Anesthetic Care of a Patient With Bernard-Soulier Syndrome for Posterior Spinal Fusion

Abstract

Bernard-Soulier syndrome (BSS), a rare autosomal recessive disorder first identified in 1948, is characterized by excessive and prolonged bleeding due to thrombocytopenia and platelet dysfunction with increased platelet size and deformability. The primary defect in BSS involves the glycoprotein (GP) Ib-IX-V complex, which is important in initiating platelet aggregation and thrombosis after vascular injury by facilitating the adhesion of platelets to von Willebrand factor. The coagulation defect in BSS can lead to significant bleeding during traumatic injury or surgical intervention. We present a 17-year-old adolescent with BSS who presented for posterior spinal fusion for idiopathic scoliosis. Previous reports of perioperative care of patients with BSS are reviewed and options for anesthetic care including perioperative control of the coagulation defect are presented.




J Med Cases. 2018;9(10):341-344
doi: https://doi.org/10.14740/jmc3142w