Kacper from Zelechow was born on 18th of May 2005 with congenital skeletal system defect -  PFFD. As a result, one of his legs is shorter.  Nowadays, the difference is 29 cm and it gets deepen. Kacper’s dream is to become a sportsman, play football and run fast. To make the dream comes true, about 200 thousand dollars is necessary for the first complex surgery, which must be held as soon as possible. Unfortunately such amount is beyond the family’s capability, therefore we kindly ask you to JOIN the action and to make a donation or entrust 1% of your tax.






What is PFFD?

Proximal femoral Focal Deficiency - is "the closest femoral focal deficiency." This is a rare non-inherited birth defect that affects the pelvis, especially the hips and the proximal femur. This defect is usually combined with a lack or severe short femur, a lack of patella in the knee joint, absence of the fibula, tibia short and crooked, or the occurrence rate of deformation. A typical feature of this defect is short the thigh is usually positioned in external rotation. Dr. classification. There are four types JDAitkien'a PFFD (A, B, C, D). They have been developed based on a classification system for x-ray results. They range in severity from a mild degree in the case of a short femur, but a normal hip joint for severe cases where there is no hip.

Perhaps because of the generality, classification Aitken'a seems to be the most widely used and recognized.

Sketch of a subclass of Aitken

Figure schematically shows the four radiographic subclasses PFFD.

 A more detailed classification system, Dr. Dror Paley presents, a surgeon specializing in musculoskeletal reconstruction. His classification is descriptive and is based on factors that affect the lengthening and reconstruction of congenital short femur. The system is based on the structure of the hip and knee.

Type 1: intact femur with mobile hips and knees
a) normal proximal femoral ossification
b) delayed ossification of the proximal femur

Type 2: The hip is not fully formed,
a) of the femoral head in acetabulum cell
b) of the femoral head absent or stiffness in the acetabulum

Type 3: deficiency of the femur (thigh bone does not reach the acetabulum)
a) of the knee> 45 degrees
b) motion of the knee <45 degrees

  According to the study concludes that PFFD formed in the first trimester of pregnancy (4-6tydz pregnancy) in the so-called process called morphogenesis. If at this stage in the division and the formation of the various parts of the body will mistake the fetus is a "deformation". In contrast to the "distortions" that can occur at a later stage, and the results from the mechanical response factor. Teratogenicity factors, which contribute to the formation of PFFD can be: anoxia (oxygen deficiency), ischemia (temporary blood supply shortages), chemicals, hypothermia, bacterial toxins, viral infections, hormonal changes, X-ray radiation, the radiation emitted from computers, the old type. By the end is not determined which factor has the greatest impact on the appearance of PFFD, but it is certain that this is not a child genetically transmitted defect.
hitherto conducted studies conclude that PFFD has no connection with the development of other body parts and organs (brain, heart, etc. ..) 

Depending on the treatment distortion can rely on zaprotezowaniu, include amputation of foot or leg. Lighter cases eligible for the so-called "pull" with the Ilizarov formulation, or other apparatus derived from the above extraction method. 

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