Evidence-based, cost-effective management of nontraumatic esophageal perforations: An algorithm of the Journal of Trauma and Acute Care Surgery Emergency General Surgery Algorithms Work Group

The Journal of Trauma and Acute Care Surgery Emergency General Surgery Algorithms Work Group developed this algorithm to provide an evidence-based, practical approach to the initial evaluation and management of nontraumatic esophageal perforations presenting in the emergency setting. This algorithm (Fig. 1) is designed as a bedside reference for clinicians and includes letter annotations that link to explanatory text detailing the rationale and supporting references; it is intended to inform clinical decision making but not to replace the judgment and experience of bedside practitioners, nor should it be interpreted as a “standard of care.” Institutions are encouraged to incorporate these recommendations into their local clinical protocols, with the understanding that individual patient characteristics and local system constraints may justify deviations from the algorithm.Figure 1: Nontraumatic esophageal perforations diagnosis and management. NPO, nil per os; IV, intravenous; ABX, antibiotic; AFT, antifungal therapy; G, gastrostomy; J, jejunostomy.Nontraumatic esophageal perforations, characterized by transmural disruption of the esophagus, are uncommon but potentially catastrophic events. The reported incidence typically ranges from approximately 3 to 14 cases per million population per year,1–3 and the estimated mortality rate ranges from 5% to 60%, depending on the time to presentation and location in the esophagus.3 One-half of cases are iatrogenic, resulting from diagnostic or therapeutic endoscopic procedures (e.g., ligation of varices, esophageal dilation, etc.).4–6 Other causes include transmural rupture associated with forceful emesis (Boerhaave syndrome), necrosis related to foreign-body impaction, caustic injury, and others (Table 1).1,3,7–9 Additionally, risk factors that increase the likelihood of perforation include advanced age, underlying esophageal pathology (such as stricture, motility disorder, or neoplasm), recent instrumentation of the esophagus, and comorbid conditions that impair tissue integrity or healing.10,11 TABLE 1 - Causes of Nontraumatic Esophageal Perforations Cause Percentage of Presentation Iatrogenic injury 50 Boerhaave syndrome 15 Foreign body ingestion 12 Traumatic injury 9 Intraoperative injury 2 Malignancy 1 Others* 11 *Includes erosive or ulcerative disease, caustic injury, severe gastroesophageal reflux disease and Mallory-Weiss tear, other esophageal motor disorders, medications, infective causes (candida, herpes, syphilis, tuberculosis), and, less commonly, ischemic or inflammatory processes. Esophageal perforations carry a substantial morbidity and mortality rate that varies based on the etiology, location of perforation, the extent of contamination, and, most importantly, the timeliness of treatment.12,13 Therefore, they require early recognition and coordinated management by emergency physicians, surgeons, gastroenterologists, and critical care teams to limit mediastinal and pleural contamination and sepsis. Given the rarity of this problem, its acuity, and its variable presentation, a structured approach is essential for the early evaluation and triage of patients presenting to the emergency setting. Initial Evaluation and Management: The initial evaluation of a patient with chest or epigastric pain requires a detailed history and thorough physical examination. Patients with an esophageal perforation usually present with acute and sudden pain referring directly to the site of perforation.10,14,15 In patients with an intrathoracic perforation, chest pain is the cardinal symptom and often radiates to the back or left shoulder;2 in about 25% of cases, it is followed by vomiting and shortness of breath, classically described as Mackler's triad (vomiting, pain, and subcutaneous emphysema).16,17 Since this pattern of symptoms could also be consistent with acute myocardial infarction, cardiac etiology should be ruled out. In patients with an intra-abdominal esophageal perforation, severe epigastric pain can be accompanied by hematemesis or melena.18,19 In patients with a cervical perforation, neck pain can be concurrent with dysphagia, dysphonia, or hoarseness.3 Patients with esophageal perforations are often in significant distress with tachycardia and fever.3,20 Crepitus in the neck region or chest wall may be present in 60% of perforations, suggesting subcutaneous emphysema.15,18,20 As soon as the diagnosis of esophageal perforation is considered, treatment should be started. Patients should be admitted to hospital, given nothing by mouth, and resuscitated with isotonic crystalloid solutions. Broad-spectrum intravenous antibiotics should be administered along with antifungal coverage in select cases (e.g., immunosuppressed patients, those previously on broad-spectrum antibiotics, etc.).21,22 The imaging modality of choice is a contrast-enhanced computed tomography (CT) scan of the chest and abdomen.23–25 Both intravenous and water-soluble enteral contrast (oral route recommended if safe) should be used since this can help delineate the injury.10,26 Besides having a high sensitivity for the diagnosis of esophageal perforation, it allows for the visualization of the site of perforation and the degree of contamination or damage to surrounding structures. It can also exclude alternative diagnosis such as aortic dissection, esophageal intramural hematoma, and Mallory-Weiss tear.10,26,27 If the CT is equivocal and there is a high suspicion, the next step is a contrast esophagogram, which has a high sensitivity and specificity for the diagnosis of esophageal perforation.3,28,29 Gastrografin or barium could be used, although the first one has a lower risk of causing mediastinitis.30,31 Although useful, it requires patients to be awake, follow commands, and tolerate oral intake. Once the diagnosis of nontraumatic esophageal perforation has been made, the choice of treatment depends on the site of perforation, the patient's physiology, the timing of the presentation, and the extent of contamination.18,20,32,33 Mortality increases with the interval between perforation and intervention, so delay should be avoided.9,34 For hemodynamically stable patients with contained esophageal disruption, minimal contamination of surrounding spaces, and early presentation (</= 24 hours), nonoperative management may be considered. Altorjay et al.35 developed the classic criteria for nonoperative management of these perforations.36 Additionally, the Pittsburgh esophageal perforation severity scoring system (Table 2) was also developed and validated to guide this decision making, with a score of δ</= 2 suggesting eligibility for nonoperative management.37–39 Besides antibiotics, antifungal therapy, and proton pump inhibitors, parenteral nutrition or feeding via an enteral route should be considered. In this scenario, consultation with an experienced esophageal surgeon is suggested. In selected patients with small esophageal perforations, endoscopic management may be effective.40,41 The American Gastroenterological Association clinical practice update recommends that endoscopic closure of esophageal perforations should be pursued when feasible, using through-the-scope clips or over-the-scope clips for esophageal perforations <2 cm and endoscopic suturing for perforations >2 cm, reserving esophageal stenting with self-expanding metal stent for cases where primary endoscopic closure is not possible.9,40,42 As stents cover the perforation, they allow for healing of the defect. The stents are then left in place for approximately 4 to 8 weeks.43,44 Stenting has also been used after a trial of nonoperative management.45 One also needs to consider potential complications including stent migration or obstruction, erosion, and failure to adequately seal the defect.45,46 Within the last decade, endoluminal vac therapy (EVT; also known as endoscopic vacuum therapy, endoscopic vacuum–assisted closure, and endosponge) has demonstrated effective outcomes for the endoluminal treatment of esophageal perforations. The therapy uses the same principles as cutaneous negative-therapy vacuum-assisted devices in which a porous wound sponge attached to an nasogastric tube is placed either in the esophagus or in the abscess cavity under endoscopic guidance with negative pressure. This results in removal of infected secretions, decreased edema, and gradual closure of the defect. A recent review of EVT for esophageal defects including 11 studies with 210 patients reported a success rate of 90% for healing esophageal anastomotic leaks and 96% for the closure of esophageal perforations at a median follow-up of 235 days with a median duration of therapy of 17 days.47 While EVT may be a helpful tool to decrease healing time, it does require hospitalization while in use and may require thoracic surgery or gastroenterology consultation for placement.21 Nevertheless, depending on the extent of the perforation, placement of a gastrostomy or jejunostomy tube should be considered. If there is no clinical deterioration (e.g., tachycardia or fever), the patient can complete a swallow study after 5 to 7 days and slowly resume oral intake if there is no leak.48 Patients treated with a stent should undergo a swallow study to make sure it is occlusive, with subsequent discharge on full liquids and plan for stent removal and a final swallow study in 6 weeks.1,48 If the stent is not occlusive, patient should be closely monitored, and the next step will depend on their clinical status, and the location and size of the perforation. In a stable patient, another endoscopic procedure may be attempted. However, if endoscopic alternatives fail, or the patient's condition deteriorates, then a surgical approach is indicated.49 When resuming oral intake, one should consider a process that includes a 3-week progression of full liquids to pureed liquids, to soft solids for 1 week each.50,51 Active involvement of the nutrition team is recommended. However, if there is clinical deterioration and with evidence of leak, surgical management needs to be considered and a CT scan should be performed to rule out mediastinitis or abscess.52 For patients who do not meet the criteria for nonoperative or endoscopic management, including those with a noncontained esophageal rupture, surgical management is required. Primary surgical repair is the optimal choice; however, alternative procedures such as diversion or esophagectomy may be performed when primary repair is not feasible (e.g., presence of malignancy, diffuse necrosis, or defect greater than 10 cm).9,50,53–55 If the patient is stable and presents within 24 hours of perforation, the surgical approach will vary based on the location of the perforation.10 Compared with other locations, cervical perforations tend to be more easily treated.7 The cricopharynx is the most common site of injury in diagnostic endoscopy.56 Standard treatment is conservative management with drainage unless the perforation is greater than 2 cm, the diagnosis is delayed, or the patient shows signs of sepsis.56 These features require prompt surgical intervention, most commonly primary repair via open or endoscopic approach. Although the open surgical approach has been most common, the use of endoscopic clips, stents, and endosuturing is rapidly increasing, and individualized treatment is recommended. If the esophageal defect can be visualized, and there is no distal obstruction, a primary repair can be performed. This includes mobilization of the esophagus, debridement of the site of perforation, tension-free closure of the perforation, buttressing with vascularized tissue, and appropriate drainage.7,29,30 To facilitate early nutritional support and promote healing, placement of a feeding tube is also recommended.57 If the perforation cannot clearly be located, then external drainage is recommended to control the leak. To repair a perforation of the intra-abdominal segment of the esophagus, the preferred approach is a midline laparotomy, followed by debridement of necrotic tissues and tension-free closure of the perforation. A complete or partial fundoplication is usually used for buttressing the repair site, plus irrigation of the peritoneum with isotonic saline, placement of Jackson-Pratt drains, and a jejunostomy tube.29,30 For thoracic perforations, the procedure depends on which side the perforation is on and the extent of damage to the esophagus. If there is extensive esophageal damage or distal obstruction, esophagectomy with diversion and delayed reconstruction should be performed.57,58 A thoracotomy is usually performed for mobilization and resection of the affected esophagus, along with debridement and drainage of the mediastinum. In this scenario, placement of a gastrojejunostomy tube or separate gastrostomy and jejunostomy tubes is recommended (open or minimally invasive); however, this can be completed at a close interval setting if the patient is too unstable.59 Reconstruction of the alimentary tract is usually performed 6 to 12 months after the perforation and diversion. Creation of a retrosternal gastric conduit can be used to restore continuity if the gastroepiploic artery is intact. If the stomach is not suitable, colonic interposition or supercharged jejunum are appropriate alternatives.60–62 If the damage is not extensive and there is no distal obstruction, primary repair is the optimal choice, including debridement of the perforation and external drainage to control the mediastinal and pleural contamination.30,59 A right thoracotomy will be performed for upper thoracic esophageal perforations, and a left thoracotomy for perforations in the lower third.3 Once the perforation has been localized, a two-layer repair is recommended, with separate suturing of the mucosa and muscle.57 To decrease the risk of leakage, both the proximal and distal ends of the mucosal defect should be appreciated and debrided to healthy tissue prior to closure. The repair should then be buttressed with a pedicled flap using intercostal muscle or a pleural or pericardial patch. In addition to mediastinal and pleural drainage, provision of enteral nutrition is essential; this can be facilitated by the placement of a jejunostomy tube at the time of the esophageal repair.3,10,30 When the diagnosis is delayed for more than 24 hours, primary repair remains the treatment of choice; however, the use of a vascularized pedicle flap as an enhancement to the repair is highly recommended. If primary repair is not possible, surgically placing a latex/rubber T-tube (along with a gastrostomy or jejunostomy tube for enteral nutrition) can prevent further contamination of the mediastinum and chest with reported high success rates in patients with delayed presentation.50,52,63 Esophagectomy with diversion, as described previously, can also be considered. TABLE 2 - Pittsburgh Severity Score for Esophageal Perforation Variables Points Assigned Age >75 y 1 Tachycardia >100 beats per minute) 1 Leukocytosis >10,000 WBC/mL 1 Pleural effusion 1 Fever, temperature >38.5°C 2 Noncontained leak 2 Respiratory distress, respiratory rate >30 or increasing oxygen requirement 2 Time to diagnosis >24 h 2 Hypotension, systolic blood pressure <90 mm Hg 3 Esophageal cancer 3 Adapted from Moletta et al.39WBC, white blood cell.