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The PDF of the article you requested follows this cover page.  This is an enhanced PDF from The Journal of Bone and Joint Surgery 1955;37:977-984. J Bone Joint Surg Am.Elden C. Weckesser     THUMB (CONGENITAL "CLASPED THUMB")CONGENITAL FLEXION-ADDUCTION DEFORMITY OF THE This information is current as of July 24, 2010 Reprints and PermissionsPermissions] link. and click on the [Reprints andjbjs.orgarticle, or locate the article citation on to use material from thisorder reprints or request permissionClick here to Publisher Information www.jbjs.org20 Pickering Street, Needham, MA 02492-3157The Journal of Bone and Joint Surgery NEUROGENICARTHROPATHYASSOCIATEDWITHDIABETESMELLITUS17.SCHWEIGER,LUDWIG:lYberdietabiformenVeranderungenderHinterstrangebeimDiabetes.Arb.a.d.Neurol.Inst.a.d.WienerUniv.,14:391—405,1908.18.SHORE,T.H.G.:DiabeticNeuropathy.(LettertotheEditor.)Lancet,2:738—739,1947.19.SPEAR,G.E.:DiabeticArthropathy.(LettertotheEditor.)Lancet,2:963—964,1947.20.WILKINs,R.W.,andKOLB,L.C.:VasomotorDisturbancesinPeripheralNeuritis.Am.J.Med.Sciences,2O2@216—221,1941.21.WILLIAMSON,R.T.:ChangesintheSpinalCordinDiabetesMellitus.BritishMed.J.,1:122—123,1904.22.WILSON,I.H.;MCINTYRE,C.H.;andALBERTSON,H.K.:Charcot'sJoint,withUnusualFeatures,inaDiabeticPatient.CaliforniaMed.,70:420—422,1949.CONGENITALFLEXION-ADDUCTIONDEFORMITYOFTHETHUMB(CONGENITAL“¿CLASPEDTHUMB“¿)BYELDENC.WECKESSER,M.D.,CLEVELAND,OHiOFromtheDepartmentofSurgery,WesternReserveUniversityandl'heHandClinic,UniversityHospitals,ClevelandFivepatientswithcongenitalflexion-adductiondeformityofthethumbhavebeenseenduringthepastfiveyears.Threeofthesepatients,seenshortlyafterbirth,respondedwelltoplasterimmobilization;thefourthpatient,seenatagetwo,respondedfairlywell;thefifthpatient,seenatagenine,requiredsurgery.CongenitalabsenceoftheextensorpollicislongustendonwasreportedbyZadekinachildtwoandone-halfyearsofage.Thischildwassuccessfullytreatedbytendontransfer.PollexvaruswasreportedbyMillerintwopatients.Oneofthepatients,eightweeksofage,wastreatedbycastsandmanipulation.MorerecentlyWhiteandJensenreportedeightcasesofthisdeformity,whichtheycalled“¿theinfant'spersistentthumb-clutchedhand.”Threeofthesepatientsweretreatedbytendontransfer.ItisouropinionthattheconditiondescribedbytheseauthorsisofthesamenatureasthatseeninthefivepatientsinourClinic.Congenitalflexion-adductiondeformityofthethumbischaracterizedbyextremeflexionofthedigitatthemetacarpophalangealjointandbyadductionintothepalm*ReadattheAnnualMeetingoftheAmericanSocietyforSurgeryoftheHand.LosAngeles,California,January28,1955.VOL.37-A,NO.5,OCTOBER1955 E.C.WECKESSER(Fig.1-A).Thedigitishelditsthepalmbythegraspoftheoverlyitsgfitsgers.Hypermobilityexistsatthemetacarpophalaisgealjoust.Itstheitsfant.,whetsthegraspoftheoverlyitsgfitsgershasbeetsreleased,passiveextetssioniscarriedoutwithease.Activeextetssion,however,istsotmatsifest(assumitsgextensortendonsarepresent)utstilthethumbhasbeetsheldoutofthepalmbythesplintforseveralmouths(Cases1,2,4,and5).Theconditiotsshouldbedifferentiatedfromotherswhicharesimilar.Chronicstenosingten'lovaginitis(triggerthumb):Passiveextensiotiofthedistaljointislimitedorassociatedwithsnappitsg.Treatmetitistendovagitsotomy.Thethumbinspasticparalysis:HerethethumbiscaughtbeneaththetightlyflexedFIG.1-AFig.I—¿A:Case1,onemontii01(1.Thumbsseverelyadductedintopalms.Noactiveextension.Fig.1—B:Twoandone—halfyearsaftercorre('tion.fingers.Flexiotsattheproximaltlsumbjointisusuallynotsomarkedasitisitsflexiots-adductiotsdeformit.y.Whenthetightlyflexedfitsgershavebeetsopeised,thethumbcxhibitsact.i@'eextetssiots.Thethumobinarthrogryposis:-—¿.rfhis(otsditiOtsismatsifestedbylimFIG.1-Bitatiotsofa('tiveandpassivejointmotioti.Usuallythereisresistancetopassiveextetssioisofthethumbratherthanhypermobilityofthemetacarpophalangealjoint,assectsitscasesofflexiots-adductiotsdeformity.CASEREPORTSCASE1.C.M.,onemonthold,wasbornwithdeformitiesofbothhandsandfeetandwithmildflexioncontractureofbothkneejoints.Therewasnoknownfamilialhistoryofcongenitaldeformities.Thethumbswereseverelyflexedandadductedintothepalms(Fig.1-A),wheretheywereheldtightlyclaspedbythefingers.Therewereflexioncontracturesofthefingers:allthedigitswerelong.Thecleftbetweenthethumbandindexfingerwastighti)ilaterallywhenthethumbswerepassivelyextended.Therewasabnormalmobilityatthemeta('arpophalangealjointofeachthumb.Therewasnoobservableactivecxtensionofthethumbs,althoughpassivelytheycouldbebroughtintonormalfunctionalalignmentwithease.THEJOURNALOFBONEANDJOINTSURGERYI.:t Plastercasts@vereapplied,holdingthethumbsinfunctionalalignmentandthefingersinextension.Toallowforgrowth,thecastswerechangedatintervalsofsixtoeightweeks.Therightthumbwasimmobilizedforfiveandone-halfmonthsandtheleft,forsevenandone-halfmonths.Whenthecastshadbeenremoved,thepatientspontaneouslyheldhisthumbsingoodalignment,andatonceitwasapparentthattherewasactiveextension.Figure1-Bshowstheresultsatfollow-upexaminationtwoandone-halfyearslater.Subsequently,PlasticproceduresoftheZtypewerecarriedoutonthetightbandbetweenthethumbsandindexfingers.Atpresent,thechildisfiveyearsoldanduseshisthumbsandfingersquitenormally.CASE2.C.F.,sixweeksofage,wasunableatbirthtoextendthethumbs.Nootherabnormalitywasnoted.Themotherexhibitedabnormalmobilityofherproximalthumbjointsbuthadnootherabnormalities.Onesil)lingwassul)se(@uentlybornwithaclubfoot.Examinationshowedboththumbsflexedandadductedintothepalm(Fig.2-A),wheretheywereheldinthishyperfiexedpositionbytheoverlyingfingers.TherewerenoassociatedflexioncontracturesoftheCONGENITALFLEXION-ADDUCTIONDEFORMITYOFTHETHUMBCase2.X-ravsofhandswhenpatientwassixweeksofage.Thumbsheldconstantlyinthispositionwithfingers(‘laslx'(loverthem.Noactiveextensionofthumbs.Fiveyearsaftercorrection.Activeextensionexcellent.FIG.2-AFIG.2-BVOL.37-A,NO.5,OCTOBER1955 fingers.Noactiveextensionwasobservedinthethumbs,butpassivelytheycouldbereadilyextended.Activeflexionwaspresentbutdidnotseemtobeexcessivelystrong.Whenthethumbhadbeenbrought_______________intoextension,abandofsofttissuewasfoundbetweenthebaseofthethumbandthebaseoftheindexfinger,Bilateralplastercasts,changedatintervalsofsixtoeightweeks,heldthethumbsinfunctionalalignmentforsevenandone-halfmonths.Thechildthenextendedherthumbsactivelyandnormally.Figure2-Bshowsnormalalignmentofthumbsatagefive.CASE3.W.M.,nineyearsofage,hadbeenbornwithcongenitaldeformitiesofbothhandsandtorticollisandlaterdevelopedseverescoliosis.Theleftupperextremityshowedabsenceofthethumbandflexioncontracturesofthefingersandelbow.Therightupperextremityshowedflexion-adductiondeformityofthethumbandflexioncontracturesofthefingers,whichwereheldtightlyflexedovertheadductedthumb.Figure3-A(obtainedfromanoldhospitalrecord)showsthechildatfifteenmonths.Figure3-Bshowstheconditionofthehandswhenthepatientwasfirstseenatnineyearsofage.Theflexioncontracturesofthefingersandtheflexion-adductiondoformityoftherightthumbremains.Thereweretightsoft-tissuebandsonthevolarsurfacesofthefingersandbetweenthebaseofthethumbandtheindexfingerontherighthand,Theproximalthumbjointwasfusedsurgicallytoholdthethumbinfunctionalalignment,andfreefullthicknessskingraftswereappliedtothevolarsurfaceof@Jthethumbandfingerstoovercomethesoft-tissuecontractures.Figure3-Cshowsthyresult.Thethumbwasmadefunctional,althoughactiveextensionremainedE.C.WECKESSERFIG.3-Afifteenmonths(fromoldhospitalCase3,ageFIG.3-BHandswhenfirstseen,thepatientthenbeingnineyearsofage.THEJOURNALOFBONEANDJOINTSURGERY CONGENITALFLEXION-ADDUCTIONDEFORMITYOFTHETHUMBl)oor.Theflexioncontracturesofthefingerswereimprovedbutnotcompletelyovercome.Partialfailureinthisinstanceisattributedtothelatestageatwhichcorrectionwascarriedout.Permanentjointchangeshadalreadyoccurredinthefingers,anditisfeltthattheoverstretchedextensortendonsofthethumbhadbeenpermanentlydamaged.CASE4.W.M.,twoyearsofage,hadbeenbornwithcongenitalofbothhands,clubfeet,largeinguinalherniae,andpyloricstenosis.Familyhistorywasnegativeforpreviouscongenitalanomalies.Examinationshowedflexion-adductiondeformityofthethumb,bilaterally,associatedwithflexioncontracturesofthefingers.Thepositionoftheleftthumbwasmoreextremethanthatoftheright.Castswerewornforthreemonths.Therightthumbshowsgoodcorrection,buttheleftisstillratherCASE5.M.W.,elevenweeksofage,wasbornwiththethumbsclaspedinthepalms.TheonlyassociatedFIG.3CabnormalitywasaslightlimitationofextensionoftheAfterfusionofproximalthumbjoint.Activeleftelbow.Therewasnoknownfamilialhistoryofextensionpoorduetolatetreatment.congenitalanomalies.Thethumbswereadductedintothepalmsandheldtherebytheflexedfingers.Noactiveextensionofthethumbswasobserved.Passively,thethumbscouldbeextendednormally.Nocontracturesofthefingersandnoparticulartightnessbetweenthethumbandtheindexfingerexisted.Plastercastswerewornforonemonthontherighthandandthreemonthsontheleft(Fig.4-A).Theresult,sixmonthslater,isshowninFigure4-B.Normalnewborninfantsholdtheirhandsclosedmuchofthetime.Theyalsoclasptheirthumbs.Amongthirty-sixnewborninfantsobservedinthenurseryatUniversityHospitals,fifteen(42percent.)heldtheirfingersclaspedoverthethumbspartofthetime.Itwasalsonoted,however,thatthegraspofthefingerswasreleasedperiodicallyandthatthethumbthenmovedfreelyoutofthepalm.Bieberstatesthatthethumbinthenewborninfantplaysnoroleingraspandthatitusuallyliesdormantinthepalmsurroundedbytheotherdigits.ThestudiesofGesellindicatethattheinfantbeginstouseitsthumbingraspataboutthreetofourmonthsofage.Inthefivecasespresentedhere,itappearsthateitherthereflexclaspofthefingersuponthethumbwasgreaterthannormalorthatweaknessoftheextensortendonsofthethumballowedittoremainbeneaththeclaspofthefingers.Duchennehasshownthattheproximalphalanxofthethumbbecomesmoreflexedwhentheshortextensorlosesitsaction.WhiteandJensenascribethedeformityintheircasestoabsenceoftheextensorpollicisbrevistendon.Inourcases,withthepossibleexceptionofCase3,thedeformitywascausedbyweaknessratherthanbyabsenceofthethumbextensor.Weaknessoftheextensorsisundoubtedlyaccentuatedbytheover-pulloftheflexorsandtheclaspoftheAssociatedflexioncontracturesofthefingersmighthavebeenanadditionalfactorinthreeofourcases;intheothertwotheywerenotpresent.Sometightnessinthewebspacebetweenthethumbandtheindexfinger,whichwaspresentinthreeofthecases,mayalsohavebeenafactor.Allthepatientsexhibitedabnormalmobilityofthemetacarpophalangealjoints.ThepossibleroleofthegraspingreflexwhenthethumbisintheVOL.37-A,NO.5,OCTOBER1955 B.C.W'ECKESSEIIFIG.4-ACastswhenfirstapplied,thepatietitthenbeingelevenweeksofage.FIG.4-BSixmonthslater,followingplasterimmobilizationofonemonthsdurationontherighthandandthreemonths'onthelefthand.palmisinteresting.ThemotherofC.F.(Case2)exhibitedabtsormalmobilityofherproximalthumbjointsbuthadtsootherabtsormalityofherhatsds.Theperiodofimmobilizationitsthefirsttwocaseswasfiveandone-halftosevetsandTHEJOURNALOFBONEANDJOINTSURGERY CONGENITALFLEXION-ADDIJCTIONbEFORMITYOFTHETHUMBone-halfmonths.Inthemostrecentcase,onetothreemonthsgaveagoodresult.Itisthoughtthatplasterimmobilizationismostdesirableinthatitgivesthemostconstant.support.Thecastsmustbechangedeachsixtoeightweekstoallowforgrowth.Associatedflexioncontracturesofthefingers,whenpresent,aretreatedsimultaneously.Atrialperiodofplasterimmobilizationwillshowwhetherornotextensortendonsarepresent.Ifnoextensionreturnsafterseveralmonthsofplasterimmobilization,tendontransfertorestoreactiveextensionisindicated.SUMMARYANDCONCLUSIONSOfthefivecasesofcongenitalflexion-adductiondeformitypresentedinthisreport,allwerebilateralexceptCase3.ThepatientinCase3hadacongenitalabsenceoftheleftthumb.Ineachinstanceofthedeformity,thethumbwasfirmlyclaspedbeneaththeflexedfingersanddidnotexhibitactiveextension.Threepatientsalsohadanassociatedtightnessofthewebspacebetweenthethumbandindexfinger.Allthepatientshadabnormalmobilityofthemetacarpophalangealjointsoftheirthumbs.Inthreeofthepatients,seenonetothreemonthsafterbirth(Cases1,2,and5),plasterimmobilizationgaveexcellentresults.Inthefourthpatient,seenattheageoftwoyears(Case4),afairtogoodresultwasachievedafterthreemonths'immobilization.Inanotherpatient,seenatagenine(Case3),afairresultwasobtainedbyfusionofthemetacarpophalangealjointofthethumb.Congenitalflexion-adductiondeformityofthethumbshouldbecorrectedbyplasterimmobilizationinfunctionalpositionattheearliestpossibletime,beforepermanentjointandtendonchangesoccur.Ifactiveextensiondoesnotreturn,tendontransferisindicatedtorestoreactiveextensiontothethumb.1.BIEBER,IRVING:GraspingandSucking.J.Xciv.andMent.Dis.,91:31—36,1940.2.DUCHENNE,G.B.A.:PhysiologyofMotion,DemonstratedbyMeansofElectricalStimulationandClinicalObservationandAppliedtotheStudyofParalysisandDeformities.TranslatedandeditedbyE.B.Kaplan,p.167.Philadelphia,J.B.LippincottCo.,1949.3.GESELL,ARNOLD:AnAt-lasofInfantBehavior.Vol.I,pp.243—249.NewHaven,YaleUniVersityPress,4.MILLER,J.W.:PollexVarus.AReportofTwoCases.Univ.Hosp.Bull.,AnnArbor,10:10—11,1944.5.WHITE,J.%V.,andJENSEN,W.E.:TheInfant'sPersistentThumb-ClutchedHand.J.BoneandJointSurg.,34-A:680—688,July1952.6.ZADEK,ISADORE:CongenitalAbsenceoftheExtensorPolli-:sLongusofBothThumbs.OperationandCure.J.BoneandJoint-Surg.,16:432—434,Apr.1934.DR.GEORGES.PHALEN,CLEVELAND,OHIO:IwasveryinterestedtohearofDr.Weckesser'sexperiencesintreatingfivecasesofcongenitalclaspedthumb.Heismostfortunatetohavehadtheopportunityofseeingsomanycaseswiththisunusualcongenitaldeformity.Iamsurethatveryfewofushereatthismeetingcanduplicatehisexperience.Onewonderswhythisanomalyisnotobservedmoreoften.Forthefirstfewmonthsoflife,thethumbactuallyservesnousefulpurpos@intheinfant'sexistence.Itisnotuntilhelearnstousethethumbingraspingthathehasanygoodreasontoraisehisthumboutofhispalm.Allofushaveobservedhowmuchofthetimeanormalinfantkeepshisthumbstightlyclaspedinhispalms.Itiseasytoseehowcongenitaladductionofthefirstmetacarpophalangealjointmightbepartofageneralizedarthrogryposis.Fouroftheauthor'sfivecasesmightfallintothisgroup.Theeaseandpermanencyofthecorrectionofthedeformity,however,wouldleadonetobelievethatcongenitalclaspedthumbisnotsimilartoanarthrogryposis.IwouldliketoaskDr.Weckesserifheobservedthefateofthesoft-tissuebandsinthefirstwebinthosecasesinwhichanadequatecorrectionofthedeformitywasobtained.Didthesebandssoften,stretch,ordisappearentirely?Also,inthetwocasesrequiringoperativecorrection,wasthecontractureinthefirstwebfoundtobeduetoasubcutaneousfibrousbandorduetoskincontractureonly?Ifasubcutaneousfibrousbandwasfoundatoperation,wasitobservedtobeathickenedpartofthenormalpalmarfascia,suchasweVOL.37-A.NO.5.OCTOBER195S seeinDupuytren'scontracture?Ifanyfascialbandswereremovedinthesetwocases,wereanyhistologicstudiesmadeofthistissue?IwouldcertainlyagreewithDr.WeckesserthatplasterimmobilizationisthebestIflethodoftreatingthisdeformity.Thegoodresultsobtainedwithearlytreatmentsserveagaintoemphasizetheimportanceofcorrectingallsoft-tissuecontracturesassoonaspossiblebeforepermanentjointcontracturesorbonydoformitiesdevelop.DR.ARTHLrRJ.BARSKY,NEWYORK,N.Y.:Dr.Weckesserstatedthatinfourofhisfivecasesflexion01'extensiondeformitieswerepresentinjoint-sotherthanthoseofthethumb.Thecasesinwhichthecondit.ionwasnotlimitedtothethumbjointsalonebelong,Ibelieve,inthecategoryofwhatisvariouslycalledarthrogryposismultiplexcongenita,multiplearticularrigidities,or,touseatermthatisperhapsbetter,amyoplasiacongenita.Curiouslyenough,thissameconditionhasbeenfoundinlambs,acircumstancewhichhasprovidedadditionaldata.Thereappearstobepresentahereditaryfactorofarecessivenature.Gilmourhadtheopportunityofautopsyingababywithhandinvolvementandfoundthatnoextensorpollicislonguswaspresent.Themusclesaffectedinthisbabyweremuchsmallerthanthenormalduetoanactualdecreaseinthenumberoffibers.Therewasadefiniteincreaseinthenumberofcollagenousfibersintheendomysiumandperimysium.Inanothercase,Middletonfoundthattheextensorpollicislonguswasreplacedbyadiposetissuecontainingfibrousstrandsandremnantsofmusclefibers.Weakelectricalresponsehasbeenreportedbutnotreactiontodegeneration.Thebeliefhasbeenexpressedthatstiffnessofthejointsistheprimarycauseofthelimitationofmotion.Althoughthisfixityofthejointssometimeshastheclinicalappearanceofafibrousankylosis,thereisnodefiniteevidenceofinflammatorychange.Thejointfixityisprobablylargelyduetodisusearisingoutofalackofactiveandpassivemotion;withdisuseonewouldexpecttheperiarticulartissuesandeventheskintobecometight.Middleton'sobservationonlambsindicatesthattheprocessisoneofdegenerationofformedanddifferentiatedmusclefibers.Thereisnodefiniteevidenceastothenatureoftheabnormalitywhichcausestheatrophyanddegenerationofthealreadyformedmusclefibers,butitisthought(byGilmour)thattheprocessisnotdependentonexternalinfluencesbutispossiblydependentonanintrinsicgeneticabnormalitycenteredinthemusclecells.Wehavehadfourcasesofamyoplasia,threeofwhichgiveagoodideaofthegradationsofthecondition.[Alanternslidewasshownofthemildestcase,inwhichinvolvementofonlytheindexfingersofbothhandswasapparent.Asecondslideshowedacaseinwhichthewristandelbowjointwereaffected,andathirdslideshowedinvolvementofpracticallyallthejointsofallfourextremities.]Dr.Weckesserhasmadeanexcellentpoint,onethatbearsemphasis,namely,thatveryearlytreatmentbysplintingwillprobablyresultinimprovementifenoughmusclefibersarelefttofunction.DR.WECKESSER(closing):InanswertoDr.Phalen'squestionsaboutthethumb-webcontractures,Iwishtostatethatthetightbandsbetweenthethumbandindexfingerseemedtoremainessentiallyunchanged.Inthetwocasesrequiringsurgery,thetightbandwasfoundtobeduetoskincontractureonly.Nosubcutaneousbandswerepresent.NON-OPERATIVETREATMENTOFLUMBARDISCSYNDROME(Continuedfrompage936)9.MENNELL,JAMES:Backache.Philadelphia,P.Blakiston'sSonandCo.,1931.10.MIXTER.W.J.,andBARR,J.S.:RuptureoftheIntervertebralDiscwithInvolvementoftheSpinalCanal.NewEnglandJ.Med.,211:210—215,1934.11.Prrxu@i,H.C.:SacrarthrogeneticTelalgia.V.APlanforTreatment.J.BoneandJointSurg.,19:169—184,Jan.1937.12.RAMSEY,R.H.:ConservativeTreatmentofIntervertebralDiskLesions.InInstructionalCourseLectures,TheAmericanAcademyofOrthopaedicSurgeons,1954,Vol.11,pp.118—120.AnnArbor,J.W.Edwards,1954.THEJOURNALOFBONEANDJOINTSURGERY