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Perforation of the esophagus

AARON M. CHENG, DOUGLAS E. WOOD AND CARLOS A. PELLEGRINI

HISTORY

The first reported spontaneous rupture of the esophagus was described by Herman Boerhaave, a “leading physician of the age,” in early mid-eighteenth century Germany. His patient, the Baron de Wassenaer, consumed a large meal and subsequently “strove to excite vomiting by tickling his fauces,” resulting in a postemetic esophageal perforation. Not unexpectedly, the baron died within 24 hours, and his postmortem revealed a linear esophageal perforation that had ruptured into both pleural spaces. A number of similar reports followed Boerhaave’s seminal description. Attempted suture repair was only contemplated in the mid-twentieth century, and initial attempts in the United States (1944) and England (1946) were unsuccessful. Successful suture repair was performed by Norman Barrett in 1947, the year following his insightful and comprehensive review of the subject. Although postemetic perforation is no longer the principal etiology for esophageal perforation, it is notable that successful surgical repair followed two centuries later.

PRINCIPLES AND JUSTIFICATION

Despite improvements in the management of critically ill patients, perforation of the esophagus can be fatal unless diagnosed promptly and treated effectively. Most esophageal perforations today are caused by instrumentation (usually therapeutic) of the esophagus, especially during forced dilatation of an esophageal stricture. Injury to the cervical esophagus can occur during endoscopy and endotracheal intubation, and, together, these iatrogenic events account for 60% of cervical perforations. External trauma due to stab or gunshot wounds is the second most common cause of cervical and thoracic perforations of the esophagus. Other etiologies include the “spontaneous” or emetogenic disruption of the esophagus (Boerhaave’s syndrome), perforation of an esophageal cancer, sloughing of the esophageal wall after injection sclerotherapy or caustic injury, foreign body impaction, surgical injury, and infectious processes.

A very important consideration in the management of esophageal perforation is the evaluation and management of underlying esophageal pathology. It is critical to obtain an accurate history that may identify symptoms or signs of preexisting abnormalities, such as esophageal stricture or cancer, which may require different or concomitant treatment.

Historically, mortality associated with esophageal perforation has been reported as high as 80%. However, with modern improvements in diagnosis and management, the current overall mortality rate for patients who develop esophageal perforation is 18%. The risk of dying from an esophageal perforation varies markedly with the location and extent of the perforation, the time elapsed prior to treatment, the age and general condition of the patient, and the presence of intrinsic esophageal disease. Key to understanding the pathophysiology of esophageal perforation is the recognition that it causes a rapidly evolving infection of the mediastinum, with substantial spread and necrosis of poorly vascularized mediastinal fat tissue. In addition, and particularly pertinent to treatment options, there is prompt deterioration of the esophageal wall at the site of the rupture. Thus, ideally, an esophageal perforation should be treated within 12 hours of its occurrence—attempts at primary repair after the first 24-48 hours are more challenging.

PREOPERATIVE ASSESSMENT

Clinical diagnosis

The most common symptom is pain. When the perforation is iatrogenic, patients may attest to pain symptoms during, or immediately after, completion of the instrumentation. Pain is constant, most often radiates to the back, and may be felt in the upper abdomen and chest, particularly when the perforation involves the thoracic esophagus. Perforations in the neck often present with pain on neck flexion or manipulation of the thyroid cartilage. Subcutaneous emphysema and crepitation are often evident following cervical perforation and are present in approximately 20% of thoracic esophageal perforations. Many patients also complain of dysphagia, odynophagia, and profuse salivation. Fever and leukocytosis are common within the first 4-6 hours after perforation and some patients rapidly develop features of systemic shock (hypotension, tachycardia, sweating). Airway compromise is uncommon following injury to the esophagus, but may be of concern in the case of a cervical perforation.

The commonly quoted triad of symptoms described by Mackler—thoracic pain, followed by vomiting associated with cervical emphysema—is only present in approximately 40% of patients. These findings are only relevant to postemetic esophageal perforation.

Radiological diagnosis

Chest and abdominal radiography are routinely obtained when the diagnosis of esophageal perforation is suspected but are usually insufficient to confirm the diagnosis, in particular the location of the perforation. Nonetheless, a perforation may be suspected from plain films, as free air may dissect adjacent tissues, creating subcutaneous emphysema, pneumomediastinum, pneumoperitoneum, or even pneumothorax. Cervical perforation may present with subtle findings such as air in fascial planes, widening of the retroesophageal space, and loss of cervical lordosis. Thoracic perforations often demonstrate widened mediastinal silhouette and air-fluid levels within the mediastinal space. A left pleural effusion is also an indirect sign of esophageal perforation. In the experience of the authors and of others, however, these signs are present only in approximately 30% of cases.

A contrast esophagogram will reliably confirm an esophageal perforation and is the most useful diagnostic test (see Figure 37.1). The esophagogram will define the site and extent of the perforation, the amount of extravasation, the communication with the pleural or peritoneal cavity, and the presence of distal obstruction. Thus, a contrast study is not only important for the diagnosis of esophageal perforation but is essential to correct planning of surgery.

The authors prefer to use diatrizoate meglumine 66% and sodium diatrizoate 10% (Gastrografin), as the contrast medium in the initial investigation. This water-soluble material is rapidly absorbed from the gastrointestinal tract and from the pleural or peritoneal cavity if extravasated. It allows even small amounts of extravasation to be detected by a follow-up computed tomography (CT) scan. Gastrografin may be more caustic to the airway if aspirated and generates a false negative study in approximately 20% of cervical esophageal perforations and 10% of intrathoracic perforations. In cases where aspiration is a concern or a false negative is suspected, a thin barium study may be more informative.

A CT scan is a useful adjunct to an esophagogram, predominantly to identify the extent of pleural and mediastinal fluid and contamination that may require debridement and drainage (see Figure 37.2 ). However, CT scanning is neither a sufficient nor appropriate modality for guiding clinical management of an esophageal perforation, and is definitely not an alternative or replacement for an esophagogram. Often the CT scan is the first study that is performed, and may reveal subtle signs of perforation. In these cases, the CT scan should be followed by an appropriate contrast study of the esophagus.

A CT scan provides invaluable information on patients who present with delayed perforations. These are patients who survived the initial insult and who have developed an abscess that effectively contains the perforation and prevents further mediastinal or pleural soiling. To plan adequate drainage, one must define the site, the extent, and the relation of the abscess to adjacent structures, and CT scanning is the test of choice.

Endoscopic diagnosis

Endoscopic examination often adds little information to that obtained from a high-quality contrast esophagogram. In some patients, however, particularly those suffering from foreign body perforation or penetrating trauma, endoscopy may help to identify and characterize the injury (see Figure 37.3). For example, a patient who has stab or gunshot wounds may also have a perforated esophagus, and, due to other concomitant injuries, the patient may be hemodynamically unstable to undergo a contrast esophagogram. The overall sensitivity of esophagoscopy in detecting subtle injuries is inferior to a contrast study, and, not unexpectedly, it is operator dependent. Therefore, we recommend that in trauma patients with penetrating injuries where there is high suspicion for esophageal injury, a contrast esophagogram be performed once the patient is stabilized, even if endoscopy is unrevealing.

Endoscopic examination may be helpful in those patients that have intrinsic esophageal disease and esophageal perforation. In these cases, discovery of a cancer, stricture, or other underlying esophageal pathology will affect planning of the operation. For this reason, the authors routinely perform endoscopy immediately following induction of anesthesia in all patients with symptoms or signs of preexisting esophageal pathology who are undergoing an operation for esophageal perforation.

INITIAL MANAGEMENT

The initial management of an esophageal perforation involves several steps.

Aggressive resuscitation

These patients suffer rapid dehydration and overwhelming contamination if there is free perforation involving the mediastinum and pleural space. Large bore intravenous access or central access is warranted. A secure airway is also paramount in patients requiring large volume resuscitations and who have compromised ventilation. Early pleural drainage may be required to evacuate a pneumothorax or drain a large pleural fluid collection.

Antibiotic administration

As soon as a perforation is suspected, the patient should be started on broad-spectrum antibiotics directed against oral flora to adequately cover aerobic Gram-positive and Gram-negative bacteria, as well as anaerobic microorganisms. Antifungal coverage is also routinely administered at our institution because of the high incidence of pathologic oral fungal flora.

Assessment of the perforation

The surgeon should ask the following before deciding on the approach and treatment strategy:

• Is the perforation contained or free? Contained esophageal perforations are often incomplete intraluminal injuries without true mediastinal contamination, or limited to soilage of the tissue immediately adjacent to the esophagus. Free perforations with extravasation of esophageal contents into the neck, the pleural cavity, or the peritoneal space are the most common type of perforations and those that require intervention.
  
• How much time has elapsed since the perforation occurred?
  
• What is the location and the extent of the perforation?
  
• What is the etiology of the perforation? Is the perforation iatrogenic, spontaneous, traumatic, etc.?
  
• Is there preexisting underlying esophageal disease?
  
• Is there an obstruction distal to the perforation site?
  
• What is the general health and functional status of the patient? What is the patient’s current hemodynamic stability?

Traditional management of esophageal perforations relied on open operative repair; however, the contemporary role of esophageal stenting has gained prominence in the management paradigm for select esophageal perforations and will be discussed in the section on “Alternative approaches.”

Contained perforations

Contained perforations, which may be partial thickness or localized injuries without extensive contamination, may be treated without surgery, provided that: the perforation is small, the contrast material flows immediately back into the esophagus and distally into the stomach, no underlying esophageal disease is present distal to the perforation (i.e., stricture), and the clinical manifestations are minimal (i.e., low-grade fever, minimal pain, etc.). Such nonoperative management is the exception, not the rule, when treating esophageal perforation. In fact, in some instances, the surgeon ought to look “beyond the horizon” in making the decision, as may be the case for a patient who has achalasia and has a “minimal” perforation. It is unlikely that anyone will attempt subsequent dilatation of this achalasia; thus, operating early, closing the perforation, and performing a myotomy will address both aspects of the problem: the underlying disease and the complication of the dilation.

One perforation that may be treated successfully with conservative (nonoperative) management is that which occurs a few weeks after injection sclerotherapy. The inflammatory reaction caused by the sclerosing agent adheres the periesophageal tissues to the wall of the esophagus, effectively blocking the perforation and decreasing the chance of mediastinal spread of infection. Furthermore, the underlying general state of the patient (cirrhosis) and the esophagus (varices) would make any attempt at open repair very difficult. When this strategy is followed, the patient should be given enteral or parenteral nutrition, and broad-spectrum antibiotics should be administered for 7-10 days. The most important aspect of management is close clinical observation, as any clinical deterioration or recurrence of symptoms (e.g., pain) warrants reevaluation and consideration of surgical intervention. The esophagus should be evaluated periodically with contrast radiography and CT scanning to monitor the progress. Any evidence of spread of infection or lack of adequate response to this treatment should elicit an immediate change of treatment, prompting more aggressive intervention.

Free perforations

Free perforations are much more common than contained perforations, and almost always require intervention, regardless of location or size. Traditionally, open surgical repair was the mainstay of treatment, but, more recently, esophageal stenting has become an important alternative treatment modality. Regardless of which interventional approach is undertaken, the principles of treating free perforations are control/drainage of the esophageal leak, eradication of mediastinal and pleural sepsis, and reexpansion of the lung.

Time elapsed since perforation

The time elapsed since the perforation determines, to some extent, the intervention to be used. Patients who suffered their perforation 24-72 hours previously should undergo an exploration of the area, and, if possible, the perforation should be closed with buttressing. The mediastinal and pleural spaces should be debrided and drained and the lung decorticated. Patients who present several days after perforation are likely to have a periesophageal abscess. In these cases, primary closure of the perforation may no longer be possible; if mediastinal and pleural sepsis is controlled and the lung is reexpanded, interventional radiological techniques, plus or minus esophageal stenting, may be used to drain the infected areas.

Location and extent of perforation

Injuries to the esophagus above the thoracic inlet should be treated by neck incision on the side of the extravasation, or on the left side (the esophagus is easier to access from the left). Occasionally, cervical esophageal perforations will extend into the mediastinum or right chest and require open mediastinal and pleural debridement through a right fifth intercostal posterolateral thoracotomy. Nonoperative treatment of perforations in the neck has been advocated, on the basis that most heal by apposition of adjacent tissue (no “real” space is present around the esophagus in the neck). The authors believe that early closure or drainage of these injuries accelerates recovery and allows for treatment of associated injuries, which are common when external trauma is the cause of the perforation. If the perforation has occurred in a Zenker’s diverticulum, resection of the diverticulum, and a concomitant cricopharyngeal myotomy are recommended. Most other esophageal perforations should be approached through a thoracotomy. Upper thoracic and mid-esophageal injuries are best approached by thoracotomy in the right posterolateral fourth to sixth intercostal space. Most distal lesions should be approached through the left posterolateral seventh or eighth intercostal space, even if the extravasation is in the abdomen. Occasionally bilateral thoracotomies will be required to decorticate both pleural spaces.

Presence of underlying esophageal disease

Underlying esophageal disease plays a critical role in determining the kind of procedure to be performed. As perforation occurs most commonly during dilatation of strictures, and because the mechanism of injury is such that the wall of the esophagus is injured at or just above the stricture, therapy should be planned accordingly. If the stricture is chronic, fibrotic, and recalcitrant to previous dilations, the best treatment is to resect the stricture and perforated area, and immediately reconstruct the gastrointestinal tract. If the perforation is caused by dilatation for achalasia, closure of the perforation and a Heller myotomy on the other side of the esophagus are recommended. Likewise, if an early stage or locally advanced esophageal cancer is perforated and promptly identified, immediate esophagectomy and primary reconstruction should be considered if the patient is a candidate for esophagectomy. Whatever the choice, the key surgical principle is to never close primarily a perforation above an esophageal obstruction.

General health and condition of the patient

Unfortunately, early discovery of a free perforation mandates treatment intervention, regardless of the fitness of the patient. The intervention may be operative surgical repair or endoscopic stenting, dependent on the extent of injury, timing of the procedure, and experience of the managing team.

OPERATIVE PROCEDURES

Repair of perforation of the unobstructed esophagus

Most spontaneous and instrumental perforations occur in the distal esophagus. The best way to approach these lesions is by thoracotomy through the left posterolateral seventh or eighth intercostal space (see Figure 37.4). We routinely harvest an intercostal muscle flap on entry into the chest. The pedicle is then wrapped in a warm saline sponge and left in the chest until it is required for buttress of the perforation. The parietal pleura overlying the esophagus should be opened at a site near the perforation. Occasionally, the esophageal lesion has lacerated the pleura, and the site of perforation is obvious from the beginning. If the perforation is not evident, the chest can be filled with saline while the anesthetist blows air through the esophageal lumen. Bubbles of air will appear at the site of perforation. Alternatively, an endoscope can be passed transorally and an examination performed while the patient is anesthetized and the chest is open. Most often, the perforation is easily identified by surrounding inflammation as well as the presence of Gastrografin or thin barium in the esophageal lumen.

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