PFFD | Proximal Femoral Focal Deficiency -

Classification

Type	Description
A	Radiographic defect in the upper femur that later ossifies, femoral head & acetabulum form, varus deformity & shaft may be higher than the head
B	Tuft at proximal end of femur, never get connection between head & shaft (pseudoarthrosis), acetabulum & head are present, femur short
C	Absent femoral head, flat-absent acetabulum, short femur
D	As in C but with very short femur represented by femoral condyles

Aitken Classification of PFFD

Short thigh
Hip flexed, abducted & externally rotated
Usually a flexion contracture of the knee
45% fibular hemimelia with short tibia & equinovalgus foot, commonly with absent lateral rays
Children generally meet the normal motor milestone
May be related to congenital short femur, anterolateral femoral bowing, AP laxity of the knee, ↓ SLR secondary to short hamstrings

Length at maturity
- Proportionate shortening will continue
- > 20 cm should be thinking prosthesis or amputation
What is present
- is there a normal hip
- Is there a normal knee
- Is there a normal foot

If end length > 20 cm short
Unstable hip
Non functional foot
- Often young child will get an extension prosthesis
- Later Symes amputation with knee fusion versus a rotationplasty with knee fusion
- Get the limb to end slightly above the contralateral knee to accommodate a prosthetic knee joint
- Most of the hip configurations are stable requiring no intervention, an abductor lurch is inevitable
Foot – amp, rotate or save
Knee – fuse versus nothing
Hip – fuse versus nothing
most common approach is a Symes amputation with knee fusion

Most for congenital short femur
Must have discrepancy < 20 cm, stable hip & good foot
A stable hip configuration must be obtained & prior to lengthening the hip may require corrective femoral or pelvic osteotomy