Proximal Focal Femoral Deficiency

PFFD is congenital femoral deficiency & deformity of proximal femur. This problem is also known as congenital femoral deficiency. This congenital anomaly may have instability & mal orientation of hip & knee, deformity of femur, acetabular dysplasia, muscular contracture and limb shortening. Some time they can also have knee ligament deficiency. Paley has classified this congenital deficiency in four categories depending upon severity of pathology, reconstructability of congenitally deficient femur. Type 1 will have intact femur with mobile hip & knee. Type 2 will have mobile pseudoarthrosis with mobile knee. Type 3 will have different grade of diaphysial deficiency. Type 4 have different grade of distal deficiency of femur. Lots of radiological & clinical evaluation is required for planning of treatment. Usually all these children need reconstruction at the age of 2 year then repeated limb lengthening. In mildest problem of femoral shortening shoe raise or limb lengthening is required but most of time they required complex reconstruction of hip joint for good outcome. Hip reconstruction in PFFD is known as super hip surgery and knee joint reconstruction is known as super knee surgery. Type 1 is most reconstructable. In this variety super hip 1 is required in which we need to correct the proximal femur deformity by osteotomy and soft tissue release. Limb lengthening is being performed after final correction of hip problem. type 3 & 4 may need rotationplasty & prosthesis fitting. In rotationplasty ankle joint will be converted in knee joint by surgicaly rotating limb by 180 degree. Type 2 variety will need very complex hip reconstruction. All these procedure is not without complication and should be practice by only expert. Trishla foundation under the supervision of Dr jitendra kumar jain, pediatric orthopaedic surgeon has given new lease of life in many children affected with this problem.