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
- 0-5% inequality : orthosis, epiphysiodesis
- 6-10% inequality: epiphysiodesis, limb lengthening
- 11-30% inequality: limb lengthening / amputation
- >30% inequality: limb lengthening, amputation (>2 procedures)
- II : non functional foot
- functional upper limb : early amputation
- 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
- PARTIAL HEMIMELIA
- LENGTHENING : if shortening <8cm
- plantigrade stable ankle jt.
- Classification predicting outcome – based on wedge shaped distal tibial epiphysis – Choi et al
- mild wedge
- mild growth retardation
- minimal foot deformity
- moderate wedge
- worsened asymmetrical growth
- progressive deformity
- 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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