Early Onset Scoliosis: An Overview

Introduction

Like many aspects of growth in young children, healthy development of the spine can vary slightly from child to child with small curves constituting a normal part of spine anatomy. But if curves are observed by a parent, teacher, school nurse, or physician, evaluation for early onset scoliosis is advisable.

Early onset scoliosis is the name given to curves greater than 10 degrees in a child under the age of five years. This umbrella term includes those children diagnosed with infantile scoliosis; congenital scoliosis (a curve that is present at birth); scoliosis arising from neuromuscular conditions such as cerebral palsy, spina bifida or spinal cord tumors; and syndromic scoliosis that results from a variety of conditions such as Marfan syndrome or a skeletal dysplasia. 

Diagnosis

Pediatric orthopaedists use physical examination and x-rays to diagnose early onset scoliosis. An initial x-ray is taken to determine the magnitude, location, and direction of the curve. Based on that x-ray, a determination is made regarding the type of scoliosis present, as well as its possible cause, and a treatment strategy is instituted.

X-ray showing curvature of the spine in a patient with early onset scoliosis

At HSS, in children younger than ten years of age, an MRI of the entire spine is often recommended to ensure that there are no other problems affecting the spinal cord.  Children with congenital scoliosis should be assessed for the presence of any cardiac or kidney problems associated with their condition.

Treatment Options

Treatment decisions must take into account the age of the patient, the type of scoliosis, the size of the deformity, and the  anticipated progression of the curve, according to John S. Blanco, MD, Associate Attending Orthopaedic Surgeon at Hospital for Special Surgery (HSS). “The period from birth to five years is crucial, because it is during this time that the lungs grow dramatically,” explains Dr. Blanco. If the chest cavity is constricted owing to scoliosis or other spinal deformities, lung growth can be significantly restricted and serious pulmonary complications may develop.

For all patients with scoliosis, the goals of treatment are to slow or prevent progression of the curve and to achieve cosmetic improvement where possible. Based on all the information available, the pediatric orthopaedist may recommend one or more of the following:

Observation

For patients with smaller curves, those greater than 10 degrees and up to 20 degrees, the pediatric orthopaedist may recommend careful monitoring of the condition with physical examinations and follow-up x-rays taken at three to four month intervals. If the curve progresses, additional treatment measures are introduced. 

Bracing

For curves in the range of 20-40 degrees, bracing can be an effective means of controlling some forms of early onset scoliosis, such as idiopathic scoliosis and some syndromic forms of the condition. (However, bracing is not appropriate for neuromuscular or congenital scoliosis.) Moreover, it must be emphasized that bracing does not correct the curve. Bracing is intended to prevent progression.

Surgical Correction

Young children with curves that exceed 40 degrees and that are progressing despite non-operative treatment are in danger of developing cardiac and/or respiratory problems and are therefore candidates for surgical intervention. Pediatric orthopaedists use two primary devices: growing rods and vertical expandable prosthetic titanium rib prostheses (VEPTR). These are growth-sparing techniques that allow for control and correction of the scoliosis while the spine continues to grow.

X-ray images showing a spinal curve in excess of 40 degrees (top) and the same patient undergoing treatment with growing rods (bottom)

Growing rods are expandable devices that are attached to the top and base of the spine using screws or hooks. Every six months the orthopaedic surgeon lengthens the rod by about one centimeter, which is the amount of growth expected in the spines of young children. While the initial surgery to attach the growing rods lasts two or more hours, subsequent adjustments are brief procedures involving only a small incision and, in otherwise healthy children, may not require an overnight stay in the hospital. When the device has reached its full extension, the child may require another surgery to introduce a new longer set of growing rods.

X-ray images showing a spinal curve in excess of 40 degrees (top) and the same patient undergoing treatment with a VEPTR device (bottom)

For children with chest wall deformities, such as those seen in congenital scoliosis, the VEPTR device is usually the best option since bracing is ineffective in this population. In contrast to growing rods, the VEPTR is attached to the patient’s ribs. It not only helps to straighten the spine, but also separates the ribs to prevent deterioration of breathing function that can develop with untreated scoliosis. As with growing rods, small adjustments are made to the VEPTR every six months to allow for growth.  

While most patients with growing rods or VEPTR devices will eventually undergo a fusion to permanently maintain the correction, Dr. Blanco notes that the creator of the original VEPTR device, Robert Campbell, MD, has patients who have finished growing and continue functioning well with the VEPTR in place and no formal fusion performed.

Some families of children in whom growing rods or VEPTR devices are placed have concerns about the risks associated with repeat surgeries, according to Dr. Blanco. However, he says, even children with multiple medical problems can tolerate these procedures well in the hands of experienced specialists.

Casting - A “New” Option for Early Onset Scoliosis

A renewed interest in casting for early onset scoliosis has occurred. Casting can produce good results in children with infantile idiopathic scoliosis and those with syndromic scoliosis. Popularized recently by British physician Dr. Min Mehta, the technique employs a series of body casts to correct the curve. Extending from just under the arm pit - some also have “straps” that go over the shoulders - to the curve of the waist area, Risser casts remain on the patient for six weeks at a time. The cast is then changed to increase the amount of correction. “The theory behind the technique is that by keeping the child in the cast around the clock, you actually help the spine start growing in a more normal way,” says Dr. Blanco. This process continues for several months to years, usually with a two- or three-day interval between castings to allow the patient to bathe and to address any skin problems that may develop.

“Because the cast remains on at all times, except for these brief breaks, the technique offers an advantage over braces which are removed for bathing and changing clothes,” says Dr. Blanco. Braces can be effective in arresting progression of a curve, but can’t correct it. “With casting, in the right patient, complete correction of the deformity is possible.” he adds. Presently, casting for the treatment of scoliosis is only available at a few centers in the United States. 

The prognosis for patients with early onset scoliosis has improved significantly over the last ten years, according to Dr. Blanco. “We have lots of tools to treat the condition. It’s a matter of finding which one is best for the individual patient.” However, he stresses, when considering where to seek treatment, it’s important to look for an institution where a high volume of procedures are performed, there is a good medical support team in place, and the anesthesiology staff have experience working with patients with early onset scoliosis.

Looking to the Future

The next advances in the treatment of early onset scoliosis - as well as for other forms of scoliosis - may grow out of genetic research that seeks to predict which curves are going to progress. One Utah-based group has developed a DNA analysis of saliva that is showing promise in this area.

Genetic counseling can also benefit patients with syndromic or neuromuscular types of early onset scoliosis that run in families. While it won’t affect the course of treatment for the individual patient, counseling can help parents understand and anticipate what other organ systems may be affected and alert them to the possible need for other children in the family to be evaluated.

To find out more about the treatment of early onset scoliosis at HSS, please visit the Physician Referral Service or call 1 (877) 606-1555.