2. Objectives
• The main objective of the following slides are
to better understand the femoral deficiency
condition. Following are the sub-objectives:
– Definition
– Etiology
– Clinical presentations
– Gait pattern
– Classification
– Limitation
3.
4.
5.
6. Longitudinal Deficiency of Femoral
partial
• Classically known as Proximal Focal femoral
Deficiency (PFFD)
• Is the complex congenital absence of
– part or all the femur
– Associated with other L/L soft tissue
– Osseous abnormalities.
• May be unilateral/bilateral
• Involvement of hip may vary from normal to
complete absence of both hip joints
7. Etiology
• Tends to arise spontaneously without a clear
cut genetic pattern of inheritance,
• Some theory says possible linked mode of
inheritance in one family autosomal dominant
inheritance
• Embryological/ Teratologic Theory:
– Vascular epiphysial disruption
– Infection
– Medication such as thalidomide
8. Clinical presentation
• Shortened lower limb
• Thigh is short-thickened & funnel shaped
• Fibular deficiency/foot deficiency associated
• Spine, heart or upper limb may be involved
(61-92% chances)
• 15% chances are of bilateral
• In bilateral cases, the acetabular dysplasia is
more severe
9. Clinical presentation..
• Affected side hip is kept in flexed, abducted &
externally rotated pattern which is due the
combined effect of osseous deformity, soft-
tissue contracture
– This classic position of the thigh makes the limb
appear smaller than actual , as do the associated
fibular and foot deficiency.
• The knee is frequently involved and may be
unstable, with a flexion contracture.
10. Clinical presentation..
• Further, Genu valgum is common which get
enhanced by angular deformity of distal femoral
condyle
• The cruciate ligaments are absence, hence A-P &
M-L subluxation/dislocation are common
• Partial or complete absence of fibula together
with foot and ankle valgus deformity accentuates
the femoral shortening
• A non-functional foot may influence the decision
of limb salvage vs. amputation
11. Gait pattern
• Depending upon severity of the affected side
LDFP cases, patient walk
– By keeping ankle plantarflexed to compensate for the
shortening compared to sound side that is in case of
least affected unilateral LDFP condition.
– By keeping flexed hip and knee on the sound side in
case of the affected side is short and its foot is at the
level of mid of the leg of the sound side.
– By kneeling so that taking load on the knee of sound
side so that to keep pelvis square in case of severely
short affected side with foot at the level of knee of the
sound side
12. Classification of Femoral Deficiency
• Aitken is the most commonly
used classification system for
LDFP.
• It is Based on the X-
ray/radiological finding of
acetabulum and proximal
femur
• This classification of A through
D is designed with class A
being least involved while class
D most severely involved.
• Associated fibular deficiency is
present in large percentage of
PFFD.
13. Aitken classification
Type A
Femoral head & acetabulam
are present with femur
short. Lucency
representing a
pseudarthosis in the
proximal femur is seen
in radiographs; this may
unite by skeletal
maturity. Varus of the
proximal femur present
14. Aitken type B
Proximal femur is more
deficient, but the
acetabulam is present
and usually is well
formed.
The femoral head may
ossify late, but the
discontinuity between
the head and shaft
persist through
skeletal maturity.
15. Type C
• There is little or no
femoral head, the
acetabulam is severely
dysplastic or absence. A
ossified tuft is present
on the proximal end of
the femur
16. Type D
• The femur is extremely
short, with the head
absent and acetabulam
severely dysplastic or
absence and there is no
tuft in proximal femur