Boerhaave syndrome, also known spontaneous esophageal rupture, is a rare (accounting for only 16% of all traumatic ruptures of the esophagus) but serious condition. Boerhaave Syndrome is characterized by a complete transmural rupture of the esophagus resulting from forceful vomiting (causing barogenic trauma).
Uncoordinated vomiting with pyloric closure and diaphragmatic contraction against a contracted cricopharyngeal muscle causing a sudden rise in intraluminal pressure is theorized to be the underlying cause of Boerhaave syndrome. The most common site of rupture is the left posterolateral wall of the lower third of the esophagus, which is the weakest point of the esophagus.
There is an association between Boerhaave syndrome and an overindulgence in alcohol and food, as expected from a condition that is associated with vomiting. Boerhaave syndrome has a significantly increased incidence in men as compared with women, with a ratio of approximately 2:1.
Boerhaave syndrome typically presents with a history of nausea and vomiting, followed by severe lower thoracic and epigastric pain. The pain may radiate to the back or to the left shoulder, and may be worsened by swallowing. Vomiting blood (hematemesis) is typically not a complaint with esophageal rupture, and this can help to differentiate it from a Mallory-Weiss tear.
Another common complaint with Boerhaave syndrome is shortness of breath, resulting from pleuritic chest pain or a pleural effusion.
The classic presentation seen in Boerhaave syndrome is known as The Mackler Triad. Other classic findings include rapid respirations (tachypnea) and abdominal rigidity.
Unusual (atypical) findings in Boerhaave Syndrome include peripheral cyanosis, hoarseness caused by involvement of the recurrent laryngeal nerve, tracheal shift, cervical vein distention, and proptosis. Nonspecific findings include tachycardia, diaphoresis, fever, and hypotension, especially as the condition evolves. More advanced stages of rupture typically present as sepsis with progression toward multi-organ failure. Despite these symptoms and signs, the clinical presentation of Boerhaave Syndrome can be variable and, often, the classic triad of symptoms and signs (ie, the Mackler Triad) can be absent, which can make establishing the diagnosis difficult.