Abstract
Patients with inherited bleeding disorders are likely to require surgery at various
times throughout life. In some cases, this may be major orthopaedic surgery required
due to their underlying condition, for example, hemophilic arthropathy. For inherited
disorders with significant bleeding risk, surgery should take place in a hemophilia
treatment center with the availability of hematologic, anesthetic, surgical, nursing,
scientific laboratory, and allied health expertise. Preoperative assessment, planning,
and communication between team members are crucial. Specific replacement therapy is
usually required, as may other adjunctive therapies. Venous thromboembolism (VTE)
risk assessment is individualized, taking account of other underlying risks in the
patient and risks associated with the procedure, including the replacement agent itself,
and use of mechanical prophylaxis may be the safest approach in many cases. Pain management
is also modified to take account of risks associated with the bleeding disorder, and
spinal or epidural analgesia is restricted compared with patients who do not have
hemostatic defects. In patients with acquired bleeding disorders, the preoperative
assessment includes decisions regarding how best to optimize management of the underlying
condition prior to proceeding with surgery. If this is achieved, specific replacement
therapy may not be required. If complete remission of the acquired bleeding disorder
is achieved preoperatively, then VTE prophylaxis and pain management options will
not be limited by the bleeding disorder. The perioperative management of both hereditary
and acquired bleeding disorders is discussed and contrasted.
Keywords inherited bleeding disorders - acquired bleeding disorders - perioperative management
- factor concentrates - thromboprophylaxis
