyndromerarelyoccurwithaunilateralvagotomy[24].Nevertheless,extracare
shouldbetakentoidentifyandpreservebothnerves.
SplenicinjuryTheriskofinjurytothespleenrangesfrom<1to5percent[2527].Thehigherratesare
fromreportswhenlaparoscopicsurgerywasintheearlystagesofuse.Managementofanintraoperative
splenicinjuryisdiscussedelsewhere.(See"Managementofintraabdominal,pelvic,andgenitourinary
complicationsofcolorectalsurgery",sectionon'Splenicinjury'.)
OPERATIVECONSIDERATIONSThereareseveraltechnicalvariationsforperformingalaparoscopicoropen
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Hellermyotomy,reflectingareasofcontroversy.Theseincludelengthofthegastriccomponentofthemyotomy
incision,optimaltypeofantirefluxprocedure[10],managementofasigmoidmegaesophagus,andtheroleof
roboticsurgery.
ExtendedgastricmyotomyAnextendedgastricmyotomy,whichisdefinedasa3cmmyotomyincisiononto
thecardiaofthestomach,isanimportanttechnicalcomponentforreducingrecurrenceofsymptomsofachalasia.
Thestandardesophagealmyotomyincludesa1.5to2.0cmextensionoftheincisionontothecardiaofthe
stomach[2830].A3cmextensionoftheesophagealmyotomyincisionontothestomachprovideslongterm
durabledysphagiareliefduetodisruptionofthegastricsling,whichconsistsofshorttransversemusclefiberson
thelessercurveofthestomach[28,29].Theimportanceofthegastricslingfibersintheloweresophageal
sphincter(LES)mechanismwasrecognizedwhentransitioningfromthethoracoscopictolaparoscopicapproach
(figure3)[19].
Thefollowingstudiesillustratetheoutcomesofperformingastandardoranextendedmyotomy:
AcaseseriesfromaprospectivedatabasefoundthatsymptomsfollowingthelaparoscopicHellermyotomy
wereimprovedwithanextendedgastricmyotomy[28].Patientstreatedwitha3cmextendedgastric
myotomy(n=58)andaToupetfundoplicationhadasignificantlylowerLESpressure(9.5versus15.8
mmHg),lessfrequentdysphagia(1.2versus2.1percent),lessseveredysphagiaonVASpainscore(3.2
versus5.3),andfewerrecurrencesrequiringdilatation(2versus9patients)comparedwithpatientstreated
withastandard1.5cmgastricmyotomyandaDorfundoplication(n=52).Patientstreatedwiththe
extendedgastricmyotomyhadsimilarratesofheartburn(1.3versus1.7percent),regurgitation(0.3versus
0.8percent),chestpain(0.3versus0.6percent),andproximal(1.7versus2.3percent)anddistal(6.0versus
5.9percent)esophagealacidexposure.
Aretrospectivereviewof113patientsundergoingalaparoscopicHellermyotomyreportedgoodorexcellent
resultsin90percent[31].Tenpatients(8.7percent)wereconsideredsurgicalfailures,includingtwowho
reportednoreliefofsymptoms.Thereasonsforrecurrenceoforpersistentsymptomsincludedanincomplete
myotomy,sclerosisofthemyotomy,andasigmoidmegaesophagus.Patientswhoweresymptomatic
followingsurgeryhadasignificantlylargeresophagealdiameteridentifiedonapostoperativebariumswallow
(25.5versus20mm),andasignificantlylongerintactLESoverall(42.5versus37.5mm),longerintactlength
ofthegastriccomponentoftheLES(32verus27mm),andahigheramplitudeofcontractionabovetheLES
(32versus18mmHg).
AdditionofafundoplicationTherationaleforperforminganantirefluxfundoplicationistoreducetheriskof
symptomaticgastroesophagealrefluxthatmayresultfromobliteratingtheLESmechanism.Theindicationsfor
additionofafundoplicationvary,inpart,withtheapproachtothemyotomy.Afundoplicationisincludedinmost
patientsinwhomalaparoscopicmyotomyisperformed.Arandomizedtrialof43patientsundergoingamyotomy
forachalasiafoundthatpatientstreatedwiththeadditionofananterior(Dor)fundoplicationhadsignificantlyfewer
gastroesophagealrefluxsymptoms(9versus48percent)andaloweracidexposuretimetothedistalesophagus
comparedwithpatientstreatedwithamyotomyalone[10].Therewasnodifferenceinthefrequencyofdysphagia.
Bothanterior(Dor)andposterior(Toupet)partialfundoplicationtechniquesareused,andcurrentlythereisno
consensusonwhichprocedureissuperior:
TheDorprocedureavoidsmobilizationofthestomachduringtheinitialdissectionandistechnicallyeasierto
perform.Inaddition,itisausefultechniquetobuttresstherepairofaninadvertentesophagealperforation.
TheToupetfundoplicationsplaystheedgesofthemyotomy,whichmaydecreasefibrosisattheincisionsite
andreducerecurrenceofdysphagia.
Unfortunately,arandomizedtrialcomparingtheDor(n=36)andToupet(n=24)fundoplicationtechniquesin
patientsundergoinglaparoscopicHellermyotomywasinconclusiveduetosmallsamplesizeandincomplete
followup[32].Nodifferencesinesophagealsymptomswerefoundbetweenthetwogroupsat6to12months.
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TheDorgrouphadahigherpercentageofpatientswithabnormal24hourpHresultscomparedwiththeToupet
group,butthedifferencewasnotsignificant(42versus21percent)[32].Acircumferentialcompletefundoplication
(Nissen)isusuallynotperformedtoavoidexcessiveconstrictionatthegastroesophagealjunction.Ourpreference
istoperformaToupetpartialfundoplicationaswebelieveitreducestheriskofrecurrentdysphagiabysplaying
opentheedgesofthemyotomy,thuspreventingscarring.WeperformaDorfundoplicationtoprovidecoverageof
theprimaryrepaironlywhenthereisanesophagealperforation[33].
Whileafundoplicationisroutinelyperformedwithalaparoscopicmyotomy,thereareseveralinstanceswhena
fundoplicationisnotperformed.Thesesettingsinclude:
Themyotomyisperformedwithaleftvideoassistedthorascopicsurgery(VATS)approachwithoutdisruption
ofthephrenoesophagealligament[3].
Latestagediseasemanifestedbythepresenceofasigmoidmegaesophagusonthepreoperative
esophagogram.(See'Sigmoidmegaesophagus'below.)
Patientswhoaremorbidlyobese.Althoughachalasiaisrarelyseeninobesepatients,webelievethatthe
optimalprocedureforthesepatientsisalaparoscopicHellermyotomywithaconcomitantRouxenYgastric
bypass,whichwehaveperformedwithsuccessinasmallseriesofpatients[34].
SigmoidmegaesophagusSigmoidmegaesophagusisanenlargedandatonicesophagusthatresultsfrom
longstandingincreasedLESpressure.Traditionally,astandardHellermyotomywasnotperformedonpatients
withasigmoidmegaesophagus,asthedegreeofesophagealdilationandreliefofsymptomswerenotthoughtto
improvewithmyotomy[35].Hence,patientswithasigmoidesophagusweretypicallytreatedwithan
esophagectomypriortothelaparoscopicera[36].
Theresultsofanesophagealmyotomywithafundoplicationforpatientswithasigmoidmegaesophagusare
illustratedinthefollowingretrospectivestudies:
Areviewof113consecutivepatientswithachalasiawhoweretreatedwithalaparoscopicHellermyotomy
andDorfundoplication[35].Thetwelvepatientswithasigmoidmegaesophagushadanequivalentoperating
time,lengthofhospitalstay,rateofpostoperativecomplications,andreliefofsymptomscomparedwith
patientswithoutesophagealdilatation.
Areviewof51patientswithachalasiatreatedwithaHellermyotomyandDorfundoplicationbylaparoscopy
orlaparotomyfoundthatthe14patientswithasigmoidmegaesophagushadequivalentpostoperative
changesinesophagealwidth,LESpressure,dysphagiascore,andregurgitationscoreasthe37patients
withoutamegaesophagus[37].
Incontrasttotheprevioustwostudies,aretrospectivereviewof394patientswithachalasiaundergoinga
laparoscopicHellermyotomyfoundthatsigmoidmegaesophaguswasanindependentpredictorforfailureof
alaparoscopicmyotomyastheprimaryorsecondarytreatmentofachalasia[4].Therewere13patientswith
amegaesophagusinthisstudy:fivehadagoodoutcomeandeightpatientswereconsideredasurgical
failure.
Currently,themajorityofsurgeonswillinitiallyofferpatientswithasigmoidmegaesophagusalaparoscopicHeller
myotomy,andifthisfails,asubsequentesophagectomy.IfaHellermyotomyisperformedinpatientswitha
sigmoidmegaesophagus,oneshouldbecarefulifaddingaconcomitantpartialfundoplication,whichismorelikely
tocauseangulationand/orresistanceinamegaesophagus.Analternativetoanesophagectomyoramyotomy
withafundoplicationmaybealaparoscopicesophagogastrectomy[38].However,therearenoshorttermorlong
termstudiesontheefficacyofthisproceduretoimprovesymptomsandnohighqualitydatafromrandomized
trialstosuggestthebestoperativeapproachformanagementofsigmoidmegaesophagus.
RoboticsurgeryAnevolvingsurgicalmodalityfortreatmentofachalasiaisrobotassistedminimallyinvasive
surgery.Theoreticaladvantagesforthesurgeonoverstandardlaparoscopicsurgeryincludeincreasedrangeof
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motion,decreasedtremor,improvedvisibilitywithrespecttodepthperception,andimprovedabilitytoperformfiner
movementsinasmaller,confinedspace[39].Thelargestprospectiveseriesincluded104patientswithachalasia
treatedwitharobotassistedHellermyotomywithpartialfundoplication[40].Therewerenoesophageal
perforations,eightminorcomplications,andtwoconversionstoanopenprocedurebecauseofbleedingand
computersystemfailure.Almosttwothirdsofpatientsweredischargedonthefirstpostoperativeday.Asymptom
surveywascompletedin79patients(76percent)allhadasignificantimprovementinpostoperativesymptom
scorecomparedwithapreoperativescore(0.5versus5.0),andnonerequiredareoperationtocontrolsymptoms.
POSTOPERATIVEMANAGEMENTTheprinciplecomponentsofpostoperativecareforlaparoscopicand/or
openmyotomyincludeadvancementofdietandcontrolofnausea.Thefollowingisasummaryofthemajor
issues:
Whilesomeadvocateperformingabariumswallowpriortoinitiatingadiet,wefindthisunnecessaryifthere
hasbeengoodintraoperativevisualizationoftheesophagusandstomachaswellasintraoperative
endoscopy.However,oneshouldhavealowthresholdforobtainingabariumswallowifapatientdevelops
symptomsandsignssuggestiveofanesophagealorgastricperforation(eg,chestpain,epigastricpain,
fever,tachycardia,subcutaneousormediastinalemphysema,and/orotherwiseunexplainedleukocytosis).
Clearliquidsaretypicallystartedthenightoftheprocedurefollowinglaparoscopicsurgery,orwhenbowel
functionreturnsfollowinganopenprocedure.Ifnodysphagiaoccurs,thedietisadvancedtoasoftdietthe
followingday.Ifepisodesofdysphagiaoccur,patientsaremaintainedonafullliquiddietforalongerperiod
oftimepriortoadvancingthediet.
Aggressivetreatmentwithantiemeticsisprovidedforpatientscomplainingofnauseatoavoidemesis,which
canleadtoruptureoftherepair.Medicationscanbeadministeredinacrushedformuntilthepatientis
toleratingaregulardiet.
InpatientsundergoingHellermyotomywithpartialfundoplication,antacidsorprotonpumpinhibitorsareused
onlyifpatientsexperiencesymptomsofheartburnandregurgitationandapHmonitoringstudyreveals
gastroesophagealreflux.Antacidsorprotonpumpinhibitorsaretypicallyrecommendedforpatients
undergoingaHellermyotomyalone.(See"Medicalmanagementofgastroesophagealrefluxdiseasein
adults",sectionon'Histamine2receptorantagonists'.)
POSTOPERATIVECOMPLICATIONSThemostcommoncomplicationsfollowinglaparoscopicandopen
myotomywithafundoplicationincludeperforation,recurrentdysphagia,andgastroesophagealreflux.Following
laparoscopicHellermyotomy,themorbidityraterangesbetween1and10percentandthemortalityrateis<0.1
percentinthe30dayperioperativeperiod[4,29,4143].
PerforationThemostcommonearlypostoperativecomplicationisgastricoresophagealperforation,which
occursin1to7percentofpatients[4,29,4143].Lateperforationsusuallyresultfromeitherdirectmucosalinjury
thatisnotrecognizedduringsurgery,orinadvertentthermalinjury.Perforationsusuallyresultindiffuseperitonitis
and/ormediastinitisandmaybelifethreatening[44].
Ifaperforationissuspected,awatersolublecontrastradiographshouldbeobtained.Managementofalate
perforationrequiresdiversionandagastrostomytubeoranesophagectomy.
RecurrentdysphagiaRecurrentdysphagiaisalatecomplicationofaHellermyotomyandfundoplication.It
occursinapproximately3to10percentofpatientsundergoingaHellermyotomy,withtheonsetofsymptomssix
monthsorlateraftersurgery[4,29,31,42,45].Themostcommoncauseisincompletemyotomy.Thiscanoccur
withincompletedivisionofthecircularfibersontheesophagusorfailuretodividethecollarslingfibersonthe
stomachatleast3cmbelowthegastroesophagealjunction.Thisismorecommoninpatientsthatweremanaged
withathoracoscopicapproachtomyotomy[30].Insomecases,theincisionwassuboptimal,evenwiththe
laparoscopicapproachasaresult,arepeatproceduremaybewarrantedtoextendtheincisionfurtherintothe
stomach[28,29].
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Otherpotentialreasonsforrecurrentdysphagiainclude:

Herniatedfundoplication
Perihiatalscarring
Pepticstricture
Obstructingtumors

Theapproachtothepatientwithrecurrentdysphagiaistoidentifythecauseandtoruleoutsignificantpathology,
suchasamalignancy,stricture,orhernia.Thediagnosticevaluationtypicallybeginswithupperendoscopyor,if
endoscopyisnotavailable,abariumswallow.Manometrymaybeperformedifachalasiaisapossiblecauseof
thedysphagia.(See"Overviewofdysphagiainadults"and"Clinicalmanifestationsanddiagnosisofachalasia",
sectionon'Evaluation'.)
GastroesophagealrefluxTherateofgastroesophagealreflux(GER)developinginpatientsundergoing
myotomywithapartialfundoplication(DororToupet)rangesfrom2to26percentindifferentseries[10,29,4648].
TherateofGERforpatientsundergoingmyotomyaloneismuchhigher.Thiswasdemonstratedinaprospective
randomizedtrialof43patientswithachalasiainwhichpatientsundergoingaHellermyotomywithaDor
fundoplicationhadasignificantlylowerrateofpathologicGERat6monthsaftersurgerycomparedwithpatients
undergoingaHellermyotomywithoutafundoplication(9versus48percent)[10].
PatientswhodevelopGERshouldhavepHmonitoringperformedthreetosixmonthsaftermyotomyandrepeated
withanychangeinsymptomsthatcouldsuggestGER,especiallyheartburnandregurgitation.Patientswith
abnormalesophagealacidexposurearetreatedmedically.Incompletemyotomywithstasisinthedistal
esophaguscanmimicGER.(See"Medicalmanagementofgastroesophagealrefluxdiseaseinadults".)
OthercomplicationsPneumothorax,bleeding,inadvertentvagalinjury,andinfectionareuncommon
complications,withatleastoneoccurringinapproximately3percentofcases[49].Apneumothoraxcanbe
causedfrominadvertentinjurytothepleuraduringmobilizationofthemediastinalesophagus.Ifthisoccurs,the
defectcanberepairedwithaprimarysutureclosureifthepatientdevelopsintraoperativehypotensionorhypoxia.
Otherwise,neitheraprimaryrepairnorachesttubeisnecessaryinmostpatients.
RISKOFESOPHAGEALCANCERUntreatedachalasiaisassociatedwithanincreasedriskofsquamous
cellesophagealcancer.Thereisapaucityoflongtermdataonthedevelopmentofesophagealcancerfollowinga
myotomy.Inaretrospectivereviewof226patientssurgicallytreatedforachalasia,four(1.8percent)developeda
squamouscellcarcinomaat2,8,13,and18yearsaftertheoperation[48].Theriskofesophagealcancerin
patientswithachalasiaandthepossibleroleofscreeningarediscussedseparately.(See"Clinicalmanifestations
anddiagnosisofachalasia",sectionon'Naturalhistoryandprognosis'.)
SUMMARYANDRECOMMENDATIONSThelaparoscopicHellermyotomywithapartialfundoplicationisthe
optimalsurgicaltreatmentofachalasia,witheffectivecontrolofsymptomsin90to97percentofpatients.The
primarygoaloftheoperativetreatmentistorelievethefunctionalobstructionoftheloweresophagealsphincter
(LES)whilepreventingreflux.
AlaparoscopicHellermyotomyisthefirstlineofsurgicaltherapyforpatientswithaconfirmeddiagnosisof
achalasiaandwhoareoperativecandidates.Anopenapproach(laparotomy)totheHellermyotomyisrarely
performedastheinitialtreatment,andisreservedforpatientswhohavehadmultiplepriorabdominal
operationsorwhocannottolerateapneumoperitoneumbecauseofcardiacorpulmonarydisease(see
'Patientselectioncriteria'above).Forthosepatients,orthosewhopreferanonoperativeapproach,
pneumaticdilatationisperformed.
Thesurgeonmustreviewtheresultsofthemanometry,endoscopy,andpathologyifnotpersonally
performingthetests.(See'Reviewofdiagnosticevaluation'above.)
Itiscriticalwhenperformingthemyotomythatvisualizationandexposureareadequatetoprevent
inadvertentmucosalinjuries.Thecardioesophagealfatpadandtheanteriorvagusnervemustbecleared
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fromtheesophagusandthegastroesophagealjunction.Acontinuousmyotomyisperformedfor6cmupthe
esophagusand3cmontothestomachasmeasuredfromthegastroesophagealjunction.(See'Surgical
myotomy'aboveand'Operativeconsiderations'above.)
Werecommendapartialfundoplicationwhenperformingalaparoscopicmyotomyforachalasia(Grade1B)
(see'Fundoplication'aboveand'Additionofafundoplication'above).OurpreferenceistoperformaToupet
partialfundoplication(270degreeposteriorwrapofthefundusaroundtheesophagus)aswebelieveit
reducestheriskofrecurrentdysphagiabysplayingopentheedgesofthemyotomy,thuspreventing
scarring.WeperformaDorfundoplication(180degreeanteriorwrap)toprovidecoverageoftheprimary
repaironlywhenthereisanesophagealperforation.
Themajorintraoperativerisksincludeanunrecognizedperforationoftheesophagealorgastricmucosa,
divisionorinjurytotheanteriorvagusnerveandsplenicinjury.(See'Intraoperativetechnicalrisks'above.)
IfaHellermyotomyisperformedinpatientswithasigmoidmegaesophagus,oneshouldbecarefulifadding
aconcomitantpartialfundoplication,asthisismorelikelytocauseangulationand/orresistancetoavery
abnormalesophagus.(See'Sigmoidmegaesophagus'above.)
Themostcommoncomplicationsfollowinglaparoscopicandopenmyotomywithafundoplicationinclude
esophagealorgastricperforation(1to7percent),recurrentdysphagia(3to10percent),and
gastroesophagealreflux(2to26percent).(See'Postoperativecomplications'above.)
UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.
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47.LuketichJD,FernandoHC,ChristieNA,etal.Outcomesafterminimallyinvasiveesophagomyotomy.Ann
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48.ZaninottoG,RizzettoC,ZambonP,etal.Longtermoutcomeandriskofoesophagealcanceraftersurgery
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Topic15060Version10.0

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GRAPHICS
Highresolutionmanometryachalasia

Thishighresolutionmanometrydepictsthefindingsofachalasia.
Graphic67060Version2.0

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HighresolutionmanometryVigorousachalasia

Thispicturedepictsthehighamplitudesimultaneouscontractionsandnolower
esophagealsphincter(LES)contractionsofvigorousachalasia.
Graphic59926Version1.0

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Achalasia

Bariumswallowshowingadilatedesophagusandbird'sbeak
appearancetypicalofachalasia.Retainedfoodisalsovisible.
CourtesyofRamDickman,MD.
Graphic53672Version3.0

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PortplacementlaparoscopicHellermyotomy

Thisfigureillustratesthelocationsofthetrocarsforlaparoscopicesophageal
myotomy.
Graphic68596Version2.0

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Hellermyotomyperformedlaparoscopically

Thisisanintraoperativephotographofthelaparoscopicperformance
ofthemyotomyontheesophagus.
Graphic50030Version2.0

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GastriccomponentoftheLES

Theproximalmarginoftheloweresophagealsphincter(LES)extends
uptoandashortdistanceproximaltothesquamocolumnarjunction
(notshown).ThedistalmarginoftheLESismoredifficulttodefine
butcarefulanatomicstudiessuggestthatitiscomposedofelements
ofthegastricmusculature,theopposingclaspandslingfibersofthe
gastriccardia.
Adaptedfrom:LiebermannMeffertD,AllgwerM,SchmidP,BlumAL.Muscular
equivalentoftheloweresophagealsphincter.Gastroenterology197976:31.
Graphic69389Version4.0

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HellermyotomywithToupetfundoplication

Reproducedwithpermission.CopyrightUniversityofWashington.
Graphic62809Version3.0

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LaparoscopicHellermyotomywithToupet
fundoplication

ThisisanintraopertivephotographshowingtheedgeoftheToupet
fundoplicationsuturestotheedgeofthemyotomy.Theprocedure
wasperformedlaparoscopically.
Graphic72012Version2.0

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Hellermyotomyandfundoplication

Thisisanintraoperativephotographoftheanteriorfundusofthe
stomachfoldedovertheesophagealmyotomy.
Graphic52028Version1.0

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Disclosures
Disclosures:BrantKOelschlager,MDNothingtodisclose.RebeccaPPetersen,MD,MScNothingtodisclose.JosephSFriedberg,
MDNothingtodisclose.WenliangChen,MD,PhDNothingtodisclose.
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseareaddressedbyvettingthrougha
multilevelreviewprocess,andthroughrequirementsforreferencestobeprovidedtosupportthecontent.Appropriatelyreferenced
contentisrequiredofallauthorsandmustconformtoUpToDatestandardsofevidence.
Conflictofinterestpolicy

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