Aloplasty of an ankylosed knee in a patient with severe haemophilia A

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Case report/Kazuistyka

Aloplasty of an ankylosed knee in a patient with severe haemophilia A

Aloplastyka stawu kolanowego z ankyloz ą u pacjenta z ci ężką postaci ą hemo ﬁlii A

Jerzy Mirosław Jaworski

^1,

*, Adam Zawojski

¹

, Joanna Zdziarska

²

, Krzysztof Czernicki

³

, Magdalena Wilk-Fra ńczuk

⁴

1OrthopaedicWard,CracowCenterofRehabilitationandOrthopaedics,Cracow,Poland

2HematologyDepartment,UniversityHospitalinCracow,Poland

3RehabilitationWard,CracowCenterofRehabilitationandOrthopaedics,Cracow,Poland

4RehabilitationClinic,InstituteofPhysiotherapy,JagiellonianUniversity–CollegiumMedicum,Cracow,Poland

Introduction

Aloplasties inpatients with haemophilia are performed in theCracowCentreofRehabilitationandOrthopaedicsstart- ingfromJune2010.Theperioperativeperiodismanagedin closecooperationwiththeHaematologyDepartment ofthe University Hospitalin Cracow.Thusfar, 160procedures in

patientswithhaemophiliawerecompletedwithgoodfunc- tionalresults.

Material and methods

M. T., was a 40 year old male with severe haemophilia Awithout inhibitorand generalizedsecondaryhaemophilic article info

Articlehistory:

Received:04.07.2017 Accepted:20.07.2017 Availableonline:29.07.2017 Keywords:

Kneealoplasty

Rotating-hingetypeprosthesis

Haemophilia

Ankylosis

Słowakluczowe:

Aloplastykakolana

Endoprotezarotacyjno-zawiasowa

Hemoﬁlia

Ankyloza

abstract

Thegoalofthisreportistodescribeacaseofkneearthroplastyinapatientwithover a20yearlonghistoryofankylosisoftheleftkneejointinthecourseofseverehaemop- hilia A. Clinical and functional conditions were assessed by meansof a Knee Society Score(KSS);painintensitybyVisualAnalogueScale(VAS);andintensityofdegeneration ofbone formingthe joint bymeans ofthe Kellgren-LawrenceScale. ROM ofthe knee jointbeforeandaftertheoperationwasmeasured.WeusedhingeprosthesisofS-ROM byDePuytorestorethekneejoint.Asaresultoftheoperationwegotaxialalignment ofthelimb.Duringtwoyearsofclinicalfollow-up,thepatientachievedincreasingmobi- lityoftheoperatedknee,fullextension,and80degreesofactiveﬂexion.Therewereno radiologicalsignsoflooseningoftheimplant.Thepatients’functionalcapabilitiesimpro- vedsigniﬁcantly;andpainoccurredonlyoccasionally.

*Correspondingauthorat:Al.Modrzewiowa22,30-224Kraków,Poland.Tel.:+48124287308;mobile:+48602434176.

E-mailaddress:jerzymir@poczta.onet.pl(J.M.Jaworski).

ContentslistsavailableatScienceDirect

Acta Haematologica Polonica

journal homepage:www.elsevier.com/locate/achaem

http://dx.doi.org/10.1016/j.achaem.2017.07.008

zo.o.Allrightsreserved.

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arthropathyofmultiplejoints.HewasreferredtoourCentre for surgical correction of the left lower limb axis. The progressivedegenerativeprocessoftheleftknee,secondary tooftenintraarticular bleedings,started early inchildhood and resulted in ankylosis of the knee joint. In addition, disturbance of the anatomical axis of the limb, making it functionallyshorter, ledto a signiﬁcantimpediment while walking. Through a compensatory mechanism, the left equinus footdeveloped. Thepatientmoved using crutches and presented with a pathologic, insufﬁcient pattern of walking. Radiosynovectomies of both knee joints were performedin10thand11thyearoflife.

In 2011, the patient underwent aloplasty of the right knee,andin2013aloplastyofthelefthip.Clinicalfollow-up examinationsandthepatient'ssubjectiveassessmentofthe resultsofpreviousoperationscausedhimtotrytoimprove life quality and functional possibilities of the left limb as awhole.

An orthopaedic examination revealed functional short- eningof the limb by4cm, disturbance of anatomicallimb axis, 30 degrees of valgus, and a 40 degree of ﬂexion contracture(Fig. 1), nomovement of theleft knee joint.X- rayexamination showed ankylosis – grade 4, of degenera- tivearthritisaccordingtotheKellgren-Lawrencescale[1].

Thepatient'sstated dreamwas tocorrect the long axis of the limb toget back supportive functionof the limb at theleast.Hewasinformedaboutthepossibilityofcorrective

osteotomy followed by osteo-synthesis with an external stabilizing device. Hewas also informed about an alterna- tive treatmentsuch as implantationof revision rotational- hinge prosthesis, providedanatomical conditions(muscles, bones) were favourable intraoperatively [2]. All possible problems and complications perioperatively were widely discussed withthe patient.Heconsented toall procedures andactionstobetakenonbytheoperatingteam.

Method of treatment

TheoperationwasperformedinJanuary2014undergeneral anaesthesia,withnotourniquetuse.Apreoperativedoseof 48IU/kg of plasma-derived factor VIII concentrate was administered and thepost-infusion level was confirmed to besufficient(118%).Thejointwasopenedinatypicalway, with parapatellar access toankylosis. Severearthrofibrosis of patello-femoral joint and ankylosis between the femur and tibiawereascertained.Afterwideliberationof thesoft tissue,bothtibial,femoralcondyles,andcicatrixofmenisci were revealed. The next step of operation was biplanal mediallyopenedwedge-shapedosteotomy.Afterosteotomy was accomplishedweestimatedthefunctional stateof the muscles, especiallythe extensorapparatus,andfounditto be quite good, giving chance for a successful prosthesis implantation.Usinganintramedullarysight-rod,wecutthe Fig.1–PreoprativeX-rayoftheleftkneeina-pandlateralview

Ryc.1–ZdjęcieRtgprzedoperacyjnelewegostawukolanowegowprojekcjiAPibocznej

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futurearticularends ofbothfemurand tibia,andprepared them to imbedboth metaphyseal sleeves and the femoral and tibial components of the S-ROM de Puy prosthesis, toughenedbyintramedullarystems,duetothepoorquality of bone (Fig. 2). Prosthesis was ﬁxed by means of bone cement withantibiotic gentamicin. Intraoperatively wegot the ROM of full extension and 808 ﬂexion of the operated knee. The operation lasted two hours. An intraoperative dose of 12IU/kg factor VIII concentrate was administered.

Postoperatively, the patient lost about 1500ml of blood during the ﬁrst two hours; he received 600ml of his own bloodinre-transfusion.Inaddition,hewasinfused4units of RBC concentrate and 2 unitsof FFP. Factor VIIIconcen- tratewasgivenevery8hinadoseof24IU/kg.Weusedtwo suction drains, which we maintainedfor 48h after operation.Theoperatedlimb wassplintedinaplaster splintfor 48h, followed by early rehabilitation using a CPM device (continuous passive motion). Nocomplications occurred in the early postoperative period or during the follow-up period.FactorVIIIplasmalevelwasmeasureddailyandthe concentrate dose adjusted to maintain the trough level above 80% in the ﬁrst week and above 60% inthe second week.

Results

Initially, the patientwalked withcrutches. During the ﬁrst twoweeksofthepostoperativeperiodhegotROMfrom 5 degreesofextensionto45degreesof ﬂexion.Rehabilitation was then continued in the Rehabilitation Ward of our Centre,wherehespent6weeks.ThefactorVIIIplasmalevel

was maintainedabove 30–40%withtwicedailyconcentrate injections. On discharge from the Centre the patient achieved ROM from full extension to 70 degrees of active ﬂexion. Passive ﬂexion with a CPM device reached 808.

Duringtheclinicalexaminationafter 2years follow-up,the patient demonstrated effortless walking, with no more crutches and inconspicuous limping; the patient did not complain about the ailment. ROM revealed 80 degrees of activeﬂexionandfullextension,painoccurredoccasionally and never exceeded 2 on VAS. According to the KSS functional scale, the patient achieved 90 points, and 86 points on clinical measure. Both functional and clinical resultsimprovedincomparisontothepre-operative period, when they were 30 and 66 respectively. X-ray control did notrevealanyradiologicalsignsofprosthesisloosening.He returned to work. In the patient's opinion the ﬁnal result was excellent, and he reported that if he had to decide again,hewouldundergotheoperation.

Discussion

Haemophilia is a genetically transmitted, X-linked disease affecting mainly males. Due to deﬁciency of a coagulation factor, patientssuffer frombleedings, usuallyintraarticular – mostly to the ankles, knees and elbows, but also to muscles,CNS,andparenchymatousorgans[3–5].Numerous bleeds during the course of a lifetime lead to secondary degenerativejointdisease,alsoseeninyoungadults[6].

The main challenge in the reported case was the fact that the patient suffered no pain. He complained of the problemwithmovingasaderivateof thedeviationof limb Fig.2–Postoprativex-rayoftheleftkneeina-pandlateralview

Ryc.2–ZdjęcieRtgpoooeracyjnelewegostawukolanowegowprojekcjiAPIbocznej

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axisandankylosedknee.Goodclinicalresultsof previously performedaloplasties inthispatientencouragedhimtotry to improve his quality of life by improving the functional conditionof thelimbandmovingpossibilities.Heexpected fromthesurgeononlythecorrectionoflimbaxis.Basedon previous operativeexperiencesand the resultsachieved in otherpatientswithhaemophilia,wedecidedtodomore:to implantthekneeprosthesis.

According to Comanho [7], who described 9 patients with ankylosed knee who underwent TKA, all showed improvementbothinpainandROM.Basedonthisobserva- tioninvestigatorshypothesizedthatTKA isatreatmentof choiceinanankylosedknee.Straussetal.[8]dividedtheir patientswith haemophilia into two groups: group 1:with stiff knee, ROM to 508 – 23 knees; and group 2: with no stiffness, ROM more than 508 – 27 knees. After TKA was performed,themeanimprovementofROMingroup1with previously stiffknee was 468, andinthe other group 98.

Investigatorsconﬁrmed,thatTKAinpatientswithhaemo- philia with progressing loss ofROM is an efﬁcient proce- dure.Cohenetal.[9]demonstratedthatinspiteofpossible complications that can occur after TKA in patients with haemophilia,thisprocedureinthelong-termimprovesthe qualityoflife.

After reviewing the patient's medical history and cur- rent clinical condition, a mobile-hinge prosthesis was recognized as the most suitable one. Felli et al. [10]

observed91.7%survivalofmobile-hingedprosthesesduring a 13-year follow-up in patients with RA used as primary andrevisionaloplasty. Petrou et al.[11] describe usage of thiskindofprosthesesin80patientswithameanfollow- upof13years,also, with91%ofgoodtoexcellentresults.

Wefound onereport describing the useofS-ROM DePuy prosthesisin16casesofprimaryandrevisionaloplastiesin patientswithlargeinstabilityofthekneejoint[12].During a 2-year follow-up the authors observed no prosthesis loosening.

Sunnasseeetal.[13]describetheuseofmega-prosthesis in the treatment of musculoskeletal complications in 5 patients with haemophilia: two of them presented with pseudotumors; two with periprosthetic fracture; and one withmixedcontractureROMof( 18)upto568.Onepatient withapseudotumorhadalimbamputationduetorecurrent bleeding;therestdemonstrateddiminishingcomplaintsand improvedROM.Acaseofstiffknee,ROMimprovedupto08–

1108.They concludedthat mega-prosthesis isagood alter- nativemethodoftreatmentofcomplicatedTKAaloplasties.

Conclusion

Our opinion is that using rotating hinge prosthesis in the reconstruction of an ankylosed knee of haemophiliac patients is the treatment of choice. Prosthesis gives the chancetosetthekneeinmotion,despiteyearsofimmobi- lizationandatrophyofmusculoskeletalsystem.Oneshould pointoutthatthesuccessistheresultofclosecollaboration between the haematologist, orthopaedic surgeon, patient and rehabilitants [14], which is the gold standard in our Centre.

Authors’ contributions/ Wkład autorów

JJ –workconcept,datacollectionandinterpretation,critical reviewing, work preparation, collection of literature. AZ – work concept, datacollectionandinterpretation,work preparation, collection of literature. JZ – data collection and interpretation, work preparation. KC – data collection and interpretation.MWF–datacollectionandinterpretation.

Conﬂict of interest/ Konﬂikt interesu

Nonedeclared.

Financial support/ Finansowanie

Nonedeclared.

Ethics/ Etyka

The work described in this articlehas been carried out in accordance with The Code of Ethics of the World Medical Association (Declarationof Helsinki)for experiments invol- ving humans; EU Directive 2010/63/EU for animal experiments;UniformRequirementsformanuscriptssubmittedto Biomedicaljournals.

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