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Fibular hemimelia

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Congenital anomaly of lower limb

Published in: Health & Medicine
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Fibular hemimelia

  1. 1. Fibular Hemimelia Dr. Ajay Alex CMC Ludhiana
  2. 2. Fibular hemimelia Its is a congenital disorder characterised by partial or complete absence of the fibula. It is the most common deficiency of long bones, and consists of a spectrum of anomalies ranging from mild fibular shortening with Limb length discrepancy to bilateral involvement with associated defects of the femur, tibia, ankle and foot.College of M
  3. 3. • Etiology- related to isolated mutation of the Limb bud. (sporadic) • Fibular hemimelia is usually not an inheritable condition. • 1:40,000
  4. 4. Fibular hemimelia Associated anomalies • Ankle instability– ball and socket joint • Valgus foot with the absence of one or more lateral rays • Tarsal coalition • Congenital short femur and Femur deficiency. • Diaphyseal angular deformity apex anteromedial. • Fibular deficiency • Hypoplasia of the lateral femoral condyle or patella • Knee instability – ACL insufficiency
  5. 5. Shortening, absence of lateral rays anterior bowing
  6. 6. Equinus Hind foot valgus
  7. 7. Agenesis of femur with FH Equino varus with FH
  8. 8. Fibular hemimelia • Achterman–Kalamchi classification Type IA Type IB Type II
  9. 9. • Type IA- the proximal fibular epiphysis is distal to the level of the tibial growth plate, whereas the distal fibular growth plate is proximal to the dome of the talus • Type IB- there is a partial absence of the fibula and proximally the fibula is absent for 30 to 50 percent of its length, whereas distally it is present but unable to support the ankle. • Type II- includes all limbs when there is a complete absence of the fibula or when only a distal, vestigial fragment is present
  10. 10. PALEYS Classificaton • Type 1- is a congenitally short tibia and fibula with a stable ankle joint. fibula is only slightly shorter at its upper end compared to the opposite side. • Type 2- short tibia and fibula with a foot that stands flat to the ground but often goes into a valgus position. Ball and socket ankle joint. • Type 3A- the fixed equinovalgus , due to a malorientation of the ankle joint. • Type 3B- the fixed equinovalgus deformity, due to a malunited subtalar coalition.
  11. 11. •Type 3C- it is due to a combination of maloriented ankle joint and a malunited subtalar coalition. •Type 3D- the subtalar joint is not fused and does not have the coalition but is maloriented. In fact in this condition, there is hypermobility of the subtalar joint. •Type 4- also known as the club foot type of fibular hemimelia has a subtalar coalition maloriented into varus (foot turned inwards) and appears more like a club foot deformity instead of the equinovalgus typical fibular hemimelia
  12. 12. Paleys Type-4
  13. 13. Goals of treatment • Plantigrade foot with stable ankle • Restore LLD • Correction of associated deformities of knee, tibia & ankle • Facilitate optimal prosthetic fitting if foot is ablated
  14. 14. Fibular hemimelia : Management options • Ablative - Syme’s or Boyd’s amputation. • Limb lengthening - Limb lengthening using Ilizarov technique & foot deformity correction
  15. 15. Knee Valgus
  16. 16. Stabilisation of ankle a) _ b) Gruca operation c) Bending osteotomy of exner d) Reconstruction of lateral malleolus e) Tibiotalar arthrodesis
  17. 17. Post Gruca’s
  18. 18. Foot & ankle deformity • Superankle procedure • Aim : to give fuctional plantigrade foot • 1st surgery • Youngest age 12 months without lengthening 18 month with lengthening
  19. 19. Super ankle procedure • Step 1: fibrous fibular anlage removal • Step 2: lengthen peroneal & Achilles tendon • Step 3: decompress peroneal & posterior tibial nerve • Step 4: reshaping dome of talus
  20. 20. • Step 5 : osteotomies depending upon type, 3A supra-malleolar, 3B subtalar, 3C combined, 3D special subtalar to correct mal-orientation • Step 6: pinning through heel pad • Step 7 : repair of lengthened Achilles & peroneal tendons • Step 8 : compartment release
  21. 21. Limb length discrepency • May be at tibia, femur & foot • Estimated LLD: Paley multiplier • 5 cm can be corrected in 4 years • 8 cm at 8year • 8 cm at 12 year • 5 cm by femoral epiphysoidesis of contralateral side • Total 25 cm LLD correctable
  22. 22. Dr. Dror Paley’s experience • After doing limb reconstruction in 2000 FH cases strongly recommended limb lengthening with super ankle procedure and suggested there is no role of amputation Only foot deformity no amputation Only LLD no amputation Why? In combination • Redifined foot deformity • Paley classification • Estimated LLD
  23. 23. THANK YOU

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