A 61-year-old woman with a history of Roux-en-y gastric bypass for morbid obesity presents with dysphagia and vomiting. She undergoes esophagogastroscopy at an outside institution where food impaction at the level of the gastrojejunostomy is found. A push-and-pull technique is used to remove the food. During the course of endoscopy, bleeding at the level of the upper esophageal sphincter is noted. After the procedure, the patient complains of sub-sternal chest pain.
A few hours later, crepitus is noted at the level of the sternal notch extending to both angles of the jaw. She has clear breath sounds bilaterally, and her abdomen is non-tender. She remains afebrile, but is tachycardic with a heart rate in the 120s. She has a leukocytosis with a white blood cell count of 20,000.
Esophageal perforation occurs in a variety of clinical scenarios. Iatrogenic trauma of the esophagus is the leading cause of esophageal perforation, with instrumentation accounting for approximately two-thirds of all cases. Instrumentation includes esophagoscopy, by far the leading iatrogenic cause, as well as pneumatic dilatation and bougienage. The risk of esophageal perforation with esophagoscopy alone is 0.03%, and rises to between 1% and 5% for endoscopic procedures such as variceal sclerotherapy and pneumatic dilatation for achalasia.1
Spontaneous perforation accounts for approximately 15% of esophageal ruptures and is the next leading etiology after esophageal instrumentation. Less common causes of esophageal perforation include foreign body ingestion, penetrating trauma, and neoplasm.
The esophagus originates at the level of the cricopharyngeus in the neck, traverses the posterior mediastinum, and ends in the upper abdomen just underneath the esophageal hiatus. Esophageal perforations can occur at any level, and the location often dictates the symptoms and the severity of the illness. While cervical perforations are tolerated relatively well, thoracic esophageal perforations and the ensuing mediastinal sepsis are potentially lethal if not promptly addressed.
Esophagoscopy most commonly perforates the esophagus at the level of the upper esophageal sphincter, which is the narrowest section. The esophagus is also prone to perforation at the level of the left mainstem bronchus and the gastroesophageal junction, the two other sections of esophageal narrowing.
Spontaneous perforations of the esophagus typically arise after violent vomiting or retching. The perforation is usually located in the distal esophagus just short of the gastroesophageal junction. The perforation is often directed toward the left, which is predisposed to disruption from the pathologic intraluminal pressure generated against a closed upper esophageal sphincter.
Esophageal perforation may lead to contamination of the deep cervical and prevertebral fascial planes, posterior mediastinum, pleural cavities, and peritoneum. A chemical inflammatory response is rapidly followed by local and systemic septic responses, as oropharngyeal flora contaminate the tissues surrounding the perforation. The mediastinal pleura is easily disrupted by the inflammation and leads to complicated pleural effusions and occasionally pneumothorax.
The clinical presentation is variable and depends on the etiology, severity, and location of the perforation, as well as the time lapse between occurrence and presentation for medical care. As symptoms are often nonspecific, a relatively high index of suspicion is necessary to avoid a delay in diagnosis. Neck, chest, or epigastric pain developing after esophageal instrumentation represents perforation until proven otherwise. Likewise, chest or epigastric pain after an episode of vomiting or retching should raise the suspicion of possible perforation. Other common symptoms include dysphagia, odynophagia, and dyspnea.
Physical examination may reveal crepitus, as air that is swallowed or insufflated by the endoscope tracks from the mediastinum into subcutaneous tissue. Pneumomediastinum may produce Hamman’s sign, which is an auscultatory finding in which a crunchy sound is made with each heartbeat as the heart moves against air in the mediastinal tissue. Signs of peritonitis may be present occasionally, when the perforation has disrupted the phrenoesophageal membrane and esophageal contents contaminate the peritoneal cavity. Fever, tachycardia, and tachypnea develop relatively early, while signs of systemic sepsis such as hypotension and oliguria may develop several hours after perforation. Leukocytosis is often present early in the course.
See Table 22–1.
Acute coronary syndrome
Ruptured aortic aneurysm
Gastric or peptic ulcer disease
Pneumothorax (iatrogenic and spontaneous)
WORKUP AND CHOICE OF IMAGING
It is well established that a delay in diagnosis of esophageal perforation significantly increases morbidity and mortality. As clinical presentations are quite disparate and findings are often subtle shortly after perforation, a high index of suspicion is necessary to facilitate prompt diagnosis.
Plain films of the neck, chest, and abdomen are often the initial imaging tests obtained. They are abnormal in approximately 90% of patients with an esophageal perforation.2 The sensitivity is reduced when the films are obtained immediately after a perforation. While plain films are frequently abnormal, they are relatively nonspecific, and moreover are limited in their ability to localize and characterize a leak.
Contrast esophagography is the ideal imaging modality to test for esophageal perforation. The sensitivity of this modality is 60% to 70% for cervical perforations and approximately 90% for intrathoracic perforations.3 Esophagography not only demonstrates the presence of a perforation, but also localizes its anatomic level and trajectory. Water-soluble contrast is often administered initially, followed by barium to increase sensitivity.3 Concerns about mediastinal extravasation of barium are often overstated, and there is little downside to using this agent to rule out a perforation. It is imperative that administration of water-soluble contrast is performed in a patient with minimal risks of aspiration, as these agents can incite a severe pneumonitis response. Dilute barium can be substituted in patients at risk for aspiration.
Computed tomography (CT) plays a role for some perforations that are not easily demonstrated by esophagography. CT scans are also invaluable when a patient cannot cooperate with a swallow study, as with the intubated patient. The sensitivity of neck, chest, and abdominal CT scans for esophageal perforation is about 90%, and combining CT imaging with esophagography raises the sensitivity to nearly 100%.4 The specificity of CT imaging is significantly less than that of esophagography. Signs such as pneumomediastinum or pleural effusion may be detected even when there is no clinically significant perforation, and may arise from other clinical conditions. CT imaging assists in the visualization of collections or abscesses that require operative or interventional radiology drainage.