PB0842 - Surgical and Procedural Outcomes in Pediatric Patients with Mild Type 1 Von Willebrand Disease

Monday, June 26, 2023

18:30 – 19:30 ET

Room: Exhibition

Presenting Author(s)

Samuel H. Heery

Medical Student
Emory University School of Medicine
Atlanta, Georgia, United States

Co-Author(s)

KZ

Karen Zimowski

Assistant Professor of Pediatrics
Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine
Atlanta, Georgia, United States
SM

Sadie F. Mason

Clinical Fellow
Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine
Atlanta, Georgia, United States
MW

Michael H. White

Assistant Professor of Pediatrics
Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine
Atlanta, Georgia, United States
SD

Stefanie M. DiGiandomenico

Clinical Fellow
Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine
Atlanta, Georgia, United States
CS

Caroline Scales

Nurse Practitioner
Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta
Atlanta, Georgia, United States
Robert F. Sidonio, Jr., MD, MSc

Associate Professor of Pediatrics
Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine
Atlanta, Georgia, United States
Megan C. Brown, MD, MSc

Assistant Professor of Pediatrics
Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine
Atlanta, Georgia, United States

Background: Persons with mild quantitative deficiency of von Willebrand factor (VWF) with levels between 30-50 IU/dL and a bleeding phenotype are now classified as type 1 Von Willebrand Disease (VWD). Recent VWD treatment guidelines suggest tailoring surgical management by procedure type; these conditional recommendations are based on very low certainty, due to limited data on surgical outcomes in this population.

Aims: Characterize bleeding prophylaxis strategies and bleeding outcomes for surgeries and procedures in children with mild type 1 VWD.

Methods: This is a retrospective cohort study. Patients were between the ages of 0-21 years with mild Type 1 VWD (defined as VWF:Ag, VWF:RCo and/or VWF:GpIbM 30-50 IU/dL with no levels < 30 IU/dL) who underwent a procedure from 7/1/17 to 7/1/22. Demographic, surgical, medication and bleeding data were collected by manual chart review.

Results: A total of 161 procedures in 108 unique patients were performed. The population was primarily female (75%), white (77.8%) and non-Hispanic (79.6%). Median age at the time of surgery was 15.8 years (IQR: 8.2-17.6). Fifty-nine surgeries were classified as major, 66 as minor and 36 as dental (Figure 1). For most procedures, patients received only antifibrinolytics for bleeding prophylaxis (128, 79.5%); DDAVP was used in seventeen (10.6%) procedures, and VWF concentrate was used in 12 (7.5%) procedures. Bleeding complications occurred in 8 (5.0%) procedures: per ISTH definitions, these included 1 major, 4 clinically relevant non-major and 3 minor bleeding events (Figure 2). No patients required blood transfusion, or an additional procedure to achieve hemostasis. Most bleeding complications were seen in patients undergoing IUD placement (5/8), which is known to cause irregular vaginal bleeding after insertion. Nearly 30% of patients undergoing IUD placement reported bleeding complications.

Conclusion(s): Pediatric patients with mild Type 1 VWD can safely undergo procedures using a tailored approach. Bleeding complications were uncommon, with the majority following IUD placement.