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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