Esophageal Perforations

Emerg Med 37(7):33-37, 2005

The authors discuss how certain procedures can lead to iatrogenic perforations of the esophagus, how such perforations manifest themselves, and how they should be managed. Mallory-Weiss tears of the esophagus due to retching are also covered.

By James W. Smith, MD, and John Lang Gosserand, MD

In 1724 Hermann Boerhaave recorded the first clinicopathologic description of esophageal perforation when he documented the case of Lord High Admiral Wassenaer of the Dutch navy. The admiral, a man who liked to feast and indulge in large amounts of alcohol, had developed a transverse perforation of his esophagus after an episode of vomiting. During the autopsy three days later, Boerhaave placed his finger through the hole in the admiral's esophagus and found duck meat in the pleural space.

Rupture of the esophagus caused by vomiting or retching thus acquired the name Boerhaave syndrome. It is associated with very high morbidity and mortality and has been described as the most rapidly fatal and serious perforation of the gastrointestinal tract. But the admiral's demise represents a rare scenario compared with other clinical situations leading to the same outcome. Spontaneous rupture of the esophagus caused by vomiting or severe retching probably accounts for about 15% of cases. The more common scenario—about 75% of cases—is iatrogenic rupture of the esophagus.

What are the situations in which perforations of the esophagus can occur?

Diagnostic and therapeutic esophagogastroduodenoscopy (EGD) and dilations of the esophagus can be associated with esophageal perforation. Dilation is usually accomplished by one of three techniques: Maloney bougienage, Savary dilators, or through-the-scope hydrostatic balloon dilators. Any of these can induce a bursting type of injury to the esophagus if excessive radial force is exerted on the esophageal walls, especially where there is fibrotic tissue such as a stricture.

Considering all of the different types of instruments that are passed either orally or transnasally into the throat and esophagus, it is not surprising that there are rare reports of esophageal perforations with these procedures. Sengstaken-Blakemore tubes, used often in the past but now infrequently for control of variceal bleeding, are notorious for perforations. Pneumatic dilation, one of the methods for treating achalasia, is performed using a large-diameter (30- to 35-mm) balloon and causes esophageal perforation in about 5% of cases. Over-tubes passed over an endoscope to protect the airway in cases of foreign body extraction can produce injury at the cricopharyngeus. Rarely, even an endotracheal tube introduced at the time of anesthesia induction can inadvertently enter the esophagus and cause perforation.

Other causes include transesophageal echocardiography and, in rare cases, the passage of a simple nasogastric tube. There are particularly dangerous therapeutic procedures such as laser ablation of esophageal cancer and endoscopic mucosal resection in which the physician and patient accept this increased risk. Surgery, such as fundoplication for reflux disease, poses a slight risk of perforation, which may be difficult to diagnose early in the postoperative setting. Purely diagnostic endoscopy is complicated in exceedingly few instances.

Is the esophagus more likely to perforate than other parts of the gastrointestinal tract?

The esophagus is the part of the upper gastrointestinal tract most likely to be injured during an EGD, although this occurs less often than traumatic complications of other forms of endoscopy, such as colonic perforation during colonoscopy. Two factors lead to the vulnerability of the esophagus. The esophageal layers from inner to outer are the mucosa, the basement membrane, the lamina propria, a thin muscle layer called the muscularis mucosa, the submucosa, the muscularis propria, and finally the adventitia. Unlike other parts of the gastrointestinal tract, the esophagus lacks a serosal layer. This layer, which contains collagen and elastic fibers, is a protective barrier for other regions of the gastrointestinal tract. The absence of a serosal layer in the esophagus may lead to not only a greater risk of perforation, but also a greater likelihood of bacterial contamination if perforation does occur. The other factor that may pose additional risk for the esophagus is the greater likelihood of stricture formation, which is usually a result of reflux disease.

Are there specific conditions of the esophagus that create increased risk for perforations?

A variety of conditions or clinical scenarios can pose an increased risk for esophageal perforation during instrumentation. A Zenker's diverticulum can lead to a proximal perforation. Patients with a Zenker's often give a history of delayed postprandial regurgitation of undigested food, and all patients should be questioned about this symptom, especially the elderly.

Diverticula of the esophagus can occur, although somewhat rarely. They occur in the mid-esophagus (usually from "traction" and inflammatory disease of the mediastinum or lungs) or the distal esophagus (usually related to motility disorders of the esophagus). A diverticulum can be perforated when any instrument, especially a bougie dilator or nasogastric tube, is inadvertently passed into it rather than the true esophageal lumen.

Endoscopic sclerotherapy, which is performed less often now than in the past for bleeding esophageal varices, can cause deep esophageal ulcerations and damage leading to what has been called a "thinning" type of injury.

There are other specific causes of esophageal perforation, notably ingestion of foreign bodies such as fish bones, pins, glass, and other sharp objects. The injuries usually occur at areas of acute angulation or physiologic narrowing. For instance, the level of the cricopharyngeal muscle is a frequent site for this occurrence. After ingestion of the sharp object, it may take two weeks for the foreign body to erode through the esophageal wall.

Finally, the risk of perforation is always increased in the presence of a stricture, as previously noted, or a tumor.

What are the signs and symptoms of an esophageal perforation?

Chest pain is the cardinal symptom and the most frequent presenting symptom, seen in about 70% of cases. But it is important to remember that the clinical presentation of esophageal perforation is variable and can be nonspecific. If the perforation has occurred in the more common iatrogenic setting, the chest pain may occur immediately after the procedure, upon first ingestion of food or liquid after the procedure, after 24 hours, or not until days or even weeks later. Usually the pain is acute and sudden in onset, and it may radiate to the back or left shoulder. It can be followed by vomiting (25% to 30% of cases) and shortness of breath (20% to 25% of cases). Chest pain and dyspnea are symptoms seen more often with acute perforation, whereas dysphagia and supraventricular arrhythmia are features seen more with chronic perforation. A key point to remember is that perforations—unlike Mallory-Weiss tears, which are discussed at the end of this article—will not manifest with hematemesis or other signs of gastrointestinal bleeding such as melena.

The triad of vomiting, chest pain, and subcutaneous emphysema is known as Mackler's triad. Rarely, a patient may have back pain rather than chest pain. Perforations of the cervical esophagus may cause neck pain. Rupture at the gastroesophageal junction may lead to epigastric pain and an acute abdomen. Fever occurs as a later sign for any location. When spontaneous rupture occurs, there is generally a history of vomiting followed by chest pain. Any time chest pain or the other symptoms mentioned above occur after instrumentation or vomiting, the diagnosis of esophageal perforation must be considered.

On physical examination the patient is generally in significant distress. Tachycardia is common. Assessment of the lung fields may reveal dullness in either base, signifying a pleural effusion. Crepitus, characteristic of subcutaneous emphysema, should be specifically sought and may be found in the neck or chest wall. There are physical findings typically associated with a pneumomediastinum. On chest auscultation, the examiner can appreciate a crackling sound very similar to a pericardial friction rub. This is known as Hamman's crunch.

What is the initial workup for a patient with a suspected esophageal perforation?

Unfortunately, the diagnosis of esophageal perforation or tear is often delayed for hours to days after presentation. Diagnosis most often relies on radiographic evidence. If a thoracic perforation is suspected, a posteroanterior and lateral chest radiograph can be used to detect cervical or mediastinal emphysema, pneumopericardium, or pleural effusion. The chest x-ray is often abnormal but may be nonspecific. A pneumoperitoneum can be seen in distal perforations, so it is important that a good quality upright film visualizes the diaphragm. If a pleural effusion is present, which is often the case, its location can be a guide to the location of the perforation. Pleural effusions develop rapidly in esophageal perforation; characteristically, mid-esophageal lesions create right-sided effusions and distal injuries go to the left. The accumulated fluid will demonstrate a high amylase content that is a diagnostic clue to its cause.

Meglumine diatrizoate, a water-soluble contrast medium, can be used to verify the presence of a perforation. Barium is avoided in favor of meglumine diatrizoate as the first test ordered because of the potential for severe inflammation if barium gets into the mediastinum. However, if the meglumine diatrizoate study is negative, a barium study should be ordered, as the heavier barium may demonstrate the tear. Contrast esophagograms have a false positive rate of 10%.

If the initial contrast swallowing study is negative, but the clinical suspicion remains, the test should be repeated several hours later. There have been reports of repeat studies demonstrating perforations not seen on initial studies, probably as a consequence of edema. Computed tomography of the chest may provide additional information such as fluid collections or air in soft tissue or the mediastinum.

What are the initial management and treatment options for esophageal perforation?

The main principle of initial management is to suspect esophageal perforation and aggressively pursue diagnostic confirmation in the appropriate setting. The patient must not have anything by mouth, so intravenous fluids are initiated as the diagnostic process begins. Broad-spectrum antibiotics should also be started at this point and thoracic surgery should be consulted as early as possible. The patient often requires monitoring in an intensive care unit due to advanced age or severe underlying medical problems.

After the diagnosis is confirmed, the most important decision is medical versus surgical therapy. Criteria for this decision are listed in the box below. Nonsurgical interventions have gained popularity because thoracotomy is associated with high morbidity and its results are frequently less desirable.

If the patient is medically treated, a special tube with multiple side holes is inserted to establish adequate drainage above and below the site of perforation. Broad-spectrum antibiotic therapy must cover anaerobes and both gram-negative and gram-positive aerobes.

Regardless of the treatment approach chosen, meeting the nutritional needs of these severely ill patients is very important and can be achieved by jejunal feedings or parenteral nutrition.

What are the short- and long-term outcomes of esophageal perforation?

Mortality is significant, often reflecting underlying medical conditions. A study from the 1990s quotes 30% to 40% mortality, but that figure is probably lower now. Hospital length of stay can be considerable; one analysis found an average of 26 days. Mortality and morbidity are probably directly related to how quickly the diagnosis is made and the treatment initiated, again underscoring the need to presume that chest pain after instrumentation is a perforation until it is proven otherwise.

Regarding the long-term outlook, development of an esophageal stricture and dysphagia after a perforation is not uncommon. Predictably, these patients are often reluctant to undergo endoscopy and dilation.

What are Mallory-Weiss tears?

The Mallory-Weiss tear is part of a specific clinical syndrome of upper gastrointestinal hemorrhage caused by a retching-induced injury of the distal esophagus or the esophagogastric junction. It is rarely associated with complete esophageal perforation. The tear goes into the highly vascular layers of the esophagus, which can result in substantial blood loss. Historically, there has been an association of alcohol abuse with this syndrome. The patient may or may not describe the classic history of vomiting and forceful retching followed by hematemesis. A Mallory-Weiss tear is a relatively common cause of upper gastrointestinal hemorrhage, probably accounting for 2% to 5% of all cases. Profuse bleeding is especially likely in patients with hematochezia or hypotension. Outcome may be poor in the presence of severe underlying disease, portal hypertension, or coagulopathy.

How is Mallory-Weiss syndrome managed?

An episode of gastrointestinal bleeding related to a Mallory-Weiss tear should be initially managed like any other cause of bleeding. In fact, since the diagnosis is not known until EGD has been performed, the initial management is identical. Stabilization of hemodynamic status is of paramount importance. Intravenous fluids, judicious use of blood transfusions, and intensive care unit observation for the unstable patient are appropriate. As in any gastrointestinal bleeding case, EGD is done when the patient is hemodynamically stable.

A Mallory-Weiss tear can be easily seen as the endoscope passes the distal esophagus. Active bleeding may be encountered and can be arrested by endoscopic injections of epinephrine. In addition, thermal treatment with a special probe can be utilized to try to achieve permanent hemostasis. The thin-walled esophagus must be approached more cautiously than more common bleeding sites like the duodenum and stomach. Most Mallory-Weiss tear bleeding is controlled easily and these tears can heal quickly. Rebleeding can occur in the early period.

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