Congenital Femoral Deficiency: Paley Type 2a

Case 56: Congenital Femoral Deficiency: Paley Type 2aCongenital femoral deficiency (CFD) is a spectrum of severity of femoral deficiency and deformity. Deficiency implies a lack of integrity, stability, and mobility of the hip and knee joints. Deformity refers to bone malorientation, bone malrotation, and soft tissue contractures of the hip and knee. Both deficiencies and deformities are present at birth, nonprogressive, and of variable degree (Paley D, Standard SC Lengthening reconstruction surgery for congenital femoral deficiency. In: Rozbruch SR, Ilizarov S (eds) Limb lengthening and reconstruction surgery, ch 29. Informa Healthcare, New York, pp 393–428, 2007). The Paley classification system is based on the factors that influence lengthening reconstruction of the congenitally short femur. Type 2 has a mobile proximal femur pseudarthrosis with a mobile knee. Type 2a has a femoral head mobile in the acetabulum, 2b has a femoral head fused to the acetabulum, and type 2c has absent femoral head and acetabulum. The SuperHip 2 (SH2) procedure for types 2a and some 2b combines bone and soft tissue reconstruction to reliably achieve union between the upper femur and the femoral head with the creation of a new femoral neck. The treatment for types 2c (absent femoral head) and 2b fused femoral heads that are not candidates for SH2 is a pelvic support osteotomy.