Scoliosis

NOTES FOR PARENTS OF MUSCLE AND NERVE DISORDER PATIENTS

Introduction

Scoliosis is the medical term for curvature of the spine. In diseases of the muscles and nerves, curvature of the spine occurs because the muscles on either side of the spine are weak. They are not working effectively to hold the spine straight. Once significant scoliosis has developed, it will worsen unless surgery is performed. Braces and special seating are not usually effective in preventing progression of the problem.

Complications of Scoliosis

1. Pain and Discomfort-

When the spine curves, ligaments and other tissues are stretched. This causes backache, discomfort and pain and difficulty sitting for long periods of time. Eventually the patient is unable to sit without discomfort and may become bedridden.

2. Impairment of Breathing -

If the spine curves too much, the rib cage is deformed and this in turn deforms the lungs. Breathing becomes less efficient.

What Can Be Done About Scoliosis?

A number of operations have been devised to overcome the effects of progressive scoliosis. During the operation, the spine is straightened out as much as possible and metal rods are placed on each side of the spine to keep the spine straight. These metal rods prevent the spine from curving again.

What Are The Risks Of The Operation?

Operations for scoliosis are major procedures. They do carry some risk as follows.

1. Haemorrhage : This can be dealt with by the surgeon before the operation is finished.

2. Wound breakdown or infection : These are very uncommon and can be treated with antibiotics.

3. Damage to the spinal cord : Although the nervous tissue of the spinal cord is not touched at the time of the operation, straightening out the spine can sometimes lead to pressure on the nervous tissue or cause the blood supply to the nervous tissue to be impaired. Paralysis of the legs, impairment of sensation in the legs and impairment of bladder function are possible resulting problems but these are rare.

4. Anaesthetic problems : Although the operation takes several hours, the risks of the anaesthetic itself are no greater for this operation than for other equivalent operations.

5. Post-operative respiratory failure : Most patients with muscle and nerve disease having this operation have significantly poor breathing capacity before the operation. This is because of weakness of the muscles of the chest wall. Often the child's ability to cough is also very poor. These factors, along with poor chest movement due to discomfort in the days immediately following the operation, can make children susceptible to chest infection which even further affects breathing capacity.

Occasionally it is necessary for the patient to be in the intensive care unit for a few days following the operation. Sometimes the patients breathing requires assistance by a mechanical ventilator. Very rarely, if post operative chest problems are severe and prolonged, the patient's chest muscles becomes weaker than they were before the operation and it becomes difficult for the patient to breath by him or herself again. It should be stressed that this is a rare but nevertheless serious problem.

Implications Of Not Having The Spinal Surgery

In patients with muscle and nerve disease, once the spine has curved significantly, the curvature or collapse will continue. Ultimately this leads to such discomfort and pain that the child is unable to sit in his or her wheelchair and will become bedridden. Lifestyle becomes dramatically more restricted than previously. Breathing capacity will be impaired and the risk of chest infection increases. Once this situation has developed it is very difficult to keep the patient comfortable.

Risks Versus Benefits

The operation for scoliosis is usually suggested before the patient is experiencing major difficulties. The operation is usually suggested to prevent the disastrous consequences of the spine collapsing before it has gone too far. The consequences of not doing the operation are very serious. Given this, most parents decide to have the procedure performed despite the fact that it does carry some risks. In most situations the potential benefits far outweigh the risks.

After surgery, almost all families have been pleased that the decision was made to proceed, despite that decision having been a difficult one. The children themselves comment that they feel better about themselves and about how they look when they are sitting straighter in their chair. Some mobility of the trunk is lost because of the rods holding the spine straight but this is usually not a major problem.

What Can You Do?

Surgery for scoliosis should be discussed thoroughly with the medical team and with the family. The child and family will be given the opportunity to raise any questions or concerns about the procedure.
Dr. LK SHIELD
Department of Neurology
ROYAL CHILDREN'S HOSPITAL
Melbourne Australia


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