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Boerhaave syndrome refers to an esophageal 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 (Mackler's triad) of vomiting, chest pain and subcutaneous emphysema. Other symptoms include epigastric pain, back pain, dyspnea 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 esophagus against a closed upper esophageal sphincter/cricopharyngeus. The tears are vertically oriented, 1-4 cm in length. Approximately 90% occur along the left posterolateral wall of the distal esophagus, 3-6 cm above the esophageal hiatus of the diaphragm 10.
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.
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
- esophagopleural fistula
Features reported on unenhanced CT scans include the presence intramural hematoma with a typical localization and peri-esophageal air collections indicating esophageal perforation 2. Post contrast CT imaging may show direct contrast leakage/tracks and esophageal wall thickening.
Other reported findings include:
- the presence of peri-aortic air tracks
- pneumothorax: has a left sided predilection
- pleural effusion: usually left sided
- mediastinal fluid collections
- oral contrast extravasation from the esophagus
- esophageal 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 esophagopleural 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 can 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 (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.