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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.