Scoliosis, Kyphosis and Spine Deformity

scoliosis

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,

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,

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 .

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.

 

  • 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 ‘ How is it diagnosed? ‘ section).
  • Most infants that develop abnormal curves do so in the first 6 months of life
  • Infantile scoliosis is a rare condition, accounting for less than 1% of all cases of idiopathic scoliosis
  • The condition is more common in Europe than in the U.S.
  • For unknown reasons, the curve in the spine tends to bend to the left in infants with scoliosis. However, girls who develop curves that bend to the right have a worse prognosis than other infants.
  • While there are several types of scoliosis for which the causes or origins of the disease are well understood, infantile scoliosis is considered idiopathic , which means the causes are, for the most part, unknown.
  • For reasons not yet understood, it has been found that children who develop scoliosis before the age of 5 are more likely to have cardiopulmonary abnormalities in infancy.
  • Infants with idiopathic scoliosis usually do not experience any pain from the condition.

 

The normal infant 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 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.

 

causes infantile scoliosis

As previously mentioned, the exact cause of infantile scoliosis is unknown. However, the considerable amount of medical research that has gone into understanding scoliosis has led to the development of two main hypotheses for how the condition may be caused. There is evidence to suggest that intrauterine molding may be responsible for the development of infantile scoliosis. With intrauterine molding, the spine is affected during fetal growth because of abnormal pressures exerted by the walls of the uterus on one side of the fetus’ body, or abnormal positioning of the fetus within the uterus. This hypothesis is supported by epidemiological data, which demonstrate that there are higher rates of plagiocephaly (a slight flattening of one side of the head) and developmental dysplasia of the hip (a condition that affects one of the hips) on the same side as the spinal curve in infants with idiopathic scoliosis. A second theory suggests that postnatal external pressures are exerted on the spine after birth, perhaps due to an infant being positioned on his/her back for extended periods of time in the crib. This may explain the higher incidence of the condition in Europe, where infants have traditionally been positioned more on their backs and less on their abdomens, compared to in North America. However, a few years ago the American Academy of Pediatrics (AAP) announced a formal recommendation that newborns be positioned on their backs when sleeping, due to research studies suggesting that Sudden Infant Death Syndrome (SIDS) and other respiratory problems are associated with babies being positioned on their abdomens. Despite the possible relation of this recommended position to infantile scoliosis, our pediatric orthopaedic group supports the policy of the AAP. A final hypothesis for a possible cause of infantile scoliosis is that of genetic inheritance. While the exact genes that may be involved have yet to be identified, research has shown that there is a higher incidence of the condition within some families, lending considerable weight to the probability of a genetic component.

Despite the notable amount evidence for these 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 infantile scoliosis and improve the treatment and prevention of the condition.

 

diagnose

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:

 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.

Managing 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.

Naturally, 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:


Juvenile (Idiopathic) 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.
 
facts about juvenile 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 ‘ How is it diagnosed? ‘ section).
  • Juvenile scoliosis is more common than infantile idiopathic scoliosis, but less common than adolescent idiopathic scoliosis. The juvenile form accounts for between 12% and 21% of all cases of idiopathic scoliosis.
  • The condition is different than the other two types of scoliosis because it develops during a period in which the spine does not undergo significant growth.
  • While juvenile is, overall, more common in adolescent girls than boys, the presence of curves in children 3 to 6 years old is actually equally common between the two sexes. However, in the age group of 6 to 10 year-olds many more girls than boys have curves, suggesting that some cases of infantile scoliosis goes undetected in males.
  • For unknown reasons, the curve in the spine tends to bend to the right in children with juvenile scoliosis. Children with curves that bend to the left tend to have a better prognosis than those bending to the right.
  • While there are several types of scoliosis for which the causes or origins of the disease are well understood, juvenile scoliosis is considered idiopathic , which means the causes are, for the most part, unknown.
  • Unlike adolescent idiopathic scoliosis, in which curves rarely become progressively more severe, the curves in juvenile scoliosis often progress. Seven out of ten children with this condition will worsen and require active treatment. Juvenile curves almost never resolve spontaneously.
  • Children with juvenile idiopathic scoliosis usually do not experience 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 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.

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.

diagnose

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:

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.

 Managing 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.

Naturally, 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:

 

 Congenital Scoliosis

Congenital scoliosis is a condition that affects newborns or young infants and is characterized by the presence of an abnormal lateral curvature of the spine. Children with this condition actually begin to develop the curvature before birth, during the period of fetal growth in the womb. The curvature causes the spinal column to bend to the left or the right, in the shape of an S or a C. Abnormal development is considered the cause of the condition. It occurs when the bony spinal segments, or vertebrae , fail to form properly, seemingly creating ?extra’ segments, or when normally separate vertebrae 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. Congenital scoliosis has a relatively high rate of other, concurrent spinal deformities associated with it, such as kyphosis (an abnormal forward-bending curvature) and lordosis (an abnormal backward-bending curvature), which can also occur independently. Treatment ranges from observation to surgery, depending on the appearance of the spinal curvature.
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 ‘ How is it diagnosed ?’ section).
  • The abnormal curves in congenital scoliosis tend to be more rigid than those in idiopathic scoliosis, making them more resistant to correction.
  • Curve progression in congenital scoliosis, particularly with mild curves, is somewhat unpredictable, underscoring the importance of frequent follow-ups with an orthopaedic surgeon. Around 10% to 25% of curves, usually mild, remain stable and never progress at all. Most curves, however, progress and require active treatment.
  • Sometimes the ribs of children with progressive early scoliosis become fused together, thereby reducing the volume of the rib cage.  In this condition, known as thoracic insufficiency syndrome , the limited chest volume interferes with lung growth, causing problems with breathing.
  • Neonates born with scoliosis have a relatively high rate of other congenital abnormalities, such as anatomical anomalies of the genito-urinary tract (found in around 20% of congenital scoliosis patients) or congenital heart defects (found in around 10% of congenital scoliosis patients).
  • Children with congenital 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 vertebral 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.

 

As mentioned above, there are two major causes of congenital scoliosis. The first is called failure of formation , in which portions of one or more vertebrae do not grow together to completion during fetal development, making the spine unstable in certain regions and creating the appearance of ?extra’ spinal segments. The second major cause is referred to as an error of segmentation , in which bony regions of the vertebral column that normally grow into distinct segments actually fail to separate and end up fused together. When evidence for both causes is present in a scoliotic spine, it is called a mixed deformity . However, with either cause, the abnormalities often occur in multiple areas of the spine, and they often occur more on one side than another, at least at some level of the spinal column. This asymmetry is responsible for the development of the lateral curvature in the womb, for as the muscles and ligaments develop around the spine and apply their natural forces to the vertebra, the two different sides have unequal stability and unequal responses to those forces.

Frequently, failures of formation and errors of segmentation also occur to an unequal degree on the front and back portions of one or more vertebrae. The result is not an abnormal lateral curve, but an abnormal forward-bending or backward-bending curve, known as kyphosis and lordosis , respectively. These conditions are commonly observed concurrently in newborns with scoliosis, and require treatment as well. Another set of conditions commonly seen in congenital scoliosis is rib abnormalities. Like the vertebrae, ribs are segmented structures that extend from the spinal segments in the back to the sternum , or breastbone, in the front. Thus, they are also affected by failures of formation, which leads to missing ribs, and errors of segmentation, which leads to fused ribs. Rib abnormalities may alter the normal shape of the chest cavity and compromise a child’s ability to breathe. In such instances, correcting the problems with the ribs will be incorporated into the treatment plan.gnosed?

Congenital scoliosis is usually first detected in the initial newborn physical examination by a pediatrician or noticed by a child’s parents soon after birth. Because of the increased incidence of other congenital abnormalities in the setting of scoliosis in the newborn, a very thorough examination must be performed on such children, including auscultation (i.e. listening with a stethoscope) for heart murmurs and an evaluation of the renal tract (i.e. kidney and urinary tract). Therefore, all patients suspected of having congenital scoliosis should receive an abdominal ultrasound to inspect the kidney and surrounding tissues. In addition, a 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. The physical will be followed by a series of x-rays, which allow for a more precise measurement of the presence and severity of a 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:

Frequently, a spinal MRI, another radiological imaging technique, will be taken to compliment the spinal x-rays. Once a clear radiological picture of the spine has been attained, a pediatric orthopaedic surgeon can often predict the chance of curve progression based on the specific appearance of the curve, and will make treatment decisions based on this prediction.  

 

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.  

Bracing treatment is less commonly used in congenital scoliosis than other types of scoliosis, mostly because the curves tend to be more rigid in newborns, and do not respond to the gentle forces of a brace. In some instances, however, bracing is appropriate and will be fitted individually for the patient, with a number of pads to maximize the child’s comfort in the device. Except for bathing, the brace is to be worn all the time, and the progression of the curve will be monitored closely by your orthopaedic surgeon. Occasionally, the curve is completely controlled the brace, and after many years of bracing, no further treatment is needed. For curves that progress despite bracing treatment, prompt surgery is warranted.

In most instances of congenital scoliosis, a surgical operation is the most appropriate treatment. Operations may consist of removal of the ?extra’ vertebrae or some type of 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. Fusion operations may be followed by casting or bracing treatment, or may involve instrumentation , in which metal rods are attached to the spine to maintain curve correction. A device known as a ?growing rod? may also be utilized, which is attached to the spine and periodically lengthened by a simple procedure, and will minimize any stunting of growth that could occur in certain cases.

 

For children that suffer from thoracic insufficiency syndrome, the titanium rib prosthesis is a revolutionary new type of treatment used to compliment scoliosis surgery.  The expandable prosthesis is implanted in a vertical position and attached to an upper and lower rib. The device helps maintain the chest space where there are fused or missing ribs, thus allowing room for heart and lung development. As the patient grows, the prosthesis is simply expanded through an outpatient surgical procedure.

While this procedure is new and only appropriate for a small number of children with scoliosis, it offers an exciting new option to children with congenital scoliosis.

 

A major spine operation during a child’s neonatal or infant stage can be an emotional experience for many parents. Managing the demands of bracing or casting treatment for scoliosis has its drawbacks as well. However, the importance of such treatment steps at an appropriate, early age cannot be overemphasized for the future health of your child. Moreover, research has shown that the various treatments for congenital scoliosis are generally extremely successful. The vast majority of children grow up without major limitations to their activities and daily functioning, from simple outdoor games to competitive sports. Thus, despite some challenging hurdles very early in childhood, with treatment

Neuromuscular Scoliosis
 Neuromuscular scoliosis is a condition that affects children with neuromuscular disorders and is characterized by the presence of one or more abnormal lateral curvatures 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. Because there is such a wide variety of the types of diseases that may cause neuromuscular scoliosis, the clinical presentation and severity of this condition is extremely variable. However, most children with this disease have poor balance and poor coordination of their trunk, neck, and head. There is also a high frequency of concurrent kyphosis , which is an abnormal forward-bending curve of the spine. The condition is generally classified into two categories, based on the type of disease that the child suffers from, and which causes the abnormal curve. Neuropathic scoliosis involves diseases that are primarily afflictions of the neurvous system, such as cerebral palsy or spinal cord trauma, whereas myopathic scoliosis involves muscular disorders, like muscular dystrophy or arthrogryposis. Unlike the other types of scoliosis, treatment of neuromuscular scoliosis is very unpredictable, mostly because the abnormal curves of the spine are also unpredictable.
 
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.

 

 

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.

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.

 

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.  

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.

REFERENCE

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.
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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.
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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.
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email : judarwanto@gmail.com,

 

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