pollicization using the second metacarpus: A clinical case series Clinical and functional results of radial club hand with centralizationand International Journal of Surgery Case Reports

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ContentslistsavailableatScienceDirect

International Journal of Surgery Case Reports

j o u r n al ho m e p a g e :w w w . c a s e r e p o r t s . c o m

Case Series

Clinical and functional results of radial club hand with centralization and pollicization using the second metacarpus: A clinical case series

Farivar A. Lahiji

^a

, Farhang Asgari

^b

, Fateme Mirzaee

^c

, Zohreh Zafarani

^d

, Hamidreza Aslani

^a,∗

aShahidBeheshtiUniversityofMedicalSciences,Iran

bLorestanUniversityofMedicalScience,Iran

cUniversityofSocialWelfareAndRehabilitationSciences,KneeandSportMedicineResearchCenter,MiladHospital,Tehran,Iran

dKneeandSportMedicineResearchCenter,MiladHospital,Tehran,Iran

a r t i c l e i n f o

Articlehistory:

Received15April2019

Receivedinrevisedform22July2019 Accepted24July2019

Availableonline1August2019

Keywords:

Radialclubhand Centralization Pollicization Metacarpalbone

a b s t r a c t

INTRODUCTION:Radialclubhand(RCH)isararecongenitaldeformityleadinginseveralfunctionaland psychologicalproblems.However,ourknowledgeaboutthelong-termfunctionaloutcomesoftreating RCHislimited.Incurrentstudy,weinvestigatedtheoutcomesofcentralizationandpollicizationusing secondorthirdmetacarpalboneinRCHpatients.

METHODS:Therewere15hands(13patients)withRCHunderwentcentralizationandpollicizationusing secondorthirdmetacarpalboneortendontransfer.Thepatientsaged1.2±1yearsatthetimeofthe surgery.Onearlypostoperativex-rays,theforearm-handanglewasmeasured.Thepatientswerefol- lowedfor6.2±2.3years.Attheﬁnalvisit,disabilitiesofarm,shoulderandhand(DASH)scorewas completed.Furthermore,forearm-handangleandrangeofmotionofbothwristsinsagittalandcoronal planesweremeasured.

RESULTS:Themeanofforearm-handangleincreasedsigniﬁcantly.In11wrists,forearm-handangle increasedonly10degreesofless.Therangeofoperatedwristwasimprovedinsagittalandcoronal planes.Therelativerangeofwristmotioninpatientswithunilateraldeformityinsagittalandcoronal planeswas83±11percentand61±12percent.Threepatientsdevelopedskinnecrosis.

CONCLUSION:Earlycentralizationandpollicizationusingsecondorthirdmetacarpalbonecansigniﬁ- cantlyrestoretherangeofmotionandfunctioninpatientswithRCH.

1. Introduction

Radialclubhand(RCH)orradialdysplasiaisa complexcon- genitaldifferenceoccurringinalongitudinaldirectionresultingin radialdeviationofthewristandshorteningoftheforearm.Inthis condition,handradiallydeviatedatthedistalforearmintheshape ofagolfclub[1–3].Bonedisordersarethemostimportantpartof RCH,butabnormalitiesofmuscles,arteries,nervesandjointscan greatlyaffectthefunctionandtreatmentofupperlimb[4].The reasonofnamingRCHisthatalltheradialcolumnofforearmare hypoplasticandasaresultthesechildrenareoftendeprivedofhav-

∗Correspondingauthorat:ShahidBeheshtiUniversityofMedicalSciences,Knee andSportMedicineResearchCenter,TehranProvince,Tehran,HemmatExpy,Iran.

E-mailaddresses:[email protected](F.A.Lahiji),

[email protected](F.Asgari),[email protected](F.Mirzaee), [email protected](Z.Zafarani),[email protected](H.Aslani).

ingafunctionalthumbandmayinthefuturerequirepollicization [4,5].

Theﬁrstnewly-bornmaleinfantcasewithbilateralclubhand duetototallackoftheradiuswasreportedin1733byPetit[6].

Thisdeformityisanuncommonconditionanditsprevalenceis estimatedat1:20000to1:100000livebirthsandslightlyhigher inboys,at3:2[1,7,8].Thisanomalyisbilateralandasymmetricin 38%–58%ofcases[9,10].ChildrenwithbilateralandsevereRCH haveconsiderablefunctionallimitationsduetothumbandwrist dysfunction,andshortupperlimbs[1].

AlthoughRCHhasahighrangeofphenotypesfromhypoplasia ofthethumbtocompletelackoftheradiusandthefirstray,Bayne etal.dividedthisdeficiencyintofourtypesbasedontheradio- graphicseverityoftheradialraydeficiency[11]:TypeI,themildest type,isdeterminedbyradiusshortening.Peoplewithhypoplastic radiusareconsideredastypeII,partialradialabsenceastypeIII,and completeabsenceofradiusastypeIV.RCHtreatmentcanbehighly variableanddifferentbasedonthepatient’sageandseverityofthe https://doi.org/10.1016/j.ijscr.2019.07.076

org/licenses/by/4.0/).

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deformity.Treatmentgoalsusuallyinvolvecreatingafunctional andcentralizedhand,maintainingtherangeofmotion,stabilityof thewrist,andsustaininglongitudinalgrowthofforearm[12,13].

TreatmentofRCHshouldbeginassoonaspossibleafterbirth.

TypeIandII,usuallyrespondwelltostretchingandsplinting,which willstretchthetightsofttissuesandradialstructuresandallows passivemodiﬁcationofthemalformationbyaligningthehandand wristwiththeulna[1].TypesIIIandIVareconsideredasthemost commonformsofdeformitythatareusuallyassociatedwiththe greatestamountofradialdeviationofthewrist[4,14].Themost commonmethodof treatmentis centralization of thewrist, in whichpatient’swristwillbemovedtothecentralpartofthedis- talulnaanditsmainobjectiveiscorrectionofsemi-subluxation andradialdeviation,whichmayunfortunatelyhaveahighrateof recurrenceofdeformity,damagingulna’sphysis,andwriststiffness [4,9,15].

Oneofthemostimportantstepsiscreatingathumbwithagood functionforthepatients,whichdependingontheseverityofthumb anomaliesisperformedbyvarioustechniquessuchastendontrans- fer(incasesofnonfunctional thumb)andpollicizationbyindex ﬁngerorthesecondﬁngerofthefoot(incaseswithoutthumb)and somestudieshaveprovidedrelativelygoodresultsofthisproce- dure[14–20].Despitevariousstudiesrelatedtointroductionofthe treatmentmethodsofRCHandevaluationoftheresults,limited dataisavailableonthelong-termoutcomesofdifferenttreatment methods.Thepresentretrospectivestudyaimedtoevaluatethe long-termtreatmentoutcomesforRCHpatientswithcorrectionof deformitywithcentralizationandpollicizationusingthesecond metacarpus.

2. Materialsandmethods

Thepresentcaseseriesof13patientswithRCHwasconducted intwohospitalsfrom2006to2016,onpatientsundergoingtwo- stagedcentralizationandpollicizationsurgerywiththesecondor thirdmetacarpusortendontransferwhowererecruitedaftersign- ingthewritteninformedconsent.ThreepatientshadbilateralRCH andatotalof16deformedlimbsenteredtheﬁrstphaseofthestudy.

Inonepatientwithbilateraldisease,RCHwastypeIthatdidnot requiresurgeryandwasexcludedfromthestudy.Intotal,thestudy wasperformedon15limbsin13patientswithRCH.

Firstly,thechartsofallpatientsevaluatedandpatient’sRCHdata wereextracted.Then,demographicinformation,includingsex,side ofthedisease,ageatﬁrstandsecondsurgery,typeofsurgery,type ofRCHbasedonBaynecategory,typeofthumbanomalyaccord- ingtoBuck-Gramckocategory,andcomorbiditieswererecorded.

Informedconsentwasobtainedfromallparents.Accordingtothe radiographstakenimmediatelyafterthesurgery, forearm-hand anglewasmeasuredastheanglebetweenthelongitudinalaxis of ulna and the longitudinalaxis of thethird metacarpus.Our surgicaltechniquewasthesameaswhathasbeendescribedby Buck-Gramckoforpolicization.

Afterdiagnosiswasestablished,serialsplintingwasperformed forstretchingandpatientswerereferredtoaneonatologistforrule outcomorbiditiesthatiscontraindicationsforsurgery.Inthefirst stageofoperativemanagementtheradializationorcentralization wasconsidered.Inmostofthecasestheagesofthepatientswere between6to18monthsattimeofthefirststageofoperation.The patientwasplacedinsupineposition.Undergeneralanesthesia, surgerywasperformedusingbilobedflapapproachofEvans. A transverseincisionwasstartedontheradialsidehsattimeofthe firststageofoperation.Thepatientwasplacedinsupineposition.

Undergeneralanesthesia,surgery wasperformedusingbilobed ﬂapapproachofEvans.Atransverseincisionwasstartedonthe radialsideofthewrist,extendingtoulnaatanangleofapproxi- mately90^◦.Theﬂapwaselevated,whiledorsalveinsandsensory

Fig.1.PollicizationwithBuck-Gramckotechnique.

nerveswerepreserved.Radialsideincisionswereverysuperficial topreventdamagetothemediannerve.Theextensorretinaculum wascutintworadialandulnarsides.Extensorandflexorstructures weredeterminedbydrawingtendonormusclemass.Ifbrachiora- dialis,FCR,¹ECRL²andECRB³existed,theywereseparatedfromthe insertion.Longdigitextensorswereretractedtotheradialsidewith specialcaretomaintaintheulnarartery.Dorsalandpalmarcapsule werecuttransverselytoreleasecarpoulnarjoint.Anyfibroticand contractedstructurewasexcised.Then,thehandwastransposedto theulnarsideandwrist’sboneswereplacedontheulnarheadanda pinwasplacedfromthesecondmetacarpusinradializationorthird incentralizationintotheulnarbone.Ulnarsideofthewristwas reinforcedusingshorteningandtighteningofECU⁴muscle.Then with90^◦rotation,theulnarandthendorsalflapwerecoveredon theradialsideofthewristandsuturedwithabsorbable4-0sutures.

Then,thehandwasplacedinalongcastfor6to8weeks.Afterward, thepinsremovedandshortarmsplintweremadeforthepatientfor full-timeusefor6months.After6monthsthesplintshouldbeused overnightuntilskeletalmaturity.Ifthesecondoperationwasnec- essaryforpollicization,Buck–Gramckotechniquewithsomeminor modiﬁcationswasdone.Wetriedtodothepolicizationbeforeage oftwo(Fig.1).

Thepatientswerecalledandaskedtocometohospitalclinic;if theywishedtocooperateinthestudy,andshoulderandhandscore (DASH)⁵questionnairewasusedtoassesstheirfunctionalstatusof disabilitiesofarm.Thisscorerangesfromzeroto100,andrep- resenttheeffectivenessofthecurrentsituationoftheupperlimb (unilateralorbilateral)ontheabilitytoperformeverydaytasksasa percentage.Onthisscale,scorecloserto100meansgreaterimpact oftheupperextremitiesdiseaseontheindividual’sfunction.Inthe ﬁnalvisit,anterior-posteriorandlateralradiographswereobtained andforearm-handanglewasrecalculatedandcomparedwiththe radiographstakenimmediatelyaftersurgery.Therangeofmotion ofbothwristswasmeasuredinthesagittalandcoronalplanesusing agoniometer.Incaseswherethepatienthadunilateraldeformity, wrist’srangeofmotionwascompared,relativetothenormalwrist.

Quantitativedatawerepresentedasmean±SD⁶ andqualitative dataasnumbersandpercentages.Tocheckthedataandforstatis-

1FlexorCarpiRadialis.

2ExtensorCarpiRadialisLongus.

3ExtensorCarpiRadialisBrevis.

4ExtensorCarpiUlnaris.

5Disabilitiesofarmshoulderandhand.

6StandardDeviation.

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Table1

Demographicandbackgroundinformationofthepatient.

Numberofpatients 13

Numberoflimbs 15

Age(atthetimeofﬁrstsurgery,year) 1.2±1(11.5)

Durationofthefollowup 6.2±2.3(19)

Gender M F

(M/F) 6(46.1%) 7(53.9%)

Secondaryprocedure Pollicization4 WristTendontransfer3

Radialclubhandtype II1(6.6%) III7(46.7%) IV7(45.7%)

Table2

Comparisonofoutcomesimmediatelyaftersurgeryandﬁnalvisit.

Immediatelyaftersurgery Finalvisit Pvalue

SagittalROM(^o) 90.92±21.48(20140) 103±32(20140) 0.221

CoronalROM(^o) 21.92±8.47(1040) 25±8(1040) 0.556

Forearmhandangle(^o) 13.8±5(1023) 22.2±13.5(1060) 0.005

DASHscore 44.38±11.42(2060) 34.2±9.7(1855) 0.069

ROM:rangeofmotion.

Table3

Comparisonofsoundwristandunderwentsurgerywristoutcomesinunilateral patients.

variables soundwrist underwentsurgerywrist Pvalue SagittalROM(^o) 140±6(130150) 117±17(75140) 0.003 CoronalROM(^o) 47±5(4055) 28±6(2040) 0.001<

ROM:rangeofmotion.

ticalanalysis,SPSS⁷ver.16softwarewasused.TheWilcoxontest wasusedtocompareforearm-handangle.P<0.05wasconsidered asthesigniﬁcancelevel.

ThistheresearchworkhasbeenreportedinlinewiththePRO- CESScriteria[21].

3. Results

Asstated,15limbsin13patientswithRCH wereexamined.

ItshouldbenotedthatamongthreepatientswithbilateralRCH, onelimb wasexcludedfromthestudy duetoresponse tonon operativetreatment.Thepatients’demographicinformationsare providedinTable1.Thetableshows,thedistributionofgenders wasalmostequal.Intheﬁrststageofcentralizationsurgery,rota- tionalﬂapandwristtendontransferwereappliedforallpatients.

Duetotheabnormalityofthumb,tendontransferofthumbwas requiredin4handsandpollicizationin6patients,butduetothe losttofollow-up,tendontransferofthumbwasperformedin3 patientsandpollicizationin4patients.

Meanforearm-handangleinRadiographytakenimmediately after surgery was 13.8±5^◦ (range: 10–23) that significantly increasedin thefinal visitto22.2±13.5^◦ (10–60) (P=0.005). It shouldbesaidthatin11hands,theincreaseinforearm-handangle wasnegligible(≤10^◦).ThemeanDASHscoreinthefinalvisitwas 34.2±9.7 (range:18–55) (Table 2).Themean DASHscore in 4 patientswithpollicizationwere39.3±12(range:28.1–55.2).

Rangeofmotionintheoperatedwristinsagittalandcoronal planeswas103±32^◦ (20–140) and24±8(10–40). Infact,in 4 hands,limitationofmotioninthesagittalplanewasverysevere andrangeofmotionwaslessthan80^◦.Inthesecases,therangeof motioninthecoronalplanewasmuchmorerestrictedthanothers.

Wecomparedrangeofmotionofnormalandoperatedwristsin patientswithunilateraldeformities.Theresultsofthecomparison arepresentedinTable3.Therelativerangeofmotionofthewrist

7StatisticalPackagefortheSocialSciences.

inthesagittalandcoronalplaneintheoperatedhandwas83±11%

(range:55–96)ofthenormalhandandinthecoronalplanewas 61±12%(range:40–78%).Skinnecrosisoccurredinthreepatients.

4. Discussion

Themost importantﬁndingof this studywasthat two-step treatmentofRCH,includingcentralizationwithrotationalﬂapand wristtendontransferandsubsequentlyattemptstocreateafunc- tionalthumbwithpollicizationusingthesecondorthirdRayor tendontransferthatisassociatedwithfavorableclinicaloutcomes andfunctioninmostpatients.

RCH is a longitudinaldefect that cancausea wide range of upper extremity deformities and its etiology may be sporadic, unknown,orassociatedwithaclinicalsyndromelikeHolt-Oram syndrome,Thrombocytopenia-AbsentRadius(TAR),VACTERLand Fanconianemia[4].Thisdiseasecanhaveawiderangefromamild illnesswithnonsurgicaltreatmenttoseverediseasewithmulti- staged surgical treatment.In this disease,the radialcolumn of forearmishypoplastic,thusthethumbisoftennotfunctionaland,if untreated,thedisabilitywillremain.Ontheotherhand,becauseof thelowincidenceofthisdeformity,thenumbersofrelevantstudies arelimitedandaremostlycaseseries.So,wehaveverylittleinfor- mationandappropriatetreatmentandprognosisisuncleartous.

Duetoproblemsassociatedwiththesepatients,regardlessofthe severityofanomaly,initialassessmentofgeneralhealthstatussuch asheart,kidney,gastrointestinalandblooddisease,isnecessary.

Therefore, electrocardiography, renal ultrasound, blood analysis andchromosomalbreakagemustbeperformedforallpatients.

In previousstudies, differentsurgicaland nonsurgical treatment methodshavebeenproposed. Thepatientis candidateof serial splinting and stretching, aimedto maintain passive cor- rectionofwristdeformity[4,12].Absolutecontra-indicationsfor surgeryincludethefollowing:

1Olderkidswithestablisheddisease 2Associatedmedicalsanomalies 3Elbowcontractureinextension

4Milddeformitywithacceptablecosmeticstatusandfunction.

Thegoalofsurgicaltreatmentistoachievethedesiredlength oftheupperlimb,correctionofforearmdeviation,reconstruction ofthumb,and pollicization.Wristrealignmentincludescentral- ization and radializationwithsimilarsurgicalexposurein both methods.Inradialization,itwastriedthattheradialulnardefor- mitybeovercorrectedtoalignulnawiththeindexﬁnger,andall

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theforceofextrinsicmuscles(ﬂexorcarpiradialis,brachioradialis, extensorcarpiradialislongusandbrevis)movetotheulnarside.

Incentralization,ulnaraxisisalignedwiththethirdmetacarpus.

Regardingthethumbstatus,itmustbesaidthatinhypopla- siagradeI,thereisoftendonotneedsurgery.Inhypoplasiagrade II,treatmentincludesopponensplasty,ﬁxationofmetacarpopha- langealjointand1^stwebDeeping.InseveredegreesofgradeIII, IVandV,usuallypollicizationisusedtoimprovepinchandgrasp status.OthermethodsoftreatmentinhypoplasiagradesIVandV includemicrovascularjointtransferthatusesthesecondtoeofthe leftfoot.In1998,Vilkkietal,introducedthismethodandreported favorableresultsin9patientswithRCHgradeIV[20].In2008,Vilkki andhiscolleaguesincreasedthenumberofpatientsandreported thetreatmentoutcomesof19handsin18patients.Theresearchers foundthatattheﬁnalvisit,meanforearm-handanglewas28^◦of radialdeviation,meanrangeofmotionofthewristjointwas83^◦, meanulnargrowthwas15.4cm,andmeanrelativelengthofulna, comparedtotheotherside,was67%[14].

Somescholars,inadditiontofavorabletreatmentresults,have evaluatedtherecurrencerateofdeformityinRCHfollowingcen- tralizationandhaveachievedsignificantresults.ShariatZadehand colleaguesexaminedtherecurrenceofdeformityaftercentraliza- tion.In this study,11 handsunderwent centralization. Patients werefollowed for 90 months. Meanpre-surgical hand-forearm anglewas75^◦thatreached25^◦immediatelyaftersurgeryand52^◦ inthefinalvisit.Thus,thecorrectionratewas66%andtherateof lossofcorrectionwas54%.However,theresearchersdidnotstate howmanypatientshada recurrenceofdeformity[15].Damore andcolleaguestreated19casesofRCHwithcentralizationin2000 andobservedthatangulationdeclinedfrom83^◦to25^◦immediately afterthesurgery(58^◦ improvement).However,inthefinal visit after3.3years,itreached63^◦ (38^◦ lossofcorrection)[22].Lamb andhiscolleaguesalsosaid7 patientshad recurrenceof deformity(46.7%)[23].Wehadrecurrencein4hands(26.7%)thatseems acceptableandislowerthanotherstudies,comparedwithprevious studies.

Anotherstudyin2008byKanojiaandhiscolleaguesexamined theoutcomesofRCHtypeIIIandIVin18hands.Inthisstudy,soft tissuedistractionbeforecentralizationandtransferofflexorcarpi radialisandflexorcarpiulnaristendonstothefifthfingerwasper- formed.Themeanageattheonsetoftreatmentwas8months.In 16cases,treatmentwascompletedbefore10monthsofage.Inthis study,beforecentralization,softtissueswerestretchedsufficiently andtheresultsafter31monthsaftersurgerywasgoodin7patients, satisfactoryin8cases,andunsatisfactoryinonecase[24].Itshould benotedthatwealsousedstretchingandserialsplintingtreatment ofpatientssixmonthsbeforesurgery,however,ourresultsseemed weaker,comparedtotheresultsofKanojia’sstudy.Itislikelythat bettershort-termfollow-upofstudywasoneofthereasonsforthis difference.

Likethisstudy,Fujiwaraandhiscolleaguespresentedatwo-step methodfortreatingRCHthatcorrectedangulationandprevented necrosisoftherenovatedthumb.Inthismethod,thesurgeonused centralizationwiths-shapeincisiononthedorsumofthehandand one-thirdofdistalforearm.ThenFCRandECRwerereleasedand ECUwasretractedtothedistalside.FCU⁸wastransferredtoECU andwristswerefixedbya1.8-mmpin.Woundwasclosedusing rotationalflapandlimbswereimmobilized inalong castfor 4 weeks.Inthesecondstage,theindexfingerwasusedforpolliciza- tionat31monthsofagethatwascarriedoutwithoutremovalofthe primarypin.Ifthepindoesnotbreak,itwasdrawn14monthsafter pollcization.Inthisstudy,4patientsweretreatedandnorecurrent

8 FlexorCarpiUlnaris.

deformitywasobservedafterfouryearsandtheresultswereexcel- lent.Justbecauseoflong-termmaintenanceofpins,therewasarisk ofbreakingpinsandpintractinfection[25].Itshouldbenotedthat thereasonofnocasesofrecurrenceinthisstudycouldbeduetothe limitednumberofpatients.Inaddition,long-termpreservationof pincanalsogreatlyhelppreventtherecurrenceofdeformity,but itissopainfulandincreasestheriskofinfection.Fujiwaraandhis colleaguesalsodidnotmentiontoanystiffnessofthejoint.

Paley et al in 2008 reported on 21 hands in 14 patients who underwent ulnarization between2000 and 2006. Theage ofpatientsrangedfromoneto14years(mean6years.)with46 monthsfollowup.Wristdorsiﬂexion(passive),arcofmotionand ﬂexioncontracture,hand-to-forearmangleandposition,palmar carpusdisplacement,ulnarlengthimprovedafterPaleyulnariza- tion.Recurrenceofdeformity,skinnecrosisandgrowtharrestofthe ulnarepiphysiswasnotobserved.Overgrowthofthedistalulna relativetothecarpusand excessiveulnardeviationreportedas thebiggestproblemsofulnarization.Topreventthiscomplication, Paleyrecommendedtoreducedistractionforceontheulnarhead byulnarshortening[26].

Romana,C.treated 13patientsmeanageof37.5bydistrac- tionofminirailexternalﬁxator.Thismethodprovidedsufﬁcient distractionintheconcavityofthedeformitytoallowsatisfactory correctioninallcases.Meandistractiontimewas53.2daysand ulnarosteotomy wasrequiredin 8cases (61%).They improved sagittalandcoronalcorrectionaftercentralization[27].

Manske,M.C. etalin2014recordedastudytoevaluatethe effectofsofttissuedistractiononrecurrenceofdeformityaftercen- tralizationforradiallongitudinaldeficienc.13upperlimbstreated withcentralizationalonewerecomparedwith13treatedwithring externalfixatordistractionfollowedbycentralizationwith2–10 yearsfollowup.TheyobservedCentralization,withorwithoutdis- tractionwithanexternalfixator,correctedalignmentofthewrist.

Distractionfacilitated centralization, butit didnotavoid defor- mityrecurrenceandwasassociatedwithaworseradialdeviation andvolarsubluxationpositioncomparedwithwriststreatedwith centralizationalone[28].

Inourstudy,weusedatwo-stepmethodfortreatmentofRCH.

As mentioned, beforesurgery, soft tissue stretching, and serial splintingwasdone.Atthebeginning,patientswereevaluatedfor comorbidities.Aftersplinting,thefirstsurgery ofwristrealign- ment(centralization)andwristtendontransferweredone.Atthis stage,rotational flap wasused toclosethe wound,which was inthree caseswithminorflapedgenecrosisnoneedforsurgi- calintervention(Fig.2).Then,inthesecondsurgeryforpatients withabnormalitiesgradeIIandIII, tendontransferwasusedto promotethumb’sfunction.Incaseswithoutthumb(gradesIVand V),thesecondthumbwasusedforpollicization,exceptonecase thatwasperformedyoungerthan2yearsofage.Ourstudyisone ofthefewstudiesthathaveexaminedthelong-termresultsofRCH treatment.Weobservedadeformityrecurrencerateof>20^◦ in4 patients(26.7%)thatwasnotsignificantinothercases.Rangeof motioninsagittalandcoronalplanes,wasacceptableinoperated handcomparedwithnormalhandthatprovidedtheabilitytoper- formdailyactivitiestosomeextent.Ofcourse,therangeofmotion inthesagittalplanewasbetterthanthatofcoronalthatisfully justifiableregardingtheradialdeviationofwrist’sdeformity.To evaluatethetreatmentoutcomesandimpactofresidualdeformity onperson’sfunction,weusedDASHscoreandobservedthatthis deformityaffectedtheabilityofindividualstoaconsiderableextent toperformeverydaytasks.Infact,accordingtoourresults,residual deformitydisruptedabout34%oftheperson’sfunction.However,it shouldbenotedthat,ifuntreated,thiseffectsignificantlyincreased.

Anotherpointthatshouldbenotedhereisthatinthetreatment ofRCH,patient’s cooperationandhisfamilyisalsoveryimpor- tant.Inourstudy,threepatientsrequiredthumbsurgery,didnot

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Fig.2.A)FlapnecrosisB)healingofthenecroticarea.

return,whichmaybeoneofthereasonsoftheimpactonfunction outcomesinthisstudy.

Likeallotherstudies,thisstudyhadalsolimitations.Oneofthe mostimportantlimitationsofthisstudywastocomparethepre- andpost-treatmentresultswithoutcontrolgroup.Also,thenumber ofpatientswasnotsufﬁcient,andpatientswerefollowedshortly.

Thus,itseemsnecessarythatfurtherstudiesevaluatemorepatients withlongerfollow-up.

5. Conclusion

TreatmentofpatientswithRCHbyprimarytractionandcen- tralization and pollicization surgery, using the second or third metacarpusortendontransfercangreatlyimprovethedeformity, togain rangeofmotionandacceptablefunctionalability.How- ever,thismethodhasitsownlimitationsandisnotfollowedby thedesiredresults,insomecases.Furtherstudieswithlongterm follow-uparerequiredinthefuture.

Funding

Thisresearchdidnotreceiveanyspeciﬁcgrantfromfunding agenciesinthepublic,commercial,ornot-for-proﬁtsectors.

Ethicalapproval

Theethicalapprovalforthepublicationofthiscaseserieswas exemptedbyourinstitutionbecauseallofthedatawerecollected fromclinicalrecordsandimagingsystemsforroutineperioperative planning.

Consent

Writteninformedconsentwasobtainedfromallofthepatient’s fathersastheyareminors,forpublicationofthiscasereportand accompanyingimages.Acopyofthewrittenconsentisavailable forreviewbytheEditor-in-Chiefofthisjournalonrequest.

Authorcontribution

1-Authorname:FarivarA.Lahiji.

Contribution(Type):Therapist.

2-Authorname:FarhangAsgari.

Contribution(Type):Therapist.

3-Authorname:FatemeMirzaee.

Contribution(Type):Writer.

4-Authorname:ZohrehZafarani.

Contribution(Type):Editingthemanuscript.

5-Authorname:HamidrezaAslani.

Contribution(Type):Editingthemanuscript&Corresponding author.

Registrationofresearchstudies

StudyregisteredwithIranianRegistryofClinicalTrials40883.

Guarantor

HamidrezaAslani.

Provenanceandpeerreview

Notcommissioned,externallypeerreviewed.

DeclarationofCompetingInterest

thorsdeclarenoconflictsofinterest.Theauthorshavenofinan- cial,consultative,institutionalandotherrelationshipsthatmight leadtobiasorconflictofinterest.

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