osteogenesis imperfecta

what is osteogenesis imperfecta

genetic disorder of connective tissue

characteristics

• brittle bones

• fracture susceptibility

• reduced bone mass

• bowing of long bones

• muscle weakness

• short stature

• blue sclera

• joint laxity/dislocation

• dentinogenesis imperfecta

severity type in order of least severe to most

• type 1

• types 4,5,6

• types 3, 7, 8

• type 2

type A binder classification system of OI

most severe

dependent for ADLs

focus on positioning

type B binder classification system of OI

strength usually 3/5

some ADLs independent 

focus on posture, ROM , and strength

type C binder classification system of OI

lease severe

poor endurance 

independent with ADLs

focus on strengthening 

bisophosphonates

improve bone density

decrease back pain

improve function

what is the big part of overall management

fracture management

keep immobilization to a min

what should be the PT management for positioning in an infant

midline positioning of head and spine

orthotics for children with OI

should be fitted once pulling to stand

HKAFOs are recommended