Rev. bras. ortop. vol.49 número4

rev bras ortop.2014;49(4):401–404

w w w . r b o . o r g . b r

Case

Report

Garré’s

sclerosing

osteomyelitis:

case

report

夽

,

夽夽

Frederico

Barra

de

Moraes

,

Tainá

Melo

Vieira

Motta

,

Alessandra

Assis

Severin

,

Deniel

de

Alencar

Faria

_,

_Fernanda

_de

_Oliveira

_César

_,

_Siderlei

_de

_Souza

_Carneiro

a_{DepartmentofOrthopedicsandTraumatology,SchoolofMedicine,UniversidadeFederaldeGoiás,Goiânia,GO,Brazil} b_{AnatomopathologyService,SchoolofMedicine,UniversidadeFederaldeGoiás,Goiânia,GO,Brazil}

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Articlehistory: Received22April2013 Accepted12July2013 Availableonline24April2014

Keywords:

Osteomyelitis/diagnosis Osteomyelitis/surgery Osteomyelitis/therapy

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TheaimofthisstudywastoreportonararecaseofGarré’ssclerosingosteomyelitis.The patientwasa54-year-oldwomanwithahistoryoftreatmentforlupususingcorticoidsfor 20years,andforosteoporosisusingalendronateforfiveyears.Shepresentededemaand developedalimitationofleftkneemovementoneyearearlier,withmildeffusionandpain onmetaphysealpalpation,butwithoutfever.Shewasinagoodgeneralstate,withoutlocal secretion.Imagesofherkneeshowedtrabecularosteolysisofthedistalmetaphysisofthe femurandaperiostealreactioninbothproximaltibiasandbothdistalfemurs, compati-blewithchronicosteomyelitisoflowvirulenceandslowprogression.Magneticresonance imagingshowedT2hypersignalinthefemurandtibia.Curettagewasperformedonthe leftdistal femur,withreleaseofsecretion,butthiswasnegativeonculturing.Abiopsy showedchronicinfectionandinflammation,fibrosis,xanthogranulomatousreactionand fociofsuppuration.Antibiotictherapywasadministeredforsixmonths.Theetiologywas notclarified:bacterialinfectionwassuspected,butculturingwasgenerallynegative.The chronicprocesswasmaintainedbylow-virulenceinfectionorevenaftertreatment.The dif-ferentialdiagnoseswerefibrousdysplasia,syphilis,pustulosispalmoplantaris,rectocolitis, Crohn’sdisease,SAPHO(synovitis,acne,pustulosis,hyperostosisandosteitis)andPaget’s disease.Theunifocaldiseaseswereosteoidosteoma,Ewing’sdisease,osteosarcomaand eosinophilicgranuloma.

©2014SociedadeBrasileiradeOrtopediaeTraumatologia.PublishedbyElsevierEditora Ltda.Allrightsreserved.

Osteomielite

esclerosante

de

Garrè:

relato

de

caso

Palavras-chave:

Osteomielite/diagnóstico Osteomielite/cirurgia Osteomielite/terapia

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RelatarumcasorarodeosteomieliteesclerosantedeGarrè.Pacientefeminino,54anos, comhistóriadetratamentodelúpuscomcorticoidehavia20anoseosteoporose,emuso dealendronatohaviacincoanos.Apresentavaedemaelimitac¸ãodojoelhoesquerdohavia umano,derrameleve,doràpalpac¸ãometafisária,afebril,bomestadogeral,semsecrec¸ão local.Imagensdojoelhoevidenciaramosteólisetrabeculardametáfisedistaldofêmure reac¸ãoperiostealnasduastíbiasproximaisenosdoisfêmuresdistais,compatíveiscom

夽_{Pleasecitethisarticleas:deMoraesFB,MottaTMV,SeverinAA,deAlencarFariaD,deOliveiraCésarF,deSouzaCarneiroS.Osteomielite}

esclerosantedeGarrè:relatodecaso.RevBrasOrtop.2014;49:401–404.

夽夽_{WorkperformedintheDepartmentofOrthopedicsandTraumatology,SchoolofMedicine,UniversidadeFederaldeGoiás,Goiânia,}

GO,Brazil.

∗ _{Correspondingauthor.}

E-mail:fredericobarra@yahoo.com.br(F.B.deMoraes).

2255-4971/$–seefrontmatter©2014SociedadeBrasileiradeOrtopediaeTraumatologia.PublishedbyElsevierEditoraLtda.Allrightsreserved.

402

osteomielitecrônica,debaixavirulênciaeprogressãolenta.HipersinalemT2nofêmur etíbiaàressonância.Curetagemdofêmurdistalesquerdo,comsaídadesecrec¸ão,mas culturanegativa.Biópsiaevidenciouinfecc¸ãoeinflamac¸ãocrônica,fibrose,reac¸ão xan-togranulomatosaefocosdesupurac¸ão.Feitaantibioticoterapiaporseismeses.Etiologianão esclarecida,suspeitadeinfecc¸ãobacteriana,masgeralmenteaculturaénegativa,processo crônicomantidoporinfecc¸ãodebaixavirulênciaoumesmoapósotratamento. Diagnósti-cosdiferenciais:displasiafibrosa,sífilis,pustulosepalmoplantar,retocolite,Crohn,Sapho (sinovite,acne,pustulose,hiperostose,osteíte)ePaget.Unifocais:osteomaosteoide,Ewing, osteossarcomaegranulomaeosinofílico.

©2014SociedadeBrasileiradeOrtopediaeTraumatologia.PublicadoporElsevier EditoraLtda.Todososdireitosreservados.

Introduction

Garré’sosteomyelitisisarareinflammatorydiseaseofchronic nature,characterizedbyperiostealreactions,whichinduces bone neoformation. It mainly affects the region of the mandibleand,inrarecases,maybelocatedinthe metaphy-sealregionofthelongbones.Thisdiseaseisalsoknownas Garré’ssclerosingosteomyelitis(GSO),chronicosteomyelitis withproliferativeperiostitis,chronicsclerosing osteomyeli-tis,ossifyingperiostitisornon-suppurativechronicsclerosing osteomyelitis.1–3_{Theclinicalconditionischaracterizedbyan} insidiousstart,withlocalpain andreactionintheaffected bone.Thesymptomshaveanepisodicnon-progressivenature and may persist for several months. On the other hand, the duration ofGSO is very variable among patients, who may present the disease for several years. The function of the affected bone is generally preserved and, during the interval between crises, most patients are seen to be healthy.4,5

TheaimofthisstudywastoreportonararecaseofGarré’s sclerosingosteomyelitis,inwhichtheproximalregionofthe tibiasanddistalregionofthefemurswereaffectedina 54-year-oldpatient.

Case

report

Thepatientwasa54-year-oldwomanwithahistoryof treat-mentforlupususingcorticoid,overthepreceding20years. Shepresentedsevereosteoporosisandhadbeenusing alen-dronate for five years. The specific clinical condition had beenevolvingovertheprecedingyear,withpain,edemaand chroniclimitationofmovementinherleftknee,which pre-sentedslightjointeffusionandpainonpalpationofthedistal femoralmetaphysis.Shewasnotfebrile,inagood general stateandwithoutreddeningordischargeofsecretionsatthe site.

Radiographsandtomographicscanswereproducedonthe leftknee.Theimagesshowedtrabecularosteolysisofthe dis-tal metaphysisofthe left femurand periostealreaction in bothproximaltibiasandbothdistalfemurs.Thiswas com-patiblewithchronicosteomyelitis,oflowvirulenceandslow progression(Figs.1A–C,2A–Cand3A–B).Theimaging diag-nosiswascomplementedbymeansofmagneticresonance, whichshowedT2hypersignalbothinthefemurandinthe tibia,thusindicatingGSO.

Surgicaltreatmentconsistingofcurettageoftheleft dis-talfemurwasperformed.Puncturingthekneejointdidnot show anypurulentsecretion.Thebonewindowthusmade subsequently dischargedsecretion,but thisproduced nega-tive cultures. Thematerial obtained from the surgery was sentforbiopsyanalysis,whichshowedinfectionandchronic inflammationmarkedbyfibrosis,xanthogranulomatous reac-tion,fociofsuppurationandabsenceofacid-alcoholresistant bacilli(AARB),fungiorsignsofmalignity(Fig.4).

The patient underwent venous antibiotic therapy with oxacillin(4g/day)andsubsequentlyoralmedication consist-ingofcephalexin(2g/dayforsixmonths),withimprovement ofinflammationintheleftknee.

Discussion

Carl Alois Philipp Garré was a Swiss surgeon and bacteri-ologist who in 1893 published an article dealing with the manifestationsofosteomyelitis.Hisnamebecameassociated withthedisease,whichbecameknownasGarré’ssclerosing osteomyelitis,althoughhewasnotresponsiblefor describ-ingit.1,2 _{Hereportedthattherewasperipheralformationof} a bone reaction due to irritation or mild infection, which resultedinthickeningoftheperiosteumoflongbones.The etiologyofthisdiseasehasstillnotbeenfullyclarified. Bac-terial infection issuspected, but the culturesare generally negativeandthechronicprocessmaybemaintainedthrough low-virulenceinfectionorevenaftertheinfectionhasbeen treated. If the germ cannotbe detectedthrough culturing, investigationbymeansofthepolymerasechainreaction(PCR) shouldberequested.3

Amultifocalsclerosingbonereactionwithclinical, radio-logical and histologicalcharacteristics similar to GSO may beencounteredinseveral diseases,suchasfibrous dyspla-sia,syphilis,pustulosispalmoplantaris,ulcerativerectocolitis, Crohn’sdisease,SAPHOsyndrome(synovitis,acne, pustulo-sis,hyperostosisandosteitis)andPaget’sdisease.Additional differential diagnoses in cases of unifocal sclerosing bone reaction may include osteoid osteoma, Ewing’s disease, osteosarcomaandeosinophilicgranuloma.3,4

r e v b r a s o r t o p . 2014;49(4):401–404

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Fig.1–Radiographicappearanceofthelesion.(A)Radiographinanteroposteriorviewshowingperiostealreactioninthe femur.(B)Radiographinlateralviewshowingperiostealreactioninthetibia.(C)Radiographinlateralviewshowingdistal femuraftertheoperation,withabonewindowfordrainage,curettage,culturingandbiopsy,andshowingthevarious layersoftheperiostealreaction.

Fig.2–Computedtomographyoftheleftknee,withbonewindow,showingtrabecularosteolysisandperiostealreaction. Coronal(A),sagittal(B)andaxialslice(C).

osteomyelitis.5,6_{Anotherpointofinterestinthepresentcase} canbehighlighted:useofcorticoidsisanimportant trigger-ingfactorforosteoporosis.Thus,itisreasonabletosuppose thatthetreatmentforlupusinthepresentcasemayhave con-tributedtowardtheappearanceofosteoporosisand,together, thetwodiseasesmayhavefavoreddevelopmentofGSO.

Chronicosteomyelitispresentslowmortalitybuthigh mor-bidity.Clinically,thechronicphaseisalwaysprecededbyan

acuteinfectiousprocess,withphlogisticsigns(pain,heat, red-ness,tumorgrowth,deformityand limitation).However,in patientswhohavebecomedebilitatedthroughotherdiseases, aswasthecaseinourpatient,theinitialconditionmaybe masked,whichhastheconsequencethatthediagnosiswill onlybecomepossibleinthechronicphase.7

Inrelationtotheimagingdiagnosis,Vasilievetal.8 eval-uated 121 patients between the ages of 4 and 74 years,

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Fig.4–Histologicalstudyonbonebiopsyfromtheleftfemur,inhematoxylin-eosin(A)and(B),showinginfectionand chronicinflammationmarkedbyfibrosis,xanthogranulomatousreactionandfociofsuppurationindicativeofGSO.

withadiagnosisofosteomyelitisinlimbs.Themostprecise examination was spiral computed tomography, which had anaccuracyof96.7%,sensitivityof99.1%and specificityof 80%,whileradiographspresented81.8,84.9and60%, respec-tively.Inthebeginning,acombinationofscleroticareasand cysticareas couldbeobservedinthe regionofthe lesions, andthe latterisgraduallyreplacedbyscleroticareas. Dur-ingexacerbations,boneformationduetoperiostealreaction could be seen, resembling an onion skin. Thus, the dis-ease ismarked bythickening and sclerosis ofthe affected bone.3,8

Surgical treatment in association with antibiotic ther-apy is the best option for chronic osteomyelitis. Antibi-otic therapy alone is ineffective, independent of the administration route, because the “bone sequestration” found in the chronic disease comprises fragments of necrotic bone and thus does not present a blood sup-ply that would allow antibiotic to arrive at the infected tissue.3,7

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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1.GarrèCA,BorchardA.LehrbuchderChirurgie.London:Oxford UniversityPress;1920.

2.WoodRE,NortjéCJ,GrotepassF,SchmidtS,HarrisAM. PeriostitisossificansversusGarrè’sosteomyelitis.PartI.What didGarrèreallysay?OralSurgOralMedOralPathol.

1988;65(6):773–7.

3.VienneP,ExnerGU.Garrèsclerosingosteomyelitis.Orthopade. 1997;26(10):902–7.

4.Bernard-BonninAC,MartonD,BrochuP.Chronicsclerosing osteomyelitis(so-calledGarrè’s).Reviewof12cases.ArchFr Pediatr.1987;44(4):277–82.

5.VieiraCL,AyresdeMeloVEV.Osteomielite:relatodecaso clínico.IntJDent.2006;1(1):35–40.

6.XavierR,VecchiAA.Infecc¸õesosteoarticulares.In:HebertS, XavierR,PardiniJúniorAG,BarrosFilhoTEP,editors.Tratado deortopediaetraumatologia:princípiosepráticas.3rded. PortoAlegre:Artmed;2007.p.822–30.

7.SumaR,VinayC,ShashikanthMC,SubbaReddyVV.Garrè’s sclerosingosteomyelitis.JIndianSocPedodPrevDent.2007;25 Suppl.:S30–3.