Boerhaave syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Feham Tariq, MD [2]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]

Boerhaave syndrome was first described in 1724 by the physician Herman Boerhaave, after whom it is named.

• Boerhaave syndrome was first described by the physician Herman Boerhaave, Professor of Medicine at Leiden University, in a publication entitled “History of a Grievous Disease Not Previously Described”.^[1]
• Dr. Herman Boerhaave (1668-1738) described esophageal rupture and the subsequent mediastinal sepsis based upon his careful clinical and autopsy findings. Hundreds of references have since been written about Boerhaave’s syndrome.^[2][3]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamed Diab, MD [2] Ajay Gade MD[3]] Feham Tariq, MD [4] Shaghayegh Habibi, M.D.[5]

Boerhaave syndrome may be classified according to the location into three groups: distal intrathoracic, intra-abdominal and cervical esophageal perforation. It also may be classified according to the time of presentation into three groups: acute, subacute and chronic perforation.

Classification according to the location into three groups:^[1][2][3]

• Distal intrathoracic esophageal perforation
• Intra-abdominal esophageal perforation
• Cervical esophageal perforation

Also Boerhaave syndrome may be classified according to the time of presentation:^[4]

• Acute perforation: presents with symptoms within twenty-four hours after rupture
• Subacute perforation: symptoms develop between twenty four hours to two weeks following perforation
• Chronic perforation: the onset of symptoms is more insidious, often delaying presentation and diagnosis for weeks to months after rupture

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamed Diab, MD [2] Shaghayegh Habibi, M.D.[3]

Boerhaave syndrome is a spontaneous longitudinal perforation of the esophagus due to a sudden rise in intraesophageal pressure combined with negative intrathoracic pressure. It is commonly associated with the consumption of excessive food and/or alcohol or underlying medication-induced esophagitis, eosinophilic esophagitis, Barrett’s or infectious ulcers.

• Boerhaave syndrome is a spontaneous perforation of the esophagus due to a sudden rise in intraesophageal pressure combined with negative intrathoracic pressure. Some situations that can induce Boerhaave syndrome include:^[1]
  • Severe straining
  • Vomiting
  • Seizures
• In Boerhaave syndrome, the spontaneous transmural perforation may be as a result of neuromuscular incoordination resulting in a longitudinal esophageal perforation.^[2]
• The most common anatomical location of the esophageal perforation in Boerhaave syndrome is at the left posterolateral wall of the distal intrathoracic esophagus (the distal third of the esophagus is inherently weaker than the rest of the esophagus) and 2-3 cm before the stomach. However, the esophageal perforation in Boerhaave syndrome can also occur in the cervical or intra-abdominal esophagus.^[3]

• Boerhaave syndrome usually occurs in patients with a normal underlying esophagus while some patients have underlying medication-induced esophagitis, eosinophilic esophagitis, Barrett’s or infectious ulcers.^[4][5]
• Boerhaave syndrome is commonly associated with the consumption of excessive alcohol or over eating, or both. Either of these can induce vomiting.^[6][7]

• In Boerhaave syndrome the gross pathology is based on the underlying condition of esophagus which could be normal, eosinophilic esophagitis, Barrett’s or infectious ulcer.

• In Boerhaave syndrome the microscopic pathology is based on the underlying condition of the esophagus which could be normal, eosinophilic esophagitis, Barrett’s or infectious ulcer.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamed Diab, MD [2] Ajay Gade MD[3]] Feham Tariq, MD [4] Shaghayegh Habibi, M.D.[5]

Boerhaave syndrome (BHS) causes include seizures, severe straining, vomiting, child birth, Iatrogenic, prolonged coughing or laughing.

The causes of BHS are as follows:^{[1][2][3][4][5][6]}

• Seizures

• Severe straining
• Vomiting

• Childbirth
• Prolonged coughing or laughing

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamed Diab, MD [2] Shaghayegh Habibi, M.D.[3]

Boerhaave syndrome must be differentiated from other diseases that cause abdominal pain such as Mallory-Weiss syndrome, myocardial infarction, pancreatitis, and peptic ulcer disease.

 Acute onset of chest or abdominal pain may also be seen with disorders such as:

To review the differential diagnosis of dysphagia click here.

To review the differential diagnosis of dysphagia and weight loss click here.

To review the differential diagnosis of dysphagia and heartburn click here.

To review the differential diagnosis of dysphagia, weight loss and heartburn click here.

Boerhaave syndrome must be differentiated from other diseases that cause dysphagia such as reflux esophagitis, esophageal carcinoma, systemic sclerosis, esophageal spasm, pseudoachalasia, stroke, esophageal candidiasis and Chagas disease.^{[1][2][3][4][5][6][7][8][9][10][11]}

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamed Diab, MD [2] Ajay Gade MD[3]] Shaghayegh Habibi, M.D.[4]

The incidence of Boerhaave syndrome is 3.1 per 100,000 individuals. Boerhaave syndrome accounts for 15% of all cases of spontaneous perforations of the esophagus. Boerhaave syndrome is most frequently seen among patients 50-70 years of age, and the least susceptible age group is 1-17 years. Boerhaave syndrome is slightly more common in males.

• The incidence of Boerhaave syndrome syndrome is 3.1 per 100,000 individuals.^[1]
• Boerhaave syndrome accounts for 15% of all cases of spontaneous perforations of the esophagus.^[2]

• Boerhaave syndrome is most frequently seen among patients 50-70 years of age, and the least susceptible age group is 1-17 years.

• Boerhaave syndrome is slightly more common in males.^[3][4]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamed Diab, MD [2], Ajay Gade MD[3]], Feham Tariq, MD [4] Shaghayegh Habibi, M.D.[5]

Boerhaave syndrome (BHS) usually occurs in patients with a normal underlying esophagus. The most potent risk factors in the development of Boerhaave syndrome is overindulgence in food and alcohol and sometimes iatrogenic. Other risk factors include Eosinophilic esophagitis, medication-induced esophagitis, and Infectious ulcers.

The risk factors of BHS is as follows:^{[1][2][3][4][5][6]}

• Overindulgence in food
• Iatrogenic 
• Alcohol

• Eosinophilic esophagitis
• Medication-induced esophagitis
• Barrett’s esophagus
• Infectious ulcers

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamed Diab, MD [2] Ajay Gade MD[3]] Feham Tariq, MD [4] Shaghayegh Habibi, M.D.[5]

There is insufficient evidence to recommend routine screening for Boerhaave syndrome.

There is insufficient evidence to recommend routine screening for Boerhaave syndrome.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2], Ajay Gade MD[3]], Feham Tariq, MD [4]

Boerhaave syndrome (BHS), if left untreated, mortality will reach 100%. Survival of Boerhaave’s syndrome is witin days. Most common complications of Boerhaave syndrome include pneumomediastinum, mediastinitis, sepsis, posterior mediastinal abscess. Boerhaave’s syndrome has a high mortality rate (14-40%).

• The natural history of BHS is as follows:^[1][2]
• Diagnosis of Boerhaave’s syndrome can easily be missed or delayed, leading to complications like dehydration, mediastinitis, sepsis and shock.
• This accounts for the high mortality rate. If appropriate treatment is not started on time and if left untreated, the mortality rate reaches 100%.
• Without adequate treatment, survival of Boerhaave’s syndrome is within days.

Most common complications of Boerhaave syndrome include:^[3][4]

• Pneumomediastinum (air in the mediastinum)
• Mediastinitis (inflammation of the mediastinum)
• Infection and sepsis (fever, hypotension, and altered level of consciousness)
• Posterior mediastinal abscess

Boerhaave’s syndrome has a high mortality rate (14-40%).The non-specific clinical signs contribute to the poor outcome of Boerhaave syndrome. ^[5][6]

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