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.
4. • Etiology- related to isolated mutation of the Limb bud.
(sporadic)
• Fibular hemimelia is usually not an inheritable condition.
• 1:40,000
5. 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
10. • 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
11. 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.
12.
13. •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
20. Foot & ankle deformity
• Superankle procedure
• Aim : to give fuctional plantigrade foot
• 1st
surgery
• Youngest age
12 months without lengthening
18 month with lengthening
22. • 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
23. 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
24.
25. 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
In SA with lengthening bending of tibia will be corrected during lengthening, but if done alone bending should be corrected by removing wedge from tibia and fixation with internal pins & cast
1) To prevent valgus reccurence & helps in lengthening, 3) to prevent damage during deformity correction 4) increase arc of motion of ankle joint
