dwarfism

Definition Short Stature

< 1.25 m in adult

Types

1.  Proportionate dysplasia

- mucopolysaccharidoses

- Morquio's, Hurler's, Hunter's

2.  Dysproportionate dysplasia

- short trunk / SED

- short limb / achondroplasia & MED

Proportionate dysplasia / Symmetrical decrease in limb and trunk length 

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