Early Treatment of Scoliosis in Spinal Muscular Atrophy

Early Treatment of Scoliosis in Spinal Muscular Atrophy David Spiegel, M. D. Jim Walker, C. O. Children’s Hospital of Philadelphia Presented at the June 2005 FSMA Conference

Outline • Terminology – Scoliosis, Kyphosis, Lordosis, pelvic obliquity • Alternatives for treatment of scoliosis in younger patients – “positional curve control” • Wheelchair modifications • Bracing – Surgery?

Front Side Cervical (Lordosis) Thoracic (Kyphosis) Lumbar (Lordosis)

Neuromuscular Curves

Pelvic Obliquity

Non- Structural: Does correct on bending x-rays Standing Left Bend

Structural: Does NOT correct on bending x-rays Standing Right Bend

Effects of Scoliosis • Pulmonary – Change in shape of chest cavity • Functional – Loss of sitting balance – Loss of upper extremity use

Scoliosis in Spinal Muscular Atrophy • • • Often diagnosed at 4 -6 years Most progress with time Overall goals of treatment – Straight spine over a level pelvis – Maximize comfort, positioning, and ease of care

Scoliosis in Spinal Muscular Atrophy • Short term goals – Slow progression, delay definitive treatment • Maximize lung growth, trunk height – Monitor curve magnitude and flexibility

Early Treatment • “Positional curve control” – Does not arrest curve progression – May delay curve progression • Surgery? – Spinal fusion – ‘’Growing rods” – VEPTR

“Positional Curve Control” • Wheelchair modifications • Bracing (soft spinal orthosis) • Goals – Improve function • Sitting balance, upper extremity use – Delay definitive treatment • Allow for trunk growth • Allow for lung growth

Lung Growth Most lung growth occurs very early— first 6 -8 years of life

Soft Spinal Orthosis • Challenges and Considerations – Pulmonary function – Curve rigidity • Skin integrity – G-tube • Casting method – Apply corrective forces

SOFT • Total contact –Improve comfort –Distribute pressure • Layering –Flexibility –Soft edges FIRM RIGID

• Crest Rolls –Control Brace Migration –Level the pelvis –Distract pelvis from rib cage

Surgical Curve Control Without Fusion • “Growing rod” • VEPTR • Roles to be determined…. .

Summary • Progressive curves in juveniles with SMA are a challenge • “Positional” curve control may improve function and buy time • Indications and results for “fusionless” surgery remain to be determined