Under the Knife

by Arnold van de Laar, Laproscopic surgeon

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  If Hercule Poirot is the master of induction, that other great detective in world literature, Sherlock Holmes, is the master of deduction. Holmes solves his cases in a completely different way, just as a surgeon reaches a working diagnosis differently from an internist. Sherlock Holmes is tall and slim, and stern in appearance. He hardly eats, but smokes all the more for it. He solves mysteries in foggy London, wraithlike and shrouded in secrecy. The basis of his success is the enormous repository of random knowledge in his head. He has studied the meanings of sailors’ tattoos, knows the colour and composition of the soil in every part of England by heart, and knows what font is used by each newspaper. These are the general facts on which his deduction is based. The strength of Sherlock Holmes’s method is observation. ‘The world is full of obvious things which nobody by any chance ever observes,’ he says, courtesy of his spiritual father and creator Arthur Conan Doyle – who was also a doctor. He uses deduction to compare what he observes with what he knows. He leaps from observation to observation, moving forward all the time. And, because he does it so well, he rarely has to look at other possibilities or change tack. His method is therefore much more efficient than that of Poirot, more direct, but also more vulnerable as success depends on how well he observes and how much he knows. That is why he works alone. He does have a companion, his friend Dr Watson, but Holmes treats him more as a kind of pupil, from whom he expects little assistance. Watson seems to have been conjured up by Conan Doyle purely to allow the thoughts in the detective’s lonely mind to be translated into a dialogue, so that the reader can also benefit from them.

  It is immediately clear that deduction relies entirely on what is in the mind of the detective or the surgeon. By comparison, induction is much more complex, but is also more transparent and objective. Sherlock Holmes could afford not to go into detail about many of his deductions and only explain the whole thing at the end, because his adventures almost always ended in success. Medical specialists, including surgeons, can no longer permit themselves such a luxury. The times when Sherlock Holmes, superior and unfathomable, could outsmart a criminal in the London fog are now over. A modern surgeon no longer presents himself as an individually focused expert who determines the quality of the investigation into a patient’s problem all on his own. Difficult decisions are increasingly made in multidisciplinary consultation, where specialists from various disciplines discuss patients on a case-by-case basis and decisions are thoroughly justified and recorded. The days of deduction are therefore numbered and, who knows, perhaps surgeons and internists will begin to understand each other in the not too distant future?

  But one thing will never change. Once a surgeon is standing at the operating table, scalpel at the ready, he is completely alone and everything that he does from that moment, everything that happens to his patient, remains his own, personal responsibility. Then you want to be sure of what you are doing, and you do not help your conscience by working on the basis of probabilities.

  13

  Complications

  The Maestro and the Shah: Mohammed Reza Pahlavi

  DURING THE SECOND World War, German actress and singer Marlène Dietrich warmed the heart of many a soldier in the front line with her sensual song Ich bin von Kopf bis Fuß auf Liebe eingestellt’ (‘I am, from head to toe, ready for love’1). That was quite a statement from a woman with such long legs. It was even claimed that she had the most beautiful legs in the world. In photographs, she was often portrayed with a cigarette in her hand and that famous sultry expression on her face. All those cigarettes eventually led to the arteries in those beautiful legs clogging up and Dietrich had to be operated on by a vascular surgeon. In her eyes, there was only one man good enough to be permitted to work his magic on her world-famous pins: Michael DeBakey.

  A vascular surgeon is a surgeon who specialises in blood vessels, and arteries in particular. Vascular surgical techniques of joining together arteries and veins were devised and tried out in the early years of the twentieth century by just one man, French surgeon Alexis Carrel. Carrel’s contribution was considered so important to the advance of general surgery that he was awarded the Nobel Prize for Medicine in 1912. The conditions under which vascular operations are carried out are exceptional. As blood vessels are relatively small, the needles and thread used in operations must be smaller than those used for other parts of the body. And because blood spurts out immediately if you cut open a blood vessel, it has to be temporarily clamped shut. But the clamps must not stay on for too long, as a limb or organ cannot go without blood for very long. Moreover, once blood stops flowing it can coagulate. And, even after the blood vessel has been sewn up and the blood is flowing again, it can be clogged up again by blood clots on the stitching in the wall of the vessel. Because healthy blood vessels are essential for the survival of organs and other body parts, there is often a greater sense of urgency surrounding vascular operations and a successful operation often feels more like a rescue. No wonder, therefore, that it was a vascular surgeon who was considered an international hero by so many celebrities in the twentieth century.

  Vascular surgery was new and exciting and opened up the way to the ultimate organ, the heart. The development of cardiac surgery, operations on the heart, led to a feeling of omnipotence in the surgical world and when the ultimate peak was reached in 1967 – the first successful heart transplant by Christiaan Barnard in Cape Town – it was of the same order as the first moon landing two years later. Michael DeBakey, cardiovascular (heart and blood vessels) surgeon at the Methodist Hospital in Houston, had been at the centre of all these developments. He had conducted groundbreaking work and was involved in the development of the first artificial heart. But he was especially a pioneer in the treatment of a less common disorder, aortic dissection – a very complex problem for a vascular surgeon. Aortic dissection occurs when a tear develops in the inner layer of the aorta, the main artery in the body, which originates in the heart. The blood is forced at high pressure through the tear and between the inner and outer layers of the aorta, which are pushed further and further apart. This is not only very painful, but also threatens the supply of blood to the brain, the arms, ultimately, the rest of the body. DeBakey’s operation made it possible to cure this dramatic problem.

  DeBakey was known as the maestro. He acquired worldwide renown (and his nickname) thanks to his most famous patient, former King Edward VIII of Great Britain, who went to America unannounced in 1964 to be operated on by DeBakey. Like Dietrich, Edward was a heavy smoker – as indeed most of a vascular surgeon’s patients are. At this point Edward was seventy years of age and needed what at that time was a life-threatening vascular operation. But he did not go into any detail with the media, saying only ‘I came to see the maestro.’ When Russian president Boris Yeltsin needed a quintuple bypass operation thirty- two years later, in 1996, he clearly did not entirely trust his Russian cardiac surgeons and had the now eighty-seven-year-old maestro flown over from America to assist them. Boris called DeBakey ‘the magician’. All of the other celebrities who were DeBakey’s patients – King Leopold III of Belgium, King Hussein of Jordan, Hollywood stars Danny Kaye and Jerry Lewis, multimillionaire Aristotle Onassis, American presidents Kennedy, Johnson and Nixon, and Yugoslav dictator Tito – must have shared this opinion. It also did no harm to Michael DeBakey’s reputation that he was anything but modest and enjoyed his fame.

  So when Mohammed Reza Pahlavi, the deposed Shah of Iran, had to undergo a splenectomy (surgical removal of the spleen) in 1980, in his eyes there was only one surgeon on the planet who could do it. The fact that, as a cardiovascular surgeon, DeBakey actually had nothing to do with the spleen, was apparently not relevant, either for himself or for his esteemed patient.

  When the shah fled the revolution in Iran on 16 January 1979 and boarded a plane in Tehran never to see his home country again, he was not only threatened with death by Ayatollah Khomeini and the Islamic rebels but also by cancer. His exile was to beco
me not only a wandering quest from one country to another, where he was always unwelcome, but also a fight against malignant non-Hodgkin lymphoma in his abdomen.

  The shah was treated by the French oncologist Professor Georges Flandrin, who followed him from one country to another. An oncologist is an medical specialist in general internal medicine – not a surgeon – who specialises in treating cancer. Flandrin’s patient suffered continually from anaemia and pain and, to make things worse, had developed an infection of the gall bladder. He underwent a cholecystectomy, surgical removal of the gall bladder, in New York. The American surgeons confirmed that the shah’s liver, and more especially his spleen, were enlarged as a consequence of his malignant disease. He had a hepatosplenomegaly, a medical term that literally means simultaneous enlargement of the liver and the spleen. The large spleen meant that his blood cells were continually broken down; it was also the cause of his pain. The shah recovered reasonably well from his gall-bladder operation, though his admission to the hospital led to demonstrations and riots outside the building, and he and his family no longer felt safe in America. The problem with his gallstones had been solved, but that had no impact on his illness. His pain and fatigue increased and the time came to remove his enormous spleen.

  Shortly afterwards, there was a hostage drama in the American embassy in Tehran and President Jimmy Carter probably wanted to be rid of his high-ranking guest as quickly as possible. The shah and his wife, Empress Farah Diba, moved on to Mexico, the Bahamas and Panama, but everywhere they went, the threat of extradition hung over their heads. An operation could not be carried out under these circumstances. But President Sadat of Egypt was willing to offer his old friend shelter and medical care, so in March 1980 the shah arrived at the Maadi Military Hospital in Cairo. Five days later, DeBakey arrived with his assistants, an anaesthetist and a pathologist. On 28 March, the operation to remove the spleen was performed by two surgeons, DeBakey and the Egyptian Fouad Nour. The patient’s wife and eldest son watched the operation live via a television connection with the operating room. The operation went well and, according to DeBakey, the shah’s spleen was as large as an American football.

  The spleen has relatively little function in the body and you can afford to lose it, if necessary. It plays a role in maintaining the quality of the blood by filtering out old blood cells and, especially at a younger age, is part of the body’s immune system. Because you can sometimes feel a strange sensation near the spleen when running or when you get the giggles, Pliny the Elder thought that the function of the spleen had something to do with these activities. There are two references to splenectomies performed in the sixteenth century. In 1549, Adriano Zacarelli is recorded as having removed a young woman’s spleen in Naples and, in 1590 Franciscus Rosetti allegedly removed half a one, again in Italy. It seems unlikely, however, that these operations really did remove the spleen, as the first abdominal operation in which the patient survived was not carried out until 1809. More probably, in both cases, it was a large clot of blood resulting from a deep subcutaneous contusion. Such a clot can closely resemble the spleen, with the same colour and the same solid texture, explaining why the two Italians thought the clots they had removed were spleens. The first genuine successful splenectomy was performed in Paris in 1876 by Jules-Émile Péan. It was the spleen of a twenty-year-old woman and weighed more than a kilogram.

  A splenectomy does not have to be a difficult operation, as long as you stick to the rules. The procedure can be learned in the third or fourth year of training to be a surgeon. There are a couple of things to watch out for, but the spleen itself is relatively straightforward. It is normally the size of half an avocado and looks a little like a toadstool, with the blood vessels carrying blood to and from the organ on one side, resembling the toadstool’s stalk. But it is difficult to get at, hidden away deep in the top left of the abdominal cavity. You have to stick both hands into the abdomen past your wrists to reach it. And the spleen is very delicate. If you pull or push it too hard, it can rip, which is dangerous as a spleen can bleed heavily. And if it ruptures, you can easily lose sight of it because of all the blood, so you have to avoid that at all costs. And then there is the final warning that surgeons give when teaching the operation: watch out for the tail of the pancreas!

  The pancreas is an elongated organ, described in German as the ‘abdominal saliva gland’. The digestive juices that the pancreas produces, however, are much more aggressive than saliva. They digest, for example, the meat in our food. The tail of this organ runs alongside the blood vessels of the spleen and can extend as far as the spleen stalk. If you place the clamp on the blood vessels of the spleen a little too far to the right, you not only remove the spleen, but also a piece of the pancreas. That can be very dangerous, as pancreatic juices can leak into the abdominal cavity and literally digest the tissues of the body, producing pus. With a normal spleen, it is fortunately not overly difficult to place the clamp correctly and spare the pancreas. But the shah’s splenectomy was especially difficult because the organ was so big.

  Nour had asked DeBakey, ‘Isn’t the tail of the pancreas caught in the clamp?’ But DeBakey dismissed his Egyptian colleague’s observation with a wave of the hand and tied the tissue under his clamp with a large ligature. Nour cautiously suggested at least leaving a drain behind, a small tube to allow any excess fluid to run out of the abdomen, just in case, but DeBakey thought that was unnecessary and closed the abdomen without a drain. He was applauded when he removed his gloves. The spleen weighed 1,900 grams. Cancer was found in the spleen and in the pieces of liver that had been removed for testing. Unfortunately, the microscopic study also found pancreatic tissue …

  The third day after the operation, the patient developed pain in the back of his left shoulder and a fever. But the wound from the operation healed quickly and the shah was able to walk in the garden of the hospital again when DeBakey left for Houston. There, he allowed himself to be interviewed like a hero, but his patient, far away, was slowly deteriorating. The fever refused to go away and the shah felt sick and tired. He had little pain, but lay in bed the whole day.

  The fever continued unabated for several months, day after day. The shah was given blood transfusions and antibiotics, a procession of American doctors came and went, but DeBakey himself remained in Houston and had X-ray photographs sent over. He guessed that the shah had pneumonia in the lower left lung. A bronchoscopy was performed – an unpleasant examination of the airways – but no problems were found. The many specialists involved had completely lost sight of the big picture and, in Paris, Professor Flandrin followed the situation with mounting amazement. Did no one see that the patient simply had an abscess under his diaphragm?

  It is a classic cause of error in surgery: an infection in the abdominal cavity produces fever and irritation of the peritoneum, unless the infection is located below the diaphragm. Then the only symptom is fever. The medical term for ‘under the diaphragm’ is subphrenic. Pus under the diaphragm is therefore referred to as a subphrenic abscess. If a patient has an infection in the abdominal cavity and the peritoneum is irritated, he will experience severe pain, which will increase with the slightest movement; this is an overtly clear indication for every doctor. But if only the diaphragm is irritated and not the peritoneum, these telltale symptoms do not arise. The patient suffers only a fever, with perhaps the hiccups, or pain in the shoulder. Flandrin saw this, and he was not even a surgeon. Even the X-rays of the lungs fitted the picture. He decided to do something about it, flew to Egypt and started arguing with everybody. He had a surgeon, Pierre-Louis Fagniez, flown out from France. On 2 July, Fagniez made a small incision in the upper left of the shah’s abdomen and drained a litre and a half of pus from the abdominal cavity. The shah had thus been left for three months with a large abscess below his diaphragm. He immediately felt better, was able to walk around, got his appetite back and started to concern himself with affairs of state again. But three and a half weeks later, he suddenly collapsed.
His blood pressure fell, he became deathly pale, and he lost consciousness. He was given blood, but not operated on. The shah died unexpectedly of internal haemorrhaging on 27 July 1980. He was sixty years old.

  He had been suffering from Waldenström’s macroglobulinaemia, a rare, not very aggressive form of non-Hodgkin cancer that can develop in the liver and the spleen. Yet this was not the cause of the shah’s death; that was due to the damage to his pancreas during DeBakey’s splenectomy. That complication was iatrogenic, i.e. ‘caused by a doctor’. The leakage of pancreatic juices after the surgeon had snipped through the tail of the pancreas had led to infection of the large hollow cavity below the diaphragm left behind after removal of the large spleen, which had filled with pus. The aggressive pancreatic fluids would then have eaten away at the wall of the splenic artery, which could lead to sudden arterial haemorrhaging in the upper abdomen.