Selfassessmentcorner753lkli0hills53ilgGll1s11WIE111111INaISs111111sll111lllllll1Illi11 ID: 953433
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752Self-assessmentcornerSeparationoftheproximalhumeralepiphysisAlaricAroojis,HaroldD'Souza,MGYagnikDepartmentofPaediatricOrthopaedics,BaiJerbaiWadiaHospitalforChildren,Parel,Mumbai-400012,IndiaAAroojisHD'SouzaMGYagnikCorrespondencetoDrAlaricAroojis,8/424ChurchView,14thRoad,TPS3,Bandra,Mumbai-400050,IndiaAccepted23April1998A42-month-oldgirlpresentedwithahistoryofsuddenonsetswellingoftheleftshoulderof4-5daysdurationwithnoprecedinghistoryofsignificanttraumaorfall.Thechildwasirritableandmildlyfebrileonexamination.Atenseglobularswellingmeasuring6x5x5cmwaspresentintheleftshoulderregionwhichwastenderandfluctuantonpalpation(figure1).Therewasanincreaseinthelocaltemperatureandtheskinoverlyingtheswellingwasstretchedandglossy.Shouldermovementswerepainfulandrestricted.Therewasnoregionallymphadenopathynoranydistalneurovascularcompromise.Otherjointswerenormalandnoabnormalitywasdetectedinothersystems.Thechildhadchronicnon-pittingoedemaofbothlegsandwasreluctanttowalk.Therewasnohistoryofableedingdisordernorwasanysuchfamilyhistoryelicitable.AnX-rayoftheleftshoulderrevealedacompleteseparationoftheproximalhumeralepiphysiswithosteopeniaandsurroundingsofttissueswelling(figure2).Haemogramrevealedanaemia(haemoglobin5.3g/dl)butanormalleucocytecountof7.2x109/l(polymorphs44%,lymphocytes48%,andeosinophils8%).Reticulocytecountwas1%,plateletcountwasadequate,andtheerythrocytesedimentationratewas10mmatonehour.Bleedingtime,clottingtimeandothertestsforbleedingprofilewerewithinnormallimits.Serumcalciumandphosphoruslevelswerenormalbutalkalinephosphataselevelwaselevatedto306IU/l(normalrange80-180IU/l).Needlebiopsyoftheswellingresultedintheevacuationofapproximately20mlofalteredbloodandafewsofttissueandbonyfragmentswhichonmicroscopicexaminationrevealedthepresenceofproliferatingfibroblasts,spiculesofnecroticboneandabundantvascularproliferation.Asin-gleX-rayoftheknee(figure3),however,showedtypicalchangeswhichaidedinthediagnosisofthiscondition..........eFigureIClinicalphotographFigure2X-Rayofleftshoulder Self-assessmentcorner753...lk,li0h.ill:.:.s53]|-ilgGll1-|--s1|1W--}--|IE--$111111-$IN--a--|IS--s111111-sl|--*-l111-=|lllll--|--*-|l*---||-----||----
-||----||---|*|----|*|--|l-1Ill|---|*|--i--||*|--11-||*|---i|*|--1111I-i*|-e--B|*|--elll15|llli511-g||f--iE|*|--R11i3WI*11.:^...ww;-.^e^-s-Figure3X-RayofkneeandlegQuestions1Whatisthemostprobablediagnosis?2Whatcharacteristicfindingsareseeninfigure3?3Whatistheunderlyingpathologicalprocess?4Whatisthemanagementoftheproximalhumeralepiphysealseparationinthiscondition? 754Self-assessmentcornerAnswersQUESTION1Theclinicalandradiographicfeaturesarecon-sistentwiththediagnosisofscurvyorvitaminCdeficiency,causingspontaneousepiphysealseparationandsubperiostealhaemorrhage.Otherdifferentialdiagnosistobeconsideredinviewofthepain,localtendernessandpseudo-paralyticstatearelistedinthebox.Scurvy:differendaldiagnosis*acuteosteomyelitis*septicarthritis*bleedingdisorders*Henoch-Schonleinpurpura*thrombocytopenicpurpura*leukaemia*syphilis*acuterheumaticfeverQUESTION2Radiographicchangestypicalofscurvy'2arebestvisualisedattheknees,wristsandcostochondraljunctions,allsitesofrapidbonegrowth.Theinitialpictureisthatofgeneralisedosteoporosisofthespongiosaandadecreaseinthecorticalthickness.Thereisanincreaseinthewidthandopacityofthezoneofprovisionalcalcificationattheendsofthemetaphysis(Fraenkel'swhiteline)andaroundthemarginsoftheepiphysealcentresofossification(Wim-berger'sringingofepiphysis).Outwardprojec-tionofthezoneofprovisionalcalcificationbeyondthelimitsoftheshaft,produceslateralspurformationattheendsofthemetaphysis(Pelkan'sspur).'The'scurvyline'orscorbuticzoneisseenasatransversebandofdecreaseddensityadjacenttothedensezoneofprovi-sionalcalcification.Subperiostealhaemorrhageisanotherdistinctivesign;thelowerendsofthefemurandtibiaandtheupperendsofthehumerusbeingfrequentsites.Spontaneousepiphysealseparationsmayoccasionallyoccur.45QUESTION3Skeletalchangesinscurvyhavebeenstudiedinthebonesofbothanimalsandhumans.6Ascorbicaciddeficiencycausesadysfunctionoftheosteoblasts;thishasbeenascribedtoalossofribonucleicacidandthedisappearanceofcytochromeoxidaseactivityinthecytoplasmoftheosteoblasts.7Theresultisafailuretoproduceosteoidtissueandformnewbone.Thechondroblasts,however,continuetoproliferatenormally,alignincolumnsandformchondroidtissue.Thereisnodist
urbanceinmineralisation;thedegeneratingcartilagecol-umnscalcifynormallybutarenotconvertedintobone.3Theplumposteoblastsbecomeflattenedandresembleconnectivetissuefibroblasts.6Thebonetrabeculaeandcorticesoflongbonesarethinandthezoneofprovisionalcal-cification,throughwhichepiphysealseparationmayoccur,isfragile.Collagensynthesisisalsointerferedwithandinsteadofnormalcollagen-isedfibroustissue,aprimitivetypeofconnec-tivetissueisformed.Inexperimentalstudiesinguineapigs,asuppressionoftheentiregrowthzone,eventuallyleadingtocompletecessationofendochondralossification,hasbeendemonstrated.6QUESTION4Rapidrecoveryoccursfollowingdailyoralorparenteraladministrationofascorbicacid(vitaminC)atadoseof100-200mgperday.Painandtendernessdisappear,subperiostealhaemorrhagescalcifyandbodygrowthsoonresumes.Separationoftheepiphysisinscurvyisbesttreatedconservativelybysplintageandobser-vationandaclosedoropenreductionofthedisplacedepiphysisisrarelyrequired.Com-pleterecoverybyremodellingistheruleandresidualdeformityordisturbanceinlongitudi-nalbonegrowthareseldomseen.8DiscussionTheclinicalmanifestationsofscurvyhavebeenwellchronicledintheliteraturesince1757,whenJamesLindpublishedATreatiseontheScurvyandrecommendedtheinclusionoflemonjuiceinnavaldiets.Scurvydevelopsfol-lowing6-12monthsofdietaryvitaminCdefi-ciency.Earlymanifestationsconsistoflossofappetite,irritabilityandaslowgaininweight.Withtheprogressionofscurvy,thetypicalscorbuticsignsappear.Haemorrhageofthegums,especiallyaroundtheupperincisorsiscommon.Subperiostealhaemorrhageisyetanotherdistinctivesign,thelowerendsofthefemur,tibiaandhumerusbeingfrequentsites.Thechildisveryapprehensiveandpreferstoliesupineinafrog-legposition-apseu-doparalyticpostureinresponsetopain.Thelegsareswollenandoedematous.Spontaneousepiphysealseparationmaytakeplace.Beadingoftheribsatcostochondraljunctionsmaydevelop.Haemorrhagesmayoccurinsub-cutaneoustissue,inandaroundmusclesintheurinaryorgastrointestinaltractorinthesubchondralspace.Anaemiaanddelayedwoundhealingarecommon.'Fracturesofthecalcifiedcartilageoftheepi-physealplateandepiphysealseparationsareknowntooccurinscurvy,buthaverarelybeenreportedinliterature.458Themanag
ementofepiphysealseparationinscurvyiscontroversial,althoughthereappearstobeageneralconsen-susthatpermanentorresidualdeformityordisturbanceoflongitudinalbonegrowthisrare.68Notwithstandingtheoftenextensiveskeletalinvolvement,thelesionshealpromptlyfollowingadequatedietarytreatment.Thiscanbeexplainedbythefactthattheperiosteumhasitsstrongestattachmentatthegrowthplate,whichisdetachedtogetherwiththeepi-physis.Theepiphysistherefore,remainslineduptothedenudedshaftbyaperiosteal Self-assessmentcorner755envelopecontainingblood(subperiostealhaemorrhage).Uponadministrationofascor-bicacid,thesubperiostealhaemorrhagerapidlycalcifiesandremodelswithinafewmonthsleavingnoresidualdeformityorgrowthdistur-bance.Furthermore,epiphysealseparationinscurvyoccursthroughtherelativelyavascularzoneofprovisionalcalcificationcausinglittleornodamagetotheepiphysealormetaphysealbloodvesselssupplyingthegrowthplate.8Aone-yearfollow-upofourcase(figure4)amplydemonstratestheintenseremodellingpotentialfollowingvitaminCsupplementationanddietaryadvice,andconfirmsthefactthatanopenorclosedreductionofthedisplacedepiphysisisrarelyrequired.FinaldiagnosisScurvycausingproximalhumeralepiphysealseparation.-.......-..........Figure4CompleteremodellingoftheproximalhumerusfollowingvitaminCtherapyfor12monthsKeywords:scurvy;epiphysealseparation;proximalhumerusWethanktheDean,BaiJerbaiWadiaHospitalforChildren,forpermissiontoutilisehospitaldata.1BrailsfordJW.Someradiologicalmanifestationsofearlyscurvy.ArchDisChild1953;28:31.2BromerRS.Acriticalanalysisoftheroentgensignsofinfantilescurvy.AJYR1943;49:575.3TachdjianMO.Pediatricorthopedics,vol2,2ndedn.Philadelphia:WBSaunders,1990;p918.4NerubayJ,PilderwasserD.Spontaneousbilateraldistalfemoralphysiolysisduetoscurvy.ActaOrthopaedScand1984;55:18.5SilvermanFN.Anunusualosseoussequeltoinfantilescurvy._JBoneJointSurg1953;35-A:215.6BanksSWBonechangesinacuteandchronicscurvy.Anexperimentalstudy.JfBoneJointSurg1943;25:553.7FollisRH.Histochemicalstudiesoncartilageandbone.II.Ascorbicaciddeficiency.BullJohnHopkinsHosp1951;89:9.8SilvermanFN.Recoveryfromepiphysealinvagination.Sequeltoanunusualcomplicationofscurvy.JfBoneJointSurg1970;52-A:38