Short-term and mid-term outcome of total ankle replacement in haemophilic patients
Introduction
Haemophilia A and B are X-linked clotting disorders caused either by factor VIII (FVIII) or IX (FIX) deficiency; significant reduction in these clotting factors (<1 IU/dL) leads to spontaneous bleeding into joints and muscles.
Recurrent bleeding into the same “target” joint [1] leads to inflammatory changes [2], synovial proliferation and eventual chronic synovitis [1], [3].
Small, recurrent haemorrhages can cause degenerative lesions that gradually provide the site for an arthropathy.
Accompanying altered joint motion and poor alignment of component bones due to damaged epiphyseal growth plates exacerbate the progression of the arthropathy. These factors [1] can also affect the overlying knee and hip by adding an abnormal mechanical constraint to the multiple joints in the lower limbs (analysis of gait and tread) [4].
Joints in the elbows, knees and ankles [5] are most affected by bleeding [6]. The ankle is the first target joint in childhood [7] and is the most frequently affected joint in the second decade of life [8], [9], characterised by pain, stiffness and deformity. It is the most prevalent cause of morbidity in patients with severe haemophilia A [7] often associated with mobility-reducing foot/ankle deformities [10] that interfere with daily activities and reduce the quality of life.
Regular prophylaxis with concentrated clotting factors is the most effective method of preventing haemophilic arthropathy [11]. To prevent recurrent bleeding, radio synovectomy or chemical synovectomy can be considered at an early stage [12] and, if unsuccessful, arthroscopic synovectomy. Nonetheless, cautious management and conservative treatment are sometimes inadequate, and invasive surgery may be required.
The current standard treatment for patients with painful, progressive arthropathy is tibio-talar arthrodesis [10], [11], [12], [13], which demonstrates good results in terms of pain and bleeding episodes but with loss of mobility [13]. Based on initial reports [6], [7], which are now outdated, total ankle replacement (TAR) is also indicated [12], [14], [15] in a limited number of cases, but its value is under debate.
The development of third-generation prostheses (cementless, with three components using cross-linked polyethylene meniscal bearings) should make it possible to conserve mobility, restore rolling and rotation, and protect other overlying and underlying joints [16], [17], [18], [19].
Defects in the mechanical axis of the lower limb, ligament instabilities and other growth defects are contraindications. Additional procedures, such as Achilles tendon lengthening, ligament reconstruction, subtalar and midtarsal arthrodesis and malleolar osteotomy, can be combined with arthroplasty to correct these various disorders.
Good short, medium [2], [19], [20], [21] and long-term [22], [23], [24] outcomes have been reported. However, most of these publications were written solely by the inventors of the various prostheses.
Interestingly, neither arthrodesis nor arthroplasty seem to be the sole solution [25].
If selected, arthroplasty is preferable as first intention, even though some suggest performing it after arthrodesis. Arthrodesis is a solution if prosthesis fails [26].
In haemophilic patients, ankle replacement poses special challenges: preventing postoperative bleeding to provide the most favourable conditions for physiotherapy and to avoid complications that may require additional surgery.
Studies available on the outcomes of TAR in a small number of haemophilic patients (1–8) report high levels of patient satisfaction in terms of pain relief, increased range of motion and the low rate of complications [2], [5], [27], [28]. Although reporting the outcomes in 531 cases of TAR, the Swedish national registry does not differentiate particular cases [24] and 21 separate cases with different aetiologies are grouped together – not only haemophilia, but also haemochromatosis, psoriasis and other disorders – making it impossible to draw comparisons.
In this paper, we describe our experience and the medium-term outcomes of 32 TARs in 21 haemophilic patients.
Section snippets
Patients
This is a retrospective study involving all patients with haemorrhagic arthropathy who underwent total ankle replacement (TAR) in our hospital. The patients were fully informed and provided their written consent before the start of data collection.
The protocol was approved by the independent Ethics Committee (Comité consultatif sur le traitement de l’information en matière de recherche [CCTIRS], Paris, France).
Patients were aged at least 18 at the time of the TAR and presented with sequelae of
Results
TARs were performed between July 2002 and September 2009 in 21 patients (17 haemophilia A, 3 haemophilia B, 1 von Willebrand disease type III).
The mean age was 44 ± 12 (range: 24–67 years).
11 patients underwent bilateral TAR (in total, 32 TARs): 2 of these patients during the same procedure, 2 patients had undergone previously unsuccessful TARs, and received the revision AES (Ramses prosthesis, 2 sides for one patient, 1 side for the other).
12 TARs were performed in 7 HIV-positive patients.
All
Discussion
Until now, the standard treatment for haemophilic arthropathy of the ankle has been arthrodesis [7], [10], [13], [28], [29]. This technique is safe, substantially reduces pain, prevents the occurrence of other intra-articular bleeding and allows the positional correction of pre-existing equinus [8]. Nonetheless, it irreversibly sacrifices ankle mobility, worsened by an additional abnormal constraint on the multiple joints of the lower limb (analysis of gait and tread) [4] and mechanical stress
Conclusion
In view of our results, we believe, in agreement with various publications cited in the literature concerning osteoarthritis of the ankle, that, even in haemophilic patients, TAR can provide an alternative to tibiotalar arthrodesis, the current standard treatment. TAR is indicated because of its excellent outcomes in pain reduction and improved ankle motion, in particular dorsiflexion, in our group of 21 patients (32 ankles).
This technique conserves tibiotalar mobility and, by contiguity
Conflict of interest
None declared.
References (38)
- et al.
Pathobiology of hemophilic synovitis I: overexpression of mdm2 oncogene
Blood
(2004) - et al.
Postoperative infection following orthopaedic surgery in human immunodeficiency virus-infected hemophiliacs with CD4 counts < or =200/mm3
J Arthroplasty
(1995) - et al.
The target joint
Haemophilia
(2004) - et al.
Haemophilic arthropathy of the ankle treated by total ankle replacement: a case series
Haemophilia
(2010) - et al.
Intermediate and long term outcomes of total ankle arthroplasty and ankle arthrodesis. A systematic review of the literature
J Bone Joint Surg Am
(2007) - et al.
Upper ankle joint prostheses in haemophilia patients
Hamostaseologie
(2009) - et al.
Total joint arthroplasty in haemophilia
Haemophilia
(2000) - et al.
Management of haemophilic arthropathy
Haemophilia
(2008) - et al.
Arthropathy of the ankle in hemophilia
J Bone Joint Surg Am
(1991) - et al.
Changing patterns of bleeding in patients with severe haemophilia A
Haemophilia
(2009)
Total ankle replacement in patients with rheumatoid arthritis
Clin Orthop Relat Res
Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia
N Engl J Med
Orthopaedic management of haemophilia arthropathy of the ankle
Haemophilia
Outcome of surgicalconcepts in haemophilic arthropathyof the hind foot
Haemophilia
Surgical management of advanced hemophilic arthropathy. An overview of 20 years’ experience
Clin Orthop Relat Res
Revision of total ankle replacement
Techniq. Foot Ankle Surg
Functional outcomeof meniscal bearing total ankle replacement: a gait analysis study
J Am Podiatr Med Assoc
Uncemented STAR total ankle prostheses. Three to eight-year follow-up of fifty-one consecutive ankles
J Bone Joint Surg Am
Total ankle replacement: medium-term results in 200 Scandinavian total ankle replacements
J Bone Joint Surg Br
Cited by (19)
Musculo-skeletal manifestations of haemophilia
2016, Blood ReviewsCitation Excerpt :The standard treatment for end-stage osteoarthritis of the ankle joint in haemophilic patients has been fusion of the ankle joint [70]. TAR is still controversial as a treatment option [42,43]. The main risks of orthopaedic surgery in haemophilia are infection and bleeding [4].
Scope assisted ankle arthrodesis in a young male with hemophilic arthritis: A case study
2021, Foot and Ankle Surgery: Techniques, Reports and CasesSevere Ankle Arthritis After Multiple Hemarthrosis Secondary to Factor XI Deficiency: A Case Report
2018, Journal of Foot and Ankle SurgeryCitation Excerpt :They also reported ankle joint dorsiflexion had improved from 0.3° preoperatively to 10.3° after TAR. They concluded that TAR is a promising alternative to arthrodesis for hemophilic patients (20). Our patient's ankle dorsiflexion improved by 10° after TAR and gastrocnemius recession.
Ankle arthrodesis and total ankle replacement in patients with congenital bleeding disorders suffering from severe ankle arthropathy
2024, Expert Review of Hematology