The perioperative management of Bernard-Soulier syndrome: a case report and review of the role of perioperative factor VIIa

Abstract

This article presents the perioperative anaesthetic management of a patient with Bernard-Soulier syndrome (BSS). A literature search was conducted to examine the perioperative haemostatic management of BSS, with particular focus on the developing role of recombinant factor VIIa. The early use of factor VIIa at doses of 90 to 100 μg/kg as a first-line therapy, alongside platelet transfusion, may result in a reduction in the perioperative use of blood products.