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Physical Examination

Before the physical examination, your child's doctor will discuss your child's family and medical histories and overall health, including which symptoms are present and when they began.

During the physical exam, your doctor will closely examine your child's back and spine. In addition, they will assess any problems with sitting balance, skin breakdown, or daily function.

(Left) This adolescent girl with cerebral palsy uses a wheelchair and has a large sweeping curve to the left. (Right) Her X-ray shows a pelvic tilt, which may cause sitting imbalance and lead to pressure sores.

Courtesy of Texas Scottish Rite Hospital for Children

Imaging Tests

Imaging tests help doctors assess the severity of your child's NMS.

X-rays. X-rays provide clear images of dense structures such as bone. An X-ray will allow your child's doctor to see the location of the curve on the spine and to measure the degree of the curvature.

Magnetic resonance imaging (MRI) scans. An MRI scan provides clear images of soft tissues and helps your child's doctor evaluate the health of the spinal cord.

Nonsurgical Treatment

Although nonsurgical treatments will not prevent your child's curve from progressing, they may delay the progression and help improve function and quality of life. Nonsurgical treatments include:

Wheelchair modification. This is the most commonly recommended nonsurgical treatment. If your child has a shifted pelvis that affects balance, their wheelchair can be modified to improve posture. Modifications may include custom-molded chair backings and adjustment of the side positioners to help improve balance while sitting.

Bracing. For some patients in wheelchairs, the treatment team may recommend wearing a molded plastic brace around the upper body. This may provide a more stable sitting position and allow for use of the arms and hands. However, bracing will not prevent the progression of NMS curves, as it can in idiopathic scoliosis, and is not definitive treatment for NMS.

For NMS patients who are able to walk, wearing a brace may make them more likely to fall or lose their balance while walking — especially if they have significant muscle weakness, lung problems, or an unsteady gait.

Physical therapy. Specific exercises may help address your child's muscular imbalances and improve physical functioning.

Surgical Treatment

Indications for surgery. Your child's treatment team will consider several things when determining whether to recommend surgery to control the spinal curve, including how severe the curve is and how much it affects your child's function. The most common indications for surgery include:

• Curves greater than 50 degrees (in patients who are still growing)
• Curves greater than 50 degrees that have progressed more than 10 degrees (in patients who have reached skeletal maturity)
• Difficulty sitting and functional deterioration
• Pain
• Problems with heart or lung function

The decision to proceed with surgery in patients who cannot communicate is often very difficult and relies on the size of the spinal curve, the impact of the curve on sitting balance, and the care of the patient. Input from the family and/or caregiver is critical in this decision-making process.

Because NMS results from an underlying neuromuscular condition, the underlying condition guides the treatment strategy. For example, surgical decompression — a surgery that involves removing bone that may be compressing structures within the spine — may prevent curve progression in patients with hydrosyringomyelia (a fluid sac within the spinal cord). Patients who undergo this procedure may not need spinal stabilization.

Surgical stabilization of the spine. The goals of this treatment are to:

• Stabilize the curve and stop its progression
• Balance the spine and pelvis, thereby increasing sitting tolerance if the patient is in a wheelchair or improving walking if the patient can walk
• Improve lung function
• Reduce pain

Growing Rods. Most operations to stabilize the spine also stop growth in the segment of the spine that is operated on. Temporary spinal stabilization with growing rods may be necessary in young children to allow the spine, chest, and lungs to grow as much as possible. The goal of a growing rod is to allow continued growth while correcting the curve and improving daily function.

During the procedure, the doctor attaches one or two rods to the spine above and below the curve. Every 6 to 8 months, the child returns to the doctor for a minor procedure to lengthen the rod(s) to keep up with the child's growth. When the child is fully grown, the doctor replaces the rod(s) and performs a spinal fusion.

A newer alternative is to implant magnetically-powered rods (MAGEC) that can be lengthened in the doctor's office, eliminating the need for repeated invasive surgeries. Some patients with NMS may not be candidates for MAGEC rods because of other magnetically or electrically placed implants. 

Growing rods can stabilize the spine and also allow for continued spine and chest wall growth. (Left) This side-view of the spine shows how the rods are fixed into the upper and lower vertebrae with screws. (Right) This illustration shows how the growing rods are placed in the spine during the surgery. Also shown are the surgical instruments used to hold back the skin and soft tissues to allow the surgeon to access the spine.

Reproduced from Akbarnia BA, Blakemore LC, Campbell RM, Dormans JP: Approaches for the very young child with spinal deformity: what's new and what works. Instr Course Lect 2010; 59:407-424.

Spinal Fusion. Spinal fusion is the most common surgical procedure used to treat NMS.

During the procedure, the curved vertebrae are fused together so that they heal into a single, solid fusion (bone mass). This will stop the growth completely in the abnormal segment of the spine and prevent the curve from worsening in the future.

Spinal fusion involves placing small pieces of bone graft between the vertebrae to be fused in an effort to increase the likelihood of a successful fusion. These graft pieces are obtained from a bone bank or made from an artificial material. Over time, the bone grows together — similar to when a broken bone heals.

Metal rods are typically used to hold the spine in place until the fusion happens. The rods are attached to the spine by hooks, screws, and/or wires. Once the bone graft fuses with existing bone, the straightened spine does not move, and the curve will not progress.

In patients who are still very immature and growing rapidly, spinal fusion may require both an anterior (front) and posterior (back) approach. This two-sided approach can help to avoid crankshaft syndrome, a problem in which areas of the spine that are still growing continue to rotate, while the instrumented areas remain fixed.

Because patients in wheelchairs often also have a pelvic tilt, it is common for the fusion to extend from the upper thoracic spine down to the sacrum (base of the spine) and pelvis.

(Left) This patient with cerebral palsy has a large spinal curve to the left. (Right) The spine has been stabilized by two rods and multiple screws, bringing the curve to a more normal alignment.

Courtesy of Scottish Rite Hospital for Children

Complications

Because NMS is associated with other underlying neuromuscular disorders and because surgery for NMS typically involves more areas of the spine, the complication rate after surgery is higher than with some other types of scoliosis. Your child's doctor will talk with you about potential complications and the steps taken prior to surgery to prevent them. For example, your child's treatment team will work to maximize nutrition and heart and lung status prior to surgery to minimize the risk of infection and the prolonged need for being on the ventilator after surgery. Similarly, before surgery, the team will fully assess your child's skin in the region of the spine and pelvis to minimize the risk for infection.