NEUROMUSCULARSCOLIOSISAHANDBOOKFORPATIENTSANDPARENTS

IntroductionNeuromuscularscoliosisisaconditionthataffectschildrenwithneuromusculardisordersandis

characterizedbythepresenceofoneormoreabnormalcurvaturesofthespine.Thiscanbecausedin

childrenbyverylowmuscletone(hypotonia)orinchildrenwithaveryhighmuscletone(spasticity).It

alsooccursinchildrenwithneurologicalconditionssuchasmusculardystrophyorspinalmuscular

atrophy(SMA).Becausethereissuchawidevarietyofthetypesofdiseasesthatmaycause

neuromuscularscoliosis,theclinicalpresentationandseverityofthisconditionisextremelyvariable.

Mostchildrenwithneuromusculardiseasehavepoorbalanceandpoorcoordinationoftheirtrunk,neck

andhead.Asthechildrenwithaneuromuscularconditiongrowandtheirtrunkmusclesgetweaker,

thespineprogressivelycollapses,producingalongCshapedscoliosis.Thecurvescanalsoprogress

duringgrowthspurts.Forchildrenwhoprimarilyusewheelchairs,severecurvescanaffectthechild’s

abilitytositcomfortably,affectingtheirqualityoflifeandfunction.Childrenwithverylargecurvescan

developlungdysfunction,whichcanleadtorecurrentpneumonias(chestinfections).

Thebehaviorofneuromuscularscoliosiscanbeunpredictable.Theearlierthecurvedevelops,the

morelikelyitistoprogresstoamoreseverecurve.Neuromuscularscoliosisisoftenassociatedwitha

longcurvethatextendstothepelvis,whichcausesaconditionknownaspelvicobliquity,inwhichthe

child’spelvisisunevenlytiltedwithonesidepositionedhigherthantheotherside.Thismayresultis

difficultysitting,hipproblems(subluxationordislocation)orpressuresoresinsomecases.

A)ClinicalphotographofapatientwithcerebralPalsyB)ClinicalphotographofapatientwithSMA

C)LongCshapedcurvecharacteristictoneuromuscularscoliosis

Classification

Neuromuscularspinaldeformitiesareusuallyclassifiedintotwocategoriesbasedonthetypeofdisease

thatthechildsuffersfrom:

A.Neuropathicscoliosis:Thisinvolvesdiseasesthatareprimarilyafflictionsofthenervoussystem.B.

Myopathicscoliosisinvolvesmuscledisorders.Manydifferentneuromuscularconditionscanleadto

neuromuscularscoliosis.Theyinclude:

1. Cerebralpalsy.

2. Spinabifida(myelomeningocele).

3. Musculardystrophy

4. Spinalmuscleatrophy

5. Mitochondrialdisorders

6. Freidreichataxia

7. Traumaticspinalcordinjury.

8. Otherconditions

TreatmentofNeuromuscularSpinalDeformity

Decisionsregardingtheappropriatetreatmentforneuromuscularscoliosiscanbedifficult,anddepend

ontheseverityofthespinalcurvature,theageofthepatient,theunderlyingdiagnosis,othermedical

problems,andthegoalsandwishesofthefamily.

Inneuromuscularscoliosis,curveprogressionislikely,somostpatientsandtheirfamilieswillfacea

choiceregardingsurgicalintervention.Observation,toallowtimetofollowthenaturalhistoryofthe

scoliosis,andtoreassessdecision-making,isavalidtreatmentoption.Insomeinstances,bracing

maybeusedtoimproveseatingbyprovidingtrunksupport,butunfortunately,bracingdoesnotslow

progressionofthistypeofscoliosis.

Surgicaltreatmentcanallowfunctionalimprovements,intermsofeaseofdailyactivitiessuchas

eating,dressing,andbathing.Itmayalsoprovidebettersittingbalance,decreasethetimeneededfor

resting,alleviatediscomfort,improvebreathingandoverallhealthstatus,andpreventworsening.

Non-SurgicalTreatment

Observation

Observationisavalidoptionforpatientswhenthenaturalhistoryofthescoliosisisnot

clear,whenmoretimemaybeneededtomakedecisions,andwhentherisksofsurgery

mayoutweightheperceivedbenefits.Observationrequiresnoextracareorexpense

comparedtothepatients’currentroutine.

Bracing

Bracingforneuromuscularscoliosismayhelpimprovesittingpositionandprovidetrunk

support.However,therearealsosomerisks.Bracesdonotpermanentlystraightenthese

curves,orpreventthemfromprogressing.Somebracesmayexacerbatepre-existing

pulmonarydiseaseiftheyaretoorestrictive,andinhibitbreathing.Softermaterialsmaybe

usedforbracesforneuromuscularpatientstoavoidtheseadverseeffects.

WheelchairModification

Wheelchairmodificationsmaybeusedtoaidseatingpositionandcomfort.Custom-

molded,paddedseatsmayadapttopatients’bodies,preventpressureulcers,andallow

improveduprightposture.Theysharesomeofthebenefitsofbracing,butsparethetime

anddifficultyneededtoputbracesonchildren.Theyalsosharesomeofthesamerisksas

braces,inthattheydonotpermanentlystraightenscoliosis,orpreventitsprogression.

AB

A:TLSOsuitableinsomeparalyticcurves

B:Moldedseatinsertsuitabletospastictypeofneuromuscularscoliosis

TheRoleofSurgicalTreatment

Improvementsinsurgicaltechniques,intensivecare,neurologic,cardiac,andanesthetictechniquesnow

allowsurgicalsolutionsforneuromuscularscoliosis,evenforfragilepatients.However,the

complicationratesforthesesurgeriesremainsignificant,andsoassessingtheexpectedrisksand

benefitsofsurgeryforeachindividualpatientisimportant...Manychildrenwithneuromuscularscoliosis

haveotherunderlyingmedicalconditions.Itiscrucialtounderstandspecificgoalsforthesurgery,

whichcanincludebettersittingtolerance,bettersittingbalance,betterlungfunction,lessdiscomfort,

betteroverallhealth,andthepreventionofworsening.Risksfromsurgeryforneuromuscularscoliosis

includeinfection,implantlooseningorbreakage,progressionofacurvedespitesurgery,backpain,

pneumonias,gastrointestinalcomplications,urinarytractinfection,spinalcordinjury,ordeath.Because

thecomplicationsaresubstantial,andsomecomplicationoccursinabout1outofevery4children,the

decisionmakingforsurgeryiscomplex.Thesurgeonandthepatient’sfamilymustworkcloselytogether

toensurethecorrectdecisionismadeforeachpatient.

TimingofSurgery

Whenpossible,mostsurgeonsprefertodelayspinefusionsurgeryuntilthespineandlungsaremostly

grown.Fusingthespinepreventsfurthergrowthanddevelopmentofthechest,whichisnotdesirablein

veryyoungpatients.Fusingonlythebackofthespineinyoungpatientsmayallowthefrontofthespine

tocontinuetogrow,allowingthedeformitytoworsendespitethesurgery.However,somedeformities

aredifficulttocontrolconservatively,andtherefore,earlysurgerymaybejustified.Insomepoorly

controlleddeformities,rodsthatallowgrowthmaybeuseful.Theserodsareattachedonlytopartsof

thespine;withconnectorsthatallowtherodstobelengthenedasthespinegrow.Iflengtheningsare

needed,theycanbedoneduringsmallersurgeriesevery6months,orbyusinganexternalmagnetic

device,dependingonthesituation.Apatientwithneuromuscularscoliosismustbetreatedwithateam

approach.Itisveryimportanttotreattheentirepatient.Closecollaborationwiththerapists,primary

careproviders,physiatrists,orthotists,nurses,pulmonologists,anesthesiologists,neurologists,and

surgeonsisessential.

Pre-operativeAssessment

Themostimportantpartofthedecisionmakingprocessisthepreoperativeassessment.Thesurgeon

willwanttodiscussthecasewiththeotherphysiciansinvolvedinyourchild’scare.Consultationwith

severaldifferenttypesofphysiciansmayberequiredtoensurethatthechildishealthyenoughtosafely

undergotheprocedure.Thesephysiciansmayincludeneurologists,cardiologists,pulmonologists,

nutritionists,gastroenterologists,neurosurgeons,anesthesiologists,orothers.Theotherphysiciansmay

helpassessthechild‘ssurgicalrisk,andmayhelpcareforthechildbeforeandaftersurgery..Other

proceduresmayneedtobedonepriortospinesurgery,suchasplacingaG-tubefornutrition,ora

tracheostomyforpulmonarycare,inordertominimizetherisksoftheprocedure.

RisksRelatedtoSurgery

Specificrisksrelatedtospinefusionforneuromuscularscoliosisincludethefollowing:

Infection:Infectionratesaftersurgeryforneuromuscularscoliosisarehigherthanforothertypesof

scoliosissurgeries,rangingfrom4toashighas25outof100children.Halfarethesearesuperficial,and

canbetreatedwithantibiotics.Halfaredeeper,andrequirerepeatsurgerytocleantheinfection,and

possiblyimplantremoval.

BloodLoss:Bleedingcanoftenbesignificant,andplansforbloodmanagementshouldbeestablished

preoperatively.Optionsmayincludeusingadevicecalleda“cellsaver”thatprocessesapatient’slost

bloodsoitcanbereturnedtothebody,orhavingunitsofbloodavailabletobetransfused.

PulmonaryDysfunction:Theneedtohaveabreathingtubeforrespiratorysupportmaylastlongerthan

expected.Lungcongestionorpneumoniasmayalsodevelop.

SpinalCordInjury:Thereisasmallriskofinjurytospinalnervesduringthesurgerywhichmayleadto

postoperativeweakness,numbness,ordysfunctionofthebowelsandbladder..Forthisreason,many

surgeonsmonitorthespinalcordduringtheoperation,whenpossible.

ImplantFailure:Rarely,thesurgicalimplantsmaydislodge,orloosen,andrequirearevisionprocedure.

GastrointestinalIssues:Slowingofbowelfunction,calledconstipationoranileus,mayoccurduetothe

stressofsurgery,theuseofpainmedications,orprolongedimmobilization.Pancreatitisdevelopsin

somepatientsaftersurgery,andmayrequirealongerperiodofnoteatingaftersurgerytoresolve.

UrinaryTractInfection:About5outof100patientswillhaveaurinarytractinfectionaftersurgery.

Thesecantypicallybetreatedandresolvedwithantibiotics.

Death:Theriskofdeathwithin30daysfollowingneuromuscularscoliosissurgeryintheUnitedStatesis

lessthan2outof100children.Overall,about1outof4patientsundergoingneuromuscularscoliosis

surgerywillexperienceatleastonecomplication.Itisimperativethatfamilieshaveafrankdiscussion

withthetreatingsurgeonsothattheycanunderstandtherisksthatpertaintotheirchildandmakean

informeddecisionregardingsurgicaltreatment.Mostcomplicationsareminorormoderateandcanbe

managedfairlyeasily.

SurgicalTechniques

Thesurgicaltreatmentforneuromuscularscoliosishasevolvedovertime,andisindividualizedforeach

patient.Foryoungpatients,growth-friendlysurgerycanbeperformed,asdiscussedintheprevious

section.Formorematurepatients,aspinalfusionisrequired.Aspinalfusioninvolvesmakingan

incisioninthepatient’sback,andplacinghooks,wires,orscrewsinthebonesasanchors.Metalrods

arethenattachedtotheanchors,andareusedtostraightenthespineasmuchaspossible.Bonegraft

isthenplacedoverthespinetoallowittofuse.Thespinethenbecomesonesolidunitthatprevents

collapseorfurthercurving.Insomecases,thespineissostiffthatthesurgeonwillperformasurgery

fromthefrontofthespineaswell.Thisallowsremovalofthediscsandligamentsbetweenthebonesto

makethecurvemoreflexible.Thefrontandthebackpartsoftheprocedurecanbedoneonseparate

days,oronthesamedayinsomecases.Tractionpriortothespinefusion,duringthespinefusion,or

boneremovaltechniquescalledosteotomiesmaybehelpfulincaseswithespeciallylarge,stiffcurves.

AB

A.Pre-opx-rayofapatientwithseverescoliosis.

B.Post-opx-raywithsatisfactorycorrectionofdeformitywithscrewsandrods.

Similarpatientwithseverescoliosis,pre-operativex-rays.

Post-opx-raysoftheabovepatientafterinstrumentationwithrodsandwires.

Pre-opphotoofapatientwithmusculardystrophy.

Post-opphotoofthesamepatientabout2weeksaftersurgery

Conclusion

Scoliosisisacommonconditionseeninpatientswithneuromusculardisease.Thedecisiontotreatthe

curvatureiscomplex,andmusttakeintoaccountthepatient’sunderlyingfunction,theneedsofthe

patientandfamily,andtherisksspecifictothatpatient.Byemployingateamapproach,toinclude

physicians,othermedicalcaregivers,andthepatient’sfamily,itispossibletoindividualizetreatmentto

providethebestpossibleoutcomeforthepatient.