RESUMEN
Proximal focal femoral deficiency (PFFD) is a developmental disorder of the proximal segment of the femur and of acetabulum resulting in shortening of the affected limb and impairment of the function. It is a spectrum of congenital osseous anomalies characterized by a deficiency in the structure of the proximal femur. The diagnosis is often made by radiological evaluation which includes identification and description of PFFD and evaluation of associated limb anomalies by plain radiographs. Contrast arthrography or Magnetic Resonance Imaging is indicated when radiological features are questionable and to disclose the presence and location of the femoral head and any cartilagenous anlage. The disorder is more commonly unilateral and is apparent at birth. However, bilateral involvement is rarely seen. Therapy of the disorder is directed towards satisfactory ambulation and specific treatment depending on the severity of dysplasia.
RESUMEN
Rotation-plasty provides useful knee function and permits use of what is essentially an end-weight-bearing below-the knee prosthesis. This principle of rotating the foot was first used by Borggreve in 1930(3), in a patient with a short femur secondary to infection. Since then, this procedure has been used in congenital short femurs(4,6,7,), malignant bone tumors of the distal femur(1,5)and compound fracture with wide bone defect around the knee(2) We have performed this procedure in a case of focal femoral deficiency with 14cm shorter than the opposite femur due to long standing persistant chronic osteomyelitis in the mid-shaft femur of a nine-year old Korean boy and gave the good result with a conventional but slightly modified below- the-knee brace. The inverted foot was good to control the brace as a below-knee stump and heel functioned as a patella to support the body weight.