Esophageal perforation

INTRODUCTION — Esophageal perforation is a diagnostic and therapeutic challenge because of the rarity of the condition and the variability in presentation.

The following basic principles are applied to the management of a patient with an esophageal perforation:

●Rapid diagnosis

●Appropriate hemodynamic monitoring and support

●Antibiotic therapy

●Restoration of luminal integrity when feasible

●Control of extraluminal contamination

The etiology, presentation, diagnosis, and management of perforations to the cervical, thoracic, and abdominal esophagus will be reviewed here. Specific types of esophageal perforation are also discussed elsewhere. (See "Adverse events related to endoscopic dilation of esophageal strictures" and "Boerhaave syndrome: Effort rupture of the esophagus".)

ANATOMY — The esophagus has three anatomic points of narrowing: the cricopharyngeus muscle, the broncho-aortic constriction, and the esophagogastric junction (figure 1) [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 "Adverse events related to endoscopic dilation of esophageal strictures", section on 'Esophageal perforation'.)

Other causes of esophageal perforation include [2]:

●Spontaneous perforation (Boerhaave's Syndrome): 15 percent (see "Boerhaave syndrome: Effort rupture of the esophagus", section on 'Clinical manifestations')

●Foreign body ingestion: 12 percent (see "Ingested foreign bodies and food impactions in adults" and "Foreign bodies of the esophagus and gastrointestinal tract in children")

●Trauma: 9 percent (see "Penetrating neck injuries: Initial evaluation and management", section on 'Pharyngoesophageal injuries' and "Initial evaluation and management of blunt thoracic trauma in adults", section on 'Esophageal injury')

●Intraoperative injury: 2 percent

●Malignancy: 1 percent

PRESENTATION — The clinical presentation of esophageal perforation varies depending on the location of the perforation [8]:

●Cervical perforation – Patients usually present with neck pain which may be accompanied by dysphagia, hoarseness, dysphonia, or subcutaneous emphysema. Systemic signs are less common with cervical perforation.

●Intrathoracic perforation – Sudden onset chest pain is the cardial sign of intrathoracic esophageal perforation [9]. The pain may radiate to the back or the left shoulder. In about 25 percent of patients, the chest pain is accompanied by vomiting and shortness of breath (Mackler triad [10]).

●Intra-abdominal perforation – Patients usually present with acute onset severe epigastric pain, which may be accompanied by either hematemesis or melena.

Patients with a perforated esophagus are typically in significant distress with tachycardia and possibly fever. There may be crepitus in the neck or chest area suggestive of subcutaneous emphysema. If untreated, systemic inflammatory response syndrome, sepsis, or multiorgan system failure usually sets in over 24 to 48 hours due to mediastinal contamination. Thus, timely diagnosis and initiation of treatment is of essence.

DIAGNOSIS — Esophageal perforation is typically diagnosed radiographically [8]. Contrast-enhanced computed tomography (CT) of the chest and abdomen is the imaging test of choice for patients suspected of having a perforated esophagus [11]. CT is not only highly sensitive in diagnosing esophageal perforation but can also assess the damage to surrounding structures (eg, mediastinal or pleural contamination) and exclude alternative diagnosis (eg, aortic dissection) [12].

In select cases where CT findings are equivocal, esophageal perforation can also be diagnosed by contrast esophagogram or endoscopy. Although plain chest films may show indirect evidence of esophageal perforation (eg, mediastinal air) (image 3), they are neither sensitive nor specific for making the diagnosis. The diagnostic studies performed to diagnose specific etiologies of esophageal perforation are discussed elsewhere. (See "Boerhaave syndrome: Effort rupture of the esophagus", section on 'Diagnosis' and "Adverse events related to endoscopic dilation of esophageal strictures", section on 'Diagnosis'.)

DIFFERENTIAL DIAGNOSIS — 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) [2]. However, the rarity of esophageal perforation and the variability in clinical presentation often lead to diagnostic treatment delays. This is especially true of spontaneous perforation where the clinical suspicion is low [13]. This often leads to the evaluation of more common medical conditions such as (see "Evaluation of the adult with chest pain of esophageal origin" and "Approach to the adult with nontraumatic chest pain in the emergency department"):

●Pneumonia

●Pleural effusion or empyema

●Gastroesophageal reflux disease/peptic ulcer disease

●Gastric volvulus/hiatal hernia

●Pneumothorax

●Myocardial infarction

●Pulmonary embolism

●Aortic dissection

INITIAL MANAGEMENT — Once the diagnosis is suspected, treatment is started immediately:

●Patient is made NPO (nothing by mouth, nil per os).

●A large-bore intravenous line is started, and fluid resuscitation is performed with isotonic saline solution or Lactated Ringer solution.

●Broad-spectrum intravenous antibiotics that provide coverage for aerobes and anaerobes are administered intravenously, such as ampicillin/sulbactam (3 grams every six hours), piperacillin/tazobactam (3.375 grams every six hours), or a carbapenem. In the setting of beta lactam hypersensitivity, use of clindamycin (900 mg every eight hours) plus a fluoroquinolone, such as ciprofloxacin (400 mg every 12 hours), is acceptable.

●Antifungal coverage (eg, fluconazole 400 mg once a day) is warranted in selected cases. These include patients who have been hospitalized or received broad-spectrum antimicrobial agents prior to perforation, patients on long-term antacid therapy, patients who have received steroids or other immunosuppressive therapy prior to perforation, patients with HIV infection, patients with known esophageal candidiasis, patients with chronic obstructive motility pathology with stasis in the esophagus, and/or patients who fail to improve after several days of appropriate antibacterial therapy.

●The patient can be transferred to an intensive care unit for hemodynamic monitoring, stabilization, and volume resuscitation. Insertion of a central venous catheter, arterial catheter, and urinary catheter should be considered. These preparations should not delay surgical evaluation and management.

●The patient should be prepared in case of operative management, including laboratory evaluation (eg, complete blood count, coagulation studies, type and screen) and a chest radiograph.

DEFINITIVE MANAGEMENT — Definitive management of an esophageal perforation can be achieved surgically, endoscopically, or with conservative/medical management. The appropriate treatment is selected based on the etiology of the perforation and patient condition as detailed below (algorithm 1).

Endoscopy-related perforation — Most endoscopy-related esophageal perforations can be treated endoscopically [14,15]. Three techniques are most commonly used depending on the size of the perforation: primary closure with clips or suturing devices, endoscopic stenting, and endoscopic vacuum therapy (EVAC/EVT).

Endoscopic primary closure — Endoscopic primary closure may be used to treat small iatrogenic esophageal perforations with healthy, compliant surrounding mucosa that can be approximated with minimal tension in the absence of significant extraluminal contamination [14].

Failure to manage extraluminal contamination will result in a primary closure failure and possible fistula formation. Attempts to manage more chronic defects endoscopically requires attention to extraluminal source control of infection and ablation of fistula tract epithelialization and removal of foreign material.

Postprocedure management includes a course of intravenous antibiotics with duration determined by extent of contamination, a period of gastrointestinal rest based on confidence with defect closure, and subsequent contrast esophagography prior to resuming oral intake.

The technique of endoscopic clipping for perforation is further discussed elsewhere. (See "Endoscopic clip therapy in the gastrointestinal tract: Bleeding lesions and beyond", section on 'Perforations and fistulas'.)

Endoscopic stent placement — Esophageal endoscopic covered stents can be used for the management of an esophageal perforation in selected patients who have extensive comorbidities, advanced mediastinal sepsis, large esophageal defects, or an inability to tolerate more extensive surgery [16-20]. (See 'Surgical-endoscopic hybrid procedures' below.)

Stents should be placed only by an experienced clinician, and decisions regarding stenting should be made by a multidisciplinary team including an experienced gastroenterologist and thoracic surgeon. Guidelines from the American College of Gastroenterology support the use of stents in carefully selected patients, albeit "based on weak evidence" [21]. (See "Boerhaave syndrome: Effort rupture of the esophagus", section on 'Endoscopic therapy'.)

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. Based upon a seven-year retrospective review of 187 patients with esophageal stent placement for the management of intrathoracic anastomotic leak, perforation, or fistula, the four factors that were identified as risks for stent failure included [22]:

●Injury in the proximal cervical esophagus

●Injury that traversed the gastroesophageal junction

●Length of injury longer than 6 cm

●Anastomotic leak associated with a more distal conduit leak

The fundamental technical components of endoscopic stent placement include:

●Diagnostic endoscopy is performed first to localize the perforation and measure the length of the injury.

●A covered stent at least 4 cm longer than the size of the injury is used so that there is at least 2 cm of overlap proximal and distal to the perforation.

●If percutaneous endoscopic gastrostomy (PEG) tube placement is considered in conjunction with stent placement for enteral access, it should be placed with minimal insufflation prior to stenting the perforation. Otherwise, stent migration can occur as the PEG tube is drawn through the stent lumen. Alternatively, a push technique utilizing T-fasteners can be utilized, thus avoiding drawing the end of the PEG tube through the area of injury.

●Stent positioning in the distal esophagus can be challenging as the distal flare of the covered stent is placed in the lumen of the stomach. This gastric position decreases the ability of the stent to anchor by radial tension and results in frequent migration. In addition, it may not effectively seal the area of perforation and result in continued retrograde leakage of gastric contents. Attention to thoracic drains to identify this problem is important. A bridle attached to the proximal stent and anchored around the nasal septum can help prevent distal migration in these circumstances.

●Debridement and drainage of extraluminal contamination.

Postoperative management includes a contrast esophagography to assess placement of the stent and to ensure that the perforation is excluded. Oral intake is initiated if the clinical status allows and the perforation is controlled. A soft or liquid diet is advisable for patients who are able to ultimately take oral intake with the stent in position. If the stent traverses the upper esophageal sphincter, reflux precautions are advisable. Stent positioning can be monitored with plain radiographs, particularly when placed in the distal esophagus. Complications of this procedure include stent malpositioning and migration, especially when used in close proximity to the gastroesophageal junction, and stent obstruction.

EVAC/EVT — EVAC/EVT is an evolving technique for the management of esophageal injuries [23]. The procedure should be performed by clinicians familiar with the applications. It is generally best for smaller defects, contained defects with extraluminal contamination, and for patients who cannot tolerate more invasive thoracic procedures. It does require maintenance of the device with long-term use of nasogastric tube and regular device changes. (See "Endoluminal vacuum therapy (EVAC/EVT)".)

Nonendoscopy-related perforation — Following confirmation of the diagnosis and stabilization of the patient with a nonendoscopy-related esophageal perforation, the clinician must decide if the patient should undergo operative or nonoperative management. Stable patients with minimal contamination of surrounding spaces who present early can be managed nonoperatively. Otherwise surgical and/or endoscopic management is mandatory (algorithm 2).

Low-risk perforation in stable patients — Nonoperative management of esophageal perforation may be feasible in stable patients with early presentation, contained esophageal disruption, and minimal contamination of surrounding spaces. The specific criteria published more than two decades ago still hold true [24]:

●Presentation within 24 hours

●Stable hemodynamics

●Cervical or thoracic perforation on imaging

●On contrast imaging, the perforation is contained by surrounding tissue and extraluminal collections can freely drain back into the esophagus

●No existing esophageal disease

●Personnel available for surveillance

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 [24-26]. This requires clinicians experienced in the care of esophageal pathology, careful patient monitoring, and early involvement of the appropriate surgical team. Additional studies have derived clinically scoring systems [27] which may help objectify the patient selection criteria [28].

●Patients are maintained on intravenous fluids, nil per os (NPO), proton-pump inhibitor, and broad-spectrum antibiotics for five to seven days.

●For patients with evidence of malnutrition prior to diagnosis, early nutritional supplementation can be achieved parenterally or enterally. For enteral nutrition, a nasogastric tube needs to be placed with endoscopic assistance.

●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.

●Patients who show evidence of clinical deterioration (eg, fever, tachycardia) require surgical or endoscopic intervention to control extraluminal contamination and restore luminal integrity.

For esophageal perforations that are small and diagnosed early, it is very reasonable to attempt endoscopic repair or stent placement as an alternative to nonoperative management. The techniques are the same as for endoscopy-related perforations. (See 'Endoscopy-related perforation' above.)

High-risk perforation or unstable patients — Surgical treatment is required for all patients who do not meet the criteria for nonoperative management outlined above. Primary surgical repair is preferable, but alternative surgical treatments such as drainage, diversion, or esophagectomy may be required when primary repair is not possible (algorithm 2).

Preferred surgical technique — Primary repair of the perforation site is the optimal procedure, even if the diagnosis is delayed more than 24 hours. While the general principles of the repair are the same, the requirements for exposing the esophagus and buttressing the repair vary by the location of perforation.

General principles for esophageal repair — A primary repair is performed when, in the judgment of an experienced surgeon, the closure can heal. The same general principles are used to perform a repair of a perforation of the cervical, thoracic, or abdominal esophagus (figure 4):

●First, devitalized tissue is debrided from the perforation site.

●Second, the muscular layer is incised longitudinally along the muscle fibers superior and inferior to the perforation to expose the entire extent of the mucosal injury. Failure to visualize the proximal and distal extent of the mucosal disruption is the most common reason for a persistent leak.

●Third, the mucosa is closed with absorbable interrupted sutures and the muscularis layer is closed with interrupted nonabsorbable sutures. Narrowing of the esophageal lumen should be avoided using precise reapproximation.

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.

Cervical perforation — Cervical perforations are typically more easily treated than perforations of the thoracic or intra-abdominal esophagus [2]. 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 [29]. The surgical dissection proceeds with identification of the anatomic structures such as the carotid sheath, trachea, spine, and 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 in order to avoid recurrent laryngeal nerve injury.

The following surgical techniques are used to expose and manage the perforated cervical esophagus (figure 9) [1,29]:

●The SCM muscle and carotid sheath are retracted laterally.

●The middle thyroid vein and the omohyoid muscle are divided.

●The trachea and esophagus are bluntly retracted medially.

●The esophagus is carefully and bluntly dissected posteriorly along the retropharyngeal plane.

●All devitalized tissue is debrided.

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 that are brought out through the bottom of the incision or through a separate dependent site, 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 author does 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):

●Prior to entering the thoracic cavity, a posteriorly based intercostal muscle flap is harvested as a potential buttress of a primary repair (figure 5).

●The thoracotomy is performed with care to preserve the intercostal muscle vascular pedicle, and the lung is retracted anteriorly, which can be facilitated by the mobilization of the pleural reflection and inferior pulmonary ligament (figure 12).

●The pleural space is evacuated of debris and the devitalized tissue in the mediastinum is debrided.

●The esophagus is encircled with a Penrose drain proximal to the perforation to facilitate dissection (figure 13).

●The perforation is localized and the repair is planned based on the size of the perforation, the friability of the esophagus, the degree of surrounding contamination, and the clinical status of the patient.

●A nasogastric tube is guided past the defect and into the stomach under direct visualization prior to repair.

●The repair is then buttressed with a pedicled flap (eg, intercostal muscle flap) utilizing interrupted absorbable sutures. Alternatives to the intercostal muscle flap (figure 6) include a parietal pleura (figure 14), thymus, and latissimus dorsi. Omentum or gastric fundus may also be considered, although this could lead to future paraesophageal hernia and/or gastroesophageal reflux disease.

●The wound is copiously irrigated and drained utilizing large-bore chest tubes. One chest tube is positioned in proximity to the site of injury to ensure adequate drainage if the repair breaks down and additional tubes to drain the remaining space.

●Pulmonary decortication is performed if exudate and debris are present to facilitate adequate lung expansion.

●A jejunostomy feeding tube can be inserted by a mini-laparotomy procedure at the time of the esophageal repair. The author prefers to place feeding tubes in clinically stable patients with significant extraluminal contamination when a prolonged intensive care unit admission is anticipated or in patients who present with significant malnutrition.

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, removal 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 [30]) 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, the hiatus is closed posteriorly with interrupted silk sutures to create an opening that accommodates only the esophagus and a fingertip. The gastroesophageal junction should be placed in the normal intra-abdominal location to avoid gastroesophageal reflux.

A Dor (partial 180° anterior wrap), Toupet (partial 270° posterior 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. (See "Surgical treatment of gastroesophageal reflux in adults", section on 'Fundoplication procedures'.)

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.

Alternative surgical techniques — Primary repair of the perforation site is the optimal procedure, even if the diagnosis is delayed more than 24 hours. The exceptions to performing a primary repair include [2,31-33]:

●A cervical perforation that cannot be accessed but can be drained

●Diffuse mediastinal necrosis

●A perforation too large for the esophagus to be reapproximated

●An esophageal malignancy

●Preexisting end-stage benign esophageal disease (eg, achalasia)

●Patient is clinically unstable

Options in these circumstances include drainage only, esophagectomy with either reconstruction or diversion, and surgical-endoscopic hybrid procedures.

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. Esophagectomy with either reconstruction or diversion is appropriate in the presence of a distal obstruction. (See 'Esophagectomy with reconstruction' below.)

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 spaces (ie, pleura, peritoneum).

Esophagectomy with reconstruction — 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; otherwise, diversion is a more prudent option.

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:

●For patients who have maintained an adequate nutritional status and do not have a dilated, tortuous thoracic esophagus (sigmoid esophagus), primary repair of the perforation site and a myotomy on the contralateral side of the esophagus with a fundoplication can be performed. Many surgeons perform a partial fundoplication procedure to prevent reflux as well as to buttress the repair. The edges of the fundoplication are sewn to the edge of the myotomy, and the stomach covers the repair. (See "Surgical myotomy for achalasia".)

●For patients with end-stage achalasia, generally an esophagectomy is indicated. If there is minimal contamination and the patient is stable, such as following an iatrogenic injury, the surgeon may choose to proceed with esophagectomy and reconstruction with a gastric tube. If the diagnosis is delayed, an esophageal resection and cervical esophagostomy is indicated.

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.

Esophagectomy with diversion — A procedure to divert the esophageal contents rather than perform a primary repair of a perforation is indicated when:

●The patient is unstable

●Repair is not possible secondary to the size of the defect or friability of surrounding tissue

●Preexisting esophageal disease is present

The goals of a diversion procedure include:

●Control and drain extraluminal contamination

●Divert the esophagus proximally with a cervical esophagostomy

●Resection of the remaining esophagus

●Obtain gastric diversion with a gastrostomy tube and feeding tube access with a jejunostomy

●Close the diaphragmatic hiatus

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 closed 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. In such circumstances, it can be difficult to obtain significant length of the esophagus to form a functional cervical esophagostomy. 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:

●The cervical esophagus is approached by a left neck incision, as described in a previous section. (See 'Cervical perforation' above.)

●Proximal length of the esophagus should be as long as possible to facilitate esophagostomy construction and ostomy appliance on the anterior chest wall (figure 19). This point cannot be overemphasized.

●A blind cervical stump should never be left in place, except in dire circumstances, as the stump will invariably rupture and lead to uncontrolled mediastinal contamination within days of its construction. A blind-ended esophagus drained by a nasogastric tube is not a durable solution; plans should be made for formal diversion as quickly as the patient can be stabilized.

●A subcutaneous tunnel is bluntly fashioned over the sternocleidomastoid muscle, clavicle, and pectoralis muscle and exits on the anterior chest wall (figure 20). The site of the esophagostomy should facilitate easy application of the ostomy appliance on a relatively flat surface. The esophagostomy should be placed in a lateral position that will not interfere with a tracheostomy, should one be necessary.

●A 1 to 2 cm disc of skin is excised at the distal point of the tunnel to prevent stricture formation. The proximal end of the esophagus is drawn through the tunnel and secured full thickness to the skin with interrupted absorbable sutures (figure 21).

●The neck incision is irrigated with isotonic saline and closed. A drain is placed from the neck into the superior mediastinum to assist in drainage of mediastinal contamination.

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 [34]. (See "Nutrition support in intubated critically ill adult 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 can be accomplished with a retrosternal gastric conduit. Retrosternal colonic interposition is an alternative should the stomach not be suitable [35-37].

Surgical-endoscopic hybrid procedures — Although open surgical intervention has traditionally been the standard treatment of esophageal perforation, a gradual shift toward less invasive treatment has occurred over the last decade [38]. In a 2015 meta-analysis, surgery was the primary treatment in less than half of the patients [39].

In contemporary practice, endoscopic therapy is accepted as the primary treatment for not only endoscopy-related esophageal perforation as discussed above, but also spontaneous perforation, anastomotic leak, and chronic fistulae [38]. (See 'Endoscopy-related perforation' above.)

Due to a lack of high-quality comparative studies, it is not possible to recommend endoscopic over surgical therapy, and vice versa. Nevertheless, at facilities where endoscopy therapy for esophageal perforation is available, it should be considered in stable patients with contained perforation. Patient instability, clinical deterioration, as well as peritonitis or pleural contamination indicating uncontained perforation remain strict indications for emergency surgical intervention, in which case endoscopic therapy should not be attempted [15].

When endoscopy therapy is used as primary treatment for esophageal perforation, the liberal use of open and percutaneous drainage procedures in conjunction with endoscopic therapy is a key concept in the successful management of these patients. As an example, when endoscopic stenting is used with open drainage for patients with advanced mediastinal sepsis, it is referred to as a hybrid procedure.

For high-risk patients with clinical evidence of mediastinal sepsis, significant comorbid illness, and large thoracic esophageal perforations, the author utilizes a hybrid technique that includes aggressive debridement, drainage, muscle flap coverage of the defect, and endoscopic stent placement. 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 reestablish continuity of the esophagus. Alternatively, a T-tube may be inserted into the perforation and exteriorized through the chest wall to a drainage bag to create a controlled fistula when a patient cannot tolerate more extensive surgery [36,37,40]. Wide drainage around the site remains essential in this circumstance. (See 'Endoscopic stent placement' above.)

Postoperative management — This approach is used to manage patients with an esophageal perforation at any site:

●Nutritional support is necessary until oral feedings can be initiated and effectively sustained. The patient remains NPO for approximately seven days. Jejunal tube feedings are started on postoperative day 2 or 3 in stable patients without evidence of an ileus.

●If a jejunostomy feeding tube was not inserted at the time of the repair of the perforation, a central line can be inserted for administration of parenteral nutrition when a prolonged period of delay of enteral feedings is anticipated. Generally, this is not indicated in previously well-nourished patients with iatrogenic injuries and minimal contamination.

●The patient is maintained on intravenous broad-spectrum antibiotics typically for 7 to 10 days, depending on clinical status.

●A contrast esophagram is obtained on postoperative day 7 if the patient is clinically stable. If there is no evidence of an esophageal leak or postoperative ileus, the nasogastric tube is removed and oral feedings are initiated.

●Drains remain in place until patients are tolerating oral feedings and without clinical evidence of a leak.

OUTCOMES

Mortality — Most published data on esophageal perforation consist of small single-center retrospective series. Even though those studies originated from centers of expertise, the mortality rate remains relatively high at 12 to 13 percent according to two meta-analyses [41,42]. The mortality rates from population studies are even higher, ranging from 14 to 30 percent at 30 days, and from 22 to 39 percent at 90 days [43-46]. Furthermore, the mortality and morbidity rates of esophageal perforation have not changed significantly over time [43,46,47] except in England [45].

The most common cause of death is sepsis leading to multiorgan failure [31]. 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). A lower mortality rate is associated with:

●Benign versus cancer-related perforation [43,44,46].

●Traumatic versus iatrogenic or spontaneous perforation (7 versus 19 versus 36 percent, respectively) [2,48]. 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.

●Cervical versus thoracic or abdominal perforation (6 versus 27 versus 21 percent, respectively) [2,41,48]. 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).

●Early versus delayed diagnosis (14 versus 27 percent, respectively) [2,41,42,49].

●Surgical repair has been associated with a lower mortality rate than other treatment modalities in most [43,49], but not all [41], studies. The discrepancy is most likely due to selection bias.

Morbidities — Major complication rate was 30 to 55 percent, with a 19 percent failure to rescue (death after major complication) rate at 90 days [43,49]. Complications are more frequent and more severe with surgical than nonsurgical treatment.

The most common complication is pneumonia (50 percent) [43]. Other frequently reported complications include persistent leak, fistula formation, mediastinitis, empyema, esophageal stricture, abscess, mediastinitis, and sepsis [31,32,50-52].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Gastrointestinal perforation" and "Society guideline links: Esophagectomy".)

SUMMARY AND RECOMMENDATIONS

●Etiology – More than half of esophageal perforations are iatrogenic (eg, endoscopic or surgical procedures). Other causes include spontaneous/Boerhaave syndrome (15 percent), foreign body ingestion (12 percent), and trauma (9 percent). (See 'Etiology' above.)

●Presentation – Esophageal perforation is a diagnostic and therapeutic challenge because of the rarity of the condition and the variability in presentation. (See 'Presentation' above.)

●Diagnosis – Esophageal perforation is usually diagnosed radiographically. Contrast-enhanced CT of the chest and abdomen is the imaging test of choice. (See 'Diagnosis' above.)

●Initial management – Prompt diagnosis and management are critical to minimizing mortality. Initial management includes nil per os (NPO), intravenous fluid, antibiotics, and intensive care unit monitoring. For select high-risk patients, we also suggest adding antifungal coverage (Grade 2C). (See 'Initial management' above.)

●Definitive management – Definitive management of an esophageal perforation can be achieved surgically, endoscopically, or with conservative/medical management. The appropriate treatment is selected based on the etiology of the perforation and patient condition (algorithm 1):

•Endoscopy-related perforations – For endoscopy-related esophageal perforation, we suggest endoscopic treatment (Grade 2C). Common techniques include endoscopic primary closure with clips or suturing devices, endoscopic stenting, and endoscopic vacuum therapy (EVAC/EVT). (See 'Endoscopy-related perforation' above.)

•Nonendoscopy-related perforations – Nonendoscopy-related esophageal perforations can be managed either nonoperatively or surgically.

-Low-risk perforation in stable patients – For clinically stable patients with no evidence of systemic inflammation, expediently diagnosed perforations, and no drainage of any collection into the pleura or peritoneum, we suggest nonoperative management (Grade 2C). Endoscopic repair may be attempted for small perforations that present early. (See 'Low-risk perforation in stable patients' above.)

-High-risk perforation or unstable patients – Those who do not meet the criteria for nonoperative management require surgical repair, or a surgical-endoscopic hybrid procedure. (See 'High-risk perforation or unstable patients' above.)

●Surgical techniques

•Preferred surgical technique – For perforations of the thoracic and abdominal esophagus, as well as for visualized perforations of the cervical esophagus (algorithm 2), we suggest a primary repair when feasible (Grade 2C). (See 'Preferred surgical technique' above.)

•Alternative surgical techniques – An alternative technique may be required when primary repair is not feasible for anatomic or clinical reasons (algorithm 2). (See 'Alternative surgical techniques' above.)

-Drainage – When cervical perforations cannot be visualized on surgical exploration, we perform a drainage procedure when there is no distal obstruction, or an esophagectomy when there is a distal obstruction. (See 'Drainage only' above.)

-Esophagectomy – For thoracic perforations with extensive esophageal damage that precludes repair or distal esophageal obstruction due to either end-stage benign esophageal disease (eg, achalasia or undilatable stricture) or cancer, an esophagectomy is required in patients who can tolerate that procedure.

Following esophagectomy, patients who are hemodynamically stable and have minimal extraluminal contamination may undergo reconstruction when there is <24-hour delay between perforation and intervention. Otherwise, diversion is a more prudent option. (See 'Esophagectomy with reconstruction' above and 'Esophagectomy with diversion' above.)

-Hybrid procedure – For high-risk patients with clinical evidence of mediastinal sepsis, significant comorbid illness, and large thoracic esophageal perforations, an alternative to esophagectomy is a hybrid technique that includes aggressive debridement, drainage, muscle flap coverage of the defect, and endoscopic stent placement. (See 'Surgical-endoscopic hybrid procedures' above.)

●Outcomes – Esophageal perforation is associated with a high mortality rate, especially in those with a malignant perforation, thoracic perforation, spontaneous perforation, or delayed diagnosis. Surgical repair has been associated with a lower mortality but a higher morbidity rate compared with other treatment modalities. (See 'Outcomes' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Carolyn Jones, MD, who contributed to an earlier version of this topic review.