1. 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

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

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

7. Neuromuscular Curves

9. Pelvic
Obliquity

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

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

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

15. 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

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

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

18. “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

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

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

21. SOFT FIRM RIGID Total contact Layering Improve comfort
Distribute pressure
Layering
Flexibility
Soft edges
FIRM
RIGID

22. Crest Rolls Control Brace Migration Level the pelvis
Distract pelvis from rib cage

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

25. 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

26. Thank You !