Neuromuscular scoliosis

Neuromuscular scoliosis is a curvature of the spine caused by a neurological or muscular condition. Neurological conditions affect the body’s nervous system. Examples are cerebral palsy and spina bifida. Not all people with a neurological condition will develop scoliosis.

Neurological conditions happen when there is damage to the brain or nerves caused by illness or injury. They can affect the muscle-nerve pathways of the body from the brain down to the spinal cord.

Muscular conditions such as Duchenne muscular dystrophy or spinal muscular atrophy stop the muscles from working. When the muscles do not work, scoliosis can develop.

Patients with these conditions often develop scoliosis or kyphosis, or both. As they grow and their trunk muscles get weaker, the spine gradually begins to collapse, which creates a long, C-shaped collapsing scoliosis.

These curves are often progressive. Progressive means they continue to get bigger. The rate of progression increases during rapid growth, so these curves worsen during growth spurts, such as puberty. 

For children who use a wheelchair, progressive curves can make it difficult for them to sit comfortably. Large curves (measuring 80o or above) in the upper or middle parts of the spine may cause lung problems.

Treatment

Patients with neuromuscular scoliosis should be seen by a scoliosis specialist. Children with neuromuscular conditions will be monitored by specialist doctors from a young age. This monitoring is essential to make sure that curvatures do not get too big. X-rays of the spine will be taken to measure the size of the curve.

MRI scans of the brain and the spine may be needed to rule out any other problems. The treatment of neuromuscular scoliosis will be tailored to the needs of each individual patient. They will need to have a number of specialists involved in their care.

Scoliosis can make walking more difficult. For wheelchair users it can affect stability and comfort when seated.

Bracing might be used to provide support for the patient’s trunk (central part of the body) when they are sitting but it will not usually stop the curve from progressing (getting bigger). Adjustments and aids for seating such as inserts into wheelchairs may help with the positioning and comfort of a child, but these will not correct the scoliosis.

A key issue for families and their doctors is whether surgery is the best choice. Surgery would aim to help the child to keep the ability to sit. The question would be whether that surgery would maintain or improve the child’s quality of life and function (In medical language, function is a word to describe the normal and proper action of any body-part or organ.)

For some children with cognitive or sensory impairment, these decisions are difficult. Parents often struggle with the fact that they can’t explain to the child why they are having surgery. It can be very hard or even impossible to help them understand the pain that goes along with this operation. These can be difficult issues to deal with when taking into account the risks and the recovery process of this type of surgery.

These decisions should be made with great care. Talking with other families who have been through this decision process is very helpful. SAUK can put families in touch with other members who have first- hand experience of neuromuscular scoliosis. In the end, it will often be down to the parents to make this difficult decision for their child.

Surgery

Surgery is usually offered only to patients who have a progressive (growing) curve that interferes with function, affects breathing ability, causes pain, or is likely to cause difficulties in the years ahead.

Surgery may also be offered to patients with curves larger than 50 degrees. The patient may have a tilted pelvis (pelvic asymmetry) as well as a large spinal curve. In these cases the spine and the pelvis will be operated on to correct the asymmetry. (The pelvis is the large frame at the bottom of the spine. The legs are attached to it.)

The aim of surgery is to rebalance the spine and preserve function, comfort, and quality of life. The spinal curve(s) are partly corrected but the main aim is to stop the curve from getting worse. Although many patients are severely disabled, they can usually live fulfilling and productive lives.

Prognosis (the outcome)

Scoliosis is common in neuromuscular conditions and often occurs when a child is young. Because it is important to spot scoliosis early, children with neuromuscular conditions need to be checked every year for any problems with the spine. Treatment is usually an operation, and it is usually necessary to fuse (join together) a large section of the spine. In many cases spinal fusion surgery has improved the quality of life for children with neuromuscular conditions

Definitions

Cognitive- Mental activities such as thinking, understanding, learning, and remembering

Function-The special, normal, or proper action of any body part or organ

Frequently asked questions

Here is a selection of common questions we are asked by callers on our Helpline. If there is other information needed or for further advice please do not hesitate to call us on: 020 8964 1166 or e-mail: info@sauk.org.uk

Scoliosis is a sideways curvature of the spine. Neuromuscular scoliosis is curvature of the spine in patients with any neurological condition such as cerebral palsy, spina bifida, muscular dystrophies, and spinal cord injuries.

Scoliosis can make walking more difficult. For wheelchair users it can affect stability and comfort when seated. Patients with neuromuscular scoliosis should be seen by a scoliosis specialist.

Children with neuromuscular conditions will be monitored by doctors from a young age looking at the child as a whole including the spine. This overview is essential to look for scoliosis and make sure that curvatures do not grow too big. X-rays will be used to measure the size of the curve and an MRI scan may also be needed.

The treatment of neuromuscular scoliosis will be tailored to the needs of each individual patient. They will need to have a number of specialists involved in their care.

Bracing might be used to provide support for the patient’s trunk (central part of the body) when they are sitting but it will not usually stop the curve from progressing (getting bigger). Adjustments and aids for seating such as inserts into wheelchairs may help with the positioning and comfort of a child, but these will not correct the scoliosis.

Surgical treatment is only recommended for patients who have progressive (curves that are getting bigger) a curve that interferes with function or is likely to cause difficulties in the years ahead. In medical language, ‘function’ is a word to describe the special, normal, or proper action of any body-part or organ. An operation is otherwise usually done for curves larger than 50 degrees.

The aim of surgery is to rebalance the spine and preserve function, comfort, and quality of life. The spinal curve(s) are partly corrected but the main aim is to stop the curve from getting worse.

For some children with cognitive or sensory impairment, these decisions are difficult. Parents often struggle with the fact that they can’t explain to the child why they are having surgery. It can be very hard or even impossible to help them understand the pain that goes along with this operation. These can be difficult issues to deal with when taking into account the risks and the recovery process of this type of surgery.

Scoliosis is common in neuromuscular conditions and often occurs when a child is young. Because it is important to spot scoliosis early, children with neuromuscular conditions need to be checked every year for any problems with the spine, often by their paediatrician or other doctors involved in their care. Treatment can include putting the child in a brace to try to stop the curve getting bigger as quickly, or an operation. For this operation it is usually necessary to fuse (join together) a large section of the spine. In many cases spinal fusion surgery has improved the quality of life for children with neuromuscular conditions.

It is quite normal to feel confused about what course of treatment to choose. There is a lot of information to take in and some of the questions about scoliosis have no clear answer. It is absolutely fine to ask your specialist further questions over several visits to outpatients or for a second opinion. It is often useful to make a list of questions and take them to an appointment so that all of your concerns can be answered.

If you need names of additional centres or specialists then you can get this information from us by calling or emailing the SAUK Helpline. You may also find that it helps to talk through the specialist’s advice with someone. The friendly SAUK team are always happy to offer advice, support, and a listening ear. You can ask us as many questions as you like and we will try to help. It sometimes helps to speak to people who have been in a similar position. If you would like to speak to people affected by scoliosis, the SAUK membership scheme can help you to do so.

It is your right to ask for a second opinion from another specialist at the same or a different hospital.

Also every hospital has a patient advice and liaison service (PALS). If you are unhappy with the way that you have been treated or have any other worry or concern about your care, you can seek advice from the PALS service linked to your hospital, which will be happy to help.

For patients in Scotland you can contact the Patient Advice and Support Service http://www.patientadvicescotland.org.uk/

Patients in Northern Ireland can contact http://www.patientclientcouncil.hscni.net/

If you would like to talk further about any aspect of scoliosis, SAUK is here to help; please call our helpline or contact us via post or using our e-mail address info@sauk.org.uk.