Boerhaave syndrome refers to an oesophageal rupture secondary to forceful vomiting and retching.
It tends to be more prevalent in males, with alcoholism a risk factor. The estimated incidence is ~ 1:6000.
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.
It is thought to 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 oesophagus, 3-6cm above the esophageal hiatus of the diaphragm 10.
Chest radiograph findings are often non-specific, and the may be completely absent. The classic CXR 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. 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
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
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
- the presence of peri-aortic air tracts
- pneumothorax: has a left sided predilection
- 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
- 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
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 esophageal stenting. Mortality has been to be as low as 6.2% when identified and treated in the first 24 hours 11.
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.
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- oesophageal dysmotility
- oesophageal tumours
- benign oesophageal neoplasms
- malignant oesophageal neoplasms
- gastro-oesophageal reflux disease
- oesophageal stricture