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 Functional 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 Casting method Pulmonary function Curve rigidity Skin integrity G-tube Casting method Apply corrective forces

SOFT FIRM RIGID Total contact Layering 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

Thank You !