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Introduction: People with haemophilia (PWH) not administered primary haematological prophylaxis since childhood, that is, those treated haematologically on demand or not treated at all, often experience the degeneration of the ankles, leading to pain and functional impairment. Aim: To analyse the outcomes and complications of arthroscopic ankle surgery performed on PWH. Methods: For this narrative review of the literature, a search was conducted in PubMed on 2, December 2023, using the keywords "haemophilia", "ankle" and "arthroscopy". Of the 29 articles identified, 15 specifically related to ankle arthroscopy in PWH were selected (inclusion criterion). The remaining articles did not meet this requirement (exclusion criterion) and were therefore eliminated. Results: Arthroscopic procedures (arthroscopic synovectomy, debridement and arthrodesis of the ankle) are increasingly used in the surgical treatment of haemophilic ankle arthropathy. Although arthroscopic ankle surgery offers good outcomes in patients with haemophilia, the procedure is not free of complications, which range from 7.9% for arthroscopic ankle debridement to 13.1% in arthroscopic ankle synovectomy and 17.8% in arthroscopic ankle arthrodesis, respectively. The non‐union rate of arthroscopic ankle arthrodesis is 7.1% (2/28). Conclusion: Although arthroscopic interventions in the haemophilic ankle (synovectomy, debridement, arthrodesis) offer good functional outcomes, they are associated with a non‐negligible rate of complications. Arthroscopic ankle surgery in PWH is major surgery and should be treated as such. [ABSTRACT FROM AUTHOR]

It is important to investigate which factors are associated with the development of arthropathy in people with mild haemophilia (PWMH), in order to prevent it and to limit its effect on function and quality of life. To examine the risk factors associated with arthropathy and to predict its presence and degree of involvement in our PWMH population. This was an observational, cross-sectional cohort study of 85 PWMH under follow-up in our centre. Patient variables (age, body mass index, haemophilia type, genetic mutations, baseline factor levels, age at diagnosis, history of inhibitor, level of physical activity and years of practice, history of haemarthrosis and muscle haematoma) were analysed and related to the presence or absence of arthropathy and the degree of arthropathy (measured with HJHS and HEAD-US). Multivariable models were performed. Some 36.5% of PWMH had arthropathy. The variables that showed an independent association with the presence of arthropathy were patient age (median 42 years) and clotting factor levels (median 10.5 IU/dL). The risk of developing arthropathy increased by 7.9% for each additional year of age and decreased by 7.7% for each 1 IU/dL increase in clotting factor. When arthropathy developed, its degree of involvement might have been influenced by the type of physical activity performed. Patient age and factor levels appear to be the most determinant risk factors associated with the development of arthropathy in mild haemophilia. Monitoring joint health is desirable in this patient population. In patients with arthropathy, physical activities with a low risk of bleeding are the most recommended. • We examined the risk factors associated with arthropathy in 85 patients with mild haemophilia (PWMH). • Patient variables were analysed and related to the presence or absence of arthropathy and the degree of arthropathy (measured with HJHS and HEAD-US). • Some 36.5% of PWMH had arthropathy. Patient age and clotting factor levels showed an independent association with the presence of arthropathy. • The risk of developing arthropathy increased by 7.9% for each year of age and decreased by 7.7% for each 1 IU/dL increase in clotting factor. • Monitoring joint health is desirable in this patient population. [ABSTRACT FROM AUTHOR]

Background: The ability of clinical tools to identify early joint changes is limited. Synovitis is a fundamental finding in understanding haemophilia activity and the response to its therapies; thus, there is a need for sensitive methods to better diagnose subclinical synovitis early. Purpose: Our aim was to compare the frequency with which clinical assessment and ultrasound detected synovial hypertrophy in the most frequently affected joints in patients with haemophilia (elbows, knees and ankles). Methods: We analysed patients with haemophilia older than 16 years who came to the haemophilia centre for routine follow‐up. From the clinical assessment carried out in the consultation, the swelling, pain and history of haemarthrosis were evaluated and compared with the findings of synovial hypertrophy detected by ultrasound. This comparison was also analysed independently for elbows, knees and ankles. Results: A total of 203 joints of 66 patients with haemophilia (mean age 34 years), most of them on secondary, tertiary prophylaxis or on demand treatment, were included. In joints with swelling, pain and history of haemarthrosis, 78% of the joints showed synovial hypertrophy on ultrasound. However, in joints with no swelling, no pain and no history of haemarthrosis, 40% presented subclinical synovial hypertrophy on ultrasound. This percentage was higher in elbows than in knees and ankles. Conclusion: In adults with haemophilia, physical examination and point‐of‐care ultrasound study provide complementary data on their joint disease. However, without ultrasound, the ability to detect subclinical synovitis is considerably reduced, especially in the elbows. [ABSTRACT FROM AUTHOR]

Introduction: Emicizumab‐kywh (ACE910) is a recombinant, humanized, asymmetric bispecific antibody that functions to bring activated FIX (FIXa) and zymogen FX into an appropriate steric conformation to medicate the activation of FX to FXa thereby mimicking the cofactor function of FVIIIa. Aim: The objective of this manuscript was to review the development and potential role for emicizumab in the treatment of patients with haemophilia A with and without inhibitors. Methods: A Cochrane Library and PubMed (MEDLINE) search focusing on emicizumab in haemophilia was conducted. Results: In total, 37 citations were retrieved and serve as the database for the literature reviewed herein. Once‐weekly subcutaneous injection of emicizumab at three dose levels has been shown to be effective as prophylaxis to prevent bleeding in a majority haemophilia A patients with inhibitors to FVIII. Likewise, prevention of bleeding was also observed in more than two thirds of patients without inhibitors to FVIII. One antidrug antibody to emicizumab has been reported in over 600 treated patients, two have developed thromboembolic events and three thrombotic microangiopathy. These thrombotic complications have occurred in conjunction with FVIII‐bypassing agents, and none have been observed following recommendations from the manufacturer regarding concomitant use of bypassing agents. The median annual treated bleeding rates were decreased in patients with as well as those without an inhibitor to FVIII. Conclusion: The principal advantage of emicizumab is subcutaneous administration and effectiveness irrespective of the presence of inhibitors. Emicizumab could conceivably represent a new epoch in the treatment of people with haemophilia A. [ABSTRACT FROM AUTHOR]

Planning and undertaking elective surgery in people with haemophilia (PWH) is most effective with the involvement of a specialist and experienced multidisciplinary team (MDT) at a haemophilia treatment centre. However, despite extensive best practice guidelines for surgery in PWH, there may exist a gap between guidelines and practical application. For this consensus review, an expert multidisciplinary panel comprising surgeons, haematologists, nurses, physiotherapists and a dental expert was assembled to develop practical approaches to implement the principles of multidisciplinary management of elective surgery for PWH. Careful preoperative planning is paramount for successful elective surgery, including dental examinations, physical assessment and prehabilitation, laboratory testing and the development of haemostasis and pain management plans. A coordinator may be appointed from the MDT to ensure that critical tasks are performed and milestones met to enable surgery to proceed. At all stages, the patient and their parent/caregiver, where appropriate, should be consulted to ensure that their expectations and functional goals are realistic and can be achieved. The planning phase should ensure that surgery proceeds without incident, but the surgical team should be ready to handle unanticipated events. Similarly, the broader MDT must be made aware of events in surgery that may require postoperative plans to be changed. Postoperative rehabilitation should begin soon after surgery, with attention paid to management of haemostasis and pain. Surgery in patients with inhibitors requires even more careful preparation and should only be undertaken by an MDT experienced in this area, at a specialized haemophilia treatment centre with a comprehensive care model. [ABSTRACT FROM AUTHOR]

Background There are several scales for evaluating the functional capacity of people with haemophilia (PWH). Objective To demonstrate the value and simplicity of the “Health Assessment Questionnaire Disability Index” (HAQ-DI) for making a functional assessment of PWH in those contexts in which the specific physical assessment scales cannot be used [Functional Independence Score in Haemophilia (FISH) and Haemophilia Activities List (HAL)]. The HAQ-DI is a validated generic self-administered questionnaire that is completed in < 5 min. Methods Data was collected on the physical and functional status of 62 adult PWH having haemophilia. Their average age was 34.7 years. The impairment of the PWH was assessed using the generic self-administrable functionality questionnaire (HAQ-DI) and the following scales: The “World Federation of Haemophilia Physical Examination Score” (WFH-PES) and the Haemophilia Joint Health Score 2.1 (HJHS 2.1). We evaluated the correlation between generic HAQ-DI score and physical assessment scores WFH-PES, HJHS 2.1 (correlation analysis). Results The correlation between WFH-PES and HAQ-DI showed a Spearman's correlation coefficient of r = 0.804 (p < 0.05). The correlation between HJHS 2.1 and HAQ-DI showed a Spearman's correlation coefficient of r = 0.823 (p < 0.05). A positive and fairly strong correlation was found between them. Conclusions The presented associations established the application of HAQ-DI as a PROXY clinical indicator of functional status. HAQ-DI is a valid alternative for assessing functional capacity in adult PWH, especially in cases in which, for some reason, it is not possible to use other tools that are specific for haemophilia. Clinical practice needs a less time demanding assessment tools. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented regarding the literature about joint distraction in haemophilic ankle arthropathy and the comparison of joint distraction among patients with ankle osteoarthritis (OA) without haemophilia.

Introduction Haemophilic arthropathy (HA) is an inevitable consequence of repeated haemarthroses in people with haemophilia, and principally affects their ankles, knees and elbows. It is advisable that haematological treatment be complemented with rehabilitation therapy and surgery. Orthoses are devices that are used within the framework of rehabilitation, in order to change the functional or structural characteristics of the neuromusculoskeletal system. Materials and Methods This article reviews the design, the materials and characteristics of orthoses and the indications for the various orthoses used in HA. Ankle, knee, elbow orthoses, insoles, footwear modifications and orthopedic shoes are discussed. Results Orthoses can control or prevent joint movement, stabilise a specific joint or relieve the load or stress on it. These devices must be prescribed by a physician within the framework of rehabilitation. Recommendations for the proper selection and use of orthoses are highly complex. Discussion To maximise the benefits of this mode of therapy, a profound understanding of anatomy and biomechanics is crucial, as is an understanding of the devices available for the various joints that may be affected by HA (ankles-feet, knees, elbows). Conclusions Orthotic devices can reduce pain and improve quality of life of people with HA, delaying surgery in many cases. [ABSTRACT FROM AUTHOR]

Many paediatric patients with haemophilia who might benefit from radiosynovectomy ( RS) for the control of synovitis do not undergo the procedure as there is controversy in the literature regarding the safety of radiation exposure after two cases of acute lymphocytic leukaemia in children with haemophilia treated with 32P RS were reported. The purpose of this review was to analyse the safety of RS in paediatric patients with haemophilia and provide a risk-benefit assessment, which practitioners could apply to their patients. Children undergoing knee RS receive a radiation dose of approximately 0.74 mSv (90 megabecquerels-MBq) and elbow and ankle RSs a dose of approximately 0.32 mSv (30-40 MBq). The radiation dose from natural sources is approximately 2 mSv and the recommended limit for patients (apart from natural sources) is 1 mSv per year. The lifetime cancer risk increases about 0.5% per 100 mSv per year. Considering the risks and benefits of RS, the authors recommend that clinicians consider this procedure in children with inhibitors or in patients without inhibitors when bleeding is recurrent and persistent despite aggressive factor replacement. [ABSTRACT FROM AUTHOR]

Background Radiosynovectomy (RS) can reduce the number of haemarthroses in chronic haemophilic synovitis. The purpose of this study was to assess the effectiveness of RS in terms of the objective improvement of five parameters (number haemarthroses, articular pain, degree of clinical synovitis, clinical score of the World Federation of Haemophilia (WFH), and radiological score of the WFH. Methods In a 38-year period (1976-2013), five hundred radiosynovectomies were performed in 443 joints of 345 patients with haemophilia diagnosed with chronic synovitis. The mean patient age was 23.7years (range, 6-53). The mean follow-up was 18.5years (range: 6months-38years). The RS was carried out with either yttrium-90 or rhenium-186. We performed 1 to 3 injections (RS-1, RS-2, RS-3), with a 6-month interval between them. Results RS resulted in significant improvement in all the parameters studied, except in the WFH radiologic score that showed no improvement. On average, the number of haemarthroses decreased by 64.1% and articular pain decreased by 69.4%. The degree of synovitis showed a reduction of 31.3%. The WFH clinical score revealed an improvement of 19%. The WFH radiological score showed no improvement. There were four complications (0.9%) of RS. Twenty-eight (6.3%) joints eventually had to be subjected to arthroscopic synovectomy or total knee replacement (TKR). No cancer was observed in this group of patients during the 38-year period. Conclusions Radiosynovectomy (RS) is an effective, safe, minimally invasive, well tolerated procedure in the long-term for the treatment of chronic haemophilic synovitis. Moreover, it is very easy to perform. The knee required more injections than the elbow or the ankle and more severe synoviums required a higher number of RS procedures. [ABSTRACT FROM AUTHOR]

Close collaboration between haematologists, orthopaedic surgeons, rehabilitation physicians, pediatricians, pharmacist, radiologist, pain specialist, psychologists, physiotherapists and nurses is essential for the proper handling of knee problems in haemophiliac patients. The ideal scenario would be for the primary prophylaxis (prevention of the disease) to prevent major degenerative changes that we still see so frequently in this type of patients. Until we achieve this we will continue having to perform multiple invasive orthopaedic procedures, such as articular punctures (joint aspiration) to drain haemarthrosis, radiosynovectomies, arthroscopic synovectomies, tendon lenghtenings, realignment osteotomies and total knee replacements (primary and revision) on the knees of people with haemophilia. As a result of this, we will improve the quality of life of haemophiliac patients with orthopaedic knee problems. The rate of potential complications following knee orthopaedic procedures, especially in patients with inhibitors, must not be underestimated. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 46-year-old patient with severe haemophilia A, without circulating inhibitor, HIV-positive and hepatitis C-positive. The patient suffered severe arthropathy of both knees with intense pain in the left knee for several years. It highlights the severe postoperative haemarthrosis following a total knee replacement in the haemophiliac patient. It also emphasizes the importance of early diagnosis and treatment of the complication through arterial embolization.

Abstract: Arthroscopic synovectomy (AS) of the elbow, while providing similar pain relief to open synovectomy (OS), may place patients at higher risk for recurrence. The primary predictor of outcome is degree of pre-existing degenerative changes within the joint. Regarding haemophilia patients, radiosynovectomy (RS) is the best choice for patients with persistent synovitis of the elbow. In the elbow we recommend a dose of 30–40 megabecquerels (mBq) in children and a dose of 56–74 mBq in adults. If three consecutive RSs with 6months intervals are ineffective, an AS or OS must be indicated. Synovectomy (by any method) significantly reduces bleeding episodes. Although the dose of radiation of RS is minimal, 0.32 millisieverts (mSv) in children, 0.54mSv in adults, and neither articular nor systemic neoplastic changes related to RS have been reported so far, all patients must be given opportunity to consider risk/benefit ratios. Radiation dose due to natural sources is 2mSv per year and the recommended limit for patients (apart from natural sources is 1mSv per year). My current recommendation is to use RS in children older than 12years of age. Therefore, in children younger than 12years of age an AS should be indicated. OS should be reserved for adults requiring radial head excision (due to a severe limitation of pronation-supination) and synovectomy in the same surgical session. [Copyright &y& Elsevier]

Background: Patellar tendinopathy (PT) presents a challenge to orthopaedic surgeons. The purpose of this review is to revise strategies for treatment of PT MATERIALS AND METHODS: A PubMed (MEDLINE) search of the years 2002-2012 was performed using "patellar tendinopathy" and "treatment" as keywords. The twenty-two articles addressing the treatment of PT with a higher level of evidence were selected.Results: Conservative treatment includes therapeutic exercises (eccentric training), extracorporeal shock wave therapy (ESWT), and different injection treatments (platelet-rich plasma, sclerosing polidocanol, steroids, aprotinin, autologous skin-derived tendon-like cells, and bone marrow mononuclear cells). Surgical treatment may be indicated in motivated patients if carefully followed conservative treatment is unsuccessful after more than 3-6 months. Open surgical treatment includes longitudinal splitting of the tendon, excision of abnormal tissue (tendonectomy), resection and drilling of the inferior pole of the patella, closure of the paratenon. Postoperative inmobilisation and aggressive postoperative rehabilitation are also paramount. Arthroscopic techniques include shaving of the dorsal side of the proximal tendon, removal of the hypertrophic synovitis around the inferior patellar pole with a bipolar cautery system, and arthroscopic tendon debridement with excision of the distal pole of the patella.Conclusion: Physical training, and particularly eccentric training, appears to be the treatment of choice. The literature does not clarify which surgical technique is more effective in recalcitrant cases. Therefore, both open surgical techniques and arthroscopic techniques can be used. [ABSTRACT FROM AUTHOR]

Background: Patellar tendinopathy (PT) presents a challenge to orthopaedic surgeons. The purpose of this review is to revise strategies for treatment of PT Materials and methods: A PubMed (MEDLINE) search of the years 2002-2012 was performed using "patellar tendinopathy" and "treatment" as keywords. The twenty-two articles addressing the treatment of PT with a higher level of evidence were selected. Results: Conservative treatment includes therapeutic exercises (eccentric training), extracorporeal shock wave therapy (ESWT), and different injection treatments (platelet-rich plasma, sclerosing polidocanol, steroids, aprotinin, autologous skin-derived tendon-like cells, and bone marrow mononuclear cells). Surgical treatment may be indicated in motivated patients if carefully followed conservative treatment is unsuccessful after more than 3-6 months. Open surgical treatment includes longitudinal splitting of the tendon, excision of abnormal tissue (tendonectomy), resection and drilling of the inferior pole of the patella, closure of the paratenon. Postoperative inmobilisation and aggressive postoperative rehabilitation are also paramount. Arthroscopic techniques include shaving of the dorsal side of the proximal tendon, removal of the hypertrophic synovitis around the inferior patellar pole with a bipolar cautery system, and arthroscopic tendon debridement with excision of the distal pole of the patella. Conclusion: Physical training, and particularly eccentric training, appears to be the treatment of choice. The literature does not clarify which surgical technique is more effective in recalcitrant cases. Therefore, both open surgical techniques and arthroscopic techniques can be used. [ABSTRACT FROM AUTHOR]

Cartilage therapy for focal articular lesions of the knee has been implemented for more than a decade, and it is becoming increasingly available. What do we know on the healing response of cartilage lesions? What do we know on the treatment of focal cartilage lesions of the knee and the prognostic factors involved? PubMed articles related to articular cartilage regeneration of the knee in clinical studies were searched from January 2006 to November 2012, using the following key words: articular cartilage, regeneration, clinical studies, and knee. A total of 44 reports were found. They showed the following possibilities for the treatment of focal lesions of the articular cartilage of the knee: cartilage regeneration and repair including cartilage reparation with gene-activated matrices, autologous chondrocyte implantation (ACI) and matrix-induced ACI (MACI), microfracture, osteochondral autograft transfer (mosaicplasty), biological approaches (scaffolds, mesenchymal stem cells-MSCs, platelet-rich plasma, growing factors-GF, bone morphogenetic proteins-BMPs, magnetically labeled synovium-derived cells-M-SDCs, and elastic-like polypeptide gels), osteotomies, stem-cell-coated titanium implants, and chondroprotection with pulsed electromagnetic fields. Untreated cartilage lesions on the femoral condyles had a superior healing response compared to those on the tibial plateaus, and in the patellofemoral joint. Clinical outcome regarding the treatment of medial defects is better than that of the lateral defects. Improvement from baseline was better for patients < or = 30 years compared with patients > or = 30 years. ACI, MACI, and mosaicplasty have shown similar results. The results of comparative clinical studies using ACI have shown some superiority over conventional microfracturing in medium or large defects and in long-term durability. Some biological methods such as scaffolds, MSCs, GF, M-SDCs, BMPs, and elastic-like polypeptide gels still need more research. [ABSTRACT FROM AUTHOR]

Due to improvements in the treatment and medical care of haemophilia, the life expectancy of individuals with haemophilia has approached that of the general population. To review the main co-morbidities of the musculoskeletal system in elderly persons with haemophilia, we have performed a review of the literature on the musculoskeletal problems of elderly haemophiliacs. Chronic arthropathy is the main co-morbidity in the ageing person with haemophilia. Age-related orthopaedic co-morbidities include degenerative joint changes, osteoporosis, muscle atrophy or sarcopenia, muscle weakness and disturbance of gait and balance. Increased pain, muscle weakness and atrophy along with an increased risk of falling are key features of advanced haemophilic arthropathy and ageing. An ageing haemophilia population in which arthropathy continues to be the primary co-morbidity is a current challenge for those responsible for their care. Exercise programmes undertaken two to three times per week for at least 12 weeks seem most effective in reducing the impact of age-related changes on the musculoskeletal system. Establishing effective exercise programmes and strategies to identify individuals who would benefit from early surgical intervention together with presurgical physiotherapy prehabilitation is a priority for future research. [ABSTRACT FROM AUTHOR]

. Animal experiments have shown that a number of bleeding disorders may affect wound healing (WH), including haemophilia B, deficiency of factor XIII and abnormalities of fibrinogen. Therefore, normal healing requires adequate haemostatic function for the appropriate time frame (up to 4 weeks in the clean and uncontaminated wound). Many factors may affect WH, including impaired haemostasis, diabetes, poor nutrition, insufficient oxygenation, infection, smoking, alcoholism, old age, stress and obesity. The gold standard for the correct care of surgical wounds in patients with bleeding disorders includes wound dressing and comprehensive standard care (haemostasis, nutritional support, treatment of co-morbidities, offloading, reperfusion therapy and compression). Although complications of surgical wounds healing in patients with bleeding disorders are uncommon, a low level of the deficient factor for an insufficient period of time could cause WH complications such as haematomas, infection, and skin necrosis and dehiscence. Clinical experience and animal experiments appear to indicate that, to get a satisfactory healing of surgical wounds and avoid potential complications of WH, a good level of haemostasis is necessary for 2-3 weeks after surgery. However, many treaters would regard this recommendation at odds with (i.e. more aggressive than) current standards. Unfortunately no additional clinical evidence for this recommendation can be provided. [ABSTRACT FROM AUTHOR]

. Recurrent haemarthrosis is the final cause of haemophilic arthrosic disease in haemophilia patients. Therefore, it is essential to diagnose it early, both clinically and by imaging. In addition, haemophilia patients experience chronic synovitis, joint degeneration, muscle haematoma and pseudotumours. The objective of this article is to highlight the value of ultrasounds in the diagnosis and control of the evolution of musculo-skeletal problems in haemophilia patients. To this end, we have performed a literature search in the PubMed, Web of Science® (WOS) and SciVerse bases, using the following keywords: hemophilia or haemophilia and ultrasonography (US), ultrasound, echography and sonography. The search was limited to studies published in English between the years 1991 and 2011, finding a total of 221 references. After reviewing the title or abstract for evidence of the use of US for the diagnosis of musculo-skeletal lesions in haemophilia, we selected 24 of these references. We added data collected from our experience to the most important data found in the references. Our main conclusion is that US is highly valuable for the diagnosis of musculo-skeletal diseases in haemophilia. It is a fast, effective, safe, available, comparative, real-time technique that can help us confirm the clinical examination. It is particularly important in acute haemarthrosis, as it can be used to objectively identify the presence of blood in the joints, measure its size, pinpoint its location, assess its evolution and confirm its complete disappearance. [ABSTRACT FROM AUTHOR]

In this article, the knee instruments and rating scales that are designed to measure outcomes are revised. Although the International Knee Documentation Committee Subjective Knee Form can be used as a general knee measure, no instrument is currently universally applicable across the spectrum of knee disorders and patient groups. Clinicians and researchers looking to use a patient-based score for measurement of outcomes must consider the specific patient population in which it has been evaluated. The Western Ontario and McMaster Universities Osteoarthritis Index is recommended for the evaluation of treatment effect in persons with osteoarthritis (OA). This is a generic health status questionnaire that contains 36 items, is widely used, and easy to complete. The Knee Injury and Osteoarthritis Outcome Score (KOOS) questionnaire evaluates the functional status and quality of life (QoL) of patients with any type of knee injury who are at increased risk of developing OA; i.e., patients with anterior cruciate ligament (ACL) injury, meniscus injury, or chondral injury. So far, the KOOS questionnaire has been validated for several orthopedic procedures such as total knee arthroplasty, ACL reconstruction, and meniscectomy. The utilization of QoL questionnaires is crucial to the adequate assessment of a number of orthopedic procedures of the knee. The questionnaires are generally well accepted by the patients and open up new perspectives in the analysis of prognostic factors for optimal QoL of patients undergoing knee surgery. [ABSTRACT FROM AUTHOR]

In this article, the knee instruments and rating scales that are designed to measure outcomes are revised. Although the International Knee Documentation Committee Subjective Knee Form can be used as a general knee measure, no instrument is currently universally applicable across the spectrum of knee disorders and patient groups. Clinicians and researchers looking to use a patient-based score for measurement of outcomes must consider the specific patient population in which it has been evaluated. The Western Ontario and McMaster Universities Osteoarthritis Index is recommended for the evaluation of treatment effect in persons with osteoarthritis (OA). This is a generic health status questionnaire that contains 36 items, is widely used, and easy to complete. The Knee Injury and Osteoarthritis Outcome Score (KOOS) questionnaire evaluates the functional status and quality of life (QoL) of patients with any type of knee injury who are at increased risk of developing OA; i.e., patients with anterior cruciate ligament (ACL) injury, meniscus injury, or chondral injury. So far, the KOOS questionnaire has been validated for several orthopedic procedures such as total knee arthroplasty, ACL reconstruction, and meniscectomy. The utilization of QoL questionnaires is crucial to the adequate assessment of a number of orthopedic procedures of the knee. The questionnaires are generally well accepted by the patients and open up new perspectives in the analysis of prognostic factors for optimal QoL of patients undergoing knee surgery. [ABSTRACT FROM AUTHOR]

Background: Chronic ankle instability (CAI) is a very common injury but still remains an area of debate. Questions: This review aims to define the current diagnosis and treatment of persons with CAI. Methods: A PubMed (MEDLINE) search of the years 2010 and 2011 (1 January 2010 to 15 July 2011) was performed using three keywords: ankle and chronic and instability. The number of articles in English language that was identified was 57. Results: Low reliability has been found in effectively testing CAI in a clinical setting. No single force-plate measure is very effective in predicting if an individual had CAI or not. Availability of a nonradiographic device (ankle arthrometer) to measure ankle instability could improve diagnostic accuracy and facilitate decision making in patients with CAI. The sensitivity of magnetic resonance imaging (MRI) may not be adequate to detect lesions in these patients before surgery. In a symptomatic patient, negative results on MRI must be viewed with caution and an arthroscopy may still be required for a definitive diagnosis and treatment. Conservative treatment (rehabilitation, taping, brace) may reduce the occurrence of recurrent ankle sprains and may be effective in managing CAI. In patients with CAI, strengthening of the muscles around the ankle with well-planned proprioceptive exercises helped the patients return to normal living and sports activities, and prevents unnecessary surgery, especially in cases with functional instability. There is no consensus regarding optimum surgical treatment for CAI, but all of them often have good results. Conclusions: Conservative treatment of patients with CAI must be the first-line therapy. Surgical treatment must be indicated only when conservative treatment fails. [ABSTRACT FROM AUTHOR]

Hemophilia is an inherited disorder of clotting factor deficiencies resulting in musculoskeletal bleeding, including hemarthroses, leading to musculoskeletal complications. The articular problems of hemophiliac patients begin in infancy. These include: recurrent hemarthroses, chronic synovitis, flexion deformities, hypertrophy of the growth epiphyses, damage to the articular cartilage, and hemophilic arthropathy. The most commonly affected joints are the ankle, the knee, and the elbow. Hematologic prophylactic treatment from ages 2 to 18 years could avoid the development of hemophilic arthropathy if the concentration of the patient's deficient factor is prevented from falling below 1% of normal. Hemarthroses 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. Prevention of joint arthropathy needs to focus on prevention of hemarthroses 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. Major hemarthrosis and chronic hemophilic synovitis should be treated aggressively to prevent hemophilic arthropathy. [ABSTRACT FROM AUTHOR]

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). [ABSTRACT FROM AUTHOR]

Deep infections are among the most severe complications in total knee arthroplasty; infection rates described in recent articles range between 1 and 2%. The most important infection risk prevention measures are skin preparation, antibiotic prophylaxis, and screening and management for methicillin-resistant Staphylococcus aureus (MRSA). Debridement and prosthesis preservation is indicated for acute postoperative infections or for acute hematogenous disseminations over well performing prostheses. The following criteria should be met for the result to be favorable: short delay from onset of symptoms (less than 2-3 weeks), Gram-positive germs, absence of prolonged wound drainage and presence of a stable implant. Two-stage replacement is currently the gold standard for treating chronic prosthetic knee infections. The survival rate free of implant removal for reinfection with this technique stands at 93% at 5 years and 85% at 10 years. Moreover, initial recovery figures are preserved with the passage of time. Survivorship free of implant removal for any reason is 90% at 5 years and 77% at 10 years. Articulated spacers seem to be better than static ones. Polymicrobial and Gram-negative infections can be controlled in late knee arthroplasty infections. On the other hand, infections by MRSA are less likely to be controlled by the standard regimens. Preoperative prophylactic antibiotics do not affect the results of intraoperative cultures and therefore they should not be withheld before surgery for an infected TKA when an organism has been identified on aspiration preoperatively, and there has been no recent (4 weeks) antimicrobial therapy. [ABSTRACT FROM AUTHOR]

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). [ABSTRACT FROM AUTHOR]

We report on a series of 92 surgical procedures (90 patients). It includes 35 orthopaedic procedures (33 patients) and 57 non-orthopaedic procedures (57 patients). The orthopaedic procedures include 27 radiosynovectomies (minor surgery) and eight major orthopaedic procedures. The non-orthopaedic procedures include 52 minor interventions and five major procedures. The average age of patients was 34 years (range: 8–56), and the average follow-up time was 3 years (range: 1–6). Of the 92 surgical procedures, 42 were performed with activated prothrombin complex concentrates [factor eight inhibitor bypassing agent (FEIBA)] and 47 with recombinant-activated factor VIIa (rFVIIa; NovoSeven, Novo Nordisk, Bagsvaerd, Denmark). Regarding FEIBA treatment in minor surgery, the initial dose was 100 IU kg−1. After 6 h, we continued with 50 IU kg−1 every 12 h for at least 4 days (radiosynovectomies). In minor non-orthopaedic procedures, the dose was continued until day 14. In patients who underwent surgery with the haemostatic control achieved by means of rFVIIa, the initial dose of rFVIIa in minor procedures (both orthopaedic and non-orthopaedic) was 90–120 μg kg−1. In postoperative days 1–5, the dose was 2–4 × 90–120 μg kg−1 q3–6 h for 24 h. In major procedures (both orthopaedic and non-orthopaedic), the dose was 120 μg kg−1 pre-operatively, 120 μg kg−1 q 3 h day 2/day 3–5, and then 90–120 μg kg−1 q 6 h until day 14. There were 87 good results, four fair results and one poor result. Our study has shown that haemophilic patients with inhibitors requiring surgery can undergo orthopaedic and non-orthopaedic procedures with a high expectation of success. In other words, surgery (orthopaedic and non-orthopaedic) is now possible in haemophilia patients with inhibitors, leading to an improved quality of life for these patients. [ABSTRACT FROM AUTHOR]

We present the case of two patients aged 12 years and 7 years who were referred to our hospital for factor VII deficiency inherited in an autosomal recessive pattern, who had suffered from previous multiple joint haemarthroses. They presented with fine motor symptoms and difficulty in walking. During physical examination we observed neurological symptoms (general hypotonia, muscular hypotrophy, exaggerated tendon reflexes, pes cavus, and spastic gait). Given that the symptoms were not justified by the deficiency of coagulation factor VII and on suspicion of hereditary spastic paraplegia (HSP), tests were carried out. Findings from the tests confirmed the diagnosis of HSP (axonal degeneration of the central motor pathway and pyramidal tracts), further complicated by mixed neuropathy. This disease was also inherited in an autosomal recessive pattern with no direct genetic association with factor VII deficiency. Neurological symptoms had gone unnoticed due to a history of multiple joint haemarthrosis; musculoskeletal examination led to a satisfactory differential diagnosis. Haematological prophylaxis was commenced with rFVIIa at 30 mcg/kg, three days per week. A rehabilitation programme was prescribed so that the patient could remain independent for as long as possible, based on orthosis, physiotherapy, and occupational therapy. Response to treatment is currently satisfactory and no new bleeding has presented. As far as we are aware, the coexistence of these two diseases (factor VII deficiency and HSP) has not been previously reported in the literature. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented regarding the use of a multimodal blood loss prevention approach (MBLPA) including intraarticular tranexamic acid (TXA) in primary total knee arthroplasty (TKA) for patients with haemophilia A.

The incidence of haemarthrosis as a result of a spontaneous periarticular aneurysm in haemophilia is very low. In these circumstances, angiographic embolization might be considered as a promising therapeutic and coagulation factor saving option in joint bleeds not responding to replacement of coagulation factor to normal levels. Moreover, embolization should be considered as a possible treatment for postoperative pseudoaneurysms complicating total knee arthroplasty in haemophilia. However, the pathological process of aneurysmal bleeding and clotting factor replacement is entirely different. While embolization is the treatment of choice for some periarticular complications that may occur, it is by no means a panacea for all resistant periarticular bleeds in haemophilia or for postoperative bleeding which usually settles with clotting factor replacement. Another use of arterial embolization is for the treatment of haemophilic tumours of the pelvis, because they can act as a focus for infection and cause cutaneous fistulas. When they present perforations and infections of endogenous origin, their course is usually fatal. Suitable treatment has been investigated on numerous occasions, most of the literature agreeing that the only curative treatment is surgical resection. However, surgical resection after performing arterial embolization to reduce the vascularization of the pseudotumour is a good alternative, thereby reducing the size of the pseudotumour and the risk of bleeding complications during surgery. It is important to bear in mind that despite its efficacy, arterial embolization is an invasive procedure with a reported rate of complications up to 25% (16% minor, 7% serious, 2% death). [ABSTRACT FROM AUTHOR]

Haemophilia patients with inhibitor have a higher level of arthropathy and more severe joint morbidity than patients without inhibitors. In recent years, interest has grown in the possibility that bypassing agent regimens could prevent bleeding and, consequently, arthropathy in inhibitor patients. Nevertheless, doubts about efficacy, complications and cost exist, questioning the justification of an uncertain prophylaxis in patients with inhibitors. Activated prothrombin complex concentrate (aPCC) has been used in more than 70 haemophilia patients with inhibitors in different clinical situations. aPCC prophylaxis seems to be safe and effective for the reduction of bleeding episodes in some patients. Recombinant activated factor VII (rFVIIa) has been employed prophylactically in over 44 haemophilia patients with inhibitors; 22 patients were included in the only randomized, prospective clinical trial of bypassing agents in prophylaxis. Bleeding frequency was reduced and this reduction was maintained during the postprophylaxis period. No thromboembolic events were reported during prophylaxis with rFVIIa. Although the effect of aPCC can last longer than that of rFVIIa, their efficacy rates are similar, suggesting that the biological effect of rFVIIa is actually much longer than indicated by its short plasma half-life. aPCC contains residual factor VIII antigen and may cause an anamnestic response in the inhibitor titre. This is crucial when immune tolerance induction is postponed to allow the inhibitor titre to decline to <10 Bethesda Units. In this setting, aPCC is not recommended as a first-line prophylaxis because of its potential to protract anamnesis, and rFVIIa is the preferred agent. [ABSTRACT FROM AUTHOR]

We recommend prophylaxis in haemophilic children with an inhibitor as a way of preventing the musculoskeletal impairment that is likely to affect them. This approach has been used for children without inhibitors with excellent results. If prophylaxis is not feasible, we suggest that intensive on-demand treatment should be given. Two agents, recombinant activated FVII (rFVIIa) and activated prothrombin complex concentrates (aPCC), are currently used to control haemostasis either for prophylaxis or intensive on-demand treatment. As it is recombinant, rFVIIa would seem more appropriate to be employed in children. aPCC could be used in adults, or in the event of an unsatisfactory response to rFVIIa. We recommend prophylaxis or, at least, intensive on-demand treatment in haemophilia children with inhibitors. Both rFVIIa and aPCC are being used for this purpose. It would seem that rFVIIa might be more appropriate for children as it is a recombinant product. Nevertheless, after skeletal maturity (in adults), both agents could be used indistinctively (taking into consideration that FEIBA is a plasma-derived product). We still need more well-designed comparative studies in order to be able to assert that our consensus-based conclusion is evidence based. In orthopaedic surgery, both aPCC and rFVIIa have been reported to be effective in controlling perioperative haemostasis, although in practice most centres have so far used rFVIIa for their orthopaedic procedures. We recommend rehabilitation programmes for all patients with inhibitors in order to mitigate the disabling and handicapping impact of their condition and thereby enable them to achieve social integration. Programmes for haemophilic children without inhibitors can be applied to children with inhibitors but should be individually tailored. [ABSTRACT FROM AUTHOR]

Haemophilia patients with inhibitors commonly present much more severe musculoskeletal problems than patients without inhibitors. With haematological advances using recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrate (aPCC), it is now possible to perform orthopaedic procedures in these patients with a high rate of success. In Spain, the healthcare system sets no financial limits for the treatment of haemophilia, and children with haemophilia today have a much better musculoskeletal status than 30 years ago. However, the children of 30 years ago are now adults and are afflicted by severe articular deformities that frequently require the intervention of an orthopaedic surgeon. Our view is that the well-known concepts of prophylaxis in patients without inhibitors should be followed and applied to patients with inhibitors. In fact, prophylaxis seems to be the only way to prevent the development of haemophilic arthropathy in patients with haemophilia. Until this goal can be achieved, major and minor orthopaedic procedures will be necessary in patients with inhibitors more frequently than in patients without inhibitors. [ABSTRACT FROM AUTHOR]

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). [ABSTRACT FROM AUTHOR]

In this paper, the most recent developments on arthropathy and inhibitors in haemophilia are reviewed. Firstly, early long-term prophylaxis is strongly recommended in children with severe haemophilia. Secondly, in joint bleeds not responding to substitution of factor VIII to normal factor VIII levels angiographic embolization might be considered as a promising therapeutic option. Thirdly, recent data indicate disruption of normal development for adolescents with inhibitors. Fourthly, some case reports indicate the benefit of secondary prophylaxis with recombinant factor VIIa (rFVIIa) or factor eight inhibitor bypassing activity (FEIBA) in patients with severe haemophilia A and inhibitors. Finally, surgical procedures in haemophilia patients with factor inhibitors can be accomplished safely and effectively with FEIBA and/or rFVIIa, although the risk of complications is higher than in haemophilia patients without inhibitors. [ABSTRACT FROM AUTHOR]

Radioactive synoviorthesis, with 90Y (knees) and 186Rh (elbows and ankles) is a highly effective procedure that decreases both the frequency and the severity of recurrent intra-articular bleeds related to joint synovitis. The procedure should be performed as soon as possible to minimize the degree of articular cartilage damage, which based on many studies is irreversible. It can also be used in patients with inhibitors. On average, radioactive synoviorthesis has a 75–80% satisfactory outcome in the long-term. From the clinical standpoint, such efficacy can be measured by the decrease in the number of haemarthroses, with complete cessation for several years in some cases. One should bear in mind that in 20–25% of cases, radioactive synoviorthesis fails to control haemarthroses. In such cases, it can be repeated. Personal experience and the general recommendation among orthopaedic surgeons and haematologists is that when three early consecutive radioactive synoviortheses (repeated every three months) fail to halt synovitis, a surgical synovectomy (open or by arthroscopy) should be immediately considered. [ABSTRACT FROM AUTHOR]

Prophylaxis is paramount to try to avoid the development of haemophilic synovitis. The best treatment for synovitis in patients with inhibitors is radioactive synoviorthesis (rhenium for ankle and elbows, yttrium for knees). With both methods (prohylaxis and radioactive synoviorthesis), we can delay the development of severe haemophilic arthropathy, that eventually will require major orthopaedic surgery. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

We report on a series of 27 orthopaedic surgical procedures. It includes 20 radiosynoviortheses and seven major orthopaedic procedures, performed on 26 patients. The average age of patients was 36 years (range: 8–53) and the average follow-up time was 2.5 years (range:1–5). There were 23 good results and four fair. In the synoviorthesis group (20 patients, 20 synoviortheses) the average age was 13.5 years (range: 9–26) and the average follow-up was 4.5 years (range: 1–7). There were 19 good results and one fair. All synoviortheses were done with activated prothrombin complex concentrates (FEIBA), all the responses being good except in one case (which had the final fair result). The total dose of FEIBA used was 600 IU kg−1, except in a patient that had a haemorrhagic complication. In fact, he required a prolongation of treatment up to a total dose of 2000 IU kg−1. In the group of major orthopaedic procedures, the average age of the six patients was 30.5 years (range: 11–53) and the average follow-up was 2.5 years (range: 1–5). There were six good results and one fair. Postoperative bleeding complications occurred in one of the seven major orthopaedic procedures performed (arterial pseudoaneurym after a total knee arthroplasty). Despite such complication, which had the final fair result, our study has shown that haemophilic patients with high inhibitor titres requiring orthopaedic surgery can undergo such procedures with a high expectation of success. In other words, orthopaedic surgery is now possible in haemophilia patients with high-titre inhibitors, leading to an improved quality of life for these patients. [ABSTRACT FROM AUTHOR]

Radiation synoviorthesis is a very effective procedure that decreases both the frequency and the intensity of recurrent ankle bleeds related to ankle synovitis. The procedure should be performed as soon as possible to minimize the degree of articular cartilage damage. It can also be used in patients with inhibitors with minimal risk of complications. On average, the efficacy of the procedure ranges from 76% to 80%, and can be performed at any age. The procedure slows the cartilaginous damage which intra-articular blood tends to produce in the long term. After 30 years of using radiation synovectomy worldwide, no damage has been reported in relation to the radioactive materials. Radiation synovectomy is currently the preferred procedure when radioactive materials are available; however, chemical synoviorthesis is an effective alternative method if radioactive materials are not available. Personal experience and the general recommendation among orthopaedic surgeons and haematologists is that when three early consecutive synoviorthesis (repeated every 3 months) fail to halt synovitis, a surgical synovectomy (open or by arthroscopy) should be immediately considered. For advanced haemophilic arthropathy of the ankle, the best solution is an ankle arthrodesis. Primary prophylaxis and radioactive synoviorthesis are the best ways that we have today of protecting against haemophilic synovitis and arthropathy of the ankle joint. [ABSTRACT FROM AUTHOR]

In the era of contemporary orthopaedics, haematology and internal medicine, it is obvious that surgery can be indicated in human immunodeficiency virus (HIV) and hepatitis C virus (HCV) coinfected haemophilia patients suffering from severe and painful musculoskeletal problems. However, the expected high risk of infection and other postoperative complications is a concern due to the poor health status that many of these patients present. In a HIV and HCV coinfected haemophilia patient, the orthopaedic surgeon together with the multidisciplinary haemophilia team should weigh the risks and benefits carefully. Clinical and immunological status should be considered before suggesting a surgical procedure (specially a joint arthroplasty) in this group of patients. If a surgical procedure is contraindicated, conservative treatment could be an alternative, although many times with inferior results. Usually, surgical procedures can better relieve pain for several years and improve the quality of life in this cohort of patients. Regarding anaesthetics, drugs metabolized by the liver or the kidney should be avoided depending on every particular case. Rachianaesthesia is more recommendable than epidural anaesthesia in elderly patients in whom general anaesthesia sometimes could be dangerous, although in all ages we prefer general anaesthesia. The size of the needle should be small (size G-27), and we never use spinal catheters. [ABSTRACT FROM AUTHOR]

A careful review of the available literature has shown with a moderate degree of evidence that surgery can be safely performed in haemophilic patients with either low-responding or high-responding inhibitors. Until recently, it was generally advised that surgery should be carried out in high responding haemophilic patients only if absolutely necessary, i.e. in emergency situations. Elective surgery was usually discouraged, with the exception of the placing of an indwelling catheter to carry out immune tolerance protocols. However, in reality elective surgery is often advisable in haemophilic patients with inhibitors because it may lead to an improved quality of life. At the present time, several options of treatment exist which appear to be sufficiently safe to assure haemostasis in different surgical procedures However, a more informed choice between different treatment options, based on estimation of advantages and risks linked to each treatment modality is desirable. Furthermore, the choice between different options has to be made taking into consideration costs of treatment. Ideally, randomised prospective studies are needed in order to provide a higher level of evidence of safety and efficacy of different treatment options and specifically comparing the two main bypassing options: activated prothrombin complex concentrates (aPCCs) and recombinant factor (rF)VIIa. In addition, such studies should be done with an adequate protocol, providing sufficient haematological data and an appropriate prolonged postoperative assessment. [ABSTRACT FROM AUTHOR]

Summary. Outcomes may be measured in terms of both objective and subjective variables and also on the basis of cost-efficiency. Most tools currently used to quantify outcomes, especially in orthopaedics, involve measurements of general health and of specific body part or organ system function. Patients' expectations of medical care are linked to their requests for treatment and to their assessments of outcome and satisfaction. Rating results are fundamental to evaluation and comparison of different methods of treatment. The discrepancies existing among rating scales have been an impediment in the evaluation of haemophilic patients from the orthopaedic point of view. Progress depends on international cooperation to improve communication and on the evaluation of new surgical techniques. The World Federation of Haemophilia (WFH) physical joint examination instrument and the Colorado physical examination (PE) instruments made important contributions by developing forms that will serve as a foundation for future evaluation systems. Some authors have suggested that the Colorado PE instruments are more indicative of early joint dysfunction than the WFH instrument. Future goals of the Colorado PE instruments are to refine the standard forms, identify additional important reproducible criteria and develop a comprehensive method of evaluation. It is paramount to develop valid and reliable surveys that can be used preoperatively to direct patient education and shared decision making and to provide a framework for setting reasonable goals. Re-examining patients' responses postoperatively could provide a way to assess fulfilment of expectations, which is a crucial patient-derived measure of outcome and satisfaction. [ABSTRACT FROM AUTHOR]

Summary. The political implications of haemophilia in the marriage of King Alfonso XIII of Spain and Princess Victoria Eugenie Battenberg of England have been reviewed in recent books on history. However, the fact that they had haemophilic sons also affected their personal relationship. In this article, we review the consequences haemophilia bore on their lives. We feel great compassion for families who suffer the illness, be it ordinary people or members of royalty; however, in this case, it can be said that when the disease affected a royal couple, the political consequences were great. [ABSTRACT FROM AUTHOR]

Close co-operation between haematologists, orthopaedic surgeons, rehabilitation physicians and physiotherapists is essential for obtaining satisfactory results after orthopaedic procedures that are performed on haemophilic patients. Although continuous prophylaxis could avoid the development of the orthopaedic complications of haemophilia that we still see in the 21st century, such a goal has not been achieved so far, not even in developed countries. Therefore, orthopaedic surgeons are still required to carry out many different surgical procedures, such as arthrocentesis, synoviorthesis, synovectomies, tendon lengthening, articular debridements, alignment osteotomies, joint arthroplasties, nerve releases, opening of compartment syndromes, removal of pseudotumours and osteosynthesis of fractures. Furthermore, the emergence of human immunodeficiency virus has meant that immunosuppressed patients in developed countries sometimes require an arthrotomy for the treatment of spontaneous septic arthritis, or the surgical drainage of a spontaneously infected haematoma (abscess). In addition, they have a high risk of postoperative infection after any surgical procedure, particularly a joint arthroplasty. [ABSTRACT FROM AUTHOR]

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 noninfective 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[sup -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. [ABSTRACT FROM AUTHOR]

Our experience and a review of the literature on inhibitors have shown that, with the availability of recombinant factor VIIa (rFVIIa), haemophilic patients with high inhibitor titres requiring elective orthopaedic surgery can undergo such surgery with a high expectation of success. The advent of rFVIIa has made major elective orthopaedic surgery possible in haemophilic patients with high-titre inhibitors, resulting in an improved quality of life. Recombinant FVIIa appears to be an efficient haemostatic product for surgery in patients suffering from haemophilia A and B with inhibitors. The series of major elective orthopaedic surgical procedures using rFVIIa (n = 53) is the largest ever reported in haemophilic patients with inhibitors, despite the long-standing presence of other treatment modalities, such as high-dose human factor VIII (FVIII), porcine FVIII (n = 8), and prothrombin complex concentrates/activated prothrombin complex concentrates (n = 9). Thorough analysis of each case as part of a multidisciplinary team will allow us to perform elective orthopaedic procedures in patients with inhibitors. [ABSTRACT FROM AUTHOR]

It is very likely that with the advent of widespread maintenance therapy, pseudotumours will be less common in the future. It is important that they are diagnosed early, and prevention of muscular haematomas is the key to reducing their incidence. There are a number of therapeutic alternatives for this dangerous condition: surgical removal, percutaneous management, exeresis and filling of the dead cavity, irradiation, and embolization. The management of a patient with a haemophilic pseudotumour is complex, with a high rate of potential complications. Surgical excision is the treatment of choice but should only be carried out in major haemophilia centres by a multidisciplinary surgical team. The main postoperative complications are death, infection, fistulization, and pathological fractures (even requiring amputations of affected limbs). Pelvic pseudotumours can even become complicated by fistulization to the large bowel and by obstruction of the ureters. Untreated, proximal pseudotumours will ultimately destroy soft tissues, erode bone, and may produce neurovascular complications. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The goal of modern fracture treatment is to obtain an optimal outcome, with the patient's return to full activity as soon as possible. Nowadays, internal stabilization is indicated in most displaced fractures in adults, whereas external fixation remains the best choice for initial stabilization with severe soft-tissue injuries. If a fracture is correctly treated in a haemophilic patient, it will progress to consolidation in a similar time-frame to fractures occurring in the general population. [ABSTRACT FROM AUTHOR]