TABLE 713 Causes of Esophageal Perforation

Perforation causes a dramatic presentation if esophageal contents leak into the mediastinum. A fulminant, necrotizing mediastinitis with polymicrobic infection that rapidly leads to shock and death can ensue. Perforation into the pleural or peritoneal spaces can occur as well, and contamination of these large potential spaces also tends to result in rapidly progressive infection and shock. If the perforation is small and leakage is contained by contiguous structures, the course may be significantly more indolent. Most spontaneous perforations occur through the left posterolateral wall of the distal esophagus. 24 Proximal perforation, seen mostly with instrumentation, tends to be less severe than distal and can be contained locally as a periesophageal abscess with minimal systemic toxicity.

Pain is classically described as acute, severe, unrelenting and diffuse; reported in the chest, neck and abdomen; and with radiation to the back and shoulders. Back pain may be the predominant symptom. Pain is often exacerbated by swallowing. Dysphagia, dyspnea, hematemesis, and cyanosis can be present as well. Less acute and atypical presentations are also described. Esophageal perforation is often ascribed to acute myocardial infarction (MI), pulmonary embolus, peptic ulcer disease, aortic catastrophe, or acute abdomen, resulting in critical delays in diagnosis, the most important factor in determining morbidity and mortality outcome.

Physical examination varies with the severity of the rupture and the elapsed time between the rupture and presentation. Abdominal rigidity with hypotension and fever often occur early. Tachycardia and tachypnea are common. Cervical subcutaneous emphysema is common in cervical esophageal perforations. Mediastinal emphysema takes time to develop. It is less commonly detected by examination or radiography in lower esophageal perforation, and its absence does not rule out perforation.25 Hammon's crunch, caused by air in the mediastinum being moved by the beating heart, can sometimes be auscultated. Pleural effusion develops in half of patients with intrathoracic perforations and is uncommon in those with cervical perforations. Pleural fluid can be due to either direct contamination of the pleural space or a sympathetic serous effusion from mediastinitis.

Making the correct diagnosis in a timely manner in an ill patients with esophageal perforation requires suspicion on the clinician's part. Chest radiography and contrast esophagography with water-soluble contrast most often make the diagnosis. Endoscopy, computed tomography (CT) of the chest, and thoracentesis can be useful adjuncts if esophagography (10 to 25 percent false-negative rate) is unrevealing in the face of high clinical suspicion. Endoscopy especially is often done after negative esophagography in penetrating trauma with suspicion of esophageal perforation.

Perforation of the esophagus is associated with a high mortality rate regardless of the underlying cause. The elapsed time between perforation and the initiation of therapy, the location of the perforation, and the etiology all affect outcome. Rapid, aggressive management is key to minimizing the morbidity and mortality associated with esophageal perforation. In the ED, resuscitation of shock, broad spectrum parenteral antibiotics, and emergent surgical consultation should be obtained as soon as the diagnosis is seriously entertained. Patients with systemic symptoms and signs after perforation need operative management. Criteria are developing for the nonoperative management of perforation in select patients.23

Instrumentation, especially endoscopic dilatation, has a relatively high rate of perforation; therefore, it is patients with strictures who sustain these injuries, usually perforating distally around the level of the obstruction. A patient with a relatively healthy esophagus undergoing instrumentation will more commonly perforate proximally. Perforation from other instrumentation, including nasogastric tube placement, has been reported. 26

Boerhaave's syndrome refers to full-thickness perforation of the esophagus following a sudden rise in intraesophageal pressure. The mechanism is sudden, forceful emesis in about three-fourths of the cases; coughing, straining, seizures, and childbirth have been reported as causing perforations as well. Alcohol is frequently an antecedent to this syndrome, which is seen more commonly in males. The perforation is usually in the distal esophagus on the left side.

Trauma to the esophagus accounts for roughly 10 percent of all esophageal perforations. Rupture from blunt injury is rare. Penetrating wounds to the esophagus from neck trauma occur but are often masked by more rapidly fatal injuries to the surrounding critical structures, such as the airway and major vessels. A combination of esophagography and esophagoscopy is used to assess patients for potential esophageal injury.

Foreign-body ingestion may result in perforation of the esophagus as well. The perforation is almost always at one of the sites of anatomic narrowing, where foreign bodies become wedged. The injury can be due to pressure necrosis from the object (coin), penetration from the object (pin, bones), or chemical irritation from the object (battery, pill).

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