Perioperative care of an adolescent with hemophilia B undergoing posterior spinal fusion.

Abstract

Hemophilia B is a coagulation disorder related to the de- ficiency of clotting factor IX. Depending on the severity of the deficiency, patients may experience significant hemorrhage spontaneously or following minor trauma. Additionally, given the associated coagulation defect, any surgical procedure or traumatic event carries the risks of severe hemorrhage, an increased incidence of periop- erative complications, and even death. We present a 13- year-old adolescent who was diagnosed with hemophilia B during investigation for a prolonged partial thrombo- plastin time during a routine preoperative laboratory evaluation. The perioperative management of a newly diagnosed hemophilia B patient is discussed including the perioperative administration of blood, blood prod- ucts, and coagulation adjuncts.

Keywords

• hemophilia b
• posterior spinal fusion