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 curvature of the spine. This curvature causes the spinal column to bend to the left or right, forming an S or C shape. It is more common in girls than in boys. The abnormal spinal curvatures in children with juvenile scoliosis are generally at high risk of progression to more severe curves. They usually require bracing, and many will go on to require surgery.

Basic Facts About Juvenile Scoliosis

• Scoliosis is technically defined by the presence of a lateral curvature in the spine of >10 degrees.
• 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 differs from the other two types of scoliosis because it develops during a period when the spine does not undergo significant growth.
• For unknown reasons, 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 that bend to the right.
• While several types of scoliosis have well-understood causes, juvenile scoliosis is considered idiopathic, meaning its causes are, for the most part, unknown.
• 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.

What Causes Juvenile Scoliosis?

The exact cause of juvenile scoliosis is unknown. However, the considerable amount of medical research into scoliosis has led to the acceptance of several hypotheses about how the condition may develop. Because the juvenile form shares clinical features with the adolescent form, the two conditions have several of the same proposed causes.

For example, there is evidence suggesting that a lack of understanding of where their body is positioned in space 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. Over time, that bend tends to persist because the ligaments surrounding the vertebral joints may stretch and then tighten 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. Lateral bending can be difficult to assess on physical exam because the spine is obscured by several layers of muscle and skin, and cannot be visualized clearly without an X-ray. It is the rotational change of the spine that is most frequently detected on physical exam.

Another possible cause of juvenile scoliosis is 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, meaning the degree to which the genes affect an individual is unpredictable.

Research at several institutions has identified genes on different chromosomes that appear to be associated with scoliosis. Many thoughtful orthopedic 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, ongoing research at a number of academic medical centers throughout the U.S. is seeking to further uncover the mechanisms underlying the development of juvenile scoliosis and to improve its treatment and prevention.

How Is It Diagnosed?

Juvenile idiopathic scoliosis is usually first suspected during a standard physical examination by a pediatrician, noticed by a child’s parents or during a screening test at school. Such examinations have the child bend forward at the waist until the spine is parallel with the floor, while the physician observes the shape of the spine as it bends.  If scoliosis is present, when the child bends forward, the abnormal rotation of the spine creates a rib hump, with the ribs on one side sticking out slightly.  If a rib hump or sign of scoliosis is found, then a standing x-ray series is recommended to make a definitive diagnosis of scoliosis. As mentioned before, this requires a curvature angle of at least 10 degrees.

If scoliosis is found, you will be referred to a Pediatric Orthopaedic Surgeon to discuss the available treatment options.

A neurologic exam of the back and extremities will be performed during the physical exam and, if indicated, with additional diagnostic testing to ensure that no other spinal conditions are present and that the spinal cord is not affected by the abnormal curvature.

A spinal MRI, a sophisticated radiological imaging technique, is essential to confirm the neurological exam findings and rule out other potential causes of scoliosis. A series of X-rays should also be taken, allowing for a more precise measurement of the curvature's severity.  As mentioned before, this requires a curvature angle of at least 10 degrees, measured with the Cobb angle.

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 patient's age, 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 rapidly, 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.  

Treatment could consist of:

Scoliosis casting

Scoliosis bracing

Schroth Therapy.  

The type of treatment initiated depends on several factors, including age and the severity of the curve.

The younger patient may be a candidate for Scoliosis Casting. Learn more about Scoliosis Casting.

Some children, depending on their age, curve size, and other factors, may be able to participate in a scoliosis-specific type of physical therapy.  This therapy is called Schroth therapy.  This may be started before bracing or concurrently with bracing. Read more about Schroth Physical Therapy.

Either after Scoliosis Casting or in older Juvenile Scoliosis patients, bracing is initiated.  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 may 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. Read more about Scoliosis Bracing.

For curves that continue to progress during bracing treatment, surgery may be the appropriate option. For children who experience progression of scoliosis, early intervention is indicated to prevent chest wall deformity and to allow normal lung development.

The Pediatric Orthopedic Surgeons at NewYork-Presbyterian Morgan Stanley Children’s Hospital are dedicated to advancing treatment options for Early Onset Scoliosis through research and clinical innovation.

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 lengthened as the child grows. This correction, without fusing the spine, minimizes the growth stunting that can occur with fusion. Several other types of surgical treatments are currently under investigation and have shown promising results, including those performed by our Pediatric Orthopaedic group here at Columbia.

Different types of procedures:

• Growing Rod/VEPTR
• MAGEC Rod
• Vertebral Stapling

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 treatments for juvenile idiopathic scoliosis are generally highly effective. The vast majority of children grow up without any limitations to their activities and daily functioning, from simple outdoor games to competitive sports. So, despite some challenging hurdles early in childhood, with treatment, your child can expect to live a normal, healthy, active life and walk tall into adolescence and adulthood.