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Proximal Femoral Focal Deficiency (PFFD)

Congenital Femoral Deficiency can lead to a congenitally short femur with different degrees of a lack of formation of the hip and knee joints. In the mildest cases, the hip and knee are properly formed and only the femur is short. In more severe cases, the hip is severely deformed, the knee is unstable, or has a flexion contracture and the femur is short. In the most severe cases, there is either no connection between the femoral head and the femoral shaft, or there is no hip joint at all and the knee is not only contracted, but also stiff (limited in its motion). The treatment for cases with a well-formed femur is lengthening. For the more severe deformity, the superhip and superknee procedures are used to reconstruct the hip and knee before lengthening. If there is a true pseudarthrosis (false joint), the superhip 2 procedure is used to build a femoral neck and connect the femoral shaft with the femoral head. Finally, in the most severe cases, Syme’s amputation and rotationplasty should be considered.

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