Boerhaave syndrome
Encyclopedia
Esophageal rupture is rupture of the esophageal
wall due to vomiting. 56% of esophageal perforations are iatrogenic, usually due to medical instrumentation such as an endoscopy
or paraesophageal surgery. In contrast, the term Boerhaave's syndrome is reserved for the 10% of esophageal perforations which occur due to vomiting.
Spontaneous perforation of the esophagus most commonly results from a sudden increase in intraesophageal pressure combined with negative intrathoracic pressure caused by straining or vomiting (effort rupture of the esophagus or Boerhaave's syndrome). Other causes of spontaneous perforation include caustic ingestion, pill esophagitis, Barrett's ulcer, infectious ulcers in patients with AIDS, and following dilation of esophageal strictures.
In most cases of Boerhaave's syndrome, the tear occurs at the left postero-lateral aspect of the distal esophagus 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 esophagus, leading to localized cervical perforation, may be more common than previously recognized and has a generally benign course. Preexisting esophageal disease is not a prerequisite for esophageal perforation but it contributes to increased mortality
This condition was first documented by the 18th-century physician Herman Boerhaave
, after whom it is named. A related condition is Mallory-Weiss syndrome
.
, tachypnea
, dyspnea
, cyanosis
, fever
, and shock develop rapidly thereafter.
The following features were described in an illustrative review:
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 esophageal perforation with a myocardial infarction.
It may also be audibly recognized as Hamman's sign
.
, pancreatitis
, lung abscess
, pericarditis
, and spontaneous pneumothorax
. If esophageal perforation is suspected, even in the absence of physical findings, contrast radiographic studies of the esophagus and a CT scan should be obtained promptly.
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.
The most common anatomical location of the tear in Boerhaave syndrome is at left posterolateral wall of the lower third of the esophagus, 2–3 cm before the stomach
.
Unfortunately, in the present time, the most common cause of esophageal perforation is iatrogenic. However, it should also be noted that iatrogenic perforations, while still constituting a serious medical condition, are easier to treat and less prone to complications, particularly mediastinitis and sepsis. This owes to the fact that they usually do not involve contamination of the mediastinum with gastric contents.
Hours to days later, pleural effusion(s) with or without pneumothorax, widened mediastinum, and subcutaneous emphysema are typically seen. CT scan may show esophageal wall edema and thickening, extraesophageal air, periesophageal fluid with or without gas bubbles, mediastinal widening, and air and fluid in the pleural spaces, retroperitoneum or lesser sac.
The diagnosis of esophageal perforation could also be confirmed by water-soluble contrast esophagram (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.
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%.
Esophagus
The esophagus is an organ in vertebrates which consists of a muscular tube through which food passes from the pharynx to the stomach. During swallowing, food passes from the mouth through the pharynx into the esophagus and travels via peristalsis to the stomach...
wall due to vomiting. 56% of esophageal perforations are iatrogenic, usually due to medical instrumentation such as an endoscopy
Endoscopy
Endoscopy means looking inside and typically refers to looking inside the body for medical reasons using an endoscope , an instrument used to examine the interior of a hollow organ or cavity of the body. Unlike most other medical imaging devices, endoscopes are inserted directly into the organ...
or paraesophageal surgery. In contrast, the term Boerhaave's syndrome is reserved for the 10% of esophageal perforations which occur due to vomiting.
Spontaneous perforation of the esophagus most commonly results from a sudden increase in intraesophageal pressure combined with negative intrathoracic pressure caused by straining or vomiting (effort rupture of the esophagus or Boerhaave's syndrome). Other causes of spontaneous perforation include caustic ingestion, pill esophagitis, Barrett's ulcer, infectious ulcers in patients with AIDS, and following dilation of esophageal strictures.
In most cases of Boerhaave's syndrome, the tear occurs at the left postero-lateral aspect of the distal esophagus 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 esophagus, leading to localized cervical perforation, may be more common than previously recognized and has a generally benign course. Preexisting esophageal disease is not a prerequisite for esophageal perforation but it contributes to increased mortality
This condition was first documented by the 18th-century physician Herman Boerhaave
Herman Boerhaave
Herman Boerhaave was a Dutch botanist, humanist and physician of European fame. He is regarded as the founder of clinical teaching and of the modern academic hospital. His main achievement was to demonstrate the relation of symptoms to lesions...
, after whom it is named. A related condition is Mallory-Weiss syndrome
Mallory-Weiss syndrome
Mallory–Weiss syndrome or gastro-esophageal laceration syndrome refers to bleeding from tears in the mucosa at the junction of the stomach and esophagus, usually caused by severe retching, coughing, or vomiting.-Causes:...
.
Signs and symptoms
The classic history of esophageal rupture is one of severe retching and vomiting followed by excruciating retrosternal chest and upper abdominal pain. OdynophagiaOdynophagia
Odynophagia is painful swallowing, in the mouth or esophagus. It can occur with or without dysphagia, or difficult swallowing....
, tachypnea
Tachypnea
Tachypnea means rapid breathing. Any rate between 12-20 breaths per minute is normal. Tachypnea is a respiration rate greater than 20 breaths per minute. - Distinction from other breathing terms :...
, dyspnea
Dyspnea
Dyspnea , shortness of breath , or air hunger, is the subjective symptom of breathlessness.It is a normal symptom of heavy exertion but becomes pathological if it occurs in unexpected situations...
, cyanosis
Cyanosis
Cyanosis is the appearance of a blue or purple coloration of the skin or mucous membranes due to the tissues near the skin surface being low on oxygen. The onset of cyanosis is 2.5 g/dL of deoxyhemoglobin. The bluish color is more readily apparent in those with high hemoglobin counts than it is...
, fever
Fever
Fever is a common medical sign characterized by an elevation of temperature above the normal range of due to an increase in the body temperature regulatory set-point. This increase in set-point triggers increased muscle tone and shivering.As a person's temperature increases, there is, in...
, 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
Subcutaneous emphysema
Subcutaneous emphysema, sometimes abbreviated SCE or SE and also called tissue emphysema, or Sub Q air, occurs when gas or air is present in the subcutaneous layer of the skin. Subcutaneous refers to the tissue beneath the cutis of the skin, and emphysema refers to trapped air...
(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 esophageal perforation with a myocardial infarction.
It may also be audibly recognized as Hamman's sign
Hamman's sign
Hamman's sign is a crunching, rasping sound, synchronous with the heartbeat, heard over the precordium in spontaneous mediastinal emphysema produced by the heart beating against air-filled tissues....
.
Differential diagnosis
Common misdiagnoses include myocardial infarctionMyocardial infarction
Myocardial infarction or acute myocardial infarction , commonly known as a heart attack, results from the interruption of blood supply to a part of the heart, causing heart cells to die...
, pancreatitis
Pancreatitis
Pancreatitis is inflammation of the pancreas. It occurs when pancreatic enzymes that digest food are activated in the pancreas instead of the small intestine. It may be acute – beginning suddenly and lasting a few days, or chronic – occurring over many years...
, lung abscess
Lung abscess
Lung abscess is necrosis of the pulmonary tissue and formation of cavities containing necrotic debris or fluid caused by microbial infection....
, pericarditis
Pericarditis
Pericarditis is an inflammation of the pericardium . A characteristic chest pain is often present.The causes of pericarditis are varied, including viral infections of the pericardium, idiopathic causes, uremic pericarditis, bacterial infections of the precardium Pericarditis is an inflammation of...
, and spontaneous pneumothorax
Pneumothorax
Pneumothorax is a collection of air or gas in the pleural cavity of the chest between the lung and the chest wall. It may occur spontaneously in people without chronic lung conditions as well as in those with lung disease , and many pneumothoraces occur after physical trauma to the chest, blast...
. If esophageal perforation is suspected, even in the absence of physical findings, contrast radiographic studies of the esophagus and a CT scan should be obtained promptly.
Pathophysiology
Esophageal 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 sphincterSphincter
A sphincter is an anatomical structure, or a circular muscle, that normally maintains constriction of a natural body passage or orifice and which relaxes as required by normal physiological functioning...
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.
The most common anatomical location of the tear in Boerhaave syndrome is at left posterolateral wall of the lower third of the esophagus, 2–3 cm before the stomach
Stomach
The stomach is a muscular, hollow, dilated part of the alimentary canal which functions as an important organ of the digestive tract in some animals, including vertebrates, echinoderms, insects , and molluscs. It is involved in the second phase of digestion, following mastication .The stomach is...
.
Unfortunately, in the present time, the most common cause of esophageal perforation is iatrogenic. However, it should also be noted that iatrogenic perforations, while still constituting a serious medical condition, are easier to treat and less prone to complications, particularly mediastinitis and sepsis. This owes to the fact that they usually do not involve contamination of the mediastinum with gastric contents.
Diagnosis
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 esophageal 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 esophageal wall edema and thickening, extraesophageal air, periesophageal fluid with or without gas bubbles, mediastinal widening, and air and fluid in the pleural spaces, retroperitoneum or lesser sac.
The diagnosis of esophageal perforation could also be confirmed by water-soluble contrast esophagram (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.
Treatment
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 therapyAntibiotic
An antibacterial is a compound or substance that kills or slows down the growth of bacteria.The term is often used synonymously with the term antibiotic; today, however, with increased knowledge of the causative agents of various infectious diseases, antibiotic has come to denote a broader range of...
to prevent mediastinitis
Mediastinitis
Mediastinitis is inflammation of the tissues in the mid-chest, or mediastinum. It can be either acute or chronic.Acute mediastinitis is usually bacterial and due to rupture of organs in the mediastinum. As the infection can progress rapidly, this is considered a serious condition...
and sepsis, surgical repair of the perforation, and if there is significant fluid loss it should be replaced with IV fluid therapy
Intravenous therapy
Intravenous therapy or IV therapy is the infusion of liquid substances directly into a vein. The word intravenous simply means "within a vein". Therapies administered intravenously are often called specialty pharmaceuticals...
since oral rehydration is not possible. Even with early surgical intervention (within 24 hours) the risk of death is 25%.
External links
- Radiology Esophageal rupture