الفهرس 
EMBRYOLOGYOnly 14 pages are availabe for public view
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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.