neuromuscular scoliosis a handbook for patients … · 2016-08-18 · neuromuscular scoliosis a...
TRANSCRIPT
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.