Congenital scoliosis

Children’s Hospital Boston’s Spinal Program provides comprehensive care—including evaluation, diagnosis, consultation, surgery, non-surgical therapies and follow-up care.

Because congenital scoliosis may progress (worsen) with growth, your child must be followed until the end of his growth, with closest observation during his periods of rapid growth. How we treat your child’s congenital scoliosis will depend on several factors, including:

• the nature and severity of his curves
• his age upon presentation and his current age
• the rapidity of his curve's progression
• any associated conditions
• his general health, including lungs, chest, urologic and other systems

Treatment options include:

• observation and measurement:
  • If your child has smaller curves and curves that form balanced patterns, they can be minor enough to be followed and measured without treatment.
  • Your child’s doctor will closely observe the curve with sequential x-ray documentation, most frequently during his periods of rapid growth.
  • Observation of certain curve types can predict his curve’s likely progression, as well its likely speed of progression.
     
• bracing:
  • Bracing is not an effective therapy for treating congenital scoliosis curves, which are usually rigid and inflexible.
  • Bracing can be effective for controlling the secondary (compensatory) curves that can develop above or below the congenital curve.
  • A brace worn after spinal fusion surgery can protect your child’s spine while his fusion heals and becomes fully solid.
     
• surgery:
  • Some children with congenital scoliosis are treated by spinal fusion or other surgery to prevent further—or anticipated—progression of a child’s curve, usually by the time he reaches adolescence (earlier if a severe curve presents in a very young child).
  • The decision for surgery is made by assessing many factors—including your child’s age, the progression of his curve, and the amount, type and location of the deformity.

Surgical goals

The goals of spinal surgery for congenital scoliosis are to:

• reach the end of the child’s growth with a reasonably straight, balanced spine and a balanced torso
• allow the spine to grow normally as much possible (greater torso length than if he hadn’t been treated)
• prevent compromise of lung function by creating room for chest cavity and lungs to grow (especially in young children)
• prevent future back pain
• prevent damage to nervous system

Surgical options for spinal fusion

Your child’s surgeon has several surgical options for spinal fusion, depending on the specific features of your child’s congenital scoliosis—including, but not limited to his age, the progression of his curve, and the amount, type and location of the deformity.

Surgical approaches include:

• in situ spinal fusion: This surgery is performed “in place” to stop the abnormal growth of the spine before it becomes seriously deformed.
  
  Disc material in front, or cartilage in back, is removed and replaced with bone graft. The graft of healthy bone will eventually fuse with the abnormal vertebrae into a solid bone mass as it heals. Instrumentation (metal rods, wires, hooks and screws) can be used to help stabilize the fusion and partially straighten the spine. Casting or bracing for four to six months may be needed until the fusion occurs.
   
• hemivertebra resection (excision): Hemivertebrae are triangular- or trapezoidally-shaped vertebrae (instead of the normal rectangular shape). If located at the lower part of the spine, a hemivertebra can cause the base of the spine to tilt and the child to lean to one side.
  
  In this procedure, the abnormally-shaped vertebrae are removed, and the next vertebra above and below the hemivertebrae are fused together. Instrumentation is often inserted to support the fusion, and the child will wear a cast while the spine heals.
   
• reconstructive osteotomies and instrumentation: If your child’s spinal deformity is particularly severe and rigid, it may cause tilting of his spine, deterioration of his torso shape, breathing problems, pain and risk to the spinal cord.
  
  This severity of deformity may not be manageable with the treatments described above. Your child’s fixed, severe deformity may need resection of part of his vertebral column (vertebral column resection), followed by instrumentation to restore spinal balance.

Surgical alternatives to spinal fusion in the very young

If your child is developing a severe curve while very young, he can be treated with growth-friendly (growth preserving) surgical procedures such as growing rods and/or VEPTR. Metal rods inserted during these procedures can help control the curve until he’s ready for spinal fusion. The rods are made longer as the child’s spine grows:

• growing rods: Spinal fusion performed on a growing child can lead to some stunting of his torso height, and possible impairment of his chest and lung growth. Inserting growing rods (instrumentation without fusion) allows for continued growth of the spine while controlling the curve progression.
  
  The rods are attached to the spine above and below the curve. They help correct the curve, and are expandable through day surgery every four to six months as the child grows, until he’s ready for spinal fusion surgery.

• VEPTR (vertical expandable titanium rib): VEPTR surgery can treat a child with combined chest and spine deformity, particularly if the chest deformity is the more severe.
  
  The goal is to make one or both sides of the chest larger, longer or more normal in shape, to allow the child’s lungs to expand or grow. A larger, longer or straighter chest can also help his abnormal spine stay as straight as possible without spinal fusion, while still allowing for spine growth.
  
  One or more ribs are spread apart to make the chest larger (expansion thoracostomy). Sometimes, congenitally abnormal (fused) ribs are separated. The VEPTR expandable rod is then attached to healthy ribs above and below, or to ribs above and spine or pelvis below. For children with congenitally small chests on both the right and left, operations can be done on both sides.

Complications after surgery

Complications are uncommon after spinal surgery for congenital scoliosis. There’s a slight chance of infection or failure to heal, as well as a slight chance of developing a new curvature above or below the fusion. And there is a risk of serious neurological injury (paralysis) that’s higher than with most other forms of scoliosis.

Caring for your child after surgery

After your child’s spinal surgery, his Children’s pediatric orthopedist and physical therapist can advise you on at-home therapies, as well as any home therapy equipment you might need.

Longer-term outlook

The outlook for your child greatly depends on the nature and severity of his congenital scoliosis, as well as any other accompanying conditions:

• Many children function perfectly normally.
   
• Commonly, the spine is shorter than normal, even if treatment isn’t needed.
   
• The spine is stiffer than normal.
  • The degree of stiffness is relative to how much of the spine is involved.
  • Fusion makes the spine stiffer.
  • For some children, this is visible and dictates restricted activities.
     
• For some children with early, severe curves, breathing may be affected.

With successful spine surgery and attentive post-operative care, he can have a spine that grows as normally as possible, with greater torso length than if he hadn’t been untreated.

Coping and support

At Children’s, we understand that a hospital visit can be difficult, and sometimes overwhelming. So, we offer many amenities to make your child’s—and your own—hospital experience as pleasant as possible. Visit The Center for Families for all you need to know about:

• getting to Children’s
• accommodations
• navigating the hospital experience
• resources that are available for your family

In particular, we understand that you may have a lot of questions when your child is diagnosed with scoliosis and perhaps another associated condition, as well. How will this affect my child long term? What do we do next? Children’s can connect you with extensive resources to help you and your family through this stressful time, including:

• patient education: From doctor’s appointments to physical therapy and recovery, our nurses and physical therapists will be on hand to walk you through your child’s treatment and help answer any questions you may have—Why will my child need surgery? Are there non-surgical options? How long will his recovery take? How should we manage home exercises and therapy? We’ll help you coordinate and continue the care and support your child received while at Children’s.
   
• parent-to-parent: Want to talk with someone whose child has been treated for congenital scoliosis? We can often put you in touch with other families who’ve been through the same process that you and your child are facing, and who will share their experiences.
   
• faith-based support: If you’re in need of spiritual support, we’ll connect you with the Children’s chaplaincy. Our program includes nearly a dozen clergy—representing Protestant, Jewish, Muslim, Catholic and other faith traditions—who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.
   
• social work: Our social workers and mental health clinicians have helped many other families in your situation. We can offer counseling and assistance with issues such as coping with your child’s diagnosis, stresses relating to coping with illness and dealing with financial issues.