Successful Perioperative Management Strategies in Surgical Correction of Craniosynostosis for Patients With von Willebrand Disease

This study evaluates the rate of von Willebrand disease (vWD) in patients with craniosynostosis and describes the management of patients with vWD who require surgical correction of craniosynostosis (SCC). This is a retrospective cohort study of 190 consecutive patients who underwent initial SCC at a university-affiliated community hospital between January 2016 and May 2024. Before surgery, patients were evaluated by the Pediatric Blood Management Service and underwent laboratory tests for anemia and vWD. Patients who screened positive for vWD received a hematology and oncology (Heme/Onc) referral, preoperative infusion of antihemophilic factor/von Willebrand factor complex (Humate-P), and postoperative administration of aminocaproic acid (Amicar). Univariate analysis was used to compare transfusion volumes between patients with and without vWD. A total of 13.2% of patients were referred to Heme/Onc due to abnormal vWD labs, and 6.8% of patients were ultimately diagnosed with vWD by Heme/Onc. All patients diagnosed with vWD received Humate-P preoperatively, and 77% of patients with vWD also received postoperative Amicar. Compared with all other patients, patients with vWD demonstrated no difference in estimated blood loss (EBL) or intraoperative and total pRBC volumes. There was also no difference in EBL nor pRBC volumes when comparing patients with vWD to anemia protocol-adherent and relative anemia protocol-adherent cohorts. vWD in our craniosynostosis population was higher than in the general American population, which is ∼1%. Preoperative screening for vWD and appropriate perioperative management can effectively address blood loss and transfusion needs for patients undergoing SCC.