Cochrane: In haemophilia and other congenital
bleeding disorders blood does not clot properly, which can cause
excessive bleeding. This is particularly relevant during surgery, when
the risk
of bleeding depends on the type and severity of the clotting disorder
and on the type of surgery. Therefore, during and after surgery, these
individuals should receive treatment to improve the ability of their
blood to clot and so prevent bleeding. Clotting factor concentrates
(when available and appropriate in those individuals missing specific
clotting proteins) or other non-specific drugs for clotting, or a
combination of both, are administered. It is not known what is the
optimal dose or duration or method of administration of these treatments
in these circumstances.
We searched for randomised controlled trials comparing the efficacy (mortality, blood loss, need for re-intervention, subjective assessment of efficacy, duration and dose of therapy) and the safety of any type of treatment given to people with congenital bleeding disorders during any type of surgery. We found four trials to be included in this review. Two trials evaluated 59 people with haemophilia A or B receiving antifibrinolytic drugs (agents that reduce the breakdown of clots) or placebo in addition to the initial standard treatment before dental extractions. The remaining two trials evaluated 53 people with haemophilia A or B and inhibitors (antibodies that act against the factor concentrate therapy) receiving an different clotting concentrate, recombinant activated factor VII, both during and after surgery. These two trials evaluated different treatment options: high-dose compared with low-dose and a single large (bolus) infusion compared with continuous infusion.
The trials included in this review provide some information in two specific situations in people with congenital bleeding disorders undergoing surgery. However, on the whole, there is not enough evidence from trials to define the best treatments for the various types of disease and types of surgery. Further trials would be useful to improve our knowledge but are difficult to carry out and currently do not appear to be a clinical priority. Indeed, both major and minor surgery are safely performed in clinical practice in these individuals based on local experience and data from uncontrolled studies.
We searched for randomised controlled trials comparing the efficacy (mortality, blood loss, need for re-intervention, subjective assessment of efficacy, duration and dose of therapy) and the safety of any type of treatment given to people with congenital bleeding disorders during any type of surgery. We found four trials to be included in this review. Two trials evaluated 59 people with haemophilia A or B receiving antifibrinolytic drugs (agents that reduce the breakdown of clots) or placebo in addition to the initial standard treatment before dental extractions. The remaining two trials evaluated 53 people with haemophilia A or B and inhibitors (antibodies that act against the factor concentrate therapy) receiving an different clotting concentrate, recombinant activated factor VII, both during and after surgery. These two trials evaluated different treatment options: high-dose compared with low-dose and a single large (bolus) infusion compared with continuous infusion.
The trials included in this review provide some information in two specific situations in people with congenital bleeding disorders undergoing surgery. However, on the whole, there is not enough evidence from trials to define the best treatments for the various types of disease and types of surgery. Further trials would be useful to improve our knowledge but are difficult to carry out and currently do not appear to be a clinical priority. Indeed, both major and minor surgery are safely performed in clinical practice in these individuals based on local experience and data from uncontrolled studies.