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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.

Some basic 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 juvenile scoliosis 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 percent and 21 percent 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.

Causes of 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 juvenile scoliosis 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.

Diagnosing juvenile scoliosis

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.

Treating juvenile scoliosis

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 four weeks and can often return to full activity within a year, though contact sports are not recommended.

Coping with juvenile scoliosis

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.

For more information

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.

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