Surgical management of hemophilic pseudotumor complicated by destructive osteoarthropathy

Hemophilic pseudotumor gradually erodes bone and induces fracture or deformity, causing joint dysfunction or destructive osteoarthropathy. Reports about surgery for hemophilic pseudotumor complicated by destructive osteoarthropathy are scarce. The object of this study was to evaluate the results and complications of surgical management for patients of pseudotumor complicated by destructive osteoarthropathy. We retrospectively reviewed records from July 1996 to July 2013, and found eight patients with pseudotumor complicated by destructive osteoarthropathy. We recorded their demographic data, time of surgery, amount of blood loss and transfusion, bone union, and complications. Seven patients were diagnosed with hemophilia A and one with hemophilia B. The mean age at surgery was 31.9 ± 8.3 years. Two of the eight underwent excision of the pseudotumor and metallic fixation, one had amputation, and five underwent autogenous or exogenous bone grafting and fixation with an absorbable screw. The median operating time was 170 min (135-315 min). The median amount of intraoperative blood loss was 1350 ml (100-4000 ml). The amount of red blood cells, plasma, and whole blood transfusion after surgery were 0-24 units, 0-2000 ml, and 0-4600 ml, respectively. After a median follow-up of 75 months, the numbers of pseudotumor recurrence, fracture nonunion, coagulation factor inhibitor formation, and wound complications were one, one, two, and four, respectively. Surgery is an effective treatment for hemophilic pseudotumor complicated by destructive osteoarthropathy. However, the incidences of wound infection, coagulation factor inhibitor formation, hemophilic pseudotumor recurrence, and fracture nonunion are high.