关键词: 骨折, 血友病, 围手术期

Abstract: Objective：To investigate the characteristics and perioperative management of hemophilia patients with fracture.Methods: Retrospectively, we analyzed 8 patients with hemophilia combined with fracture, who were admittted to our department from 2005 to 2013. Six patients were with hemophilia A and two with hemophilia B; Based on the severity of hemophilia, 2 cases were light, 3 moderate and 3  severe; Based on the location of fracture, 4 cases were femoral neck fractures, 1 femoral intertrochanteric fracture, 1 bilateral distal femur fractures, 1 tibiofibula fracture, and 1 humerus intercondylar fracture. Blood coagulation factor replacement therapy was conducted preoperatively, intraoperatively and postoperatively, All the patients underwent closed or open reduction and internal fixation or joint replacement. Also, we analyzed the perioperative complications and observed whether the fracture healed.  Results: The average age was 33.5 years (14 to 47 years); In 6 cases, fractures occurred at femur, accounting for 75% of all the fractures; Femoral neck fracture was treated by closed reduction and hollow screws  fixation; Femoral intertrochanteric fracture, distal femur fracture, and tibiofibula fracture were treated by open reduction and internal fixation with plate; Humerus intercondylar fracture was treated by elbow joint replacement. Intraoperative bleeding was from 50 to 600 mL, an average of 262 mL; Perioperatively, the average use of FⅧ/activated prothrombin complex concentrates (APCC) was 358 U/kg (125 to 554 U/kg). Postoperatively, poor wound healing was observed in 2 patients, and the condition improved after symptomatic treatment; In patients with internal fixation, all the fractures united, and the average healing time was 14 weeks. No complications such as fixation loosening or rupture occurred after internal fixation.Conclusion: Hemophilia combined with fracture mainly occurred in the young, and the site of fracture was given priority to femur. With perfect preoperative preparation, on the basis of the replacement therapy, hemophilia combined with fractures was safe for surgical treatment, and postoperative fractures healing wasgood. But the risk of poor wound healing was high.

Key words: Fractures, bone, Hemophilia, Perioperative period