Effectiveness of ankle fusion in patients with hemophilia, advanced ankle degeneration, and unbearable pain for whom nonsurgical and surgical treatments have been ineffective

ABSTRACT

Introduction: In underdeveloped countries, patients with hemophilia often experience repetitive ankle joint hemorrhages due to a shortage of coagulation factors (factor VIII [FVIII] and factor IX [FIX] for hemophilia A and B, respectively).

Areas covered: This is a narrative literature review in which we searched the Cochrane Library and PubMed for articles related to ankle arthrodesis in patients with hemophilia. The searches covered the period from the databases´ inception to 28 February 2021. In the event of unsuccessful hematologic prophylaxis and conservative measures (e.g. analgesics, cyclooxygenase-2 inhibitors, taping, intra-articular injections of hyaluronic acid and corticosteroids, physical and rehabilitation medicine, orthoses, radiosynovectomy, and joint-preserving surgery (e.g. removal of the distal tibia by open surgery or by arthroscopic surgery, joint debridement by arthroscopic surgery), the classical surgical solution is ankle arthrodesis, which does not preserve the ankle joint.

Expert opinion: Ankle pain is reduced after ankle arthrodesis (75% of patients experience no pain). Approximately 5% of patients require reoperation due to lack of fusion, and deep infection occurs in 2.5%. After tibiotalar fusion, a self-reported activity scale shows that approximately 12% of patients improve, 9% worsen, and 79% show no improvement. The results of ankle arthrodesis therefore appear to be poor.Therefore, although 75% of the patients stopped having ankle pain after arthrodesis, according to a self-reported activity scale 88% of them did not improve or worsened.

KEYWORDS:

• Hemophilia
• ankle
• arthrodesis
• fusion
• indications
• results

Article highlights

• There are several alternatives for treating the initial phases of hemophilic ankle arthropathy when hematological prophylaxis fails.

• Analgesics, COX-2 inhibitors, taping, intra-articular injections (hyaluronic acid, corticosteroids), physical medicine and rehabilitation, and orthoses should be the first options.

• When these approaches fail and there is chronic hemophilic synovitis, radiosynovectomy can alleviate the problem.

• If the aforementioned conservative measures fail, there are a number of surgical techniques that preserve the ankle joint: excision of the anterior osteophyte of the distal tibia by open surgery or by arthroscopic surgery and debridement of the joint by arthroscopic surgery.

• In the final phases of degeneration of the articular cartilage (advanced arthropathy), the only method for alleviating ankle pain is surgery that does not preserve the ankle joint. There are 2 options: ankle arthrodesis and total ankle arthroplasty.

• Currently, ankle fusion is the most recommended technique, although improvements in prosthetic designs portend an increasing number of ankle arthroplasties for patients with hemophilia in the future.

Declaration of interest

ECR Merchan has received reimbursement for collaborating as a speaker and researcher from Pfizer and Roche and honoraria for consulting or collaborating as a speaker from Pfizer, Roche, Sobi, Takeda, NovoNordisk, and Bayer. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosure

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.