Author: Dale K Mueller, MD Clinical Associate Professor of Surgery, Section Chief, Department of Surgery, University of Illinois College of Medicine; Co-Medical Director, Thoracic Center of Excellence, Vice-Chair, Department of Cardiovascular Medicine and Surgery, OSF St Francis Medical Center; Director, Adult ECMO, Cardiovascular and Thoracic Surgeon, HeartCare Midwest, SC.

Background
Almost 300 years ago, Herman Boerhaave, a Dutch physician, described the case of Barron Wassenaer, the Grand Admiral of Holland.[1, 2] In 1724, Boerhaave was called to the bedside of the admiral, who complained of severe chest pain and exclaimed that something had burst in his chest. The admiral had consumed a huge meal, had taken a self-prescribed emetic, and “shortly afterwards he vomited, but only a little and this not easily.” Over the next 16 hours, his condition progressively worsened until he died. Autopsy revealed a rent in an otherwise normal-looking esophagus, with food and medicine in the left chest cavity. Spontaneous esophageal rupture then became known as Boerhaave syndrome.[3]

Until the middle of the 20th century, many similar uniformly fatal cases were described without full explanation. As technology improved, however, instrumental perforation became more common, and the pathophysiologies of rupture, perforation, and esophageal disruption (anastomotic leak) were elucidated, although the definitions of these entities became blurred. This article discusses adult esophageal rupture.

Epidemiology–Frequency
The frequency of esophageal perforation is 3 in 100,000 in the United States. The distribution by location is cervical (27%), intrathoracic (54%), and intra-abdominal (19%). The most common cause of esophageal perforation is medical instrumentation for diagnostic and therapeutic endeavors; in one series, such instrumentation caused 65% of all perforations. The frequencies of other causes include postemetic (16%) and trauma, including postoperative trauma (11%). All other causes (caustic, peptic ulcer disease, foreign body, aortic pathology, and diseases of the esophagus) occur rarely, with a frequency of approximately 1%.

Esophagogastroduodenoscopy is the most common procedure instrumenting the esophagus. Risk of perforation with diagnostic esophagogastroduodenoscopy is extremely low (0.03%). The risk of perforation is increased when therapeutic procedures are performed at the time of endoscopy.

Risk increases as follows:
Esophageal dilation – 0.5%
Esophageal dilation for achalasia – 1.7%
Endoscopic thermal therapy – 1-2%
Endoscopic variceal sclerotherapy – 1-6%
Endoscopic laser therapy – 5%
Photodynamic therapy – 4.6%
Esophageal stent placement – 5-25%
Esophageal perforation is rare with nonendoscopic esophageal instrumentation.

Pathophysiology
The esophagus lacks a serosal layer and is, therefore, more vulnerable to rupture or perforation. Once a perforation (ie, full-thickness tear in the wall) occurs, retained gastric contents, saliva, bile, and other substances may enter the mediastinum, resulting in mediastinitis.

The degree of mediastinal contamination and the location of the tear determine the clinical presentation. Within a few hours, a polymicrobial invasion of bacteria supervenes, which can lead to sepsis and, eventually, death if the patient is not treated with conservative management or surgical intervention.[5] The mediastinal pleura often ruptures, and gastric fluid is drawn into the pleural space by the negative intrathoracic pressure. Even if the mediastinal pleura is not violated, a sympathetic pleural effusion often occurs. This effusion is usually left-sided but can be bilateral. Rarely, isolated right-sided effusions occur.

The site of perforation varies depending upon the cause. Instrumental perforation is common in the pharynx or distal esophagus. Spontaneous rupture may occur just above the diaphragm in the posterolateral wall of the esophagus. Perforations are usually longitudinal (0.6-8.9 cm long), with the left side more commonly affected than the right (90%).

Esophageal perforation remains a highly morbid condition. Mortality rates are reported from 25-89% and are based predominantly on time of presentation and etiology of perforation. Postemetic perforation has a higher reported mortality; it has been reported to occur at 2% per hour. Mortality rates have varied depending on the time from symptomology until treatment was instituted. If treatment is instituted within 24 hours of symptoms, mortality rates are 25%; rates rose to above 65% after 24 hours and 75-89% after 48 hours.

Presentation -History
The classic presentation of spontaneous esophageal rupture is in a middle-aged man with a history of dietary overindulgence and overconsumption of alcohol, with chest pain and subcutaneous emphysema after recent vomiting or retching (Mackler triad). Typical symptoms include the following:

Pain of variable location, commonly in the lower anterior chest or upper abdomen
Vomiting
Subcutaneous emphysema
Neck pain
Dysphagia
Dyspnea
Hematemesis
Melena
Back pain
Atypical symptoms include shoulder pain, facial swelling, hoarseness, and dysphonia. Because spontaneous esophageal rupture is a life-threatening emergency, clinicians should be aware of its atypical presentations.[9]

Physical signs include the following:

Fever
Crepitus
Tachycardia
Tachypnea
Cyanosis
Dyspnea
Upper abdominal rigidity
Shock
Local tenderness
The classic Mackler triad, which includes vomiting, lower chest pain, and cervical subcutaneous emphysema, is present in approximately 50% of cases.

The Anderson triad [/b]also refers to subcutaneous emphysema, rapid respirations, and abdominal rigidity.

Indications for Surgery
Controversy exists regarding indications for surgery for esophageal rupture; however, operative therapy depends on a number of factors, including etiology, location of the perforation, and the time interval between injury and diagnosis.[8, 11] Other considerations include the extension of the perforation into an adjacent body cavity and the general medical condition of the patient. General recommendations for surgery include the following:

Clinical instability with sepsis
Recent postemetic perforation
Intra-abdominal perforation
Lack of medical contraindications to surgery (eg, severe emphysema, severe coronary artery disease)
Leak outside the mediastinum (ie, extravasation of contrast into adjacent body cavities)
Malignancy, obstruction, or stricture in the region of the perforation
Some authors believe that if treatment is instituted more than 24 hours after the perforation, the mode of treatment does not influence the outcome and can be conservative, tube thoracostomy (drainage), repair, or diversion.

Relevant Anatomy
The esophagus is the muscular tube that serves to pass food from the oropharynx to the stomach. It is the narrowest part of the gastrointestinal tract, and its configuration is flat in the upper and middle portion and rounded in the lower portion. It has no mesentery or serosal coating, which is a unique feature of this portion of the gastrointestinal tract. The connective tissue in which the esophagus and trachea are embedded is surrounded by long continuous sheaths of fibroareolar laminae that cover and bind together muscles, vessels, and bony constituents of the neck and chest. The arterial blood supply to the esophagus includes the superior and inferior thyroid arteries, direct aortic branches, left gastric artery, and splenic artery.

Apart from the lack of a serosal coating, the construction of the esophagus is similar to other organs in the gastrointestinal tract. It consists of four layers: external fibrous layer, intermediate muscular layer, intermediate submucosal layer, and internal mucosal layer.