Under the Knife

by Arnold van de Laar, Laproscopic surgeon

  It is tempting to compare this story with what happened to Abraham, but there is no official evidence of Louis undergoing a circumcision or any other operation on his foreskin. Yet it may be no coincidence that Dr Lassone was something of an expert in the surgical treatment of phimosis. He had even developed his own method for performing the operation, which he did not describe until much later, in 1786. It involved the smallest possible intervention, making only a few shallow scratches across the foreskin, rather than cutting it open completely, so that it could be pulled more easily over the glans. In this way, the foreskin was preserved completely intact without being deformed. Is it possible that Lassone performed this minor operation on Louis?

  Because no clear – surgical – explanation was given for Marie-Antoinette’s sudden pregnancy, the people of France most likely believed that she had committed adultery. Later, too, the royal couple rarely shared their marital bed and Marie-Antoinette was seen with other men. Before long, the French Revolution broke out and Louis and his wife were taken prisoner. What finally happened to them in 1793 is history. They had four children in total, of whom only the eldest, Marie-Thérèse, survived the Revolution.

  According to an estimate by the World Health Organisation, in the year 2006 some 665 million men and boys underwent circumcision. Although a single foreskin weighs only a few grams, this means that hundreds of tons of foreskin are cut away every year. An estimated 30 per cent of the world’s current population has been circumcised. That makes circumcision without doubt the most widely performed operation, not only now, but of all time.

  In historic times, the foreskin was perhaps rightly considered unhygienic. In Arabic, the word for circumcision literally means ‘cleaning’. In modern times, however, the removal of the foreskin no longer has any demonstrable medical benefits. And, although today’s surgical conditions mean that complications are now rare, serious bleeding and infections do still occur, sometimes with fatal consequences. From a surgical perspective, it is unacceptable to conduct a futile operation on children who are too young to ask if they approve of their foreskin being removed for ever.

  For men and boys suffering from a genuine phimosis, as was perhaps the case with Abraham and Louis XVI, full circumcision is not actually necessary. In the case of children, the problem often rights itself or is cured with a salve. If not, it can be put right with a much less invasive operation than circumcision. For adults, too, there are various methods that leave the function of the foreskin intact, as with the operation devised by Lassone.

  4

  Shock

  The Lady and the Anarchist: Empress Sisi

  IN MEDICAL TERMS, shock means a failure of the blood’s circulatory system. A constant flow of blood is essential for every organ in our bodies. And that requires sufficient blood pressure. Shock is what happens if our blood pressure falls so low that our organs do not receive sufficient oxygen, and it has catastrophic consequences.

  Not all organs can last equally long without a sufficient supply of blood. The brain and the kidneys will be the first to fail. Then our consciousness decreases and our urine production stops. That is followed by the intestines, lungs, liver and heart. A state of shock that persists for too long therefore leads to multiple organ failure (MOF). To understand the mechanisms of shock, it is first important to know that the walls of the arteries in our bodies contain small muscles that allow the blood vessels to dilate or contract, known medically as vasodilation (widening of the blood vessel) and vasoconstriction (narrowing of the vessel). This is one way in which our bodies can regulate blood pressure. The heart can also affect blood pressure by beating slower or faster or by pumping more strongly.

  The circulatory system comprises three essential components: the heart, the blood and the blood vessels. The heart pumps the blood through the vessels. Failure of the circulatory system can be caused by any of these three components, resulting in different kinds of shock. Firstly, there is cardiogenic shock (literally ‘caused by the heart’), which may occur because of a heart attack, a faulty heart valve or an injury to the heart. Secondly, there is hypovolemic shock (literally ‘with too little volume’), which is caused by insufficient blood being pumped around the system, for example as a result of dehydration or bleeding. In both cases, the blood vessels will constrict (vasoconstriction) to keep the blood pressure up. This reflex is triggered by nerves that run to the blood vessels, and by adrenaline released by the adrenal glands. By contrast, the third form of shock (septic shock) occurs when the blood vessels dilate excessively as a result of toxic substances paralysing and damaging the vessel wall. That causes the blood pressure to fall, the mechanism that regulates the blood pressure to stop working and fluid to leak to the surrounding tissues. The toxic substances that invoke septic shock come mostly from bacteria or dead tissue resulting, for example, from burns, gangrene or blood poisoning.

  An operation can cause all three kinds of shock: cardiogenic by overtaxing the heart, hypovolemic through blood loss, or septic shock as a result of damaged tissue and infection. Shock can sometimes be treated surgically by, for example, stopping massive haemorrhaging, draining pus from an infection, or cutting away dead or damaged tissue. In this chapter we look at the story of an exceptional woman with a case of shock, unfortunately with an unhappy ending.

  On 10 September 1898 an Italian anarchist named Luigi Lucheni attacked the Austrian empress, Elisabeth, commonly known as Sisi, by thrusting a small, triangular file into her chest. But when the sixty-year-old simply got to her feet again, straightened her hat and calmly continued on her way, he must have watched the aftermath of his assault with astonishment. It was only later, when two police officers arrested him for murder, that he realised he had apparently succeeded, after all.

  Lucheni would testify that his main motive was to kill someone royal, no matter who. His victim had been spotted by paparazzi some days previously in the Hôtel Beau Rivage on the shores of Lake Geneva, and Lucheni had read about it in the newspapers. The empress was in many ways the Lady Diana of her time. Not only because both their deaths were indirectly caused by paparazzi but, like Diana, she was a princess who married the handsome prince of an important country, just as in a fairy tale. In 1854, when she married the twenty-three-year-old Emperor Franz Joseph at the age of sixteen, she became both Empress and Queen of the mighty Habsburg Empire, which extended from Russia to Milan and from Poland to Turkey. The popularity of the beautiful Empress Elisabeth of Austria soared again in the 1950s, with the release of the ‘Sisi’ films, in which she was played by the beautiful Romy Schneider. The real Sisi’s life, however, was much less like a fairy tale than the film suggests. Elisabeth suffered from an eating disorder, which we would now call anorexia nervosa. In her youth, she weighed only 46 kilograms. In addition, she always wore a tight corset to preserve her wasp waist, which was less than 50 centimetres in circumference – the equivalent of a diameter of only 16 centimetres! She was wearing one of these contraptions when she left her hotel in Geneva on that day to take the steamboat to Montreux.

  Her lady-in-waiting Countess Irma Sztáray de Sztára et Nagymihály, who accompanied her, stated later that, as they were walking along the waterfront, her Royal Highness was suddenly knocked to the ground by a man. But she stood up again quickly, said that she was fine, and carried on walking so as not to miss the boat. Once on board, she became pale and fainted, but regained consciousness rapidly and asked what had happened. The boat was by then in open water and the captain was asked to turn about. To relieve her mistress’s distress, the lady-in-waiting loosened Elisabeth’s tight corset, at which the empress passed out again. Only then did the countess see a small spot of blood the size of a silver coin on the dying empress’s undergarment. The boat moored and members of the crew carried Elisabeth, who was probably already dead, back to the hotel on an improvised stretcher made of two oars. In the hotel, the death of the empress was confirmed by a doctor cutting open an artery in her arm. No blood came out. It was ten past two i
n the afternoon.

  An autopsy revealed a stab wound 8.5 centimetres deep near the fourth rib on the left side, which extended through the lung and across the whole width of the heart, causing internal bleeding. How could someone with such severe wounds through their heart still have got to the Montreux boat?

  Our bodies possess a number of regulatory and reserve systems to provide a first response to serious problems. The fact that the sixty-year-old Elisabeth was able to survive for that long with a punctured heart is, first and foremost, a sign that she was generally in good health. Sisi was a healthy woman. She was not overweight, had grown up in the mountains, had never smoked and had ridden horses her whole life. That healthy condition explains why all the organs and systems in her body were functioning well when the attack took place.

  Elisabeth was of course alarmed immediately after the incident. And she was afraid of missing the boat. This state of agitation stimulated a part of her nervous system known as the sympathetic nervous system, which had immediately made her body alert. Her heartbeat increased, her muscles received more blood and her adrenal glands were activated to release adrenaline into the bloodstream. The name ‘adrenal’ comes from the location of these two small glands, on top of (Latin ‘ad’) each kidney (Latin ‘ren’). High concentrations of adrenaline therefore flowed through her blood, strengthening the effects of the sympathetic nervous system. This must have given her enough energy to get to the boat on time.

  Sisi did not faint until she was on board. The cause was shock, a sudden fall in blood pressure. The first organ to suffer from low blood pressure is the one that needs the most oxygen, the brain. That is why a reduction in consciousness – fainting – is often the first sign of shock. The fall in blood pressure may have already been caused by the loss of blood from the heart – in other words, bleeding leading to hypovolemic shock – but that is not very likely. The internal blood loss from a punctured heart would, after all, have been so severe that Elisabeth would never have been able to walk another hundred metres. The loss of blood therefore had to be restricted by something and the shock must have been caused by something else.

  Sisi had developed a cardiac tamponade. The word tamponade comes from the French tamponner, meaning ‘to tamp’ or ‘plug up’. With a cardiac tamponade, blood from a wound to the heart accumulates in the pericardium, the rigid sac that surrounds (peri) the heart. As Luigi’s file was quite thin, the hole in the pericardium was too small for blood to escape easily. Therefore, the loss of blood was initially limited, but as it accumulated in the pericardium, the heart had less and less room and came under increasing pressure. In this way, an apparently small loss of blood could have serious consequences for the functioning of the heart.

  The shock was thus initially caused by the constriction of the heart and not by the loss of blood. A constricted heart cannot beat properly, so that Sisi first experienced cardiogenic shock. As a result of the reduced functioning of the heart, the blood pressure falls. That low blood pressure is registered at various points in the body. There are sensors on both sides of the arteries in the neck, which record low blood pressure and pass the information on to the brain stem. There the sympathetic nervous system is activated, contracting the blood vessels throughout the body to boost the blood pressure. The kidneys also register low blood pressure and temporarily retain the body’s reserves of fluid. If you could have asked her, Elisabeth would have said that she was terribly thirsty.

  * * *

  Specialisation

  If you tell people you are a surgeon, they usually ask ‘What kind of surgeon?’ Many do not seem to know that being a surgeon is a profession in itself, and that you can therefore be a general surgeon. Medical specialisations can be divided into medicine (the ‘non-cutting’ professions) – which includes internal medicine, paediatrics, neurology, psychiatry and pathology – and surgery (the ‘cutting’ professions). For many centuries, surgeons applied themselves to all aspects of surgery, but in the twentieth century a number of specialisations went their own way. Gynaecologists operate on the female reproductive organs, and urologists on the kidneys, the urinary tract and the male reproductive organs. Cosmetic surgery, reconstructive surgery, microsurgery and hand surgery are all performed by plastic surgeons. Neurosurgeons operate on the brain, the spine and the nerves. Orthopaedic surgeons focus on the musculoskeletal system, while ear, nose and throat specialists need no further explanation. The rest can be classified horizontally, by theme, or vertically, by organ system. Horizontally, there is traumatology (operations following accidents), oncological surgery (cancer operations) and paediatric surgery (operations on children). Vertically, there is cardiac surgery (on the heart), thoracic surgery (the lungs), vascular surgery (the blood vessels) and gastrointestinal or abdominal surgery (the organs in the abdomen). General surgery still embraces five of these components: traumatology, oncological surgery, thoracic surgery, gastrointestinal or abdominal surgery, and vascular surgery. Paediatric surgery and cardiac surgery are separate specialisations. In some countries, breast cancer is not treated by a surgeon but a gynaecologist and traumatology by orthopaedic surgeons. There are also a number of ‘super-specialisations’ within general surgery, including head and neck surgery, transplant surgery and bariatric surgery.

  * * *

  The lady-in-waiting reported that Sisi had become remarkably pale. The normal pink colour of the skin is caused by the flow of blood. If that pink becomes paler, it can be due to anaemia resulting from a severe loss of blood. But the contraction of the blood vessels also reduces the flow of blood in the skin, so the paleness that afflicted the empress when she fainted was consistent with cardiogenic shock. Contraction of the blood vessels can also make you go pale from fright. The lady-in-waiting probably looked as pale her mistress.

  A cardiac tamponade reduces the functioning of the heart in two ways. The heart is a hollow muscle that dilates to fill itself with blood and then contracts to pump it out again. In the event of a cardiac tamponade, the heart muscle can pump the blood out, but is unable to refill itself sufficiently because of the pressure in the pericardium. Consequently, with the next heartbeat, there is less blood to pump away. But something else happens, too. The strength of the heart muscle depends heavily on the heart being optimally filled. With a cardiac tamponade, the heart will therefore not only pump less amounts of blood but also with less power.

  Elisabeth fainted on the boat. Yet, shortly afterwards, she came round again in the arms of her lady-in-waiting. That is because, after fainting, she ended up lying horizontally. That increased the flow of blood from the legs and the abdomen to the heart, as it no longer had to fight gravity and flow upwards. As a result, Sisi’s heart was able to fill up with more blood, and thus pump more blood around and, above all, more strongly. Several minutes passed. It can be assumed that, during that time, a large volume of blood did actually flow through the small hole in the pericardium into the chest cavity. This was later confirmed by the autopsy. So how was it possible that Sisi was still alive and was able to talk to her lady-in-waiting?

  The answer to this medical puzzle is probably her corset. Because her abdomen and pelvis were compressed by the tight corset, there was relatively more blood in her upper body than normal. When the lady-in-waiting released the corset, this reserve of blood was once again able to flow throughout Elisabeth’s whole body, leaving relatively less blood close to her heart.

  After the corset was opened, therefore, the heart no longer filled with enough blood. The body had no more emergency plans left to call upon. The blood vessels were already contracted as far as possible and the heartbeat had reached its maximum rate, at Elisabeth’s age probably around 160 beats a minute. She may also have been struck by one last catastrophe. As a result of the shock, her heart itself could have received insufficient oxygen. The electrical circuit of the heart muscle is the first to notice this problem. Normally, the circuit ensures that the heartbeat is regular and coordinated, so that the heart can function optimally
. But a lack of oxygen can cause a fatal fault in this circuit. Elisabeth’s heart would have started to fibrillate, contracting chaotically with no effect at all, causing her death.

  If Elisabeth had made it to a hospital instead of the boat, it is doubtful whether they would have taken the chance of operating on her. Professor Theodor Billroth, the world-renowned surgeon who had called the shots in Vienna for many years, had died four years previously, but his words were still seen as the gold standard in surgery. He had been very resolute about heart surgery. Without a single ounce of evidence to support his statement, this bully of a professor had left the surgical world cowering under the threat: ‘Surgeons who attempt to operate on the heart can no longer count on the respect of their colleagues.’ Only two years after Billroth’s death did a surgeon, Ludwig Rehn, dare to stitch a stab wound in the heart for the first time. Although his patient, whose heart had been pierced by a sword, survived the operation, it would be many years before surgeons would begin to explore the field of cardiac surgery.

  Today, thanks to that breathtaking new branch of surgery, Elisabeth would have a greater chance of surviving a stab wound to the heart. It is only 2.5 kilometres from where she was attacked, the Quai du Mont-Blanc, to what is now Geneva University Hospital. An ambulance could be on the spot within ten minutes. A happy ending would, however, require bystanders on the quay or the jetty to start treating her shock immediately. The lady-in-waiting would have to start CPR as soon as Sisi fainted after her corset was opened. The rhythmic up and down movement of the sternum turns the whole chest into one large pump. This would have kept Elisabeth’s blood pressure up at a safe level. Performing CPR is very tiring and the lady-in-waiting’s pale face would soon become scarlet. Other people would have to take over and keep going until the ambulance arrived. The ambulance crew would then immediately insert a breathing tube into the windpipe and a needle into a vein to introduce litres of fluid directly into the blood vessels – the most effective way to treat shock. If the heart was fibrillating, they could use a defibrillator to deliver an electric shock to normalise the heartbeat. They would also administer adrenaline through the intravenous needle and oxygen through the breathing tube, and the empress would be made ready for transport to hospital. In the meantime, at the hospital, an operating team would be called together and, in the operating theatre, the heart–lung machine would be prepared. Once she was on the operating table, her sternum would be sawn open vertically to connect the input and output tubes of the heart–lung machine in the open chest cavity. The machine would take over the pumping action of her heart and the respiratory function of her lungs. The surgeons would pour ice water into her chest cavity to stop her heart and cool it off, and the operation could start. But, in 1898, that was all far into the future.