Congenital Femur Deficiency and Fibular Hemimelia – Amputation ...

Congenital Femur Deficiencyand Fibular Hemimelia –Amputation and ProthetisationMaria José Costa, Francisco TavaresPhysical Medicine and RehabilitationHospital de Dona EstefâniaLisboa – Portugal

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationPediatric Amputations• About 70% of pediatric amputee are congenital limbdeficiencies:– Fibular Hemimelia (1 / 40.000) (1)– Congenital Femur Deficiency (1 / 50.000 – 200.000) (2)• 40% of the children with congenital limb deficiencies will havemultiple limb involvement (3)(1) Limb Reconstruction Surgery for Fibular Hemimelia; Dror Paley(2) Oppenheim WL, Setoguchi Y, Fowler E. Overview and comparison of Syme's amputation and knee fusion with the van Nes rotationplasty procedure inproximal femoral focal deficiency. In: Herring JA, Birch J, eds. The Child With a Limb Deficiency. Chicago, Ill:. American Academy of OrthopaedicSurgeons;1998.(3) Gibson DA. Child and juvenile amputee. In: Banjerjee SN, ed. Rehabilitation management of amputees. Baltimore/London: Williams & Wilkins, 1982

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationPediatric AmputationsISPO classification• Transverse Deficiencies– Easy decision prothetisation• Longitudinal Deficiencies– Complex problem– Multiple theapeutic options– Lower limb most frequent:• Fibular Hemimelia• Congenital Femur Deficiency

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationFibular Hemimelia• Associated with other deformities:– foot ray deficiency– sub-talar coalition– ball and socket ankle joint– ankle joint malorientation– diaphyseal angular deformity apex anteromedial– fibular deficiency– congenital shortening of the tibia– femural malformation (coxa vara, CFD, lateral femural condylohypoplasic,…)Limb reconstruction Surgery for fibular hemimelia; Dror PaleyLimb lenght discrepancy and congenital limb anomalies in fibular hemimelia; Rodriguez-Ramirez

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationFibular Hemimelia• Clinical presentation:– Shorter limb (below knee)– Frequently with footdeformity– Possible ankle and kneeinstability

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationCongenital Femur Deficiency• Spectrum of severity of femoral deficiency anddeformity:– Lack of integrity, stability and mobility of hip and kneejoints– Bone malorientation, bone malrotation– Soft tissue contractures of the hip and kneeLengthening Reconstruction Surgery for Congenital Femoral Deficiency, Dror Paley

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationCongenital Femur DeficiencyClinical Presentation:– Shorter lower limb (above knee)– Knee apparently inexistent or inproximal position– Usually normal foot

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationCFD and FH – What they have in common?• Abnormal stand position and gait– Dismetry– Deformity

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationPediatric Amputation• Primary goal: maximize function• Approach based on:– Preservation limb length and growth plates– Disarticulation– Proximal portion stabilization/normalization

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationLevel of Amputation – FH• In Fibular Hemimelia:– Incapacity to have aplantigrad foot– Unstable ankleSyme’samputation

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationElective Surgery for CFD• Varies according to Paley’s classificationType 1aType 1bType 2aType 2bType 3a Type 3b Type 3c Type 4

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationElective Surgery for CFD• Rotationplasty• Syme’s amputation– With or without knee fusionAdaptado de Tachdjian, 1 ed

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationCFD Prothetisation• Some patients refuse chirurgicaltreatment– Requiring non-standard prosthesisAdaptado de Tachdjian, 1 ed

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationDifferences between children's and adultsamputations• No sense of lossProsthesis:Assistivedevice

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationWhen to amputate?• Before 2 years old– before the foothas become fullyincorporated intothe child’s bodyimage

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationProsthesis Fitting• Between 6-12 months– Standing– Gait7 M 8M 9M 10M11M 12M 15M 18M 3A

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationTherapeutic training

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationAdequate psychomotor development

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationEnergy Expenditure• Through or below the knee amputation:– maintain a normal walking speed– without significantly increasing their energy• Above the knee amputation:– significantly slower walk (72-80%)– elevated heart rate (124%) and energy cost (VO2 151%-161%)• Bilateral BK amputation:– Slower velocity (87%)– Elevated heart rate (119%); similar energy costEffect of Amputation Level on Energy Expenditure During Overground Walking by Children with an Amputation; Kelly A. Jeans, Richard H. Browne and LoriA. KarolJ Bone Joint Surg Am. 2011;93:49-56.

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationFollow-up• Follow-up– 3-4 months intervals– Specially during growth spurts until maturity• New lower limb prosthesisLiteratureOur experience – HDE< 5 years5 to 12 years13–21 yearsAnuallyEveryother yearOnce every3 years16 yearsAnuallyEveryother year

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationProsthesis Selection• Age– Toddler:• Wide based, hip flexion andabduction, knee flexion,total foot initial contact• Prosthesis without kneecomponent• Function– Varies according topsychomotor development,personality and sportspreferences

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationProsthesis ComponentsSuspension andSockets:Knees:.Monocentric.Polycentric.Mechanic.Pneumatic.HydraulicFoots:• SACH• Dynamic• Flex Footjunior

Congenital Femur Deficiency and Fibular HemimeliaAmputation and Prothetisation

Congenital Femur Deficiency and Fibular HemimeliaAmputation and ProthetisationGive childrenthe prosthesisthat works as hard as theyplayObrigada