Boerhaave syndrome
Updates to Article Attributes
Boerhaave syndrome refers to an oesophageal rupture secondary to forceful vomiting and retching.
Epidemiology
It tends to be more prevalent in males, with alcoholism a risk factor. The estimated incidence is ~ 1:6000.
Clinical presentation
They are often associated with the clinical triad of vomiting, chest pain and subcutaneous emphysema (Mackler's triad). Other symptoms include epigastric pain, back pain, dyspnoea and shock. This condition was universally fatal before the age of surgery.
Pathology
It is thought occur due to a forceful ejection of gastric contents in an unrelaxed oesophagus against a closed glottis. The tears are vertically oriented, 1-4 cm in length. Approximately 90% occur along the left posterolateral wall (left diaphragmatic crus) of the distal oesophagus, 3-6cm above the esophageal hiatus of the diaphragm 10.
Radiographic features
Plain radiograph
Chest radiograph findings are often non-specific and the may be completely absent. The classic CXR findings include pneumomediastinum and/or, left pleural effusion and left pneumothorax. Gas may also be seen with the soft tissue spaces of the chest wall and the neck. Other signs include
- V sign of Naclerio: a focal, sharply marginated region of paraspinal radiolucency in on the left side immediately above the diaphragm 3
Fluoroscopy
On contrast swallow:
- up to 10% of patients have a false negative result 3, 10
- may directly demonstrate contrast medium leakage, often at a supradiaphragmatic level
- submucosal contrast collections
- oesophagopleural fistula
CT
Features reported on unenhanced CT scans include the presence intramural haematoma with a typical localisation and peri-oesophageal air collections indicating oesophageal perforation 2. Post contrast CT imaging may show direct contrast leakage/tracts and oesophageal wall thickening.
Other reported findings include
- presence of peri-aortic air tracts
- pneumothorax: has a left sided predilection
- pneumomediastinum
- pleural effusion: usually left sided.
- mediastinal fluid collections
- oral contrast extravasation from the esophagus
- oesophageal wall thickening 6
- gas within soft tissue spaces of the chest wall and neck, and around the great vessels
- gas extending into spinal epidural, peritoneal and retroperitoneal spaces
Differential diagnosis
- esophageal perforation from iatrogenic injury
- Mallory-Weiss tear: partial thickness tear
- epiphrenic diverticulum: mimicking pneumomediastinum
- esophageal or pulmonary malignancy causing oesophagopleural fistula
Treatment and prognosis
ItMediastinal infection and sepsis can potentially cause abe life threatening sepsis that can result in a(mortality as high mortality (~35as 35%) 1), especially if there is a delay in diagnosis. Surgery is the gold standard treatment however there is emerging use of conservative methods, namely esophageal stenting. Mortality has been to be as low as 6.2% when identified and treated in the first 24 hours 11.
Complications
- acute mediastinitis
- oesophagopleural fistula
- pneumonia
- empyema
- sepsis
History and etymology
It is named after Hermann Boerhaave,professor of clinical medicine, Netherlands (1668-1738) 4. The syndrome was described after the case of Dutch admiral Baron Jan von Wassenaer, who died of the condition.
-<p><strong>Boerhaave syndrome</strong> refers to an <a href="/articles/oesophageal-perforation">oesophageal rupture</a> secondary to forceful vomiting.</p><h4>Epidemiology</h4><p>It tends to be more prevalent in males. The estimated incidence is ~ 1:6000.</p><h4>Clinical presentation</h4><p>They are often associated with the clinical triad of vomiting, chest pain and subcutaneous emphysema (Mackler's triad). This condition was universally fatal before the age of surgery.</p><h4>Pathology</h4><p>It is thought occur due to a forceful ejection of gastric contents in an unrelaxed oesophagus against a closed glottis. The tears are vertically oriented, 1-4 cm in length. Approximately 90% occur along the left posterolateral wall (left diaphragmatic crus) of the distal <a href="/articles/oesophagus">oesophagus</a>.</p><h4>Radiographic features</h4><h5>Plain radiograph</h5><p><a href="/articles/chest-radiograph">Chest radiograph</a> findings are often non-specific and include <a href="/articles/pneumomediastinum">pneumomediastinum</a> and/or <a href="/articles/pleural-effusion">pleural effusion</a>. Gas may also be seen with the soft tissue spaces of the chest wall and the neck. Other signs include</p><ul><li>- +<p><strong>Boerhaave syndrome</strong> refers to an <a href="/articles/oesophageal-perforation">oesophageal rupture</a> secondary to forceful vomiting and retching.</p><h4>Epidemiology</h4><p>It tends to be more prevalent in males, with alcoholism a risk factor. The estimated incidence is ~ 1:6000.</p><h4>Clinical presentation</h4><p>They are often associated with the clinical triad of vomiting, chest pain and subcutaneous emphysema (Mackler's triad). Other symptoms include epigastric pain, back pain, dyspnoea and shock. This condition was universally fatal before the age of surgery.</p><h4>Pathology</h4><p>It is thought occur due to a forceful ejection of gastric contents in an unrelaxed oesophagus against a closed glottis. The tears are vertically oriented, 1-4 cm in length. Approximately 90% occur along the left posterolateral wall of the distal <a href="/articles/oesophagus">oesophagus</a>, 3-6cm above the esophageal hiatus of the diaphragm <sup>10</sup>.</p><h4>Radiographic features</h4><h5>Plain radiograph</h5><p><a href="/articles/chest-radiograph">Chest radiograph</a> findings are often non-specific and the may be completely absent. The classic CXR findings include <a href="/articles/pneumomediastinum">pneumomediastinum</a>, left <a href="/articles/pleural-effusion">pleural effusion</a> and left <a href="/articles/pneumothorax">pneumothorax</a>. Gas may also be seen with the soft tissue spaces of the chest wall and the neck. Other signs include</p><ul><li>
-<li>up to 10% of patients have a false negative result <sup>3</sup>- +<li>up to 10% of patients have a false negative result <sup>3, 10</sup>
- +<li>submucosal contrast collections</li>
- +<li><a href="/articles/oesophageal-pleural-fistula">oesophagopleural fistula</a></li>
- +<li>pleural effusion: usually left sided.</li>
-<li>oesophageal thickening <sup>6</sup>- +<li>oral contrast extravasation from the esophagus</li>
- +<li>oesophageal wall thickening <sup>6</sup>
-</ul><h4>Treatment and prognosis</h4><p>It can potentially cause a life threatening sepsis that can result in a high mortality (~35%) <sup>1</sup>.</p><h4>History and etymology</h4><p>It is named after <strong>Hermann Boerhaave</strong>,<strong> </strong>professor of clinical medicine, Netherlands (1668-1738)<sup> 4</sup>. The syndrome was described after the case of Dutch admiral Baron Jan von Wassenaer, who died of the condition. </p>- +</ul><h4>Differential diagnosis</h4><ul>
- +<li>esophageal perforation from iatrogenic injury</li>
- +<li>
- +<a href="/articles/mallory-weiss-tear-1">Mallory-Weiss tear</a>: partial thickness tear</li>
- +<li>
- +<a href="/articles/epiphrenic-diverticulum">epiphrenic diverticulum</a>: mimicking pneumomediastinum</li>
- +<li>esophageal or pulmonary malignancy causing <a href="/articles/oesophageal-pleural-fistula">oesophagopleural fistula</a>
- +</li>
- +</ul><h4>Treatment and prognosis</h4><p>Mediastinal infection and sepsis can be life threatening (mortality as high as 35% <sup>1</sup>), especially if there is a delay in diagnosis. Surgery is the gold standard treatment however there is emerging use of conservative methods, namely esophageal stenting. Mortality has been to be as low as 6.2% when identified and treated in the first 24 hours <sup>11</sup>.</p><h5>Complications</h5><ul>
- +<li>acute <a href="/articles/peroneus-brevis-1">mediastinitis</a>
- +</li>
- +<li><a href="/articles/oesophageal-pleural-fistula">oesophagopleural fistula</a></li>
- +<li><a href="/articles/pneumonia">pneumonia</a></li>
- +<li><a href="/articles/thoracic-empyema-1">empyema</a></li>
- +<li>sepsis</li>
- +</ul><h4>History and etymology</h4><p>It is named after <strong>Hermann Boerhaave</strong>,<strong> </strong>professor of clinical medicine, Netherlands (1668-1738)<sup> 4</sup>. The syndrome was described after the case of Dutch admiral Baron Jan von Wassenaer, who died of the condition. </p>
References changed:
- 9. Katabathina VS, Restrepo CS, Martinez-Jimenez S et-al. Nonvascular, nontraumatic mediastinal emergencies in adults: a comprehensive review of imaging findings. Radiographics. 2011;31 (4): 1141-60. <a href="http://dx.doi.org/10.1148/rg.314105177">doi:10.1148/rg.314105177</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/21768244">Pubmed citation</a><span class="auto"></span>
- 10. de Schipper JP, Pull ter Gunne AF, Oostvogel HJ et-al. Spontaneous rupture of the oesophagus: Boerhaave's syndrome in 2008. Literature review and treatment algorithm. Dig Surg. 2009;26 (1): 1-6. <a href="http://dx.doi.org/10.1159/000191283">doi:10.1159/000191283</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/19145081">Pubmed citation</a><span class="auto"></span>
- 11. Shaker H, Elsayed H, Whittle I et-al. The influence of the 'golden 24-h rule' on the prognosis of oesophageal perforation in the modern era. Eur J Cardiothorac Surg. 2010;38 (2): 216-22. <a href="http://dx.doi.org/10.1016/j.ejcts.2010.01.030">doi:10.1016/j.ejcts.2010.01.030</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/20304664">Pubmed citation</a><span class="auto"></span>
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