Evidence-Based Management of the Knee in Hemophilia

Abstract

➢ Hemophilia is among the commonest bleeding disorders encountered in orthopaedic practice and results from an X-chromosome-linked failure of coagulation factors VIII (hemophilia A) or IX (hemophilia B).➢ Morbidity in hemophilia is a result of repeated hemarthroses, and the aim of orthopaedic management is to prevent these, to treat them promptly, and to address the joint pathology that results.➢ Both radiosynovectomy and arthroscopic synovectomy are effective in reducing the degree of synovitis and the number of hemarthroses, although, to our knowledge, there is little evidence that it prevents progression to end-stage arthritis. There is substantial evidence for the safety and effectiveness of radiosynovectomy, and this is the first line of treatment in our practice.➢ In end-stage disease, total knee arthroplasty is challenging as a result of severe contractures and is associated with a higher rate of complications such as infection (7% compared with 1% to 2% in osteoarthritis) and need for blood transfusions and a higher revision rate than that seen in total knee arthroplasty for osteoarthritis.➢ However, many series of total knee arthroplasty for hemophilia have now been published, and excellent results can be gained in terms of pain relief and quality of life.

Analysis of the Early Clinical Effect of Simultaneous Ipsilateral Total Hip and Knee Arthroplasty in the Treatment of End-stage Hemophilic Arthritis

“…Some studies have shown that 90% of hemophiliacs develop HA in the second and third decade of their lives [4,10] Total joint arthroplasty is the gold standard for the treatment of advanced hemophilic hip and knee arthritis. Carulli et al and Liddle et al [6,11] reported 23 patients with hemophilic hip arthritis who were treated with THA, with an average follow-up of 8.4 years. When discharged, all patients were able to walk with full weight.…”

Hemofili A Hastalarındaki Diz Artropatisi ve Bilateral Total Diz Artroplastisi Oranı

“…5 If the factor concentration is between 1 and 5%, it is considered as moderate and if below 1%, named as severe. 4,5 Haemarthrosis can develop as spontaneous or trauma-related and the presence of blood in the joint may provoke syn-ovial tissue reaction, this may eventually lead to cartilage damage and the progression of the damage can cause hemophilic arthropathy. 1,6 Plane x-rays may show subchondral irregularities, joint space loss and osteophytes.…”

“…[1][2][3] One of the most common complications is musculoskeletal bleeding and especially in moderate and severe forms, it leads to intraarticular bleeding (haemarthrosis). 4 Hemophilia is classifi ed according to the amount of factor defi ciency. 5 If the factor concentration is between 1 and 5%, it is considered as moderate and if below 1%, named as severe.…”

“…8 Early diagnosis and prevention of haemarthosis with prophylactic treatment in the bleeding disorders is the most important stage of the disease. 4,9 Modifi cation of life style, physical therapy and medical treatment are useful, and radiosynovectomy in chronic synovitis and intraarticular injections are the other available treatment options. [10][11][12] When these measurements are not enough, next option is surgical treatment, which includes open or arthroscopic synovectomy, releasing soft tissue for contracture, arthroplasty and joint fusion.…”

“…[10][11][12] When these measurements are not enough, next option is surgical treatment, which includes open or arthroscopic synovectomy, releasing soft tissue for contracture, arthroplasty and joint fusion. 1,4 Th e main indication for total knee arthroplasty (TKA) in hemophiliac patients is a disabling pain. However, functional mobility loss and developing knee deformity in the absence of pain could be also indication for TKA.…”