Updated: 6/14/2021

Tibial Deficiency

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Flashcards
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Questions
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Evidence
6
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Cases
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Topic
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https://upload.orthobullets.com/topic/4058/images/tib hemimelia_moved.jpg
https://upload.orthobullets.com/topic/4058/images/tibia hemi_moved.jpg
  • summary
    • Tibial Deficiency is a congenital condition characterized by a longitudinal deficiency of the tibia with varying degrees of tibial absence.
    • Diagnosis is made radiographically with xrays of the tibia. 
    • Treatment is a variety of surgical options depending on the extent of deficiency and stability of knee joint. 
  • Etiology
    • Genetics
      • autosomal dominant inheritance pattern
        • obtain genetic counseling
    • Associated conditions
      • musculoskeletal conditions present in 75% of patients
        • ectrodactyly
        • preaxial polydactyly
        • ulnar aplasia
  • Classification
    • Jones classification
    • Jones Classification
      Type 1a
      • No proximal tibia visible on radiograph  
      • Extensor mech absent
      • Hypoplastic distal femoral epiphysis
      Type 1b 
      • Proximal tibia eventually ossifies and extensor mechanism will function
      • Distal femoral epiphysis appears normal
      Type 2
      • Proximal tibia present at birth but short tibia
      Type 3
      • Diaphyseal and distal tibia present but proximal tibia absent
      Type 4
      • Short tibia, fibula migrated proximal, diastasis of distal tib-fib joint
    • Alternate system is Kalamachi and Dawe (types 1-3)
  • Presentation
    • 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
  • Imaging
    • Radiographs
      • recommended
        • AP and lateral tibia/fibula
      • findings
        • show deficiency of the tibia
        • early radiographs may show small and minimally ossified distal femoral epiphysis
  • Treatment
    • 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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Flashcards (2)
Cards
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Questions (1)

(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 most predictive of a good outcome with a Syme amputation?

QID: 201
FIGURES:
1

35 degree flexion contracture

1%

(22/2629)

2

Absence of tibiofibular synostosis

10%

(269/2629)

3

Bilateral involvement

0%

(13/2629)

4

Full functional quadriceps strength

87%

(2292/2629)

5

Family history of tibial hemimelia

1%

(20/2629)

L 2 D

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Evidence (6)
CASES (1)
EXPERT COMMENTS (22)
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