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Boerhaave Syndrome Last Updated: August 9, 2006 |
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| Synonyms and related keywords: spontaneous rupture of the esophagus, Boerhaave's syndrome, esophageal rupture, esophagus rupture, ruptured esophagus, forceful emesis, emesis complications, transmural perforation of the esophagus, esophageal perforation, perforated esophagus
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AUTHOR INFORMATION
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| Author: Praveen K Roy, MD, Assistant Professor of Medicine, Associate Director of Research, Division of Gastroenterology, Department of Internal Medicine, University of Missouri at Columbia; Chief of Gastroenterology, Harry Truman Veteran Affairs Memorial Hospital Coauthor(s): Mohamed Othman, MD, Staff Physician, Department of Internal Medicine, University of New Mexico School of Medicine; Mark Murphy, MD, FACP, Assistant Professor of Internal Medicine, Mercer University Medical School; Program Director, Gastroenterology and Hepatology Education, Dept of Internal Medicine, Memorial Health University Medical Center; Viswanath Kalapatapu, MD, Staff Physician, Department of Internal Medicine, Memorial Health University Medical Center; Prakash Ramanathan, MD, Staff Physician, Department of Internal Medicine, Memorial Health University Medical Center |
| Praveen K Roy, MD, is a member of the following medical societies:
American College of Gastroenterology,
American Gastroenterological Association, and
Canadian Association of Gastroenterology |
| Editor(s): Manoop S Bhutani, MD, FACG, FACP, Professor, Department of Medicine, Division of Gastroenterology, Director, Center for Endoscopic Ultrasound, Co-Director, Center for Endoscopic Research, Training and Innovation, University of Texas Medical Branch at Galveston; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine;
James L Achord, MD, Professor Emeritus, Department of Medicine, Division of Digestive Diseases, University of Mississippi School of Medicine;
Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine;
and Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania |
Disclosure
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INTRODUCTION
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Background: Herman Boerhaave first described spontaneous rupture of the esophagus in 1724. It typically occurs after forceful emesis. Boerhaave syndrome is a transmural perforation of the esophagus to be distinguished from Mallory-Weiss syndrome, a nontransmural esophageal tear also associated with vomiting. Because it usually is associated with emesis, Boerhaave syndrome usually is not truly spontaneous. However, the term is useful for distinguishing it from iatrogenic perforation, which accounts for 85-90% of cases of esophageal rupture.
Diagnosis can be difficult because often no classic symptoms are present and delays in presentation for medical care are common. Approximately one third of all cases are clinically atypical. Prompt recognition of this potentially lethal condition is vital to ensure appropriate treatment. Mediastinitis, sepsis, and shock frequently are seen late in the course of illness, which further confuses the diagnostic picture.
A reported mortality estimate is approximately 35%, making it the most lethal perforation of the GI tract. The best outcomes are associated with early diagnosis and definitive surgical management within 12 hours of rupture. If intervention is delayed longer than 24 hours, the mortality rate (even with surgical intervention) rises to higher than 50% and to nearly 90% after 48 hours. Left untreated, the mortality rate is close to 100%. Pathophysiology: Esophageal rupture in Boerhaave syndrome is postulated to be the result of a sudden rise in intraluminal esophageal pressure produced during vomiting, as a result of neuromuscular incoordination causing failure of the cricopharyngeus muscle to relax. The syndrome commonly is associated with overindulgence in food and/or alcohol. The most common anatomical location of the tear in Boerhaave syndrome is at the left posterolateral wall of the lower third of the esophagus, 2-3 cm proximal to the gastroesophageal junction, along the longitudinal wall of the esophagus. The second most common site of rupture is in the subdiaphragmatic or upper thoracic area. Frequency:
- Internationally: Although likely underreported, incidence is relatively rare. A 1980 review by Kish cited 300 cases in the literature worldwide. A 1986 summary by Bladergroen and Postlethwait described 127 cases. Of these, 114 were diagnosed antemortem; the others were diagnosed at autopsy. Overall, Boerhaave syndrome accounts for 15% of all traumatic rupture or perforation of the esophagus.
Mortality/Morbidity: The mortality rate is high. It is the most lethal perforation of the GI tract. Survival is contingent largely upon early recognition and appropriate surgical intervention.
- Overall mortality rate is approximately 30%.
Mortality is usually due to subsequent infection including mediastinitis, pneumonitis, pericarditis, or empyema
- Patients who undergo surgical repair within 24 hours of injury have a 70-75% chance of survival. This falls to 35-50% if surgery is delayed longer than 24 hours and to approximately 10% if delayed longer than 48 hours.
- Cases of patients surviving without surgery exist but are rare enough to warrant case reports in the medical literature.
Race:
- Cases have been reported in all races and on virtually every continent.
Sex:
- The syndrome is described more commonly in males than in females, with ratios ranging from 2:1 to 5:1.
Age:
- It is seen most frequently among patients aged 50-70 years.
- Reports suggest that 80% of all patients are middle-aged men.
- Cases have been described in neonates and in persons older than 90 years. Although no clear explanation exists for this, the least susceptible age group appears to be children aged 1-17 years.
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CLINICAL
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History: - The classic clinical presentation usually consists of repeated episodes of retching and vomiting, typically in a middle-aged man with recent excessive dietary and alcohol intake.
- This is followed by a sudden onset of severe chest pain in the lower thorax and upper abdomen.
- The pain may radiate to the back or left shoulder.
- Swallowing often aggravates it.
- Typically, hematemesis is not seen after esophageal rupture, which helps distinguish it from the more common Mallory-Weiss tear.
- Swallowing may precipitate coughing because of communication between the esophagus and the pleural cavity.
- Atypical clinical features sometimes delay a prompt diagnosis and appropriate intervention. This may result in an increase in morbidity and mortality.
- Shortness of breath is a common complaint and is due to pleuritic pain or pleural effusion.
Physical: - The Mackler triad defines the classic presentation. It consists of vomiting, lower thoracic pain, and subcutaneous emphysema.
- Presentation may vary depending on the following:
- The location of the tear
- The cause of the injury
- The amount of time that has passed from the perforation to the intervention
- Patients with cervical esophagus perforation may present with neck or upper chest pain.
- Patients with middle or lower esophagus perforation may present with interscapular or epigastric discomfort.
- Findings of pleural effusion are common.
- If present, subcutaneous emphysema is particularly helpful for confirming diagnosis.
- It is seen in 28-66% of patients at initial presentation.
- More typically, it is found later.
- Other classic findings, as described by Anderson and Barrett, include tachypnea and abdominal rigidity.
- Tachycardia, diaphoresis, fever, and hypotension are common, particularly as the illness progresses. However, these findings are nonspecific.
- Unusual findings may include the following:
- Hoarseness of voice due to recurrent laryngeal nerve involvement
- Tracheal and mediastinal shift
- Pneumomediastinum is a very important finding.
- It may cause a crackling sound upon chest auscultation, known as the Hamman crunch.
- The crunch typically is heard coincident with each heartbeat and may be mistaken for a pericardial friction rub.
- This is present in 20% of patients.
- Later stages of illness may manifest as signs of infection and sepsis.
- Symptoms may include fever, hemodynamic instability, and progressive obtundation.
- Establishing a diagnosis in the later stages can be quite difficult because septic complications begin to dominate the clinical picture.
- Early diagnosis is critical.
Causes: - It is a barogenic injury resulting from a sharp increase in intraluminal pressure against a closed cricopharyngeus.
- Perforation typically occurs at the weakest point in the esophagus.
- Usually the left lower esophagus below the diaphragm in adults
- Usually into the right pleural cavity in very young patients
- Alcoholism and overindulgence in food and drink are primary risk factors. Both can lead to emesis, which is a major component of the classic presentation.
- No genetic predilection is apparent.
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DIFFERENTIALS
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Aortic Dissection Esophageal Rupture Mallory-Weiss Tear Myocardial Infarction Pancreatitis, Acute Peptic Ulcer Disease Pneumothorax
Other Problems to be Considered:
Spontaneous intramural esophageal perforation
Spontaneous intramural hematoma of the esophagus (esophageal apoplexy) |
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WORKUP
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Lab Studies:
- Laboratory findings often are nonspecific.
- Patients may present with leukocytosis and a left shift.
- As many as 50% of patients have a hematocrit value that approaches 50%. This may be due to fluid loss into pleural spaces and tissues.
- Serum albumin is normal but may be low, while the globulin fraction may be normal or slightly elevated.
- Many patients present with a pleural effusion.
- Thoracentesis with examination of the pleural fluid can aid in diagnosis.
- Undigested food particles and gastric juices usually are found.
- If no gross particles are found, cytology can confirm or exclude their presence, but time is of the essence.
- The pH of the pleural fluid will be less than 6, and the amylase content will be elevated.
- Squamous cells from saliva may be found.
Imaging Studies:
- This is useful in the initial diagnosis because 90% of patients reveal an abnormal finding after perforation.
- The most common finding is a unilateral effusion, usually on the left. This corresponds with the fact that most perforations occur in the left posterior aspect of the esophagus.
- Other findings may include pneumothorax, hydropneumothorax, pneumomediastinum, subcutaneous emphysema, or mediastinal widening.
- The V-sign of Naclerio has been described as a chest radiograph finding in as many as 20% of patients. This involves the presence of radiolucent streaks of air that dissect the fascial planes behind the heart to form the shape of the letter V. It is a fairly specific, although insensitive, radiographic sign of esophageal perforation.
- Overall, 10% of chest radiographs are normal. This can be at least partly explained by the delayed radiographic development of mediastinal and subcutaneous emphysema. These findings may take an hour or more after perforation to appear on the chest radiograph.
- This helps confirm the diagnosis.
- It typically shows extravasation of contrast into the pleural cavity.
- The study outlines the length of the perforation and its location, which aids in the decision of whether to use a thoracic or abdominal surgical approach.
- Initially, use a water-soluble contrast, such as Gastrografin. It has 90% sensitivity.
- It may have false-negative results in up to 20% of patients.
- The use of barium in patients affected with Boerhaave syndrome has been associated with severe mediastinitis. This complication may contribute significantly to increased morbidity and mortality.
- If the contrast study is negative and the clinical index of suspicion remains high, placing the patient in the left and right lateral decubitus positions often is helpful. The use of barium would then be warranted.
- It can reveal decisive criteria for diagnosis.
- It is helpful in patients too ill to tolerate esophagrams.
- It localizes collections of fluid for surgical drainage.
- Visualization of adjacent structures is possible, which expands the differential diagnosis in patients with chest pain and vomiting.
- It can demonstrate periesophageal air tracks that are suggestive of perforation.
- CT scan may not precisely localize the site of perforation.
Procedures:
- Endoscopy is not commonly used to aid in diagnosis.
- It carries the additional risks of increasing the size and extent of the original perforation and forcing additional air through the perforation into the mediastinum or pleural cavity.
- It is more useful in the thoracic esophagus because it has poor sensitivity in the cervical esophagus.
- Endoscopy may be useful when a perforation is suspected but not proven, especially when trauma (eg, ingested foreign body) is known or suspected to be present.
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TREATMENT
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Medical Care: - Ideal management involves a combination of both conservative and surgical interventions.
- Mainstays of therapy include the following:
- Intravenous volume resuscitation
- Administration of broad-spectrum antibiotics
- Prompt surgical intervention
- The decision to use a conservative (medical intervention only) or an aggressive (medical plus surgical intervention) approach depends on the following factors:
- Time delay in presentation and diagnosis
- Extent of perforation
- Overall medical condition of the patient
- Surgical intervention is the standard of care in most cases, but Cameron et al established a set of criteria in which conservative (nonsurgical) management might be appropriate. These include the following:
- The esophageal disruption should be well contained in the mediastinum.
- The cavity should be well drained back into the esophagus.
- Few symptoms should be present.
- Evidence of clinical sepsis should be minimal.
- Conservative management consists of the following:
- Intravenous fluids should be instituted.
- Antibiotics: Imipenem/cilastatin (Primaxin) offers good broad-spectrum coverage.
- Nasogastric suction should be applied.
- Keep the patient NPO.
- Adequate drainage with tube thoracostomy or formal thoracotomy is vital.
- Early use of nutritional supplementation: Recent evidence suggests that, for hastening recovery, a jejunostomy tube feeding may be favored over hyperalimentation.
Surgical Care: - Barrett described the first successful surgical repair of the esophagus in 1947. Prior to this, Boerhaave syndrome had virtually 100% mortality.
- Most physicians advocate surgical intervention if the diagnosis is made within the first 24 hours after perforation.
- Direct repair of the rupture and adequate drainage of the mediastinum and pleural cavity provide the best survival rates.
- A left thoracotomy is the preferred approach, although laparotomy may be necessary if the tear extends into the distal esophagus.
- Various techniques, such as the use of an omental flap, may be used to support the primary closure.
- Gastrostomy and jejunostomy tubes often are placed to aid in drainage and nutrition, respectively.
- The vitality of the surrounding tissue is an important factor in selecting the surgical procedure.
- For patients in whom a delay in diagnosis (>24 h) occurred, primary repair may not be possible.
- After 24 hours, the wound edges frequently are edematous, stiff, and friable.
- Various alternatives to primary repair are available.
- The most common includes the creation of an esophageal diversion through the use of a loop or end-cervical esophagostomy. This allows the wound to heal by secondary intention.
- The use of T-tubes also has been described. T-tubes result in the formation of a controlled fistula and a route for drainage of esophageal secretions and refluxed gastric materials.
- One recent study noted that the option of primary repair may be considered for perforations as old as 72 hours.
- Newer techniques involve the use of plastic-covered self-expanding metallic stents.
- They are considered acceptable alternatives only when all other interventional options have been exhausted. Their use in nonmalignant disease is highly controversial because they cannot be removed without considerable risks or not at all.
- Currently, the use of stents in Boerhaave syndrome is recommended for cases that involve extreme delays in diagnosis or a failure of conservative management.
- Expandable metal stents are most commonly used as palliative interventions for unresectable malignant esophageal obstruction.
- These devices bridge the esophageal tear.
- Several types of stents are available, and they vary in flexibility. Recent research holds the promise of biodegradable stents, obviating the necessity for removal.
- Esophageal stents have been associated with a risk of delayed massive hemorrhage in patients with esophageal malignancy.
- The long-term effects of stent placement in Boerhaave syndrome have not been adequately evaluated.
- Late complications of surgical intervention may include the following:
- Empyema that often requires tube drainage or decortication
- Esophagotracheal or esophagobronchial fistulas
Consultations: - Consultation with a thoracic or general surgeon is indicated as soon as the diagnosis is suspected.
- Infectious disease specialist should be consulted for assistance with antimicrobial therapy.
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MEDICATION
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The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Drug Category: Antibiotics -- Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting. A broad-spectrum antibiotic, such as imipenem/cilastatin (Primaxin), is recommended. Drug Name
| Imipenem/cilastatin (Primaxin) -- Offers good broad-spectrum coverage. For treatment of multiple-organism infections in which other agents do not have wide-spectrum coverage or are contraindicated due to potential for toxicity. |
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| Adult Dose | Base initial dose on severity of infection, and administer in equally divided doses
250-500 mg IV q6h, not to exceed 3-4 g/d
Alternatively, 500-750 mg IM q12h or intra-abdominally| Pediatric Dose | <12 years: Not established; 15-25 mg/kg/dose IV q6h suggested for >3 months
Fully susceptible organisms: not to exceed 2 g/d
Infections with moderately susceptible organisms: not to exceed 4 g/d| Contraindications | Documented hypersensitivity |
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| Interactions | Coadministration with cyclosporine may increase adverse CNS effects of both agents; coadministration with ganciclovir may result in generalized seizures |
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| Pregnancy |
C - Safety for use during pregnancy has not been established.
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| Precautions | Adjust dose in renal insufficiency; avoid use in children <12 years |
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FOLLOW-UP
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Complications:
- Esophageal rupture may lead to the development of septicemia, pneumomediastinum, mediastinitis, massive pleural effusion, empyema, pneumomediastinum, or subcutaneous emphysema.
- If the rupture extends directly into the pleura, hydropneumothorax is expected.
- In adults, this occurs more commonly on the left side of the pleura.
- In neonates, the rupture usually occurs on the right side.
- After the rupture, free air enters the mediastinum and also may spread to the adjacent structures and result in mediastinal abscess or superimposed secondary infection.
- Other complications include acute respiratory distress syndrome, pneumomediastinum, pneumothorax, and hydrothorax.
Prognosis:
- Prognosis is directly contingent on early recognition and appropriate intervention.
- Early diagnosis allows prompt surgical repair.
- Diagnosis and surgery within 24 hours carry a 75% survival rate. This drops to approximately 50% after a 24-hour delay and approximately 10% after 48 hours.
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MISCELLANEOUS
| Section 9 of 10  |
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Medical/Legal Pitfalls:
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BIBLIOGRAPHY
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Boerhaave Syndrome excerpt |