Etiology and Management of Esophageal Perforation

Etiology

The first report of spontaneous esophageal perforation was attributed to the Dutch physician Hermann Boerhaave in 1724. He described the demise of Barron van Wassenaer, the Grand Admiral of the Dutch fleet, after an episode of self-induced vomiting following a large meal. Spontaneous esophageal perforation, which has come to be known as Boerhaave syndrome, is due to an abrupt increase in the intraluminal pressure of the esophagus during forceful emesis leading to full-thickness rupture of the esophageal wall. The perforation is generally toward the left and may involve the thoracic or abdominal portion of the esophagus.

Fortunately, Boerhaave syndrome remains relatively uncommon, accounting for only 15% of esophageal perforations. With the frequent utilization of flexible endoscopy and its adjuncts for esophageal diagnostics and therapeutics, the most common cause of esophageal perforation currently is iatrogenic injury following esophageal instrumentation, accounting for nearly 60% of all cases. Given the elective nature of most such interventions and the fact that the perforations are often both immediately recognized and occur with minimal initial contamination of surrounding tissues due to an empty stomach, the outcomes may be better than those following spontaneous perforation, where diagnosis may be delayed and extensive contamination may occur. Other contemporary etiologies of esophageal perforation include foreign body ingestion, blunt or penetrating trauma, iatrogenic operative injury, tumor, and tumor necrosis following cancer-related therapy, such as radiation or chemotherapy.

Presentation

Esophageal perforations are quite variable in their manifestations and presentations depending upon multiple factors, including:

• Etiology

• Size

• Location

• Associated esophageal pathology

• Time interval since perforation

• Extent of neck, mediastinal, pleural or abdominal contamination

• Patient comorbidities

The most common presenting symptom is chest pain, although complaints of odynophagia, neck or abdominal pain, dyspnea, crepitus, fevers, and chills may occur in addition or in isolation. Symptoms may be mild, particularly if the perforation is contained or of recent onset. Within the first 8 to 24 hours following injury, frank sepsis with tachycardia, hypotension, altered mental status and respiratory failure may develop, particularly if the contamination is extensive. Given the potentially serious consequences of diagnostic delays, a high index of suspicion for esophageal perforation is necessary in any patient presenting with moderate to severe upper gastrointestinal symptoms of recent onset, especially following vomiting or a history of recent esophageal instrumentation.

During the initial evaluation, a thorough history focused on preexisting dysphagia, heartburn, or regurgitation, as well as prior esophageal surgery or known esophageal disorders, is important because concomitant esophageal pathology may have contributed to the perforation or may influence the treatment paradigm.

Diagnosis

After a detailed clinical history and physical examination have been completed, the most useful and expeditious initial screening examination for evaluation of the patient with a suspected esophageal perforation is a plain upright chest radiograph. This quick, readily available, and inexpensive study may reveal a pleural effusion, pneumothorax, pneumoperitoneum, subcutaneous or mediastinal emphysema, or mediastinal widening suggestive of perforation. However, of importance, is the fact that a normal chest x-ray does not rule out the possibility of a leak, especially in the early time period after the suspected event, because it may be contained or inadequate time may have elapsed to allow for the development of a recognizable radiographic abnormality.

The most frequently used radiologic test specifically to assess for esophageal perforation has been the contrast esophagram ( Fig. 48.1 ). The study traditionally is commenced with a water-soluble contrast agent, such as diatrizoate meglumine and diatrizoate sodium solution (Gastrografin), due to a concern for exacerbating mediastinal, pleural, or abdominal contamination if barium were used and leaked. However, contrast esophagography requires an alert and cooperative patient who is able to swallow without aspirating. In the intubated patient, contrast can be administered via a nasoesophageal tube. A risk of water-soluble contrast is the potential to induce a severe chemical pneumonitis if aspirated into the lung parenchyma. Therefore judgment must be exercised in using this study for the elderly patient or others considered at high risk for aspiration, because a resultant pulmonary injury could be a catastrophic insult added to the morbidity of the perforation.

The exam typically provides a reliable assessment of esophageal anatomy, the location and size of a perforation, and the presence of significant coexisting esophageal pathology, such as an esophageal stricture, malignancy, diverticulum, or motility disorder. In addition, a determination can be made as to whether the perforation is contained or freely leaking into the mediastinum, pleural spaces, abdomen, or neck, as well as the size of associated fluid collections. A negative study with water-soluble contrast should be followed by one using thin barium to increase the sensitivity of the examination. Films also should be taken with the patient in both the left lateral and right lateral decubitus positions because proper positioning also may increase the sensitivity of the study in detecting a leak. However, a negative esophagram does not exclude a perforation because false-negative rates run in the range of 10% to 38%.

Computed tomography (CT) has proven to be an extremely useful diagnostic modality for assessing esophageal perforations and guiding their management ( Fig. 48.2 ). Findings on CT suggestive of perforation include pneumomediastinum, pneumoperitoneum, subcutaneous emphysema, mediastinal fluid or inflammation, pleural effusion, or abdominal abscess. Importantly, CT provides accurate information regarding the location and size of any extraesophageal fluid collections in need of operative intervention or percutaneous drainage.

The finding of frank leakage of oral contrast is not sensitive and should not be the sole criterion to prove or disprove the diagnosis.

Flexible fiberoptic upper endoscopy serves a critical role in the assessment of a wide array of esophageal pathology, including perforations ( Fig. 48.3 ). Not only does endoscopic assessment facilitate the determination of the location and size of a mucosal injury, it also allows identification of concomitant mucosal ischemia or ulceration, as well as more chronic or subacute pathology such as a stricture, diverticulum, or malignancy. Some perforations are subtle and do not demonstrate an obvious mucosal tear but rather only an ecchymotic or slightly disrupted mucosa that flutters with insufflation. Although concern may exist about the safety of flexible endoscopy in the setting of acute perforation, given the need for insufflation and the risk of inducing a tension pneumothorax or exacerbating pneumoperitoneum, the examination typically can be completed safely in experienced hands and with appropriate attention to detail. Insufflation should be kept to a minimum, and consideration should be given to placement of a chest tube prior to the procedure if concern exists about the consequences of a pneumothorax.

No single diagnostic study is absolutely reliable in the evaluation of esophageal perforation. Esophagography with water-soluble contrast agents is limited by its false negative rate and risk of aspiration pneumonitis, as well as by its relative insensitivity in assessing mucosal pathology. In addition, leaked barium risks exacerbating mediastinal, pleural, or abdominal sepsis. CT lacks sensitivity in locating the exact site of a perforation and detecting concomitant esophageal pathology. Endoscopy is invasive, requires sedation, risks exacerbating pneumothorax or pneumoperitoneum, and does not facilitate a determination of the extent of extraesophageal contamination.

As a result of the limitations of each of these diagnostic modalities, considerable judgment is required on the part of the managing physician to determine the sequence and optimal utilization of studies, taking into consideration the clinical suspicion for a leak, the information desired, patient comorbidities and performance status, and possible therapeutic alternatives. A point worthy of emphasis is that the location of a perforation and extent of contamination must be determined prior to surgical intervention because the location of incisions and the types of procedures chosen are dependent on the findings.

Treatment