Continually Updated Clinical Reference
 
 
  All Sources     eMedicine     Medscape     Drug Reference     MEDLINE
 
eMedicine - Esophageal Rupture : Article by

Quick Find
Authors & Editors
Introduction
Indications
Relevant Anatomy
Workup
Treatment
Complications
Outcome and Prognosis
Future and Controversies
Multimedia
References




Patient Education
Click here for patient education.



Author: Dale K Mueller, MD, Section Chief, Department of Surgery, University of Illinois at Peoria, Co-Medical Director, Thoracic Center of Excellence, Vice-Chair, Department of Cardiovascular Medicine and Surgery, OSF St Francis Medical Center

Dale K Mueller is a member of the following medical societies: American College of Chest Physicians, American College of Surgeons, American Medical Association, Chicago Medical Society, Illinois State Medical Society, and Society of Thoracic Surgeons

Coauthor(s): Yogesh Govil, MD, MRCP, Consulting Staff, Department of Internal Medicine, Division of Gastroenterology, Crozer-Chester Medical Center; Thomas E Kowalski, MD, Assistant Professor, Department of Medicine, Director, Gastrointestinal Endoscopy Unit, Thomas Jefferson University, Consulting Staff, Thomas Jefferson University Hospital; Jeffrey C Milliken, MD, Chief, Division of Cardiothoracic Surgery, University of California at Irvine Medical Center; Clinical Professor, Department of Surgery, University of California at Irvine School of Medicine

Editors: Benson B Roe, MD, Emeritus Chief, Division of Cardiothoracic Surgery, Emeritus Professor, Department of Surgery, University of California at San Francisco Medical Center; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Shreekanth V Karwande, MBBS, Chair, Professor, Department of Surgery, Division of Cardiothoracic Surgery, University of Utah School of Medicine and Medical Center; Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy; Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center

Author and Editor Disclosure

Synonyms and related keywords: Boerhaave syndrome, iatrogenic rupture, esophagus, esophageal perforation, esophageal disruption, anastomotic leak, esophagitis, esophageal ulcer, esophageal neoplasm, necrotizing mediastinitis, mediastinal emphysema, hydropneumothorax, sepsis, Mackler triad, endoscopy, esophagogastroduodenoscopy

Two hundred seventy-six years ago, Herman Boerhaave, a Dutch physician, described the case of Barron Wassenaer, the Grand Admiral of Holland. 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.

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.

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)
  • 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
  • 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. 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. 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.

Clinical

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.

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.



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. 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.



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.



Lab 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:

    • Hydrothorax (usually 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, 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. 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.
  • CT scanning may be performed if contrast esophagraphy cannot be performed, cannot localize rupture, or is nondiagnostic. If the patient has been sedated, delay contrast studies pending the return of the gag reflex. 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

Other Tests

  • Other tests depend on the results of esophagram.

    • MRI, CT scanning, or both may be indicated for dissection of aorta.
    • Ventilation/perfusion (V/Q) and/or CT scanning of the lungs may reveal pulmonary embolism.
    • 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:

    • Admission to medical/surgical ICU
    • Nothing by mouth
    • Parenteral nutritional support
    • Nasogastric suction
    • Broad-spectrum antibiotics
    • Narcotic analgesics
  • 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 to surgery (eg, severe emphysema, 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
    • 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.
    • 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
  • Diversion
  • Diversion and exclusion
  • Esophageal resection
  • Thoracoscopic repair
  • Esophageal stent
  • Endoscopic placement of fibrin sealant

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.



  • Mediastinitis

  • Intrathoracic abscess

  • Sepsis

  • Respiratory failure

  • Shock



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.



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. 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.



Media file 1:  Water-soluble contrast esophagram from a patient with esophageal perforation after esophageal dilation shows contrast leak (arrowheads) and normal esophageal lumen (arrows).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  X-RAY



  • Anderson RL. Spontaneous rupture of the esophagus. Am J Surg. Feb 1957;93(2):282-90. [Medline].
  • Bladergroen MR, Lowe JE, Postlethwait RW. Diagnosis and recommended management of esophageal perforation and rupture. Ann Thorac Surg. Sep 1986;42(3):235-9. [Medline].
  • Bobo WO, Billups WA, Hardy JD. Boerhaave's syndrome: a review of six cases of spontaneous rupture of the esophagus secondary to vomiting. Ann Thorac Surg. 1969;172:1034-1038.
  • Bradley SL, Pairolero PC, Payne WS, Gracey DR. Spontaneous rupture of the esophagus. Arch Surg. Jun 1981;116(6):755-8. [Medline].
  • Brewer LA, Carter R, Mulder GA, Stiles QR. Options in the management of perforations of the esophagus. The American Journal of Surgery. Jul 1986;152:62-69. [Medline].
  • Brown RH Jr, Cohen PS. Nonsurgical management of spontaneous esophageal perforation. JAMA. Jul 14 1978;240(2):140-2. [Medline].
  • Cameron JL, Kieffer RF, Hendrix TR. Selective nonoperative management of contained intrathoracic esophageal disruptions. Ann Thorac Surg. 1979;27:404-408.
  • Curci JJ, Horman MJ. Boerhaave's syndrome: The importance of early diagnosis and treatment. Ann Surg. Apr 1976;183(4):401-8. [Medline].
  • DeMeester TR. Perforation of the esophagus. Ann Thorac Surg. Sep 1986;42(3):231-2. [Medline].
  • Derbes VJ, Mitchell RE Jr. Hermann Boerhaave's (1)atrocis, nec Descripti priu, morbi Historia; (2)the first translation of the classic case report of rupture of the esophagus, with annotations. Bull Med Libr Assoc. 1955;43:217.
  • Fischer A, Thomusch O, Benz S, von Dobschuetz E, Baier P, Hopt UT. Nonoperative treatment of 15 benign esophageal perforations with self-expandable covered metal stents. Ann Thorac Surg. Feb 2006;81(2):467-72. [Medline].
  • Graeber GM, Niezgoda JA, Albus RA, Burton NA, Collins GJ, Lough FC, et al. A comparison of patients with endoscopic esophageal perforations and patients with Boerhaave's syndrome. Chest. Dec 1987;92(6):995-8. [Medline].
  • Harries K, Masoud A, Brown TH, Richards DG. Endoscopic placement of fibrin sealant as a treatment for a long-standing Boerhaave's fistula. Dis Esophagus. 2004;17(4):348-50. [Medline].
  • Henderson JA, Peloquin AJ. Boerhaave revisited: spontaneous esophageal perforation as a diagnostic masquerader. Am J Med. May 1989;86(5):559-67. [Medline].
  • Infatolino A, Ter RB. Rupture and perforation of the esophagus. In: The esophagus. 3rd ed. 1999:595-605.
  • Jaworski A, Fischer R, Lippmann M. Boerhaave's syndrome. Computed tomographic findings and diagnostic considerations. Arch Intern Med. Jan 1988;148(1):223-4. [Medline].
  • Justicz AG, Symbas PN. Spontaneous rupture of the esophagus: immediate and late results. Am Surg. Jan 1991;57(1):4-7. [Medline].
  • Kossick PR. Spontaneous rupture of the oesophagus. S Afr Med J. Oct 6 1973;47(39):1807-9. [Medline].
  • Larrieu AJ, Kieffer R. Boerhaave syndrome: report of a case treated non-operatively. Ann Surg. 1974;181:452-454.
  • Lyons WS, Seremetis MG, deGuzman VC, Peabody JW Jr. Ruptures and perforations of the esophagus: the case for conservative supportive management. Ann Thorac Surg. Apr 1978;25(4):346-50. [Medline].
  • Netter FH. Upper digestive tract. The Ciba collection of medical illustrations. 1971;3:44.
  • O'Connell ND. Spontaneous rupture of the esophagus. Am J Roentgenol Radium Ther Nucl Med. Jan 1967;99(1):186-203. [Medline].
  • Orringer MB, Stirling MC. Esophagectomy for esophageal disruption. Ann Thorac Surg. Jan 1990;49(1):35-42; discussion 42-3. [Medline].
  • Pate JW, Walker WA, Cole FH Jr. Spontaneous rupture of the esophagus: a 30-year experience. Ann Thorac Surg. May 1989;47(5):689-92. [Medline].
  • Richardson JD, Martin LF, Borzotta AP, Polk HC Jr. Unifying concepts in treatment of esophageal leaks. Am J Surg. Jan 1985;149(1):157-62. [Medline].
  • Sabanathan S, Eng J, Richardson J. Surgical management of intrathoracic oesophageal rupture. Br J Surg. Jun 1994;81(6):863-5. [Medline].
  • Scott HJ, Rosin RD. Thoracoscopic repair of a transmural rupture of the oesophagus (Boerhaave's syndrome). J R Soc Med. Jul 1995;88(7):414P-415P. [Medline].
  • Shaffer HA Jr, Valenzuela G, Mittal RK. Esophageal perforation. A reassessment of the criteria for choosing medical or surgical therapy. Arch Intern Med. Apr 1992;152(4):757-61. [Medline].
  • Sherr HP, Light RW, Merson MH, Wolf RO, Taylor LL, Hendrix TR. Origin of pleural fluid amylase in esophageal rupture. Ann Intern Med. Jun 1972;76(6):985-6. [Medline].
  • Tong BC, Yang SC, Harmon J. Esophageal Perforation. In: Principles of Surgery. 8th Edition. 2004:10-14.
  • Troum S, Lane CE, Dalton ML Jr. Surviving Boerhaave's syndrome without thoracotomy. Chest. Jul 1994;106(1):297-9. [Medline].
  • Walker WS, Cameron EW, Walbaum PR. Diagnosis and management of spontaneous transmural rupture of the oesophagus (Boerhaave's syndrome). Br J Surg. Mar 1985;72(3):204-7. [Medline].

Esophageal Rupture excerpt

Article Last Updated: Jun 12, 2007