PediatricLimbDeficiency

RamonaM.Okumura,C.P./L.P.
ClinicalProsthetist
SeniorLecturer,DivisionofProstheticsOrthotics
DepartmentofRehabilitationMedicine
SchoolofMedicine
UniversityofWashington
Introduction
smallnumberbornwithoracquiringalimbdeficiency
vastmajorityhavenoknownetiology
childschangingdevelopmentalcapabilitiescontinuously
altertheteamtreatmentplan
mustkeepthedoorsopenforlongtermgoalsandyet
provideforoptimalfunctioning
successfuloutcomedependsontreatmentofthewhole
family
verypleasantclientswhichusuallydoverywellina
healthyfamilyunit
Objectives

BeabletoclassifyLimbDeficiencies
Identifyparticularmanagementissues
Knowhowoftentoreviewtheprescriptionasthe
childgrows
Predictafunctionaloutcome
Epidemiology
Incidenceestimatedat4per10,000births
Congenital60%toAcquired40%
Male:Female
Congenital1.2:1
Acquired3:2
Left:Right
2:1inunilateralUETransverseDeficiency
Epidemiology
Commonpresentations
Unilateraltransverse
deficiencyofthe
forearmmiddlethird
 Epidemiology
Commonpresentations
Unilateralconversion
byankle
disarticulationfor
longitudinalfibular
deficiency
 Embryology

Limbsform47weeksgestation
Proximaltodistalinsequence
Upperlimbdevelopsslightlyaheadofthe
lowerlimb
Simultaneouslywithorgandevelopment
AssociatedwithRadialdeficiency
Etiologyof
CongenitalDeficiencies

Environmental
Genetic
EnvironmentalEtiology
preciseoriginunknowninthemajorityof
cases
EnvironmentalEtiology
preciseoriginunknown
SpeculateVascularcausesparticularlysome
kindofThromboembolism
EnvironmentalEtiology
preciseoriginunknown
SpeculateVascular/Thromboembolism
Mechanical:AmnioticBandsorStreeters
dysplasiainwhichmultiplelimbsare
involved
EnvironmentalEtiology
preciseoriginunknown
SpeculateVascular/Thromboembolism
Mechanical:AmnioticBands
Maternal:
diabetesmellitus
intrauterineinfection
EnvironmentalEtiology
preciseoriginunknown
SpeculateVascular/Thromboembolism
Mechanical:AmnioticBands
Maternalcauses
Pharmaceutical:
Thalidomideonlyprovendrug
Otherssuspected,noconvincingevidence
GeneticEtiology
Chromosomal:ex.
Turners
syndromeXXX
GeneticEtiology
SingleGene
Autosomal
Dominant:
Longitudinaltibial
deficiency
GeneticEtiology
SingleGene
Autosomal
Dominant
Autosomal
Recessive:TAR
Thrombocytopenia
AbsentRadius
Etiologyof
AcquiredDeficiencies
Trauma67%
especiallytrainsand
lawnmowers
Etiologyof
AcquiredDeficiencies
Disease33%
majoritycausedby
malignancy,
particularly1221
yearsofage
Etiologyof
AcquiredDeficiencies
Surgicalconversion
forcongenitallimb
deficiency
Classificationof
CongenitalLimbDeficiency
InternationalOrganizationfor
Standardization(ISO)
restrictedtoskeletalradiologicaldeficiency
ISOClassificationof
CongenitalLimbDeficiency
Transversedeficiency:
noskeletalelementspresentdistally
Nametheleveloftheportionofthelimb
involved(UpperArm)
Statetheportionwheretheabsenceoccurs
(middlethirdortotal)
ISOClassificationof
CongenitalLimbDeficiency
LeftTransverse
deficiency:
Forearm
middlethird
ISOClassificationof
CongenitalLimbDeficiency
Longitudinaldeficiency:
skeletalelementspresentaxiallyordistally
Namethebonesinvolved
Statepartialortotalabsence
ISOClassificationof
CongenitalLimbDeficiency
RightLongitudinal
deficiency:
Fibulatotal
Tarsalspartial
Rays3,4,5total
FrantzandORahillyClassification
CongenitalLimbDeficiency

terminalorintercalary
transverseorparaxial
completeorincomplete
additionalterms
ameliatotalabsenceofthelimbinvolved
hemimeliapartialabsenceofthelimbinvolved
phocomeliaabsenceofthelongbones
Classificationof
AcquiredLimbDeficiency
Throughlongbones
UpperExtremity
Transradial=BelowElbow(BE)
Transhumeral=AboveElbow(AE)
LowerExtremity
Transtibial=BelowKnee(BK)
Transfemoral=AboveKnee(AK)
LimbSalvageandTurnplasty(VanNess)
Classificationof
AcquiredLimbDeficiency
Throughthejoint:
Namethejoint+Disarticulation
UpperExtremity:WristDisarticulation,
etc.
LowerExtremity:AnkleDisarticulation,
etc.
ClinicalPrinciples
Treatmentgoals
Healthybody
image
Maintainchoicefor
prostheticoptions
Optimalfunction
ClinicalPrinciples
Generalconsiderations

Teamapproach
Developmentalfocus
Returnappointments
34monthstoevalprostheticfit&function
Annuallyforteamtoassessdevelopmental
needs
ClinicalPrinciples
PsychosocialSupport
Clientsneedtomeetotherswithsimilar
presentations
Guiltandassociatedfamilialproblems
Givechildcontrolanddecisionmaking
opportunities
Geneticcounselingshouldbeprovidedto
boththechildandparents
ClinicalPrinciples
SurgicalPlanning
Timingfor
congenital
conversions
ClinicalPrinciples
SurgicalPlanning
Timing
Growthplate
considerations
ClinicalPrinciples
SurgicalPlanning
Timing
Growthplate
considerations
Overgrowthwith
longbone
transections
ClinicalPrinciples
SurgicalPlanning
Timing
Growthplate
considerations
Overgrowth
Planningfor
multiplesurgical
procedures
ClinicalPrinciplesOTandPT
Wheninfants,wemusttrainparents
andcaregivers
Childrenneedminimaltraining
insteadneedopportunity
ClinicalPrinciples
Prostheticdesigns
Endoskeletalvs.exoskeletal
Flexiblevs.rigid
Growthadjustabledesigns
Sockswhenapplicablecanallowforgrowth
Forunilateraldeficiencies,legsareused,but
armsoftenrejected
Recreation
ClinicalPrinciples
LEProstheticConsiderations
Wearingguidelines
Fitwhenpullthe
standandcruising
(912months)
ClinicalPrinciples
LEProstheticConsiderations
Foot/Ankle
Toddlergait
Lacksheelstrike
Widebaseofsupport
ClinicalPrinciples
LEProstheticConsiderations
Childsgait
morenormalgait
benefitfrom
dynamicfoot/ankle
ClinicalPrinciples
LEProstheticConsiderations
Knee
Toddlerhas
fixed/lockedknee
Somecenters
experimentingwith
afreeknee
ClinicalPrinciples
LEProstheticConsiderations
Knee
Toddlerhas
fixed/lockedknee
At3y.o.temporary
reductionofKnee
ROMwhilelearning
ClinicalPrinciples
LEProstheticConsiderations
Hip
usesalignment
stability
ClinicalPrinciples
UEProstheticConsiderations
Wearingguidelines
3mosforsupinegrasp
Fitwhensit
Bestbefore12mos.
Commonperiodsfor
rejection
Unilateralsfunctional
withoutprosthesis,but
morereceptivelearners
thanadults
ClinicalPrinciples
UEProstheticConsiderations
Grasp
Passiveasaninfant
forgrossgrasp
ClinicalPrinciples
UEProstheticConsiderations
Grasp
Activewhen
developmentally
readyandableto
understandgrasping
function(1824
months)
ClinicalPrinciples
UEProstheticConsiderations
Grasp
Electricswitchcontrol
canprovideactive
controlatanearlier
age
ClinicalPrinciples
UEProstheticConsiderations
Elbow
Fixedforsitting
balanceasaninfant
ClinicalPrinciples
UEProstheticConsiderations
Elbow
Fixedasaninfant
Passivefrictionfor
toddler
Activelockingat3
yearsold
ClinicalPrinciples
UEProstheticConsiderations
Highlevel
prostheticfunction
poorsubstitute
andoftenrejected
SpecialCaseDiscussion
ProximalFemoral
FocalDeficiency
(PFFD)
AitkenAorB
SpecialCaseDiscussion
ProximalFemoral
FocalDeficiency
(PFFD)
AitkenAorB
AitkenCorD
SpecialCaseDiscussion
Longitudinalfibular
deficiency
Normalfoot
withstableankle,
centralizationsurgery
AFO
ShoeLifts/Lengthening
SpecialCaseDiscussion
Longitudinalfibular
deficiency
Abnormalfoot
Ankledisarticulation
conversion
foraProsthesis
SpecialCaseDiscussion
Septicemia/Purpura
Fulminans
Skinmanagement
Overgrowth
SpecialCaseDiscussion
RadialDeficiency
Associatedpathologies
genetics
SpecialCaseDiscussion
TibialDeficiency
Surgicalconversion
genetics
SpecialCaseDiscussion
Bilateralupper
extremity
SpecialCaseDiscussion
Bilateralupper
extremity
Bilaterallower
extremity
SpecialCaseDiscussion
HighLevel
Quadramembral
Deficiencies

FUNCTION