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)

The muscles supporting the spine either are too weak or tight (called spasticity) which exert distorting forces on the spine resulting in a curved spine (scoliosis).

X-rays are used to measure the angle of the curve (called the Cobb angle).

Larger the angle, more severe the scoliosis. Larger curves tend to develop more problems.

• Smaller 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.
• Curve larger than 40-50º can continue to increase in size, sometimes as fast as 10º per year
• As curves get larger, it is likely your child will develops symptoms and problems.

For your child

• As curves progressively get larger, the pelvis starts tilting making it difficult to sit independently. Sometimes sitting problems are also cause because of hip issues (hip dislocation and contractures). The child then starts using arms to prop up the trunk and thus progressively social interaction with hands reduces as arms are no longer available to interact with the environment.
• A pelvic obliquity will cause your child to sit on one buttock putting extra pressure on that side. The skin over these areas might break down (sore) because of these abnormal pressures.
• Increase in pain in the back or the ribs. The ribs can touch the pelvic bone causing pain (costopelvic impingement)
• Breathing problems can develop in large 80º+ curves as the lungs get compromised because of the curve. Sometimes muscle weakness contributes to breathing problems.
• Nutrition and feeding suffers and your child can get malnourished with time.

For the care-giver or parent

• Feeding problems increase.
• Dressing difficulty
• Constant repositioning is required
• Wheelchair transfers are more difficult

• 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 of the wheelchair are modified and sometimes moulded as per your child's curvature

• Obviously avoids the risk of surgery
• Bracing and wheelchair modification can improve trunk support and sitting posture

• 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 with increase in curve size
• Bracing or wheelchair modification will not straighten the curve
• 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.

• 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, like Duchenne’s this threshold is lower and surgery is offered for smaller curves)
• There is an optimal window in the child life when the surgery can be done with relative safety. Waiting and watching for problems to develop is not a good idea.
• 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.
• Surgery can correct the deformity and level the pelvis so that sitting balance improves and stays that way for the rest of the child’s life.

• Improvement in posture and sitting balance.
• Pain relief
• Improvement in health and nutrition
• Improvement in lung function
• Curve will stop from worsening
• With a level pelvis, body weight gets evenly distributed. Skin breakdown is avoided.
• Improvement in appearance and self-esteem

• Surgery in neuromuscular scoliosis carries some risk
• Overall complications occur in about 1 in 4 patients. 3 out of 4 patients do not have complications.
• Risk of complications depends on the severity of the curve and the severity of the underlying neuromuscular problem.
• Wound infection can occur in about 4 of 100 children. 96 of 100 children do not get wound infection. Some infections require repeated surgery to wash out the infection.
• Lung problems (like pneumonia, prolonged breathing tube, ventilator support) may occur after surgery.
• Urinary tract infection in 5 of 100 children; 95 of 100 do not get this complication
• Screw loosening or rod breakage may occur in about 16% children. 84 out of 100 patients do not have this problem. Many times repeat surgery to fix the problem may not be necessary.
• Back pain sometimes may not resolve or can develop as a new problem.
• Risk of spinal cord injury and worsening of spasticity with new bladder and bowel problems are rare complications. Sometimes intraoperative neuromonitoring is used to minimize these problems.
• Risk of death within 30 days is less than 2 per 100 children. The risk of death is higher in sicker children with larger curves.
• Most complications can be successfully treated. Even if complications occur, 90 of 100 parents are satisfied with the results of the surgery because of the benefits.