Managing Scoliosis

Non-sitters

To try to prevent scoliosis and to assist with sitting or being in a more upright position, children who are non-sitters may find it helpful and will generally be more comfortable if they wear a spinal brace during the day. The brace should be custom-made and fitted by a specialist (an orthotist) so that it goes around the back and chest and fits comfortably.

Sitters

Anyone with a scoliosis over 20^o may be provided with a spinal brace or body jacket, also called a TLSO (thoraco-lumbar sacral orthosis) to provide corrective support when in an upright sitting position; wearing this will not stop the further possible development of spinal curvature. Spinal braces are recommended for children who still have lots of growing to do; a soft or semi-rigid spinal orthosis is recommended. A brace helps to relieve pain, provide stability and may slow curve progression.

There may come a point when spinal surgery to straighten the spine is advised. This is to help with balance when sitting as well as allowing the lungs to expand more fully and so improve breathing. If this is being considered, there should be plenty of time to talk with the multidisciplinary medical team about the options and what is involved.

A recommendation to have spinal surgery is based on a number of factors. Most importantly these are when the spinal curve Cobb angle has progressed to 50^o or more and when the curve is increasing by 10^o or more each year.

When discussing the possibility of surgery, other factors that will be assessed and considered include:

Age in years and months (chronological age) and whether their bones and skeleton are fully grown (skeletal maturity). Since skeletal age and actual age often don’t match, skeletal maturity can be estimated by taking an X-ray of the bones in the left wrist
How much breathing ability has decreased
If Chest wall shape has changed or their ribs are collapsing
If the curve is causing problems with their day-to-day activities
If there is persistent back and hip pain
If hips and pelvis are positioned unevenly, making sitting in a balanced way difficult

There are two types of spinal surgery:

Spinal fusion involves straightening the spine by attaching two metal rods, one on each side of the spine, to many bones of the spine (vertebrae) and ribs along the spinal curve. This is to correct the curve and support the spine against gravity. In addition, the vertebrae are fused together to provide additional support.

‘Growth friendly’ spinal surgery is recommended for children less than 10 years old who have significant spine and rib cage growth remaining. The spinal rods are only anchored to the spine or ribs at the top and bottom of the curve, leaving the spine segments and ribs in-between untouched to allow for continued growth. The spinal curve is straightened as much as possible when the rods are placed, then every two to six months the rods supporting the spine are gradually lengthened. This procedure allows for continued spine and chest growth. New technologies are becoming available for growth-friendly spinal surgery, for example magnetically controlled growing rods may be available which allow for lengthening without the need of repeated surgery.

Recommended age of spinal interventions:

The SoC recommendations to medical teams vary depending on age and are as follows:

Under 4 years: in general, spinal surgery should be delayed until after four years of age.
Age 4 – 10 years: As children are skeletally immature, growthfriendly spinal surgery is recommended.
Age 10 – 12 years: At this age children are transitioning to skeletal maturity. If surgery is needed, the type will depend on the child’s skeletal maturity and how much more their spine is likely to grow.
Age over 12 years: This is when children are skeletally mature. If surgery is needed, spinal fusion surgery is recommended.

Before any operation, the multidisciplinary medical team should carry out a full evaluation of health and well-being. This includes any weight or dietary challenges, bone health and breathing ability, and any need for support with breathing during surgery. So that decision making is shared, the team should have an indepth discussion about the risks and benefits of the procedure. The medical team that will be involved should have a plan for how breathing will be managed after the operation.

Experts advise that any future spinal surgery should leave an unfused area in the middle of the lower back (midlumbar) to allow for the administration of therapies, such as Spinraza®, that are injected via a needle directly into the spinal canal (intrathecally) (see Section, Administration of New Treatments for SMA).

Impact on the hips

It is common for non-sitters and sitters to have unstable hips which may affect one hip or both. If someone can stand, manage assisted transfers and/or walk, and their hip instability is interfering with these activities or causing pain, surgical hip reconstruction to stabilise a dislocated or partly dislocated hip is recommended. Surgical stabilisation is also recommended for non-walkers if they have persistent pain or their limited hip mobility makes managing any activities such as eating, going to the toilet, dressing or sitting, difficult.

Impact on the joints

Tightening of joints (contractures) is a common problem which can lead to pain and difficulty moving. Physiotherapy and splints are recommended to help manage this (see Section, Physiotherapy and Rehabilitation). Surgical intervention should only be considered when contractures cause pain or limit the ability to move and use the joint.

Impact on the bones

Non-sitters and sitters are at higher risk of bone fracture due to osteoporosis from not standing (bearing weight) and not using the bones and muscles. It is important to make sure that children and adults with SMA have enough dietary calcium and vitamin D3 (see Section, Nutrition, Growth and Bone Health).

If a bone is fractured, the SoC advise medical teams:

For sitters and non-sitters	For walkers
Avoid using a plaster cast which restricts movement for longer than four weeks	Consider surgery if someone is usually able to walk and has fractured their leg bone
Avoid surgery whenever possible and perhaps instead use a cast	Consider surgery using rods or plates if someone can’t usually walk and has fractured their leg. This can speed up healing of the fracture and gives the greatest chance of maintaining movement.

Figure 2. Clinical management route a clinician will take when they suspect a person has curvature of the spine (figure adapted from E. Mercuri et al. 2018, p.109)