Tibial Deficiency

Topic updated on 02/16/16 11:33am
  • 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 (type I divided into a and b based on distal femoral epiphysis and extensor mechanism function)
  • 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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Qbank (1 Questions)

(OBQ06.90) A 13-month-old boy has shortening and deformity of the right leg. A clinical photograph and radiograph are provided in Figures A and B. Which of the following findings are indicative of a good outcome with a tibiotalar Syme amputation? Topic Review Topic
FIGURES: A   B        

1. 35 degree flexion contracture
2. Absence of tibiofibular synostosis
3. Bilateral involvement
4. Full functional quadriceps strength
5. Family history of tibial hemimelia



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