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INTRODUCTION — Esophageal perforation is a diagnostic and therapeutic challenge because of the rarity of the condition and the variability in presentation. Surgical management is primarily based upon small retrospective studies and expert opinion. The following basic principles are applied to the management of a patient with an esophageal perforation:
The etiology and surgical management of a perforation to the cervical, thoracic, and abdominal esophagus will be reviewed here. The risk factors, clinical presentation, diagnosis, and nonoperative management of an esophageal perforation are discussed elsewhere. (See "Complications of endoscopic esophageal stricture dilation" and "Boerhaave's syndrome: Effort rupture of the esophagus".)
ANATOMY — The esophagus has three anatomical points of narrowing, the cricopharyngeus muscle, the broncho-aortic constriction, and the esophagogastric junction (figure 1) . Perforation may occur anywhere along the esophagus, but there is a predilection for rupture at these key anatomic areas. As an example, iatrogenic injuries to the cervical esophagus can occur during endoscopy at Killian’s triangle (figure 2), an area lacking a posterior esophageal muscularis and bordered by the horizontal cricopharyngeus muscle inferiorly and the oblique inferior constrictor muscles superiorly.
ETIOLOGY — Increased intraluminal pressure at the anatomic sites of narrowing, as well as sites narrowed by a malignancy, foreign body, or physiologic dysfunction, can lead to rupture of the esophagus (image 1 and image 2).
More than one half of all esophageal perforations are iatrogenic and most of these occur during endoscopy [2-7]. The rate of esophageal perforation during diagnostic and therapeutic esophageal endoscopy is discussed separately. (See "Overview of upper gastrointestinal endoscopy (esophagogastroduodenoscopy)", section on 'Perforation' and "Complications of endoscopic esophageal stricture dilation", section on 'Esophageal perforation'.)
Other causes of esophageal perforation include :
PRIMARY MANAGEMENT — The major principles of the primary and immediate management of an esophageal perforation include prompt diagnosis, stabilization of the patient, and assessment for operative or nonoperative management. (See 'Primary surgical repair' below and 'Alternatives to primary surgical repair' below.)
Regardless of etiology, an esophageal perforation is a surgical emergency [8,9]. Leakage of esophageal and gastric contents into the mediastinum creates a necrotizing inflammatory process that can lead to sepsis, multiorgan failure, and death [10,11]. The near doubling of overall mortality from 14 to 27 percent with a delay in diagnosis greater than 24 hours after perforation emphasizes the importance of a prompt diagnosis and treatment (figure 3) . The diagnostic studies performed to confirm the clinical suspicion of esophageal perforation are discussed in detail elsewhere. (See "Boerhaave's syndrome: Effort rupture of the esophagus", section on 'Diagnosis' and "Complications of endoscopic esophageal stricture dilation", section on 'Diagnosis'.)
The rarity of the diagnosis and the variability in clinical presentation often lead to diagnostic treatment delays. This is especially true of spontaneous perforation, in contrast to iatrogenic perforation, where the clinical suspicion is low, which often leads to the evaluation of more common medical conditions such as myocardial infarction, pneumonia, and peptic ulcer disease. (See "Chest pain of esophageal origin" and "Evaluation of chest pain in the emergency department".)
Initial management — Once the diagnosis is suspected, treatment is started immediately:
Selection of operative candidates — Following confirmation of the diagnosis and stabilization of the patient, the clinician must decide if the patient should undergo operative or nonoperative management. While operative management is required for most patients to minimize morbidity and mortality, there are specific clinical settings (eg, minimal extraluminal contamination) where nonoperative management is an acceptable alternative. (See 'Primary surgical repair' below and 'Nonoperative management' below.)
PRINCIPLES OF SURGICAL MANAGEMENT — Primary repair of the perforation site is the optimal procedure, even if the diagnosis is delayed greater than 24 hours. The exceptions to performing a primary repair include a cervical perforation that cannot be accessed but can be drained, diffuse mediastinal necrosis, a perforation too large for the esophagus to be re-approximated, an esophageal malignancy, pre-existing end-stage benign esophageal disease (eg, achalasia), or the patient is clinically unstable [12-14]. Surgical alternatives to primary repair in these settings are discussed below (see 'Alternatives to primary surgical repair' below).
Primary surgical repair — A primary suture or pedicle repair is performed when, in the judgement of an experienced surgeon, the closure can heal and not re-perforate.
General principles for esophageal repair — The following general principles are used to perform a repair of a perforation of the cervical, thoracic, or abdominal esophagus (figure 4):
When there has been a delay in diagnosis greater than 24 hours, and/or substantial extraluminal contamination from the leakage of fluid and debris has occurred, the integrity of the repair can be enhanced with the use of a vascularized pedicle flap. The most common flap used is the intercostal muscle flap (figure 5 and figure 6). Other options for a flap include serratus muscle, latissimus dorsi muscle, diaphragm, parietal pleura, omentum, and gastric fundus.
If the gastric fundus is used to buttress a lower esophageal repair, the gastroesophageal junction should be placed in the normal intra-abdominal location to avoid severe and debilitating gastroesophageal reflux. A Dor fundoplication (partial anterior wrap) is an excellent alternative for an intra-abdominal perforation.
Cervical perforation — Cervical perforations are typically more easily treated than perforations of the thoracic or intra-abdominal esophagus . A primary repair of a cervical perforation is performed if the perforation can be clearly visualized and there is no distal obstruction. Otherwise, drainage of the perforation is adequate to control the leak since the anatomic structures of the neck typically confine extraluminal contamination to a limited space and thereby enhance spontaneous healing (figure 7). (See 'Drainage only' below.)
The surgical approach to control a perforation in the cervical esophagus begins with an incision in the left neck along the lower third of the sternocleidomastoid (SCM) muscle (figure 8), unless the perforation is documented or visualized from the right neck . The surgical dissection proceeds with identification of the anatomic structures such as the recurrent laryngeal nerve, which should be preserved. Soft retractors, including the fingers of the surgeon and first assistant, are used to retract the esophagus and trachea.
Blunt dissection should be carried into the mediastinum posterior to the esophagus and anterior to the prevertebral fascia to assure adequate drainage of the infection. The perforation should be primarily repaired when clearly visualized, as described above. However, if the perforation is not clearly visualized, then the perforation site is drained. (See 'General principles for esophageal repair' above.)
The wound is irrigated, widely drained with Jackson-Pratt drains, and loosely closed in layers with interrupted absorbable sutures. Alternatively, when heavy contamination is present, the wound may be left open and packed with wet to dry dressings or a wound vac. (See "Negative pressure wound therapy".) The authors do not routinely use a nasogastric tube. A feeding tube is only considered in patients presenting with significant malnutrition.
Thoracic perforation — A thorough knowledge of the relationship of the esophagus to the adjacent vital structures is necessary when planning the surgical approach to a thoracic perforation (figure 1 and figure 10). The level of the perforation of the thoracic esophagus determines the surgical approach to controlling the leak and repairing the perforation. As an example, a mid-esophageal perforation is approached through a right thoracotomy at the sixth or seventh intercostal space while a distal esophageal perforation is approached through a left thoracotomy at the seventh or eighth intercostal space (figure 11).
The following surgical techniques are used to expose the thoracic esophagus (figure 12):
Abdominal perforation — The general principles for the management of an intra-abdominal esophageal perforation are the same as those described for perforations of the cervical and thoracic esophagus (figure 15). These surgical principles include a careful dissection to isolate the esophagus without damaging vital structures, evacuation of debris and devitalized tissues, and debridement of the area of perforation.
A laparotomy is the preferred approach to repair a perforation of an intra-abdominal esophagus. The left triangular ligament (peritoneal attachment of the liver to the diaphragm ) of the liver is divided and the liver is retracted laterally (figure 16). This maneuver provides access to the esophageal hiatus, which is proximal to the perforation. Division of the short gastric vessels will help mobilize the gastroesophageal junction for improved exposure and access to the perforation (figure 17).
Following the primary suture repair, a Dor (partial 180° anterior wrap) or a Nissen (complete 360° posterior wrap) fundoplication is used to buttress the site of repair depending on the site of perforation and patients’ preoperative history of swallowing dysfunction. The peritoneum is then copiously irrigated with isotonic saline, Jackson-Pratt drains are placed near the site of repair, and a feeding jejunostomy tube is placed for postoperative alimentation.
Postoperative management — This approach is used to manage patients with an esophageal perforation at any site:
Alternatives to primary surgical repair — Several approaches have been described for cases when a primary repair is technically not feasible, the patient is hemodynamically unstable, or the perforation is diagnosed immediately after the event. As an example, severe mediastinitis associated with extra-esophageal tissue friability and necrosis from a delay in diagnosis can preclude a primary repair . Options in these circumstances include drainage and/or diversion procedures.
Endoscopic covered stent placement has been described as an alternative procedure to primary repair, however, there are no patient selection guidelines. For patients with a distal malignancy or achalasia, an esophagectomy may be warranted. Nonoperative management may be an option for highly select cases in which the patient is diagnosed early, and has evidence of a contained perforation and limited extraluminal soilage.
Drainage only — Surgical drainage as the sole operative management is reserved for perforations of the cervical esophagus when the perforation site cannot be completely visualized and when there is no distal obstruction. Drainage alone is contraindicated in the management of a perforation of the thoracic or intra-abdominal esophagus because of uncontrolled leakage and contamination of adjacent tissues (ie, pleura, peritoneum).
For patients with clinical evidence of mediastinal sepsis, significant comorbid illness, and large thoracic esophageal perforations, the authors utilize a hybrid technique that includes aggressive debridement, drainage, muscle flap coverage of the defect, and endoscopic stent placement. (See 'Endoscopic stent placement' below.) The goal of this approach is to reconstruct the esophageal lumen and control the leakage and septic contamination of adjacent tissues in patients unlikely to tolerate a second operative procedure to re-establish continuity of the esophagus. Alternatively, a T-tube may be inserted into the perforation to create a controlled fistula when a patient cannot tolerate more extensive surgery [17-19].
Diversion — A procedure to divert the esophageal contents rather than perform a primary repair of a perforation is indicated when:
The goals of a diversion procedure include:
A thoracotomy is typically performed to mobilize and resect the esophagus, and debride and drain the mediastinum. Alternatively, for an intra-abdominal perforation the thoracic esophagus may be bluntly dissected. A laparotomy is performed to insert a gastrostomy tube, a jejunostomy feeding tube, and to suture close the diaphragmatic hiatus to prevent subsequent hiatal hernia formation.
For the patient who is hemodynamically unstable and critically ill, a diversion without an esophageal resection is performed. The perforation site must be adequately drained to control esophageal contamination. A cervical esophagostomy is constructed and the distal esophagus divided at the diaphragmatic hiatus to exclude the site of perforation (figure 18). A gastric feeding tube is inserted at this time. Once the patient is stabilized, definitive operative management can proceed.
The fundamental technical components of a cervical esophagostomy include:
Postoperative management unique to this population includes flexible fiberoptic laryngoscopy for evaluation of the vocal cord function, digital dilation of the esophagostomy to prevent stricture formation and reduce the risk of aspiration pneumonia, and aggressive nutritional support . (See "Nutrition support in critically ill patients: Enteral nutrition".)
Reconstruction of the esophagus is typically performed six months to one year following the perforation, pending full recovery. Restoration of alimentary tract continuity often requires a retrosternal colon interposition [17,18,21].
Endoscopic stent placement — Esophageal endoscopic covered stent can be used for the management of an esophageal perforation in selected patients [22-25]. While no guidelines exist, stents may be appropriate for patients with extensive comorbidities, advanced mediastinal sepsis, or large esophageal defects and the patient’s inability to tolerate more extensive surgery. Stents should be placed only by an experienced clinician.
Precise stent placement can restore luminal integrity and prevent further extraluminal soilage. However, control and drainage of the extraluminal contamination must be achieved for this approach to effectively manage the esophageal perforation.
Complications of this procedure include stent malpositioning and migration, especially when used in close proximity to the gastroesophageal junction, and stent obstruction. The types of esophageal stents are described in another topic. (See "Expandable stents in the treatment of esophageal obstruction", section on 'Types of stents'.)
The authors perform endoscopic stent placement for an esophageal perforation in the operating room with fluoroscopic guidance. The fundamental technical components include:
Postoperative management includes a contrast esophagography to assess placement of the stent and to identify potential leakage from the perforation. Oral intake is initiated if the clinical status allows and the perforation is controlled. Stent positioning can be monitored with plain radiographs, particularly when placed in the distal esophagus.
Esophagectomy — A primary repair alone of an esophageal perforation proximal to untreated achalasia, an undilatable stricture, or a malignancy should not be performed. An esophagectomy at the time of perforation may be performed if the patient is clinically stable and there is minimal contamination.
A perforation of the distal esophagus following dilatation in patients with achalasia requires special mention. The degree of preoperative dysfunction dictates treatment strategies as illustrated in the following examples:
The same principles can be applied to patients with perforated cancers or undilatable strictures as these patients are not candidates for a primary repair of the esophageal perforation.
OUTCOMES FOLLOWING OPERATIVE MANAGEMENT — The principal variables associated with mortality from an esophageal perforation include delay in diagnosis, type of repair, location of perforation, and etiology of the perforation (table 1 and figure 3). The most common cause of death is sepsis leading to multiorgan failure .
The following two retrospective reviews from two different eras report similar findings and illustrate the variables associated with mortality:
The lower mortality rate associated with a traumatic rupture may be due to an earlier diagnosis while the diagnosis of a spontaneous rupture is frequently delayed.
Mortality rates by location of the perforation were:
The lower mortality rate associated with a cervical perforation may be related to the anatomic tissue planes of the neck that limit the spread of contamination and infection (figure 2).
There are no reports from large series detailing the frequency and type of postoperative complications. Based upon small series of patients, the most frequently reported complications include persistent leak, fistula formation, mediastinitis, empyema, esophageal stricture, pneumonia, abscess, and sepsis [12,13,27-29].
NONOPERATIVE MANAGEMENT — The role of nonoperative management has evolved rapidly in the past several decades, likely due to the increasing incidence of iatrogenic injuries, which are often diagnosed more quickly and are associated with less extraluminal contamination. Essential to nonoperative management is careful patient selection; appropriate patient selection can achieve 100 percent survival rates [30-32]. This requires clinicians experienced in the care of esophageal pathology, careful patient monitoring, and the early involvement of the appropriate surgical team.
Cervical perforation is most commonly considered for nonoperative management due to the anatomic confinement of the esophagus by surrounding surgical structures. Perforation into the pleural or peritoneal cavity is a relative contraindication to nonoperative management due to the difficulties of controlling spillage of contaminated contents in large, free spaces.
Evidenced based criteria for patient selection for nonoperative management and the medical management of an esophageal perforation are discussed separately. (See "Complications of endoscopic esophageal stricture dilation", section on 'Medical management' and "Complications of endoscopic esophageal stricture dilation", section on 'Approach to medical management'.)
The authors utilize nonoperative management in patients diagnosed early in settings where there is evidence of contained perforations and limited extraluminal soilage. Patients are maintained on intravenous fluids, NPO, and broad spectrum antibiotics for five to seven days. As long as patients remain clinically stable, contrast esophagography is performed at five to seven days and resumption of oral intake under observation is considered depending on the results. We generally do not utilize parenteral nutrition unless the patient has evidence of malnutrition prior to diagnosis. Patients who show evidence of clinical deterioration require surgical intervention to control extraluminal contamination and to restore luminal integrity.
SUMMARY AND RECOMMENDATIONS — Esophageal perforation is a diagnostic and therapeutic challenge because of the rarity of the condition and the variability in presentation.
All topics are updated as new information becomes available. Our peer review process typically takes one to six weeks depending on the issue.