Esophageal Rupture

Practice Essentials

One of the earliest descriptions of spontaneous esophageal rupture in the medical literature was the fatal case of Baron Jan von Wassenaer, Grand Admiral of Holland, which was reported by the Dutch physician Herman Boerhaave in 1724.^[1]  Spontaneous esophageal rupture subsequently came to be referred to as Boerhaave syndrome. 

Until the middle of the 20th century, many similar uniformly fatal cases were described without full explanation. As technology improved, however, instrumental perforation became more common, and the pathophysiologies of rupture, perforation, and esophageal disruption (anastomotic leak) were elucidated, though the definitions of these entities became blurred. This article discusses adult esophageal rupture.

General recommendations for surgery include the following:

• Clinical instability with sepsis
• Recent postemetic perforation
• Intra-abdominal perforation
• Absence of medical contraindications for surgery (eg, severe emphysema or severe coronary artery disease)
• Leak outside the mediastinum (ie, extravasation of contrast into adjacent body cavities)
• Malignancy, obstruction, or stricture in the region of the perforation

Features that support conservative therapy include the following:

• Absence of clinical signs of infection
• Contained perforation in the mediastinum and the visceral pleura without penetration to another body cavity
• Perforation draining back into the esophagus

Criteria for nonoperative treatment include the following:

• Recent iatrogenic perforation or late iatrogenic or postemetic esophageal perforation
• Intrathoracic perforation
• Absence of sepsis
• Medical contraindications for surgery (eg, severe emphysema or severe coronary artery disease)
• Isolation of the leak within the mediastinum or between the mediastinum and visceral pleura (no extravasation of contrast into adjacent body cavities)
• No malignancy, obstruction, or stricture in the region of the perforation
• Minimal symptoms
• Drainage of perforation into the esophagus

Guidelines for the management of esophageal perforation and other esophageal emergencies have been published by the World Society of Emergency Surgery (WSES).^[2]

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Anatomy

The esophagus is the muscular tube that serves to pass food from the oropharynx to the stomach. It is the narrowest part of the gastrointestinal (GI) tract, and its configuration is flat in the upper and middle portions and rounded in the lower portion.

A unique feature of this portion of the GI tract is that it has no mesentery or serosal coating. The connective tissue in which the esophagus and trachea are embedded is surrounded by long continuous sheaths of fibroareolar laminae that cover and bind together muscles, vessels, and bony constituents of the neck and chest. The arterial blood supply to the esophagus includes the superior and inferior thyroid arteries, direct aortic branches, the left gastric artery, and the splenic artery.

Apart from the lack of a serosal coating, the construction of the esophagus is similar to that of other organs in the GI tract. It consists of the following four layers:

• External fibrous layer
• Intermediate muscular layer
• Intermediate submucosal layer
• Internal mucosal layer

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Pathophysiology

Because the esophagus lacks a serosal layer, it is more vulnerable to rupture or perforation than other portions of the GI tract are.^[3] Once a perforation (ie, full-thickness tear in the wall) of the esophagus occurs, retained gastric contents, saliva, bile, and other substances may enter the mediastinum, resulting in mediastinitis.

The degree of mediastinal contamination and the location of the tear determine the clinical presentation. Within a few hours, a polymicrobial bacterial invasion occurs, which can lead to sepsis and, eventually, death if the patient is not treated with conservative management or surgical intervention.

The mediastinal pleura often ruptures, and gastric fluid is drawn into the pleural space by the negative intrathoracic pressure. Even if the mediastinal pleura is not violated, a sympathetic pleural effusion often occurs. This effusion usually is on the left but can be bilateral. Rarely, isolated right-side effusions occur.

The site of perforation varies depending upon the cause. Instrumental perforation is common in the pharynx or distal esophagus. Spontaneous rupture may occur just above the diaphragm in the posterolateral wall of the esophagus. Perforations are usually longitudinal (0.6-8.9 cm long), with the left side more commonly affected than the right (90%).

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Etiology

The most common cause of esophageal perforation is medical instrumentation for diagnostic and therapeutic endeavors; in one series, such instrumentation caused 65% of all perforations. The frequencies of other causes are as follows:

• Postemetic - 16%
• Trauma, including postoperative trauma - 11%
• All other causes (caustic, peptic ulcer disease, foreign body, aortic pathology, and diseases of the esophagus) - Rare, approximately 1%

Esophagogastroduodenoscopy (EGD) is the most common procedure for instrumentation of the esophagus. The risk of perforation is extremely low (0.03%) with diagnostic EGD but becomes substantially higher when therapeutic procedures are performed at the time of endoscopy. The degree of increased risk varies according to therapeutic procedure being performed, as follows:

• Esophageal dilation - 0.5%
• Esophageal dilation for achalasia - 1.7%
• Endoscopic thermal therapy - 1-2%
• Endoscopic variceal sclerotherapy - 1-6%
• Endoscopic laser therapy - 5%
• Photodynamic therapy - 4.6%
• Esophageal stent placement - 5-25%

In a study including 62 children with benign esophageal strictures who sustained 22 esophageal ruptures after 129 fluoroscopic balloon dilatation procedures, Zhou et al categorized the ruptures as intramural (type 1), transmural (type 2), or transmural with free leakage (type 3).^[4]  Of the 22 ruptures, 21 were type 1 or 2 and were treated conservatively, whereas one was type 3 rupture and was treated surgically with esophagoesophagostomy. The overall rupture rate was 17.1% (22/129), but the rate of rupture that required aggressive treatment (ie, type 3) was only 0.8% (1/129).

Esophageal perforation is rare with nonendoscopic esophageal instrumentation.

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Epidemiology

The frequency of esophageal perforation is 3 in 100,000 in the United States. The distribution by location is as follows:

• Cervical - 27%
• Intrathoracic - 54%
• Intra-abdominal - 19%

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Prognosis

Esophageal perforation remains a highly morbid condition, and if it is not diagnosed and treated promptly, mortality is high (5-89%, depending predominantly on time of presentation and etiology of perforation). Postemetic perforation has a higher reported mortality (2% per hour and 25-89% overall), whereas iatrogenic instrumental perforation has a lower mortality (5-26%). Treatment instituted within 24 hours of symptoms is associated with a lower mortality than treatment instituted after this period.^[5] Mortality is higher in patients with delayed presentation or treatment, thoracic or abdominal rupture, spontaneous rupture, or underlying esophageal disease.

An international study comparing the outcome of endoscopic stent insertion with that of primary operative management for spontaneous rupture of the esophagus found that the former had no advantage over the latter with regard to morbidity, stay in the intensive care unit (ICU), or hospital stay.^[6]

A small retrospective single-institution study (N = 12; median age, 63 y; median Pittsburg perforation severity score, 6.5) by Willems et al described outcomes after hybrid minimally invasive treatment of Boerhaave syndrome.^[7]  Mortality was relatively low, even among patients with high perforation severity scores: 30-day mortality was 17% (n = 2), and in-hospital mortality was 25% (n = 3).

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History

The classic presentation of spontaneous esophageal rupture is that of a middle-aged man with a history of dietary overindulgence and overconsumption of alcohol who experiences chest pain and subcutaneous emphysema after recent vomiting or retching (Mackler triad). The classic Mackler triad is present in approximately 50% of cases.

Typical symptoms include the following:

• Pain of variable location, commonly in the lower anterior chest or upper abdomen
• Vomiting
• Subcutaneous emphysema
• Neck pain
• Dysphagia
• Dyspnea
• Hematemesis
• Melena
• Back pain

Atypical symptoms include the following:

• Shoulder pain
• Facial swelling
• Hoarseness
• Dysphonia

Because spontaneous esophageal rupture is a life-threatening emergency,^[2] clinicians should be aware of its atypical presentations.

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Physical Examination

Physical signs include the following:

• Fever
• Crepitus
• Tachycardia
• Tachypnea
• Cyanosis
• Dyspnea
• Upper abdominal rigidity
• Shock
• Local tenderness

The combination of subcutaneous emphysema, rapid respirations, and abdominal rigidity is commonly referred to as the Anderson triad.

An underrecognized possible presenting feature of spontaneous esophageal rupture is pneumothorax, which may be present in as many as 20% of such cases.^[8]

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Complications

Complications of esophageal rupture include the following:

• Mediastinitis
• Intrathoracic abscess
• Sepsis
• Respiratory failure
• Shock

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Laboratory Studies

Diagnosis of esophageal rupture depends on a high index of clinical awareness and relies on confirmatory radiographic findings. However, laboratory tests (eg, complete blood count [CBC] and pH test) should be ordered to establish baseline values and to help with follow-up care.

Evidence of leukocytosis on the CBC is commonplace for almost all esophageal perforations. Esophageal perforations with penetrance into the pleural cavity have pH levels lower than 7.2.

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Plain Radiography

Although diagnostic images may not yield significant findings if obtained early, posteroanterior and lateral chest and upright abdominal radiographs (diagnostic in 90% of cases) should be obtained on an urgent basis to look for the following conditions:

• Hydrothorax - This will usually be on the left
• Hydropneumothorax
• Pneumothorax
• Pneumomediastinum
• Subcutaneous emphysema
• Mediastinal widening without emphysema
• Subdiaphragmatic air
• Pleural effusions - These are more common on the left but can occur bilaterally and, in rare cases, on the right only

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Contrast Esophagography

Water-soluble contrast (eg, diatrizoate meglumine–diatrizoate sodium) or barium esophagography following plain radiography may be performed to look for extravasation of contrast and to determine the location and extent of the rupture or tear (see the image below). In 22% of patients considered to have a strong likelihood of esophageal perforation whose water-soluble contrast studies reveal negative results, barium contrast studies reveal esophageal perforation.

View Image

Water-soluble contrast esophagogram from patient with esophageal perforation after esophageal dilation shows contrast leak (arrowheads) and normal eso....

If the patient has been sedated, contrast studies should be delayed until the gag reflex has returned. Look for the following signs:

• Air in the soft tissue of the mediastinum surrounding the esophagus
• Abscess cavities in the pleural space/mediastinum
• Communication of the esophagus with mediastinal fluid collections

For more information on imaging of this condition, see Esophagus, Tear.

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Computed Tomography

If contrast esophagography cannot be performed, cannot localize a rupture, or is nondiagnostic, computed tomography (CT) may be performed. A study by Suarez-Poveda found CT esophagography to yield good diagnostic results in the setting of suspected esophageal rupture.^[9]  Multidetector CT (MDCT) is increasingly being used in the setting of esophageal rupture.^[10]  The 2019 World Society of Emergency Surgery (WSES) guidelines for esophageal emergencies stated that contrast-enhanced CT and CT esophagography are the imaging examinations of choice in the setting of suspected esophageal perforation.^[2]  

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Other Tests

Other tests may be considered, depending on the results of prior studies. Magnetic resonance imaging (MRI), CT, or both may be indicated for aortic dissection. Ventilation/perfusion (V/Q) scanning or CT of the lungs may reveal pulmonary embolism. Electrocardiography (ECG) may exclude myocardial infarction (MI) or associated cardiac abnormalities. Shear-wave elastography may prove useful for detecting hemorrhage from esophageal rupture.^[11]

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Procedures

Esophagogastroduodenoscopy (EGD) has not been recommended for acute esophageal rupture. Diagnostic endoscopy can be helpful in cases of suspected perforation when CT findings are inconclusive; although it is reliable and safe in experienced hands, its use as a first-line method is limited by its potential risks.^[2]

Thoracocentesis, though rarely needed, may reveal acidic pH, elevated salivary amylase, purulent malodorous fluid, or the presence of undigested food in pleural aspirate, which help confirm the diagnosis.

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Approach Considerations

Controversy exists regarding indications for surgery for esophageal rupture.^{[12, 13]} In general, however, operative therapy depends on a number of factors, including etiology, location of the perforation, and the time interval between injury and diagnosis.^[14]  Early diagnosis (< 24 hr) is associated with improved clinical outcome.^[5]

Other considerations include the extension of the perforation into an adjacent body cavity and the general medical condition of the patient. A retrospective study by Luan et al suggested that operative management is preferable to nonoperative management for low- and medium-risk patients with spontaneous esophageal rupture but may not have clear advantages for high-risk patients.^[12]

General recommendations for surgery include the following:

• Clinical instability with sepsis
• Recent postemetic perforation
• Intra-abdominal perforation
• Absence of medical contraindications for surgery (eg, severe emphysema or severe coronary artery disease)
• Leak outside the mediastinum (ie, extravasation of contrast into adjacent body cavities)
• Malignancy, obstruction, or stricture in the region of the perforation

Some authors believe that if treatment is instituted more than 24 hours after the perforation, the mode of treatment does not influence the outcome and can consist of conservative therapy, tube thoracostomy (drainage), repair, or diversion.

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Medical Therapy

Standard medical therapy for esophageal rupture includes the following:

• Admission to a medical or surgical intensive care unit (ICU)
• Nothing by mouth
• Parenteral nutritional support
• Nasogastric suction - This should be maintained until there is evidence to indicate that the esophageal perforation has healed, is smaller, or is unchanged
• Broad-spectrum antibiotics - No randomized clinical trials exist for antibiotics and esophageal perforation; however, empiric coverage for anaerobic and both gram-negative and gram-positive aerobes should be initiated when the initial diagnosis is suspected
• Narcotic analgesics

Features that support conservative therapy include the following^[15] :

• Absence of clinical signs of infection
• Contained perforation in the mediastinum and the visceral pleura without penetration to another body cavity
• Perforation draining back into the esophagus

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Surgical Therapy

Deterioration of a patient's condition should prompt consideration of surgery, the need for which may be confirmed by contrast esophagography to look for leakage or computed tomography (CT) to detect an abscess.

If the institution does not have an experienced thoracic surgeon, the patient should be transferred to a hospital with an experienced surgical team.

Surgical techniques reported to have been used for esophageal rupture include the following:

• Tube thoracostomy (drainage with a chest tube or operative drainage alone)^[16]
• Primary repair^{[17, 16]}
• Primary repair with reinforcement with pleura, intercostal muscle, diaphragm, pericardial fat, pleural flap^[18]
• Diversion
• Diversion and exclusion
• Esophageal resection^{[19, 16]}
• Thoracoscopic repair^{[20, 21]}
• Esophageal stenting^{[22, 23, 24, 25, 26]}
• Endoscopic placement of fibrin sealant^[27]
• Endoscopic suture ligation^{[28, 29]}
• Endoscopic negative-pressure (vacuum) therapy^[30]

A multicenter retrospective study (N = 57; median age, 68 y; 41 male, 16 female) by Wannhoff et al compared endoscopic vacuum therapy (EVT; n = 25) with surgery (n = 14) and endoscopic stenting (n = 15) as primary treatment of Boerhaave syndrome.^[31]  Primary EVT yielded a higher success rate (80.0%) than the two non-EVT treatments (43.8%), as well as a lower mortality (8.0% vs 25.0%).

A small (N = 7) single-center case-series study from Veltri et al reported successful treatment of Boerhaave syndrome with laparoscopic transhiatal suture and gastric valve (rather than the more common thoracic approach).^[32]

Early surgical repair should be considered when indicated because delayed repair (>24 hr) may alter the surgical approach and increases mortality.

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