Elective surgery in patients with inherited bleeding disorders: A retrospective analysis

Published guidelines are available to assist in the management of patients with inherited bleeding disorders in the elective surgical setting however good quality outcome data is lacking.

- Evaluate the outcomes of adult patients with inherited bleeding disorders, who received factor replacement for elective surgery in NSW/ACT, Australia. - Assess adherence to relevant guidelines including Haemophilia Treatment Centre (HTC) utilisation and appropriate factor replacement.

A retrospective analysis was performed between 2000 and 2018 to describe patient characteristics, surgical details, factor provision and outcomes. Univariate analysis was used to determine variables associated with guideline adherence. Covariates with p < 0.1 were included in the multivariate analysis.

A total of 1065 surgeries were performed on 571 patients. Diagnoses included Haemophilia A (43.5%), Haemophilia B (9.7%), von Willebrand disease (VWD) (45.3%) and rare bleeding disorders (RBDs) (1.6%). Bleeding complications were reported in 14 surgeries and 19 patients received factor replacement beyond standard duration of prophylaxis. Approximately 50% of all surgeries were performed in a HTC. Multivariate analysis demonstrated that diagnosis, surgical specialty, sex and year (p < 0.001) were associated with non-compliance with variable pattern within each category. Factor replacement was as expected except for plasma-derived Factor VIII/VWF usage in patients with VWD undergoing major bleeding risk surgery. VWD classification (p < 0.001) was associated with this deviation.

Low complication rates demonstrate that elective surgery in Australia is being safely performed in patients with inherited bleeding disorders however non-compliance with published guidelines exists highlighting areas of practice and policy discrepancies that warrant further exploration.