Pre-operative synovial hyperaemia in haemophilia patients undergoing total knee replacement and the effects of genicular artery embolization: A retrospective cohort study.

AIM: Haemophilia is characterized by recurrent joint bleeding caused by a lack of clotting factor VIII or IX. Due to repeated joint bleeding, end-stage arthropathy occurs in relatively young patients. A total knee replacement (TKR) can be a solution. However, TKR may be complicated by perioperative and postoperative bleeds despite clotting factor therapy. The aim of this study was to evaluate the prevalence of pre-operative synovial hyperaemia and the effects of Genicular Artery Embolization on synovial hyperaemia and 3-month postoperative joint bleeding. METHODS: In this retrospective cohort study, all patients with haemophilia who underwent periarticular catheter angiography between 2009 and 2020 were evaluated after written informed consent. Synovial hyperaemia on angiography was scored by an interventional radiologist. RESULTS: Thirty-three angiography procedures in 24 patients were evaluated. Median age was 54.4 years (IQR 48.4-65.9). Preoperative synovial hyperaemia was observed in 21/33 joints (64%). Moderate and severe synovial hyperaemia was observed in 10/33 joints (30%). Synovial hyperaemia decreased in 13/15 (87%) joints after embolization. Three-month postoperative joint bleeding occurred in 5/32 joints: in 2/18 joints (11%) without synovial hyperaemia and in 3/14 joints (21%) with mild synovial hypertrophy. Non-embolized and embolized joints did not differ regarding 3-month postoperative bleeding (P = .425). No complications were observed after embolization. CONCLUSION: One-third of patients with haemophilia requiring a TKR had moderate or severe synovial hyperaemia which can be reduced safely by Genicular Artery Embolization prior to TKR. Three-month postoperative bleeding appears to occur independently of the presence of residual mild synovial hyperaemia.

Hemostatic cover in orthopedic surgery.

Bleeding into joints, known as hemarthrosis, is the most common kind of bleeding experienced by patients with hemophilia. Repeat bleeds into the same joint lead to the so-called hemophilic arthropathy. Patients with this condition tend to require surgery earlier and most frequently than the general population. Successful hemostasis is essential to carry out such procedures. Thanks to the advances made in the treatment of hemophilia, most surgical techniques can be performed safely and reliably. The present review shall focus on the international recommendations related to the performance of these surgical procedures. We shall be examining the available treatments, including the way they should be administered as well as the requirements regarding the postoperative period and the subsequent rehabilitation program.

Ankle joint distraction is a promising alternative treatment for patients with severe haemophilic ankle arthropathy.

INTRODUCTION: Haemophilic ankle arthropathy (HAA) causes major morbidity. When conservative treatment fails, major surgical interventions are indicated. An alternative treatment to maintain joint mobility and postpone these interventions is desired. AIM: To gather prospective data on clinical/structural changes after ankle joint distraction (AJD) in HAA. METHODS: This study includes patients with severe HAA insufficiently responding to conservative treatment. AJD was performed during 8-10 weeks by use of an external frame. Questionnaires, physical examination and radiology were used to evaluate pain, function and structural changes before and 6, 12, 24 and 36 months after distraction. Mixed effect models were used for analysis. RESULTS: This study includes eight cases (21-53 years). The fixed effects estimates of the visual analogue score (0-10) improved from 7.5 at baseline to 3.4 (p = .023) 3 years after distraction. The Haemophilia Activities List (HAL, 0-100) for basic/complex lower extremities functions improved from respectively 29.6 and 31.5 to 54.3 (p = .015) and 50.7 (p = .031). Joint mobility was maintained. Magnetic resonance imaging (MRI) showed thickened cartilage and reduced bone marrow oedema and subchondral cysts. Pin tract infections (n = 6) were effectively treated and no adverse bleeding events occurred. At 3-year follow-up, in none of the patients the originally indicated arthrodesis was performed. CONCLUSION: This first prospective study showed that AJD in HAA results in decreased pain, improved function and decreased arthropathy-related MRI findings in the majority of patients for prolonged time. Although the study population is small and follow-up is relatively short, AJD may be promising to postpone invalidating interventions and might be a breakthrough treatment.

Total knee arthroplasty in hemophilia: lessons learned and projections of what's next for hemophilic knee joint health.

INTRODUCTION: The purpose of this article has been to review the literature on total knee arthroplasty (TKA) in people with hemophilia (PWH), to mention the lessons we have learned from our own experience and to try to find out what the future of this type of surgery will be. AREAS COVERED: A Cochrane Library and PubMed (MEDLINE) search of studies related to TKA PWH was analyzed. In PWH, the complication rate after TKA can be up to 31.5%. These include infection (7.1%) and bleeding in the form of hematoma, hemarthrosis or popliteal artery injury (8.9%). In a meta-analysis the revision arthroplasty rate was 6.3%. One-stage or two-stage revision arthroplasty due to infection (septic loosening) is not always successful despite providing correct treatment (both hematological and surgical). In fact, the risk of prosthetic re-infection is about 10%. It is necessary to perform a re-revision arthroplasty, which is a high-risk and technically difficult surgery that can sometimes end in knee arthrodesis or above-the-knee amputation of the limb. EXPERT OPINION: TKA (both primary and revision) should be performed in centers specialized in orthopedic surgery and rehabilitation (knee) and hematology (hemophilia), and with optimal coordination between the medical team.

Recurrent Hemarthrosis Secondary to Erosive Patellofemoral Arthritis Treated with Arthroplasty: A Report of 3 Cases.

CASE: Three patients presented with recurrent hemarthrosis secondary to erosive patellofemoral arthritis. Recurrent hemarthrosis from the eroded patellofemoral subchondral bone has not been well described. Each patient presented with symptoms secondary to painful effusions that were identified by aspiration. Each patient was successfully treated with patellofemoral or total knee arthroplasty. CONCLUSION: Spontaneous or recurrent effusions in the setting of erosive patellofemoral arthritis should prompt orthopaedic surgeons to consider hemarthrosis as the cause of such effusions. Patellofemoral or total knee arthroplasty is effective in resolving the hemarthroses, resolving pain, and restoring function in these patients.

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

BACKGROUND: 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. METHODS: 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). RESULTS: 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. CONCLUSION: 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.

Perioperative haematological outcomes following total knee arthroplasty in haemophiliacs.

PURPOSE: Patients with haemophilia suffer from recurrent joint haemarthrosis. This can progress to symptomatic arthropathy commonly affecting the knee. While modern coagulation strategies have reduced those proceeding to end-stage arthropathy, total knee arthroplasty (TKA) remains the optimal treatment for some patients. Despite innovation in perioperative haematological management, concerns about the potential for excessive haemorrhage still exist. The aim of this study is to quantify immediate postoperative blood loss and haematological complications in haemophiliacs following TKA. METHODS: A retrospective study of patients with haemophilia types A or B who underwent a TKA over a 12-year period at a single institution was conducted. These patients were compared to both a non-haemophiliac control group and to published standards in non-haemophiliacs undergoing TKA. RESULTS: Twenty-one TKA procedures in 18 patients (72% haemophilia A, 28% haemophilia B) were suitable for inclusion with a mean age of 44 years. The mean haemoglobin drops at 24 and 48 h postoperatively were 2.7 g/dl and 3.8 g/dl respectively. There was no significant difference in haemoglobin drop at 48 h postoperatively when compared to the non-haemophiliac control group (P = 0.2644). There were no immediate perioperative complications and two patients (9.6%) required postoperative transfusion. CONCLUSION: Haemophiliacs undergoing a unilateral primary TKA in a specialised tertiary referral centre appear to have comparable rates of perioperative blood loss when compared to both a non-haemophiliac control group as and published haemostatic standards in non-haemophiliac patients following TKA. Perioperative management with expert orthopaedic and haematological input is recommended to optimise outcomes in this complex patient group.

Treatment of Recurrent Hemarthrosis Following Total Knee Arthroplasty Using Surgical Interventions.

BACKGROUD: Recurrent hemarthrosis following total knee arthroplasty (TKA) is a rare complication. Its pathophysiology and standard treatments have not yet been established. In this study, we report 7 cases of recurrent hemarthrosis after TKA in which failure of the initial conservative treatment was followed by angiographic embolization; in 1 of the 7 cases, arthroscopic electrocauterization was also performed after treatment failure with selective embolization. METHODS: From January 2015 to May 2018, 7 patients visited our hospital due to recurrent hemarthrosis after TKA. Their medical records and serologic test results were reviewed to check for the presence of any bleeding disorder and history of anticoagulant use. Implant malalignment and instability were checked using X-ray. In all cases, the conservative treatment failed, so interventional angiography with selective embolization was performed, which was also followed by arthroscopic electrocauterization if the outcome was unsatisfactory. RESULTS: The interval between TKA and the onset of hemarthrosis ranged from 3 to 76 months (average, 34.1 months). There was no coagulopathy and instability. All patients underwent conservative treatment at an interval of 4.3 months and the rate of relapse was 3.1 on average. On the interventional angiography, 6 cases showed vascular blush, and 1 case had pulsatile bleeding. The average duration for interventional angiography was 90.9 minutes. The average length of follow-up was 38.8 months. Embolization was successfully performed in 4 cases. In 2 of 3 failed cases, the symptoms improved without further treatment. In the remaining 1 failed case, the patient had a relapse of hemarthrosis, so an arthroscopic procedure was performed, which led to identification of the suspicious bleeding point by using preoperative angiographic findings. Electrocauterization was performed and active bleeding was stopped. All cases with recurrent hemarthrosis achieved improvement. CONCLUSIONS: Interventional angiography was used to aid in the diagnosis of recurrent hemarthrosis, and therapeutic selective embolization provided satisfactory clinical results. Even if selective embolization fails, interventional angiography may be helpful for further surgical procedures because it reveals vascular blush of a bleeding site. Therefore, interventional angiography and selective embolization should be considered to be a useful treatment for recurrent hemarthrosis after TKA.

Spontaneous atraumatic knee haemarthrosis.

Spontaneous knee haemarthrosis is a rare condition, most prevalent in the elderly with osteoarthritis. Recent reports have proposed that the source of bleeding is the peripheral arteries supplying the posterior horn of the lateral meniscus. In this case, a 62-year-old active man presented with acute postero-lateral left knee pain and swelling with limited weight bearing or movement of the knee. No recent history of trauma but history of lateral compartment dominant osteoarthritis and arthroscopic partial lateral meniscectomy of left knee. Aspiration showed a large haemarthrosis and following an MRI scan displaying large areas of full thickness chondral loss, complex tearing of lateral meniscus and loose bodies, the patient had an arthroscopy, washout, debridement of osteochondral tibial defect and diathermy of suspected bleeding point. This report supports the peripheral arteries supplying the posterior horn of the lateral meniscus as the source of bleeding in spontaneous haemarthrosis of the knee.

Geniculate Artery Embolization in Patients With Recurrent Hemarthrosis After Knee Arthroplasty: A Retrospective Study.

BACKGROUND: Recurrent hemarthrosis after knee arthroplasty is an uncommon and disabling complication of this frequently performed procedure. Selective endovascular embolization of the geniculate arteries is one of the therapeutic options to manage this complication. The purpose of this study is to analyze the effectiveness of this treatment in patients suffering from recurrent hemarthrosis after knee arthroplasty. METHODS: We performed a retrospective study of 31 patients (39 embolization procedures) with recurrent hemarthrosis after knee arthroplasty. There were 17 men and 14 women with a median age of 67 years (range 48-90). All patients were referred for geniculate artery embolization between January 2007 and November 2016. RESULTS: Twenty-seven procedures were executed on the right side and 12 on the left side. Total knee arthroplasty was performed on 29 patients, only 2 patients underwent unicompartmental knee arthroplasty. Embolization of the superior geniculate arteries was achieved in all patients. In 12 of 39 procedures (31%), at least 1 of the inferior geniculate arteries could not be catheterized, therefore embolization was achieved through collaterals. Symptomatic improvement was observed in 26 of 31 patients (84%). Discomfort or mild postprocedural pain was observed in most patients, needing only minor pain medication, mostly resolving within 24 hours. Two patients presented with a severe complication: a 48-year-old male patient developed septic arthritis and an 85-year-old hypertensive female patient treated with anticoagulants showed aseptic necrosis of the femoral condyles. CONCLUSION: Embolization of geniculate arteries is a safe and effective treatment in recurrent hemarthrosis post knee arthroplasty. Clinical improvement was seen in most patients.

Surgical approaches to hemophilic arthropathy.

: The role of the orthopedic surgeon is to use invasive and/or surgical methods to treat the musculoskeletal disorders suffered by persons with hemophilia, always within the context of a multidisciplinary team. Muscle hematomas must be diagnosed as early as possible and be subjected to continuous treatment until full resolution, as they are associated with the risk of severe complications (compartment syndromes and pseudotumors). Arthrocentesis (extraction of intra-articular blood) is recommended in cases of acute and profuse hemarthrosis. Synovectomy is mandatory in the case of synovitis. Radiosynovectomy plays a key role as it has been shown to reduce bleeding by 65%. Our department uses Yttrium-90 in knees and Rhenium-186 in elbows and ankles. Radiosynovectomy is our treatment of choice for synovitis whereas arthroscopic synovectomy is resorted to as second-line treatment. Total knee replacement (TKR) has shown itself to be effective for treating severe hemophilic arthropathy, although the infection risk in patients with hemophilia is higher than in patients with osteoarthritis (1-2 vs. 7%).

Acute Compartment Syndrome After Knee Synovectomy in a Patient with Hemophilia A: A Case Report.

CASE: A 19-year-old man with severe hemophilia A underwent open surgical synovectomy to treat recurrent hemarthrosis of the knee. He developed symptoms and signs of acute compartment syndrome (ACS) despite receiving full-dose factor replacement. Arthrotomy was performed twice, and massive hemarthrosis was evacuated. The symptoms subsided after supplementing clotting factor with prednisone. CONCLUSIONS: ACS may complicate hemophilia. Diagnosis is mainly based on clinical judgment. Proper factor replacement is the mainstay of treatment. Surgery is reserved for patients with no improvement following factor administration.

Accelerating recovery from acute hemarthrosis in patients with hemophilia: the role of joint aspiration.

: Arthrocentesis of an acute hemarthrosis in hemophilia remains a controversial issue. The purpose of this study is to define the role that joint aspiration can play in the recovery from acute hemarthrosis in patients with hemophilia. The study sample included 33 hemophilic patients (55 joints) with acute elbow, knee, and ankle hemarthrosis as confirmed by ultrasonography. Patients were distributed into a treatment group and a control group. Patients in the first group were subjected to joint aspiration, whereas patients in the second were not. Arthrocentesis was carried out immediately after diagnosis of acute hemarthrosis in liquid phase. Patients were infused with the deficient coagulation factor and were instructed to observe relative rest until resolution of hemarthrosis. The following parameters were analyzed: time to full resolution of hemarthrosis (determined by ultrasonography), duration of treatment with the deficient coagulation factor, time to pain relief, time to recovery of prebleed range of motion, and time to resumption of school/work (all of these measured in days). The joints treated with joint aspiration exhibited a significantly faster resolution of bleeding (fewer days). In addition, this group required fewer days of pharmacological treatment, with faster achievement of functional recovery and resumption of school/work activities. No complications were observed. This study shows that joint aspiration under hemostatic cover and in strictly aseptic conditions is a well-tolerated technique that makes the recovery of acute hemarthrosis of hemophilic patients faster.

Risks and patient outcomes of surgical intervention for hemophilic arthropathy.

INTRODUCTION: Surgical interventions usually needed for hemophilic arthropathy include arthroscopic synovectomy, alignment osteotomy, arthroscopic joint debridement, radial head removal, arthrodesis of the ankle, and total joint arthroplasty (elbow, hip, knee, ankle). Areas covered: Whatever the surgical technique, appropriate surgical hemostasis must be achieved through infusion of concentrate of the deficient factor (factor VIII or factor IX), either in recombinant or plasma-derived form, at the correct dosage (ideally for 10-14 days). In patients with inhibitors, there are also the aPCCs (activated prothrombin complex concentrates) and rFVIIa (recombinant activated FVII). These surgical procedures are safe even in the most complex cases such as patients with inhibitors or who are human immunodeficiency virus-positive (HIV+) and hepatitis C virus-positive (HCV+). Expert opinion: The risk of bleeding in surgical procedures is higher for hemophilic patients than for other patients and there is also a greater risk of infection. Both these factors increase the risk of a poor outcome. Orthopedic surgery in hemophilia improves the patient's quality of life.

Anterior osteophyte resection of the ankle joint to increase range of motion in haemophilic ankle arthropathy.

INTRODUCTION: Haemophilic ankle arthropathy may cause joint bleeding, pain, stiffness, erosion and progressive motion limitations, including a decreased range of motion (ROM) secondary to anterior osteophyte impingement. AIM: The purpose of this study was to investigate changes in the ROM and symptoms after synovectomy and anterior osteophyte resection for haemophilic ankle arthropathy. METHODS: We retrospectively reviewed 41 ankles in 35 patients with haemophilic arthropathy who underwent arthroscopic and/or open synovectomy. The mean follow-up period was 59.5 months. Indications for the procedure included a lack of improvement with conservative management, and the presence of haemarthrosis and arthralgia. We assessed radiographic images, ankle joint range of motion, the American Orthopaedic Foot and Ankle Society (AOFAS) Ankle-Hindfoot score and patients' subjective satisfaction preoperatively and at the last follow-up. RESULTS: Arthroscopic synovectomy was performed in 32 ankles, open synovectomy in 6, and arthroscopic synovectomy with additional mini-open debridement in 3. Ankle dorsiflexion improved from a mean of 2.7° preoperatively to a mean of 7.5° at the final follow-up (P = 0.003), whereas plantar flexion improved from a mean of 30.7° preoperatively to a mean of 32.7° at the final follow-up (P = 0.276). The AOFAS score improved from 62.2 preoperatively to 79.1 at the final follow-up (P < 0.001). Patient satisfaction was "excellent" in 11 ankles, "good" in 22, "fair" in 3 and "poor" in 5. CONCLUSION: Arthroscopic and/or open synovectomy with anterior osteophyte resection and early rehabilitation in adolescents and adults with haemophilic ankle arthropathy showed improved ankle dorsiflexion and AOFAS scores.

Long-term results of total knee arthroplasty in hemophilic arthropathy.

PURPOSE: Knee arthropathy is a frequent complication affecting hemophilic patients, which can cause severe pain and disability. When conservative measures fail, total knee arthroplasty (TKA) may be performed. METHODS: Eighteen TKA were performed in 15 patients with hemophilia during a 24-year period in a Hemophilia Comprehensive Care Center. All patients were evaluated by a multidisciplinary team constituted by a hematologist, an orthopedic surgeon, and a physical medicine and rehabilitation physician. Mean follow-up was 11.3 years. RESULTS: Ten-year survival rate with prosthesis removal as end point was 94.3%. At last, follow-up visual analog pain scale score was 3.2 points, knee osteoarthritis outcome scale was 86.7 points, and mean range of motion was 88°. Only two patients required perioperative transfusion. Complication rate was 27.8% and included two infections, two prosthesis stiffness, and one case of recurrent hemarthrosis. CONCLUSION: After appropriate medical optimization and with prompt rehabilitation, TKA can be performed in hemophilic patients with good clinical results and survivor rates comparable to nonhemophilic patients.

Recurrent Hemarthrosis After Total Knee Arthroplasty: Evaluation and Treatment.

Recurrent hemarthrosis (RH) is a rare complication (∼1%) after total knee arthroplasty (TKA). It is a complex and particularly frustrating problem for both patient and surgeon. Typically, patients present several months to years after their index TKA surgery with a painful and swollen joint. Although conservative management may provide temporary relief, the rate of recurrence is high. Despite extensive case series in the literature, no consensus was made on the criteria needed to establish this diagnosis, or how to best provide treatment. Several management strategies have been described for RH, including immobilization, arthroscopic versus open synovectomy, angiographic embolization, and revision arthroplasty. All of these have demonstrated variable effectiveness, with limited evidence for their use in specific situations. This review synthesizes the available literature and suggests an algorithm for the diagnosis and treatment of RH after TKA.

Hindfoot malalignment in adults with haemophilic ankle arthropathy: The importance of early detection and orthotic treatment.

INTRODUCTION: Haemophilic arthropathy (osteoarthritis secondary to haemophilia) of the ankle may result in painful hindfoot malalignment. PURPOSE: To analyse hindfoot alignment in subjects with haemophilic arthropathy of the ankle and evaluate the response (improvement of pain, function and alignment) to the orthotic treatment prescribed in patients with malalignment. METHODS: The study included 163 patients with haemophilia, all of them over 16 years of age. Hindfoot alignment and footprint were analysed in patients with and without haemophilic arthropathy of the ankle (as determined by the Pettersson score). Response to the use of an orthosis was evaluated at 6 months by means of the AOFAS Ankle-Hindfoot Scale. RESULTS: Fifty-six (59.5%) patients with haemophilic arthropathy presented with concomitant  hindfoot malalignment. The most common abnormality was a valgus alignment combined with a neutral footprint. In 14 cases, valgus was associated with a pes planus or a pes cavus. Only 5 patients without haemophilic arthropathy (7.2%) presented with some form of malalignment. The differences between the groups were statistically significant. The probability of having malalignment increased with the degree of arthropathy. Patients with haemophilic arthropathy and malalignment were treated with an orthosis, with insoles as the most commonly used alternative (86%). Such treatment significantly improved patients' pain and function-related scores on the AOFAS Ankle-Hindfoot Scale. CONCLUSION: Given the high rates of valgus malalignment in subjects with haemophilic arthropathy of the ankle, and the good response rates obtained following individualised orthotic treatment, it seems reasonable to routinely evaluate hindfoot alignment in this group of patients.