Early Treatment of Early Treatment of Scoliosis in Spinal Scoliosis in Spinal Muscular AtrophyMuscular Atrophy

David Spiegel, M.D.David Spiegel, M.D.

Jim Walker, C.O.Jim Walker, C.O.

Children’s Hospital of PhiladelphiaChildren’s Hospital of Philadelphia

Presented at the June 2005 FSMA Conference

OutlineOutline• TerminologyTerminology

– Scoliosis, Kyphosis, Lordosis, Scoliosis, Kyphosis, Lordosis, pelvic obliquitypelvic obliquity

• Alternatives for treatment of Alternatives for treatment of scoliosis in younger patientsscoliosis in younger patients– ““positional curve control”positional curve control”

• Wheelchair modificationsWheelchair modifications• BracingBracing

– Surgery?Surgery?

Cervical (Lordosis)

Thoracic (Kyphosis)

Lumbar (Lordosis)

Front Side

Neuromuscular Neuromuscular CurvesCurves

PelvicPelvicObliquityObliquity

NonNon- Structural:- Structural:DoesDoes correct on bending x- correct on bending x-

raysrays

Standing

Left Bend

Structural:Structural:Does Does NOTNOT correct on bending x- correct on bending x-

raysrays

Standing Right Bend

Effects of ScoliosisEffects of Scoliosis

• PulmonaryPulmonary– Change in shape of chest Change in shape of chest

cavitycavity

• FunctionalFunctional– Loss of sitting balanceLoss of sitting balance– Loss of upper extremity useLoss of upper extremity use

Scoliosis in Spinal Muscular Scoliosis in Spinal Muscular AtrophyAtrophy

• Often diagnosed at 4-6 yearsOften diagnosed at 4-6 years• Most progress with timeMost progress with time• Overall goals of treatmentOverall goals of treatment

– Straight spine over a level Straight spine over a level pelvispelvis

– Maximize comfort, positioning, Maximize comfort, positioning, and ease of careand ease of care

Scoliosis in Spinal Muscular Scoliosis in Spinal Muscular AtrophyAtrophy

• Short term goalsShort term goals– Slow progression, delay Slow progression, delay

definitive treatmentdefinitive treatment•Maximize lung growth,trunk Maximize lung growth,trunk heightheight

– Monitor curve magnitude and Monitor curve magnitude and flexibilityflexibility

Early TreatmentEarly Treatment

• ““Positional curve control”Positional curve control”– Does not arrest curve Does not arrest curve

progressionprogression– May delay curve May delay curve

progressionprogression• Surgery?Surgery?

– Spinal fusionSpinal fusion– ‘’‘’Growing rods”Growing rods”– VEPTRVEPTR

““Positional Curve Positional Curve Control”Control”

• Wheelchair modificationsWheelchair modifications• Bracing (soft spinal orthosis)Bracing (soft spinal orthosis)• GoalsGoals

– Improve functionImprove function•Sitting balance, upper extremity Sitting balance, upper extremity useuse

– Delay definitive treatmentDelay definitive treatment•Allow for trunk growthAllow for trunk growth•Allow for lung growthAllow for lung growth

Lung Lung GrowthGrowthMost lung growth Most lung growth occurs very early—occurs very early—first 6-8 years of first 6-8 years of lifelife

Soft Spinal OrthosisSoft Spinal Orthosis

• Challenges Challenges and and ConsiderationConsiderationss– Pulmonary Pulmonary

functionfunction– Curve rigidityCurve rigidity

• Skin integritySkin integrity

– G-tubeG-tube• Casting methodCasting method

– Apply corrective Apply corrective forcesforces

SOFT

FIRM

RIGID

•Total contact–Improve comfort–Distribute pressure

•Layering–Flexibility–Soft edges

•Crest RollsCrest Rolls–Control Brace MigrationControl Brace Migration–Level the pelvisLevel the pelvis–Distract pelvis from rib cageDistract pelvis from rib cage

Surgical Curve Control Without Surgical Curve Control Without FusionFusion

• ““Growing rod”Growing rod”• VEPTRVEPTR• Roles to be Roles to be

determined…..determined…..

SummarySummary

• Progressive curves in juveniles Progressive curves in juveniles with SMA are a challengewith SMA are a challenge

• ““Positional” curve control may Positional” curve control may improve function and buy timeimprove function and buy time

• Indications and results for Indications and results for “fusionless” surgery remain to “fusionless” surgery remain to be determinedbe determined