Osteogenesis Imperfecta

Incidence: OI occurs in 1:10,000-20,000 live births.

Owing to the fragility of bones, caution is needed in positioning or transporting these pts.

Excessive neck extension may lead to fracture.

Scoliosis may cause difficulty with regional anesthesia.

High risk for difficult intubation.

Temperature control; tendency toward hyperthermia due to a hypermetabolic state.

Cardiac events (pts may have cardiac abnormalities).

Consider advanced monitoring in case of cardiac lesions (valvulopathy).

Coagulopathy may be present owing to reduced collagen-induced platelet aggregation.

Difficult mask ventilation due to bone deformities

Difficult intubation

Temperature regulation

Positioning and monitoring

Inherited disease of connective tissue with tendency to bone fractures

Brittle teeth (dentinogenesis imperfecta), blue sclerae, progressive deafness

Bone fragility leading to major complications