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Esophageal Rupture

Author: Dale K Mueller, MD; Chief Editor: Mary C Mancini, MD, PhD more...

Overview
Workup
Treatment

Updated: Sep 5, 2008

Background
Epidemiology
Etiology
Pathophysiology
Presentation
Indications
Relevant Anatomy
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[ ] References
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Background
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." Over the next 16 hours,
his condition progressively worsened 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, although 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 cervical (27%), intrathoracic (54%), and 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 include postemetic (16%) and trauma, including
postoperative trauma (11%). All other causes (caustic, peptic ulcer disease, foreign body,
aortic pathology, and diseases of the esophagus) occur rarely, with a frequency of
approximately 1%.
Esophagogastroduodenoscopy is the most common procedure instrumenting the esophagus.
Risk of perforation with diagnostic esophagogastroduodenoscopy is extremely low (0.03%).
The risk of perforation is increased when therapeutic procedures are performed at the time of
endoscopy. Risk increases 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.

Etiology
Causes of esophageal rupture may include the following:

Iatrogenic (eg, diagnostic and therapeutic endoscopy, passage of nasogastric tubes,


transesophageal echocardiography, endotracheal tube misplacement, chiropractic
manipulation[4] )
Postemetic (spontaneous)
Diseases of the esophagus such as esophagitis, esophageal ulcer, paraesophageal
hernia, Zollinger-Ellison syndrome, and esophageal neoplasm
Postoperative (eg, esophagectomy, aortic aneurysm surgery, thoracotomy,
fundoplications)
Caustic injury
Foreign body[5]
Neck hyperextension
Aortic pathology (eg, aortic aneurysm)

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.[6] 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.[7, 8, 9, 6, 10, 11] 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
History
The classic presentation of spontaneous esophageal rupture is in a middle-aged man with a
history of dietary overindulgence and overconsumption of alcohol, with chest pain and
subcutaneous emphysema after recent vomiting or retching (Mackler triad). 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.[10]
Physical signs include the following:

Fever
Crepitus
Tachycardia
Tachypnea
Cyanosis
Dyspnea
Upper abdominal rigidity
Shock

Local tenderness

The classic Mackler triad, which includes vomiting, lower chest pain, and cervical
subcutaneous emphysema, is present in approximately 50% of cases.
The Anderson triad also refers to subcutaneous emphysema, rapid respirations, and
abdominal rigidity.

Indications
Controversy exists regarding indications for surgery for esophageal rupture; however,
operative therapy depends on a number of factors, including etiology, location of the
perforation, and the time interval between injury and diagnosis.[9] 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
Lack 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 be conservative, 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 tract, and its configuration is flat in the
upper and middle portion and rounded in the lower portion. It has no mesentery or serosal
coating, which is a unique feature of this portion of the gastrointestinal tract. 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 other
organs in the gastrointestinal tract. It consists of four layers: external fibrous layer,
intermediate muscular layer, intermediate submucosal layer, and internal mucosal layer.

Laboratory Studies

Diagnosis depends on a high index of clinical awareness and relies on confirmatory


radiographic findings. However, order lab tests to establish baselines and to help with
follow-up care.
Complete blood count (CBC): Evidence of leukocytosis is commonplace for almost
all esophageal perforations.
pH level: Esophageal perforations with penetrance into the pleural cavity have pH
levels less than 7.2.

Imaging Studies

Although findings may not include significant results if taken early, order urgent
posteroanterior and lateral chest and upright abdominal radiographs (diagnostic in
90% of cases) to look for the following conditions:
o Hydrothorax (usually on the left)
o Hydropneumothorax
o Pneumothorax
o Pneumomediastinum
o Subcutaneous emphysema
o Mediastinal widening without emphysema
o Subdiaphragmatic air
o Pleural effusions (These are more common on the left but can occur bilaterally
and, rarely, only of the right.)
Gastrografin (water-soluble contrast) and/or barium esophagram following plain
radiography may be performed to look for extravasation of contrast and location and
extent of rupture/tear (see image below). Twenty-two percent of patients considered
to have a strong likelihood of esophageal perforation whose water-soluble contrast
studies reveal negative results are found to have esophageal perforation on barium

contrast studies.
Water-soluble contrast esophagram from
a patient with esophageal perforation after esophageal dilation shows contrast leak
(arrowheads) and normal esophageal lumen (arrows).
CT scanning may be performed if contrast esophagraphy cannot be performed, cannot
localize rupture, or is nondiagnostic.[12] If the patient has been sedated, delay contrast
studies pending the return of the gag reflex. Look for the following signs:
o Air in the soft tissue of the mediastinum surrounding the esophagus
o Abscess cavities in the pleural space/mediastinum
o Communication of the esophagus with mediastinal fluid collections
For more information on imaging of this condition, see eMedicine Radiology article
Esophagus, Tear.

Other Tests

Other tests depend on the results of esophagram.


o MRI, CT scanning, or both may be indicated for dissection of aorta.
o Ventilation/perfusion (V/Q) and/or CT scanning of the lungs may reveal
pulmonary embolism.
o ECG may exclude myocardial infarction or associated cardiac abnormalities.

Diagnostic Procedures

Esophagogastroduodenoscopy is not recommended for acute esophageal rupture.


Thoracentesis, 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.

Medical Therapy

Standard therapy includes the following:[9, 13, 14]


o Admission to medical/surgical ICU
o Nothing by mouth
o Parenteral nutritional support
o Nasogastric suction
o Broad-spectrum antibiotics
o Narcotic analgesics
Features that support conservative therapy include the following:[15]
o Absence of clinical signs of infection
o Contained perforation in the mediastinum and the visceral pleura without
penetration to another body cavity
o Perforation draining back into the esophagus
Criteria for nonoperative treatment include the following:
o Recent iatrogenic perforation or late iatrogenic or postemetic esophageal
perforation
o Intrathoracic perforation
o Absence of sepsis
o Medical contraindications to surgery (eg, severe emphysema, severe coronary
artery disease)
o Isolation of the leak within the mediastinum or between the mediastinum and
visceral pleura (no extravasation of contrast into adjacent body cavities)
o No malignancy, obstruction, or stricture in the region of the perforation
o Minimal symptoms
o 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
be conservative, tube thoracostomy (drainage), repair, or diversion.
o Drainage of perforation into the esophagus

Medication

Drug Category: 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 done when the initial diagnosis is suspected.

Surgical Therapy
Surgical techniques used for esophageal rupture include the following:

Tube thoracostomy (Drainage with a chest tube or operative drainage alone)


Primary repair
Primary repair with reinforcement with pleura, intercostal muscle, diaphragm,
pericardial fat, pleural flap[16]
Diversion
Diversion and exclusion
Esophageal resection
Thoracoscopic repair[2]
Esophageal stent[17, 18]
Endoscopic placement of fibrin sealant[19]

Follow-up
Further inpatient care (conservative management)

Consider early surgical repair when indicated because delayed repair (>24 hours) may
alter the surgical approach and increases the mortality rate.
Maintain nasogastric suction until evidence exists that esophageal perforation has
healed, is smaller, or is unchanged.
Deterioration in a patient's condition should prompt consideration of surgery, the need
for which may be confirmed by contrast esophagrams to look for leakage or CT scans
to detect an abscess.

Transfer

Transfer patients from hospitals without an experienced thoracic surgeon to a hospital


with an experienced surgical team.

Complications

Mediastinitis
Intrathoracic abscess
Sepsis
Respiratory failure
Shock

Outcome and Prognosis


Esophageal perforation remains a highly morbid condition with a high mortality rate if not
diagnosed and treated promptly. Mortality rates are reported from 5-89%, based

predominantly on time of presentation and etiology of perforation. Postemetic perforation has


a higher reported mortality rate of 2% per hour and an overall mortality of 25-89%, while
iatrogenic instrumental perforation has a lower mortality of 5-26%. If treatment is instituted
within 24 hours of symptoms, reported mortality rates are 25%; rates rose to above 65% after
24 hours and 75-89% after 48 hours. The mortality rates are higher in patients with delayed
presentation or treatment, thoracic/abdominal rupture, spontaneous rupture, and underlying
esophageal disease.

Future and Controversies


Controversy exists regarding indications for surgery for esophageal rupture.[7, 9, 20, 21, 15, 22]
However, operative therapy depends on a number of factors, including etiology, location of
the perforation, and the time interval between injury and diagnosis. Other considerations
include the extension of the perforation into an adjacent body cavity and the general medical
condition of the patient. Currently, no randomized trials exist for the appropriate treatment of
esophageal perforation in regard to this controversy; therefore, future studies could be
considered.