Surgical management of esophageal perforation

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:

●Rapid diagnosis

●Appropriate hemodynamic monitoring and support

●Antibiotic therapy

●Restoration of luminal integrity when feasible

●Control of extraluminal contamination

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 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) [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 "Complications of endoscopic esophageal stricture dilation", 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

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) [2]. The diagnostic studies performed to confirm the clinical suspicion of esophageal perforation are discussed in detail elsewhere. (See "Boerhaave 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 where the clinical suspicion is low. This often leads to the evaluation of more common medical conditions such as myocardial infarction, pneumonia, and peptic ulcer disease. (See "Evaluation of the adult with chest pain of esophageal origin" and "Evaluation of the adult with chest pain in the emergency department".)

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 for operative management, including laboratory evaluation (eg, complete blood count [CBC], coagulation studies, type and screen) and a chest radiograph (image 3).

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. (See "Overview of esophageal injury due to blunt or penetrating trauma in adults", section on 'Clinical features and diagnosis'.)

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 more 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 reapproximated, an esophageal malignancy, preexisting end-stage benign esophageal disease (eg, achalasia), or if 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 repair is performed when, in the judgment of an experienced surgeon, the closure can heal.

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):

●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.

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 [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 [15]. 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) [1,15]:

●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, 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.

●The repair is then buttressed with a pedicled flap (eg, intercostal muscle flap) utilizing interrupted absorbable sutures. An alternative to the intercostal muscle flap (figure 6) is the parietal pleura flap if the intercostal muscle is not available or the intercostal blood supply has been compromised (figure 14).

●The wound is copiously irrigated and drained utilizing two 28 to 32 French chest tubes. One chest tube is positioned in proximity to the site of injury to ensure adequate drainage if the repair breaks down and the second posterior to the repair.

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

●A nasogastric tube is guided past the site of repair and into the stomach, taking care to avoid damaging the repair site.

●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 [16]) 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. 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:

●Nutritional support is necessary until oral feedings can be initiated and effectively sustained. The patient remains nil per os (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 the 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.

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 an intervention. As an example, severe mediastinitis associated with extraesophageal tissue friability and necrosis from a delay in diagnosis can preclude a primary repair [2]. 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 suitable for selected patients. (See 'Nonoperative management' below.)

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 spaces (ie, pleura, peritoneum).

For 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 (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 reestablish 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 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 (see "Enteral feeding: Gastric versus post-pyloric")

●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. 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 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 must allow for uncomplicated application of the ostomy appliance. 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 [20]. (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 requires a retrosternal colon interposition [17,18,21].

Endoscopic stent placement — Esophageal endoscopic covered stents can be used for the management of an esophageal perforation in selected patients [22-26].

Stents may be appropriate for patients with extensive comorbidities, advanced mediastinal sepsis, or large esophageal defects, or for a patient's inability to tolerate more extensive surgery. 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" [27].

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 [28]:

●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

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 "Endoscopic stenting for palliation of malignant esophageal obstruction", section on 'Types of stents'.)

The author performs endoscopic stent placement for an esophageal perforation in the operating room with fluoroscopic guidance. The fundamental technical components 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 a 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. However, it is critical that the distal end of the stent be placed in this location to completely cover and control the distal esophageal perforation.

●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. Stent positioning can be monitored with plain radiographs, particularly when placed in the distal esophagus.

Endoscopic clipping — With the evolution of endoscopic technology, endoscopic clipping has emerged as an alternative means of addressing an esophageal mucosal defect in carefully selected clinical scenarios [29,30]. Experience with this technique has increased significantly with the popularization of peroral endoscopic myotomy (POEM) for the treatment of achalasia. (See "Peroral endoscopic myotomy (POEM)".)

Fundamental principles of endoscopic clipping for treatment of esophageal perforation include:

●Careful patient selection. The defect should be small and have healthy, compliant surrounding mucosa that can be approximated with minimal tension.

●Extraluminal contamination must be drained and debrided.

●Epithelialization associated with chronic fistulas requires ablation. Foreign material at the site of the fistula must be removed.

Endoscopic clipping is generally best suited for iatrogenic injuries with minimal extraluminal contamination. Failure to control extraluminal contamination will result in a significant risk of fistulization. Postprocedure management would be similar to stent management and include 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 vacuum therapy is an evolving technique for the management of esophageal injuries [31]. The procedure should be performed by clinicians familiar with the applications. It is generally best for smaller defects, contained defects with extraluminal spaces, and for patients who cannot tolerate more invasive 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)".)

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:

●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.

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 [12].

The following two retrospective reviews from two different eras report similar findings and illustrate the variables associated with mortality:

●The first review in the era of nutritional support and antibiotics included 450 patients with an esophageal perforation diagnosed between 1980 and 1990 [32]. The mortality rates were dependent upon etiology and location of the perforation.

The mortality rates by etiology were:

•Spontaneous (39 percent)

•Iatrogenic (19 percent)

•Traumatic (9 percent)

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:

•Cervical (6 percent)

•Thoracic (34 percent)

•Intra-abdominal (29 percent)

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).

●The largest retrospective review included 726 patients diagnosed with an esophageal perforation between 1990 and 2003 [2]. The overall mortality rate was 18 percent. Other pertinent results from this review include:

•The overall mortality rate for a diagnosis within 24 hours was significantly lower compared with a greater than 24 hour delay (14 versus 27 percent).

•Primary repair, with or without reinforcement with a flap, offered the highest probability of survival regardless of the interval between perforation and repair. The mortality rate was significantly lower when either a primary or a primary reinforced repair was performed within 24 hours of perforation compared with a repair after 24 hours (4 versus 14 percent).

•A perforation of the thoracic esophagus was associated with the highest mortality rate compared with a perforation of the cervical and intra-abdominal esophagus (27 versus 6 versus 21 percent).

•The mortality rate was higher with a spontaneous rupture of the esophagus compared with an iatrogenic and traumatic rupture (36 versus 19 versus 7 percent).

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, mediastinitis, and sepsis [12,13,33-35].

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 [36-38]. This requires clinicians experienced in the care of esophageal pathology, careful patient monitoring, and 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.

Evidence-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 author utilizes 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, nil per os (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 (eg, fever, tachycardia) require surgical or endoscopic intervention to control extraluminal contamination and restore luminal integrity.

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

●Prompt diagnosis and management is critical to minimizing mortality. A delay of greater than 24 hours in diagnosis and treatment of an esophageal perforation is associated with a higher mortality rate compared with an early diagnosis and treatment initiation (27 versus 14 percent). (See 'Initial management' above.)

●The mortality rate following operative management of an esophageal perforation is dependent on location of the perforation, with cervical perforations having the lowest mortality rate (6 percent) compared with thoracic perforations (27 to 34 percent) and intra-abdominal perforations (21 to 29 percent). (See 'Outcomes following operative management' above.)

●A primary repair is the gold standard of care and should be utilized for perforations of the thoracic and abdominal esophagus, as well as for visualized perforations of the cervical esophagus. (See 'Primary surgical repair' above.)

●Drainage alone should only be performed for perforation of the cervical esophagus when the perforation cannot be visualized and when there is no distal obstruction. (See 'Drainage only' above.)

●Diversion is appropriate when patients are clinically unstable and cannot tolerate a more extensive operative procedure, when patients have preexisting esophageal diseases that preclude repair, or when extensive esophageal damage precludes a primary repair. (See 'Diversion' above.)

●Esophageal stents may be used to treat esophageal perforation in patients with extensive comorbidities, advanced mediastinal sepsis, or large esophageal defects, and in patients who cannot tolerate more extensive surgery (see 'Endoscopic stent placement' above). The stent should be placed by a clinician experienced with this procedure.

●Endoscopic clipping may be used to treat small esophageal perforations with healthy, compliant surrounding mucosa that can be approximated with minimal tension and minimal extraluminal contamination. Following endoscopic clipping, epithelialization associated with chronic fistulas requires ablation, and foreign material at the site of the fistula must be removed. (See 'Endoscopic clipping' above.)

●Esophagectomy should be performed when the patient presents with malignancy, extensive esophageal damage that precludes repair, or end-stage benign esophageal disease. The patient must be hemodynamically stable and have minimal extraluminal contamination; otherwise, diversion is likely a more prudent option. (See 'Esophagectomy' above.)

●Nonoperative management should be reserved for clinically stable patients with no evidence of systemic inflammation, expediently diagnosed perforations, and no drainage of any collection into the pleura or peritoneum. (See 'Nonoperative management' above.)

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