11
Other aspects of orthopaedics 23 Management of haemophilic arthropathy F. APARISI, F. QUEROL, J. BELTRAN, C. CIFRIAN, C. MIGUEL and J. A. AZNAR Radiodiagnostic Service, Hospital Rehabilitation La Fe Unidad de Coagulopatı´as Conge´nitas, Hospital La Fe Physiotheraphy Department, Universidad de Valencia, Spain The initial event consists of synovial or subsynovial haemorrhage. After blood enters the joint cavity, the toxic effects of haemosiderin and blood products provoke an inflammatory reaction, possibly through the generation of toxic oxygen radicals. MRI and US are able to study the synovial layer. MR images demonstrate an irregularly thickened synovial lining of low to intermediate signal intensity on T1- and T2-weighted images, probably as a result of the presence of haemosiderin deposited within the synovial membrane. Ultrasonography is a particularly attractive means of performing serial noninvasive studies to monitor response to joint bleeding and various thera- peutic endeavours. Synovial ablation with radioisotopes has also been advocated, with ultrasonography used in an effort to diagnose synovial thickening prior to the onset of radiographic bony changes. We present a 40-case series of haemophilic arthropathy studied by X-ray plain film, MRI, and ultrasound. After reviewing this series we found four types of change: joint effusion, soft tissue thickening, bone erosions and bone cysts. X-ray plain film and MRI are able to confirm the presence of bone lesions that correspond with nonreversible arthropathies, there are no significant differences between these methods of confirming the bone lesions. MRI and ultrasound are able to confirm synovial lesions and distinguish between reactive and proliferative synovitis. Reactive and proliferative synovitis are reversible findings after bleeding control treatment and radioisotopic ablation. According to our results we prefer the ultrasound as a fast, safe and easily available method to analyse the synovial changes, however, MRI is the most sensitive method to evaluate the joint damage. 24 Develop treatment of haemophilic pseudotumours: haemophilic cave treatment and reconstruction J. C. BALCAZAR Rebagliati Martins Hospital, Essalud, Peru Introduction Kønig first described the secondary joint alterations in haemophilia nearly a century ago. Two decades later, Starker reported a large haematoma of the thigh associated with extensive bone destruction; thus becoming the first case description of haemophilic pseu- dotumours. Countries with fewer resources and a deficient healthcare structure continue to observe haemophilic pseudotumours on a regular basis. The incidence in devel- oped countries is in second place after haemophilic arthropathy. Materials and methods This exposition is the result of orthopaedic surgery on haemophilic pseu- dotumours, all of them in patients with severe haemophilia type A, which had presented previous episodes except in one of them. In the 1° episode there are inhibitors and a high level of undernourished ‘osseous’ that has been classified on four levels based on osseous destruction and existence of inhibitors. Level I; extension of osseous destruction. Level II; loss of osseous mass. Level III; multiple loss of osseous mass (haemophilic caves). Level IV; patient with presence of inhibitors. We treated young haemophiliacs, between 12 and 30 years old, with pain and increase in the volume of the affected zone. We did studies by non surgical methods (1) Simplex RX forehead shot, profile shot, oblique shot with follow-up of 3 years; (B) TACC-RMN. After the study we did open surgery under the control of the haemophiliac unit from Edgardo Rebagliati Martins Hospital, Essalud. We performed the excavation of the pseudotumour capsule and its contents. Following this, we washed the osseous with 2 L of 0.9% saline solution plus one ampoule of aminoglucoside 80 mg. We found osseous perforations which we called haemo- philiac osseous caves. We rasped and filled the osseous cavity closed the planes with Teflon. After Jones dressing, passive exercises were used on the third day (external flexor), and walking in the eighth week. Results Progres- sive fill in 80% of all osseous parts of the handicap left by the haemophiliac pseudotumour in a period of 2 years 5 months. 25 Orthopaedics and haemophilia in Mexico G. CAPETILLO and M. D. MORALES Hospital del IMSS, Villahermosa, Tabasco, Mexico The Mexican Republic, which is formed by a federation of 31 states and a Federal district, has a population of approximately 3800 registered haemophiliacs. However, the total population, including haemophiliacs that have not been registered, must be double. The care these patients receive is offered by the medical institutions of the health sector, which are the Secretaria de Salud Publica (Ministry of Public Health), Instituto Mexicano del Seguro Social IMSS (Social Insurance Institute), the medical services of Petroleos Mexicanos PEMEX and the Instituto de Segur- idad Social de los Trabajadores del Estado ISSTE (Institute of Social Security for State Workers). At the same time the Federacion de Hemofllia de la Republica Mexicana A.C. promotes patient care. At the IMSS factor VIII and IX are used with an adequate supply. At the other institutions factor is used and in some of them still factor and cryoprecipitates. The orthopaedic viewpoint as to the care of osteomuscular complications varies in relation to a ORTHOPAEDIC ISSUES 385 Ó 2000 Blackwell Science Ltd Haemophilia (2000), 6, 376–395

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23Management of haemophilic arthropathyF. APARISI, F. QUEROL, J. BELTRAN,

C. CIFRIAN, C. MIGUEL and J. A. AZNAR

Radiodiagnostic Service, Hospital Rehabilitation La Fe

Unidad de CoagulopatõÂas CongeÂnitas, Hospital La Fe

Physiotheraphy Department, Universidad de Valencia,

SpainThe initial event consists of synovial or subsynovialhaemorrhage. After blood enters the joint cavity, the toxiceffects of haemosiderin and blood products provoke anin¯ammatory reaction, possibly through the generation oftoxic oxygen radicals. MRI and US are able to study thesynovial layer. MR images demonstrate an irregularlythickened synovial lining of low to intermediate signalintensity on T1- and T2-weighted images, probably as aresult of the presence of haemosiderin deposited within thesynovial membrane. Ultrasonography is a particularlyattractive means of performing serial noninvasive studiesto monitor response to joint bleeding and various thera-peutic endeavours. Synovial ablation with radioisotopeshas also been advocated, with ultrasonography used in aneffort to diagnose synovial thickening prior to the onset ofradiographic bony changes. We present a 40-case series ofhaemophilic arthropathy studied by X-ray plain ®lm, MRI,and ultrasound. After reviewing this series we found fourtypes of change: joint effusion, soft tissue thickening, boneerosions and bone cysts. X-ray plain ®lm and MRI are ableto con®rm the presence of bone lesions that correspondwith nonreversible arthropathies, there are no signi®cantdifferences between these methods of con®rming the bonelesions. MRI and ultrasound are able to con®rm synoviallesions and distinguish between reactive and proliferativesynovitis. Reactive and proliferative synovitis are reversible®ndings after bleeding control treatment and radioisotopicablation. According to our results we prefer the ultrasoundas a fast, safe and easily available method to analyse thesynovial changes, however, MRI is the most sensitivemethod to evaluate the joint damage.

24Develop treatment of haemophilic pseudotumours:haemophilic cave treatment and reconstructionJ. C. BALCAZAR

Rebagliati Martins Hospital, Essalud, PeruIntroduction Kùnig ®rst described the secondary jointalterations in haemophilia nearly a century ago. Twodecades later, Starker reported a large haematoma of thethigh associated with extensive bone destruction; thusbecoming the ®rst case description of haemophilic pseu-dotumours. Countries with fewer resources and a de®cient

healthcare structure continue to observe haemophilicpseudotumours on a regular basis. The incidence in devel-oped countries is in second place after haemophilicarthropathy. Materials and methods This exposition isthe result of orthopaedic surgery on haemophilic pseu-dotumours, all of them in patients with severe haemophiliatype A, which had presented previous episodes except inone of them. In the 1° episode there are inhibitors and ahigh level of undernourished `osseous' that has beenclassi®ed on four levels based on osseous destruction andexistence of inhibitors. Level I; extension of osseousdestruction. Level II; loss of osseous mass. Level III;multiple loss of osseous mass (haemophilic caves). LevelIV; patient with presence of inhibitors. We treated younghaemophiliacs, between 12 and 30 years old, with painand increase in the volume of the affected zone. We didstudies by non surgical methods (1) Simplex RX foreheadshot, pro®le shot, oblique shot with follow-up of 3 years;(B) TACC-RMN. After the study we did open surgeryunder the control of the haemophiliac unit from EdgardoRebagliati Martins Hospital, Essalud. We performed theexcavation of the pseudotumour capsule and its contents.Following this, we washed the osseous with 2 L of 0.9%saline solution plus one ampoule of aminoglucoside 80 mg.We found osseous perforations which we called haemo-philiac osseous caves. We rasped and ®lled the osseouscavity closed the planes with Te¯on. After Jones dressing,passive exercises were used on the third day (external¯exor), and walking in the eighth week. Results Progres-sive ®ll in 80% of all osseous parts of the handicap left bythe haemophiliac pseudotumour in a period of 2 years5 months.

25Orthopaedics and haemophilia in MexicoG. CAPETILLO and M. D. MORALES

Hospital del IMSS, Villahermosa, Tabasco, MexicoThe Mexican Republic, which is formed by a federationof 31 states and a Federal district, has a population ofapproximately 3800 registered haemophiliacs. However,the total population, including haemophiliacs that have notbeen registered, must be double. The care these patientsreceive is offered by the medical institutions of the healthsector, which are the Secretaria de Salud Publica (Ministryof Public Health), Instituto Mexicano del Seguro SocialIMSS (Social Insurance Institute), the medical services ofPetroleos Mexicanos PEMEX and the Instituto de Segur-idad Social de los Trabajadores del Estado ISSTE (Instituteof Social Security for State Workers). At the same time theFederacion de Hemo¯lia de la Republica Mexicana A.C.promotes patient care. At the IMSS factor VIII and IX areused with an adequate supply. At the other institutionsfactor is used and in some of them still factor andcryoprecipitates. The orthopaedic viewpoint as to the careof osteomuscular complications varies in relation to a

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particular health institution, although the Federation ofHemophilia has organized workshops and congresses indifferent states of the country to promote involvement inup-to-date developments, and publishes the works of thedifferent international forums on haemophilia. The compli-cations that a Mexican orthopaedist has to face aregenerally the same as in any other country. Particularly,the specialist receives patients with established grade II andIII arthropathies with a decrease of movement arcs, andmuscular atrophies, mainly in knees, ankles, elbows, hipsand shoulders. As regards the ®rst, radiosynoviorthesis inknees and ankles with Yttrium has been used for manyyears. To correct knee contractions tensor plaster hasbeen used. Intra-articular in®ltrations have been caredfor with bethametasone dipriopionate. Orthopaedic sur-gery on haemophiliacs has started with a correctiveosteothomy of varoaduct equinus foot, after a bleed inthe calf occurred at 7 years in a haemophiliac patient of22 years old, which is the case we present below. Thissurgery was performed at the General Hospital Zone 1of IMSS Tabasco in collaboration with Dr FedericoFernandez Palazzi from Venezuela. The patient wasstudied previously in order to discount the presence ofinhibitors. The surgery was performed in a surgical timeof 2 h. 3500 units of factor were used during thesurgery. While in hospital the patient did not have anycomplications whatsoever and a total of 30 000 factorunits were used ($300 000 approximately). The result isa foot of a smaller size, but well planted and with goodsupport. Also there is a psychological bene®t for thepatient who will now be able to continue his career inhuman medicine, which was interrupted because of hisdisability. Conclusion In this case the availability offactor VIII and surgical techniques made the patient'simprovement possible, both physically and psychologi-cally, because the deformity of the varo equinus foot inadults raises serious technical dif®culties, which in thiscase were magni®ed by haemophilic illness and becauseit was secondary to a tricepsural bleeding that hadoccurred in the patient's childhood.

26Partial diaphysal femur replacementin a haemophilia patientG. CHEMIS, V. ZORENKO, M. RYASHENTSEV

and Y. ANDREEV

Special Orthopaedic Department, Haematological

Scienti®c Center, Moscow, RussiaA 32-year-old patient with severe haemophilia B, who hadpreviously undergone corrective osteotomy (2 years beforepresented case) due to incorrect consolidation in his rightfemur, was hospitalized in our clinic after trauma to theright femur causing fracture, large open suppuratedhaematoma in the affected area, osteomyelitis in thefractured bone margins, purulent left knee arthritis, deepanaemia and sepsis. All suppurative focuses were drained,

and femur wound debridement with excision of infectedbone margins was performed. A 16 cm length femur defectwas formed after procedure. When septic process subsideda partial diaphysal femur replacement covered by factor IXconcentrate Ahaem®lum B (Russia) infusions was carriedout. Neither surgical nor transfusion complication wasobserved. Follow-up At 2 years, there are no signs ofosteomyelitis, prothesis is stable. Conclusion Modernhaemostatic therapy and surgical care allow not only saveslives for haemophiliacs but prevents their disability.

27Rheumatoid arthritis in a patient with haemophilicarthropathy ± a case reportH. EICKHOFF,* G. RADERSCHADT,* A. SEUS-

ER,  T. WALLNYà and H. BRACKMANN*±*Orthopaedic Hospital St. Josef, Troisdorf, Germany;

 Kaiser Karl Klinik, Bonn, Germany; àOrthopaedic

±Hemostasiological Departments of the University

of Bonn, Bonn, GermanyThe prevalence of haemophilia in Europe is about1:10 000. According to the literature, the prevalence ofrheumatoid arthritis in males is about 1:400. Therefore thetheoretical occurrence rate of a combination of bothdiseases is 1:4 000 000. In haemophilia, it is mainly theknee, elbow and ankle joints that are affected. The jointinvolvement in rheumatoid arthritis usually relates to®nger, hand, elbow, knee and ankle joints. However, withthe exception of the distal interphalangeal joints, allsynovial joints can be affected. An additional extraarticularinvolvement is frequently observed. In the combination ofhaemophilic arthropathy and rheumatoid arthritis anadditional effect concerning the number and distributionof affected joints as well as the degree of joint involvementcan be expected. The case presented is one of a 54-year-oldpatient from the former East Germany with haemophiliaA, factor activity <1%. Due to recurrent bleeding episodes,severe arthropathy of all major joints developed (knee, hip,ankle, elbow, shoulder). In addition, in 1980, the patientdeveloped a seropositive rheumatoid arthritis which dete-riorated the joint situation dramatically. In 1989 he wascon®ned to a wheelchair. In October 1990, the patient wasadmitted to the Bonn University Hospital Orthopaedicdepartment for combined haemostatic and rheumato-logical treatment. A cementless total hip arthroplasty wasimplanted on the right side (October 1990) and on the leftside (January 1991). A cemented knee arthroplasty (typeGSB) was implanted on the left side in April 1991. InFebruary 1992, a cementless total condylar prosthesis wasimplanted on the right side (type Tricon). During follow-up, the second knee arthroplasty showed a signi®cant lossof range of movement. That led to a revision in May 1997with arthrolysis and exchange of the prosthesis to acemented GSB knee arthroplasty (Orthopaedic HospitalSt. Josef, Troisdorf). At the ®nal follow-up in October

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1999 the patient walked unaided and is very satis®ed withthe result.

28Orthopaedic surgery experience in the nationalhaemophilia center of CaracasF. FERNANDEZ-PALAZZI, A. BOADAS,

A. RUIZ-SAEZ and N. DE BOSCH

National Haemophila Center, Banco de Sangre del D.F,

Caracas, VenezuelaFrom January 1973 until December 1999, we haveperformed at our centre 50 orthopaedic interventions and33 nonorthopaedic surgeries in 80 patients between 3 and64 years of age. Orthopaedic surgery was performed in 46haemophilic patients and one patient with Glanzmann'sthrombastenia. Nonorthopaedic surgeries were performedin 22 haemophilia A patients, three B haemophiliacs, ®ve invon Willebrand, two female haemophilia A carriers andone haemophilia B carrier. The most frequent orthopaedicoperations were voidance of bone cysts and ®lling with`®brin seal' in 13 cases; knee synovectomies in six cases (1by arthroscopy); resection of haemophilic pseudotumoursin four cases; hip joint prosthesis in four cases; femoralosteotomies in three cases; knee arthrodesis in three cases;elbow synovectomy + radial head resection in one case andWilson knee capsulotomy in one case. In 39 of oursurgeries (orthopaedics and non) we used ®brin seal(TissucolÒ; Baxter or BeriplastÒ; Centeon). In 10 ortho-paedic and two general surgeries performed from June1998 till June 1999, all in A haemophiliacs, we used asfactor coverage continuous infusion of factor VIII. Thenonorthopaedic most frequent operations were appendec-tomies in eight cases; haematoma drainage (subdural orretroperitoneal) in six cases; adenotonsillectomies in threecases; and haemioplasties in three cases. In three vonWillebrand type 1 patients we used DDAVP repeatedly,two for adenotonsillectomies and one for duodenal resec-tion for a perforated ulcer.

29Survey of surgical interventions performed inchildren with haemophilia in the Czech republicsince January 1989±December 1999V. KOMRSK, L. CRHOVA, J. MRACEK,

V. VAVRA and J. STARY

Department of Hematology, Department of Orthopaedics,

University Hospital Motol, Prague, Czech RepublicIn the Czech Republic, with a population of 10.3 millioninhabitants, there are about 3 million (29.3%) people atthe age of 0±22 years. The incidence of haemophilia is>0.06% with a total number of 180 haemophiliacs among22 years old or younger. 73 patients with haemophiliaand 14 patients with von Willebrand's disease (vWD)were followed in our centre from January 1989 till

December 1999. 76 urgent or planned surgery procedureswere performed in 47 of these patients during this period.There were 30 patients with haemophilia A without, andeight with inhibitor, ®ve with haemophilia B and fourwith vWD. Orthopaedic surgery was indicated in 65.7%(50 procedures); synoviorthesis (18), knee synovectomy(14), elbow synovectomy (2), knee release (11), elbowrelease (1), reposition of fracture (1), Achilles tendonlengthening (3). Other consisted of orchidopexy (2),hernioplasty (4), cleft lip repair and palatoplasty (2),circumcision (1), revision of abdomen (2), central venouscatheters (4), sutures of different lesions (4), nail ablations(3), excision of naevi (2), adenotomy (1) and tonsillecto-my (1). Continuous infusion of factors was used in only10 procedures (13%) while other surgeries were securedby bolus replacement therapy. Results Orthopaedic sur-gery had favourable outcome. In all cases reducedfrequency of joint bleeding episodes was achieved. Nineearly complications had appeared after 16 synovectomieswith fever in one, allergic reaction after replacementtherapy (1), bleeding (4) and re-surgery (2). Later com-plications had developed after 3 procedures, demandingelbow re-surgery after 2 years in one, and knee re-surgeryafter 5 months in another patient, respectively. Conse-quent joint contracture appeared in one patient. Incontrast, there were only late complications after synov-iorthesis in four out of 18 procedures. No effect wasachieved in three of them. No complications wereobserved after joint release (12) with only one treatmentfailure. In our cohort of patients shorter hospital stay anddecreased incidence of bleeding complications were seenwhen continuous replacement therapy was used instead ofbolus therapy. Excellent results were obtained after plasticsurgery in patients with cleft defects. Summary Wepresent results of surgery procedures performed in youngpatients with haemophilia in a single institution betweenJanuary 1989 and December 1999.

30Outpatient treatment with RSO for haemophilicarthropathyD. LAZOVIC,* G. TOENSHOFF,  M. FINK,àA. ROSENSTOCK,à M. VON DEPKA± and

A. GANSER±*Department of Orthopaedics, àDepartment of Physical

and Rehabilitation Medicine; ±Department of

Haematology and Oncology, Medical School Hannover;

 Radiological Institute, Hannover, GermanyPurpose Characteristics of haemophilic arthropathy arerecurrent bleedings, chronic synovitis and osteochondraldestruction. Prophylactic treatment to prevent intra-artic-ular bleeding is essential. If chronic arthritis has alreadyoccurred operative treatment is considered. Alternativelyradiosynoviorthesis (RSO) is discussed We investigated theeffect of outpatient treatment with RSO on advanced

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haemophilic arthropathic joints. Method In 1998 and1999 we saw 48 patients with haemophilic arthropathyin our interdisciplinary consulting hours. In 12 joints (twoshoulders, three elbows, three ankles, four knees) thesymptoms matched our criteria for RSO. It was done in anoutpatient procedure with 90Yttrium after a clinical sono-graphic, radiographic and scintigraphic evaluation.Results Synovitis and frequency of bleeding could bereduced or totally suppressed in all joints. Reduction ofpain was seen as a major advantage. Complicationsoccurred in one shoulder as a burn by disregarding thedirected after-treatment and in one knee as progressivedestruction by overuse. Conclusion RSO seems to be afavourable alternative to operative procedures even insome cases of advanced haemophilic arthropathy. It can bedone in an outpatient procedure. It does not reduce theprogression of joint destruction but leads to a reduction ofsymptoms, especially of pain and joint bleedings.

31Haemophilic arthropathy ± a single centre'sexperienceM. JADHAV, I. WARRIER, C. KOEHLMANN,

C. MANCINI, D. STANITSKI, C. BECKER,

S. KOTTAMASU and J. LUSHER4

Children's Hospital of Michigan, Detroit, MI, USAChronic haemophilic arthropathy is one of the majorcauses of debilitation in patients with haemophilia (H).Factor prophylaxis has made a signi®cant difference byprevention of recurrent joint bleeds. However, oncerecurrent bleeding occurs in a joint a vicious cycle setsin. Surgical synovectomy and more recently chemical andradionuclide synovectomy (RS) offer promising results forcontrol of recurrent joint bleeding. Over 3 years we haveperformed RS using 32P on ten target joints (®ve knees,three ankles and two elbows) in eight patients (ages 6±22 years) being followed by our multidisciplinary haemo-philia programme. Seven patients had HA and one hadHB. Five of seven patients with HA had inhibitors; threehigh titre inhibitors and two low titre inhibitors that hadresolved by the time of the procedure. The noninhibitorpatients were on routine prophylaxis therapy for 3±6 months prior to and for 3 months following theprocedure. All patients had radiological (XRay andMRI) evaluation prior to RS. Six of the eight patientshad advanced joint disease by Petterson's scale. Allpatients were evaluated by a physician and a physio-therapist prior to and 1 week, 1 month, 3 months and6 months following RS. In patients <13 years of age thedose of intra-articular 32P was 0.25 mCi for small jointsand 0.5 mCi for large joints, while older patients received0.5 mCi for small joints and 1 mCi for large joints.Patient charts and clotting factor usage logs were evalu-ated to estimate the bleeding episodes into the treatedtarget joint for 1 year prior to the procedure and for

every following year. RS signi®cantly reduced, but did noteliminate the number of haemarthroses. This effect wasseen maximally in the ®rst year following RS. Joint size,mobility and overall function of the joint improved in allpatients. No adverse events were observed in any patientfollowing the procedure. Chromosomal analyses per-formed on four patients were normal pre- and post-procedure. Follow-up radiological evaluation performedin ®ve patients after 6 months did not reveal anyimprovement in the appearance of the joints. In conclu-sion, RS was effective in reducing the number of recurrentbleeds into the joint but did not change the course ofexisting haemophilic arthropathy.

32Spectrum of myositis ossi®cans (MO) in haemophiliaG. MASSEY,* J. KUHN,* J. NOGI, S. E. SPOTTSWOOD, à C. JOBNSON,±N. DUNN* and E. C. RUSSELL*

Departments of *Paediatrics,  Orthopaedics, àRadiology

and ±Radiation Oncology, Virginia Commonwealth

University, Medical College of Virginia, USAMO is heterotopic soft tissue ossi®cation that has severalaetiologies (genetic vs. acquired). Most common is MOtraumatica which is seen in normal young adults with ahistory of trauma. We present here three adolescent boyswith haemophilia and MO of varying severity. MY wasa 14-year-old male with severe (<1%) factor VIIIde®ciency. He was treating himself with on-demandtherapy and had mild arthropathy of his ankles. He hashad no inhibitor. In May 1997 he began to complain ofleft hip pain with no known history of trauma. Radio-graphs revealed ossi®cation medial to the lesser tro-chanter consistent with MO. He was treated with dailyfactor: infusions and is now on secondary prophylaxis.Symptoms resolved and no further intervention wasrequired. PK was a 19-year-old male with mild (5±7%)factor IX de®ciency. He was receiving on-demandtherapy with a mild clinical course. In November1996, he sustained an injury to his right upper armplaying football. By December 1996, although he had nopain, he had decreased range of motion (ROM) of thearm and a palpable mass over the humerus. Radiographsshowed MO in the soft tissues lateral to the righthumerus. Bone scan con®rmed these ®ndings. He wastreated conservatively and by July 1997, ROM andradiographs were normal. JW was a 16-year-old malewith severe (1%) Factor VIII de®ciency, with a mildclinical course, no arthropathy and no inhibitor. In April1995, he sustained a mild injury to his right hip while®shing. Despite aggressive therapy he had decreasedROM and gait alterations, without pain. Radiographs,showed a 7.6 ´ 2.5 cm ossi®cation in the quadricepsextending to the lesser trochanter. Secondary prophylaxiswas started and serial bone scans were performed to

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assess activity of the lesion. In April 1996 he underwentsurgical resection of the lesion and adjuvant radiation of8 Gy. He remains well with normal ROM. MO inhaemophilia is rarely reported. Given the increasedincidence of soft tissue bleeds in this population, MOshould be considered in the differential diagnosis ofpatients with signi®cant loss of ROM without acutepain. Conservative management is recommended asspontaneous resolution can occur.

33Peculiarities of arthroscopic operation usagefor patients with haemophiliaL. PASSOIAN, V. ZORENKO, V. MAMONOV

and Y. ANDREEV

Department of Orthopaedic Surgery, Haematological

Scienti®c Center, Moscow, RussiaRegular research on usage of arthroscopic operations forpatients with haemophilia (children and adults) has beenstarted since March 1999 in our department. At thepresent stage, our purpose was limited to determinationof dates and ®nding out of peculiarities of arthroscopicoperation usage for this disease. At the present time, ourexperience includes operative treatment of 18 patients(16 operations) at the ages of 7±25 years (mean 12).Among them, 13 with severe hameophilia A, Ai, 1; onewith haemophilia B and one with von Willebrand'sdisease. Standard X-ray of knee joint in two views wasdone to all patients. Additional researches were necessaryfor nine patients including ®ve CT scans, two contrastX-ray and two MRT. In the ®rst two cases, arthroscopywas for diagnosis and was done before planned opensynovectomy. In 11 cases indications for surgery treat-ment were frequent haemarthroses due to synovitis (allpatients were children), severe pain in joint during activeand static processes (2), fracture of eminentia inter-condylaris of tibia (1) and in one more case, arthroscopicdebridement was necessary for patient as a consequenceof frequent knee haemarthroses in the 6 months afteropen surgery treatment done by the classic method. Highpurity factor VIII, FEIBA and cryoprecipitate was usedfor regulation of haemostasis during the operation andnearest postoperative period for patients with haemo-philia A and von Willebrand's disease; Agem®l B(domestic factor IX) for the patient with haemophiliaB. Besides this, all patients got preventive antibiotictreatment. To take away postoperative in¯ammation andpromote quick rehabilitation they were given a course ofhyperbaric origination intra-articular injections of ste-roids, an accepted method in the clinic. Preliminaryresearch results revealed some advantages of the use ofarthroscopic operations over open surgery. Arthroscopicoperations, synovectomy in particular, have less bleeding,and are less painful, which is very important forchildren. Recovering motion in joints takes place in 1±

2 months after operation (in open synovectomy during6±12 months). There were no serious complications afterillness. Haemostatic therapy duration and total volumefor haemostatic preparations were 2 times less thanduring open surgery.

34Pettersson scores in haemophilia patients with earlyonset and long duration of prophylactic treatmentR. PREJS,* K. FISCHER, à J. G. VAN DER

BOM, à T. H. D. WITKAMP,* E. P. MAUSER-

BUNSCHOTEN,  G. ROOSENDAAL  and

H. M. VAN DEN BERG *Department of Radiology,  Van Creveldkliniek,

àJulius Center for Patient Oriented Research, University

Medical Center Utrecht, the NetherlandsIn patients with severe haemophilia it is commonlybelieved that the knees are the joints in which joint damageis most pronounced. This study was set up to examinewhether this is also true in patients with early onset andlong duration of prophylactic treatment. From the cohortof patients treated in Van Creveldkliniek we selected 51patients who started prophylactic treatment before the ageof 6 years and were on prophylactic treatment for at least60% of life at the time of scoring. Pettersson score consistsof eight items: osteoporosis, enlargement of epiphysis,irregularity of subchondral surface, narrowing of jointspace, subchondral cysts formation, erosions on jointmargins, joint incongruity and deformity, scored for knees,elbows and ankles. Totals were calculated for every item inPettersson score, separately for each joint (total maximumof 78 points). Mean age at latest Pettersson score was15.5 years. Mean total score per patient was 4.2 points. Ofall 214 points scored, 108 points (50.5% of total points)were recorded in ankles, 89 points (41.6%) in elbows, andonly 17 points (7.9%) in knees. 79 of 108 points withinankles was scored for irregularity of subchondral surface,narrowing of joint space and subchondral cysts formation.Enlargement of epiphysis was almost exclusively (96%)seen in elbows and ankles. Knees mainly scored inosteoporosis (9/17 points, 53%). We observed that thepredilection for joint damage due to haemarthrosis in ourgroup of patients is completely opposite to what is knownfrom the textbooks and other publications: the mostseverely affected joints in our group are ankles, followedby elbows and by knees. The most likely reason for thispattern of joint damage is the early onset of prophylaxisthat allows for more mobility, which results in moreintensive use of ankles.

35An analysis of clinical results of the use of hyaluronicacid in the treatment of haemophilic arthropathy

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S. PROVELENGIOS, O. KATSAROU,*

A. TSIBINOS, E. LOUCOPOULOU,*

R. TSAKNIS and A. KARAFOULIDOU*

Department of Orthopaedic Surgery, and *Comprehensive

Haemophilia Care Center, Laiko General Hospital of

Athens, GreeceHyaluronic acid has used successfully in the treatment ofosteoarthritis since the early 1990s, but experience inhaemophiliacs remains poor. Methods In a prospectivestudy of 14 patients with haemophilic arthropathy (tenknees, three ankles and 1 elbow) we performed a total of70 injections of 20 mg hyaluronic acid sodium salt intra-articularly for 5 consecutive weeks (once a week). Allpatients suffered of pain and limited range of motion. Allof them were HCV+ and among them were six HIV+.Patients' age varied from 17 to 52 years (mean 35 years).For the clinical and radiographic evaluation we used theWFH score and Petersson score, respectively. The meanWFH score was 7 points (5±11) and the Pettersson score 9points (3±12). Results The follow-up time ranged from 3to 28 months (mean 18.5 months). The WFH scoredecreased to 5.5 points. All but two patients continue tobe almost free to pain and to present slight improvement inthe range of motion. Ankles had a poorer result than theother joints. Conclusion Despite no experience in largerstudy groups, the intra-articular injection of hyaluronicacid seems to be bene®cial, especially in cases where thedecision for more radical procedures is critical. In ourseries, results were independent of the severity of thearthropathy (according to the clinical and radiographicscore).

36Orthotics in haemophiliaF. QUEROL, M. C. SOTOS, S. HAYA,

J. I . LORENZO and J. A. AZNAR

Unidad de CoagulopatõÂas CongeÂnitas, Hospital La Fe

Physiotheraphy Department, Universidad de Valencia,

SpainIn addition to replacement therapy, the treatment andprevention of disorders to the musculoskeletal system inthe haemophilic patient requires rehabilitation and phys-iotherapy, as well as the use of orthotic devices adaptedto the part of the body that we want to protect. When theinjury has required surgery, the purpose of orthosis is toimmobilize the part of the body affected, relieve the jointpartially or completely of its weightbearing function,facilitate alignment of the articular axes, and assistanalytical movement. In the acute phase of haemarthrosisthe main priority of orthosis is to rest the joint. In ourexperience the use of articulated splints enables us tomaintain antalgic position for the ®rst 24±48 h, afterwhich the same splint can be placed in the functionalposition, allowing physical activity in the movementranges established by the therapist or physician, and

accelerating the process of return to full weightbearingfunction. In the chronic phase the fundamental objectiveof orthosis is to protect, stabilize, or relieve the joint ofload. The characteristics of elbow, knee, and ankle padsare described according to the therapeutic objectivepursued. Basic protection consists of a tubular bandagesystem adapted to the characteristics of the joint andwhich is made up of materials like silicone, which absorband cushion impact, at the same time as reducing swellingand stabilizing analytical movement. The characteristicsof the `Air Cam Walker' load-relief splints are alsodescribed, which are especially useful in the haemarthrosisof the ankle. For the absorption of talar impact and itsrepercussion on microtrauma to the ankle, we use asystem of instrumented insoles, designed with the help ofcomputer programs that have enabled us to know, amongother things, the distribution of load on a surface onweightbearing. By using this method, we know thedistribution of load in the foot, which enables us tomake orthopaedic insoles with material of differentdensities. To prevent spontaneous haemartrosis at night,we use `Neofrakt' splints because they are easy and quickto manufacture, and comfortable to use. The character-istics of these type of orthotics for elbows, knees andankles are described in this paper.

37Incidence of joint haemorrage in haemophiliaF. QUEROL, J. I . IGNACIO and J. A. AZNAR

Unidad de CoagulopatõÂas CongeÂnitas, Hospital La Fe

Physiotherapy Department, Universidad de Valencia,

SpainDuring an 8-year-period between January 1991 toDecember 1998 we collected data on the haemarthrosesof our haemophilic patients, affecting the shoulder,elbows, hips, knees and ankles. Our haemophilic patientsreceived home replacement therapy. Patients receiving on-demand treatment were given 20 IU d)1 for 1±3 days oruntil the cessation of the symptoms. Patients receivingprophylactic treatment were administered 20 IU body-weight 3 days week)1. The follow-up visits wereplanned, and patients received 1±3 visits a year, depend-ing on the severity of the haemophilia. In the pro-grammed visit each patient kept a record on the treatmentthat was prescribed for him, in which the occurrence,location, and duration of bleeding episodes are noted, aswell as the amount of factor concentrate administered.We studied 183 patients, who received a total of 2318programmed visits. Our patients suffered 5412 haemarth-roses: elbows, 1874; ankles, 1431; knees, 1344; shoulders,595; and hips, 168. The incidence of injuries was greaterin elbows (34.8%), followed by ankles (26.4%), knees(24.8%), shoulders (10.9%), and hips (3.1%). Severehaemophiliacs suffered 4043 episodes, moderate 1253and mild 53. Severe, moderate and mild haemophilia

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showed the same order of injury incidence: elbows,ankles, knees, shoulders and hips.

38Cartilage is more susceptible to blood-induceddamage at young than at old ageG. ROOSENDAAL, M. E. VIANEN,

J. M. TEKOPPELE, H. M. VANDENBERG,

F. P. LAFEBER and J. W. BIJLSMA

Van Creveldkliniek and Department of Rheumatology and

Clinical Immunology, UMC Utrecht, PO Box 85500,

3508GA Utrecht, The NetherlandsIt has been demonstrated that cartilage is damaged uponintra-articular haemorrhage (patients with haemophilicarthropathy). The present study investigates differences inthe susceptibility of young and old cartilage to blood-induced joint damage in a canine in vivo model. Rightknees of six young (2.2 � 0.1 years) and six adult(7.4 � 0.3 years) dogs (beagles) were intra-articularly in-jected twice in 4 days with autologous blood. Dogs werekilled 4 or 16 days after the ®rst injection and cartilagematrix proteoglycan content and synthesis, and collagendamage were determined. Shortly after blood injection(day 4), proteoglycan synthesis was inhibited and theproteoglycan content was decreased in young and oldcartilage. However, the degree of the inhibition of prote-oglycan synthesis was signi®cantly greater in young carti-lage than in old cartilage. On day 16 proteoglycansynthesis was increased in both young and old cartilage,but more elevated in old cartilage. The proteoglycancontent remained decreased in both young and old carti-lage, but signi®cantly more so in young cartilage than inold cartilage. The results show that intra-articular bleedingdamages joint cartilage and they suggest that the cartilageof young animals is more susceptible to such damage thanis the cartilage of old animals. Differences in the ageing ofchondrocytes and age-related changes in matrix integritymay be involved. Prevention and appropriate treatment ofjoint bleeding is indicated and this is especially relevant foryoung cartilage (and probably for young patients withhaemophilia).

39Biomechanical research in haemophiliaA. SEUSER,* T. H. WALLNY,  G. SCHUMPE,àH-H. BRACKMANN± and H. EICKHOFF§

*Department of Orthopaedics Kaiser-Karl-Klinik, Bonn,

Germany;  Orthopaedic Department, University Clinic

Bonn, Germany; àDepartment of Biomechanics,

University Clinic Bonn, Germany; ±Hemophilia Treatment

Center, University of Bonn, Germany; §Orthopaedic

Department, St. Josef Hospital, Troisdorf, GermanyMaterials and methods The on-line 3D motion analysiswas performed with the Original Ultrasound Topometer(UST, Bonn, Germany). All recorded data was processed

with special software, providing analysis of the relativejoint angles, angle velocity and angle acceleration to beshown in any of three dimensions. In addition, we canmeasure the roll and glide mechanism of the knee jointrelated to the joint angle. We examined in several studiesknees, ankles and elbows of haemophilic patients.Results We used the roll and glide mechanism tocharacterize the inner knee motion, to detect functionaldisturbances and to optimize conservative treatment. Wecan tell the exact location of the disturbance during knee¯exion. Control analysis will show the changes andindicate the need of surgery if the monocentric characterof the joint cannot be in¯uenced by conservative treat-ment due to severe structural damage. We investigatedthe in¯uence of intra-articular hyaluronic acid on theknee joint. Whereas the clinical and radiological scoresdid not show signi®cant changes, the roll and glidecurves before and after therapy did. We measured aspeci®c rotation pattern of the normal knee. Even slightpathologies have a long-term in¯uence on the joint'skinetics. In haemophiliacs we see an early loss of rotationcapacity and characteristics even before restriction of the¯exion arc occurs. Looking at the ankle we saw adramatic change in gait pattern with silicon heel cush-ioning. The angular velocity of the ankle was increased,producing 2 times higher acceleration at the ankle joint.The in¯uence of the heel cushioning diminished withrestricted ankle motion. As a matter of fact, late stagehaemarthropathic joints are not able to react any moreto external forces. Gait analysis of the correspondingknee joints however, showed that the compensation tookplace there and resulted in false movements, higher anglevelocities and accelerations, and therefore in higherloading of these joints. Without muscular control, theankle joint is loaded with shear forces and the dyscoor-dinated motion might cause impingement of the synovialtissue, which is a major reason for joint bleedings inhaemophilia. We examined the rotation of the elbowjoint. The results show that there is a special distributioncharacteristic of the whole range of rotation throughoutthe extension ¯exion ROM of the elbow. In thehaemophilic elbow we detected pathologies in quantityand curve characteristic. There was no correlationbetween clinical or radiological ®ndings. Even elbowswith Pettersson 0 show changes in distribution ofrotation and diminished rotational ROM. We have toconclude that even slight disturbances in the elbow jointcausing clinically undetectable changes in muscle andligaments will result in a functional pathology. Thiscould be the beginning of the end of the joint. Earlyphysiotherapy with special regards to rotation throughout the whole ¯exion is compulsory. Conclusion Theexisting biomechanic motion data teaches us that thereare deeper levels of joint kinetics than we can realizewith clinical or radiological means. Biomechanical diag-nosis and motion analysis are of growing importance,especially in chronic illnesses. To keep the locomotivesystem healthy we need a lot more information than the

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usual diagnostic and scores can offer. As we deal withmoving beings, motion analysis will be a tool forobjectively planning and monitoring successful therapy.

40Haemophilic arthropathy in a referral haemophiliacentre in IranR. A. SHARIFIANSH and S. M. LAK

Imam Khomeini Hospital, Hemophilia Centre Keshavarz

Avenue, Tehrantul, IranChronic haemophilic arthropathy is the most commondebilitating complication of severe haemophilia A and B,especially in developing countries, where the HIV positiv-ity is not a major limiting factor for patients' survival (as awhole) and treatment facilities are not adequate. Materialand methods We examined a total of 208 patients(patients) with severe (factor level <1%) and moderate(factor level 1±5%) haemophilia A (176 patients) andhaemophilia B (32 patients) for chronic knee, elbow andankle joint arthropathy. The severity of knee joint wasgraded according to the WFH criteria for haemophilicarthropathy. Patients less than 5 years old were excluded.12% of patients had antibody to FVIII (or FIX). The studywas a randomized, cross-sectional descriptive one. Radio-logical evaluation is also planned. Patients with acute jointbleeding were excluded or reassessed 3 weeks later.Results A total of 208 patients were evaluated; of those89.9% had some degrees of chronic arthropathy in one ofthe evaluated joints (knee arthropathy 75%, elbow 55.3%,ankle 37%). The frequency and severity of joint diseaseincreases with increasing age. The results are shown inTables 1 and 2.

Conclusion The main problem in our haemophilia centreat this time is inadequate replacement treatment, leading torepeated haemarthrosis and chronic arthropathy even inchildren (5±10-year-old, 78.5%). This ®gure would in-crease over a 10-year period to 95%, making over 2000young haemophilic patients debilitated and requiringhundreds of joint replacement procedures. Although weare facing inadequate health budgets, we have to prepareand arrange some kind of regular prophylactic factorreplacement therapy in near future.

41Time course of haemophilic arthropathy evaluatedby X-ray ®ndingsA. SHIRAHATA and M. SAKAI

Department of Paediatrics, School of Medicine, University

of Occupational and Environmental Health, Kitakyushu,

JapanHaemophilic arthropathy limits the range of motion,leading to dif®culties in their activities of daily life. Inorder to improve the QoL of haemophiliacs with avariety of problems, we opened the North KyushuHemophilia Center in 1984. Our centre has put emphasison prevention of haemophilic arthropathy and guidancefor delaying its progress. For this purpose, X-rayexamination as well as measurement of the range ofmotion and muscle strength are performed at everycomprehensive outpatient clinic service (Each patientreceives this service once a year, in principle), and anychanges are carefully observed. If arthropathy becomesworse in spite of adequate infusion, which should beperformed according to the fundamental rule as soon aspossible after bleeding, then a regular infusion should berecommended. As another approach, guidance for reha-bilitation training at home for strengthening or restoringthe range of motion is given based on ®ndings from theout patient clinic service. Preparation of supporters orprosthetic appliance, and adjustment of shoes are pro-vided as necessary. Using data collected through the15 years that our centre has been in operation, weexamined the joints' X-ray history of each of the patientsincluded in the study. The X-ray ®ndings were evaluatedby two orthopaedic doctors using a modi®ed De Palmamethod. Sixty-one haemophiliac patients who had at-tended the comprehensive outpatient clinic service formore than 8 years were enrolled in this study. Thepatients were divided into four groups according to theirages (group A 15±19 years; group B 20±24 years; groupC 25±34 years; group D 35±45 years). The results of thestudy indicate that the progress of haemophilic arthro-pathy of the elbow or knee joints (grade 2 or more) wasfound in only three patients during the observationperiod of 8 years or more, although severe arthropathy(grade 3 or 4) was found in many older patients on their®rst visits to our centre. Our study also found aworsening of the ankle in one third of the young

Table 2 The frequency and severity of knee joint arthropathy in

different age groups

Age(years)

No.patients

Grade 0(Normal)

Grade I(Minimal)

Grade II(Mild)

Grade III(Moderate)

Grade IV(severe)

5±10 28 11

(39.2%)

4

(14%)

11

(39.2%)

0

(0%)

1

(3%)

10±15 33 12

(36%)

3

(9%)

9

(27%)

7

(21%)

1

(3%)

15±20 62 15

(25%)

8

(13%)

23

(36%)

12

(19%)

3

(5%)

>20 85 13

(15%)

3

(3.5%)

24

(28%)

31

(36%)

14

(16%)

Table 1 The frequency of arthropathy by age

Age (years) No. patients Joint lesion (all) Per cent

5±10 28 22 78.5

10±15 33 28 84.8

15±20 62 57 91.9

>20 85 80 94.1

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patients, even those born after 1980. These resultssuggest that a prophylactic regular infusion of coagula-tion products from an early stage (1 or 2 years old) isneeded for the prevention of haemophilic arthropathy ofthe ankle.

42Joint range of motion (ROM) limitations amongyoung males with haemophilia in the USAJ. M. SOUCIE, S. CRUDDER, B. LEVATT and

THE UNIVERSAL DATA COLLECTION (UDC)

PROJECT INVESTIGATORS

Hematologic Diseases Branch, Centers for Disease Control

and Prevention (CDC), Atlanta, GA, USATo assess the extent of joint disease, ROM measurementswere obtained on all persons with haemophilia receivingcare in U.S. haemophilia treatment centres who voluntarilyparticipated in a national surveillance project (UDC) fromMay 1998 through October 1999. Measures were obtainedduring a clinic visit on ten joints (shoulders, elbows, hips,knees, and ankles) by trained medical care providersfollowing a standardized protocol. The sum of themeasures of ¯exion and extension (including hyper-exten-sion) of all joints was subtracted from the sum of thenormal adult values for these measures (American Associ-ation of Orthopaedic Surgeons) to estimate the overallROM limitation for each patient. Haemophilia severitywas based on factor activity (FA) levels and categorized asmild (FA 6%±50%), moderate (1%±5%), or severe (<1%).Complete ROM measures were obtained on 3367 haem-ophilic males, of whom 81% had haemophilia A. Themean (median) age was 21.4 (15.9) years and 21% hadmild, 23% had moderate, and 56% had severe haemo-philia. Overall, the mean (+SD) percentage joint ROMlimitation was 6.9% (10.5) and varied by age and diseaseseverity. The ®gure shows trend lines based on mean jointROM limitations of the 2020 haemophilic males <20 yearsof age.

43Ultrasonography: a dynamic imaging toolto evaluate bleedsD. STEPHENSEN and M. WINTER

Kent Haemophilia Centre, East Kent Hospitals Trust,

Canterbury, UKUltrasound imaging has been used since the early 70s as adiagnostic tool in haemophilia, almost exclusively for thedifferential diagnosis of psoas muscle bleeds. However itsuse has never been expanded further. Recent advances inultrasonography equipment now allow exceptional softtissue resolution, thus revolutionizing the assessment ofmusculoskeletal pathology in the patient with haemophil-ia. It allows the clinician to look inside muscles andjoints. The exact size, shape and location of joint and

muscle bleeds can be observed and monitored. The effectof bleeding on structures within the joint (e.g. synoviumand articular cartilage) can be observed. The effect ofmuscle bleeds on surrounding structures (e.g. nerve tissueand other muscles) can also be observed. The response tofactor replacement therapy can be observed and moni-tored. Ultrasound technology now allows the clinician todynamically evaluate and view joint and muscle struc-tures during movement. Ultrasound in the future willprovide vital information and knowledge on the state ofbleeding and the cause of bleeding in joints and muscles.This presentation describes the daily clinical use ofmusculoskeletal ultrasound in the haemophilia clinic.The advantages and uses of this diagnostic tool inassessing acute bleeds, synovitis and chronic musculo-skeletal changes are demonstrated. The scanning anddiagnosis is made by the clinician at the bedside. This notonly makes diagnosis and assessment quicker but allowsthe patient to view the images as well. A good educa-tional tool, particularly for children. The equipment usedis a Diasus PC driven high resolution diagnostic ultra-sound scanning unit. Two transducers are utilized, a 5±12 MHz transducer for deep muscle evaluation and a8±16 MHz transducer for super®cial joint assessment. Intodays healthcare environment where cost containmentand outcome reporting are becoming major issues thebene®ts of musculoskeletal ultrasound cannot be ignored.Ultrasonography is inexpensive and safe. To assessmusculoskeletal pathology it is far easier to use thanother forms of imaging. The results of ultrasoundimaging can be used to evaluate and report outcomesof treatment. In countries where factor replacementtherapy is a scarce resource ultrasonography could beused to quantitatively evaluate the severity of a bleedingepisode and prioritize treatment protocols.

44Calci®c tendinitis and ultrasound therapyF. TALEVSKA,* O. TRAJKOVSKA and N. TALEVA*

Medical Center-Bitola, Medical Center Kavadarci,

MacedoniaBackground and methods Although ultrasound therapy isused to treat calci®c tendinitis of the shoulder, its ef®cacyhas not been rigorously evaluated. We conducted arandomized, double-blind comparison of ultrasonographyand sham insinuation in patients with symptomatic calci®ctendentious veri®ed by radiography. Patients were assignedto receive 24 15-minute sessions of either pulsed ultra-sound (frequency, 0.89 MHz, intensity, 2.5 W cm)1

pulsed mode, 1:4) or an indistinguishable sham treatmentto the area over the calci®cation. The ®rst 15 treatmentswere given daily (5 times week)1), and the remainder weregiven 3 times week)1 for 3 weeks. Randomization wasconducted according to shoulders rather than patients, so apatient with bilateral tendencies might receive either or

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both therapies. Results We enrolled 63 consecutive pa-tients (70 shoulders). Fifty-four patients (61 shoulders)completed the study. There were 32 shoulders in theultrasound-treatment group and 29 in the sham-treatmentgroup. After 6 weeks of treatments, calcium deposits thatresolved in the six shoulders (19%) in the ultrasound-treatment group and decreased by at least 50% in nineshoulders (28%), as compared with respective values ofzero and three (10%) in the sham-treatment group(P � 0.003). At the 9-month follow-up visit, calciumdeposits had resolved in 13 shoulders (42%) in theultrasound treatment group and improved in seven shoul-ders (23%), as compared with respective values of two(8%) and three (12%) in the sham-treatment group(r � 0.002). At the end of the treatment, patients whohad received ultrasound treatment had greater decrease inpain and greater improvement in the quality of life thanthose who had received sham treatment; at 9 months, thedifferences between the groups were no longer signi®cant.Conclusions In patients with symptomatic calci®c tenden-tis of the shoulder, ultrasound treatment helps resolvecalci®cations and is associated with short-term clinicalimprovement.

45Follow-up on periarticular aneurysm formationhaemophiliaA. C. ZIJL, G. ROOSENDAAL,

A. C. VAN RINSUM, D. B. F. SARIS,

E. P. MAUSER-BUNSCHOTEN,

H. M. VAN DEN BERG and A. J. VERBOUT

Van Creveldkliniek and Department of Orthopaedic Sur-

gery, University Medical Center Utrecht, PO Box 85500,

3508 GA, Utrecht, the NetherlandsThe detection of periarticular aneurysms in patients withsevere haemophilia has been reported by us previously(Lancet 1997; 349: 766±68). These aneurysms could be thecause of periarticular haemorrhage after total knee arthro-plasty (TKA). This resulted in standardized angiography todiagnose and, if possible, treat periarticular aneurysmsperioperatively. Factor VIII/IX activity was supplementedroutinely before TKA. Preoperative angiograms were taken1 day prior to or on the day of surgery. Postoperativeangiograms were performed in case of bleeding, includ-ing in patients whose preoperative angiogram had notrevealed pathology. No major complications were seenand all angiographies were performed without the use ofextra clotting factor. Since 1992 we have performed 43angiographies in 54 TKAs, of which 32 were preoper-ative and 11 postoperative. A total of 14 (33%), ofwhich three (9%) were preoperative, and 11 (100%)postoperative angiograms showed a periarticular aneu-rysm. These malformations could all be treated success-fully by placement of embolization coils through highly

selective catherization. We conclude that perioperativeangiography and, when indicated, embolization, provedsimple and effective in both diagnosis and treatment ofperiarticular aneurysms. Keywords joint arthroplasty/angiography/periarticular aneurysm.

46Haemophilic arthropathy council: a big challengefor a developing societyB. ZULFIKAR,*  M. CAKMAK,*àY. BUYUKPINARBASILI,* O. TASER,àF. BEZGAL,* and N. TOKER*

*The Hemophilia Society of Turkey;  University

of Istanbul Oncology Institute; àIstanbul

Medical School Department of Orthopaedics,

Istanbul, TurkeyThe most important problem of haemophiliacs ofdeveloping countries are recurrent haemarthrosis, insuf-®cient treatment, lack of an expert team and treatmentcentres with resultant chronic synovitis, ®brosis, arthro-sis and disabled people. 102/391 haemophilic patientswho were registered with the Hemophilia Society ofTurkey and had joint problems were assessed periodi-cally 275 times during May 1997 and December 1999.Each patient was evaluated at the haemophilia council,consisting of orthopaedic surgeons, haematologist, phys-ical therapists, paediatricians and haemophilia nurses.This was the ®rst of its kind in our country. 32 cases in1997, 75 cases in 1998 and 168 cases in 1999 wereevaluated, 43 cases were discussed once, and 59 caseswere discussed 2±12 times in the council. Of the 99haemophilia A (52 severe, 37 moderate and eight mild)and four haemophilia B (all severe) and one factor XIIIde®ciency, median age were 16 (range 1±64) years. 31cases were less than 10 years of age, 37 cases werebetween 11 and 20 years, 27 cases were between 21and 40 years and seven cases were more than 40 years.The knee was effected in 66 (65%) the ankle in 26(25%) the elbow in 23 (23%) the hip in 15 (15%) theshoulder in four (4%) of cases. Mean haemorthrosisoccurrence per month was 1.9 (range 0±5). As a resultof these ®ndings, it was decided that 80 patients receivephysiotherapy and 46 cases needed orthopaedic surgery.Since then, 80% of all cases needed physiotherapy, 46(45%) of them have been enrolled to the physiotherapyprogramme after brief factor replacement and their jointproblems improved. Ten patients had had arthroscopicsynoviectomy with factor replacement, three casesphonxions, two cases each had debridement and TEP,one case each had high tibial osteotomy and a pelvi-pedalic cast. Two of these interventions were done in1997, eight of them in 1998 and nine of them in 1999.The costs of physiotherapy and orthopaedic surgerywere afforded by SSK (blue colour insurance) in 60% of

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cases, by state insurance company in 20% of cases andby our Society in 20% of cases. The results of thesetherapies are presented elsewhere. The joint problemsbegan early in our haemophiliacs. We believe that byclose management by an expert team including thepsychosocial aspects, establishing haemophilia centres,

supplying factor concentrates and implementing exerciseand physiotherapy, there are going to be fewer problemsin future. The number of damaged joints will decreaseand the problems will be solved more easily. Possiblyprophylaxis will also decrease the morbidities of thesepatients.

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