Practice Essentials
Almost 300 years ago, Herman Boerhaave, a Dutch physician, described the case of Baron Jan von Wassenaer, the Grand Admiral of Holland. [1, 2] In 1724, Boerhaave was called to the bedside of the admiral, who complained of severe chest pain and exclaimed that something had burst in his chest. The admiral had consumed a huge meal, had taken a self-prescribed emetic, and "shortly afterwards he vomited, but only a little and this not easily."
The Grand Admiral's condition progressively worsened over the following 16 hours, until he died. Autopsy revealed a rent in an otherwise normal-looking esophagus, with food and medicine in the left chest cavity. Spontaneous esophageal rupture then became known as Boerhaave syndrome. [3]
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:
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Clinical instability with sepsis
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Recent postemetic perforation
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Intra-abdominal perforation
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Absence of medical contraindications for surgery (eg, severe emphysema or severe coronary artery disease)
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Leak outside the mediastinum (ie, extravasation of contrast into adjacent body cavities)
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Malignancy, obstruction, or stricture in the region of the perforation
Features that support conservative therapy include the following:
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Absence of clinical signs of infection
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Contained perforation in the mediastinum and the visceral pleura without penetration to another body cavity
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Perforation draining back into the esophagus
Criteria for nonoperative treatment include the following:
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Recent iatrogenic perforation or late iatrogenic or postemetic esophageal perforation
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Intrathoracic perforation
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Absence of sepsis
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Medical contraindications for surgery (eg, severe emphysema or severe coronary artery disease)
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Isolation of the leak within the mediastinum or between the mediastinum and visceral pleura (no extravasation of contrast into adjacent body cavities)
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No malignancy, obstruction, or stricture in the region of the perforation
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Minimal symptoms
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Drainage of perforation into the esophagus
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:
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External fibrous layer
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Intermediate muscular layer
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Intermediate submucosal layer
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Internal mucosal layer
Pathophysiology
Because the esophagus lacks a serosal layer, it is more vulnerable to rupture or perforation. [4] Once a perforation (ie, full-thickness tear in the wall) 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. [5]
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%).
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:
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Postemetic - 16%
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Trauma, including postoperative trauma - 11%
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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:
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Esophageal dilation - 0.5%
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Esophageal dilation for achalasia - 1.7%
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Endoscopic thermal therapy - 1-2%
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Endoscopic variceal sclerotherapy - 1-6%
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Endoscopic laser therapy - 5%
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Photodynamic therapy - 4.6%
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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). [6] 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.
Epidemiology
The frequency of esophageal perforation is 3 in 100,000 in the United States. The distribution by location is as follows:
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Cervical - 27%
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Intrathoracic - 54%
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Intra-abdominal - 19%
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%).
Mortality figures have varied according to the time interval between the appearance of symptoms and the institution of treatment. [7, 8, 9, 5, 10, 11] If treatment is instituted within 24 hours of symptoms, the reported mortality is 25%; this rate rises to more than 65% after 24 hours and to 75-89% after 48 hours. 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. [12]
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. [13] 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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Water-soluble contrast esophagogram from patient with esophageal perforation after esophageal dilation shows contrast leak (arrowheads) and normal esophageal lumen (arrows).
