Spontaneous esophageal rupture is a rare and commonly known as Boerhaave syndrome.
Boerhaave syndrome usually secondary to vomiting after heavy food and alcohol intake, but any action that abruptly increases intra-abdominal pressure against a closed superior esophageal sphincter can be responsible.
Boerhaave syndrome is rare, accounting for 15% of all cases of esophageal perforation.
Most instances are iatrogenic.
Frequency 3 in 100,000 in the U.S.
Boerhaave syndrome is more commonly observed in males than in females.
Iatrogenic perforation has no gender preference.
Iatrogenic causes account for 50-75% of esophageal perforations.
Boerhaave syndrome is most common among patients aged 40-60 years old.
Since iatrogenic perforations are associated with preexisting pathology they are more common in fifth and later decades of life.
Rigid endoscopy carries a perforation rate 0.1-0.4%, while that of flexible endoscopy varies from 0.01-0.06%.
When procedures are undertaken, such as pneumatic balloon dilatation, rates of perforation increase significantly.
Perforation rate increased in the presence of a large hiatal hernia or esophageal diverticula.
Distribution in the esophagus is 27% in the cervical area, 54% in the intrathoracic area and 19% in the intraabdominal area.
Most common cause today is related to medical instrumentation.
Estimated that 65% of cases related to medical instrumentation.
Other causes include postemesis,16%, and trauma, 11%.
All other causes such as peptic ulcer disease, foreign bodies occur with a frequency of approximately 1%.
Potentially lethal condition.
Rapid diagnosis and therapy essential for best chance for survival.
Delay in diagnosis is common, resulting in significant morbidity and mortality.
The esophagus is vulnerable to perforation due to the lack of a serosal layer, which provides stability through elastin and collagen fibers.
Perforation may be due to direct piercing, shearing, bursting from radial forces, and thinning from necrosis of the esophageal wall.
Penetrating trauma to the neck associated with 2-9% of cases, thinning of esophageal membrane secondary to variceal sclerotherapy accounts for 1-3% of cases and foreign body or toxic ingestions account for 5-15%of cases.
Iatrogenic injury by esophageal instrumentation is the leading cause of perforation by endoscopic piercing or shearing injuries.
Iatrogenic etiologies accounting for up to 85% of cases, and include Instrumentation endoscopy, sclerotherapy, variceal ligation, pneumatic dilation, bougie management, and laser treatment.
Placement of nasogastric, and Blakemore tubes are uncommon iatrogenic causes.
Iatrogenic perforations often occur near the pharyngoesophageal junction where the wall is weakest.
The esophagus is surrounded by loose connective tissue and the infectious and inflammatory response to perforation can easily affect nearby vital organs.
Esophageal tumor or stricture increases the likelihood of perforation.
Perforation during surgery most often occurs in the abdominal esophagus.
Spontaneous esophageal rupture, also known as Boerhaave syndrome, occurs secondary to a sudden increase in intraluminal pressures.
Spontaneous esophageal rupture usually is due to violent vomiting or retching, and often follows heavy food and alcohol intake.
Perforation occurs in the lower third of the esophagus in about 90% of cases.
The esophageal tear is in the left posterolateral region in 90% of cases.
The predilection for left-side is due to the lack of adjacent supporting structures, thinning of the musculature in the lower esophagus, and anterior angulation of the esophagus at the left diaphragmatic crus.
Fifty percent of ruptures occur in patients with gastroesophageal reflux disease.
The cervical esophagus is the most common site of perforation by trauma, almost uniformly penetrating.
Toxic ingestions and foreign bodies can directly damage the cervical esophagus, become lodged and cause erosion of the muscle wall.
Intraoperative esophageal perforation can occur with fundoplication, accounting for around 2% of all perforations.
The mortality rate can be very high, and varying from 5-75%.
The mortality rate correlates with delays in both presentation and diagnosis.
The highest mortality rates are associated the Boerhaave syndrome , followed by iatrogenic and traumatic perforations.
Cervical perforations have the lowest mortality rates, compared with abdominal and thoracic perforations due to containment of potential contamination by tight fascial attachments.
The morbidity and mortality in esophageal perforation is due to inflammatory response to gastric contents in the mediastinum, pleural spaces, and adjacent tissues, and to spread of infection to surrounding tissues.
Complications include pneumonia, mediastinitis, sepsis, empyema, adult respiratory distress syndrome, and multiorgan failure.
Esophageal injuries secondary to penetrating trauma often involve adjacent structures such as the spinal cord and trachea.
Patients with spontaneous esophageal rupture generally present with severe vomiting or retching followed by acute, severe chest or epigastric pain.
Esophageal perforation has been reported to occur following blunt trauma, childbirth, coughing, fits of laughing, forceful swallowing, hiccuping, seizures, straining, and weight lifting.
Following esophageal instrumentation the onset of fever or pains in the neck, abdomen, or upper back, shortness of breath, dysphagia, dysphonia, or odynophagia suggest the presence of esophageal perforation.
Patients may also present with low back pain, shoulder pain from diaphragmatic irritation, increased discomfort when lying down, or true acute abdomen.
A history of ingestion of a caustic substance or foreign body preceding any of the above symptoms may indicate perforation.
The presence of gastrointestinal abnormalities such as GERD, hiatal hernia, carcinoma, strictures, radiation therapy, Barrett esophagus, varices, achalasia, or infection increases the risk of perforation.
Hematemesis is not a typical feature of esophageal perforation.
Physical examination may show the presence of subcutaneous emphysema in the neck or chest in up to 60% of ceases but requires at least an hour to develop after the initial injury.
Tachycardia and tachypnea and fever may be present on clinical examination.
The Mackler triad, consisting of vomiting, chest pain, and subcutaneous emphysema, is classically associated with spontaneous esophageal rupture.
The triad above is known as the Mackler triad, and is present in about 50% of cases of EP.
A raspy, crunching sound heard over the precordium with each heartbeat caused by mediastinal emphysema, may be present with thoracic or abdominal esophageal perforations.
In cases of delayed diagnosis the patient may be critically ill with significant hypotension.
Differential Diagnoses include: acute coronary syndrome, abdominal or dissecting aortic aneurysm, gastritis, peptic ulcer disease, acute myocardial infarction, cardiac tamponode, and pericarditis, pancreatitis, pneumonia, pneumothorax, and pulmonary embolus.
Laboratory studies contribute little to the diagnosis, but if a diagnostic thoracentesis is performed on a pleural effusion, it may show the presence of food particles, a pH< 6, or an elevated pleural fluid amylase.
Chest X-rays reveal abnormalities in about 90% of cases that support the diagnosis of EP, and those findings include: peumomediastinum and subcutaneous emphysema found shortly after and are highly suggestive of perforation.
Later radiographic findings include: mediastinal air-fluid levels, pleural effusions, free air under diaphragm,, hydropneumothorax and pneumothorax.
Lateral neck X-rays may demonstrate air,in cases of cervical perforations.
Patients should undergo a contrast esophagogram, with a water soluble agent, if suspected to have an esophageal perforation.
The use of a water soluble agent, such as Gastrograffin, has a sensitivity of 60-75% in making the diagnosis of EP, by demonstrating an esophageal leak and should be the initial imaging diagnostic study.
If the initial study is negative, a barium study should be performed as it has a higher sensitivity of 90% for detecting small perforations.
A barium esophogram is not the initial study of choice, because it may cause a severe inflammatory mediastinitis in the presence of a EP.
Esophagoscopy can often visualize a perforation, especially with in acute traumatic perforations.
Esophagoscopy is not helpful or appropriate when small mucosal tears are suspected, as insufflated air can increase the degree of perforation.
With a high degree of clinical suspicion of an EP a contrast-enhanced CT scan of the chest should be performed if it is not possible to obtain a contrast esophagogram, or if the esophagogram was negative.
CT chest may indicate the presence of mediastinal air, extravasated contrast material, periesophageal fluid collections, pleural effusions, or communication of an air-filed esophagus with an adjacent mediastinal air-fluid collection.
Any patient with an esophageal tear should be expeditiously transported to the hospital with intravenous access, supplemental oxygen, a secure airway, and pain medication as necessary.
Broad-spectrum intravenous antibiotics should be instituted early in the evaluation of the patient suspected of having an esophageal perforation, and should be continued or 10 days in patients managed conservatively.
Patients are made NPO and have a nasogastric tube placed to clear gastric contents.
Thoracostomy may be needed decompress the chest of accumulated fluid.
In patients with small tears and minimal extraesophageal involvement non-operative conservative treatment Is provided.
Intravenous parenteral nutrition is considered for a prolonged course.
Repeat esophageal studies are necessary to determine conservative management treatment success.
Surgery may be necessary for primary repair, stent placement, resection of perforation, or drain placement.
The prognosis can be poor, with high rates of morbidity and mortality, especially if diagnosis is delayed.
The prognosis of cervical iatrogenic perforation is better than that of spontaneous perforation.