Surgery in patients with inherited bleeding disorders

Abstract

It is estimated that up to 1% of the general population has a congenital bleeding disorder. With this level of disease burden, it is more likely than not that any practising surgeon or anaesthetist will, at one time or another, have occasion to manage one such patient. Congenital haemophilia, both A and B, von Willebrand's disease, and inherited qualitative platelet defects, constitute the bulk of these disorders, with the rest distributed between much rarer conditions. Although looking after such patients will continue to pose a challenge to anaesthetists, recent and continuing advances in haemostatic products, coupled with increasing awareness of haemostatic care, means that surgery in this challenging group of patients is safer now than ever before, and can now be undertaken with a degree of confidence not possible even two decades ago. Central to these recent successes has been the continuing evolution of specialised healthcare services; in particular, Haemophilia Comprehensive Care Centres. Of equal importance, at least in developed countries, has been the ease of access to highly purified, safe and effective haemostatic products. The key to successful surgical management of the patient with a bleeding disorder is a multidisciplinary approach involving not only surgeons, anaesthetists and haematologists, but also laboratory scientists, specialist physiotherapists and haemophilia nurses. With careful planning, most surgical and invasive procedures can be carried out safely in persons with haemophilia and other bleeding disorders.