Boerhaave SyndromeBoerhaave Syndrome

Preethi Yeturu and Erik Mikaitis Preethi Yeturu and Erik Mikaitis MS IVMS IV

BackgroundBackground

Transmural perforation of the esophagusTransmural perforation of the esophagus

Distinguished from Mallory-Weiss Distinguished from Mallory-Weiss syndrome (non-transmural tear)syndrome (non-transmural tear)

Diagnosis is difficult because often no Diagnosis is difficult because often no classic symptoms are presentclassic symptoms are present

HistoryHistory

First described in 1724 by Hermann BoerhaaveFirst described in 1724 by Hermann Boerhaave

His patient, Baron van Wassenaer, would eat His patient, Baron van Wassenaer, would eat large meals and induce vomiting by ingesting large meals and induce vomiting by ingesting ipecac so that he could immediately have ipecac so that he could immediately have another large mealanother large meal

After vomiting, he began having severe chest After vomiting, he began having severe chest pain & dyspnea and died 18 hours laterpain & dyspnea and died 18 hours later

At autopsy, Boerhaave found olive oil and roast At autopsy, Boerhaave found olive oil and roast duck in the left pleural cavity.duck in the left pleural cavity.

PathophysiologyPathophysiology

Rupture is caused by a sudden rise in Rupture is caused by a sudden rise in intraluminal esophageal pressure produced intraluminal esophageal pressure produced during vomiting.during vomiting.Neuromuscular incoordination results in failure Neuromuscular incoordination results in failure of the cricopharyngeus muscle to relax.of the cricopharyngeus muscle to relax.Most common location of the tear is the left Most common location of the tear is the left posterolateral wall of the lower third of the posterolateral wall of the lower third of the esophagus. esophagus. (2(2ndnd most common is subdiaphragmatic or upper most common is subdiaphragmatic or upper thoracic area)thoracic area)

CausesCauses

Commonly associated with:Commonly associated with: AlcoholismAlcoholism BulimiaBulimia Overindulgence in food and drinkOverindulgence in food and drink

EpidemiologyEpidemiology

Rare but most lethal perforation of the GI Rare but most lethal perforation of the GI tracttractMost studies report a 100% mortality Most studies report a 100% mortality within 7 days without surgerywithin 7 days without surgeryOnly a 70% overall survival with surgeryOnly a 70% overall survival with surgeryHowever, the syndrome is very rare:However, the syndrome is very rare: Only 16 cases reported from 1958-1973Only 16 cases reported from 1958-1973 A 1980 review cited only 300 cases in A 1980 review cited only 300 cases in

literature worldwideliterature worldwide

EpidemiologyEpidemiology

Accounts for 15% of all traumatic ruptures Accounts for 15% of all traumatic ruptures or perforations of the esophagusor perforations of the esophagus

Other 85% of ruptures are from iatrogenic Other 85% of ruptures are from iatrogenic perforation (Not Boerhaave’s syndrome)perforation (Not Boerhaave’s syndrome)

Mortality and MorbidityMortality and Morbidity

The overall mortality of 30% is due to:The overall mortality of 30% is due to: Subsequent infectionSubsequent infection MediastinitisMediastinitis PneumonitisPneumonitis PericarditisPericarditis EmpyemaEmpyema

EpidemiologyEpidemiology

There is equal distribution of Boerhaave’s There is equal distribution of Boerhaave’s throughout all racesthroughout all races

Male-to-female ratio ranges from 2:1 to 5:1Male-to-female ratio ranges from 2:1 to 5:1

Most frequently seen in patients aged 50-Most frequently seen in patients aged 50-70 years70 years

80% of patients are middle aged men80% of patients are middle aged men

DiagnosisDiagnosis

Clinical HistoryClinical History

Repeated episodes of retching and Repeated episodes of retching and vomitingvomiting

Sudden onset of chest pain in lower thorax Sudden onset of chest pain in lower thorax and upper abdomenand upper abdomen

Pain may radiate to the back or to the left Pain may radiate to the back or to the left shouldershoulder

Swallowing can aggravate the painSwallowing can aggravate the pain

Clinical HistoryClinical History

Hematemesis is not seen after rupture Hematemesis is not seen after rupture (which helps distinguish from Mallory-(which helps distinguish from Mallory-Weiss)Weiss)

Swallowing may precipitate coughSwallowing may precipitate cough

SOB is common due to pleuritic pain or SOB is common due to pleuritic pain or pleural effusionpleural effusion

Physical ExamPhysical Exam

Mackler triad is the classic presentation, Mackler triad is the classic presentation, including:including: VomitingVomiting Lower thoracic painLower thoracic pain Subcutaneous emphysemaSubcutaneous emphysema

Presentation may depend on:Presentation may depend on: Location of tearLocation of tear

Cervical tear may have neck of upper chest painCervical tear may have neck of upper chest painMid to lower esophagus tear may have interscapular or Mid to lower esophagus tear may have interscapular or epigastric pain.epigastric pain.

Cause of the injuryCause of the injury Time since the perforationTime since the perforation

Physical ExamPhysical Exam

Pleural effusion is commonPleural effusion is commonSubcutaneous emphysema is very helpfulSubcutaneous emphysema is very helpful It is seen in 28-66% of patients It is seen in 28-66% of patients Typically found laterTypically found later

Tachypnea and abdominal rigidity are other Tachypnea and abdominal rigidity are other classic findingsclassic findingsPneumomediastinum is an important findingPneumomediastinum is an important finding May cause crackling on chest auscultation (Hamman May cause crackling on chest auscultation (Hamman

crunch)crunch) Heard coincident with each heartbeat (can be Heard coincident with each heartbeat (can be

mistaken for pericarial friction rub)mistaken for pericarial friction rub) Found in 20% of casesFound in 20% of cases

Later stages of IllnessLater stages of Illness

Can manifest as signs of infection and Can manifest as signs of infection and sepsissepsis

Symptoms may include fever, Symptoms may include fever, hemodynamic instability, progressive hemodynamic instability, progressive obtundationobtundation

Diagnosis at later stages is more difficult Diagnosis at later stages is more difficult as septic complications begin to dominate as septic complications begin to dominate the clinical picturethe clinical picture

WorkupWorkup

Laboratory StudiesLaboratory Studies Findings are non-specificFindings are non-specific May present with leukocytosis with left shiftMay present with leukocytosis with left shift 50% of patients have hematocrit over 50%. 50% of patients have hematocrit over 50%. Thought to be due to fluid loss into pleural Thought to be due to fluid loss into pleural

spaces and tissuesspaces and tissues

ThoracentesisThoracentesis

If patient presents with pleural effusion:If patient presents with pleural effusion: Pleural fluid can aid in diagnosisPleural fluid can aid in diagnosis May find undigested food particles and gastric May find undigested food particles and gastric

juicesjuices If no gross particles are found, cytology can If no gross particles are found, cytology can

confirm its presenceconfirm its presence pH of the fluid will be less than 6 and amylase pH of the fluid will be less than 6 and amylase

will be elevatedwill be elevated Squamous cells from saliva may be foundSquamous cells from saliva may be found

Imaging StudiesImaging Studies

Upright Chest X-rayUpright Chest X-ray 90% of patients have an abnormality after perforation90% of patients have an abnormality after perforation Most common finding is a left unilateral effusionMost common finding is a left unilateral effusion May have: May have:

PneumothoraxPneumothoraxHydropneumothoraxHydropneumothoraxPneumomediastinumPneumomediastinumSubcutaneous emphysemaSubcutaneous emphysemaMediastinal wideningMediastinal widening

V-sign of NaclerioV-sign of NaclerioStreaks of air that dissect the planes behind the heart and form a Streaks of air that dissect the planes behind the heart and form a ‘V’.‘V’.Fairly specific, but very insensitiveFairly specific, but very insensitiveFound of 20% of patientsFound of 20% of patients

Imaging StudiesImaging Studies

EsophagramEsophagram Helps confirm the diagnosisHelps confirm the diagnosis Shows extravasation of contrastShows extravasation of contrast Outlines the length of the perforation and its location Outlines the length of the perforation and its location

(aids in decision of surgical approach, thoracic vs. (aids in decision of surgical approach, thoracic vs. abdominal)abdominal)

Initially use water soluble contrast (Gastrografin) Initially use water soluble contrast (Gastrografin) which has 90% sensitivitywhich has 90% sensitivity

Barium is associated with severe medistinitisBarium is associated with severe medistinitis If study is negative but suspicion remains high, try left If study is negative but suspicion remains high, try left

and right lateral decubitus imagesand right lateral decubitus images

Imaging StudiesImaging Studies

CT ScanCT Scan Can reveal decisive criteria for diagnosisCan reveal decisive criteria for diagnosis Helpful in patients too ill to tolerate Helpful in patients too ill to tolerate

esophagramsesophagrams Shows localized collections of fluidShows localized collections of fluid Visualizes adjacent structures to help narrow Visualizes adjacent structures to help narrow

the differential diagnoses.the differential diagnoses. Can demonstrate periesophageal air tracksCan demonstrate periesophageal air tracks It may not precisely localize the site of It may not precisely localize the site of

perforationperforation

ProceduresProcedures

EndoscopyEndoscopy Not commonly usedNot commonly used Carries a risk of increasing the size and Carries a risk of increasing the size and

extent of the perforation as well as pushing extent of the perforation as well as pushing more air through the perforationmore air through the perforation

More useful in thoracic esophagusMore useful in thoracic esophagus May be useful when perforation is suspected May be useful when perforation is suspected

but not provenbut not proven

TreatmentTreatment

Medical careMedical care

Therapy includes:Therapy includes: IV volume resuscitationIV volume resuscitation Broad-spectrum aantibioticsBroad-spectrum aantibiotics Prompt surgical interventionPrompt surgical intervention

Conservative vs aggressive treatment Conservative vs aggressive treatment depends on:depends on: Time delayTime delay Extent of perforationExtent of perforation Overall medical conditionOverall medical condition

Medical managementMedical management

Conservative management may be Conservative management may be appropriate if:appropriate if: The disruption is well contained within the The disruption is well contained within the

mediastinummediastinum The cavity should be drained back into the The cavity should be drained back into the

esophagusesophagus Few symptomsFew symptoms Clinical sepsis should be minimalClinical sepsis should be minimal

Conservative ManagementConservative Management

Consists of the following:Consists of the following: IVFIVF Antibiotics (Primaxin)Antibiotics (Primaxin) NGT on suctionNGT on suction Keep patient NPOKeep patient NPO Drainage with tube thoracostomyDrainage with tube thoracostomy Early us of nutritional supplements (via Early us of nutritional supplements (via

jejunostomy tube)jejunostomy tube)

Surgical CareSurgical Care

First successful surgical repair in 1947First successful surgical repair in 1947Goals in surgery:Goals in surgery: Direct repair of the ruptureDirect repair of the rupture Adequate drainage of the mediastinum and pleural Adequate drainage of the mediastinum and pleural

cavitycavity

Left thoracotomy is the preferred approachLeft thoracotomy is the preferred approachOmental flap may be used to support the Omental flap may be used to support the primary closureprimary closureGrastrostomy and jejunostomy tubes are placed Grastrostomy and jejunostomy tubes are placed for drainage and nutrition respectivelyfor drainage and nutrition respectively

Surgical CareSurgical Care

Alternatives to primary repair:Alternatives to primary repair: Creation of an esophageal diversion through Creation of an esophageal diversion through

the use of a loop or end-cervical the use of a loop or end-cervical esophagostomyesophagostomy

T-tubes result in the formation of a controlled T-tubes result in the formation of a controlled fistula and cause a drainage of esophageal fistula and cause a drainage of esophageal secretions and refluxed gastric materialssecretions and refluxed gastric materials

Primary repair can be considered for Primary repair can be considered for perforations as old as 72 hoursperforations as old as 72 hours

Surgical CareSurgical Care

Late complicationsLate complications

EmpyemaEmpyema

Esophagotrachael fistula Esophagotrachael fistula

esophagobronchial fistulaesophagobronchial fistula

ConsultationsConsultations

Thoracic or general surgery as soon as Thoracic or general surgery as soon as diagnosis is suspecteddiagnosis is suspected

Infectious disease for antimicrobial therapyInfectious disease for antimicrobial therapy

ComplicationsComplications

Esophageal rupture which may lead to:Esophageal rupture which may lead to: SepticemiaSepticemia PneumomediastinumPneumomediastinum MedistinitisMedistinitis Pleural effusionPleural effusion EmpyemaEmpyema Subcutaneous emphysemaSubcutaneous emphysema

Other complicationsOther complications

A rupture extending into the pleura will A rupture extending into the pleura will cause a hydropneumothoraxcause a hydropneumothorax

ARDSARDS

PrognosisPrognosis

Directly related to early recognition and Directly related to early recognition and appropriate interventionappropriate interventionEarly intervention allows for prompt Early intervention allows for prompt surgical repairsurgical repairPatients who undergo repair within 24 Patients who undergo repair within 24 hours have a 70-75% survival.hours have a 70-75% survival.Repair at 24-48 hours, survival drops to Repair at 24-48 hours, survival drops to 35-50%35-50%At more than 48 hours, survival is 10%At more than 48 hours, survival is 10%

Questions?Questions?