Teaching Case

An 84-year-old woman presented with a sudden onset of dyspnoea and epigastric and left-sided pleuritic chest pain after suffering from diarrhoea and vomiting for the past days. An examination revealed decreased sounds in the left hemithorax, tachypnoea and tachycardia. ABG shows hypoxaemia (75mmHg), hypocapnia (31mmHg), and low bicarbonate (18.8mmol/L). Blood test: Leukocytosis 16.000, CRP 54 and Lactate 4.2

Frontal chest radiograph (Fig. 1) showed moderate left pleural effusion with underlying atelectasis/consolidation of the left lower lung zone. Multiple areas of hyperlucency project over the left midline. Subcutaneous emphysema is seen in the soft tissues of the left lower neck. Contrast-enhanced CT (Fig. 2, 3) depicted extensive free gas throughout the neck and chest. Significant pneumomediastinum was seen extending along the soft tissue planes of the neck and upper chest, producing surgical emphysema. There was a moderately-sized, left-sided pneumothorax as well as a bilateral pleural effusion (Fig. 2). Oral contrast medium was seen within the oesophagus and free contrast medium was seen exiting the distal third of the oesophagus and entering the mediastinum (Fig. 3). The patient underwent a thoracotomy with repair of the oesophagus. Postoperatively the patient made a slow but full clinical recovery.

Boerhaave's syndrome or postemetic rupture of the oesophagus represents a special instance of barogenic trauma to the oesophagus leading to a challenging clinical syndrome [1]. Vomiting represents the most frequent underlying cause. Nevertheless, a myriad of circumstances have been reported such as straining, weight-lifting, severe coughing, childbirth, blunt trauma, seizures, Cushing's ulcers, seasickness and asthma. A rapid rise in intraluminal pressure with sudden distension of the distal oesophagus causes the ''barogenic rupture''.

Boerhaave's syndrome classical presentation consists of vomiting, chest pain and subcutaneous emphysema [2]. Physical examination is not reliable as typical subcutaneous crepitation emphysema is only present in 27% of cases and Meckler triad in 30-50%. An accurate clinical history is therefore mandatory: excessive food/alcohol intake, vomiting and sudden severe chest pain are highly suggestive. When the diagnosis is made in the first 12 hours, mortality rate is about 30-40%; if it takes more than 48h, it rises to 90%. Regardless of its cause a fulminant mediastinal inflammatory response may result from extrusion of bacteria and enzyme-rich salivary, gastric and biliary excretions. Furthermore, circulation of these noxious stimuli throughout the mediastinum and pleural spaces may be exacerbated by the negative intrathoracic pressure that results from the mechanics of ventilation. This results in extensive fluid transit across the excoriated mediastinal and pleural surfaces leading to systemic hypovolaemia, hypoperfusion, systemic inflammation, sepsis and multisystem organ dysfunction, the respiratory system typically resulting affected in the first place. When left untreated this injury has a mortality approaching 100% [1].

Chest radiograph is the initial imaging procedure and the simple erect X-ray delivers the most information [3]. Approximately 80% of cases reveal a left pneumothorax plus an effusion. Noteworthy is the fact that a negative or normal chest radiograph doesn't exclude the diagnosis. Computed tomography (CT) shows oesophageal wall oedema and thickening, extraoesophageal air, perioesophageal fluid with or without gas bubbles, mediastinal widening, and air and fluid in the pleural spaces, retroperitoneum or lesser sac [4]. A high index of suspicion must be maintained in patients presenting within one hour of perforation as surgical emphysema (which represents a pathognomonic finding ) might not be evident at the time [3]. Contrast oesophagography using barium represents the most reliable test to confirm a perforation and is mandatory for complete and accurate evaluation.

Management consists on limiting further mediastinal contamination with cessation of oral intake, antibiotic therapy and nasogastric decompression. The majority of cases require urgent surgical intervention.