- Discussion:
 - rare skeletal dysplasia characterized by change in body proportions w/growth;
 - because of the short limbs and relatively long trunk during infancy, this condition often has been confused with achrondroplasia;
 - unlike pts with achrondroplasia, the head and face appear normal;
 - severe kyphoscoliosis may develop, w/ a marked shortening of trunk and an apparent reversal of body proportions;
 - curves are severe and rigid;
- Diff Dx:
  - Morquio's
  - Achondroplasia