melorheostosis: a multi-center case seriesllrs.org/llrs 2016 pdfs/melorheostosis-a multi-center case...
TRANSCRIPT
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Melorheostosis:
AMulti-centerCaseSeries
MarcelWileyMD,DavidA.PodeszwaMD,DmitryPopkovMD,MikhailSamchukovMD,
AlexCherkashinMD,JohnG.BirchMD,
LLRSAnnualMeetingJuly22,2016Charleston,S.C.
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Disclosures
Theauthorshavenorelevantdisclosures
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Melorheostosis
• Raremesenchymalbonedysplasia(associatedwithLEMD3genemutations)
• Typicallypresentsinchildhood
• Softtissuescarring/contracturestypicalpresentingproblem
• Radiographs• Rlowinghyperostosis• “drippingcandlewax”• followsasclerotomaldistribution
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Melorheostosis
• Raremesenchymalbonedysplasia(associatedwithLEMD3genemutations)
• Typicallypresentsinchildhood
• Softtissuescarring/contracturestypicalpresentingproblem
• Radiographs• Rlowinghyperostosis• “drippingcandlewax”• followsasclerotomaldistribution
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Melorheostosis
• Onlysinglecasesreportedinlast30years
MarshallandBradishJBJS(Br)1993;Choietal.CORR2003;Gagliardietal.JFootAnkleSurg2010;Aranovichetal.EurOrthopTruamatol2013
• Purpose:Identifythepresentingcharacteristics,treatmentandcomplicationsofacohortofpatientswithmelorheostosisfromtwoinstitutions
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Methods
• IRBapproved,retrospectivecaseseries(1990–2014)
• TSRHandIlizarovScientiRicCenter(Kurgan,Russia)
• AlldiagnosesconRirmedradiographically
• Data• Patientdemographics• Clinicalmanifestations• Treatments• RadiographicRindings
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Results
• 11pts(6male,5female)
• Avgageatdiagnosis:8.7y.(2mon–15.8y.)
• Avgfollow-up:42.2mon.
• ExtremityInvolvement• UnilateralUE:n=2• UnilateralLE:n=9
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Results–AssociatedDeformities
• LowerExtremity• Ankleequinus(2)• Calcaneovalgusfoot(2)• Toedeformities(3)• Associatedhemiatrophy(2)• Concomitantgenuvalgum(5)• ConcomitantLLD(4)
• UpperExtremity• Hand–softtissuechangeswithlimitedRingerROM
• Nointerventionneeded
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Results–LowerExtremity
• ThosewithmultipleboneinvolvementtendedtohavegreaterLEdeformity.
• SurgicalTreatment(5patients)• Excisionalbiopsytibia• OpenTAL(equinuscontracture)• Anklerelease,multipletendonlengthening(calcaneovalgusfoot)• TAL,plantarfasciaandposteriorrelease(x3)(recurrentequinocavus)
• Distalfemorallengthening(4.5cm)withconcomitantgradualcorrectionofkneeRlexioncontractureandequinocavovalgusfoot
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Summary
• Commonpresentation• Lowerlimbdeformity• Genuvalgum• Anklecontractures• PhalangealRlexioncontractures
• Mostmanagednon-operatively
• Skeletalsurveymaybehelpfullyatinitialevaluation
• Complicationsrelatedtowoundhealing/scarringandrecurrenceofdeformity
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CaseExample–
11y.o.male,4.5cmLLD,equinocavovalgusfoot,kneeJlexioncontracture
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Conclusions
• Raredisease–multi-centercollaborationimportantforthesetypesofconditions
• EarlyidentiRicationmayhelpavoidneedforinterventionlater(i.e.bracingorthotics)
• Avoidsofttissueprocedureswhenpossibleduetopotentialsofttissuecomplications
• Lengthening/deformitycorrectionwithexternalRixationmaybeappropriate• Boneformationdidnotseemtobeeffected• Averagehealingindex• Minimizessofttissuemanipulations
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ThankYou!
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ThankYou!