Boerhaave's Syndrome – Spontaneous Rupture Of The Esophagus

Case Presentation:

An 80 year old female with a past medical history of hypertension, renal cell carcinoma, and status-post nephrectomy, experienced an episode of choking, coughing and vomiting when she had difficulty swallowing her Centrum Silver tablet. Soon after vomiting, the patient developed sever chest pain with radiation to her back. The pain was significantly worse with deep inspiration.

Introduction:

Boerhaave's Syndrome is a spontaneous rupture of the esophagus, classically described as post -emic, 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 training 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:

Classicly, the patient is a middle-aged, white male with a history of overindulgence in food or drink. The patient often 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 will 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 inductive an inflammatory reaction leading to granuloma formation.

Management:

The initial emergency department management of Boerhaave's Syndrome includes strict NPO, broad spectrum antibiotic, fluid resuscitation, and continuous nasal gastric suction. 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.

Continued 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 adjunct to a short esophageal restriction in the mid to distal 1/3 of the esophagus.

The gastro-intestinal and cardio-thoracic surgery services were informed and the patient was transferred to the ICU

References:

1. UpToDate online 13.2, Boerhaave's Syndrome: Effort rupture of the esophagus. 2005.

2. Hospital Physician, A 55 year old man with chest pain, November 2005.

3. Emergency Physician Monthly, Boerhaave's Syndrome, January 2006.

4. Khan AZ, Strauss D, Mason RC. Boerhaave's syndrome: diagnosis and surgical management. Surgeon. 2007 Feb; 5 (1): 39-44.

5. Vial CM, Whyte RI. Boerhaave's syndrome: diagnosis and treatment. Surg Clin North Am. 2005 Jun; 85 (3): 515-24.

6. Janjua KJ. Boerhaave's syndrome. Postgrad Med J. 1997 May; 73 (859): 265-70.