Overall, perforation is associated with a 20% mortality rate. The etiologies may be broadly divided into intra- and extraluminal categories.
Instrumentation injuries represent 75% of esophageal perforations and may occur during endoscopy, dilation, sclerosis of esophageal varices, transesophageal echocardiography, and tube passage. The most common sites are the anatomic sites of narrowing of the esophagus (e.g., at the cricopharyngeus and GE junction).
Foreign bodies can cause acute perforation, or more commonly follow an indolent course with late abscess formation in the mediastinum or development of empyema.
Ingested caustic substances, such as alkali chemicals, can produce coagulation necrosis of the esophagus.
Cancer of the esophagus may lead to perforation.
Barotrauma induced by external compression (e.g., Heimlich maneuver), forceful vomiting (Boerhaave syndrome), seizures, childbirth, or lifting can produce esophageal perforation. Almost all of these injuries occur in the distal esophagus on the left side.
Penetrating injuries to the esophagus can occur from stab wounds or, more commonly, gunshot wounds.
Blunt trauma may produce an esophageal perforation related to a rapid increase in intraluminal pressure or compression of the esophagus between the sternum and the spine.
Operative injury to the esophagus during an unrelated procedure occurs infrequently but has been reported in association with thyroid resection, anterior cervical spine operations, proximal gastric vagotomy, pneumonectomy, and laparoscopic fundoplication procedures.
Esophageal perforations initially manifest with dysphagia, pain, and fever and progress to leukocytosis, tachycardia, respiratory distress, and shock if the perforation is left untreated. Cervical perforations may present with neck stiffness and subcutaneous emphysema, and an intrathoracic perforation should be suspected in patients with chest pain, subcutaneous emphysema, dyspnea, and a pleural effusion (right pleural effusion in proximal perforations, left effusion in distal perforations). Patients with intra-abdominal perforations usually present with peritonitis.
The diagnosis of esophageal perforation is suggested by pneumomediastinum, pleural effusion, pneumothorax, atelectasis, and soft-tissue emphysema on chest x-ray or mediastinal air and fluid on computed tomography (CT) scan. Rapid evaluation with water-soluble or dilute barium contrast esophagography is mandatory, although contrast studies carry a 10% false-negative rate for esophageal perforations. Because esophagoscopy is used primarily as an adjunctive study and can miss sizable perforations, any discoloration or submucosal hematoma should be considered highly suspicious for perforation after trauma to the posterior mediastinum. Whenever an esophageal perforation is suspected, diagnosis and treatment must be prompt because morbidity and mortality increase in direct proportion to the delay.
Principles of management include (1) adequate drainage of the leak, (2) intravenous antibiotics, (3) aggressive fluid resuscitation, (4) adequate nutrition, (5) relief of any distal obstruction, (6) diversion of enteric contents past the leak, and (7) restoration of GI integrity. Initially, patients are kept on nothing-by-mouth status, a nasogastric tube is placed carefully in the esophagus or stomach, and they receive intravenous hydration and broad-spectrum antibiotics.
Definitive management generally requires operative repair, although a carefully selected group of nontoxic patients with a locally contained perforation may be observed. Patients with an intramural perforation after endoscopic procedures or dilation have a characteristic radiographic finding of a thin collection of contrast material parallel to the esophageal lumen without spillage into the mediastinum. Management with a nasogastric tube and antibiotics almost always is successful in these patients.
Cervical and upper thoracic perforations usually are treated by cervical drainage alone or in combination with esophageal repair.
Thoracic perforations should be closed primarily and buttressed with healthy tissue, and the mediastinum should be drained widely. Even when perforations are more than 24 hours old, primary mucosal closure usually is possible. When primary closure is not possible, options include wide drainage alone or in conjunction with resection, or with exclusion and diversion in cases of severe traumatic injury to the esophagus.
Abdominal esophageal perforations typically result in peritonitis and require an upper abdominal midline incision to correct.
Perforations associated with intrinsic esophageal disease (e.g., carcinoma, hiatal hernia, or achalasia) require addressing the perforation as described previously and surgically correcting the associated esophageal disease concomitantly.