Future Horizons in Medicine
For centuries the art of medicine has been dominated by bumps, bruises, or other symptoms, felt by the patient or discovered by the physician, with eyes ever-magnified by increasingly sophisticated scanning technology: the microscope, the x-ray, and eventually the MRI. But however powerful the machine, the underlying model remained the same. To find the illness, doctors first had to look for the symptom. To diagnose the cancer, they had to see the tumor. To find a drug, they had to undergo a long, costly, and laborious process of trial and error, trying millions of natural compounds on animals to find one that seemed to work.
This approach to medicine may be coming to an end. As drug discovery becomes an information-based science, fueled by rapid increases in computer processing power and the marriage of test tubes with microchips, we are transforming the way we diagnose and treat many of the worst human diseases. New drugs currently in clinical trials are no longer scattershot one-size-fits-all affairs, but carefully targeted to the molecular fingerprints of specific diseases. Some of these drugs are even targeted to a patient’s unique DNA profile. In a breathtaking paradigm shift, medicine is moving from the species level—the ingrained assumption that drugs and diseases work the same in all human beings—to the individual level, unlocking new healing possibilities in the minute differences between seemingly similar diseases and their individual victims. The result will be a new age of medical therapy, dominated not by cell, tissue, and organ replacements but by early diagnosis and individualized drug treatments.
Union of Biology and silicon.
To understand this new scientific paradigm, first consider how it is changing the way doctors diagnose disease. In conventional medicine, diagnosis remains mostly the art of neglecting remote dangers in favor of likelier ones. Diagnostic tools are often too expensive or too inaccurate to be deployed widely. But in the near future, diagnostic gene chips will rely not on spying crude symptoms but detecting the underlying molecular processes that trigger disease in the weeks, months, or years before the patient feels a twinge.
DNA chips are elegantly simple in concept: thin wafers of glass or plastic embedded with strips of DNA rather than, like silicon chips, tiny transistors. They exploit the natural tendency of double-stranded DNA molecules to bind with their complementary partners, in a process called hybridization. Once researchers have identified a particular strip of DNA within a virus or bacteria or genetic disease, that strip can be used to track down a matching strand from a patient’s blood sample or biopsy specimen. Dozens, even hundreds of potentially offending pathogens, genetic diseases, or other ailments can be diagnosed on the surface of a single chip—at a cost of hundreds or sometimes a few thousand dollars for each chip, although prices of these new diagnostic tools are dropping dramatically.
The first step is to fix a single strand of a known DNA sequence (or hundreds of such known disease-causing sequences) to a chip so that it can be used to search and bind to a complementary strand found in the patient’s own tissue. In hours, a remarkable feat of pattern matching occurs. Genes from the blood sample are allowed to bind to their complementary probes on the silicon surface. Then the entire chip is placed in an analyzer that can read the patterns of gene binding and transfer the information directly into computers capable of interpreting the results.
Last summer, scientists at the National Cancer Institute used gene chips to isolate DNA sequences that can differentiate among pediatric cancers called “blue cell tumors,” even in instances where the best-trained pathologists, peering through powerful microscopes, were once stumped. Previously, many of these tumors were considered to be one disease. But the gene chips discovered important distinctions among these similar tumors, revealing that doctors were actually dealing with several distinct cancers. More than a simple clinical breakthrough, it has led to a dramatic discovery: the criteria doctors are currently using to classify different cancers are not accurate.
This is likely to hold true for a myriad of common cancers. Doctors are currently treating most cancers in a uniform fashion. For example, everyone with pancreatic cancer or liver cancer ends up getting similar treatments, even though genetic markers are beginning to tell us that these cancers really come in a wide variety, each requiring its own unique approach. This is perhaps one reason why most cancer treatments usually leave a significant number of people behind, often with more than half (and in the case of pancreatic cancer, 90 percent) failing standard treatments.
All of these innovations, from targeted medicines to precision diagnostics on a chip, represent the new paradigm that medicine is entering as biology meets silicon. The history of medical progress has been the history of moving from surface to cause, from symptoms to underlying processes. Hippocrates and his fellow physicians probably killed as many patients as they saved with “cures” that ranged from leeches to arsenic. With the Renaissance came anatomy; anatomy begat physiology, and medicine for the first time moved towards science. Germ theory marked the next great leap from surface symptom to underlying process. A whole class of deadly diseases—typhus, whooping cough, measles, malaria—could be cured or prevented, because for the first time we understood their cause.
Understanding the body’s internal disease process took longer. In the twentieth century, biologists gained some understanding into what genes actually do: they make proteins. Proteins consist of strings of different amino acids. Scientists in labs can construct an almost infinite variety, but nature, it turns out, makes just 20 different kinds. All the millions of different proteins on Earth are compounded from that basic amino acid set, just as all 228,000 words in the Oxford English Dictionary are compounded from 26 letters.
The task of genes is to make sure that amino acids line up in the right order to produce the right protein. In biology, the right protein is everything. Cell membranes consist of proteins and fats. Fingernails, hair, and muscles are proteins. Proteins function as hormones, antibodies, or enzymes. They form the body’s cellular structure, direct the metabolism, carry cellular messages, and form defensive forces.
In 1953, with the discovery of the structure of DNA, the scientific basis for investigating how proteins cause and cure disease was finally at hand. But research on the cellular pathways to disease was characterized by a doomed reductionism: for years the big picture of how different genes interacted with different proteins to produce different symptoms was ignored, largely because scientists lacked the processing power to generate and analyze the huge volumes of information necessary to perform this task.
The merger of medicine and microchip is in one sense only natural. DNA can be thought of as a three-billion-year-old Fortran code easily transduced into bits of data, captured in databases, and analyzed with sophisticated software. But until recently, the body’s digital code was just too complex to crack. The true potential of emerging genetic knowledge remained locked in a box of complexity, awaiting the development of a sufficiently advanced information technology. The key was abundant processing power to generate and manage huge data sets linking gene sequences to body functions and dysfunctions.
In the simplest cases, such as sickle-cell anemia, diseases can be linked to a single gene. But most important diseases are far more complicated, determined by multiple gene markers at many different locations. Finding the culprits requires large sample sets and powerful software algorithms that can hunt down genetic patterns among millions of data points, tracking which ones seem to be associated with disease. Only now is such technology becoming available.
George Weinstock, co-director of the human genome center at Baylor College of Medicine, believes this new computer-assisted ability to crack the DNA code is as significant as the microscope. “Before the microscope they never realized the structure of cells and the presence of disease-causing microbes in water,” Weinstock says. “The gene sequence will likewise have an impact over a number of centuries.” Our growing mastery of genetics has finally met up with an information technology sufficiently advanced to exploit it for commercial medical purposes. These new technologies are being adopted throughout the drug industry, but they are being most effectively implemented in the R&D infrastructure of some of the smaller biotechnology companies, which are gaining a competitive advantage by embracing new tools.
Protein in a Haystack
One of the best illustrations of how computational tools are redefining drug discovery is a new process called rational drug design. Traditionally, pharmaceutical companies discovered leads on new drugs by a process virtually akin to blind luck. Most drugs work by binding to proteins and altering their function in some small way. So the first step en route to new “miracle cures” was finding a molecule that binds to a protein. In the old “wet lab” model, that required mixing millions of different chemicals and hoping one of them stuck. Pharmaceutical companies sank billions of dollars into technology upgrades that made this antiquated model work a little better—with automated systems that helped scientists synthesize and survey thousands of chemical compounds a week, hoping to stumble upon a few hits. But most of these sticky compounds still failed the minute they left the test tube.
The emerging paradigm marries computational tools with biology to develop a different model: deploying information technology to design drug-like qualities right into the molecules from the very start. Instead of mixing compounds in test tubes at random, this process begins by teaching computers what the molecular structure of an effective drug ought to look like, and what molecular structures it ought to avoid. For example, certain molecular structures, promising in the test-tube, bind to sites in the liver’s P450 system, where enzymes break them down, leading to poor absorption in the body. The idea behind rational drug design is to construct medicines atom by atom, fitting drugs like finely cut jewels onto settings made of protein.
One of the first drugs to be designed in this way is the AIDS medication Agenerase, from the powerful class of drugs known as protease inhibitors. The first step in the development of Agenerase, which the FDA approved in 1999, was to create a three-dimensional picture of a key enzyme that HIV uses to reproduce (i.e., a protease) in a process called x-ray crystallography. In this process, scientists crystallize the virus particle and then aim radiation at it. Computers capture the radiation as it bounces off the crystal, and reconstruct the diffracted signals into a three-dimensional picture of the enzyme.
On computer screens, the protease looks like a mass of sticks and balls. Computers home in on the parts of the enzyme where drugs can bind. The site contains about 10 different regions on which different drugs could be hooked. Most drugs work, as described above, by binding in some way to a protein involved in causing a disease. For example, many cancers are caused by the overabundance of mutant forms of naturally occurring proteins that instruct certain cells to divide continuously even when they should turn themselves off. Many new cancer drugs work by binding and disabling these mutant proteins.
In rational drug design, computers screen different drugs against a protein’s binding site, digitally docking them with the protease to see what fits. One early version of the drug Agenerase seemed to fit its pocket well, but part of the molecule hung out of the edge, meaning the drug was easily knocked around and became dislodged. So scientists involved in the development of Agenerase snipped off three carbon atoms in a key area of the drug, which gave it a smaller profile and thus a snugger fit.
The Coming of Genetic Medicine
The knowledge made available by precise diagnostics and targeted drugs is helping to shape another great paradigm shift in medicine: from species to individual. Medicine has been based on the largely unexamined assumption that disease process and treatment is species-specific. People all have the same basic biological processes. What cures disease in one human being will cure the same disease in all other human beings. But in reality, human bodies differ, and so do individual responses to drugs and other treatments.
For example, researchers recently discovered that one likely reason African-Americans die more often from heart attacks is that ACE inhibitors, one of the drugs given to heart attack patients, are much less effective in people of African than European ancestry. Computational tools give scientists the ability to collect and analyze such ethnic, family, and individual genetic variations. “Medicine never really focused on our differences,” explains Huntington Willard, President of the American Society of Human Genetics. “Our hearts are all different and the differences have implications for function and performance. Sequence knowledge will change doctors’ perspective to providing care for the individual.”
“By learning about what makes each patient’s tumor grow, what makes it spread or not spread, hopefully you could tailor therapies to the individual patient rather than use a one-size-fits-all kind of approach,” said Dr. Paul Meltzer, a senior investigator at the National Human Genome Research Institute. Researchers at one Cambridge-based company developed a test called Melastatin which detects a protein that accurately predicts whether a melanoma will recur. The next step is to come up with a drug that turns on the proteins that turn on Melastatin, blocking skin cancer from metastasizing. It’s not that the patient with the Melastatin gene would lose it. The gene would still be there, but the drug blocks the body from turning on the disease process.
This kind of research is already having an impact on clinical practice. Melanoma cases that slip through today’s antiquated screening processes account by themselves for six percent of the money awarded each year in malpractice suits. Scientists at the University of California, San Francisco, recently developed a test which detects characteristic chromosomal abnormalities, allowing doctors to diagnose melanoma even when normal tissue biopsies have pathologists stumped. Another strategy focuses on using receptors on the surface of the cancer cells as targets for drugs.
In short, as diagnostic tools and drug design techniques are transformed by the merger of medicine and microchip, so are the drugs that scientists are creating. Consider the advent of a large new class of drugs referred to as monoclonal antibodies. In 1975, two future Nobel Prize-winning scientists, Georges Köhler and César Milstein, stimulated an immune reaction in mice, cloned the antibody-rich immune cells, and then harvested the antibodies. Such refined antibodies are called monoclonal because, unlike the antibody cocktails our bodies create, they all react in a uniform way to a particular “antigen,” a piece of protein or carbohydrate on the surface of an “invader” cell. In many ways, monoclonal antibodies represent the low hanging fruit of genomics; they are among the first genetically engineered medical products and the leading edge of the new, personalized, and targeted treatments.
The concept of using an antibody as a drug is fairly simple. The first step is to identify the antigen marker on the surface of a disease-causing cell. In the case of cancer, researchers identify a protein expressed on the surface of every cancer cell and then engineer an antibody that is programmed to recognize and attach itself to that protein. Once attached to its target cell, monoclonal antibodies can be engineered to disable a protein, flag a diseased cell for destruction by a person’s own immune system, or kill a cell outright by interfering with its growth or by punching holes into it. There are six therapeutic monoclonal antibodies currently approved and marketed for several different diseases in the United States. More than 15 percent of the hundreds of drugs in clinical trials in the United States are antibodies.
Monoclonal antibodies can be designed to disable cell signals that, for example, tell a particular system how to go awry or carry the messages that instruct cancer cells how to grow. Alternatively, antibodies can be used as transport vehicles to identify cancer cells and deliver toxic payloads. Like tiny divining rods, these drugs hunt down only diseased cells, avoiding the shotgun approach of past chemotherapies. Best of all, scientists can make lots of them.
This is not, however, an overnight success story: researchers spent more than 20 years doing the underlying work that led to the arrival of monoclonal antibodies in the marketplace. Monoclonal antibodies were first produced in mice because it was comparatively easy to do so. But the drugs triggered rejection from human patients’ immune systems, which recognized them as foreign proteins. The result was that patients who received them often suffered life-threatening immune reactions. By the 1980s, researchers had begun to humanize the antibodies by replacing parts of the mouse antibody with human antibody, which ensured that the engineered antibodies would be better tolerated in humans. In effect, scientists re-engineered the antibodies to look more familiar by replacing at least half of the mouse DNA with human DNA.
The first of these “humanized antibodies” to reach the market, in 1994, was ReoPro, a clot-busting drug that reduces the risk of death during angioplasty procedures by 57 percent. ReoPro—which is half-mouse, half-human—was low-tech by current standards. The breast cancer drug Herceptin, which came to market four years later, is five percent mouse, 95 percent human. Better versions are already on the way.
The View from Industry
Some analysts point to the declining number of novel drugs submitted for approval by the Food and Drug Administration over the past two years as evidence that this new technology is not yielding the types of breakthroughs once envisioned, despite increasing investments in research and development. According to the pharmaceutical industry’s calculation, its R&D investment doubled to an estimated $30.5 billion in 2001. Despite the increased effort, output as measured by the number of new drugs and biologics approved or submitted for approval has been steady or declining across almost every major therapeutic area. Meanwhile, if you look at the trend over the next five years, it is not likely to change dramatically. If the technology being brought to the task of drug development is so impressive, why haven’t these innovations resulted in more new drugs being developed and approved? The answer is not so much technological as it is structural: some revolutions take time, and old ways of doing things die hard.
Nevertheless, the drug industry has been gradually reorienting itself around this new drug-discovery technology, and it has been adapting itself to the realization that its business model is to create medicine, not simply to identify new targets or pathways at which to aim new drugs. While the generation of these new targets is bearing fruit, some of these targets will also prove dead ends once drugs are tested in people. This is not because the science was wrong, but because humans are more than the single pathway that a particular drug is created to target. People are complete with systems that can not only override medical interventions but can cause unintended consequences. If you look at genomics and proteomics as a way of finding novel ways of attacking a disease, the next step is to build an understanding into the drug discovery process of not only what target or biochemical pathway a new compound will attack but what role this molecular intervention will have in the entire organism. This is the technical challenge that the industry is grappling with and why it is at a watershed moment in the evolution of its development skills.
The Real Future of Medicine
Taken together, the marriage of biology and silicon and the shift from species-based to individualized therapy will change the face of medicine forever. Traditional human efforts to treat disease are being empowered with digital tools that annotate life with silicon technology. The enormous material effort to find symptoms is being replaced by a combined genetic and artificial intelligence that knows where to look and how to find problems before we do. In the new medical paradigm, disease will be diagnosed before it is made fully manifest. Highly targeted drugs will be used to intervene before organs are ravaged or tissue is destroyed.
This new ability to diagnose and treat certain diseases early, from infectious agents like hepatitis C to degenerative ailments such as Alzheimer’s and Parkinson’s, may obviate the need for the types of tissue, organ, or stem cell therapies that often attract the most public attention. Moving from wet lab to computer, from random to rational drug design, from species biology to the individual unique DNA profile, companies adopting the in silico paradigm are unlocking the long-hyped promise of genomic medicine, making targeted drugs and diagnosis a reality and drug development faster, cheaper, and better.
In the future, a supercomputer sitting in an air-conditioned room will work day and night, crunching billions of bits of information to design new drugs. Multiplying at the speed of Moore’s Law, which predicts that computer processing power doubles every three years, this drug discovery machine will never need to rest or ask for higher pension payments. It will shape how we use the abundance of genomic information that we are uncovering and will be the deciding factor for the success of medicine in an age of digitally driven research.
Of course, there are reasons to question whether this new medical revolution will come to pass, and there are many things that could go wrong: Regulatory procedures need to keep pace with technological change and government agencies need to create frameworks for evaluating drugs that look and behave differently than previous medicines. The industry needs to maintain its financial footing to fund the new research. And, of course, many parts of this new technology still need to be validated in the clinical setting. Scientists still need to prove that their cool new tools can also make important new medicines.
But if one had to guess where the future of medicine really lies, it is in DNA chips, supercomputers, and new drugs, not embryo research, tissue transplants, or stem cells. It is time for our public debate to pay more attention to this fact, since a medical and technological revolution of this significance is sure to have lasting political, economic, and social consequences.
Tuesday, December 29, 2009
Thursday, December 10, 2009
Jakob Lewin Joseph -The Father of modern aesthetic surgery
The history of modern facial plastic surgery began more than one hundred years ago, when a few men independently began to explore a new surgical frontier of reconstructive and functional repairs that also improved appearance. As one might expect, they had trained in different specialties, including otolaryngology, and so brought to the work somewhat different knowledge and skills. By observing one another - there were few formal training programs at the time - these surgeons rapidly improved and expanded the procedures each could perform. They interacted with each other, observed one another and were soon able to both expand the repertoire of their work and the finesse in it. These were the newer generation of Plastic and Reconstructive Surgeons.
Out of this group of dedicated new generation specialists one surgeon in particular stands out from this time. Jakob Lewin (Jacques) Joseph was born on 6 th September 1865 in Kφnigsberg. He was the third child of the Rabbi Israel Joseph and his wife Sara. From 1885 to 1889 he studied medicine at the Friedrich-Wilhelm University in Berlin. He completed his studies in 1889 and obtained his doctorate in 1890 in Leipzig.
The humble beginning.
After the stipulated period of his medical licensing and medical practical training in 1892, Joseph became a general practitioner in the district of Berlin-Mitte. Though he did exceptionally well in his practice a desire to specialize always kindled inside his heart. In 1892 he applied to the University Polyclinic to work in Orthopaedic Surgery and was selected for training. The Unit was headed by Professor Julius Wolff (1836-1902) - a recognized surgeon, called 'Knochenwolff' (Bonewolf) by the Berlin populace. Dr. Wolff, who is considered one of the pioneers of modern orthopaedics, liked young Joseph and took keen interest in his training. This relationship of mutual respect between the teacher and the taught however came to an abrupt end one day when Joseph, true to his nature, chose to think out of the box. Joseph undertook to surgically correct the ears of a ten-year-old boy, who refused to attend school because he suffered such ridicule from classmates for his large, protruding ears, which were too large and stuck out much too far ('donkey's ears'). Though Joseph was not sure whether such surgery had ever been performed in the past, he felt it was possible and after careful planning he operated upon the child successfully. He took his success story to the Berlin Medical Society and was highly appreciated by his peers but this show of originality also cost him his job with Wolff, who felt that Joseph had risked the reputation of Wolff's clinic by performing the maverick procedure. Thus, after training with Wolff for 4 years, a training which if completed could have earned him a University degree, in 1896 Joseph was back to square one and he returned to private practice.
Conceptualizing Aesthetic Surgery
28-year-old man whose extremely large nose caused him such embarrassment that he could not bear to appear in public. The man had heard about the ear reduction Joseph had performed and wondered if he might also be able to reduce the size of a nose.
Joseph too felt that this was possible, but as he had never thought about it he thought should study the anatomy of the nose further. He practiced his nose reduction surgery on cadavers and later on operated on the patient, much to the latter's satisfaction. Again he reported his success in May 1898 to the Berlin Medical Society, but this time with the case report he propounded a landmark theory.
Joseph had been developing this theory for quite some time, and it postulated that the psychological aspect of aesthetic surgery was as important as its physical success. According to the theory, a person whose looks caused social or economic disadvantage was as severely afflicted as a person who suffered from a debilitating disease. Now considering that the year was 1898, this theory was way too radical for all those purists of his era as it was outside the mainstream of the then serious curative medicine. The "serious" surgeons were in a habit of scorning the use of their skills for petty cosmetic purposes. It was also contrary to Joseph's Prussian background, which sternly admonished one to make do with what life dealt. Joseph called the desire to look normal "anti-dysplasia" not vanity. Hence for his time and society his was a very bold step towards authenticating aesthetic surgery. So there is little doubt why the courageous Jacques Joseph today is considered the father of modern aesthetic surgery!
The First World War
When the First World War started Joseph, by then a reputed facial plastic surgeon amongst both his peers and his patients, was entrusted with responsibility of staff physician in reserve. This was a whole new war with newer and more lethal ammunitions that would inflict injuries of a frequency and severity unknown until then. Jacques Joseph, rose up to the newer challenges and fired by his patriotic sense of service to the German fatherland increased the number and extent of his operations to the most extreme degree. As it was expected because of his grasp of the fundamentals of facial surgery, he worked with extraordinary success in the field of reconstructive surgery as well, attaining quite spectacular successes. It is said that the 'Supreme Commander' Wilhelm II himself came to notice him, or at least Joseph was brought to his attention for his extraordinary services.
Joseph had no desire to chair any department but in 1915 the Emperor personally offered him the Chair for Plastic Surgery at the Charitι hospital - but only under the condition that he, Joseph, convert to Christianity. Joseph refused. He was proud of his Jewish heritage but why the Supreme Commander made this extraordinary offer is not very clear. Did he want to quiet his conscience by offering the wounded soldiers, some of whom were horribly disfigured, the 'best facial surgeon in the world' in such an exposed position? Then again did such scruples and sentimentality come naturally to Wilhelm II perhaps no one would ever know!
On 2 June 1916 the Prussian Ministry for Ecclesiastical and Educational Matters (Ministerium für geistliche und Unterrichtsangelegenheiten) gave Joseph a Department of Facial Plastic Surgery, which was opened at the Ear and Nose Clinic of the Charitι, then headed by Adolf Passow (1859-1926). This was a post which carried no remunerations for Joseph. Three years later in 1919 he was named professor - but this time not by the Emperor, and without any impossible religion conversion conditions being attached. During his time at the Charitι he accomplished great reconstructive feats using regional or forehead and upper arm flaps. He also performed plastic surgery on one hand, and free cartilage and bone transplants on the other. His bulk of work remained facial plastic surgery and he succeeded in reconstructing faces even in cases of extensive injury. His Wartime services earned him the Iron Cross.
Nasen Joseph in Private Practice
In 1922 the Army had no further need for Joseph's services as the war victims were mostly well managed or dead by then. They stopped financing his department and Joseph returned to his practice. This proved to be a boon in disguise and he dedicated himself increasingly to corrective and aesthetic surgery. In 1922 a report on Jacques Joseph was published by "raving reporter" Egon Erwin Kisch. A much reputed and respected surgeon by now, most of his work was corrective procedures for the nose and 'hanging cheeks', as well as breast operations. He was known as 'Nasen-Joseph' (Nose-Joseph) or 'Noseph' by the Berliners, such was the popularity of his rhinoplasties! He attracted the newer generation of Plastic surgeons like a magnet and his Unit almost always had visitors from both Europe and America. One of his famous assistants Gustave Aufricht, later went to New York and contributed greatly to the spreading of the Joseph procedure in the USA. Yet another assistant was the American Joseph Safian who reported that up to six doctors from home and abroad were permitted to observe the operations from a platform at the foot of the operating table, for an appropriate payment. They were not permitted to ask questions and there were no explanations and comments on the operative procedure. Was it because Joseph disliked being questioned or because his private patients were being operated upon under local anaesthesia and he did not want any discussion in their presence, giving them the impression that nothing less than 100% of his attention was towards their wellbeing? No one will ever know! To the observers too, whether this exposure was discouraging rather than encouraging and informative, again one will never be able to fathom.
Those who did not know Jacques Joseph personally had an impression that he was a rather bad-tempered and un-cordial teacher. But those who did manage to break through his tough exterior valued his warmth of heart and his humour, and appreciated that there was hidden inside a deeply sensitive man, fascinated by a classical ideal of beauty, who, as a physician, was honestly fond of his unfortunately disfigured patients. Moving testimonials sent to his family in the 1970's and 80's from his disfigured patients and War Veterans long after he died are ample proof of the goodness of his heart and sensitivity of his soul!
Both his surgery and his compassionate view on the subject of aesthetic improvement made him immensely popular and patients gravitated to him from all parts of Europe. This popularity and his consistent good results made him the premier facial plastic surgeon in Europe of his time. Perhaps his greatest contribution is the fact that he systematized corrective, reconstructive and aesthetic rhinoplasty and defined it anew with regard to its aims and techniques. For the distinction of the founder of modern nasal surgery he has perhaps no competition. Both his surgery and his published work have ultimately earned him a place in history as the father of modern facial plastic surgery.
Joseph would have many famous admirers in days to follow, amongst them was Hugo Ganzer, himself an extremely experienced exponent of the Plastic Surgical skills. He was of the view that Joseph was a talented surgeon, who had not only excellent surgical know-how, but also that artistic feeling for form at his disposal. Erich Lexer (1867-1937) too was a fan of his artistic temper and said that everyone who wishes to carry out cosmetic operations must have it. While Joseph had mastered certain chronological operative steps while operating on an injured face or a deformed nose, he would not shy away from newer designs and follow un-trodden roads if the situation demanded. Every defect or disfigurement was thoroughly analyzed before surgery much like the planning session of today. He would plan every step painstakingly never left anything to intuition during the operation. Such was his mastery on the subject of aesthetic and reconstructive Rhinoplasty that even till date some of his methods and some of his results have not been bettered.
Publications
In 1928 and 1929 the first two sections of Jacques Joseph's book on 'nasal plastic surgery' were published at Curt Kabitzsch Publishers, Wuerzburg / Leipzig. This book, Nasenplastik und sonstige Gesichtsplastik nebst Mammaplastik ('Nasal Plastic Surgery and Other Facial Procedures, and the Plastic Surgery of the Breast') remains Jacques Joseph's major scientific contribution and was completed in 1931. He had over 30 publications to his credit. Through his monumental work Joseph systematized corrective, reconstructive and aesthetic Rhinoplasty in particular and facial plastic surgery in general and redefined them in their aims and techniques. He emphasized that Rhinoplasty was to address both form and function, and authenticated aesthetic surgery as a serious surgical speciality. His book and his work makes him the unchallenged founder of modern Rhinoplasty and one of the most important pioneers of facial plastic surgery. The book, a masterpiece of 842 pages - which according to Joseph is an ' Atlas and textbook'- is a comprehensive survey of both the principles of analysis and operation planning and techniques for patients with extensive facial wounds. It also deals with the functional and aesthetic indications of Rhinoplasty such as crooked, saddle, and hump nose. Corrective, reconstructive and aesthetic nasal surgery all find a mention as does facial surgery for drooping cheeks, plastic surgery of forehead, jaw, lip, cheek, and lid. The surprise pack is an 80-page appendix on reconstructive breast surgery and mammoplasty techniques. With time and continuing research many of his operative techniques and indications, e.g. resectional septum surgery, have been further developed or changed. Nevertheless, his book 'Nasal Plastic Surgery and Other Facial Reconstructive Procedures, With an Appendix on Reconstructive Breast Surgery and Some Other Procedures in the Area of External Plastic Surgery still remains a landmark of such importance that Rhinoplasty and Facial Plastic Surgery can easily be subdivided into a rather gloomy and ill defined era before Jacques Joseph and a better documented and brighter era after his age and time!
A doyen of reconstructive plastic surgery
The basic principles of planning skin flaps, suture techniques or wound treatment which Joseph practiced are relevant even today and ought to be understood and followed by every plastic surgeon. He has in his book emphasized the importance of the exact analysis of a deformity or defect and how it is essential to a clear, pre-operative plan. The diagrams and illustrations depicting the general fundamentals of anatomy, facial proportions, the morbid anatomy of a deformity, what is missing and from where, all help the reader to become a better treatment planner. The didactic structure of his book, which illustrates the various causes of each deformity, guides the reader through specific surgical problem situations and their solutions, often in apparently hopeless cases. The language is simple and gives a clear idea of how the author was thinking and encourages the reader to do the same. What we today refer to as looking at the third dimension of a deformity is very clearly apparent. In fact he adds a fourth dimension too by emphasizing the psychological aspect rather boldly. For example, in the chapter "Grounds for rhinomiosis and the relevance of the operation to everyday living", we read: "One who wishes to be rid of a deformity, who simply wants a 'normal' appearance - wishes to be inconspicuous - should not suffer the odium of vanity."
Not only the author Jacques Joseph but his team of illustrator, photographer and publisher all deserve rich praises for the outstanding publication. The illustrations and pastels are easy to understand, clear and didactically brilliant, making the steps of each procedure and its principles thoroughly comprehensible and enjoyable to read. The balanced and harmonic design, the layout, and the technical excellence visible in the black-and-white photographs all combined can match the best publications of our present day and age. The reprint, using a complex reprographic procedure, is quite comparable in quality to the original and those who possess the book treasure it and pass it on to the next generation with a lot of hope. The original German edition of Joseph's book published in 1931 is a legendary rarity. The English translation by Stanley Milstein was published in 1987. It has 843 pages with 1718 illustrations, some in colour. Bound in Red Morocco leather spine tooled in gold, black cloth boards, and presented in gold-stamped cloth slipcase, it is a collector's dream besides being an outstanding reference book of aesthetic plastic surgery.
Joseph developed many new instruments for his operations, the most well-known being the Joseph's elevator which is still in daily use in operating theatres throughout the world. The book also conveys an understanding of the relationship between operative goal and instrument knowledge. The clever use of novel instruments to suit tricky situations was perhaps his hobby as in his lifetime he designed many of them.
Cultural history of the then Germany
This major work of Jacques Joseph is also an impressive reminder of an important, bygone era of medicine. Joseph's career began in Germany at the time of emperor Wilhelm II, brought him the highest recognition as a doctor and ended sadly under the National Socialists with extreme humiliation. In 1933 the National Socialists came to power in Germany and a catastrophe was in the making. Jewish Germans, who were unanimously labeled as 'dissidents', were in for a hard time, to put it mildly after so many years. Joseph, being an aristocrat and a jewel amongst Berliners had not taken the increasing Brown danger seriously. Little did he realize that despite the greatest recognition and esteem that he enjoyed in the recent past there would follow - nearly overnight - the deepest official disdain. The Gestapo had engaged the steno typist whom Joseph had employed to finish his textbook, and who lived in his house. He spied on him and blackmailed him on the instructions of his Nazi authorities. The authorities would allow Joseph to carry out only few 'special approval procedures'. The wave of violent anti-Semitism of the time took a toll on both his practice and his private life.
In Germany today, the importance of Jacques Joseph and his work has largely fallen into oblivion. This is primarily due to the fact that after his death, his achievements, like those of all other Jewish doctors and scientists during the Nazi era, were ignored. After the war, the first publications and praises about Jacques Joseph came from abroad, particularly from his trainees and peers in the USA, where his wife Eleonore and daughter Bella had emigrated. It is thanks to the publisher Dr. Reinhard Kaden and the luxurious reprint of Jacques Joseph's work that this scientific legacy of a great physician is once again accessible to his cynosures.
Death of an unsung Hero
On 12 th February 1934 Jacques Joseph died of a heart attack in the hallway of his house on his way to his practice. Though theories of death by violence or suicide are mostly unfounded, the fact that his myocardial infarction was precipitated by the repeated mishandlings which Joseph suffered at the hands of Nazi thugs can not be ruled out. The German specialist press, already mostly brought into line by the Nazis by then, took no more notice of Joseph's death. Obituaries appeared only in overseas journals and mourners were mostly his trainees and his peers.
Recognition after death
Jacques Joseph was buried in the Jewish Cemetery in Berlin-Weissensee. After the bombing raid in the Second World War his grave was destroyed and was believed to be no longer identifiable. After much difficult research, it was possible to find the partially buried and overgrown gravestone of Joseph's grave in August 2003. The stone, made of black granite, has since been retrieved, identified and the former inscription deciphered.
Jacques Joseph's gravestone was consecrated on the occasion of the re-erection of his tomb on 17 th October 2004, at the same Jewish Cemetery in Berlin-Weissensee. The unveiling and consecration of the stone was conducted by the eminent rabbi Dr. Andreas Nachama in the presence of some 70 guests from home and abroad. Thus, Jacques Joseph was paid the respect which was denied him at his death . Many private persons and professional associations throughout the world donated generously for the re-erection of his tomb. They included many private citizens and organizations like American Academy of Facial Plastic and Reconstructive Surgery; European Academy of Facial Plastic Surgery; Deutsche Gesellschaft fuer Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie; Norddeutsche Gesellschaft fuer Otorhinolaryngologie und zervikofaziale Chirurgie; Oto-Laryngologische Gesellschaft zu Berlin; Vereinigung der Westdeutschen Hals-Nasen-Ohren-Aerzte von 1897. The stone, though refurbished, was purposely left unchanged despite the bomb-damage it sustained to reflect the eventful history of the grave. On the front is the new inscription on polished Swedish granite but the back of the stone has been left just as it was when it was found broken, overgrown, damaged, with fragments of inscription. It has been proposed to the Senate that the tomb receives recognition as an official Berlin memorial.
When the International Federation of Plastic. Reconstructive and Aesthetic Surgery (IPRAS), met in Berlin for its 14 th . Congress in June 2007, Jacques Joseph was honoured in its Inaugural Function. Prof. Robert Goldwyn unveiled his bust statue and all the dignitaries spoke very highly of him and his contributions to enrich our science, which we all practice and love so passionately.
Out of this group of dedicated new generation specialists one surgeon in particular stands out from this time. Jakob Lewin (Jacques) Joseph was born on 6 th September 1865 in Kφnigsberg. He was the third child of the Rabbi Israel Joseph and his wife Sara. From 1885 to 1889 he studied medicine at the Friedrich-Wilhelm University in Berlin. He completed his studies in 1889 and obtained his doctorate in 1890 in Leipzig.
The humble beginning.
After the stipulated period of his medical licensing and medical practical training in 1892, Joseph became a general practitioner in the district of Berlin-Mitte. Though he did exceptionally well in his practice a desire to specialize always kindled inside his heart. In 1892 he applied to the University Polyclinic to work in Orthopaedic Surgery and was selected for training. The Unit was headed by Professor Julius Wolff (1836-1902) - a recognized surgeon, called 'Knochenwolff' (Bonewolf) by the Berlin populace. Dr. Wolff, who is considered one of the pioneers of modern orthopaedics, liked young Joseph and took keen interest in his training. This relationship of mutual respect between the teacher and the taught however came to an abrupt end one day when Joseph, true to his nature, chose to think out of the box. Joseph undertook to surgically correct the ears of a ten-year-old boy, who refused to attend school because he suffered such ridicule from classmates for his large, protruding ears, which were too large and stuck out much too far ('donkey's ears'). Though Joseph was not sure whether such surgery had ever been performed in the past, he felt it was possible and after careful planning he operated upon the child successfully. He took his success story to the Berlin Medical Society and was highly appreciated by his peers but this show of originality also cost him his job with Wolff, who felt that Joseph had risked the reputation of Wolff's clinic by performing the maverick procedure. Thus, after training with Wolff for 4 years, a training which if completed could have earned him a University degree, in 1896 Joseph was back to square one and he returned to private practice.
Conceptualizing Aesthetic Surgery
28-year-old man whose extremely large nose caused him such embarrassment that he could not bear to appear in public. The man had heard about the ear reduction Joseph had performed and wondered if he might also be able to reduce the size of a nose.
Joseph too felt that this was possible, but as he had never thought about it he thought should study the anatomy of the nose further. He practiced his nose reduction surgery on cadavers and later on operated on the patient, much to the latter's satisfaction. Again he reported his success in May 1898 to the Berlin Medical Society, but this time with the case report he propounded a landmark theory.
Joseph had been developing this theory for quite some time, and it postulated that the psychological aspect of aesthetic surgery was as important as its physical success. According to the theory, a person whose looks caused social or economic disadvantage was as severely afflicted as a person who suffered from a debilitating disease. Now considering that the year was 1898, this theory was way too radical for all those purists of his era as it was outside the mainstream of the then serious curative medicine. The "serious" surgeons were in a habit of scorning the use of their skills for petty cosmetic purposes. It was also contrary to Joseph's Prussian background, which sternly admonished one to make do with what life dealt. Joseph called the desire to look normal "anti-dysplasia" not vanity. Hence for his time and society his was a very bold step towards authenticating aesthetic surgery. So there is little doubt why the courageous Jacques Joseph today is considered the father of modern aesthetic surgery!
The First World War
When the First World War started Joseph, by then a reputed facial plastic surgeon amongst both his peers and his patients, was entrusted with responsibility of staff physician in reserve. This was a whole new war with newer and more lethal ammunitions that would inflict injuries of a frequency and severity unknown until then. Jacques Joseph, rose up to the newer challenges and fired by his patriotic sense of service to the German fatherland increased the number and extent of his operations to the most extreme degree. As it was expected because of his grasp of the fundamentals of facial surgery, he worked with extraordinary success in the field of reconstructive surgery as well, attaining quite spectacular successes. It is said that the 'Supreme Commander' Wilhelm II himself came to notice him, or at least Joseph was brought to his attention for his extraordinary services.
Joseph had no desire to chair any department but in 1915 the Emperor personally offered him the Chair for Plastic Surgery at the Charitι hospital - but only under the condition that he, Joseph, convert to Christianity. Joseph refused. He was proud of his Jewish heritage but why the Supreme Commander made this extraordinary offer is not very clear. Did he want to quiet his conscience by offering the wounded soldiers, some of whom were horribly disfigured, the 'best facial surgeon in the world' in such an exposed position? Then again did such scruples and sentimentality come naturally to Wilhelm II perhaps no one would ever know!
On 2 June 1916 the Prussian Ministry for Ecclesiastical and Educational Matters (Ministerium für geistliche und Unterrichtsangelegenheiten) gave Joseph a Department of Facial Plastic Surgery, which was opened at the Ear and Nose Clinic of the Charitι, then headed by Adolf Passow (1859-1926). This was a post which carried no remunerations for Joseph. Three years later in 1919 he was named professor - but this time not by the Emperor, and without any impossible religion conversion conditions being attached. During his time at the Charitι he accomplished great reconstructive feats using regional or forehead and upper arm flaps. He also performed plastic surgery on one hand, and free cartilage and bone transplants on the other. His bulk of work remained facial plastic surgery and he succeeded in reconstructing faces even in cases of extensive injury. His Wartime services earned him the Iron Cross.
Nasen Joseph in Private Practice
In 1922 the Army had no further need for Joseph's services as the war victims were mostly well managed or dead by then. They stopped financing his department and Joseph returned to his practice. This proved to be a boon in disguise and he dedicated himself increasingly to corrective and aesthetic surgery. In 1922 a report on Jacques Joseph was published by "raving reporter" Egon Erwin Kisch. A much reputed and respected surgeon by now, most of his work was corrective procedures for the nose and 'hanging cheeks', as well as breast operations. He was known as 'Nasen-Joseph' (Nose-Joseph) or 'Noseph' by the Berliners, such was the popularity of his rhinoplasties! He attracted the newer generation of Plastic surgeons like a magnet and his Unit almost always had visitors from both Europe and America. One of his famous assistants Gustave Aufricht, later went to New York and contributed greatly to the spreading of the Joseph procedure in the USA. Yet another assistant was the American Joseph Safian who reported that up to six doctors from home and abroad were permitted to observe the operations from a platform at the foot of the operating table, for an appropriate payment. They were not permitted to ask questions and there were no explanations and comments on the operative procedure. Was it because Joseph disliked being questioned or because his private patients were being operated upon under local anaesthesia and he did not want any discussion in their presence, giving them the impression that nothing less than 100% of his attention was towards their wellbeing? No one will ever know! To the observers too, whether this exposure was discouraging rather than encouraging and informative, again one will never be able to fathom.
Those who did not know Jacques Joseph personally had an impression that he was a rather bad-tempered and un-cordial teacher. But those who did manage to break through his tough exterior valued his warmth of heart and his humour, and appreciated that there was hidden inside a deeply sensitive man, fascinated by a classical ideal of beauty, who, as a physician, was honestly fond of his unfortunately disfigured patients. Moving testimonials sent to his family in the 1970's and 80's from his disfigured patients and War Veterans long after he died are ample proof of the goodness of his heart and sensitivity of his soul!
Both his surgery and his compassionate view on the subject of aesthetic improvement made him immensely popular and patients gravitated to him from all parts of Europe. This popularity and his consistent good results made him the premier facial plastic surgeon in Europe of his time. Perhaps his greatest contribution is the fact that he systematized corrective, reconstructive and aesthetic rhinoplasty and defined it anew with regard to its aims and techniques. For the distinction of the founder of modern nasal surgery he has perhaps no competition. Both his surgery and his published work have ultimately earned him a place in history as the father of modern facial plastic surgery.
Joseph would have many famous admirers in days to follow, amongst them was Hugo Ganzer, himself an extremely experienced exponent of the Plastic Surgical skills. He was of the view that Joseph was a talented surgeon, who had not only excellent surgical know-how, but also that artistic feeling for form at his disposal. Erich Lexer (1867-1937) too was a fan of his artistic temper and said that everyone who wishes to carry out cosmetic operations must have it. While Joseph had mastered certain chronological operative steps while operating on an injured face or a deformed nose, he would not shy away from newer designs and follow un-trodden roads if the situation demanded. Every defect or disfigurement was thoroughly analyzed before surgery much like the planning session of today. He would plan every step painstakingly never left anything to intuition during the operation. Such was his mastery on the subject of aesthetic and reconstructive Rhinoplasty that even till date some of his methods and some of his results have not been bettered.
Publications
In 1928 and 1929 the first two sections of Jacques Joseph's book on 'nasal plastic surgery' were published at Curt Kabitzsch Publishers, Wuerzburg / Leipzig. This book, Nasenplastik und sonstige Gesichtsplastik nebst Mammaplastik ('Nasal Plastic Surgery and Other Facial Procedures, and the Plastic Surgery of the Breast') remains Jacques Joseph's major scientific contribution and was completed in 1931. He had over 30 publications to his credit. Through his monumental work Joseph systematized corrective, reconstructive and aesthetic Rhinoplasty in particular and facial plastic surgery in general and redefined them in their aims and techniques. He emphasized that Rhinoplasty was to address both form and function, and authenticated aesthetic surgery as a serious surgical speciality. His book and his work makes him the unchallenged founder of modern Rhinoplasty and one of the most important pioneers of facial plastic surgery. The book, a masterpiece of 842 pages - which according to Joseph is an ' Atlas and textbook'- is a comprehensive survey of both the principles of analysis and operation planning and techniques for patients with extensive facial wounds. It also deals with the functional and aesthetic indications of Rhinoplasty such as crooked, saddle, and hump nose. Corrective, reconstructive and aesthetic nasal surgery all find a mention as does facial surgery for drooping cheeks, plastic surgery of forehead, jaw, lip, cheek, and lid. The surprise pack is an 80-page appendix on reconstructive breast surgery and mammoplasty techniques. With time and continuing research many of his operative techniques and indications, e.g. resectional septum surgery, have been further developed or changed. Nevertheless, his book 'Nasal Plastic Surgery and Other Facial Reconstructive Procedures, With an Appendix on Reconstructive Breast Surgery and Some Other Procedures in the Area of External Plastic Surgery still remains a landmark of such importance that Rhinoplasty and Facial Plastic Surgery can easily be subdivided into a rather gloomy and ill defined era before Jacques Joseph and a better documented and brighter era after his age and time!
A doyen of reconstructive plastic surgery
The basic principles of planning skin flaps, suture techniques or wound treatment which Joseph practiced are relevant even today and ought to be understood and followed by every plastic surgeon. He has in his book emphasized the importance of the exact analysis of a deformity or defect and how it is essential to a clear, pre-operative plan. The diagrams and illustrations depicting the general fundamentals of anatomy, facial proportions, the morbid anatomy of a deformity, what is missing and from where, all help the reader to become a better treatment planner. The didactic structure of his book, which illustrates the various causes of each deformity, guides the reader through specific surgical problem situations and their solutions, often in apparently hopeless cases. The language is simple and gives a clear idea of how the author was thinking and encourages the reader to do the same. What we today refer to as looking at the third dimension of a deformity is very clearly apparent. In fact he adds a fourth dimension too by emphasizing the psychological aspect rather boldly. For example, in the chapter "Grounds for rhinomiosis and the relevance of the operation to everyday living", we read: "One who wishes to be rid of a deformity, who simply wants a 'normal' appearance - wishes to be inconspicuous - should not suffer the odium of vanity."
Not only the author Jacques Joseph but his team of illustrator, photographer and publisher all deserve rich praises for the outstanding publication. The illustrations and pastels are easy to understand, clear and didactically brilliant, making the steps of each procedure and its principles thoroughly comprehensible and enjoyable to read. The balanced and harmonic design, the layout, and the technical excellence visible in the black-and-white photographs all combined can match the best publications of our present day and age. The reprint, using a complex reprographic procedure, is quite comparable in quality to the original and those who possess the book treasure it and pass it on to the next generation with a lot of hope. The original German edition of Joseph's book published in 1931 is a legendary rarity. The English translation by Stanley Milstein was published in 1987. It has 843 pages with 1718 illustrations, some in colour. Bound in Red Morocco leather spine tooled in gold, black cloth boards, and presented in gold-stamped cloth slipcase, it is a collector's dream besides being an outstanding reference book of aesthetic plastic surgery.
Joseph developed many new instruments for his operations, the most well-known being the Joseph's elevator which is still in daily use in operating theatres throughout the world. The book also conveys an understanding of the relationship between operative goal and instrument knowledge. The clever use of novel instruments to suit tricky situations was perhaps his hobby as in his lifetime he designed many of them.
Cultural history of the then Germany
This major work of Jacques Joseph is also an impressive reminder of an important, bygone era of medicine. Joseph's career began in Germany at the time of emperor Wilhelm II, brought him the highest recognition as a doctor and ended sadly under the National Socialists with extreme humiliation. In 1933 the National Socialists came to power in Germany and a catastrophe was in the making. Jewish Germans, who were unanimously labeled as 'dissidents', were in for a hard time, to put it mildly after so many years. Joseph, being an aristocrat and a jewel amongst Berliners had not taken the increasing Brown danger seriously. Little did he realize that despite the greatest recognition and esteem that he enjoyed in the recent past there would follow - nearly overnight - the deepest official disdain. The Gestapo had engaged the steno typist whom Joseph had employed to finish his textbook, and who lived in his house. He spied on him and blackmailed him on the instructions of his Nazi authorities. The authorities would allow Joseph to carry out only few 'special approval procedures'. The wave of violent anti-Semitism of the time took a toll on both his practice and his private life.
In Germany today, the importance of Jacques Joseph and his work has largely fallen into oblivion. This is primarily due to the fact that after his death, his achievements, like those of all other Jewish doctors and scientists during the Nazi era, were ignored. After the war, the first publications and praises about Jacques Joseph came from abroad, particularly from his trainees and peers in the USA, where his wife Eleonore and daughter Bella had emigrated. It is thanks to the publisher Dr. Reinhard Kaden and the luxurious reprint of Jacques Joseph's work that this scientific legacy of a great physician is once again accessible to his cynosures.
Death of an unsung Hero
On 12 th February 1934 Jacques Joseph died of a heart attack in the hallway of his house on his way to his practice. Though theories of death by violence or suicide are mostly unfounded, the fact that his myocardial infarction was precipitated by the repeated mishandlings which Joseph suffered at the hands of Nazi thugs can not be ruled out. The German specialist press, already mostly brought into line by the Nazis by then, took no more notice of Joseph's death. Obituaries appeared only in overseas journals and mourners were mostly his trainees and his peers.
Recognition after death
Jacques Joseph was buried in the Jewish Cemetery in Berlin-Weissensee. After the bombing raid in the Second World War his grave was destroyed and was believed to be no longer identifiable. After much difficult research, it was possible to find the partially buried and overgrown gravestone of Joseph's grave in August 2003. The stone, made of black granite, has since been retrieved, identified and the former inscription deciphered.
Jacques Joseph's gravestone was consecrated on the occasion of the re-erection of his tomb on 17 th October 2004, at the same Jewish Cemetery in Berlin-Weissensee. The unveiling and consecration of the stone was conducted by the eminent rabbi Dr. Andreas Nachama in the presence of some 70 guests from home and abroad. Thus, Jacques Joseph was paid the respect which was denied him at his death . Many private persons and professional associations throughout the world donated generously for the re-erection of his tomb. They included many private citizens and organizations like American Academy of Facial Plastic and Reconstructive Surgery; European Academy of Facial Plastic Surgery; Deutsche Gesellschaft fuer Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie; Norddeutsche Gesellschaft fuer Otorhinolaryngologie und zervikofaziale Chirurgie; Oto-Laryngologische Gesellschaft zu Berlin; Vereinigung der Westdeutschen Hals-Nasen-Ohren-Aerzte von 1897. The stone, though refurbished, was purposely left unchanged despite the bomb-damage it sustained to reflect the eventful history of the grave. On the front is the new inscription on polished Swedish granite but the back of the stone has been left just as it was when it was found broken, overgrown, damaged, with fragments of inscription. It has been proposed to the Senate that the tomb receives recognition as an official Berlin memorial.
When the International Federation of Plastic. Reconstructive and Aesthetic Surgery (IPRAS), met in Berlin for its 14 th . Congress in June 2007, Jacques Joseph was honoured in its Inaugural Function. Prof. Robert Goldwyn unveiled his bust statue and all the dignitaries spoke very highly of him and his contributions to enrich our science, which we all practice and love so passionately.
Wednesday, December 9, 2009
Health care-The future and beyond
We have seen the future of medicine-and it is tough on the eyes. The secret to radically improved health care lies at the cellular level, ground zero for disease, where everything is roughly 1,000 times as small as the period at the end of this sentence. Go ahead a decade or so, and doctors will wield molecular tools to switch genes on and off, taming diabetes and obesity, among many ills.
Researchers will harness tiny proteins to ward off any strain of influenza.
Precision-guided cancer killers will lay waste to tumors without so much as grazing the surrounding healthy tissue. No more chemotherapy side effects.
From a nano-sewing kit that mends severed nerves to a genetic switch that turns off fat genes, the future of molecular medicine looks bright. And that's no small thing.
This is what the not-so-distant future of medicine will look like. Over the next two decades, medicine will change from its current reactive mode, in which doctors wait for people to get sick, to a mode that is far more preventive and rational. People call it P4 medicine—predictive, personalized, preventive and participatory. What's driving this change are powerful new measurement technologies and the so-called systems approach to medicine. Whereas medical researchers in the past studied disease by analyzing the effects of one gene at a time, the systems approach will give them the ability to analyze all your genes at once. The average doctor's office visit today might involve blood work and a few measurements, such as blood pressure and temperature; in the near future physicians will collect billions of bytes of information about each individual—genes, blood proteins, cells and historical data. They will use this data to assess whether your cell's biological information-handling circuits have become perturbed by disease, whether from defective genes, exposure to bad things in the environment or both.
Several emerging technologies are making this holistic, molecular approach to disease possible. Nano-size devices will measure thousands of blood elements, and DNA sequencers will decode individual human genomes rapidly, accurately and inexpensively. New computers will sort through huge amounts of data gathered annually on each individual and boil down this information to clear results about health and disease.
Medicine will begin to get more predictive and personalized (the first two aspects of P4 medicine) over the next five to 10 years. First, doctors will be able to sequence the genome of each patient, which together with other data will yield useful predictions about his or her future health; it will be able to tell you, for example, that you have a 30 percent chance of developing ovarian cancer before age 30. Second, a biannual assessment of your blood will make it possible to get an update on the current state of your health for each of your 50 or so organ systems. These steps will place the focus of medicine on individual patients and on assessing the impact that genes and their interactions with the environment have in determining health or disease.
In preventive medicine (the third P), researchers will use systems medicine to develop drugs that help prevent disease. If, say, you have a 50 percent chance of developing prostate cancer by the time you're 50, you may be able to start taking a drug when you're 30 that would reduce substantially reduce that probability. In the next 10 to 20 years the focus of health care will shift from dealing with disease to maintaining wellness.
Participatory medicine acknowledges the unparalleled opportunities that patients will have to take control of their health care. To participate effectively, though, they will have to be educated as to the basic principles of P4 medicine. New companies that can analyze human genome variation, like 23andMe and Navigenics, are already planning to provide patients with genetic information that may be useful in modifying their behavior to avoid future health problems. In the future, patients will need not just genetic data but insight into how the environment is turning genes on and off to cause disease—just as smoking often causes lung cancer and exposure to sunlight can cause skin cancer.
P4 medicine will have a big impact on many industries, including pharmaceuticals, food and insurance, as well as health care. The interesting question is whether preexisting businesses and entrenched bureaucracies will be able to respond to these winds of change, or whether a host of new companies will emerge to replace them—focused precisely on these new opportunities.
Research will also have to change. Because most important diseases such as diabetes, cancer, heart disease, obesity and Alzheimer's are so complex, the traditional approaches to studying them have had only marginal results. Powerful new systems approaches, individual measurements and computational technologies will transform our ability to deal with complexity and fashion new drugs and approaches for therapy and prevention.
Medical education will also need to be transformed. Although today's medical students will be practicing P4 medicine within the next five to 20 years, their training is still focused on a classification of disease based on observation of relatively few measurements of health parameters. Tomorrow's physicians will need to be familiar with the complexity of the human biological system as never before, and they'll have to be handy with computer-based tools. Physicians will need to deal with patients who have an enormous amount of information at their disposal. And doctors will need to deal with maintaining wellness more than with disease.
The key benefits of P4 medicine, to the system and mostimportantly the patient, include innovative techniques to:
• Disease detection at an earlier stage, thus not only making the treatment more effective but also reducing the cost
• Stratify patients into groups that enable the selection of optimal therapy
• Reduce adverse drug reactions by more effective early assessment of individual drug responses
• Improve the selection of new biochemical targets for drug discovery
• Reduce the time, cost, and failure rate of clinical trials for new therapies
• Shift the emphasis in medicine from reaction to prevention and from disease to wellness
The digitization of medicine—that is, our ability to extract and store disease-relevant information from DNA and molecules in the blood of each individual—together with the revolutionary changes in diagnosis, therapy and prevention will allow those of us in the developed world to export P4 medicine to the developing world and thus transform the quality of its health care. The new P4 medicine will eventually lead to a universal democratization of health care, bringing to billions the fundamental right of health, unimaginable even a few years ago.
Researchers will harness tiny proteins to ward off any strain of influenza.
Precision-guided cancer killers will lay waste to tumors without so much as grazing the surrounding healthy tissue. No more chemotherapy side effects.
From a nano-sewing kit that mends severed nerves to a genetic switch that turns off fat genes, the future of molecular medicine looks bright. And that's no small thing.
This is what the not-so-distant future of medicine will look like. Over the next two decades, medicine will change from its current reactive mode, in which doctors wait for people to get sick, to a mode that is far more preventive and rational. People call it P4 medicine—predictive, personalized, preventive and participatory. What's driving this change are powerful new measurement technologies and the so-called systems approach to medicine. Whereas medical researchers in the past studied disease by analyzing the effects of one gene at a time, the systems approach will give them the ability to analyze all your genes at once. The average doctor's office visit today might involve blood work and a few measurements, such as blood pressure and temperature; in the near future physicians will collect billions of bytes of information about each individual—genes, blood proteins, cells and historical data. They will use this data to assess whether your cell's biological information-handling circuits have become perturbed by disease, whether from defective genes, exposure to bad things in the environment or both.
Several emerging technologies are making this holistic, molecular approach to disease possible. Nano-size devices will measure thousands of blood elements, and DNA sequencers will decode individual human genomes rapidly, accurately and inexpensively. New computers will sort through huge amounts of data gathered annually on each individual and boil down this information to clear results about health and disease.
Medicine will begin to get more predictive and personalized (the first two aspects of P4 medicine) over the next five to 10 years. First, doctors will be able to sequence the genome of each patient, which together with other data will yield useful predictions about his or her future health; it will be able to tell you, for example, that you have a 30 percent chance of developing ovarian cancer before age 30. Second, a biannual assessment of your blood will make it possible to get an update on the current state of your health for each of your 50 or so organ systems. These steps will place the focus of medicine on individual patients and on assessing the impact that genes and their interactions with the environment have in determining health or disease.
In preventive medicine (the third P), researchers will use systems medicine to develop drugs that help prevent disease. If, say, you have a 50 percent chance of developing prostate cancer by the time you're 50, you may be able to start taking a drug when you're 30 that would reduce substantially reduce that probability. In the next 10 to 20 years the focus of health care will shift from dealing with disease to maintaining wellness.
Participatory medicine acknowledges the unparalleled opportunities that patients will have to take control of their health care. To participate effectively, though, they will have to be educated as to the basic principles of P4 medicine. New companies that can analyze human genome variation, like 23andMe and Navigenics, are already planning to provide patients with genetic information that may be useful in modifying their behavior to avoid future health problems. In the future, patients will need not just genetic data but insight into how the environment is turning genes on and off to cause disease—just as smoking often causes lung cancer and exposure to sunlight can cause skin cancer.
P4 medicine will have a big impact on many industries, including pharmaceuticals, food and insurance, as well as health care. The interesting question is whether preexisting businesses and entrenched bureaucracies will be able to respond to these winds of change, or whether a host of new companies will emerge to replace them—focused precisely on these new opportunities.
Research will also have to change. Because most important diseases such as diabetes, cancer, heart disease, obesity and Alzheimer's are so complex, the traditional approaches to studying them have had only marginal results. Powerful new systems approaches, individual measurements and computational technologies will transform our ability to deal with complexity and fashion new drugs and approaches for therapy and prevention.
Medical education will also need to be transformed. Although today's medical students will be practicing P4 medicine within the next five to 20 years, their training is still focused on a classification of disease based on observation of relatively few measurements of health parameters. Tomorrow's physicians will need to be familiar with the complexity of the human biological system as never before, and they'll have to be handy with computer-based tools. Physicians will need to deal with patients who have an enormous amount of information at their disposal. And doctors will need to deal with maintaining wellness more than with disease.
The key benefits of P4 medicine, to the system and mostimportantly the patient, include innovative techniques to:
• Disease detection at an earlier stage, thus not only making the treatment more effective but also reducing the cost
• Stratify patients into groups that enable the selection of optimal therapy
• Reduce adverse drug reactions by more effective early assessment of individual drug responses
• Improve the selection of new biochemical targets for drug discovery
• Reduce the time, cost, and failure rate of clinical trials for new therapies
• Shift the emphasis in medicine from reaction to prevention and from disease to wellness
The digitization of medicine—that is, our ability to extract and store disease-relevant information from DNA and molecules in the blood of each individual—together with the revolutionary changes in diagnosis, therapy and prevention will allow those of us in the developed world to export P4 medicine to the developing world and thus transform the quality of its health care. The new P4 medicine will eventually lead to a universal democratization of health care, bringing to billions the fundamental right of health, unimaginable even a few years ago.
Sunday, December 6, 2009
First evidence of successful Rhinoplasty
In surgery, ancient Hindu medicine reached its zenith. Operations performed by Hindu surgeons included excision of tumours, incision and draining of abscesses, punctures to release fluid in the abdomen, extraction of foreign bodies, repair of anal fistulas, splinting of fractures, amputations, cesarean sections, and stitching of wounds.In two types of operations especially, the Hindus were outstanding. Stone in the bladder (vesical calculus) was common in ancient India, and the surgeons frequently removed the stones by lateral lithotomy. They also introduced plastic surgery. Sushruta, one of the earliest surgeons of recorded history (600 B.C.) is believed to be the first individual to describe Rhinoplasty.
The historical evidences suggest that plastic surgery originated in India more than two millennia ago and the oldest plastic surgery operation probably relate to nasal reconstruction.Sushruta, an ancient Indian surgeon in 600 BC. is believed to be the first individual to describe Rhinoplasty.
Sushruta (also known as the ‘‘Father of Indian surgery'‘ and ‘‘Father of Indian plastic surgery'‘) authored famous ancient encyclopaedic treatise 'Sushruta Samhita' (Sushruta's compendium) and vividly described the technique of Rhinoplasty. ‘Sushruta Samhita' is considered to be the most advanced compilation of surgical practices prevalent in India around two thousand millennia ago. In‘Sushruta Samhita', Sushruta emphasized all the basic principles of plastic surgery and vividly described numerous operations in various fields of surgery with significant contributions to Plastic Surgery .The notable contributions in Plastic Surgery are technique of pedicle flap, repair of ear lobe defects ,repair of traumatic and congenital clefts of the lip, classification of burns ,description of sharp (20 types) and blunt (101 types)instruments, practice of mock operations, cadveric dissection ,use of wine to dull the pain of surgical incisions, code of ethics; however the Rhinoplasty remains the greatest highlight of Sushruta's surgery. The nose in Indian society has remained a symbol of dignity and respect throughout antiquity. In ancient times, amputation of nose was frequently done as a punishment for criminals, war prisoners or people indulged in adultery. The practice of Rhinoplasty slowly started as a result of the need to reconstruct the external nose and later developed to the full fledged science.
Sushruta is considered as the innovator of the Rhinoplasty technique practised since 600 B.C.The detailed description of the Rhinoplasty operation in the Sushruta Samhita is amazingly precise and comprehensive. The English translation of Sushruta's original Sanskrit description of the method is as follows 3:
“The portion of the nose to be covered should be first measured with a leaf. Then a piece of skin of the required size should be dissected from the living skin of the cheek, and turned back to cover the nose, keeping a small pedicle attached to the cheek. The part of the nose to which the skin is to be attached should be made raw by cutting the nasal stump with a knife. The physician then should place the skin on the nose and stitch the two parts swiftly, keeping the skin properly elevated by inserting two tubes of eranda (the castor-oil plant) in the position of the nostrils, so that the new nose gets proper shape. The skin thus properly adjusted, it should then be sprinkled with a powder of liquorice, red sandal-wood and barberry plant. Finally, it should be covered with cotton, and clean sesame oil should be constantly applied. When the skin has united and granulated, if the nose is too short or too long, the middle of the flap should be divided and an endeavor made to enlarge or shorten it.”
The Sanskrit text of 'Sushruta Samhita' was later translated in Arabic by Ibn Abi Usaybia (1203-1269 AD)4.As the historical pages started opening up, the knowledge of Rhinoplasty spread from India to Arabia and Persia and from there to Egypt. However, it took centuries for the principles and the technique of Rhinoplasty to travel to Europe and other parts of the world. In the 15th century, Gaspare Tagliacozzi from Italy documented similar technique of nasal reconstruction. He successfully reconstructed the nose by using the skin of the upper arm. The principle of Italian procedure was precisely the same as of the pedicle flap which was described two millennia ahead by Sushruta. Ackernecht aptly observed “There is little doubt that plastic surgery in Europe which flourished in medieval Italy is a direct descendant of classical Indian surgery”.5
The classical cheek flap Rhinoplasty of Sushruta was later modified by using a rotation flap from the adjacent forehead, The Traditional Indian Method of Rhinoplasty. This technique was kept a secret for centuries in India, and practiced by Marathas of Kumar near Poona, certain Nepali families and Kanghairas of Kangra (Himachal Pradesh) 4.
The resurgence of Indian method began in the 1700s when British surgeons working for the East India Company saw the work done by Indian surgeons. During Mysore War of 1792 between Tipu Sultan and the British. Cowasjee, a cart-driver with the British and four other native sepoys were captured by the Sultan's soldiers. Their noses and a hand each were cut off by the Mysore army. After a year without a nose, he and four of his colleagues submitted themselves to treatment by a man who had a reputation for nose repairs. The operations were witnessed by Thomas Cruso and James Findlay ,surgeons at the British Residency in Poona. They appear to have prepared a description of what they saw and diagrams of the procedure. The technique used for Rhinoplasty was a modification of the ancient Rhinoplasty described by Sushruta. Sushruta's version has the skin flap being taken from the cheek; Cowasjee's was taken from the forehead. A photo feature on the sensational surgery was published in the Madras Gazette. Subsequently, the details and an engraving from the painting were reproduced in the October 1794 issue of the Gentleman's Magazine of London 6.
The operation was described as follows:
“A thin plate of wax is fitted to the stump of the nose so as to make a nose of good appearance; it is then flattened and laid on the forehead. A line is drawn around the wax, which is then of no further use, and the operator then dissects off as much skin as it had covered, living undivided a small slip between the eyes. This slip preserves the blood circulation till a union has taken place between the new and the old parts. The cicatrix of the stump of the nose is next paired off, and immediately behind the new part, an incision is made through the skin which passes around both alae, and goes along the upper lip. The skin, now brought down from the forehead and being twisted half around, is inserted into this incision, so that a nose is formed with a double hold above and with its alae and septum below fixed in the incision. A little Terra Japonica (pale-catechu) is softened with water and being spread on slips of cloth, five or six of these are placed over each other to secure the joining. No other dressing but this cement is used for four days. It is then removed, and cloths dipped in ghee are applied. The connecting slip of skin is divided about the twentieth day, when a little more dissection is necessary to improve the appearance of the new nose. For five or six days after the operation, the patient is made to lie on his back, and on the tenth day, bits of soft cloth are put into the nostrils to keep them sufficiently open. This operation is always successful. The artificial nose is secured and looks nearly as well as the natural nose, nor is the scar on the forehead very observable after a length of time.”
This story encouraged Carpue, an English surgeon, to study the details and soon he recognized the immense potential of the operation. Carpue successfully performed the first Rhinoplasty operation (37 minutes) on October 23, 1814 followed by a second successful operation7. Subsequently, through the publication of these successful operations by Carpue in 1816, the use of Indian technique gained popularity amongst British and European surgeons. By 1897, at least 152 rhinoplasties had been performed in Europe.
One of the earliest European descriptions of Indian rhinoplasty is as follows 8:
“The surgeons belonging to the country cut the skin of the forehead above the eyebrows, and made it fall down over the wounds on the nose. Then, giving a twist so that a live flesh might meet the other live surface, by healing applications, they fashioned for them other imperfect noses. There is left above, between the eyebrows, a small hole, caused by the twist given to the skin to bring the two live surfaces together. In a short time the wounds heal up, some obstacle being placed beneath to allow of respiration. I saw many persons with such noses, and they were not so disfigured as they would have been without any nose at all.” (Storia do Mogor 1653-1708 AD).
These Rhinoplasties were widely appreciated as the 'Indian Nose' and generated tremendous interest in the medical fraternity paving way for corrective Rhinoplasty in Europe , United states and other part of the world. Later, with the dissemination and refinement of the technique it became an established procedure worldwide.
Though today the technique has received few modifications but the basic principles laid down by Sushruta still remains true. Today, the world acknowledges India as the cradle of Rhinoplasty and the contemporary use of the “Indian flap” for nasal reconstruction testifies to its practicality and success for more than 2500 years.
The historical evidences suggest that plastic surgery originated in India more than two millennia ago and the oldest plastic surgery operation probably relate to nasal reconstruction.Sushruta, an ancient Indian surgeon in 600 BC. is believed to be the first individual to describe Rhinoplasty.
Sushruta (also known as the ‘‘Father of Indian surgery'‘ and ‘‘Father of Indian plastic surgery'‘) authored famous ancient encyclopaedic treatise 'Sushruta Samhita' (Sushruta's compendium) and vividly described the technique of Rhinoplasty. ‘Sushruta Samhita' is considered to be the most advanced compilation of surgical practices prevalent in India around two thousand millennia ago. In‘Sushruta Samhita', Sushruta emphasized all the basic principles of plastic surgery and vividly described numerous operations in various fields of surgery with significant contributions to Plastic Surgery .The notable contributions in Plastic Surgery are technique of pedicle flap, repair of ear lobe defects ,repair of traumatic and congenital clefts of the lip, classification of burns ,description of sharp (20 types) and blunt (101 types)instruments, practice of mock operations, cadveric dissection ,use of wine to dull the pain of surgical incisions, code of ethics; however the Rhinoplasty remains the greatest highlight of Sushruta's surgery. The nose in Indian society has remained a symbol of dignity and respect throughout antiquity. In ancient times, amputation of nose was frequently done as a punishment for criminals, war prisoners or people indulged in adultery. The practice of Rhinoplasty slowly started as a result of the need to reconstruct the external nose and later developed to the full fledged science.
Sushruta is considered as the innovator of the Rhinoplasty technique practised since 600 B.C.The detailed description of the Rhinoplasty operation in the Sushruta Samhita is amazingly precise and comprehensive. The English translation of Sushruta's original Sanskrit description of the method is as follows 3:
“The portion of the nose to be covered should be first measured with a leaf. Then a piece of skin of the required size should be dissected from the living skin of the cheek, and turned back to cover the nose, keeping a small pedicle attached to the cheek. The part of the nose to which the skin is to be attached should be made raw by cutting the nasal stump with a knife. The physician then should place the skin on the nose and stitch the two parts swiftly, keeping the skin properly elevated by inserting two tubes of eranda (the castor-oil plant) in the position of the nostrils, so that the new nose gets proper shape. The skin thus properly adjusted, it should then be sprinkled with a powder of liquorice, red sandal-wood and barberry plant. Finally, it should be covered with cotton, and clean sesame oil should be constantly applied. When the skin has united and granulated, if the nose is too short or too long, the middle of the flap should be divided and an endeavor made to enlarge or shorten it.”
The Sanskrit text of 'Sushruta Samhita' was later translated in Arabic by Ibn Abi Usaybia (1203-1269 AD)4.As the historical pages started opening up, the knowledge of Rhinoplasty spread from India to Arabia and Persia and from there to Egypt. However, it took centuries for the principles and the technique of Rhinoplasty to travel to Europe and other parts of the world. In the 15th century, Gaspare Tagliacozzi from Italy documented similar technique of nasal reconstruction. He successfully reconstructed the nose by using the skin of the upper arm. The principle of Italian procedure was precisely the same as of the pedicle flap which was described two millennia ahead by Sushruta. Ackernecht aptly observed “There is little doubt that plastic surgery in Europe which flourished in medieval Italy is a direct descendant of classical Indian surgery”.5
The classical cheek flap Rhinoplasty of Sushruta was later modified by using a rotation flap from the adjacent forehead, The Traditional Indian Method of Rhinoplasty. This technique was kept a secret for centuries in India, and practiced by Marathas of Kumar near Poona, certain Nepali families and Kanghairas of Kangra (Himachal Pradesh) 4.
The resurgence of Indian method began in the 1700s when British surgeons working for the East India Company saw the work done by Indian surgeons. During Mysore War of 1792 between Tipu Sultan and the British. Cowasjee, a cart-driver with the British and four other native sepoys were captured by the Sultan's soldiers. Their noses and a hand each were cut off by the Mysore army. After a year without a nose, he and four of his colleagues submitted themselves to treatment by a man who had a reputation for nose repairs. The operations were witnessed by Thomas Cruso and James Findlay ,surgeons at the British Residency in Poona. They appear to have prepared a description of what they saw and diagrams of the procedure. The technique used for Rhinoplasty was a modification of the ancient Rhinoplasty described by Sushruta. Sushruta's version has the skin flap being taken from the cheek; Cowasjee's was taken from the forehead. A photo feature on the sensational surgery was published in the Madras Gazette. Subsequently, the details and an engraving from the painting were reproduced in the October 1794 issue of the Gentleman's Magazine of London 6.
The operation was described as follows:
“A thin plate of wax is fitted to the stump of the nose so as to make a nose of good appearance; it is then flattened and laid on the forehead. A line is drawn around the wax, which is then of no further use, and the operator then dissects off as much skin as it had covered, living undivided a small slip between the eyes. This slip preserves the blood circulation till a union has taken place between the new and the old parts. The cicatrix of the stump of the nose is next paired off, and immediately behind the new part, an incision is made through the skin which passes around both alae, and goes along the upper lip. The skin, now brought down from the forehead and being twisted half around, is inserted into this incision, so that a nose is formed with a double hold above and with its alae and septum below fixed in the incision. A little Terra Japonica (pale-catechu) is softened with water and being spread on slips of cloth, five or six of these are placed over each other to secure the joining. No other dressing but this cement is used for four days. It is then removed, and cloths dipped in ghee are applied. The connecting slip of skin is divided about the twentieth day, when a little more dissection is necessary to improve the appearance of the new nose. For five or six days after the operation, the patient is made to lie on his back, and on the tenth day, bits of soft cloth are put into the nostrils to keep them sufficiently open. This operation is always successful. The artificial nose is secured and looks nearly as well as the natural nose, nor is the scar on the forehead very observable after a length of time.”
This story encouraged Carpue, an English surgeon, to study the details and soon he recognized the immense potential of the operation. Carpue successfully performed the first Rhinoplasty operation (37 minutes) on October 23, 1814 followed by a second successful operation7. Subsequently, through the publication of these successful operations by Carpue in 1816, the use of Indian technique gained popularity amongst British and European surgeons. By 1897, at least 152 rhinoplasties had been performed in Europe.
One of the earliest European descriptions of Indian rhinoplasty is as follows 8:
“The surgeons belonging to the country cut the skin of the forehead above the eyebrows, and made it fall down over the wounds on the nose. Then, giving a twist so that a live flesh might meet the other live surface, by healing applications, they fashioned for them other imperfect noses. There is left above, between the eyebrows, a small hole, caused by the twist given to the skin to bring the two live surfaces together. In a short time the wounds heal up, some obstacle being placed beneath to allow of respiration. I saw many persons with such noses, and they were not so disfigured as they would have been without any nose at all.” (Storia do Mogor 1653-1708 AD).
These Rhinoplasties were widely appreciated as the 'Indian Nose' and generated tremendous interest in the medical fraternity paving way for corrective Rhinoplasty in Europe , United states and other part of the world. Later, with the dissemination and refinement of the technique it became an established procedure worldwide.
Though today the technique has received few modifications but the basic principles laid down by Sushruta still remains true. Today, the world acknowledges India as the cradle of Rhinoplasty and the contemporary use of the “Indian flap” for nasal reconstruction testifies to its practicality and success for more than 2500 years.
Saturday, December 5, 2009
Bilateral Cleft Lip Repair
Introduction
The presence of bilateral cleft lip and palate has the potential to significantly alter facial form and structure. The central third of the face is distorted by the bilateral cleft, and restoring the normal facial form is one of the primary goals for the reconstructive surgeon.
· Craniofacial, Bilateral Cleft Nasal Repair
· Craniofacial, Cleft Palate
· Craniofacial, Cleft Palate Repair
· Craniofacial, Unilateral Cleft Lip Repair
· Craniofacial, Unilateral Cleft Nasal Repair
History of the Procedure
The history of bilateral cleft lip repair has evolved from discarding the premaxilla and prolabium and approximating the lateral lip elements to a definitive lip and primary cleft nasal repair utilizing the underlying musculature. Accompanying the evolution of surgical repair is the increasingly important role of orthodontic support with early presurgical alveolar and nasal molding.1 Repositioning the maxillary and alveolar segments into a more anatomic position allows the surgeon to repair the lip and associated nasal deformity under more optimal conditions. The reader is referred to the references cited for a more detailed historical review of individual repairs and supportive management.
Problem
The cleft affects the obvious facial form as an anatomic deformity and has functional consequences, affecting the child's ability to eat, speak, hear, and breathe. Consequently, rehabilitation of a child born with a facial cleft must involve a multidisciplinary approach and be staged appropriately with the child's development. The need for intervention must be balanced against its subsequent effect on normal growth. In the child born with a bilateral cleft, the surgeon initially is faced with a protrusive premaxilla and the difficulty of achieving adequate columellar length and vertical height to the lip during reconstruction.
Bilateral cleft lip repair. (A) The prolabial width is typically set at 4-5 mm. (B) The prolabial flap is elevated to the base of the columella. The adjacent flaps are turned over to create a labial sulcus. (C) The orbicularis oris muscle, dissected from the overlying skin, is approximated across the midline. (D) The skin is approximated, and the Cupid's bow is created from the lateral vermilion flaps.
Frequency
The overall occurrence of cleft lip with or without cleft palate is approximately 1 in 750-1000 live births, making it the second most common congenital deformity (after club foot). Incidence varies by race, with clefts occurring more commonly in Asians (1 in 500 births), less frequently in Caucasians (1 in 750 births), and even less frequently in African Americans (1 in 2000 births). These racial variations in incidence are not observed with the isolated cleft palate. In terms of gender, the incidence of cleft lip/palate is more common in males, while the incidence of cleft palate alone is more common in females.
The most common presentation is cleft lip and palate (approximately 45%), followed by cleft palate alone (35%) and cleft lip alone (approximately 20%). Unilateral cleft lips are more common than bilateral cleft lips and occur more commonly on the left side (left cleft lip:right cleft lip:bilateral cleft lip - 6:3:1). In a large series reported in the literature, bilateral clefts are observed in approximately 15% of patients with clefts.2,3,4
Bilateral clefts occur in approximately 1 in 5000-6500 births based on the overall occurrence. According to CDC estimates, the overall incidence of orofacial clefts is 1 in 596, with the highest incidences occurring in Native Americans (3.6 in 1000) and the lowest in African Americans.5,6
According to the American Society of Plastic Surgeons (ASPS), 18,859 cleft lip and palate reconstructive procedures were performed in the United States in 2007.7 These statistics include procedures performed by ASPS members as well as other board-certified physicians most likely to perform plastic surgery procedures.
Etiology
Clefting is a multifactorial issue, with both genetic and environmental causes cited. The observation of clustered cases of facial clefts in a particular family indicates a genetic basis. Clefting of the lip and/or palate is associated with more than 150 syndromes. The overall incidence of associated anomalies (eg, cardiac) is approximately 30% (more common with isolated cleft palate).
Environmental causes, such as viral infection (eg, rubella) and teratogens (eg, steroids, anticonvulsants), during the first trimester of pregnancy have been linked to facial clefts. The risk also increases with parental age, especially when older than 30 years, with the father's age appearing to be a more significant factor than the mother's age. Nevertheless, most presentations are of isolated patients within the family without an obvious etiology.
Midfacial development involves several sets of genes, including those involved in cell patterning, proliferation, and signaling. Mutations in any of these genes can change the developmental process and contribute to cleft development. Some of these genes include the DIX gene, sonic hedgehog (SHH) gene, transforming growth factor (TGF) alpha and beta, and interferon regulatory factor (IRF6) gene.
Pathophysiology
While the normal embryologic development of the face is detailed in Head and Neck Embryology, a brief outline relevant to the formation of facial clefts follows.
In short, the branchial arches are responsible for the formation of several areas, including the mouth and lip. Mesenchymal migration and fusion occurs during weeks 4-7 of gestation. The first branchial arch is responsible for the formation of the maxillary and mandibular processes. The maxillary and mandibular prominences form the lateral borders of the primitive mouth or stomodeum.
Mesenchymal migration and fusion of the primitive somite-derived facial elements (central frontonasal, 2 lateral maxillary, mandibular processes), at 4-7 weeks gestation, are necessary for the normal development of embryonic facial structures. When migration and fusion are interrupted for any reason, a facial cleft develops along embryonic fusion lines. The embryonic development of the primary palate (lip and palate anterior to the incisive foramen) differs from the secondary palate (palate posterior to the incisive foramen).
The developing processes of the medial nasal prominence, lateral nasal prominence, and maxillary prominences form the primary palate. Fusion occurs, followed by "streaming" of mesodermal elements derived from the neural crest. In contrast, the secondary palate is formed by the fusion of palatal processes of the maxillary prominence alone. The difference in embryonic development suggests the possibility of differing degrees of susceptibility to genetic and environmental influences and accounts for the observed variation in incidences. In summary, a bilateral cleft lip results from the failure of fusion of the medial nasal prominences with the maxillary prominences bilaterally.
Kernahan developed a classification scheme in which the defect can be classified onto a Y-shaped symbol. In this diagram, the incisive foramen is represented as the focal point. This system has been applied to both cleft lip and palate.
Millard modification of Kernahan striped-Y classification for cleft lip and palate. The small circle indicates the incisive foramen; the triangles indicate the nasal tip and nasal floor.
Presentation
As with the unilateral cleft, a wide range of clinical presentations of the bilateral cleft lip and palate is possible, from the simple microform cleft to the complete cleft bilaterally involving the lip, alveolus, palate, and nose. Bilateral cleft lip is typically classified as either complete or incomplete.
In addition, the cleft may present with varying degrees of asymmetric involvement of the lip and palate. Any degree of combination of involvement of the lip, primary palate, and secondary palate can exist. Recognizing these potentially asymmetric variations is essential in planning operative treatment and evaluating postoperative results.
In the complete form of clefts, the premaxilla is unrestrained by the lateral maxillary segments and the vomer grows anterior to the lateral segments. This distorts the alar cartilages away from the nasal tip and stretches the alae across a widening cleft. The columella is significantly deficient, can be virtually nonexistent and without a distinct demarcation from the prolabium, which also is vertically deficient. No prolabial muscle, philtral columns, dimples, or Cupid's bow is present. No nasal floor is present, as the cleft continues through the palatal shelf along either side of the vomer through the soft palate.
Further treatment planning
Orthodontic treatment can be initiated a few weeks following birth, prior to surgical intervention. Other adjunct procedures include lip adhesion, presurgical orthopedics, primary nasal correction, and nasoalveolar molding. These procedures attempt to reduce the deformity. Nasoalveolar molding is the active molding and repositioning of the nasal cartilage and alveolar processes with an appliance. This orthodontic intervention takes advantage of the plasticity of the cartilage.
Presurgical nasal alveolar molding allows repositioning of the maxillary alveolus and surrounding soft tissues in hopes of reducing wound tension and improving results. Definitive repair is delayed until approximately 3 months of age, depending on physician comfort. A multidisciplinary approach should be carried out over a period of several years for patients with unilateral cleft lip. This multidisciplinary treatment team should include specialists in audiology, ENT, and speech therapy, among others.
The key difference between the treatment of unilateral versus bilateral cleft lips centers around the concept of aligning the 3 maxillary segments (the lateral lip segments with the prolabial segments). Presurgical orthopedics may be used to prevent the maxillary segments and premaxilla from collapse.
Presurgical orthopedics can involve the following:
· Finger massage of prolabium
· Pressure tape on prolabium
· Intraoral fixation devices
· Lip adhesion (can be used to achieve symmetry in asymmetric bilateral cleft situations)
· Nasoalveolar molding
The most common appliances used are nasoalveolar molds and intraoral fixation devices. Botulinum toxin is currently being investigated to decrease tension in cleft repair.
In general, bilateral cleft lip repair techniques have been modified from unilateral cleft lip repair techniques. If the repair is staged, the more severe side is repaired first, and the second side is repaired approximately 3-6 months later.
Indications
Patients born with a cleft lip should undergo surgical repair unless otherwise contraindicated. The goal of reconstruction is to establish normal morphologic facial form and function in order to provide the optimal conditions for the development of dentition, mastication, hearing, speech, breathing, and psychosocial status.
Relevant Anatomy
Understanding normal lip and nasal anatomy is essential to recognize the distortion caused by a facial cleft. The elements of the normal lip are the central philtrum, which is demarcated laterally by the philtral columns and inferiorly by the cupid's bow and tubercle. Just above the junction of the vermilion-cutaneous border is a mucocutaneous ridge referred to as the white roll. Within the red vermilion of the lip are 2 distinct areas: the dry vermilion (the more keratinized portion of the lip that is exposed to air) and the wet vermilion (exposed to the moist environment of the labial mucosa).
The primary muscle of the lip is the orbicularis oris. It has 2 well-defined components: the deep (internal) layer and the superficial (external) layer. The deep fibers run circumferentially from commissure (modiolus) to commissure (modiolus) and function as the primary sphincter for oral feeding. The superficial fibers run obliquely, interdigitating with the other muscles of facial expression to terminate in the dermis, and function to provide subtle shades of expression and precise movements of the lip for speech.
The superficial fibers of the orbicularis decussate in the midline and insert into the skin lateral to the opposite philtral groove, forming the philtral columns. The resulting philtral dimple is centrally depressed, as no muscle fibers directly insert into the dermis in the midline. The tubercle of the lip is shaped by the pars marginalis, the portion of the orbicularis along the vermilion forming the tubercle of the lip with eversion of the muscle.
In the upper lip, the levator labii superioris contributes to the form of the lip. Its fibers arise from the medial aspect of the infraorbital rim and sweep down to insert near the vermilion cutaneous junction. The most medial fibers of the levator labii superioris sweep down to insert near the corner of the ipsilateral philtral column and vermilion-cutaneous junction, helping to define the lower philtral column and the peak of the Cupid's bow.
The nasal muscles are equally important. The levator superioris alaeque arises along the frontal process of the maxilla and courses inferiorly to insert on the mucosal surface of the lip and ala. The transverse nasalis arises along the nasal dorsum and sweeps around the ala to insert along the nasal sill from lateral to medial into the incisal crest and anterior nasal spine. These fibers join with the oblique fibers of the orbicularis and the depressor septi (nasalis), which arises from the alveolus between the central and lateral incisors to insert into the skin of the columellar to the nasal tip and the footplates of the medial crura.
A bilateral cleft thus disrupts the normal termination of the muscle fibers that cross the embryologic fault line of the maxillary and nasal processes, resulting in symmetric but abnormal muscular forces between the normal equilibrium that exists with the nasolabial and oral groups of muscles. With an unrestrained premaxilla, the deformity accentuates with differential growth of the various elements. The alar cartilages are splayed apart and rotate caudally, subluxed from the normal position. Consequently, the nasal tip broadens, the columellar is foreshortened, and the alar bases rotate laterally and cephalad.
Workup
Laboratory Studies
· Perform a thorough physical examination, not limited to the head and neck region, to uncover associated anomalies in the infant presenting with a unilateral cleft lip with or without a palatal cleft. Additional workup is determined by physical findings that suggest involvement of other organ systems.
· The child's weight, oral intake, and growth and/or development are of primary concern and must be followed closely.
· Routine laboratory studies typically are not required, other than a hemoglobin study shortly before the planned lip repair.
· Routine imaging is not indicated in a healthy patient with isolated cleft lip.
Treatment
Surgical Therapy
Children born with a facial cleft benefit from a multidisciplinary approach in a team-based setting so that all aspects of their care can be coordinated efficiently.
Beyond lip repair are hearing, speech, dental, and psychosocial integration issues. These should be addressed throughout the child's growth and development from birth through adolescence. These issues are as important as the anatomic reconstruction. Ultimately, the functional outcome of the reconstruction depends on cooperation between the surgeon, orthodontist, and speech pathologist.
Each specialty involved must evaluate the child individually, formulate a treatment plan, and integrate it with those of the other specialties based on the child's needs. The Parameters of Care Guidelines established by the American Cleft Palate Craniofacial Association should be followed.8 However, rather than strictly adhering to one protocol, physicians should assess each child individually and formulate the treatment plan based on the team's experience, its overall philosophy of treatment, and available resources.
Preoperative Details
While the lip repair is the initial focus for many parents, treatment begins by assessing the child's nutritional status and assisting the parents with oral feeding techniques so that appropriate weight gain occurs. Parents who suddenly are faced with caring for a child with a facial cleft can be overwhelmed. The importance of spending sufficient time with them to allay their fears, to discuss staging and timing of reconstruction, to stress the need for involvement of other specialists, and to stress the importance of long-term and consistent follow-up care from birth through adolescence cannot be overemphasized.
No agreement is found in the literature regarding the ideal timing of lip repair. Some have advocated surgery in the early neonatal period, with a theoretical benefit in scar appearance and nasal cartilage adaptability, thus minimizing the nasal deformity. To minimize anesthetic risks, some still adhere to the rule of 10s: perform surgical repair of cleft lip when the child has a hemoglobin level of 10 g, weight of 10 lb, and is aged 10 weeks. In general, most centers prefer to perform the lip reconstruction when the patient is aged 2-4 months; anesthesia risks are lower, the child is better able to withstand the stress of surgery, and lip elements are larger and allow for a meticulous reconstruction.
A presurgical, fabricated, passive, intraoral orthodontic palatal appliance can be used to maintain the arch width to prevent the nearly inevitable collapse that occurs with lip repair. Rarely is there an indication for active expansion of the maxillary segment. This passive plating and gentle traction is an integral component of surgical cleft lip repair. The repair reestablishes the soft tissue and muscular forces on the easily moldable maxillary arch segments. Recently, the palatal appliance has been modified to include a nasal extension to help improve the nasal tip form. The orthodontist takes impressions, and the custom appliance is fitted as soon as possible after birth and well before the lip repair.
The appliance also aids in the child's oral feeding, helping to decrease nasal regurgitation and assisting oral suction. Some centers have chosen either no presurgical orthopedic intervention or an active pin-based appliance (eg, Latham) to align the maxillary arch segments. For bilateral clefts, external pressure is routinely used to help maintain the premaxillary component within the arch alignment. Soft elastic tape (eg, 3M Microfoam tape) across the premaxilla, a head cap with elastic traction, or lip adhesion can be used prior to a definitive lip repair once the arch segments have approximated.
Intraoperative Details
The ideal lip repair results in symmetrically shaped nostrils, nasal sill, and alar bases; adequate columellar length; a well-defined philtral dimple and columns; a natural-appearing cupid's bow with a pout to the vermilion tubercle; and an adequate labial sulcus. In addition, lip scars are used to approximate the natural landmarks. The ideal repair results in functional muscle repair that with animation that mimics a normal lip.
Intraoperative technique.
(A) The anatomic landmarks are tattooed and the planned incisions are marked.
(B) The orbicularis is dissected from the overlying skin and divided into bundles to allow interdigitation with its opposing element. Inferiorly, an element of the muscle is left attached with the triangular vermilion flap used to create a Cupid's bow.
(C) The prolabial flap is developed. The lateral lip elements are discarded and the mucosal flaps are turned over to create a labial sulcus.
(D) The lower lateral cartilages are freed from the overlying nasal skin from the base of the ala and columella. The nasal domes are approximated to each other and the cartilages are suspended from the upper lateral cartilages.
(E) The series of interdigitating bundles of the orbicularis muscle are approximated to each other.
(F) The skin is inset with a series of fine nylon sutures, which are removed 5-7 days postoperatively if a skin adhesive is not used. Xeroform gauze bolsters are placed as a temporary nasal stent.
A number of surgical procedures with many variations for the repair of bilateral cleft lip are well described. Among these are the repairs of Veau, Tennison, Manchester, Millard, and others. The Veau III operation is a straight-line closure without elevation of the prolabial skin and correspondingly without any attempt at restoring the continuity of the orbicularis oris. The central cupid's bow and tubercle is constructed from the vermilion of the lateral lip elements. In contrast, Millard's repair involved complete elevation of the prolabium and reconstitution of the orbicularis across the premaxilla. In addition, Millard banked lateral segments of the prolabium as "forked flaps" that were meant to add columellar height at a later stage. As with Veau, the central vermilion is recreated from the lateral lip elements.
Unlike Veau and Millard, Manchester preferred to maintain the prolabial vermilion to create the cupid's bow and tubercle, but similarly to Veau, Manchester's repair did not involve repairing the orbicularis, as he felt this would create an overly tight lip. In recent years, significant contributions by McComb, Mulliken, Nakajima, and Cutting have integrated the correction of the associated nasal deformity with simultaneous lip repair that appears to achieve adequate primary columellar lengthening and nasal tip projection.9,10,11,12,13,14 More recently, McComb's experience led him to stage the repair.9 The initial stage involves approximating and repositioning the splayed alar cartilages through a V-Y nasal tip "gull-wing" incision that allows redraping the overlying skin with a simultaneous bilateral lip adhesion. A definitive lip repair follows at a second stage.
Mulliken's extensive experience has evolved from a median nasal tip incision for exposure to bilateral rim incisions that allow adequate access to correct the nasal cartilage deformity.15 Nakajima and Cutting have introduced presurgical molding of the nasal tip and columellar with acrylic outriggers attached to a palatal appliance.
· Use general anesthesia with a noncuffed Oral RAE endotracheal tube positioned midline. Typically, the otolaryngologist then examines the ears; if needed, myringotomy and pressure equalizing tubes are placed.
· Prior to infiltration with a local anesthetic (0.5% lidocaine with 1:200,000 epinephrine), tattoo the anatomic landmarks with a methylene blue dye and mark the proposed incisions.
· Two key elements are involved in the preoperative skin marking for the elevation of two crucial flaps (the prolabial flap and lateral lip advancement flaps). Make the prolabial flap in a trapezoid shape, with the peak of the Cupid's bow between 2-2.5 mm on either side of the midline. The flap narrows superiorly to the columella base. On the lateral lip element, the peak of the Cupid's bow is determined where the dry vermilion is maximal in width before it tapers off superiorly. Mark the incision of the lateral lip along the vermilion mucosa cutaneous junction to the ala base. The incision continues vertically intranasally along the mucocutaneous junction. Design the vermilion of the lateral lip flaps to fit into the inferior edge of the prolabial flap and to each other in the midline, forming a tubercle. The white roll should be included with the vermilion flaps.
· Elevate the prolabial flap to the base of the columella. The lateral cutaneous elements are primarily discarded, except at the columella base, where it is tailored to reconstruct the nasal sill (not as banked fork flaps). The mucosa is then turned down to create the labial sulcus.
· Elevate the lateral lip flaps by incising the marking just above the white roll through the full thickness of the lip. Identify the orbicularis oris muscle and separate it from the overlying skin and underlying labial mucosa. The muscle is divided inferiorly to allow it to accompany the vermilion flap. Make an upper buccal incision above the attached gingival to allow medial mobilization of the labial mucosa.
· Free the alar bases from their attachments to the piriform region to allow medial and inferior mobilization of the ala and the corresponding transversus nasi muscles (to prevent alar flare).
· The lower lateral alar cartilages are freed from the overlying nasal skin through an infracartilaginous incision laterally and medially. The domes are approximated to each other with intradomal sutures, and the complex is suspended from the upper lateral cartilages with temporary fixation sutures.
· Reconstructing the lip begins with creating the labial sulcus by approximating the labial mucosa of the lateral lip elements to the turned over prolabial mucosa. To approximate the orbicularis oris muscle, it is divided into bundles and interdigitated with its opposing element with a series of sutures. The alar bases are then set into place (inferior and medial) to the nasal spine. Approximate the prolabial skin flap and lateral lip flaps either with dermal sutures or in combination with an adhesive. The vermilion flaps are tailored to create a central tubercle. Tailor the flaps at the nasal sill and then close the alar and intranasal incisions.
· Place the Xeroform bolsters and nasal stents. Apply a topical antibiotic ointment to the lip.
Postoperative Details
· Oral feedings: For a child who is breastfed, the authors encourage uninterrupted breastfeeding after surgery. Bottle-fed children can resume feedings immediately following surgery with a crosscut nipple. Some centers still advocate having the child use a soft catheter tip syringe for 10 days following surgery, followed by resumption of normal nipple bottle feeding. The authors have found this degree of caution to be unnecessary.
· Activities: The authors instruct the parents to avoid giving the child pacifiers or toys with pointed edges for 2 weeks after surgery. No other restrictions on activity are necessary. Some centers advocate the use of Velcro elbow immobilizers on the patient for 10 days following surgery to minimize the risk of inadvertent injury to the lip repair. These are periodically removed during the day under supervision.
· Lip care: Any exposed suture line, at the base of the nose and lip, should be cleaned using cotton swabs with diluted hydrogen peroxide several times a day, followed by the liberal application of topical antibiotic ointment. The authors then remove any permanent sutures 5-7 days after surgery. If cyanoacrylate adhesive is used, no additional care is required in the immediate postoperative period until the adhesive film comes off. The parents are told to expect noticeable scar contracture, erythema, and firmness for about 4-6 weeks postsurgery and that this gradually begins to improve 3-12 months after the procedure. The authors typically instruct parents to massage the upper lip during this phase and to avoid placing the child in direct sunlight until the scar matures.
Follow-up
Following cleft lip repair, patients are evaluated periodically by the various cleft team members. Oral hygiene and proper dental care need to be emphasized. Psychosocial evaluation and treatment should be made available. Follow-up appointments with speech pathologists should be continued until normal or near normal speech is achieved. Close cooperation among the members of the cleft team is necessary for optimal outcomes.
Complications
Aside from an unsatisfactory appearance of the surgical result, possible complications include dehiscence of the repair, hypertrophic scar formation, or contracture of the lip scars. If dehiscence occurs, re-operation is postponed until the induration has subsided completely. In the intervening period, control of the premaxilla with orthodontic devices to prevent rotation caused by asymmetric forces may be needed. With lip scars that remain red, thick, and contracted, the authors use an occlusive tape dressing and, occasionally, Kenalog-10 (triamcinolone acetonide) injection and/or flurandrenolide tape. For most repairs, the observed contracture is part of normal healing and improves with time. Wait to perform intervention until the lip scar matures (generally 1 y), and the intervention should be guided by the severity of the residual deformity with the goal of minimizing the number of revisions.
A number of secondary deformities of a less than ideal outcome are well characterized. These primarily include the whistle deformity of the lip, a vertically deficient upper lip, a constricted lip, and muscular diastasis. Correction of these residual deformities must be specifically tailored and range from minor revisions to fully re-creating the defect and reconstructing each of the elements of the lip to discarding the prolabial element and replacing it with a lower lip Abbe reconstruction.
Outcome and Prognosis
While maintaining symmetry typically is not the main issue in reconstructing the bilateral cleft lip, the outcome can be less satisfying than with unilateral clefts. Careful preoperative assessment of the cleft lip deformity and attention to appropriate presurgical management and detail in the reconstruction typically results in an acceptable repair that achieves some of the characteristics of the natural lip and nose. Many variables are involved beyond the technical aspects of a particular repair. Ultimately, the outcome depends on the natural course of uncomplicated healing of the initial repair, alignment of the skeletal framework on which the lip rests, and the differential effect of normal growth and development on the operated lip.
While a poor initial result is unlikely to improve with time, an excellent initial result may require some revision because of uncontrolled variables. Moreover, while the lip repair may be acceptable, additional procedures to achieve nasal symmetry are commonly required, despite the initial primary nasal surgery incorporated as an integral part of lip repair. Realistically, one must realize that, despite physicians' best attempts, the stigmata of a bilateral cleft deformity remains in many children.
Future and Controversies
Bilateral cleft lip surgery has evolved from discarding the premaxillary and prolabial elements in a simple approximation of the cleft margins to, currently, a definitive single-stage lip and primary cleft nasal repair that incorporates the underlying musculature. Accompanying the evolution of the surgical repair is the increasingly important involvement of early presurgical alveolar and nasal molding to possibly improve surgical outcome.
The basics of cleft surgery are to achieve a good philtrum size, shape, and positioning of the cartilages, and muscular continuity. Although the basics are the same, the development of presurgical techniques continue to evolve and provide an exciting scaffold to the management of bilateral cleft lip.
The presence of bilateral cleft lip and palate has the potential to significantly alter facial form and structure. The central third of the face is distorted by the bilateral cleft, and restoring the normal facial form is one of the primary goals for the reconstructive surgeon.
· Craniofacial, Bilateral Cleft Nasal Repair
· Craniofacial, Cleft Palate
· Craniofacial, Cleft Palate Repair
· Craniofacial, Unilateral Cleft Lip Repair
· Craniofacial, Unilateral Cleft Nasal Repair
History of the Procedure
The history of bilateral cleft lip repair has evolved from discarding the premaxilla and prolabium and approximating the lateral lip elements to a definitive lip and primary cleft nasal repair utilizing the underlying musculature. Accompanying the evolution of surgical repair is the increasingly important role of orthodontic support with early presurgical alveolar and nasal molding.1 Repositioning the maxillary and alveolar segments into a more anatomic position allows the surgeon to repair the lip and associated nasal deformity under more optimal conditions. The reader is referred to the references cited for a more detailed historical review of individual repairs and supportive management.
Problem
The cleft affects the obvious facial form as an anatomic deformity and has functional consequences, affecting the child's ability to eat, speak, hear, and breathe. Consequently, rehabilitation of a child born with a facial cleft must involve a multidisciplinary approach and be staged appropriately with the child's development. The need for intervention must be balanced against its subsequent effect on normal growth. In the child born with a bilateral cleft, the surgeon initially is faced with a protrusive premaxilla and the difficulty of achieving adequate columellar length and vertical height to the lip during reconstruction.
Bilateral cleft lip repair. (A) The prolabial width is typically set at 4-5 mm. (B) The prolabial flap is elevated to the base of the columella. The adjacent flaps are turned over to create a labial sulcus. (C) The orbicularis oris muscle, dissected from the overlying skin, is approximated across the midline. (D) The skin is approximated, and the Cupid's bow is created from the lateral vermilion flaps.
Frequency
The overall occurrence of cleft lip with or without cleft palate is approximately 1 in 750-1000 live births, making it the second most common congenital deformity (after club foot). Incidence varies by race, with clefts occurring more commonly in Asians (1 in 500 births), less frequently in Caucasians (1 in 750 births), and even less frequently in African Americans (1 in 2000 births). These racial variations in incidence are not observed with the isolated cleft palate. In terms of gender, the incidence of cleft lip/palate is more common in males, while the incidence of cleft palate alone is more common in females.
The most common presentation is cleft lip and palate (approximately 45%), followed by cleft palate alone (35%) and cleft lip alone (approximately 20%). Unilateral cleft lips are more common than bilateral cleft lips and occur more commonly on the left side (left cleft lip:right cleft lip:bilateral cleft lip - 6:3:1). In a large series reported in the literature, bilateral clefts are observed in approximately 15% of patients with clefts.2,3,4
Bilateral clefts occur in approximately 1 in 5000-6500 births based on the overall occurrence. According to CDC estimates, the overall incidence of orofacial clefts is 1 in 596, with the highest incidences occurring in Native Americans (3.6 in 1000) and the lowest in African Americans.5,6
According to the American Society of Plastic Surgeons (ASPS), 18,859 cleft lip and palate reconstructive procedures were performed in the United States in 2007.7 These statistics include procedures performed by ASPS members as well as other board-certified physicians most likely to perform plastic surgery procedures.
Etiology
Clefting is a multifactorial issue, with both genetic and environmental causes cited. The observation of clustered cases of facial clefts in a particular family indicates a genetic basis. Clefting of the lip and/or palate is associated with more than 150 syndromes. The overall incidence of associated anomalies (eg, cardiac) is approximately 30% (more common with isolated cleft palate).
Environmental causes, such as viral infection (eg, rubella) and teratogens (eg, steroids, anticonvulsants), during the first trimester of pregnancy have been linked to facial clefts. The risk also increases with parental age, especially when older than 30 years, with the father's age appearing to be a more significant factor than the mother's age. Nevertheless, most presentations are of isolated patients within the family without an obvious etiology.
Midfacial development involves several sets of genes, including those involved in cell patterning, proliferation, and signaling. Mutations in any of these genes can change the developmental process and contribute to cleft development. Some of these genes include the DIX gene, sonic hedgehog (SHH) gene, transforming growth factor (TGF) alpha and beta, and interferon regulatory factor (IRF6) gene.
Pathophysiology
While the normal embryologic development of the face is detailed in Head and Neck Embryology, a brief outline relevant to the formation of facial clefts follows.
In short, the branchial arches are responsible for the formation of several areas, including the mouth and lip. Mesenchymal migration and fusion occurs during weeks 4-7 of gestation. The first branchial arch is responsible for the formation of the maxillary and mandibular processes. The maxillary and mandibular prominences form the lateral borders of the primitive mouth or stomodeum.
Mesenchymal migration and fusion of the primitive somite-derived facial elements (central frontonasal, 2 lateral maxillary, mandibular processes), at 4-7 weeks gestation, are necessary for the normal development of embryonic facial structures. When migration and fusion are interrupted for any reason, a facial cleft develops along embryonic fusion lines. The embryonic development of the primary palate (lip and palate anterior to the incisive foramen) differs from the secondary palate (palate posterior to the incisive foramen).
The developing processes of the medial nasal prominence, lateral nasal prominence, and maxillary prominences form the primary palate. Fusion occurs, followed by "streaming" of mesodermal elements derived from the neural crest. In contrast, the secondary palate is formed by the fusion of palatal processes of the maxillary prominence alone. The difference in embryonic development suggests the possibility of differing degrees of susceptibility to genetic and environmental influences and accounts for the observed variation in incidences. In summary, a bilateral cleft lip results from the failure of fusion of the medial nasal prominences with the maxillary prominences bilaterally.
Kernahan developed a classification scheme in which the defect can be classified onto a Y-shaped symbol. In this diagram, the incisive foramen is represented as the focal point. This system has been applied to both cleft lip and palate.
Millard modification of Kernahan striped-Y classification for cleft lip and palate. The small circle indicates the incisive foramen; the triangles indicate the nasal tip and nasal floor.
Presentation
As with the unilateral cleft, a wide range of clinical presentations of the bilateral cleft lip and palate is possible, from the simple microform cleft to the complete cleft bilaterally involving the lip, alveolus, palate, and nose. Bilateral cleft lip is typically classified as either complete or incomplete.
In addition, the cleft may present with varying degrees of asymmetric involvement of the lip and palate. Any degree of combination of involvement of the lip, primary palate, and secondary palate can exist. Recognizing these potentially asymmetric variations is essential in planning operative treatment and evaluating postoperative results.
In the complete form of clefts, the premaxilla is unrestrained by the lateral maxillary segments and the vomer grows anterior to the lateral segments. This distorts the alar cartilages away from the nasal tip and stretches the alae across a widening cleft. The columella is significantly deficient, can be virtually nonexistent and without a distinct demarcation from the prolabium, which also is vertically deficient. No prolabial muscle, philtral columns, dimples, or Cupid's bow is present. No nasal floor is present, as the cleft continues through the palatal shelf along either side of the vomer through the soft palate.
Further treatment planning
Orthodontic treatment can be initiated a few weeks following birth, prior to surgical intervention. Other adjunct procedures include lip adhesion, presurgical orthopedics, primary nasal correction, and nasoalveolar molding. These procedures attempt to reduce the deformity. Nasoalveolar molding is the active molding and repositioning of the nasal cartilage and alveolar processes with an appliance. This orthodontic intervention takes advantage of the plasticity of the cartilage.
Presurgical nasal alveolar molding allows repositioning of the maxillary alveolus and surrounding soft tissues in hopes of reducing wound tension and improving results. Definitive repair is delayed until approximately 3 months of age, depending on physician comfort. A multidisciplinary approach should be carried out over a period of several years for patients with unilateral cleft lip. This multidisciplinary treatment team should include specialists in audiology, ENT, and speech therapy, among others.
The key difference between the treatment of unilateral versus bilateral cleft lips centers around the concept of aligning the 3 maxillary segments (the lateral lip segments with the prolabial segments). Presurgical orthopedics may be used to prevent the maxillary segments and premaxilla from collapse.
Presurgical orthopedics can involve the following:
· Finger massage of prolabium
· Pressure tape on prolabium
· Intraoral fixation devices
· Lip adhesion (can be used to achieve symmetry in asymmetric bilateral cleft situations)
· Nasoalveolar molding
The most common appliances used are nasoalveolar molds and intraoral fixation devices. Botulinum toxin is currently being investigated to decrease tension in cleft repair.
In general, bilateral cleft lip repair techniques have been modified from unilateral cleft lip repair techniques. If the repair is staged, the more severe side is repaired first, and the second side is repaired approximately 3-6 months later.
Indications
Patients born with a cleft lip should undergo surgical repair unless otherwise contraindicated. The goal of reconstruction is to establish normal morphologic facial form and function in order to provide the optimal conditions for the development of dentition, mastication, hearing, speech, breathing, and psychosocial status.
Relevant Anatomy
Understanding normal lip and nasal anatomy is essential to recognize the distortion caused by a facial cleft. The elements of the normal lip are the central philtrum, which is demarcated laterally by the philtral columns and inferiorly by the cupid's bow and tubercle. Just above the junction of the vermilion-cutaneous border is a mucocutaneous ridge referred to as the white roll. Within the red vermilion of the lip are 2 distinct areas: the dry vermilion (the more keratinized portion of the lip that is exposed to air) and the wet vermilion (exposed to the moist environment of the labial mucosa).
The primary muscle of the lip is the orbicularis oris. It has 2 well-defined components: the deep (internal) layer and the superficial (external) layer. The deep fibers run circumferentially from commissure (modiolus) to commissure (modiolus) and function as the primary sphincter for oral feeding. The superficial fibers run obliquely, interdigitating with the other muscles of facial expression to terminate in the dermis, and function to provide subtle shades of expression and precise movements of the lip for speech.
The superficial fibers of the orbicularis decussate in the midline and insert into the skin lateral to the opposite philtral groove, forming the philtral columns. The resulting philtral dimple is centrally depressed, as no muscle fibers directly insert into the dermis in the midline. The tubercle of the lip is shaped by the pars marginalis, the portion of the orbicularis along the vermilion forming the tubercle of the lip with eversion of the muscle.
In the upper lip, the levator labii superioris contributes to the form of the lip. Its fibers arise from the medial aspect of the infraorbital rim and sweep down to insert near the vermilion cutaneous junction. The most medial fibers of the levator labii superioris sweep down to insert near the corner of the ipsilateral philtral column and vermilion-cutaneous junction, helping to define the lower philtral column and the peak of the Cupid's bow.
The nasal muscles are equally important. The levator superioris alaeque arises along the frontal process of the maxilla and courses inferiorly to insert on the mucosal surface of the lip and ala. The transverse nasalis arises along the nasal dorsum and sweeps around the ala to insert along the nasal sill from lateral to medial into the incisal crest and anterior nasal spine. These fibers join with the oblique fibers of the orbicularis and the depressor septi (nasalis), which arises from the alveolus between the central and lateral incisors to insert into the skin of the columellar to the nasal tip and the footplates of the medial crura.
A bilateral cleft thus disrupts the normal termination of the muscle fibers that cross the embryologic fault line of the maxillary and nasal processes, resulting in symmetric but abnormal muscular forces between the normal equilibrium that exists with the nasolabial and oral groups of muscles. With an unrestrained premaxilla, the deformity accentuates with differential growth of the various elements. The alar cartilages are splayed apart and rotate caudally, subluxed from the normal position. Consequently, the nasal tip broadens, the columellar is foreshortened, and the alar bases rotate laterally and cephalad.
Workup
Laboratory Studies
· Perform a thorough physical examination, not limited to the head and neck region, to uncover associated anomalies in the infant presenting with a unilateral cleft lip with or without a palatal cleft. Additional workup is determined by physical findings that suggest involvement of other organ systems.
· The child's weight, oral intake, and growth and/or development are of primary concern and must be followed closely.
· Routine laboratory studies typically are not required, other than a hemoglobin study shortly before the planned lip repair.
· Routine imaging is not indicated in a healthy patient with isolated cleft lip.
Treatment
Surgical Therapy
Children born with a facial cleft benefit from a multidisciplinary approach in a team-based setting so that all aspects of their care can be coordinated efficiently.
Beyond lip repair are hearing, speech, dental, and psychosocial integration issues. These should be addressed throughout the child's growth and development from birth through adolescence. These issues are as important as the anatomic reconstruction. Ultimately, the functional outcome of the reconstruction depends on cooperation between the surgeon, orthodontist, and speech pathologist.
Each specialty involved must evaluate the child individually, formulate a treatment plan, and integrate it with those of the other specialties based on the child's needs. The Parameters of Care Guidelines established by the American Cleft Palate Craniofacial Association should be followed.8 However, rather than strictly adhering to one protocol, physicians should assess each child individually and formulate the treatment plan based on the team's experience, its overall philosophy of treatment, and available resources.
Preoperative Details
While the lip repair is the initial focus for many parents, treatment begins by assessing the child's nutritional status and assisting the parents with oral feeding techniques so that appropriate weight gain occurs. Parents who suddenly are faced with caring for a child with a facial cleft can be overwhelmed. The importance of spending sufficient time with them to allay their fears, to discuss staging and timing of reconstruction, to stress the need for involvement of other specialists, and to stress the importance of long-term and consistent follow-up care from birth through adolescence cannot be overemphasized.
No agreement is found in the literature regarding the ideal timing of lip repair. Some have advocated surgery in the early neonatal period, with a theoretical benefit in scar appearance and nasal cartilage adaptability, thus minimizing the nasal deformity. To minimize anesthetic risks, some still adhere to the rule of 10s: perform surgical repair of cleft lip when the child has a hemoglobin level of 10 g, weight of 10 lb, and is aged 10 weeks. In general, most centers prefer to perform the lip reconstruction when the patient is aged 2-4 months; anesthesia risks are lower, the child is better able to withstand the stress of surgery, and lip elements are larger and allow for a meticulous reconstruction.
A presurgical, fabricated, passive, intraoral orthodontic palatal appliance can be used to maintain the arch width to prevent the nearly inevitable collapse that occurs with lip repair. Rarely is there an indication for active expansion of the maxillary segment. This passive plating and gentle traction is an integral component of surgical cleft lip repair. The repair reestablishes the soft tissue and muscular forces on the easily moldable maxillary arch segments. Recently, the palatal appliance has been modified to include a nasal extension to help improve the nasal tip form. The orthodontist takes impressions, and the custom appliance is fitted as soon as possible after birth and well before the lip repair.
The appliance also aids in the child's oral feeding, helping to decrease nasal regurgitation and assisting oral suction. Some centers have chosen either no presurgical orthopedic intervention or an active pin-based appliance (eg, Latham) to align the maxillary arch segments. For bilateral clefts, external pressure is routinely used to help maintain the premaxillary component within the arch alignment. Soft elastic tape (eg, 3M Microfoam tape) across the premaxilla, a head cap with elastic traction, or lip adhesion can be used prior to a definitive lip repair once the arch segments have approximated.
Intraoperative Details
The ideal lip repair results in symmetrically shaped nostrils, nasal sill, and alar bases; adequate columellar length; a well-defined philtral dimple and columns; a natural-appearing cupid's bow with a pout to the vermilion tubercle; and an adequate labial sulcus. In addition, lip scars are used to approximate the natural landmarks. The ideal repair results in functional muscle repair that with animation that mimics a normal lip.
Intraoperative technique.
(A) The anatomic landmarks are tattooed and the planned incisions are marked.
(B) The orbicularis is dissected from the overlying skin and divided into bundles to allow interdigitation with its opposing element. Inferiorly, an element of the muscle is left attached with the triangular vermilion flap used to create a Cupid's bow.
(C) The prolabial flap is developed. The lateral lip elements are discarded and the mucosal flaps are turned over to create a labial sulcus.
(D) The lower lateral cartilages are freed from the overlying nasal skin from the base of the ala and columella. The nasal domes are approximated to each other and the cartilages are suspended from the upper lateral cartilages.
(E) The series of interdigitating bundles of the orbicularis muscle are approximated to each other.
(F) The skin is inset with a series of fine nylon sutures, which are removed 5-7 days postoperatively if a skin adhesive is not used. Xeroform gauze bolsters are placed as a temporary nasal stent.
A number of surgical procedures with many variations for the repair of bilateral cleft lip are well described. Among these are the repairs of Veau, Tennison, Manchester, Millard, and others. The Veau III operation is a straight-line closure without elevation of the prolabial skin and correspondingly without any attempt at restoring the continuity of the orbicularis oris. The central cupid's bow and tubercle is constructed from the vermilion of the lateral lip elements. In contrast, Millard's repair involved complete elevation of the prolabium and reconstitution of the orbicularis across the premaxilla. In addition, Millard banked lateral segments of the prolabium as "forked flaps" that were meant to add columellar height at a later stage. As with Veau, the central vermilion is recreated from the lateral lip elements.
Unlike Veau and Millard, Manchester preferred to maintain the prolabial vermilion to create the cupid's bow and tubercle, but similarly to Veau, Manchester's repair did not involve repairing the orbicularis, as he felt this would create an overly tight lip. In recent years, significant contributions by McComb, Mulliken, Nakajima, and Cutting have integrated the correction of the associated nasal deformity with simultaneous lip repair that appears to achieve adequate primary columellar lengthening and nasal tip projection.9,10,11,12,13,14 More recently, McComb's experience led him to stage the repair.9 The initial stage involves approximating and repositioning the splayed alar cartilages through a V-Y nasal tip "gull-wing" incision that allows redraping the overlying skin with a simultaneous bilateral lip adhesion. A definitive lip repair follows at a second stage.
Mulliken's extensive experience has evolved from a median nasal tip incision for exposure to bilateral rim incisions that allow adequate access to correct the nasal cartilage deformity.15 Nakajima and Cutting have introduced presurgical molding of the nasal tip and columellar with acrylic outriggers attached to a palatal appliance.
· Use general anesthesia with a noncuffed Oral RAE endotracheal tube positioned midline. Typically, the otolaryngologist then examines the ears; if needed, myringotomy and pressure equalizing tubes are placed.
· Prior to infiltration with a local anesthetic (0.5% lidocaine with 1:200,000 epinephrine), tattoo the anatomic landmarks with a methylene blue dye and mark the proposed incisions.
· Two key elements are involved in the preoperative skin marking for the elevation of two crucial flaps (the prolabial flap and lateral lip advancement flaps). Make the prolabial flap in a trapezoid shape, with the peak of the Cupid's bow between 2-2.5 mm on either side of the midline. The flap narrows superiorly to the columella base. On the lateral lip element, the peak of the Cupid's bow is determined where the dry vermilion is maximal in width before it tapers off superiorly. Mark the incision of the lateral lip along the vermilion mucosa cutaneous junction to the ala base. The incision continues vertically intranasally along the mucocutaneous junction. Design the vermilion of the lateral lip flaps to fit into the inferior edge of the prolabial flap and to each other in the midline, forming a tubercle. The white roll should be included with the vermilion flaps.
· Elevate the prolabial flap to the base of the columella. The lateral cutaneous elements are primarily discarded, except at the columella base, where it is tailored to reconstruct the nasal sill (not as banked fork flaps). The mucosa is then turned down to create the labial sulcus.
· Elevate the lateral lip flaps by incising the marking just above the white roll through the full thickness of the lip. Identify the orbicularis oris muscle and separate it from the overlying skin and underlying labial mucosa. The muscle is divided inferiorly to allow it to accompany the vermilion flap. Make an upper buccal incision above the attached gingival to allow medial mobilization of the labial mucosa.
· Free the alar bases from their attachments to the piriform region to allow medial and inferior mobilization of the ala and the corresponding transversus nasi muscles (to prevent alar flare).
· The lower lateral alar cartilages are freed from the overlying nasal skin through an infracartilaginous incision laterally and medially. The domes are approximated to each other with intradomal sutures, and the complex is suspended from the upper lateral cartilages with temporary fixation sutures.
· Reconstructing the lip begins with creating the labial sulcus by approximating the labial mucosa of the lateral lip elements to the turned over prolabial mucosa. To approximate the orbicularis oris muscle, it is divided into bundles and interdigitated with its opposing element with a series of sutures. The alar bases are then set into place (inferior and medial) to the nasal spine. Approximate the prolabial skin flap and lateral lip flaps either with dermal sutures or in combination with an adhesive. The vermilion flaps are tailored to create a central tubercle. Tailor the flaps at the nasal sill and then close the alar and intranasal incisions.
· Place the Xeroform bolsters and nasal stents. Apply a topical antibiotic ointment to the lip.
Postoperative Details
· Oral feedings: For a child who is breastfed, the authors encourage uninterrupted breastfeeding after surgery. Bottle-fed children can resume feedings immediately following surgery with a crosscut nipple. Some centers still advocate having the child use a soft catheter tip syringe for 10 days following surgery, followed by resumption of normal nipple bottle feeding. The authors have found this degree of caution to be unnecessary.
· Activities: The authors instruct the parents to avoid giving the child pacifiers or toys with pointed edges for 2 weeks after surgery. No other restrictions on activity are necessary. Some centers advocate the use of Velcro elbow immobilizers on the patient for 10 days following surgery to minimize the risk of inadvertent injury to the lip repair. These are periodically removed during the day under supervision.
· Lip care: Any exposed suture line, at the base of the nose and lip, should be cleaned using cotton swabs with diluted hydrogen peroxide several times a day, followed by the liberal application of topical antibiotic ointment. The authors then remove any permanent sutures 5-7 days after surgery. If cyanoacrylate adhesive is used, no additional care is required in the immediate postoperative period until the adhesive film comes off. The parents are told to expect noticeable scar contracture, erythema, and firmness for about 4-6 weeks postsurgery and that this gradually begins to improve 3-12 months after the procedure. The authors typically instruct parents to massage the upper lip during this phase and to avoid placing the child in direct sunlight until the scar matures.
Follow-up
Following cleft lip repair, patients are evaluated periodically by the various cleft team members. Oral hygiene and proper dental care need to be emphasized. Psychosocial evaluation and treatment should be made available. Follow-up appointments with speech pathologists should be continued until normal or near normal speech is achieved. Close cooperation among the members of the cleft team is necessary for optimal outcomes.
Complications
Aside from an unsatisfactory appearance of the surgical result, possible complications include dehiscence of the repair, hypertrophic scar formation, or contracture of the lip scars. If dehiscence occurs, re-operation is postponed until the induration has subsided completely. In the intervening period, control of the premaxilla with orthodontic devices to prevent rotation caused by asymmetric forces may be needed. With lip scars that remain red, thick, and contracted, the authors use an occlusive tape dressing and, occasionally, Kenalog-10 (triamcinolone acetonide) injection and/or flurandrenolide tape. For most repairs, the observed contracture is part of normal healing and improves with time. Wait to perform intervention until the lip scar matures (generally 1 y), and the intervention should be guided by the severity of the residual deformity with the goal of minimizing the number of revisions.
A number of secondary deformities of a less than ideal outcome are well characterized. These primarily include the whistle deformity of the lip, a vertically deficient upper lip, a constricted lip, and muscular diastasis. Correction of these residual deformities must be specifically tailored and range from minor revisions to fully re-creating the defect and reconstructing each of the elements of the lip to discarding the prolabial element and replacing it with a lower lip Abbe reconstruction.
Outcome and Prognosis
While maintaining symmetry typically is not the main issue in reconstructing the bilateral cleft lip, the outcome can be less satisfying than with unilateral clefts. Careful preoperative assessment of the cleft lip deformity and attention to appropriate presurgical management and detail in the reconstruction typically results in an acceptable repair that achieves some of the characteristics of the natural lip and nose. Many variables are involved beyond the technical aspects of a particular repair. Ultimately, the outcome depends on the natural course of uncomplicated healing of the initial repair, alignment of the skeletal framework on which the lip rests, and the differential effect of normal growth and development on the operated lip.
While a poor initial result is unlikely to improve with time, an excellent initial result may require some revision because of uncontrolled variables. Moreover, while the lip repair may be acceptable, additional procedures to achieve nasal symmetry are commonly required, despite the initial primary nasal surgery incorporated as an integral part of lip repair. Realistically, one must realize that, despite physicians' best attempts, the stigmata of a bilateral cleft deformity remains in many children.
Future and Controversies
Bilateral cleft lip surgery has evolved from discarding the premaxillary and prolabial elements in a simple approximation of the cleft margins to, currently, a definitive single-stage lip and primary cleft nasal repair that incorporates the underlying musculature. Accompanying the evolution of the surgical repair is the increasingly important involvement of early presurgical alveolar and nasal molding to possibly improve surgical outcome.
The basics of cleft surgery are to achieve a good philtrum size, shape, and positioning of the cartilages, and muscular continuity. Although the basics are the same, the development of presurgical techniques continue to evolve and provide an exciting scaffold to the management of bilateral cleft lip.
Craniofacial, Unilateral Cleft Lip Repair
Introduction
The presence of unilateral cleft lip is one of the most common congenital deformities. A broad spectrum of variations in clinical presentation exists. Unilateral cleft lip involves deformity of the lip in addition to the alveolus and nose. Patients with this deformity require short-term care and long-term care and follow-up from practitioners in multiple specialties. Patients may need multiple surgical interventions, from infancy to adulthood, in order to achieve necessary function and aesthetic quality.
No universal agreement has been reached as to the timing and technique of repair. Several methods are used with comparable long-term results, which serves as an indication that more than one treatment option exists for definitive repair. Treatment goals include the restoration of facial appearance and oral function, improvement of dental skeletal and occlusal relationships, improvement of speech, and the psychosocial state.
History of the Procedure
Hippocrates (400 BC) and Galen (150 AD) mention cleft lip, but not cleft palate in their writings. For centuries, perforations of the palate were considered to be secondary to syphilis, and cleft palate was not recognized as a congenital disorder until 1556, by Fanco. The first successful closure of a soft palate defect was reported in 1764 by LeMonnier, a French dentist. The first closure of the hard palate was performed in 1834 by Dieffenbach. In the 1930's, Kilner and Wardill independently developed the "pushback" procedure. In 1843, closure of the unilateral cleft lip with local flaps was described by Malgaigne. The following year, Mirault modified Malgaigne’s technique by using the lateral lip flap to fill the medial defect. All future methods of cleft lip closure are based on Mirault’s technique. LeMesurier and Tennyson modified this technique with a quadrilateral and triangular flap, respectively. In 1976, Millard published his definitive repair in which the lateral flap advancement into the upper portion of lip was combined with downward rotation of medial lip. Other modifications have been published by Noordhoff, Mohler, and Onizuka. Fisher has described an anatomical subunit approximation for definitive cleft lip repair. Millard’s methods, including variations, remain among the most popular method for unilateral cleft lip closure.
Cleft lip surgery has evolved from a simple adhesion of paired margins of the cleft to an understanding of the various malpositioned elements of the lip to a more complicated geometric reconstruction using transposition, rotation, and advancement flaps.
Problem
The cleft affects the facial form as an anatomic deformity and has functional consequences. These include the child's ability to eat, speak, hear, and breathe. Consequently, rehabilitation of a child born with a facial cleft must involve a multidisciplinary approach and staged appropriately with the child's development, balancing the timing of intervention against its effect on subsequent normal growth.
Incidence
The overall occurrence of cleft lip with or without cleft palate is approximately 1 in 750-1000 live births. Racial differences exist, with the incidence in Asians (1:500) greater than in Caucasians (1:750) greater than in African Americans (1:2000). The incidence of cleft lip/palate is more common in males.
The most common presentation is cleft lip and palate (approximately 45%), followed by cleft palate alone (35%) and cleft lip alone (approximately 20%). Unilateral cleft lips are more common than bilateral cleft lips and occur more commonly on the left side (left cleft lip: right cleft lip: bilateral cleft lip = 6:3:1).
The risk of a newborn having a cleft lip increases if a first-degree relative also has a cleft. If one child already has a cleft lip, the chance of a second child being born with the deformity is 4%. If a parent has a cleft lip, the chance of a newborn having a cleft is 7%. If both a parent and a sibling have a cleft lip, the newborn's risk rises to 15%.
Etiology
Clefting has a multifactorial basis, with both genetic and environmental causes cited. The observation of clustered cases of facial clefts in a particular family indicates a genetic basis. Clefting of the lip and/or palate is associated with more than 150 syndromes. The overall incidence of associated anomalies (eg, cardiac) is approximately 30% (more common with isolated cleft palate).
Environmental causes, such as viral infection (eg, rubella) and teratogens (eg, steroids, anticonvulsants), during the first trimester have been linked to facial clefts. The risk also increases with parental age, especially when older than 30 years, with the father's age appearing to be a more significant factor than the mother's age. Nevertheless, most presentations are of isolated patients within the family without an obvious etiology.
Midfacial development involves several sets of genes, including those involved in cell patterning, proliferation, and signaling. Mutations in any of these genes can change the developmental process and contribute to cleft development. Some of these genes include the DIX gene, sonic hedgehog (SHH) gene, transforming growth factor (TGF) alpha/beta, and interferon regulatory factor (IRF6).
Classification
Kernahan developed a classification scheme in which the defect can be classified onto a Y-shaped symbol.
Millard modification of Kernahan striped-Y classification for cleft lip and palate.
Pathophysiology
While the normal embryologic development of the face is detailed in Head and Neck Embryology, a brief outline relevant to the formation of facial clefts follows.
In short, the branchial arches are responsible for the formation of several areas, including the mouth and lip. Mesenchymal migration and fusion occurs during weeks 4-7 of gestation. The first branchial arch is responsible for the formation of the maxillary and mandibular processes. The maxillary and mandibular prominences form the lateral borders of the primitive mouth or stomodeum.
Mesenchymal migration and fusion of the primitive somite-derived facial elements (central frontonasal, 2 lateral maxillary, mandibular processes), at 4-7 weeks gestation, is necessary for the normal development of embryonic facial structures. When migration and fusion are interrupted for any reason, a facial cleft develops along embryonic fusion lines. The embryonic development of the primary palate (lip and palate anterior to the incisive foramen) differs from the secondary palate (palate posterior to the incisive foramen).
The developing processes of the medial nasal prominence, lateral nasal prominence, and maxillary prominences form the primary palate. Fusion occurs, followed by "streaming" of mesodermal elements derived from the neural crest. In contrast, the secondary palate is formed by the fusion of palatal processes of the maxillary prominence alone. The difference in embryonic development suggests the possibility of differing degrees of susceptibility to genetic and environmental influences and accounts for the observed variation in incidences.
In summary, unilateral cleft lip results from failure of fusion of the medial nasal prominence with the maxillary prominence.
Presentation
For treatment purposes, unilateral cleft lip can be placed into one of three categories: microform/forme fruste, incomplete, or complete cleft lip.
Microform cleft (forme fruste): This defect is characterized by a "light" furrow along the vertical length of the lip with a small vermilion notch and minor imperfections in the white roll. A small component of vertical lip length deficiency and associated nasal deformity may be present.
Incomplete cleft lip: This defect is characterized by the varying degree of vertical lip separation. By definition, it has an intact nasal sill, commonly termed the Simonart band.
Complete cleft lip: This involves the full-thickness defect of the lip and alveolus (primary palate), extends into the base of the nose (no Simonart band exists), and is often accompanied by a palatal cleft (secondary palate). The premaxilla is typically rotated outward and projects anteriorly in relation to a relatively retropositioned lateral maxillary alveolar element.
As a consequence of the clefting of the lip, an associated nasal deformity occurs. The structures of the ala base, nasal sill, vomer, and septum are distorted significantly. The lower lateral cartilage on the cleft side is positioned inferiorly, with an obtuse angle as it flattens across the cleft. The alar base is rotated outward. The developing nasal septum pulls the premaxilla away from the cleft, and the septum and the nasal spine are deflected toward the noncleft side. The cleft may continue through the maxillary alveolus and palatal shelf, extending to the palatal bone and soft palate.
Further treatment planning
Orthodontic treatment can be initiated a few weeks following birth, prior to surgical intervention. Other adjunct procedures include lip adhesion, presurgical orthopedics, primary nasal correction, and nasoalveolar molding. These procedures attempt to reduce the deformity. Nasoalveolar molding is the active molding and repositioning of the nasal cartilage and alveolar processes with an appliance.6 This orthodontic intervention takes advantage of the plasticity of the cartilage. Presurgical nasal alveolar allows repositioning of the maxillary alveolus and surrounding soft tissues in hopes of reducing wound tension and improving results.7,8
Definitive repair is delayed until approximately 3 months of age; this varies, depending on physician comfort. A multidisciplinary approach should be carried out over several years for patients with unilateral cleft lip. This team should include practitioners from audiology, otolaryngology, and speech therapy, among other specialities.
Indications
Patients born with a cleft lip should undergo surgical repair unless otherwise contraindicated. The goal of reconstruction is to establish normal morphologic facial form and function in order to provide the optimal conditions for the development of dentition, mastication, hearing, speech, and breathing, and psychosocial status.
Anatomy
Normal lip and nasal anatomy is essential for an understanding of the distortion caused by a facial cleft. The elements of the normal lip are composed of the central philtrum, demarcated laterally by the philtral columns and inferiorly by the Cupid's bow and tubercle. Just above the junction of the vermilion-cutaneous border is a mucocutaneous ridge frequently referred to as the white roll. Within the red vermilion of the lip is a noticeable junction demarcating the dry and wet vermilion, the increased keratinized portion of the lip that is exposed to air from the moist environment of the labial mucosa.
The primary muscle of the lip is the orbicularis oris, and it has two well-defined components: the deep (internal) and the superficial (external) components. The deep (internal) fibers run horizontally or circumferentially from commissure (modiolus) to commissure (modiolus) and functions as the primary sphincteric action for oral feeding. The superficial (external) fibers run obliquely, interdigitating with the other muscles of facial expression to terminate in the dermis. They provide subtle shades of expression and precise movements of the lip for speech.
The superficial fibers of the orbicularis decussate in the midline and insert into the skin lateral to the opposite philtral groove forming the philtral columns. The resulting philtral dimple centrally is depressed as there are no muscle fibers that directly insert into the dermis in the midline. The tubercle of the lip is shaped by the pars marginalis, the portion of the orbicularis along the vermilion forming the tubercle of the lip with eversion of the muscle.
In the upper lip, the levator labii superioris contributes to the form of the lip. Its fibers, arising from the medial aspect of the infraorbital rim, sweep down to insert near the vermilion cutaneous junction. The medial-most fibers of the levator labii superioris sweep down to insert near the corner of the ipsilateral philtral column and vermilion-cutaneous junction, helping to define the lower philtral column and the peak of the Cupid's bow.
The nasal muscles are equally important. The levator superioris alaeque arises along the frontal process of the maxilla and courses inferiorly to insert on the mucosal surface of the lip and ala. The transverse nasalis arises along the nasal dorsum and sweeps around the ala to insert along the nasal sill from lateral to medial into the incisal crest and anterior nasal spine. These fibers join with the oblique fibers of the orbicularis and the depressor septi (nasalis), which arises from the alveolus between the central and lateral incisors to insert into the skin of the columellar to the nasal tip and the footplates of the medial crura.
A unilateral cleft thus disrupts the normal termination of the muscle fibers that cross the embryologic fault line of the maxillary and nasal processes, resulting in symmetric but abnormal muscular forces between the normal equilibrium that exists with the nasolabial and oral groups of muscles. With an unrestrained premaxilla, the deformity accentuates with differential growth of the various elements. The alar cartilages are splayed apart and rotate caudally, subluxed from the normal position. Consequently, the nasal tip broadens, the columellar is foreshortened, and the alar bases rotate outwardly cephalad.
Workup
Laboratory Studies
· Perform a thorough physical examination, not limited to the head and neck region, to uncover associated anomalies in the infant presenting with a unilateral cleft lip with or without a palatal cleft. Additional workup is determined by physical findings that suggest involvement of other organ systems.
· The child's weight, oral intake, and growth and/or development are of primary concern and must be followed closely.
· Routine laboratory studies typically are not required, other than a hemoglobin study shortly before the planned lip repair.
· Routine imaging is not indicated in a healthy patient with isolated cleft lip.
Treatment
Surgical Therapy
Children born with a facial cleft benefit from multidisciplinary clinical care. This is a team-based approach allowing efficient coordination of all aspects of care. Beyond the lip repair are other issues such as hearing, speech, dental, and psychosocial integration. With the multidisciplinary approach, as the child grows, comprehensive care can be given from birth through adolescence. These associated issues are as important as the anatomic reconstruction, and ultimately the functional outcome of the reconstruction depends on addressing them.
Each specialty involved must evaluate the child individually and formulate a treatment plan, then the team forms a combined individual integrated protocol that follows the Parameters of Care Guidelines established by the American Cleft Palate Craniofacial Association. Rather than strictly adhering to any one protocol, each child is assessed based on the present need in his or her development, and a treatment plan is created based on the team's experience.
Preoperative Details
While the lip repair is the initial focus for many parents, treatment begins by assessing the child's nutritional status and assisting the parents with oral feeding techniques so that appropriate weight gain occurs.
Parents who suddenly are faced with caring for a child with a facial cleft are overwhelmed. The importance of spending sufficient time with them to allay their fears, to discuss staging and timing of reconstruction, to stress the need for involvement of other specialists, and to instruct them on the importance of long-term and consistent follow-up care from birth through adolescence cannot be overemphasized.
The optimal timing of the surgical repair is still somewhat controversial. Some centers have advocated surgery in the early neonatal period, with a theoretical benefit in the scar appearance and nasal cartilage adaptability, thus minimizing the nasal deformity. To minimize anesthetic risks, some still adhere to the rule of 10s: perform surgical repair of cleft lip when the child has a hemoglobin of 10 g, weight of 10 lb, and is aged 10 weeks. In general, however, most centers prefer to perform the unilateral lip repair when the infant is aged 2-4 months; anesthesia risks are lower, the child is better able to withstand the stress of surgery, and lip elements are larger and allow for a meticulous reconstruction.
Before the definitive lip surgery, cleft centers utilize lip taping, alone or in combination with a passive intraoral appliance or an active pin-based appliance (eg, Latham) to align the maxillary arch segments; or no presurgical orthopedic intervention at all. This choice depends on the center's protocol and resources.
A number of cleft centers prefer to use a passive intraoral orthodontic palatal appliance to maintain the arch width to prevent the nearly inevitable collapse that occurs with the lip surgery.6 The lip repair reestablishes the soft tissue and muscular forces on the easily moldable maxillary arch segments. Additionally, this appliance may include a nasal extension to help improve the nasal tip form. This nasal alveolar molding device is incorporated into the intraoral appliance. Several weeks of treatment prior to the surgery and regular adjustments are needed to mold the alar cartilages into a more favorable position, thus facilitating the surgical correction of the nasal deformity. Impressions are taken soon after birth so that the custom appliance can be applied as soon as possible before the lip repair. The appliance also assists in the child's oral feeding, helping to decrease nasal regurgitation and assisting oral suction.
Intraoperative Details
The ideal lip repair results in symmetrically shaped nostrils, nasal sill, and alar bases; a well-defined philtral dimple and columns; and a natural appearing Cupid's bow with a pout to the vermilion tubercle. In addition, it results in a functional muscle repair that with animation mimics a normal lip. While ideally the lip scars approximate natural landmarks, ultimately the eye first focuses on symmetry and then normal contours of the lip at rest and in animation.
A number of surgical procedures for the repair of a unilateral cleft lip are well described, with a multitude of variations, including the LeMesurier quadrilateral flap repair, Randall-Tennison triangular flap repair, Millard rotation-advancement repair, and Skoog and Kernahan-Bauer upper and lower lip Z-plasty repairs. Many other variations exist; of particular note are the repairs by Delaire and by Poole.
The Rose-Thompson repair involves curved or angled paring of the cleft margins to lengthen the lip as a straight-line closure.
Hagedorn-LeMesurier repair. The medial lip element is lengthened by introducing a quadrilateral flap developed from the lateral lip element.
Tennison-Randall repair. The medial lip element is lengthened by introducing a triangular flap from the inferior portion of the lateral lip element.
Skoog repair. The medial lip element is lengthened by introducing two small triangular flaps developed from the lateral lip element.
Each of these techniques ultimately has the common goal of achieving symmetry and restoring the continuity of the underlying orbicularis muscle. All attempt to lengthen the foreshortened philtrum on the cleft side by interposing tissue from the lateral lip element into the medial lip element through various combinations of rotation, advancement, and transposition flaps.
While none of the repairs is ideal, each has advantages and disadvantages, and each results in an excellent repair in experienced hands, underscoring the fact that more than a single acceptable technique, rather than a single ideal repair, is available. However, because of the limitations of this article, the authors choose to focus on the repair Millard first described in 1955, as today it is perhaps the most commonly adapted repair of cleft lip.
The rotation-advancement method of Millard advances a mucocutaneous flap from the lateral lip element into the gap of the upper portion of the lip resulting from the inferior downward rotation of the medial lip element.9 The repair attempts to place the lip scars along anatomic lines of the philtral column and nasal sill. Conceptually, Millard's approach is elegant but it is not always technically easy to accomplish without some modifications to deal with the wide variation in clefts. As with any other repair, consistency in achieving a good result is operator-dependent.
A cursory description of a modified Millard operative technique used is as follows:
· Use general anesthesia with a noncuffed oro-Rae endotracheal tube positioned midline. Typically the otolaryngologist then examines the ears; if needed, myringotomy and pressure equalizing tubes are placed.
· Prior to infiltration with a local anesthetic (0.5% lidocaine with 1:200,000 epinephrine), mark the anatomic landmarks and tattoo them with a methylene blue dye.
Important anatomic landmarks used in all cleft lip repairs. Measurements of various distances are used to guide the surgeon in creating a symmetric lip.
Two key elements are involved in the markings: the placement of the final position of the new Cupid's bow peak and the vertical length of the philtral column to be created on the cleft side. Referring to the diagram, Point 3 is determined as the mirror image of Point 2 based on the distance from the midpoint to the peak of the Cupid's bow on the noncleft side. The peak on the cleft side, Point 4, is not determined as easily but typically is placed level with Point 2, where the dry vermilion is widest and the white roll above is well developed. The white roll and dry vermilion taper off medial to this point. It is unreliable to determine the peak on the cleft side using the distance between the peak of the Cupid's bow from the commissure on the noncleft side because of unequal tension of the underlying orbicularis muscle.
· Once the anatomic points are marked, draw incision lines that define the 5 flaps involved in the lip reconstruction. These are the inferior rotation flap (R) of the medial lip element, the medial advancement flap (A) of the lateral lip element, the columellar base flap (C) of the medial lip element, and the two pared mucosal flaps of the medial (m) and lateral (l) lip elements. Two additional flaps that refine the repair often are used: a white roll flap and a vermilion triangular flap to allow for a smoother transition at the vermilion cutaneous junction and at the vermilion contour.
· The essential marking is the line that determines the border between the R and C flaps. This line becomes the new philtral column on the cleft side. For the vertical lengths of the philtrum on the cleft side and noncleft side to be symmetric, the length of the rotation advancement flap (y) should equal the vertical length of the philtral column (x) on the noncleft side (distance between alar base and Cupid's bow peak). For the two lengths, x and y, to be equal, the path of y must be curved as illustrated. In marking the curve, take care to avoid a high arching curve that comes too high at the columellar base to create a generous philtrum, as this significantly diminishes the size of the C flap.
· While all flaps are marked, the authors typically refine the design of the A flap after the R and C flaps are repositioned appropriately so that it more accurately is tailored to fill the gap left by the inferior rotation of the R flap and the final placement of the C flap.
· Pare the margins of the cleft and develop the m and l flaps. The l flap can be used to inset into the nasal vestibule lining, and the m flap can be used as part of the orolabial vestibule lining as needed. Alternatively, both flaps can be used to reconstruct the nasal and orovestibular lining of the nasal floor depending on the situation. The pars marginalis of the orbicularis typically is tethered by its abnormal insertion and further is pared, allowing the constricted muscle to expand.
· In the region of the vermilion-cutaneous junction, incise the muscle for approximately 2-3 mm on either side of the cleft paralleling the vermilion border to allow development of vermilion-cutaneous muscular flaps for final alignment.
· Develop the R and C flaps by incising the line (x) between the flaps to allow inferior rotation of the R flap so that it lies horizontally tension free with Point 3, level with Point 2. For this to occur, release must be at all levels (skin, subcutaneous tissue, muscle, fibrous attachments to the anterior nasal spine, labial mucosa). Occasionally an additional 1- to 2-mm back cut just medial to the noncleft philtral column is required along with a mucosal back cut to allow for adequate inferior rotation of the R flap. The back cut occasionally can be limited to the subdermal portion to avoid lengthening the cutaneous scar.
Millard repair. With maximal rotation of the R flap, any residual lip length discrepancy can be corrected with an inferior Z-plasty or a triangular flap. In a secondary correction, further rotation of the R flap can be considered.
· Correspondingly free the C flap with the medial crus of the alar cartilage and allow it to be repositioned, creating a large gap to be filled by the A flap.
· Develop the A flap from the lateral lip element for advancement into the gap between the R and C flaps. In developing the A flap, keep the incision along the alar base at a minimum; it rarely is required to extend much beyond the medial-most aspect of the alar base. The key to allowing adequate mobilization of the A flap is the subcutaneous release of the fibrous attachments of the alar base to the piriform margin of the maxilla and not necessarily a continued cutaneous incision along the alar margin. Other surgeons have chosen to mobilize the ala at the subperiosteal level.
Millard repair. The medial lip element [R] is rotated inferiorly and the lateral lip element [A] is advanced into the resulting upper lip defect. The columellar flap [C] is then used to create the nasal sill (see text for details).
· A lateral labial mucosal vestibular release also is required to mobilize the A flap medially and to avoid a tight-appearing postoperative upper lip deformity. Do not forget that the maxillary alveolar arches typically are at different heights in the coronal plane, and the ala must be released completely and mobilized superior medially to achieve symmetry, although ultimately its maxillary support is inadequate until arch alignment and bone grafting can be accomplished.
· As part of the mobilization of the ala, make an incision along the nasal skin-mucosal vestibular junction (infracartilaginous) where the previously developed l flap may be interposed if needed. Currently, the trend is toward more aggressive mobilization and repositioning of the lower lateral cartilages as an integral part of the cleft lip repair.
· Widely undermine the nasal tip between the cartilage and the overlying skin approaching laterally from the alar base and medially from the columellar base.
· While the A flap can be inserted as a mucocutaneous flap incorporating the orbicularis, the authors repair the muscle separately to allow for differential re-orientation of its vectors. Dissect the muscle from the overlying skin and the underlying mucosa to accomplish this and divide it into bundles that can be repositioned and interposed appropriately.
· Once all the flaps are developed and the medial and lateral lip elements are well mobilized, begin reconstruction. Typically, this begins with creating the labial vestibular lining from superior to inferior and then proceeding to the junction of the wet-dry vermilion with completion of the remainder of the vermilion after the cutaneous portion of the lip is completed.
· At this point, the labial mucosa can be advanced as needed, with additional lengthening and a back cut to allow for adequate eversion of the lip and to avoid a tight-appearing lip postoperatively.
· Direct attention to approximating the muscle bundles. Appropriately reorient the nasolabial group of muscles toward the nasal spine. Follow this by approximating the orbicularis, interdigitated with its opposing element along the full length of the vertical lip. Inset the C flap to create a symmetric columellar length and flare at its base. Millard originally described the C flap to cross the nasal sill to insert into the lateral lip element as a lateral rotation-advancement flap. Millard later refined the C flap as a medial superior rotation flap to insert into the medial lip element, augmenting the columellar height and creating a more natural flare at the base of the medial footplate. The latter method occasionally results in a nexus of scars at the base of the columellar with unfavorable healing if the flaps are not well planned. However, the authors and others continue to use the C flap in either position as needed.
Millard repair. Two of the most common variations described with utilization of the C Flap to correct the hemi-columellar deficiency (Millard II] and the nasal sill alar base region [Millard I]
· Set the ala base in place. As the C and A flaps and the ala are inset, take care to leave an appropriate width to the nasal sill to avoid a constricted-appearing nostril, which is nearly impossible to correct as a secondary deformity.
· Approximate the vermilion-cutaneous junction and inset the vermilion mucocutaneous triangular flap. If the lip appears to be vertically short at this point, the authors inset a small, 2- to 3-mm triangular flap into the medial lip just above the vermilion.
· Use dermal sutures to approximate the skin edges. Final approximation is with either rapidly absorbing sutures or nylon sutures, ideally removed at 5 days. If the cutaneous edges are well approximated with dermal sutures alone, the authors occasionally use a cyanoacrylate-type adhesive. Reposition the cleft alar cartilage with suspension/transfixion sutures and a stent. Further shape the ala with through-and-through absorbable sutures as needed.
Postoperative Details
· Oral feedings: For the child who is breastfed, the authors encourage uninterrupted breastfeeding after surgery. Bottle-fed children can resume feedings immediately following surgery with the same crosscut nipple used before surgery. Some centers still advocate having the child use a soft catheter-tip syringe for 10 days and then resuming normal nipple bottle feeding, but the authors have found this degree of caution to be unnecessary.
· Activities: The authors instruct the parents to avoid giving the child pacifiers or toys with sharp edges for 2 weeks after surgery. No other particular restrictions on activity are necessary. Some centers do advocate the use of Velcro elbow immobilizers on the patient for 10 days to minimize the risk of inadvertent injury to the lip repair. These are periodically removed several times a day under supervision.
· Lip care: The exposed suture line at the base of the nose and red lip can be cleaned using cotton swabs with diluted hydrogen peroxide, and topical antibiotic ointment can be applied several times a day. The authors then remove the permanent sutures on postoperative day 5-7. If cyanoacrylate adhesive is used, no additional care is required in the immediate postoperative period until the adhesive film comes off. The authors tell the parents to expect noticeable scar contracture, erythema, and firmness 4-6 weeks postsurgery, and that this gradually begins to improve 6-12 months after the procedure. Typically, the authors instruct parents to massage the upper lip during this phase and to avoid placing the child in direct sunlight until the scar matures.
Follow-up
Following cleft lip repair, patients are evaluated periodically by the various cleft team members. Oral hygiene and dental care must be promoted, hearing and speech must be assessed, and psychosocial evaluation and treatment should be made available.
Despite technical advances and simultaneous correction of the nasal deformity performed at the time of lip repair, a significant number of patients still require a secondary procedure to restore nasal symmetry and improve function.10 Such procedures should be individualized. The alar base symmetry is unlikely to be improved until the alveolar alignment is corrected and grafted with bone. The remaining components of cleft care are addressed in other articles, including the following:
· Cleft Lip and Palate
· Cleft Palate
· Craniofacial, Bilateral Cleft Lip Repair
· Craniofacial, Bilateral Cleft Nasal Repair
· Craniofacial, Unilateral Cleft Nasal Repair
· Cleft Lip Nasal Deformity
Complications
Several common mistakes are made in the rotation-advancement method of unilateral cleft lip repair. These include insufficient rotation of the R flap, vermilion-cutaneous mismatch, vermilion notching and a tight-appearing lateral lip element, a lateral muscle bulge, a laterally displaced ala, and a constricted-appearing nostril.
Aside from unsatisfactory appearance of the surgical result, possible complications include dehiscence of the repair (more common if the repair is delayed until the child is learning to walk and falls) and excessive scar formation and/or contracture of lip scars. If dehiscence occurs, postpone re-operation until the induration has subsided completely. With lip scars that appear red, thick, and contracted, the authors use an occlusive tape dressing and if needed, Kenalog-10 (triamcinolone acetonide) injection and/or flurandrenolide tape. For most repairs, the observed contracture is part of the normal healing process and improves with time. Postpone revisional surgery until the scar matures. Intervention should be guided by the severity of the residual deformity. Keep revisions to a minimum.
Outcome and Prognosis
Careful preoperative assessment of the cleft lip deformity and attention to detail in the reconstruction typically results in an excellent repair that achieves many characteristics of the natural lip. Realistically, many variables are involved beyond the technical aspects of a particular repair. Ultimately, the outcome depends on the natural course of uncomplicated healing of the initial repair, alignment of the skeletal framework on which the lip rests, and the differential effect of normal growth and development on the operated lip.
While a poor initial result is unlikely to improve with time, do not assume that an excellent initial result will not require some revisional procedure because of uncontrolled variables. Moreover, while the lip repair may be acceptable, additional procedures required to achieve nasal symmetry are not uncommon, despite the initial primary nasal surgery incorporated as an integral part of lip repair.
The presence of unilateral cleft lip is one of the most common congenital deformities. A broad spectrum of variations in clinical presentation exists. Unilateral cleft lip involves deformity of the lip in addition to the alveolus and nose. Patients with this deformity require short-term care and long-term care and follow-up from practitioners in multiple specialties. Patients may need multiple surgical interventions, from infancy to adulthood, in order to achieve necessary function and aesthetic quality.
No universal agreement has been reached as to the timing and technique of repair. Several methods are used with comparable long-term results, which serves as an indication that more than one treatment option exists for definitive repair. Treatment goals include the restoration of facial appearance and oral function, improvement of dental skeletal and occlusal relationships, improvement of speech, and the psychosocial state.
History of the Procedure
Hippocrates (400 BC) and Galen (150 AD) mention cleft lip, but not cleft palate in their writings. For centuries, perforations of the palate were considered to be secondary to syphilis, and cleft palate was not recognized as a congenital disorder until 1556, by Fanco. The first successful closure of a soft palate defect was reported in 1764 by LeMonnier, a French dentist. The first closure of the hard palate was performed in 1834 by Dieffenbach. In the 1930's, Kilner and Wardill independently developed the "pushback" procedure. In 1843, closure of the unilateral cleft lip with local flaps was described by Malgaigne. The following year, Mirault modified Malgaigne’s technique by using the lateral lip flap to fill the medial defect. All future methods of cleft lip closure are based on Mirault’s technique. LeMesurier and Tennyson modified this technique with a quadrilateral and triangular flap, respectively. In 1976, Millard published his definitive repair in which the lateral flap advancement into the upper portion of lip was combined with downward rotation of medial lip. Other modifications have been published by Noordhoff, Mohler, and Onizuka. Fisher has described an anatomical subunit approximation for definitive cleft lip repair. Millard’s methods, including variations, remain among the most popular method for unilateral cleft lip closure.
Cleft lip surgery has evolved from a simple adhesion of paired margins of the cleft to an understanding of the various malpositioned elements of the lip to a more complicated geometric reconstruction using transposition, rotation, and advancement flaps.
Problem
The cleft affects the facial form as an anatomic deformity and has functional consequences. These include the child's ability to eat, speak, hear, and breathe. Consequently, rehabilitation of a child born with a facial cleft must involve a multidisciplinary approach and staged appropriately with the child's development, balancing the timing of intervention against its effect on subsequent normal growth.
Incidence
The overall occurrence of cleft lip with or without cleft palate is approximately 1 in 750-1000 live births. Racial differences exist, with the incidence in Asians (1:500) greater than in Caucasians (1:750) greater than in African Americans (1:2000). The incidence of cleft lip/palate is more common in males.
The most common presentation is cleft lip and palate (approximately 45%), followed by cleft palate alone (35%) and cleft lip alone (approximately 20%). Unilateral cleft lips are more common than bilateral cleft lips and occur more commonly on the left side (left cleft lip: right cleft lip: bilateral cleft lip = 6:3:1).
The risk of a newborn having a cleft lip increases if a first-degree relative also has a cleft. If one child already has a cleft lip, the chance of a second child being born with the deformity is 4%. If a parent has a cleft lip, the chance of a newborn having a cleft is 7%. If both a parent and a sibling have a cleft lip, the newborn's risk rises to 15%.
Etiology
Clefting has a multifactorial basis, with both genetic and environmental causes cited. The observation of clustered cases of facial clefts in a particular family indicates a genetic basis. Clefting of the lip and/or palate is associated with more than 150 syndromes. The overall incidence of associated anomalies (eg, cardiac) is approximately 30% (more common with isolated cleft palate).
Environmental causes, such as viral infection (eg, rubella) and teratogens (eg, steroids, anticonvulsants), during the first trimester have been linked to facial clefts. The risk also increases with parental age, especially when older than 30 years, with the father's age appearing to be a more significant factor than the mother's age. Nevertheless, most presentations are of isolated patients within the family without an obvious etiology.
Midfacial development involves several sets of genes, including those involved in cell patterning, proliferation, and signaling. Mutations in any of these genes can change the developmental process and contribute to cleft development. Some of these genes include the DIX gene, sonic hedgehog (SHH) gene, transforming growth factor (TGF) alpha/beta, and interferon regulatory factor (IRF6).
Classification
Kernahan developed a classification scheme in which the defect can be classified onto a Y-shaped symbol.
Millard modification of Kernahan striped-Y classification for cleft lip and palate.
Pathophysiology
While the normal embryologic development of the face is detailed in Head and Neck Embryology, a brief outline relevant to the formation of facial clefts follows.
In short, the branchial arches are responsible for the formation of several areas, including the mouth and lip. Mesenchymal migration and fusion occurs during weeks 4-7 of gestation. The first branchial arch is responsible for the formation of the maxillary and mandibular processes. The maxillary and mandibular prominences form the lateral borders of the primitive mouth or stomodeum.
Mesenchymal migration and fusion of the primitive somite-derived facial elements (central frontonasal, 2 lateral maxillary, mandibular processes), at 4-7 weeks gestation, is necessary for the normal development of embryonic facial structures. When migration and fusion are interrupted for any reason, a facial cleft develops along embryonic fusion lines. The embryonic development of the primary palate (lip and palate anterior to the incisive foramen) differs from the secondary palate (palate posterior to the incisive foramen).
The developing processes of the medial nasal prominence, lateral nasal prominence, and maxillary prominences form the primary palate. Fusion occurs, followed by "streaming" of mesodermal elements derived from the neural crest. In contrast, the secondary palate is formed by the fusion of palatal processes of the maxillary prominence alone. The difference in embryonic development suggests the possibility of differing degrees of susceptibility to genetic and environmental influences and accounts for the observed variation in incidences.
In summary, unilateral cleft lip results from failure of fusion of the medial nasal prominence with the maxillary prominence.
Presentation
For treatment purposes, unilateral cleft lip can be placed into one of three categories: microform/forme fruste, incomplete, or complete cleft lip.
Microform cleft (forme fruste): This defect is characterized by a "light" furrow along the vertical length of the lip with a small vermilion notch and minor imperfections in the white roll. A small component of vertical lip length deficiency and associated nasal deformity may be present.
Incomplete cleft lip: This defect is characterized by the varying degree of vertical lip separation. By definition, it has an intact nasal sill, commonly termed the Simonart band.
Complete cleft lip: This involves the full-thickness defect of the lip and alveolus (primary palate), extends into the base of the nose (no Simonart band exists), and is often accompanied by a palatal cleft (secondary palate). The premaxilla is typically rotated outward and projects anteriorly in relation to a relatively retropositioned lateral maxillary alveolar element.
As a consequence of the clefting of the lip, an associated nasal deformity occurs. The structures of the ala base, nasal sill, vomer, and septum are distorted significantly. The lower lateral cartilage on the cleft side is positioned inferiorly, with an obtuse angle as it flattens across the cleft. The alar base is rotated outward. The developing nasal septum pulls the premaxilla away from the cleft, and the septum and the nasal spine are deflected toward the noncleft side. The cleft may continue through the maxillary alveolus and palatal shelf, extending to the palatal bone and soft palate.
Further treatment planning
Orthodontic treatment can be initiated a few weeks following birth, prior to surgical intervention. Other adjunct procedures include lip adhesion, presurgical orthopedics, primary nasal correction, and nasoalveolar molding. These procedures attempt to reduce the deformity. Nasoalveolar molding is the active molding and repositioning of the nasal cartilage and alveolar processes with an appliance.6 This orthodontic intervention takes advantage of the plasticity of the cartilage. Presurgical nasal alveolar allows repositioning of the maxillary alveolus and surrounding soft tissues in hopes of reducing wound tension and improving results.7,8
Definitive repair is delayed until approximately 3 months of age; this varies, depending on physician comfort. A multidisciplinary approach should be carried out over several years for patients with unilateral cleft lip. This team should include practitioners from audiology, otolaryngology, and speech therapy, among other specialities.
Indications
Patients born with a cleft lip should undergo surgical repair unless otherwise contraindicated. The goal of reconstruction is to establish normal morphologic facial form and function in order to provide the optimal conditions for the development of dentition, mastication, hearing, speech, and breathing, and psychosocial status.
Anatomy
Normal lip and nasal anatomy is essential for an understanding of the distortion caused by a facial cleft. The elements of the normal lip are composed of the central philtrum, demarcated laterally by the philtral columns and inferiorly by the Cupid's bow and tubercle. Just above the junction of the vermilion-cutaneous border is a mucocutaneous ridge frequently referred to as the white roll. Within the red vermilion of the lip is a noticeable junction demarcating the dry and wet vermilion, the increased keratinized portion of the lip that is exposed to air from the moist environment of the labial mucosa.
The primary muscle of the lip is the orbicularis oris, and it has two well-defined components: the deep (internal) and the superficial (external) components. The deep (internal) fibers run horizontally or circumferentially from commissure (modiolus) to commissure (modiolus) and functions as the primary sphincteric action for oral feeding. The superficial (external) fibers run obliquely, interdigitating with the other muscles of facial expression to terminate in the dermis. They provide subtle shades of expression and precise movements of the lip for speech.
The superficial fibers of the orbicularis decussate in the midline and insert into the skin lateral to the opposite philtral groove forming the philtral columns. The resulting philtral dimple centrally is depressed as there are no muscle fibers that directly insert into the dermis in the midline. The tubercle of the lip is shaped by the pars marginalis, the portion of the orbicularis along the vermilion forming the tubercle of the lip with eversion of the muscle.
In the upper lip, the levator labii superioris contributes to the form of the lip. Its fibers, arising from the medial aspect of the infraorbital rim, sweep down to insert near the vermilion cutaneous junction. The medial-most fibers of the levator labii superioris sweep down to insert near the corner of the ipsilateral philtral column and vermilion-cutaneous junction, helping to define the lower philtral column and the peak of the Cupid's bow.
The nasal muscles are equally important. The levator superioris alaeque arises along the frontal process of the maxilla and courses inferiorly to insert on the mucosal surface of the lip and ala. The transverse nasalis arises along the nasal dorsum and sweeps around the ala to insert along the nasal sill from lateral to medial into the incisal crest and anterior nasal spine. These fibers join with the oblique fibers of the orbicularis and the depressor septi (nasalis), which arises from the alveolus between the central and lateral incisors to insert into the skin of the columellar to the nasal tip and the footplates of the medial crura.
A unilateral cleft thus disrupts the normal termination of the muscle fibers that cross the embryologic fault line of the maxillary and nasal processes, resulting in symmetric but abnormal muscular forces between the normal equilibrium that exists with the nasolabial and oral groups of muscles. With an unrestrained premaxilla, the deformity accentuates with differential growth of the various elements. The alar cartilages are splayed apart and rotate caudally, subluxed from the normal position. Consequently, the nasal tip broadens, the columellar is foreshortened, and the alar bases rotate outwardly cephalad.
Workup
Laboratory Studies
· Perform a thorough physical examination, not limited to the head and neck region, to uncover associated anomalies in the infant presenting with a unilateral cleft lip with or without a palatal cleft. Additional workup is determined by physical findings that suggest involvement of other organ systems.
· The child's weight, oral intake, and growth and/or development are of primary concern and must be followed closely.
· Routine laboratory studies typically are not required, other than a hemoglobin study shortly before the planned lip repair.
· Routine imaging is not indicated in a healthy patient with isolated cleft lip.
Treatment
Surgical Therapy
Children born with a facial cleft benefit from multidisciplinary clinical care. This is a team-based approach allowing efficient coordination of all aspects of care. Beyond the lip repair are other issues such as hearing, speech, dental, and psychosocial integration. With the multidisciplinary approach, as the child grows, comprehensive care can be given from birth through adolescence. These associated issues are as important as the anatomic reconstruction, and ultimately the functional outcome of the reconstruction depends on addressing them.
Each specialty involved must evaluate the child individually and formulate a treatment plan, then the team forms a combined individual integrated protocol that follows the Parameters of Care Guidelines established by the American Cleft Palate Craniofacial Association. Rather than strictly adhering to any one protocol, each child is assessed based on the present need in his or her development, and a treatment plan is created based on the team's experience.
Preoperative Details
While the lip repair is the initial focus for many parents, treatment begins by assessing the child's nutritional status and assisting the parents with oral feeding techniques so that appropriate weight gain occurs.
Parents who suddenly are faced with caring for a child with a facial cleft are overwhelmed. The importance of spending sufficient time with them to allay their fears, to discuss staging and timing of reconstruction, to stress the need for involvement of other specialists, and to instruct them on the importance of long-term and consistent follow-up care from birth through adolescence cannot be overemphasized.
The optimal timing of the surgical repair is still somewhat controversial. Some centers have advocated surgery in the early neonatal period, with a theoretical benefit in the scar appearance and nasal cartilage adaptability, thus minimizing the nasal deformity. To minimize anesthetic risks, some still adhere to the rule of 10s: perform surgical repair of cleft lip when the child has a hemoglobin of 10 g, weight of 10 lb, and is aged 10 weeks. In general, however, most centers prefer to perform the unilateral lip repair when the infant is aged 2-4 months; anesthesia risks are lower, the child is better able to withstand the stress of surgery, and lip elements are larger and allow for a meticulous reconstruction.
Before the definitive lip surgery, cleft centers utilize lip taping, alone or in combination with a passive intraoral appliance or an active pin-based appliance (eg, Latham) to align the maxillary arch segments; or no presurgical orthopedic intervention at all. This choice depends on the center's protocol and resources.
A number of cleft centers prefer to use a passive intraoral orthodontic palatal appliance to maintain the arch width to prevent the nearly inevitable collapse that occurs with the lip surgery.6 The lip repair reestablishes the soft tissue and muscular forces on the easily moldable maxillary arch segments. Additionally, this appliance may include a nasal extension to help improve the nasal tip form. This nasal alveolar molding device is incorporated into the intraoral appliance. Several weeks of treatment prior to the surgery and regular adjustments are needed to mold the alar cartilages into a more favorable position, thus facilitating the surgical correction of the nasal deformity. Impressions are taken soon after birth so that the custom appliance can be applied as soon as possible before the lip repair. The appliance also assists in the child's oral feeding, helping to decrease nasal regurgitation and assisting oral suction.
Intraoperative Details
The ideal lip repair results in symmetrically shaped nostrils, nasal sill, and alar bases; a well-defined philtral dimple and columns; and a natural appearing Cupid's bow with a pout to the vermilion tubercle. In addition, it results in a functional muscle repair that with animation mimics a normal lip. While ideally the lip scars approximate natural landmarks, ultimately the eye first focuses on symmetry and then normal contours of the lip at rest and in animation.
A number of surgical procedures for the repair of a unilateral cleft lip are well described, with a multitude of variations, including the LeMesurier quadrilateral flap repair, Randall-Tennison triangular flap repair, Millard rotation-advancement repair, and Skoog and Kernahan-Bauer upper and lower lip Z-plasty repairs. Many other variations exist; of particular note are the repairs by Delaire and by Poole.
The Rose-Thompson repair involves curved or angled paring of the cleft margins to lengthen the lip as a straight-line closure.
Hagedorn-LeMesurier repair. The medial lip element is lengthened by introducing a quadrilateral flap developed from the lateral lip element.
Tennison-Randall repair. The medial lip element is lengthened by introducing a triangular flap from the inferior portion of the lateral lip element.
Skoog repair. The medial lip element is lengthened by introducing two small triangular flaps developed from the lateral lip element.
Each of these techniques ultimately has the common goal of achieving symmetry and restoring the continuity of the underlying orbicularis muscle. All attempt to lengthen the foreshortened philtrum on the cleft side by interposing tissue from the lateral lip element into the medial lip element through various combinations of rotation, advancement, and transposition flaps.
While none of the repairs is ideal, each has advantages and disadvantages, and each results in an excellent repair in experienced hands, underscoring the fact that more than a single acceptable technique, rather than a single ideal repair, is available. However, because of the limitations of this article, the authors choose to focus on the repair Millard first described in 1955, as today it is perhaps the most commonly adapted repair of cleft lip.
The rotation-advancement method of Millard advances a mucocutaneous flap from the lateral lip element into the gap of the upper portion of the lip resulting from the inferior downward rotation of the medial lip element.9 The repair attempts to place the lip scars along anatomic lines of the philtral column and nasal sill. Conceptually, Millard's approach is elegant but it is not always technically easy to accomplish without some modifications to deal with the wide variation in clefts. As with any other repair, consistency in achieving a good result is operator-dependent.
A cursory description of a modified Millard operative technique used is as follows:
· Use general anesthesia with a noncuffed oro-Rae endotracheal tube positioned midline. Typically the otolaryngologist then examines the ears; if needed, myringotomy and pressure equalizing tubes are placed.
· Prior to infiltration with a local anesthetic (0.5% lidocaine with 1:200,000 epinephrine), mark the anatomic landmarks and tattoo them with a methylene blue dye.
Important anatomic landmarks used in all cleft lip repairs. Measurements of various distances are used to guide the surgeon in creating a symmetric lip.
Two key elements are involved in the markings: the placement of the final position of the new Cupid's bow peak and the vertical length of the philtral column to be created on the cleft side. Referring to the diagram, Point 3 is determined as the mirror image of Point 2 based on the distance from the midpoint to the peak of the Cupid's bow on the noncleft side. The peak on the cleft side, Point 4, is not determined as easily but typically is placed level with Point 2, where the dry vermilion is widest and the white roll above is well developed. The white roll and dry vermilion taper off medial to this point. It is unreliable to determine the peak on the cleft side using the distance between the peak of the Cupid's bow from the commissure on the noncleft side because of unequal tension of the underlying orbicularis muscle.
· Once the anatomic points are marked, draw incision lines that define the 5 flaps involved in the lip reconstruction. These are the inferior rotation flap (R) of the medial lip element, the medial advancement flap (A) of the lateral lip element, the columellar base flap (C) of the medial lip element, and the two pared mucosal flaps of the medial (m) and lateral (l) lip elements. Two additional flaps that refine the repair often are used: a white roll flap and a vermilion triangular flap to allow for a smoother transition at the vermilion cutaneous junction and at the vermilion contour.
· The essential marking is the line that determines the border between the R and C flaps. This line becomes the new philtral column on the cleft side. For the vertical lengths of the philtrum on the cleft side and noncleft side to be symmetric, the length of the rotation advancement flap (y) should equal the vertical length of the philtral column (x) on the noncleft side (distance between alar base and Cupid's bow peak). For the two lengths, x and y, to be equal, the path of y must be curved as illustrated. In marking the curve, take care to avoid a high arching curve that comes too high at the columellar base to create a generous philtrum, as this significantly diminishes the size of the C flap.
· While all flaps are marked, the authors typically refine the design of the A flap after the R and C flaps are repositioned appropriately so that it more accurately is tailored to fill the gap left by the inferior rotation of the R flap and the final placement of the C flap.
· Pare the margins of the cleft and develop the m and l flaps. The l flap can be used to inset into the nasal vestibule lining, and the m flap can be used as part of the orolabial vestibule lining as needed. Alternatively, both flaps can be used to reconstruct the nasal and orovestibular lining of the nasal floor depending on the situation. The pars marginalis of the orbicularis typically is tethered by its abnormal insertion and further is pared, allowing the constricted muscle to expand.
· In the region of the vermilion-cutaneous junction, incise the muscle for approximately 2-3 mm on either side of the cleft paralleling the vermilion border to allow development of vermilion-cutaneous muscular flaps for final alignment.
· Develop the R and C flaps by incising the line (x) between the flaps to allow inferior rotation of the R flap so that it lies horizontally tension free with Point 3, level with Point 2. For this to occur, release must be at all levels (skin, subcutaneous tissue, muscle, fibrous attachments to the anterior nasal spine, labial mucosa). Occasionally an additional 1- to 2-mm back cut just medial to the noncleft philtral column is required along with a mucosal back cut to allow for adequate inferior rotation of the R flap. The back cut occasionally can be limited to the subdermal portion to avoid lengthening the cutaneous scar.
Millard repair. With maximal rotation of the R flap, any residual lip length discrepancy can be corrected with an inferior Z-plasty or a triangular flap. In a secondary correction, further rotation of the R flap can be considered.
· Correspondingly free the C flap with the medial crus of the alar cartilage and allow it to be repositioned, creating a large gap to be filled by the A flap.
· Develop the A flap from the lateral lip element for advancement into the gap between the R and C flaps. In developing the A flap, keep the incision along the alar base at a minimum; it rarely is required to extend much beyond the medial-most aspect of the alar base. The key to allowing adequate mobilization of the A flap is the subcutaneous release of the fibrous attachments of the alar base to the piriform margin of the maxilla and not necessarily a continued cutaneous incision along the alar margin. Other surgeons have chosen to mobilize the ala at the subperiosteal level.
Millard repair. The medial lip element [R] is rotated inferiorly and the lateral lip element [A] is advanced into the resulting upper lip defect. The columellar flap [C] is then used to create the nasal sill (see text for details).
· A lateral labial mucosal vestibular release also is required to mobilize the A flap medially and to avoid a tight-appearing postoperative upper lip deformity. Do not forget that the maxillary alveolar arches typically are at different heights in the coronal plane, and the ala must be released completely and mobilized superior medially to achieve symmetry, although ultimately its maxillary support is inadequate until arch alignment and bone grafting can be accomplished.
· As part of the mobilization of the ala, make an incision along the nasal skin-mucosal vestibular junction (infracartilaginous) where the previously developed l flap may be interposed if needed. Currently, the trend is toward more aggressive mobilization and repositioning of the lower lateral cartilages as an integral part of the cleft lip repair.
· Widely undermine the nasal tip between the cartilage and the overlying skin approaching laterally from the alar base and medially from the columellar base.
· While the A flap can be inserted as a mucocutaneous flap incorporating the orbicularis, the authors repair the muscle separately to allow for differential re-orientation of its vectors. Dissect the muscle from the overlying skin and the underlying mucosa to accomplish this and divide it into bundles that can be repositioned and interposed appropriately.
· Once all the flaps are developed and the medial and lateral lip elements are well mobilized, begin reconstruction. Typically, this begins with creating the labial vestibular lining from superior to inferior and then proceeding to the junction of the wet-dry vermilion with completion of the remainder of the vermilion after the cutaneous portion of the lip is completed.
· At this point, the labial mucosa can be advanced as needed, with additional lengthening and a back cut to allow for adequate eversion of the lip and to avoid a tight-appearing lip postoperatively.
· Direct attention to approximating the muscle bundles. Appropriately reorient the nasolabial group of muscles toward the nasal spine. Follow this by approximating the orbicularis, interdigitated with its opposing element along the full length of the vertical lip. Inset the C flap to create a symmetric columellar length and flare at its base. Millard originally described the C flap to cross the nasal sill to insert into the lateral lip element as a lateral rotation-advancement flap. Millard later refined the C flap as a medial superior rotation flap to insert into the medial lip element, augmenting the columellar height and creating a more natural flare at the base of the medial footplate. The latter method occasionally results in a nexus of scars at the base of the columellar with unfavorable healing if the flaps are not well planned. However, the authors and others continue to use the C flap in either position as needed.
Millard repair. Two of the most common variations described with utilization of the C Flap to correct the hemi-columellar deficiency (Millard II] and the nasal sill alar base region [Millard I]
· Set the ala base in place. As the C and A flaps and the ala are inset, take care to leave an appropriate width to the nasal sill to avoid a constricted-appearing nostril, which is nearly impossible to correct as a secondary deformity.
· Approximate the vermilion-cutaneous junction and inset the vermilion mucocutaneous triangular flap. If the lip appears to be vertically short at this point, the authors inset a small, 2- to 3-mm triangular flap into the medial lip just above the vermilion.
· Use dermal sutures to approximate the skin edges. Final approximation is with either rapidly absorbing sutures or nylon sutures, ideally removed at 5 days. If the cutaneous edges are well approximated with dermal sutures alone, the authors occasionally use a cyanoacrylate-type adhesive. Reposition the cleft alar cartilage with suspension/transfixion sutures and a stent. Further shape the ala with through-and-through absorbable sutures as needed.
Postoperative Details
· Oral feedings: For the child who is breastfed, the authors encourage uninterrupted breastfeeding after surgery. Bottle-fed children can resume feedings immediately following surgery with the same crosscut nipple used before surgery. Some centers still advocate having the child use a soft catheter-tip syringe for 10 days and then resuming normal nipple bottle feeding, but the authors have found this degree of caution to be unnecessary.
· Activities: The authors instruct the parents to avoid giving the child pacifiers or toys with sharp edges for 2 weeks after surgery. No other particular restrictions on activity are necessary. Some centers do advocate the use of Velcro elbow immobilizers on the patient for 10 days to minimize the risk of inadvertent injury to the lip repair. These are periodically removed several times a day under supervision.
· Lip care: The exposed suture line at the base of the nose and red lip can be cleaned using cotton swabs with diluted hydrogen peroxide, and topical antibiotic ointment can be applied several times a day. The authors then remove the permanent sutures on postoperative day 5-7. If cyanoacrylate adhesive is used, no additional care is required in the immediate postoperative period until the adhesive film comes off. The authors tell the parents to expect noticeable scar contracture, erythema, and firmness 4-6 weeks postsurgery, and that this gradually begins to improve 6-12 months after the procedure. Typically, the authors instruct parents to massage the upper lip during this phase and to avoid placing the child in direct sunlight until the scar matures.
Follow-up
Following cleft lip repair, patients are evaluated periodically by the various cleft team members. Oral hygiene and dental care must be promoted, hearing and speech must be assessed, and psychosocial evaluation and treatment should be made available.
Despite technical advances and simultaneous correction of the nasal deformity performed at the time of lip repair, a significant number of patients still require a secondary procedure to restore nasal symmetry and improve function.10 Such procedures should be individualized. The alar base symmetry is unlikely to be improved until the alveolar alignment is corrected and grafted with bone. The remaining components of cleft care are addressed in other articles, including the following:
· Cleft Lip and Palate
· Cleft Palate
· Craniofacial, Bilateral Cleft Lip Repair
· Craniofacial, Bilateral Cleft Nasal Repair
· Craniofacial, Unilateral Cleft Nasal Repair
· Cleft Lip Nasal Deformity
Complications
Several common mistakes are made in the rotation-advancement method of unilateral cleft lip repair. These include insufficient rotation of the R flap, vermilion-cutaneous mismatch, vermilion notching and a tight-appearing lateral lip element, a lateral muscle bulge, a laterally displaced ala, and a constricted-appearing nostril.
Aside from unsatisfactory appearance of the surgical result, possible complications include dehiscence of the repair (more common if the repair is delayed until the child is learning to walk and falls) and excessive scar formation and/or contracture of lip scars. If dehiscence occurs, postpone re-operation until the induration has subsided completely. With lip scars that appear red, thick, and contracted, the authors use an occlusive tape dressing and if needed, Kenalog-10 (triamcinolone acetonide) injection and/or flurandrenolide tape. For most repairs, the observed contracture is part of the normal healing process and improves with time. Postpone revisional surgery until the scar matures. Intervention should be guided by the severity of the residual deformity. Keep revisions to a minimum.
Outcome and Prognosis
Careful preoperative assessment of the cleft lip deformity and attention to detail in the reconstruction typically results in an excellent repair that achieves many characteristics of the natural lip. Realistically, many variables are involved beyond the technical aspects of a particular repair. Ultimately, the outcome depends on the natural course of uncomplicated healing of the initial repair, alignment of the skeletal framework on which the lip rests, and the differential effect of normal growth and development on the operated lip.
While a poor initial result is unlikely to improve with time, do not assume that an excellent initial result will not require some revisional procedure because of uncontrolled variables. Moreover, while the lip repair may be acceptable, additional procedures required to achieve nasal symmetry are not uncommon, despite the initial primary nasal surgery incorporated as an integral part of lip repair.
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