What is neuromuscular scoliosis?

Neuromuscular scoliosis is the name given to the type of scoliosis that happens in people with problems with their nervous systems (brain, spinal cord or nerves) or muscles. Common conditions that can result in a neuromuscular scoliosis include:

• Cerebral palsy
• Spinal muscular atrophy
• Syringomyelia
• Muscular dystrophy (eg Duchenne’s muscular dystrophy)
• Poliomyelitis

A scoliosis means that the spine is curved abnormally when viewed from the front or the back. It can lead to the trunk and shoulders no longer being symmetrical. Sometimes the shoulders aren’t level anymore. Sometimes the head is not level or the pelvis is not level. The rib cage can also push out on one side so that it becomes prominent - this is known as a rib hump. The severity of the abnormalities can vary between people and can change with time.

How would I know there was a neuromuscular scoliosis?

Either you, your child or someone else may notice that there are changes in the shape of the spine or that they are sitting or standing as straight as before.

You may notice:

• Rib cage sticking out on one side especially at the back
• Shoulders not being level
• The hip or waist sticking out
• The head tilting over to one side of the trunk
• The pelvis not being level
• Clothes not fitting well anymore
• Not being able to stand up straight or sit up straight
• There may be pain as well as the changes above

Why does it matter?

Sometimes the shape changes can become a big concern. The shape can change as your child grows, particularly at the time of growth spurts.

If scoliosis worsens, it can affect the mechanics of the spine and result in aching or pain.

It can affect walking efficiency, especially when there are other issues affecting walking due to the neuromuscular condition. It can affect seating, and make seating in a wheelchair uncomfortable. If the spine results in a lot of tilting of the trunk, it can affect function in a wheelchair and the ability to sit up straight and look ahead to interact with the environment.

A big curve can result in the ribs touching the pelvis bone causing pain and skin problems.

Occasionally, especially if the scoliosis affects someone early in life, it can affect the development and function of the lungs. This does need to be treated or at least monitored. The younger the age when the scoliosis starts, the more likely this may happen. If not monitored and treated when appropriate, this type of scoliosis can result in serious health problems related to the lungs in adulthood.

Generally, a scoliosis that has started early in life, can get a lot bigger due to all the growth that the spine will go through. A bigger curve can cause more of the problems described above.

What should I do if I think there is a neuromuscular scoliosis?

Speak to your GP, Paediatrician, Orthopaedic Surgeon or Physiotherapist looking after you or your child. They will be able to assess and see if there is a scoliosis.

Once a scoliosis is confirmed, you will need to see a Spine Surgeon at a recognised spine centre who will assess you fully and talk about what you may need. The GP, Paediatrician or Orthopaedic Surgeon will refer you on to the Spine Surgeon.

Do not ignore it if you think there is a scoliosis. More often than not, it will be nothing serious and just require monitoring at the hospital. However it is important to be sure, so having someone check you properly is important.

How will the doctors check for a scoliosis?

The first thing the specialist will do is talk to you about your concerns. They will ask questions about health generally, how the problems are affecting your child and try to get an idea how much more growth and development there is left.

The doctors will want to examine the spine and so your child will need to get undressed for this. Often the hospital will give you a gown, but wearing shorts and a T shirt may make things easier. The examination will look at the back and see how flexible it is. They will often look at the back whilst bent forwards if possible, as this reveals the scoliosis more (Adam’s position). Your child’s arms and or legs will often be examined to make sure the spinal cord is working normally. If standing or bending is not possible, the doctors will do the examination in the wheelchair and then on the couch in the clinic.

The Spine Surgeon will send your child for X - rays. These are necessary to see if there is a scoliosis and how bad it is. The Spine Surgeon will measure the angle of the scoliosis on the X - ray (Cobb angle). The X - rays also show if there are causes for the scoliosis. Sometimes the X - rays help to identify if there is a high risk of the scoliosis worsening.

After all of this, the doctors may want you to go for further tests such as an MRI scan. An MRI scan may need a general anaesthetic to help them stay still in the scanner. The doctors will talk that through with you.

If the plan is to monitor your scoliosis, you will be asked to return in a few months (often 6 months) for another examination and X - ray.

How can you treat neuromuscular scoliosis?

Observation

The commonest initial plan is to be monitored. This will be by 6 - 9 monthly appointments at the hospital by the Spine Surgeon. The appointment will usually involve being examined again and having an X - ray. This is to see if the scoliosis is worsening. If it remains roughly the same each time you are seen then they will continue to monitor you until you are fully grown.

Exercise

There is no need to avoid sport or exercise when you have neuromuscular scoliosis. It is actually better for you to remain active and to have good core muscles. Unfortunately there is no strong evidence that exercise alone stops a scoliosis progressing or improves it. There are many ongoing studies that may help prove the role of exercise one way or another.

Bracing

Braces may be recommended. They are usually rigid braces and need to be worn 23 hours a day. They can be worn under your child’s clothes. It has been found that braces only work if they are used more or less constantly. You can take the brace off to wash, dress and during sporting activities.

Surgery

If the neuromuscular scoliosis is felt to be at high risk of worsening or worsens whilst being monitored, surgery will be advised. Surgery may also be advised if function is affected.

Surgery in a growing child’s spine aims to reduce the risk of the curve worsening at the same time as trying to ensure the spine overall still grows and lengthens with time.

If your child is under 10 year of age, neuromuscular scoliosis will be treated like an early onset scoliosis. Further information can be found here.

Once it is felt that there is no longer much growth left in the spine, the commonest operation in the UK would be a posterior instrumented correction and fusion. This means an operation through a long scar on the back to put rods and screws into the bones of the spine and straighten the spine. Sometimes, the screws and rods are attached from the very top of the spine (where the neck joins the trunk) to the pelvis bone. This operation also involves using some form of bone graft to fuse the spine to stop the curve deteriorating or coming back. This does stiffen as well as straighten the spine.

The aim of the operation is to stop things worsening and to hopefully improve function and appearance. Your Spine Surgeon will talk to you about the risks and benefits of this type of an operation. As people with neuromuscular conditions often have other illnesses as well as their scoliosis, your surgeon will have to make choices with you about the appropriateness of surgery balanced with all the potential risks. This may involve other specialists and tests to make sure surgery is as safe as possible.