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Scoliosis, Kyphosis and Spine Deformity The Division of Pediatric Orthopaedic Surgery at the Children's Hospital of New York Presbyterian offers comprehensive care for infants, children, and adolescents with scoliosis and spinal deformities. The orthopaedic surgeons at our Center work collaboratively with pediatric specialists in our children's hospital. Every specialty is represented from Pediatric Anesthesia and intensive care to Pediatric neurology, pulmonology and cardiology. Spinal cord monitoring at the Center is performed by the world renowned neurophysiology division at Columbia University. We perform routine monitoring of somatosensory evoked potentials and motor evoked potentials More children's spine surgery is performed at Children's Hospital of New York Presbyterian, Columbia University Medical Center than any other single center in New York. Surgery for infantile scoliosis, juvenile scoliosis, and adolescent idiopathic scoliosis are frequently performed. The Center is engaged in cutting edge research including investigations into the effect of chest wall deformity on pulmonary development, outcomes of various treatments for scoliosis and spine deformity and the effect of spine deformity on quality of life. The Center provides the most advanced care for children and adolescents with idiopathic scoliosis. Our bracing program is integrated into the offices with the orthotist available for immediate consultation with the physician at every visit. When surgery is indicated, it is performed using state of the art blood salvage techniques and spinal cord monitoring techniques. Innovative surgery is performed at the CHONY Scoliosis and Spine Deformity Center. Our center is performing expansion thoracoplasty in infants and children with thoracic insufficiency syndrome. These children have early onset spine and chest wall deformity that threatens lung growth. We perform thoracoscopic surgery in indicated cases thus avoiding long incisions in the chest wall. Our faculty members belong to the professional organizations and the national collaborative groups that monitor the outcomes of surgery. The physicians of the Center for Pediatric and Adolescent Scoliosis and Spine Deformity are leading the field in the application and evaluation of new techniques. Scoliosis is a musculoskeletal condition that primarily affects children and adolescents, in which there is an abnormal lateral curvature of the spine, causing the spinal column to bend to the left or right. Whereas most people's spines have the appearance of a straight line, when viewed from directly behind the person, the spine of someone with scoliosis more closely resembles the letter S or C, because of the abnormal curvature. The name is derived from the Greek word ? skoliosis ', which means ?crookedness'. Scoliosis is actually relatively common compared to other musculoskeletal diseases, affecting approximately 3% of the population. The condition has affected children for thousands of years, and was described in even the earliest medical histories. The different types of scoliosis Abnormal curvature of the spine can occur in children in a wide range of ages and through a variety of causes. Therefore, scoliosis has actually been categorized into a number of different types, each with its own clinical characteristics. More importantly, each type of scoliosis is managed, or treated, by orthopaedic surgeons slightly differently than the others, so it is important to classify the distinct conditions in this manner. The most common type of scoliosis is idiopathic scoliosis, which means that it occurs without known causes. Because this type of scoliosis can affect children at different ages, it has been further broken down into an even more specific set of conditions. There are therefore 3 sub-types of idiopathic scoliosis that affect children: infantile, juvenile, and adolescent. Infantile idiopathic scoliosis occurs before the age of 3 and is seen more frequently in boys than girls. Most cases resolve spontaneously, but some may progress to more severe deformity. Treatment may include observation, physical therapy, bracing and, under rare circumstances, surgery. For more in-depth information on infantile scoliosis, click here.Juvenile idiopathic scoliosis is defined as scoliosis occurring between the ages of 3 and 9. It is found more frequently in girls than boys. The abnormal spinal curvatures in children with juvenile scoliosis are generally at a high risk for progression to more severe curves. They usually require bracing and many will go on to require surgery. For more in-depth information on juvenile scoliosis, click here. Adolescent idiopathic scoliosis occurs between the age of 10 and young adulthood. This condition, also called AIS, is by far the most common type of scoliosis, as well as the type with the best prognosis. AIS may begin to manifest itself at the initial onset of puberty or become apparent or worsen during an adolescent's growth spurt. Girls, or young women, are at higher risk than boys, or young men. Most children with AIS do not develop worsening curvature, and therefore treatment usually begins only with observation. For more in-depth information on adolescent scoliosis, click here. While most cases of scoliosis are considered ?idiopathic' and occur during childhood without a clear cause, a small number of children develop a lateral curvature of the spine even before birth, during the period of fetal growth in the womb. These cases are referred to as congenital scoliosis , which means that abnormal development is the cause of the condition. Congenital scoliosis occurs when bony portions of the spine fail to form properly or normally separated segments fuse together during fetal development. The defects in the spine can be minor, involving only one segment of the vertebral column, or the condition can involve nearly every level and result in a more severe deformity. Treatment ranges from observation to surgery, depending on the appearance of the spinal curvature. For more in-depth information on congenital scoliosis, click here . Another major type of scoliosis is neuromuscular scoliosis , which means that it is caused by the presence of a neurological or muscular disease, such as cerebral palsy or muscular dystrophy. Because there is such a wide variety of these types of diseases that may cause neuromuscular scoliosis, the clinical presentation and severity of this condition is extremely variable. Unlike the other types of scoliosis described here, treatment of neuromuscular scoliosis is very unpredictable, mostly because the abnormal curves of the spine are also unpredictable. For more in-depth information on neurogenic scoliosis, click here . While there are adults with scoliosis, these are cases that usually begin in childhood or adolescence and persist into adulthood. Adult degenerative scoliosis is a distinct condition that results from wear and tear on the spinal column with disc collapse and actually begins in adulthood. Both entities should be managed by adult spine specialists, and therefore will not be described on this website. For more information about adult spine conditions and possible treatment options, please access the website for the New York-Presbyterian Spine Center: http://nypspine.org/spi_cen_tea.html . More in-depth information on the different types of scoliosis For more in-depth information on the different types of scoliosis that affect children and adolescents, please click on the links below: Infantile (Idiopathic) Scoliosis
Infantile idiopathic scoliosis is a condition that affects children before the age of 3 and is characterized by the presence of an abnormal lateral curvature of the spine. This curvature causes the spinal column to bend to the left or the right, in the shape of an S or a C. The condition is seen more frequently in boys than girls. Most cases resolve spontaneously, but some may progress to more severe deformity. Treatment of infantile scoliosis may include observation, physical therapy, bracing and, under rare circumstances, surgery. Some basic facts about infantile scoliosis
The normal infant spineThe spine, or spinal column, as it is also called, is a complex structure made up of 33 vertebrae , or bony segments, arranged vertically in succession from just below the skull to the tailbone. The spine has two main functions: to support the weight of the skull and upper body, and to provide a protective encasement for the spinal cord, the long, cylindrical nervous structure that sends messages to and from the brain and the rest of the body. The vertebrae are attached to other vertebrae above and below them by a number of ligaments , thick fibrous structures found throughout the musculoskeletal system that attach bones to other bones. The connection points between the vertebrae are called joints , in which small areas of smooth cartilage lined with a lubricating material, known as synovial fluid , allow the bony vertebrae to slide past each other with minimal friction, allowing movement. Unlike joints such as the knee or elbow, which allow for considerable movement, each joint of the spinal column allows for very little movement because of the number of tightly attached ligaments, thereby ensuring the strength and stability of the vital supportive column. However, the joints of the spine act collectively, with the sum of movements in all the joints allowing movements such as bending forward, back, and to the side. The normal spine actually has a number of natural curves to it, which stems from the evolution of humans from four-legged mammalian ancestors who walked on the ground bent over, with the spine parallel to the ground. However, these normal curves are antero-posterior curves only; that is, they can only be seen when looking at the spine from the side. On the other hand, when looking from directly behind or in front of a person, the spine maintains a rigid upright structure, like a straight line. In other words, there are normally no lateral curves to the human spine. Even during development in the womb, when the position of the fetal body is somewhat compacted, the spine is supposed to maintain its straight position, without any excessive lateral pressures. What causes infantile scoliosis?
How is it diagnosed?Infantile scoliosis is usually first detected in the first 6 months of life during a standard physical examination by a pediatrician, or noticed by a child’s parents. When scoliosis is suspected, careful neurologic exam as well as exam of the head, back, and extremities should follow, to inspect for plagiocephaly, as described above, and to insure that the spinal cord is not being affected by another disease entity. A spinal MRI, a sophisticated radiological imaging technique, is essential to confirm the findings of the neurological exam and rule out other potential causes of scoliosis. A series of x-rays should also be taken, which allow for a more precise measurement of the severity of the curvature. As mentioned before, this requires the presence of a curvature angle of at least 10 degrees, measured with the Cobb method, as shown here:
How is it treated?Decisions regarding the appropriate treatment for infantile scoliosis depend mostly on the severity of the spinal curvature and the likelihood that the curvature will worsen in the future. Because mild curves (10-25 degrees) very rarely progress to more severe curves and instead usually correct themselves, these children are treated only with observation . This entails simply that the child be seen regularly by an orthopaedic surgeon, who will monitor the possible progression of the curve with physical exams and x-rays. Visits must continue even into adolescence, because growth spurts may trigger progression, even in a previously non-progressive curve. When progression does occur in this group, which is rare, the patient will require casting and bracing treatment. Infants with moderate and severe curves have a greater chance of progressing, and often require casting and bracing treatment. Casting treatment consists of serial casting , which entails that the cast be changed every 6 to 12 weeks and a new cast applied, in an attempt to gradually correct the curvature. The cast is made of plaster or fiberglass, and is applied in the operating room under general anesthesia , which means that the infant will be put to sleep through the application process, so as not to fuss or experience discomfort. Correction is usually achieved by around 18 months of age. Casting treatment is followed up with bracing treatment, which is needed to maintain the correction. There are a number of braces available for treatment of infantile scoliosisand there is no concensus on which is the best. Further, there is no scientific evidence that bracing in infants alters the natural history of this condition. Except for bathing,and exercise the brace is to be worn all the time, usually for 2 to 3 years, after which time the child is weaned off the brace, provided correction of the curvature has been maintained. Occasionally, curves recur after brace removal, which necessitates reinstitution of full-time bracing treatment, or curves progress during bracing treatment, in which case surgery is warranted. Surgical treatment options are generally the same for both infantile and juvenile idiopathic scoliosis. Operations may consist of instrumentation , in which metal rods are attached to the spine to maintain curve correction and/or spinal fusion , in which two or more of the vertebrae are fused together with bone bridges made of bone grafts . Surgeries may be performed through a posterior approach , in which the operation is performed with an incision in the back, or also with an anterior approach , which that requires making an opening in the chest wall to reach the front part of the spinal column. Because of the stability and effectiveness of the devices used in spine operations today, patients can be mobile the day following their surgery, and hospital stays are generally under 1 week. Our spinal deformity service has added another technique in the last three years. This technique is uses instrumentation on the rib cage to provide growth of the spine and the chest without fusion. The technical name, distraction thoracoplasty speaks to the dual goals of distraction of the hemi thoracaa to get room in the chest for lung growth and lengthening of the spinal column to control the scoliosis.
Coping with infantile scoliosisManaging the demands of bracing treatment for scoliosis can be a difficult task for infants and their parents alike. Surgery at such a young age, if necessary, can seem somewhat daunting as well. However, research has shown that the various treatments for infantile idiopathic scoliosis are generally extremely successful. The vast majority of children grow up without any limitations to their activities and daily functioning, from simple outdoor games to competitive sports. Thus, despite some challenging hurdles early in childhood, with treatment your child may be expected to live a normal, healthy, active life, and walk tall into adolescence and adulthood.
Other questionsNaturally, you may have other questions about infantile scoliosis that are not answered in the above summary. If your child is not in the infant age group or may have another type of scoliosis, please refer to our descriptions of other types of scoliosis, please refer to our descriptions of other types of scoliosis, found here . We have also included links to other scoliosis websites, listed below, that detail some of the services and support groups available to people with the condition: National scoliosis foundation: http://www.scoliosis.org/ Scoliosis Research Society: http://www.srs.org/ Scoliosis World: http://www.scoliosis-world.com/cat21.htm Scoliosis Support Group: http://www.geocities.com/Athens/Troy/4314/index.html http://www.espine.com http://www.iscoliosis.com
Thank you for accessing our website. The physicians of the Pediatric Orthopaedic Surgery division of Columbia University College of Physicians & Surgeons, Morgan Stanley Children's Hospital of New York Presbyterian, and the New York Orthopaedic Hospital: David P. Roye, Jr., M.D. ? chief Juvenile (Idiopathic) Scoliosis
What is juvenile scoliosis?Juvenile idiopathic scoliosis is a condition that affects children between the ages of 3 and 9 and is characterized by the presence of an abnormal lateral curvature of the spine. This curvature causes the spinal column to bend to the left or the right, in the shape of an S or a C. It is found more frequently in girls than boys. The abnormal spinal curvatures in children with juvenile scoliosis are generally at a high risk for progression to more severe curves. They usually require bracing, and many will go on to require surgery.
The normal spineThe spine, or spinal column, as it is also called, is a complex structure made up of 33 vertebrae , or bony segments, arranged vertically in succession from just below the skull to the tailbone. The spine has two main functions: to support the weight of the skull and upper body, and to provide a protective encasement for the spinal cord, the long, cylindrical nervous structure that sends messages to and from the brain and the rest of the body. The vertebrae are attached to other vertebrae above and below them by a number of ligaments , thick fibrous structures found throughout the musculoskeletal system that attach bones to other bones. The connection points between the vertebrae are called joints , in which small areas of smooth cartilage lined with a lubricating material, known as synovial fluid , allow the bony vertebrae to slide past each other with minimal friction, allowing movement. Unlike joints such as the knee or elbow, which allow for considerable movement, each joint of the spinal column allows for very little movement because of the number of tightly attached ligaments, thereby ensuring the strength and stability of the vital supportive column. However, the joints of the spine act collectively, with the sum of movements in all the joints allowing movements such as bending forward, back, and to the side. The normal spine actually has a number of natural curves to it, which stems from the evolution of humans from four-legged mammalian ancestors who walked on the ground bent over, with the spine parallel to the ground. However, these normal curves are antero-posterior curves only; that is, they can only be seen when looking at the spine from the side. On the other hand, when looking from directly behind or in front of a person, the spine maintains a rigid upright structure, like a straight line. In other words, there are normally no lateral curves to the human spine. This upright structure is maintained by the body's natural positioning system, known as proprioception . When we move around on a normal basis, special nerve sensors called proprioceptors in the regions of all of our joints send millions of rapid signals to the brain with even the slightest movements. The brain processes all of these signals, monitoring exactly what the different parts of the body are doing, and sending signals back down to the muscles to alter the position of our body parts. Because the spine is made up of a large number of joints, all of which have their own proprioceptors, the brain is able to maintain finely tuned control over the spine and preserve its straightness, eliminating even the slightest lateral curves on a normal basis. Should there be even minor defects in the communication from the proprioceptors to the brain or in the brain's processing of the signals, one's ability to maintain an upright position would be compromised.
What causes juvenile scoliosis?As previously mentioned, the exact cause of juvenile scoliosis is unknown. However, the considerable amount of medical research that has gone into understanding scoliosis has led to the acceptance of several hypotheses for how the condition may develop. Because the juvenile form has similar clinical features to the adolescent form, the two conditions actually have a number of the same proposed causes. For example, there is evidence to suggest that deficits in proprioception, as described above, may be responsible for the development of juvenile scoliosis. Children may therefore not even realize when they are not positioning themselves exactly upright much of the time. As a result, the spine may progressively bend to one side to compensate for the deficit. In time, that bend maintains itself because the ligaments surrounding the vertebral joints may get stretched and then tighten according to whatever position is most often maintained. Because that position is one of lateral bending in some children, scoliosis ensues. As a result of the condition, the spine also rotates slightly on its axis, which causes the ribs on one side to rotate as well. The lateral bending can be difficult to assess on physical exam, because the spine is buried under several layers of muscle and skin, and cannot be viewed clearly without an x-ray. It is the rotational change of the spine that actually gets detected most frequently on physical exam, and a full clinical work-up for scoliosis follows (described further in the ' How is it diagnosed ' section). Another possible cause of developing juvenile scoliosis may be genetic inheritance. Because a number of reports have emerged of the condition occurring in many members of a single family, many scientists believe that a set of genes that code for the deficits causing scoliosis may be inherited in a dominant fashion. However, because the incidence of scoliosis does not occur in a traditional dominant pattern (i.e., not every child of parents who had scoliosis develops the condition, and vice versa), most likely not everyone expresses these genes to the same extent. This suggests that the scoliosis gene, or genes, may be dominant, but there is extreme variability in genetic penetrance , which means that the degree to which the genes end up affecting an individual is quite unpredictable. Research at several institutions has uncovered several different genes on different chromosomes that appear to be associated with scoliosis. Many thoughtful orthopaedic surgeons believe we will discover that scoliosis is a more heterogeneous condition than it appears from the clinical description. Despite the emergence of these and other hypotheses, much about the condition remains to be discovered and fully understood. As a result, there is ongoing research at a number of academic medical centers throughout the U.S., attempting to further uncover the keys to the development of juvenile scoliosis and improve its treatment and prevention.
How is it diagnosed?Juvenile idiopathic scoliosis is usually first suspected during a standard physical examination by a pediatrician. Such examinations include the Adams forward bending test , which consists of the child or adolescent, with his/her shirt removed, bending forward at the waist until the spine is parallel with the floor, while the physician observes the shape of the spine as it bends. As mentioned above, the abnormal rotation of the spine, called truncal rotation , will create a rib hump characteristic in AIS, in which the ribs on one side protrude slightly, compared to the other side, when bending forward. Mild curves in the spine generally do not cause back pain early in the condition, but may cause some stiffness or difficulty in bending forward completely, which may also be observed by a physician. When scoliosis is suspected, careful neurologic exam as well as exam of the head, back, and extremities should follow, to inspect for plagiocephaly, as described above, and to insure that the spinal cord is not being affected by another disease entity. A spinal MRI, a sophisticated radiological imaging technique, is essential to confirm the findings of the neurological exam and rule out other potential causes of scoliosis. A series of x-rays should also be taken, which allow for a more precise measurement of the severity of the curvature. As mentioned before, this requires the presence of a curvature angle of at least 10 degrees, measured with the Cobb method , as shown here: How is it treated?Decisions regarding the appropriate treatment for juvenile scoliosis depend on the severity of the spinal curvature at the time of diagnosis, the age of the patient, and the likelihood that the curvature will worsen in the future. Mild curves (10-25 degrees) are initially treated only with observation . This entails simply that the child be seen again within 6-8 months by his/her orthopaedic surgeon, who will monitor the progression of the curve with follow-up physical exams and x-rays. If a mild curve is observed to progress at a significant rate, or a child has a moderate to severe curve (>25 degrees) at the time of diagnosis, treatment is promptly begun, because of the high probability that the condition will worsen if untreated. If a child's curve is flexible (meaning that with some maneuvering, the spine can temporarily be moved towards an upright position, despite the abnormal curve), an external brace is the appropriate first line of treatment. Though there are a number of braces available for treatment of scoliosis, our pediatric orthopaedic group favors the use of a custom-made TLSO brace, or thoracic-lumbar-sacral orthosis, which has floating pads, designed to maximize patient comfort. Each patient's schedule for wearing the brace is determined by his/her orthopaedic surgeon, and is customized according to the response of his/her spine. If the curve is progressing quickly, full-time brace wearing is suggested, consisting of 18 to 23 hours per day. If the curve responds over time and decreases in severity, the brace wear time is reduced. Occasionally with juvenile scoliosis, bracing may be discontinued for one or more years, with a return to observation, as described above. As the child begins an adolescent growth spurt, he/she will likely be re-braced to maintain the previously achieved correction of the curve. In other instances, curves worsen significantly after brace removal, which may necessitate reinstitution of full-time bracing treatment or possibly surgery. For curves that continue progressing during bracing treatment, surgery is the appropriate treatment option. A small number of children have rigid curves at the time of diagnosis, which means that the spine is fixed in its abnormal position, and cannot be temporarily straightened. In such cases, the most appropriate initial treatment is serial casting , which entails that a cast be applied and changed every 6 to 12 weeks, in an attempt to gradually correct the curvature. The cast is made of plaster, and is applied in the operating room under general anesthesia , which means that the child will be put to sleep through the application process, so as to minimize discomfort. Casting treatment is followed up with bracing treatment, which is needed to maintain the correction. Surgical treatment options are generally the same for both infantile and juvenile idiopathic scoliosis. Operations may consist of instrumentation , in which metal rods are attached to the spine to maintain curve correction and/or spinal fusion , in which two or more of the vertebrae are fused together with bone bridges made of bone grafts . Surgeries may be performed through a posterior approach , in which the operation is performed with an incision in the back, or also with an anterior approach , which is a more extensive surgery that requires making an opening in the chest wall to reach the front part of the spinal column. Surgeries utilizing the anterior approach have been revolutionized in the last decade or so with the advent of thoroscopic spine surgery, in which smaller openings are made and special cameras are used to visualize the spine, thereby minimizing scars and recovery time. For a more detailed description of this type of surgery, click here. For some children in the 3-8 year-old age range, a device known as a ?growing rod? may be utilized. In this type of treatment a metal rod attached to the spine is periodically lengthened by a simple procedure. This correction without fusing the spine minimizes any stunting of growth that can occur with fusion. Several other types of experimental surgical treatments are currently under investigation and have shown promising results, including those performed by our pediatric orthopaedic group here at Columbia. One procedure, called an anterior epiphysiodesis, is designed to limit the growth of the spine in the region of the instrumentation, so that the existing curve does not worsen and new curves not emerge. Artificial ligaments, attached to one side of the spine, may be effective in stabilizing the spine over time and are also being investigated. Because of the stability and effectiveness of the devices used in spine operations today, patients are usually able to walk the day following their surgery, and hospital stays are generally under 1 week. The majority of children return to school within 4 weeks and can often return to full activity within a year, though contact sports are not recommended.
Coping with juvenile scoliosisManaging the demands of bracing treatment for scoliosis can be a challenging task for children and their parents alike. Surgery at such a young age, if necessary, can seem somewhat daunting as well. However, research has shown that the treatments for juvenile idiopathic scoliosis are generally extremely successful. The vast majority of children grow up without any limitations to their activities and daily functioning, from simple outdoor games to competitive sports. Thus, despite some challenging hurdles early in childhood, with treatment your child may be expected to live a normal, healthy, active life, and walk tall into adolescence and adulthood.
Other questionsNaturally, you may have other questions about juvenile scoliosis that are not answered in the above summary. If your child is not in the juvenile age group or may have another type of scoliosis, please refer to our descriptions of other types of scoliosis, found here . We have also included links to other scoliosis websites, listed below, that detail some of the services and support groups available to people with the condition: National scoliosis foundation: http://www.scoliosis.org/ Scoliosis Research Society: http://www.srs.org/ Scoliosis World: http://www.scoliosis-world.com/cat21.htm Scoliosis Support Group: http://www.geocities.com/Athens/Troy/4314/index.html http://www.espine.com http://www.iscoliosis.com
Thank you for accessing our website. The physicians of the Pediatric Orthopaedic Surgery division of Columbia University College of Physicians & Surgeons, Morgan Stanley Children's Hospital of New York Presbyterian, and the New York Orthopaedic Hospital: David P. Roye, Jr., M.D. ? chief
Adolescent Idiopathic Scoliosis (AIS)
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Decisions regarding the appropriate treatment for congenital scoliosis depend on the type and location of the spinal deformity and the likelihood that the curvature will worsen in the future. Some mild curves (10-25 degrees) may be treated only with observation . This entails simply that the child be seen regularly by an orthopaedic surgeon, who will monitor the possible progression of the curve with physical exams and x-rays over time. Visits must continue into adolescence, because growth spurts often trigger progression, even in a previously non-progressive curve. If progression does occur in this group, the patient may require bracing or surgical treatment.
Some basic facts about neuromuscular scoliosis |
- Scoliosis is technically defined by the presence of a lateral curvature in the spine of >10 degrees deviation from straight upright (described further in the 'n the ' How is it diagnosed ?' section).
- The earlier the curve develops in neuromuscular scoliosis, the more likely it is to progress to a more severe curve. Likewise, the more severe a curve is when it is first detected, the faster it will progress, on average.
- Unlike most cases of idiopathic scoliosis, in which the curves occur in a limited region of the spinal column and are relatively short, so to speak, neuromuscular scoliosis is associated with long curves that often extend to the sacrum, the region at the bottom of the spinal column.
- Neuromuscular curves are often associated with a condition known as pelvic obliquity , in which the child's pelvis is unevenly tilted, with one side positioned higher than the other side.
- Children with neuromuscular scoliosis usually do not experience any pain from the condition.
The normal spine
The spine, or spinal column, as it is also called, is a complex structure made up of 33 vertebrae , or bony segments, arranged vertically in succession from just below the skull to the tailbone. The spine has two main functions: to support the weight of the skull and upper body, and to provide a protective encasement for the spinal cord, the long, cylindrical nervous structure that sends messages to and from the brain and the rest of the body. The vertebrae are attached to other vertebrae above and below them by a number of ligaments , thick fibrous structures found throughout the musculoskeletal system that attach bones to other bones. The spinal column is also surrounded by a large number of muscles, known as paraspinal muscles, which offer support for the spine and contract to dictate its movement. The connection points between the vertebrae are called joints , in which small areas of smooth cartilage lined with a lubricating material, known as synovial fluid , allow the bony vertebrae to slide past each other with minimal friction, allowing movement. Unlike joints such as the knee or elbow, which allow for considerable movement, each joint of the spinal column allows for very little movement because of the number of tightly attached ligaments, thereby ensuring the strength and stability of the vital supportive column. However, the joints of the spine act collectively, with the sum of movements in all the joints allowing movements such as bending forward, back, and to the side.
The normal spine actually has a number of natural curves to it, which stems from the evolution of humans from four-legged mammalian ancestors who walked on the ground bent over, with the spine parallel to the ground. However, these normal curves are antero-posterior curves only; that is, they can only be seen when looking at the spine from the side. On the other hand, when looking from directly behind or in front of a person, the spine maintains a rigid upright structure, like a straight line. In other words, there are normally no lateral curves to the human spine. This upright structure is maintained by the body's natural positioning system, known as proprioception . When we move around on a normal basis, special nerve sensors called proprioceptors in the regions of all of our joints send millions of rapid signals to the brain with even the slightest movements. The brain processes all of these signals, monitoring exactly what the different parts of the body are doing, and sending signals back down to the muscles to alter the position of our body parts. Because the spine is made up of a large number of joints, all of which have their own proprioceptors, the brain is able to maintain finely tuned control over the paraspinal muscles and preserve its straightness, eliminating even the slightest lateral curves on a normal basis. Should there be even minor defects in the communication from the proprioceptors to the brain, in the brain's processing of the signals, or in the tone or contraction of the paraspinal muscles, one's ability to maintain an upright position would be compromised.
Because these myriad neuromuscular diseases are so varied in their clinical presentation, the actual pathophysiology (i.e. the set of events or process that ultimately brings about a condition) of scoliosis in these diseases is also varied. However, there are several features that are common across many, if not all, of the predisposing diseases.
What causes neuromuscular scoliosis?
Unlike idiopathic scoliosis, which is the most common type of scoliosis and does not have an established cause, neuromuscular scoliosis is generally quite well understood, with regard to causation. In all cases, the underlying neuromuscular condition is felt to be the trigger for the development of scoliosis.
In neuropathic scoliosis, defects or abnormalities in the central nervous system (i.e. the brain and spinal cord) leads to altered proprioception, described above, or diminished control of the paraspinal muscles. As a result, abnormal forces will be transmitted upon the vertebral units of the spine, either because the muscles show increased motion (termed spasticity , as seen in cerebral palsy), decreased motion (also referred to as flaccidity , as occurs in Friederich's ataxia), or motion that is out-of-sequence (called dyskinesia, also seen in cerebral palsy).
Myopathic scoliosis is caused in a similar fashion, but through the direct affects of various diseases upon the muscles themselves, despite normal nervous systems. In many of these conditions, the most common of which is muscular dystrophy, the muscles undergo atrophy , or gradual wasting of the muscle tissue. Because the muscles will atrophy at different rates, scoliosis can result when the paraspinal muscles or other muscles of the trunk waste more quickly or significantly on one side of the body than the other, if even in a very small section of the spine. This throws off the stability of the spinal column, and leads to the curvatures seen in scoliosis. Other muscle diseases, such as arthropryposis, cause contractures of the muscles, which means they are excessively flexed, and remain fixed in a contracted position because of fibrosis of the muscles. This can occur in trunk and paraspinal muscles, obviously transmitting abnormal lateral forces on the normally symmetrical spine.
Because the neuromuscular changes in so many of these diseases arise in childhood, the spine is still in the process of growing when it experiences these abnormal forces, which generally worsens curves as growth spurts occur.
How is it diagnosed?
Neuromuscular scoliosis is usually first detected during a standard physical examination by a pediatrician, noticed by a child's parents, or during a full workup for the child's neuromuscular condition. The physical will be followed by a series of x-rays, which allow for a more precise measurement of the possible presence and severity of one or more curves. As previously mentioned, this requires the presence of a curvature angle of at least 10 degrees, measured with the Cobb method , as shown here:
A full neurologic exam of the back and extremities will be performed to insure that no other spinal conditions are present and that the spinal cord is not being affected by the presence of the abnormal curvature, if it is not already affected by the underlying condition. Occasionally, a spinal MRI, another radiological imaging technique, will be taken to compliment the spinal x-rays.
How is it treated?
Decisions regarding the appropriate treatment for neuromuscular scoliosis depend on the severity of the spinal curvature at the time of the diagnosis, the age of the patient, and the symptoms of the existing neurological or muscular disorder. The curves in almost all of the predisposing diseases have a high rate of progression, and almost all children will therefore require surgery at some point. However, in some instances, bracing, though not a definitive form of treatment, will be utilized to slow the progression of the curve until a later time that surgery can be safely performed.
Bracing treatment usually involves wearing an external brace when the child is upright, but not when they are sleeping or lying flat. Though there are a number of braces available for treatment of scoliosis, the molded TLSO brace (thoraco-lumbo-sacral orthosis) is the preferred design, because it is effective when worn periodically and does not significantly constrict breathing, as might occur with some of the other designs. In general, the brace should improve a patient's ability to sit or stand, as well as perform certain functions otherwise not possible. The brace should be worn for only several years, or until the decision has been made to pursue surgical treatment. Wheelchair seating adaptations also exist that can act similarly to bracing, and may be appropriate for some patients.
Surgeries for neuromuscular scoliosis are relatively complex, because of the other biological effects that are common in underlying neuromuscular diseases, such as respiratory difficulties, weak bones, and poor nutrition. Operations generally consist of instrumentation , in which metal rods are attached to the spine to maintain curve correction and spinal fusion , in which two or more of the vertebrae are fused together with bone bridges made of bone grafts . Fusion between the spine and the pelvis may also be necessary with cases of pelvic obliquity and particularly in children with muscular dystrophy. Surgeries may be performed through a posterior approach , in which the operation is performed with an incision in the back, or also with an anterior approach , which is a more extensive surgery that requires making an opening in the chest wall to reach the front part of the spinal column. Because of the stability and effectiveness of the devices used in these spine operations today, patients usually do not require bracing treatment following surgery, and hospital stays are usually limited to 7 to 10 days.
Coping with neuromuscular scoliosis
The development of scoliosis on top of an existing neuromuscular condition can be a difficult challenge for children and their parents alike. Surgery at a young age, if necessary, can seem somewhat daunting as well. However, because of the affects that scoliosis can have if left untreated, it is imperative that parents promptly seek out diagnosis and management of the condition by an orthopaedic surgeon. Surgical treatment of neuromuscular scoliosis is generally quite successful in reducing curves and improving the quality of patients' lives. Our pediatric group here at the Children's Hospital of New York is dedicated not only to the outcome of the treatment steps along the way, but also to the end goal of making your child's present and future life as comfortable, happy, and fulfilling as possible.
Other questions
Naturally, you may have other questions about neuromuscular scoliosis that are not answered in the above summary. If your child does not have a neurologic or muscular disorder or may have another type of scoliosis, please refer to our descriptions of other types of scoliosis, found here . We have also included links to other scoliosis websites, listed below, that detail some of the services and support groups available to people with the condition:
National scoliosis foundation: http://www.scoliosis.org/
Scoliosis Research Society: http://www.srs.org/
Scoliosis World: http://www.scoliosis-world.com/cat21.htm
Scoliosis Support Group: http://www.geocities.com/Athens/Troy/4314/index.html
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The physicians of the Pediatric Orthopaedic Surgery division of Columbia University College of Physicians & Surgeons, Morgan Stanley Children's Hospital of New York Presbyterian, and the New York Orthopaedic Hospital:
David P. Roye, Jr., M.D. ? chief
Joshua E. Hyman, M.D.
Francis Y. Lee, M.D.
Michael G. Vitale, M.D., M.P.H.
Innovative techniques in spine surgery
Thoracoscopic spine surgery
- Introduction
- What happens during the procedure?
- Who can have thoracoscopic spine surgery?
- Who should not have thoracoscopic spine surgery?
- What are the advantages for thoracoscopic surgery over the open approach?
- What are potential complications of thoracoscopic spine surgery?
- What are future developments with thorascopic spine surgery?
- Conclusion
- References
Introduction
Spine surgery is one of the most rapidly evolving fields in orthopaedics. New techniques have revolutionized the post-operative course of patients, curtailing post-operative pain and accelerating a return to normal function. Video-assisted thoracoscopic surgery (VATS) is one of these promising techniques. Surgeons have performed thoracoscopic surgery (using cameras to operate within the chest cavity) for over 80 years. In the early 1990s spine surgeons began applying thoracoscopic techniques to correct spine deformities including scoliosis. Since then, VATS has become a new powerful tool in the surgeon's armamentarium. (1, 5, 7) At the Morgan Stanley Children's Hospital of New York we are optimizing the use of this tool to benefit our patients.
Surgeons use two main approaches to correct scoliosis curves.
1. The anterior approach: Vertebrae are visualized and fused from the front through a chest wall opening.
2. The posterior approach : Vertebrae are visualized and fused from the back through a midline back incision.
Each approach can incorporate:
a) Fusion : eliminating the vertebrae growth plates and connecting them with bone ?bridges? so that they will not bend or curve as the patient grows.
b) Release : removing the intervertebral discs and ablating the joints between the vertebrae to ?free up? the spine.
c) Instrumentation : placing laminar hooks or inserting screws through the vertebral pedicles and connecting them with a rod to straighten the curve.
Traditionally, the anterior open approach has required a full thoracotomy (chest wall opening) to access the thoracic vertebrae. The approach, though effective, does leave a significant chest wall scar. With the advent of VATS, surgeons strived to obviate the need for the large incision and leave the patient with only the small incisions from the VATS camera and tool portholes. Currently, we employ the technique to successfully perform anterior fusion with instrumentation and can also combine it with a conventional posterior approach.
What happens during the procedure?
During the thoracoscopic procedure, the anesthesiologist placed a breathing tube in the patient that has a double lumen (one lumen in each lung bronchus) in order to control the inflation of each lung independently. Using this tube, the anesthesiologist collapses one lung to allow the surgeon access and workspace within the chest. The surgeon then cuts several small skin incisions along the side of the chest wall that will serve as portholes for a fiber-optic camera and surgical instruments. [Figure 1.] The camera magnifies and makes the structures inside the chest easier to identify .
During the procedure, fluoroscopy (live x-ray imaging) is used to orient the surgeons to the vertebral levels that are being fused. Once the surgeon identifies the thoracic spine, vertebral discs are removed (release), bone-graft is added to fuse the vertebrae together and instrumentation (screws and rods) are placed. The bone graft can be harvested from the patient herself (e.g. a piece of rib) or from prepared bone graft material such as demineralized bone matrix. In our experience, children do well with either type. After the operation, the patient usually wears a brace for about 3 months. (5)
Who can have thoracoscopic spine surgery?
Thoracoscopic surgery is most helpful for patients who require a release of the anterior thoracic spine. Instrumentation can also be added to the procedure.
Anterior release is typically indicated in:
1. Severe or rigid scoliosis and kyphosis:(1,4) Removal of anterior vertebral disks and anterior spinal ligaments increases the flexibility of the spine and facilitates a better correction of the curve.
2. Young patients: Because they have much growth left, young children are at risk for developing a post-operative crankshaft phenomenon. This phenomenon results from continued growth of the anterior spine after fusing the posterior spine only. Ablating the anterior vertebral growth plates during VATS prevents this from happening.
Indications for VATS with instrumentation (rods and screws) include:
1. A single thoracic curve with either no or a very mild lumbar curvature that does not cross the midline.
2. Curves that are relatively supple and present in young patients
3. Low thoracic curves (below vertebral level T5)
Who should not have thoracoscopic spine surgery? |
Contraindications for thoracoscopic spine surgery include:
1. Patients with curves larger than 90°: These curves result in a small chest cavity that makes VATS difficult. (2)
2. Small children: Children have to be large enough to handle a double lumen breathing tube, and have adequate lung function to tolerate single lung ventilation.
3. Patients with scarring in the chest cavity: (History of previous chest cavity surgery, empyema or sever pneumonia) This makes thoracoscopic surgery difficult, (2, 5)
4. Certain patients with neuromuscular disease: These patients are more likely to suffer from osteopenia (thin, brittle bones) be on seizure medications, both of which are predisposing factors for more bleeding. (1) Excessive bleeding obstructs the thoracoscopic camera view, making the surgery very difficult. Open procedures should be considered for these patients.
Contraindications for thoracoscopic instrumentation include:
1. Curves above vertebral level T5 or below L2 are technically very difficult to instrument thoracoscopically(2, 5)
2. Male patients with severe, rigid curves: VATS uses smaller rods than in open surgery. This makes the rods more prone to break or loosen, especially in the rigid curves seen in male patients.
Many studies have overwhelmingly shown that thoracoscopic spine surgery anterior release and fusion is safe and effective, at least within a two year follow-up period. (1, 4, 6-8, 15, 17, 18, 19) In fact, it is at least as effective as the open procedure. Long term results are not yet available.
Ultimately, success with VATS can only improve with time, experience and a motivated, thorascopically trained team of surgeons, anesthesiologists, nurses and surgical technicians. (1,2) The pediatric orthopaedic surgery team at the Morgan Stanley Children's Hospital of New York has been and continues to be at the forefront of VATS development and is among the best trained in the procedure.
What are the advantages of thoracoscopic surgery over the open approach?
Thoracoscopic surgery can be preferable to the open approach for several reasons. One, it spares the chest wall muscles, decreasing postoperative pain, improving cosmetic results and hastening a return to normal function.(2,5) Two, it leaves smaller skin incisions, which are more aesthetically appealing and which should decrease the risks of wound infection. Three, it should lead to a shorter stay in the hospital and less pain for the patient. VATS does not add any time in the hospital for a child who has also had a simultaneous posterior fusion. Furthermore, studies are beginning to show that VATS leads to a shorter hospital stay than the open approach. (5) This is exactly what we have seen in our experience.
What are potential complications of thoracoscopic spine surgery?
There are also drawbacks to the thoracoscopic approach. The one major drawback is that we do not have the long term follow up data or large studies to truly know how well the procedure works and what the complication rates are.
Open thoracotomy and thoracoscopic spine surgery share some of the same potential complications. (4) These include:
1. Pulmonary complications
2. Urine tract infections.(11)
3. Injury to blood vessels leading to blood loss,
4. Injury Injury to lymphatics leading to lymphatic fluid in the chest cavity (chyothorax)
5. Injury to nerves and spinal cord.
6. Pleural scarring/ lung damage: However, some studies have shown that the thoracoscopic approach results in better pulmonary function than the open approach. (9, 10)
Other complications are associated uniquely with the thoracoscopic approach. These include:
1. Intercostal neuralgia: Nerves located around the ribs are injured by the portal placements(5)
2. Tension pneumothorax (lung collapse) can occur if the guiding pin for the screws injures the lung. This is treatable with careful monitoring.(3) 3. Complications of anesthesia: Related to single lung ventilation.
4. Hardware problems: The screws on the upper vertebrae may come out.
5. Pseudoarthrosis (failure of the bone to fuse) has also been reported.(1, 2)
With With proper monitoring, most complications are reversible and treatable.(2, 4, 6) In addition, occasionally, cases that are attempted thoracoscopically may need to be converted into open approaches. Reasons for this are difficulty ventilating with in just one lung, unknown scarring and excessive bleeding.(16)
What are future developments with thorascopic spine surgery?
Recent technological advances, including new tools for controlling bleeding and more refined instruments have improved the thoracoscopic procedure.(1) For example, soon we will have instruments that can remove disk fragments while still remaining in the chest cavity. The instrumentation rods and screws are always being modified to give better results. (5) Development and approval of better bone graft material is being actively researched. For example, bone morphogenic proteins, contained in bone marrow, accelerate bone formation. By using these proteins, it may be possible to improve fusion rates without using an autogenous bone graft (a rib from the patient). This would decrease both the invasiveness and duration of the procedure, since there would be no need to harvest bone from the patient (12) Ultimately, thoracoscopic gene therapy may play a role in anterior fusion by decreasing the amount of dissection necessary.(13) Technologies to assist the surgeon, such as voice-activated robotics to guide the camera, special ?virtual? glasses with inbuilt monitors and three-dimensional video assistance, may be further developed and refined. (5)
Conclusion
Thoracoscopic anterior spinal surgery is a relatively new treatment. The surgery team at the Morgan Stanley Children's Hospital of New York boasts considerable experience and success with this minimally invasive approach to anterior release of the thoracic spine. In experienced hands, it is a safe, effective treatment option for a sub-group of patients. With time and further experience, we will learn more about additional uses of minimally invasive spine surgery, including thoracoscopic instrumentation, in an effort to provide patients with the best options for the treatment of scoliosis.
References |
1. Newton PO, Shea KG, Granlund KF. Defining the Pediatric Spinal Thorascopy Learning Curve ? Sixty-Five Consecutive Cases. Spine 2000; 25(8):1028-35.
2. Regan JJ. Point of View. Spine 2000; 25(8):1028-35.
3. Roush TF, Crawford AH, Berlin RE, Wolf RK. Tension Pneumothorax as a Complication of Video-Assisted Thorascopic Surgery for Anterioir Correction of Idiopathic Scoliosis in an Adolescent Female. Spine 2001; 26(4): 448-50.
4. Newton PO, Wenger Dr, Mubarak SJ, Meyer RS. Anterior Release and Fusion in Pediatric Spinal Deformity ? A Comparison of Early Outcome and Cost of Thoracoscopic and Open Thoracotomy Approaches. Spine 1997; 22(12):1398-1406.
5. Crawford AH, Wall EJ, Wolf RK. Video-Assisted Thoracoscopy. Orthopedic Clinics of North America 1999; 30(3):367-385.
6. McAfee PC, Regan JR, Zdeblick T, Zuckerman J, Picetti GD, Heim S, Geis WP, Fedder IL. The Incidence of Complications in Endoscopic Anterior Thoracolumbar Spinal Reconstructive Surgery ? A Prospective Multicenter Study Comprising the First 100 Consecutive Cases. Spine 1995; 20(14):1624-32.
7. Huntington CF, Murrell WD, Betz RR, Cole BA, Clements DH 3 rd , Balsara RK. Comparison of Thoracoscopic and Open Thoracic Discectomy in a Live Ovine Model for Anterior Spinal Fusion. Spine 1998; 23(15):1699-702.
8. Graham EJ, Lenke LG, Lowe TG, Betz RR, Bridwell KH, Kong Y, Blanke K. Prospective Pulmonary Function Evaluation Following Open Thoracotomy for Anterior Spinal Fusion in Adolescent Idiopathic Scoliosis. Spine 2000; 25(18):2319-25.
9. Furrer M, Rechsteiner R, Eigenmann V, Signer C, Althaus U, Ris HB. Thoracotomy and thocoscopy: Postoperative Pulmonary Function, Pain and Chest Wall Complaints. Eur. J Cardiothorac Surg 1997; 12(1):82-7.
10. Kaseda S, Aoki T, Hangai N, Shimizu K. Better Pulmonary Function and Prognosis with Video-Assisted Thoracic Surgery than with Thoracotomy. Ann Thorac Surg 2000; 70(5):1644-6.
11. McDonnell MF, Glassman SD, Dimar JR 2 nd , Puno RM, Johnson JR. Perioperative Complications of Anterior Procedures on the Spine. J Bone Surg Am 1996; 78(6):839-47.
12. Sucate DJ, Hedequist D, Pierce B, Zhang H, Colby S, O'Brien S, Welch RD. rh BMP-Induced Anterior Spinal Fusion in a Thoracoscopically Instrumented Animal Model. Presented at Pediatric Orthopaedic Society of North America (POSNA), Annual Meeting 2001, May 1-5. Paper No. 26.
13. Riew KD, Lou J, Wright NM, Cheng S.-L, Bae T, Aviolo LV. Thoracoscopic Intradiscal Spine Fusion Using Gene Therapy. Scoliosis Research Society 35 th Annual Meeting, Oct 18-21, 2000. Paper No. 72.
14. Newton PO, Lee SS, Mahar AT, Farnsworth CL, Weinstein C. Comparison of Autograft Vs. Demineralized Allograft in Anterior Thoracoscopically Instrumented ?Scoliosis? Fusions ? A Caprine Model. Scoliosis Research Society 35 th Annual Meeting, Oct 18-21, 2000. Paper No. 71.
15. Betz RR, Lenke LG, Clements DH, Newton PO. Thoracoscopic Versus Open Thoracotomy Correction by Anterior Instrumentation for the Treatment of Thoracic Adolescent Idiopathic Scoliosis. Scoliosis Research Society 35 th Annual Meeting, Oct 18-21, 2000. Exhibit #18.
16. Knapp DR. VATS, An Initial Learning Curve and Reasons for Conversion to Thoracotomy. Scoliosis Research Society 35 th Annual Meeting, Oct 18-21, 2000. Exhibit #63.
17. Picetti GD, Bueff HU. Endoscopic Instrumentation, Correction and Fusion of Thoracic Curves in Idiopathic Adolescent. Scoliosis. Research Society 35 th Annual Meeting, Oct 18-21, 2000. Paper #54.
18. Arlet V. Anterior Thoracoscopic Spine Release in Deformity Surgery: A Meta-Analysis and Review. Eur Spine J 2000 Suppl 1:S17-23.
19. Early SD, Newton PO, Wenger DR and Mubarak SJ. The Results of Anterior Thoracoscopic Spine Surgery in Children Under Thirty Kilograms. . Scoliosis. Research Society 36 th Annual Meeting, Oct 18-21, 2001. Paper #60
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The physicians of the Pediatric Orthopaedic Surgery division of Columbia University College of Physicians & Surgeons, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center:
David P. Roye, Jr., M.D. ? chief
Joshua E. Hyman, M.D.
Francis Y. Lee, M.D.
Michael G. Vitale, M.D., M.P.H.
Growing Rods
For some children in the 3-8 year-old age range, a device known as a "growing rod" may be utilized. In this type of treatment a metal rod attached to the spine is periodically lengthened by a simple procedure. This correction without fusing the spine minimizes any stunting of growth that can occur with fusion. Several other types of experimental surgical treatments are currently under investigation and have shown promising results, including those performed by our pediatric orthopaedic group here at Columbia. One procedure, called an anterior epiphysiodesis, is designed to limit the growth of the spine in the region of the instrumentation, so that the existing curve does not worsen and new curves not emerge. Artificial ligaments, attached to one side of the spine, may be effective in stabilizing the spine over time and are also being investigated. Because of the stability and effectiveness of the devices used in spine operations today, patients are usually able to walk the day following their surgery, and hospital stays are generally under 1 week. The majority of children return to school within 4 weeks and can often return to full activity within a year, though contact sports are not recommended.
The Titanium Rib
The spine center at the Morgan Stanley Children's Hospital of New York Presbyterian is unique in New York in offering this new and exciting treatment for the infant or child with thoracic insufficiency syndrome. Thoracic Insufficiency Syndrome has been defined as the inability of the chest to support normal breathing or lung growth. If the chest cannot grow normally, the child's lungs cannot grow and life threatening breathing problems may develop. The VEPTR has been designed to allow the rib cage to grow while controlling spinal deformity without fusion of the spine. Before this technique was developed, there was no effective treatment for the combination of chest wall deformity and scoliosis that created problems with lung growth and pulmonary function.
Some testing is required before the surgery. Regular x-rays are of course performed. The children will require a CT scan and an MRI. Pulmonary function evaluation and consultation with pediatric pulmonology is also required. All of our patients are part of a clinical research protocol that allow us to track and measure the outcome of the surgery and thus to insure the best possible results.
The surgery is performed under general anesthesia with the child on his or her side. The incision is seen in the illustration. The ribs are exposed and are separated allowing correction of the rib cage. One or two of the VEPTR devices are placed depending on the extent and type of deformity. On some occasions, the devices are attached to the spine and to the ribs. The initial procedure requires admission to the hospital for three to five days. The child is treated in the pediatric intensive care unit until the staff is confident that she is healthy enough to be moved to the regular floor.
In the post operative period there is no casting or bracing necessary and regular activities are not limited. To keep the rib cage growing, the VEPTR must be lengthened every 4 to 6 months. The lengthening surgeries are generally performed as outpatient surgery and only a small portion of the incision is used to perform the lengthening.
In this special group of children, the VEPTR technique offers real promise in preventing the late sequelae of inadequate growth of the chest wall and thus inadequate growth of the lung and subsequent breathing problems. We are excited to be able to treat this set of patients, to offer a solution that truly changes the prognosis of thoracic insufficiency syndrome.
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