AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Background

In 1724, Dr Hermann Boerhaave described the first, and likely most well known, case of esophageal perforation. Baron von Wassenaer, the Grand Admiral of Holland, followed a large meal with his customary bout of emetic-induced vomiting. However, on this occasion, the Admiral experienced a sudden and severe pain in his upper abdomen after violent but minimally productive retching. Dead less than 24 hours later, his autopsy revealed a transverse tear of his distal esophagus and gastric contents in the pleural spaces. Spontaneous esophageal rupture is a rare and dangerous entity, which today is commonly known as Boerhaave syndrome.

Today, most instances of esophageal perforation are iatrogenic, but this remains a potentially devastating condition. Rapid diagnosis and therapy provide the best chance for survival; however, delay in diagnosis is common, resulting in substantial morbidity and mortality. This article discusses the causes, investigation, and initial therapy for this potentially lethal gastrointestinal condition.

Pathophysiology

The esophagus is more vulnerable than the rest of the alimentary tract due to the lack of a serosal layer, which provides stability through elastin and collagen fibers. Perforation may be due to several mechanisms, including direct piercing, shearing along the longitudinal axis, bursting from radial forces, and thinning from necrosis of the esophageal wall.

Iatrogenic injury through esophageal instrumentation is the leading cause of perforation by either piercing or shearing and may be due to any number of procedures, especially endoscopy and dilatation of strictures. Such tears often occur near the pharyngoesophageal junction where the wall is weakest. Because the esophagus is surrounded by loose stromal connective tissue, the infectious and inflammatory response can disseminate easily to nearby vital organs, thereby making the esophageal perforation a medical emergency and increasing the likelihood of serious sequelae. Underlying esophageal disease (tumor, stricture) predisposes toward perforation with instrumentation, which often occurs distal to the affected area. Perforation during surgery most often occurs in the abdominal esophagus.

Spontaneous esophageal rupture (Boerhaave syndrome) occurs secondary to a sudden increase in intraluminal pressures, usually due to violent vomiting or retching, and often follows heavy food and alcohol intake. In more than 90% of cases, perforation occurs in the lower third of the esophagus; most frequently, the tear is in the left posterolateral region (90%) and may extend superiorly. The predilection for left-side perforation is due to the lack of adjacent supporting structures, thinning of the musculature in the lower esophagus, and anterior angulation of the esophagus at the left diaphragmatic crus. Fifty percent of ruptures occur in patients with gastroesophageal reflux disease, suggesting that ease of pressure transfer from the abdominal to thoracic esophagus may facilitate rupture.

Shearing forces due to rapid increases in intragastric pressure against a closed pylorus result in a Mallory-Weiss tear (MWT). These longitudinal mucosal lacerations occur most commonly at the gastroesophageal junction or gastric cardia, especially if a hiatal hernia is present, and often present with hematemesis. Ultimately, these tears can perforate if the pressure increases are unrelieved. Further discussion of MWTs is reserved for another section.

The cervical esophagus is the most common site of perforation by several other mechanisms as well, particularly in the region of the pyriform sinus. Trauma, almost uniformly penetrating, shows an affinity for the upper esophagus, while toxic ingestions and foreign bodies can directly damage the cervical esophagus or become lodged and cause insidious erosion of the muscle wall.

Frequency

United States

Boerhaave syndrome is rare, accounting for 15% of all cases of esophageal perforation.

Iatrogenic causes account for 50-75% of esophageal perforations. The actual incidence depends on the procedure; rigid endoscopy carries a perforation rate 0.1-0.4%, while that of flexible endoscopy varies from 0.01-0.06%. Rates increase quickly when interventions are undertaken, such as pneumatic balloon dilatation in achalasia (2-6%) or any procedure involving strictures secondary to radiation or tumor (10%). Furthermore, the rate of perforation is increased in the presence of a large hiatal hernia or esophageal diverticula.

Penetrating trauma to the neck (2-9%), thinning of esophageal membrane secondary to variceal sclerotherapy (1-3%), and foreign body or toxic ingestions (5-15%) make up the bulk of the remaining causes.

Mortality/Morbidity

Even with prompt therapy and advances in surgical technique, the mortality rate can be very high, varying from 5-75%; higher rates correlate with delays in both presentation and diagnosis.

• Mortality also varies by etiology and location of the perforation. The highest rates are attributed to Boerhaave syndrome (up to 72%), partly because of the difficulty in making the diagnosis, followed by iatrogenic (19%) and traumatic perforations (7%). Cervical perforations portend a lower mortality compared with abdominal and thoracic perforations due to containment of potential contamination by tight fascial attachments and mechanisms, which may make injury more obvious.
• The morbidity and mortality in esophageal perforation is most often due to an overwhelming inflammatory response to gastric contents in the mediastinum, pleural spaces, and adjoining tissues, as well as swift spread of infection to paraesophageal structures. Negative intrathoracic pressure may draw gastric material out of the esophagus, exacerbating injury.
• Morbidity may be due to pneumonia, mediastinitis, empyema, polymicrobial sepsis, and multiorgan failure.

Race

No information on racial predilection is available.

Sex

Boerhaave syndrome is generally associated with vomiting and customarily occurs after drinking and eating binges. It is more commonly observed in males than in females. Iatrogenic perforation shows no predilection.

Age

• Boerhaave syndrome is most common among patients aged 40-60 years old, but isolated case reports in children have been described.
• Iatrogenic perforations are associated with preexisting pathology and so are more common in fifth and later decades of life.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

History

• The classic presentation of spontaneous esophageal rupture is severe vomiting or retching followed by acute, severe chest or epigastric pain.
• Boerhaave syndrome has also been reported with abdominal or chest pain following straining, childbirth, weight lifting, fits of coughing or laughing, hiccuping, blunt trauma, seizures, and forceful swallowing.
• The presence of fever; pain in the neck, upper back, chest, or abdomen; dysphagia; odynophagia; dysphonia; or dyspnea following esophageal instrumentation should raise suspicion for perforation.
• Patients with thoracic or abdominal perforations may present with any of the above symptoms, as well as low back pain, shoulder pain referred from diaphragmatic irritation, increased discomfort lying flat, or true acute abdomen.
• The ingestion of a caustic toxin or foreign body preceding any of the above symptoms may indicate perforation.
• A history of preexisting upper gastrointestinal pathology (gastroesophageal reflux disease, hiatal hernia, carcinoma, strictures, radiation therapy, Barrett esophagus, varices, achalasia, infection) raises a patient's risk of perforation.
• Hematemesis, while occasionally present, is normally not a predominant symptom.

Physical

• Although the physical examination is often nonspecific, certain findings can be helpful.
• Subcutaneous emphysema is palpable in the neck or chest in up to 60% of perforations but requires at least an hour to develop after the initial injury.
• Tachycardia and tachypnea are common initial physical examination findings, but fever may not be present for hours to days.
• The Mackler triad, consisting of vomiting, chest pain, and subcutaneous emphysema, is classically associated with spontaneous esophageal rupture, though it is only fully present in about 50% of cases.
• Auscultation of the chest can be of particular value.
• • The Hamman sign is a raspy, crunching sound heard over the precordium with each heartbeat caused by mediastinal emphysema, often present with thoracic or abdominal perforations.
  • Breath sounds may be reduced on the side of the perforation due to a contamination of the pleural space, often on the left.
• In cases of delayed presentation, patients may be critically ill and present with significant hypotension.

Causes

• Iatrogenic etiologies predominate the causes of esophageal perforation, accounting for up to 85% of cases.
• • Instrumentation modalities commonly include endoscopy, sclerotherapy, variceal ligation, pneumatic dilation, bougienage, and laser treatment.
  • Placement of endotracheal, nasogastric, and Blakemore tubes represent less common iatrogenic causes.
• As detailed above, Boerhaave syndrome consistently accounts for about 15% of all perforations, normally secondary to vomiting after heavy food and alcohol intake, but possible by any action that abruptly increases intra-abdominal pressure against a closed superior esophageal sphincter.
• Swallowed foreign bodies may directly injure the esophagus by penetrating the tissue or becoming lodged at a point of esophageal narrowing, leading to pressure necrosis and wall weakness; pills and coins are common culprits. Ingestion of caustic chemicals may lead to direct wall inflammation and damage.
• Trauma represents an important cause of perforation, estimated at up to 10% of cases. Penetrating trauma is much far more prevalent than blunt, often in the form of knife or gun wounds, and is associated with significant injury to important adjacent cervical structures. Blunt trauma may affect any portion of the esophagus, and the diagnosis is often delayed secondary to other injuries.
• Intraoperative esophageal perforation is a recognized complication of surgery, especially cardiothoracic or fundoplication, accounting for around 2% of all perforations.

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Other Problems to be Considered

Mallory-Weiss tear

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Lab Studies

• Laboratory studies are of little value in confirming the diagnosis.
• If a diagnostic thoracentesis is performed on a pleural effusion, the presence of food particles, pH <6, or an elevated pleural fluid amylase are compatible with esophageal perforation.

Imaging Studies

• Chest radiographs can be very helpful in diagnosing esophageal perforation, with suggestive abnormalities reported in about 90% of cases.
• • Pneumomediastinum and subcutaneous emphysema are often present an hour after the injury and are highly suggestive of perforation.
  • Mediastinal air-fluid levels, pleural effusions (often left sided), free air under diaphragm, pneumothorax, and hydropneumothorax encompass later potential findings.
  • The V sign classically seen in esophageal perforation is indicative of pneumomediastinum. Air outlines the left lower mediastinal border and medial left hemidiaphragm, forming a "V".¹
• Lateral neck radiographs may demonstrate air in the fascial planes early in cases of cervical perforations.
• A contrast esophagogram should be performed in any patient with suspected perforation.
• • Despite a modest sensitivity (60-75%), a water-soluble contrast agent (Gastrografin) should be the initial study of choice.
  • A barium study should be undertaken immediately afterward should the initial study show no evidence of perforation.
  • Barium has a higher sensitivity (90%) for detecting small perforations but may cause a severe inflammatory response in tissues, most notably a mediastinitis.
  • Studies should be performed with the patient in the right lateral decubitus position.
• A contrast-enhanced CT scan of the chest should be obtained if it is not possible to obtain a contrast esophagogram, if the esophagogram was negative despite a high clinical suspicion, or if seeking to evaluate for a more likely alternative diagnosis. Perforation may be suggested by mediastinal air, extravasated luminal contrast, periesophageal fluid collections, pleural effusions, or actual communication of an air-filed esophagus with an adjacent mediastinal air-fluid collection; definitive esophageal communication with outside structures is often difficult to visualize.

Other Tests

• Esophagoscopy often can be used to visualize perforations and is especially useful in acute traumatic perforations, but it is not appropriate when small mucosal tears are suspected, as insufflated air can cause further dissection of the perforation.

TREATMENT

Section 6 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Prehospital Care

Any patient with an esophageal tear should be expeditiously transported to the emergency department with intravenous access, supplemental oxygen with a secure airway, and pain medication as necessary.

Emergency Department Care

• Consideration of esophageal perforation as a diagnosis is the first and most important step in management. Emergency department treatment of any patient with suspected esophageal perforation depends on the severity of the injury and the patient's hemodynamic stability, but will always include large-bore intravenous access, supplemental oxygen as necessary, and cardiopulmonary monitoring before further treatment is considered.
• Administration of broad-spectrum intravenous antibiotics should be instituted early in the evaluation.
• Patient should be made NPO and have a nasogastric tube placed to clear gastric contents and limit further contamination.
• Patient pain and discomfort may be significant; narcotic analgesia should be given as needed, judiciously in hypotensive patients.
• Patients with tenuous hemodynamic stability or any degree of airway compromise, especially those with Boerhaave syndrome, should undergo treatment in a setting with complete resuscitative facilities, including emergency airway equipment, as clinical decompensation can be precipitous.
• Rarely, tube thoracostomy may be urgently used to decompress the chest. Fluid removed is often gastric contents, occasionally pus, which is often present after significant delay in diagnosis.
• While historically treated exclusively with surgery, emerging evidence indicates that patients with small well-defined tears and minimal extraesophageal involvement may be better served by conservative treatment as outlined above.
• Originally put fourth by Cameron et al in 1979 and modified by Altorjay in 1997², the following represent suggested criteria for nonoperative management: Early diagnosis or delayed diagnosis with contained leak; tear outside abdomen, contained to mediastinum, draining to esophagus; draining to esophageal lumen by esophagography; tear does not involve neoplasm or obstruction; no signs or symptoms of sepsis; experienced thoracic surgeon and contrast imaging available.
• Specific surgical technique (primary repair, stent, resection, or drain placement) depends on the extent and location of injury, and is beyond the scope of this discussion.

Consultations

Obtain an emergent surgical consultation, cardiothoracic if available, as even patients initially managed nonoperatively could require surgery.

MEDICATION

Section 7 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Analgesia and antibiotics are required for management.

Drug Category: Analgesics

Pain control is essential to quality patient care. Ensures patient comfort, promotes pulmonary toilet, and enables physical therapy regimens. Many analgesics have sedating properties that are beneficial to patients who have sustained trauma.

Drug Category: Antiemetics

Useful in treating symptomatic nausea and preventing further contamination of pleural space.

Drug Category: Antibiotics

Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.

FOLLOW-UP

Section 8 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Further Inpatient Care

• Patients almost uniformly require intensive care unit admission because they may become septic and can have a complicated hospital course.
• Patients managed conservatively should take nothing by mouth and be administered parenteral antibiotics for 7-10 days; consideration should be given to intravenous nutrition in the event of a prolonged course. Repeat esophageal studies are utilized to ascertain treatment success.

Transfer

• Patients with Boerhaave syndrome must be treated in a center with access to intensive care and cardiothoracic surgery. Transfer may be required to a tertiary care facility if these services are not available at the presenting hospital.

Complications

• Complications include pneumonia, mediastinitis, sepsis, empyema, and adult respiratory distress syndrome.
• Because of improved management, a significant number of patients now survive; recurrent spontaneous ruptures of the esophagus have been described.
• Esophageal injuries secondary to penetrating trauma often involve adjacent structures such as the spinal cord and trachea.

Prognosis

• The prognosis can be poor, especially if diagnosis is delayed. Even with prompt diagnosis and definitive therapy, the hospital course may be prolonged with high rates of morbidity and mortality.
• The prognosis of cervical iatrogenic perforation is far better than that of spontaneous perforation.

MISCELLANEOUS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Medical/Legal Pitfalls

• With a high index of suspicion, a negative water-soluble contrast study is not sufficient to rule out the diagnosis of esophageal tear.
• While evidence has begun to accumulate regarding specific treatment approaches, this remains a rare condition and, as such, many suggestions are based on case series or personal experience.

Special Concerns

• Early diagnosis is crucial in Boerhaave syndrome. It is important to consider the possibility in any patient presenting with atypical chest or abdominal pain.

ACKNOWLEDGMENTS

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Martin J Carey, MD, to the development and writing of this article.

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

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Article Last Updated: Feb 15, 2008