Fractures needing orthopaedic surgery in haemophilic patients: long-term experience of a dedicated team at a single institution

Abstract

Purpose: Haemophilia is a rare inherited X-linked bleeding disorder associated with various levels of coagulative factor VIII (type A) or IX (type B) deficit. Persons with haemophilia (PWH) can be affected by trauma and fractures just like the general population. Due to their bleeding disorder, PWH usually need specific multidisciplinary management, from the emergency room to the operating theatre, in order to limit severe complications. Worldwide, there are few specialized orthopaedic centres dedicated to the management of PWH. The purpose of this paper is to report our experience on the management of fractures in PWH by a multidisciplinary team at a single institution. Methods: In the period 2000-2017, 19 PWH were treated in our centre: 15 with haemophilia type A, 4 with type B. Patients were classified according to fracture site (lower extremities: 16 patients; upper extremities: 3) and haematological treatment (secondary prophylaxis: 15 patients; “on demand” regimen: 4). All patients were treated by the same multidisciplinary team of orthopaedic-haemophilia specialists. They received specific haematologic protocols during the peri-operative period according to their characteristics. All patients were also evaluated using the Numeric Rating Scale and Short Form-36 preoperatively and at specific times postoperatively. Results: All patients enrolled in the study had a minimum of 2 years of follow-up (mean 8.5 yrs). No patient showed early postoperative complications; no major bleeds or deep venous thromboembolisms were recorded. Satisfactory fracture healing and functional recovery were reported by all the patients except for three, who presented symptoms or functional impairments at mid-term, needing further surgery. Conclusion: Fractures in PWH are uncommon and their management requires close cooperation with haemophilia specialists. Through multidisciplinary treatment it is possible to manage these challenging conditions in a safe manner and with a low rate of complications.