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WHITE TEAM PRESENTATION

WHITE TEAM PRESENTATION. WED, 7 TH MAY 2014. MANAGEMENT OF FIBULAR HEMIMELIA. DR TELLA A.O NOH, DALA. OUTLINE. INTRODUCTION CLINICAL EVALUATION DECISION MAKING LIMB RECONSTRUCTION AMPUTATION/PROSTHETIC FITTING AMPUTATION VS LIMB RECONSTRUCTION THE DALA EXPERIENCE CONCLUSION.

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WHITE TEAM PRESENTATION

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  1. WHITE TEAM PRESENTATION WED, 7TH MAY 2014

  2. MANAGEMENT OF FIBULAR HEMIMELIA DR TELLA A.O NOH, DALA

  3. OUTLINE • INTRODUCTION • CLINICAL EVALUATION • DECISION MAKING • LIMB RECONSTRUCTION • AMPUTATION/PROSTHETIC FITTING • AMPUTATION VS LIMB RECONSTRUCTION • THE DALA EXPERIENCE • CONCLUSION

  4. INTRODUCTION • Fibular hemimelia was initially described as aplasia or hypoplasia of the fibula. • Associated with constellation of lower extremity deformities • Associated deformities should be evaluated and treated • Extent of involvement of other bones dictates management

  5. INTRODUCTION • Relatively rare, with variable expression- Occurs 1 in 40,000 live births • Bilateral involvement is less common • Usually not an inheritable condition, but Family history must be sought • Historically, amputation was always recommended as a last resort. • Cause of fibular hemimelia is unknown

  6. HOW IS THE CHILD AFFECTED? • Limb length discrepancy (LLD)- affected side grows at a slower rate • Foot and ankle deformities • Knee joint deformities- Valgus deformity • Absent knee ligaments- Hypoplastic or absent ACL

  7. CLINICAL EVALUATION • Adequate history • Clinical examination • Work-up for treatment • Treatment options

  8. CLINICAL PRESENTATION

  9. CLINICAL EVALUATION • General examination • Knee & Patella • Leg • Ankle • Foot • Limb shortening

  10. WORK UP • Judicious use of appropriate imaging studies • Standing X-rays to fully evaluate the problem • Pelvic/Hip X-rays → PFFD • Knee series • Ankle series • Foot series

  11. WORK UP

  12. DECISION MAKING • GOALS OF TREATMENT:- Equalize limb length by skeletal maturity- Obtain a plantigrade foot with stable ankle- Correct associated deformities- Facilitate prosthetic fitting if the limb is to be ablated • Involve parents and families • Goals and expectations must be realistic

  13. TREATMENT OPTIONS • NON-OPERATIVE TREATMENT • LIMB RECONSTRUCTION- Limb lengthening or Epiphysiodesis- Stabilization of the ankle- Correction of tibia bowing- Correction of knee valgus- Correction of foot deformities • AMPUTATION & PROSTHETIC FITTING

  14. LIMB LENGTHENING • Prediction of LLD at skeletal maturity- Green/Anderson chart- Paley’s multiplier method- Moseley’s straight line graph • Have a reconstructive plan- Sequential lengthening advocated- Adequate counselling of parents

  15. PALEY’S MULTIPLIER TABLE

  16. MULTIPLIER TABLE (GIRLS)

  17. LIMB LENGTHENING

  18. LIMB LENGTHENING

  19. STABILIZATION OF THE ANKLE • Gruca operation • Bending osteotomy of Exner • Reconstruction of lateral malleolus with iliac graft • Wiltsesupramalleolarosteotomy • Superankle procedure by Paley • Tibiotalararthrodesis

  20. CORRECTION OF KNEE VALGUS Staple hemiepiphyseodesis Distal Femoral Osteotomy Right Knee GenuValgum

  21. AMPUTATION & PROSTHETIC FITTING • This is controversial • May be recommended if;- Failed limb reconstruction (salvage)- LLD > 25cm (Paley)- Absence of 2 or more rays of the foot- Associated PFFD

  22. PRINCIPLES OF AMPUTATION • Timing:- Ideal age < 2 years- Treatment completed by 3 years • The Procedure:- Syme’s amputation (preferred)- Boyd amputation- Below Knee Amputation- Above Knee amputation • Prosthetic fitting

  23. SYME’S AMPUTATION

  24. AMPUTATION VS LIMB RECONSTRUCTION • JUSTIFICATION FOR AMPUTATION- Failure to obtain satisfactory results with limb lengthening- Fewer surgical interventions- Fewer days of hospitalization- Lower complication rates- Excellent function with prosthetics (modern prosthetics)

  25. AMPUTATION VS LIMB RECONSTRUCTION

  26. AMPUTATION VS LIMB RECONSTRUCTION • JUSTIFICATION FOR LIMB RECONSTRUCTION- Are there unreconstructable limbs?- Are there fundamental errors in treatment strategy?- Well-aligned stable ankle is more functional than a prosthetic ankle- Psychological impact on the child- Cost of limb salvage vs amputation

  27. INFERENCES • AMPUTATION MAY NOT BE THE BEST OPTION OF TREATMENT • Limb reconstruction involves limb length equalization and correction of associated deformities • Where modern prosthetics is not available, amputation is useless

  28. PROGNOSIS • Depend on some factors:- Severity of LLD (<15% - mild, 15-25% - moderate, >25% - severe) • Stability of the ankle • Presence of other deformities • Bilateral involvement • No systemic involvement

  29. THE DALA EXPERIENCE • Fibular hemimelia is rare in our environment • TWO patients in the last 10 years • Treatment challenges- Paediatric LRS not available- Delay initiating treatment- Socio-cultural factors- Motivated parents

  30. CONCLUSION • The final result of limb lengthening is dependent on foot position after reconstruction • Parental choice and social factors are considered • Cases should be individualized • Surgeon and parents have to be motivated

  31. APPRECIATION DR DROR PALEY

  32. THANK YOU

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