The Hip: Preservation, Replacement and Revision

Kniest Dysplasia

- See: Metatrophic Dysplasia:

- Discussion:
    - this disorder is frequently confused with metatrophic dysplasia;
    - characterized by disproportionate short trunk dwarfism, kyphoscliosis, and joint stiffness, and contractures of the hands;
    - assessment of atlantoaxial instability is essential;
    - major orthopaedic deformity is limited joint motion and contractures;
    - these patients are predisposed to early osteoarthritis;
    - may be caused by a mutation in type II collagen

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

Last updated by Data Trace Staff on Thursday, September 8, 2011 12:43 pm