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PediatricDeformity– EvaluationandNon-operativeManagement

GregoryMMundisJr.,MDScrippsClinicMedicalGroupSanDiegoSpineFoundation

Outline• Scoliosis

•Definitionsandreview• Types:

• Congenital•Neuromuscular• AIS

• Atypicalcurves• Emergingtechnologies

• 3-Dimaging•Magneticrods• Anteriorvertebralbodytethering

Scoliosis• Scoliosisisdefinedasalateralcurvatureofthespine>10°

• Cobbangleendtoendvertebraemeasurement

• Typesofscoliosis• Idiopathic• Congenital• Neuromuscular• Syndromic

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ClassificationsoftheScoliosis• Congenital(5%)• Neuromuscular(20%)• Idiopathic(65%)• Syndromic(10%)

Weinsteinetal,2004

CongenitalScoliosis• Abnormaldevelopmentofthespineduringpregnancy

• Ifonepartisabnormal,otherpartsmayalsobeabnormal

• Kidneys• Heart• Spinalcord

McMasteretal,2001

EmbryologyofCongenitalScoliosis/Kyphosis

• Failureofsegmentation

• Failureofformation

• Mixedmalformations

WeinsteinSL,ed.ThePediatricSpine,2nded.,2001,LippincottWilliams&Wilkins.

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NaturalHistoryofCongenitalScoliosisMcMaster&Ohtsuka,1982

• 251patients• Greatestprogressionafter10yearsofage(pre-pubescentgrowthspurt)

• Dependentonvertebralanomaly• TL>Tworse• 143nottreatedafter10yearsofage

• 51(36%)curve40-60º• 40(28%)curve>60º

McMasterMJ,Ohtsuka K:Thenaturalhistoryofcongenitalscoliosis.JBJS64-A;8:1128-47,1982.

EvaluatingtheExtentofClinicalUncertaintyinEarlyOnsetScoliosisVitaleetal,JBJS2013

• 315patientcasescenarios• 8treatmentoptions

•Observation,casting,bracing,growthguidance,growthmodulation,distractionbased(spineorrib),fusion

• 11surgeons(averageexperienceof22years)selectedtreatment

EvaluatingtheExtentofClinicalUncertaintyinEarlyOnsetScoliosisVitaleetal,JBJS2013

• CollectiveEquipoiseforoverone-thirdofclinicalscenarios

• Coronaldeformityandpatientageamongmostimportantpatientvariablesinfluencingtreatment

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Indications•Magnitudeofcurve

• Rapidlyprogressivecurve

• Locationandtypeofanomaly

54°

Age4 Age5

70º

PulmonaryFunctionFollowingEarlyThoracicFusioninNon-NeuromuscularScoliosisKaroletal,JBJS2008

• 28patients•Meanageatsurgery3years•Minimum5yearf/u• Proximalthoracicandfusion>4levelsathighestriskfordevelopingrestrictivelungdisease

PatientCL• UnderwentT2/3toL1VEPTRwithexpansionthoracoplasty

• Didwellpostoperatively• Age7

• 6lengthenings• Nomorelengthobtained

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Complications:GrowingSpineStudyGroup(2010)

• N=140,1987– 2005• 140implantations,633lengthenings,and50definitivefusions• 177complicationsin81patients(58%)

• 59patientswithoutcomplications(42%)• Patientswithdualrodshadmorelengtheningsandgreatercorrectionandwereyounger

• Woundinfectionsweremorecommonindualrodsandimplantfailuremorecommoninsinglerods

• Nodifferenceinsinglerodswithsubmuscularvs.subcutaneous• Butmorecomplicationswithsubcutaneousdualrods

Bessetal.Complicationsofgrowing-rodtreatmentforearly-onsetscoliosis.JBJS92:2533-43,2010

Whatifnogrowingsystem?

• Presentedatage8withprogressive50ºcurve

• Recommendedgrowingsystem• Losttofollow-up

Whatifnogrowingsystem?

6yearslater

Post-op Post-op

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PatientDP

• Initially• Importanceofgrowingsystems

• Increaseinsurgicalrisk• Later

• Savedmultiplesurgeries

CaseIllustration

• 14yoboywithspinalcordinjurysinceage4

• Curveprogressedto90°• Admitted

• Halotractionfor6weekscurvedecreasedto55°

• G-tube• Weightincreasedby15

CaseIllustration• T2topelvisPSF• Intraoperativehalofemoraltraction;osteotomiesT3toT12

• EBL3.8L(noAmicar)• Fluids

• 14Lofcrystalloid• 6unitsofpRBC’s

• Operatingtime8hours• Lungsoverloadedandremainedintubated/sedated

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VisionLossAfterSpineSurgeryBaig etal:Visionlossafterspinesurgery:reviewoftheliteratureandrecommendations.NeurosurgFocus2007

• 0.1%• Corticalblindness• Centralretinalarteryocclusion• Ischemicneuropathy

VisionLossAfterSpineSurgeryPatil etal:Visionlossafterspinesurgery.Spine2008

• Avoiddirectpressureonglobe• Avoidperioperativehypotension• Avoidperioperativeanemia• Consider10ºofreverseTrendelenbergduringpronesurgery• Lowertransfusionthresholdtokeephematocritabove30inhighriskpatients

• Avoidinfusionsoflargeamountsofcrystalloid• Considerstaginglongspinalsurgeries(>8hours)• Maintainmeanarterialpressureatpatient’sbaseline• Performapostoperativevisualexamasearlyaspossibleinhighriskpatients

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NeuromuscularScoliosis

SRSClassificationPrimaryneuropathies

UppermotorneuronCerebralpalsyCharcot-Marie-ToothSyringomyeliaSpinalcordtumorSpinalcordtrauma

LowermotorneuronPoliomyelitisTraumaticSMAWerdnig-HoffmannRiley-Daysyndrome

Combinedupper/lowerMyelomeningocele

PrimarymyopathiesMusculardystrophyDuchenne’smusculardystrophyLimb-girdledystrophyFacioscapulohumeraldystrophyArthrogryposisFiber-typedisproportionCongenitalhypotoniaMyotonia dystrophica

IncidenceofScoliosis

lCerebralpalsy:20-70%lQuadriplegia>diplegia

lSpinalcordinjury:40-100%lSkeletallyimmature>mature

lComplete>incompletelMyelomeningocele:25-90%

lIncreasesaslevelascends

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ProblemswithSpineDeformity

• Pelvicobliquity• PoorsittingabilitycausingreducedUEfunction

• Pressuresores• Pain• PoorLEorthoticfittinganduse• Gastrointestinaldysfunction• Cardiopulmonarydysfunction

BracinglPotentialbenefits

lMayprovidesittingsupport

lSkeletallyimmaturepatientsmayholdcurve

lNogooddatainCPlSpinalcordinjury

lMehtaetal JSpinalCordMed2004

lMaydelay/preventneedforsurgery

IndicationsforOperation

lVeryvariablelCurves>50ºinagrowingchild

lSkeletallymaturepatients:lFunctionalproblemslCurveprogression

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ResultsSmall Curves

<70°Large Curves

>70°P Value

Mean age (years) 12.5 ± 2 14.7 ± 2 0.001

Major curve 54 ± 12° 85 ± 11° 0.000

Operative time (minutes) 463 ± 87 536 ± 122 0.016

Blood loss (cc) 2673 ± 1437 3524 ± 2199 0.075

Mean hospital stay (days)

10.9 ± 4 14.9 ± 9 0.048

Major perioperativecomplication

21% 36%

Samdanietal,IMAST2011

Complications• Smallcurves

• 21%(4/19)•Duraltear• Pneumonia• Prolongedintubation

•Woundinfection

Complications• Largercurves

• 36%(9/26)• Woundinfections(3)• Aspiration(2)• Prolongedintubation(2)• Duraltear• Unplannedstagedsurgery(excessivebloodloss)

• Sepsis• ARDS• Visionloss

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AdolescentIdiopathicScoliosis

Prevalence• >10° 2to3%(F:M5:1)• >20° 0.3to0.5%• >30° 0.2to0.3%(F:M10:1)

LonsteinJE:Surgicalversusnonsurgicaltreatment.ClinOrthop443:248,2006

ProposedEtiologies• Remainsunknown• Genetic

• Inheritancepatternunknown• 27%ofdaughtersofwomenwithscoliosisdevelopedscoliosis

• Monozygotictwinsdisplayhighrateofconcordance(upto70%)

Riseborough EJ,Wynne-DaviesR:AgeneticsurveyofidiopathicscoliosisinBoston,MA.JBJS55A:974,1973

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GeneticTestingWardK,NelsonLM,etal:SRS2008

• Salivatest• 30genemarker• “…wecannowpredictwhichpatientsarelikelytoprogresstoaseverescoliosisatthetimeofinitialclinicalevaluation.”

PhysicalExamination

Paininonly20%Backexam

• Shoulderheights• R>L• Even• L>R

• Ribprominence(Adam’sforwardbend)

• Lumbarprominence• Trunkshift

Lenke Classification:AdvancedCareofPatientswithAIS

Lenkeetal,JBJS83A:1169,2001

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WhatisanatypicalcurvepatterninAIS?

lLeftsidedthoraciccurvelKyphosis>40ºlOther:

lRapidprogressionlDouble/triplemajorcurves

lLongcurves

Spiegeletal,Spine2003Wangetal,Spine2010

IndicationsforMRIinPresumedAISDavids etal,JBJS2004

• 1280patients•Defined‘atypical’as:

• Leftthoraciccurve• Lossofapicallordosis• Lackofvertebralrotation• Rapidprogression

• Incidenceofanatypicalcurve:4.5%(58/1280)

AdolescentIdiopathicScoliosis

• Whyareweconcernedaboutit?• Appearance• Backpain• Breathing

• Heartfunction• Worseningofthecurve

• ADULTissues

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AxialPlane:ClinicalRelevance

• Ribdeformityismajorcomponentofspinedeformity

Geissele AEetal:Thoracoplastyforthetreatmentofribprominenceinthoracicscoliosis.Spine19:1636-42,1994Theologis TNetal:Quantifyingthecosmeticdefectofadolescentidiopathicscoliosis.Spine18:909-12, 1993Thulbourne T,GillespieR:Theribhumpinidiopathicscoliosis.Measurement,analysisandresponsetotreatment.JBJS58B:64–71,1976

NaturalHistory:PercentageofCurvesthatProgress

Lonstein JE, Carlson JM. The prediction of curve progression in untreated idiopathic scoliosis during growth. J Bone Joint Surg Am. 1984;66:1061–71.

Risser 5-19º 20-29º

0-1 22% 68%

2-4 1.6% 23%

OutcomesintoAdulthood

• Curves<30ºatskeletalmaturityareunlikelytoprogress• Thoraciccurves>50ºwillprogress1º/year• Backpainlikelynodifferencefromgeneralpopulation

Weinstein SL et al. Idiopathic scoliosis: long-term follow-up and prognosis in untreated patients. J Bone Joint Surg. 1981;63:702–12.

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OutcomesintoAdulthood

• Pulmonaryfunctiondecreasesat60ºandmorepronouncedat100º

• Pregnancylikelynoeffect• Betzetal:355patientswithscoliosis

•Nodifferenceincurveprogression

Betz RR et al: Scoliosis and pregnancy. J Bone Joint Surg Am. 1987;69:90–6.

BracesworkforpatientswhowearthemBRAISTClinicalTrial,2013

75%successforbracetreatmentcomparedto42%whowerenottreated