Osteogenesis Imperfecta

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

 

OI Scoliosis