Orthopaedic Surgery In Haemophilia Research Articles

The role of orthopaedic surgery in haemophilia: current rationale, indications and results.

The musculoskeletal problems of haemophilic patients begin in infancy when minor injuries lead to haemarthroses and haematomas.Early continuous haematological primary prophylaxis by means of the intravenous infusion of the deficient coagulation factor (ideally from cradle to grave) is of paramount importance because the immature skeleton is very sensitive to the complications of haemophilia: severe structural deficiencies may develop quickly.If primary haematological prophylaxis is not feasible due to expense or lack of venous access, joint bleeding will occur. Then, the orthopaedic surgeon must aggressively treat haemarthrosis (joint aspiration under factor coverage) to prevent progression to synovitis (that will require early radiosynovectomy or arthroscopic synovectomy), recurrent joint bleeds, and ultimately end-stage osteoarthritis (haemophilic arthropathy).Between the second and fourth decades, many haemophilic patients develop articular destruction. At this stage the main possible treatments include arthroscopic joint debridement (knee, ankle), articular fusion (ankle) and total joint arthroplasty (knee, hip, ankle, elbow).Cite this article: EFORT Open Rev 2019;4:165-173. DOI: 10.1302/2058-5241.4.180090

The role of recombinant activated factor VII in the haematological management of elective orthopaedic surgery in haemophilia A patients with inhibitors.

The clinical profile and expectations of haemophilic patients with inhibitors have changed over the last three decades, mainly because of the prolongation of life-expectancy, often resulting in an increase of the orthopaedic burden. Recombinant activated factor VII (rFVIIa) is the most frequently used bypassing agent in haemophilia patients with inhibitors during elective orthopaedic surgery. For nearly 30 years, rFVIIa has been successfully used to control haemostasis in several major and minor surgical procedures. Clinical trials, case series, reports and surveys were progressively aimed at optimising rFVIIa usage in very demanding conditions managed in highly specialised centres. Recommendations from consensus opinions and guidelines have been provided on the basis of this clinical experience.

Elective total knee arthroplasty in haemophilic patients. Proposal for a clinical pathway.

Total knee arthroplasty (TKA) provides significant pain relief and better function in patients with end-stage haemophilic knee arthropathy. Peri- and postoperative care tends to be more complex than in non-haemophilic patients undergoing TKA and requires a multidisciplinary team approach. The implementation of standardized clinical pathways in non-haemophilic patients undergoing TKA has been shown to increase quality of care and to reduce postoperative complication rates. Consequently, the use of clinical pathways in haemophilic patients undergoing TKA may be beneficial to this particular subpopulation of patients. A clinical pathway for TKA for haemophilic patients was designed in a consensus process involving all participating departments. We propose a specifically adjusted clinical pathway for TKA for haemophilic patients to show that standardization of elective orthopaedic surgery in haemophilia is feasible. The authors emphasize that there are limitations on categorizing haemophilic patients and stress that individual interdisciplinary treatment should take precedence over a standardized approach.

Aspects of current management: orthopaedic surgery in haemophilia

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

Operative orthopädische Hämophilie behandlung

SummaryIn particular health economists promise to improve healthcare processes, cost-effectiveness and outcomes by standardised procedures. In the area of EOS indication, operative procedures and postal-surgical pain therapy can partly be standardised. In addition, clinical pathways have impact on the organisation of care if the care process is structured in a standardised way. On these four subjects examples are demonstrated. Elective orthopaedic surgery in haemophilia can be standardised. Nevertheless, it may not be forgotten that these patients can be categorised only restrictedly and the individual, interdisciplinary treatment must be prior to clinical standards.

Orthopaedic surgery in haemophilia--The Tel Hashomer experience: a tribute to Professor Henri Horoszowski.

Orthopaedic surgery in haemophilia--The Tel Hashomer experience: a tribute to Professor Henri Horoszowski.