Surgical correction of spinal deformity

This page is intended to help you understand what scoliosis is and the reasons for spinal surgery being considered and help you appreciate what needs to be taken into account before a decision is made to go ahead with surgery.

Please read the following information carefully and discuss it with relatives, friends and your family doctor, if you wish. If there is anything that is not clear, or if you have any further questions, please do not hesitate to ask.

What is scoliosis?

Scoliosis means an abnormal sideways or twisting curvature of the spine and affects about 3-4 children in every 1,000. There are a variety of reasons for a person to develop scoliosis. Scoliosis develops more often in people with muscular dystrophy and some of the other neuromuscular disorders because the spinal muscles are weak and unable to support the spinal column in the upright position, either standing or sitting.

When does scoliosis develop?

Scoliosis can be already present at birth in rare conditions, for example in some children with specific congenital muscular dystrophy (CMD) variants or Spinal Muscular atrophy (SMA) type I. Scoliosis can develop in the first decade of life, very commonly in children with SMA type II and congenital muscular dystrophy variant such as Ullrich CMD and Rigid Spine syndrome (RSMD1). Some children with SMA III can also develop scoliosis in the first decade of life. Scoliosis develops very frequently in the early teens and adolescence in Duchenne muscular dystrophy (DMD) and in young people with SMA III in whom walking is not possible any more. Scoliosis can also develop in other conditions such as some congenital myopathies and in Charcot-Marie-Tooth disease.

How does scoliosis develop?

Children grow and change in height and weight, which adds greater demands to their already weak muscles. For some children with neuromuscular conditions, as they get older they become less mobile and spend much of the time sitting. Scoliosis develops and tends to gradually increase more rapidly in children who are unable to stand and walk. It is unclear which additional risk factors could contribute to the onset and progression of the scoliosis. It is not known whether operating the electric wheelchair with the dominant hand will influence the side the scoliosis curve develops for example. Initially most of the scoliosis is quite mobile. However, even a flexible scoliosis may over time become fixed reducing the correction which can be achieved.