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Esophageal Perforation and RuptureĀ
Key Points
- Esophageal perforation or rupture is a life-threatening condition in which the cervical, thoracic, or intra-abdominal esophageal sections are perforated or ruptured iatrogenically, traumatically, spontaneously, or through the ingestion of foreign bodies
- Often results in the dissection of air along the subcutaneous planes or into the mediastinum, presenting with chest pain, dyspnea, fever, and neck pain
- Diagnosed clinically based on patient history, signs, and symptoms, and confirmed through visualization; chest radiograph (with water-soluble contrast if needed) is obtained in all patients, with CT scan obtained if needed, and esophagram (chest fluoroscopy with a water-soluble contrast medium) performed if other imaging is inconclusive
- Patients with esophageal perforation or rupture are considered critically ill and require immediate treatment and management in an ICU
- Treatment is dependent on degree of containment of esophageal contents and extent of mediastinal contamination; it may be surgical or nonsurgical
- Patients with esophageal perforation or rupture have decreased morbidity and mortality rates if treated within the first 24 hours
Pitfalls
- Lack of physician experience with this condition (owing to its rarity) along with the diversity of clinical symptoms may delay diagnosis, increasing the risk of morbidity and mortality 2
Terminology
Clinical Clarification
- Esophageal perforation or rupture is a life-threatening condition in which the cervical, thoracic, or intra-abdominal esophageal sections are perforated or ruptured iatrogenically, during trauma, spontaneously, or through the ingestion of foreign bodies 3
- May result in the esophageal contents passing into the mediastinum, pleural cavity, and peritoneum
- A hallmark symptom is the dissection of air along the subcutaneous planes or into the mediastinum as identified by radiography in up to 90% of cases 3
Classification
- Classified by cause of perforation or rupture
- Iatrogenic 4
- Most common (up to 70% of all cases 5)
- Most common sites of iatrogenic esophageal perforation are at anatomically narrowing zones, such as the cricopharyngeal region of the cervical esophagus and the lower esophageal sphincter
- Spontaneous 3
- Commonly affects the left posterolateral wall of the lower third of the esophagus 2 to 3 cm proximal to the gastroesophageal junction
- Usually associated with intense retching and vomiting
- Represents 15% of cases 6
- Foreign bodies 1
- Can partially or completely lacerate the anatomic narrowing of the esophagus
- Trauma
- Such as blunt trauma or gunshot wound
- Iatrogenic 4
Diagnosis
Clinical Presentation
History
- Cervical perforation 7
- Cervical pain (90%)
- Classically associated with dysphagia and/or odynophagia that worsens with neck flexion and swallowing; dysphonia may also be noted
- Thoracic perforation 7
- Thoracic pain (79%); usually lateralizes to side of perforation
- Vomiting (84%)
- Dyspnea (53%)
- Epigastric pain (47%)
- Dysphagia (21%)
- Mackler triad (thoracic pain, vomiting, subcutaneous emphysema) is highly suggestive of the condition but is present in fewer than one-third of cases
- Abdominal perforation 7
- Dorsal pain (worsened by lying on the back) or epigastric pain radiating to the back or scapula (due to diaphragmatic irritation)
- Other reported associated symptoms include:
Physical examination 3
- Patients are typically in distress 1
- Emphysematous crepitus is evident in 60% and 30% of patients with cervical and thoracic esophageal perforations, respectively 1
- Systolic crunching sound (Hamman sign) may be detectable over the cardiac apex
- Systemic inflammatory response may develop depending on length of time since perforation and the degree of contamination, with signs including the following:
- Fever (over 38.5 Ā°C); detected in approximately 51% of cases
- Tachycardia is common
- Tachypnea
- Hemodynamic instability
- Left-sided pleural effusion may be present with distal thoracic esophageal perforation/rupture causing dullness to percussion over the involved area 9
- Right-sided pleural effusion is associated with more proximal perforation/rupture
Causes and Risk Factors
Causes
- Iatrogenic esophageal perforation/rupture accounts for up to 70% of cases and results from: 10 11
- Endoscopic procedures
- Occurs at a rate of approximately 0.03% of cases using a flexible endoscope and 0.11% with a rigid scope
- 1% to 6% risk for endoscopic variceal sclerotherapy
- Esophageal dilation for strictures and achalasia
- Risk of 0.5% for esophageal dilation, increasing to 1.7% for dilation for achalasia
- Surgery on tissue in close proximity to the esophagus
- Increased risk with certain procedures (eg, mediastinal surgery, cervical spine surgery, thyroidectomy)
- Transesophageal echocardiography
- 0.1% to 0.3% risk
- Endoscopic procedures
- Spontaneous rupture, also known as Boerhaave syndrome, occurs in the absence of preexisting pathology (15% of cases) 1
- Typically results from intense vomiting or severe retching
- Foreign objects or trauma 1
- Perforation tends to occur where there is anatomic esophageal narrowing:
- Cricopharyngeus muscle (level of C5-C6)
- Left main-stem bronchus
- Aortic arch
- Lower esophageal sphincter
- Swallowed foreign bodies account for 80% of cervical perforations 7
- Materials that were inappropriately ingested (eg, metal safety pins)
- Ingestion of caustic liquids
- Blunt trauma 12
- External air-blast 4
- Gunshot wounds
- Perforation tends to occur where there is anatomic esophageal narrowing:
- Rarely, esophageal rupture is due to the following:
- Parturition
- Weight lifting
- Status epilepticus
- Use of Heimlich maneuver
Risk factors and/or associations
Sex
- Esophageal rupture affects men more often than women (55% versus 45%, respectively) 10
Diagnostic Procedures
Primary diagnostic tools
- Initial diagnosis is clinical and is based on symptoms, signs, and patient history 3
- Lack of physician experience with this condition (owing to its rarity), along with the diversity of clinical symptoms, may delay diagnosis, increasing risk of morbidity and mortality 2
- Begin diagnostic work-up immediately upon clinical suspicion of esophageal rupture 1
- Definitive diagnosis is based on radiographic evidence 1
- Posteroanterior and lateral plain chest radiography may help diagnose esophageal rupture; it can be done first if diagnosis is uncertain and often is performed first in practice 1
- Sufficient for identifying indirect signs of esophageal rupture
- If patient can swallow, consider using an oral water-soluble contrast medium such as diatrizoate meglumine (Gastrografin)
- If radiographic evidence is negative and clinical symptoms persist, obtain a CT scan or repeat after 4 to 6 hours
- CT of the chest and upper abdomen with oral contrast medium is a first line diagnostic tool used to provide both esophageal and extraesophageal information and may be chosen instead of chest radiography 1
- Indicated for all patients but especially for those with external trauma, caustic injuries, or critical illness
- Key examination in patients with suspected perforation or other foreign bodyārelated complications 13
- Esophagram is the second line imaging modality, indicated if CT scan with contrast enhancement is inconclusive 8
- Posteroanterior and lateral plain chest radiography may help diagnose esophageal rupture; it can be done first if diagnosis is uncertain and often is performed first in practice 1
- Flexible upper endoscopy is used only when there is a high clinical suspicion of a perforation but radiographic imaging result appears negative/equivocal, or when swallowing contrast media is not an option 1
- Definitive diagnosis is based on radiographic evidence 1
Imaging
- Plain chest radiography 9
- May obtain in all patients; add water-soluble contrast medium if needed
- May show indirect signs of esophageal rupture that include the following:
- Pneumomediastinum diagnosed as
- Radiolucent outline around the heart and mediastinum
- Subcutaneous emphysema diagnosed as
- Radiolucent striation
- Pneumothorax and lung collapse diagnosed as
- Air in the pleural space
- A shift of the mediastinum
- Hemothorax diagnosed as
- Partial or complete opacification of the hemithorax
- Pleural effusion
- Pneumomediastinum diagnosed as
- CT of the chest and upper abdomen with oral contrast medium is best imaging procedure 1 13
- May be used as first line instead of chest radiography or if chest radiography is inconclusive
- Key examination in patients with suspected perforation or other foreign bodyārelated complications 13
- Diagnostic findings of esophageal rupture include the following:
- Collection of fluid and/or air in the mediastinum
- Esophageal thickening
- Chest fluoroscopy with a water-soluble contrast medium (esophagram) 1
- Second line imaging procedure if chest radiograph and/or CT scan is inconclusive
- Diatrizoate meglumine (Gastrografin) will show contrast leak in most cases
- Diagnostic findings of esophageal rupture include contrast medium extravasation accompanied by large pleural effusion
- Avoid barium sulfate owing to extravasation into mediastinum leading to the following:
- Fibrosing mediastinitis
- Long-term presence may make interpretation of future mediastinal imaging difficult
- 10% false-negative rate 9
Procedures
- Insertion of a thin, flexible tube equipped with fiber optics and a camera into the upper gastrointestinal tract to visualize a suspected esophageal rupture
- High suspicion of perforation and negative radiographic findings
- Patient unable to swallow contrast agent
- Esophageal perforation observed with radiographic study
- Hemodynamic instability
- Conversion of small tear to a larger perforation during air insufflation
- Direct visualization of perforation site indicates its location and extent
Differential Diagnosis
Most common
- Acute aortic dissection 14 (Related: Acute Aortic Syndrome)
- Patients typically present with sudden, severe pain that may radiate from chest or abdomen to back or extremities; hypertension is present in approximately 75% of cases
- Differentiate by presentation:
- Sudden chest or back pain accompanied by at least 1 of the following symptoms:
- Pulse deficit
- Aortic regurgitation
- Neurologic manifestations, including the following:
- Stroke
- Spinal cord ischemia
- Ischemic neuropathy
- Hypoxic encephalopathy
- Sudden chest or back pain accompanied by at least 1 of the following symptoms:
- Diagnose radiographically
- Contrast-enhanced CT of the chest and abdomen is commonly used to detect aortic dissection
- Bedside transesophageal echocardiogram can be used to diagnose aortic dissection when managing a hemodynamically unstable patient
- Acute pericarditis 15 (Related: Pericarditis)
- Differentiate by presentation:
- Typically sudden-onset, high-intensity pleuritic chest pain that worsens with deep inspiration radiating to the neck and upper limbs
- Pain worsening in supine position and improving when sitting
- Pericardial friction rub is useful to discern pericarditis when present (85% of cases at some time during the disease course)
- Diagnose
- ECG showing typical changes of diffuse upwardly concave ST-segment elevation with PR depression
- CT showing the enlargement of the ascending or descending aorta
- Chest radiograph showing excess fluid in the pericardium; appearance of an enlargement of the heart may occur in the presence of pericardial effusion of more than 200 mL
- Differentiate by presentation:
- Mallory-Weiss tear 16
- Longitudinal mucosal laceration (intramural dissection) at the gastroesophageal junction resulting from sudden increase in pressure within the stomach (eg, vomiting) accounts for up to 15% of upper gastrointestinal bleeding cases
- Differentiate by presentation
- Acute upper gastrointestinal bleeding accompanied by epigastric or back pain and weakness
- Diagnose
- Esophagogastroduodenoscopy facilitates visualizing site of mucosal tear at gastroesophageal junction and allows for treatment at the same time
- Pneumonia (Related: Community-Acquired Pneumonia in Adults)
- Differentiate clinically
- Patient presents with fever, cough, pleuritic chest pain, dyspnea, and tachypnea
- Diagnose
- Chest radiography facilitates the visualization of airspace opacity, lobar consolidation, or interstitial opacities
- Sputum and blood culture to identify organism causing the infection
- Differentiate clinically
- Myocardial infarction (Related: Acute Coronary Syndromes)
- Differentiate clinically
- Initially causes substernal pressure or pain with radiation to neck, lower jaw, or arms; may be precipitated by exertion
- Often associated with dyspnea, nausea, and diaphoresis
- Initially causes substernal pressure or pain with radiation to neck, lower jaw, or arms; may be precipitated by exertion
- Diagnose
- Typical ECG changes and positive cardiac biomarkers (eg, troponins) diagnose infarction
- Differentiate clinically
- Spontaneous pneumothorax
- Differentiate clinically
- Sudden onset of pleuritic chest pain and dyspnea
- Classically occurs in tall, thin males in their teens or twenties; also can occur in patients who smoke and have lung disease
- Findings on examination may include absent tactile fremitus and decreased breath sounds over the involved area of lung
- Diagnose
- Plain chest radiograph shows radiolucent air and absence of lung markings in area of collapse
- Differentiate clinically
Treatment
Goals
- Prevent further contamination of body cavities with adequate drainage and antibiotics
- Eliminate any focus of infection and inflammation
- Restore alimentary tract continuity
- Establish nutritional support
Disposition
Admission criteria
Esophageal perforation rupture is a medical emergency; admit suspected cases to the hospital immediately
Criteria for ICU admission
- Suspected or confirmed cases of esophageal perforation/rupture require ICU observation and management
Recommendations for specialist referral 3
- Gastroenterology referral/consultation is recommended for all patients with esophageal perforation/rupture
- Thoracic surgical consultation is recommended for all patients with esophageal perforation/rupture, even if nonoperative treatment is chosen
- If thoracic surgery is not available, transport patient to a facility with available thoracic surgery as soon as possible 1
Treatment Options
Begin empiric broad-spectrum antibiotic coverage for anaerobes and both gram-negative and gram-positive aerobes when diagnosis is first suspected 5
Typically, cervical esophageal perforation is managed by placement of a drain alone; thoracic or abdominal perforation involves more contamination and is more difficult to contain, requiring surgical management 9
- Endoscopy is sometimes used to repair a perforation if treated immediately
Surgical treatment
- Gold standard of treatment for patients with early diagnosis (within 24 hours) of unconfined cervical, thoracic (especially when greater than 6 cm in length 18), and abdominal esophageal perforations 17
- For rapid closure of the esophageal leak and drainage of the mediastinal or pleural collection
- Immediate parenteral nutrition and broad-spectrum antibiotics are necessary to minimize mediastinitis
- Necessary for the treatment of clinically unstable patients
- Specific operative strategies are chosen based on location of injury, presence of underlying esophageal disease, and degree of tissue damage, combined with patient’s overall condition
- Percutaneous drainage
- Recommended for patients with cervical perforation or those who seek medical treatment after 48 hours from time of incident
- Extensive surgical drainage and tube thoracostomy
- Recommended for patients with thoracic or abdominal perforations to contain leakage into mediastinum; occasionally required for cervical esophageal perforation
- As a general principle, all thoracic or abdominal esophageal perforations require wide mediastinal drainage by opening the parietal pleura in its entire length of the esophagus
- Debridement with primary closure with or without inclusion of buttressing with adjacent tissue can be done at this time
- Primary closure with or without autologous tissue
- Perforations in cervical esophagus
- Primary closure can be done with interrupted sutures
- Feeding jejunostomy and draining gastrostomy for nutrition and to allow distal drainage
- Esophagectomy is recommended as a secondary surgical procedure followed by immediate or delayed reconstruction 5
- Indicated to close an esophageal rupture that was not closed during primary surgery owing to severe mediastinitis or underlying esophageal pathology
- Immediate reconstruction is indicated when pathologic process is a localized resectable cancer or an undilatable or malignant stricture reconstruction
- Delayed reconstruction is indicated when there is significant devitalized tissue that does not allow a primary repair (eg, caustic ingestion; when primary repair failed and there is ongoing mediastinal sepsis) 8
- Involves source control by debridement, drainage, and cervical esophagostomy for proximal drainage, and gastrostomy for distal drainage with feeding jejunostomy for nutritional support
- Esophageal T-tube 9
- For patients with thoracic or abdominal perforations who are hemodynamically unstable
- Indicated for patients whose perforation cannot be repaired with primary closure
- Delayed presentation of thoracic or abdominal perforation
- Percutaneous drainage
Endoscopic treatment
- Use of endoscopic treatment is increasing for perforations recognized immediately, not associated with pleural or mediastinal contamination, in clinically stable patients with no signs of sepsis present 13 19
- Increasingly used to treat intrathoracic esophageal perforation smaller than 6 cm with immediate placement of esophageal endoscopic clips or stent to close the defect 18
- Emergent endoscopy (less than 6 hours) is recommended for sharp-pointed objects, batteries, and magnets 13
- Endoscopy is not employed in clinically unstable patients
Nonoperative treatment (approximately 25% of cases) 9
- Indicated for most patients with cervical esophageal perforation; appropriate for those with the following:
- Well-contained perforation and minimal mediastinal soilage on imaging or endoscopic evaluation 17
- Injury limited to the esophageal wall 17
- No malignancy, stricture, or obstruction in area of perforation 8
- No evidence of systemic infection 8
- Hemodynamic stability 17
- Late presentation (24 hours after incident) but good clinical condition 17
- Contained cavity that drains back into esophagus 8
- No perforation into abdominal cavity 8
- No free extravasation of contrast medium into body cavities 8
- Availability of advanced imaging modalities and thoracic surgery
- Nonoperative treatments include the following: 9
- Securing the airway and ordering NPO status
- Nasogastric suction
- Pain management with opioid analgesics as needed
- Broad-spectrum IV antibiotics
- Proton pump inhibitors
- Indicated for patients with severe erosive esophagitis, esophageal stricture, or malignancies, and/or those who are experiencing dysphagia
- Nutritional support
- Patients treated nonoperatively remain NPO for 7 days, after which a meglumine diatrizoate (Gastrografin) swallow radiographic study is obtained 8
Drug therapy
- Begin empiric broad-spectrum antibiotic coverage for anaerobes and both gram-negative and gram-positive aerobes when diagnosis is first suspected. 5
- Ī²-lactam antibiotics
- Cefuroxime
- Cefuroxime Sodium Solution for injection; Adults: 750 mg IV every 8 hours for 7 to 10 days.
- Ampicillin
- Ampicillin Sodium Solution for injection; Adults: 500 mg IV every 8 hours for 7 to 10 days.
- Cefuroxime
- Nitroimidazole antimicrobial 20
- Metronidazole
- Metronidazole Solution for injection; Adults: 500 mg IV every 8 hours for 7 to 10 days.
- Metronidazole
- Oxazolidinone antibiotic
- Linezolid 21
- Linezolid solution for injection; Adults: 600 mg IV every 12 hours.
- Linezolid 21
- Ī²-lactam antibiotics
- Proton pump inhibitors 5
- Pantoprazole
- Pantoprazole Sodium Solution for injection; Adults: 40 mg IV every 12 hours. Switch to oral therapy when feasible.
- Pantoprazole
Nondrug and supportive care
Nonoperative treatment (approximately 25% of cases) 9
- Indicated for most patients with cervical esophageal perforation; appropriate for those with the following:
- Availability of advanced imaging modalities and thoracic surgery
- Well-contained perforation and minimal mediastinal soilage 17
- No perforation into abdominal cavity 8
- Injury limited to the esophageal wall 17
- No malignancy, stricture, or obstruction in area of perforation 8
- No evidence of systemic infection 8
- Uncompromised circulation 17
- Late presentation (24 hours after incident) but good clinical condition 17
- Contained cavity that drains back into esophagus 8
- No free extravasation of contrast medium into body cavities 8
- Supportive care 9
- Securing the airway and ordering NPO status
- Nasogastric suction
- Pain management with opioid analgesics as needed
- Broad-spectrum IV antibiotics
- Proton pump inhibitors
- Indicated for patients with severe erosive esophagitis, esophageal stricture, or malignancies, and/or those experiencing dysphagia
- Nutritional support
- Patients treated nonoperatively remain NPO for 7 days, after which a meglumine diatrizoate (Gastrografin) swallow radiographic study is obtained 8
Parenteral nutrition 5
- To provide nutrition to patients with a dysfunctional gastrointestinal tract and those who are experiencing dysphagia
- Total parenteral nutrition through a central catheter or indwelling IV line
- May be customized to individual patient requirements, or a standardized solution may be used
- Transition to oral feeding is varied among patients owing to multiple factors
Procedures
Primary closure with or without anchoring using autologous tissue 3
General explanation
- Surgical closure of the rupture primarily using interrupted sutures
- Repair can be further stabilized with vascularized autologous tissues (eg, pleural flap, diaphragmatic pedicle graft, omentum onlay graft, rhomboid and latissimus dorsi muscles, intercostal muscles, pericardial fat pad)
Indication
- Perforations without extravasation of contrast medium into surrounding body cavities
- Hemodynamic instability
Contraindications
- Mediastinal sepsis
- Underlying malignancy, stricture, or obstruction at site of perforation
Complications
- Site leakage
Tube thoracostomy and surgical drainage (thoracotomy) 22
General explanation
- A chest tube is inserted into the pleural space and placed to suction (tube thoracostomy)
- In general, all thoracic or abdominal esophageal perforations require wide mediastinal drainage by opening the parietal pleura in its entire length of the esophagus
- Tube placement and surgical drainage (thoracotomy) are typically carried out at the same time; in the absence of cancer or an irreversible distal obstruction, meticulous repair of an intrathoracic esophageal perforation is the preferred approach, regardless of the duration of the injury 23
Indication
- Thoracic or abdominal perforations (to contain leakage into mediastinum)
- Cervical perforation only if the patient’s pleural effusion becomes grossly contaminated and/or the perforation/rupture remains unconfined after initial percutaneous drainage and nonoperative treatments have been done
Contraindications
- Cervical perforation in a stable patient
Complications
- Bleeding or hemothorax
- Perforation of internal organs
- Intercostal neuralgia
- Subcutaneous emphysema
- Pneumonia
- Empyema
Esophagectomy 5
General explanation
- Partial or full surgical removal of the esophagus, with concurrent reconstruction from sections of the stomach or the large intestine
Indication
- Thoracic or abdominal perforations that were not closed during primary surgery owing to severe mediastinitis or underlying esophageal pathology
- Thoracic or abdominal perforations caused by megaesophagus, carcinoma, caustic ingestion, stenosis, or multiple reflux strictures that cannot be dilated
Contraindications
- Hemodynamic stability and perforation/rupture amenable to primary closure
- Hemodynamic instability and perforation/rupture that can be repaired with esophageal T-tube
Complications
- Pneumonia
- Atrial fibrillation
- Recurrent laryngeal nerve injury
- Chyle leak/chylothorax
- Anastomotic leak
- Functional abnormalities (eg, dumping syndrome, reflux)
Esophageal T-tube 17
General explanation
- The T-tube creates a controlled esophagocutaneous fistula, allowing drainage of the esophagus and time for tissues to heal
Indication
- Hemodynamic instability
- Esophageal injuries that cannot be repaired with primary closure
- Delayed presentation of thoracic or abdominal esophageal perforation
Contraindications
- Perforations caused by megaesophagus, carcinoma, caustic ingestion, stenosis, or multiple undilatable reflux strictures
- Severe mediastinitis or underlying esophageal pathology
Complications
- Leakage
- Atrial fibrillation
- Empyema
- Sepsis
- Chronic fistula formation
Percutaneous drainage 9
General explanation
- Recommended to stabilize or minimize leakage into surrounding tissues and mediastinum, and to contain pleural contamination
- Use of fluoroscopy, CT, or ultrasonography to guide needle placement for drainage
- A drainage tube may be inserted until no further drainage is produced
Indication
- Cervical esophageal perforation for patients without sepsis who are hemodynamically stable
- For patients with perforations confined to the mediastinum or who seek medical attention more than 48 hours after the injury
Contraindications
- Gross mediastinum contamination
- Perforations caused by megaesophagus, carcinoma, caustic ingestion, stenosis, or multiple undilatable reflux strictures
Complications 24
- Inadvertent catheterization of adjacent organs
- Vascular fistula formation
- Pseudoaneurysm formation
- Infection
Monitoring
- All patients hospitalized with esophageal perforation or rupture require close monitoring for hemodynamic instability and evidence of systemic infection, typically provided in an ICU 8
- Before discharge, imaging is repeated (CT, chest radiograph, or esophagram) using oral meglumine diatrizoate (Gastrografin) to test the perforation site 5
- Patients treated nonoperatively are imaged after 7 days without oral intake to determine closure of the perforation before initiating an oral diet 8
Complications and Prognosis
Complications
- Complications are reported in approximately 14% of patients with cervical perforation, 39% with thoracic perforation, and 25% with abdominal perforation 3
- Pneumonia
- Empyema
- Esophageal narrowing or stricture
- Periesophageal abscess
- Esophageal leakage
- Mediastinitis
- Peritonitis
- Cardiopulmonary collapse
- Multiorgan failure
Prognosis
- Mortality of esophageal rupture or perforation ranges from 10% to 40% 25 8 and is improved with early recognition and treatment initiation within 24 hours; 3 treatment begun more than 48 hours after perforation is associated with 40% to 60% mortality 5
- Iatrogenic perforation has lower mortality of 10%, whereas rupture after emesis is associated with mortality of 60% to 70%
Sources
1:Ā Soreide JA et al: Esophageal perforation: diagnostic work-up and clinical decision-making in the first 24 hours. Scand J Trauma Resusc Emerg Med. 19:66, 2011