End-stage Haemophilic Arthropathy Research Articles

TKR in Hemophilic Arthropathy: A Combination of Special Surgical Considerations and Novel Nonacog Beta Pegol: A Case Report.

A 29-year-old man with hemophilia B presented with advanced arthropathy of the right knee, resulting in poor knee functional scores and difficulties in his livelihood. The patient underwent total knee replacement while receiving nonacog beta pegol factor IX by a multidisciplinary approach. Hemophilias commonly result in end-stage hemophilic arthropathy of the joints at a young age that may warrant joint replacement surgeries. This case report illustrates the surgical protocol of total knee arthroplasty in a patient who received a long-acting factor IX preparation.

Clinical Efficacy of Intra-articular Tranexamic Acid Injection in the Management of Hemophilia with Total Hip Arthroplasty: A 24-month Retrospective Cohort Study.

Total hip arthroplasty (THA) effectively treats end-stage hemophilic hip arthropathy. Given hemophilia's unique characteristics, perioperative bleeding remains a significant risk for patients undergoing THA. Tranexamic acid (TXA), an efficient antifibrinolytic agent, may benefit the outcomes of THA for patients with hemophilia (PWH). This study aims to explore the clinical efficacy of intra-articular injection of TXA in treating perioperative bleeding in PWH and assess its additional clinical benefits. The retrospective study comprised data of PWH who received THA from January 2015 to December 2021 in the research center. A total of 59 individuals were included in the study, divided into a TXA group (n = 31) and a non-TXA group (n = 28). We compared various parameters, including total blood loss (TBL), visible blood loss (VBL), occult blood loss (OBL), intraoperative coagulation factor VIII (FVIII) consumption, perioperative total FVIII consumption, hemoglobin (HB), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), length of hospital stay, hospitalization costs, length of surgery, total protein, activated partial thromboplastin time (APTT), D-dimer, rate of joint swelling, hip joint range of motion (ROM), visual analogue scale (VAS), and Harris hip joint function scale (HHS) between the two groups. Follow-up assessments were conducted for up to 24 months. A Student's t test was utilized for the statistical analysis. This study demonstrated that intra-articular TXA effectively reduced TBL (1248.19 ± 439.88 mL, p < 0.001), VBL (490.32 ± 344.34 mL, p = 0.003), and OBL (757.87 ± 381.48 mL, p = 0.004) in PWH who underwent THA. TXA demonstrated effectiveness in reducing VAS scores on POD1, POD7, and POD14 and joint swelling rates on POD1, POD7, POD14, and at discharge (p < 0.05). Additionally, the TXA group achieved higher HHS ratings at all follow-up time points (p < 0.05), showing superior hip joint mobility, lower postoperative inflammation levels, reduced factor VIII consumption during surgery, and less postoperative nutritional loss. No statistically significant differences were observed between the two groups in terms of hospital stay, hospitalization costs, surgery duration, and coagulation indicators. Intra-articular injection of TXA reduces perioperative bleeding in PWH undergoing THA while also improving joint mobility, post-operative rehabilitation, and quality of life. This may provide value for the future application of TXA in PWH.

Outcome measures analysis following total knee arthroplasty in patients with severe haemophilic arthropathy of the knee.

Total knee arthroplasty (TKA) has been the gold standard for treating severe haemophilic arthropathy of the knee when all conservative measures fail. However, performing a TKA in patients with haemophilic arthropathy is difficult due to severe joint deformity and destruction, and poor bone quality. The aim of the present study was to evaluate the short-term results of TKA in the treatment of knee haemophilic arthropathy in a tertiary referral centre, with an emphasis on health-related quality of life and knee function. A prospective study was conducted that included 19 male patients with end-stage haemophilic knee arthropathy who underwent TKA in a tertiary referral centre. Clinical outcome and health-related quality of life were assessed by the Western Ontario and McMaster Universities Arthritis (WOMAC) index and the Short Form-36 (SF-36) both pre-operatively and at 1-year post-operatively. The mean age of the patients was 50.37±7.63 years (range, 40-65 years). Pre-operative health-related quality of life was impaired in all patients in all SF-36 domains but was markedly improved after TKA. Knee function in all dimensions (pain, stiffness and physical function), as measured by the WOMAC questionnaire, significantly improved after TKA. Pre-operative pain, stiffness and function, along with total WOMAC score, were strongly and negatively correlated with pre-operative SF-36. Overall, the present study indicated a significant improvement in quality of life and clinical outcome after TKA in patients with advanced haemophilic arthropathy. More studies with longer follow-up periods in a larger population are needed to fully elucidate the mid- and long-term values of TKA in haemophilic patients.

Physical therapy for end-stage hemophilic arthropathy: a case report

This report introduces a young adult who has been in bed for more than ten years with end-stage hemophilic arthropathy. He didn’t have access to factor VIII (FVIII) in the early stage of hemophilia due to the high costs of clotting replacement therapy. As a result, he is experiencing some difficulties, such as joint contracture, muscular atrophy, severe pain, and poor function of cardiopulmonary. He came to visit us for a comprehensive rehabilitation program, and, finally, he achieved the basic goal of self-care in daily life.

Long-term clinical outcomes following total knee arthroplasty in patients with hemophilic arthropathy: a single-surgeon cohort after a 10- to 17-year follow-up.

Total knee arthroplasty (TKA) can reduce severe joint pain and improve functional disability in hemophilia. However, the long-term outcomes have rarely been reported in China. Therefore, this study aimed to evaluate the long-term outcomes and complications of TKA in Chinese patients with hemophilic arthropathy. We retrospectively reviewed patients with hemophilia who underwent TKA between 2003 and 2020, with at least 10 years of follow-up. The clinical results, patellar scores, patients' overall satisfaction ratings, and radiological findings were evaluated. Revision surgery for implants during the follow-up period was recorded. Twenty-six patients with 36 TKAs were successfully followed up for an average of 12.4 years. Their Hospital for Special Surgery Knee Score improved from an average of 45.8 to 85.9. The average flexion contracture statistically significantly decreased from 18.1° to 4.2°. The range of motion (ROM) improved from 60.6° to 84.8°. All the patients accepted patelloplasty, and the patients' patellar score improved from 7.8 preoperatively to 24.9 at the last follow-up. There was no statistically significant difference in clinical outcomes between the unilateral and bilateral procedures, except for a better ROM at follow-up in the unilateral group. Mild and enduring anterior knee pain was reported in seven knees (19%). The annual bleeding event was 2.7 times/year at the last follow-up. A total of 25 patients with 35 TKAs were satisfied with the procedure (97%). Revision surgery was performed in seven knees, with 10- and 15-year prosthesis survival rates of 85.8% and 75.7%, respectively. TKA is an effective procedure for patients with end-stage hemophilic arthropathy, which relieves pain, improves knee functions, decreases flexion contracture, and provides a high rate of satisfaction after more than ten years of follow-up.

A Retrospective, Long-term Follow-up of the Clinical Outcomes and Cost-effectiveness of Single-anesthetic Multiple Total Joint Procedures in Hemophilic Arthropathy.

Although total joint replacement (TJR) procedures are efficacious, perioperative high-dose factors replacement therapy (FRT) to avoid catastrophic bleeding represents a significant hurdle, particularly for patients with multiple joint affection. Double simultaneous bilateral TJRs were reported as safe and cost-effective. However, little is known about multiple TJRs. The feasibility and effects remain debatable. Surgeons need to weigh the high cost of FRT against safety. Accordingly, we aimed to evaluate the clinical outcomes and cost-effectiveness of single-anesthetic multiple-joint procedures of lower limbs in end-stage hemophilic arthropathy. Our retrospective cohort study retrieved data from an inpatient database of patients with hemophilia who underwent total knee arthroplasty (TKA), total hip arthroplasty (THA), and/or ankle arthrodesis from January 2000 to April 2016. Complications, hospital stays, transfusion, doses of clotting factor, medical costs, range of motion (ROM), Harris hip scores (HHSs) and Hospital for special surgery knee scores (HSSs) were recorded. A P value < 0.05 was considered significant. A total number of 81 patients were included in this study, among which 89 TKAs and 52 THAs were performed. Compared to the single TJR group, the simultaneous multiple TJR group showed a significantly higher rate of blood transfusions (P < 0.05). But no significant differences were found in the length of hospital stays, factor consumption, hospitalization costs excluding prosthesis expenses, and total complication rates. Finally, similar postoperative ROM, HHS, and HSS were witnessed in two groups (P value > 0.05). Our data indicated that simultaneous multiple TJRs are a safe and cost-effective choice for treating hemophilic patients with multiple HA-affected lower limb joints.

Influence of RANKL and OPG in Hemophilic arthropathy

Background: Hemophilia is a bleeding disorder characterized by recurrent bleeding, especially in joints (hemarthrosis), leading to destructive effect to the most of joint components, resulting in synovitis, osteochondral degeneration, and eventually, end-stage hemophilic arthropathy. Objective: Since few published data exist about the influence of OPG (Osteoprotegrin) and RANKL (receptor activator of nuclear factor-kappa B ligand) in haemophilic arthropathy, the present study is conducted to investigate their serum level in hemophilic patients. Methodology: This study has been carried out on Iraqi patients with hemophilia in the National Center of Hematology/Mustansiriyah University. Forty male patients involved in this study with a range of (11-44) years old, Hemophilia A affected 25 of them, while the other 15 were hemophilia B. Along with patient group, 20 healthy subjects with matched age and gender were involved as control group. The presence and severity of arthropathy among patients was determined based activated partial thromboplastin time (aPTT)) in hemophilic patients, while RANKL and OPG were determined in the serum samples of healthy and patient subjects. Result: The serum level of RANKL in Hemophilia A patients was (204.6pg/ml) is significantly (p0.001) higher than in control (164.5pg/ml) and in Hemophilia B patients was (200pg/ml). While the serum level of OPG in Hemophilia A patients was (67.2pg/ml) is significantly (p0.002) higher than in control (30.21pg /ml) and in Hemophilia B patients (48.85pg/ml). Conclusion: It can be concluded that this system RANKL, RANK, and OPG are important for the metabolism of bones.

IMPACT OF STATIC PROGRESSIVE STRETCH ON RANGE OF MOTION AFTER TOTAL KNEE REPLACEMENT IN PATIENTS WITH HAEMOPHILIA.

BackgroundHaemophilic arthropathy is often associated with a loss of range of motion. Total knee arthroplasty is an effective treatment option for patients with end-stage haemophilic arthropathy of the knee. However, even after arthroplasty, the range of motion sometimes remains insufficient.ObjectiveTo evaluate static progressive stretch as a treatment method for haemophilic patients with decreased range of motion after total knee arthroplasty.MethodsStatic progressive stretch was used to improve range of motion in patients with a postoperative extension lag of more than 10° and flexion of less than 80°. A total of 7 knees were treated for a mean of 21.7 weeks.ResultsStatistically significant increases in range of motion and in Knee Society Score were observed when comparing pre-treatment and post-treatment values.ConclusionsStatic progressive stretch using an orthotic device could be a successful adjuvant method for treating joint stiffness in patients with haemophilia after total knee arthroplasty.LAY ABSTRACTHaemophilic arthropathy often results in loss of range of motion. Total knee arthroplasty is an effective treatment option for patients with end-stage haemophilic arthropathy of the knee. However, despite arthroplasty, the range of motion is sometimes insufficient. Static progressive stretch using special knee orthoses is an adjunct technique to traditional physiotherapy, intending to restore functional range of motion and decrease knee stiffness and pain. The aim of this study was to evaluate static progressive stretch as a treatment method for haemophilic patients with decreased range of motion after total knee arthroplasty.

A comparison of arthrodesis and arthroplasty for hemophilic arthropathy of the ankle: a systematic review

Objective: To conduct a systematic review of the literature on surgical treatment for end-stage hemophilic arthropathy of the ankle joint, describing the results for arthroplasty and arthrodesis. Methods: We conducted a systematic literature review according to Cochrane (Cochrane Handbook for Systematic Reviews of Interventions version 6.1, 2020) and PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) recommendations. The primary outcome was clinical improvement in pain and function of the affected limb. Secondary outcomes included adverse effects and complications from the surgical procedures. Results: After reviewing 514 studies, 10 were included for data extraction and qualitative analysis (180 patients: 100 arthroplasties and 117 arthrodeses). Conclusions: Both methods showed low overall complication rates and effectively reduced pain and improved function, allowing a return to work, activities of daily living and, in some cases, sports. One procedure was not clearly better than the other: the choice must be based on the patient’s characteristics and clinical judgment. Level of Evidence III; Therapeutic Studies; Systematic Review of Level III Studies.

The mid-term clinical outcomes of total knee arthroplasty in the management of end-stage hemophilic arthropathy.

This study aimed to evaluate the mid-term clinical outcomes of total knee arthroplasty (TKA) in the management of end-stage hemophilic arthropathy. Eleven patients (15 knees) undergoing TKA with stiff knees were retrospectively evaluated. TKA was performed in all patients without additional surgical interventions such as posterior capsular release, hamstring release, synovectomy, VY quadricepsplasty, or tibial tubercle osteotomy. All patients were evaluated for clinical and radiological results at follow-ups. Functional evaluation and pain status were assessed using the Knee Society Score and Visual Analogue Scale. The mean age at the time of operation was 40.8 ± 11.8 years (range=30-64 years). The mean follow-up was 51.2 ± 20.6 months (range=24-95). The mean flexion contracture significantly decreased from 17.6 ± 11.3 to 1.7 ± 2.8 degrees, and the mean maximum flexion increased dramatically from 55.6 ± 20.5 to 109.2 ± 16.2 degrees (P < 0.001). Statistical significant improvement in flexion and flexion contracture degrees continued up to the postoperatively 18 months. The mean Knee Society Score increased from 22.7 ± 2.4 points preoperatively to 87.8 ± 3.8 points at the last follow-up (P < 0.001). The mean cost of coagulation factor consumption and blood transfusion accounted for 78% of the total cost. This study has shown that TKA is an effective treatment for relieving pain and improving both ranges of motion and quality of life in managing end-stage hemophilic arthropathy of the knee joint. Level IV, Therapeutic Study.

Total Knee Arthroplasty in Hemophilia: Survivorship and Outcomes-A Systematic Review and Meta-Analysis.

Patients with hemophilia (PWHs) may experience spontaneous or traumatic bleeding episodes. Recurrent bleeding can lead to end-stage hemophilic arthropathy and total knee replacement (TKR) provides an effective treatment. The aim of this study is to investigate outcomes in PWHs who undergo TKR. A systematic review and meta-analysis was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Study outcomes included patient-reported functional outcomes, implant survivorship, and complications. Subgroup analysis was performed assessing the effect of recombinant prophylaxis medication by comparing studies that included only TKRs performed after the year 2000 (period A), to those that included TKRs before 2000 (period B). Twenty-eight studies were included, with a total of 1210 TKRs performed in 917 PWHs. The mean age of patients was 38.5 years (standard deviation 5.1) with a mean length of follow-up of 7.1 years (standard deviation 2.9). The total complication rate was 28.7%, with 19.3% requiring a further procedure. Hospital for Special Surgery Knee Score improved by 44.6 points (confidence interval 38.9-50.4) and Knee Society Score function improved by 35.9 points (confidence interval 30.1-41.8). Total range of motion improved by 22.3°. The most common complication was post-operative hemarthrosis (7.6%, 92 TKRs). Deep infection (6.2% vs 3.9%) and aseptic loosening (3.8% vs 2.1%) rates fell between period B and period A. TKR in PWHs is a successful procedure improving function, reducing pain, and improving range of motion. PWHs undergo TKR at a younger age and have a higher risk of complications, though contemporary treatment has reduced these risks. PWHs can expect similar survivorship to the general population.

Comparison of analgesic consumption of hemophilic and non-hemophilic patients in knee arthroplasty.

Hemophilia is a rare hereditary bleeding disorder that develops as a result of factor VIII or IX deficiency. Long-term complications of hemophilia such as arthropathy, synovitis, and arthritis can lead to the development of recurrent chronic pain. Pain is therefore a critical aspect of hemophilia. The gold standard treatment for end-stage hemophilic knee arthropathy is total knee arthroplasty (TKA). The hypothesis of this study was that after knee replacement surgeries that cause severe post-operative pain, hemophilia patients with chronic analgesic consumption may experience higher levels of pain than non-hemophilic patients, and use more opioid and non-opioid drugs. This retrospective study included 82 patients who were hemophilic and non-hemophilic TKA patients operated under general anesthesia. Seventy-three patients were evaluated and divided into two groups according to the diagnosis of hemophilia: 36 patients were investigated in the hemophilic group and 37 patients in the non-hemophilic group. Post-operative tramadol consumption (p=0.002) and pethidine consumption (p=0.003) were significantly higher in the group hemophilia. The length of stay in the hospital was also significantly longer in the hemophilic group (p=0.0001). In the light of these informations, we think that acute post-operative pain management of hemophilia patients should be planned as personalized, multimodal preventive, and pre-emptive analgesia.

Clinical and patient reported outcome in total ankle replacement compared to ankle fusion in end-stage haemophilic arthropathy.

Ankle arthropathy is a frequent complication of haemophilia, reducing the patients' quality of life. Despite intensive conservative therapy, end-stage arthropathy requires surgical treatment, either by ankle fusion (AF) or total ankle replacement (TAR). Eleven consecutive AFs were performed in nine patients and 11 TARs were implemented in 10 patients. Outcomes were assessed clinically by AOFAS score and radiologically by the Pettersson and Gilbert scores. The mean age of the patients in these groups were 35.7 years and 49.4 years, respectively. Of the 11 ankles that underwent fusion, 10 showed bony consolidation not later than 12weeks after surgery, whereas one still showed non-union after 6months. VAS pain scores decreased significantly in both groups. Mean AOFAS scores also improved significantly, from 28.1 before to 80.3 after AF and from 21.5 before to 68.0 after ankle replacement. No perioperative complications were observed in either group. Late deep infection was observed in two patients that underwent TAR, which required removal of the implant. Our data indicate that both AF and TAR result in significantly reduced pain in patients with haemophilia with end-stage haemophilic arthropathy. While TAR is associated with a higher risk of deep infection and minimal persistent pain, it preserves the pre-operative range of motion. AF on the other hand is associated with the risk of non-union and a longer post-operative recovery period but results in greater pain reduction.

Inflammation, angiogenesis and sensory nerve sprouting in the synovium of bony ankylosed and not bony ankylosed knees with end-stage haemophilic arthropathy.

Clinical practice showed that patients with haemophilia (PwH) with bony ankylosed end-stage haemophilic arthropathy knees reported milder pain than those with not bony ankylosed knees. To compare the differences in pain sensation and the histopathological differences in synovial samples of affected knee joints between PwH with bony ankylosed end-stage haemophilic arthropathy knees and those with not bony ankylosed knees. From January 2011 to December 2019, the synovial samples of knee joints were collected during total knee arthroplasty (TKA) surgery for end-stage haemophilic arthropathy. The visual analogue scale (VAS, 0-10) pain score was reviewed from the chart data of the patients. The thickness of the inner layer of the synovium in haematoxylin and eosin (H&E) staining sections was measured. The expression levels of Ki67, IL-1β, TNF-α, CD31, VEGF, NGF and PGP9.5 in the synovium were detected by immunohistochemistry (IHC) method. Fifty-two end-stage haemophilic arthropathy knee synovial samples from 36 male PwH (34 type A and 2 type B) were collected. Fifteen knees had bony ankylosed (BA-group), and 37 were not bony ankylosed (Not-BA-group). The mean age of patients at TKA surgery of BA-group and Not-BA-group was 32years (15) and 32years (10), respectively (p=0.824). Before TKA surgery, the mean VAS pain scores of patients in the Not-BA-group were significantly higher than those in the BA-group (p<0.001). The mean thickness of the inner layer of the synovium, the mean rate of Ki67+ cells, the mean density of CD31+ vascular endothelial cells and the expression levels of IL-1β, TNF-α, VEGF and NGF in samples in the Not-BA-group was significantly higher than those in samples in the BA-group (p<0.001, p=0.02, p=0.001, p=0.117, p<0.001, p=0.003 and p=0.008), respectively. The mean density of PGP9.5+ sensory neural fibres in the Not-BA-group was slightly higher than in the BA-group (p=0.131). Linear regression analysis showed a significant positive correlation between the VAS pain score and indicators including the synovial thickness, the rate of Ki67+ cells, the expression level of IL-1β, TNF-α, VEGF, NGF and the densities of CD31+ vascular endothelial cells and PGP9.5+ nerve fibres (p<0.05). Worsened hypertrophic synovitis, angiogenesis and sensory nerve sprouting in the synovium may play a critical role in causing worse pain sensation in PwH with not bony ankylosed haemophilic arthropathy knees than in those with bony ankylosed knees.

The role of total ankle replacement in patients with haemophilia and end-stage ankle arthropathy: A review.

Haemophilia is the most common X-linked bleeding disorder, affecting over 1 million individuals throughout the world. Patients are subclassified into mild, moderate and severe disease based on per cent factor activity level. Nearly, all patients with haemophilia develop haemophilic arthropathy (HA) by age 30 and HA is known to have a negative impact on physical health subscores in Haem-A-QOL, a validated quality of life scoring system for patients with haemophilia. Unfortunately, many patients progress to end-stage HA of the ankle, which is characterized by pain, contractures, decreased range of motion and muscle atrophy. Ankle arthrodesis (AAD) has been the standard of care in the definitive surgical management of end-stage HA of the ankle. While AAD is a safe surgical procedure known to improve HA-related pain, it decreases functional mobility and has been associated with secondary hindfoot arthritis as well as subtalar degeneration. In recent years, total ankle replacement (TAR) has emerged as an alternative surgical procedure that strives to improve functional mobility, pain and quality of life in end-stage HA of the ankle. However, the safety, durability, and efficacy of this procedure in these patients are unknown. In this review, we analyse the clinical studies investigating TAR in patients with end-stage HA of the ankle. We also discuss important considerations in the perioperative management of patients with haemophilia and compare the risks and benefits of AAD and TAR for patients with end-stage HA of the ankle.

Comparative Analysis of the Occurrence and Role of CX3CL1 (Fractalkine) and Its Receptor CX3CR1 in Hemophilic Arthropathy and Osteoarthritis.

Objective Hemophilic arthropathy is characterized by recurrent bleeding episodes in patients with hemophilia leading to irreversible joint degeneration. The involvement of CX3CL1 (fractalkine) and its receptor CX3CR1 was observed in the pathogenesis of numerous arthritis-associated diseases. Taking this into account, we have presented a study investigating the role of the CX3CL1/CX3XR1 axis in the course of hemophilic arthropathy, including the CX3CL1-dependent expression of CD56+, CD68+, and CD31+ cells along with evaluation of articular cartilage and synovial membrane morphology. Methods The study was carried out using cases (n = 20) of end-stage hemophilic arthropathy with a severe type of hemophilia A and control cases (n = 20) diagnosed with osteoarthritis. The biofluids including blood serum and synovial fluid were obtained intraoperatively for the evaluation of CX3CL1 using the ELISA test. Tissue specimens including articular cartilage and synovial membrane were similarly collected during surgery and stained immunohistologically using selected antibodies including anti-CX3CR1, anti-CD56, anti-CD68, and anti-CD31. Additionally, the analysis included the assessment of articular cartilage, synovial membrane, and blood vessel morphology. Results In our study, we have documented increased average concentration of CX3CL1 in the blood serum of the study group (7.16 ± 0.53 ng/ml) compared to the control group (5.85 ± 0.70 ng/ml) without statistically significant difference in synovial fluid concentration at the same time. We have observed an increased macrophage presence with more marked proliferation and fibrosis of the synovial membrane in the study group. Remaining results such as expression of CX3CR1 presence of NK cells and larger surface area of blood vessels within the synovial membrane were noted also without statistical significance. Conclusions This study has demonstrated collective CX3CL1/CX3CR1 axis involvement in hemophilic arthropathy pathogenesis introducing new interesting diagnostics and a therapeutic target.

Complications of total knee arthroplasty in patients with haemophilia compared with osteoarthritis and rheumatoid arthritis: A 20-year single-surgeon cohort.

Patients with haemophilia are prone to haemophilic arthropathy (HA). For end-stage HA, total knee arthroplasty (TKA) is an effective procedure. However, limited data were available regarding complications of TKA in HA patients. To provide comprehensive comparisons of TKA complications among patients with osteoarthritis (OA), rheumatoid arthritis (RA) or HA. The present study was a single-surgeon cohort study including patients who underwent TKAs for OA, HA or RA from January 1997 to December 2017. The information of patients was extracted from medical records and follow-up database. The primary outcome was complications. Potential risk factors of complications in HA patients were also evaluated. A total of 1515 patients with 2083 TKAs were selected following the criteria. The overall complication rate in the HA group was 21.79%, which was much higher than the OA or RA group (7.08% and 8.70%, respectively, P<.05). The dominate complications were prosthetic loosening and wound dehiscence. For HA, more complications occurred in the period more than 1year after TKA when compared with OA (33.33% vs 11.43%, P=.028). Among the potential risk factors, patients with haemophilia B and severe haemophilia had significantly higher complication rates (P<.05 for both). When compared with OA or RA, HA patients had different characteristics on complications, including the higher complication rate, different complication distribution and later occurring time. In HA patients who underwent TKA, haemophilia B and severe haemophilia were risk factors of complications which should be paid more attention to.

Total Knee Arthroplasty in Patients with Hemophilia: What Do We Know?

Total knee arthroplasty is considered as the treatment of choice for those with end stage hemophilic arthropathy. Compared to other patients undergoing TKA, these patients have specific features such as bleeding tendency, younger age, pre-operative restricted range of motion (ROM), altered anatomy, and increased complications. This narrative review of literature is going to investigate several issues regarding the TKA in hemophilic patients including indications, perioperative factor replacement, surgical challenges, postoperative rehabilitation, outcomes, and complications.

Ankle arthrodesis for end-stage haemophilic ankle arthropathy using a Ilizarov method.

Only a few studies in the literature have reported on the outcome of ankle arthrodesis in haemophilia patients, and the optimal surgery technique for end-stage haemophilic ankle arthropathy remains controversial. The purpose of this study was to evaluate the results of ankle arthrodesis using the Ilizarov method in treating end-stage haemophilic ankle arthropathy. Fourteen haemophilia patients with end-stage ankle arthropathy who underwent ankle arthrodesis using the Ilizarov method from December 2013 to May 2018 were enrolled in this study. The hospital day, fusion rate, complications, and functional outcomes were recorded and analyzed. Functional outcomes were evaluated according to the pre-operative and final follow-up of the American Orthopedic Foot and Ankle Society (AOFAS) ankle-hind foot score, visual analog scale (VAS), and Medical Outcomes Short Form 12-item questionnaire score (SF-12) . All patients achieved bony fusion as confirmed by post-operative radiographs. The average time to fusion was 12.9weeks (range, 10-17weeks). Superficial pin track infection was observed in two patients, and subtalar arthritis was found in one patient. The mean AOFAS ankle-hind foot score, VAS score, physical component summary (PCS), and mental component summary (MCS) at final follow-up were significantly improved compared with the pre-operative data. All patients were satisfied with the surgery. Ankle arthrodesis using the Ilizarov method is a minimally invasive, effective, and safe surgical method and can be a viable option for hemophilia patients with end-stage ankle arthropathy.

Joint disease in haemophilia: Pathophysiology, pain and imaging.

Haemarthroses cause major morbidity in patients with haemophilia. Blood has devastating effects on all joint components, resulting in synovitis, osteochondral degeneration and ultimately end-stage haemophilic arthropathy. Key players in this process are iron and inflammation. Preventing joint bleeds is of utmost importance to maintain joint health as targeted therapies directed against blood-induced inflammation and iron-mediated processes are lacking. Joint bleeds result in acute pain as well as chronic pain due to synovitis or arthropathy. Acute pain originates from nociceptors activated by tissue damage. In chronic inflammation, central and peripheral sensitization of nociceptors might occur resulting in chronic pain. This also triggers a series of brain disorders such as emotional fear, anxiety, mood depression and impairment of cognitive functions. Treatment of haemophilia-related pain not only consists of analgesics, but also of exercise, education and in selected cases antidepressants and anticonvulsants. For objective assessment of joint structural outcome and detecting earlier changes of haemophilic arthropathy, both ultrasound (US) and magnetic resonance (MR) imaging have shown valuable. Both can be considered equally able to reveal signs of disease activity. MR imaging is able to visualize haemosiderin deposition and is more comprehensive in depicting osteochondral changes. Disadvantages of MR imaging are the duration of the examination, evaluation of a single joint at a time, costs and may require sedation, and it may need intraarticular contrast injection to depict initial osteochondral changes with accuracy. As such, US is a more useful screening tool and can be used for repeated follow-up examinations.