Incidence
Spinal deformities are found in 20% to 80% of patients with OI
Risk factors
Severe disease with nonambulatory status
Progression
Scoliosis in OI may progress after skeletal maturity
- may be related to weakened osteoporotic bone
Management
Non operative
Bracing
Poor results
- risk of rib fractures
Operative
Posterior segmental instrumentation / arthrodesis
A progressive curve that has reached 35 - 40o should be fused
- regardless of the patient's age
- significant correction cannot be expected
Augmentation with segmental sublaminar wiring
- to improve purchase in the osteoporotic bone