Perioperative Management of a Frail Patient With Bernard-Soulier Syndrome.

Abstract

Bernard-Soulier syndrome (BSS) is an autosomal recessive inherited bleeding disorder characterized by prolonged bleeding time, thrombocytopenia, and giant platelets. Patients with BSS are at an increased risk of bleeding, especially during traumatic injury and surgical procedures. The literature on the anesthetic management of patients with BSS is limited. In this report, we detail the successful management of a frail patient with BSS who underwent a major surgical procedure. Despite comprehensive clinical monitoring and an extended pharmacological strategy, a hemorrhagic complication occurred in the later postoperative phase, emphasizing the necessity for continued support and vigilant clinical monitoring due to the ongoing bleeding risk associated with these patients. In this case, a combined strategy involving antifibrinolytics, recombinant factor VII, and platelet transfusions proved effective.