eMedicine Specialties > Gastroenterology > Esophagus

Boerhaave Syndrome

Author: Praveen K Roy, MD, Comments and Criticisms Editor, Cochrane Colorectal Cancer Group
Coauthor(s): Mark E Murphy, MD, FACP, Assistant Professor of Internal Medicine, Mercer University Medical School; Program Director, Gastroenterology and Hepatology Education, Department of Internal Medicine, Memorial Health University Medical Center; Abhishek Choudhary, MD, Resident, Department of Internal Medicine, University Hospital of Missouri; Mohamed Othman, MD, Staff Physician, Department of Internal Medicine, University of New Mexico School of Medicine; Viswanath Kalapatapu, MD, Staff Physician, Department of Internal Medicine, Memorial Health University Medical Center; Jack Bragg, DO, FACOI, Assistant Professor, Department of Clinical Medicine, University of Missouri School of Medicine; Gautam Dehadrai, MD, Department Chair, Section Chief, Department of Interventional Radiology, Norman Regional Hospital
Contributor Information and Disclosures

Updated: Aug 12, 2008

Introduction

Background

Boerhaave first described the spontaneous rupture of the esophagus in 1724. It typically occurs after forceful emesis. Boerhaave syndrome is a transmural perforation of the esophagus to be distinguished from Mallory-Weiss syndrome, a nontransmural esophageal tear also associated with vomiting. Because it usually is associated with emesis, Boerhaave syndrome usually is not truly spontaneous. However, the term is useful for distinguishing it from iatrogenic perforation, which accounts for 85-90% of cases of esophageal rupture.

Diagnosis of Boerhaave syndrome can be difficult because often no classic symptoms are present and delays in presentation for medical care are common. Approximately one third of all cases of Boerhaave syndrome are clinically atypical. Prompt recognition of this potentially lethal condition is vital to ensure appropriate treatment. Mediastinitis, sepsis, and shock frequently are seen late in the course of illness, which further confuses the diagnostic picture.

A reported mortality estimate is approximately 35%, making it the most lethal perforation of the GI tract. The best outcomes are associated with early diagnosis and definitive surgical management within 12 hours of rupture. If intervention is delayed longer than 24 hours, the mortality rate (even with surgical intervention) rises to higher than 50% and to nearly 90% after 48 hours. Left untreated, the mortality rate is close to 100%.

Pathophysiology

Esophageal rupture in Boerhaave syndrome is postulated to be the result of a sudden rise in intraluminal esophageal pressure produced during vomiting, as a result of neuromuscular incoordination causing failure of the cricopharyngeus muscle to relax. The syndrome commonly is associated with overindulgence in food and/or alcohol. The most common anatomical location of the tear in Boerhaave syndrome is at the left posterolateral wall of the lower third of the esophagus, 2-3 cm proximal to the gastroesophageal junction, along the longitudinal wall of the esophagus. The second most common site of rupture is in the subdiaphragmatic or upper thoracic area.

Frequency

International

Although likely underreported, incidence of Boerhaave syndrome is relatively rare. A 1980 review by Kish cited 300 cases in the literature worldwide.1 A 1986 summary by Bladergroen et al described 127 cases.2 Of these, 114 were diagnosed antemortem; the others were diagnosed at autopsy. Overall, Boerhaave syndrome accounts for 15% of all traumatic rupture or perforation of the esophagus.

Mortality/Morbidity

The mortality rate is high. Esophageal perforation is the most lethal perforation of the GI tract. Survival is contingent largely upon early recognition and appropriate surgical intervention.

  • Overall mortality rate is approximately 30%.Mortality is usually due to subsequent infection, including mediastinitis, pneumonitis, pericarditis, or empyema
  • Patients who undergo surgical repair within 24 hours of injury have a 70-75% chance of survival. This falls to 35-50% if surgery is delayed longer than 24 hours and to approximately 10% if delayed longer than 48 hours.
  • Cases of patients surviving without surgery exist but are rare enough to warrant case reports in the medical literature.

Race

  • Cases have been reported in all races and on virtually every continent.

Sex

  • The syndrome is described more commonly in males than in females, with ratios ranging from 2:1 to 5:1.

Age

  • Boerhaave syndrome is seen most frequently among patients aged 50-70 years.
  • Reports suggest that 80% of all patients are middle-aged men.
  • Cases of Boerhaave syndrome have been described in neonates and in persons older than 90 years. Although no clear explanation exists for this, the least susceptible age group appears to be children aged 1-17 years.

Clinical

History

  • The classic clinical presentation of Boerhaave syndrome usually consists of repeated episodes of retching and vomiting, typically in a middle-aged man with recent excessive dietary and alcohol intake.
  • These repeated episodes of retching and vomiting are followed by a sudden onset of severe chest pain in the lower thorax and the upper abdomen. The pain may radiate to the back or to the left shoulder. Swallowing often aggravates the pain.
  • Typically, hematemesis is not seen after esophageal rupture, which helps distinguish it from the more common Mallory-Weiss tear.
  • Swallowing may precipitate coughing because of communication between the esophagus and the pleural cavity.
  • Atypical clinical features sometimes delay a prompt diagnosis and appropriate intervention. This may result in an increase in morbidity and mortality.
  • Shortness of breath is a common complaint and is due to pleuritic pain or pleural effusion.

Physical

  • The Mackler triad defines the classic presentation of Boerhaave syndrome. It consists of vomiting, lower thoracic pain, and subcutaneous emphysema.
  • Presentation may vary depending on the following:
    • The location of the tear
    • The cause of the injury
    • The amount of time that has passed from the perforation to the intervention
  • Patients with cervical esophagus perforation may present with neck or upper chest pain.
  • Patients with middle or lower esophagus perforation may present with interscapular or epigastric discomfort.
  • Findings of pleural effusion are common.
  • If present, subcutaneous emphysema is particularly helpful for confirming diagnosis.  
    • Subcutaneous emphysema is seen in 28-66% of patients at initial presentation.
    • More typically, subcutaneous emphysema is found later.
  • Other classic findings include tachypnea and abdominal rigidity.
  • Tachycardia, diaphoresis, fever, and hypotension are common, particularly as the illness progresses. However, these findings are nonspecific.
  • Unusual findings may include the following:  
    • Peripheral cyanosis
    • Hoarseness of voice due to recurrent laryngeal nerve involvement
    • Tracheal and mediastinal shift
    • Cervical vein distention
    • Proptosis
  • Pneumomediastinum is a very important finding.  
    • It may cause a crackling sound upon chest auscultation, known as the Hamman crunch.
    • The crunch typically is heard coincident with each heartbeat and may be mistaken for a pericardial friction rub.
    • This is present in 20% of patients.
  • Later stages of illness may manifest as signs of infection and sepsis.  
    • Symptoms may include fever, hemodynamic instability, and progressive obtundation.
    • Establishing a diagnosis in the later stages can be quite difficult because septic complications begin to dominate the clinical picture.
    • Early diagnosis is critical.

Causes

  • It is a barogenic injury resulting from a sharp increase in intraluminal pressure against a closed cricopharyngeus.
  • Perforation typically occurs at the weakest point in the esophagus.
    • Usually the left lower esophagus below the diaphragm in adults
    • Usually into the right pleural cavity in very young patients
  • Alcoholism and overindulgence in food and drink are primary risk factors. Both can lead to emesis, which is a major component of the classic presentation.
  • No genetic predilection is apparent.

More on Boerhaave Syndrome

Overview: Boerhaave Syndrome
Differential Diagnoses & Workup: Boerhaave Syndrome
Treatment & Medication: Boerhaave Syndrome
Follow-up: Boerhaave Syndrome
References
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Further Reading

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Keywords

Boerhaave syndrome, Boerhaave's syndrome, esophageal perforation, esophageal rupture, perforated esophagus, esophagus perforation, esophagus rupture, ruptured esophagus, esophagus tear, esophageal tear, spontaneous esophageal rupture, spontaneous rupture of the esophagus, transmural perforation of the esophagus, forceful emesis, emesis complications

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Contributor Information and Disclosures

Author

Praveen K Roy, MD, Comments and Criticisms Editor, Cochrane Colorectal Cancer Group
Praveen K Roy, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and Canadian Association of Gastroenterology
Disclosure: Nothing to disclose.

Coauthor(s)

Mark E Murphy, MD, FACP, Assistant Professor of Internal Medicine, Mercer University Medical School; Program Director, Gastroenterology and Hepatology Education, Department of Internal Medicine, Memorial Health University Medical Center
Mark E Murphy, MD, FACP is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Medical Association, American Society for Gastrointestinal Endoscopy, Medical Association of Georgia, and Southern Medical Association
Disclosure: Nothing to disclose.

Abhishek Choudhary, MD, Resident, Department of Internal Medicine, University Hospital of Missouri
Abhishek Choudhary, MD is a member of the following medical societies: American College of Physicians
Disclosure: Nothing to disclose.

Mohamed Othman, MD, Staff Physician, Department of Internal Medicine, University of New Mexico School of Medicine
Disclosure: Nothing to disclose.

Viswanath Kalapatapu, MD, Staff Physician, Department of Internal Medicine, Memorial Health University Medical Center
Viswanath Kalapatapu, MD is a member of the following medical societies: American College of Physicians and American Medical Association
Disclosure: Nothing to disclose.

Jack Bragg, DO, FACOI, Assistant Professor, Department of Clinical Medicine, University of Missouri School of Medicine
Jack Bragg, DO, FACOI is a member of the following medical societies: American College of Osteopathic Internists and American Osteopathic Association
Disclosure: Nothing to disclose.

Gautam Dehadrai, MD, Department Chair, Section Chief, Department of Interventional Radiology, Norman Regional Hospital
Gautam Dehadrai, MD is a member of the following medical societies: American College of Radiology, Medical Council of India, and Radiological Society of North America
Disclosure: Nothing to disclose.

Medical Editor

Manoop S Bhutani, MD, FACG, FACP, Professor, Department of Medicine, Division of Gastroenterology, Director, Center for Endoscopic Ultrasound, Co-Director, Center for Endoscopic Research, Training and Innovation, University of Texas Medical Branch at Galveston
Manoop S Bhutani, MD, FACG, FACP is a member of the following medical societies: American Association for the Advancement of Science, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Institute of Ultrasound in Medicine, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

James L Achord, MD, Professor Emeritus, Department of Medicine, Division of Digestive Diseases, University of Mississippi School of Medicine
James L Achord, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Medical Association, American Society for Gastrointestinal Endoscopy, Mississippi State Medical Association, New York Academy of Sciences, Sigma Xi, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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