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.

Scoliosis is diagnosed as several types:

• Idiopathic Scoliosis: of unknown origin
• Congenital Scoliosis: present at birth, a congenital scoliosis curve is where bones are asymmetrical at birth and the vertebrae may be partially formed (hemivertebra) or wedge-shaped
• Neuromuscular Scoliosis: is symptomatic of a systemic condition (syndromic scoliosis) such as cerebral palsy, muscular dystrophy, spina bifida, spinal cord tumors, or paralysis
• Syndromic Scoliosis: a unique group of spine conditions. Diseases such as Marfan’s Syndrome, Ehlers-Danlos Syndrome, osteogenesis imperfecta, neurofibromatosis, Prader-Willi Syndrome, arthrogryposis, and Riley-Day Syndrome are some of the common syndromic causes of this condition.

Causes of Scoliosis

The vast majority of scoliosis cases are due to unknown causes and can develop during infancy, childhood or adolescence. Patients with syndromic scoliosis often have curves in the spine early in life. As children with this condition grow, the curvature can progress and worsen. It’s important for a pediatric orthopedic surgeon to monitor the condition closely, because in some cases the curvature may eventually need to be treated.

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 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.

• Idiopathic Scoliosis: The most frequently seen form of the condition, idiopathic scoliosis may first be recognized during a routine pediatrician’s visit or in a school screening. While children with idiopathic scoliosis may not experience any pain, parents may see cosmetic signs of the condition, such as a shoulder that appears higher than the other or protruding ribs on one side, owing to a twisting aspect of the spine. Patients with idiopathic scoliosis are further categorized by age:
  • Infantile scoliosis, affecting children from birth to three years of age
  • Juvenile scoliosis from 3 to 9 years of age
  • Adolescent scoliosis, from 10 to18 years of age and seen more frequently in girls than in boys
• Syndromic Scoliosis: Children with syndromic scoliosis should also be evaluated by a geneticist and neurologist to determine which one of the disorders mentioned above, or others, could be the cause of the spine curvature. Sometimes these patients may have respiratory and cardiac conditions, related to the syndrome or secondary to severe spinal curvatures. Due to this risk, it is also important for these patients to be evaluated by a pediatric pulmonologist and cardiologist.
• Congenital Scoliosis: 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.

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. The period from birth to five years is crucial, because it is during this time when the most dramatic lung growth occurs. 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.

Casting: The Risser Cast

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 called the Risser Cast. 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 two to three months at a time, based on the age of the child. 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, sometimes 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.

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, such as the MAGEC (MAGnetic Expansion Control) System 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 Rod Technique

Growing rods (MAGec 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)

The VEPTR Technique

The VEPTR (Vertical Expandable Prosthetic Titanium Rib) technique works to straighten the spine and separate the ribs, to prevent respiratory problems. 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.

Fusion

In spinal fusion, two or more vertebrae are fused together with bone grafts and internal devices, such as metal rods, to stabilize the spine or correct a deformity. Sophisticated fusion techniques and new instrumentation to surgically correct progressive curves, enhances the recovery of patients. Such techniques include new endoscopic procedures that allow specialists to access the spine through the chest cavity, and perform the fusion with three or four small incisions.

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.

Learn more about pediatric orthopedic surgery in the HSS Guide to Pediatric Scoliosis.

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.

Updated: 3/14/2018

Summary by Nancy Novick

Authors

John S. Blanco, MD
Associate Attending Orthopedic Surgeon, Hospital for Special Surgery
Associate Professor of Orthopedic Surgery, Weill Cornell Medical College