Upload
austin-richard
View
225
Download
0
Tags:
Embed Size (px)
Citation preview
Pediatric Limb Deficiency
Ramona M.Okumura, C.P./L.P.Ramona M.Okumura, C.P./L.P.
Clinical ProsthetistClinical Prosthetist
Senior Lecturer, Division of Prosthetics OrthoticsSenior Lecturer, Division of Prosthetics Orthotics
Department of Rehabilitation MedicineDepartment of Rehabilitation Medicine
School of MedicineSchool of Medicine
University of WashingtonUniversity of Washington
IntroductionIntroduction small number born with or acquiring a limb deficiencysmall number born with or acquiring a limb deficiency vast majority have no known etiologyvast majority have no known etiology child’s changing developmental capabilities continuously child’s changing developmental capabilities continuously
alter the team treatment planalter the team treatment plan must keep the doors open for long-term goals and yet must keep the doors open for long-term goals and yet
provide for optimal functioningprovide for optimal functioning successful outcome depends on treatment of the whole successful outcome depends on treatment of the whole
family family very pleasant clients which usually do very well in a very pleasant clients which usually do very well in a
healthy family unithealthy family unit
ObjectivesObjectives
Be able to classify Limb DeficienciesBe able to classify Limb Deficiencies Identify particular management issuesIdentify particular management issues Know how often to review the prescription as the Know how often to review the prescription as the
child growschild grows Predict a functional outcomePredict a functional outcome
EpidemiologyEpidemiology
Incidence estimated at 4 per 10,000 birthsIncidence estimated at 4 per 10,000 births Congenital 60% to Acquired 40%Congenital 60% to Acquired 40% Male : FemaleMale : Female
Congenital 1.2:1Congenital 1.2:1 Acquired 3:2Acquired 3:2
Left : Right Left : Right 2:1 in unilateral UE Transverse Deficiency2:1 in unilateral UE Transverse Deficiency
EpidemiologyCommon presentationsEpidemiologyCommon presentations
Unilateral transverse Unilateral transverse deficiency of the deficiency of the forearm middle thirdforearm middle third
Epidemiology Common presentationsEpidemiology Common presentations
Unilateral Unilateral conversion by conversion by ankle ankle disarticulation for disarticulation for longitudinal longitudinal fibular deficiencyfibular deficiency
EmbryologyEmbryology
Limbs form 4-7 weeks gestationLimbs form 4-7 weeks gestation Proximal to distal in sequenceProximal to distal in sequence Upper limb develops slightly ahead of the Upper limb develops slightly ahead of the
lower limblower limb Simultaneously with organ developmentSimultaneously with organ development Associated with Radial deficiencyAssociated with Radial deficiency
Etiology ofCongenital DeficienciesEtiology ofCongenital Deficiencies
EnvironmentalEnvironmental GeneticGenetic
Environmental EtiologyEnvironmental Etiology
precise origin unknown in the majority of precise origin unknown in the majority of casescases
Environmental EtiologyEnvironmental Etiology
precise origin unknownprecise origin unknown Speculate Vascular causes particularly some Speculate Vascular causes particularly some
kind of Thromboembolismkind of Thromboembolism
Environmental EtiologyEnvironmental Etiology
precise origin unknownprecise origin unknown Speculate Vascular/ThromboembolismSpeculate Vascular/Thromboembolism Mechanical: Amniotic Bands or Streeter’s Mechanical: Amniotic Bands or Streeter’s
dysplasia in which multiple limbs are dysplasia in which multiple limbs are involvedinvolved
Environmental EtiologyEnvironmental Etiology
precise origin unknownprecise origin unknown Speculate Vascular/ThromboembolismSpeculate Vascular/Thromboembolism Mechanical: Amniotic BandsMechanical: Amniotic Bands Maternal: Maternal:
diabetes mellitusdiabetes mellitus intrauterine infectionintrauterine infection
Environmental EtiologyEnvironmental Etiology
precise origin unknownprecise origin unknown Speculate Vascular/ThromboembolismSpeculate Vascular/Thromboembolism Mechanical: Amniotic BandsMechanical: Amniotic Bands Maternal causesMaternal causes Pharmaceutical:Pharmaceutical:
Thalidomide only proven drugThalidomide only proven drug Others suspected, no convincing evidenceOthers suspected, no convincing evidence
Genetic EtiologyGenetic Etiology
Chromosomal: ex. Chromosomal: ex. Turner’s Turner’s syndrome XXXsyndrome XXX
Genetic EtiologyGenetic Etiology
Single GeneSingle Gene Autosomal Autosomal
Dominant: Dominant: Longitudinal tibial Longitudinal tibial deficiencydeficiency
Genetic EtiologyGenetic Etiology
Single GeneSingle Gene Autosomal Autosomal
DominantDominant Autosomal Autosomal
Recessive: TARRecessive: TAR TThrombocytopenia hrombocytopenia AAbsent bsent RRadiusadius
Etiology ofAcquired DeficienciesEtiology ofAcquired DeficienciesTrauma 67%Trauma 67%
especially trains and especially trains and lawn mowerslawn mowers
Etiology ofAcquired DeficienciesEtiology ofAcquired Deficiencies DiseaseDisease 33%33%
majority caused by majority caused by malignancy, malignancy, particularly 12-21 particularly 12-21 years of ageyears of age
Etiology ofAcquired DeficienciesEtiology ofAcquired DeficienciesSurgical conversionSurgical conversion
for congenital limb for congenital limb deficiencydeficiency
Classification ofCongenital Limb DeficiencyClassification ofCongenital Limb Deficiency
International Organization for International Organization for Standardization (ISO)Standardization (ISO)
restricted to restricted to skeletal radiologicalskeletal radiological deficiency deficiency
ISO Classification ofCongenital Limb DeficiencyISO Classification ofCongenital Limb Deficiency
Transverse deficiency:Transverse deficiency:
no skeletal elements present distallyno skeletal elements present distally Name the level of the portion of the limb Name the level of the portion of the limb
involved (Upper Arm)involved (Upper Arm) State the portion where the absence occurs State the portion where the absence occurs
(“middle third” or “total”)(“middle third” or “total”)
ISO Classification ofCongenital Limb DeficiencyISO Classification ofCongenital Limb DeficiencyLeft Transverse Left Transverse
deficiency:deficiency: ForearmForearm middle thirdmiddle third
ISO Classification ofCongenital Limb DeficiencyISO Classification ofCongenital Limb Deficiency
Longitudinal deficiency:Longitudinal deficiency:skeletal elements present axially or distallyskeletal elements present axially or distally Name the bones involvedName the bones involved State partial or total absenceState partial or total absence
ISO Classification ofCongenital Limb DeficiencyISO Classification ofCongenital Limb DeficiencyRight Longitudinal Right Longitudinal
deficiency:deficiency: Fibula totalFibula total Tarsals partialTarsals partial Rays 3,4,5 totalRays 3,4,5 total
Frantz and O’Rahilly Classification Congenital Limb DeficiencyFrantz and O’Rahilly Classification Congenital Limb Deficiency
terminal or intercalaryterminal or intercalary transverse or paraxialtransverse or paraxial complete or incompletecomplete or incomplete additional termsadditional terms
amelia total absence of the limb involvedamelia total absence of the limb involved hemimelia partial absence of the limb involvedhemimelia partial absence of the limb involved phocomelia absence of the long bonesphocomelia absence of the long bones
Classification ofAcquired Limb DeficiencyClassification ofAcquired Limb DeficiencyThrough long bonesThrough long bones Upper ExtremityUpper Extremity
Transradial = Below Elbow (BE)Transradial = Below Elbow (BE) Transhumeral = Above Elbow (AE)Transhumeral = Above Elbow (AE)
Lower ExtremityLower Extremity Transtibial = Below Knee (BK)Transtibial = Below Knee (BK) Transfemoral = Above Knee (AK)Transfemoral = Above Knee (AK)
Limb Salvage and Turnplasty (Van Ness)Limb Salvage and Turnplasty (Van Ness)
Classification ofAcquired Limb DeficiencyClassification ofAcquired Limb DeficiencyThrough the joint:Through the joint:
Name the joint + “Disarticulation”Name the joint + “Disarticulation” Upper Extremity: Wrist Disarticulation, Upper Extremity: Wrist Disarticulation,
etc.etc. Lower Extremity: Ankle Disarticulation, Lower Extremity: Ankle Disarticulation,
etc.etc.
Clinical PrinciplesTreatment goalsClinical PrinciplesTreatment goals
Healthy body Healthy body imageimage
Maintain choice for Maintain choice for prosthetic optionsprosthetic options
Optimal functionOptimal function
Clinical PrinciplesGeneral considerationsClinical PrinciplesGeneral considerations
Team approachTeam approach Developmental focusDevelopmental focus Return appointmentsReturn appointments
3-4 months to eval prosthetic fit & function3-4 months to eval prosthetic fit & function Annually for team to assess developmental Annually for team to assess developmental
needs needs
Clinical PrinciplesPsychosocial SupportClinical PrinciplesPsychosocial Support Clients need to meet others with similar Clients need to meet others with similar
presentationspresentations Guilt and associated familial problemsGuilt and associated familial problems Give child control and decision making Give child control and decision making
opportunitiesopportunities Genetic counseling should be provided to Genetic counseling should be provided to
both the child and parents both the child and parents
Clinical PrinciplesSurgical PlanningClinical PrinciplesSurgical Planning Timing for Timing for
congenital congenital conversionsconversions
Clinical PrinciplesSurgical PlanningClinical PrinciplesSurgical Planning TimingTiming Growth plate Growth plate
considerationsconsiderations
Clinical PrinciplesSurgical PlanningClinical PrinciplesSurgical Planning TimingTiming Growth plate Growth plate
considerationsconsiderations Overgrowth with Overgrowth with
long bone long bone transectionstransections
Clinical PrinciplesSurgical PlanningClinical PrinciplesSurgical Planning TimingTiming Growth plate Growth plate
considerationsconsiderations OvergrowthOvergrowth Planning for Planning for
multiple surgical multiple surgical proceduresprocedures
Clinical Principles OT and PTClinical Principles OT and PT
When infants, we must train parents When infants, we must train parents and caregiversand caregivers
Children need minimal “training” Children need minimal “training” instead need opportunityinstead need opportunity
Clinical Principles Prosthetic designsClinical Principles Prosthetic designs Endoskeletal vs. exoskeletalEndoskeletal vs. exoskeletal Flexible vs. rigidFlexible vs. rigid Growth adjustable designsGrowth adjustable designs Socks when applicable can allow for Socks when applicable can allow for
growthgrowth For unilateral deficiencies, legs are used, For unilateral deficiencies, legs are used,
but arms often rejectedbut arms often rejected RecreationRecreation
Clinical PrinciplesLE Prosthetic ConsiderationsClinical PrinciplesLE Prosthetic ConsiderationsWearing guidelinesWearing guidelines Fit when pull the Fit when pull the
stand and cruising stand and cruising (9-12 months)(9-12 months)
Clinical PrinciplesLE Prosthetic ConsiderationsClinical PrinciplesLE Prosthetic ConsiderationsFoot/AnkleFoot/Ankle Toddler gaitToddler gait
Lacks heel strikeLacks heel strike Wide base of supportWide base of support
Clinical PrinciplesLE Prosthetic ConsiderationsClinical PrinciplesLE Prosthetic ConsiderationsChild’s gaitChild’s gait more normal gaitmore normal gait benefit from benefit from
dynamic foot/ankledynamic foot/ankle
Clinical PrinciplesLE Prosthetic ConsiderationsClinical PrinciplesLE Prosthetic ConsiderationsKneeKnee
Toddler has Toddler has fixed/locked kneefixed/locked knee
Some centers Some centers experimenting with experimenting with a free kneea free knee
Clinical PrinciplesLE Prosthetic ConsiderationsClinical PrinciplesLE Prosthetic ConsiderationsKneeKnee
Toddler has Toddler has fixed/locked kneefixed/locked knee
At 3 y.o. temporary At 3 y.o. temporary reduction of Knee reduction of Knee ROM while learningROM while learning
Clinical PrinciplesLE Prosthetic ConsiderationsClinical PrinciplesLE Prosthetic ConsiderationsHip Hip uses alignment uses alignment
stabilitystability
Clinical PrinciplesUE Prosthetic ConsiderationsClinical PrinciplesUE Prosthetic ConsiderationsWearing guidelinesWearing guidelines 3 mos for supine grasp3 mos for supine grasp ““Fit when sit”Fit when sit” Best before 12 mos. Best before 12 mos. Common periods for Common periods for
rejectionrejection Unilaterals functional Unilaterals functional
without prosthesis, but without prosthesis, but more receptive learners more receptive learners than adultsthan adults
Clinical PrinciplesUE Prosthetic ConsiderationsClinical PrinciplesUE Prosthetic ConsiderationsGraspGrasp Passive as an infant Passive as an infant
for gross graspfor gross grasp
Clinical PrinciplesUE Prosthetic ConsiderationsClinical PrinciplesUE Prosthetic ConsiderationsGraspGrasp Active when Active when
developmentally developmentally “ready” and able to “ready” and able to “understand” grasping “understand” grasping function (18-24 function (18-24 months)months)
Clinical PrinciplesUE Prosthetic ConsiderationsClinical PrinciplesUE Prosthetic ConsiderationsGraspGrasp Electric switch control Electric switch control
can provide active can provide active control at an earlier control at an earlier ageage
Clinical PrinciplesUE Prosthetic ConsiderationsClinical PrinciplesUE Prosthetic ConsiderationsElbowElbow Fixed for sitting Fixed for sitting
balance as an infantbalance as an infant
Clinical PrinciplesUE Prosthetic ConsiderationsClinical PrinciplesUE Prosthetic ConsiderationsElbowElbow Fixed as an infantFixed as an infant Passive friction for Passive friction for
toddlertoddler Active locking at 3 Active locking at 3
years oldyears old
Clinical PrinciplesUE Prosthetic ConsiderationsClinical PrinciplesUE Prosthetic ConsiderationsHigh level High level
prosthetic function prosthetic function poor substitute poor substitute
and often rejectedand often rejected
Special Case DiscussionSpecial Case Discussion
Proximal Femoral Proximal Femoral Focal Deficiency Focal Deficiency (PFFD)(PFFD)
Aitken A or BAitken A or B
Special Case DiscussionSpecial Case Discussion
Proximal Femoral Proximal Femoral Focal Deficiency Focal Deficiency (PFFD)(PFFD)
Aitken A or BAitken A or B Aitken C or DAitken C or D
Special Case DiscussionSpecial Case Discussion
Longitudinal fibular Longitudinal fibular deficiencydeficiency
Normal foot Normal foot with stable ankle, with stable ankle, centralization surgerycentralization surgery AFOAFO Shoe Lifts/LengtheningShoe Lifts/Lengthening
Special Case DiscussionSpecial Case Discussion
Longitudinal fibular Longitudinal fibular deficiencydeficiency
Abnormal foot Abnormal foot Ankle disarticulation Ankle disarticulation
conversion conversion for a Prosthesisfor a Prosthesis
Special Case DiscussionSpecial Case Discussion
Septicemia/Purpura Septicemia/Purpura FulminansFulminans
Skin managementSkin management OvergrowthOvergrowth
Radial DeficiencyRadial Deficiency Associated pathologiesAssociated pathologies geneticsgenetics
Special Case DiscussionSpecial Case Discussion
Tibial DeficiencyTibial Deficiency Surgical conversionSurgical conversion geneticsgenetics
Special Case DiscussionSpecial Case Discussion
Bilateral upper Bilateral upper extremityextremity
Special Case DiscussionSpecial Case Discussion
Bilateral upper Bilateral upper extremityextremity
Bilateral lower Bilateral lower extremityextremity
Special Case DiscussionSpecial Case Discussion
Special Case DiscussionSpecial Case Discussion
High Level High Level Quadramembral Quadramembral DeficienciesDeficiencies
FUNCTIONFUNCTION