Boerhaave’s syndrome has also been called “barogenic perforation” and “postemetic perforation.” Although the term “spontaneous esophageal rupture” is also used for Boerhaave’s syndrome, truly spontaneous cases are rare, because most cases occur after severe vomiting.

Although Boerhaave’s syndrome is classically postemetic, other causes of a sudden rise in intraesophageal pressure, including weight lifting, laughing, hyperemesis gravidarum, and seizures, can result in “spontaneous” rupture. Spontaneous rupture almost always occurs on the left side of the distal third of the esophagus. A review of l84 cases of spontaneous rupture revealed that the vast majority (90%) were in the lower third of the esophagus, with 8% occurring in the middle third and 2% in the upper third. Other large series have confirmed this site distribution. Most tears occur along the longitudinal axis, and they vary from 0.6 to 8.9 cm in length. The mucosal tear is often longer than the muscle tear, which is important to repair the esophageal wall completely. The reason for the predilection for the lower third of the esophagus on the left side remains unclear, but weakening of the wall at the sites of entry of nerves and vessels, lack of adjacent supporting structures, the vertical orientation of the longitudinal muscle bundles, and the anterior angulation of the esophagus are all regarded as contributing factors.

The incidence of esophageal perforation as a result of trauma is 8% to 15.3%. Trauma-related perforation is divided into two types: blunt trauma and penetrating trauma. The incidence of esophageal perforation as a result of blunt trauma is very low (0.001%). The most common cause is chest injury by a steering wheel in a traffic accident. The intraesophageal pressure rises rapidly and causes perforation of the hypopharyngoesophageal junction. Esophageal perforation sometimes occurs as a result of intratracheal injury, fracture of the second rib, fracture of the cervical spine, and hyperextension of the neck.

Penetrating injuries produced by knives and bullets are more common. The incidence of esophageal perforation by penetrating injuries is 11% to 17%. Perforation is more common in the cervical esophagus than in the thoracic esophagus. The overall mortality rate remains high (15% to 40%).

Locally advanced esophageal cancer is sometimes associated with an esophageal fistula to the mediastinum, an esophagotracheal, or an esophagobronchial fistula.

Foreign body ingestion and endoscopic removal are sometimes associated with esophageal perforation (7% to 14%). The site of the perforation is at physiological narrowing. Penetrating injuries by sharp or pointed metallic objects, animal or fish bones, and toothpicks can cause perforation. Pressure
necrosis by foreign body, such as a coin, is a rare cause of perforation except in children. The initial clinical manifestations of esophageal perforation may occur within 48 hours or as late as 2 weeks.

Ingestion of caustic substances can cause devastating injury to the esophagus. Liquefaction necrosis of the esophageal wall after ingestion of lye causes the wall of the esophagus to degenerate and results in perforation. The perforation occurs as a result of inflammation, vascular thrombosis, ulceration, and necrosis of the esophageal wall 3 to 5 days after ingestion.

The drugs that are the most common cause of esophageal perforation are tetracycline preparations, potassium chloride, quinidine, and nonsteroidal anti-inflammatory drugs. Sustained-release formulations are more likely to cause injury. Long-term contact between drugs and the esophageal mucosa can cause esophageal perforation.

Infectious esophagitis, including Candida esophagitis, herpes simplex viral esophagitis, and cytomegaloviral esophagitis, usually develops in immunocompromised hosts. Although infectious esophagitis is a rare cause of esophageal perforation, there have been reports of spontaneous perforation associated with Candida esophagitis and herpes simplex viral esophagitis.

Barrett’s ulcer and ulcerative esophagitis with Zollinger–Ellison syndrome sometimes cause perforation. Aneurysms and aberrant right subclavian artery also sometimes result in esophageal rupture.

The surgical approach depends on the location of the perforation. When the perforation is in the cervical esophagus, the esophagus can usually be exposed through a left oblique incision in the neck. If the perforation is in the upper two-thirds of the thoracic esophagus, it can be approached through a right posterolateral thoracotomy in the fourth or fifth intercostal space. The perforation of the lower third of the thoracic esophagus can be approached through a left posterolateral thoracotomy in the sixth or seventh intercostal space. If the perforation is in the abdominal esophagus,
the esophagus can be approached through an upper midline laparotomy. Minimally invasive approaches, such as thoracoscopic or laparoscopic approaches, have recently been reported. Although simple primary closure and primary closure reinforced with gastric fundus are possible, it seems difficult to completely debride all the infected and necrotic tissues and to secure closure of the perforation by reinforcing it with muscle, such as with a pedicled intercostal muscle flap. The minimally invasive approaches, therefore, may be limited to early and uncontaminated perforations of the esophagogastric junction.