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.     

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.     

Intra-articular hyaluronic acid in the treatment of haemophilic arthropathy of the knee. Clinical, radiological and sonographical assessment

Hyaluronic acid has been used successfully in the treatment of osteoarthritis since 1989. There is no experience in haemophiliacs in larger study groups. In a prospective study, 20 patients (21 knees) with haemophilic arthropathy of the knee received 20 mg hyaluronic acid by intra-articular injection for 5 consecutive weeks. Assessment included clinical scores, X-ray, magnetic resonance imaging (MRI) and biomechanical motion analysis before and 3 months after the first injection. The score of the WFH advisory committee and the Aichroth score for special evaluation of the knee were used. After an average period of 26 months, the World Federation of Hemophilia (WFH) score, the Aichroth score and the visual analogue scale were evaluated again. All patients had pain caused by their arthropathy, nine of them had positive antibodies to human immunodeficiency virus, and 15 had chronic hepatitis C. The mean WFH score was 8.1 points, the Petterson score was 7.3 points and the Aichroth score was 38 points (maximum 55 points). The WFH score decreased to 7.3 points, the Aichroth score improved to 40 points and the subjective assessment measured with a visual analogue scale improved from 5.3 to 3.7 points. No differences from MRI controls were detected. After 3 months, 14 of 20 patients improved subjectively, particularly in longer walking distance, stair-climbing or initial pain. These positive aspects were limited by arthropathy in adjacent joints. After 26 months 10 patients still are benefiting for up to 31 months follow-up. The average WFH score was 7.3 points, the Aichroth score 39 points, the visual analogue scale 4.0 points. We recommend hyaluronic acid for haemophilic arthropathy of the knee when regular conservative therapy has failed and operative treatment is not feasible.     

Viscosupplementation in haemophilic arthropathy: a long-term follow-up study

Haemophilic arthropathy is the most common clinical manifestation of haemophilia, secondary to recurrent haemarthroses and chronic synovitis. Modern bleeding-preventing drugs have limited significantly the incidence of severe arthropathy, and primary approach is usually conservative. Use of intra-articular injections of hyaluronan acid is considered one of the most efficient treatments for early stages of articular degenerative diseases. Assessment of long-term effectiveness of intra-articular administration of hyaluronic acid (HA) in knees, ankles and elbows of patients affected by haemophilic arthropathy was done for 46 patients (10 elbows, 24 knees and 25 ankles) affected by haemophilic arthropathy. They received injections of HA and were evaluated with Visual Analogue Scale, Short Form-36, World Federation of Haemophilia score and Petterson score with a 6-year mean follow-up. Most of the patients showed improvement in pain relief and functional recovery without any complications: only a limited number of patients (8.6%) found poor results, undergoing surgery or other further treatments in the follow-up period for persistent pain or limitation. Viscosupplementation is an effective therapeutic strategy in early stages of haemophilic arthropathy, with no complications and long-term good clinical results.     

Pathogenesis of haemophilic arthropathy

Summary.   The pathogenetic mechanism of haemophilic arthropathy is multifactorial and includes degenerative cartilage-mediated and inflammatory synovium-mediated components. Intra-articular blood first has a direct effect on cartilage, as a result of the iron-catalysed formation of destructive oxygen metabolites (resulting in chondrocyte apoptosis), and subsequently affects the synovium, in addition to haemosiderin-induced synovial triggering. Both processes occur in parallel, and while they influence each other they probably do not depend on each other. This concept resembles degenerative joint damage as found in osteoarthritis as well as inflammatory processes in rheumatoid arthritis. These processes finally result in a fibrotic and destroyed joint.     

Computer‐navigated total knee arthroplasty in haemophilic arthropathy

Total knee arthroplasty (TKA) in end‐stage haemophilic arthropathy is complex and challenging due to the altered bony anatomy, arthrofibrosis and muscle contractures. Computer navigation is especially advocated in patients with deformity or altered anatomy to improve alignment and to assist in ligament balancing. The objective of this study was to evaluate the results of computer‐navigated TKA in haemophilic arthropathy. A consecutive series of computer‐assisted TKA for the end‐stage haemophilic arthropathy between February 2007 and December 2009 were evaluated. A total of 27 TKA were performed in 25 patients. Pre‐ and postoperative full‐length weight‐bearing radiographs were assessed for the axial limb alignment. The orientation of the components was measured on anteroposterior radiographs. Clinically, Knee Society score and Short Form‐36 were evaluated. The mechanical axis of the leg was within a range of ±3° varus/valgus in 92% of the TKA. The coronal alignment of the femoral and tibial components was within a range of ±3 degrees in 96% of the knees. The clinical outcomes were significantly improved after the operation. There were no complications specific to the computer navigation. Computer‐navigated TKA helps in restoring the mechanical axis and improves accuracy of orientation of the components in patients with end‐stage haemophilic arthropathy. Potential benefits in long‐term outcome require further investigation.     

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.     

Value of magnetic resonance imaging for the diagnosis and follow-up of haemophilic arthropathy

An orthopaedic assessment of the joint status of seven severe haemophiliacs (mean age 12.5 y - range 8.9-15.5) on prophylactic treatment (PT) by magnetic resonance imaging (MRI) and physical examination was carried out. Median duration of PT of these patients was 84 months (range 32-107). A locally designed MRI joint score considering seven parameters (1 - joint effusion, 2 - synovial membrane thickening, 3 - haemosiderin deposits, 4 - joint cartilage injury, 5 - subchondral bone erosion, 6 - subchondral bone cysts, 7 - osteonecrosis) was used (maximum score = 13 points). MRI scans were performed in 17 joints (nine ankles, seven knees and one elbow): mean MRI scores for the affected joints was 5.1. A poor consistency between physical examination and MRI findings or the real extent of joint damage was found (Cohen kappa index 0.320). MRI is a precise non-invasive tool for the assessment of early joint cartilage and synovium pathological changes still undetectable by physical examination or conventional X-rays in the haemophilic setting.     

Intra-articular rifamycin in haemophilic arthropathy

Synoviorthesis is the intra-articular injection of chemical or radioactive substances able to produce fibrosis of hypertrophied synovium, which has proved effective in the treatment of chronic haemophilic synovitis. Between September 1999 and October 2001 we treated 28 outpatients (25 with haemophilia A, three with haemophilia B). Our treatment was focused on pain and functional limitation of joints. A schedule was adopted to treat each joint using intra-articular rifamycin once a week, repeated five times. Patients were covered with factor replacement on demand. Oral analgesia was offered as required because of acute but transient painful inflammatory reaction. Their median age was 34 years (range 15-60 years). The indication for synoviorthesis was chronic synovitis characterized by recurrent haemarthroses, persistent pain and limited range of motion (ROM). Thirty-five joints were treated with a total of 169 injections, including six joints (20%) in patients with inhibitors. In five patients two joints were treated in the same session. Thirty procedures were completed: 24 (80%) were considered effective (as excellent or good), while six were considered insufficient (20%). Pain was reduced in 96% of cases and in 70% the ROM was improved. In our experience intra-articular infiltration with rifamycin appears to be effective in reducing joint pain and in improving the ROM. The procedure presents a low risk of bleeding, can be used for patients with inhibitors and multiple joints can be treated without any additional cost.     

Current approaches in haemophilic arthropathy of the hip

Summary.  The hip is considered to be one of the main load bearing joints of the body. In the haemophilic patient joint bleeds can be catastrophic, leading to long-term joint degeneration and accompanying arthritis. In this review we explore the mechanisms of joint destruction, with particular consideration of the anatomy of the hip and how it may influence disease progression. We also review current strategies for treatment including hip replacement in the haemophilic patient and describe our experiences as a unit. Finally we evaluate future prospects in the management of hip disease in haemophilia.     

Outcome of surgical concepts in haemophilic arthropathy of the hindfoot

The ankle is one of the most frequent joint affected by haemophilia, in the second decade of life it can be considered as the most common site for haemophilic arthropathy. The aim of this study is to evaluate the results of 15 surgical interventions of the hindfoot performed on 11 patients. Twenty-seven of 70 male patients suffering from haemophilia were treated for haemophilic arthropathy of the hindfoot. We performed 15 operations on 11 patients with a mean age of 33.5 (range: 10-53) years. The surgical treatment consisted of 15 operations, 12 of which concerned the talocrural joint (six synovectomies, three radiosynoviorthesis, four arthrodeses, one achilles tendon lengthening) and one the talocalcaneonavicular joint (one triplearthrodesis). The arthrodeses of the talocrural joint were undertaken by screw, the triplearthrodesis by clamp fixation. The synovectomies were all late synovectomies and performed by open means. The mean follow-up period was 32.0 (range: 12-84) months. The mean age at surgery was 29.6 (range: 8-51) years. No perioperative complications were registered when adequate replacement therapy was carried out. The consolidation of the arthrodeses was accomplished within 8-12 weeks. All synovectomies stopped or reduced significantly recurrent joint bleeding. With both procedures we achieved pain relief and walking ability improvement. The aim of synovectomy is to reduce bleeding episodes. If synovectomy can halt the progression of the haemophilic arthropathy is controversially discussed, especially when surgery was carried out in the presence of an established arthropathy. If synovectomy fails, the arthrodesis proves to be a good treatment option.     

The outcome of cementless total hip arthroplasty in haemophilic hip arthropathy

Summary.  Total hip arthroplasty (THA) in haemophilic arthropathy is reported to be less successful than in non-haemophilic indications. Although preliminary results are encouraging, the survival and functional outcome of cementless THA in haemophilia are not known. The aim of this study was to analyse mid-term results of cementless THA in haemophilia. Twenty-seven consecutive cementless THAs with 23 patients performed between June 1995 and June 2003 were reviewed. Mean age at time of operation was 36 years and mean follow-up period was 92 months (range, 60–156). Radiographic assessment was done for fixation of components, loosening, osteolysis, wear and bone responses around the implants. The factor requirements, amount of transfusion and complications associated with bleeding were studied. The mean preoperative Harris hip score changed from 57 to 95.9 at the latest follow-up. The survival at mean follow-up was 95.2%. One patient with osteolysis around acetabular cup was re-operated with bone-grafting and change of polyethylene liner. One loose cup was revised with a cemented cup. All other components were deemed stable at the latest follow-up. A standardized management protocol and dedicated team approach comprising of haematologist, physicians, physical therapist, nurses and coordinators is needed for excellent results. The present retrospective study shows that the functional results of cementless THA in haemophilia are satisfactory as it happens in osteoarthritic patients according to the current literature, mainly the younger. Thus, taking into account that the majority of haemophilia patients requiring a THA are relatively young, cementless THA is currently recommended.     

Digital scoring of haemophilic arthropathy using radiographs is feasible

Summary. Radiographs are important tools to evaluate structural changes in many joint diseases. In the case of haemophilic arthropathy (HA), the Pettersson score is widely used. The rising of digital radiography enables evaluation of these changes in a more quantitative and detailed manner, potentially improving diagnosis and follow‐up. The aim of this study was to evaluate whether digital image analysis in the case of HA is feasible, using a presently available method for radiographic changes in knee osteoarthritis (OA), knee image digital analysis (KIDA). Sixty‐two knee radiographs were scored according to Pettersson and with KIDA, each by two independent observers. Inter‐observer variation and correlations between the two scoring methods were determined. The inter‐observer variation was smaller for KIDA than for Pettersson and for KIDA not significantly different from evaluation of OA joints. Good correlations were found for the two methods where comparison of parameters was appropriate. Importantly, for each of the parameters within one point in the ordinal Pettersson score, a large window still existed in the continuous KIDA grading. Digital analysis of radiographs to quantify joint damage in HA is feasible. The use of continuous variables, as used in a digital method such as KIDA has the advantage that it enables objective and much more sensitive detection of small changes than by use of an ordinal analogue method such as the Pettersson score. Based on the present results, it would be worthwhile to adapt the KIDA method for the specific characteristics of HA and to extend the method to elbow and ankle radiographs.     

Ultrasound detects joint damage and bleeding in haemophilic arthropathy: a proposal of a score

Summary. Haemarthrosis triggers haemophilic arthropathy (HA) because bleeding starts synovitis immediately, damages cartilage and leads to loss of function and disability. The aim of our study was to investigate the capacity of ultrasonography (US) in detecting bleeding and joint damage in HA. The joints of 62 patients (pts) with haemophilia A or haemophilia B were consecutively evaluated and scored (score ranging from 0 to 21) for effusion (E), bone remodelling (BR), cartilage damage (CD), synovial hypertrophy (SH), haemosiderin (H), osteophytes (O), haemarthrosis (Hae), erosion (Er) and fibrotic septa (FS) with US. X‐rays [Pettersson Score (PXS)] were performed in 61 patients and clinical evaluation [World Federation Haemophiliac orthopaedic score (WFHO)] was performed in all patients. A total of 20 healthy subjects and 20 patients affected by Rheumatoid Arthritis (RA) were used as controls. Power Doppler US (PDUS) was performed in all patients on the knee, ankle and elbow joints. A total of 83 joints were studied (50 knees; 12 elbows and 21 ankles). US showed effusion in 57 joint, bone remodelling in 62, cartilage damage in 64, synovial hypertrophy in 45, haemosiderin in 39, osteophytes in 30, haemarthrosis in 24, erosion in 5 and fibrotic septa in 3. The X‐rays score showed remodelling in 47 joints, narrowing joint space in 44, displacement/angulation in 39, osteoporosis in 42, subchondral irregularity in 44, subchondral cyst formation in 37, osteophytes in 36 and erosions in 25. The US score in healthy subjects was always ≤5 (range 0 to 4). In haemophiliacs, 34 of 83 joints showed US score ≤5, and 49 US score >5. Joints with US score ≤5 had a low PXS (SRCC = 0.375, P < 0.01) and joints with US score >5 showed a high PXS (SRCC = 0.440, P < 0.01). A significant correlation between US score and PXS for bone remodelling [Spearman’s rho Correlation Coefficient (SRCC) = 0.429, P < 0.01] and for osteophytes (SRCC = 0.308 P < 0.05) was found. The correlation between the US score and number of bleedings in 83 joints was very significant (SRCC = 0.375, P < 0.01). A total of 24 bleeding joints were identified and verified with aspiration of haematic fluid. US may detect bone and cartilage alterations and synovitis. Indeed, PDUS identified bleeding also in asymptomatic joints and was able to show different entity of haemarthrosis. US may be a feasible and reliable tool to evaluate joint modifications in HA.     

Compatible scales for progressive and additive MRI assessments of haemophilic arthropathy

The international MRI expert subgroup of the International Prophylaxis Study Group (IPSG) has developed a consensus for magnetic resonance imaging (MRI) scales for assessment of haemophilic arthropathy. A MRI scoring scheme including a 10 step progressive scale and a 20 step additive scale with identical definitions of mutual steps is presented. Using the progressive scale, effusion/haemarthrosis can correspond to progressive scores of 1, 2, or 3, and synovial hypertrophy and/or haemosiderin deposition to 4, 5, or 6. The progressive score can be 7 or 8 if there are subchondral cysts and/or surface erosions, and it is 9 or 10 if there is loss of cartilage. Using the additive scale, synovial hypertrophy contributes 1-3 points to the additive score and haemosiderin deposition contributes 1 point. For osteochondral changes, 16 statements are evaluated as to whether they are true or false, and each true statement contributes 1 point to the additive score. The use of these two compatible scales for progressive and additive MRI assessments can facilitate international comparison of data and enhance the accumulation of experience on MRI scoring of haemophilic arthropathy.