title: Orthopaedic limb salvage with a mega prosthesis in a patient with haemophilia A and inhibitors - a case report.
abstract: Inhibitors against FVIII or FIX in patients with haemophilia are a common and serious complication. Until recently, elective surgery was associated with major bleeding despite the availability of a sufficient substitution therapy. We report about the major orthopaedic reconstruction of the right limb in a patient with severe haemophilia A and inhibitors. This reconstruction was the after effect of a traumatic periprosthetic fracture of the right femur after total knee replacement 6 months ago. This fracture could be stabilized by internal fixation. Two months later, a non-traumatic femur fracture occurred. Therefore, we removed the distal part of the femur and the joint replacement, and implanted a custom made tumour prosthesis (Type MUTARS (c), Münster). These three successive operations, which included emergency and elective surgery, were performed within 8 months. This is, to the best of our knowledge, the first patient with inhibitors undergoing such a complicated reconstruction of a limb. We conclude that successful elective orthopaedic surgery could be accomplished safely in this patient with high responding inhibitors using recombinant FVIIa. After a follow-up of 9 months, no major complications were seen.
authors.affiliation: Department of Traumatology and Handsurgery, University Hospital Duesseldorf, Heinrich Heine-University, Duesseldorf, Germany. ulla.stumpf@med.uni-duesseldorf.de
authors.forename: U C
authors.initials: UC
authors.lastname: Stumpf
authors.forename: C
authors.initials: C
authors.lastname: Eberhardt
authors.forename: A A
authors.initials: AA
authors.lastname: Kurth
mesh_terms: D000328:Adult; D019645:Arthroplasty, Replacement, Knee / Q000379:methods*; D015942:Factor VIIa; D005264:Femoral Fractures / Q000601:surgery*; D005592:Fracture Fixation / Q000379:methods*; D017048:Health Care Costs; D006467:Hemophilia A / Q000150:complications*; D006488:Hemostasis, Surgical / Q000379:methods*; D006801:Humans; D007718:Knee Injuries / Q000150:complications*; D008297:Male; D019736:Prostheses and Implants / Q000706:statistics & numerical data*; D011994:Recombinant Proteins; D018570:Risk Assessment; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa