Eleven total knee replacements were performed in eight patients with severe haemophilia A and the patients were followed up for two to eight years. All the patients had disabling haemophilic arthropathy of one or both knees, which had not responded to conservative treatment. Postoperative complications occurred in 10 knees, including nose bleeding, haemarthrosis, anaphylactic reactions, urinary tract infection with haematuria, recurrent phlebitis at infusion sites, and fever for a few days. There were no wound infections. The outcome, as determined by a standard scoring system, was rated as excellent or good in nine knees, fair in one and poor in one. Nevertheless, all patients were free of pain and all but one returned to full-time or part-time employment. Total knee arthroplasty appears to be a satisfactory procedure in the treatment of disabling haemophilic arthropathy of the knee

Among 41 adult haemophiliacs 15 suffered from shoulder symptoms. We examined 12 patients by radiography and ultrasound. Four had bilateral symptoms making a total of 16 symptomatic shoulders. Of these, 10 had abnormal ultrasound scans with eight having evidence of rotator cuff tears. Evidence of bicipital tendonitis was found in two. Pain with loss of range of movement and a positive impingement sign was the most reliable clinical indicator of a cuff tear. Joint incongruity and superior migration of the humeral head were the best radiographic indicators. Rotator cuff tears are a common component of haemophilic arthropathy of the shoulder

Patients with severe haemophilia have a tendency towards recurrent haemarthroses resulting in chronic synovitis and leading to end stage haemophilic arthropathy. From 1997 to 2001 five patients underwent sequential bilateral total knee replacement. We compared these patients with 13 haemophilic patients undergoing primary unilateral total knee replacement. One senior surgeon performed all surgery using an identical prosthesis under similar surgical and haematological conditions. We reviewed information regarding pre-operative medical condition, antibiotic prophylaxis, blood replacement requirement and tourniquet time were all recorded. The rate of post-operative complications and economic evaluation between the two cohorts was calculated. Functional results were assessed using the Hospital for Special Surgery knee scoring system both pre and postoperatively. We have shown that complication rates following bilateral and unilateral total knee replacements are comparable and that there are no differences in the functional outcomes or complication rates between the two groups. Furthermore, we found that bilateral procedures were advantageous with respect to total rehabilitation times, length of in-patient stay clotting factor usage and cost efficiency

Specimens of tissue from haemophilic synovium and articular cartilage were collected from 39 patients during reconstructive surgery. They were studied by histochemistry, electron microscopy and microprobe analysis. The detailed findings are presented and discussed. It is suggested that haemophilic arthropathy is the result of a number of mechanisms affecting the synovial lining which becomes progressively fibrotic and the hyaline cartilage which disintegrates and is eventually lost. Mechanical and chemical processes cause degeneration of cells but enzymatic processes appear to be primarily responsible for the degradation of the matrix of the articular cartilage

Thirteen elbows affected by severe haemophilic arthropathy and treated by silastic interposition arthroplasty were followed up for at least five years. The severity of pain, the frequency and severity of spontaneous haemorrhage and the range of movement were assessed before operation and at review. All patients were much improved and needed less factor replacement. Three elbows were revised, one for infection and two because of fragmentation of the silastic sheet. They regained good function following revision

We report the results of supramalleolar varus osteotomy on seven ankles (in six patients) for haemophilic arthropathy and secondary valgus deformity. The operation reduced pain and the frequency of intra-articular bleeding while preserving joint function for a mean of nine years. The procedure is an attractive alternative to the more commonly used surgical option of arthrodesis

Background Recurrent haemarthroses in patients with haemophilia commonly affect the ankles. It can result in haemophilic arthropathy and necessitate arthrodesis. The purpose of this report was to present the results of arthroscopic arthrodesis performed for this condition and to highlight some of the potential difficulties encountered as part of the surgical management. The operative technique is also described. Method Between January 2001 and May 2004, eight male patients underwent nine arthroscopic arthrodeses. The mean age was 34 years (range 19–44). The patients were identified retrospectively from a surgical database and the radiographs and outpatient notes reviewed. Patients were also contacted to determine their level of satisfaction with the procedure. Results The mean length of follow-up was 9 months (range 3–18). All patients had united both clinically and radiographically by the time of maximal follow-up and all were satisfied with the result. One patient had minor post-operative bleeding which settled spontaneously. No other significant complications were encountered. Bony cysts were observed in three patients pre-operatively and these all resolved following the attainment of union. Conclusion Arthroscopic arthrodesis of the ankle is a safe and reliable treatment for haemophilic ankle arthropathy. The union rate is high, the complication rate is low, the risk of disease transmission from patient to staff is lessened and the post-operative rehabilitation regime, including allowing immediate weight bearing is less arduous than with traditional open procedures. Factor requirements are lessened and the length of stay is also reduced compared with open arthrodesis. Collaboration with a haematology unit is essential for a good result to be achieved

Six patients with severe haemophilic arthropathy of the elbow have been treated by limited excision arthroplasty using a sheet of silicone rubber as an interposition membrane. There were no complications. The symptoms and signs before and after operation have been assessed using the grading system advocated recently by the World Federation of Haemophilia. In all cases there was relief of pain and increased movement. Moreover, the subsequent incidence of spontaneous haemorrhage into the elbow has been reduced with considerable cost benefit

Purpose of study:. Total elbow replacement (TER) is one of the surgical options for treatment of severe haemophilic arthropathy of the elbow. There are only a few small series described in the literature. The goal of our study was to evaluate our cases and add these data to the known literature. Methods:. A retrospective analysis of a series of 8 TER's (5 patients) was performed. Patients were seen in clinic and evaluated by physical examination, a VAS score for pain, the Mayo Elbow Performance Score (MEPS, 100 points=excellent) and X-rays of the affected elbow. Mean follow-up was 91 (4–236) months, and 103 months when we excluded a patient with only 4 months follow-up. Primary diagnosis was Hemophilia type A in 4 and von Willebrand disease in 1 patient. 2 patients were positive for Hepatitis C and 3 for HIV. Mean age at primary surgery was 46.7 (31.8–63.1) years. Results:. The mean VAS score for pain improved from 7.9 to 0.5 and the mean Mayo Elbow Performance Score from 36 to 93. The arc for flexion/extension improved from 68° to 91° and from to 75° to 153° for pro- and supination. Revision surgery was performed in 3 out of 8 TER's; 1 because of loosening of the humeral component (18.7 years postop), 1 for loosening of the ulnar component (9.9 years postoperatively) and 1 due to a late deep infection, 4 years postoperatively. The last case was treated with an excision arthroplasty, the others with a revision. No replacement currently shows signs of loosening. Conclusion:. This is a small retrospective study, as are the other studies on TER in haemophilic patients. It demonstrated excellent clinical outcome and an acceptable survival rate of a TER, with a mean follow-up of 7.6 years even in this young patient population

Five patients severely affected by haemophilia, in whom six knees showed advanced haemophilic arthropathy, have been studied. The patients presented with painful limitation of movement and increasingly frequent episodes of spontaneous bleeding in the affected knees. These all showed secondary degenerative changes and varus deformity. A Corrective osteotomy of the proximal tibia was performed under full haematological cover without complications. Follow-up ranged from 6 to 36 months, with a mean of 18 months. At follow-up all patients were free of pain and had retained their range of movement. In the three patients with a longer follow-up there was radiological improvement. The most significant feature was that there was almost complete cessation of bleeding episodes into the knee in all patients and no progression of the arthropathy. The cost benefit implications of this corrective procedure are discussed

Haemophilia is an x-linked inherited bleeding disorder which can cause severe arthropathy. We have reviewed the results of 70 primary total knee replacements (TKR) performed in 57 haemophilic patients between 1983 and 2007. The functional results were assessed using the Hospital for Special Surgery (HSS) knee scoring system and Kaplan-Meier survivorship analysis. Six patients died. HSS scores were available for 60 TKRs at a mean follow-up of 9.2 years (2 to 23); 57 (95%) had good or excellent results. Deep infection was recorded in one patient. Kaplan-Meier analysis using infection and aseptic loosening as endpoints showed the survival rate at 20 years to be 94.0%.

A reduction in infection, spontaneous haemarthrosis and improvement in the quality of life were noted to justify surgery in our series of patients with a mean age of 43 (25 to 70). We have found that using the latest techniques of continuous infusion of clotting Factor have significantly helped to reduce the complication rates and have achieved results which match those of the non-haemophilic population undergoing TKR.

The results of primary total knee replacement performed on a group of haemophiliac patients in a single institution by the same surgeon using the same surgical technique and prosthesis are reported.

A total of 35 primary replacements in 30 patients were carried out between 1996 and 2005 and were reviewed retrospectively. The mean age of the patients was 31 years (24 to 42) and the mean follow-up was for 7.5 years (1 to 10). There were 25 patients with haemophilia A and five with haemophilia B. The HIV status and CD4 count were recorded, and Knee Society scores determined. Two patients had inhibitors to the deficient coagulation factor.

There were no early wound infections and only one late deep infection which required a two-stage revision arthroplasty, with a good final result. The incidence of infection in HIV-positive and negative patients was thus similar. One knee in a patient with inhibitor had excessive bleeding due to a pseudoaneurysm which required embolisation. The results were excellent in 27 knees (77%), good in six (17%) and fair in two (6%). The survival rate at 7.5 years taking removal of the prosthesis for loosening or infection as the end-point was 97%.

The mechanical survival of total knee replacements in haemophiliacs is very good. Our results confirm that this is a reproducible procedure in haemophilia, even in HIV-positive patients with a CD4 count > 200 mm³ and those with inhibitors. Our rate of infection was lower than previously reported. This could be due to better control of the HIV status with highly active anti-retroviral therapy and the use of antibiotic-loaded cement.

Purpose

This study was performed to evaluate clinical and radiographic outcomes of Hip Resurfacing Arthroplasty for treatment of haemophilic hip arthropathy.

Purpose: Total knee arthroplasty (TKA) is safe and effective in patients with advanced hemophilic arthropathy. This procedure is extremely successful in pain relief and improving functional status, however the limited restoration in motion due to preexisting soft tissue contractures, remains a concern. The purpose of this study is to report the results of TKA in patients with hemophilia using posterior cruciate ligament (PCL) sacrificing prostheses.

Materials and Methods: The records of 18 consequtive hemophilic arthropathy patients who underwent total knee arthroplasty at our institution between 1998 and 2003 were retrospectively reviewed. The patients were evaluated by International Knee Society (IKS) Scoring system with specific attention to range of motion parameters and functional status at preoperative and postoperative clinical evaluation. Postoperative radiologic evaluation was based on the knee Society roent-genographic evaluation and scoring system.

Results: The average age at operation was 34 years (range, 18–60) with an average follow-up of 51 months (range, 12–74). TKA resulted in an improvement in functional and knee scores, and range of motion parameters (p< 0,01). However, the functional status of the patients improved better than the knee status (p < 0,01). No signs of radiographic loosening were seen in any of the components at the final follow-up evaluation. There were two early hemarthrosis which required open lavage. Four patients had late complications. One patient had a periprosthetic fracture, which was treated with open reduction and internal fixation. Another patient had tibial tubercle avulsion and conservative treatment was choosen. One patient had revision secondary to progression of flexion contracture due to repetitive intraarticular hemorrhage, at the third year (26th month). One patient had deep infection.

Conclusions: Our results demonstrate the excellent results in terms of functional scores and to a lesser extent in knee scores. Evaluation of the subcategories of the knee score demonstrated the limited restoration of motion as the reason for lower success rate in knee scores. Although PCL sacrificing designs allow better motion restoration, futher techniques should be developed to release the extraarticular structures that contribute to the flexion contracture, such as hamstring release.

Purpose: Recurrent hemarthrosis is a common clinical entity in hemophiliacs. They not only interfere with daily life but also trigger hemophilic arthropathy. Synovial hypertrophy has a pivot role in hemarthrosis related joint damage and ablation of the synovium prevents further deterioration. Current treatment strategy is to ablate synovium in the early stages in order to prevent progression of the arthropathy. We report a series of hemophilia patients with advanced arthropathy who had been treated with radionuclide synoviorthesis.

Materials and Methods: The patients who had been treated with radionuclide synoviorthesis for either knee or elbow disease between 2002 and 2004 were included in this study. The inclusion criteria were having > 1 hemarthrosis episode per month in the index joint, chronic synovial hypertrophy, advanced degenerative changes of grade III and IV as defined by Arnold-Hilgartner Classification, F8 inhibitor level < 3. The frequency of hemarthrosis, range of motion of the affected joint, pain level that was evaluated by visual analog scale, was recorded during each follow-up. 90Y was used for the synoviorthesis of the knee, whereas 186Re was used for the elbow cases. Patients were screened for radionuclide leak by using a gamma camera following the injection.

Results: Twenty radionuclide synoviorthesis were performed in 14 knees. The average age was 20 (range, 10–31) with an average follow-up of 17 months (range, 3–29). There were 12 severe hemophilia A and 2 severe hemophilia B patients. The frequency of intaarticular bleeding episodes was significantly reduced at the final follow-up (p< 0,05). Although the range of motion and the pain scores were improved, the change was not statistically significant (p> 0,05). Six patients required repeated treatment because of inadequate response. No radioactive material leakage were detected at the draining lymph nodes.

Conclusions: Our results demonstrate that radionuclide synoviorthesis significantly reduces the number of the bleeding episodes even in the knees presenting with advanced arthritis, and increases the quality of life. Although most patients respond to single injection, some patients may require more than one injection, to achieve a satisfactory clinical outcome.

Introduction

Although total knee arthroplasty (TKA) in end-stage hemophilic arthropathy can reduce the severe joint pain and improve the functional disability, it is technically demanding. In addition, it has generally reported a high rate of complication including periprosthetic joint infection (PJI) and component loosening up to 20%. Although the Knee Society classification system of TKA complication was introduced, the complications of TKA in hemophilic arthropathy has not stratified using this classification system in previous articles to the best of our knowledge.

The purpose of this study was to evaluate the mid-term outcomes and complications of TKA in hemophilic arthropathy.

This is a non controlled experimental prospective4 clinical study that obtained satisfactory results in the chemical Synovectomy with Oxitetracycline Clorhydrate (Emicine), in dfferent joints, demonstrating that it is an Effective method in the treatment of recurrent haemarthrosis in haemophilia.

Materials: 84 patients of whom 77 concluded the complete treatment. 82 joints were injected. The dosage injected was 5 cc of the drug (25 mgms.) in 5 cc of anaesthesia for the knee. 2 cc with 1 cc anaesthesia for the elbow. 1 cc plus 1 cc anaesthesia for the ankle. These injections were performed once weekly with a reinforcement in one month. In case of failure the same can be done repeatedly.

Evaluation and results: Subjective parameters. Pain, range of movement and use of the joint involved. Pain decreased from a mean of 6.5 to 0.9 (Likert scale). Range of movement increased from 5.9 to 9 and joint use increased from 5.9 to 9.2. Objective parameters. Joint diameter and range of movement. Range of movement for flexion and extensiòn improved from 72.2 and 149.2 to 73.7 and 167 respectively for the knees. From 57.3 and 160 to 66.6 and 170 respectively for the shoulder. And from 22.7 and 10.8 to 34 and 18.6 respectively for the ankle.

Conclusion: This procedure has multiple advantages such as immediate therapeutic effect, short period of treatment, easy technique, much les AHF coverage (•0% above coagulation level, less cost than radiocolloid treatment which makes it an perfect alternative of treatment for developing countries and not so underdeveloped.

Besides the experimental demonstration of its action in rabbits is exposed.

Haemophilic arthropathy is characterised by iron deposits in synovial tissues. We investigated the suggestion that iron plays an important role in synovial changes. We obtained synovial tissue from six patients with haemophilia during arthroplasty, finding that brown haemosideritic tissue was often adjacent to tissue with a macroscopically normal appearance in the same joint. Samples from both types of synovial tissue were analysed histologically and biochemically to determine catabolic activity. Macroscopically haemosideritic synovium showed a significantly higher inflammatory activity than that with a normal appearance. Cultures of abnormal synovial tissue gave a significantly enhanced production of IL-1, IL-6 and TNFα compared with cultures of synovial tissue with a normal appearance. In addition, the supernatant fluids from the cultures showed greater catabolic activity from haemosideritic tissue, as determined by the inhibition of the synthesis of articular cartilage matrix. We conclude that in patients with haemophilic arthropathy, local synovial iron deposits are associated with increased catabolic activity

The surgical treatment of a secondary gonarthrosis caused by haemophilic arthropathy needs high quality in soft tissue balancing and accurate alignment in total knee arthroplasty (TKA), which are essential for good long-term results. Due to the early-onset severe arthropathy, haemophiliacs undergo prosthetic surgery at a younger age than general population; therefore to ensure a longer duration of implantation is a major objective to be reached in this setting. As several prospective randomised studies could show, Computer navigation in prosthetic surgery improve precision concerning geometry of axes, resection planes and implant alignment, by the determination of joint centres (actual axis), amount of bone resection, size of prostheses and check of ligament balance. At our department, since January 2006, we implanted four TKA in four patients (age range 45–52 years) affected by severe Haemophilia B; the same surgeon used a single system (Orthopilot system) in all cases. The quality of implantation was studied on postoperative standardized long leg coronal and lateral x-rays. Our results showed that CAS had greater consistency and accuracy in implant placement. Complications influencing the clinical outcome did not occur. In our experience, drawbacks of the navigation systems are the additional costs and the additional operation time between 15 and 25 min. However, one of the most important advantages of using of this technique in patient affected by coagulation disease, according to the international literature, is the reduction of blood loss after operation. A long-term follow-up of these and of larger samples of patients is needed for testing cost/risk-benefit ratio of Orthopilot in prosthetic surgery of haemophiliacs. Therefore navigated total knee arthroplasty in haemophilic arthropathy is not yet a standard procedure, but this technique could become an important surgical choice in management of severe secondary osteoarthritis in the future