Instrumental injuries occur during diagnostic or therapeutic procedures. The incidence, at about 0.4%, is low in both flexible and rigid esophagoscopy.¹²⁷ The naturally occurring narrowing sites of the thoracic esophagus are the more common areas of perforation: at the level of the aortic arch, the left mainstem bronchus, and the distal esophagus as it courses through the diaphragm to join the stomach. Esophageal biopsy or therapeutic dilation may lead to rupture. The reported incidence of perforation following pneumatic dilation for achalasia ranges from 1.4% to 4.9%.^12,37 These injuries, however, constitute up to 20% of esophageal perforations in several series.⁵⁴ Rupture may also occur with improper placement and inflation of the gastric balloon of a Sengstaken–Blakemore tube to control bleeding esophageal varices.

Other causes of perforation include the palliative placement of an indwelling tube for malignant strictures and endoscopic injection sclerotherapy for esophageal varices¹⁰⁷ or a Mallory–Weiss laceration, which has been described to evolve to gangrene and perforation.¹⁰² It has been reported that small pleural effusions complicate sclerotherapy in about 50% of the cases without perforation.³³ However, if the effusions persist or occupy more than 25% of the hemithorax, a thoracentesis is indicated to rule out empyema or esophageal perforation, as suggested by a high
pleural fluid amylase level.⁶⁸ When either is present, a contrast swallow should be done.

Transesophageal echocardiography (TEE) during cardiac operations is another cause of perforation. It might be more common than originally thought, being reported at 0.18%, but it is associated with a low mortality rate of 0.0098%.²⁹ Fatal outcomes are mainly due to delay in diagnosis. How TEE causes injury is still unknown, but it has been suggested to result from high intraluminal pressure where the probe sits, coupled with an area that has been fixed to surrounding tissues by scarring, be it from previous infections, irradiation, or chronic ischemia. On the other hand, thermal or pressure injuries from the TEE probe have not been reproduced in animal and human models,¹¹⁷ thus adding to the uncertainty of the etiology. However, since its description in 1991,²⁹ multiple reports have been published confirming its existence.^19,59,73,93 Regardless of the mechanism, one should consider the possibility of significant injury in performing TEE.

Particularly vulnerable to the ingestion of foreign bodies are children, individuals with psychiatric disorders, and those who wear dentures.⁵ Most commonly, injuries occur in the cervical esophagus, but any point of normal narrowing in the thoracic segment or at a diseased segment may be the site of perforation. All varieties of objects cause perforation; they are limited only by the imagination of the ingestor. Of note, though, is the fact that fish bones usually do not cause esophageal injury because they lodge in the hypopharynx. In the pediatric population, the most frequent foreign bodies ingested are coins (57%), button batteries (21%), sharp objects (12%) and chicken bones (3%).⁶⁹

Li and associates⁶⁷ reported their experience with 988 patients and 1,090 foreign bodies in a 25-year period. The age range was 1 day to 96 years. These investigators found objects lodged in the pharynx in 1.1%, the esophagus in 52.9%, the stomach in 40.4%, the duodenum in 4.5% and at a surgical anastomosis in 0.9%.

In some instances, sharp foreign bodies lacerate the wall partially or completely; a perforation can also occur spontaneously or during the extraction of the foreign body. Although acute illness is possible, an indolent course with late abscess formation or pyopneumothorax is more common (Fig. 146-3), particularly if the foreign body ingestion goes unrecognized until it becomes symptomatic.

The diagnosis may be suggested by the history and physical examination. Conventional radiographic examinations may reveal the culprit, but frequently studies are negative, owing to the low density of many of the foreign bodies. The use of multislice CT imaging will improve the diagnostic yield. Other radiographic features depend on the extent of the inflammatory process; for example, an unexplained hydropneumothorax will prove to be a pyogenic process on thoracentesis.

Endoscopy with foreign body removal is the treatment of choice and was successful in over 94% of the cases in Li and colleagues’⁶⁷ series. Appropriate drainage of the involved area is also part of the integral management. Esophagotomy is indicated when endoscopic retrieval fails, most commonly due to dentures, iron pieces, and complex bodies. In the neck, this procedure is accomplished through a cervical mediastinotomy, whereas in the chest, either a posterior thoracotomy or thoracoscopy is used, depending on the extent of the disease process. The morbidity and mortality of foreign body ingestion vary with the time of recognition and the appropriateness of the therapy, with a reported survival of 94% to 100% in this group of patients.^15,67

A rapid increase of the intraluminal pressure within the esophagus may result in partial or complete rupture of the esophageal wall. This rupture is usually the result of internal changes in pressure accompanying emesis, but it may also occur with significant Valsalva maneuver, blunt abdominal trauma, and deceleration injuries.

Rarely, it may result from compressed air. There are reports of accidental pneumatic ruptures of the esophagus.^43,66 Common sources of the barotrauma were accidents involving compressed-air hoses and tanks or explosion of an inflated tire when a young child bit a bulge on an inner tube. In some instances perforation occurred in children who were playing with carbonated beverages.²⁶

Hermann Boerhaave is credited with the first description of an esophageal rupture in 1724. He found it during the autopsy of Baron von Wassenaer, Grand Admiral of the Dutch Fleet, who indulged himself excessively with food and drink only to then induce emesis by taking an ipecac-like drug; he died as a result. It was not until 1955 that Derbes and Mitchell translated and published Boerhaave’s findings, which then became
widely known.³⁰ Meyer was the first to diagnose an antemortem Boerhaave’s syndrome in 1852, at that time a fatal condition. Successful treatment was finally reported in 1947, almost simultaneously, by Barrett,⁸ Frink,⁴¹ and Olsen and Clagett.⁸⁵

The etiology of Boerhaave’s syndrome is a rapid increase of intraluminal pressure of the esophagus that occurs during emesis. This creates a high pressure that is forced through a patent lower esophageal sphincter and against a closed upper esophageal sphincter—i.e., the cricopharyngeus muscle. Hardy and Wallace⁵⁰ in 1977 noted that a force of 0.5 to 1.5 kg was necessary to rupture a normal esophagus, but that less pressure is needed when esophageal disease is present. Most often, the full-thickness rupture is located in the distal portion of the intrathoracic esophagus as it lies exposed anterior and to the left of the aorta (the distal 6 to 8 cm). This particular weak area could be the result of the distribution pattern of the smooth muscle layer. The longitudinal fibers taper out at that point as they pass onto the stomach wall. Other hypotheses presented in previous editions of this textbook suggest that the rupture occurs there because of the lack of support from adjacent structures.⁴⁷

A partial-thickness tear may occur. In that situation, it is rare to find a significant submucosal dissection.¹³ The partial laceration may extend into the proximal stomach and cause major bleeding, as first described by Mallory and Weiss in 1929.⁷² The main problem is control of the bleeding. If expectant and supportive management or endoscopic sclerosing injections fail, surgical intervention is required. The operative intervention is typically completed through an abdominal approach, making a gastrotomy to access and suture-ligate the bleeding points.

Massive bleeding with complete rupture of the esophageal wall is rare. Classically, patients with so-called spontaneous rupture of the esophagus present with severe chest pain and dyspnea after an episode of emesis. Pain may radiate to the shoulders, but typically patients complain of epigastric pain only. Marked thirst is occasionally noted. In many patients, however, the history is unobtainable owing to mental status changes (intoxication or shock); hence the diagnosis relies on the physical examination, radiographic findings, and at times thoracentesis.

The physical findings vary with the duration of the disease and the involvement of either pleural space. Subcutaneous emphysema is present in most patients, and cyanosis may be evident. The abdominal findings may vary from epigastric discomfort to an overt acute abdomen. Radiographic findings of the chest and abdomen that suggest the diagnosis are pneumomediastinum, pleural effusion(s), hydropneumothorax, and pneumoperitoneum (rare). The “V sign” is a patchy, irregular density behind the left cardiac silhouette seen early on in the process.⁷⁹ CT with contrast may localize the injury. It is especially helpful when the clinical features are obscure (Fig. 146-4). A thoracentesis may be diagnostic in the setting of a hydropneumothorax without suggestive history and physical findings. In adults, a pyopneumothorax confirms the diagnosis of a perforated esophagus.

Inflammatory diseases of the esophagus are mostly prevalent in immunocompromised patients, as discussed in Chapter 148. Some of them may develop invasive infections, necrotizing esophagitis, perforations, or tracheal/bronchoesophageal fistulas. Therefore every immunosuppressed individual (those undergoing transplantation regimens or chemotherapy and patients with AIDS, among others) should be considered at high risk to present such complications. Offending organisms include drug-resistant bacteria as well as fungal, viral, and mycobacterial organisms.

Gaissert and colleagues⁴¹ followed four patients over a 7-year period and reviewed the literature identifying 21 cases with severe esophageal infections; 10 patients were treated conservatively, with only 1 survival (90% mortality), while 11 patients were managed surgically (ranging from salivary diversion to esophagectomies), with 8 survivals (27% mortality). This difference in mortality, as emphasized by the authors, was mainly due to sepsis. Overall mortality was 48%; however, there were several patients whose outcome was not reported. Nonetheless, it is clear that invasive infections pose a great challenge and that an especially aggressive management might be needed to prevent or control sepsis.

Tuberculous infection of the esophagus is rare and is usually the result of extension from the lung or involved mediastinal nodes. In the immunocompromised patient, however, the infection may be primary in nature. Ulceration, fistulization, or even perforation may occur. According to Grubbs and coworkers,⁴⁶ perforation per se is rare, further evidenced by these investigators being able to find only four such cases in the literature. An adequate course of antituberculosis medications and subsequent surgical closure are indicated, although, in the case of Grubbs et al., the perforation healed without surgical intervention.

Perforation of the thoracic esophagus leads initially to contamination of the visceral compartment of the mediastinum; it may involve one or both pleural spaces as well. The left pleural space is usually involved in the most distal perforations, while the right side is affected when the perforation is proximal. Because of the necrotizing process that accompanies these injuries, the infection may spread and involve other structures in or adjacent to the mediastinum; the pericardium or even the central nervous system (CNS) may become involved.

The occurrence of CNS involvement is quite rare and is seen most often with perforations of the proximal thoracic esophagus. In those cases, a localized mediastinal abscess fistulizes into the esophageal–subarachnoid space, with subsequent infection and air in the thecal space that may migrate into the meninges of the cranium (Fig. 146-5). CT and magnetic resonance imaging (MRI) demonstrate air in the subarachnoid space. Esophageal–subarachnoid fistulas have been described since 1975 by multiple authors, in some cases describing spontaneous esophageal perforations presenting as meningitis.^{18,28,58,61,98} In other cases, the ruptures resulted from dilation of lye strictures, rupture of a carcinoma of the esophagus, blunt trauma, and placement of an esophageal stent for a benign stricture.

Injuries to the thoracic esophagus are associated with pain, fever, dysphagia, and, frequently, respiratory distress. The pain may be thoracic, precordial, or even epigastric, and radiation of the pain occurs to the intrascapular region. With contamination of the pleural space, the patient may experience severe unilateral pleural pain. Tachycardia is frequently disproportionate to the degree of fever. The patient may be able to localize the pain to the vicinity of perforation. Excessive thirst may be present. Respiratory distress may occur, depending on the degree of
pleural contamination. Physical examination findings vary from subclinical to overtly septic. Focused examination should aim at identifying subcutaneous air initially in the neck area, and signs suggestive of a hydropneumothorax or sepsis should be elicited.

Conventional radiographic examination of the chest may show a widened mediastinal silhouette, mediastinal air or fluid or both, air or fluid or both in either pleural space, or, rarely, pneumopericardium. Radiologic examination may be normal in 12% to 33% of the cases.⁴⁹

Demonstration of the site of the leak may be accomplished by fluoroscopy and ingestion of contrast medium (Fig. 146-6). A water-soluble medium can be used, but better results are obtained with water-suspended barium sulfate. Practice preferences vary; thus the diagnostic approach to the patient with suspected esophageal leak should be individualized; water-soluble contrast can be followed, if negative, by barium contrast, enabling the physician to detect smaller injuries. Some prefer water-soluble contrast using barium from the beginning, the argument being that if there is indeed extravasation of the medium, surgical intervention is immediately indicated, not giving enough time to the extravasated barium to complicate the surgical procedure. In addition, if contrast aspiration is to occur, particularly in the obtunded patient, the pulmonary inflammatory reaction may be severe with water-soluble contrast.

CT imaging may be helpful when the other diagnostic studies are negative. In addition, flexible esophagoscopy can be a valuable adjunct in accurately localizing and directly visualizing the site of perforation.^56,76

Sometimes the diagnosis is obscure. In those cases, thoracentesis may be beneficial. The fluid analysis demonstrates an exudate with a high amylase. The most useful test is microscopic evaluation. If vegetable fibers are seen, it is diagnostic of gastrointestinal contents.

The management of intrathoracic perforations is based on four principles⁷⁴: (a) elimination of the source of contamination, (b) wide surgical drainage, (c) broad-spectrum antibiotics, and (d) maintenance of adequate nutrition. Specific interventions are determined on the basis of the status of the esophagus before and after the injury, whether the esophagus is normal or diseased, whether the lesion is benign or malignant, the extent to which the mediastinum or the pleural space is contaminated, and the patient’s nutritional status.

At present, most surgeons recommend immediate surgical intervention except under unusual circumstances. The urgency to provide definitive treatment less than 24 hours postinjury prevails. However, the time of recognition and institution of therapy seems to be of less importance than formerly believed. Patients are frequently partially treated with antibiotics and/or drained with a chest catheter or nasogastric tube for other presumed diagnoses and survive the formerly fatal mediastinitis to develop a subacute perforation. Jougon and collaborators⁵⁷ published a successful primary repair strategy, regardless of the time interval between injury and surgical intervention, with 64% of their patients having surgical intervention >24 hours after the injury occurred.

Contained perforations are managed expectantly. Cameron²² advanced the concept for perforations where ima- ging studies show that the contrast material drains back into the esophageal lumen in a setting of minimal or no clinical signs of sepsis. Suggested criteria for nonsurgical treatment stipulate that the perforation site is not proximal to a high-grade stenosis, the patient has minor symptoms without sepsis or hemodynamic compromise, contrast studies show prompt drainage back into the esophageal lumen, there is no pleural contamination, and the patient has had no food intake after the episode of perforation.^93,103 The patients are admitted and monitored, nasogastric suction is initiated, appropriate antibiotics are administered, and parenteral nutrition or postpyloric feeding is established.

For the perforations that do not fit the conservative management approach, multiple surgical options are available (Table 146-3). In most cases, primary closure, usually with buttressing of the suture line, is recommended^{84,93,125,126} regardless of the amount of time that has elapsed since the occurrence of the esophageal perforation.¹²² The actual technique of closure with or without the use of a buttress of tissue to cover the closure varies from group to group. The techniques used by the University of Michigan and the Massachusetts General Hospital staffs are seen in Figure 146-7. Correction of any distal obstructing process (functional or organic) is essential for success, as is adequate mediastinal and pleural drainage.

In the case of perforation after dilation for achalasia, the surgeon should mobilize the lower esophagus and perform a modified Heller myotomy (see Chapter 135) opposite the perforation site. An alternative approach consists of esophageal wound debridement, exposure of the entire tear, and secure closure of the mucosal layer only. An intercostal muscle flap is sutured to the edges of the esophageal muscle layer (using 4-0 silk sutures
with a horizontal mattress technique) to ensure a tight closure. This technique maintains the myotomy already created by the balloon dilation.¹¹⁶ To complete the procedure, regardless of the technique used, drainage of the thorax is established, and a draining gastrostomy as well as feeding jejunostomy should be performed.