Boerhaave syndrome refers to an oesophageal rupture secondary to forceful vomiting and retching.
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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 (Mackler's triad) of vomiting, chest pain and subcutaneous emphysema. Other symptoms include epigastric pain, back pain, dyspnoea and shock. This condition was universally fatal before the age of surgery.
Pathology
It is thought to occur due to a forceful ejection of gastric contents in an unrelaxed oesophagus against a closed upper oesophageal sphincter/cricopharyngeus. The tears are vertically orientated, 1-4 cm in length. Approximately 90% occur along the left posterolateral wall of the distal oesophagus, 3-6 cm above the oesophageal hiatus of the diaphragm 10.
Radiographic features
Plain radiograph
Chest radiograph findings are often non-specific, and the radiograph may be normal. The classic chest radiographic findings include pneumomediastinum, left pleural effusion and left pneumothorax. Gas may also be seen with the soft tissue spaces of the chest wall and the neck.
Another sign that may be present is the Naclerio V sign, which describes a focal, sharply marginated region of paraspinal radiolucency 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/tracks and oesophageal wall thickening.
Other reported findings include:
- the presence of peri-aortic air tracks
- pneumothorax: has a left sided predilection
- pneumomediastinum
- pleural effusion: usually left sided
- mediastinal fluid collections
- oral contrast extravasation from the oesophagus
- 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
- oesophageal perforation from iatrogenic injury
- Mallory-Weiss tear: partial thickness tear
- epiphrenic diverticulum: mimicking pneumomediastinum
- oesophageal or pulmonary malignancy causing oesophagopleural fistula
Treatment and prognosis
Mediastinal infection and sepsis can be life-threatening (mortality as high as 35% 1), especially if there is a delay in diagnosis. Surgery is the gold standard treatment. However, there is an emerging use of conservative methods, namely oesophageal stenting. Mortality can 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 (1668-1738), a Dutch professor of clinical medicine 4,8. The syndrome was described after the case of Dutch Admiral Baron Jan von Wassenaer, who died of the condition.
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