Correction of knee flexion contracture at the time of surgical fixation of a femoral supracondylar fracture in a haemophiliac with inhibitors.

Introduction Haemophilia A is a congenital bleeding disorder due to the lack of coagulation factor VIII (FVIII). The standard treatment is based on administration of plasma-derived or recombinant FVIII concentrates. The natural history of the disease includes recurrent bleeding into joints and the major long-term disability in affected patients is chronic arthropathy. The life expectancy of patients with haemophilia is now nearing that of the normal population thanks to replacement therapy and major surgery has also become feasible1. Unfortunately, after treatment with FVIII concentrates, a subgroup of patients become sensitised, developing neutralising antibody inhibitors which render standard replacement treatment ineffective. Surgery becomes a challenge for these patients and even more so when haemostasis is urgently necessary, as in the case of fractures. When treatment with FVIII is unfeasible in patients with high titres of inhibitor, haemostasis may be achieved by using the so-called bypassing agents such as activated recombinant factor VII (rFVIIa; NovoSeven, Novo Nordisk, Denmark) or activated prothrombin complex (aPCC; Feiba, Baxter, Deerfield, IL, USA)2,3. The advantages of rFVIIa are the possibility of concurrently using antifibrinolytic agents such as tranexamic acid and avoiding an anamnestic response to FVIII, which has been reported with aPCC. There have now been a few descriptions of major surgery carried out with the use of rFVIIa in patients with haemophilia A and high-titre FVIII antibodies, including a single joint replacement and a case of concurrent double prostheses4,5. Although achieving haemostasis is the main challenge in these instances, once obtained, the critical and complex picture of the chronic, multiple haemophilic arthropathy must be tackled by orthopaedists6.