FIBULAR HEMIMELIA

FIBULAR HEMIMELIA

• 
  
• 
  
• most common long bone deficiency

 

• ETIOLOGY
  
  
  • not much defined
  
  
  • vascular dysgenesis and relative ischemia affect the developing mesenchyme
  
  
  • intercalary paraxial deficiency with normal foot / terminal deficiency with absent rays of foot
  
  
  
  

 

• CLASSIFICATION


• ACHTERMAN & KALAMCHI CLASSIFICATION

• IA : hypoplasia fibula
  
  
  
  
  • proximal fibular epiphysis distal to tibial epiphysis
  
  
  • distal fibular epiphysis proximal to talar dome
  
  
  
  
  
  


• IB : severe deficiency of fibula ( 30-50% length missing)
  
  
  
  
  • no support for ankle joint
  
  
  
  
  
  

 

• II : fibula absent
  
  
  
  
  • tibial angulation
  
  
  
  
  
  

 

 

• BIRCHI CLASSIFICATION FOR MANAGEMENT
  
  
  • I : functional foot
  
  
  
  

1. 0-5% inequality : orthosis, epiphysiodesis


2. 6-10% inequality: epiphysiodesis, limb lengthening


3. 11-30% inequality: limb lengthening / amputation


4. >30% inequality: limb lengthening, amputation (>2 procedures)

 

• II : non functional foot

1. functional upper limb : early amputation


2. non functional upper limb : limb salvage procedure

 

• CLINICAL FEATRUES
  
  
  • limb length discrepancy
  
  
  • % of discrepancy remains same throughout the growth
  
  
  • IA : ball and socket ankle
    
    
    
    
    • mild valgus at ankle
    
    
    • lateral malleolus above the ankle mortise
    
    
    
    
    
    
  
  
  
  

 

• IB : severe ankle and foot abnormalities
  
  
  • equino valgus
  
  
  • flexion contracture knee
  
  
  • femoral shortening
  
  
  • knee, ankle instability
  
  
  • stiff hind foot
  
  
  • absent lateral rays
  
  
  
  

 

• TREATMENT
  
  
  • PARTIAL HEMIMELIA
    
    
    • Syme amputation + prosthetic fitting
    
    
    • heel pad migration ( as complication to syme's) – countered by prosthetic fitting
    
    
    • advantages of early amputation
      
      
      • excellent emotional adaptation
      
      
      • low cost good functional outcome
      
      
      
      
    
    
    
    
  
  
  
  

 

• LENGTHENING : if shortening <8cm
  
  
  
  
  
  
  
  
  
  
  
  
  • plantigrade stable ankle jt.
  
  
  
  
  
  
  
  
  
  
  
  
  
  

 

• Classification predicting outcome – based on wedge shaped distal tibial epiphysis – Choi et al

1. mild wedge

• mild growth retardation


• minimal foot deformity

1. moderate wedge

• worsened asymmetrical growth


• progressive deformity

1. severe wedging

• severe growth retardation


• severe foot deformity

 

ANKLE VALGUS

• corrected with varus supramalleolar osteotomy


• Wiltse Varus Supramalleolar Osteotomy


• prevent medial malleolar prominence

 

• COMPLETE HEIMIMELIA
  
  
  • amputation/ limb lengthening if discrepancy <5cm at birth, no foot deformity + combined osteotomy for tibia
  
  
  
  

 

• B/L
  
  
  • amputation/ limb lengthening

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