History’s Medical Mysteries

 
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Contents

History’s Medical Mysteries
> Dead Men Talking
> View Masters
> Bewitched, Bothered, & Bewildered
> The Curious Case of the
    Incurable Epicure

> Mystery Theater
> Murder Most Harvard
> Name That Tool [pdf only]

Other Harvard Resources
> The Center for the History
    of Medicine

> The Warren Anatomical Museum
> Contagion: Historical Views of Diseases
    and Epidemics

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The Curious Case of the Incurable Epicure
The sudden, baffling death of the grand admiral of the Dutch fleet handed a premier physician a mystery—and medical immortality.
by Anthony S. Patton

A
utumn wind and rain lashed Hermann Boerhaave’s carriage as it hurtled down dark village lanes and past sodden fields on its rush to theHermann Boerhaave
nearby castle. There the doctor found the grand admiral of the Dutch fleet, the Baron Jan Gerrit van Wassenaer, slumped at the edge of his bed, pressing his belly into his lap and groaning. A trio of servants hovered to help prop the admiral in a sitting position.

When Boerhaave approached his patient just past midnight on October 30, 1723, the admiral struggled to raise himself and offer his hand, but his pain grew too sharp. Speaking proved agonizing as well, so his son’s tutor hastened to provide the details.

During the previous week, van Wassenaer had suffered a particularly vexing bout of his usual stomach trouble. After three days of near fasting, though, he had felt well enough to treat himself to a sumptuous lunch. He tucked into veal soup and cabbage boiled with mutton, then devoured calf sweetbreads, spinach, a generous helping of duck, two larks, apple compote, and bread, all washed down with beer. Moselle wine had accompanied his dessert of pears, grapes, and sweetmeats. After a brief rest, he joined his son for a horseback romp across his estate.

By the time the admiral returned to the castle, he was feeling ill. To induce vomiting—his customary cure for an upset stomach—he gulped several glasses of tepid water blended with musk thistle extract and perhaps ipecac root extract as well. When the concoction failed to produce results, the admiral swilled four more glasses.

This remedy proved unwise. The admiral began vomiting violently. Suddenly he emitted a piercing howl. When alarmed servants rushed to his chamber, they found him doubled over. Something at the top of his stomach felt torn, he gasped out, and death would soon follow. He asked his servants to lower him to his knees so he could surrender himself to God. While he prayed, his body began to shake violently. Cold sweat formed, and his pulse turned thready. His servants lifted him to his hunched position again. Over the next few hours they warmed him with hearth-heated blankets, moistened his head and chest with the juice of crushed herbs, made him choke down several ounces of olive oil, and offered him medicated Danzig beer.

During these ministrations, the admiral’s family summoned James de Bye, a highly respected physician from the Hague. The doctor gave his patient soft ptisans of oats to swallow; he also prescribed a milk-and-corn preparation to be rubbed gently over the chest and abdomen. No tonic provided relief, though, and the grand admiral, surrounded by family members and servants, remained doubled over in agony.

Close to midnight, the admiral’s brother, James, also a powerful baron, galloped through the storm to the nearby city of Leiden. By the time he reached Hermann Boerhaave’s imposing residence, he was in tears. His brother’s last hope lay with the world-famous physician’s ability to diagnose and treat the admiral’s mysterious ailment.


Cultivating Genius

In 1575, after Leiden gained fame for its brave stance against a Spanish siege, William I, Prince of Orange, rewarded its citizens for their courage by granting a charter for a university. Leiden University soon grew into one of the finest academic institutions in the world, with a celebrated medical school. Benjamin Waterhouse, one of the three original faculty members of Harvard Medical School, trained there in the early 1780s.

Heading Leiden’s medical school at the time of the admiral’s woes was Boerhaave, whose stature as a clinician and scientist had reached unparalleled heights. Letters from Asia addressed loosely to “The Best Doctor in Europe” arrived on his doorstep. He wrote brilliant books on chemistry and medicine and, following the examples of Hippocrates and Thomas Sydenham, revived the lost art of clinical teaching at the bedside.

Boerhaave trained scores of physicians from all over the globe, including the founders of Edinburgh’s influential medical school. He was one of the first to use the microscope and thermometer as tools of medicine, and his description of glandular function remains valid to this day. At one time he was a fellow of the Royal Society of London, a fellow of the French Academy of Sciences, and chairman of the Surgical Guild in Leiden—quite a feat for a non-surgeon.

Although he had originally intended to become a Calvinist minister like his father, Boerhaave had become transfixed by the works of Spinoza, Descartes, and other philosophers whose ideas clashed with the tenets of the Dutch Reformed Church. So he abandoned his ecclesiastic ambitions and earned instead a medical degree with distinction. He wrote his thesis on the value of fecal examination in the diagnosis of disease.

Boerhaave opened a small medical practice in Leiden, taught private courses out of his home, and began lecturing at the university. His classes proved so popular—noblemen often hired men to arrive at the classroom early and reserve seats for them—that university officials offered him the first opening on the permanent faculty: a professorship in botany. Boerhaave had little experience with the subject but soon mastered it with the aid of the university’s extensive gardens, where exotic plants from all over the world flourished. He wrote a seminal text on botany and later trained the noted Swedish scientist Carl Linnaeus, whose plant classification system remains in use today.

The fame of Boerhaave’s extensive talents allowed the admiral’s desperate family to cling to a shard of hope as the doctor’s carriage hurtled to the castle that dismal night.


Eat, Drink, and Be Buried

Boerhaave found his patient in dire straits, though some clinical signs were surprisingly normal: The admiral had no fever. His face had retained its natural color and his eyes their luster. No noxious odor emanated from his breath. When he managed to speak, his voice sounded clear. Yet his pulse remained weak. Despite the many liquids poured into his body, he produced not a single drop of urine. And his pain felt lacerating. Boerhaave had attended to the admiral’s gout over the years and knew him to be a good-humored man who bore discomfort stoically. Even as the admiral tried to stifle his groans, it became clear this was no ordinary affliction.

One of Boerhaave’s immediate suspicions centered on poisoning. Few people in the Netherlands enjoyed greater stature than the grand admiral. Beginning in the 1650s, the country had maintained the world’s largest merchant fleet. The navy not only provided protection for the commercial ships, but had also been embroiled in innumerable wars with Spain and England. Overseas treaties and alliances, sometimes unsavory, required a corps of expert sailors, and many members of the baron’s influential family had served in successful expeditions and battles. Although foreign intrigue and assassination plots were common, motives aside, no poison Boerhaave knew would provoke the admiral’s constellation of symptoms.

In those long hours of attending to van Wassenaer, Boerhaave reviewed all he knew about his 51-year-old patient. In his youth, the baron had led an active life, filled with sport and horseback riding. As he aged, though, the many diplomatic conferences and lavish parties caught up with him, and he seemed unable, Boerhaave noted, to “observe the exacting niceties of moderation.” Indeed, the admiral’s voracious appetite had brought him infamy, as he consumed enormous portions of food while drinking prodigious amounts of wine and beer at each meal. The baron himself suspected that his excesses likely caused his chronic gout and the intermittent inflammation of what Boerhaave described as “the mouth of his stomach.”

But this latest episode of illness was clearly far graver than any the baron had previously experienced. Boerhaave tried a range of remedies. He bled the admiral, gave him a goblet of wine to drink, applied a lukewarm preparation of flour and milk to the painful areas, and offered him a concoction made from wild poppies. Yet nothing alleviated the patient’s suffering. “All hope seemed to be diminishing,” Boerhaave later wrote. “We were horrified and stupefied at the occult nature of this unique malady.”

In the late afternoon, Boerhaave noted that the skin covering the admiral’s chest and upper abdomen had become swollen with an odd, spongy fluid. Suddenly the patient’s already excruciating pain increased. His skin grew cold and sweaty, and slowly, like a wounded ship in his fleet, the admiral rolled onto his side and sank into unconsciousness, never to awaken again.


Internal Review

Like any great scientist, Boerhaave tried to learn from his failure. In eloquent Latin, he recorded in detail the history of the patient’s illness, including the copious amounts of rich food, beer, and wine the admiral had consumed; his howl of pain; the doctors’ ineffectual remedies. Boerhaave catalogued each symptom as well: the baron’s swollen epigastrium, the strange crepitus in his skin, his falling urine volume, his weakening pulse, and then his final moments of torment, unconsciousness, and death.

Mystified, Boerhaave agonized over both his inability to render a diagnosis and his unsuccessful treatment. He worried that he and de Bye had possibly exacerbated the baron’s misery. Boerhaave himself, following an attack by a swarm of bees, had suffered a painful ulcer in his thigh for five years while a teenager; his biographer, Samuel Johnson, wrote that the ulcer “defeated all the art of the surgeons and physicians, and not only afflicted him with most excruciating pains, but exposed him to such sharp and tormenting applications, that the disease and remedies were equally insufferable.” Boerhaave ultimately rejected the received wisdom of his doctors and cared for himself by applying salt and urine to the stubborn wound. Later, when he entered medicine, he resolved never to exacerbate his patients’ suffering.

When the admiral died, the two physicians at his bedside agreed that a postmortem examination was essential. “What Doctor is there,” Boerhaave later wrote, “who while he treats a disease unknown to him, might be at ease until he had clearly perceived the nature of this disease and its hidden causes?” Aided by candlelight, he undertook the autopsy himself, with several men in attendance, including de Bye, the tutor, and the local minister.

Boerhaave’s knowledge of anatomy was extensive; he had long ago mastered Vesalius’s work. As a good pathologist, he first described the appearance of the corpse, noting the strange accumulation of fluid in the flanks and the spongy crepitation from air under the skin. When Boerhaave opened the abdomen he felt—and heard—a great rush of air but could find no perforation of the bowel, only generalized distension with a normal liver and spleen. As soon as he nicked the stomach he again heard a loud hissing sound as air escaped; he was astonished to find the stomach empty but for a peculiar dark fluid.

When he next opened the thoracic cavity he immediately felt more air and detected the strong odor of duck. The viscous nature of the dark fluid that bathed the lungs, heart, and mediastinum suggested the olive oil that de Bye had administered to soothe the patient’s pain. With deeper dissection, Boerhaave found a ragged communication between the pleural cavities. Then, at the bottom of the chest, he found his answer: a hole in the baron’s esophagus, just above his diaphragm. From above and below, Boerhaave poked his forefinger through this hole into the esophageal lumen, then invited his audience to do the same. He could detect no sign of chronic ulceration or inflammation, but a fresh injury resulting in massive contamination of the pleural cavity by food, saliva, and stomach contents.

Boerhaave had thus uncovered the first recorded case of an esophageal rupture due to forceful vomiting. He speculated on the stresses caused by retching and the quick regurgitation of a large amount of stomach contents into an esophagus. He realized that the esophagus, lacking the serosa, or tough outer layer, that protects the bowel, is prone to blowout. His mechanistic view concluded that compression of the lungs had led to the admiral’s death; we know now, however, that the chemical and bacterial injuries can play as important a role in such cases.

Boerhaave’s published booklet about the admiral’s demise, “Atrocis, nec Descripti Prius, Morbi Historia,” or “History of a Grievous Disease Not Previously Described,” became a classic, perhaps the first complete clinical-pathological correlation of medical history with physical examination, diagnosis, disease progression, and autopsy findings. Its narrative reveals much about the times, including the role of the doctor and the state of medical knowledge. It also marks the significance of the case for all physicians, for without early treatment, patients with esophageal ruptures can suffer long, agonizing hospitalizations and, until recently, most died.


Savings Account

Boerhaave’s prediction that no one would ever survive the syndrome named after him proved correct for more than two centuries. But in 1947 Norman Barrett, a famous surgeon at St. Thomas’s Hospital in London, was able to save a patient with an esophageal rupture. Since then, thoracic surgeons have remained alert to symptoms of Boerhaave’s syndrome. To diagnose the condition quickly and initiate immediate, lifesaving repair can establish a surgeon as a hero; to miss the diagnosis and lose a patient can create a legacy of shame.

When I met Barrett in the early 1960s, the distinguished surgeon told me he considered that groundbreaking case to be one of his proudest moments. At the time I was working at a regional thoracic surgical unit in Devonshire, England, where I, too, faced my earliest challenges with the syndrome. Two years in a row the owner of The Rock, a favorite local pub for vacationing Londoners, rang me up on Boxing Day, the day after Christmas.

“Is this the registrar?” he bellowed into the ancient phone each time. “Well, we have another bloke who has overdone and ruptured his swallowing tube just like that Dutch admiral. I’m sending him over.” The pubkeep had become an unofficial expert on Boerhaave’s syndrome. Fortunately, our surgical team was able to repair both cases. We found the tear exactly as Boerhaave had described—just above the esophagogastric junction—and were able to suture it within several hours of the injury.

Upon leaving England, I started a surgical practice north of Boston, where I saw many cases of ruptured esophagi. I remember one middle-aged man, a master electrician, who was transferred to our unit from another hospital in severe pain. His diagnosis had baffled his doctors, but my close reading of Boerhaave’s account and my experience in England led me to conduct emergency surgery. After the operation I went out to talk to the patient’s wife. I’d had little time for explanation beforehand, so I took my time in describing her husband’s initial plight and expressing my optimism for a successful outcome.

“Good God,” she exclaimed. “Ruptured his esophagus!” She paused, grabbed my hands, and added ruefully, “That man, he’s always doing to excess.”  Interestingly, he too had tried his own remedy for gastric distress—a big gulp of baking soda.

Later in my career, I called numerous patients back for study and ordered barium swallows to see how their repaired esophagi now appeared. I was amazed at how many of them still had tiny slivers of leaks or thin diverticuli that nevertheless had healed well enough to cause no trouble. Perhaps Boerhaave was right in his original report: no case will ever be completely cured.

Not all cases are as dramatic as that of the admiral, so physicians must remain alert for unusual presentations of Boerhaave’s syndrome. Although almost all cases involve vomiting and air either under the skin or in the pleural space, many have more subtle presentations. The vomiting is sometimes triggered by bowel obstruction, severe esophagitis, or even seasickness. Today surgeons can save the patients whose diagnoses are delayed, albeit with great effort. The corrosive effects caused by saliva, gastric juices, and bacteria can create havoc with normal tissues, so the passing hours and even minutes are critical. Yet with intensive care and techniques that exclude the esophagus from the normal digestive mechanisms, patients who have somehow survived weeks of perforation can be saved. The best results, though, are repairs made within a few hours of injury.

Whenever we save someone with a ruptured esophagus, we must give a nod not only to Boerhaave, but also to his brave if indulgent aristocrat, the grand admiral of the Dutch fleet whose “atrocious illness” stimulated one of history’s most important clinical descriptions and started the tradition of clinicopathological conferences. Boerhaave may have failed to save his patient, but the baron did not die in vain. The vividness with which his famous physician recorded his life—and agonizing death—helped imprint the mysterious ailment on the minds of centuries of healers—and at least one sharp-witted pubkeep.

Anthony S. Patton ’58 is a retired thoracic and vascular surgeon whose career was centered at Salem Hospital in Massachusetts.

This article appeared in the Spring 2005 issue of the Harvard Medical Alumni Bulletin.

Image: Courtesy of the Boston Medical Library in the Francis A. Countway Library of Medicine


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