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Highlights

FootandAnkleSurgeryxxx(2014)xxx–xxx

Short-termandmid-termoutcomeoftotalanklereplacementinhaemophilic patients

J.G.Asencioa_*,_C._Leonardia_,_C._Andreani_Birianib_,_J.F._Schveda

a

HoˆpitalPrive´ lesFranciscaines,Nıˆmes,France b

CentreRe´gionalduTraitementdel’He´mophilie,CHUMontpellier,France

Reportoftheexperienceontheuseof32prosthesesinhaemophilicpatients.

Gainintherangeofmotionduringdorsiﬂexionisimportantforgaitcycle.

7patientswereHIVpositive,butnoinfectionsoftheanklejointinourseries.

Limitations:retrospectivestudy,presentingonlyamedium-termreview.

ContentslistsavailableatScienceDirect

Foot

and

Ankle

Surgery

(3)

1

2

Short-term

and

mid-term

outcome

of

total

ankle

replacement

in

3

haemophilic

patients

4 Q1

J.G.

Asencio

a,

*

,

C.

Leonardi

a

,

C.

Andreani

Biriani

b

,

J.F.

Schved

a

5 a_Hoˆpital_Prive_{´ les}_{Franciscaines,}_Nıˆmes_,_France

6 b

CentreRe´gionalduTraitementdel’He´mophilie,CHUMontpellier

Q2 ,France

7

8 1. Introduction

9 Haemophilia Aand Bare X-linked clottingdisorders caused 10 either by factor VIII (FVIII) or IX (FIX) deﬁciency; signiﬁcant 11 reductionintheseclottingfactors(<1IU/dL)leadstospontaneous 12 bleedingintojointsandmuscles.

13 Recurrent bleeding intothe same ‘‘target’’joint[1] leads to 14 inﬂammatory changes [2], synovial proliferation and eventual 15 chronicsynovitis[1,3].

16 Small,recurrenthaemorrhagescancausedegenerativelesions 17 thatgraduallyprovidethesiteforanarthropathy.

18 Accompanying altered joint motion and poor alignment of 19 component bones due to damaged epiphyseal growth plates 20 exacerbatetheprogressionofthearthropathy.Thesefactors[1]

21 canalsoaffecttheoverlyingkneeandhipbyaddinganabnormal 22 mechanicalconstraint tothemultiplejointsin thelower limbs 23 (analysisofgaitandtread)[4].

24 Jointsintheelbows,kneesandankles[5]aremostaffectedby 25 bleeding[6].Theankleistheﬁrsttargetjointinchildhood[7]and 26 isthemostfrequentlyaffectedjointintheseconddecadeoflife

27

[8,9],characterisedbypain,stiffnessanddeformity.Itisthemost 28 prevalentcauseofmorbidityinpatientswithseverehaemophiliaA

29

[7]oftenassociatedwithmobility-reducingfoot/ankledeformities 30

[10]thatinterferewithdailyactivitiesandreducethequalityof 31 life.

32 Regularprophylaxiswithconcentratedclottingfactorsisthe

33 most effective method of preventing haemophilic arthropathy

34

[11]. To prevent recurrent bleeding, radio synovectomy or 35 chemical synovectomycanbeconsideredat anearlystage[12]

36 and, if unsuccessful, arthroscopic synovectomy. Nonetheless,

37 cautiousmanagementandconservativetreatmentaresometimes

38 inadequate,andinvasivesurgerymayberequired.

39 The current standard treatment for patients with painful,

40 progressivearthropathyistibio-talararthrodesis[10–13],which

41 demonstratesgoodresultsintermsofpainandbleedingepisodes

42 butwithlossofmobility[13].Basedoninitialreports[6,7],which

43 arenowoutdated,totalanklereplacement(TAR)isalsoindicated

44

[12,14,15] in a limited number of cases, but itsvalue is under 45 debate.

46 Thedevelopmentofthird-generationprostheses(cementless,

47 withthreecomponentsusingcross-linkedpolyethylenemeniscal

48 bearings) shouldmake it possibleto conservemobility, restore

49 rollingandrotation,andprotectotheroverlyingandunderlying

50 joints[16–19].

FootandAnkleSurgeryxxx(2014)xxx–xxx

ARTICLE INFO

Articlehistory:

Received8November2013 Receivedinrevisedform30May2014 Accepted9August2014

ABSTRACT

Background: Ankle arthropathy is very frequent in haemophilic patients. Prostheses are valuable alternativestoarthrodesisinnon-haemophilicpatients.Wereporttheexperienceofasinglecentrein Franceontheuseofprosthesesinhaemophilicpatients.

Methods:Retrospectivestudyof21patientswithhaemarthropathywhounderwentanklearthroplasty (32ankles),withadditionalsurgery,ifneeded,fromJuly2002toSeptember2009(meanfollow-up 4.41.7years).TheAmericanOrthopaedicFootandAnkleSociety(AOFAS)ankle–hindfootscalewasusedto evaluatepain,function,anklemobilityandalignment.

Results:TheoverallAOFASscoreimprovedfrom40.219.4(pre-surgery)to85.311.4(post-surgery). Thefunctionscoreincreasedfrom23.67.7to35.96.7anddorsiﬂexionfrom0.385.08to10.384.48. Twopatientsunderwentfurtheranklearthrodesis.OnX-ray,bothtibialandtalarcomponentswerestable andcorrectlyplacedinallankles.Alignmentwasgood.

Conclusion:Anklearthroplastyisapromisingalternativetoarthrodesisinhaemophilicpatients. ß2014PublishedbyElsevierLtdonbehalfofEuropeanFootandAnkleSociety.

* Correspondingauthor.Tel.:+33466620787

Q3 .

E-mailaddress:c.asencio@wanadoo.fr(J.G.Asencio).

G Model

FAS7471–8

Pleasecitethisarticleinpressas:AsencioJG,etal.Short-termandmid-termoutcomeoftotalanklereplacementinhaemophilic patients.FootAnkleSurg(2014),http://dx.doi.org/10.1016/j.fas.2014.08.004

ContentslistsavailableatScienceDirect

Foot

and

Ankle

Surgery

j o urn a lhom e pa g e : ww w . e l se v i e r. c om / l oca t e / f a s

http://dx.doi.org/10.1016/j.fas.2014.08.004

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51 Defects in the mechanical axis of the lower limb, ligament 52 instabilities and other growth defects are contraindications. 53 Additional procedures, such as Achilles tendon lengthening, 54 ligamentreconstruction,subtalarandmidtarsalarthrodesis and 55 malleolarosteotomy,canbecombinedwitharthroplastytocorrect 56 thesevariousdisorders.

57 Good short, medium [2,19–21] and long-term [22–24] out-58 comeshavebeenreported.However,mostofthesepublications 59 werewrittensolelybytheinventorsofthevariousprostheses. 60 Interestingly,neitherarthrodesisnorarthroplastyseemtobe 61 thesolesolution[25].

62 If selected,arthroplasty is preferable as ﬁrst intention,even 63 thoughsomesuggestperformingitafterarthrodesis.Arthrodesisis 64 asolutionifprosthesisfails[26].

65 In haemophilic patients, ankle replacement poses special 66 challenges: preventing postoperative bleeding to provide the 67 most favourable conditions for physiotherapy and to avoid 68 complicationsthatmayrequireadditionalsurgery.

69 StudiesavailableontheoutcomesofTARinasmallnumber 70 of haemophilic patients (1–8) report high levels of patient 71 satisfactionin termsof pain relief, increasedrange of motion 72 and the low rate of complications [2,5,27,28]. Although 73 reporting the outcomes in 531 cases of TAR, the Swedish 74 nationalregistrydoesnotdifferentiateparticularcases[24]and 75 21 separate cases with different aetiologies are grouped 76 together – not onlyhaemophilia, butalso haemochromatosis, 77 psoriasis andother disorders – making it impossible todraw 78 comparisons.

79 Inthispaper,wedescribeourexperienceandthemedium-term 80 outcomesof32TARsin21haemophilicpatients.

81 2. Materialsandmethods

82 2.1. Patients

83 This is a retrospective study involving all patients with 84 haemorrhagic arthropathy who underwent totalankle replace-85 ment(TAR)inourhospital.Thepatientswerefullyinformedand 86 providedtheirwrittenconsentbeforethestartofdatacollection. 87 The protocol was approved by the independent Ethics 88 Committee(Comite´ consultatifsurletraitementdel’information 89 enmatie`rederecherche[CCTIRS],Paris,France).

90 Patients were aged at least 18 at the time of the TAR and 91 presentedwithsequelaeofhaemorrhagicdisease(haemophiliaA, 92 haemophiliaBorvonWillebranddisease).

93 2.2. Clottingfactorreplacementtherapy

94 Clottingfactorreplacementtherapywassupervisedexclusively 95 by the haemophilia centre at all stages of the procedure 96 (preoperative,postoperativeandphysiotherapyperiods). 97 The target level of anti-haemophilia factor was 80% during 98 surgery,decreasingfrom80%to30%duringthepostoperativeand 99 physiotherapy periods.The dose and frequency (once or twice 100 daily) of injections were determined after pharmacokinetic 101 evaluation.

102 If necessary, one bolus of anti-haemophilia factor was 103 administered before surgery and a continuous infusion during 104 surgery.

105 The levelsof FVIIIand FIXweremonitored dailyduring the 106 postoperativeperiodandweeklyduringthephysiotherapyperiod.

107 2.3. Prosthesesandsurgicalprocedure

108 Twonon-constrainedprostheseswiththreecomponentswere 109 used:

111 -AnkleEvolutiveSystem(AES)(Transyste`meSA, Nıˆmes,France) 112 113 for27TARsuntil30June2008,then

114 -Hintegraprosthesis(NewdealSA,Lyon,France)for5TARs. 115 116 Surgeryusingatourniquetwasperformedbytwoexperienced

117 orthopaedicsurgeons.

118 Antibiotics were administered at induction of anaesthesia

119 beforeapplicationofthetourniquet.

120 The approach used to expose the ankle joint was anterior

121 longitudinal, slightly offset laterally in relation to the tibialis

122 anterior muscle. Synovitis, cartilage fragment and subchondral

123 bone,aswellassurroundingosteophytes,wereremoved.

124 The distal tibia and talar dome were cut and prepared for

125 insertion of the trial metal components. Their positions were

126 checked,andthemobilityandstabilityofthejointweremonitored

127 by X-ray with an image intensiﬁer; in addition, the size and

128 thicknessofthepolyethylenebearingwereselectedbyperforming

129 successivetests.

130 If dorsiﬂexion was less than 108, the Achilles tendon was

131 lengthened.

132 Theincisionwasclosed,leavingasuctiondraininplacewhich

133 wasremoved2daysaftertheprocedure.Aremovablebootwasused

134 toallowwoundcareandimmediatephysiotherapy.No

postopera-135 tivepreventiveanticoagulanttherapywasadministered(exceptfor

136 theﬁrst2cases,afterwhichthisprophylaxiswaseliminated).

137 Anadditionalsurgery(one-stepprocedures)wasperformed,if

138 necessary: tibiotalar arthrolysis (N=8); subtalar arthrodesis

139 (N=1);talonaviculararthrodesis(N=1);achillestendon

length-140 ening (N=9); medial malleolar osteotomy (N=1); lateral

mal-141 leolarlengtheningosteotomy(N=1).

142 Intwopatients,abilateralTARwasperformedduringthesame

143 procedure.Inninepatients,TARwasperformedontheotherside

144 severalmonthsoryearsaftertheﬁrst.

145

2.4. Physiotherapy

146 Physiotherapywascommencedthreedaysaftersurgery,with

147 passivemobilisationoftheankletwicedaily.

148 Patients were then transferred to a specialistphysiotherapy

149 departmentfor45–60daystoimproveanklemobilityandmuscle

150 strengthandtopreventtheappearanceofhaemarthrosis.

Weight-151 bearingactivitiesandactivemobilisationwereinitiated15 days

152 afterTAR.

153 Afterdischarge,physiotherapycontinuedfor2months.

154 Normalphysicalactivityandappropriatesports couldbe

re-155 startedabout3monthsaftersurgery.

156

2.5. Clinicalevaluation

157 Allpatientswereevaluatedbeforeand aftersurgery,ateach

158 visit,usingtheankle–hindfootscaleoftheAmericanOrthopaedic

159 Foot and Ankle Society (AOFAS) and that of the Association

160 Franc¸aise de Chirurgie du Pied (AFCP – French Foot Surgery

161 Association),i.e.bothscoresareoutof100points(Table1).

162

2.6. Radiologicalexamination:allpatients 163

165 -Weight-bearingfootX-raysforbothfeet:X-rayimages(anterior 166 167 andlateral).Full-length,standinganteroposteriorX-raysofthe

168 lower limb may provide information about knee and hip

169 pathologyoranymisalignment.

170 -TheMe´arytechnique,describedinFigs.1and2(anteroposterior 171 172 view of the ankle associated with metallic circling of the

173 hindfoot), was usedto evaluatethe axis of thehindfoot and

174 heightdifferencebetweenthemedialandlateralmalleolionthe

175 rightandleftsides.

J.G.Asencioetal./FootandAnkleSurgeryxxx(2014)xxx–xxx

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176

-177 Therangeofmotionofthetibiotalarjointwasevaluatedglobally 178 duringextensionanddorsiﬂexion.

179 2.7. Statisticalmethods

180 Descriptiveresultswererepresentedbythemeanstandard 181 deviation (mSD) and the variance (minimum/maximum) and

182 mean.

183 Categoricaldatawasdescribednumericallyandaspercentages 184 (ifthedenominatorwasatleast20).Forclinicalandradiological 185 results,thestatisticalunitwastheankle,asagivenpatientmay 186 haveoneortwoTARs.

187 Data was analysed by the Orgame´trie company (Roubaix, 188 France)usingSAS9.2(SASInstitute,Cary,NorthCarolina,USA)

189 3. Results

190 TARswereperformedbetweenJuly2002andSeptember2009 191 in 21 patients (17 haemophilia A, 3 haemophilia B, 1 von 192 WillebranddiseasetypeIII).

193 Themeanagewas4412(range:24–67years).

194 11patientsunderwentbilateralTAR(intotal,32TARs):2ofthese 195 patients during the same procedure, 2 patients had undergone 196 previously unsuccessful TARs, and received the revision AES 197 (Ramsesprosthesis,2sidesforonepatient,1sidefortheother).

198 12TARswereperformedin7HIV-positivepatients.

199 All patients received anti-haemophilia factor replacement

200 therapyfor6monthsbeforethesurgicalprocedure.

201 NopatienthadsevereamyotrophycontraindicatingTAR.

202 Patientswerefollowed-upfor4.41.7years (range:2.2–9.4)

203 afterTAR,andfollow-upwasterminated,ifappropriate,onthedateof

204 arthrodesis.

205 Todate,nopatientswerelosttofollow-up.

206

3.1. Clinicalresults(Fig.3)

207 ThepreoperativeAOFASscorewas40.219.4(range:16–81;

208 mean34)(Table2).Severedebilitatingpainin18patients.TheAOFAS

209 scoreafter5926daysofphysiotherapywas8511.

210 The AFCP score increased from27.917.1 preoperatively to

211 67.212.0after physiotherapy.The functionscore changedfrom

212 23.67.7to35.96.7.

Table1

AFCPscore.

Item Score

Pain 0=debilitatingpain

to45=nopain

Function: 0–55

Maximumwalkingdistance 0–8

Gaitdisturbance,limp 0–4

Climbingstair 0–3

Descendingstairs 0–3

Requiressupport(sticks) 0–3

Walkingonunevenground 0–3

Stability 0–3

Tricepsforceontiptoe 0–10

Rangeofmotionduringﬂexion/extension 0–15

Equinus 0–5

Laxity 2to0

Podoscopeexaminationofhindfoot 1to0

Fig.1.WeigthbearingX-rays,footbyfoot–heelelevatedof1.5cm–heelringedbyleadedwireorlocatedby2metalpartsonbothsides–X-rayontheaxisofthe2nd metatarsal.

Fig.2.Differenceoftheheightbetweenmedialandlateralmalleolar,comparative bothsides:(normalupto3mm)heelaxis(normal48–88).

J.G.Asencioetal./FootandAnkleSurgeryxxx(2014)xxx–xxx 3

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213 Mostpatientswere:

215

-216 Completelyfreeofpain45points(16ankles)

217

-218 Occasionalminimalpain40points(14ankles)

219

-220 Moderatedailypain30points(2ankles)

221

-222 Severepain.0points(0ankles)

223 Alignment was good for all ankles (weight-bearing axis of 224 hindfoot)accordingtoMe´aryimages.

225 Plantarﬂexiondidnotvary(19.587.08)(range:58–308).

226 Dorsiﬂexionsigniﬁcantlyincreasedfrom 0.3080.58;range:

227 108 to +108 preoperatively, to 10.384.48; range: 58–208 on

228 dischargefromthephysiotherapycentre.

229

3.2. Complicationsandrevisionsurgery

230 Twoperioperativecomplicationswereseen:

232 (a)Cardiogenicshockwithpulmonaryoedema,butwithoutother 233 234 consequences,inoneHIV-positivepatient.

235 (b)2bleedingepisodesinonepatientafteranticoagulanttherapy. 236 237 At the start of the study, 2 of our patients received

238 anticoagulant therapy, but the treatment was quickly and

239 deﬁnitivelyhaltedafterthiscomplication.

240 2 orthopaedic complications: 2 patients (1 patient with

241 worsening pain and 1 with ﬁxation failure) received an ankle

242 arthrodesis (patientnos. 8and 11, Table 3)using theHintegra

243 Newdeal Retrograde Tibio-Talar-Calcaneal Nail with graft, but

244 nonecouldbeneﬁtfromrevisionprostheses.

Fig.3.Dottedlinewithdiamonds:AOFASscore;bolddashedlines:AFCPscore;bold dottedlinewithtriangles:dorsalankleﬂexion;dottedlinewithsquares:plantar ankleﬂexion.

Valuesaremeasuredbeforeanklearthroplasty,onhospitaldischargeandatmost recentevaluation.

Table2

Clinicalﬁndingsatstudytimepoints.AOFASsubscoresandAOFAStotal. Q5

Pre-operative N=32 Athospitaldischarge N=32 Lastevaluation N=30* Pain 9.312.0 34.46.2 37.37.9 Function 23.67.7 35.96.7 33.74.7 Alignment 7.25.9 150 12.83.9 Totalscore 40.219.4 85.311.4 83.413.4 Table3

Individualdata.AOFASsubscores.

# Patient Age* AOFASscore AFCPscore

Before arthroplasty Athospital discharge Atlast evaluation Before arthroplasty Athospital discharge 1 1 30 HaemophiliaA 37 96 75 24 74 2 1 30 HaemophiliaA 28 93 90 24 74 3 2 56 HaemophiliaA 39 82 73 23 61 4 2 56 HaemophiliaA 25 96 88 11 74 5 3 39 HaemophiliaA 23 89 89 12 71 6 4 31 TypeIIIWillebrand’s disease 26 99 85 24 85 7 5 31 HaemophiliaA 16 64 75 13 50 8** 5 32 HaemophiliaA 18 72 - 8 56 9 6 32 HaemophiliaB 18 82 23 9 61 10 7 56 HaemophiliaA 28 70 86 13 44 11** 7 56 HaemophiliaA 34 96 - 23 75 12 8 57 HaemophiliaA 61 92 46 13 9 42 HaemophiliaA 28 92 92 15 75 14 10 32 HaemophiliaA 34 86 75 18 68 15 11 40 HaemophiliaA 25 76 92 12 56 16 11 40 HaemophiliaA 29 93 96 20 75 17 12 40 HaemophiliaA 25 89 90 33 71 18 12 40 HaemophiliaA 65 73 90 49 57 19 13 67 HaemophiliaB 90 89 80 20 14 60 HaemophiliaA 46 69 93 28 53 21 14 60 HaemophiliaA 46 69 89 28 53 22 15 33 HaemophiliaA 73 98 82 51 88 23 16 61 HaemophiliaA 37 73 82 25 59 24 17 24 HaemophiliaB 66 102 79 44 90 25 18 44 HaemophiliaA 21 96 93 16 79 26 18 45 HaemophiliaA 79 96 93 68 9 27 19 48 HaemophiliaA 50 86 73 28 70 28 20 52 HaemophiliaA 50 95 90 29 73 29 20 52 HaemophiliaA 30 95 72 19 73 30 21 42 HaemophiliaA 70 82 85 61 56 31 21 42 HaemophiliaA 81 82 85 69 61 32 6 31 TypeIIIWillebrand’s disease 58 86 85 40 64

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245 Theoverall rateofmajorrevision,deﬁned asanyprocedure 246 requiring the removal or replacement of one or two metal 247 components,istherefore2/32(6%).

248 3.3. Medium-termclinicaloutcomes(Figs.4and5)

249 Atthelastevaluation,theoutcomesremainedexcellent. 250 SincetheAOFASandAFCPscorescouldnotbeevaluatedforthe 251 twoanklesthatreceivedarthrodesis,theoutcomesonlyconcern 252 30ankles.

253 TheAOFASandAFCPscoresare,respectively,83.413.4and 254 81.213.8.

255 Patientspresentedwithnopainin25cases,minimalpainin4 256 anklesandmoderatepainin1ankle.

257 Dorsiﬂexionwas6.884.58(range: 58to+108),withperfect 258 stability.

259 3.4. Medium-termradiologicalresults

260 TheradiographicrangeofmotionimprovedafterTAR(Table4). 261 Atthelastexamination,thetibialandtalarmetalcomponents 262 werestableandinplaceinallcases.

263 Clinically insigniﬁcant radiolucency was revealed above the 264 platformof thetibial component8 and 4 years after TARin 2 265 patientswhoweremonitoredfor10and7yearsrespectively. 266 In1case,abonecystorcavitationaroundthetibialimplanthas 267 beenfollowed-upfor4yearswithoutclinicalsignsorworsening. 268 Inrarecases,X-rayexaminationrevealedperi-malleolarand 269 sub-malleolarosteophytes,slightlypainfulin1case.

270 Finally,thesagittalaxisofthetibiameasures4.482.88(range: 271 08–98),andthesagittal axisofthe talus(anteriorslope) measures 272 8.586.38(range:1.08–28.08)

273 Me´ary technique: the hindfoot valgus measures 5.483.28 274 (range:2.08–13.08)

275 4. Discussion

276 Untilnow,thestandardtreatmentforhaemophilicarthropathyof

277 theanklehasbeenarthrodesis[7,10,13,28,29].Thistechniqueissafe,

278 substantiallyreducespain,preventstheoccurrenceofother

intra-279 articularbleedingandallowsthepositionalcorrectionofpre-existing

280 equinus [8]. Nonetheless,it irreversiblysacriﬁces ankle mobility,

281 worsenedbyanadditionalabnormalconstraintonthemultiplejoints

282 ofthelowerlimb(analysisofgaitandtread)[4]andmechanicalstress

283 onotherunderlyingandoverlyingjointsthatwerealreadydamaged

284

[30,31]. Arthrodesis can lead to secondary osteoarthritis of the 285 hindfootandmidfootandalsoaffecttheoverlyingkneeorhip[16].

286 On the otherhand,totalankle replacement(TAR) preserves

287 anklemobility,restoresrollingofthetalus[28,32]andprotectsthe

288 otherjoints.Inaddition,haemophilicarthropathyoftheankleis

289 often bilateral [33]. In our study, 11 of 21 patients received

290 bilateralTARs,withoutcomesassuccessfulasthoseofpatients

291 affectedunilaterally.

292 Sincebilateralarthrodesisoftheankleoftenresultsinmediocre

293 outcomes [16] (tibiotalar blockage and major stress on both

294 hindfeet), this may represent another advantage for TAR over

295 arthrodesis.

296 AsinpreviousstudiesonTARinhaemophilicpatients[2,7,27]

297 ourgroupof21 patients(32 TARs)achievedhighlysatisfactory

298 medium-termoutcomes.Insertionofnon-constrainedprostheses

299 withthreecomponents,includinganintermediatepolyethylene

300 mobilebearing[13],considerablyreducedpain[16]andrestored

301 rangeofmotiontonearlyphysiologicallevels.Thealignmentofthe

302 metalcomponentsisperfectlystable.

303 Clinicaloutcomesaregood;onlytwopatientshadtoundergo

304 arthrodesis, performedwithout problemsandperfectly

consoli-305 dated,underscoringthatTARisnotaone-wayprocedure[16].

306 Thegainintherangeofmotionduringdorsiﬂexionisimportant

307 forthegaitcycle[32–34],whileplantarﬂexionvarieslittle.Barg

Fig.4.PatientwithseverehaemophiliaA,30yearsoldandveryseverehaemophilicarthropathy(2kneesand2ankles).Programme:07/10/2002leftTAR,09/12/2002leftTKR, 26/03/2003rightTAR,03/06/2003rightTKR.Totalrehabilitationin6months.

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308 etal.reportlimitedgain inmobility,probablysecondarytothe 309 releaseofperiarticular softtissueduetorecurrenthaematoma. 310 TARdoesnotsignificantlyimprovetherangeofmotionofjoints 311 duringflexion,but this is notof majorimportance; thegain is 312 mostlyrelatedtojointindolence.Inaddition,itseemstousthat 313 Achillestendonlengtheningisindispensablewhendorsiflexionis 314 lessthan108onperioperativeX-ray.

315 Whenreleasingbylengthening,itisoftenpossibletoobtaina

316 greater posterior rangeof motionthannecessary;gradually the

317 patient limits the range of motion during dorsiﬂexion to an

318 appropriatelevelfortakingnormalsteps,withtheprogressionof

319 thetibiafromthe reartowardsthefrontduringthegaitwaiting

320 phase,calledthe‘‘secondrocker’’[32];thisexplainsthedifferencein

321 dorsiﬂexionbetweenthepostoperativeperiodandlastevaluation.

322 Knecht et al. [23] performed long-term X-ray monitoring

323 (mean: 7.2 years; range: 2–16 years for 117 TARs

[three-324 component constrained Agility TAR, De Puy, Warsaw, Indiana,

325 USA])innon-haemophilicpatients,showingthat 76%presented

326 with signs of radiolucency, sometimes with large cavitations

327 aroundtheimplant.

328 Compared to the medium-term (mean: 4.41.7 years), we

329 detected signs of radiolucency in 2 ankles (Fig. 6) and 1 cyst

330 (cavitation)aroundthetibialimplantwithnoclinicalsigniﬁcanceat4

331 and8yearsaftersurgery[35].

Fig.5.Follow-up:10yearsaftersurgery.

Table4

Radiographicﬁndings.Tibio-talarrangeofmotion. Athospital discharge

Last evaluation Globaltibio-talarrangeofmotion 25.2011.19 29.8612.08 Tibio-talarrangeofmotionin

plantarﬂexion

9.864.67 10.706.42

Tibio-talarrangeofmotionin dorsiﬂexion

5.863.29 7.034.43

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332 Jointreplacementsurgeryinhaemophilicpatientssometimes 333 raises several issues: perioperativeand postoperativebleeding, 334 aseptic debonding, infection and heterotopic bone formation 335 leading to disruption of physiotherapy, limp and limitation of 336 walkingdistance.Theuseofatourniquetontheipsilateralthigh 337 preventsperioperativebleeding.No postoperativebleeding was 338 observed;this isthankstocareful managementofthedosesof 339 clotting factor, monitored before surgery and postoperatively. 340 Otherauthorshaveemphasisedtheimportanceofanexperienced 341 teamofhaematologistsandsurgeons[27].

342 Publicationsonkneeand hipprostheses[36]underscorethe 343 riskofhigherratesof asepticdebondinganddeepinfectionsin 344 haemophilic patients [10,37] although these results are under 345 debate[24].Wedidnotobserveasepticdebondingorinfection.In 346 theliterature,theriskofdeepinfectionaftersurgeryappearstobe 347 higherinHIV-positivepatients[26,37].IntheBargetal.study,on 348 10Hintegraprosthesesin8patientswithhaemophilic arthropa-349 thy,noinfectionswerereported,butnopatientswereHIVpositive. 350 Inourstudy,7patientswereHIVpositive(CD4counthigherthan 351 200/mm3atsurgery),butnoinfectionsontheimplantedmaterial 352 inthe12TARssuggestthatTARcanhavelowerinfectionriskthan 353 hiparthroplasty[37].

354 Two cases required secondary arthrodesis after prosthesis 355 removal;haemophiliacsrequiringTARaregenerallyyoung,which 356 tends to increase the risk of prosthetic revision. We have not 357 observedadverseclinicalprogressionsintheyoungestpatients, 358 buthaemophiliacsconsciousoftheirmedicalhistoryappeartobe 359 morecarefulandcautiousandlessdare-devil.

360 Whydidweusetwotypesofprostheses?

361 In 1989, when we performed our ﬁrst implantation for 362 osteoarthritis, we used the Buechel–Pappas prosthesis. Its 363 distributionwasstoppedinFrancein1995.In1996,wetherefore 364 usedtheAESprosthesis.In2008,themanufacturerdecidedtostop 365 itsdistributioncompletely,astheprosthesiswastheonlysuspect 366 inthegenerationofcavitations.

367 Sincethen,wehaveoptedfortheHintegraprosthesis. 368 Today,allprosthesesusedinEurope(Buechel–Pappas,Doets, 369 Hintegra,Mobility,Salto,Starandothers)alsopresent[19,20,38]

370 cavitationssimilar tothose that werepresented withthe AES, 371 whetherinthetibiaortalus,inabout20–30%ofpatients. 372 Innocircumstancescanoneimplicatebysimplesuspicion–as

373 somehaveattemptedtodo–themanufacturingprocess,metal, 374 polyethylene, shape or even the surgeon. To date, no credible 375 explanationhasbeenfound,andresearchisongoing[16]. 376 Thisphenomenontakesusbacktosimilarproblemsoccurring 377 inTHRs(TotalHipReplacements)20–30yearsago,which,happily, 378 didnotleadtoTHRsbeingstoppedbutdidmakeitpossibletoﬁnd 379 explanationsandsolutionsintelligently.

380 AlthoughourTARstudyinhaemophilicpatientsisthelargest

381 published to date, it hasa few limitations: Itis retrospective,

382 presentingonlyamedium-termreview,nonethelessperformedby

383 an independent clinical research assistant using the hospital

384 registry;somedatamayhavebeenmissed,butnomajorfactshave

385 beenomitted.Allpatientswerereviewed,andX-rayresultswere

386 carefullyexaminedbeforeﬁnalisingthisreport.

387 5. Conclusion

388 Inviewofourresults,webelieve,inagreementwithvarious

389 publicationscitedintheliteratureconcerningosteoarthritisofthe

390 ankle, that, even in haemophilic patients, TAR can provide an

391 alternative totibiotalararthrodesis, the currentstandard

treat-392 ment.TARisindicatedbecauseofitsexcellentoutcomesinpain

393 reductionandimprovedanklemotion,inparticulardorsiﬂexion,in

394 ourgroupof21patients(32ankles).

395 Thistechniqueconservestibiotalarmobilityand,bycontiguity

396 preserves neighbouring joints; combined with pain relief, it

397 improvesqualityoflifeinhaemophilicpatients.

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