BoerhaaveSyndrome

Author:PraveenKRoy,MD,AGAFChiefEditor:JulianKatz,MDmore...
Updated:Mar15,2013

Background
Boerhaavefirstdescribedthespontaneousruptureoftheesophagusin1724.Ittypicallyoccursafterforceful
emesis.BoerhaavesyndromeisatransmuralperforationoftheesophagustobedistinguishedfromMalloryWeiss
syndrome,anontransmuralesophagealtearalsoassociatedwithvomiting.Becauseitusuallyisassociatedwith
emesis,Boerhaavesyndromeusuallyisnottrulyspontaneous.However,thetermisusefulfordistinguishingitfrom
iatrogenicperforation,whichaccountsfor8590%ofcasesofesophagealrupture.
DiagnosisofBoerhaavesyndromecanbedifficultbecauseoftennoclassicsymptomsarepresentanddelaysin
presentationformedicalcarearecommon.ApproximatelyonethirdofallcasesofBoerhaavesyndromeare
clinicallyatypical.Promptrecognitionofthispotentiallylethalconditionisvitaltoensureappropriatetreatment.
Mediastinitis,sepsis,andshockfrequentlyareseenlateinthecourseofillness,whichfurtherconfusesthe
diagnosticpicture.
SeeCan'tMissGastrointestinalDiagnoses,aCriticalImagesslideshow,tohelpdiagnosethepotentiallylife
threateningconditionsthatpresentwithgastrointestinalsymptoms.
Areportedmortalityestimateisapproximately35%,makingitthemostlethalperforationoftheGItract.Thebest
outcomesareassociatedwithearlydiagnosisanddefinitivesurgicalmanagementwithin12hoursofrupture.If
interventionisdelayedlongerthan24hours,themortalityrate(evenwithsurgicalintervention)risestohigherthan
50%andtonearly90%after48hours.Leftuntreated,themortalityrateiscloseto100%.

Pathophysiology
EsophagealruptureinBoerhaavesyndromeispostulatedtobetheresultofasuddenriseinintraluminal
esophagealpressureproducedduringvomiting,asaresultofneuromuscularincoordinationcausingfailureofthe
cricopharyngeusmuscletorelax.Thesyndromecommonlyisassociatedwithoverindulgenceinfoodand/oralcohol.
ThemostcommonanatomicallocationofthetearinBoerhaavesyndromeisattheleftposterolateralwallofthe
lowerthirdoftheesophagus,23cmproximaltothegastroesophagealjunction,alongthelongitudinalwallofthe
esophagus.Thesecondmostcommonsiteofruptureisinthesubdiaphragmaticorupperthoracicarea. [1,2]

Epidemiology
Frequency
International
Althoughlikelyunderreported,incidenceofBoerhaavesyndromeisrelativelyrare.A1980reviewbyKishcited300
casesintheliteratureworldwide. [3]A1986summarybyBladergroenetaldescribed127cases. [4]Ofthese,114
werediagnosedantemortemtheotherswerediagnosedatautopsy.Overall,Boerhaavesyndromeaccountsfor
15%ofalltraumaticruptureorperforationoftheesophagus.

Mortality/Morbidity
Prognosis
Prognosisisdirectlycontingentonearlyrecognitionandappropriateintervention.EarlydiagnosisofBoerhaave
syndromeallowspromptsurgicalrepair.Diagnosisandsurgerywithin24hourscarrya75%survivalrate.Thisdrops
toapproximately50%aftera24hourdelayandapproximately10%after48hours.
Morbidity/mortality
Themortalityrateishigh.EsophagealperforationisthemostlethalperforationoftheGItract.Survivalis
contingentlargelyuponearlyrecognitionandappropriatesurgicalintervention.
Overallmortalityrateisapproximately30%.Mortalityisusuallyduetosubsequentinfection,includingmediastinitis,
pneumonitis,pericarditis,orempyema.
Patientswhoundergosurgicalrepairwithin24hoursofinjuryhavea7075%chanceofsurvival.Thisfallsto35
50%ifsurgeryisdelayedlongerthan24hoursandtoapproximately10%ifdelayedlongerthan48hours.
Casesofpatientssurvivingwithoutsurgeryexistbutarerareenoughtowarrantcasereportsinthemedical
literature.
Complications
Esophagealrupturemayleadtothedevelopmentofsepticemia,pneumomediastinum,mediastinitis,massive
pleuraleffusion,empyema,pneumomediastinum,orsubcutaneousemphysema.
Iftheesophagealruptureextendsdirectlyintothepleura,hydropneumothoraxisexpected.Inadults,thisoccurs
morecommonlyontheleftsideofthepleura.Inneonates,theesophagealruptureusuallyoccursontherightside.

Aftertheesophagealrupture,freeairentersthemediastinumandalsomayspreadtotheadjacentstructuresand
resultinmediastinalabscessorsuperimposedsecondaryinfection.
Othercomplicationsincludeacuterespiratorydistresssyndrome,pneumomediastinum,pneumothorax,and
hydrothorax.

Race
Caseshavebeenreportedinallracesandonvirtuallyeverycontinent.

Sex
Thesyndromeisdescribedmorecommonlyinmalesthaninfemales,withratiosrangingfrom2:1to5:1.

Age
Boerhaavesyndromeisseenmostfrequentlyamongpatientsaged5070years.Reportssuggestthat80%ofall
patientsaremiddleagedmen.
CasesofBoerhaavesyndromehavebeendescribedinneonatesandinpersonsolderthan90years.Althoughno
clearexplanationexistsforthis,theleastsusceptibleagegroupappearstobechildrenaged117years.

ContributorInformationandDisclosures
Author
PraveenKRoy,MD,AGAFGastroenterologist,MarshfieldClinicAdjunctAssociateResearchScientist,
LovelaceRespiratoryResearchInstitute
PraveenKRoy,MD,AGAFisamemberofthefollowingmedicalsocieties:AmericanGastroenterological
AssociationandAmericanSocietyforGastrointestinalEndoscopy
Disclosure:Nothingtodisclose.
Coauthor(s)
RahamanMujibur,MDAcademicHospitalist/Hospitalist,MarshfieldClinic,StJoseph'sHospital
RahamanMujibur,MDisamemberofthefollowingmedicalsocieties:AmericanCollegeofPhysicians,
AmericanMedicalAssociation,andWisconsinMedicalSociety
Disclosure:Nothingtodisclose.
MarkEMurphy,MD,FACPAssistantProfessorofInternalMedicine,MercerUniversityMedicalSchool
ProgramDirector,GastroenterologyandHepatologyEducation,DepartmentofInternalMedicine,Memorial
HealthUniversityMedicalCenter
MarkEMurphy,MD,FACPisamemberofthefollowingmedicalsocieties:AmericanAssociationfortheStudy
ofLiverDiseases,AmericanCollegeofGastroenterology,AmericanGastroenterologicalAssociation,American
MedicalAssociation,AmericanSocietyforGastrointestinalEndoscopy,MedicalAssociationofGeorgia,and
SouthernMedicalAssociation
Disclosure:Nothingtodisclose.
ViswanathKalapatapu,MDStaffPhysician,DepartmentofInternalMedicine,MemorialHealthUniversity
MedicalCenter
ViswanathKalapatapu,MDisamemberofthefollowingmedicalsocieties:AmericanCollegeofPhysiciansand
AmericanMedicalAssociation
Disclosure:Nothingtodisclose.
ShowkatBashir,MDAssistantProfessor,DepartmentofMedicine,DivisionofGastroenterology,George
WashingtonUniversity,Washington,DC
ShowkatBashir,MDisamemberofthefollowingmedicalsocieties:AmericanCollegeofGastroenterology,
AmericanCollegeofPhysicians,AmericanGastroenterologicalAssociation,andAmericanMedicalAssociation
Disclosure:Nothingtodisclose.
AbhishekChoudhary,MDResidentPhysician,DepartmentofInternalMedicine,UniversityHospitalof
MissouriColumbia
AbhishekChoudhary,MDisamemberofthefollowingmedicalsocieties:AmericanCollegeofPhysicians
Disclosure:Nothingtodisclose.
SpecialtyEditorBoard
ManoopSBhutani,MDProfessor,CoDirector,CenterforEndoscopicResearch,TrainingandInnovation
(CERTAIN),Director,CenterforEndoscopicUltrasound,DepartmentofMedicine,DivisionofGastroenterology,
UniversityofTexasMedicalBranchDirector,EndoscopicResearchandDevelopment,TheUniversityofTexas
MDAndersonCancerCenter
ManoopSBhutani,MDisamemberofthefollowingmedicalsocieties:AmericanAssociationforthe
AdvancementofScience,AmericanCollegeofGastroenterology,AmericanCollegeofPhysicians,American
GastroenterologicalAssociation,AmericanInstituteofUltrasoundinMedicine,andAmericanSocietyfor
GastrointestinalEndoscopy
Disclosure:Nothingtodisclose.

FranciscoTalavera,PharmD,PhDAdjunctAssistantProfessor,UniversityofNebraskaMedicalCenter
CollegeofPharmacyEditorinChief,MedscapeDrugReference
Disclosure:MedscapeSalaryEmployment
AlexJMechaber,MD,FACPSeniorAssociateDeanforUndergraduateMedicalEducation,Associate
ProfessorofMedicine,UniversityofMiamiMillerSchoolofMedicine
AlexJMechaber,MD,FACPisamemberofthefollowingmedicalsocieties:AlphaOmegaAlpha,American
CollegeofPhysiciansAmericanSocietyofInternalMedicine,andSocietyofGeneralInternalMedicine
Disclosure:Nothingtodisclose.
ChiefEditor
JulianKatz,MDClinicalProfessorofMedicine,DrexelUniversityCollegeofMedicine
JulianKatz,MDisamemberofthefollowingmedicalsocieties:AmericanCollegeofGastroenterology,
AmericanCollegeofPhysicians,AmericanGastroenterologicalAssociation,AmericanGeriatricsSociety,
AmericanMedicalAssociation,AmericanSocietyforGastrointestinalEndoscopy,AmericanSocietyofLaw,
Medicine&Ethics,AmericanTraumaSociety,AssociationofAmericanMedicalColleges,andPhysiciansfor
SocialResponsibility
Disclosure:Nothingtodisclose.
AdditionalContributors
TheauthorsandeditorsofMedscapeDrugs&Diseasesgratefullyacknowledgethecontributionsofprevious
coauthor,PrakashRamanathan,MD,tothedevelopmentandwritingofthisarticle.

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