Boerhaave disease-easily missed in ED with 90% mortality

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http://www.emedicine.com/emerg/topic176.htm

Esophageal perforation initially was described in a landmark paper by Hermann Boerhaave in 1724. It was one of the first clinicopathological correlations, and described spontaneous rupture of the esophagus of Lord High Admiral Wassenaer of the Dutch navy. The admiral had a transverse tear, an event that rarely occurs.

Boerhaave syndrome is a potentially devastating injury. Rapid diagnosis and therapy provide the best chance for survival; however, delay in diagnosis is common, resulting in substantial morbidity and mortality. This article discusses the causes, investigation, and initial therapy for this potentially lethal gastrointestinal perforation.

Esophageal perforations also may be caused by instrumentation of the esophagus (eg, after gastric lavage or gastroscopy).


Pathophysiology: Two distinct entities, esophageal rupture and esophageal perforation, are described.

Esophageal rupture (ie, Boerhaave syndrome) occurs because the esophagus, unlike the rest of the alimentary tract, lacks a serosal layer, which usually contains collagen and elastic fibers. Thus, the wall is weaker and may rupture at a lower intraluminal pressure. Vomiting usually precedes development of spontaneous perforation.

In more than 90% of cases, perforation occurs in the lower third of the esophagus; most frequently, the tear is in the left posterolateral region (90%). Tears usually are 0.6-8.9 cm long. Predilection for left-side perforations may be caused by lack of adjacent supporting structures, thinning of musculature in lower esophagus, and anterior angulation of esophagus at left diaphragmatic crus. Tears usually extend superiorly. Perforation of esophagus resulting from instrumentation or trauma does not appear to have a specific site predilection.


Frequency:


In the US: Boerhaave syndrome is rare, with an estimated incidence of 1 in more than 6,000 patients. It accounts for 15% of cases of esophageal rupture or perforation.
Iatrogenic perforations account for approximately 55% of esophageal perforations. In a large case series of patients undergoing esophagoscopy, incidence of perforations was 1.7% (17 cases in 1011 procedures). Of these 17 cases, tumors were found in 8 and achalasia was found in 4; the remaining 5 had strictures or an esophageal anastomosis. The generally accepted complication rates are from 2-6% after pneumatic dilatation for achalasia.

All other cases of esophageal perforation are caused by foreign bodies or traumatic perforations.

Esophageal rupture secondary to blunt external trauma is very rare, with few cases reported in world literature.

Mortality/Morbidity:

Even with prompt therapy, the mortality rate is very high, varying from 30-50%. With delay in diagnosis, the mortality rate exceeds 90%.
Mortality rates from perforation caused by instrumentation (ie, iatrogenic perforation) are lower than other causes (15-20%), although clearly still very significant.
Race:

No information on racial predilection is available.
Sex:

Boerhaave disease generally is associated with vomiting and customarily occurs after drinking and eating binges. It is more commonly observed in males than females.
Age:

Spontaneous esophageal perforation usually is observed in patients aged 40-60 years.
Boerhaave syndrome is very rare in younger patients. Isolated case reports have documented its occurrence in children.
Iatrogenic perforation generally is associated with preexisting pathology; thus, it is a disease of the fifth and later decades.

CLINICAL

History:

Reports suggest that approximately 50% of patients with Boerhaave syndrome are not diagnosed upon initial presentation to the ED. Delay is caused by rareness of presentation and nonspecific findings on history and physical examination.
Classic presentation for spontaneous rupture is vomiting followed by severe chest pain. However, many patients have less dramatic presentations.
In addition to classical pictures of vomiting, Boerhaave syndrome has been ascribed to straining, childbirth, weight lifting, severe coughing, hiccuping, blunt trauma, seizures, asthma, and forceful swallowing.
In several large series, patients have complained of abdominal pain, often epigastric, and chest pain.
Dyspnea is a common complaint, occurring in 30-54% of patients. Pain may radiate to the neck.
Hematemesis usually is not profuse. It occurs in as many as 55% of patients.
Iatrogenic perforation usually occurs during a procedure and may be at any point in the esophagus. Although usually detected at time of injury, delayed presentation is reported. This injury usually coincides with preexisting esophageal pathology (eg, strictures, carcinoma).
Physical:

Physical examination usually is nonspecific; however, subcutaneous emphysema may be present, palpable in neck or chest. This sign takes at least an hour to develop after injury.
The triad of vomiting, chest pain, and subcutaneous emphysema is called Mackler triad; however, this cannot be relied on for diagnosis because it is uncommon.
Patients usually appear acutely ill, having tachycardia and tachypnea. Depending on the length of time to presentation, patients may have a fever. If a delay in presentation or diagnosis has occurred, patients may present hypotensive and septic.
Examination of chest may reveal decreased breath sounds on the side of perforation, usually the left side. A crunching sound (ie, Hamman sign) may be heard with each heartbeat in as many as 20% of cases.
Causes:

As noted above, Boerhaave syndrome is responsible for approximately 15% of esophageal perforations. Iatrogenic perforations account for approximately 55%, and foreign body perforation and trauma account for the remainder. Direct trauma causing injury to esophagus is often secondary to gunshot wounds and frequently is associated with injury to trachea and spinal cord.

DIFFERENTIALS

Dissection, Aortic
Gastritis and Peptic Ulcer Disease
Myocardial Infarction
Pancreatitis
Pericarditis and Cardiac Tamponade
Pneumonia, Aspiration
Pneumonia, Bacterial
Pneumonia, Empyema and Abscess
Pneumothorax, Iatrogenic, Spontaneous and Pneumomediastinum
Pulmonary Embolism

Other Problems to be Considered:

Mallory-Weiss tear

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http://www.emedicine.com/ped/topic1359.htm

In 1929, Kenneth Mallory and Soma Weiss first described a syndrome characterized by esophageal bleeding caused by a mucosal tear in the esophagus as a result of forceful vomiting or retching. The initial description was associated with alcoholic bingeing; however, with the advent of endoscopy, Mallory-Weiss tears have been diagnosed in many patients with no antecedent history of alcohol intake. Although the tear typically occurs after repeated episodes of vomiting or retching, it may occur after a single incident. While most of the written reports of these tears relate to adults, Mallory-Weiss tears also occur in children.


Pathophysiology: Any disorder that initiates vomiting may result in the development of a Mallory-Weiss tear, which develops as a linear laceration at the gastroesophageal junction because the esophagus and stomach are cylindrical. The cylindrical shape makes longitudinal tears occur more easily than circumferential tears. These tears have been postulated to occur either by a rapid increase in intragastric pressure and distention, which increases the forceful fluid ejection through the esophagus, or secondary to a significant change in transgastric pressure (ie, difference in pressure across the gastric wall) because negative intrathoracic pressure and positive intragastric pressure leads to distortion of the gastric cardia, resulting in a gastric or esophageal tear. Because of these factors, Mallory-Weiss tears occur more commonly in people with hiatal hernias.


Frequency:


In the US: Mallory-Weiss tears are the cause for approximately 10-15% of all episodes of hematemesis in adults; however, these tears occur much less commonly in children (<5% of all upper GI bleeding episodes).
Mortality/Morbidity: The major complication of a Mallory-Weiss tear is bleeding. Patients present with variable bleeding, which can range from a few specks or streaks of blood mixed with mucus to copious amounts of fresh red blood. In adults, shock occurs in up to 20% of patients bleeding from Mallory-Weiss tears who present to emergency departments, while up to 45% of patients develop postural hypotension. In contrast, children rarely have hemodynamic instability.