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A development defect of the proximal femur recognizable at birth in which the femur is shorter than normal and there is apparent discontinuity between the femoral neck and shaft.PFFD consists of a partial skeletal defect in the proximal femur with a variably unstable hip joint, shortening.. Defin
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1. Proximal Femoral FocalDeficiency By
Mustafa Abbas . TMO Orthopaedic and Trauma Unit KTH
2.
A development defect of the proximal femur recognizable at birth in which the femur is shorter than normal and there is apparent discontinuity between the femoral neck and shaft.
PFFD consists of a partial skeletal defect in the proximal femur with a variably unstable hip joint, shortening. Defination
3. Unknown cause – majority of cases
Definite cause
Anoxia
Irradiation
Ischaemia
Bacterial and viral infection
Toxins and hormones
Thalidomide
Etiology
4.
Sclerotome subtraction theory-- injury to neural crest cells
Boden’s theory -- a defect in proliferation and maturation of chondrocytes in the proximal growth plate
Theories
5. 3rd most common longitudinal deficiency of lower extremity
bilateral involvement is seen in 15%
one per 50,000 live births
Maternal diabetes has been implicated in femoral hypoplasia Incidence
6.
fibular hemimelia 70-80%
agenesis of cruciate ligaments of the knee
clubfoot
congenital heart anomalies
spinal dysplasia
facial dysplasias Associated Anamolies
7. Short thigh
Hip is held in flexion, abduction, and external rotation
Position and stability of the knee and foot are variable. Clinical Findings
8. Limb length inequality
proximal joint instability
Malrotation
weakness of the proximal musculature
Primary clinical problems
9.
Aitken's four-part classification scheme
Gillespie & Torode classification
Nine Pappas Classes of Congenital Abnormalities of the Femur
Hamanishi classification
Classification
10. most widely used classification
divides PFFD into 4 categories based on the radiographic appearance
Aitken's four-part classification scheme
11. Aitken classification