Boerhaave Syndrome

Boerhaave syndrome typically occurs after forceful emesis and retching. Boerhaave syndrome is a transmural perforation of the esophagus and should be distinguished from Mallory-Weiss syndrome, a nontransmural esophageal tear also associated with vomiting. Since the perforation occurs with emesis, Boerhaave syndrome is usually not truly spontaneous, but this term helps distinguish it from iatrogenic perforation. Vomiting is the most common cause, but any activity that increases intraesophageal pressure can result in this syndrome. This condition can manifest in patients with a typically functioning esophagus, yet there is a subgroup where specific esophageal abnormalities or pathology are identified. Boerhaave syndrome accounts for 10% to 15% of all esophageal perforations.

Diagnosis of this condition can be challenging because the presentation can vary significantly, thus requiring an index of suspicion. Boerhaave syndrome is classically associated with the Mackler triad of vomiting, chest pain, and subcutaneous emphysema. However, patients rarely present with all of these symptoms and often have vague, nonspecific complaints. This can contribute to a delay in diagnosis and poor outcomes. Boerhaave syndrome is one of the most lethal gastrointestinal tract disorders, with a mortality rate of up to 60% with intervention, increasing to nearly 100% without intervention. Treatment is varied and depends on the time of diagnosis and the patient’s clinical condition at presentation. Management can range from conservative management to major surgical resection.