When Linda Corsetti Carnivale returned to Boston from Bermuda, she went back on PTU and, predictably, her symptoms cleared up. It was now clear that she was going to require definitive treatment for her Graves’ disease. There are two time-honored possibilities: radioactive iodine and surgery. Arky discussed the options with his patient and her husband. Older patients generally choose the radioactive iodine; former first lady Barbara Bush selected this option for her Graves’ disease. The treatment is also used in younger patients, but because of the radioactivity, some women in their childbearing years opt for surgery. The surgery —a thyroidectomy—can be tricky. Aside from the possibility of infection, heavy bleeding and damage to the adjacent parathyroid glands, accidental damage to the nerves of the larynx can lead to permanent hoarseness.
Then something happened to Linda Carnivale that forced the issue: she became pregnant. Administering PTU late in a pregnancy is dangerous, because the drug crosses the placenta and can make the baby hypothyroid, and radioactive iodine was now out of the question. So early in her second trimester, thyroid surgery was scheduled.
The evolution of thyroid surgery is one of the more colorful chapters in the history of medicine, and some of its pioneers are giants of modern surgery—Theodor Billroth, Theodor Kocher, Charles Mayo, and William Halsted. Even aside from the generic problems of adequate anesthesia and postoperative infection that confronted surgeons during the second half of the nineteenth century and the first half of the twentieth, there were other issues that were specific to thyroid surgery.
In 952 AD, a Moorish physician, Khalaf Egn Abbas, reportedly performed a successful operation to remove a goiter. He apparently used opium for sedation and hot cautery irons to stanch the bleeding. In 1791, the French surgeon Pierre-Joseph Desault operated on a twenty-eightyear-old woman with a goiter. She recovered. Despite these scattered successes, however, most surgeons avoided the procedure altogether. In 1846, Robert Liston, one of England’s most audacious and skilled surgeons, said, “You could not cut the thyroid gland out of a living body in its sound condition without risking the death of the patient from hemorrhage; [thyroid surgery is] a proceeding by no means to be thought of.”
The thyroid is a very vascular gland, with major arteries running in every direction, and bleeding during the operation was a major problem. Twenty years later, the Philadelphia surgeon Samuel Gross summed up this concern when he wrote: “Can the thyroid in the state of enlargement be removed? Should the surgeon be so foolhardy to undertake it . . . every stroke of the knife will be followed by a torrent of blood and lucky it would be for him if his victim lived long enough for him to finish his horrid butchery. No honest and sensible surgeon would ever engage it.”
In his treatise on the subject of thyroid surgery, William Halsted, chief surgeon at the newly formed Johns Hopkins Hospital, wrote that mortality from thyroidectomy before 1850 was 40 percent. But during the second half of the nineteenth century, doctors gradually overcame the problems of anesthesia, sterile technique, and infection. European surgeons began using small metal clamps to stop intraoperative bleeding. The ability to operate in a bloodless field on an anesthetized patient, who had a good chance of not dying from a postoperative infection, set the stage for more intricate operations.
In 1860, at the age of thirty-one, Theodor Billroth became the chair of surgery at Vienna. Previously he had worked in Zurich. One of the areas in which goiter is endemic due to low dietary iodine is the region that encompasses southeastern France, northern Italy, and parts of Switzerland. This provided surgeons in this area with a steady supply of patients with large goiters (but who were not thyrotoxic). It was here that Billroth began his work in the field. Of his first thirty-six procedures (done without sterile technique), sixteen died. This dismal 36 percent mortality rate led him to abandon the procedure until the late 1870s. Then, using the sterile techniques that had since become standard, between 1877 and 1881 he did forty-eight thyroidectomies with a mortality rate of just over 8 percent.
It was Billroth’s pupil, Theodor Kocher, who was a professor of surgery at Bern, who took thyroid surgery to the next level. Because Bern is part of the area of endemic goiter, Kocher rapidly gained experience in the field. During his first ten years in his post at Bern, he removed 101 goiters with only 13 deaths. Kocher became so renowned that he received the Nobel Prize in Medicine for “his work in physiology, pathology, and surgery on the thyroid gland” in 1909, the first surgeon ever to be so honored.
In 1917, weeks before he died, he presented his lifelong experience with benign goiter; in approximately five thousand cases, his mortality rate was less than 1 percent. Part of the large reduction in mortality rates had to do with general improvements in surgical procedures, including anesthesia, clamps to prevent bleeding, and sterile technique, and part was a result of the accumulated experience of surgeons.
Just as Graves had done, Halsted made his medical pilgrimage to Europe in 1878. He spent two years at the great European surgical centers, observing and learning from both Kocher and Billroth. He brought the techniques he acquired back to America and became an authority on thyroid surgery.
Mortality, however, is just one measure of the success (or failure) of a surgical procedure. Other complications must also be considered. In the case of thyroid surgery, there are several important ones. The first complication was causing the patient to become hypothyroid—too little thyroid. Kocher was the first to recognize this problem. On January 8, 1874, he performed a total thyroidectomy on an eleven-year-old girl named Marie Richsel. Kocher later reported that the referring physician contacted him to report that “the girl had become quite cretinoid. This seemed so important to me that I made every effort to examine the girl, which was not easy since this physician had died very shortly after making his report. We were all the more intent upon it since our colleague, Reverdin of Geneva, had informed us that he had observed two patients who had suffered diminution of mental capacity following goiter operations. I was highly astonished at the striking appearance of my patient. . . . She had an ugly, almost idiotic appearance. As soon as this was determined, I immediately requested all of my goiter patients to return for examination.”
Of thirty-four such patients, Kocher could locate only eighteen; of those, sixteen had developed hypothyroidism. So Kocher changed his standard operation to leave a little bit of thyroid tissue behind to prevent this complication. This was before the days when thyroid extract could simply be given to treat this possible complication of thyroidectomy.
There were other pitfalls too, however. Some patients would develop tetany after thyroid surgery. Tetany, after the Greek tetanus, which means painful contracture, developed shortly after thyroid surgery in a variable percentage of patients. Sometimes it was transient; other cases were permanent. Patients with tetany had painful muscle spasms, especially in the hands and feet, very twitchy reflexes, and odd sensations in the body. In severe cases spasm of the larynx could result. Over time, the surgeons learned that this was due to the inadvertent removal of the tiny parathyroid glands, four glands the size of watermelon seeds that are responsible for calcium metabolism and are located on the rear surface of the thyroid. The frequency of this complication was a function of how meticulous the surgeon’s technique was.
Last, nerves to the larynx lie in close proximity to the thyroid gland. If a surgeon inadvertently cut the recurrent laryngeal nerve during the operation, permanent hoarseness would result. Again, once this anatomic detail was clarified, surgeons developed techniques to avoid it. By 1900, Europeans were light years ahead of the Americans in thyroid surgery. Halsted could find reports of only 45 operations in America up to the year 1883; by that same date, Billroth had performed 125 by himself. The metal clamps that the Europeans were using were nearly nonexistent in the United States and late to be adopted. Halsted wrote: “Few hospitals in New York, at least, possessed as many as six artery clamps in 1880. I recall vividly an operation performed by Mikulicz in 1879 in Billroth’s cli
nic. Americans, newly arrived in Austria, we were greatly amused at seeing perhaps a dozen clamps left hanging in a wound of the neck while the operator proceeded with his dissection, and were inclined to ridicule the method as being untidy or uncouth. Slowly it dawned upon us that we in America were the novices in the art as well as the science of surgery. The value of artery clamps is not likely to be over estimated. They determine methods and effect results impossible without them. They tranquilize the operator. In a wound that is perfectly dry, and in tissues never permitted to become even stained by blood, the operator, unperturbed, may work for hours without fatigue.”
The Americans caught up rapidly, though. Halsted, who performed only six cases in the first ten years that he was at Hopkins, did ninety in the next ten years (with a mortality rate of 2 percent), and by 1914, he reported on over five hundred cases of thyroidectomy in Graves’ disease alone. Others, such as Charles Mayo, George Crile, and Frank Lahey, followed. By the time Lahey died in 1953, he had personally performed nearly ten thousand thyroidectomies.
Most surgeons in the United States had only a tiny fraction of that experience, however. Today, the recommendation for patients needing thyroid surgery is to have it done by a surgeon who performs at least fifty procedures per year, which is ten times more than what most American surgeons in general practice do. Linda was a sophisticated patient, and her fears were not totally unjustified. And on top of everything else, she was pregnant. Ironically, her mother also had needed thyroid surgery for Graves’ disease during one of her pregnancies. Dr. Arky consulted with an experienced neck surgeon, who performed the procedure on October 10, 1988.
“Linda was hysterically afraid,” recalls her husband, “and I was a wreck. The morning of surgery, I got to the hospital at four-thirty in the morning so I could be with her before they took her to the operating room at six. We knew that there was risk to the baby and to Linda.” The surgery was successful, and about six months later, the Carnivales had a healthy baby boy.
Since her surgery, Linda Carnivale has felt fine. Her husband remarks on the patience she has with their son and thinks how different it all would have been if the hyperthyroidism hadn’t been diagnosed and treated. “We went back to Grotto Bay last summer,” he says, “and it was just as hot as during out honeymoon. Linda couldn’t believe how cold the water was. Of course, it was the same temperature as before, but she couldn’t stand it this time.”
12 Feeling His Oats
By midmorning on Tuesday, December 13, 1988, Henry Schachte of Weston, Connecticut, knew something was dreadfully wrong. That was when the abdominal pain began. “It felt like my stomach was being inflated with air,” recalls the seventy-six-year-old retired advertising executive. As the day wore on, the pain intensified, and by 9 PM, Schachte knew he needed help. He threw on some pajamas and drove himself to Norwalk Hospital.
There, in the emergency department, Schachte was examined by Dr. Edward Tracey, the surgeon who had operated on him for diverticulitis eight years earlier, removing part of his colon. He had mild high blood pressure and was on a medication for that. He had had knee surgery in 1980, and some minor prostate issues. A widower for three years, Schachte lived alone and cooked for himself. He ate prudently and had recently increased his fiber consumption to lower his cholesterol. Overall, Schachte was in pretty good health for a man his age.
In the emergency department, Dr. Tracey did not like what he saw. Schachte was in moderate distress from the pain. His blood pressure was elevated. Most important, his abdomen was distended and diffusely tender. When the surgeon pressed on it, there was an involuntary tensing of the abdominal wall muscles, which is often a harbinger of serious mischief in the belly.
In an era before the routine availability of CT scans, Dr. Tracey ordered a series of plain x-rays of the abdomen. These films revealed dilated loops of small bowel, confirming the surgeon’s initial diagnostic impression: Schachte had an obstruction in his small intestine.
The human intestine is a long, hollow tube that begins at the stomach and ends at the anus. It consists of two basic parts: the small intestine and the large intestine. In the stomach, various acids, enzymes, and, to some extent, mechanical motion begin the first steps of digestion of the food that is swallowed. Once the material finds its way into the first part of the intestines (called the duodenum), the principal task is to further break down the food into its basic elements and complete the process of digestion, absorbing what is useful and eliminating what is not. The three portions of the small intestine are the duodenum, which empties into the jejunum, which in turn becomes the ileum. The ileum empties into the large intestine, or colon, at the point where the appendix lies. The small intestine gets its name not from its length (it is much longer than the large intestine) but from its smaller diameter.
Although most nutrients (protein, fats, and carbohydrates) are absorbed in the small intestine, its contents remain uniformly liquid from start to finish. It is in the large intestine that water is absorbed and the waste product, stool, assumes a solid consistency.
Because it is narrow, the small intestine can become obstructed relatively easily, making such blockages a common cause for admission to a surgical ward. In patients with small bowel obstructions, the portion of the intestine proximal to the obstruction swells. As the swelling increases, the blood supply to that part of the gut is compromised, and if the obstruction is not relieved, that part of the bowel will die. This leads to perforation and infection of the abdominal cavity, or peritoneal cavity, as doctors call it. This inflammation or infection of the peritoneum is called peritonitis. The inflammation leads to fluid being drawn out of the blood vessels and accumulating in the peritoneal cavity; this in turn can cause the blood pressure to fall. Unchecked and untreated, a bowel obstruction usually results in death.
Diagnosing the presence of a bowel obstruction is the first step. The next step, finding out its cause, is also important. Although there are dozens of potential causes, most result from one of two problems—hernias and adhesions resulting from prior surgery. Sometimes, a hernia of a portion of the bowel causes a kink that blocks the flow of intestinal material. An adhesion is an area of scarring in the peritoneal cavity that also causes a loop of bowel to twist or kink, resulting in a bowel obstruction. Fifty years ago, hernias were the most common cause of obstructions, but over the past several decades, adhesions have caught up and surpassed them in frequency. Most abdominal adhesions, by far, are the result of prior abdominal surgery. Sometimes the bowel obstruction from an adhesion will fix itself; however, surgery is often necessary.
After the patient is put to sleep by an anesthesiologist, the surgeon makes an incision into the abdomen, and then “runs” the bowel, meaning that he will inspect the bowel from the duodenum to the anus, to find any pathology. Often there is a clear-cut transition point at the site of the obstruction. The surgeon snips or otherwise releases the adhesion or reduces an internal hernia, the bowel floats back to a normal position, and the surgeon closes the incision. If all goes well, it is a fairly quick and routine operation.
“I remember distinctly telling Mr. Schachte that we’d be in and out of the operating room in about an hour,” says Tracey. “I should have known better. When we opened him up, it was a real mess. There were massive adhesions from the old surgery.”
His dictated postoperative report spells out the details:
On entering the peritoneum, [we found] totally fixed adhesions of the bowel to the omentum [ fatty tissue that lies within the abdomen] and to the overlying peritoneum, from the diaphragm right down to the pelvis. The most proximal end and the distal ileum were of normal caliber. By tedious blunt and sharp dissection, the small bowel loops were carefully separated from one another in two areas where the bowel was fixed.
After about three hours, the bowel was finally cleared and there was no one area that could be seen as the site of his obstruction. The middle aspect of the small bowel was packed with a very thick material that may have been t
he causative factor. This was some type of undigested vegetable material.
Dr. Tracey recalls, “We made an incision in the suspicious area of bowel. That’s when this paste, the consistency of toothpaste, came out; it was totally obstructing the segment of small intestine. We filled a small bucket with the stuff.” Tracey removed a two-foot-long concretion of the thick impacted goo, the consistency of soft concrete that hasn’t set. He sent the material to the pathologist, but he was already pretty sure he knew what the problem was—a bezoar.
Bezoars are solid clumps of ingested material found in the alimentary canal of animals, mostly ruminants. Ruminants include cows, goats, sheep, deer, and antelopes—animals that have special four-chambered stomachs that are designed to digest their food in two steps. First they eat their food. The food sits for a while in the first two chambers; eventually, the animal regurgitates the semi-digested food, known as cud, and then begins to chew the cud. This process is called ruminating (which accounts for the use of the word “ruminate” to mean taking a long time to think about something). Occasionally, partially digested foodstuff in the stomachs of these animals begins to form. They become firm concretions, which sometimes form a solid mass as hard as a rock.
Although it may seem somewhat fantastic today, bezoars have been prized by humans for at least three millennia. The original bezoars are thought to have come from goats in the mountains of western Persia. The word “bezoar” probably derives from a combination of two old Persian words, pâd, meaning protector, and zahr, meaning poison. The combination, pâdzahr, is the likely the root for the Arabic badzehr and the Turkish panzehir. All of these words mean the same thing—protector from poison, or, in modern parlance, an antidote.
Consistent with this meaning, bezoars were thought to possess curative powers in times past. Bezoars taken from goats, sheep, gazelles, and probably other animals were collected and preserved to be used as medical “charms” since at least 1000 BC. Galen, a Greek physician who practiced in Rome in the second century AD and wrote extensively, had an enormous influence on medical practice well into the Renaissance. According to an article in the British Journal of Homeopathy written in 1841, Galen prescribed a bezoar stone in cases of jaundice and apparently was most fond of bezoars made from the stags of “eastern countries.”
The Deadly Dinner Party: and Other Medical Detective Stories Page 19