(ii) Orthopaedic surgery and haemophilia

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MINI-SYMPOSIUM: SPECIAL CARE PATIENTS

(ii) Orthopaedic surgery and haemophilia

Timothy Matthews, Andrew Carr*

Nuffield Orthopaedic Centre, Nuffield Department of Orthopaedic Surgery, Oxford OX3 7LD, UK

Summary Disorders of haemostasis present considerable challenges to the ortho-paedic surgeon. The x-linked inherited condition of haemophilia is characterised byspontaneous haemarthrosis; if left inadequately treated, recurrent bleeding willoccur, leading to chronic synovitis and ultimately haemophilic arthopathy. Soft-tissue haemorrhage accounts for up to a third of all bleeding episodes and may becomplicated by compartment syndrome or development of a pseudo-tumour.Chronic synovitis unresponsive to factor replacement and physiotherapy is treated

initially by chemical or radioactive synoviorthesis and failure to respond will requiresurgical synovectomy.Surgical procedures are now safer and more common due to the advances in blood

product technology and the multi-disciplinary approach of care from specialisthaemophilia centres.Joint debridement and replacement remain the mainstay of surgical treatment for

end-stage haemophilic arthropathy.& 2004 Elsevier Ltd. All rights reserved.

Introduction

Haemophilia and other disorders of haemostasishave always presented a challenge to the surgeon.However, advances in blood product technology andavailability have revolutionised the peri-operativemanagement of these patients as well as thosesuffering on a day-to-day basis. The need forsurgery and the risks associated with it arebecoming less. Patients with haemophilia can nowanticipate a life-expectancy approaching that ofnormal.

Epidemiology

Haemophilia A and B have an incidence of one in5000 and one in 30,000 males, respectively. Otherinherited coagulation factor deficiencies (V, VII, Xand XI) are rare.

There are currently 6000 registered haemophi-liacs in the UK.

Pathogenesis and genetics

Haemophilia A is due to a deficiency or abnormalityof factor VIII, which functions in the coagulationsystem as a co-factor with activated factor IX toactivate factor X (Fig. 1). It is an x-linked recessivedisorder so that daughters of affected males areobligate carriers, whereas there sons are unaf-fected (Figs. 2–9).

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KEYWORDS

Haemophilia;

Orthopaedic surgery

*Corresponding author. Tel.: þ 44-1865-227377; fax: þ 44-1865-227354.E-mail addresses: [email protected]

(T. Matthews), [email protected] (A. Carr).

0268-0890/$ - see front matter & 2004 Elsevier Ltd. All rights reserved.doi:10.1016/j.cuor.2004.06.008

Current Orthopaedics (2004) 18, 345–356

Haemophilia B, or Christmas Disease is an x-linked deficiency of factor IX and is clinicallyindistinguishable from haemophilia A. Factor IX

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Figure 1 Diagram illustrating the clotting cascade.

Figure 2 Photograph showing an acute on chronichaemarthrosis of the left knee; showing also flexioncontracture of the left knee resulting from recurrenthaemarthroses.

Figure 3 Photograph showing left elbow with chronicarthropathic changes and associated varus deformity.

346 T. Matthews, A. Carr

works at the same point in the coagulation systemas factor VIII, which when activated, activatesfactor X (in the presence of factor VIII).

It is important to note that in up to a third ofpatients their disease is the result of new mutationsand therefore have no positive family history.

Inhibitors

A proportion of patients with haemophilia willdevelop antibodies directed towards their absent

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Figure 4 Radiograph showing advanced haemophilicarthropathy of the right elbow.

Figure 5 Photograph showing a large pseudo-tumour ofthe distal right tibia.

Figure 6 Radiograph showing extensive bone destruc-tion and a pathological fracture of the left femur from alarge pseudo-tumour.

Figure 7 Radiograph showing left femur followingexcision of pseudo-tumour and internal fixation ofassociated pathological fracture.

Orthopaedic surgery and haemophilia 347

coagulation factor. In all, 5–7% of patients withhaemophilia A develop antibodies despite notreatment with coagulation factors and a cumula-tive risk of up to 39%, will develop antibodiesduring their life-time. Less than 1% of patients withhaemophilia B develop antibodies.1

Therefore due to the presence of an antibodythat inhibits the activity of factor VIII or factor IX,

infusion of factor concentrate does not result in anincrease in the factor level. Instead, additionalexposure to factor may stimulate production of theantibody and increase the inhibitor titre, measuredin Bethesda units. As a result surgery poses asignificant risk to these patients.

Porcine factor VIII, activated prothrombin com-plex concentrates and recombinant activated fac-tor VIII have been used successfully to managebleeding during surgery in patients with inhibi-tors.2,3 Despite this it is highly recommended thatsuch patients should be managed only in specialistcentres and are likely to require increased in-patient stays following surgery.

Clinical features

Both factor deficiencies have identical clinicalfeatures.

These depend on the frequency and severity ofbleeds and have been classified in haemophilia Aaccording to the level of factor VIII present asfollows:

Severe o1 iu/dl (or 1% of normal)Spontaneous haemorrhage into jointsand muscleSevere bleeding after minor trauma

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Figure 8 Photograph of end-stage haemophilic arthro-pathy of the left knee.

Figure 9 Radiographs of left knee showing end-stage haemophilic arthropathy changes.


Moderate 1–5 iu/dl (or 1–5% of normal)Bleeding after minor traumaFew other symptoms

Mild 5–40 iu/dl (or 5–40% of normal)Bleeding only after trauma or opera-tive procedures

It is unusual for an infant to have spontaneoushaemarthrosis in the first few months of life.However, the knees are the most commonlyaffected joints as the baby begins to crawl.

For those patients with mild haemophilia recogni-tion of the diagnosis may not come until into theirearly teens. It is usually diagnosed following moresignificant traumatic episodes where there is bleedingor bruising out of proportion to the trauma sustained.

HIV infection

Before routine screening of blood for HIV in 1985,thousands of haemophiliacs received factor con-centrates containing HIV. Many subsequently devel-oped AIDS. During the early 1990s 90% of severehaemophiliacs were HIV positive and approximately1% of all AIDS cases in USA were haemophiliacs.4

The current practice of using heat treated factorconcentrates derived from the blood of sero-negative HIV donors and recombinant factor pro-ducts has virtually eliminated the risk of transmis-sion and as a result the number of haemophiliacswith HIV is reducing significantly.5

Haemarthroses

Frequency

Recurrent spontaneous haemarthrosis is the hall-mark of haemophilia.

The knee is the most commonly affected joint at44%, followed by the elbow 25%, ankle 15%, and theremainder being made up most commonly of theshoulder and hip joint.

Bleeding more than two times in a month or morethan three times in a 2-month period in a particularjoint is termed a ‘target joint’.

Practice pointsHaemarthroses

* Haemarthroses rarely occur in infantsbefore crawling.

* Ankle most commonly effected joint inteenagers.

* Frequency of adult target joint: knee(44%), elbow (25%), ankle (15%).

Acute and chronic features

The patient often experiences an ‘aura’ prior to thejoint becoming hot, swollen, painful and held inflexion. There is usually no external discolourationor bruising around the joint.

Repeated bleeding into the same joint leads tochronic haemophilic arthropathy

Pathology

Repeated haemorrhage causes the synovium tohypertrophy. This is characterised by villousformation, increased vascularity and chronic in-flammatory cellular infiltrate. The type A synovio-cytes absorb a limited amount of the ironpresent, and once their capacity is exceeded,they disintegrate and lysosomes are released.These not only destroy articular cartilage but alsofurther inflame the synovium. Joint fibrosis andankylosis soon follow if the condition remainsuntreated.

Acute management

The goal in the management of haemophilia is therestoration of normal haemostasis with replace-ment of the deficient factor.

One unit of factor VIII per kilogram raises theplasma factor VIII coagulant level by 2%. Thereforeto achieve a 60% level in a 70 kg man with severehaemophilia, 30 iu/kg (or 2100 units) of factor VIIImust be given. The half-life of factor VIII isapproximately 12 h; therefore, replacement shouldbe given at 12 h intervals.

Treatment is aimed firstly at prevention and thentreatment of acute bleeding episodes.

PreventionPrimary prophylaxis is used to prevent bleeding insevere haemophiliacs and factor concentratesare given 2–3 times a week. The aim is to maintainthe level of factor greater than 1% of normal.Lyophilised coagulation factor is stored in thedomestic refrigerator and patients and carers aretaught to inject the factor intravenously. This ishowever expensive, and has been estimatedfor a child being treated in this way until

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skeletal maturity in the region of 2.5–3 million USdollars.

Secondary prophylaxis is aimed at preventing thedevelopment of chronic synovitis and arthropathy.This is the more commonly used preventativemeasure and replacement factor is given over aperiod of 3–4 months following an acute bleed toprevent further bleeds and the subsequent devel-opment of synovitis.

Pooled plasma products are gradually beingreplaced by recombinant produced factor, whichfurther reduces the risk of pathogen (e.g. CJD)transmission.

Practice pointsPrevention

* Primary prophylaxis: Aims to preventspontaneous bleeding, children treated‘from cradle to college’ and cost estimatedat $3 million per patient.

* Secondary: Aims to prevent or resolvesynovitis and treatment over 3–6 months

TreatmentFor most acute bleeds treatment at home willsuffice. The patient is able to inject replacementfactor intravenously for several days to manage theacute episode. In addition elevation of the affectedlimb, rest and application of ice packs will bebeneficial. If treatments are ineffective at homethen admission to hospital for assessment andtreatment may be required.6

AspirationJoint aspiration is accepted practice for non-haemophilia haemarthrosis; however, it remainscontroversial for haemophiliacs. Pain relief andmore rapid rehabilitation are the obvious benefits;however, removal of the tamponade affect and theintroduction of infection have led many centres todiscontinue this practice.

Practice pointsTreatment of acute haemarthrosis

* Restoration of normal haemostasis withfactor replacement.

* Rest, ice and elevation.* Role of aspiration is controversial.* Early physiotherapy with replacement fac-

tor cover.

Soft-tissue haemorrhage

Bleeding within the muscle accounts for 30% of allbleeds in haemophilia and is usually preceded by ahistory of trauma.

The clinical features largely depend on the sizeand location of the bleed together with theextensibility of the surrounding fascia.

Treatment is similar to that of haemarthroses innormalising clotting, and the majority respond toconservative management.

Complications

Compartment syndromeBleeding within a compartment of the forearmor leg has a high risk of developing into acompartment syndrome unless adequatelytreated. Patients with inhibitory factors are parti-cularly at risk of this condition. It is suggestedthat treatment of an established acute compart-ment syndrome should first be managed by normal-isation of clotting prior to consideration offasciotomy. Compartment pressures may in factreduce in response to treatment by clottingfactors, thus avoiding the need for fasciotomy. Ifno response is made then fasciotomy shouldproceed.

Pseudo-tumour (blood cyst)This encapsulated haematoma presents as a pro-gressive painless, enlarging, hard mass over aperiod of 2–3 months. It develops followingrecurrent bleeds into soft tissue or bone. Sub-periosteal bleeding within more proximal bones(femur and pelvis) is the most common source,although it can develop in any bone. Inadequatelytreated soft-tissue bleeds may also develop intopseudo-tumours.

CT/MRI serves as the most useful imagingmodality to determine the extent and nature ofthe pseudo-tumour.7

Untreated pseudo-tumours will erode bone andmay lead to pathological fractures, destroy soft-tissue and may produce neurovascular lesions. Inaddition infection may develop within the pseudo-tumour with rupture leading to septicaemia.8 Thisis particularly the case in patients infected withHIV.

Surgical excision is the treatment of choicealthough it is associated with high morbidity andhas a significant mortality rate (20%). Conservativemanagement with replacement therapy and im-mobilisation may cause some regression, however,will not achieve a cure.9 Additionally divided doses

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of irradiation have been reported to be useful inearly treatment.

Practice pointsSoft-tissue haemorrhage

* Accounts for 30% of all bleeds in haemo-philiacs.

* Treatment aimed at restoration of haemos-tasis.Complications of soft-tissue haemorrhage

* Compartment syndrome: Patients with in-hibitor most at risk

* Pseudo-tumour: Progressively enlarging,encapsulated haematoma, causes boneerosion and soft-tissue destruction

Joint contractures

These occur almost exclusively in the severelyaffected patients with less than 1% of normalclotting factors.

The most commonly affected joints are fixedflexion at the knees and elbows or equinus at theankle.

Contractures result from recurrent intra-articu-lar and intra-muscular bleeding episodes leading tochronic haemophilic arthropathy and muscle fibro-sis, respectively. These pathological processes canin themselves or combined together, lead to jointcontractures. The process is further complicated bydevelopment of peripheral nerve lesions secondaryto the haemorrhage.

Conservative treatment

Treatment is initially aimed at prevention byreducing the frequency and severity of bleeds.Home treatment programmes and recombinanttherapy enable the patient to have earlier andmore adequate treatment in the event of an acutebleed.

Once the acute bleeding phase has been con-trolled measures should be taken to maintainpassive joint movement and prevent musculo-tendinous contracture. This is achieved by earlyphysiotherapy and splintage.

Operative treatment

Treatment of established joint contractures shouldbe aimed at restoring the patient’s lifestyle and

mobility rather than anatomic and radiographicnormality.

Treatment modalities are made up of physiother-apy, orthoses and corrective devices, and surgery.

Serial casting can gradually correct joints; how-ever, hinged braces allow an arc of movement to begradually increased and as lighter, are moreacceptable to patients.

Surgical procedures are divided into soft-tissueand osseous.

Soft-tissue procedures include tendon release,lengthening and tendon transfer. These are usedalone or in combination.

Synovectomy either open or arthroscopic willreduce the incidence of further bleeding; howeverarthrodesis may be required for painful arthropa-thy. Corrective osteotomies can be used when thesoft-tissue procedures are inadequate; however,altered biomechanics can generate secondaryproblems when the normal weight-bearing axis ischanged.

Practice pointsJoint contractures

* Occur almost exclusively in severe haemo-philiacs (o1% of normal factor).

* Knee and elbow most commonly effected.* Non-operative treatment: physiotherapy,

serial casting and hinged braces.* Operative interventions: tendon release/

lengthening and corrective osteotomies.

Management of fractures

There is no evidence to suggest a correlationbetween the incidence of fractures and the severityof haemophilia. Additionally it appears that thepattern and degree of energy involved in producingthe fracture is no different to that in the normalpopulation.10

The principles of fracture management in thehaemophiliac patient are no different to theunaffected individual; however, there are addi-tional considerations to be made.

Fracture healing appears to occur in thesame way as the unaffected patient andtherefore a fracture in a haemophiliac can betreated on its own merits and dealt with eitherconservatively or surgically as the fracture patterndictates.11

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Factor replacement therapy remains the same aswith any other acute episode and should becommenced without delay. Factor replacement willneed to continue through the treatment andrehabilitation period to allow for dressing changesand physiotherapy.

Additional considerations

Early treatment is recommended particularly as therisk of compartment syndrome is increased.

Factor levels should be in excess of 70% duringthe initial manipulation under anaesthesia (MUA)and not drop below 30% in the early stagesparticularly if cast changes are to be made.

The use of pneumatic tourniquets is not contra-indicated, although exsanguination with Eschmarchbandages should be avoided.

Skeletal traction pins may loosen prematurelydue to micro-haemorrhages around the pin sites.10

Peri-operative management

Hospital organisation

The treatment of these patients is done best at acentre with a multi-disciplinary approach to theirsurgical management. There are 26 haemophiliacomprehensive care centres in the UK with othersmaller ‘satellite’ haemophilia centres. These centresprovide specialist experienced staff in the manage-ment of haemophilia, specialist laboratory services,direct links to other specialist clinical departments(e.g. orthopaedics and trauma, infectious diseases,hepatology, paediatrics, dentistry and physiotherapy),24h advice to patients and smaller satellite units andeducation and counselling facilities. Also necessary is asurgeon who has experience operating on persons withclotting disorders and effective communication be-tween all involved professionals.12

Preoperative planning should also include prepara-tion of the operating theatre and communication withother operative staff concerning the case. In particularthe increased risk of transmission of blood-borneinfections, hepatitis B&C and HIV must be emphasised.It is suggested that additional precautions should betaken; wearing of double gloves changed hourly,enclosed hood and face masks, knee length imperme-able gowns and the use of disposable drapes.4

Preoperative care

Patients should be admitted 1–2 days prior to theprocedure for assessment and blood tests. Baseline

information should include factor levels, patient’sweight and the presence or absence of inhibitors.

It may be possible to coordinate proceduresduring the in-patient stay for additional proceduressuch as routine dental work or minor surgery. This isadvantageous to the patient as well as making asubstantial reduction in cost.

Factor replacement

For major surgery 50 units/kg should be givenpreoperatively to increase the factor VIII levels to100%, and then 25 units/kg given every 8–12 h tomaintain a factor VIII level for 5–7 days post-operatively. After that maintenance at 30–50%should be satisfactory until healing is completeand the sutures are removed.

If post-operative physiotherapy is needed infu-sions should continue until therapy complete.

One unit of factor IX concentrate is needed toraise the factor IX level to 1%.

The half-life of factor IX is approximately 24 h,replacement should be given at 12–24 h doses.

Continuous infusions of both factors VIII and IXcan be given and may be more appropriate thanrepeated bolus doses. For example, after an initialbolus dose of 25 units/kg a continuous infusion of 2units/kg/h can be administered.

Regular monitoring of factor levels is importantthroughout factor replacement and appropriateadjustment made as necessary.

Post-operative care

Adequate analgesia is required and may in factnecessitate stronger and more prolonged analgesiathan the normal patient undergoing similar sur-gery.13 It should be noted that intra-muscularinjections of analgesics or other drugs are contra-indicated as is aspirin.

Dressing changes and physiotherapy are bestdone soon after bolus doses of factor concentrateare given to minimise bleeding in the wound.

Complications

InfectionThe haemophilic patient is at increased risk ofdeveloping infection either within the joint orwound following surgery in comparison to theunaffected patient. The incidence is increasedfurther as a result of HIV infection and the resultingimmuno-suppression.14

Overall rates of post-operative infection havebeen reported as high as 26% and led some units to

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doubt the wisdom of undertaking certain electiveprocedures.15

Post-operative haemorrhagePrevention of post-operative bleeding is bestachieved by adequate preoperative planning andadministration of required factors. Patients withinhibitors are most at risk.

Some studies have shown that continuous infu-sion of factors rather than bolus doses, during andfollowing surgery gives a better outcome andreduced level of post-operative bleeding.16

Management should be aimed at normalisation ofclotting prior to any subsequent surgery.

Practice pointsPeri-operative management

* Patients should be managed in a haemo-philia centre.

* Important to identify preoperatively thepresence of inhibitors and blood-borneinfections.

* Factor levels should be increased to 100%prior to surgery.

* Factor levels to be maintained above 30%until wounds have healed and to allowearly physiotherapy.

* Most common complications are infectionand post-operative haemorrhage.

Chronic synovitis

Chronic synovitis is characterised by persistent jointswelling and proliferate synovitis. As the synovitisincreases so does the frequency of bleeding into thatjoint. Unless the vicious cycle of haemarthrosis–synovitis–haemarthrosis is stopped, haemophilicarthropathy will soon develop.

Chronic synovitis is predominantly a clinicaldiagnosis based on chronic swelling of the affectedjoint and an increased bleeding tendency; however,ultrasound scanning and MRI can confirm thedisease process.

Prevention in childhood can be achieved bycontinuous prophylaxis through to skeletal matur-ity, preventing the concentration of factor fromfalling below 1% of normal. In adults a treatmentprogramme of 3–6 months of prophylactic factorreplacement will protect the joint from furtherbleeds after an initial bleed.

Conservative treatment includes an activeprogramme of physiotherapy combined with an

intensive programme of prophylactic treatmentwith the missing factor and, when indicated, theuse of anti-inflammatory agents and orthoses.6,17

Intra-articular and oral steroids have both beenshown to be effective in the treatment of chronicsynovitis.18,19

If conservative treatment is ineffective thenpatients should be considered for synoviorthesis.The term synoviorthesis means to ‘straighten’ or‘stabilise’ the synovium.

Synoviorthesis is considered ahead of surgicalsynovectomy as studies have shown it to be aseffective, with significantly less risk of transmissionof blood-borne infection, safer in patients withinhibitors, complication free and requires no gen-eral anaesthetic. There are two types: radioactiveand chemical.

Radioactive synoviorthesis

Most centres use yttrium-90 (90Y) although somehave used equally effective agents such as radio-active gold-198 (198Au). Essentially a chemicallypure, non-toxic isotope, is introduced into the jointby aseptic injection and emits b-radiation, pene-trating up to a 4mm depth, into the tissue. Thehalf-life of yttrium-90 is 2.7 days; therefore, wholebody radiation is unlikely in the event of leakage.20

The radiation causes fibrosis within the sub-synovial connective tissue of the joint capsule andsynovium and the vascular arcade is also affectedwith vessels becoming obstructed. The articularcartilage remains unaffected.

Following injection the joint is immobilised for aperiod of 2–3 days in a splint, before activemovement is resumed.

Studies indicate that 75–80% of patients under-going radioactive synoviorthesis obtain a goodresult.20,21

If three synoviortheses in a 3-month period areineffective then surgical synovectomy is the treat-ment of choice.

Chemical synoviorthesis

Radioactive agents are less available in developingcountries; therefore, rifampicin has been success-fully used as a synoviorthetic agent.

Despite the fact that studies comparing radio-active agents with rifampicin have indicated that itis as effective, it is not generally used in thedeveloped world.22

This is due to the fact that it is very painful wheninjected and requires more doses to achieve thesame results.

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Surgical synovectomy

Synovectomy is routinely used in patients withother inflammatory arthropathies such as rheuma-toid arthritis. Storti et al.23 first proposed its use inthe treatment of chronic synovitis in the haemo-philiac. It is used to reduce the frequency ofhaemarthroses and therefore slow down articulardestruction.

It is done by means of an open or arthroscopictechnique. In joints that lend themselves well toarthroscopic techniques, a more rapid rehabilitationand improved range of movement has been shownwhen comparing it to the open procedure.24,25

More recently devices such as lasers have beenused in conjunction with arthroscopic techniqueswhich have been proposed to improve localhaemostasis and the speed of recovery.26

Practice pointsTreatment of chronic synovitis

* Physiotherapy with appropriate factor re-placement, anti-inflammatories andorthoses.

* Radioactive synoviorthesis: yttrium-90(90Y).

* Chemical synoviorthesis: rifampicin (indeveloping countries).

* Failure of three synoviortheses in a 3-month period indicates surgical synovect-omy.

Surgical management of chronichaemophiliac arthropathy

Knee

Joint debridementYounger patients with long life-expectancy havebeen shown to benefit from open joint debride-ment. For those who fail this treatment and olderpatients with shorter life-expectancy, joint repla-cement should then be considered.9

OsteotomyRibbans reported high tibial osteotomy for thetreatment of varus deformity of the knee secondaryto haemophilic arthropathy. However, as the diseasepattern is normally pan-articular, rehabilitation is poordespite the normal mechanical axis being restored.27

Proximal tibial valgus osteotomy has been shownto be effective in the treatment of painful genuvarum.28

Joint replacementTotal knee replacement for haemophiliacs began inthe early 1970s with relatively small numbers. Bythe 1980s larger studies had been completed anddemonstrated a large proportion of patientsachieving good and excellent results based onoutcome scores. Most studies also reported moder-ate improvements in the range of motion post-operatively although patients without patella res-urfacing showed significantly worse results.

Post-operative complications are dominated byinfection particularly related to those patientsinfected with HIV. As a result some authors havesuggested that TKR should be reserved for haemo-philiacs without HIV infection. However, Birchet al.29 published a series of 15 patients, eight ofwhom had HIV, with only one developing infection.This was supported by Lofqvist et al.30 and Ungeret al.31, who showed similarly low levels ofinfection.

A retrospective multi-centre study concludedthat TKR on patients with CD4 counts of less than200/ml will result in a significantly greater risk ofpost-operative infection. In addition knee arthro-plasty has a three times greater risk of infectionthan non-arthroplasty procedures.32

Overall knee arthroplasty provides considerablebenefits in terms of pain relief and improvement offunction and flexion contractures. However, thepotential risks and benefits should be carefullyconsidered before surgery is embarked upon.

Hip

Joint replacementHip arthropathy is less common in comparison withthe knee, elbow or ankle. However, in severearthopathy, arthroplasty may need to be consid-ered as there are few other options available.

The relative rarity of hip arthroplasty in thehaemophiliac may be due to the fact that haemar-throsis occurs less frequently in the hip joint incomparison with other major joints.

Of the few studies that report outcome measuresthe majority of patients demonstrated good re-sults.33–36 These results are however taken inperspective with relatively high rates of asepticloosening, Nelson et al.36 reporting a rate of 36%,and post-operative infection associated with HIV,reported at a rate of over 10% by Kelley et al.37

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The high rate of loosening may be due to ayounger patient with higher demands than theusual patient for hip arthroplasty.

Despite the complications, both short and long-term, THA can provide excellent pain relief,improved range of motion and improved function.

Elbow

Despite being the second commonest joint affectedby haemophilic arthropathy considerably few sur-gical interventions or series are reported. This maybe due to adequate function despite advancedarthropathy, particularly in the presence of otheraffected joints predisposing to poor function, e.g.,knee and hip joint.

There does however appear to be a coexistingrelationship between elbow and knee haemophilicarthropathy probably due to a load-sharing issue.38

Radial head excisionRadial head excision is used for predominatelylateral joint disease and has shown satisfactoryoutcome in pain relief and movement. It shouldhowever be avoided in patients where the proximalradial physis is yet to close, thus preventingunequal longitudinal growth deformity of theforearm.39

Joint replacementTotal elbow replacement using hinged prostheseshave shown moderate to good results but with a smallnumbers of cases.40,41 The majority of these caseshave used semi-constrained or unlinked devices.

Ankle

By the fifth decade 80% of patients with severehaemophilia reported that ankle arthropathy washaving a significant effect on their occupational andleisure activities. Well over half of these patients byage 30 will require orthoses or walking aids.

Joint debridementDebridement is the first choice of treatment in theyounger patient and can also be considered in theolder patient yet to approach end-stage arthro-pathy. This may be done arthroscopically togetherwith a synovectomy and can proceed to arthrodesisif required.

ArthrodesisThis is the treatment of choice for end-stagearthropathy of the ankle according to Gambleet al.,42 who showed that it satisfactorily relieves

pain, reduces haemarthroses and can correct a pre-existing equinus contracture.

There is only one reported ankle arthroplasty forhaemophilic arthropathy which relieved pain andreduced bleeding.43

Shoulder

Total shoulder arthroplasty in haemophilic artho-pathy has been reported as small series and casereports. Satisfactory results have been achieved inthese studies although were not significantly betterthan shoulder arthrodesis.43

Genetic counsellingPatients are educated in their disease by thetreating haemophilia centre. This is aimed atproviding information regarding treatment andprognosis.

Investigation into the patient’s family history andblood tests may be requested to delineate themutation further.

With this information the patient and theirrelatives can be counselled into the risk of anyfuture offspring as well as their current offspringhaving haemophilia or being carriers.

Despite this genetic investigation it may not bepossible to trace a mutation and therefore somesiblings may not know if they are carriers or not.

Ante-natal testing by means of chronic villoussampling can diagnose haemophilia in-utero; how-ever, it may not be until umbilical cord blood hasbeen sampled that newly born babies are diag-nosed.

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