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.     

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.     

Cementless total hip arthroplasty for haemophilic arthropathy: follow‐up result of more than 10 years

The improvement of prophylaxis and adequate replacement of clotting factors, the quality of life and natural history of haemophilia have been significantly improved. However, significant functional impairment is inevitable. This study was performed to evaluate over 10 years clinical and radiographic outcomes of cementless total hip arthroplasty (THA) for treatment of haemophilic hip arthropathy. Between 1995 and 2003, 27cases of cementless total hip arthroplasties were performed in 23 haemophilic patients. A total of 21 cases from 17 patients were available for follow‐up analysis over 10 years. Modified Harris hip score, the range of motion of the hip joint, perioperative coagulation factor requirements and complications associated with bleeding were evaluated as part of the clinical assessment. For the radiographic assessment, fixation of component, osteolysis, loosening and other complications were evaluated. Clinically, the mean Harris hip score improved from 57 points before the operation to 94 points at the last follow‐up. The mean flexion contracture was 10° preoperatively and 0.9° at the final follow‐up. The further flexion improved from 68.4° to 90.5° after surgery. The mean monthly requirement of factor VIII reduced from 3150 units before surgery to 1800 units at the time of the last follow‐up. There were three cases of rebleeding. In one case, a progressive haemophilic pseudotumour was found. Reoperation for any reason including revision was performed in three cases. We believe that cementless THA in patient with haemophilic hip arthropathy can bring reliable pain relief and functional improvement for longer than 10 years.     

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.     

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.     

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.     

Charcot arthropathy of the knee. A case-based review

The diagnosis of Charcot arthropathy in the knee is rare. However, there is an increasing number of diabetic patients, and they are living longer due to improvements in treatment. Because neuropathic arthropathy is a late effect of peripheral neuropathy, we can expect an increasing incidence of neuropathic arthropathy. Total knee arthroplasty is the preferred choice of treatment by patients, although it may also be associated with a high incidence of serious complications. This case report presents a young female with diabetes mellitus and Charcot arthropathy of the knee managed by total knee arthroplasty and a literature review. The report encompasses a 5-year follow-up of the patient, from the first contact after knee distortion through diagnosis of Charcot arthropathy and the performance of total knee arthroplasty with outpatient controls. The diagnosis was established on the basis of the rapid destruction of the medial tibial condyle after knee distortion in a patient with neuropathy. Neuropathic arthropathy was confirmed by histology. The patient refused knee fusion and total knee arthroplasty was performed. The patient quickly achieved a painless, stable knee with a 130-degree range of motion. However, a radiolucent line appeared under the tibial component due to premature weight-bearing. The patient was ordered to refrain from weight-bearing for the next 3 months, and the knee was healed. While the management of Charcot arthropathy in the knee remains controversial, total knee arthroplasty is not a contraindication. Early diagnosis, appropriate choice of implant and operative technique, and long-term weight protection are essential.     

Pre-operative flexion contracture determines the functional outcome of haemophilic arthropathy treated with total knee arthroplasty

End-stage haemophiliac arthropathy can be successfully treated with total knee arthroplasty. However, the functional results may not be as good as anticipated and certain pre-op knee characteristics may alter the functional results. The purpose of this study was to evaluate the functional outcome of TKA in haemophilic patients with specific attention to final range of motion and residual flexion contracture of the joint. Twenty-one consecutive patients were retrospectively reviewed. The average age was 34 years with an average follow-up of 5.7 years. Functional status was evaluated with Hospital for Special Surgery Knee Score. Receiving Operating Characteristics analysis was used to determine the threshold of pre-operative flexion contracture degree to avoid residual knee contracture. The range of motion was increased in 16 joints and unchanged in three joints and decreased in the remaining two. Preoperative average range of motion was 37.6°, improved to 57.1° post-operatively. The average knee score increased from 27.85 (15-30) points pre-operatively to 79.42 (12-94) points at the last follow-up. The degree of pre-operative flexion contracture was found to be a good predictor for residual flexion contracture. (Specificity: 85.7%, sensitivity: 100%, cut-off: 27.5°). Total knee replacement improves the quality of life in patients with advanced haemophilic arthropathy. Statistical analysis revealed that pre-op flexion contracture of 27.5° is an important threshold. Patients should be operated before that stage to gain maximum benefit with minimal gait abnormalities.     

Haemophilic; Arthropathy: Assessment of Quality of Life After Total Knee Arthroplasty

The goal of this study was to examine how the known effects of total knee arthroplasty (TKA) on clinical outcome parameters translate into improved quality of life, as measured with validated condition-specific and generic questionnaires (Knee Society Score, WOMAC, SF-12, transition questions), addressing physical, mental and social health. Eleven patients (13 knees) undergoing TKA from 1986 to 1994, with the diagnosis of severe haemophilic arthropathy of the knee, were followed-up over a 4-year period on average. TKA was found to reduce the burden of disease to levels similar to patients with osteoarthritis undergoing hip arthroplasty. Clinical and functional improvement after TKA translated into a substantial and significant increase in quality of life and patient satisfaction, found in objective as well as in patient-perceived measures. However, the physical functional ability did not reach the same level as in the corresponding population not affected by haemophilia, due to residual symptoms and impairment of other joints. Received: 30 September 1998 / Accepted: 17 March 1999     

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.     

Shoulder hemiarthroplasty to manage haemophilic arthropathy: two case studies

Bleeds within the shoulder joint can lead to significant joint destruction and may have associated pain, decreased range of movement (ROM), and impaired function. Conservative management should involve prompt administration of Factor VIII and physiotherapy to address all surrounding structures so as to minimize further damage. If conservative management fails to relieve severe, unremitting shoulder pain in the presence of underlying arthropathy, then arthroplasty may be considered. Outcomes of arthroplasties performed for osteoarthritis and rheumatoid arthritis appear favourable. Few articles, however, have addressed shoulder arthroplasty to manage haemophilic arthropathy, and no reports have documented the rehabilitation process. Three hemiarthroplasties were performed at the Royal Brisbane and Women's Hospital on two men with haemophilia. There were no surgical or postoperative complications. Rehabilitation included intensive physiotherapy. The results in each case revealed a decrease in pain, and an increase in ROM and function postoperatively. These findings suggest that hemiarthroplasty with postoperative physiotherapy may be a feasible option to manage severe, chronic and progressing shoulder pain as a result of haemophilic arthropathy of the shoulder.     

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.     

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.     

Ankle surgery in haemophilia with special emphasis on arthroscopic debridement

Summary. Various conservative options are available to treat ankle problems in haemophilia, which achieve a high rate of satisfactory results in the majority of cases. They are usually employed in combination and include the use of patellar tendon bearing (PTB) orthoses and radiosynoviorthesis. The effect of the PTB orthoses is that it allows the patient to walk without weight bearing of the ankle. Radiosynoviorthesis can be performed in cases of haemophilic arthropathy of the ankle, provided there is at least a minimal degree of synovitis within the joint, with a 75% of satisfactory results. If the conservative options fail, surgical intervention can be indicated. Alternatives to total ankle arthroplasty or arthrodesis include arthroscopic debridement, removal of osteophytes of the anterior aspect of the distal tibia and peri‐articular osteotomies to correct angular, rotational or translational malalignment. Ankle arthrodesis is the standard technique for end‐stage ankle arthropathy. Arthroscopic debridement of the ankle with haemophilic arthropathy can offer temporary relief; however, it is important to stress to patients that the degree of improvement is limited. The complication rate in ankle arthroscopy is high compared with other joints, which is especially true for neurological complications and postoperative infections. Orthopaedic procedures are not very frequent in the haemophilic ankle, because the majority of problems can be solved satisfactorily by conservative means (radiosynoviorthesis and PTB orthosis).     

Aspects of current management: orthopaedic surgery in haemophilia

Summary. If continuous prophylaxis is not feasible due to expense or lack of venous access, we must aggressively treat major haemarthroses (including arthrocentesis) to prevent progression to synovitis, recurrent joint bleeds, and ultimately end‐stage osteoarthritis (haemophilic arthropathy). For the treatment of chronic haemophilic synovitis, radiosynovectomy should always be indicated as the first procedure. If, after three procedures with 6‐month interval, radiosynovectomy fails, an arthroscopic synovectomy must be indicated. Between the second and fourth decades, many haemophilic patients develop joint destruction (arthropathy). At this stage possible treatments include alignment osteotomy, arthroscopic joint debridement, arthrodesis (joint fusion) and total joint arthroplasty. For the hip press‐fit uncemented components (hemispherical acetabulum, flanged femoral stem, metal‐to‐polyethylene) are recommended whilst for the knee a posterior‐stabilized (PS) cemented design is advised. Muscular problems must not be underestimated in haemophilia due to their risk of developing compartment syndromes (which will require surgical decompression) and pseudotumours (which will require surgical removal or percutaneous treatment). Regarding patients with inhibitors, the advent of APCCs and rFVIIa has made major orthopaedic surgery possible, leading to an improved quality of life for haemophilia patients. Concerning local fibrin seal, it is not always necessary to achieve haemostasis in all surgical procedures performed in persons with haemophilia. However, it could be a good adjunct therapy, mainly when a surgical field potentially will bleed more than expected (i.e. patients with inhibitors), and also in some orthopaedic procedures (mainly the surgical removal of pseudotumours).     

Correction of fixed contractures during total knee arthroplasty in haemophiliacs

Encouraging clinical experience has increased the indications for prosthetic knee arthroplasty in haemophiliacs. The medical status, physical disability, age and projected activity levels are the major factors in determining treatment for the patient with unilateral or bilateral haemophilic arthropathy of the knee. In more severely involved knees of patients with haemophilia, flexion contracture is a common deformity. In addition, valgus, external rotation deformity and posterior subluxation of the tibia may exist. The surgeon must have the expertise and experience to correct these deformities sufficiently when performing a total knee arthroplasty. A properly performed soft-tissue release which achieves balance between the medial and lateral ligamentous structures and posterior capsule can provide stability to the knee with a semiconstrained prosthesis. In cases with severe deformity requiring resection of the posterior cruciate ligament a posterior cruciate substituting prosthesis may be necessary.     

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.     

Total knee replacement in haemophilia

We believe that total knee replacement (TKR) is a safe and effective procedure for the management of haemophiliac joint arthropathy; however, the increased risk of infection and non-infective complications remain a cause for concern. TKR in haemophilic patients carries with it an increased risk of post-operative infection in comparison to non-haemophiliac patients. Those patients at particular risk are the HIV-positive haemophiliac patients whose CD4 count is less than 200 cells mm-3. The latest techniques have gone a long way to reducing the complication rate and to achieving results that match those of a similar non-haemophiliac population.     

Preliminary results for the use of knee mega‐endoprosthesis in the treatment of musculoskeletal complications of haemophilia

Complications of haemophilia in the knee region are rare and difficult to treat. Use of surgical treatments such as total knee arthroplasty cannot satisfactorily restore knee function in patients with these complications, which include massive haemophilic pseudotumour, fracture around the knee and haemarthrosis. To analyse the postoperative results of patients suffering from complications of haemophilia and treated with a knee mega‐endoprosthesis, to discuss and compare this type of surgical management with other types of treatments used in similar cases. We retrospectively analyse the surgical results of patients who were treated with a knee mega‐endoprosthesis for complications of haemophilia. Three severe haemophilic arthritic knees, of which two were combined with femoral condylar fractures, were treated in a one‐stage surgery, and another two knees which presented with massive haemophilic pseudotumours and bony defects were treated in a two‐stage operation. Mean age at time of surgery was 28.5 years old and mean follow‐up time was 22.8 months; the mega‐endoprosthesis surgery was successfully performed in four cases and the mean range of motion increased from 29.5° preoperatively to 96.75° postoperatively. The Knee society score function score value increased from 25 to 82.5. One knee was amputated because of uncontrollable recurrent haemorrhage. Roentgenograms did not show any signs of loosening of the prostheses. Use of Mega‐endoprosthesis in the treatment of complications of haemophilia can offer patients suffering from massive pseudotumours with bone defect, severe contracture knee haemophilic arthritis and fractures around a haemophilic knee a viable treatment option.     

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.