Boerhaave syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Feham Tariq, MD [2]
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Overview
Boerhaave syndrome (BHS) or esophageal perforation, is the rupture of the esophageal wall. It is an emergency, life-threatening condition. It is most often caused by excessive vomiting, eating disorders such as bulimia, although it may rarely occur in extremely forceful coughing or other situations such as obstruction by food. It can cause pneumomediastinum (air in the mediastinum) and/or mediastinitis (inflammation of the mediastinum) and sepsis. Boerhaave syndrome was first described in 1724 by the physician Herman Boerhaave, after whom it is named. Boerhaave syndrome may be classified according to the location of involvement into three groups or according to the time of presentation into three groups of acute, subacute and chronic perforation. Some of the causes of BHS include seizures, severe straining, vomiting, child birth, Iatrogenic, prolonged coughing or laughing. It is important to differentiate BHS from other diseases that cause abdominal pain such as Mallory-Weiss syndrome, myocardial infarction, pancreatitis, and peptic ulcer disease. The incidence of Boerhaave syndrome is 3.1 per 100,000 individuals. The most potent risk factor in the development of Boerhaave syndrome is overindulgence in food and alcohol, Iatrogenic. Other risk factors include Eosinophilic esophagitis, medication-induced esophagitis, and infectious ulcers. The mainstay of therapy for BHS is conservative which consists of intravenous fluids instituted as soon as the diagnosis is suspected, antibiotics, nasogastric suction, NPO, and adequate drainage with tube thoracostomy or formal thoracotomy. Surgical intervention is reserved primarily for the debridement of non-viable esophagus and repair of the perforation.
Historical Perspective
Boerhaave syndrome was first described in 1724 by the physician Herman Boerhaave, after whom it is named.
Classification
Boerhaave syndrome may be classified according to the location of involvement 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.
Pathophysiology
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.
Causes
Boerhaave syndrome (BHS) causes include seizures, severe straining, vomiting, child birth, Iatrogenic, prolonged coughing or laughing.
Differentiating Boerhaave syndrome overview from Other Diseases
Boerhaave syndrome must be differentiated from other diseases that cause abdominal pain such as Mallory-Weiss syndrome, myocardial infarction, pancreatitis, and peptic ulcer disease.
Epidemiology and Demographics
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 to age group is 1-17 years. Boerhaave syndrome is slightly more common in males.
Risk Factors
Boerhaave syndrome (BHS) usually occurs in patients with a normal underlying esophagus. The most potent risk factor in the development of Boerhaave syndrome is overindulgence in food and alcohol, Iatrogenic. Other risk factors include Eosinophilic esophagitis, medication-induced esophagitis, and Infectious ulcers.
Screening
There is insufficient evidence to recommend routine screening for Boerhaave syndrome.
Natural History, Complications, and Prognosis
If Boerhaave syndrome (BHS) is left untreated, the mortality reaches 100%, and without adequate treatment, survival of Boerhaave's syndrome is within days. Most common complications of Boerhaave syndrome include pneumomediastinum, Mediastinitis, Sepsis, Posterior mediastinal abscess. Boerhaave's syndrome has a high mortality rate (14-40%).
Diagnosis
History and Symptoms
The clinical manifestations of Boerhaave syndrome (BHS) depend on the location of the perforation. Boerhaave syndrome often presents with excruciating retrosternal chest pain due to an intrathoracic esophageal perforation. Boerhaave syndrome classically associated with a history of severe retching and vomiting, however, 25 to 45 percent of patients have no history of vomiting.
Physical Examination
The patient might be in moderate distress with epigastric pain and bleeding. Patients may experience Tachycardia, weak pulse, hypotension, Diaphoresis and fever.
Laboratory Findings
Laboratory findings often are non-specific. Patients may present with leukocytosis. As many as 50% of patients with Boerhaave syndrome have a hematocrit value of 50% due to fluid loss into pleural spaces and tissues.
Imaging Findings
Erect radiograph chest posteroanterior view is the most useful in early diagnosis. In most patients with Boerhaave syndrome, chest x-ray shows one-sided effusion, pneumothorax, hydropneumothorax, pneumomediastinum and subcutaneous emphysema.
Other Diagnostic Studies
Contrast esophagram usually establishes the diagnosis of an esophageal perforation the location and extent of perforation. The accuracy of the test depends on the size and location of the perforation and the technique used for the study. Gastrografin, a water-soluble contrast, is used for the esophagram procedure. If the Gastrografin esophagram is inconclusive, a barium esophagram should be performed. Gastrografin is preferred over the barium esophagram to prevent a barium-induced inflammatory response in mediastinum and pleural space, whereas the barium esophagram is very efficient in picking the small perforations.
Treatment
Medical Therapy
The mainstay of therapy for BHS is conservative which consists of intravenous fluids instituted as soon as the diagnosis is suspected, antibiotics, nasogastric suction, NPO, and adequate drainage with tube thoracostomy or formal thoracotomy.
Surgery
Most physicians advise surgical intervention if the diagnosis is made within the first 24 hours after perforation. The main objectives of surgical management in patients undergoing primary repair are debridement of non-viable esophagus and repair of the perforation. The surgical procedure often depends on the general condition of the patient, level of intrathoracic contamination and eligibility of the esophagus for primary repair.
Prevention
There are no established measures for the prevention of Boerhaave syndrome.