Having any major operation is a disadvantage and there is always some danger which must be balanced against the benefits. In addition, rigidly stabilising the spine in this manner, from just below the neck to the pelvis, does restrict the mobility of the trunk which in a healthy person would be a distinct disadvantage. But in a child with a neuromuscular disorder this movement is already impaired; hence rigid spinal stability is not such a great problem. Nevertheless some children find that eating is more difficult after the operation because they cannot lean forward and their hands can no longer easily reach their mouth. This problem can often be overcome by the use of simple aids.
The spinal operation greatly restricts further growth of the spine. This is why it is usually postponed in very young children unless the curve is severe.
The decision
The decision as to whether to undertake this operation can only be made by the parents of young children or by the individual in consultation with the family and the paediatrician and orthopaedic surgeon. As the operation itself calls for a high degree of skill it is necessary to have an experienced medical team. This will include a paediatrician and anaesthetist experienced in neuromuscular disorders and an orthopaedic surgeon experienced in spinal surgery, along with appropriate paramedical and nursing staff.
Conclusion
Improved surgical techniques along with careful spinal cord monitoring and combined with skilled anaesthetic cover, now make it possible to advise children with Dmd to have surgical correction of their spinal deformity.
It is clear from published results that surgery should be undertaken early, that is when the child starts to develop a significant curve, quite soon after he has become unable to walk or stand. On the whole, the results of surgery are good, as they control the spinal collapse and remove the need for extensive bracing, jackets or supports.
The results published also show that there is no loss of respiratory function following this operation and indeed there may be an improvement at least for a period of time.
Similar advice applies in SMA and in the congenital myopathies. The situation in these and in other neuromuscular disorders is much more variable and the many different factors involved make it especially important to assess the particular situation of each child.
This information sheet is intended to present a balanced picture from which families can see the arguments for and against surgical correction and can then examine their own personal situation.
It will often be found very helpful for families contemplating spinal surgery to contact other families who have been through the experience and with whom they can discuss it.