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Chondroectodermal Dysplasia



- Discussion:
    - rare disorder characterized by short limb dwarfism, polydactyly, dysplasia of the fingernails and toenails, dental deficiencies, & congenital 
         heart disease;
    - common in the Amish;
    - short limb disproportionate dwarves are characterized by acromesomelia, w/ the forearms, hands, legs, and feet involved to a greater 
          extent than the proximal limb segments;
    - postaxial polydactyly of hands is universal, occasionally involving feet as well;
    - excision of the postaxial digits and release of the syndactylys are indicated for most patients, if there is a stable cardiac status;
    - characteristically, a valgus alignment of the knees is present and frequently progresses produces a marked deformity;
         - these usually require surgical correction