Diaphyseal Dysplasia

Engelmann's Disease

 

Progressive diaphyseal dysplasia

- marked thickening of cortices of long bones

 

Present

 

Young child with gait disturbance and limb pain

 

Clinical

 

Bilateral symmetrical diaphyseal sclerosis of long bones

- Affects endosteal & periosteal new bone

- Metaphysis & epiphysis are normal

 

Skull base sclerotic

- can get blindness and deafness