Proximal Femoral Focal Deficiency - Philippa Rust 19/5/2006

Definition : developmental defect of the proximal femur recognizable at birth

 

Clinical presentation: short, bulky thigh, held flexed, abducted and externally rotated

1:6 bilateral

1:50,000 live births

Associated with coxa vara or fibular hemimelia (50%),

Absent lateral rays of the foot and tarsal coalition,

 

Ref: Grogan DP, Holt GR, et al. Talocalcaneal coalition in patients with fibula hemimelia or proximal femoral focal deficiency. 1994 JBJS-A;76:63-70

Congenital knee ligamentous deficiency and contractures are common.

Percentage of shortening remains constant through growth

 

 

Classification

1. Aitken :

 

Femoral head

Acetabulum

Femoral segment

Relationship at skeletal maturity

A

Present

Normal

Short

Gap between prox & distal femur (subtroc varus) may ossify or pseudoarthrosis

B

 

Present, hypoplastic

Moderately dysplastic

Short, prox bony tuft

No ossification between head and bony shaft

Head in acetabulum

C

 

Absent or ossicle only

Severely dysplastic

Short, proximal taper

May ossify between shaft and proximal ossicle, if not prox migration of femur

No articulation between head & acetabulum

D

 

Absent

Absent

Short, deformed

No acetabulum or head

Often bilateral

Ref: Aitken GT. Proximal femoral focal deficiency: definition, classification and management, Washington DC 1969, National Academy of Sciences.

 

Therefore A& B as head present potential for reconstruction

A – Proximal femoral osteotomy to correct varus

B - Proximal femoral osteotomy to correct varus & secure union

C&D – amputation and prosthesis, femoral-pelvic fusion

 

2. Pappas

Expanded classification with 9 classes, based on evaluation of 125 patients.

Pappas

Aitken class

% femoral shortening

femoral-pelvic abnormality

associated abnormally

Rx

1

 

-

Femur absent, tibia at level of acetabulum, but lack acetabulum development

Fibula absent

Prosthetic

2

D

70-90

 

Femoral head absent

Tibia short, fibula, foot, knee & ankle abnormality

Pelvic-femoral stability & prosthetic

3

B

45-80

 

No ossification between head and bony shaft, acetabulum may be absent

 

Short fibula, tibia, knee instability, foot malform

Union between shaft & hip & prosthesis

4

A

40-67

 

Gap between prox & distal femur joined by irreg cartilage pseudoarthrosis

Less freq than 3: Short fibula, tibia, knee instability, foot malform

Union between shaft, neck & head & prosthesis

5

A

48-85

 

Femur incompletely ossified, hypoplastic, & irreg. Abnorm midshaft femur

Short fibula, tibia, knee instability, foot malform

Prosthesis

6

 

30-60

 

Distal femur short, irreg & hypoplastic

Single lower leg bone, foot abnorm

Prosthesis

7

 

10-50

 



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