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A Christmas Story: Richard von Volkmann, Charles Dickens, and the Children Peter R. Carter, MD
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ODAY I WANT TO TELL YOU A STORY,
a story of my personal experience with Volkmann contracture—the nightmare of every orthopedic and plastic surgeon. It began a very long time ago at what was then called the Texas Scottish Rite Hospital for Crippled Children. I was a third-year resident. My mentor there was a wonderful man, Dr. Brandon Carrell, the Chief of Surgery. He was highly experienced and the most respected pediatric orthopedic surgeon in my community. Together we saw a young boy with a useless hand due to Volkmann contracture. Kindly old Dr. Carrell did a careful examination and then gently told the family the situation was hopeless. It was during that rotation when I decided that I wanted to be a hand surgeon, and later I did my fellowship at Roosevelt Hospital in New York City. There, Dr. J. William Littler taught me that for many of these children there was something that could be done. After my fellowship, I returned to Dallas in private hand surgery practice. As luck would have it, I was soon referred a 3-year-old boy with Volkmann contracture (Fig. 1A). He had been jumping on his bed and fell, sustaining the dreaded supracondylar humerus fracture. The same night, a good orthopedist carried out a closed reduction and percutaneous pin fixation of the fracture. The patient stayed overnight in the hospital to watch for swelling. The next morning, the surgeon recognized a compartment syndrome, took the child immediately to the operating room, and performed a wide fasciotomy. Retired from the Department of Orthopaedic Surgery, University of Texas Southwestern Medical School; and Texas Scottish Rite Hospital for Children, Dallas, TX. Received for publication October 16, 2014; accepted in revised form October 17, 2014. No benefits in any form have been received or will be received related directly or indirectly to the subject of this article. The Robert Carroll Founders Lecture, Annual Meeting of the American Society for Surgery of the Hand, Boston, MA, September 18, 2014. Corresponding author: Peter R. Carter, MD, 9320 Stratford Way, Dallas, TX 75220; e-mail: [email protected]. 0363-5023/14/3912-0022$36.00/0 http://dx.doi.org/10.1016/j.jhsa.2014.10.027
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I saw the patient 6 weeks later. The fracture was healed in perfect position, but the hand was useless. The parents were frightened and the orthopedist was horrified. Figure 1B shows the result after one year, following the 2 procedures taught to me by Dr. Littler. A successful reconstruction of Volkmann contracture of the forearm is one of the most dramatic and rewarding results in all of hand surgery. You can imagine how grateful the family was. The orthopedist was relieved; as far as I know, he never sent another hand case to anyone else. I was hooked. I had to find out more about this. I started by asking myself: Just who was this guy Volkmann? My old chief of orthopedics used to tell us as residents that if, on our boards, we ever were asked who some eponymous surgeon was, we should say: “a famous British orthopedist around the turn of the last century.” Richard von Volkmann was certainly a famous surgeon of the 19th century, and he was famous for so much more that the muscle contracture that bears his name. But he was German, not English. The story of his life is part of this story. Volkmann was born in the university town of Leipzig. His family soon moved 30 miles up the road to Halle, where he grew up, lived, and practiced his whole life. He died in Jena, a few miles to the south. Halle was and still is one of Germany’s ancient university towns. The university at Halle was founded in 1502 and the medical school in 1694. The medical school was nearly 200 years old when Volkmann taught there. I suspect you know Richard’s father, too: Alfred Volkmann. He was professor of anatomy and physiology at Halle and discovered the micro-canals in cortical bone that bear his name. Growing up in an academic family, Richard received a classical education and became conversant in several European languages as well as Latin and Greek. He finished medical school in 1854 at a time when surgery was well on its way to becoming a science. Just 10 years before, the discovery of anesthesia had changed surgery forever. A little more than a decade
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FIGURE 1: A The author’s first personal case of Volkmann’s contracture. B The result after a year and 2 procedures. (Source: author.)
later, Lister, based on the germ theory popularized by Pasteur, discovered a technique to prevent the nearly inevitable fatal outcome of most surgery— wound infection. When Volkmann finished medical school, he went to work for the Halle Professor of Surgery, Ernst Blasius. Blasius had invented the z-plasty and yev-plasty but was 61 years old and going blind. Apparently, Blasius and Volkmann did not get along and Richard lasted a year before he left the university and entered private practice in Halle. Volkmann knew the first rule of private practice: availability. It was said, “He would go anywhere and do anything to save a patient.” After he became successful, Volkmann himself said, “A surgeon either has no bread or no time to eat it.” In spite of his busy schedule, he found time to marry Anna von Schlechtendahl and together they had 11 children. In addition to his private practice and probably thanks to his father’s introduction, he lectured at the J Hand Surg Am.
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university and was a popular lecturer with his students. Within a few years of the practice of surgery, Volkmann became interested in bone and joint diseases, and this would be the subject of his first book. During the 19th century, tuberculosis was the cause of most of these conditions. The bacteriological etiology at the time was unknown; it would be over 20 years before Koch would isolate mycobacteria. During the early years of Volkmann’s career, when Prussia was at war with Austria, Volkmann ran a field hospital (Fig. 2). Later, during of the FrancoPrussian war, he was Surgeon General of the Army at the Siege of Paris. Out of 13,000 amputations, 10,000 died of infection. Coincidental to this disaster, Volkmann had heard of Scottish surgeon Joseph Lister’s work with carbolic acid spray and was interested enough to dispatch an assistant to Glasgow. This discovery coincided with Volkmann’s return to Halle after the war, where postoperative infections and related deaths in his hospital had reached Vol. 39, December 2014
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FIGURE 2: Volkmann in his uniform. (Source: http://www.wfbverlag.de/artikelliste/kategorie/volkmann-leander-richard-von. html.)
catastrophic levels. Volkmann applied Lister’s technique of an atomized carbolic acid (phenol) spray, and the infection rate dropped to 5% and saved the hospital from closure. He became a lifelong friend of and advocate for Lister, a fact that contributed to the adoption of the asepsis in German operating rooms even before those in Britain. For his work in the war and treatment of postoperative infections, Volkmann was made Herr Professor of Halle and his career took off like a rocket. Volkmann is considered by many to be the founder of “scientific” orthopedics. Here are a few of his contributions. Predating modern surgical journals, he published a “blog” he called A Collection of Clinical Reports, which cemented his fame in Germany. He invented the bone curette. He incorporated traction and fabricated an orthosis for fracture treatment. He identified what he called congenital dislocation of the ankle 20 years before Roentgen discovered x-rays; today we know it as fibular hemimelia. He was the first J Hand Surg Am.
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to successfully resect a rectal carcinoma. He was the first to associate an exogenous chemical as a cause of cancer . in paraffin workers. He recognized the relationship of loading to bone growth now known as the Heuter-Volkmann Law. He helped found the German Surgical Society. A Collection of Clinical Reports became the The German Surgical Journal. One hundred fifty years ago, he operated on scoliosis, resecting the rib hump. The discovery of ischemic contracture of the forearm may be one of the least of his accomplishments. By 1879, the University of Halle had built him a building with his own operating room. It became known as The School for Surgeons and surgeons came from around the world to learn from the great man. In the late 19th century, European science indisputably led the world. The birth of the fields of physiology, pathology, and bacteriology occurred there. American medicine was far behind that in Europe and a few American doctors began traveling to Europe to learn about “scientific medicine.” One of these was a pathologist from Belleview Hospital in New York, William H. Welch. One of the first scientific pathologists in America, he would discover the bacterial cause for gangrene—the organism we know as Clostridium welchii. But his greatest contribution would be as a medical administrator. Soon after returning home from Europe, Welch was asked by a wealthy industrialist in Baltimore to start a new medical school, become its dean, and hire the faculty. The industrialist had the curious first name of Johns. His last name was Hopkins. Welch was committed to raising American medicine to the highest scientific level in the world. He was responsible for opening the Rockefeller Institute and training its first director, Simon Flexner. Investigators at the Rockefeller Institute would go on to win 23 Nobel Prizes. Flexner’s younger brother Abraham would publish a report that led to the closing of proprietary medical schools in the United States and to the establishment for rigid standards in medical education in America and Europe. These standards were modeled after those of Hopkins’ medical school. Welch knew a bright young surgeon, William Halsted, in New York and after returning from Europe, Welch encouraged him to travel to Germany to see first-hand the amazing things that were happening. Later, Welch and Halsted, along with the internist William Osler and the gynecologist Howard Kelly, would be the founding faculty, “The Big Four”, of Johns Hopkins School of Medicine. In 1879, after Halsted first heard Volkmann lecture, he wrote to Welch: Vol. 39, December 2014
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Volkmann was tall, slender and animated, He had reddish hair and wore flowing Dundreary whiskers and long moustaches. His costume at the first séance of the congress, I recall vividly: Swallow-tailed coat, white silk waistcoat, spangled with embroidered flowers, with a flowing bright artist’s tie and scotch plaid trousers. He was a forceful, logical and picturesque speaker—a genius and also, as you know, a poet.
For many years I have noted on occasion, following the use of bandages too tightly applied, the occurrence of paralysis and contraction of the limb, not, as has been previously assumed, due to paralysis of the nerve by pressure, but as quick and massive disintegration of the contractile substance . The paralysis and contracture are to be understood as purely myogenic. We also get a glimpse of Volkmann the man in this paper by the way he graciously credited the previous work of a basic science researcher named Kraske:
Two years after Halsted’s visit, the European scientific medical community was soaring to new heights with leaders in every field of scientific endeavor reporting astonishing new discoveries. The 1881 International Conference of Medicine, arguably the greatest medical conference ever, assembled in London. Most of the great scientists were there. In the 1880s, Baroness Angela Burdette-Coutts, a London socialite and the richest heiress in England, was famous for her remarkable parties. Although popular on the London social scene, the Baroness was also an avid philanthropist and known as the “Queen of the Poor.” Among her many accomplishments, she, along with Charles Dickens, established a home for former prostitutes. For the 1881 International Medical Conference, the most sought-after invitation was an extracurricular event for an afternoon backyard party at the Baroness’ estate, Holly Lodge. Only the most famous scientists were invited. Wanting to record the event, Coutts shunned a photographer and instead engaged the painter A. P. Tilt to paint what would become a famous portrait of participants of the event. The painting hangs today in the Wellcome Library in London (Fig. 3). Note the Baroness Coutts and nearby, Volkmann. Just below Volkmann is Frederick von Esmarch, the inventor of the tourniquet and the man who gave hand surgeons the bloodless operative field. Down and to the left is Lister, and in the back row are Sir James Padget and Jean Charcot, the famous French neurologist. Over to the right is William Osler, who in 1881 was still living in Canada. Osler would not move to Johns Hopkins for another 8 years, where together with Halsted, he would create America’s first residency programs with graduated responsibility for young physician and surgeons. This is the training program style in which you and I were trained. On Christmas Eve of the same year as the International Medical Conference, Volkmann published a paper that would earn him the most well known of his 2 eponyms. Let me read it for you from an English translation:
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That animal muscle cannot endure complete interruption of its arterial supply.has been shown clearly by Kraske in his fine work. Volkmann offered no real treatment for the condition, only an admonition against tight bandages and a warning that even in his initial series, radius fracture was common. It was 30 years later when Bardenheuer described the first fasciotomy. However, because the fasciotomy may either be too late or not work at all, all surgeons must remember the admonition of Robert Jones in 1928: One must of course recognize the potential dangers in injuries and fractures about the elbow. But it cannot be too emphatically stated that, despite every precaution, ischemic contracture may occur. Thanks to Volkmann’s 1881 paper, the world knew about ischemic contracture of muscle. Effective treatment for the fully developed infarct would not be discovered until 80 years later, however. Seddon began the process in 1944 when he described the deep location of the infarct plus the frequent survival of the more superficial muscle tissue. The ulnar wrist flexor, so critical to reconstruction success, was often spared due to its extrafascial location. Seddon also noted the unique bright yellow character of the infarct and its surrounding rind of normal muscle. This infarct causes shortening of the muscle tendon unit and is manifested by the clinical sign of a tenodesis effect. Initial treatments of these patients included teonotomy, muscle origin slides, carpectomy, and forearm bone shortening. They focused incorrectly on this shortening and were misguided. Finally, in the 1960s, Littler recognized that the essential deficit was loss of sensibility. Patients with fully evolved ischemic forearm contractures completely ignore their limb. It is like a piece of wood, like it is not even there (see Video 1,
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FIGURE 3: A Wellcome Library Portrait of the Burdett-Coutts Holly Lodge Party. B The line drawing identifying the persons attending. (Reprinted with permission of the Wellcome Library, London.)
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FIGURE 5: The mother’s photograph on the day of birth. (Source: author.) FIGURE 4: A photograph of a baby with Volkmann contracture. (Source: author.)
available on the Journal’s Web site at www.jhandsurg. org). This critical sensory deficit is caused by a compression neuropathy of the median and ulnar nerves high in the forearm. Seddon was aware of the nerve compression, but he considered it an infarction of the nerves and to be irreversible. A paradigm shift began quietly in 1962 when, buried in the Proceedings section of the British edition of The Journal of Bone and Joint Surgery, Littler described his experience with 3 cases of fully evolved Volkmann contracture. He showed some of these nerve compression palsies to be reversible and described a reconstruction in 2 stages, separated by considerable time. In the first stage he did a precise but radical removal of the entire muscle infarct, carefully leaving the all of the healthy tendons in place in the distal forearm to provide active interphalangeal flexion following a subsequent muscleetendon transfer. At the first operation, Littler also carried out a neurolysis of the median and ulnar nerves from above the elbow to the distal forearm. He took special care to preserve the neurovascular supply to the ulnar wrist flexor, an important requirement for an eventual good result. Because the infarct resection immediately corrected the
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tenodesis effect, there was no longer need to shorten the skeleton. Then Littler waited for several months while the nerves regenerated into the hand. At first, intrinsic muscle function returned. The claw deformity corrected, and the re-innervated intrinsic muscles provided active but weak flexion at the metacarpophalangeal joints. The most critical benefit was return of sensibility in the hand. The patient now was aware of their hand and tried to use it—the essential milestone for further rehabilitation following the second-stage reconstruction. Because at this point there was still no interphalangeal flexion, at a second procedure, Littler powered the extrinsic flexor tendons previously left intact in the distal forearm at the first operation. He used Phalen’s transfer of the tendon of extensor carpi radialis longus. Today, microsurgical techniques allow transfer of the gracilis muscle on its neurovascular pedicle. By attaching the stump of the patient’s anterior interosseous nerve to the nerve of the transferred gracilis muscle, in time strong profundus muscle function can return. I remember when Manktelow presented his cases at the 1977 ASSH meeting and showed a movie of one of his patients doing a chin up using the transferred gracilis muscle. It was the only time I have ever seen a standing ovation for a paper at the ASSH. Here is a movie of the 20-year follow-up of a
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FIGURE 6: A Hamlin’s case at 1 hour of age. B Fasciotomy in a 3-hour-old newborn. C Result, 2 years later. (Photographs courtesy of Dr. Charles Hamlin.)
FIGURE 7: The company of Manchester child amputees in formation. (Source: www.spartacus.schoolnet.co.uk.)
gracilis transfer done when the boy was 5 years old (see Video 2, available on the Journal’s Web site at www.jhandsurg.org). He is now 25 years old with no deterioration of the transfer. Littler’s staged procedure is effective in the majority of cases. Milder cases with no neurological J Hand Surg Am.
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impairment do not need it. Sadly, the severe or longstanding cases with no viable muscle in both compartments and nonreconstructable nerves receive little improvement from it. I would encourage everyone to read or read again a few pages written by Littler about the treatment of Vol. 39, December 2014
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FIGURE 9: Richard Leander, nom de plume for Richard von Volkmann. (Source: http://en.wikipedia.org/wiki/Richard_von_ Volkmann#mediaviewer/File:Richard_von_Volkmann2.jpg.)
FIGURE 8: Charles Dickens. (Source: http://en.wikipedia.org/wiki/ Charles_Dickens#mediaviewer/File:Dickens_Gurney_head.jpg.)
Volkmann contracture (see selected reading no. 2) I think this is one of the finest things ever written on the subject. Orthopedic surgeons are especially unaware of this landmark description of the condition and its effective treatment. Although Littler wrote these words almost a half century ago, as the famous Oliver Wendell Holmes said: There is a dead medical literature and a live one. The dead is not all ancient and the live is not all modern. And then . I saw this baby along with several others like it (Fig. 4). We had no idea what caused this horrible deformity. Finally, a few of the mothers gave us our first clue by bringing a photograph of the baby’s arm taken on the day it was born (Fig. 5). All of these newborn photos showed a swollen limb and a peculiar skin lesion that turned out to be a false clue and led invariably to inappropriate treatment. Instead of consulting a surgeon, the neonatologist called the dermatologist, who applied a topical antibiotic, covered up the lesion, and started drawing blood. In fact, the baby had a compartment syndrome and needed a fasciotomy. The cause of the condition is still unknown, but now we know early treatment that may save the child from a worthless limb. J Hand Surg Am.
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I showed photos of these cases to my long-time friend, Charlie Hamlin, who said, “I saw a case just like this when the baby was 1 hour old.” Thankfully, he had the presence of mind to take also a photograph (Fig. 6A). The same swollen limb and skin lesion are present along with cyanotic fingertips. All surgeons recognize and know the treatment for a swollen adult forearm, but in one 3 inches long the medical staff fails to recognize a compartment syndrome. Not Charlie—he saw this case for what it was and he knew the treatment for a compartment syndrome, even in a 3-inch forearm, should be fasciotomy. Under local anesthesia using a large Penrose drain for a tourniquet, he did the fasciotomy (Fig. 6B). Here is the result at age 2 years; even those dead looking fingers survived and the little boy has full function in his hand (Fig. 6C). In 2005, we published a collection of 24 cases (see selected reading no. 3), most of which came from the Texas Scottish Rite Hospital for Children but included Hamlin’s landmark case. This has been one of the most gratifying publications of my career because of the many e-mails like this: “I was called to the newborn nursery and the kid’s arm looked just like the photo in your article. I did a fasciotomy immediately and now the hand is normal.” Chance favors the prepared mind. Our obligation is to teach our neonatologist colleagues what this is—a Vol. 39, December 2014
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FIGURE 10: The Royal Alexandra Hospital in Rhyl, Wales, at Christmastime. (Source: rhylhistoryclub.wordpress.com.)
FIGURE 11: Queen Alexandra inspecting her troops (the patients) with Lord Treolar, the mayor of London, at the children’s hospital. A far cry from sinister, these children were now loved, even by the Queen. (Reproduced with permission from Carter AJ. A Christmas carol: Charles Dickens and the birth of orthopaedics. J R Soc Med. 1993;86(1):45e48.)
neonatal compartment syndrome—and to call us. Several years ago, I presented these cases to a university pediatric grand rounds. Afterwards, one of the pediatricians came up to me and said: “I run the newborn nursery here. This has got to be very rare. We see 18,000 deliveries a year and I have never seen a single case.” He was shocked when I told him: “I am not sure how rare it is, but 2 of the cases I showed this morning had seen you.” But I promised you a Christmas Story. Believe it or not it has not always been easy to raise money for crippled children. I live in Texas, a place where many of our citizens are both generous and wealthy. They have given millions to the Texas Scottish J Hand Surg Am.
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Rite Hospital for Children. Volkmann, however, lived in a different time, the middle of the Industrial Revolution. Power was provided by steam generated with coal. The sky over Europe was so dark from pollution that people never saw the sun. Rickets was common, and crowded conditions in the city were desperate. Tuberculosis was rampant and the mycobacterium that caused it would not be discovered by Koch until 1882. Even worse, there were no child labor laws. In the great cotton mills of Manchester, England, mill bosses especially favored children as young as 4 years old. Because of their small size, these children were expected to crawl under and clean the moving machinery. The result was catastrophic. Figure 7 is a photograph taken in Manchester, England during the Industrial Revolution. It was said: “there were so many pediatric amputations it looked as if the children had been off to war.” But into this horror came the social reformers of the late 19th century. In England, the most important was Charles Dickens (Fig. 8). It is hard to imagine this man’s popularity at the time; he was a rock star. Dickens was the first of bestseller authors. People gathered in homes and read his works aloud. Dickens also gave public readings to standing-room only audiences. He even hit upon the idea of serializing his books, selling them a chapter at a time to reduce the price to an affordable level for the poor and to maximize his readership. Over one third of all literate people owned his books, making him one of the richest men in England. He was a friend of the Queen, and even our beloved Baroness Coutts. Until Dickens, crippled children were viewed as sinister and were to be hidden and kept out of sight. Vol. 39, December 2014
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Classical literature and characters such as Shakespeare’s Richard III and Victor Hugo’s Quasimodo reinforced a suspicion of the crippled. Dickens’ books Oliver Twist, Nicholas Nickleby, and especially A Christmas Carol began changing the public view of crippled children. Across the channel another author, Richard Leander, known as the German Dickens, was gaining fame with such works as Dreams by French Firesides. It went through 10 editions and was published in 5 languages. Here is a passage from the English translation about a little girl with Pott disease: Once upon a time there was a woman who had a child, a small pale girl who was unlike other children. When the woman took her for a walk, the people in the street stopped and whispered. When the little girl asked, “Why do they look at me so strangely?” the mother answered, “Because you wear such a very pretty new dress.” After a time the mother died, and the child had no one to take her walking. She became very pale and did not grow. A year later her father remarried, and the little girl fearfully asked if she might accompany her stepmother shopping. The stepmother cruelly replied, “What would people say if they saw me walking with you? You’re a hunchback and must stay at home!” The little girl often wondered about her hunch and what might be inside of it. Since she was never permitted to go out of doors again, she gradually grew paler and weaker and finally died. When an angel came to take her to heaven, she could not believe that hunchbacks go to heaven. The angel smiled and showed her that there were 2 magnificent white angel wings hidden in her hunch.
Leander was a pen name for none other than Richard von Volkmann (Fig. 9). Remember Halsted said in his letter to Welch: “and as you know, also a poet.” Volkmann was the most beloved citizen of Halle and Germany. Wilhelm I knighted him and changed his name to “von” Volkmann. Following his death at age 59, the turnout for his funeral was said to be the largest ever held in Halle. Early in the 20th century, the Queen of England at the time, Alexandra, was so taken by Dickens’ story A Christmas Carol and the plight of Bob Cratchet and Tiny Tim that she raised money to open crippled children’s hospitals (Figs. 10, 11). The stories of Dickens and Volkmann changed the hearts of men forever. If it were not for the immortal characters they created—Volkmann’s little girl with Pott disease who had 2 white angel wings hidden in her hunch, or Dickens’ Tiny Tim and Bob Cratchet— we might well still be hiding crippled children today instead of helping them towards a chance at a happy and meaningful life. Today, children’s hospitals exist around the world, supported by local Scrooge equivalents who, through their magnificent philanthropy, provide a safe harbor for today’s Bob Cratchet parents and their Tiny Tim. Medicine has always been an innately humanistic science. These children and their parents prove it to me every day. Thank you, and Merry Christmas to all.
SELECTED READINGS
These stories were allegories, not just fairy tales. They were commentaries on the times and changed public opinion about the disabled. By the way, Richard
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1. Carter AJ. A Christmas carol: Charles Dickens and the birth of orthopaedics. J Royal Soc Med. 1993;86(1):45e46. 2. Littler JW. Volkmann’s contracture. In Converse JM, ed. Reconstructive Plastic Surgery, vol. 6. 2nd ed. Philadelphia, PA: WB. Saunders Co, 1977: 3130e3151. 3. Ragland R 3rd, Moukoko D, Ezaki M, Carter PR, Mills J. Forearm compartment syndrome in the newborn: report of 24 cases. J Hand Surg Am. 2005;30(5):997e1003.
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A Christmas Story: Richard von Volkmann, Charles Dickens, and the Children Peter R. Carter, MD
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ODAY I WANT TO TELL YOU A STORY,
a story of my personal experience with Volkmann contracture—the nightmare of every orthopedic and plastic surgeon. It began a very long time ago at what was then called the Texas Scottish Rite Hospital for Crippled Children. I was a third-year resident. My mentor there was a wonderful man, Dr. Brandon Carrell, the Chief of Surgery. He was highly experienced and the most respected pediatric orthopedic surgeon in my community. Together we saw a young boy with a useless hand due to Volkmann contracture. Kindly old Dr. Carrell did a careful examination and then gently told the family the situation was hopeless. It was during that rotation when I decided that I wanted to be a hand surgeon, and later I did my fellowship at Roosevelt Hospital in New York City. There, Dr. J. William Littler taught me that for many of these children there was something that could be done. After my fellowship, I returned to Dallas in private hand surgery practice. As luck would have it, I was soon referred a 3-year-old boy with Volkmann contracture (Fig. 1A). He had been jumping on his bed and fell, sustaining the dreaded supracondylar humerus fracture. The same night, a good orthopedist carried out a closed reduction and percutaneous pin fixation of the fracture. The patient stayed overnight in the hospital to watch for swelling. The next morning, the surgeon recognized a compartment syndrome, took the child immediately to the operating room, and performed a wide fasciotomy. Retired from the Department of Orthopaedic Surgery, University of Texas Southwestern Medical School; and Texas Scottish Rite Hospital for Children, Dallas, TX. Received for publication October 16, 2014; accepted in revised form October 17, 2014. No benefits in any form have been received or will be received related directly or indirectly to the subject of this article. The Robert Carroll Founders Lecture, Annual Meeting of the American Society for Surgery of the Hand, Boston, MA, September 18, 2014. Corresponding author: Peter R. Carter, MD, 9320 Stratford Way, Dallas, TX 75220; e-mail: [email protected]. 0363-5023/14/3912-0022$36.00/0 http://dx.doi.org/10.1016/j.jhsa.2014.10.027
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I saw the patient 6 weeks later. The fracture was healed in perfect position, but the hand was useless. The parents were frightened and the orthopedist was horrified. Figure 1B shows the result after one year, following the 2 procedures taught to me by Dr. Littler. A successful reconstruction of Volkmann contracture of the forearm is one of the most dramatic and rewarding results in all of hand surgery. You can imagine how grateful the family was. The orthopedist was relieved; as far as I know, he never sent another hand case to anyone else. I was hooked. I had to find out more about this. I started by asking myself: Just who was this guy Volkmann? My old chief of orthopedics used to tell us as residents that if, on our boards, we ever were asked who some eponymous surgeon was, we should say: “a famous British orthopedist around the turn of the last century.” Richard von Volkmann was certainly a famous surgeon of the 19th century, and he was famous for so much more that the muscle contracture that bears his name. But he was German, not English. The story of his life is part of this story. Volkmann was born in the university town of Leipzig. His family soon moved 30 miles up the road to Halle, where he grew up, lived, and practiced his whole life. He died in Jena, a few miles to the south. Halle was and still is one of Germany’s ancient university towns. The university at Halle was founded in 1502 and the medical school in 1694. The medical school was nearly 200 years old when Volkmann taught there. I suspect you know Richard’s father, too: Alfred Volkmann. He was professor of anatomy and physiology at Halle and discovered the micro-canals in cortical bone that bear his name. Growing up in an academic family, Richard received a classical education and became conversant in several European languages as well as Latin and Greek. He finished medical school in 1854 at a time when surgery was well on its way to becoming a science. Just 10 years before, the discovery of anesthesia had changed surgery forever. A little more than a decade
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FIGURE 1: A The author’s first personal case of Volkmann’s contracture. B The result after a year and 2 procedures. (Source: author.)
later, Lister, based on the germ theory popularized by Pasteur, discovered a technique to prevent the nearly inevitable fatal outcome of most surgery— wound infection. When Volkmann finished medical school, he went to work for the Halle Professor of Surgery, Ernst Blasius. Blasius had invented the z-plasty and yev-plasty but was 61 years old and going blind. Apparently, Blasius and Volkmann did not get along and Richard lasted a year before he left the university and entered private practice in Halle. Volkmann knew the first rule of private practice: availability. It was said, “He would go anywhere and do anything to save a patient.” After he became successful, Volkmann himself said, “A surgeon either has no bread or no time to eat it.” In spite of his busy schedule, he found time to marry Anna von Schlechtendahl and together they had 11 children. In addition to his private practice and probably thanks to his father’s introduction, he lectured at the J Hand Surg Am.
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university and was a popular lecturer with his students. Within a few years of the practice of surgery, Volkmann became interested in bone and joint diseases, and this would be the subject of his first book. During the 19th century, tuberculosis was the cause of most of these conditions. The bacteriological etiology at the time was unknown; it would be over 20 years before Koch would isolate mycobacteria. During the early years of Volkmann’s career, when Prussia was at war with Austria, Volkmann ran a field hospital (Fig. 2). Later, during of the FrancoPrussian war, he was Surgeon General of the Army at the Siege of Paris. Out of 13,000 amputations, 10,000 died of infection. Coincidental to this disaster, Volkmann had heard of Scottish surgeon Joseph Lister’s work with carbolic acid spray and was interested enough to dispatch an assistant to Glasgow. This discovery coincided with Volkmann’s return to Halle after the war, where postoperative infections and related deaths in his hospital had reached Vol. 39, December 2014
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FIGURE 2: Volkmann in his uniform. (Source: http://www.wfbverlag.de/artikelliste/kategorie/volkmann-leander-richard-von. html.)
catastrophic levels. Volkmann applied Lister’s technique of an atomized carbolic acid (phenol) spray, and the infection rate dropped to 5% and saved the hospital from closure. He became a lifelong friend of and advocate for Lister, a fact that contributed to the adoption of the asepsis in German operating rooms even before those in Britain. For his work in the war and treatment of postoperative infections, Volkmann was made Herr Professor of Halle and his career took off like a rocket. Volkmann is considered by many to be the founder of “scientific” orthopedics. Here are a few of his contributions. Predating modern surgical journals, he published a “blog” he called A Collection of Clinical Reports, which cemented his fame in Germany. He invented the bone curette. He incorporated traction and fabricated an orthosis for fracture treatment. He identified what he called congenital dislocation of the ankle 20 years before Roentgen discovered x-rays; today we know it as fibular hemimelia. He was the first J Hand Surg Am.
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to successfully resect a rectal carcinoma. He was the first to associate an exogenous chemical as a cause of cancer . in paraffin workers. He recognized the relationship of loading to bone growth now known as the Heuter-Volkmann Law. He helped found the German Surgical Society. A Collection of Clinical Reports became the The German Surgical Journal. One hundred fifty years ago, he operated on scoliosis, resecting the rib hump. The discovery of ischemic contracture of the forearm may be one of the least of his accomplishments. By 1879, the University of Halle had built him a building with his own operating room. It became known as The School for Surgeons and surgeons came from around the world to learn from the great man. In the late 19th century, European science indisputably led the world. The birth of the fields of physiology, pathology, and bacteriology occurred there. American medicine was far behind that in Europe and a few American doctors began traveling to Europe to learn about “scientific medicine.” One of these was a pathologist from Belleview Hospital in New York, William H. Welch. One of the first scientific pathologists in America, he would discover the bacterial cause for gangrene—the organism we know as Clostridium welchii. But his greatest contribution would be as a medical administrator. Soon after returning home from Europe, Welch was asked by a wealthy industrialist in Baltimore to start a new medical school, become its dean, and hire the faculty. The industrialist had the curious first name of Johns. His last name was Hopkins. Welch was committed to raising American medicine to the highest scientific level in the world. He was responsible for opening the Rockefeller Institute and training its first director, Simon Flexner. Investigators at the Rockefeller Institute would go on to win 23 Nobel Prizes. Flexner’s younger brother Abraham would publish a report that led to the closing of proprietary medical schools in the United States and to the establishment for rigid standards in medical education in America and Europe. These standards were modeled after those of Hopkins’ medical school. Welch knew a bright young surgeon, William Halsted, in New York and after returning from Europe, Welch encouraged him to travel to Germany to see first-hand the amazing things that were happening. Later, Welch and Halsted, along with the internist William Osler and the gynecologist Howard Kelly, would be the founding faculty, “The Big Four”, of Johns Hopkins School of Medicine. In 1879, after Halsted first heard Volkmann lecture, he wrote to Welch: Vol. 39, December 2014
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Volkmann was tall, slender and animated, He had reddish hair and wore flowing Dundreary whiskers and long moustaches. His costume at the first séance of the congress, I recall vividly: Swallow-tailed coat, white silk waistcoat, spangled with embroidered flowers, with a flowing bright artist’s tie and scotch plaid trousers. He was a forceful, logical and picturesque speaker—a genius and also, as you know, a poet.
For many years I have noted on occasion, following the use of bandages too tightly applied, the occurrence of paralysis and contraction of the limb, not, as has been previously assumed, due to paralysis of the nerve by pressure, but as quick and massive disintegration of the contractile substance . The paralysis and contracture are to be understood as purely myogenic. We also get a glimpse of Volkmann the man in this paper by the way he graciously credited the previous work of a basic science researcher named Kraske:
Two years after Halsted’s visit, the European scientific medical community was soaring to new heights with leaders in every field of scientific endeavor reporting astonishing new discoveries. The 1881 International Conference of Medicine, arguably the greatest medical conference ever, assembled in London. Most of the great scientists were there. In the 1880s, Baroness Angela Burdette-Coutts, a London socialite and the richest heiress in England, was famous for her remarkable parties. Although popular on the London social scene, the Baroness was also an avid philanthropist and known as the “Queen of the Poor.” Among her many accomplishments, she, along with Charles Dickens, established a home for former prostitutes. For the 1881 International Medical Conference, the most sought-after invitation was an extracurricular event for an afternoon backyard party at the Baroness’ estate, Holly Lodge. Only the most famous scientists were invited. Wanting to record the event, Coutts shunned a photographer and instead engaged the painter A. P. Tilt to paint what would become a famous portrait of participants of the event. The painting hangs today in the Wellcome Library in London (Fig. 3). Note the Baroness Coutts and nearby, Volkmann. Just below Volkmann is Frederick von Esmarch, the inventor of the tourniquet and the man who gave hand surgeons the bloodless operative field. Down and to the left is Lister, and in the back row are Sir James Padget and Jean Charcot, the famous French neurologist. Over to the right is William Osler, who in 1881 was still living in Canada. Osler would not move to Johns Hopkins for another 8 years, where together with Halsted, he would create America’s first residency programs with graduated responsibility for young physician and surgeons. This is the training program style in which you and I were trained. On Christmas Eve of the same year as the International Medical Conference, Volkmann published a paper that would earn him the most well known of his 2 eponyms. Let me read it for you from an English translation:
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That animal muscle cannot endure complete interruption of its arterial supply.has been shown clearly by Kraske in his fine work. Volkmann offered no real treatment for the condition, only an admonition against tight bandages and a warning that even in his initial series, radius fracture was common. It was 30 years later when Bardenheuer described the first fasciotomy. However, because the fasciotomy may either be too late or not work at all, all surgeons must remember the admonition of Robert Jones in 1928: One must of course recognize the potential dangers in injuries and fractures about the elbow. But it cannot be too emphatically stated that, despite every precaution, ischemic contracture may occur. Thanks to Volkmann’s 1881 paper, the world knew about ischemic contracture of muscle. Effective treatment for the fully developed infarct would not be discovered until 80 years later, however. Seddon began the process in 1944 when he described the deep location of the infarct plus the frequent survival of the more superficial muscle tissue. The ulnar wrist flexor, so critical to reconstruction success, was often spared due to its extrafascial location. Seddon also noted the unique bright yellow character of the infarct and its surrounding rind of normal muscle. This infarct causes shortening of the muscle tendon unit and is manifested by the clinical sign of a tenodesis effect. Initial treatments of these patients included teonotomy, muscle origin slides, carpectomy, and forearm bone shortening. They focused incorrectly on this shortening and were misguided. Finally, in the 1960s, Littler recognized that the essential deficit was loss of sensibility. Patients with fully evolved ischemic forearm contractures completely ignore their limb. It is like a piece of wood, like it is not even there (see Video 1,
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FIGURE 3: A Wellcome Library Portrait of the Burdett-Coutts Holly Lodge Party. B The line drawing identifying the persons attending. (Reprinted with permission of the Wellcome Library, London.)
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FIGURE 5: The mother’s photograph on the day of birth. (Source: author.) FIGURE 4: A photograph of a baby with Volkmann contracture. (Source: author.)
available on the Journal’s Web site at www.jhandsurg. org). This critical sensory deficit is caused by a compression neuropathy of the median and ulnar nerves high in the forearm. Seddon was aware of the nerve compression, but he considered it an infarction of the nerves and to be irreversible. A paradigm shift began quietly in 1962 when, buried in the Proceedings section of the British edition of The Journal of Bone and Joint Surgery, Littler described his experience with 3 cases of fully evolved Volkmann contracture. He showed some of these nerve compression palsies to be reversible and described a reconstruction in 2 stages, separated by considerable time. In the first stage he did a precise but radical removal of the entire muscle infarct, carefully leaving the all of the healthy tendons in place in the distal forearm to provide active interphalangeal flexion following a subsequent muscleetendon transfer. At the first operation, Littler also carried out a neurolysis of the median and ulnar nerves from above the elbow to the distal forearm. He took special care to preserve the neurovascular supply to the ulnar wrist flexor, an important requirement for an eventual good result. Because the infarct resection immediately corrected the
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tenodesis effect, there was no longer need to shorten the skeleton. Then Littler waited for several months while the nerves regenerated into the hand. At first, intrinsic muscle function returned. The claw deformity corrected, and the re-innervated intrinsic muscles provided active but weak flexion at the metacarpophalangeal joints. The most critical benefit was return of sensibility in the hand. The patient now was aware of their hand and tried to use it—the essential milestone for further rehabilitation following the second-stage reconstruction. Because at this point there was still no interphalangeal flexion, at a second procedure, Littler powered the extrinsic flexor tendons previously left intact in the distal forearm at the first operation. He used Phalen’s transfer of the tendon of extensor carpi radialis longus. Today, microsurgical techniques allow transfer of the gracilis muscle on its neurovascular pedicle. By attaching the stump of the patient’s anterior interosseous nerve to the nerve of the transferred gracilis muscle, in time strong profundus muscle function can return. I remember when Manktelow presented his cases at the 1977 ASSH meeting and showed a movie of one of his patients doing a chin up using the transferred gracilis muscle. It was the only time I have ever seen a standing ovation for a paper at the ASSH. Here is a movie of the 20-year follow-up of a
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FIGURE 6: A Hamlin’s case at 1 hour of age. B Fasciotomy in a 3-hour-old newborn. C Result, 2 years later. (Photographs courtesy of Dr. Charles Hamlin.)
FIGURE 7: The company of Manchester child amputees in formation. (Source: www.spartacus.schoolnet.co.uk.)
gracilis transfer done when the boy was 5 years old (see Video 2, available on the Journal’s Web site at www.jhandsurg.org). He is now 25 years old with no deterioration of the transfer. Littler’s staged procedure is effective in the majority of cases. Milder cases with no neurological J Hand Surg Am.
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impairment do not need it. Sadly, the severe or longstanding cases with no viable muscle in both compartments and nonreconstructable nerves receive little improvement from it. I would encourage everyone to read or read again a few pages written by Littler about the treatment of Vol. 39, December 2014
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FIGURE 9: Richard Leander, nom de plume for Richard von Volkmann. (Source: http://en.wikipedia.org/wiki/Richard_von_ Volkmann#mediaviewer/File:Richard_von_Volkmann2.jpg.)
FIGURE 8: Charles Dickens. (Source: http://en.wikipedia.org/wiki/ Charles_Dickens#mediaviewer/File:Dickens_Gurney_head.jpg.)
Volkmann contracture (see selected reading no. 2) I think this is one of the finest things ever written on the subject. Orthopedic surgeons are especially unaware of this landmark description of the condition and its effective treatment. Although Littler wrote these words almost a half century ago, as the famous Oliver Wendell Holmes said: There is a dead medical literature and a live one. The dead is not all ancient and the live is not all modern. And then . I saw this baby along with several others like it (Fig. 4). We had no idea what caused this horrible deformity. Finally, a few of the mothers gave us our first clue by bringing a photograph of the baby’s arm taken on the day it was born (Fig. 5). All of these newborn photos showed a swollen limb and a peculiar skin lesion that turned out to be a false clue and led invariably to inappropriate treatment. Instead of consulting a surgeon, the neonatologist called the dermatologist, who applied a topical antibiotic, covered up the lesion, and started drawing blood. In fact, the baby had a compartment syndrome and needed a fasciotomy. The cause of the condition is still unknown, but now we know early treatment that may save the child from a worthless limb. J Hand Surg Am.
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I showed photos of these cases to my long-time friend, Charlie Hamlin, who said, “I saw a case just like this when the baby was 1 hour old.” Thankfully, he had the presence of mind to take also a photograph (Fig. 6A). The same swollen limb and skin lesion are present along with cyanotic fingertips. All surgeons recognize and know the treatment for a swollen adult forearm, but in one 3 inches long the medical staff fails to recognize a compartment syndrome. Not Charlie—he saw this case for what it was and he knew the treatment for a compartment syndrome, even in a 3-inch forearm, should be fasciotomy. Under local anesthesia using a large Penrose drain for a tourniquet, he did the fasciotomy (Fig. 6B). Here is the result at age 2 years; even those dead looking fingers survived and the little boy has full function in his hand (Fig. 6C). In 2005, we published a collection of 24 cases (see selected reading no. 3), most of which came from the Texas Scottish Rite Hospital for Children but included Hamlin’s landmark case. This has been one of the most gratifying publications of my career because of the many e-mails like this: “I was called to the newborn nursery and the kid’s arm looked just like the photo in your article. I did a fasciotomy immediately and now the hand is normal.” Chance favors the prepared mind. Our obligation is to teach our neonatologist colleagues what this is—a Vol. 39, December 2014
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FIGURE 10: The Royal Alexandra Hospital in Rhyl, Wales, at Christmastime. (Source: rhylhistoryclub.wordpress.com.)
FIGURE 11: Queen Alexandra inspecting her troops (the patients) with Lord Treolar, the mayor of London, at the children’s hospital. A far cry from sinister, these children were now loved, even by the Queen. (Reproduced with permission from Carter AJ. A Christmas carol: Charles Dickens and the birth of orthopaedics. J R Soc Med. 1993;86(1):45e48.)
neonatal compartment syndrome—and to call us. Several years ago, I presented these cases to a university pediatric grand rounds. Afterwards, one of the pediatricians came up to me and said: “I run the newborn nursery here. This has got to be very rare. We see 18,000 deliveries a year and I have never seen a single case.” He was shocked when I told him: “I am not sure how rare it is, but 2 of the cases I showed this morning had seen you.” But I promised you a Christmas Story. Believe it or not it has not always been easy to raise money for crippled children. I live in Texas, a place where many of our citizens are both generous and wealthy. They have given millions to the Texas Scottish J Hand Surg Am.
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Rite Hospital for Children. Volkmann, however, lived in a different time, the middle of the Industrial Revolution. Power was provided by steam generated with coal. The sky over Europe was so dark from pollution that people never saw the sun. Rickets was common, and crowded conditions in the city were desperate. Tuberculosis was rampant and the mycobacterium that caused it would not be discovered by Koch until 1882. Even worse, there were no child labor laws. In the great cotton mills of Manchester, England, mill bosses especially favored children as young as 4 years old. Because of their small size, these children were expected to crawl under and clean the moving machinery. The result was catastrophic. Figure 7 is a photograph taken in Manchester, England during the Industrial Revolution. It was said: “there were so many pediatric amputations it looked as if the children had been off to war.” But into this horror came the social reformers of the late 19th century. In England, the most important was Charles Dickens (Fig. 8). It is hard to imagine this man’s popularity at the time; he was a rock star. Dickens was the first of bestseller authors. People gathered in homes and read his works aloud. Dickens also gave public readings to standing-room only audiences. He even hit upon the idea of serializing his books, selling them a chapter at a time to reduce the price to an affordable level for the poor and to maximize his readership. Over one third of all literate people owned his books, making him one of the richest men in England. He was a friend of the Queen, and even our beloved Baroness Coutts. Until Dickens, crippled children were viewed as sinister and were to be hidden and kept out of sight. Vol. 39, December 2014
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Classical literature and characters such as Shakespeare’s Richard III and Victor Hugo’s Quasimodo reinforced a suspicion of the crippled. Dickens’ books Oliver Twist, Nicholas Nickleby, and especially A Christmas Carol began changing the public view of crippled children. Across the channel another author, Richard Leander, known as the German Dickens, was gaining fame with such works as Dreams by French Firesides. It went through 10 editions and was published in 5 languages. Here is a passage from the English translation about a little girl with Pott disease: Once upon a time there was a woman who had a child, a small pale girl who was unlike other children. When the woman took her for a walk, the people in the street stopped and whispered. When the little girl asked, “Why do they look at me so strangely?” the mother answered, “Because you wear such a very pretty new dress.” After a time the mother died, and the child had no one to take her walking. She became very pale and did not grow. A year later her father remarried, and the little girl fearfully asked if she might accompany her stepmother shopping. The stepmother cruelly replied, “What would people say if they saw me walking with you? You’re a hunchback and must stay at home!” The little girl often wondered about her hunch and what might be inside of it. Since she was never permitted to go out of doors again, she gradually grew paler and weaker and finally died. When an angel came to take her to heaven, she could not believe that hunchbacks go to heaven. The angel smiled and showed her that there were 2 magnificent white angel wings hidden in her hunch.
Leander was a pen name for none other than Richard von Volkmann (Fig. 9). Remember Halsted said in his letter to Welch: “and as you know, also a poet.” Volkmann was the most beloved citizen of Halle and Germany. Wilhelm I knighted him and changed his name to “von” Volkmann. Following his death at age 59, the turnout for his funeral was said to be the largest ever held in Halle. Early in the 20th century, the Queen of England at the time, Alexandra, was so taken by Dickens’ story A Christmas Carol and the plight of Bob Cratchet and Tiny Tim that she raised money to open crippled children’s hospitals (Figs. 10, 11). The stories of Dickens and Volkmann changed the hearts of men forever. If it were not for the immortal characters they created—Volkmann’s little girl with Pott disease who had 2 white angel wings hidden in her hunch, or Dickens’ Tiny Tim and Bob Cratchet— we might well still be hiding crippled children today instead of helping them towards a chance at a happy and meaningful life. Today, children’s hospitals exist around the world, supported by local Scrooge equivalents who, through their magnificent philanthropy, provide a safe harbor for today’s Bob Cratchet parents and their Tiny Tim. Medicine has always been an innately humanistic science. These children and their parents prove it to me every day. Thank you, and Merry Christmas to all.
SELECTED READINGS
These stories were allegories, not just fairy tales. They were commentaries on the times and changed public opinion about the disabled. By the way, Richard
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1. Carter AJ. A Christmas carol: Charles Dickens and the birth of orthopaedics. J Royal Soc Med. 1993;86(1):45e46. 2. Littler JW. Volkmann’s contracture. In Converse JM, ed. Reconstructive Plastic Surgery, vol. 6. 2nd ed. Philadelphia, PA: WB. Saunders Co, 1977: 3130e3151. 3. Ragland R 3rd, Moukoko D, Ezaki M, Carter PR, Mills J. Forearm compartment syndrome in the newborn: report of 24 cases. J Hand Surg Am. 2005;30(5):997e1003.
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