Cleidocranial Dysplasia (Dysostosis)



- Discussion:
    - autosomal dominant proportionate dwarfism that affects bones formed intramembranously (eg. facial bones, cranium, & clavicles);
    - dislocations of hips, knees, and the radial heads may occur;
    - clavicle:
          - most commlonly, absence of the central portion of the clavicle with rudimentary medial and lateral portions remaining;
          - may be confused w/ pseurdoarthrosis of the clavicle
    - coxa vara:
          - consider intertrochanteric osteotomy if varus is < 100 deg;
          - reference:
                 - Management of developmental coxa vara in cleidocranial dysostosis.
    - pubis: shows delayed ossification (hence, it may appear to be widened)



Medical Progress: Epiphyseal Disorders.

The epiphyseal dysplasias

Avascular necrosis of the hip in multiple epiphyseal dysplasia.

Development of the hip in multiple epiphyseal dysplasia. Natural history and susceptibility to premature osteoarthritis.



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Monday, May 21, 2012 3:20 pm