- Intermediate severity (type II) SMA and Congenital Myopathies: Most children (90%) with type II SMA develop progressive scoliosis, often associated with a pelvis which is not level (pelvic tilt). These children are never able to walk independently and scoliosis shows a continuous progression, often also after spinal growth is completed.
Children with congenital myopathies might be able to walk independently without obvious progression of their muscle weakness. However they often show progression of scoliosis and this can be accompanied by significant respiratory muscle weakness. A decision to perform spinal surgery might be taken if the progression of the scoliosis is significant.
For very young children when years of rapid spinal growth lie ahead bracing is often considered unless scoliosis is severe. In later childhood or the teenage years, surgery is usually advised if scoliosis is increasing and the child's health (and especially respiratory function) is sufficiently good.
- Duchenne muscular dystrophy and other forms of muscular dystrophy: Between 68-90% of boys with Duchenne muscular dystrophy (DMD) will develop scoliosis after having lost the ability to walk, starting between 11-16 years, typically 1-2 years after starting to use their wheelchairs continuously. Rarely boys with DMD might develop scoliosis whilst still walking.
Although scoliosis is a very common problem in DMD, it is also not an inevitable complication. The reasons why a boy with DMD will not develop severe scoliosis are still not completely understood. When the child is no longer on his feet, the scoliosis may start to progress. At this stage discussions may begin between you and the surgeons about spinal surgery to correct the curve. Regular monitoring of scoliosis is very helpful in planning the right time to consider spinal surgery. In DMD consideration of timing that reflects, cardio- respiratory sufficiency and nutrition is very important in planning spinal surgery.
When is the right time for surgery?
The right time to undertake surgery needs to be decided in consultation between the young person, the surgeon, the family and the rest of the multidisciplinary team (see section entitled ’The decision’). There are a variety of factors that should be considered and discussed. Ideally children/young people who are considered suitable for surgery will have a preoperative assessment. This approach gives the child/young person and the family a chance to meet people who will be involved in their care when they do come into hospital for surgery.
It can be a helpful experience for children/young people and families to ask any questions they may have. This assessment may also contribute to the final decision regarding surgery. It may be helpful for families and children/young people to consult with another family whose child/young person has had scoliosis surgery.