Neuromuscular Scoliosis

What is neuromuscular scoliosis?

Neuromuscular scoliosis is a side-to-side spine curvature that happens alongside conditions that affect your child’s nervous system and muscles. Vertebrae (spine bones) don’t align straight in the middle of your child’s back. Instead, they rotate slightly off-center, which makes your child’s spine form in a C or S shape.

It’s common for parents or caregivers to remind children to sit up straight at the dinner table or stop slouching. But with neuromuscular scoliosis, your child isn’t acting up or ignoring you. Sitting up straight is a constant challenge for them to do. It could even be uncomfortable.

The curve in your child’s back can affect more than their posture. You might also notice that your child’s clothing isn’t fitting right. One sleeve may be longer than the other or the buttons don’t align with the holes on the left and right sides. It’s not because their shirt shrunk in the wash or the clothing wasn’t manufactured well. This is the result of neuromuscular scoliosis.

Scoliosis symptoms usually become noticeable during adolescence and growth spurts. But with neuromuscular scoliosis, signs and symptoms may appear before adolescence.

The spine curvature may get worse as your child gets older. The progression could cause pain and other complications that surgery can treat.

Is neuromuscular scoliosis progressive?

Yes, neuromuscular scoliosis is progressive. This means that the curve in your child’s spine gets worse over time. Since the curve is present in early childhood, it may quickly get worse when your child has a growth spurt. Spine curves may also progress through adulthood.

How common is neuromuscular scoliosis?

Neuromuscular scoliosis is common with other nervous system and muscle conditions like muscular dystrophy, atrophy and cerebral palsy. An estimated 90% of people diagnosed with Duchenne muscular dystrophy and 80% of people diagnosed with spinal muscular atrophy develop scoliosis. Scoliosis affects between 20% to 70% of people diagnosed with cerebral palsy.

What are the signs of neuromuscular scoliosis?

The signs of neuromuscular scoliosis include:

• Uneven shoulders.
• Ribs that stick out on one side.
• Tilted hips and waist.

Among children who use a wheelchair, you may notice:

• Slouching to one side.
• A slanted pelvis.
• Difficulty sitting (using hands or arms for support).
• Pressure sores.

In addition to a sideways spine curve, your child may also have other curves like kyphosis (front-to-back curve) or lordosis (swayback).

What are the symptoms of neuromuscular scoliosis?

As the curve progresses, your child may feel the following symptoms with neuromuscular scoliosis:

• Back pain.
• Discomfort when sitting.
• Muscle weakness.
• Numbness (arms and legs).
• Limited range of motion.
• Difficulty with balance and coordination of their head, trunk and neck.

What causes neuromuscular scoliosis?

An underlying condition that affects your child’s nervous system and/or muscles causes neuromuscular scoliosis. Your child may not be able to move or control certain muscles in their body with neuromuscular conditions. This can weaken the area around their spine, causing a curve to form.

Common conditions associated with this type of scoliosis include but aren’t limited to the following:

• Cerebral palsy.
• Duchenne muscular dystrophy.
• Paralysis.
• Spina bifida.
• Spinal muscular atrophy.

What are the risk factors for neuromuscular scoliosis?

Your child is more likely to develop neuromuscular scoliosis if they have a neuromuscular condition and/or regularly use a wheelchair for mobility.

What are the complications of neuromuscular scoliosis?

Severe curves from neuromuscular scoliosis may cause the following complications:

• Respiratory problems like difficulty breathing and thoracic insufficiency syndrome.
• Difficulty maintaining independence like getting dressed on your own or eating meals, for example.
• Mobility challenges and difficulty walking.
• Nerve damage.
• Cardiovascular problems.

Your child might experience low self-esteem related to neuromuscular scoliosis and the condition that caused it. It’s difficult to manage more than one underlying health condition at a time and the toll it can take on your child’s body puts them at a higher risk of conditions like depression or anxiety. A mental health professional can help your child navigate how these diagnoses affect their emotional well-being.

How is neuromuscular scoliosis diagnosed?

To diagnose neuromuscular scoliosis, a healthcare provider will perform a physical exam to learn more about your child’s symptoms. They’ll review their medical history and family medical history. During the exam, your provider will look closely at your child’s spine, their posture, balance and skin (to look for pressure sores). They may use a scoliometer to measure the spinal curve. Your child’s provider may also order imaging tests, like a spine X-ray or MRI (magnetic resonance imaging), to get a better look at their spine.

How is neuromuscular scoliosis treated?

A healthcare provider may recommend nonsurgical neuromuscular scoliosis treatment options first, like:

• Wheelchair adjustments or modifications: If your child uses a wheelchair, a healthcare provider may suggest adding a custom seat backing or lateral supports (side positioners) to help your child balance and navigate comfortably.
• Physical therapy: A physical therapist can help your child practice exercises to strengthen affected muscle groups in their body. This can address balance issues and improve their independence.
• Brace: Wearing a brace may or may not be recommended based on your child’s situation. For example, if your child uses a wheelchair, a brace around your child’s torso may help them balance while seated but it doesn’t stop the curve from getting worse. A brace isn’t usually safe for children who walk since it might make them more at risk of falls and losing their balance.

Nonsurgical treatments may delay or slow down the progression of scoliosis, but they don’t correct the curve.

Surgery for neuromuscular scoliosis can stop the progression and stabilize your child’s spine. A healthcare provider may consider surgery if your child has:

• A curve greater than 40 degrees that continues to progress (get worse).
• Pain or discomfort.
• Complications that affect lung or heart function.

Your child’s provider will consider their general health, the underlying neuromuscular condition type and the severity of their scoliosis before determining what type of treatment is safest for your child’s situation.

Neuromuscular scoliosis surgery

The types of surgery to treat neuromuscular scoliosis include:

• Spinal fusion: A surgeon will realign your child’s spine and connect (fuse) affected vertebrae bones together. Your child’s surgeon will use bone grafts, metal rods and screws to hold the bones in place until the two bones heal into one solid bone. Spinal fusion stops the fused bones from growing after the procedure, which may result in short stature.
• Growing rods: This is a type of surgery best for children who are still growing. A surgeon will place a rod on your child’s spine. This rod stabilizes their spine and corrects the curve. A surgeon can lengthen the rod with a minor procedure or via magnetic lengthening (which can be done in office) every six to eight months to adapt to your child’s growth. A surgeon will remove the rod and perform spinal fusion when your child reaches skeletal maturity (stops growing).

Since your child is managing an underlying condition in addition to neuromuscular scoliosis, they’re at a higher risk of surgical complications like infections or nerve damage. Complications are also more likely because your child’s surgeon may need to address more than one area of their spine at a time.

Your child’s care team will evaluate whether surgery is a safe option and tell you about the possible side effects before the procedure so you can make an informed decision about your child’s care.

Can neuromuscular scoliosis be prevented?

You can’t prevent neuromuscular scoliosis. With an early diagnosis and treatment, healthcare providers may be able to slow the progression of the curve or correct it with surgery.

What is the prognosis for neuromuscular scoliosis?

Your child’s prognosis (outlook) can vary based on:

• Their general health.
• The type of condition that caused their scoliosis.
• The curve severity and progression.
• Treatment options available for their situation.

While surgery may correct the curve, there are risks associated with the procedure. Your child’s care team will offer treatment that’s the safest for your child’s physical and emotional well-being. If you have any questions about your child’s prognosis, talk to their provider.

What’s the life expectancy for neuromuscular scoliosis?

Neuromuscular scoliosis may or may not affect your child’s life expectancy. Severe cases can cause heart and lung complications that may be life-threatening. Your child’s care team can advise you on what to expect if they’re at risk of severe complications.

When should my child see a healthcare provider?

Visit your child’s healthcare provider if you notice signs of scoliosis, like difficulty sitting, uneven shoulders or pressure sores.

If your child has neuromuscular scoliosis, talk to their provider if they experience symptoms like pain or discomfort, or any complications.

What questions should I ask my child’s healthcare provider?

Questions you may want to ask your child’s healthcare provider include:

• What type of treatment do you recommend?
• Does my child need surgery?
• What are the side effects of the treatment?
• How do I keep my child safe and comfortable?

A note from Cleveland Clinic

The last thing you want to hear is that your child has not one but two conditions that can affect their overall health. You might be worried about what the future looks like and how you can help your child stay comfortable with neuromuscular scoliosis. As a parent or caregiver, this can be stressful, but you don’t have to handle it on your own.

Since neuromuscular scoliosis is a progressive condition, you’ll need to visit your child’s healthcare team more regularly. You’ll get to know them even better than you already do. During these visits, a provider will measure the curve in your child’s spine. A surgeon may consider surgery if the curve is severe, and if the risks of surgery don’t outweigh the benefits for your child. If you have any questions about your child’s outlook or how you can help them at home, talk to their providers.