Boerhaave syndrome

Boerhaave syndrome refers to an esophagealoesophageal 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 (Mackler's triad) of vomiting, chest pain and subcutaneous emphysema. Other symptoms include epigastric pain, back pain, dyspneadyspnoea 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 esophagusoesophagus against a closed upper esophagealoesophageal sphincter/cricopharyngeus. The tears are vertically orientedorientated, 1-4 cm in length. Approximately 90% occur along the left posterolateral wall of the distal esophagusoesophagus, 3-6 cm above the esophagealoesophageal 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 hematomahaematoma with a typical localisation and peri-esophageal-oesophageal air collections indicating esophagealoesophageal perforation ². Post contrast CT imaging may show direct contrast leakage/tracks and esophagealoesophageal 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 esophagusoesophagus
• esophagealoesophageal 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

• esophagealoesophageal perforation from iatrogenic injury
• Mallory-Weiss tear: partial thickness tear
• epiphrenic diverticulum: mimicking pneumomediastinum
• esophagealoesophageal 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 esophagealoesophageal 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.