Famous Patients, Famous Operations, 2002 - Part 1: The Case of the Aristocrat with Indigestion

Albert B. Lowenfels, MD

Disclosures

February 28, 2002

Introduction

The patient is a 51-year-old obese white male in previously good health with the chief complaint of severe chest pain. Three days earlier, the patient had eaten a large meal in the afternoon, which caused symptoms of indigestion and fullness of the stomach. In an attempt to relieve gastric distress, the patient took several doses of an over-the-counter drug to settle his stomach. Because of persistent symptoms, he attempted to relieve the sensation of gastric fullness by self-induced vomiting. But rather than obtaining any relief, he experienced the onset of sudden, very severe chest pain, which caused him to collapse. Severe pain persisted during the next few days, gradually becoming localized in the posterior chest.

His history and physical examination revealed the following additional relevant information:

  • Past medical history: gout of several years' duration

  • Social history: retired naval officer who had traveled widely, moderate-to-heavy alcohol consumption, smoking history unknown

  • Physical examination: obese, middle-aged male sitting upright in bed, obviously in severe pain; temperature, pulse, respirations normal; abdomen soft with no scars, masses, or tenderness

  • Course of illness: Over the next 24 hours his urinary output diminished, his pulse became weak and rapid, and his skin became cold and moist. He died approximately 4 days after the first onset of symptoms. An autopsy was performed.

This famous patient, who died in November 1723, was Jan Gerrit, Baron of Wassenaar, a wealthy Dutch aristocrat. The cause of death, as determined at autopsy, was a ruptured esophagus. Herman Boerhaave (1668-1738), the attending physician, called to see the patient at the onset of this critical illness (Figure 1), performed the autopsy, and carefully noted the findings. The main abnormality was a rupture of the lower esophagus, together with free air in the mediastinum and in the abdominal cavity. The published report of this autopsy was the first description of spontaneous rupture of the esophagus, now named after Boerhaave. At the time, Boerhaave, a highly respected European teacher and physician, remarked that it might be possible to diagnose the condition in the future, but that treatment would be ineffective.

Herman Boerhaave, M.D.

Since Boerhaave's original description of spontaneous rupture of the esophagus, we have refined our diagnostic capabilities considerably. Major symptoms include sudden onset of vomiting followed by severe, sharp chest pain (as in the first case description), and shortness of breath. Careful examination will often reveal subcutaneous crepitus along the chest wall or in the neck. Although the perforation can be on the right side, the chest x-ray will most often reveal a left-sided pleural effusion -- the usual site of rupture. A computerized tomography (CT) scan can be helpful in making the diagnosis (Figure 2). If performed after ingestion of contrast material, the CT scan usually shows extravasation of air and contrast material into the mediastinum.

Computed tomography of the chest through the superior mediastinum with the use of intravenous and oral contrast medium showed air (A) in the mediastinum adjacent to the esophagus (E) and the trachea (T), indicating the occurrence of spontaneous esophageal rupture (Boerhaave's syndrome). From: Gopalan R, Cooke C. Boerhaave's Syndrome. NEJM. 2000;343(3):190.

Despite availability of accurate diagnostic methods, the diagnosis can be difficult, mainly because Boerhaave's syndrome is uncommon. Spontaneous perforation of the esophagus can mimic a heart attack, rupture of the aorta, peptic ulcer disease, and acute pancreatitis.

The pathophysiology of Boerhaave's syndrome closely resembles that of Mallory-Weiss syndrome, which is characterized by upper gastrointestinal bleeding due to a mucosal tear at the esophago-gastric junction. Forceful vomiting may lead to either disease, but in Boerhaave's syndrome, the tear extends through all layers of the esophageal wall.

Early recognition followed by prompt surgery remains the treatment of choice for this life-threatening thoracic emergency. Since the perforation is usually on the left side of the lower esophagus, the lesion can be visualized through a left-sided thoracotomy. After the esophageal tear is identified, it should be closed and then reinforced with a patch of adjacent tissue. External drainage of the chest completes the procedure.

Stents have been used to seal iatrogenic perforations of the esophagus and for the treatment of perforations associated with esophageal cancer. There are now several favorable reports of the use of self-expanding mesh stents to close the esophageal defect in patients with spontaneous perforation of the esophagus. This approach may shorten operative time and be especially useful in high-risk patients.

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