PROXIMAL FEMORAL

FOCAL DEFICIENCY

D R . FA D I K H A S AW N E H
Definition
 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 partial skeletal defect in the

proximal femur with a variably unstable hip joint,
shortening.
Associated Anomalies
 Fibular hemimelia 70-80%
 Agenesis of cruciate ligaments of the Knee
 Clubfoot
 Congenital heart anomalies
 Spinal dysplasia
 Facial dysplasia
ETIOLOGY
 The etiology of PFFD is not known exactly
 Two Theories:
 Sclerotome Subtraction Theory- “Injury to neural crest
cells”.

 Boden’s Theory- “A defect in proliferation and

maturation of chondrocytes in the proximal growth
plate”.
Etiology
 Unknown cause – majority of cases

Anoxia
irradiation
Ischemia
Bacterial and Viral infection
Toxins and Hormones
Thalidomide definite cause
Incidence
 3rd most common longitudinal deficiency of lower
extremity
 Bilateral involvement is seen in 50%
 One per 50,000 live births
 Maternal diabetes has been implicated in femoral
hypoplasia
Clinical Findings
 Short thigh.

 Hip is held in flexion , abduction ,and external rotation.

 Position and Stability of the Knee and foot are variable.

Primary Clinical Problems
 Limb length inequality

 Proximal joint instability

 Malrotation

 Weakness of the proximal musculature

Classification
 Aitken’s four- part classification scheme

 Gillespie & Torode classification

 Nine Pappas Classes of Congenital Abnormalities of the

Femur

 Hamanishi classification
Aitken’s Four- Part Classification Scheme

 Most widely used classification

 Divides PFFD into 4 categories based on the

radiographic appearance
Aitken’s Classification
Aitken’s four- part classification scheme

 Class A

 Least severe type

 Femoral head is present and attached to the shaft
 Acetabulum is well formed
 Femur is shortened and a coxa vara deformity is present
 Cartilaginous connection b/w neck and shaft forms a sub
trochanteric pseudarthrosis
Aitken’s four- part classification scheme

Class B
 Femoral segment is short and usually has a bulbous
bony tuft
 Proximal end of femur usually positioned above
acetabulum is “adequate” on moderately dysplastic
and contains femoral head
 At maturity, no osseous connection is seen between
the femoral head and the shaft
Aitken Class B
Aitken’s four- part classification scheme
 Class C

 The acetabulum is severely dysplastic

 The femoral head is absent

 The shortened femoral segment has a tapered proximal end
Aitken Class C
Aitken’s four- part classification scheme

 Class D

 This is the most severe form , with absence of the

acetabulum and proximal femur

 No proximal tuft is present

Gillespie & Torode classification

 This is clinically based treatment oriented

classification system

 Initially 2 groups , later modified into 3 groups

Gillespie & Torode classification
 Group A

 Candidates for limb lengthening

 Congenitally short femurs but clinically stable hips

 Ipsilateral foot at or below the level of middle of

contralateral tibia

 Indicating the over all limb length discrepancy is 20% or less

Gillespie & Torode classification
 Group B

 Affected femur is 50% or less the length of contra lateral

femur
 Foot at the level of knee or above it
 Categorized as Aitken’s type A, B, C
 Limb length discrepancy of approx. 40%
 Best managed with prostheses after surgical conversion
(knee fusion, rotationplasty etc.)
Gillespie & Torode classification
 Group C
 Represent the same patients as Aitken’s class D

 They have subtotal absence of the femur

 Arthrodesis of the knee is not indicated

 Prosthetic treatment
Gillespie & Torode classification
Treatment
Goals of treatment of PFFD

 To compensate for the functional deficits

 No single treatment approach applies to all cases
 Each person with PFFD must be assessed individually
 Cosmetic is much less of an issue
Treatment
 Evaluation: The two essential factors in
treatment plan for PFFD

i. The predicted length of the femur at maturity

ii. Pelvic- femoral stability

Treatment
 The predicted length of the femur at maturity
 Calculation:
• The percentage of growth inhibition is calculated by the
difference between the normal and abnormal lengths
divided by the normal length and multiplied by 100

• The anticipated normal limb length is multiplied by the

percentage of growth inhibition, and the length of the
involved limb segment at skeletal maturity can be
determined
Treatment

• Pelvic- femoral stability :

• The acetabular index, shelf index, acetabular

radiodensity, and acetabular dysplasia are used
to predict ultimate pelvic-femoral stability at
early age.
Treatment
 Based on predicted length of the femur at maturity
and Pelvic- femoral stability koman divided patients
into two classes :
I. Class ( I )
II. Class ( II )
Treatment
 Class ( I )

• Patients have greater than 60% of the predicted

femoral length
• Evidence of pelvic-femoral stability
• Less than 17 cm of projected extremity shortening
• Presence of a stable planta- grade foot
Treatment

 Class ( II )

• Patients have less than 60% of predicted femoral length

or greater than 17 cm predicted extremity shortening
Surgical management
Surgical options:
 Equinus Prosthesis
 Ankle Disarticulation and prosthetic fitting
 Ankle Disarticulation and knee arthrodesis
 Ankle Disarticulation and femoral pelvic arthrodesis
 Rotationplasty and femoral pelvic arthrodesis
 Rotationplasty and femoral pelvic arthrodesis
 Limb lengthening procedures
Treatment
 Major problems

 Limb- length inequality

 Variable inadequacy of the proximal femoral muscular
and hip joint
 Malrotation
 Instability of proximal joints
Treatment
o Limb- length inequality

• Depend on the length whether group A or B:

• Group A > limb length discrepancy is 20% or less

• Group B > more than 20% or shortening more than 50%
Treatment
Group A

 The foot is at the mid tibial level & predicted to be 50%

length of normal side at maturity
 The ideal treatment is limb lengthening
 The goals are to eliminate the length of discrepancy
 Preexisting deformities( femoral neck varus, acetabular
dysplasia) should be corrected prior to lengthening
Treatment
Group B
 Amputation : age 2-4 years

a. Foot combined with knee arthrodesis

b. Hip stability before any attempt
c. Syme or Boyd to create limb for prosthesis
d. Arthrodesis converts functional AKA
Treatment
Group B
Rotationplasty
a. The foot is rotated 180 degrees to serve as a knee joint
b. The ankle joint should be normal ( at least 60 degree
arc of motion)
c. Ankle should be at the knee joint level
Treatment
femoral pelvic arthrodesis:
a. Elimination of lurch and stabilization of hip
b. Steel’s method; femoral segment is fused to pelvis and
the knee joint functions as a hip joint
c. Epiphysiodesis is done to maintain length
d. Brown’s method: Rotation of femur 180 degrees before
fusion with the pelvis
Treatment
 Group B
Knee Fusion for prosthetic conversion

a. Convert PFFD limb into single skeletal lever

b. Arthrodesis of knee and syme ankle disarticulation

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