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Congenital Coxa Vara

 

synonyms:

Congenital Coxa Vara ICD-9

Congenital Coxa Vara Etiology / Epidemiology / Natural History

  • Defined as any decrease below the normal values of the neck-shaft angle of the proximal femur.
  • Congital, progressive autosomal dominant developmental disorder, or a result of trauma, LCP diesae, SCFE, metabolic disorder, or neoplasm.
  • Male = female, right = left
  • 30%-50% bilateral
  • Developmental coxa vara, represents coxa vara not present at birth but develops early in childhood and produces progressive deterioration of the proximal femoral neck-shaft angle during growth

Congenital Coxa Vara Anatomy

  • Slip occurs on the metaphyseal side of the epiphyseal plate, breaking away from the plate at its distal end to separate off a flake (triangular fragment) of metaphysis (the inverted 'Y')

Congenital Coxa Vara Clinical Evaluation

  • Painless waddling or Trendelberg gait generally noted between 2-6 years old.
  • Decreased hip abduction
  • Limb-length discrepancy is unilateral.

Congenital Coxa Vara Xray / Diagnositc Tests

  • AP and lateral pelvis films, lateral can be frog-legged or cross table .
  • Inverted Y pattern of the proximal formoral physis.
  • Fairbanks Fragment: fragment at the inferior neck associated with invertd Y pattern.
  • Hilgenreiner epiphyseal angle: angle between hilgenreiner's line and a lone drawn throught the proximal femoral phsis on AP pelvic view.
  • Classic Radiographic findings: 
    -decreased femoral neck-shaft angle
    -vertical position of the physeal plate
    - triangular metaphyseal fragment in the inferior femoral neck surrounded by an inverted radiolucent Y pattern ( sine qua non of this condition)
    -decrease in normal anteversion of the proximal femur, may become true retroversion; coxa breva; and in some patients acetabular dysplasia

Congenital Coxa Vara Classification / Treatment

  • Neck shaft angle >90° or Hilgenreiner angle < 45°
    -Treatment = observation
  • Neck shaft angle <90° or Hilgenreiner angle >60° or 45° -60° with documented progression of varus deformity
    -Treatment: corrective intertrochanteric or subtrochanteric valgus osteotomy. Goal = Hilgenreiner's angle of 25° , neck shaft angle between 150° and 160°. Consider concomittant adductor tentomy, proximal femoral shortening. (Carroll J Pediatr Othrop 17:220;1997)
  • Higenreiner physeal angle should be corrected to  >38 degrees to minimize recurrence. (Carroll J Pediatr Othrop 17:220;1997)

Congenital Coxa Vara Associated Injuries / Differential Diagnosis

  • Proximal femoral focal deficiency
  • Fibular hemimelia

Congenital Coxa Vara Complications

  • Premature physeal closure
  • Greater tochanteric overgrowth
  • Recurrence of deformity
  • Osteonecrosis
  • Limb-length discrepancy, Degenerative arthritis

Congenital Coxa Vara Follow-up Care

Congenital Coxa Vara Review References

  • Weinstein JN, JPO 1984;4:70
  • °

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