Buckle up! Managing surgery in patients with bleeding disorder of unknown cause

Bleeding disorder of unknown cause (BDUC) is a diagnosis of exclusion encompassing patients who have objective clinical symptoms of a mild bleeding disorder, yet negative hemostatic evaluations. Patients with BDUC may have a history of significant postoperative bleeding, and management of subsequent procedures poses a distinct challenge. After reviewing both patient and procedural bleeding risk factors, empiric hemostatic therapies can be considered. Extrapolating from the management of phenotypically similar bleeding disorders, a therapeutic approach may include antifibrinolytic agents (tranexamic acid), desmopressin, and/or platelet transfusion. Patients should be medically optimized prior to procedures and monitored closely for excess bleeding. Education of both patients and providers is central throughout the hemostatic planning process. Primary research in BDUC remains limited; future multisite observational and therapeutic clinical trials in BDUC may help us better understand multifactorial bleeding risk and further define the role of hemostatic therapies.