Total joint arthroplasty in haemophilia

In severely affected haemophilic patients arthropathy is a common problem which can lead to considerable pain and functional deficit. Surgical management, including total joint arthroplasty, can be undertaken if conservative management fails. A search of the literature showed that a number of studies describing the use of total knee arthroplasty (TKA) and total hip arthroplasty (THA) in haemophilia have been published, whereas shoulder, elbow and ankle arthroplasties are confined to case reports. This paper reviews the functional outcome of arthroplasty in the different joints, the postoperative and long-term complications, and the impact of HIV. Although complications are commonly described and the surgery is technically demanding, the results suggest that arthroplasty, particularly of the hip and knee, can be a valuable option in the management of severe haemophilic arthropathy.     

Total joint arthroplasty: the final solution for knee and hip when synovitis could not be controlled

Summary.  In severely affected haemophilic patients knee and hip arthropathy is a common problem, which can leads to considerable pain and functional deficit. Surgical management, including total knee and hip arthroplasty, can be undertaken if conservative management fails. This paper reviews the functional outcome of arthroplasty in the knee and hip, the postoperative and long-term complications, and the impact of HIV. Although complications are commonly described and the surgery is technically demanding, the results of the author of this paper and the review of the literature suggest that arthroplasty of the hip and knee can be a valuable option in the management of severe haemophilic arthropathy.     

The role of physiotherapy after total knee arthroplasty in patients with haemophilia

Summary. With the availability of clotting factor concentrates, advances in surgical techniques, better implant design, and improvements in postoperative management, total knee arthroplasty has become the treatment of choice for haemophilia patients suffering from end‐stage haemophilic knee arthropathy. The success of this surgery is also dependent on close collaborations among the orthopaedic surgeon, the haematologist and the physiotherapist. Although haemophilic patients undergoing this surgery would likely benefit from a targeted rehabilitation programme, its specificities, modalities and limitations have thus far not been extensively studied. Employing the published data of rehabilitation after knee prosthesis in patients with osteoarthritis and haemophilic arthropathy along with clinical experience, the authors present a comprehensive and original review of the role of physiotherapy for patients with haemophilia undergoing knee arthroplasty.     

Total knee arthroplasty for severe haemophilic arthropathy: long-term experience in Taiwan

Summary. A number of articles have investigated the outcomes of total knee arthroplasty (TKA) and causes of prosthetic failure in patients with haemophilic arthropathy. The aims of this retrospective study were to evaluate the clinical and functional outcomes of TKA and causes of prosthetic failure in patients with haemophilic arthropathy. A consecutive series of 35 TKA in 26 patients with haemophilic arthropathy were performed between November 1985 and October 2006 by one experienced surgeon. The mean age at index operation was 34.2 years old (range: 23.4–47 years) and the mean follow‐up duration was 82.2 months (range: 12–218 months). Clinical assessment included range of flexion, range of extension and total range of motion (ROM). Functional evaluation comprised pain score and functional score by Dr. Insall’s Knee Society Clinical Rating System. The average preoperative ROM was 63.2° with flexion contracture 15°, whereas the average postoperative ROM was 79.8° with flexion contracture 5.5°. Improvement of range of flexion was 7.1° (P = 0.16); improvement of range of extension was 9.5° (P < 0.01). Average increase of total ROM was 16.6° (P = 0.02). Pain score by Knee Society was 7.1 points preoperatively and 48 points postoperatively (P < 0.01); functional score by Knee Society was 42 points preoperatively and 77.1 points postoperatively (P < 0.01). Three patients received manipulations because of an inadequate ROM. Three infection episodes were treated with debridement and one of them received arthrodesis after removal of prosthesis. Two patients received revision TKA. One of them was because of loosening of femoral component. The other one received revision TKA because of insert wear. Though improvement in range of flexion is insignificant in haemophilic arthropathy of knee after TKA, it showed significant increase in total ROM after operation, especially in improvement of flexion contracture. It also showed great pain relief and significant functional gain. Under the circumstance of acceptable infection rate and complication, TKA is an effective method to achieve pain relief and gain better function in patients with haemophilic arthropathy of knee. The data of this study confirm those previously published by many authors.     

Haemophilic arthropathy of the elbow

Patients affected by haemophilia commonly have recurrent intra-articular bleeding which leads to progressive destruction and instability of joints. Severe arthropathy of the elbow is complicated by pain, stiffness and loss of function which can be debilitating. Conservative measures such as analgesics, physiotherapy and orthotics are commonly used in the management of these patients. Surgery is considered when conservative measures fail to control the symptoms. Total elbow replacement is now increasingly performed with the advances made in factor replacement therapy and evolution of better implants and techniques of total elbow arthroplasty. The pathogenesis, clinical features and radiological changes of haemophilic arthropathy of the elbow are described in this review article.     

Synovectomy for haemophilic arthropathy: 6–21 years of follow-up in 16 patients

Abstract. Objectives . To assess the efficacy of synovectomy in reducing recurrent haemarthroses and joint pain in patients suffering from haemophilic arthropathy. Moreover, to study whether synovectomy could improve joint mobility or postpone progression of joint destruction. Design . A retrospective study was conducted addressing joint-related symptoms and findings, and the need of orthopaedic surgery during follow-up. Setting . Oslo Sanitetsforenings Rheumatism Hospital/The National Hospital, the National Centre for Orthopaedic Surgery for approximately 180 Norwegians suffering from severe congenital coagulation deficiencies. Subjects . Twelve patients with haemophilia A, two patients with von Willebrand's disease and two patients with factor VII deficiency in which 21 synovectomies (nine knees. six ankles and six elbows) were performed. Main outcome measures . Joint pain, joint mobility, frequency of haemarthroses and radiographic joint scores at follow-up were compared to preoperative figures, and the number of joints in need of total joint replacement or arthrodesis was evaluated. Results . Synovectomy proved efficacious in reducing recurrent haemarthroses and joint pain in all patients. The total range of motion was not improved, but correction of extension deficiency of the knee was accomplished. The progression of arthropathy was not arrested by synovectomy as judged by the radiographic assessment; and in four patients arthroplasty of the knee, and in two patients arthrodesis of the ankle, had been performed. A major wound haemorrhage and subsequent wound rupture was seen in one patient who developed high-titred neutralizing antibodies to factor VIII. Conclusions . Synovectomy for haemophilic arthropathy is safe and efficacious in reducing recurrent haemarthroses and joint pain. Synovectomy should not be performed to improve joint mobility. The progression of the arthropathy is not arrested, and subsequently many patients will be candidates for arthroplasty or arthrodesis.     

What patient,joint and isotope characteristics influence the response to radiosynovectomy in patients with haemophilia?

Summary. The literature describes radiosynovectomy (RS) as a good non‐surgical option for reducing synovial membrane size and thus the number of haemarthrosis episodes. However, there are still many aspects concerning the beneficial effects of RS that have not been quantified. A total of 156 radiosynovectomies (RS) were performed in 104 joints corresponding to 78 haemophiliacs (yttrium‐90, rhenium‐186). The mean patient age was 18 years. In another study involving the same group of patients, the parameters that improved most after RS were pain and haemarthrosis, followed by the World Federation of Hemophilia clinical score, muscle strength and range of movement (ROM). Following RS, improvement was seen to be independent of patient age, haemophilia type and grade, previous haematological treatment, the presence or absence of circulating inhibitor, synovial membrane size, the type of joint (elbow, knee and ankle), previous physical activity or lack of activity, the prior presence or absence of radiographic signs of joint degeneration (arthropathy) or the isotope used. RS is effective in treating haemophilic synovitis and may require 1–3 injections (RS‐1, RS‐2 and RS‐3) spaced 6 months apart. Following RS‐1, the knee had a 3.4‐ and 3.2‐fold greater risk of not improving in terms of pain, compared with the elbow and ankle, respectively. Regarding ROM, lesser improvement was recorded after RS‐1 in cases of severe haemophilia and the ankle. In other words, severe haemophilia implies a 2.1‐fold greater risk of no improvement in ROM compared with mild and moderate haemophilia. In addition, the ankle presented a 6‐fold greater risk of not improving in terms of ROM compared with the elbow and knee. RS affords effective treatment of chronic haemophilic synovitis. RS is effective in all patient groups, independently of the presence of circulating inhibitor antibody, the type of joint involved, the degree of synovial membrane hypertrophy and the presence of radiographic findings of joint degeneration (arthropathy).     

Comprehensive management of chronic pain in haemophilia

Chronic pain, most often due to haemophilic arthropathy, is a pervasive problem in persons with haemophilia (PWH) that adversely impacts function and quality of life. PWH with inhibitors and older PWH may be especially vulnerable to progressive arthropathy and resulting chronic pain. The development of chronic pain from acute pain involves a complex interplay of biological and psychosocial factors that may all contribute to the perpetuation of chronic pain and the outcome of therapy. In the absence of evidence‐based guidelines, an individualized, multimodal approach to chronic pain management is proposed, as it is in individuals without haemophilia who have chronic pain. Pharmacological treatment is central to the management of chronic pain and must be modified based on pain intensity, ongoing response to therapy and the risk for adverse events. Non‐pharmacological interventions, including physiotherapy, complementary treatments and surgical (e.g. orthopaedic) or other invasive procedures, may be integral to chronic pain management in this population. Ongoing psychosocial assessment is critical to identify those factors that may be contributing to the perpetuation of chronic pain or acting as barriers to effective management. Additional study is needed to identify optimal pharmacological treatments for chronic pain in PWH based on the unique pathophysiology of haemophilic arthropathy and on risk profile. Systematic determination of the particular psychosocial factors impacting the experience and management of chronic pain in PWH would likewise add value to the treatment of this pervasive problem.     

Outcomes in total elbow arthroplasty in patients with haemophilia at the University of California,San Francisco: a retrospective review

Summary. Severe haemophilic arthropathy of the elbow is a significant cause of morbidity among adults with haemophilia. However, previous reports of total elbow arthroplasty (TEA) in the haemophilic population have been based on small numbers of patients with relatively short‐term follow‐up. The records of seven total elbow arthroplasties in six adult men with haemophilia at the University of California, San Francisco who underwent TEA over a period of 25 years were retrospectively reviewed. Type of haemophilia, age at time of TEA, HIV infection status, pre‐ and postoperative range‐of‐motion (ROM) scores, complications (including infections), need for subsequent surgical revision and functional outcomes were recorded. Four patients had severe factor VIII deficiency and two patients had severe factor IX deficiency. None of the patients had an inhibitor. The mean age at the time of surgery was 34 years (range, 22–46 years) and the mean follow‐up period was 118 months (range, 37–176 months). One of the six patients had TEA in both elbows. Five of the six patients were infected with HIV. There were no immediate perioperative complications. At a mean of 19.2 months postoperatively, ROM had improved in five of seven TEAs: mean flexion had increased from 110.7° (SD = 15.0) to 120.1° (SD = 14.5), whereas mean preoperative extension increased from ?44.3° (SD = 21.5) to ?36.9° (SD = 27.0). One patient required a revision at 30 months because of ulnar component loosening. This same patient sustained a staph epidermidis infection and ultimate removal of the prosthesis 15 years postoperatively. At a mean of 118 months postoperatively, five of six patients continued to report reduced pain and preserved functionality, with ability to perform normal daily activities. TEA resulted in favourable results in six of seven procedures. Our findings support the viability of TEA for individuals with severe haemophilic arthropathy of the elbow, especially to reduce pain and preserve or restore functionality. Level of evidence .  Level IV.     

Clinical outcomes and patient satisfaction following total joint replacement in haemophilia – 23‐year experience in knees,hips and elbows

Summary. Joint replacement surgery is an available option for end‐stage haemophilic arthropathy. However, reports with long‐term follow‐up are limited. Moreover, patient satisfaction in this setting has never been measured. We share our institution’s experience with joint arthroplasty in haemophilic arthropathy and report on clinical outcomes and patient satisfaction. Between 1985 and 2007, 65 consecutive joints in 45 patients (mean age: 48.6; range: 22–83) underwent joint replacement surgery. Of these, 40 total knee replacements in 31 patients, 18 total hip replacements in 16 patients and 6 total elbow replacements in 3 patients were included. Average follow‐up was 10.7 years (2.4–24.3). Charts were reviewed retrospectively and patients were asked to return for clinical assessment and completion of questionnaires. According to the Knee Society clinical score, postoperative results were good to excellent in 83% of knees. According to the Harris Hip Score, results were good to excellent in 31% of hips. According to the Mayo Elbow Performance Score, results were good to excellent in 83% of elbows. Complication rates are higher than in the non‐haemophilic population, while prosthesis survival rates are lower. Patient satisfaction with pain relief is higher than satisfaction with functional improvement. For 88% of joints, patients are willing to have the same operation again. This study confirms previous knowledge on the role of total joint arthroplasty in haemophilic arthropathy. Despite high complication rates and modest functional outcomes, the operations are valuable for achieving pain relief. In general, patients find that risks are outweighed by the benefits.     

Total knee arthroplasty in HIV-positive haemophilic patients

In the era of total joint replacements in orthopaedics, total knee arthroplasty (TKA) should be indicated in haemophilic patients suffering from severe knee pain and disability. However, the expected high risk of infection and other postoperative complications is a concern. Although the message of this article may seem conservative, it should not be inferred that TKA should still be avoided in a haemophilic patient, but rather that the orthopaedic surgeon should weigh the risks and benefits carefully. Clinical and immunological status should be considered before suggesting a total knee replacement to a haemophilic patient. If TKA is contraindicated, knee arthrodesis is the alternative. However, knee joint debridement can relieve pain for several years and delay the need for TKA.     

Total hip and knee arthroplasty for arthropathy in a hemophiliac

We evaluated the medium-term follow-up results, effectiveness, and suitability of arthroplasty for hemophilic arthropathy. We performed 26 total knee and 9 total hip arthroplasty operations on hemophilic patients between 1988 and 1998 under general anesthesia and appropriate hemostatic management. Postoperative treatment for hemophilic arthropathy is the same as that for osteoarthritis and rheumatoid arthritis. After their operations, patients experienced relief from pain and intra-articular bleeding in affected joints but only marginal improvement in the range of motion. In general, total joint arthroplasty is not indicated for young patients. However, arthropathy can have a severe impact on the active life of patients during their youth. For severe hemophilic arthritis, total knee and hip arthroplasty can lead to a pain-free and improved quality of life. We believe total knee and hip arthroplasty is a good solutions for hemophiliac arthropathy before severe deformity occurs. Although treated relatively young hemophilic patients, age was not considered to be a contraindication.     

Joint distraction results in clinical and structural improvement of haemophilic ankle arthropathy: a series of three cases

Summary. The incidence of haemophilic arthropathy in multiple joints decreased due to treatment with clotting factor. Nowadays patients are enabled to live a rather normal life, resulting in more (sports) trauma-induced arthropathy in isolated joints like the ankle. As surgical treatment options, fusion of the tibiotalar joint and total ankle replacement are available. Both standard treatments have complications and therefore an alternative treatment is desired. In this study, treatment of haemophilic ankle arthropathy with joint distraction was explored. Three patients with haemophilic ankle arthropathy were treated with joint distraction using an Ilizarov external fixator. Clinical outcomes like function, participation and pain were evaluated in retrospect with three different questionnaires: haemophilia activities list, impact on participation and autonomy and the Van Valburg questionnaire. Structural changes were assessed blinded on X-ray by the Pettersson score and ankle images digital analysis (AIDA) and by an MRI score. All three patients were very satisfied with the clinical outcome of the procedure. They reported a clear improvement for self-perceived functional health, participation in society and autonomy and pain. Partial ankle joint mobility was preserved in the three patients. The Pettersson score remained the same in one patient and slightly improved in the two other patients, while joint space width measured by AIDA and the MRI score demonstrated improvement for all three patients after ankle distraction. This study suggests that joint distraction is a promising treatment for individual cases of haemophilic ankle arthropathy, without additional risk of bleedings during treatment.     

Is on‐demand treatment effective in patients with severe haemophilia?

Summary. On‐demand therapy enables stopping haemorrhages rapidly, reducing joint pain and restoring joint mobility, but does not prevent the beginning and subsequent development of haemophilic arthropathy. The main objective of this study was to identify the clinical and orthopaedic status of severe haemophilic patients with bleeding phenotype receiving on‐demand treatment in Spain. We conducted an epidemiological, observational, retrospective study, recruiting 167 patients from 36 centres (92% of them with haemophilia A), median age at enrolment of 35 years. Forty per cent of the patients received a combination of on‐demand and short‐term prophylaxis regimen; the rest was under on‐demand treatment. One hundred and forty‐five patients (87%) reported at least one bleeding episode and 22 (13%) of the biologically severe patients had no bleeding phenotype. Seventy‐one per cent of the studied population presented established haemophilic arthropathy, reaching 80% if we exclude patients without bleeding phenotype. Forty‐three per cent of these patients had one or two joints affected, 28% of them had three or four affected joints, 20% reported five or six affected joints and 9% more than six injured joints. An increase in established haemophilic arthropathy with age was observed. Forty‐six patients underwent orthopaedic surgery at least once. These data show that on‐demand therapy is not effective in preventing the development of haemophilic arthropathy in severe haemophilic population with bleeding phenotype. Therefore, we suggest that the optimal treatment in these patients should be based on prophylaxis. We recommend analysing the reasons for ending prophylaxis, in case its reinstatement should be necessary.     

Celecoxib in the treatment of haemophilic synovitis, target joints, and pain in adults and children with haemophilia

Joint haemorrhage and subsequent haemophilic arthropathy are significant complications of haemophilia. The pathophysiology involves inflammation and angiogenesis. Cyclo-oxygenase 2 (COX-2) inhibitors have anti-inflammatory, anti-angiogenic and analgesic properties yet do not affect platelet function in the manner of traditional non-steroidal anti-inflammatory drugs. We have previously reported the successful use of rofecoxib in the management of haemophilic arthropathy and currently report our use of celecoxib. A retrospective chart review was conducted of all patients with haemophilia A or B seen at the Children's Hospital of Orange County and treated with celecoxib for chronic synovitis, target joint or pain. Efficacy in chronic synovitis and pain reduction was judged subjectively as effective, partially effective or ineffective. Efficacy in resolution of target joints was judged as effective if the target joint resolved or ineffective if it did not resolve. Twelve patients between 9 and 54 years old were treated for a total of 14 courses of celecoxib treatment. All courses were evaluated for safety and efficacy. Celecoxib was used in eight patients with chronic synovitis, three patients with pain, and in one patient with a target joint on three occasions. A response was noted in seven of eight for synovitis, three of three with pain, but no response in the target joint patient. No serious adverse events including hypertension were observed. This is the first study evaluating celecoxib as adjunctive therapy in haemophilia and suggests that celecoxib is safe and effective in treating chronic synovitis and joint pain similar to the previous study of rofecoxib.     

Long-term follow-up after intertrochanteric varus osteotomy for haemophilic arthropathy of the hip

In view of an increasing tendency in prosthetic management of haemophilic arthropathy, we intended to evaluate the efficacy of corrective osteotomy of the hip, specifically taking long-term clinical and radiographic results into consideration. Eleven hips affected by haemarthropathy in nine patients suffering from severe haemophilia A were treated with an intertrochanteric varus osteotomy. The average follow-up period was 15 small middle dot4 years. The preoperative clinical score of the Advisory Committee of the World Federation of Haemophilia was 5 small middle dot3 points (range 4-7) and the Pettersson score was 6 small middle dot4 points (range 2-10). The average WFH score at follow-up had increased to 3 small middle dot6 points. Seven hips showed clinical improvement, two hips showed a postoperative deterioration while a further two hips remained unchanged. The Petterson score increased to an average of 7 small middle dot7 points. Here the radiographs of six patients indicated postoperative deterioration, three remained unaltered and two showed improvement. Our study cannot conclusively answer whether intertrochanteric varus osteotomy for haemophilic arthropathy of the hip is always a feasible alternative to joint arthroplasty. The decision for or against this procedure must be individually assessed and the patient must be thoroughly informed about the advantages and disadvantages of both procedures.     

Exploration of the pathogenesis of haemophilic joint arthropathy: understanding implications for optimal clinical management

Haemophilia is an inherited disorder of clotting factor deficiencies resulting in musculoskeletal bleeding, including haemarthroses, leading to orthopaedic complications. The pathogenesis of haemophilic joint arthropathy continues to be explored and there is evidence to suggest that iron, cytokines, and neo angiogenesis can initiate synovial and early cartilage damage resulting in molecular changes and the perpetuation of a chronic inflammatory state. This joint arthropathy has long term consequences for bone health resulting in chronic pain and quality of life issues in the individual with haemophilia. Haemarthroses can be prevented by the administration of clotting factor concentrates (prophylaxis). However, high costs and the need for venous access devices in younger children continue to complicate recommendations for universal prophylaxis. In patients who fail or refuse prophylaxis, procedures, such as synovectomy and arthroplasty, can provide relief from repeated haemarthroses. The optimal timing of these, however, is not well defined. Prevention of joint arthropathy needs to focus on prevention of haemarthroses through prophylaxis, identifying early joint disease through the optimal use of cost effective imaging modalities and the validation of serological markers of joint arthropathy. Screening for effects on bone health and optimal management of pain to improve quality of life are, likewise, important issues.     

Perioperative clotting factor replacement and infection in total knee arthroplasty

Total knee arthroplasty, or replacement (TKR), is now the most commonly performed surgical procedure performed in adults with haemophilia. It is indicated when end-stage haemophilic arthropathy results in intractable pain and reduced function. In patients with haemophilia, however, there has always been a concern about the high risk of infection, which carries with it potentially catastrophic consequences. The aims of this study were to review the case series of TKR for haemophilic arthropathy published in the medical literature, comparing the published infection rates and the differing clotting factor replacement regimes employed. Nineteen retrospective case series were identified; representing 556 TKR's in 455 patients with an overall infection rate of 7.9%. Case series which maintained a high level of clotting factor replacement throughout the first two postoperative weeks, however, had an infection rate of 2.15%, significantly lower than that of case series using the clotting factor replacement regime currently recommended in the World Federation of Hemophilia guidelines (9.22% P = 0.00545). We believe this study supports the use of a high level clotting factor replacement regime, replacing clotting factors to maintain them at a higher level for a longer period of time than currently recommended in international guidelines.     

Total knee and hip arthroplasty in haemophilic patients

We evaluated the long-term results of three total hip and nine total knee arthroplasties in nine haemophilic patients with disabling pain and end-stage arthropathy. These patients have been followed over a period of 2–12 years (mean 6.9). One patient had a post-operative haematoma, which was evacuated; two patients required manipulation of the knee because of a limited range of motion. There were no infections. At follow-up, all but two patients were completely free of pain; two patients had occasional pain. The functional improvement was impressive with an average increase in the knee score from 37 to 80 points post-operatively. The hip score increased from 36 to 85 points. The range of motion was increased in seven joints, unchanged in two joints and decreased in three. One total hip arthroplasty was revised after 9 years for aseptic loosening. One total knee demonstrated nonprogressive radiographic evidence of aseptic loosening. Median consumption of coagulation factor concentrate in severe haemophiliacs decreased from 47.00 U yr⁻¹ (range 16.000–144.000) before surgery, to 25.000 U yr⁻¹ (range 18.000–132.000) at 2 years after surgery. Conclusion: hip and knee arthroplasty is a valuable option in symptomatic haemophilic patients with disabling arthropathy, in order to obtain pain relief and functional improvement. It is associated with a low rate of complications and may reduce the need for substitution of factor VIII and IX.     

Osteonecrosis of the femoral head in haemophiliacs: not all joint disease is haemophilic arthropathy

Osteonecrosis (ON) is rare while arthropathy is common in persons with haemophilia. A recent case of bilateral ON of the femoral head prompted us to review our experience with hip arthroplasty. We identified nine patients with presumed end-stage haemophilic arthropathy. Four of the nine individuals had radiographic findings suggestive of ON, but without unequivocal microscopic evidence of ON. It is important to recognize ON at an early stage because there are surgical interventions which may prolong the life of the joint and improve quality of life. We suggest that ON should be included in the differential diagnosis of hip pathology in persons with haemophilia presenting with hip pain or dysfunction.