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Abstract Proximal femoral focal deficiency (PFFD), is a rare disease, but remains a very important problem in pediatric orthopedics and presents a challenge to orthopedic surgeons. PFFD is a spectrum of severity of femoral deficiencies and deformities. Deficiency refers to lack of integrity, stability and mobility of the hip and knee joints. Deformity refers to mal-orientation and mal-rotation and contractures of the hip and knee. Both deficiencies and deformities are present at birth, non-progressive and of variable degrees. The specific cause of this deficiency has not yet been elucidated. Many trials for classification of the disease have been attempted, starting with Aitken in 1969 up to Paley in 1998. Aitkin’s classification (the most widely used classification) is a four type classification, depends on the radiographic appearance of the femoral head, neck, acetabulum and femoral length. In 1974 the three type classification of Fixsen and Lloyd-Roberts appeared, this was a radiographic dependant classification of the proximal portion of the femur. Hamanishi in 1980 classified PFFD into six primary groups and ten subgroups varying from short femur with no radiographic deficiency up to complete absence of the femur.Several techniques of radiology are used to assess PFFD; the first is the teleoradigraph, which is a single large film showing both legs in the standing position. The orthoradiograph uses several films showing the hip, knee and ankle with the advantage of avoiding magnification. The scanogram also avoids magnification and reduces the size of the film. Digital radiology is the most recent technique used; it helps in the measurement of leg length and angular deformities, and it decreases the hazards of radiology by the use of the microdose technique. Assessment of leg length discrepancy is an important aspect in the management of PFFD, and it is essential to be estimated at the time of skeletal maturity, therefore, there are four methods that achieve this goal. The arithmetic method is used to determine the time of epiphysiodesis and is useful only for children whose skeletal and chronological ages are less than a year apart. The growth remaining method is used to predict the future growth of the long leg and is used to estimate the effect of epiphysiodesis. The straight line graph method is a graphical presentation of the growth of the leg to predict the effect of surgery. As for the multiplier method, it allows a quick calculation of the predicted limb length discrepancy at skeletal maturity without the need to plot graphs and is based on as few as one or two measurements.Treatment of PFFD has several aspects to be dealt with, those are: the definitive treatment of PFFD, associated anomalies with PFFD and leg length discrepancy. The associated anomalies with PFFD should be treated before attempting to treat PFFD. These anomalies are: coxavara, acetabular dysplasia, patellar or tibial dislocation, external rotation deformity of the hip, distal femoral valgus deformity, soft tissue contractures and knee instability. The treatment of leg length discrepancy has some general guide lines to be used, according to the amount of discrepancy at the age of maturity. Treatment varies from no intervention (for 0-2 cm of discrepancy), the use of a shoe lift, epiphysiodesis, leg shortening (for 2-6 cm of discrepancy), limb lengthening (for 6- 20 cm of discrepancy) and amputation and prosthetic fitting (for more than 20 cm of discrepancy. So, with the recent advances in limb lengthening, particularly by using the Ilizarov fixator, lengthening and reconstruction in PFFD has become more successful and minimizes the need for the depressive and humiliating technique of limb amputation, which is restricted to a specific group of severe forms of the disease. |