Boerhaave syndrome

Boerhaave syndrome (also called Boerhaave's syndrome), or Esophageal perforation, is rupture of the esophageal wall. It is most often caused by excessive vomiting in 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 and/or mediastinitis (air or inflammation of the mediastinum) and sepsis.

This condition was first documented by the 18th-century physician Herman Boerhaave, after whom it is named. A related condition is Mallory-Weiss syndrome.

Symptoms

It typically occurs after forceful vomiting. Boerhaave syndrome is a transmural perforation (full-thickness; a hole) of the esophagus, distinct from Mallory-Weiss syndrome, a nontransmural esophageal tear also associated with vomiting. Because it is generally associated with vomiting, 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, typically as a complication of an endoscopic procedure, feeding tube, or unrelated surgery. Boerhaave syndrome is often seen as a complication of Bulimia.

It is associated with "Mackler's triad" which consists of vomiting, lower thoracic pain and subcutaneous emphysema which the later can be heard as Hamman's crunch on physical examination.

Pathophysiology

Esophageal rupture in Boerhaave syndrome is thought to be the result of a sudden rise in internal esophageal pressure produced during vomiting, as a result of neuromuscular incoordination causing failure of the cricopharyngeus muscle (a sphincter within the esophagus) to relax. The syndrome is commonly associated with the consumption of excessive food and/or alcohol.

The most common anatomical location of the tear in Boerhaave syndrome is at left posterolateral wall of the lower third of the esophagus, 2–3 cm before the stomach.

Unfortunately, in the present time, the most common cause of oesphageal perforation is iatrogenic. However, it should also be noted that iatrogenic perforations, while still constituting a serious medical condition, are easier to treat and less prone to complications, particularly mediastinitits and sepsis. This owes to the fact that they usually do not involve contamination of the mediastinum with gastric contents.

Treatment

Untreated Boerhaave's syndrome is uniformly fatal. Its treatment includes immediate antibiotic therapy to prevent mediastinitis and sepsis, surgical repair of the perforation, and if there is significant fluid loss it should be replaced with IV fluid therapy since oral rehydration is, obviously, not possible. Even with early surgical intervention the risk of death is high.