Rates of venous thromboembolism and use of thromboprophylaxis after major orthopedic surgery in patients with congenital hemophilia A or B: a systematic review.

BACKGROUND: Venous thromboembolism (VTE) is a well-recognized complication after total joint replacement (TJR). Persons with hemophilia A or B are considered at low postoperative VTE risk due to their coagulation factor deficiencies, and administering pharmacologic thromboprophylaxis is often considered contraindicated. However, using factor replacement therapy could increase the postoperative VTE risk. OBJECTIVES: To analyze best available evidences of VTE rates in persons with hemophilia A or B undergoing lower limb TJR and the use of postoperative pharmacologic thromboprophylaxis. METHODS: We systematically screened 4 online biomedical databases to identify studies reporting VTE rates in patients with hemophilia after TJR. Case reports and case series with less than 10 patients were excluded. RESULTS: Twenty-six observational studies were included in this systematic review, reporting 1181 TJRs in patients with hemophilia A or B. Eight studies had VTE rates as the primary outcome. Five studies reported screen-detected VTE, while 21 reported symptomatic VTE events. Overall, 17 VTE events were reported (1.4%; 95% CI, 0.9%-2.3%), including 10 (6.6%) after 151 surgeries with postoperative VTE screening and 7 (0.7%) after 1080 surgeries without postoperative screening. Thromboprophylaxis protocols were specified in 21 studies; postoperative thromboprophylaxis was used in 15 (1.3%) surgeries. This information was not available for 29.0% of the analyzed population. CONCLUSION: Despite the low thromboprophylaxis use in patients with hemophilia, rates of symptomatic VTE after TJR appeared to be low. We also highlighted the need to better report the thrombotic outcome in persons with hemophilia to face the ongoing changes in the hemophilia landscape.

A single-centre experience of 29 total ankle replacement in haemophiliac patients: Therapeutic management, factor consumption and cost.

INTRODUCTION: In patients with haemophilia, repeated bleeding in large joints leads to chronic haemophilic arthropathy, a rare disease that can be managed surgically with ankle arthrodesis or with total ankle replacement (TAR). TAR has been reported to provide good surgical results in the medium/long-term and allow preservation of joint mobility but the medical therapeutic management of the patients has not been described. AIM: To describe the medical therapeutic management of TAR. METHODS: All patients with haemophilia A/B, with haemophilic ankle arthropathy, and who underwent TAR between April 2006 and October 2019 were retrospectively included. Factor consumption, perioperative and early complications, volume of blood lost, and orthopaedic data were collected. RESULTS: A total of 25 patients underwent 29 TAR (mean age was 44.7 years [range: 26-65]). In the 17 patients with HA without history of anti-FVIII inhibitor, the mean ± SD consumption the day of surgery was 116 ± 16 UI/kg when clotting factors were administered by continuous infusion, 106 ± 13 UI/kg when SHL factors were administered by bolus infusion, and 75 ± 22 UI/kg when EHL factors were administered by bolus infusion. During hospitalisation, the mean factor cost was €38,073 (83.7% of the total cost of surgery). Mean blood loss was significantly lower in patients treated with tranexamic acid (164 mL, range: 40-300) than in those not (300 mL, range: 70-800; p = .01). Six patients had haematoma. The 10-year survival free of any prosthesis removal/arthrodesis was estimated to be 92.2% (95% CI [83; 100]). CONCLUSION: The medical therapeutic management of TAR is complex, carried out by a multidisciplinary team but effective in avoiding the occurrence of complications.

[Profuse Hemorage During Open Heart Surgery in a Patient Late After Liver Transplantation and Remission Stage Acquired Hemophilia].

A 72-year-old male presented with severe mitral regurgitation, moderate tricuspid regurgitation, and chronic atrial fibrillation. One month prior, he encountered difficulties with dialysis and was subsequently referred to our department for cardiac surgery. The patient's medical history includes living-donor liver transplantation for type C cirrhosis associated with acquired hemophilia A. The preoperative liver function was categorized as Child-Pugh grade B, with a model for end-stage liver disease( MELD) score of 23. His factor Ⅷ activity was close to the lower limit of the normal range. The patient underwent mitral valve replacement, tricuspid valve repair, and left atrial appendage closure. Initially, he experienced intractable bleeding, but hemostasis was easily achieved after administrating a factor Ⅷ preparation. Upon admission to the intensive care unit, his factor Ⅷ clotting activity was slightly below the normal range. Therefore, in cases where a patient with remission stage hemophilia A resulting in liver transplantation undergoes open heart surgery, it is crucial to have coagulation factor medication readily available, regardless of normal preoperative factor Ⅷ levels.

In the era of primary prophylaxis in hemophilia, what types of orthopedic surgical interventions have been published in the 2020-2023 period and in which countries?

INTRODUCTION: Primary prophylaxis has significantly reduced the number of orthopedic surgical procedures performed on patients with hemophilia (PWH) worldwide. However, studies on orthopedic surgery in PWH are still being published in the medical literature. AREAS COVERED: The aim of this article is to determine the types of orthopedic surgical interventions in PWH published between 2020 and 2023 and the countries in which they are published. EXPERT OPINION: The following orthopedic surgical procedures are still performed on PWH: total knee, ankle, elbow and hip arthroplasty, ankle fusion, ankle distraction, and the surgical removal of hemophilic pseudotumors. The countries in which articles on orthopedic surgery in hemophilia have been published in the period 2000-2023 include China (14 articles); Republic of Korea and U.S.A. (3 articles each); Germany, Italy, Japan, Poland and Turkey (2 articles each); and 1 article each in the following countries: Austria, Belgium, Chile, France, Ireland, New Zealand, Russia, and The Netherlands. These data suggest that primary prophylaxis should be improved and extended to all patients globally.

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.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Orthopedic surgical procedures in people with hemophilia.

People with hemophilia tend to develop joint lesions secondary to the recurrent hemarthroses typical of their condition. These usually include chronic synovitis and arthropathy chiefly affecting their ankles, knees, and elbows. In addition, muscular hematomas, albeit less frequently, may also result in complications such as acute compartment syndrome, pseudotumors, bone cysts and peripheral nerve compression. Joint lesions may require some of the following surgical interventions: arthroscopic synovectomy (in cases of synovitis), arthroscopic joint debridement, radial head resection, opening-wedge tibial osteotomy, arthrodesis, arthrodiastasis (of the ankle), tendon lengthening (hamstrings, Achilles tendon), progressive extension of the knee by placing an external fixator in cases of flexion contracture of the knee, supracondylar femoral extension osteotomy in cases of knee flexion contracture and, eventually, a total joint arthroplasty when the affected joint has been destroyed and the patient experiences severe joint pain. Total knee arthroplasty in hemophilic patients is associated with a high infection risk (7% on average). As regards the complications following muscle hematomas, acute compartment syndrome requires urgent performance of a fasciotomy when hematological treatment is incapable of resolving the problem. Surgical resection of hemophilic pseudotumors is the best solution, with those affecting the pelvis (secondary to iliopsoas hematomas) being particularly difficult to resolve. Peripheral nerve lesions can often be effectively addressed with hematological treatment, although a surgical neurolysis of the ulnar nerve is indicated if nonoperative treatment fails.

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.

The effect of HIV prevalence, CD4 counts and disease severity on the outcome of total knee arthroplasty for haemophilic arthropathy: a systematic review and meta-analysis.

PURPOSE: End-stage knee arthropathy is a recognised complication of haemophilia. It is often treated by total knee arthroplasty (TKA), which is more technically challenging in patients with haemophilia (PwH). It remains unclear what factors may predict implant survivorship and deep infection rate. Therefore, we systematically review the evidence regarding TKA survivorship and infection in PwH, compared to the general population, and determine the important factors influencing survivorship, particularly HIV and CD4 + count. METHODS: A systematic literature review was conducted using MEDLINE, EMBASE, and PubMed for studies reporting Kaplan-Meier survivorship for TKA in PwH (PROSPERO CRD42021284644). Meta-analysis was performed for survivorship, and the results compared to < 55-year-olds from the National Joint Registry (NJR). Meta-regression was performed to determine the impact of relevant variables on 10-year survivorship, with a sub-analysis focusing on HIV. RESULTS: Twenty-one studies were reviewed, totalling 1338 TKAs (average age 39 years). Implant survivorship for PwH at 5, 10, and 15 years was 94%, 86%, and 76% respectively. NJR-reported survivorship for males < 55 years was 94%, 90%, and 86%. Survivorship improved over time (1973-2018), and correlated inversely with HIV prevalence. Infection rate was 5%, compared to 0.5-1% in the NJR. Infection was not significantly increased with higher HIV prevalence, and CD4 + count had no effect. Complications were inconsistently reported. CONCLUSION: Survivorship was similar at 5 years but declined thereafter, and infection rate was six-fold higher. HIV was related to worse survivorship, but not increased infection. Meta-analysis was limited by inconsistent reporting, and standardised reporting is required in future studies.

Perioperative Outcomes of Patients with Bleeding Disorders Undergoing Major Surgery at an Academic Hemophilia Treatment Center.

Persons with bleeding disorders (PwBD) are at high risk for bleeding with invasive procedures. However, the risk of bleeding in PwBD undergoing major surgery and outcomes of patients managed perioperatively at a hemophilia treatment center (HTC) are not well described. We performed a retrospective review of surgical outcomes among PwBD undergoing major surgery between January 1st, 2017 and December 31st, 2019 at the Cardeza Foundation Hemophilia and Thrombosis Center in Philadelphia, PA. The primary outcome was postoperative bleeding, assessed according to the ISTH-SSC's 2010 definition. Secondary outcomes included use of unplanned postoperative hemostatic therapy, LOS, and 30-day readmission rate. Results were compared to non-PwBD population from a surgical database, matched for surgery, age, and sex. During the study period, 50 PwBD underwent 63 major surgeries. The most common diagnoses were VWD (64%) and hemophilia A (20.0%). The most common surgical procedure category was orthopedic (33.3%), predominantly arthroplasties. Postoperatively,4.8% of procedures were complicated by major bleeding and 1.6% by non-major bleeding. The mean LOS was 1.65 days, and 30-day readmission rate was 1.6%. In comparison to matched, non-PwBD patients in a national surgical database undergoing the same procedures, study patients had a similar rate of bleeding complications per procedure (5.0% vs 1.04% P = .071, Fisher's exact test). PwBD undergoing major surgeries have low rates of major bleeding when receiving comprehensive care at an HTC. Bleeding and hospital readmission rates were similar to non-PwBD baseline in a large database.

Perioperative continuous infusions of factor VIII versus factor IX for patients with hemophilia A or B undergoing major surgery.

Continuous factor VIII (FVIII) or factor IX (FIX) infusions are commonly used for patients with hemophilia A (HA) or B (HB) undergoing surgery to secure perioperative hemostasis. To describe differences between the initial recovery and subsequent FIX and FVIII levels, and describe clinical outcomes among HB and HA patients receiving perioperative continuous infusion (CI) of recombinant FVIII and FIX concentrates. Retrospective chart review was conducted on 8 consecutive patients with HB and 7 consecutive patients with HA who underwent major surgery between 2014 and 2018 and received continuous infusions of standard half-life factor concentrate. Median initial bolus dose per kilogram was higher for HB compared to HA patients [90.8 (IQR 78.0-98.7) vs. 52.1 (IQR 48.6-55.6) IU/kg], while initial CI dose-rates were similar [4.3 (IQR 3.8-4.6) vs. 4.2 (IQR 3.8-4.4) IU/kg/h]. Median post-bolus recovery was higher for FVIII compared to FIX [1.70 (IQR 1.23-1.75) vs. 0.88 (IQR 0.75-1.00) IU/mL]. Median factor levels also were higher for FVIII on post-operative days 1 to 3. HB patients had greater mean intraoperative estimated blood loss [285.7 (range 0-1000) vs. 142.8 (range 0-400) mL] and longer median length of hospital stay [9 (IQR 8-12) vs. 5 (IQR 4-6.5) days]. Our initial evidence suggests greater in vivo yield of rFVIII compared to rFIX in the perioperative setting. We identified poorer clinical outcomes in this small cohort of perioperative HB patients indicating that they may benefit from a higher CI rate for adequate surgical hemostatic coverage.

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

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

Perioperative blood loss during joint replacement: comparison between patients with and without hemophilia.

BACKGROUND: Although arthroplasty provides satisfactory pain relief, functional improvement, and reduced flexion contracture in patients with hemophilia arthropathy, bleeding remains the primary problem associated with hemophilic arthropathy joint replacement. We aimed to explore the differences in perioperative blood loss (PBL) associated with joint replacement surgery in patients with and without hemophilia. METHODS: This study retrospectively analyzed 61 cases of PBL in patients undergoing joint replacement surgery, including 37 patients with hemophilia and 24 patients without hemophilia. All patients demonstrated severe joint flexion contractures that seriously affected their daily lives and required surgical intervention. PBL was compared between the two groups. RESULTS: In univariate analysis, the overall (p < 0.001) and hidden (p < 0.001) blood losses were significantly higher for patients with hemophilia than those for patients without hemophilia. However, after adjustment for multiple factors, there was no significant difference in overall blood loss between the two groups (p = 0.731). In addition, sex, age group, and surgical site did not affect blood loss in patients with hemophilia. CONCLUSION: Overt bleeding did not increase significantly in patients with hemophilia, compared with that in patients without hemophilia. In terms of blood loss, joint replacement surgery for patients with hemophilia is relatively safe. The results of this study must be verified by a prospective follow-up study with larger sample size. Trial registration Retrospectively registered.

Surgical outcomes in patients with haemophilia A or B receiving extended half-life recombinant factor VIII and IX Fc fusion proteins: Real-world experience in the Nordic countries.

INTRODUCTION: Perioperative dosing recommendations vary across Nordic haemophilia treatment centres (HTCs) for extended half-life (EHL) factor concentrates in haemophilia A/B (HA/HB) patients. AIM: To summarise Nordic real-world surgical experiences with EHL recombinant factor VIII/IX Fc (rFVIIIFc/rFIXFc) fusion proteins using retrospective data from clinical records at four HTCs in Finland, Sweden and Norway. METHODS: Factor dosing and surgical outcomes were recorded from HA/HB patients who underwent surgery and were treated with rFVIIIFc/rFIXFc. Perioperative factor dosing regimens were clinician-determined based on local practises. RESULTS: Twenty five surgeries were performed on 20 patients, all covered by bolus injections except one minor HA surgery; eight minor surgeries were in paediatric patients. Median preoperative rFVIIIFc dose for major HA surgeries (n = 8) was 48 IU/kg (range: 35-57), with total consumption up to Day 14 of 427 IU/kg (196-568). For the two major HB surgeries (in one patient), preoperative rFIXFc doses were 50 IU/kg and 20 IU/kg; total consumption up to Day 14 was 130 IU/kg and 40 IU/kg. Median preoperative rFVIIIFc/rFIXFc bolus doses for minor HA (n = 10) and HB (n = 4) surgeries were 50 IU/kg (24-79) and 47 IU/kg (40-71), with total consumption up to Day 5 of 138 IU/kg (49-404) and 100 IU/kg (43-125), respectively. Intraoperative and postoperative haemostatic responses were rated as at least good/excellent for 24/25 surgeries, with bleeding episodes reported in only three surgeries. CONCLUSION: Nordic real-world experiences suggest that EHL products can be used safely and effectively for peri-operative haemostasis. Further research is required to develop local dosing guidelines for optimised treatment schedules.

Ankle arthrodesis in patients with haemophilia-associated ankle arthropathy - does the technique influence the outcome?

Management of symptomatic osteoarthritis (OA) of the ankle in patients with haemophilia can be challenging. Arthroscopic ankle arthrodesis has been shown in non-haemophiliac patients to provide similar or superior rates of fusion to open ankle fusion. However, the literature regarding ankle arthrodesis in patients with haemophilia is limited. Our aim was to compare the rate of successful fusion between open and arthroscopic assisted ankle arthrodesis in patients with haemophilia. A retrospective study was performed. All patients with haemophilia who underwent ankle arthrodesis at our centre were included. Outcomes including peri- and post-operative complications, and lengths of stay were extracted from patients' records. Radiographs were reviewed for signs of successful arthrodesis. Seventeen arthrodesis procedures were performed in 13 patients between 1980 and 2017. Nine procedures were performed arthroscopically and eight were open. Ten patients were diagnosed with haemophilia A and three with haemophilia B. The success rates of arthroscopic and open tibiotalar arthrodesis were 100% and 87.5% respectively. Four complications occurred. In the open technique group, there was one non-union. The same patient also developed subsequent haematoma after revision surgery. One patient developed a superficial wound infection which resolved with antibiotics. In the arthroscopic group, one patient developed a pseudoarthrosis of the distal tibiofibular joint which required a revision procedure. The results of this study suggest that arthroscopic ankle fusion for haemophilia- associated arthropathy is a viable option, with the rate of successful fusion being comparable to open procedures.

[Treatment of chronic refractory wound with autologous platelet-rich plasma in a hemophilia A patient after knee joint surgery].

On December 22, 2017, a 35-year-old male hemophilia A patient with a secondary chronic refractory wound after left knee joint surgery was transferred from the Department of Hematology of Maoming People's Hospital to the Department of Burns and Plastic Surgery in the same hospital. The physical examination revealed that the patient's left knee joint was swollen, with a full-thickness skin defect wound of 4 cm×4 cm on the lateral side of the joint and a large number of dark red blood clots at the bottom of the wound. The wound bleeding was controlled by intravenous infusion of plasma, cryoprecipitate, and human coagulation factor Ⅷ. After con- ventional debridement and dressing changes until the wound infection was controlled and necrotic tissue was removed, a subcutaneous cavity wound of 2 cm×2 cm in area and 3 cm in depth remained in the left knee joint and was difficult to heal. Nineteen days after transfer, the patient received autologous platelet-rich plasma (PRP) treatment, and 32 days after PRP treatment, the wound in left knee joint was healed with epithelialization. This case suggests that autologous PRP therapy would be a good option for hemophilia complicated chronic refractory wounds when they could not be repaired by surgery.

Risk factors for increased perioperative blood loss during total knee arthroplasty in patients with haemophilia.

INTRODUCTION: Currently, there is a paucity of literature describing the risk factors for increased perioperative blood loss (PBL) during total knee arthroplasty (TKA) in haemophilia. Furthermore, no studies have analysed coagulation factor levels that affect PBL. AIM: To identify the risk factors for increased PBL by incorporating coagulation factor levels following TKA in patients with haemophilic arthropathy. METHODS: A total of 92 TKA (78 haemophilia A and 14 haemophilia B) were performed for haemophilic arthropathy. PBL was calculated using the haemoglobin (Hb) balance method, and patients were categorised into two groups: group H (higher blood loss than the mean PBL, n = 36) and group L (lower blood loss than the mean PBL, n = 56). Body mass index (BMI), operation day Hb, haematocrit and coagulation factor level (VIII or IX) were analysed, including demographic and laboratory data. RESULTS: The mean PBL volume during TKA for haemophilic arthropathy of the knee was 542.3 ± 361.7 mL. Multivariate analysis revealed that lower haematocrit on the operation day (odds ratio [OR], .633; 95% confidence interval [CI], .511-.786; p < .001) and coagulation factor level (OR .970, CI .941-.999; p = .046) were independent risk factors for increased PBL. Receiver-operating characteristic analysis identified these cutoff values for predicting increased PBL: operation day coagulation factor level 93.5% (sensitivity, 70.4%; specificity, 67.6%) and haematocrit level of 38.2% (sensitivity, 85.7%; specificity, 80.6%). CONCLUSIONS: The PBL increased as haematocrit and coagulation factor levels decreased on the operation day. A coagulation factor level <93.5% or haematocrit level of <38.2% may be a significant risk factor for increasing PBL. LEVEL OF EVIDENCE: Level IV, Case Series, Treatment Study.

Management of patients with inherited bleeding disorders in oral surgery: A 13-year experience.

INTRODUCTION: The management of patients with inherited bleeding disorders in oral surgery requires the systematic evaluation of bleeding risk and the setting up of an adequate treatment protocol by the referring haematologist, defining a replacement therapy and a recommended length of hospital stay. The purpose of this study was to determine the bleeding risk associated with oral surgery for each type of inherited bleeding disorder and to evaluate the efficacy of the treatment protocols set up in our tertiary care center. MATERIALS AND METHODS: We included all patients with an inherited bleeding disorder, managed in our oral and maxillofacial surgery department with a treatment protocol set up by our local Haemophilia Treatment Centre for an oral surgical procedure. RESULTS: Between January 2006 and December 2018, 295 treatment protocols were set up for the management of patients with haemorrhagic risk in oral surgical procedures. Of these, 203 were scheduled to take place in our department. A total of 180 oral surgical procedures for 147 patients were included. The incidence of bleeding complications in our study was 4.44% (8 out of 180) with a significantly higher risk in patients with haemophilia, the mean time to onset of bleeding was 11 days. DISCUSSION: The use of a treatment protocol for the management of patients with inherited bleeding disorders in oral surgery seems effective. Our rate of bleeding complications was comparable to data in the literature, and often lower.

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.

National survey of hepatobiliary and pancreatic surgery in hemophilia patients in Japan.

BACKGROUND: Approximately 8300 hemophiliacs are registered in Japan, but no comprehensive reports on hepatobiliary and pancreatic surgery (HBPS) have been conducted. This report investigates the current status of HPBS in hemophilia patients in Japan. METHODS: The subjects were hemophiliac patients seen between January 1 2007, and December 31 2017, at facilities participating in this study among the facilities for performing high-difficulty cases nationwide designated by the Japanese Society for HBPS. A retrospective examination of short-term outcomes in 49 cases was conducted to assess patient background, disease, surgical procedure, and complications. RESULTS: The types of hemophilia were A: 43 cases, B: four cases, and von Willebrand disease: two cases (hemophilia severity: mild 32, moderate seven, severe 10). The target malignant diseases for surgery were hepatocellular carcinoma (HCC) in 20 cases, intrahepatic cholangiocellular carcinoma (CCC) in four cases, combined HCC-CCC in two cases, hilar CCC in two cases, and pancreatic cancer in four cases. As for the surgical procedure, limited resection (subsegmentectomy and partial hepatectomy) was performed in 16 cases of HCC even with normal liver function tests. Pancreaticoduodenectomy and distal pacreatectomy were performed for pancreatic cancers as in the standard procedure. Postoperative complications were postoperative bleeding in two cases after hepatectomy and one after pancreatectomy in one case. When compared with Japanese National Clinical Data base, the complication rates after hepatectomy and pancreatectomy were not conspicuous in hemophilic patients. CONCLUSIONS: As long as they are performed in qualified centers, complication rate is not increased in hemophilic patients undergoing HBPS.

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

BACKGROUND: 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). METHODS: 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. RESULTS: 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 12 weeks after surgery, whereas one still showed non-union after 6 months. 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. CONCLUSION: 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.