|Classification and external resources|
Oesophageal rupture (also known as Boerhaave's syndrome) is rupture of the oesophageal wall. 56% of oesophageal perforations are iatrogenic, usually due to medical instrumentation such as an endoscopy or paraoesophageal surgery. In contrast, the term Boerhaave's syndrome is reserved for the 10% of oesophageal perforations which occur due to vomiting.
Spontaneous perforation of the oesophagus most commonly results from a sudden increase in intraoesophageal pressure combined with relatively negative intrathoracic pressure caused by straining or vomiting (effort rupture of the oesophagus or Boerhaave's syndrome). Other causes of spontaneous perforation include caustic ingestion, pill oesophagitis, Barrett's oesophagus, infectious ulcers in patients with AIDS, and following dilation of oesophageal strictures.
In most cases of Boerhaave's syndrome, the tear occurs at the left postero-lateral aspect of the distal oesophagus and extends for several centimeters. The condition is associated with high morbidity and mortality and is fatal in the absence of therapy. The occasionally nonspecific nature of the symptoms may contribute to a delay in diagnosis and a poor outcome. Spontaneous effort rupture of the cervical oesophagus, leading to localized cervical perforation, may be more common than previously recognized and has a generally benign course. Preexisting oesophageal disease is not a prerequisite for esophageal perforation but it contributes to increased mortality
Signs and symptoms
The classic history of oesophageal rupture is one of severe retching and vomiting followed by excruciating retrosternal chest and upper abdominal pain. Odynophagia, tachypnea, dyspnea, cyanosis, fever, and shock develop rapidly thereafter.
The following features were described in an illustrative review:
- A history of alcoholism or heavy drinking was present in 40 percent; presentation during an episode of alcoholism may contribute to a delay in diagnosis.
- A history of gastroduodenal ulcer disease was present in 41 percent.
- Pain occurred in 83 percent, vomiting in 79 percent, dyspnea in 39 percent, and shock in 32 percent.
Physical examination is usually not helpful, particularly early in the course. Subcutaneous emphysema (crepitation) is an important diagnostic finding but is not very sensitive, being present in only 9 of 34 patients (27 percent) in one series . A pleural effusion may be detected. A case report described presentation with an Enterococcal bacterial pericardial effusion.
Mackler's triad which includes chest pain, vomiting and subcutaneous emphysema, while classical, is only present in 14% of people.
Pain can occasionally radiate to the left shoulder, causing physicians to confuse an oesophageal perforation with a myocardial infarction.
It may also be audibly recognized as Hamman's sign.
Common misdiagnoses include myocardial infarction, pancreatitis, lung abscess, pericarditis, and spontaneous pneumothorax. If oesophageal perforation is suspected, even in the absence of physical findings, contrast radiographic studies of the esophagus and a CT scan should be obtained promptly.
Oesophageal rupture in Boerhaave syndrome is thought to be the result of a sudden rise in internal esophageal pressure produced during vomiting, as a result of neuromuscular incoordination causing failure of the cricopharyngeus muscle (a sphincter within the esophagus) to relax. The syndrome is commonly associated with the consumption of excessive food and/or alcohol as well as eating disorders such as bulimia.
Currently the most common cause of oesophageal perforation is iatrogenic. However, iatrogenic perforations, while still constituting a serious medical condition, are easier to treat and less prone to complications, particularly mediastinitis and sepsis. This is because they usually do not involve contamination of the mediastinum with gastric contents.
The diagnosis of Boerhaave's syndrome is suggested on the plain chest radiography and confirmed by chest CT scan. The initial plain chest radiograph is almost always abnormal in patients with Boerhaave's syndrome and usually reveals mediastinal or free peritoneal air as the initial radiologic manifestation. With cervical oesophageal perforations, plain films of the neck show air in the soft tissues of the prevertebral space.
Hours to days later, pleural effusion(s) with or without pneumothorax, widened mediastinum, and subcutaneous emphysema are typically seen. CT scan may show oesophageal wall edema 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.
The diagnosis of oesophageal perforation could also be confirmed by water-soluble contrast oesophagram (Gastrograffin), which reveals the location and extent of extravasation of contrast material. Although barium is superior in demonstrating small perforations, the spillage of barium sulfate into the mediastinal and pleural cavities can cause an inflammatory response and subsequent fibrosis and is therefore not used as the primary diagnostic study. If, however, the water-soluble study is negative, a barium study should be performed for better definition.
Endoscopy has no role in the diagnosis of spontaneous esophageal perforation. Both the endoscope and insufflation of air can extend the perforation and introduce air into the mediastinum.
Patients may also have a pleural effusion high in amylase (from saliva), low pH, and may contain particles of food.
With the exception of few case reports describing survival without surgery, the mortality of untreated Boerhaave syndrome is nearly 100%. Its treatment includes immediate antibiotic therapy to prevent mediastinitis and sepsis, surgical repair of the perforation, and if there is significant fluid loss it should be replaced with IV fluid therapy since oral rehydration is not possible. Even with early surgical intervention (within 24 hours) the risk of death is 25%.
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- Radiology Esophageal rupture