Congenital- Osteo Imperfecta

Question 1

Def.

Answer 1

• Group of inborn connective tissue disorders (due to abnormal collagen synthesis) -> characterized radiographically by decreased bone density
• 8 different types

Question 2

Dx

Answer 2

2 of 4 major criteria are present:

1: Osteoporosis with abnormal skeletal fragility.
2: blue sclera (Whites of the eyes may have a blue, purple, or grey tint)
3: abnormal dentition
4: premature osteosclerosis

Question 3

Radiol Fx

Answer 3

• Decr bone density
• multiple # w/ exuberant calluses
• callus present as pseudo tumour (i.e. osteosarcoma)
• bowing deformity / pseudoarthroses
• diffuse osteopenia