Proximal Focal Femoral Deficiency

- congenital malformation

- characterised by failure of normal development of the proximal femur





- 1 in 50 000


Bilateral in 15% 

- usually more severe




Fibular hemimelia in 2/3

ACL deficient


May also be associated with


- Cleft palate


- Congenital spinal deformity

- Dysraphism


Aitken's Classification


Class A

- short femoral shaft with coxa vara

- head of femur present, neck may be absent early

- adequate acetabulum


- bony connection between head & neck present at maturity

- may be pseudarthrosis at point of connection


Class B

- pseudoarthrosis between head and shaft

- shorter femoral shaft with small bony tuft on proximal end

- head of femur present

- adequate acetabulum



Class C

- no femoral head

- acetabulum severe dysplastic

- very short femoral shaft with bony tuft proximally


Class D

- femoral head & acetabulum completely absent

- femur consists of condyles only




Child with very short leg

- large shoe raise

- LLD above GT


DDx of shortening above GT

- DDH 


- Tom Smith arthritis / post septic arthritis

- Tumour



- short involved thigh segment

- characteristic posture (Flex / Abducted / ER hip)

- Ship's Funnel Sign 


Bulky upper thigh segment

- sharply tapering to knee




1.  Pseudoarthrosis

2.  Instability of the hips

3.  Coxa vara

4.  LLD

5.  Inadequate lateral femoral condyle

6.  Inadequate proximal musculature

7.  Malrotation




Class A & B


Femoral heads present


1.  Stabilize pseudarthrosis with IM Rod

2.  Hip stabilisation 

- pelvic osteotomy

- femoral osteotomy

3.  Limb lengthening


Class A


1.  Realign Coxa Vara

- subtrochanteric osteotomy


2.  Equalise LLD

- lengthen femur or

- shorten / epiphysiodesis other femur



- if lengthen femur need to watch knee doesn't dislocate 

- ACL deficient

- may have to bridge knee with external fixator


Class B


1.  Fuse pseudarthrosis 


2.  Limb lengthen or amputate as required


Class C & D


No femoral heads


Stabilise hip

- pelvic support osteotomy

- osteotomy proximal femur

- supported by ischium

- distal osteotomy for mechanical axis

- need lengthening as well


Foot above contralateral knee

- knee fusion + symes

- essentially AKA


Foot below contralateral knee

- rotate then fuse knee / Van Ness Rotational Osteotomy

- ankle acts as knee joint / essentially BKA

- BV become anterior

- require good functioning foot




Usually Class C or D

- can usually walk well without prosthesis

- surgical procedures usually detract from mobility

- should not be treated surgically unless such severe foot deformity that cannot ambulate without prosthesis / Symes