Fibular HemimeliaDr. Ajay Alex

CMC Ludhiana

Fibular hemimelia

Its is a congenital disorder characterised bypartial or complete absence of the fibula. It is the mostcommon 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

• Etiology- related to isolated mutation of the Limb bud. (sporadic)

• Fibular hemimelia is usually not an inheritable condition. • 1:40,000

Fibular hemimeliaAssociated 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

Shortening, absence of lateral rays anterior bowing

Equinus Hind foot valgus

Agenesis of femur with FH

Equino varus with FH

Fibular hemimelia• Achterman–Kalamchi classification

Type IA Type IB Type II

• 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

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.

• 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

Paleys Type-4

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

Fibular hemimelia : Management options

• Ablative - Syme’s or Boyd’s amputation.

• Limb lengthening - Limb lengthening using Ilizarov technique & foot

deformity correction

Knee Valgus

Stabilisation of anklea) _b) Gruca operationc) Bending osteotomy of exnerd) Reconstruction of lateral

malleoluse) Tibiotalar arthrodesis

Post Gruca’s

Foot & ankle deformity• Superankle procedure• Aim : to give fuctional plantigrade foot• 1st surgery• Youngest age 12 months without lengthening 18 month with lengthening

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

• 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

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

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 amputationOnly foot deformity no amputationOnly LLD no amputationWhy? In combination• Redifined foot deformity • Paley classification• Estimated LLD

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