A cemented Charnley total hip prosthesis was implanted in a 48-year-old man with mild haemophilia (factor VIII 4 IU dL-1) in his right spontaneously ankylosed hip. At the time of surgery he was anti-HCV positive, anti-HIV negative, and no circulating inhibitors were encountered. The indication for surgery was long-lasting intractable low back and ipsilateral knee pain. At 4-month follow-up, relief of pain was achieved as well as correction of limb-length discrepancy, with a good result according to the Mayo Clinic hip score. Doses of 50 IU kg-1 body weight of recombinant factor VIII (Recombinate; Baxter, Glendale, California, USA) was used during the 2 weeks of admittance to the hospital. The dosage was adjusted according to the recoveries of factor VIII, with an overall factor consumption of 68 000 IU. As far as we know this is the first case reported in the literature of a person with haemophilia in whom a spontaneous hip ankylosis has been satisfactorily converted in a total hip arthroplasty with a short-term follow-up. However, a much longer follow-up is still needed to ascertain the efficacy of this surgical procedure in haemophilia.
Because HIV and hepatitis B and C infection can be transmitted by exposure to infected blood or blood components, orthopaedists are at risk for HIV and hepatitis infection during surgical procedures. Thus, care must be taken to protect themselves from transmission of HIV and the hepatitis virus. The following precautions are recommended: double latex gloves, changed hourly, or a combination of cloth and latex gloves, enclosed hood and face-masks and operative isolator with umbilical-cord aspirator; knee-length impermeable gowns of high-count polyester weave or plastic-lined nonwoven spun-lace polyester; a combination of shoe-covers that provide waterproof coverage as high as the knee; and disposable drapes. If a member of the operating team is inadvertently pricked or cut, the wound should be washed immediately with iodine, soap and water. If the injured person has been immunized for the hepatitis B (and has adequate titres), or is positive for hepatitis B surface antigen or antibody, no further treatment is necessary. Otherwise, two doses of hepatitis B immune globulin should be given, 5 mL immediately and 5 mL after 1 month. Because HIV infection can be transmitted by exposure to infected blood, orthopaedic surgeons are also at risk for HIV infection. Prospective studies suggest that this risk is very low; nevertheless, healthcare workers need to adhere rigorously to the aforementioned infection-control precautions to minimize the risk of exposure to blood.
The most important clinical strategy for management of patients with hemophilia is the avoidance of recurrent hemarthroses by means of continuous, intravenous hematological prophylaxis. When only intravenous ondemand hematological treatment is available, frequent evaluations are necessary for the early diagnosis and treatment of episodes of intra-articular bleeding. The natural history of the disease in patients with poorly controlled intra-articular bleeding is the development of chronic synovitis and, later, multi-articular hemophilic arthropathy. Once arthropathy develops, the functional prognosis is poor. Treatment of these patients should be conducted through a comprehensive program by a multidisciplinary hemophilia unit. Although continuous prophylaxis can avoid the development of the orthopedic complications of hemophilia still seen in the twenty-first century, such a goal has not, so far, been achieved even in developed countries. Therefore, many different surgical procedures such as arthrocentesis, radiosynoviorthesis (radiosynovectomy) (yttrium-90, rhenium-186), tendon lengthenings, alignment osteotomies, joint arthroplasties, removal of pseudotumours, and fixation of fractures are still frequently needed in the care of these patients.
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