elorheostosisLeridiseaseisararenonhereditaryscle Fig1A46yearoldmanwithneckpainafterminortraumaAPandlateralradiographsshowadenselyscleroticrightparavertebralmassextendingfromtheC5toT1levelC ID: 944140
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CASEREPORTMelorheostosisInvolvingtheCervicalandUpperThoracicSpine:Radiographic,CT,andMRImagingFindingsA.M.MotimayaS.P.MeyersMelorheostosis,anuncommonmesenchymaldysplasia,rarelyaffectstheaxialskeleton.Wedescribetheimagingfindingsofmelorheostosisinvolvingthecervicalandupperthoracicspine.RadiographsandCTshowedunilateralwell-marginatedundulatingzonesofcorticalhyperostosisinvolvingmultiplevertebraethatwerecontiguouswithacoalescentossifiedrightparavertebralmass.MRimagingshowedzonesofsignalintensityvoidonallpulsesequenceswithoutcontrastenhance-ment.Conservativemanagementwaselectedbecauseoflackofintervalclinicalandimagingchangesfor8years. elorheostosis(Leridisease)isararenonhereditaryscle- Fig1.A46-year-oldmanwithneckpainafterminortrauma.AP()andlateral()radiographsshowadenselyscleroticrightparavertebralmassextendingfromtheC5toT1level.Coronal(),rightparasagittal(),andaxial()CTimagesshowundulatingzonesofcorticalhyperostosiswithadrippingcandlewaxappearanceinvolvingtherightsidesoftheC5T1vertebraewithextensionovertherightfacetjointsofC4C5toC7T1.Thehyperostosisinvolvesthevertebralbodies,rightlaminaeandtransverseprocesses,andrightsidesofthespinousprocesses.Theexpansilecorticalhyperostosisextendsmostlyperipherallyalongtheouterbonesurfacebutalsoinvolvestheendostealmarginsresultinginprominentnarrowingoftheinvolvedmarrowspace.PosteriorvertebralbodyosteophytesatC3C4andnarrowingoftherightC5C6foramenfromthehyperostosisarealsoevident.Rightparasagittal()T1-weighted(TR450,TE9),rightparasagittal()T2-weighted(TR1800,TE110),axial)T2-weighted(TR4000,TE100),andaxial()postgadolinium-contrast(Gadoteridol)T1-weighted(TR450,TE9)MRimagesobtained7yearsaftertheradiographsandCTimagesaboveshowzonesofsignalintensityvoidonallpulsesequenceswithnoenhancementcorrespondingtotheareasofhyperostosisseenonradiographsandCTscans.AposteriorspondyloticridgeatC3C4()isseen.Sagittal,midline()T2-weighted(TR,1800;TE,110)MRimageshowstheposteriorspondyloticridgeatC3C4toresultinspinalstenosis,whichislikelysecondarytothefusedspinalcolumnfrommelorheostosisimmediatelybelowthislevel.Nosignalintensityabnormalities,however,areseeninthespinalcordrelatedtothespinalstenosisatC3C4orfromthemelorheostosisinvolvingthelowercervicallevels.CASEREPORTAJNRAmJNeuroradiol27:11981200Jun-Jul2006 Thedifferentialdiagnosisforsuperficialhyperattenuatedvertebralandadjacentparaspinalabnormalitiesincludesmelorheostosis,tumoralcalcinosis,tumoralcalciumpyro-phosphatedihydratedepositiondisease(CPPD),ivoryosteo-mas,heterotopicossification(myositisossificans),andparos-tealandperiostealosteosarcoma.Theunilateralandmultifocalcorticallocations,distinctimagingfeatures,andlackofintervalchangefortheabnormalitiesinthisreportare,however,characteristicformelorheostosisandareidenticaltopriorreportedimagingfindingsforbiopsy-confirmedmelo-rheostosisinvolvinglowerthoracicandlumbarvertebrae.Theimagingfeaturesofthiscasearealsosufficientlydifferentfromtheotherdisordersinthedifferentialdiagnosis.Forex-ample,tumoralcalcinosisofthespine,whichisoftenassoci-atedwit
hsystemicdisordersofcalciummetabolismorrenaldialysis,typicallyoccursashighattenuationparaosseousle-sionsthatresultfromdystrophiccalcificationsinsofttissuescomposedofcalciumhydroxyapatitecrystals,collagenousfi-broussepta,andcollectionsofhistiocytesandforeignbodygiantcells.Unlikemelorheostosis,tumoralcalcinosisofthespineisoftennotunilateralandappearsprimarilyasex-tradurallesionswithheterogeneousmixedsignalintensityonbothT1-weightedandT2-weightedimages,whichareoftenassociatedwitherosionofadjacentbone.CPPDcanoccurastumor-likelesionsinthespine,typicallylocatedattheliga-mentumflavumandsynovialjoints.CPPDlesionscontainneedle-andrhomboid-shapedcalciumpyrophosphatecrys-talswithassociatedchondroidelements,fibrocollageneoustissue,andvariableamountsofacuteandchronicinflamma-toryreaction.CPPDlesionsmaycauseerosionofadjacentTheMRimagingfindingsofspinalCPPDarealsosimilartothosefortumoralcalcinosis,thusdifferingfromthoseformelorheostosis.Althoughivoryosteomascanhavehistologicfeaturessimilartomelorheostosis,largeosteomasofthespineareveryrareandhavebeenreportedtoinvolveonlysinglevertebra.Heterotopicossification,alsoreferredtoasmyositisossificans,typicallyoccursaslesionsinsofttissuewithpredominantperipheraldistributionsofossi-ficationandthusdiffersfromtheappearanceofcortical-basedhyperostosisseenwithmelorheostosis.Theimagingfeaturesofparostealandperiostealosteosarcomasalsodiffermarkedlyfrommelorheostosisbecausethesetumorscontainossificmineralizedmatrixthatisoftenirregularandnotuniformlyattenuatedonradiographsandhavefocalorpoorlydefinedsoft-tissuemasseswithhighsignalintensityonT2-weightedTumorinvasionintomedullarybonewithhighsignalintensityonT2-weightedimagesoccurin41%oflow-gradeand50%ofhigh-gradeparostealosteosarcomas.nally,thejuxtacorticalsofttissuemassesofperiostealosteo-sarcomastypicallycauseextrinsicerosionofcorticalbone,perpendicularperiostealreaction,andreactivemarrowzoneswithhighsignalintensityonT2-weightedimages,whicharefindingsnotseenwithmelorheostosis.Althoughradiographicandbonescintigraphicappearanceofmelorheostosishasbeenwelldescribed(ie,undulatingcorticalthickeningandmarkedincreaseduptakeofradionuclide),CTandMRimaginghelpsconfirmandaccuratelylocalizethezonesofhyperostosisinthespineandprovideassessmentofthedegreesofnarrowingofthespinalcanalandforamina.Further,eventhoughtheMRimagingappearanceofsofttissuemassesassoci-atedwithmelorheostosisisvariable,mineralizedandnonminer-alizedsofttissueabnormalitiesshouldberecognizedasanothermanifestationofthisdisease.MRimagingaidsinconfirmationofthediagnosisandintheaccuratedetectionanddetermi-nationoftheextentofsofttissueinvolvement.Althoughmelorheostosisisarareconditionaffectingtheaxialskeleton,itshouldbeadefiniteconsiderationinthedif-ferentialdiagnosisofunilateralorsegmentallesionsofcorticalhyperostosisinthespinebecauseaccuratedetectioncanpre-ventanunwarrantedbiopsy.1.LeriA,JoannyJ.Uneaffectionnonde´critedesoshyperostoseencoule´esurtoutelalongeurdunmemberoumelorhe´ostose.BullMemoiresSocMedHopitauxParis1922;46:1141452.Kalbermat
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