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Management of Esophageal Perforations and Leaks
The incidence of esophageal perforations is on the rise. Iatrogenic causes remain the most common and continue to increase in an era of frequent use of endoscopy for diagnostic and therapeutic procedures. Despite many advances in care, the mortality rate for an esophageal perforation remains high, with some series citing 12% to 50%.
The esophagus passes through the neck, chest, and abdomen, so surgeons managing perforations must be experienced with the unique anatomic considerations for approaching a perforation in any of these levels/locations. Many factors must be considered when managing these patients, including acuity of presentation, contamination, size of leak, cause of leak, and comorbid conditions. Those caring for esophageal perforation must be experienced in endoscopic procedures, esophageal resection, and complex esophageal reconstruction. Many of the current techniques involve a hybrid approach of stenting with a muscle buttress.
Anatomic Considerations
The esophagus is a long muscular tube that begins at the pharynx and ends at the gastroesophageal junction past the crura of the diaphragm, thus passing through three anatomic fields. The esophagus lacks a serosal layer, making it more susceptible to leak and less forgiving with surgical repair. The inner circular and outer longitudinal muscular layers are often weakened by perforation and do not hold sutures well. Perforations are often underestimated as the infection spreads through the submucosal plane and is covered by muscular tissue.
The cause of perforation often dictates the location. Important anatomic landmarks to appreciate, as they are common sites of perforation, are described in Table 49.1 .
TABLE 49.1
Common Sites of Esophageal Perforations
| Anatomic Landmark | Description |
|---|---|
| Piriform sinus | This sinus or recess flanks the laryngeal orifice. Trumpet players, manual laborers, and singers are prone to develop piriform sinus perforations. These can often result in massive subcutaneous emphysema and can often be treated by merely placing the patient NPO. |
| Killian triangle | A triangular region of the cervical esophagus formed by the oblique fibers of the inferior constrictor muscles of the pharynx and the transverse fibers of the cricopharyngeus muscles. Pharyngoesophageal diverticula occur here, and inadvertent cannulation of the diverticulum can result in perforation. |
| Cricopharyngeus muscle | The upper esophageal sphincter is a high-pressure zone at the proximal esophagus extending approximately 3–4 cm. It is comprised of the cricopharyngeus as the major contributor along with the inferior pharyngeal constrictor. This represents one of the most common sites of perforation. Spinal instrumentation can often protrude anteriorly toward the esophagus complicating endoscopy. Perforations at this level are often treated with mere drainage as long as the mediastinum does not become contaminated. Diversion is difficult for perforations in this location. |
| Aortic arch/bronchus | The esophagus is in direct contact with the aortic arch and the bronchus, which both create a small indentation on esophagram. This angulation provides a potential location for food to become lodged. Unique problems specific to this location include complete vascular rings, a right-sided aortic arch, and the classic dysphagia lusoria (arteria lusoria), which is impairment in swallowing due to compression from an aberrant right subclavian artery. |
| Gastroesophageal junction | The lower esophageal sphincter is another location where iatrogenic injury often occurs; however, the most common etiology of perforation here remains Boerhaave's. This unfortunately can result in contamination of the mediastinum, bilateral pleural spaces, and the abdomen because of its location. |
NPO, Nil per os.
Etiology of Esophageal Perforations and Leaks
The most common cause of esophageal perforation remains iatrogenic, accounting for approximately 60% of all cases. Most iatrogenic perforations are related to endoscopy. The risk of perforation is related to the indication of procedure—diagnostic carries a risk of 0.6% versus 6% for more complex interventional procedures. Other less common causes include spontaneous perforation (15%), ingestion of foreign body (12%), trauma (9%), and malignancy (1%). Table 49.2 presents a comprehensive list of causes and clinical findings associated with esophageal perforations.
TABLE 49.2
Etiologies of Esophageal Perforations, Leaks and Strictures
| Type | Causes | Clinical Findings |
|---|---|---|
| Anatomic | External compression from an aberrant right subclavian artery (Kommerell diverticulum) Schatzki ring |
Dysphagia |
| Piriform sinus | Endoscopy, playing brass instruments, singing or yelling | Marked mediastinal and cervical subcutaneous emphysema |
| Anastomotic | Leakage at or near the site of a surgical anastomosis | History of surgically created esophageal anastomosis. May result in perforation, leak, or stricture. |
| Boerhaave's | Vomiting, straining, retching, weight lifting, hyperemesis, seizures causing a full-thickness tear at the gastroesophageal junction | Characteristic longitudinal tear on the left side of the esophagus, typically in the distal  segment Mucosal defect typically longer than muscular defect |
| Iatrogenic | Endoscopic: Ablation, dilation, sclerotherapy, EMR, instrumentation, POEM | Recent history of surgery or endoscopy |
| Surgical: Esophageal surgery, pulmonary decortication, spine surgery | May occur secondary to nonesophageal surgery secondary to instrumentation | |
| Traumatic | Penetrating or blunt trauma to neck or torso | Strong association with neck hyperextension |
| Cancer | Erosion of an esophageal tumor Extension of surrounding tumor through esophageal wall |
Gas near or abutting the tumor on imaging |
| Paraesophageal hernia | Incarceration with necrosis of the distal esophagus | Evidence of left pleural effusion or abdominal fluid on imaging studies |
| Foreign body | Ingestion of a substance (i.e., chicken bone) that becomes lodged Esophageal webs Eosinophilic esophagitis |
Upper esophageal impaction at the sphincter |
| Esophagitis | Inflammation and erosion of ulceration Peptic ulcers Zollinger–Ellison syndrome Barrett ulcer Infection ( Candida, herpes simplex, viruses, CMV) |
Immunocompromised patient |
| Ingestion | Ingestion of caustic substance Drug ingestion/impaction |
Acid or base (lye) Tetracycline Potassium Quinidine NSAIDs Sustained-release formulations |
CMV , Cytomegalovirus; EMR , endoscopic mucosal resection; NSAIDs , nonsteroidal antiinflammatory drugs; POEM , per oral endoscopic myotomy.
Originally described by Herman Boerhaave, his eponymous esophageal perforation called Boerhaave syndrome results from a sudden increase in intraesophageal pressure. He described his findings in a 1724 pamphlet detailing postmortem observations of Baron de Wassenaer, the Grand Admiral of Holland, who suffered a fatal esophageal rupture as a result of self-induced vomiting in an attempt to relieve discomfort following unrestrained consumption of food. Although most widely perceived to be associated with vomiting, spontaneous perforations may also be seen in situations of forceful valsalvae—weight lifting, child-birth, and defecation.
Esophageal disruption may occur in the setting of penetrating or, less frequently, blunt trauma. Gunshot wounds account for 75% of penetrating injury followed by stab wounds and other mechanisms. The majority of injuries involve the cervical esophagus. Unfortunately these injuries continue to carry a very high mortality with a large series published in 2013 by Patel et al. citing 44%.
Blunt trauma resulting in esophageal perforation is exceedingly rare with only about 100 cases reported in the literature. The mechanism of injury is debated and may be caused by anterior-posterior compression of the esophagus between the sternum and spine, severe hyperextension, increased intraluminal pressure, and chest compression with perforation occurring in a manner similar to Boerhaave's, or possibly ischemic injury from rapid deceleration with delayed perforation.
Ingestion of caustic materials may result in severe esophageal injury and in some cases perforation. The extent of injury is dependent on a variety of factors including type of material, amount consumed, and length of time it is in contact with tissues. Caustic materials are generally categorized as acidic or alkali. Acids are generally poor to taste and irritating, resulting in smaller volume ingestion. They cause a coagulative necrosis, form an eschar, and have a lesser incidence of esophageal perforation. Lye or alkali liquids are tasteless and dense, and they cause liquefactive-type necrosis and have a propensity to full-thickness progression of esophageal injury.
Infection is another important cause of esophageal perforation, particularly in immunocompromised patients. Eosinophilic esophagitis, which is characterized by inflammation, esophageal dysfunction, and eosinophil penetration into the esophageal wall, may result in spontaneous esophageal perforation as a complication of food impaction.
Many diseases may result in esophageal stricture formation. Strictures may be grouped into several categories: intrinsic disease causing narrowing or extrinsic disease causing compression or invasion. Peptic strictures secondary to acid exposure are the most common cause of benign, intrinsic narrowing of the esophagus accounting for 70% to 75% of cases. Disease processes that result in inflammation or perforation and leak may also result in stricture formation.
Patient Presentation
The presenting symptoms vary largely by the anatomic location of the esophageal injury. Signs and symptoms common to all sites include fever, tachycardia, tachypnea, pain, leukocytosis, and varying grades of shock. Mackler triad describes the classic presenting syndrome of spontaneous esophageal rupture of a middle-aged man who consumes excessive food and alcohol, has active vomiting and retching, and develops chest pain and subcutaneous emphysema. The Anderson triad includes subcutaneous emphysema, rapid respirations, and abdominal rigidity.
Patients presenting with a high esophageal perforation—in the neck or piriform sinus—may describe neck pain, a change in their voice (generally a more nasal sound secondary to inflammation of the vocal cords), dysphagia, hemoptysis, or crepitus (a crunching sound or sensation when pushing on the skin secondary to the accumulation of subcutaneous emphysema; Fig. 49.1 ).
Intrathoracic perforations often present with symptoms such as chest or back pain, dysphagia, dyspnea, bleeding, vomiting, or signs and symptoms of sepsis. Signs of an intrathoracic perforation include pleural effusion, pneumopericardium, pneumomediastinum, or pneumothorax.
Intraabdominal perforations are more likely to present with abdominal pain and distention. Signs of an abdominal perforation of the esophagus include pneumoperitoneum, or free fluid detected by either exam or imaging ( Fig. 49.2 ).
With an uncontained perforation, polymicrobial infection with bacteria such as Staphylococcus , Pseudomonas , Streptococcus , and Bacteroides typically occurs within the first 12 hours. Patients begin to develop tachycardia, fluid sequestration, fever, and a leukocytosis. In addition, immunocompromised patients may fail to have a classic presentation and often warrant a more aggressive imaging approach with subtle signs such as a mere tachycardia, denoting infection.
Evaluation
A high level of suspicion leading to early identification is essential in the management of esophageal perforations and leaks as timing of intervention correlates with outcome.
The work-up of any patient with suspected esophageal perforation begins with a detailed history and physical examination. Particular attention should be given to a history of instrumentation, trauma, food consumption, occupation, recent activity, and signs and symptoms of malignancy such as weight loss or dysphagia. In patients presenting with hemodynamic instability, this should be addressed immediately with placement of large bore IVs, fluid administration, and aggressive monitoring.
If esophageal perforation is suspected, an anteroposterior, lateral upright chest, and abdominal radiographs should be obtained promptly. Subcutaneous emphysema, pneumomediastinum, new effusion, pneumothorax, and pleural thickening are indicators of perforation. A plain radiograph may prove diagnostic in 80% of patients with suspected iatrogenic perforations. Radiographs are not only diagnostic but also assist in localizing the defect; midesophageal perforations manifest with right-sided efffusion, whereas distal esophageal injuries show left-sided effusion.
The gold standard diagnostic study remains a contrast swallow study with the treating surgeon present. The esophagram is performed fluoroscopically with the patient positioned obliquely in standing or semierect during swallowing of contrast. This facilitates the identification of subtle leaks ( Fig. 49.3 ). The false-negative rate of contrast radiography is about 10%. Consequently, we prefer to use angiography (Omnipaque) or low-osmolar water-soluble contrast solutions. Gastrografin has a higher false-negative rate—extravasating in only 50% to 80% of cases of esophageal perforation—and carries a risk of severe pneumonitis when aspirated. Barium has a higher diagnostic accuracy; however, it persists in the space after imaging and may complicate further imaging, making a closed perforation appear present after it has sealed.
Patients who are unable to swallow or who are intubated may alternatively have a computed tomography (CT) scan performed ( Fig. 49.4 ). CT is also helpful in cases of suspected perforation that are unable to be identified on upper gastrointestinal (GI) evaluation, and a nasogastric tube (NGT) should be placed under fluoroscopy to allow for contrast injection to better identify and delineate the leak. It is important to ensure that endotracheal tube cuff is inflated to prevent aspiration. CT scan is useful not only in identifying the site of leak, but also in delineating associated abscess of fluid collections that may require drainage.
Endoscopy is an important adjunct to imaging as it is diagnostic ( Fig. 49.5 ) and therapeutic and allows irrigation and drainage of large perforations. Endoscopy in the setting of perforation must be approached cautiously by an expert endoscopist, but skilled thoracic surgeons are often more comfortable performing thoracic irrigation in the setting of a chronic esophageal perforation and can often immediately follow with appropriate surgical intervention while under the same anesthesia. Patient should be intubated and under general anesthesia in the operating room. In this setting, pleural effusions may also be sampled to determine if they are transudative or exudative effusions that could require additional treatment. Endoscopy is being used increasingly as an effective method for the treatment of some patients with perforation.
Esophageal strictures, similarly, are worked up with an esophagram and endoscopy to denote the location and extent of esophageal involvement. Endoscopy is an important adjunct to the diagnosis to rule out malignancy as the cause of esophageal narrowing appreciated on a contrast study.
Management
The treatment of esophageal perforations, leaks, and strictures continues to evolve, as traditional thoracotomy and repair versus diversion is now challenged by the less invasive endoscopy with stenting, dilation, and thoracoscopy.
Regardless of approach, the tenets of treatment remain the same for perforations and leaks: drainage of infection, timely intervention, prevention of progressive contamination, restoration of gastrointestinal continuity, and nutritional support.
One of the initial steps in patient management is determining whether or not the esophagus is salvageable. The traditional management of esophageal leaks and fistulas is currently being challenged by esophageal stents, which now may allow the surgeon to salvage a previously doomed esophagus. The use of temporary covered self-expanding metal and Silastic stents in the management of esophageal leakage is promising, but clinical trials in this area are being conducted for this sometimes off-label use.
Refer to Fig. 49.6 for our algorithm for the management of esophageal perforations.
To Repair or Not to Repair
Most perforations and leaks that are identified acutely are amenable to treatment rather than diversion, while large perforations may not be amenable to repair. Perforations involving more than 50% of the circumference of the esophageal wall and longer than 3 cm are likely to become undilatable strictures once healed.
Nonviable tissue is débrided, and mucosal and muscle layers are closed separately. In some cases, the esophageal muscle must be opened additionally to adequately expose the mucosal defect and ensure adequate repair. The repair may be buttressed by well-vascularized tissue. Cervical perforations may be buttressed with sternocleidoastoid muscle, rhomboid, pectoralis, or intercostal muscle. Thoracic or abdominal perforations can use pedicled intercostal muscle flap, serratus, latissimus dorsi, pericardial fat pad, omentum, diaphragm, or gastric fundal flap. Additionally, surgical repair is not typically recommended in patients with delayed presentation (>48 hours).
Alternative management strategies for complex, large, or delayed presentation include hybrid repairs, T-tube placement, temporary stenting, or diversion. Hybrid approach includes initial débridement and the placement of a buttressing muscle over the perforation to complement internal coverage with stenting, and it is essential to widely drain the region to prevent ongoing contamination. T-tubes can also be used to drain perforations and create a long, externalized fistula. However, these are unreliable means of ensuring fistula control and more frequently result in leakage around the tube than through the tube. Esophageal exclusion may also offer a proximal esophageal side diversion onto the neck and a temporary Vicryl suture tied distal to the diversion to eliminate salivary contamination of a severe, uncontrolled, distal esophageal perforation. A diverting esophagostomy may be a consideration with delayed reconstruction. In the case of very high cervical defects with insufficient length for diversion, a salivary bypass drainage tube may be required.
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