- 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