w w w . r b o . o r g . b r
Case
Report
Surgical
features
of
Trevor’s
disease
of
the
patella
夽
,
夽夽
Frederico
Barra
de
Moraes
∗,
Geraldo
dos
Passos
Barcelos
Filho,
Guilherme
Felipe
Faria
Lobo,
Cícero
Almeida
Santos,
Luiz
Augusto
Alves
Reis,
Mauro
Rodrigues
dos
Santos
FaculdadedeMedicinadaUniversidadeFederaldeGoiás,Goiânia,GO,Brazil
a
r
t
i
c
l
e
i
n
f
o
Articlehistory:
Received1May2013 Accepted6March2014 Availableonline12July2014
Keywords:
Osteochondrodysplasia Boneneoplasia Knee
a
b
s
t
r
a
c
t
Theaimofthisstudywastodescribesurgicalfeaturesofresectionofhemimelicepiphyseal dysplasiaofthepatella.Wealreadydescribedtheclinicalandimagingfeaturesinanother article.Thepatientwasasix-year-oldboywithatumorinhisrightkneemeasuring12cm longitudinallyand6cmtransversally,whichwasadheringtothe patellaandhadbeen slowlygrowingfortwoyears.Biopsyfindingsweresuggestiveofabenign osteochondro-matouslesion,withoutadefineddiagnosis.Imagingexaminationssuchasradiographyand tomographyshowedareasofboneformationandradiotransparentareas,whilemagnetic resonanceimagingshowedareasofhypoandhypersignalinT1andT2,ofestimatedsize 8.5cmlongitudinallyand6cmtransversally.Thetumorgrowthwassurgicallyresectedand curettagewasperformedontheepiphysealnucleusofossificationoftheupperandmedial centersofthepatella,withgoodpatellarremodelingandnormaldevelopment.Thepatient didnotpresentanyrecurrenceofthelesionuptothetimeofreachingskeletalmaturity.
©2014SociedadeBrasileiradeOrtopediaeTraumatologia.PublishedbyElsevierEditora Ltda.Allrightsreserved.
Aspectos
cirúrgicos
na
doenc¸a
de
Trevor
da
patela
Palavras-chave:
Osteocondrodisplasias Neoplasiasósseas Joelho
r
e
s
u
m
o
Descreveraspectoscirúrgicosdaressecc¸ãodedisplasiaepifisáriahemimélicadapatela. Osaspectosclínicosedeimagemjáforamdescritosemoutroartigo.Pacientemasculino, seisanos,comtumordecrescimentolentoemjoelhodireito,pordoisanos,com dimen-sõesde12cmnoeixolongitudinale6cmnotransversal,aderidoàpatela.Biópsiasugeriu lesãoosteocondromatosabenigna,semdiagnósticodefinido.Examesde imagem,como radiografiasetomografias,comáreasdeformac¸ãoósseaeáreasradiotransparentesena ressonânciamagnéticaáreas dehipoe hiperssinal em T1eT2, detamanho estimado em8,5cmnoeixolongitudinale6cmnotransversal.Foramfeitasressecc¸ãocirúrgicada
夽
Pleasecitethisarticleas:deMoraesFB,FilhoGPB,LoboGFF,SantosCA,ReisLAA,dosSantosMR.Aspectoscirúrgicosnadoenc¸ade Trevordapatela.RevBrasOrtop.2014;49:414–419.
夽夽
WorkperformedintheDepartmentofOrthopedicsandTraumatologyoftheSchoolofMedicine,FederalUniversityofGoiás,Goiânia, GO,Brazil.
∗ Correspondingauthor.
E-mail:[email protected](F.B.deMoraes). http://dx.doi.org/10.1016/j.rboe.2014.06.002
Hemimelicepiphysealdysplasiaisaveryrarediseaseof epi-physealosteocartilaginous developmentthat occurs during children’sgrowth.ItwasfirstdescribedbyMouchetandBelot1
in1926,withthenametarsomegaly,becauseoftheincreased volumein the regionof the ankle, and then byTrevor2 in
1950,astarsoepiphysealaclasia,sincethelesionsfrequently occurredintheepiphysealregionofthefootandankle.In1956, Fairbank3reportedfurthercasesanddescribedthiscondition
ashemimelicepiphysealdysplasia(HED),sinceitcouldoccur inotherjoints.ThefirstcaseofHEDoriginatinginthepatella wasonlydescribedin1980,inSpain,byEnriquezetal.4
In2003,weoperatedonasimilarcaseattheKneeSurgery ServiceoftheDepartmentofOrthopedicsandTraumatology, SchoolofMedicine,FederalUniversityofGoiás.Theclinical and imagingcharacteristics ofthiscase werepublished by Araújoetal.in2006,5butthesurgicalaspectsofthecasewere
notaddressed.ThetreatmentforHEDgenerallyconsistsof resectionofthelesioninordertoavoidpainandlimitations onjointmovements,and deformitiesthatcouldgenerate a conditionofearlyosteoarthritis.Theobjectiveofthepresent studywastoreportonthesurgicalaspectsofthatcaseofHED ofthepatella.
Case
report
Thepatientwasasix-year-oldmalewithahistoryofslowly growingbut progressive tumorformationinhisright knee overatwo-yearperiod.Thetumormeasuredapproximately 12cmlongitudinallyand6cmtransversallyandwas adher-ingtothepatella.Itwasassociatedwithaconditionofpain, edema,claudication,moderatehypotrophyofthequadriceps and movementlimitation,suchthat flexion–extensionwas limitedoveritslast30◦.Mildjointeffusionwaspresent, with-out pain on palpation ofthe metaphysis. Thepatient was not feverish and in a good generalstate, without redness orsecretionsatthesite.Abiopsysuggestedthatthiswasa benignosteochondromatouslesion,withoutadefined diag-nosis(Fig.1).
Over two years of evolution of this lesion in the right knee,therewasalargeincreaseinthepatellartumormass onimagingexaminations,withareasofboneformationand radiotransparentarea.Theestimatedsizeofthetumorwas 8.5cmlongitudinally,6cmtransversallyand3.5cmindepth (Figs.2and3).
Thesurgicalprocedurewasstartedwiththepatientin dor-saldecubitusundergeneralanesthesia,withasepsis,sterile fieldsandapneumatictourniquetappliedtotherightthigh.
1. A longitudinal lateral parapatellar incision of approxi-mately10cmwasmade,withdissectioninlayers(Fig.4A). 2. Thesuprapatellarquadricepstendonwaspushedbackto the lateral region ofthe knee, together with the vastus medialismuscle,whichwasfoundtohavebulgedoutdue tothetumorformation(Fig.4B).
3. Medialcapsulotomywasperformedasfarastheplaneof thetumor.Asuperficialcartilaginouscoverandbone pedi-clewereobservedattheupperandmedialcenterofthe patella,andthesewereresected(Figs.4Cand5A). 4. Surgical cleaning was performed, with curettage of the
nucleusofepiphysealossificationattheupperandmedial centerofthepatella,inordertoavoidrecurrenceofthe lesion(Fig.5BandC).
5. Hemostasis,capsulorrhaphy,reinsertionofthequadriceps tendon in the patella, suturing inlayers, skin suturing, application of dressings and immobilization were per-formed.
Thepatientcontinuedtobefollowedupatourservice,and theconditionofHEDwasseentobecomeclinicallyand radio-logicallyresolved,withoutanyrecurrenceofthe lesionand withoutanydeficitoffunctioningofthekneeextensor mech-anismuptotheendoftheskeletalmaturationprocess.The patientreturnedtohisnormalactivities(Figs.6and7).
Discussion
HED is a disease of unknown etiology that is generally restricted tothemedialorlateral sideofthe affectedlimb andwhichceasestoevolvefurtherwhenskeletalmaturityis reached.Inthecasereportedhere,itwasfoundonthemedial lineofthelimb,sinceitoriginatedattheupperandmedial centerofthepatellaoftherightknee.HEDoccursmore fre-quentlyamongmales,withproportionsofthreemalecases toeachfemalecase.Itgenerallyarisesinchildrenor adoles-centsbetweentheagesof2and14years,anditisonlyrarely diagnosedatolderages.Itsestimatedincidenceisonecasein everyonemillionindividuals,butfewerthan300caseshave beendescribedintheliterature.6,7
Fig.1–Preoperativeappearance,withmedialview(A)andlateralview(B)oftherightkneewithpatellartumorformation:
hemimelicepiphysealdysplasia.
Fig.2–Preoperativeappearanceonlateralradiographsoftherightknee,withpatellartumorformationshowingonemonth
ofevolution(A),oneyear(B)andimmediatelybeforethesurgery(C):hemimelicepiphysealdysplasia.
presentedanunusuallocation,originatingattheupper cen-terofthepatella,anditsclinicalandimagingcharacteristics weredescribedpreviously.5
Tumorsoriginatingfromthepatellaarerare;70%ofthem arebenignand theyneedtobeapproachedfrom the clini-cal,imaging,surgicalandhistologicalpointsofviewforthese casestobeconcludedinthebestwaypossible.Thereport pre-sentedshowedthatbiopsypriortosurgeryrevealedabenign osteocartilaginouslesion,butwithoutafinaldefinition.The
differential diagnosis in relation to HED should be made withseveralothertypesoftumor,including:osteochondroma, chondroblastoma,intracapsularchondroma,giant-celltumor, tumoral calcinosis and synovial chondromatosis; and also withnon-tumorallesionssuchas:ossifyingmyositis,vascular or parasiticcalcification,tuberculosisandneurological syn-dromes(CINCAorCharcot).10–12
Imaging examinations(radiographs,computed tomogra-physcansandmagneticresonanceimaging)maysuggestthe
Fig.3–Preoperativeappearanceonaxialcomputedtomographyslice(A)andonT2-weightedsagittalmagneticresonance
slice(B)oftherightkneewithvoluminouspatellartumorformation,immediatelybeforesurgery:hemimelicepiphyseal
Fig.4–Intraoperativeappearanceoftheresectionofthepatellartumorformation(hemimelicepiphysealdysplasia),
showinglaterallongitudinalparapatellarincision(A),dissectionatmuscle-tendonlayer(B)andintracapsularlayer(C).
diagnosisofHED,especiallyintheusualregionssuchasthe footand ankle. However,when thelesion isinanunusual location, such as the patella, this becomes more difficult. Thenucleusofepiphysealossificationofthepatellaappears aroundtheageoffourtofiveyearsinmalesandattheageof threeyearsinfemales.13,14
ThetreatmentforHEDcanbeclinicalorsurgical, depend-ingonthesymptoms.Thereisnosingleguidelineregarding thetreatment,butmostauthorshavereportedresectingthe lesion, which shows that observation is only for asymp-tomaticcases. Clinicaltreatmentisless invasive,and does
not have the risks of surgery. Nevertheless, surgical treat-mentproducesbetterresultsinthefollowingsituations:(1) compressionofnerves,arteriesortendons;(2)exostosisthat interfereswithgrowthoftheextremity;(3)functional limi-tationsofthejoint;and(4)deformitiesandpain.Thetumor growth isgenerallyaggressiveandsothetreatmentshould alsobeaggressive;moreover,thisapproachistakeninorder toavoidpain,deformities,limitationsandearly osteoarthro-sis.Recurrenceiscommonaftersimpleexcisionofthetumor. Extracapsularcasespresentbetterresultsthanintracapsular cases.15–17
Fig.5–Intraoperativeappearanceoftheresectionofthepatellartumorformation(hemimelicepiphysealdysplasia),
showingexcisionofthepedunculatevoluminousmasswithosteocartilaginouscharacteristics(A),surgicalremovalofthe
Fig.6–Appearanceoneyearaftertheoperation,withanteriorview(A)andlateralview(B)oftherightknee,withfull
extensionandflexion:hemimelicepiphysealdysplasia.
Fig.7–Appearanceoneyearaftertheoperation,onradiographsoftherightkneeinaxialview(A)andlateralview(B),
showingadequategrowthofthepatella.
Regarding the possible typesof surgery for suchcases, thesecanbedividedintofourtypes,accordingtothe aggres-sivenessofthetumor.Thefirsttypeisarthroscopicresection, whichpresentsleastmorbiditybuthasagreaterchanceof recurrence and has greater technical difficulty in children. Openresectionofthetumorformationenablesalarger sur-gicalfieldandshouldalwaysbeaccompaniedbycurettageof thecompromisednucleusofossification,inordertoreduce therecurrencerate.Surgicalproceduresofgreater aggressive-ness,suchashemipatellectomyandtotalpatellectomycan beperformedinordertoavoidlesion recurrenceandearly osteoarthrosis,butimplylossofthepotencyoffunctionof thekneeextensormechanismand,becausethisisinachild, hypoplasiaofthefemoralcondyles.4,10,17
Our patient presenteda very voluminous lesion,which wasresectedusinganopenprocedure,atitspedicle.Toavoid
recurrenceoftumorformation,weperformedsurgical clean-ingandcurettageofthenucleusofepiphysealossificationof the uppercenter ofthe patella. Therisk ofthis procedure isthat thepatellamay developabnormally.However,what weobservedwasgoodremodelingofthepatellaandnormal development,probablybecauseofthepatient’syoungageand thepotentialofhisimmatureskeleton,withcontinual stimu-lationofthekneeextensormechanism,giventhatthepatella isasesamoidboneandfunctionsasapulley.Ourpatientdid notpresentanyrecurrenceuptothetimeofcompletinghis skeletalmaturation.
Conflicts
of
interest
4. EnriquezJ,QuilesM,TorresC.Auniquecaseofdysplasia epiphysealishemimelicaofthepatella.ClinOrthopRelatRes. 1981;(160):168–71.
5. AraujoJrCR,MontandonS,MontandonC,TeixeiraKI,Moraes FB,MoreiraMA.BestcasesfromtheAFIP:dysplasia
epiphysealishemimelicaofthepatella.Radiographics. 2006;26(2):581–6.
6. Wynne-DavisR,HallCM,ApleyAG.Dysplasiaepiphysealis hemimelica.In:Wynne-DavisR,HallCM,ApleyAG,editors. Atlasofskeletaldysplasias.NewYork:ChurchillLivingstone; 1985.p.539–43.
7. KircherJ,WesthoffB,BittersohlB,ZiskovenC,KrauspeR.A rarecaseofTrevor’sdisease(dysplasiaepiphysealis hemimelica)intheelbow.JShoulderElbowSurg. 2013;22(2):e12–5.
8. AzouzEM,SlomicAM,MartonD,RigaultP,FinidoriG.The variablemanifestationsofdysplasiaepiphysealis hemimelica.PediatrRadiol.1985;15(1):44–9.
OnikulE.MRimagingofdysplasiaepiphysealishemimelica: bonyandsoft-tissueabnormalities.AmJRoentgenol. 1999;172(3):819–23.
14.LinYH,ChouYJ,YehLR,ChenCKH,PanHB,YangCF. DysplasiaepiphysealishemimelicaorTrevor’sdisease:acase report.ChinJRadiol.2001;26(5):215–20.
15.KeretD,SpatzDK,CaroPA,MasonDE.Dysplasiaepiphysealis hemimelica:diagnosisandtreatment.JPediatrOrthop. 1992;12(3):365–72.
16.StrujisPA,KerkhoffsGM,BesselaarPP.Treatmentofdysplasia epiphysealishemimelica:asystematicreviewofpublished reportsandareportofsevenpatients.JFootAnkleSurg. 2012;51(5):620–6.
