Esophageal Rupture

Author: Dale K Mueller, MD

Almost 300 years ago, Herman Boerhaave, a Dutch physician, described the case of Barron
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.

Epidemiology
Frequency
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%

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 with diagnostic EGD is extremely low (0.03%). The

risk of perforation is increased, however, when therapeutic procedures are performed at the
time of endoscopy. The degree of increased risk varies with the 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%

Esophageal perforation is rare with nonendoscopic esophageal instrumentation.

Pathophysiology
The esophagus lacks a serosal layer and is, therefore, more vulnerable to rupture or
perforation. 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 invasion of bacteria supervenes, which can
lead to sepsis and, eventually, death if the patient is not treated with conservative
management or surgical intervention.[4] 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
is usually left-sided but can be bilateral. Rarely, isolated right-sided 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%).
Esophageal perforation remains a highly morbid condition. Mortality rates are reported from
25-89% and are based predominantly on time of presentation and etiology of perforation.
Postemetic perforation has a higher reported mortality; it has been reported to occur at 2%
per hour. Mortality rates have varied depending on the time from symptomology until
treatment was instituted.[5, 6, 7, 4, 8, 9] If treatment is instituted within 24 hours of symptoms,
mortality rates are 25%; rates rose to above 65% after 24 hours and 75-89% after 48 hours.

Presentation

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 shoulder pain, facial swelling, hoarseness, and dysphonia.
Because spontaneous esophageal rupture is a life-threatening emergency, clinicians should be
aware of its atypical presentations.[8]
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.[10]

Indications
Controversy exists regarding indications for surgery for esophageal rupture. 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.[7, 11] Other considerations
include the extension of the perforation into an adjacent body cavity and the general medical
condition of the patient.
General recommendations for surgery include the following:

Clinical instability with sepsis

Recent postemetic perforation

Intra-abdominal perforation

Absence of medical contraindications to surgery (eg, severe emphysema, 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.

Relevant 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 portion 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, left gastric artery,
and 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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