CASE 8911 Published on 05.12.2011

A case of Boerhaave\'s syndrome- spontaneous oesophageal perforation

Section

Chest imaging

Case Type

Clinical Cases

Authors

Dr Peter S. Blair

Patient

69 years, female

Categories
Area of Interest Thorax, Gastrointestinal tract, Head and neck, Lung, Contrast agents, Anatomy ; Imaging Technique Digital radiography, CT
Clinical History
A 67-year-old woman attended the emergency department following a choking episode with associated chest pain whilst eating. On attendance in the emergency department, surgical emphysema was noted.
Imaging Findings
Chest radiograph confirmed surgical emphysema, pneumomediastinum and pneumopericardium [Figure 1].
A CT examination of neck, chest and abdomen was then performed. The CT of neck, chest and abdomen was performed following the administration of oral and intravenous contrast medium. Extensive free gas throughout the neck and chest was noted [Figure 2]. Significant pneumomediastinum was seen to extend along the soft tissue planes into the pericardium and superiorly into the soft tissues of the neck and upper chest producing surgical emphysema. It also extended inferiorly to surround the aorta at the aortic hiatus. There was a moderately sized, right-sided pneumothorax and a tiny left-sided pneumothorax. A right-sided pleural effusion was present, which was hyperdense in keeping with leakage of oral contrast medium into the pleural cavity and there was collapse/consolidation of the right lung base. Oral contrast medium was seen within the oesophagus and free contrast medium was seen exiting the distal third of oesophagus and entering the mediastinum and pleural cavity [Figure 2j]. The patient underwent a posterolateral thoracotomy with a primary repair of the oesophagus and drainage of the right hydropneumothorax. Postoperatively the patient made a slow but full clinical recovery.
Discussion
This case demonstrates the classic clinical presentation and imaging findings of Boerhaave's syndrome or spontaneous oesophageal perforation.

Boerhaave's syndrome, or postemetic rupture of the oesophagus, first described in 1724, represents a special instance of barogenic trauma to the oesophagus, leading to a challenging clinical syndrome that still bears its describer's name [1, 2].

Vomiting is the most frequent cause of Boerhaave's syndrome, but it is certainly not the only cause and it has been reported with straining, weight-lifting, severe coughing, childbirth, blunt trauma, seizures, Cushing's ulcers, seasickness and asthma. In these cases, a rapid rise in intraluminal pressure with sudden distension of the distal oesophagus causes ''barogenic rupture'' [3].

The classical presentation of Boerhaave's syndrome is vomiting, chest pain and subcutaneous emphysema, also called Mackler's triad and was present in this case [4]. Regardless of the cause of oesophageal perforation, a fulminant mediastinal inflammatory response may result from extrusion of bacteria and enzyme rich salivary, gastric, and biliary excretions. 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. Fluid transit across excoriated mediastinal and pleural surfaces can lead to systemic hypovolaemia, hypoperfusion, systemic inflammation, sepsis and multisystem organ dysfunction, typically affecting the respiratory apparatus first. Left untreated, this injury has a mortality approaching 100% [2].

It stands to reason that the longer the course of undetected mediastinal soilage, the worse the patient's condition is likely to be at the time of clinical evaluation. Indeed, delayed diagnosis and therapy have been associated with poorer outcomes historically [2].

Although surgical emphysema is pathognomonic, in patients with spontaneous oesophageal rupture it may or may not be evident in patients who present within one hour of perforation and an essential element in diagnosis of these patients is to maintain a high index of suspicion [5].

Chest radiograph is the initial imaging procedure and the simple erect film yields the most information [3, 5]. Virtually 80% of cases have a left pneumothorax plus an effusion on chest radiograph [6], however, a negative or normal chest radiograph does not suffice. Contrast oesophagography, using a water soluble agent initially, followed by a barium study if the initial study is negative, represents the most reliable test for demonstrating a perforation and is mandatory for complete evaluation. Contrast enhanced CT may be useful, as in this case, and is particularly useful when contrast studies are negative or impractical when the patient is intubated/sedated [7].

The goals of initial therapy are the same: resuscitation and limiting further mediastinal contamination with cessation of oral intake, antibiotic therapy and nasogastric decompression. In the majority of patients, operative intervention is required like in the presented case [2].

The case clearly illustrates the clinical and imaging findings of Boerhaave's syndrome. The CT findings allowed a prompt diagnosis and speedy surgical repair.
Differential Diagnosis List
Boerhaave's syndrome - spontaneous oesophageal perforation
Airway trapping secondary to airway narrowing or mucus plugging
Straining against a closed glottis like in weight lifting
Blunt trauma with injury to the trachea or bronchus
Sinus fracture
Iatrogenic manipulation following dental extraction
Perforation with dissection of air into mediastinum from retroperitoneal space
Final Diagnosis
Boerhaave's syndrome - spontaneous oesophageal perforation
Case information
URL: https://www.eurorad.org/case/8911
DOI: 10.1594/EURORAD/CASE.8911
ISSN: 1563-4086