Boerhaave’s Syndrome
BOERHAAVE’S SYNDROME
Introduction:
Boerhaave’s Syndrome is a spontaneous rupture of the esophagus, classically described as postemetic, but may also occur after lifting a heavy weight, with severe asthma, or prolonged coughing. The sudden rise in esophageal intraluminar pressure causes an esophageal tear which most commonly occurs in the lower esophagus above the left diaphragm.
Boerhaave’s Syndrome was first described in 1724 by Hermann Boerhaave, a Dutch physician. His patient was a 50 year-old admiral (Baron John van Waasenaer) who developed a sudden excruciating chest pain while straining to vomit. The patient developed shock and died 18 hours later. An autopsy demonstrated rupture of the distal esophagus into the left chest.
Clinical Presentation:
Classically, the patient is a middle-aged, white male with a history of overindulgence in food or drink. The patient develops vomiting, lower chest pain, and mediastinal or subcutaneous emphysema (Meckler’s triad – vomiting, pain and subcutaneous emphysema).
The physical exam typically reveals a critically ill patient, usually sitting up in bed with a forward-crouching position. Subcutaneous emphysema is frequently seen. Hamman’s sign is seen in up to 20 percent of Boerhaave’s patients. Hamman’s sign is a crunching, rasping sound, synchronous with the heartbeat, heard over the precordium, and is often indicative of spontaneous mediastinal emphysema. Patients also present with varying degrees of epigastric tenderness, sometimes imitating an intra-abdominal catastrophe. When the rupture is confined to the mediastinum, the patient may not look particularly sick and vital signs may be deceptively normal.
Differential Diagnosis:
The differential diagnosis of Boerhaave’s Syndrome is extensive and should include the following:
Perforated or Bleeding Ulcer
Acute Pancreatitis
Myocardial Infarction
Pulmonary Embolus
Dissecting Aneurysm
Spontaneous Pnuemothorax
Mallory-Weiss Tear
Acute Cholecystitis
Diagnostic Evaluation:
In addition to the patient history, radiography remains the cornerstone of the diagnostic evaluation for Boerhaave’s Syndrome. Plain chest radiographs may show mediastinal or free peritoneal air (most common finding on initial films), a widened mediastinum, hyrdrothorax, hydropneumothorax, or mediastinal emphysema. Ten to fifteen percent of all patients presenting with Boerhaave’s Syndrome may have a normal plain chest radiograph.
A swallow contrast radiographic study remains the diagnostic gold-standard. Either a thoracic CT scan or an esophagram is required to locate the exact site of perforation, and helps to determine the best surgical approach. A water-soluble contrast agent such as gastrografin is utilized. Most recommend avoidance of barium since its penetration into the thorcacic cavity can induce an inflammatory reaction leading to granuloma formation.
Management:
The initial emergency department management of Boerhaave’s Syndrome includes strict NPO (nothing per mouth), broad spectrum antibiotics, fluid resuscitation, and continuous nasal gastric suctioning. A cardiothoracic surgeon should be consulted urgently, and if cardiovascular services are not available at your facility, the patient should be transferred to an appropriate facility.
Patient’s are often placed on total parenteral nutrition, and early surgical repair remains the standard of care. Complications of Boerhaave’s Syndrome include persistent esophageal leak, mediastinitis, polymicrobial sepsis, pneumonia and empyema.
Despite optimal management, the mortality of patient’s with Boerhaave’s Syndrome remains high. Mortality rates have been quoted as high as 72 percent and are most likely attributable to difficulty in making the diagnosis. In contrast to spontaneous rupture of the esophagus, iatrogenic esophageal rupture carries a mortality rate of only 20 percent, and traumatic perforation has a mortality of only 7 percent.
Case Conclusion:
The patient was treated in the emergency department with Aspirin, Morphine, Reglan, one liter of normal saline, Zosyn 3.375 grams intravenous, and a nasogastric tube was placed. A thoracic CT scan with oral administration of 20 ml Redicat demonstrated bilateral pleural effusions, with a tract of contrast and air noted within the anterior wall of the esophagus. A semi erect single contrast esophagram with thin barrium solution demonstrated an esophageal tear adjacent to a short esophageal stricture in the mid to distal 1/3 of the esophagus. The gastro-intestinal and cardio-thoracic surgery services were consulted and the patient was transferred to the ICU .
References:
UpToDate online 13.2, Boerhaave’s Syndrome: Effort rupture of the esophagus. 2005.
Hospital Physician, A 55 year-old man with chest pain, November 2005.
Emergency Physician Monthly, Boerhaave’s Syndrome, January 2006.
Khan AZ, Strauss D, Mason RC. Boerhaave’s syndrome: diagnosis and surgical management. Surgeon. 2007 Feb;5(1):39-44.
Vial CM, Whyte RI. Boerhaave’s syndrome: diagnosis and treatment. Surg Clin North Am. 2005 Jun;85(3):515-24.
Janjua KJ. Boerhaave’s syndrome. Postgrad Med J. 1997 May;73(859):265-70.