msk11 program2011 lao 1 3/10/11 10:09 am page 1 wfh 12th ... · similar to osteoarthritis, causes...

WFH 12TH INTERNATIONALMUSCULOSKELETALCONGRESSMarch 31 - April 2, 2011Dubai, UAE

Organized by: In collaboration with:

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n Society of Hemophilia

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AbstractsA1.2Living with Hemophilia: The Physiotherapist’sRole in Adolescence to Young AdulthoodP. HilliardThe Hospital for Sick Children, Toronto, ON, Canada

Adolescence into young adulthood is a time when manychanges occur at varying rates and intensity includingphysical, emotional, social, and psychological alterations.Body size and structure can change dramatically, resultingin self-image concerns. The adolescent strives to gainindependence, often testing boundaries, and peeracceptance is important. For boys with hemophilia, thistransition period can present many challenges, oftenoccurring simultaneously and potentially becomingoverwhelming. Joint health may be a low priority for theyouth at this time.The hemophilia program physiotherapist can play asignificant role in educating and supportingadolescents/young adults during this transition. Duringchildhood, parents make decisions regarding bleeddetection/management, return to activities, potentialconsequences, and activity selection. During adolescence,the emphasis changes from passive to active involvement.Gradually building upon the youth’s knowledge base todevelop a good understanding of the effects of blood inthe joint, the potential development of chronic synovitisand joint arthropathy will help them find a balancebetween optimal bleed recovery and what they want todo. Stressing that factor replacement alone is not sufficientto treat joint and muscle bleeds is essential. Keying in onthe adolescent/young adult’s priorities in exploring therisks/benefits of activities and potential physicaljobs/careers and recommending possible modificationswill assist the physiotherapist to make a positive influencein the young man’s choices.Group education sessions that generate topics ofinterest/concern to youths and young adults that can beaddressed by the physiotherapist may be of particularbenefit to shy or less confident individuals. The venues canalso be varied, more casual, and less boring orintimidating.Starting the transition process from passive to activeparticipation earlier in adolescence may result in instillinggood habits at a younger age. Truly tailoring the approachto match the youth’s priorities will ensure his commitment.

Session A1 - PWH: The Way of Life

A1.1Primary Prophylaxis in Young People withHemophiliaP.E. MonahanPediatric Hematology and Gene Therapy Center, Universityof North Carolina at Chapel Hill, North Carolina U.S.A.

Decades of clinical experience support the concept thatprimary prophylactic clotting factor replacement forsevere hemophilia A and B can decrease the incidence ofjoint bleeding and prevent joint damage, when comparedto replacement in response to bleeding or secondaryprophylaxis begun to treat established joint damage.Although different definitions of primary prophylaxis havebeen used, the onset of prophylaxis prior to the onset ofjoint damage is critical. Even in countries with abundantreplacement clotting factor, institution of primaryprophylaxis does not necessarily imply immediateinstitution of “full prophylaxis” schedules. Instead,approaches tailored to minimize morbidity and optimizeadherence and empowerment of families may be pursued.An analysis of 10,262 patients in a U.S. database hasrecently confirmed that there is additional benefit thatprophylaxis is associated with a significant risk reductionfor intracranial hemorrhage. Epidemiologic datasuggesting that early institution of primary prophylaxis inthe absence of intensive treatment or bleeding maydecrease the risk of FVIII inhibitors is more controversial. Randomized clinical trial data now also demonstratessuperior clinical outcomes using primary prophylaxiscompared to a very aggressive on-demand replacementschedule. (Manco-Johnson et al, N Engl J Med 2007). Anunanticipated finding in that trial, however, was that thenumber of clinically observed hemarthroses correlatedweakly with joint outcomes as determined by MRI. Thefinding suggests the possibility that, even withoutclinically evident joint bleeding episodes, repeatedosteochondral microhemorrhages may contribute todeterioration of joints in the absence of prophylacticreplacement. Additionally, animal models suggest thatadequate initial hemostasis in response to bleeding doesnot ensure normal wound healing, which instead mayrequire maintenance of some hemostatic potential duringthe course of wound healing. New insights into the scopeof mechanisms by which clotting factor protects the jointmay be required to optimize primary prophylacticstrategies. Key words: primary prophylaxis, hemophilic arthropathy

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Abstracts (cont’d)bleeding disorder specific conditions. Diagnostic,screening, and preventive strategies for age-relatedconditions need to be identified and delivered. Clinicaldata to inform treatment are scant, and multicentreobservational and treatment studies required. Treatmentcentres should soon be adding geriatricians to themultidisciplinary team.

A1.5Hemophilia and Ageing: AddressingMusculoskeletal ProblemsA. ForsythPenn Hemophilia and Thrombosis Program, Thorofare, NJ, U.S.A.

In older men with hemophilia, musculoskeletal (MSK)complications of bleeding contribute to increasedmorbidity that occurs with age. Hemophilic arthropathy,similar to osteoarthritis, causes pain and structural jointdeterioration that often impairs functional ability andimpacts physical activity. In addition, persons withhemophilia (PWH) also experience the general MSKcomplications of ageing such as declining balance,incidence of falls, and osteoporosis. These problems maybe intensified in PWH, due to concomitant MSK bleedingissues. A regular fitness program that incorporates aerobicexercise, strength-training, and balance and flexibilityactivities is important to help improve functional mobilityand manage hemophilic arthropathy symptoms, balanceand falls risk, osteoporosis, and osteoporotic fractures.Research from the non-bleeding disorders population hasshown that exercise is an effective tool to addresssymptoms of osteoarthritis, reducing the risk of falls andhelping to maintain bone density. Because of the specialchallenges associated with hemophilia, which includeboth the underlying coagulopathy and, in many cases,extensive MSK involvement, patients beginning anexercise regime should be referred to appropriatelytrained physiotherapists for evaluation, education andinstruction, and follow-up. Although some informationfrom the general population can be applied to PWH, morestudies specifically addressing these topics in regards toPWH are needed.

Session A2 - Hemophilia and Cartilage

A2.1Hemophilia and Cartilage: The Blood EffectP.E. MonahanPediatric Hematology and Gene Therapy Center, Universityof North Carolina at Chapel Hill, North Carolina U.S.A.

The inflammatory synovitis that results from hemophilicbleeding into joints has been implicated in the subacuteand chronic secondary osteochondral destruction that

A1.3Surgical Treatment in Patients on PrimaryProphylaxis: What is the Incidence? G. PastaOrthopaedics and Traumatology Department and AngeloBianchi Bonomi Hemophilia Center, IRCCS Ca’ GrandaMaggiore Hospital Foundation, MilanE-mail: [email protected]

Prophylaxis is recommended as first choice therapy forpatients with severe hemophilia in countries where factorconcentrates are largely available. Primary prophylaxisrefers to preventive treatment started in children beforethe onset of joint damage. Although the benefits ofprimary prophylaxis are well documented, only theScandinavian and northern European countries haveadopted, since long time, prophylaxis as the standardtreatment in all children with severe hemophilia. Usually,the joint status is considered the primary endpoint ofprophylaxis studies; however data on the impact ofprimary prophylaxis in reducing the need for orthopedicsurgery are still scarce. Moreover, the wide availability ofsafe and effective replacement therapy has favoured anincreasing number of elective orthopedic surgeries (i.e.joint replacement, arthroscopic procedures). Thesechanges in surgical practice occurred through the lastdecades in a different way across the world, havingrendered difficult the comparison between differentpatients' cohorts. Furthermore, the use of primaryprophylaxis has allowed the participation to high-riskactivities of young hemophilic patients. Consequently, anincreasing need for several surgical procedures such assports traumatology may be expected. The assessment oflong-term outcomes of primary prophylaxis is warrantedand, in this light, the claim for orthopedic surgeryrepresents a relevant outcome, not only by the clinicalstandpoint but also in terms of pharmacoeconomics.

A1.4Hemophilia and Ageing: Medical AspectsA. StreetThe Alfred Hospital, Melbourne, Australia

The hemophilia population, particularly in developedcountries, is ageing. For example, 2% of patients in theU.S.A. with FVIII and FIX deficiency are over 65 years of agewith a further 15% over 45-years-old. There are problemsspecific to the hemophilia population of chronicarthropathy and long-term effects of transfusiontransmitted infection which is compounded by the widercommunity ails of cancer and vascular disease, concurrentwith the ageing process. Risk factors for vascular diseaseare identical in patients with hemophilia as for the generalpopulation, independent of the severity of hemophilia.Hemophilia centres may provide, though not necessarilybe resourced for, medical care beyond the usual array of

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Abstracts (cont’d)A2.3The Role of MovementA. SeuserOrthopeadic Departement Kaiser-Karl-Klinik, Bonn(Germany)

Cartilage is an essential structure for the joint. Blood candestroy cartilage cells and start a vicious cycle that leadsto inflammation and more deterioration of cartilage. Weobserve worsening of joins that never experiencedsymptomatic bleeds. Cartilage is a bradytrophic tissue thatneeds pressure and release of pressure for nutrition andregeneration. Movement therefore plays a major role inmaintaining and preserving cartilage. In 3D motionanalysis we can measure the movement of the joints andcan predict the loading of the cartilage. Optimum loadingis rhythmic, regular and sinusoid, with no accelerationpeaks and a good distribution of the pressure on to thecartilage. Loading should always be perpendicularbecause of the vertical lining of cartilage cells. Accelerationpeaks, saggital loading like gliding in the knee joint ordisturbed pressure distribution will hurt the laminasplendens. There is enough biomechanic proof thatpressure on the superior layers is much higher than on thebony endplate. False movements as described aboveoccur routinely in younger children. They have theadvantage of a higher regenerative power as cartilage isstill in growth. They occur as well if the joints areoverloaded by vigorous exercise. The mechanicallydestroyed cartilage will act just like the chemicallydestroyed cell, which results in faster deterioration of thejoint. Conclusion: Early moderate movement of all jointsand a good musculature that is well trained concerningstrength, flexibility, and coordination will provide verticalloading of cartilage a good distribution of pressure.

A2.4Surgical Treatment: Is It Useful?M. Silva

No Abstract Submitted.

Session A3 - Hemophilia and Bone

A3.1Osteolytic Lesions: Differential Diagnosis andTreatmentH. Caviglia

There are two types of cysts in patients with hemophilia,which have different pathophysiology and clinicalpresentation. The subchondral cyst is always presented inthe presence of arthropathy, in contrast, the intraosseouscyst is secondary to intraosseous bleeding. The first signsof arthropathy on the X-ray are narrowing of the jointspace and small abnormalities in the subchondral bone.

characterizes hemarthropathy. Nevertheless, intraarticularblood is directly toxic to cartilage, as iron-catalyzedformation of destructive oxygen metabolites results inchondrocyte apoptosis and loss of the cartilage matrix.Gaps in the mechanistic understanding of the progress ofbleeding-induced joint damage leave us unable to answerthe key question: Why do some PWH progress rapidly tocartilage loss and subchondral deformation in response tobleeding that is relatively well tolerated in otherindividuals? Much has been learned from models of blood-induced changes in joint tissues in vitro and inhemostatically normal animals in vivo, including theimplication of proinflammatory (e.g. TNFα; IL-1β) and anti-inflammatory (e.g. IL-10) cytokines in hemarthropathy.Ultimately, some processes that lead to osteochondraldegeneration in human hemophilic arthropathy (e.g.abnormal wound remodelling after bleeding) may only befully elucidated by in vivo preclinical investigations inhemophilic animals, as a path to human clinicalinvestigation. Key words: cartilage, hemophilic arthropathy

A2.2Joint Aspiration, Viscosupplementation,Growth Factors, or Nothing? G. PastaOrthopaedics and Traumatology Department and AngeloBianchi Bonomi Hemophilia Center, Fondazione IRCCS Ca’Granda, Ospedale Maggiore Policlinico, Milan, Italy [email protected]

The pathogenetic mechanism of hemophilic arthropathyis not fully understood, however general agreement existson the fact that that the repeated extravasation of bloodinto the joint cavity is the factor responsible for synovialand cartilage changes. The proper treatment strategy toprevent and/or cure cartilage damage in hemophilicarthropathy is still an open issue. Several experimentalstudies suggest the potential role of joint aspiration in caseof hemarthrosis to prevent the blood-borne damage ofthe cartilage. Nevertheless, the management ofhemarthrosis in hemophilic patients is still controversial;often the conditions to perform a safe arthrocentesis arenot fulfilled, and very few hemophilia centres are used topractising such a procedure. Since cartilage and bonedamages have very limited ability to heal spontaneously,procedures able to generate cartilage and bone tissue areneeded. It has been suggested that intra-articularadministration of hyaluronan (viscosupplementation) asgrowth factors may improve articular cartilage defects.This issue has been poorly investigated so far, howeverpreliminary results are promising. The recent researchachievements in this field and the potential clinicalapplications of new technologies in the treatment ofchronic hemophilic arthropathy will be reviewed anddiscussed.

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Abstracts (cont’d)level of physical activity, low BMI (<19), liver dysfunction,concomitant hepatitis C, HIV sero-positivity, systemic steroidtherapy, and prolonged immobilisation are some of thecommon causes leading to low peak bone mass (PBM) inthe first 20-25 years of life. This leads to reduced bonemarrow density (BMD) subsequently resulting in osteopeniaand eventually osteoporosis. Various literature shows thatthe incidence of osteopenia is up to 43% and that ofosteoporosis is 25-27% in PWH. However, the sample size isnot adequate enough to show a true picture worldwide. AU.K. consensus group published that 30% of hip fracturesand 20% of vertebral fractures occur in men in the generalpopulation, though there is no published data showingincidence of osteoporotic fractures in PWH. Dual energy X-ray absorptiometry (DEXA) scan done to measure BMD isthe hallmark of diagnosis. It measures the density aroundthe hip, wrist, and spine of the patient compared with ageand gender matched healthy individuals. It is correlatedwith laboratory values of normal serum calcium,phosphorus, and alkaline phosphatase values. High alkalinephosphatase values suggest associated osteomalacia,which can be confirmed with values of serum vit.D3. Use offactor prophylaxis since early childhood may preservenormal BMD in severe hemophilia. Vitamin D supple -mentation to prevent bone loss and fractures for ”at risk”population contemplated. Weight bearing physical activity,physiotherapy, and surgery to remobilise diseased joints,and calcium and vit. D supplements can be recommendedfor anyone at any age. Treatment with anti-resorptivemedication like bisphosphonates may be indicated forpatients in whom rapid bone loss of bone mass has beenconfirmed by sequential BMD measurements, or who havesuffered fragility fractures. Surgical treatment is a challengein presence of osteoporosis. Load sharing implants likeintramedullary locking nails are the preferred methodcompared to load shielding implants for fixation of longbone fractures. Locking compression plates have beenhelpful to a certain extent. Using sharp drills, avoidingtapping after drilling, use of bone grafts/bone cement aresome of the techniques for conventional fixation. Cementedreplacement arthroplasty for femoral, neck, and humerusfractures are choice in the older population.

A3.4Osteoporosis and ExerciseA. Sabbour Head of Physical Therapy for Women’s Health Dept.,Faculty of Physical Therapy – Cairo University, Egypt

The word osteoporosis comes from the Greek osteo andporos: osteo means bone and poros translates to porous(holey). Even healthy bones have holes in them as part oftheir intricate honeycomb-like design. Bones affected byosteoporosis, however, have bigger holes, making themweaker and more prone to breaking. Osteoporosis iscommon, occurs throughout the world, and has becomea major public health concern. Adequate bone mass

The subchondral cyst appears later, and is multiple, andirregularly distributed. The X-ray show these cysts later,however in the CT scan or MRI they are observed early.These cysts are connected and exposed in the articulationaccompanied by the disintegration of the subchondralbone. (1-2-3-4). The connection of the cyst with the jointcauses increased intraarticular pressure during synovitisand leads to progression of the cyst.The clinical presentation of the subchondral cyst is notspecific and is related to the symptoms of synovitis orarthropathy.Sometimes the cyst progression results in big osteolyticlesions that may lead to a pathological fracture. Theintraosseous cyst are caused by intraosseous bleeding, thelocations is in general in the bone metaphysis and are notrelated with the arthropathy. Most of the time, its locationis related to the location of the nutricial artery of theaffected bone. Clinical presentation is usually with acutepain in the affected bone, but unlike acute osteomyelitis,patients don’t usually present fever. Early diagnosis ismade by MRI. Radiological images are done later and arecharacterized by an osteolytic lesion in the metaphysis ofthe bone without the presence of arthropathySurgery is indicated for subchondral cysts when:

1- The size of the cysts is greater than 15% of thearea of the joint, especially if these are loadarticulations. In the knee and in the elbow eachmedial and lateral compartment is consideredseparate and should therefore be evaluatedseparately.

2- A control X-ray shows that cyst size hasprogressed; even if its subchondral extension issmaller than 15%.

The purpose of treatment is to avoid the crumbling of thejoint and to reconstruct bone stock when it is necessary tocarry out definitive arthroplasty. Intraosseous cysts requiresurgical treatment when they have not responded toreplacement therapy during 6 weeks. In both subchondraland intraosseous cysts, the joint must be studied bycomputed tomography and a magnetic nuclear resonanceMRI, which allow appropriate pre-operative planning.

A3.2Is Bioengineering the New Solution?F. Forriol


A3.3Osteoporosis: Clinical ImplicationsS.S. MohantyDept. of Orthopaedics, King Edward Memorial Hospital,Mumbai, IndiaE-mail:[email protected]

Osteoporosis compromises the quality of life of personswith hemophilia (PWH) associated with arthropathy. Low

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Abstracts (cont’d)hemophilia A patients. Musculoskeletal function wasassessed using Functional Independence Score forHemophilia (FISH), and mood status was assessed usingBeck Depression Inventory—Short Form (BDI-SF). Results:The mean FISH score was 23.32 ± 4.69 (range 13-28) andthe tasks that obtained the lowest score were stepclimbing, squatting, and walking. Of our 50 patients, 16(32%) were not depressed, 18 (36%) had mild depression,11 (22%) had moderate depression, and 5 (10%) hadsevere depression. There was highly significant negativecorrelation between mean FISH score and mean BDI-SFscore (P<0.001). Conclusion: The better the replacementtherapy regimen, the better the musculoskeletal functionthat could be obtained in hemophilia A patients and thebetter their mood.Key Words: Hemophilia A, musculoskeletal, FISH,adolescents

A4.2Measuring Balance in Individuals withHemophilia Using the Community Balanceand Mobility Scale: A Pilot Study C. Bos1, M. Wright1, A. Chan1, 2, T. Almonte 1, K. Decker1, R.Goldsmith1, K. Strike1, I. Walker2, L. Waterhouse1, K. Webert2

1 McMaster Children’s Hospital; 2 McMaster University,Hamilton, ON, CanadaE-mail: [email protected]

Introduction: This study aimed to explore the scoringpatterns of individuals with mild, moderate, and severehemophilia A and B using the Community Balance andMobility Scale (CB&M). This study also aimed to investigateany relationship between Hemophilia Joint Health Score(HJHS) and CB&M score, as well as to investigate anyrelationship between Body Mass Index (BMI) and CB&Mscore. Methods: This pilot study used a single-centered,cross-sectional study design. The CB&M and HJHS wereadministered to 15 participants. Correlation analysis, t-test,and a descriptive analysis were performed. A subgroupanalysis was conducted on individuals with severehemophilia A and B. Results: No significant differenceswere found in CB&M scores between individuals with mildand severe hemophilia A and B. A significant correlationwas observed between CB&M scores and HJHS (r= -.805,p<0.001), age, and total CB&M scores (r=.765, p=0.045).There was no significant correlation between BMI and totalCB&M scores. Specific item CB&M scoring pattern wasgenerally comparable to normative values in the samplepopulation. Conclusion: As the hemophilia populationages, balance and falls are becoming issues of primaryimportance. This pilot study suggests that individuals withhemophilia show decreased scores on the CB&Mcompared to the healthy population, regardless ofhemophilia severity. It is the first study to investigate therelationship between dynamic functional balanceperformance and joint health. It is also the first study to

accumulation in early life is important in preventingosteoporosis. Lifetime plots of bone density and age showthat late childhood and adolescence are importantperiods of bone mineral acquisition. Persons with thegreatest bone mass at the end of adolescence have thegreatest protection against the gradual decline in bonemass that occurs with aging. Physical activity has beenconsidered an important factor for bone density and as afactor facilitating prevention of osteoporosis. Bone densityhas been reported to be reduced with hemophilia. Themain cause for reduced bone mass in the hemophiliac ismost probably due to hemophilic arthropathy, typicallyassociated with chronic pain and loss of joint functionsubsequently leading to inactivity. Evidence shows thatexercise may help build and maintain bone density at anyage. Studies have seen bone density increase by doingregular resistance exercises, such as lifting weights, two orthree times a week. This type of weight-bearing exerciseappears to stimulate bone formation and the retention ofcalcium in the bones that are bearing the load. The forceof muscles pulling against bones stimulates this bone-building process. Therefore, any exercise that places forceon a bone will strengthen that bone. Weight-bearingexercises are the most effective to build bones. Theseinclude activities such as walking, stair climbing, running,hiking, and weight lifting. However, swimming andbicycling are not considered weight-bearing exercises. Key words: osteoporosis, weight-bearing exercises andhemophilia.

Session A4 – Free Papers: Non-Surgical

A4.1Assessment of Musculoskeletal Function andMood in Hemophilia A Adolescents: A Cross-Sectional Study M. Ahmed Badr1, T. Hasan Hassan1, N. Raafat Abdel Fattah2,S. Mohamed Badawy1

Departments of Paediatrics1 and Psychiatry2, ZagazigUniversity, EgyptE-mail: [email protected]

Introduction: Hemophilia A is characterized by theoccurrence of frequent spontaneous intra-articular andintramuscular bleeding. If inadequately treated, it resultsin progressive damage to joints and muscles, leading tocrippling deformities and musculoskeletal dysfunction.These complications result in lifelong chronic pain anddisability that may greatly affect the patients' mood. Weaimed to evaluate the musculoskeletal function in ourhemophilia A patients and its correlation to depressedmood in these patients, and determine the impact ofdegree of factor VIII deficiency, different replacementtherapy regimens, and frequency of hemarthrosis on bothmusculoskeletal function and mood. Methods: A cross-sectional study was carried out on 50 adolescent

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Abstracts (cont’d)ankle chronic synovitis in boy S. Both individuals weretreated according to developed guidelines including theuse of: daily factor; P.R.I.C.E (compression being verylimited); splint immobilisation and reduced activity; a 6-week course of Ibuprofen 10mg/kg with coveringLansoprazole; intra-articular steroid injection; regularrehabilitative physiotherapy; use of combined podiatryclinic in boy S; baseline MRI imaging. Results: Both boysappeared to respond favourably to the used guidelinesand showed a reduction, boy S, in clinical signs of chronicsynovitis, and complete resolution in boy A. Conclusion:Chronic synovitis may be addressed successfully withcombination therapy and the support of an extendedteam including pediatric rheumatology. Follow-up jointreviews continue to be conducted to describe thepotential longevity of treatment. Ideally repeat MRI’s mayprovide more objective data.Key words: chronic synovitis, hemophilia A

A4.5Aerobic Capacity in Egyptian Adults withHemophilia H.A. Fouda1, A.H. Sabbour2, A.A. Abdel-Hady3, M.A. El-Ekiaby4, M.H. Gad5, N.F. Mahmoud6

1Physical Therapy Department for Cardiovascular/Respiratory Disorders and Geriatrics Faculty of PhysicalTherapy, October 6 University; 2Physical TherapyDepartment for Obstetrics and Gynecology Faculty ofPhysical Therapy, Cairo University; 3Physical TherapyDepartment for Cardiovascular/Respiratory Disorders andGeriatrics Faculty of Physical Therapy Cairo University;4Hematology Department El-Shabrwishy Hospital;5National Heart Institute; 6Physical Therapy departmentfor Surgery Faculty of Physical Therapy, October 6University, Cairo, EgyptE-mail: [email protected]

Background and objectives of the study: It is wellknown that physical activity is very important forhemophilic patients and can contribute to better qualityof life. Physical training also reduces the risk of bleedingepisodes and prepares the body to manage hemophiliabetter. In spite of these benefits, participation in physicalactivity for children with hemophilia is discouraged inEgypt. The purpose of this study was to investigate thefeasibility of maximum cardiopulmonary exercise test(CPX) to evaluate aerobic capacity of hemophilic patients,also to determine the maximum oxygen consumption(VO2max) and total work load (TWL) in adult Egyptianpatients with hemophilia A. Study design: VO2max andTWL obtained during CPX test (breath by breath) from 30 subjects with hemophilia A (16 moderate and 14severe); their mean age was 17.6 ± 1.92 years; werecompared with normal matched for anthropometric dataand age. Result: All subjects were able to perform atmaximal or near maximal level on exercise tests and none

suggest a single validated measure, the CB&M, as a usefulclinical tool to measure balance in the hemophiliapopulation.

A4.3Assessment of Musculoskeletal Status of aLebanese Hemophilia PatientC Haddad, C. Djambas Khayat, A. Hage, J. Bakhos Asmar, P.Noun, H. Samaha, A. Taher, A. Inaty, S. Sakr Lebanese Association for Hemophilia Centre Care, Beirut,LebanonE-mail: [email protected]

Introduction: It is important that adequate data onoutcomes be documented to develop an optimal protocolfor management. In hemophilia care, musculoskeletaloutcome remains the major hallmark. The purpose of thestudy was to assess joint status of Lebanese hemophiliapatient (PWH). Methods: We conducted a cross-sectionalstudy at the Lebanese Hemophilia Association care centre.65 severe PWH out of the 159 registered were assessed.Patients were subdivided into 5 groups according to actualage. Group A patients were less than 5-years-old (n=8);group B, 5 to 10-years-old (n=9); group C, 10 to 20-years-old (n=12); group D, 20 to 40-years-old (n=25); and groupE over 40 years of age (n=11). Musculoskeletal status wasmeasured using the Hemophilia Joint Health Score (HJHS)and the Functional Independence Score for Hemophilia(FISH). Results: Mean age was 23 years. The means HJHSand FISH scores were 25.57 and 23 respectively. Mean gaitscore was 2.34. The most affected joints were knee,followed by elbows and ankles. Locomotion then transfersand self care were affected. Mean HJHS and FISH scoreaccording to age group were 2 and 33 in group A, , 8.77and 32 in group B, 19.08 and 30.33 in group C, 35.36 and25.48 in group D, and 43.09 and 23.09 in group E,respectively. Scores were significantly more impaired inolder patients. Conclusion: According to our result newstrategies of care must be undertaken. More data must becollected then used for lobbying to enhance standards ofcare with the health authorities.

A4.4Guideline for the Treatment of ChronicSynovitis in Pediatric Hemophilia: CaseReport Involving Synovitis of the Ankle Jointin Two Boys with Hemophilia AT. Flannery, A. South, A. Westoby Leeds Teaching Hospitals NHS Trust, U.K.E-mail: [email protected]

Objective: Review and reflection on the use of developedguidelines in the treatment of chronic synovitis in boy A &boy S. Methods: Joint review by physiotherapistidentified acute bleed, with subsequent chronic synovitis,in boy A (right ankle), and clinical evidence of bilateral

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Abstracts (cont’d)A4.7Physiotherapy Care Profile in Brazil: AnEvaluation of 18 Hemophilia TreatmentCentres M. Matta1, S. Thomas2, A. Wolff3, A. Luz4, C. Ribeiro5, J.Silveira6, A. Do Nascimento7, B. Oliveira Junior 8, L. Nunes9, M.Sayago10

1IHTC Claudio L P Correa, Hemocentro, UNICAMP;2Hemocentro de Mato Grosso E Serviço de MedicinaNuclear da UFRJ; 3Hospital de Clínicas - UniversidadeFederal do Paraná; 4Hemocentro do Estado do Rio Grandedo Sul; 5Hemocentro do Rio de Janeiro; 6HemocentroRegional de Uberlandia; 7Centro de Hematologia eHemoterapia de Pernambuco; 8Hemocentro de Alagoas;9Centro dos Hemofilicos do Estado de Sao Paulo; 10IHTCBrasilia, BrazilE-mail: [email protected]

In 2009 the Brazilian Federation of Hemophilia nominateda group comprising physiotherapists (PT) from allgeographic regions of Brazil. The main goals of theBrazilian MSK Group are to discuss MSK manifestations inhemophilia, and develop trainings. In order to obtain aprofile of the physiotherapy hemophilia care amongdifferent centres, the group sent an e-mail to 85 PTs witha questionnaire covering demand for treatment, avai -lability of auxiliary resources, multidisciplinary interaction,and patient adhesion to the programs. We obtainedcomplete answers from 18 PTs (21.1 %), 44.4% of thesededicated exclusively to hemophilia care, and mostattending patients referred by hematologists (83.3%). PTswho replied use at least 2 auxiliary equipments forelectrotherapy. All maintain exercises programs as well ascryotherapy (94.4%) and some kind of hydrotherapy(66.6%). The preferred modalities of treatment areexercises programs (100%), therapeutic ultrasound(88.8%), and cryotherapy (83.3%). Patients have 3appointments per week (77.7%), averaging 66 minutes persession, with factor infusion prior to physiotherapy(50.0%). Public transportation is used by 72.2% of thepatients attending physiotherapy. When intensiveprogram is needed, 77.7% can count on housing. Exceptfor one centre, all have a multidisciplinary team. One ofthe greatest obstacles to adhesion to treatment istransportation. Only 3 centres (16.6%) regularly provideexercise programs for home and only 2 (11.1%) providewritten electronic guidance to other PTs. We concludedthat more efforts should be done in order to guaranteephysiotherapy care focusing on the patient needs andeducation, since the infrastructure seems adequate.

of them reported bleeding or other adverse events. Theaerobic capacity indices; the VO2max and TWL were less forhemophilic subjects by 56.5% and 32.5% respectively thannormal peers. There was no significant difference betweenmoderate and severe hemophilic subjects. Conclusion: Itwas concluded that the maximum exercise test was safeand useful for hemophilic subjects. Egyptian adults withbleeding disorders have impaired aerobic capacitycompared with their peers. This may be attributed to lackof appropriate treatment, patient's overprotection andsocioeconomic factors. The severity of the bleedingdisorder did not contribute to aerobic capacity deficiency. Key words: aerobic capacity, hemophilia

A4.6Development of a Chatterbot Specialist inHemophilia CareR. Matsunaga1, M. Matta3, M. Ozelo3, M. Borges1, 2

1LIAG/FT; 2NIED; 3IHTC “Cláudio L. P. Correa” Hemocentro,Unicamp Universidade Estadual de Campinas, Sao Paulo,BrazilE-mail: [email protected]

Introduction: In the past several years, InformationTechnology has supported medical areas. The purpose ofthis project is to present a Chatterbot specialist inhemophilia. The prefix 'chatter' means to establish aninformal conversation, while the suffix 'bot' comes fromthe word robot. Thus, this computer program aims to setup a chat with the user about any particular issue. Itsdevelopment is a result of an integration of ArtificialIntelligence and hemophilia care. Methods: Thedevelopment of Chatterbot used the Java programminglanguage to implement its web interface and the Prologartificial programming language to handle its knowledgebase. The Chatterbot uses Portuguese as its defaultlanguage, but other languages will be supported in thefuture. Its knowledge base was constructed based onhemophilia courseware, with the help of hematologistsand physiotherapists,and can be improved on demand.Results: The goal of this computer program is to teachchildren how to deal with hemophilia issues. Children caninteract by asking questions and getting answers throughtext, images, and videos. Chatterbot is available on theInternet and has been evaluated by specialists inhemophilia care. Conclusion: It is challenging for childrento deal with hemophilia. They have to know whichactivities they can perform and how to proceed ifsomething unexpected happens. Chatterbot wasdeveloped to support children to understand theirrestrictions while having fun. It shows howmultidisciplinary areas can help children with hemophilialearn about hemophilia by using a computer program.

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Abstracts (cont’d)The Canadian Physiotherapists in Hemophilia Care (CPHC)conducted qualitative research with young adults withmild hemophilia. Objectives: To discuss how these menmake decisions about their injuries and their ability torecognize an injury needing medical attention, to explorethe attitudes towards physical activity, to identify effectivecommunication strategies. Method: 18 semi-structuredinterviews using grounded theory were performed, inperson or phone, with men aged 18-30-years-old withmild hemophilia from across Canada. Results: Manythemes emerged from these interviews including theimportance of previous experience with injuries, thereluctance to access care, and participation in vigorousphysical activity. Conclusion: The interviews demons -trated that communication between these young menand the healthcare team was not optimal. Gaps inknowledge also emerged regarding bleed identificationand management. Young men with mild hemophilia feelthat they are “normal”, so partake in aggressive sports.Contribution to the practice: This qualitative researchhelped develop a tool to assist young men with mildhemophilia assess injuries and to determine when factorreplacement is necessary. Recommendations regardingsports and physical activity with this population should bere-examined. It also led to the development of a set ofquestions suggested for use by the healthcare team. It ishoped that enhanced communication will lead to moreeffective interactions between the clinic team and theseyoung men. Key words: Mild Hemophilia, Self-management

A4.10Proprioceptive Training in Hemophilic ChildrenA. Osama, E. Elsayed, A. Sabbour, M. Elmasry Faculty of Physical Therapy, Cairo University, EgyptE-mail: [email protected]

Efficient movement function and the maintenance ofbalance during dynamic tasks are more complex than onlyforce production; it requires a primary sensory mechanismfor motor control which is proprioception. The objectiveof this work is to study alterations in proprioceptiveperformance to subsequently evaluate the appropriatetherapeutics. The purpose of the study: To determinewhether exercise intervention improves balance inchildren with hemophilia following participation in abalance and proprioceptive training program using theBiodex Stability System. Thirty hemophilic boys agedbetween 7 and 14 years-old with hemophilia (type A & B)participated in this study. They were classified randomlyinto two groups of equal number (control and study). Thecontrol group received factor replacement as prophylacticmedical management. The study group received theproprioceptive training program and factor replacement.Proprioception parameters were assessed using theBiodex Stability System in both groups with eye openedand with eye closed, before and after three months of the

A4.8Hemophilic Arthropathy: Can Non-Hemato -logical Factors Affect the Burden of Disease?A. McCarthy Physiotherapy Department, Royal Free Hampstead NHSTrust, Pond Street, London, U.K.E-mail: [email protected]

There is an increasing awareness that non-hematologicalfactors may influence the presentation of hemophilia andtherefore the potential to develop hemophilicarthropathy. Clinical observation seems to suggest agroup of factors that may have the ability to influence thelikelihood of a person to bleed more readily than others.These include hypermobility, proprioceptive acuity, andbiomechanical factors. Prophylaxis remains the standardof care, however recently evidence has emerged thatwould appear to indicate that some boys cope very wellon an enhanced on-demand regimen. Although there aresome potential hematological explanations for thedifferences between people who do and don’t bleed withsevere hemophilia, such as maintenance of trough levels,there is also the potential that other factors are at work.All presentations of Ehlers-Danlos Syndrome include tissuehypermobility and easy bruising in their symptomspectrum. Proprioception and muscle weakness are alsofeatures. When considered alongside hemophilia itpresents a paradigm that may explain why some personswith hemophilia persist in bleeding in the presence ofadequate prophylaxis. Biomechanics of the foot and ankleare also regularly discussed within the hemophilialiterature where a presumption exists that non-idealbiomechanics and bleeds have a cause and effectrelationship, however the author cannot identify robustevidence to wholly support this and would at least partlychallenge this view. This presentation will discuss the potential influence ofthese factors and an upcoming program of work that willbegin investigating their influence. Key words: Hemophilia, Hypermobility Etiological Factor,Proprioception

A4.9The Mild Hemophilia Experience: Attitudes,Behaviours, Communication with the HealthCare Team and Determining when Treatmentis Needed J. Nilson1, K. Mulder1, P. Hilliard1, C. Jarock1, M. Hahn1, M.Steele1, C. Schachter1

1Saskatchewan Bleeding Disorder Program, 2WinnipegHealth Sciences Center, 3Toronto Hospital for Sick Children,4Halifax IWK Health Center, 5Montreal Children's Hospital,6Southwestern Ontario Bleeding Disorder Program,7School of Physiotherapy, University of Saskatchewan,CanadaE-mail: [email protected]

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Abstracts (cont’d)Introduction: The ability of individuals to recover from asudden loss of balance is essential for preventing falls andsubsequent injuries. Balance recovery depends on theselection of a proper postural response to maintain thecentre of pressure (COP) in the limits of the base ofsupport. Patients with hemophilia often bleed inside thejoints and muscles, which could lead to poor sensorialinformation about the position of the body segment. Thepurpose of the study was to evaluate postural adjustmentafter a sudden perturbation in children with hemophiliacompared to a control group. Methods: 11 children withhemophilia (10.3±1.3 years-old) and 11 able-bodiedchildren (9.7±1.1 years-old) comprised the hemophiliagroup and control group. A controlled external posteriorperturbation (EPP) was applied by means of horizontaltraction to the subject’s dorsum, using a fixed pulleysystem. The EPP was unexpectedly removed, producingpostural adjustment. COP displacement in the anterior-posterior direction was obtained by means of a force plate.Postural responses were compared between groups usingunpaired student t test in eight intervals of 1 second (t1 tot8), beginning at the moment of EEP removal. Results:COP displacement was higher in the hemophilia group int2 (p=0.02), t6 (p=0.02), and t7 (p=0.04) intervals. Theseresults indicate that children with hemophilia have apoorer ability to control their posture under a sudden lossof balance. Conclusion: Children with hemophilia usegreater centre of pressure displacement to control balanceafter an unexpected perturbation when compared to acontrol group.

A4.13Early-Onset Administration of IL-4 and IL-10Prevents Blood-Induced Cartilage Damage inVitroM. Van Meegeren1, 2, N. Jansen1, 2, G. Roosendaal2, F. Lafeber1

1Rheumatology & Clinical Immunology; 2Hematology andVan Creveld Clinic, University Medical Center Utrecht, TheNetherlandsE-mail: [email protected]

Introduction: It was reported that interleukin (IL)-10limits blood-induced cartilage damage in vitro. In addition,IL-4 has cartilage protective properties. Hence our aim wasto study whether IL-4 in addition to IL-10 can preventblood-induced cartilage damage when administeredduring (prevention) and after the onset (treatment) ofblood-exposure. Methods: Human articular cartilageexplants were cultured for 4 days in presence or absenceof 50% v/v homologous blood (mimicking a joint bleed).IL-4, IL-10, or a combination of these two cytokines wasadded during blood exposure, and 24 or 48 hours afterstart of blood-exposure. After 4 days the medium wasrefreshed and cartilage was cultured for an additional 12days to determine cartilage matrix turnover. Results:Cartilage exposed to blood showed a decrease of

application of the treatment program. The results of thisstudy revealed statistically highly significant improvementin nearly all of the measuring variables of the study group(P<0.01) when comparing its pre- and post-treatmentresults, and when comparing the post-treatment resultsof the control group. From the obtained results of thisstudy, it can be concluded that proprioceptive training isa beneficial modality that may be used to improvestanding postural control with minimal stress to the jointin hemophilic children.Key words: hemophilia, hemarthrosis, proprioception

A4.11Clinical and Research Uses of the CDC NormalRange of Motion (ROM) Public Use DatasetJ. Michael Soucie, Sally McAlister; Division of Blood Disorders, Centers for Disease Controland Prevention, Atlanta, GA, U.S.A.

Objective: To describe the potential clinical and researchuses of a public use dataset of normal ROM measurementstaken on 674 healthy normal male and female subjectsaged 2 – 69 years. Methods: ROM life-span charts for eachjoint by gender can be made using the 95% confidenceintervals and population percentiles of the mean valuesfor ROM obtained from healthy subjects in four agegroups. One possible research use of the normal ROMdataset would be to combine these data with bilateralmeasures of ROM taken on the shoulders, elbows, hips,knees and ankles at the most recent clinic visit of 4,655patients with severe hemophilia participating in a publichealth surveillance system in the U.S. to generatesummary measures of the proportionate reduction fromnormal ROM. Results: In the clinical setting a normal ROMlife-span chart could be used to plot a patient’s ROM. Thiswould provide a visual tool to help a patient understandhow his joints compare to normal joints for his age group.In a research setting the average overall summary measureof proportionate normal ROM can be plotted forhemophilia patients over the life span. The plots can beused to compare joint outcomes among hemophiliaseverity groups. Relevance to hemophilia care: PlottingROM measurements obtained during clinic visits on a life-span chart can help monitor disease progression. Thepublic use dataset can be used by researchers incomparison studies and to study patterns of ROM changesin patient populations with chronic joint disease.

A4.12Postural Adjustment after an UnexpectedPerturbation in Children with HemophiliaF. Bessa de Souza1, N. Minuque1, R. Pereira1, C. Carmo1, M.Mello1, P. Villaça2, C. Tanaka1

1Department of Physical Therapy, CommunicationDisorders and Occupational Therapy; 2Hemophilia Center,Clinical Hospital - Faculty of Medicine, University of SãoPaulo, Brazil

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Abstracts (cont’d)185 MBq, Rhenium-186 for the elbows (56-74 MBq) andthe ankles (74 MBq). The procedure required 1 to 3injections of the appropriate radioactive material (RS-1, RS-2, RS-3) with 3 to 6-month intervals. Of the 104 joints, 68required RS-1, 20 RS-1 plus RS-2, and 16 RS-1, RS-2, and RS-3. Thus, the total number of RSs was 156. In eight cases(7.6%) surgical treatment (arthroscopic synovectomy) waseventually required after the failure of RS. We studied tenarticular parameters before RS and 6 months after RS toassess its efficacy: number of hemarthroses, articular pain(VAS), range of motion -ROM- (in flexion and in extension),muscular power (in flexion and in extension), degree ofclinical synovitis, size of the synovium (mm) measured byimage (ultrasonography and/or MRI), WFH clinical scoreand radiological score. Results: RS significantly improvedall the parameters except for the WFH’s radiological score.Moreover, such parameters improved in an independentway for each of the RSs (RS-1, RS-2, RS-3). On average, thenumber of hemarthroses and the degree of pain improvedby 70%. Synovial size improved by 35%. The WFH’s clinicalscore improved by 19%. Muscular power improved by12%, while ROM improved by 3%. We also observed thatthe efficacy of RS was not related to patient’s age, type andgrade of hemophilia, previous hematological treatment(on demand or prophylaxis), presence or absence ofinhibitor, synovial size, type of joint (elbow, knee, ankle),previous physical activity, presence or absence of jointdegeneration (arthropathy), radioactive material used, andpresence or absence of complications after RS. We alsofound that after RS-1 the knee had 3.4 and 3.2 times morerisk of not improving pain than elbows and ankles,respectively. Regarding ROM after RS-1, severe hemophiliahas 2.1 times more risk of lack of improvement of ROMthan moderate and mild hemophilia. Moreover, the anklehas 6 times more risk of not improving ROM than elbowsand knees. Conclusions: We have confirmed that RS is anefficient procedure for the conservative management ofchronic hemophilic synovitis, although it may require 1 to3 injections (RS-1, RS-2, RS-3) with 3 to 6-month intervals.On the other hand, studying ten articular parametersbefore and 6 months after RS, we have been able tomeasure the efficacy of RS and also define someprognostic factors.

B1.3Role of the Physiotherapist in Prevention andTreatment of SynovitisN. ZourikianPhysiotherapy Department, Sainte-Justine UniversityHospital Center, Pediatric / Adult Comprehensive CareHemophilia Treatment Center, Quebec Reference Centerfor Coagulation Inhibitors, Montreal, QC, CanadaE-mail: [email protected]

Hemophilic synovitis (HS) - an inflammation of the synovialmembrane - is directly and indirectly related to the effects

proteoglycan synthesis rate, an increase of -release, and adecrease of -content (all p<0.05). This blood-induceddamage to the cartilage matrix was limited both by IL-10and IL-4 alone. Proteoglycan synthesis rate improved by125% and 512% respectively, release decreased by 34%and 50%, and content increased by 23% and 22% (allp<0.05). Moreover, the combination of IL-4 and IL-10 wasclearly more protective than IL-10 alone. Addition of IL-4and IL-10 after the onset of blood-exposure did notprevent cartilage damage anymore unless blood was firstremoved. Conclusion: The combination of IL-4 and IL-10is most protective when added within 24 hours after startof blood-exposure. Therefore treatment should be startedas soon as possible and duration of blood-exposureshould be limited to prevent hemophilic arthropathy.

Session B1 - Synovitis

B1.1PathogenesisL. ValentinoRush University Medical Center, Chicago, IL, U.S.A.

Synovitis and arthropathy are frequent and seriouscomplications of repeated joint bleeding in patients withhemophilia, resulting in pain, deformity, disability, andreduced quality of life. While the pathogenesis ofhemophilic arthropathy has not been fully elucidated, itappears to have similarities with the degenerative jointdamage that occurs in osteoarthritis and the inflammatoryprocesses associated with rheumatoid arthritis. Thispresentation will review the potential actions of variousblood constituents on joint components that culminate inthe development of hemophilic synovitis and arthropathy.

B1.2Conservative Management of HemophilicSynovitisE. C. Rodriguez-Merchan, H. De La Corte-Rodriguez, V. Jimenez.YusteHemophilia Unit, La Paz University Hospital, Madrid, Spain

Introduction: Radiosynovectomy (RS) is an efficientprocedure for the conservative management of chronichemophilic synovitis. However, quantification of itsefficacy has not been fully investigated in the literature.We have analyzed ten articular parameters to try andmeasure the efficacy of RS. Materials and Methods: In agroup of 78 persons with hemophilia we performed 156RSs in 104 joints, under the diagnosis of hemophilicsynovitis. The mean age of patients was 18-years-old(range: 7-51). Fifty-eight patients required RS in a singlejoint while 20 patients required RS in more than one joint.Of the 104 joints treated 33 were elbows, 24 ankles, and47 knees. We used Yttrium-90 for the knees at a dose of

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Abstracts (cont’d)synovectomy in controlling recurrent bleeding was over80%. Most of these procedures were performed in patientswho already had extensive joint surface destruction,however, a natural progression to end-stage disease waseventually observed. Many patients had difficultyregaining range of motion following open synovectomy.Additionally, the procedure required considerableamounts of clotting factor replacement and prolongedhospitalization. For these reasons, open synovectomieshave been largely abandoned and replaced byarthroscopic synovectomies Arthroscopic synovectomy is recognized today as aneffective method of synovial deactivation. While its use asa first option has some advantages, there is generalconsensus that it is best used as a second level of defenceafter radiosynoviorthesis. The procedure requires surgicalexpertise and meticulous execution, and allows access tothe vast majority of the joint with minimal externalincisions. Patients require hospitalization, surgicalamounts of clotting factor replacement, and dedicatedphysiotherapy. The technique allows removal ofosteophytes, treatment of chondral lesions, andremodelling of meniscal tears, which are characteristic ofgrade III and IV arthropathy.Angiographic embolization of peri-articular arteries is apromising percutaneous procedure. Published case seriessuggest this intervention modifies the natural course ofchronic synovitis in hemophilia. This accomplishment ismaterialized in a decrease in bleeding frequency and involume of the synovial tissue. In the absence ofcomparative data it is early to conclude on the role thistherapeutic modality may play in the armamentarium forthe treatment of chronic synovitis. Given the intra-arterialnature of the procedure, generating data on safety isparamount before recommending for widespread use.

Session B2 – Inhibitor Patients: The Truth

B2.1Open Issues: Prophylaxis and ITIE. SantagostinoAngelo Bianchi Bonomi Hemophilia and ThrombosisCenter, IRCCS Maggiore Hospital Foundation andUniversity of Milan, Italy

Thanks to the progress achieved with by-passingtherapies, elective orthopedic surgery has becomefeasible in hemophiliacs with inhibitors; however onlycentres already experienced can tackle this challenge. Astrict collaboration amongst surgeons, hematologists andphysiotherapists is essential for a successful long-termoutcome. The choice of the therapeutic strategy is basedon the current inhibitor titre and the information onhistorical peak titres. Bypassing agents, activated

and consequences of hemarthrosis. Research over the past50 years has progressively offered insight and betteroptions for the management of joint bleeds and chronicHS. Nevertheless, the long-term consequences of jointbleeding and chronic synovitis in people with hemophiliastill remain a challenge for members of the comprehensivecare team, both in the developed and developing world.Prophylaxis to help prevent or reduce joint bleeds ispresently considered to be more effective than on-demand treatment following a joint bleed. Similarly, apreventative approach with respect to physiotherapy mayalso be more effective in decreasing joint bleed frequencyand directly-related complications such as HS and jointdegeneration. Specialists such as hematologists,orthopedists, rheumatologists, researchers, as well asother members of the comprehensive care team, such asphysiotherapists, nurses, and psychologists each have animportant role in the management of HS. Thispresentation will look at some of the common approachespresently used in physiotherapy treatment of HS, attemptto put them in perspective, as well as offer preventionrationales based on an amalgamation of the currentevidence.

B1.4Surgical Options, Including AngiographicEmbolizationA. LlinasDepartment of Orthopedics and Traumatology, FundaciónSanta Fe de Bogotá, Fundación Cosme y Damián,Universidad de los Andes and Universidad del Rosario,Bogotá, ColombiaE-mail: [email protected]

Surgical synovectomy, whether open or arthroscopic,requires extensive resources from an experienced team, adedicated hemophilia treatment centre, and a large supplyof clotting factor. Surgical synovectomy is seldomnecessary today and is only considered when other lessinvasive and equally effective procedures fail. Thepopulation with synovitis refractory to non surgicaltreatment constitutes a challenging subgroup to treat.Identifying the reasons that lead to failure of previous, lessinvasive approaches is key to achieve success. Theexplanation for the failure is most probably multi-factorial,and may include undetected inhibitors, early bleederphenotypes, association with other coagulopathies, intra-articular and peri-articular mechanical problems.Examples of the latter are torn meniscus, intra-articularloose bodies, and quadicipital palsy secondary to previousinjury of the femoral nerve during a psoas-iliacus muscularhemorrhage.Historically, synovectomy for chronic hemophilic synovitiswas performed through an open arthrotomy. Withextensive surgical approaches, most of the synovium canbe removed from a joint. The success rate of open

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Abstracts (cont’d)that patients with inhibitors often have a significantburden of disability with inevitable adverse impact of theirquality of life. Elective orthopedic surgery can certainly beundertaken with good results nowadays, although thisrequires careful planning and should only be undertakenin centres with the requisite experience. Full evaluation ofhemostasis, including platelet function tests, should becarried out prior to surgery. Patients with liver disease orHIV infection may also require treatment with fresh frozenplasma and/or platelet concentrates. Fibrin sealants canhelp to reduce blood loss during surgery and tranexamicacid should be used in conjunction with NovoSeven.Laboratory monitoring of peri-operative therapy isgenerally not needed but a number of groups are seekingto apply thromboelastography and thrombin generationtesting. Both FEIBA and recombinant activated factor VIIa(NovoSeven) have been widely used to cover electivesurgery, with good success. The cost of both bloodproducts is very high but may be recovered in subsequentyears as a consequence of reduced bleeding frequency.More conservative options may need to be considered as alternatives to surgery. These include treatment withCOX-2 anti-inflammatory agents such as Rofecoxib,radionuclide or chemical synovectomy, and embolization.

B2.4Inhibitor Patients: The RehabilitationProgram, Is It Different?A. ForsythPenn Hemophilia and Thrombosis Program, Thorofare, NJ, U.S.A.

Even with advances in preventing and treating bleedingepisodes, arthropathy continues as a common co-morbidity in persons with hemophilia (PWH). Arthropathymay result in the need for elective orthopedic surgery(EOS) to address pain and functional limitations. In PWH,EOS is common in countries where it is readily available,and with bypassing agents and accruing experience inhemophilia treatment centres (HTCs), EOS is now beingcarried out more in PWH with inhibitors. The physio -therapist is an integral member of the comprehensive,multidisciplinary team. For optimal functional outcome,physiotherapy is critical leading up to EOS in PWH withinhibitors, and during post-operative recovery. Pre-operative goals include preparation of the PWH for therehabilitation program and assuring realistic expectationsof the specific EOS. While addressing the muscles andjoints adjacent to the operative site, as well as the affectedjoint, the experienced physiotherapist may be able tojudge the PWH’s level of participation. This may in turnhelp predict adherence to prescribed treatments aftersurgery, a factor that will have a substantial impact onrecovery. During post-operative rehab, the physiotherapistcaring for the PWH with inhibitors must negotiate a finebalance between intervention and minimizing the risk ofbleeding into the operative joint or at other sites. Bleeding

prothrombin complex concentrate (aPCC), andrecombinant activated factor VII (rFVIIa), represent the firstchoice in patients with high-responding inhibitors. No by-passing product can guarantee sustained hemostasis andno laboratory assay is available to predict the efficacy oftreatment. Caution is recommended administeringbypassing agents in elderly patients or in those withthrombotic risk factors. High-dose factor replacement mayneutralize low-titre inhibitors, allowing achievement andmaintenance of the desired factor levels to cover surgery.A preliminary pharmacokinetic study is useful in thesecases to establish the optimal dose regimen. Factorreplacement therapy represents the first-choice approachfor patients with low-responding inhibitors and it couldalso be considered in high-responders who have atemporarily low inhibitor titre. Laboratory monitoring iscrucial for dose adjustment and factor recovery should bemeasured at least daily until discharge. The cornerstone ofpost-operative management is to avoid bleedingcomplications and if bleeding occurs it must be treatedwithout delay. Early mobilization should be individuallytailored and started with caution; a single concentrateinfusion may be given before the physiotherapy session inorder to reduce the bleeding risk.

B2.2Before Surgery: What the Surgeon Wants toKnowP.L. SolimenoOrtho-Trauma Unit, IRCCS Cà Granda Foundation,Policlinico Hospital, Milan, Italy

The management of recurrent hemarthroses, chronicsynovitis, and chronic arthropathy is more challenging inpatients who developed an inhibitor against FVIII/FIX, themost common and most serious complication ofreplacement therapy in patients with hemophilia A or B.In such patients, although the frequency and severity ofhemarthroses is similar to non-inhibitor patients, weregistered a severe degree of arthropathy related to minortreatment response. The introduction of bypassing agentsas rFVIIa has improved the management of recurrentbleeding episodes and synovitis in hemophilic patientswith inhibitors ,and today it is possible to performsurgeries in inhibitor patients, even if we have a highercomplication rate in comparison with non- inhibitorpatients. The author describes the pre-op managementfrom a hematological and orthopedic point of view toavoid risk of complications.

B2.3Before Surgery: A Hematologist’s ViewP. GiangrandeOxford Haemophilia & Thrombosis Centre, U.K.

The life expectancy of people with hemophilia in affluentcountries is now very similar to that of the generalpopulation. This good news is counterbalanced by the fact

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Abstracts (cont’d)Session B3 - Musculoskeletal Issues inPatients with VWD and RBD

B3.1 The HematologistD. QuonOrthopaedic Hospital Hemophilia Treatment Center, LosAngeles, CA, U.S.A.

The Hematologist’s Perspective on Musculoskeletal Issuesin Patients with VWD and RBD. Von Willebrand disease (VWD) is the most commoninherited bleeding disorder, occurring in approximately1% of the general population. It is caused by a qualitativeor quantitative deficiency in von Willebrand factor (VWF)which results in an increased risk of bleeding. In contrast,rare bleeding disorders (RBD) affect less than 5% of allpatients with bleeding disorders (hemophilia and VWDcombined constitutes the remaining 95%). The clinicalmanifestations of VWD may vary substantially from patientto patient with the majority of the patients experiencingmucocutaneous hemorrhage. Despite the prevalence ofVWD, there is a paucity of published information regardingmusculoskeletal or orthopedic complications in thesepatients, and even less is known in the cases of RBD. Themajority of the information that is available is extrapolatedfrom hemophilic arthropathy as the disease shares manysimilarities with other coagulopathies. Many studies have“lumped” taken cases of VWD and RBD into the cohorts ofhemophilic patients. The current status of these issues willbe discussed.

B3.2The Orthopedic SurgeonO.S. PerfettoSant’Ambrogio Clinic, Milan, Italy

Von Willebrand disease (VWD) is the most commonhereditary bleeding disorder affecting both males andfemales, with an estimated prevalence of 1.3% in thegeneral population. Clinical manifestations of the diseasesprim arily consist of excessive and prolonged muco -cutaneous and post-operative bleeding that result fromquantitative or qualitative defects in the adhesiveglycoprotein von Willebrand factor (VWF). Rare bleedingdisorders (RBDs) represent 3 to 5% of all the inheritedcoagulation deficiencies and include the inheriteddeficiencies of fibrinogen factor (F) II, FV, FV + FVIII, FVII,FX, FXI, and FXIII, and are usually transmitted as autosomalrecessive traits, meaning that women represent about halfof the affected patients. The authors described theirsurgical experience (surgical indications, peri-operativemanagement and complications) in the last years in thesepatients: shoulder replacement, knee replacement, hipreplacement, and shoulder and knee arthroscopy.

into the operative joint will not only hinder recovery butmay also set the stage for infection, which may lead tofurther complications, including subsequent need forsurgery. The rehab program in a PWH with inhibitors hasunique challenges above and beyond rehab of PWHwithout inhibitors, which will be highlighted.

B2.5Before Surgery: Are the Results of SurgeryDifferent?N. GoddardRoyal Free Hospital, London, U.K.

The availability of by-passing agents (rFVIIa or FEIBA) forhemostatic cover in patients with inhibitors has permittedmajor surgery that was previously considered to beimpossible. Globally, experience of performing orthopedicsurgery in patients with inhibitors is increasing. Thesurgery being performed has become more enterprisingand is certainly not for the faint-hearted. Thromboticcomplications are rare in inhibitor patients and surgeonsfrequently ignore the concept of thromboprophylaxis.However, bleeding complications, problems with woundhealing and resulting increased infection rates remain aconcern. Despite our best endeavours it has not beenpossible to establish a worldwide register of patientsundergoing such surgery and we are still somewhat in thedark as to the ultimate outcomes. There are still only alimited number of publications in this field, often singlecase reports, small series or pooled data from multiplecentres with no standardised treatment regimes. Currentpublished guidelines are seemingly based on a “bestguess” scenario rather than true “best practice” and areliable evidence base. It is probable that there is a bias inthe literature towards reporting positive outcomes andconsequently failures of treatment are under reported. Itis vital to obtain this information lest we repeat the errorsof others since only with appropriate data can we makesuitable recommendations for peri-operativemanagement. As far as I can tell the published results formajor surgery (TKR or THR) in patients with inhibitors havebeen good. However there is sufficient anecdotal evidenceto suggest that there are still serious concerns about theincidence of post-operative infection and prematurefailures. The increased risk, coupled with the vastlyincreased expense of hemostatic cover has led some toquestion the wisdom of performing surgery in thesepatients. Clearly there are some fundamental questions ofhealth economics, but in addition we should provideguidance as to which patients are, or are not suitable forsurgical intervention and to provide robust recom -mendations for peri-operative hemostatic treat mentregimes.

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Abstracts (cont’d)(4 right and 3 left) were treated surgically with inverted “V”distal femoral osteotomy, the internal fixation was withSteinman wire and external immobilization with long cast.Results: Bone healing was achieved at an average of 9.1weeks (8-10 weeks). The follow-up was 2.8 years(maximum 4 and minimum 1). Postoperatively, patientswere sent to physiotherapy for muscle strengthening andgait training. Extension was achieved in 100% of cases. Theaverage active motion was 60°. No patient had pain in themobility and gait. The average number of hemarthrosiswas 3.1 times per year. Conclusions: The inverted "V"osteotomy is very stable, easy to perform and has low risk,requires minimal internal fixation that is removed at thesame time as the external immobilization and is useful incountries with few resources because requirements areminimal.Key Words: contracture, femoral osteotomy

B4.2Cysts in Patients with Hemophilia: BoneRestoration by Means of HydroxyapatiteCorallineH.A. Caviglia1, 2, G.A. Galatro1, 2, N. Moretti1, C. Daffunchio1,2,R. Perez Bianco1

1Fundación De La Hemofilia; 2Hospital General de AgudosJuan A. Fernández, Buenos Aires, ArgentinaE-mail: [email protected]

Introduction: Subchondral bone cysts are frequent inhemophilic patients and progress to articular damage.Objective: The aim of this study is to show the restorationof the bone architecture filling with hydroxyapatitecoralline in hemophilic patients with subchondral cysts.Materials and Methods: 31 male patients with 39 lesionswere treated. 29 patients suffer from hemophilia A and 2from hemophilia B. 2 patients had inhibitors and 1 was alow responder. The mean age was 23 years (6-47). Themean follow-up was 7 (1-16). The most frequent locationof the cysts was tibia (19), talus (7), ulna (5), humerus (3),femur (2), and radius (1). Surgery was indicated when thesize of the subchondral cyst is greater than 15% of the areaof the joint, particularly if it includes load articulations orwhen the control x-ray shows the progression of the cystsize, even if its subchondral extension is less than 15%.Every cyst was evaluated with X-ray, CT scan and MRIbefore surgery. All patients were treated in the same way,guided with image intensifier, cyst aspiration, and filledwith hdroxyapatite coralline. Results: 38 lesionsprogressed to healing. Restoration took 10 months. In 1patient the filling of the cyst was inadequate and requiredfurther surgery to complete the filling, which finallyevolved to healing. Conclusions: Filling subchondral cystswith hydroxyapatite coralline prevents the progression ofjoint damage and facilitates reconstruction with aprosthetic implant in the future.

B3.3The PhysiotherapistG. BlameyWinnipeg, Canada

This presentation will focus on the musculoskeletal issuesrelated to von Willebrand disease (VWD) and rare bleedingdisorders (RBD), and how they compare to those mostcommonly experienced with hemophilia. Key differencesbetween the coagulopathies themselves as well as thetypes of patients that they affect present thephysiotherapist with challenges not usually encounteredwhen treating hemophilia. Some of the issues faced aredue to the pathology of the disorders themselves, whileothers relate to the medical treatment that is provided. Thepresentation will address how the impact of gender,longer factor half lives, tissues targeted by the variousbleeding disorders, and whether their impact poses adirect or an indirect threat to the MSK system. Thispresentation will assist the physiotherapist to determinethe types of issues that will need to be addresses withVWD and RBD, and therefore in selecting the treatmenttechniques that are most appropriate.

Session B4 – Free Papers: Surgical

B4.1Flexion Deformity in Knee: Femoral ExtensionOsteotomy in Pediatric Patients withHemophiliaR. Bernal-Lagunas1, J. Aguilera-Soriano1, I. Kalach-Bucay1,S. Fuentes-Figueroa1, A. Berges-Garcia2, F. Gonzalez-Martinez3

1Servicio de Ortopedia Pediátrica, Hospital de Ortopedia,Unidad Médica de Alta Especialidad (UMAE) Magdalenade las Salinas, Instituto Mexicano del Seguro Social (IMSS);2Servicio de Hematología Pediátrica, Hospital General,Unidad Médica de Alta Especialidad (UMAE), CentroMédico Nacional “La Raza”, Instituto Mexicano del SeguroSocial (IMSS); 3Servicio de Medicina Fisica y Rehabilitación,Hospital Colonia, Instituto Mexicano del Seguro Social(IMSS), Mexico City, MexicoE-mail: [email protected]

Objective: Display experience with the use of femoralosteotomy in inverted "V" for the correction of knee flexiondeformity. Is a very stable metaphyseal osteotomy thatprovides a wide contact surface and easy fixation withcross Steinman wires. Material and Methods: A total of7 patients with knee flexion contractures were treatedbetween 2006 and 2009, they had severe hemophilia A.Average age was 11 years (8 to 15 years). No response toprevious physiotherapy treatment. Showed flexioncontracture of the knee between 40° and 70° (3 cases: 40°,2 cases: 50°, 1 case 60°). All had pain in active-passivemobility and gait. The average number of hemarthrosiswas 13.4 times per year (1.11 times per month). The 7 cases

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Abstracts (cont’d)Y90 and Re186 in the young population with hemophilia.Micronucleus (MN) assay on peripheral blood lymphocyteshas been reported as one of the best established in vivocytogenetic assays in the field of genetic toxicology.Methods: 19 patients exposed to RS were enrolled in thestudy. Mean age was 20.6±10.4 years-old (range: 6-52),and 17 patients had hemophilia A. 18 patients withhemophilia who were not exposed to RS procedure wereselected as a control group (mean age: 22±10.6 years-old,range: 5-45 /13 hemophilia A). MN assay was performedusing cytochalasin-B and 1000 binucleated cells werescored under light microscobe in the Medical Geneticslaboratory. Results: There was no significant differencebetween patients (18.2±10.3 per 1000 cells) and controls(16.6±7.8) in respect of MN values. However, both valuesobtained in RS exposed patients and control group weremuch elevated than values reported from healthy controls(4.4-9.8/1000 cells). The mean values of patients below 20years old (15.4±8.2) were much lower those above 20 yearsold (22.2±12.0), however there was no difference. MNfrequencies between Re186 (22.5±10.0) and Y90(19.3±11.3) groups were also analysed, and no significantdifference was observed. Conclusion: In conclusion,radioisotope synovectomy seems to be a safe procedure,however further studies including larger series of patientsare needed to better understanding the effects on healthstatus.

B4.5Nerve Entrapment and Neurodiagnostics,Our Experience in HemophiliaD. Magliaro, G. Lenciza, P. Manngearelli, M. Davoli, V. Rescia Ilar, Rosario, Sante Fe, ArgentinaE-mail: [email protected]

Introduction: Often the presence of hematoma inpatients with hemophilia can occasionally cause entrap -ment neuropathies of various nerves according to theirlocation. Although the neurological symptoms of thenerve entrapment is very typical, it is necessary to confirmthe same non-invasive methods that allow us to quantifythe damage, from which the most appropriate treatmentcan be designed. Methods: Neurophysiologicaltechniques were used, motor and sensory nerveconduction in nerve stimulation and recording throughsurface electrode bar or rings. Results: Clinical suspicionof entrapment neuropathy was found in 9 patients withpositive studies, of which all but one were resolved withphysiotherapy and medication methods, only onerequired surgical intervention for resolution. The nervesaffected were mainly found in the median nerve in theulnar nerve second, then the radial nerve, and finally asingle case of femoral cutaneous lateral nerve.Conclusion: In this paper we show our experience in thedetection, confirmation, and treatment of nerveentrapment in hemophilia patients with the use of nerve

B4.3Correlation Between MusculoskeletalFunction and Radiological Joint Scores inHemophilia A AdolescentsT.H. Hassan1, M.A. Badr1, K.M. El Gerby2

Department of 1Pediatrics and 2Radiodiagnosis, ZagazigUniversity, Cairo, Egypt

Key Words: Musculoskeletal; Hemophilia

Introduction: The Functional Independence Score inHaemophilia (FISH) is a performance-based instrumentused to assess musculoskeletal function in patients withhemophilia. We aimed to evaluate the functionalindependence of hemophilia A adolescents and itscorrelation to radiological joint scores. Methods: A crosssectional study was carried out on 50 adolescenthaemophilia A patients. Musculoskeletal function wasassessed using the FISH score and individual joints wereassessed radiologically using the Pettersson score and MRIscale. Data were checked, entered, and analyzed usingSPSS version 11. Results: The mean age of our patientswas 16±1.1 with a mean FISH of 23.32 ± 4.69 (range 13-28), and a mean Pettersson score of 2.32 ± 3.09 (range 0-13) for the knees, 1.86 ± 2.67 (range 0-11) for ankles, and1.42 ± 2.17 (range 0-10) for elbows. The mean MRI scorefor the knees was 3.92 ± 2.74 (range 0-10) while that forankles was 3.16 ± 2.64 (range 0-10), and for elbows was2.34 ± 2.63 (range 0-10). There was highly significantcorrelation between both radiological joint scores andFISH and between degree of factor VIII deficiency and eachof FISH, Pettersson score and MRI score. MRI was superiorto conventional radiography in detection of subchondralcyst formation and erosions at joint margins. Conclusion:Given the highly significant correlation with bothradiological joint scores, FISH appears to be a reliable toolfor the assessment of functional independence inadolescents with hemophilia A. MRI is more sensitive thanconventional radiography in detection of early jointabnormalities.

B4.4Micronucleus Evaluation for PotentialChromosomal Breakages in HemophiliacPatients Who Have Undergone RadioisotopeSynovectomyK. Kavakli1, S. Aydogdu2, O. Cogulu3, H. Ozkilic4, E. Karaca3,B. Durmaz3, C. Balkan1, D. Yilmaz 1, Y. Ay1, F. Ozkinay3

1Dept. Hematology, Ege University Children's Hospital;2Dept. Orthopedics; 3Dept. Medical Genetics; 4Dept.Nuclear Medicine, Ege University Hospital, Istanbul, TurkeyE-mail: [email protected]

Introduction: Radioisotope Synovectomy (RS) is definedas the intra-articular injection of radioisotopic agents inthe target joint of patients with hemophilia. The aim of thisstudy was to investigate the potential genotoxic effects of

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Abstracts (cont’d)treatment of inflammatory arthropathies. Their efficacyhas been addressed by several trials, but safety concernsdue to the theoretical risk of stochastic effects andreported cases of malignancy have precluded their use inmany countries. Methods: We approach the issue ofsafety through 15 years of patient follow-up after P-32radiosynovectomy performed by our multidisciplinaryhemophilia group in collaboration with nuclear medicinespecialists. Results: Since 1995, 287 individual jointradiosynovectomies were performed in 148 patients aged4 to 71-years-old (mean 21.9). The main underlyingcondition leading to arthropathy was hemophilia (71.6%),followed by rheumatoid arthritis (9.5%), degenerativearthritis (6.1%), PVNS (6.1%), and seronegative arthritis(4.1%). Joints treated were knees (53.3%), elbows (27.5%),ankles (8.3%), hips (5.9%), and shoulders (3.1%). Doses ofP-32 in colloid form per joint range from 0.25 to 1 mCi, andthe number of joints treated per patient from 1 to 10(mean 1.97). Average follow-up for all joints is 8.5 years(range 16 months to 15.8 years). There have been nodocumented cases of malignancy in patients exposed tothe radioisotope. During follow-up, one patient died froma hemorrhagic complication of hemophilia. Conclusions:With this series we contribute cumulative data to theuniversal pool dedicated to establishing the long termsafety of radiosynovectomy. Verifying the long term safetyof radiosynoviorthesis is relevant given that when pairedwith its known efficacy for controlling synovialhypertrophy, this procedure is a highly risk-effectiveintervention. Maintaining strict clinical observation on thiscohort will provide additional safety information as thepopulation ages. Key words: hemophilic arthropathy, radiosynovectomy

B4.8CT Guided Percutaneous Decompression ofIliopsoas Hematoma in Hemophiliac Patient:Case ReportJ.H. Ramírez1, P. Ramírez2, G. Salom3

Departments of 1Orthopedic; 2General PhysicianHemophilia Centre; 3Radiology, Diagnostic Imaging,Centro Medico Imbanaco, Cali, ColombiaE-mail: [email protected]

Iliopsoas hematoma is a complication in hemophilia. Itoccurs in 0.3-13% depending on the cited source. It ispotentially life threatening with a high risk ofcompartment syndrome (CS) and femoral neuropathy. Thecurrent treatment is conservative, with high doses ofcoagulation factor and bed rest. Percutaneousdecompression is used on different pathologies, but oniliopsoas hematomas there are only 3 successful casespublished on patients with anticoagulant therapy. Wereport a case of a 14-year-old male patient, with moderatehemophilia A and severe left groin pain, hip in flexion,positive psoas sign and hypoesthesia on femoral nerve

conduction techniques for noninvasive neuro -physiological treatment.

B4.6Inter-Observer Reliability of Three Types ofRadiographic Scores for Adult HemophiliaH. Takedani1, T. Fujii2, Y. Kobayashi3, N. Haga4, S. Tatsunami5

1Department of Joint Surgery, Research Hospital of TheInstitute of Medical Science, The University of Tokyo;2Department of Transfusion Medicine, HiroshimaUniversity Hospital; 3Department of Rehabilitation,Fukuoka Houeikai Hospital; 4Department of Rehabilitation,The University of Tokyo; 5Unit of Medical Statistics, Facultyof Medical Education and Culture, St. Marianna UniversitySchool of Medicine, Tokyo, JapanE-mail: [email protected]

Introduction: The purpose of this study is to evaluate theinter-observer reliability of radiological assessmentsystems for hemophilic arthropathy. Methods: Threesenior orthopedic surgeons with expertise in hemophiliaindependently evaluated a total of 527 joint radiographsof adult hemophilia patients, without any knowledge ofthe clinical data. Results: This study was the largest studyto evaluate the reliability of radiological assessmentsystems. As for the results, the Arnold-Hilgartner stagingsystem showed moderate reliability (kappa value: к=0.44,p=0.000), and the De Palma grading system and thePettersson scoring system showed fair reliability (к=0.40,p=0.000) and slight reliability (к=0.12, p=0.000),respectively. As for the reliability of the eight findings inthe Pettersson scoring system, three findings showedsubstantial or moderate reliability. These were “narrowingof joint space” (к=0.70 p=0.000), “irregular subchondralsurface” (к=0.58, p=0.000), and “erosion of joint margins”(к=0.56, p=0.000). Other findings showed fair or lessreliability. The traditional radiological assessment systemsshowed poor inter-observer reliability. Both progressivescales showed higher reliability than the additive scale,and the three findings in the Pettersson scoring systemshowed good reliability. Conclusion: Our results suggestthat the progressive scale, including the three reliableradiological findings, might be a more reliable radiologicalassessment system.

B4.7Pharmacovigilance in Patients WhoUnderwent Radiosynovectomy with ColloidalChromic Phosphate P-32J. Pérez1, A. Llinás1, M. Duarte2, G. Ucrós3

Departments of 1Orthopedics; 2Internal Medicine, Hemat -ology Section; 3Nuclear Medicine, Fundación Santa Fe deBogotá, Bogotá D.C., ColombiaE-mail: [email protected]

Introduction: Radioisotopes as localized radiotherapy forresistant synovitis have been widely implemented for the

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Abstracts (cont’d)B4.10Arthroscopic Synovectomy with JointDistraction Using a Patella Tendon BearingBrace for Severe Hemophilic AnkleArthropathyY. Tanaka1, A. Taniguchi1, T. Matsuda1, S. Kamijo1, S.Tsukamoto1, T. Kumai1, M. Shibata2, I. Tanaka2, M. Shima2, A.Yoshioka2

Departments of 1Orthopedic Surgery and 2Pediatrics, NaraMedical University, Kashihara, JapanE-mail: [email protected]

Introduction: Although arthrodesis is a gold standard forsevere ankle arthropathy, we would like to avoid it,especially for young patients. We devised treatment withjoint distraction using a patella tendon bearing (PTB) braceafter doing synovectomy. The purpose of this study was toevaluate the clinical results of our procedures. Methods:Nine patients (all boys, 5-18-years-old, mean 10-years-old)with progressed ankle arthropathy were treated. Carefularthroscopic synovectomy was performed and defectivecartilage areas were treated with bone marrow stimulationtechnique. The PTB brace was applied for 1 year post-operatively. Clinical results and radiographic findingsusing weight-bearing views were evaluated. Follow-updurations were from 24 to 64 months, with an average of48 months. Results: Pain and disturbance of ADL weredramatically improved. An average AOFAS score wasimproved from 59 points to 88 points. Episodes of intra-articular bleeding were significantly decreased after thetreatment. Erosive changes were repaired and narrowingof joint space was recovered to nearly normal. The Arnold-Hilgartner stages also were improved. The averagePettersson score was improved from 7.7 to 4.7. Autism inone patient was cured post-operatively and he playsdrums in his band now. Conclusion: Arthroscopicsynovectomy is an established therapeutic option forhemophilic ankle arthropathy. However, it was well knownthat synovectomy could not improve existing jointdegeneration. Our devised treatment could improveradiographic stages for progressed ankle arthropathy. Itshould be done before indicating arthrodesis for youngpatients.

B4.11Feasibility of Continuous Peripheral NerveBlock for Post-Operative Analgesia inPatients with Severe HemophiliaA. Tirichine, S. Fourmas, K. Koroghli, A. Benbouzid, S.Hassam, L. Ait Elhadj Specialized Hospital of Ben-Aknoun, Algiers, AlgeriaE-mail: [email protected]

Background: Hemophilic patients with hemarthropathyare chronic pain patients. Pain treatment is essential inorthopedic surgery. Procedures are often associated withrelevant post-operative pain. Aim: Moreover in

territory. He received factor VIII replacement andanalgesics. CT reported acute left iliopsoas hematoma. Wedecided to perform a CT guided percutaneousdecompression under strictly sterile conditions, with a No.18 lumbar puncture needle. Intracompartmental pressurewas measured with a manual manometer. 30 mmHg wasthe first measurement, 10 ml of blood was drained andposterior pressure was 23 mmHg with immediate painrelief and improvement on the leg’s sensibility. Thisprocedure is a valuable alternative in severe iliopsoashematoma treatment with CS. It has to be performed bytrained physicians in a hemophilia treatment centre. Key words: Iliopsoas hematoma, percutaneousdecompression

B4.9Long-Term Outcome of Total Elbow Endo -prosthesis in Hemophilia Arthropathy May BeSuperior to Radial Head Prosthesis – SomeCase ReportsP. Staritz1, P. Lage1, S. Weiss2, Z. Rainer1, H. Angela1

1Hemophilia Care Center Heidelberg; 2Department ofOrthopedic Surgery, University of Heidelberg, GermanyE-mail: [email protected]

Introduction: The elbow represents the second mostinvolved joint in hemophilic arthropathy. In contrast to hipand knee, total elbow replacement is not yet anestablished therapy (less functional impairment, no long-term results, early loosening of the prosthesis, design ofthe implants). Long-term outcomes are still awaited.Methods: Since 2001, 6 patients (35-64 years-old) withadvanced arthropathy of the elbow received a total elbowprosthesis, and 1 patient (27-years-old) a radial headprosthesis. We evaluate every patient at least once per yearincluding functional outcome in daily life, range of motion(ROM), bleeding pattern, and pain assessment. Results:Functional outcome in everyday life is highly satisfactoryin all patients. ROM depends mainly on pre-operativestiffness of the joint capsule. The median increase ofmotion was 20°, full extension in most cases is notachieved. 5/7 patients had complete relief of pain.Remaining pain was observed in one patient with totalelbow replacement, suffering from dysesthesia of thehand improving under gabapentin therapy. No completepain relief and increasing pain in the meantime was seenin the one patient with radial head prosthesis in 2007.There seems to be a continuous trauma by the radial headprosthesis eroding the opposite joint partner, leading torepeated micro bleedings. Conclusion: Total and partialelbow arthroplasty reduces significantly or even resolvespain, improves the range of motion and the capacity of theelbow joint. Radial head prosthesis is only a minor surgicalprocedure but may have disadvantages in hemophilicarthropathy, causing micro bleedings in the oppositebone.

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Abstracts (cont’d)acid every four months for a six-month period. The primaryoutcome was change in lumbar spine BMD (LSBMD) Z-score at 6 months determined using dual X-rayabsorptiometry, secondary outcomes included adverseevents. Results: Six patients with on-demand treatmentand a median age of 12-years-old were included. Atbaseline the median value of LSBMD Z-score was -2.6 SDcompared with a -0.95 Z-score value at 6 months (p =0.027), this significant difference persisted until 12 months.The treatment was well tolerated; adverse reactionsconsisted of flu-like symptoms and pruritus, whichresolved without any further action. Conclusions: Our datasuggest that Zoledronic acid seems to be a safe andeffective option to get the LSBMD back to normal after 6months in all children with hemophilia, however, this wasan open and uncontrolled study, and double-blind,placebo-controlled studies are needed.Keywords: Children, Osteoporosis

B4.13Perioperative Clotting Factor Replacementand Infection in Total Knee Arthroplasty N. Goddard, J. Wong, H. Mann Royal Free Hospital, London, U.K.E-mail: [email protected]

Hemophilia is an X-linked inherited bleeding disorderwhich can cause severe arthropathy. The knee is the mostcommonly affected joint, and is associated with the mostsignificant disability and impaired quality of life. Over thepast few decades, total knee arthroplasty has become themost common surgical procedure performed in adulthemophiliacs. The outcomes of total knee replacements(TKR) in the published literature is, however, highlyvariable, with infection rates of 1-16% reported in studies.One contributing factor may be the differing clottingfactor replacement protocols used. Current WorldFederation of Hemophilia guidelines for perioperativeplasma factor VIII levels recommend a pre-operative levelof 80-100%, tapering down to 30-50% at 14 days. Recentanimal studies have suggested that high initial levels ofthe deficient plasma factor are not sufficient to normalisewound healing, and that normal wound healing requiresadequate hemostatic function for an extended period oftime. The purpose of this study was to compare thediffering clotting factor replacement protocols used in thepublished series of TKR for hemophilic arthropathy, inorder to determine if a relationship exists with infectionrates. A meta-analysis of the published literature shows astatistically significant relationship between infection andclotting factor replacement. This would seem to supportthe use of 100% factor replacement perioperatively andthe maintenance of high levels for 2 weeks post-operatively. A cost analysis of this protocol shows thatwhilst there may be an increased “up-front” costimplication, the ultimately reduced complication ratemakes the high dose regime more cost-effective in the

hemophiliac patients, the implementation of surgeryrequires early mobilization for rehabilitation in order tooptimize functional recovery (a further cause of majorpain). Continuous peripheral nerve block (CPNB) hasbecome the standard approach; this study assessed theirrealization (safety) and effects in these patients. Material& Methods: At our hospital, under adequate factorreplacement, CPNB (Contiplex B.BraunR) can beperformed before (35% of cases) or after the patient wasawake from general anesthesia (65%), using ultrasoundor electrical nerve stimulation guidance. The interruptionof the sensitive transmission can be obtained with lowdoses of anaesthetic, in the absence of motor block withthe use of elastomeric pump (EasypumpR8ml/h) with asolution of bupivacaine 0.125%. 27 patients were included.They underwent a five-day programme of early jointmobilization - factor activity level was maintained above30%, and they were evaluated for placement of catheteras the primary outcome; secondary outcomes included:VAS visual analogue scale, adverse effects and theirsatisfaction of pain control and compliance withphysiotherapy. Results: The success rate of catheterplacement is higher using US (100%) compared toelectrical nerve stimulation (92%). VAS diminished to 2.5in all patients except three. Adverse effects=1; satisfactionand good compliance of the patient with mobilization (allbut two). Conclusion: CPNBs may be safely performed inhemophiliac patients and provides excellent post-operative analgesia. The anesthesiologist should be incharge of adopting most adequate approaches in order tocontribute to improve treatment quality.Keywords: pain, CPNB, safety, rehabilitation

B4.12Efficacy of Zoledronic Acid to Normalize theLumbar Bone Mineral Density in Childrenwith HemophiliaA. Tlacuilo-Parra1, P. Lopez-Barrera1, M. Fernandez-Ceseña1,R. Garibaldi-Covarrubias1, J. Soto-Padilla1, J. Orozco-Alcala2

1UMAE Hospital de Pediatria CMNO IMSS; 2University ofGuadalajara, MexicoE-mail: [email protected]

Pediatric patients with hemophilia often have low bonemass for their age. Bisphosphonates are used for thetreatment of adult bone diseases associated with excessiveresorption, but there has been increasing and successfulapplication in pediatric bone diseases. Trials usingZoledronic acid have never been performed in thispopulation. Objective: To determine the safety andefficacy of Zoledronic acid to improve bone mass inchildren with hemophilia. Methods: In an open-labelphase II trial, patients with hemophilia and osteopenia(defined as lumbar spine bone mineral density (BMD) Z-score less than 2 standard deviations (SD) below the meanfor sex and age at baseline), were treated with Zoledronic

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Abstracts (cont’d)orthoses has two main goals. Firstly, to reduce the forcesthrough the ankle allowing weight to bypass the inside ofthe joint. Secondly, to physically restrict the movementsof the ankle reducing the forces passing through the jointin this way also prevents the impingement of alteredsynovia. As long as symptoms are limited to weightbearing activity and not present when resting or duringsleep, an orthotic is useful. Once the symptoms are notalleviated by wearing orthotics then more invasivetreatments such as surgical options may be considered. Key words: Hemophilia, Ankle Arthropathy, Orthoses.

C1.3Pre- and Post-Operative Physiotherapy Man -agement of Hemophilic Ankle ArthropathyS. Lobet1, 2, M. Avaux2, C. Hermans1

1Haemostasis and Thrombosis Unit, Saint-Luc UniversityHospital, Catholic University of Louvain, Brussels, Belgium. 2Institute of Neurosciences, Catholic University of Louvain,Brussels, BelgiumE-mail: [email protected]

The ankle is a common site for spontaneous hemarthrosisin patients with hemophilia. Bleeding episodes principallyaffect the tibiotalar and/or the subtalar joints, and maylead to severe degenerative changes. Several mechanicaland functional deficits have been identified that maycontribute to associated pain and functional loss.Associated muscle atrophy, decreased tibio-talar andsubtalar range of motion, muscle flexibility, andproprioception because of the pre-existing target joint aregenerally evident. One of the hidden symptoms isdecreased range of motion of the midtarsal andtarsometatarsal joints. This deficit could predispose thepatient to increased pain, stiffness, and disruptedproprioceptive input to the sensorimotor system.Therefore, improving accessory and physiological motionat the entirety of the ankle and foot joints is a clinicalconsideration. Localized joint mobilizations could be aneffective intervention. The role and effectiveness forconservative treatment, such as medication, patienteducation, shoe modification, and physical agents will bediscussed in this presentation. Surgical procedures caninclude synovectomy, synoviorthesis, debridement, orarthrodesis. Synovectomies are advocated to help stopuncontrolled, repetitive cycles of joint bleed and/orchronic synovitis. As the involved synovial membrane isdestroyed, joint bleeding is often resolved or significantlydecreased. The key is in taking advantage of thesubsequent, relatively safe, bleed-free period to proposea tailored physiotherapy program. After ankle arthrodesis,mobilizations to unfused joints may begin after two weekspost-op. Once the fusion has completely healed, low levelbalance and proprioceptive exercises can be started. Aninsert in the shoe or an orthopedic rocker sole will facilitatethe foot to move through a step.

long term. We believe that our findings support a changein the current international guidelines.Key words: Knee arthroplasty, peri-operative management

Session C1 - Ankle Arthropathy

C1.1Conservative Management: PhysiotherapyA. Thomas, H. Caviglia, G. Galatro, N. Moretti, C. DaffunchioBuenos Aires, Argentina

Ankle arthropathy is one of the most common complaintsin patients with hemophilia, even in young and adolescentpatients. There are many treatment options, but theconservative way is the first option. It is very important tolearn and understand what the typical evolution of thearthropathy of this joint is like and to identify the boys andadolescents with high risk of developing this condition.Once it is settled, it is essential to recognize the typicalpatterns that include loss of flexibility and range ofmotion, decrease of the strength of certain muscle groups,and impoverishment of proprioception and neuro-musclecontrol. In addition, it is important to emphasize that themajority of these patients have not only the ankle butmany joints affected, so their walking patterns areinvolved. In our experience treating many cases of patientswith this condition, we have obtained very good resultsadequately applying modalities, strengthening andflexibility exercises, proprioception drills, and walking re-education. This presentation hopes to transmit the way inwhich this condition can be treated choosing theconservative option, and describe in detail theaforementioned points.

C1.2Orthoses: When and Why?N. JankovićClinical Center Niš, Clinic for Physical Medicine,Rehabilitation and Prosthetics, Niš, Serbia

Despite the constant improvement in hemophiliamanagement, arthropathy remains the major cause ofsignificant clinical problems in patients with hemophiliasince joints are the most frequent location where bleedingoccurs. In literature, it is often stated that the ankle joint isless frequently affected by arthropathy than other joints,yet it is well known that the ankle joint demonstrates earlyonset of bleeding in severe hemophilia, so in thispopulation it is quite frequent. Treatment of anklearthropathy should always begin with simple stepsincluding footwear changes, activity modifications, anduse of orthoses. Orthotic management can be very usefulfor ankle arthropathy when is used in its early stages. Thereare many types of orthoses. In mild arthropathy a bracecan be applied to help hold the ankle joint in position, orin moderate cases they can be custom-made and arecalled ankle-foot-orthoses or AFOs. Application of

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Abstracts (cont’d)13 months follow-up period. Results: Synovectomyyielded good and satisfactory results. In more than half ofcases, no bleeding episodes occurred; its effect on therange of motion was moderate with a usual loss ofmobility between 8 and 14°, recovered in some patientsafter rehabilitation. Conclusion: Synovectomy inmoderate to severe chronic arthropathy produces goodresults in terms of bleeding recurrence and overallfunction when performed in a specialized centre with amulti-disciplinary approach.

P1.02Primary Prophylaxis Therapy in Hemophiliawith Low Dose and Low Intensity: Resultsfrom a Centre in TurkeyT. Celkan, G. Özdemir, G. Tüysüz Medical Faculty, Pediatric Hematology-Oncology, IstanbulUniversity, Cerrahpasa, Turkey E-mail: [email protected]

Primary prophylaxis with coagulation factor concentrateshas become the standard of care for children withhemophilia. However, while reducing the risk of bleedingand related morbidity, the optimal use of prophylaxis isnot well known. We tried to tailor the dose and scheduleof prophylaxis and reduce costs without decreasingefficacy. The aim of this study was to evaluate theeffectiveness of low dose primary prophylaxis therapy inchildren with severe hemophilia A or B. We documentedthe patients from June 2005 to June 2010. 15 patients (10hemophilia A patients and 5 hemophilia B) who had beenon prophylaxis therapy for at least 6 months weregathered. These hemophilia A and B patients received 20-30u/kg of factors VIII and IX, one or two times per week,respectively. All except 1 hemophilia A patient weretreated with recombinant factors (Recombinant, KogenateFS), only one patient began prophylaxis with plasma-derived factor concentrates and then shifted torecombinant factor. All hemophilia B patients were treatedwith plasma-derived factors. Twelve were diagnosedduring the first year of life, usually when they were 7-8months old (3.5-11 month) except three who werediagnosed when they were 4-5-years-old. The orthopedicevaluation was performed, 86.6% of patients who hadused factor regularly did not present any clinical orradiographic changes in their joints; only one patient whowas diagnosed when he was 4-years-old and put onprophylaxis had radioactive synovectomy. The preliminaryresults of low dose primary prophylaxis confirm itseffectiveness in preventing hemophilic arthropathy andbleeding.

C1.4Ankle Fusion: A Solution for DevelopingCountries Only?TBC


C1.5New Developments in Total AnkleReplacementJ.F. Asencio


Poster Session 1: Surgical

P1.01Knee Surgical Synovectomy in YoungHemophiliacs: A Report of 8 CasesF. Benmegherbi1, M. Bensadok1, M. Belhani1, S.Formas2, A. Benbouzid2, S.Khiat3

Departments of 1Hematology and Blood Transfusion;2Orthopedic and Trauma Unit, 3Physiotherapy Unit, CHUBéni Messous, Algiers, AlgeriaE-mail: [email protected]

In Algeria in 2010, 524 hemophiliacs from a total of 1465were under 19 years of age.The aim of this study is to assess the results ofsynovectomy in the treatment of hemophilicarthropathies and demonstrate the interest of amultidisciplinary collaboration (hematologist, orthopedicsurgeon, and physiotherapist). Method: Between 2008and 2010, 24 hemophilic patients (type A and B) had atotal of 29 surgical procedures, of which 14 orthopedicsurgical procedures were carried out on 11 children. Eightsynovectomies were performed in the Orthopedics Unitin 7 severe hemophilic children, of which 2 were carriedout on the same patient. Patients were between 7 and 16-years-old. Viral contamination (HIV, HCV) was absent in allpatients as were inhibitors. Indications of thesynovectomy: frequent hemarthrosis, chronichemarthrosis, pain and deterioration of function, stage IIIand IV arthropathy (Arnold-Hilgartner scale), the mobilityof the knee was 102°. Substitution by antihemophilicfactor was maintained during a couple of weekspostoperatively, then twice a week to ensure functionalrehabilitation. During hospitalization the patients used onaverage 50000 IU of factor VIII and 22500 IU of factor IX,and between 48000 IU and 96000 IU of each factor tocover functional rehabilitation. No hemorrhagiccomplications, infections, or appearance of inhibitorsoccurred after the surgery. The results were evaluated with

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Abstracts (cont’d)Aim: This study gives the musculoskeletal situation of anAlgerian sample population of hemophiliacs. It givessequence analyses of joint events, and provides a first drafttheory of sequences, vertical or passing from limb to otherof joint damage. Method: Since January 2008, wereceived 95 severe hemophiliac patients who havemusculoskeletal problems. We perform a systematicalreview of their medical history; take photos and videos ofphysical and radiological anomalies. We insist on thesequence of joint manifestation using interview andmedical documents. We statistically analyse all the data.Results: 70% are under 30-years-old. In 55.4% there are 2hemophiliacs per family, and more than 3 in 35.3%.Inhibitor factor status: 5% positive, 58% negative, and 37%unknown. Viral serology: 10% positive and 37% unknown.One joint’s lesion: 32%, more than 3 joint’s lesion: 52.5%.The sequences of joint lesion: 72.7% are passing from limbto the other; the diagram (A) gives all found sequences.Discussion: There is segmental joint adaptation inresponse to progressive joint function deterioration (M.Fearn 2010), the result of our study shows that thesequence of joint lesions is an adaptation of thisdeterioration. Our study shows that the sequence of jointdamage is secondary to different compensation strategies,and joint’s damage timing: vertical or cross from one limbto another, is guided by the first affected joint.Conclusion: The sequence of joint damage in hemophiliais the consequence of different strategies of compensationand allows to define a joint at risk.Keywords: sequence of joint damage, strategy ofcompensation

P1.05Hemophilic Chronic Synovitis: HemarthrosisTherapy Using Endovascular Embolization inKnee and ElbowE. Galli1, H. Caviglia2, 3, N. Moretti3, C. Daffunchio2, 3, G. Galatro2,3

1Servicio de Hemodinamia; 2Traumatología, HospitalGeneral De Agudos Juan A. Fernández; 3Fundación de laHemofilia, Buenos Aires, ArgentinaE-mail: [email protected]

Introduction: Chronic synovitis produce loss of quality oflife (QOL). Objective: To show the effect of endovascularembolization therapy in patients with synovitis. Materialand Methods: 23 patients, 2 with inhibitors, ranging from12-56 years-old, and presenting at least 3 hemarthrosisevents in 6 months, and synovitis. We performed 26procedures. Angiography of genicular arteries in the knee(18) and ulnar and radial arteries in the elbow (6) wasfollowed by selective gelfoam or PVA particle embolizationin blush or hypervascular lakes using standardendovascular technique. MRI was performed before andat 2 and 6 months control. Follow-up between 6 and 42months, evaluating number of hemarthrosis events,amount of coagulation, clotting factors used, and QOL.

P1.03The Results of Staged Bilateral Total KneeArthroplasty in Congenital Factor XIIIDeficiency R. Espandar1, M. Kaseb1, J. Mortazavi1, M. Managhchi1, G. Toogeh2

Departments of 1Orthopaedic Surgery and 2Haematology,Tehran University of Medical Sciences, Thrombosis andHaemostasis Research Center, Imam University Hospital,Tehran, IranE-mail: [email protected]

Introduction: Although hemarthrosis is not a raremanifestation of congenital factor XIII deficiency,destructive changes in the joints are relatively uncommon,in contrast with arthropathy changes seen in patients withclassic hemophilia. Factor replacement has significantlyhelped to reduce the complication rates of total kneearthroplasty (TKA) in classic hemophilia; making TKA aneffective method to achieve pain relief and gain betterfunction in this group of patients. According to ourknowledge, there is not any published data regarding theresults of TKA in patients with congenital factor XIIIdeficiency. Methods: Bilateral staged total kneearthroplasty with a six-month interval was performed formanagement of disabling arthropathy in a 56-year-oldfemale with congenital factor XIII deficiency. Stemmedcondylar constrained knee prosthesis was used becauseof varus and bony deficiency. According to therecommendations of our hematologist consultant,plasma-derived factor XIII concentrate and Transaminwere prescribed pre- and post-operatively. The pre- andpost-operative range of movement was assessed andcomplications after surgery were noted. Functional resultswere assessed using the Hospital for Special Surgery (HSS)knee scoring system, both pre- and post-operatively.Results: There was neither abnormal bleeding norinfection. The range of movement was increased. The kneescore (HSS) was improved considerably (from poor togood); hence, the patient’s quality of life has beenimproved. Conclusion: Like classic hemophilia, total kneearthroplasty may be a safe and effective procedure for themanagement of severe arthropathy in congenital factorXIII deficiency after infusion of plasma-derived factor XIIIconcentrate and Transamin.

P1.04Musculoskeletal Study of Algerian SevereHemophiliacs: Primary Finding, and 27 Casesof Elective Orthopedic SurgeryS. Fourmas1, A. Tirichine1, L. Aitelhadj1, A. Benbouzid1, F. Benmagharbi2, M. Bensadok2

1Orthopedic Surgery Unit, Specialized Hospital ofBenaknoun Algiers, 2Hematology Unit, University Hospitalof Benimessous Algiers, AlgeriaE-mail: [email protected]

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Abstracts (cont’d)P1.07Synoviorthesis Induced by Rifampicin inHemophilic Arthropathy; Report of 24Treated JointsM. Karimi1, S. Rezazadeh2, A. Haghighat2, Z. Babanezhad1,M. Mahmoodi1

1Hematology Research Center, Nemazee Hospital;2Department of Orthopedics, Chamran Hospital, ShirazUniversity of Medical Sciences, Shiraz, Iran

Introduction: Intra-articular injection of chemical orradioactive substances in order to produce fibrosis of thehypertrophied synovium has proved effective in thetreatment of chronic hemophilic synovitis. The aim of thisstudy is to investigate clinical outcomes of synoviorthesisinduced by rifampicin in 21 patients (which included 24joints) with hemophilic arthropathy. Methods: This quasi-experimental study was conducted between December2006 and July 2007. We treated 21 patients withhemophilic arthropathy by synoviorthesis with rifampicin(500mg) once a week. The procedures were performed on14 knees, 5 elbows, 4 ankles, and 1 shoulder (24 joints and113 injections). Results: Radiological staging using theArnold-Hilgartner scale and Fernandez-Palazziclassification were performed. Mean age was 16.7 years(range 7–49 years). According to the Fernandez-Palazziclassification, synoviorthesis was considered satisfactory(excellent or good) in 21 (87.5%) joints and unsatisfactory(fair or poor) in 3 (12.5%). A mean reduction of 6.3bleeding episode per month was obtained (p<0.0001). Themean pain score was reduced from 2.62 (range: 2–3, SD:0.49) before treatment to 0.79 (range 0-2 SD: 0.65) aftertreatment. The mean World Federation of Hemophilia(WFH) score was 5.45 (range 2–8) before synoviorthesisand 3.5 (range 1–7) after treatment. Conclusions: Thismethod effectively reduced hemarthrosis and pain, andalso improved range of motion in patients withhemophilic arthropathy. Chemical synoviorthesis withrifampicin appears to be efficient, inexpensive and simple,and also especially practical in developing countries whereradioactive agents are not easily available.

P1.08Our Follow-up Study After Synovectomy ofHemophilic Knee JointsL. Bartha1, L. Nemes2

1Orthopedic Dept. of the Semmelweis Medical University;2National Haemophilia Center and HaemostasisDepartment, State Health Center, Budapest, HungaryE-mail: [email protected]

Synovectomy of the hemophilic joints controls bleeding;however, significant loss of motion frequently results. Overthe last 16 years we performed 56 operations in ourorthopedic centre, on hemophilia patients withhemophilic arthropathy. Among these, 25 were

Results: All patients have improved QOL and madeimprovements in physiotherapy. In the knee group theaverage number of days without clinical events was112±76.8. There was a reduction in the number of eventsand the severity of them, and consumption of coagulationsubstitutive factors decreased (p<0.01) at 6 months in thewhole study and (p<0.01) at 18 months in patients havingmore than 3 bleeding events in 3 months. One patient hadto be re-embolized at 6 months. Technical success was96.15% without major complications or adverse effects. Inthe elbow group 1 patient had bad results whichcoincided with an insufficient technical embolization.Conclusion: In hemophilic patients with chronicknee/elbow synovitis, endovascular embolization seemsto offer a safe therapeutic option to treat hemarthrosis andreduce synovitis.

P1.06Botulinum Toxin-A Intramuscular Injectionsin a Boy with Severe Hemophilia and CerebralPalsyM. Bladen1, N. Hubert1, K. Edwards1, E. Main2

1Great Ormond St Hospital for Children NHS Trust;2Institute of Child Health University College London,United KingdomE-mail: [email protected]

Introduction: We report a case of a boy with severehemophilia A and cerebral palsy (CP) with spastic diplegiawho received intramuscular injections of Botulinum toxin-A (BtA) to reduce his lower-limb hypertonicity. BtAinjection was indicated for dynamic contractures whichinterfered with walking and wearing splints. No cases ofchildren with hemophilia receiving BtA for CP were foundin the literature. Methods: Prior to BtA injection, themodified Tardieu Scale was used to assess spasticity andcontracture, and the Gross Motor Function Measure –GMFM-66 was used to measure change in gross motorfunction. Before the BtA injection and the day followingthe procedure he received factor VIII cover at 60IU per kg,tranexamic acid 250 mg tds for 7 days. BtA was injectedbilaterally into hip adductors, hamstrings, andgastrocnemii. Dosage of BtA injection was calculated frominternational consensus guidelines. Orthotics wasamended to accommodate the effects of BtA. Results:Eight weeks later, parents reported improvements inactivities of daily function. There was absence of dynamicclonus in hips and knees and marked improvement at theankle joint. Before BtA injection, he scored 83% and 26%in the GMFM-66 crawling/kneeling and standingdimensions respectively. On reassessment, scores were95.2% and 33% respectively, which were consideredclinically significant. Conclusion: Assessments demons -trated improvements in ambulation, tolerance of orthotics,functional abilities, and range of movement. There wereno bleeding side effects. BtA is documented in theliterature for the management of hypertonicity in childrenwith CP and should be considered in those children withhemophilia and CP.

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Abstracts (cont’d)patient’s quality of life and delaying the symptoms ofhemophilic arthropathy.

P1.10The Role of Colloid 32P Synoviorthesis inTreatment of Hemophilic ArthropathyS. Mortazavi1, R. Gilbert2, M. Gilbert2

1Tehran University of Medical Sciences, Iran; 2Leni andPeter May Department of Orthopaedics of Mount SinaiHospital, New York, NY, U.S.A.E-mail: [email protected]

Introduction: Radioactive synoviorthesis by injection ofsafe radioisotopes into the joints affected by chronicarthritis is accounted as a novel method to treathemophilic arthropathy. The main goal of this therapywould be to decrease frequency of hemarthrosis andconsumption of coagulation factors. In this study weassessed the effect of radioactive synoviorthesis on thefrequency of hemarthrosis, factor consumption and otherrelated parameters. Methods: In an interventional studyin Imam Khomeini Hospital in Tehran, Iran, after meetingthe inclusion criteria and taking written consent, colloid32p radiosynovectomy was performed for 56 joints withhemophilic arthropathy. After local anesthesia of injectionsite, one mCi of 32P for large joints (knee) and 0.5 mCi forsmall joints (ankle and elbow) were injected, respectively.Half of these doses were considered for children (age <12years). Results: The mean age was 16.78-years-old (range:2.5-36; SD: 7.46), and 98.2% of cases were male. Injectedwere knee 80.35%, ankle 12.5%, and elbow 7%. The meanfollow-up was 43.63 months (range: 3-102), the result was62% decrease in frequency of hemarthrosis (p=0.0001)and 84% decrease in factor consumption (p=0.0001).However, the involvement of other (non-injected) jointsduring follow-up could lower the decrease of mean totalfactor consumption. Conclusions: Radioactive synovior -thesis can be a cost-effective alternative to decreasehemarthrosis and factor consumption in hemophilicarthropathy. Keywords: Hemophilic arthropathy, synoviorthesis

P1.11Cubital Tunnel Syndrome in Patients withHemophiliaS. Mortazavi1, R. Gilbert2, M. Gilbert2

1Tehran University of Medical Sciences, Iran; 2Leni andPeter May Department of Orthopaedics of Mount SinaiHospital, New York, NY, U.S.A.E-mail: [email protected]

Introduction: The elbow is the second most commonjoint involved in patients with hemophilia; however, thereis little data about the involvement of the ulnar nerve atthe elbow in patients with hemophilic arthropathy. Thepurpose of this study was to address this problem in the

arthroscopic knee synovectomies, combined with jointdebridement. Two other knee synovectomies were madeby open surgical procedure. Pre-operatively, all of thepatients had experienced an average of three bleedingepisodes into the affected joint per month, and had beenunresponsive to at least three months of substitutionmanagement. We are reporting on 10 patients whounderwent arthroscopic knee synovectomy and haddecreased bleeding episodes. The average flexioncontracture before operation was 11.5 degrees, post-operatively flexion contracture reduced to 9.5 degrees.The average loss of range of knee motion was 19 degrees.The average age of the patients at the time of the kneesynovectomy was 37-years-old and the average length offollow-up was 5.65 years (range, 1-16 years). It wasconcluded from our study that bleeding frequency andthe pain associated with persistent synovitis in thehemophilic knee can be effectively decreased with kneesynovectomy. All of our patients required less factor aftersynovectomies to control bleeding, although almost all ofour patients had loss of motion of the knee post-operatively.

P1.09Hemophilic Arthropathy: Diagnosis andTreatment of Subchondral Knee CystG. Lenciza1, H. Caviglia2, V. Rescia1, M. Davoli1, D. Magliaro1,P. Mangiarelli1

1Fundación de la Hemofilia; 2Fundación de la Hemofilia,Filial Rosario, Buenos Aires, ArgentinaE-mail: [email protected]

Introduction: The purpose of the study is to review theresult of the surgical treatment of the subchondral kneecyst with impacted morselized bone graft in a patient withsevere hemophilia. These cysts cause pain, loss of bonestock, and result in restricted range of movement anddeterioration of quality of life. Methods: We evaluated theresults of the surgical treatment of a subchondral kneecyst in a 54-year-old patient with severe hemophilia A. Thepatient had a history of recurrent bleeding, mainly in theknee. The cyst was diagnosed by conventional radiographsand MRI. The indication for surgical treatment was basedon the size of the cyst, which was larger than 50% of thesize of the tibial plateau.This procedure consisted of aspirating the contents of thecyst and packing it with impacted morselized bone graft.In addition to surgery, the patient underwent both pre-and post-operative physical therapy. Results: After threemonths, the patient had no joint pain and had improvedhis activity level. The range of movement increased by 40º(from 70 º-110 º). Signs of allograft resorption were notdetected. Conclusion: The treatment of subchondral kneecysts with morselized bone graft provides biological tissueand increases the bony reservation, improving the

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Abstracts (cont’d)P1.13Sonography Versus MRI in HemophilicArthropathy: A Comparative StudyG. Rivolta1, F. Mozzani2, C. Di Perna1, R. Polo3, A. Tagliaferri1

1Regional Reference Centre For Inherited BleedingDisorders; 2Department of Internal Medicine, Section ofRheumatology; 3Orthopaedic Department, University-Hospital of Parma, ItalyE-mail: [email protected]

Introduction: Hemophilic arthropathy is the most severecomplication of hemophilia. Physical examination and thePettersson score are routinely used to grade the severityof arthropathy and monitor the effects of the treatment.Magnetic resonance imaging (MRI) is currently the goldstandard for evaluating joint damage, but it is expensiveand younger children need to be sedated to undergo thisinvestigation. In recent years, sonography has become auseful complementary modality in the evaluation ofhemophilic arthropathy because of its prompt availability.Methods: We evaluated knee arthropathy, comparing thephysical examination score and the different imagingtechniques in three adult patients with severe hemophiliaA treated with secondary prophylaxis. Results: While theclinical examination and plain radiographs tended tounderestimate the extent of joint destruction, with MRIand sonography we obtained comparable informationabout synovitis, subchondral bone erosions, and chondraldamage. Conclusion: While MRI is presently consideredthe gold standard for the evaluation of arthropathy, theneed for sedation in children and its high costs precludeits use as a method for monitoring progression of jointdisease. Although sonography is an operator-dependenttechnique and a systematic protocol for sonographicassessment is needed, we think that it may provide auseful, more readily available and less expensive tool thanMRI for evaluating musculoskeletal involvement on aregular basis. In addition, the association of power-Doppler studies could help to detect and quantify synovialvascularity improving the quality of assessment of chronicsynovitis.

P1.14rFVIIa for Orthopedic Surgeries in FVIIDeficient PatientsE. Stefanska-Windyga1, P. Zbikowski2, P. Ambroziak2, I.Kotela2, J. Windyga1

1Institute of Haematology and Transfusion Medicine,2Central Clinical Hospital of the Ministry of Internal Affairs,Warsaw, PolandE-mail: [email protected]

Background: Inherited factor VII (FVII) deficiency has anestimated incidence of 1/500,000 in the generalpopulation and an autosomal recessive pattern ofinheritance. The hemorrhagic manifestations in affected

elbow and evaluate the results of anterior subcutaneoustransposition of the ulnar nerve in a small group ofpatients with hemophilia who had been managed in twoinstitutions. Materials: Information on 6 patients whowere diagnosed with tardy ulnar nerve palsy in twoinstitutions was retrospectively collected. All patientssuffered from severe hemophilia A. Anterior subcutaneoustransposition of the ulnar nerve had been performed in allexcept one. Results: The mean age of the patients at thetime of procedure was 45.8-years-old and the meanduration of follow-up was 60.2 months. No post-operativecomplication or recurrence was observed. No additionalsurgery was required in operated patients. Evaluation wasperformed using subjective and objective measures, anda modified Bishop score. After operation, subjectivesensory and motor disturbances were improved orresolved in all of the operated patients, while objectivemeasures improved less well. Conclusion: The ulnar nervecan be involved in cubital tunnel syndrome in patientswith hemophilia. Anterior subcutaneous transposition ofthe ulnar nerve is an effective procedure for improvingpatients' symptoms, with a low risk of complications.

P1.12Osteoporosis in Hemophilia: PreliminaryResults of a Densitometric StudyL. Abad-Franch1, A. Moret1, T. Montón2, J. Beltrán2, F. Aparisi2,S. Pérez-Alenda1, 3, N. Cabrera1, J. Aznar1, F. Querol1, 3

1Haemostasis and Thrombosis Unit, University Hospital LaFe; 2Radiology Service, University Hospital La Fe;3University of Valencia, SpainE-mail: [email protected]

In hemophilic patients, the loss of bone mineral density isassociated with osteoporosis, hemophilic arthropathy, anda decreased quality of life. The pathogenesis ofosteoporosis is multifactorial, and in hemophilic patientsthe risk factors are increased by musculoskeletal injuriesthat reduce mobility and by the common presence of viraldiseases and drugs that can affect bone metabolism. Inour hemophilia treatment centre, densitometry is includedin the patient’s assessment protocol. The aim of this studyis to describe the epidemiology of impaired bonemineralization in a group of patients with hemophilia. Weperformed axial and hip densitometry to 70 patientscontrolled in our centre. Regarding disease severity, 36were severe, 9 moderate, and 25 mild. Mean age was 31-years-old (range 14 - 62). Mean body mass index was 24.4(40% were overweight). 88% of the patients were infectedby hepatitis C virus and 51.4% by the human immun -odeficiency virus. The results of bone densitometryshowed loss of mineralization in 70% of our patients: 34had osteopenia (48.6%) and 15 had osteoporosis (21.4%).We conclude pointing out the importance of assessingosteoporosis considering it as a major clinical problem inthe hemophilic population.Keywords: Hemophilia, Osteoporosis

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Abstracts (cont’d)radiosynovectomy. Similar results were obtained inpatients with advanced osteoarthritis and in inhibitorcases. There was a significant reduction in pain in all joints.Average range of motion was maintained or increasedfrom 5 to 15 degrees of motion 1 year post-radiosynovectomy in most joints. Radiosynoviorthesis wassuccessful in 80% of the patients after 1 year of isotopeimplantation. Conclusion: Radiosynoviorthesis representsan important alternative for the treatment of chronichemophilic synovitis, markedly reducing joint bleedingfrequency and pain, irrespective of the stage of arthritisand inhibitor status.

P1.16The Results of 186Re Radiosynovectomy withIndividual Physiotherapeutic Protocol in theTreatment of Pediatric Hemophiliacs withInhibitorP. Zbikowski1, P. Laguna2, J. Cwikla1, M. Matysiak2, K. Scipiodel Campo2

1Central Clinical Hospital of Ministry of Internal Affairs;2Medical University of Warsaw, PolandE-mail:[email protected];[email protected]

Introduction: To evaluate the influence of 186Reradiosynovectomy with individual physiotherapeuticprotocol on reduction of hemarthroses in children withhemophilia and factor inhibitor. Methods: We performed8 186Re radiosynovectomies on 4 knees and 4 elbows in5 patients aged 9 to 15-years-old (median – 11.6) withsevere hemophilia A (4) or B (1) with inhibitors. The activityof 186Re colloid ranged from 60-180 MBq. We used rVIIa(NovoSeven) before the injection, after 2 hours, after 3hours, and then after 3 more hours. Then aPCC (FEIBA) wasadministered. Post-therapeutic scanning was performed1 hour after the procedure and on the 2nd and 3rd days.The number of hemarthroses and the clinical state wereassessed in 3 month intervals. Ultrasound evaluation wasperformed at each visit. Physiotherapy was adjusted topatient’s age and the state of the joint, and aimed to regaina full range of motion, muscle strength, and pro -prioception. Efforts were made to reduce residualrotational contracture and regain proper walking pattern.Progression-free survival was assessed using the Kaplan-Meier method. Results: Significant reduction inhemarthroses was noted (before: 1.5/months; after:0.4/months). Post-therapeutic scanning revealed evenintra-articular distribution of isotope and no leakage.Physiotherapeutic protocol allowed for recovery andimprovement of joint function. Conclusion: The studysuggests that the use of 186Re radiosynovectomy withtailored physiotherapy reduces the number ofhemarthroses in young hemophiliacs with inhibitors.

individuals are variable and correlate poorly with plasmaFVII activity levels. In severely affected cases, however,significant bleeding problems including spontaneoushemarthroses resulting in advanced arthropathy havebeen observed. We report here good results of orthopedicsurgeries performed in five FVII deficient patients whoreceived recombinant activated factor VII (rFVIIa) in theperi- and post-operative period. Patients and Methods:The study comprised 1 male and 4 females, aged 20-78-years-old, with FVII baseline plasma levels <10IU/dl. Twopatients required total hip replacement and three requiredvarious arthroscopic procedures. All surgeries wereperformed under hemostatic cover of rFVIIa. Results:Doses of rFVIIa ranged from 18 to 37µg/kg bw and weregiven every 8 hours on the day of surgery (first dose given10-15 minutes before the procedure), followed by 13-30µg/kg bw given every 12-24 hours for 9 to 14 days. Totaldose of rVIIa per procedure ranged from 16 to 38 mg. Labmonitoring consisted of daily determination ofprothrombin time and FVII plasma activity. In all patientsperi- and post-operative clinical course was uneventfuland there was no need to use other preparationscontaining FVII. Blood loss ranged from 30 to 700 ml perprocedure and none of the patients required bloodtransfusions. Pharmacologic thromboprophylaxis wasused in two patients undergoing hip surgery. Conclusion:rFVIIa is an effective, safe, and cost-effective haemostaticagent in hypoproconvertinaemia patients undergoingorthopedic surgery.

P1.15Radiosynoviorthesis in the Treatment ofHemophilic Joints - Hungarian ExperienceI. Szerb Orthopedic Department, Uzsoki Hospital , Budapest,HungaryE-mail: [email protected]

Aim: To present our promising results with radiosyn -oviorthesis in the treatment of hemophilic joints.Materials and Method: Hemophilic hip, knee, and anklejoints have been treated with radiosynoviorthesis at ourdepartment since 2003. 110 MBq Rhenium-186 sulfide, incolloidal suspension, is used for hip and ankle joints, whileYttrium-90, in colloidal suspension is implanted into kneejoints. Isotope implantations are performed in a verysterile operating room. Local anesthesia with 1% Lidocaineis used for the procedure. There were no cases of injurycaused by leakage of the isotope. The patients areadmitted to the hospital for three days and bed rest andimmobilization of the joints with a brace is prescribed forthe same time period, to reduce extra-articular migrationof the radiopharmaceutical. Hip joints are not braced.Results: We observed an overall reduction in jointbleeding frequency, from 18.7 to 2.4 per year post-

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Abstracts (cont’d)There is a shortage of replacement therapy drugs forhemophilia patients in the Ukraine. Therefore, it isimportant to use different methods of physicalrehabilitation for people with bleeding disorders. In manycases we are using physical therapy for hemophiliapatients without a replacement therapy. In this work wepresent the treatment of a patient with severe hemophiliaA, complicated with inhibitors (patient 1), and a patientwith von Willebrand disease (third type) (patient 2). Bothof them have a 1-year-old combined contracture (flexionand extension) of the knee joint. Both patients wereevaluated before rehabilitation and every month duringthe treatment. Evaluation includes measurements of rangeof movements in the joints, manual muscle test, and“Up&Go” test. Individual rehabilitation programs includeactive, active-passive exercises, post isometric relaxation,elements of Maitland’s manual therapy, and exercises formuscle strengthening. Both patients received individualrehabilitation programs, which lasted for half a year. Afterthis period patients show significant improvements inknee joint movement: range of movements has increasedfrom -20° – 75° to 0° – 100°, and from -35° – 85° to 0° – 120°for patient 1 and 2, respectively; manual muscle test inknee extension and flexion has increased by 2 points forboth patients; “Up&Go” test time has decreased from 22 to13 seconds and from 18 to 9 seconds for patient 1 and 2,respectively. These results show that our proposedrehabilitation program is very effective for thecomprehensive care of patients with hemophilia.Keywords: hemophilia, physical rehabilitation

P2.02Acute Effect of Exercise in Persons withHemophiliaL. Beltrame, A. Beltrame, A. Almeida, M. Sayago, T. Felix, J.Almeida IHTC, Brazil, BrasiliaE-mail: [email protected]

Hemophilia is a bleeding disorder that concerns experts.Physical activity has shown some changes in hemostasisrelated to improvements in the coagulation cascade ofnormal people, such as: increasing FVIII, increasing VWF,decreasing APTT, decreasing PT and increasing fibrinogen.Goal: This is a preliminary study that aims to analyse theacute effect of exercise on people with hemophilia.Methods: The sample consisted of 4 severe hemophilia Aand 1 moderate hemophilia A patients, physically activefor at least six months, aged 15-31 years-old (mean 19.6).The sample had blood samples taken before andimmediately after a session of 20 minutes of exercisewithout the use of clotting factor for 48 hours before.Statistical procedure for analyzing the results wasstatistical average of the values before and after exercise.Results: We found decreased APTT from 74.88 to 72.88; areduction of PT from 17.88 to 13.56; a FVIII increase from

P1.17Factor VIII Replacement in the Peri-operativePeriod in Joint Arthroplasty in Hemophilia A– A Series of 7 CasesJ. Zdziarska1, T. Parda2, J. Jaworski2, T. Iwaniec3, A. Skotnicki1

1Department of Hematology, Jagiellonian UniversitySchool of Medicine; 2Department of Traumatology,Orthopedics and Rehabilitation, Cracow RehabilitationCenter; 3Department of Internal Medicine, JagiellonianUniversity School of Medicine, Cracow, PolandE-mail: [email protected]

Introduction: Orthopedic surgeries in patients withcongenital bleeding disorders require thorough a logisticplan, an experienced team of specialists, and adequatesupplies of clotting factor concentrates. Access to electivejoint replacement surgeries in patients with hemophilia inPoland is far below the demand. Since June 2010 theCracow Rehabilitation Center, working in strictcooperation with the Department of Hematology inCracow, is the second orthopedic hospital in Polandperforming these procedures. We describe factor VIIIreplacement therapy in the peri-operative period in thefirst 7 patients with hemophilia A operated on in theCracow Rehabilitation Center. We point out the issuesconcerning optimal dosage, monitoring strategy, andfactor VIII levels in the peri-operative period. Methods:Factor VIII concentrate (Immunate, Baxter) wasadministered three times a day. Pre-dose factor VIII activity(FVIII:C) was measured daily from Monday till Friday withone-stage clotting assay (BCS XP analyzer, Siemens,Germany). Factor VIII doses were adjusted on a daily basis,based on the clinical status, FVIII and hemoglobin levels.Low-molecular-weight-heparin was used in only onepatient due to prolonged immobility. Results: Pre-surgeryFVIII level was 95-159%. Within 14 days of hospitalizationa mean of 1042.6 IU/kg (range 678-1974) of factor VIII wasutilized. In 4 of 7 patients mild to moderate bleedingcomplications were observed that did not affect theoutcome of surgery and rehabilitation. Conclusion:Routine joint replacement in patients with hemophiliarequires coordination between the surgeon, hematologist,laboratory diagnostician, and Blood Bank. Bleeding/thrombotic risk must be assessed individually for eachpatient.Key words: joint replacement, hemophilia, factor VIIIreplacement therapy

P2.01Physical Therapy of Patients with Hemophiliawithout a Replacement Therapy: ClinicalCasesI. Ablikova1, G. Baranesckij2

1Elita Rehabilitation Center; 2Lviv State University ofPhysical Culture, Lviv, The UkraineE-mail: [email protected]

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Abstracts (cont’d)Introduction: Pseudotumors are a major complication inpatients with hemophilia and can be devastating,especially in the presence of inhibitors. Only a few caseshave been reported in the literature. Objectives: Thepurpose of our work was to show the experience intreating 7 pseudotumors in patients with inhibitors at onecenter. Materials and Methods: 6 patients with 7pseudotumors were treated. All the patients have severehemophillia A. The mean age was 21.4 years (13-60). 1patient presented soft tissue pseudotumors in the arm.The most frequent location of the pseudotumors was thefemur (3), tibia (2), and calcaneus (1). 2 patients had apathological fracture, one in the femur and the other onein the tibia. 1 patient had a pseudotumor of the femur andcalcaneus on the same lower limb at the same time. In thispatient, both pseudotumors were embolized beforesurgery to control bleeding. All patients with truepseudotumors were treated surgically except the one withthe soft tissue pseudotumor who was under conservativetreatment. The bypass agent used in all patients wasrFactor VIIa. Results: 5 true pseudotumors recovered withsurgical treatment. 1 patient who had a large femoralpseudotumor with a pathological fracture died of sepsissecondary to necrotizing fasciitis. The patient with the softtissue pseudotumor healed with conservative treatment.Conclusions: Our results show that pseudotumors inpatients with inhibitors could be treated with surgery anda bypass agent.

P2.05Physiotherapy Treatment in Patients withHemophilia and Chronic Ankle Arthropathy:Experimental StudyR. Cuesta, A. Gómez Universidad De Murcia, SpainE-mail: [email protected]

Objective: This article tries to evaluate the effectivenessof two programs about physiotherapy in patients withhemophilia A and B, adults and with artropathy of theankle (figure 1), without previous surgery and also withoutthe parameters that can interfere in improvement.Methods: 9 patients took part in this double-blind study,who randomly received treatment composed ofphysiotherapy and medication with concentrates of FVIIIand FIX, over six weeks, two times per week. They wereevaluated before and after the intervention, and after 6months range of motion, pain perception, quality of life,and stability of the lower limbs was evaluated (figure 2).Results: It was noticed the effectiveness of both of theinterventions (p<0.05) in eleven and five of the studiedvariables, of the first and second group respectively.Physiotherapy treatments using thermotherapy,mobilization and passive stretching of the ankle,proprioception and cryotherapy, and manual orthopaedictherapy, were effective in the improvement of arthropathyrelated to the studied sample. After six months,

1.75% to 2.59%; and fibrinogen increasing from 299.4% to304.6% with the heart rate average 142 bpm. Conclusion:In the present study we found that there was a change inthe parameters observed. However the sample was notsufficient to statistically evaluate the acute effect ofexercise on hemostasis. We suggest further studies toevaluate the acute and chronic effect of exercise onhemostasis, the long life of FVIII and other components ofthe coagulation cascade.

P2.03Why Physical Activity for Hemophiliacs?A. Beltrame, L. Beltrame, A. Almeida, M. Sayago, T. Felix, J.AlmeidaIHTC, Brazil, BrasiliaE-mail: [email protected]

Sedentary behavior in people with hemophilia isassociated with a risk of being overweight, having jointproblems, repeated bleedings, and loss of function. Theactual recommendations for sedentary people are toaccumulate physical activity for at least 30 minutes per dayalmost every day of the week (5-7days). The ideal forhealth is to accumulate approximately 28kcal/kg/week.One kcal corresponds to 1 MET (metabolic equivalent). Theaim of this study was analyze the chronic effects ofphysical activities in hemophiliacs participating in regularphysical activities (3 times/week) for at least one year.Methods: The group had 10 males with hemophilia (A, B)aged between 15 to 34-years-old, stimulated to practicephysical activities for one year. The group was analyzed byIPAQ questionnaire, body mass index (BMI), waist-hip ratio,rest blood pressure, and rest heart rate. The statisticalanalyses were done by averaged and standard error.Results: The group were identified: Less active(<600METs), moderated active (600-1499 METs), active(1500-2999METs), and very active (>3000 METs). We found50% of the group was very active, 30% active, and 20%moderately active. Almost every result of the physicalfitness test (BMI, waist-hip ratio, blood pressure, and heartrate) was associated with health conditions and lessbleeding episodes. Conclusion: The chronic effects ofregular physical exercise were associated withmaintenance of energy, and we could observe the lowlevel of bleeding during this time. We suggest morestudies relating the recommended expenditure of energyin regular physical activity and the frequency of bleedingin joints, muscles, and factor usage throughout theprocess.

P2.04Pseudotumors in Patients with InhibitorsH.A. Caviglia1, 2, G.A. Galatro1, 2, M. Candel M1, N. Moretti1,C.R. Perez Bianco1

1Fundación De La Hemofilia; 2Hospital General de AgudosJuan A. Fernández, Buenos Aires, ArgentinaE-mail: [email protected]

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Abstracts (cont’d)Hemophilic arthropathy secondary to recurrenthemarthroses and chronic synovitis is a major orthopediccomplication in hemophilia. The ankle is a commonlyinvolved joint, trailing behind the knee and ankle, and is afrequent cause of functional limitation and disability inindividuals with hemophilia. Physical therapy is commonlyprescribed for conservative management of chronic anklesynovitis. The goal of rehabilitation is to help prevent theprogression of chronic synovitis to end-stage arthropathyand to maximize the function and quality of life of patients.This article reviews the musculoskeletal impairmentsconcomitant with chronic ankle synovitis and outlines thecurrent treatment approach, along with the evidencesurrounding rehabilitation of this specific condition.Multiple databases including PUBMED, COCHRANELIBRARY, MEDLINE, and CINHAL were utilized to obtainrelevant studies published between 1995 and 2010.Current physical therapy interventions to addressimpairments associated with chronic ankle synovitis arecomprised of manual therapy, cryotherapy, andhydrotherapy, strengthening and stretching exercises, andbalance and proprioceptive training. With the exceptionof manual therapy, these interventions, in conjunctionwith the use of Factor VIII replacement therapy, have beenshown in the existing literature to improve joint function,minimize bleeding episodes, and prevent furtherdeterioration in the knee and elbow. There is a paucity ofresearch investigating the effects of conventionalinterventions on chronic ankle synovitis in the hemophilicpopulation. More research needs to be conducted in thisarea to clearly delineate the most effective physicaltherapy treatments to address impairments and functionallimitations and prevent the development of hemophilicankle arthropathy. Keywords: chronic ankle synovitis, physical therapy

P2.08Vertical Ground Reaction Forces duringHemophilic Arthropathy Gait: A Case StudyX. García-Massó1, M. Pellicer-Chenoll1, P. Salvà1, L. González1,J. Gallach1, F. Querol2, 3

1Department of Physical Education and Sports, Universityof Valencia; 2Department of Physiotherapy, University ofValencia, 3Thrombosis and Hemostasis Unit, La FE Hospital,Valencia, SpainE-mail: [email protected]

Introduction: Hemophilic arthropathy (HA) is the clinicalmanifestation with the greatest morbidity in hemophilicpatients. Furthermore, this degenerative disease in thejoint produces disturbances in motor control (e.g. balance,proprioception). The aim of our study was to compare thevariables related to vertical ground reaction force (VGRF)depending on the arthropathic severity in the supporting

physiotherapy interventions were effective, obtainingimprovement in pain perception and the reversal of bothankles, left ankle eversion, and mental health.Conclusions: It is necessary to mix both of thephysiotherapy models, with a higher sample, todeterminate effectiveness and to delay surgery as the lasttherapeutic option.Keywords: ankle, physiotherapy

P2.06Postsurgical Mobility of Total KneeReplacement in Patients with Hemophilia C. Daffunchio1, 2, G. Galatro1, 2, N. Moretti1, M. Candela1,2, H. Caviglia1, 2

1Fundación de la Hemofilia; 2Servicio de Ortopedia yTraumatología, Hospital Juan A. Fernández, Buenos Aires;Argentina E-Mail: [email protected]

Introduction: We have analysed 8 internationalpublications and the median range of motion (ROM) postsurgery is 22°. Objective: To evaluate the range of post-surgical motion in patients with TKA in our treatmentcenter. Materials and Methods: From 2001 to 2009, 19TKAs were performed on 12 patients with severehemophilia A, 7 bilateral and 5 unilateral. Mean age atarthroplasty was 47.6 years, mean follow–up was 3.5 years(range 1- 9). ROM was evaluated pre-operative, post-operative at 6 months, in one year, and in 2010. All patientsreceived physiotherapy, including postural treatment.Results: The mean knee flexion improved from 67.6° pre-operatively to 84.7° at the final follow-up, with animprovement of 17.1°. The mean flexion contracturediminished from 24.5° to 8.4°, with an improvement of16.1°. The mean total ROM increased from 43.2° pre-operative to 76.3° at the final follow-up, with animprovement of 33.1°. TKAs with total ROM pre-operativebelow 70° had an increased total post-operative ROM of35°-55°, whereas those with total ROM of 70°-84° gained25°-30°. Those with total ROM between 85°-100° remainedwithout changes. One case with a loss of total ROM of 15°had a pre-operative ROM of 110°. ROM obtained in 6months post-operative was maintained up to the finalfollow-up. Conclusion: TKA with the techniques appliedobtains acceptable mobility, allowing patients to improvetheir functionality.Key Words: ROM (range of motion) , TKA (total kneereplacement)

P2.07Physical Therapy Management of ChronicAnkle Synovitis: A Literature ReviewC. Eria1, P. Letourneau2

1Boston Hemophilia Center, Boston, MA; 2HemophiliaTreatment Center of Nevada, Las Vegas, U.S.A.E-mail: [email protected]

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Abstracts (cont’d)P2.10Spontaneous Iliopsoas Hematoma in Patientswith von Willebrand Disease and Hemophilia AB. Keikhaei Research Center for Thalassemia & Hemoglobinopathy,Ahvaz Joundishapur University of Medical Science, Ahvaz, IranEmail: [email protected]

Introduction: Iliopsoas hemorrhage can be developed inindividuals with bleeding disorders such as hemophilia Aand von Willebrand disease (VWD). This is a review of twocases of severe hemophilia A and type 3 VWD withspontaneous iliopsoas hematoma from south-west Iran.Case Presentation: Case1: A 26–year-old male Iranianwith severe hemophilia A had right groin pain. No historyof trauma. Pelvic ultrasongraphy showed a small echo-freelesion on the right iliopsoas muscle. With impression ofiliopsoas hemorrhage he was given recombinant factor VIIIat a dose of 50 IU/Kg body weight, twice daily. Case 2: A20–year-old male Iranian with Type 3 VWD had onset ofright flank pain. Upon arrival, there was limited mobility ofthe hip without antecedents of trauma. The patientexhibited an anti-pain posture and held his right leg flexedin the hip joint (Figure 2). MRI showed a mass in rightiliopsoas muscle (Figure3). An initial clinical diagnosis ofan iliopsoas hematoma and partial femoral nerve palsywas made. He was given Humate-P at a dose of 40 IU/Kgbody weight twice daily. Discussion: Iliopsoashemorrhage is encountered in a variety of coagulationdisorders such as hemophilia A, VWD, rare bleedingdisorders, and those using anti-coagulant drugs asHeparin, Warfarin, and Ticlopidine, but most iliopsoashemorrhages occur in hemophilia patients. The first casewas explained by Tallroth in 1939. Iliopsoas hemorrhageis a potentially serious complication because delay instarting treatment may result in permanent femoral nervepalsy. Diagnosis of retroperitoneal hematoma is obtainedby ultrasongraphy, computed tomography scan and MRI.The treatment of iliopsoas muscle hemorrhage remainscontroversial. Surgical decompression and a medicalapproach are recommended. In conclusion, these caseswith spontaneous iliopsoas hematoma show a goodresponse and outcome with medical intervention.Acknowledgements: The author thanks Dr. Solani andlaboratory staff for their assistance.

leg. Methods: One patient, suffering from hemophilia Awith HA, volunteered. The reaction force signals wereacquired with a force plate NedSVE/iIBV (Institute ofBiomechanics of Valencia, Valencia, Spain) at 100Hzfrequency. Non-parametric tests were applied (U de MannWhitney) for independent samples to determine if therewere significant differences between both legs. Results:The landing force (N), the take-off force (N), the take-offratio (N.s-1), and the stance time were greater in the legwith less arthropathic affectation (p<0.05). On the otherhand, for the landing ratio (N.s-1) the values weresignificantly higher in the leg with less articular affectation(p<0.05). Conclusion: VGRF produced during walking areaffected by the HA, this fact shows the importance of gaitpattern re-education in these patients.

P2.09Kinetic and Kinematic Analysis of the AnkleJoint: A comparative Analysis between Childrenwith Hemophilia and Normal ChildrenF.H. Abd-Elazeim, S.A. HamedFaculty of Physical Therapy, Cairo University; Faculty ofPhysical Therapy October 6 University, Cairo, Egypt

Hemophilia is an inherited, congenital life-long blooddisease. Children with hemophilia are vulnerable to a highpercentage of musculoskeletal lesions. These lesionspredominantly affect lower limbs, influencing posturalcontrol, standing, and walking. The purpose of this studywas to evaluate sagittal kinetic and kinematic changes ofankle joint during stance phase of gait. 100 boys with amean age 10.560+1.110 years participated in this study.The study group examined 50 children with hemophiliaand the control group included 50 normal volunteers. Thetwo groups were evaluated using a 3D motion analysissystem, the Qualysis motion capture system which has 3Pro-Reflex 120 infra-red cameras, with Q-trac, Q-capture,and Q-view. The Q-gait software was used to capture andanalyze data. Ground reaction was also collected using aforce plate unit. Data were compared between the twogroups using unpaired student t test. Children withhemophilia were found to be significantly different in allparameters of joint angles, and moments during initialcontact, loading response, mid-stance, terminal stance,and pre-swing phase. These results suggested analteration in range of motion and decrease in all forcesaround ankle joint which affect dorsi- and plantar-flexormovements. In addition children with hemophilia werefound to have joint changes, and alteration ofproprioception and pain. Key word: ankle joint, hemophilia

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Abstracts (cont’d)as compared to age-appropriate normal values. Jointrange-of-motion (JROM) of other was within normal limits.None of the affected individuals had joint hypermobilityor hyperelasticity of the skin; genotyping for Ehlers-Danlossyndrome was not obtained. All were non-responsive toDesmopressin and required von Willebrand factor (VWF)concentrates for treatment of bleeding episodes. Othermembers of the family with VWD had normal JROM. Therewere also members in these families who were notaffected by VWD. Relevance to hemophilia care: Theabove cases demonstrate the occurrence of jointhyperextension in family members with VWD. All casesexperienced bleeding episodes of varying severity;however none had experienced an overt joint bleed.Unlike hypermobility, which results from a jointabnormality, hyperextension is the stretching of a bodypart beyond what is normal, although sometimes theterminologies are used interchangeably. To the best of ourknowledge the occurrence of joint hyperextensibility inVWD has not been reported. Skeith et al described thatbaseline joint hypermobility in hemophilia may be a riskfactor for chronic arthropathy. This paper highlights theimportance of obtaining JROM in patients with VWD.Key words: Von Willebrand Disease, Joints

P2.13Management of Ankle Pain in an Adolescentwith Severe Hemophilia A: Using Orthoticsand Rehabilitation to Improve OutcomesP. McLaughlin1, M. Mathias2

Departments of 1Physiotherapy and 2PaediatricHaematology, Katharine Dormandy Haemophilia Centreand Thrombosis Unit, Royal Free Hospital, London, U.K.E-mail: [email protected]

Objectives: To highlight the importance of physiotherapyintervention in the management of ankle pain; outcomesmeasures that are clinically and financially valuable inpatient management; the value of patient completedtreatment records and the monitoring of them.Introduction/Report: Patient A (SHA, prophylaxis) at hisroutine review appointment complained of recurrent leftankle pain, denying any injury or trauma. The painworsened on weight-bearing activity and he had difficultyhopping and running. Extra FVIII had only mild effect.Physiotherapy assessment identified no swelling, bilateralpes planus and a mild laxity of the talocrural joint. Gaitanalysis highlighted poor heel strike and foot control withglobal muscle weakness. The Beighton score was 5/9 andthe Haemophilia Joint Health Score (HJHS) was 7. The‘Treatment Direction Test’ (TDT) (lessen pronation withstrapping) improved pain on assessment. Clinicalimpression was pain from pes planus and associatedmuscle weakness. Treatment consisted of orthoticsprovision, lower limb strengthening, and education.Outcomes: Treatment record review for 12 months prior

P2.11Successful Conservative Management ofHemophilic Pseudotumour: A Case Report A. Chhabra1, R. Kulkarni1, J. Penner1, S. Pipe2; A. Scott-Emuakpor1, R. Gera1; E. Saah1

1Michigan State University, East Lansing; 2University ofMichigan, Ann Arbor, MI, U.S.A.E-mail: [email protected]

Objective: To highlight conservative management ofhemophilic pseudotumor with long-term replacementtherapy with factor concentrate. Method: Retrospectivereview of patient chart. Results: A 14-year-old nativeAmerican male with moderate hemophilia B (baselinefactor (F) IX level 2%) presented to the emergencydepartment with severe left gluteal and thigh painfollowing injury. X-rays of the left hip were normal. Hereceived 70-80units/kg of FIX concentrates (dosed to theclosest vial) daily for 5 days. A computerized tomography(CT) scan obtained for worsening pain showed left pelvicfluid collection and possible pseudoaneurysm. AnUltrasound (US) and Magnetic Resonance Imaging (MRI)revealed a 12.7 cm x 6.2 cm x 7.9 cm hematoma with noevidence of a pseudoaneurysm. Following a centralvenous access device (port) placement, the patientreceived daily FIX concentrates for a period of 2 monthsfollowed by infusions every other day for 6 months, andthen weekly infusions for a period of 1 month. Serialfollow-up CT showed interval resolution of the hematoma.At the end of 9 months, US and MRI showed completeresolution of the hematoma. Patient was able to attendschool throughout the duration of treatment. Relevanceto hemophilia care: Early institution of replacementtherapy followed by long-term prophylaxis is effective inthe management of hemophilic pseudotumors. Thecost/benefit of interventional treatment versusreplacement therapy, and the risk of recurrence should betaken into consideration when treating a hemophilicpseudotumor. Key words: Hemophilia B and pseudotumour

P2.12Joint Hyperextensibility in von WillebrandDisease (VWD): A report of Six CasesS. Chonat, R. Kulkarni, R. Gera, S. Caroline, S. Parker, S.Adkins, E. Saah, A. Scott-Emuakpor Michigan State University, East Lansing, MI, U.S.A.E-mail: [email protected]

Objective: To raise awareness of the occurrence of jointhyperextension in VWD, that may mask joint-range-of-motion loss due to bleeding. Methods: Retrospectivereview of charts from a single Hemophilia TreatmentCenter. Results: In 6 female cases of VWD (5 from a singlefamily), all had moderately severe type 1 or type 2 VWD.Joint hyperextension of elbows and knees were seen in all

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Abstracts (cont’d)radiosynoviorthesis in our group of patients. Conclusion:An early physiotherapy programme for severe hemophilicpatients, with the appropriate replacement coverage,allows rapid and adequate functional recovery afterradiosynoviorthesis. Key Words: Physiotherapy, radiosynoviorthesis

P2.15Improving Quality of Life in Women with vonWillebrand DiseaseR.M.Y. Saad1, A. H. Sabbour2, A.A. Abdel-Hady3 and M.A. El-Ekiaby4

1Physical Therapy Department for Cardiovascular/Respiratory Disorders and Geriatrics Faculty of PhysicalTherapy, October 6 University; 2Physical Therapy Depart -ment for Obstetrics and Gynecology Faculty of PhysicalTherapy, Cairo University; 3Physical Therapy Departmentfor Cardiovascular/ Respiratory Disorders and GeriatricsFaculty of Physical Therapy Cairo University; 4HematologyDepartment El-Shabrwishy Hospital; Cairo, EgyptE-mail: [email protected]

Objective of the study: This study was conducted toestimate the effect of aerobic exercises on quality of life inwomen suffering from bleeding symptoms andmenorrhagia due to VWD. Study design: 30 womensuffering from type 1 von Willebrand disease participatedin this study. All patients were divided into two groups;each group consists of 15 patients. Group (A) patients wereevaluated by blood test (Ristocetin Cofactor Assay),Congenital Rare Bleeding Disorder Questionnaire,cardiopulmonary exercise tests (Bruce Protocol), and SF-36 QoL Questionnaire, before participation in the studyand after three consecutive months. Patients in group Bwere enrolled in the exercise program using a motordriven treadmill for about three months, and weresubmitted to the same methods of evaluation as group A.Results: The results of this study showed negative findingsin VWF, VO2max, VE, limitations of activities, physicalfunctioning problems, general health, bodily pain andbleeding score for patients in group A. The results forpatients in group B showed a statistically highly significantincrease (P<0.01) in VWF, VO2max, VE, decreasedlimitations of activities, physical functioning problems,general health and a statistically highly significantdecrease (P<0.02) in bodily pain (as a domains for QoLQuestionnaire), and highly significant decrease (P<0.01) inbleeding score after the end of three consecutive monthsof walking treatment program which reflect improvementin the QoL. Conclusion: The results of this studyconcluded that walking exercises are excellent con -servative treatment for von Willebrand disease throughimproving the aerobic capacity which improves thequality of life for such cases.Key words: VWD, aerobic exercises

to physiotherapy highlighted 16 excess treatments forankle pain, in the 10 months following input there wereonly 4 extra. The cost difference in FVIII use was £11,600.The HJHS reduced to 0, muscle strength significantlyimproved, and he was pain free. Discussion and ClinicalRelevance: The cause of ankle pain in this instance wasnot related to bleeding or hemarthropathy, althougharguably if it continued, movement compensations mayhave led to a bleeding episode. Application of the TDTrefined the assessment and reasoning behind orthoticprovision. Although the actual biomechanical mechanismby which orthotics have their effect remains inconclusive,they clearly had a positive effect. They should not howeverbe considered a universal treatment, but as a targetedintervention alongside strengthening. Key words: ankle pain; orthotics

P2.14Physiotherapy and Synoviorthesis inHemophilic PatientsF. Querol 1, 2, S. Pérez-Alenda1, L. Abad-Franch1, A. Moret1, N. Cabrera1, L. Dueñas2, F. Aparisi3, E. Mesa2, X. García-Massó2,J. Aznar2

1Haemostasis and Thrombosis Unit, Hospital La Fe;2University of Valencia; 3Radiology Services, Hospital La Fe,Valencia, SpainE-mail: [email protected]

Introduction: The aim of this study is to describe aprotocol of physiotherapy for functional recovery afterradiosynoviorthesis in severe hemophilic patients.Methods: Isotopic synoviorthesis in knees wereperformed with Itrium90, using an average dose of 4-5mCi. In elbows and ankles we used Renio86 with anaverage dose of 3 mCi. The dose of replacement factorconcentrate used was 25-30 IU/kg of FVIII or FIX before theprocedure and after 24 hours, followed by two doses every48 hours. Patients with inhibitors were treated withbypassing agents. The protocol of physiotherapy afterradiosynoviorthesis consisted of: 1) During the first 24hours: joint immobilization, rest and isometric exercisesfollowed by 10 minutes of cryotherapy. 2) Days 2-7:isometric exercises, active and strength exercises,performed 3 to 5 times a day, followed by cryotherapy. Wealso allowed a progressive increase in daily activities, usingcrutches for those patients with lower limbs involved(knees or ankles). 3) During the next 6 months, patientsfollowed individualized recommendations with the aim ofreducing swollen joints, avoiding re-bleeding duringspecific exercises and daily activities, maintaining orimproving muscular strength and trophism and range ofmovement. Results: Between 2004 and 2010 weperformed 122 radiosynoviorthesis (60 knees, 35 elbows,and 26 ankles) in severe hemophilic patients. There wereno re-bleedings due to early physiotherapy after

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Abstracts (cont’d)P2.16Improving the Number of Patients withSevere Hemophilia Undergoing a Physio -therapy Annual Review E. SherlockNational Centre For Hereditary Coagulation Disorders,Dublin, IrelandE-mail: [email protected]

Introduction: All patients with severe hemophilia shouldundergo a formal physiotherapy annual review (AR)(Haemophilia Chartered Physiotherapist Association (U.K.),2002). Aims were to measure the percentage of patientsreviewed in 2009 against the target of 100%, to identifyreasons for any shortfall and instigate changes to reduceit. The number of reviews performed in 2010 would bemonitored to assess the impact of changes made. Anotheraim was to record the outcome of the reviews. Methods:A retrospective review of the physiotherapy notes ofpatients with severe hemophilia registered at the NCHCDwas used to identify those that attended for AR in 2009.The outcome of the review was also recorded. TheNCHCD’s electronic patient record was used to establishpatient’s attendance record at the hemophilia clinics.Descriptive statistics were used to present the findings tothe multidisciplinary team for discussion. Results: Barrierswere identified as lack of time, lack of space, otherdemands on the service, as well as the poor attendancerecord of some patients. Changes agreed to address thesebarriers, such as dedicated physiotherapy space and timebeing available during hemophilia clinics, resulted in ahigher percentage of patients being reviewed in 2010. Aneed for further intervention was identified in 48% ofpatients reviewed in 2010. Conclusion: With almost halfthe patients who had physiotherapy AR needing furtherin-put, maximising efforts to ensure patients are reviewedeach year is important.

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