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Proximal Focal Femoral Deficiency

- See:
       - Defects of the Fibula:
       - Van Nes Rotational Plasty:

- Discussion:
    - a development defect of the proximal femur recongnizable at birth;
    - 3rd most common longitudinal deficiency of lower extremity;
    - abnormality ranges from hypoplasia of entire femur to complete absence of the proximal end;
    - bilateral involvement is seen in 15% of patients;

- Limb Length Deformity:
    - percentage of shortening is constant over growth and allows an assessment of final outcome;

- Associated Anomalies:
    - fibular hemimelia(2/3 of pts);
    - PFFD can be associatted with coxa vara or fibular hemimelia (50%)
    - congential knee ligamentous laxity and contracture are also common;

- Clinical Findings:
    - affected extremity has short thigh, & hip is held in flexion, abduction, and external rotation;
    - position and stability of the knee and foot are variable.
    - primary clinical problems are limb length inequality, malrotation, instability at the hip (and, to a lesser extent, at knee), and weakness of the 
           proximal musculature;

- Classification:
    - Aitken:
         - Class A
               - femoral head present;
               - severe varus deformities may develop;
         - Class B
               - femoral head present, but ossification is delayed;
               - severe varus deformities & pseudoarthrosis may develop;
                   
        - Classes C
              - femoral head is not present;
              - severe dysplasia of acetabulum;
              - severe shortening of femur;
                   
        - Class D:
              - femoral head is not present;
              - severe dysplasia of acetabulum;
              - severe shortening of femur;
                   

- Treatment:
    - standard orthopaedic reconstructive procedures have proved totally ineffective in correcting leg length inequality seen in unilateral
          PFFD, esp when there is accompanying ipsilateral fibular hemimelia;
    - treatment must be individualized based on leg length discrepancy, adequacy of proximal musculature, femoral rotation, & proximal joint stability;
    - treatment options:
          - Limb lengthening or contralateral epiphysiodesis (or both) for mild cases;
          - Iliofemoral fusion
               - main disadvantage: inability for prosthesis to allow ischial containment
          - Knee fusion + Boyd (or Syme amputation or Van Nes rotationplasty)
                    - Example of Knee Fusion:
                         

                 - Example of Boyd:
                       

          - Creative prosthetic application for more severe cases;

 - Van Nes Rotationplasty:
     - if calculations indicate that foot of affected limb will be sig distal to level of knee of sound limb, consideration should be given to 
               performing Van Nes rotational osteotomy thru leg;
 - Amputation:
     - if foot of affected limb will lie proximal to or at level of knee of sound limb, ablation of foot by ankle disarticulation w/ a syme closure &
               prosthetic fitting as AKA is indicated;
     - following either ankle disarticulation & above knee prosthetic fitting or rotation-plasty & BKA fitting, consideration should be given to 
         arthrodesis of knee in order to provide to provide a more stable stump and to enhance prosthetic fitting;

- Bilateral PFFD:
    - bilateral PFFD do not present significant limb length inequalities but manifest other biomechanical deficiencies plus disproportionate dwarfism;
    - it is of interest that almost all reported bilateral cases of PFFD are of the D subtype;
    - children w/ bilateral PFFD generally walk quite well w/o any form of prosthetic restoration, & surgical procedures almost always detract
            from their ambulatory independence rather than benefit from them;
    - it is widely accepted that children w/ bilateral PFFD should not be treated surgically unless they ambulate w/o prosthesis



 Natural history and treatment of instability of the hip in proximal femoral focal deficiency.

 Talocalcaneal coalition in patients who have fibular hemimelia or proximal femoral focal deficiency. A comparison of the radiographic and
 pathological findings
.

 Proximal femoral focal deficiency: does a radiologic classification exist.

 Soft tissue anatomy of proximal femoral focal deficiency.

 Proximal femoral focal deficiency: evaluation and management.

 Rotational osteotomy for proximal femoral focal deficiency.

 Proximal femoral focal deficiency. Evidence for a defect in proliferation and maturation of chondrocytes.

 Iliofemoral fusion for proximal femoral focal deficiency.

 Familial bilateral proximal femoral focal deficiency. Report of a kindred.

 Missing cruciate ligament in congenital short femur.  

 Proximal femoral focal deficiency.

 Congenital abnormalities of the femur and related lower extremity malformations: classification and treatment.

 Proximal femoral focal deficiency: a 50-year experience.

 Proximal femoral focal deficiency: natural history and treatment.

 Proximal femoral focal deficiency: a clinical appraisal.

 Proximal femoral focal deficiency: treatment and classification in  forty-two cases.

 Tibial rotation-plasty for proximal femoral focal deficiency.

 Van Nes rotational osteotomy for treatment of proximal femoral focal deficiency and congenital short femur.

 Proximal femoral focal deficiency: results of rotationplasty and Syme amputation.

 Tibial rotation-plasty for proximal femoral focal deficiency.

 Rotational osteotomy for proximal femoral focal deficiency.

 Computed Tomographic Angiography in Proximal Femoral Focal Deficiency