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  • A longitudinal deficiency of the tibia with varying degrees of tibial absence
    • previously known as tibial hemimelia
  • Genetics
    • autosomal dominant inheritance pattern
      • obtain genetic counseling
  • Associated conditions
    • musculoskeletal conditions present in 75% of patients   
      • ectrodactyly
      • preaxial polydactyly
      • ulnar aplasia
  • Jones classification: types I- IV

1a: No proximal tibia visible on radiograph.  Extensor mech absent. Hypoplastic distal femoral epiphysis

1b- Proximal tibia eventually ossifies and extensor mechanism will function.  Distal femoral epiphysis appears normal

2- Proximal tibia present at birth but short tibia

3- diaphyseal and distal tibia present but proximal tibia absent

4- short tibia, fibula migrated proximal, diastasis of distal tib-fib joint

  • Alternate system is Kalamachi and Dawe  (types 1-3)
  • Physical exam
    • deformity
      • shortening of the affected extremity
      • anterolateral bowing of the tibia
      • prominent fibular head
    • ROM & stability
      • the knee is usually flexed with an associated knee flexion contracture 
        • it is important to evaluate for active knee extension
      • check stability of the knee joint in all planes
        • treatment is based primarily on the stability of the knee joint
    • foot deformity
      • there is often a rigid equinovarus and supination deformity of the foot
      • sole of foot faces perineum
  • Radiographs
    • recommended
      • AP and lateral tibia/fibula 
    • findings
      • show deficiency of the tibia  
      • early radiographs may show small and minimally ossified distal femoral epiphysis
  • Operative
    • knee disarticulation followed by prosthestic fitting
      • indications
        • complete  absence of the tibia
        • no active knee extension present (most cases)
    • tibiofibular synostosis with modified Syme amputation
      • indications
        • proximal tibia present with intact extensor mechanism and minimal flexion contracture 
    • Syme/Boyd amputation
      • indications
        • ankle diastasis
    • Brown Procedure (centralization of fibula under femur)
      • no longer recommended due to high failure rate

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