Neuromuscular Scoliosis

What is neuromuscular scoliosis?

Abnormal curvature of the spine is called scoliosis. The twisted spine causes prominence of ribs and creates an asymmetry of the trunk. In neuromuscular scoliosis, the curve is caused by abnormalities that cause muscle weakness or spasticity. These can be problems with the brain (cerebral palsy), spinal cord (spinal cord injury), nerves (spinal muscular atrophy), or muscles (muscular dystrophy)

What causes neuromuscular scoliosis?
  • The muscles supporting the spine either are too weak or tight (called spasticity) which exert abnormal, distorting forces on the spine resulting in a curved spine (scoliosis).
How do we know how severe the scoliosis Is?
  • X-rays are used to measure the angle of the curve (called the Cobb angle).
  • Problems are more likely to arise in large curves.
Why is neuromuscular scoliosis a problem?
  • Small curves (<40º) usually do not cause problems. The doctor will ask you to follow-up regularly to assess if the size of the curve is increasing.
  • Once curves reach 40-50º, they can continue to increase in size sometimes as fast as 10 degrees per year.
  • The larger the curve more the problems.
What are the problems associated with a large curve?
  • The pelvis starts tilting and it becomes difficult to sit independently. Sometimes sitting problems are also cause because of hip issues. The child uses arms to prop up the trunk and thus social interaction with hands reduces.
  • A pelvic obliquity will cause your child to sit on one buttock putting extra pressure on that side. The skin over these areas might ulcerate because of these abnormal pressures.
  • Increase in pain
  • Wheelchair transfers become difficult
  • The ribs can touch the pelvic bone causing pain (costopelvic impingement)
  • Breathing problems in large 80º+ curves. Sometimes muscle weakness contributes to breathing problems.
  • It gets difficult for the care-giver or parent to take care of the child. Feeding and positioning problems increase.

What are the treatment options?
Non surgical treatment
  • Observation - means to keep a watch on the curve and continue physical therapy
  • Bracing - means wearing an external jacket to support the curve
  • Wheelchair modification - means the seat and the trunk supports are modified and sometimes moulded as per your child's curvature
Spinal instrumentation and fusion operation
  • This involves putting in screws and rods (spinal instrumentation) in the spine to correct the curvature and make the individual bones stick to each other (fusion).

What are the risk of non-surgical treatment options?
  • The curve might progress in spite of all efforts
  • Breathing problems in a brace
  • Skin problems in a brace
  • Pain cause by increase in curve size
  • increased difficulty in providing care to your child
  • Bracing does not change the fate of the curve (natural history). This means that external bracing has not been shown to stop a progressive curve from increasing.
When is surgery needed for neuromuscular scoliosis?
  • If curves increase to more than 50º or if they are causing problems with sitting and balancing, then surgery is an option. (In some conditions such as Duchenne muscular dystrophy, surgery is offered when the curves are less severe, as later surgery gets difficult).
  • If neglected, these curves get bigger and stiffer as the child gets older. Surgery when the curve gets very large and stiff is risker compared to surgery done early (in mid-teens), when the curve is more flexible as easier to correct.
  • Spinal instrumentation and fusion is the only treatment that can make the curve smaller and keep it from getting big.
  • Usually, surgery can correct the curve by 50-75%. Surgery cannot make the spine completely straight.