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Procedural outcomes in children with mild type 1 von Willebrand disease
Samuel Heery1, Karen Zimowski1,2, Sadie F Mason1,2
1Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, USA.
Insights
Children with mild type 1 von Willebrand disease (VWD) can safely undergo procedures with tailored prophylaxis. Bleeding complications were rare, with most occurring after intrauterine device placement.
Area of Science:
- Hematology
- Pediatric Surgery
- Genetics
Background:
- Treatment guidelines for mild type 1 von Willebrand disease (VWD) recommend individualized surgical management.
- Evidence supporting these recommendations is limited, necessitating further research into surgical outcomes in this population.
Purpose of the Study:
- To analyze bleeding prophylaxis strategies and outcomes in pediatric patients with mild type 1 VWD undergoing procedures.
- To identify specific procedures associated with higher bleeding risks in this cohort.
Main Methods:
- Retrospective cohort study of patients aged 0-21 years with mild type 1 VWD (VWF:Ag or VWF:RCo 30-50 IU/dL).
- Data collected via manual chart review included demographics, surgical details, medications, and bleeding events for 161 procedures (July 2017-July 2022).
Main Results:
- 108 patients underwent 161 procedures; 59% were major or minor surgeries, 36% dental.
- Antifibrinolytics were the most common prophylaxis (79.5%), followed by desmopressin (10.6%) and VWF concentrate (7.5%).
- Bleeding complications occurred in 5.0% of procedures (8/161), with most linked to intrauterine device (IUD) placement (5/8).
Conclusions:
- Pediatric patients with mild type 1 VWD can undergo procedures safely with individualized prophylaxis.
- Intrauterine device (IUD) placement was associated with a higher incidence of bleeding complications in this cohort.
Background:
In patients with mild type 1 von Willebrand disease (VWD), treatment guidelines suggest individualization of surgical management. However, these conditional recommendations are based on very low-certainty evidence due to limited data on surgical outcomes in this population.
Objectives:
To characterize procedural bleeding prophylaxis strategies and outcomes in children with mild type 1 VWD.
Methods:
This is a retrospective cohort study that included patients aged between 0 and 21 years with mild type 1 VWD (defined as von Willebrand factor antigen and/or an activity of 30-50 IU/dL) who underwent a procedure from July 1, 2017, to July 1, 2022. Demographic, surgical, medication, and bleeding data were collected by manual chart review.
Results:
A total of 161 procedures were performed in 108 patients. The population was primarily female (75%), White (77.8%), and non-Hispanic (79.6%). Median age was 15.8 years (IQR, 8.2-17.6). Fifty-nine surgeries were classified as major, 66 as minor, and 36 as dental. For most procedures, patients received only antifibrinolytics for bleeding prophylaxis (n = 128, 79.5%); desmopressin was used in 17 (10.6%) procedures, and von Willebrand factor concentrate was used in 12 (7.5%) procedures. Bleeding complications occurred in 8 (5.0%) procedures: these included 1 major, 4 clinically relevant nonmajor, and 3 minor bleeding events. No patient required blood transfusion or an additional procedure to achieve hemostasis. Most bleeding complications were seen following intrauterine device (IUD) placement (5/8). Nearly 30% of patients who underwent IUD placement reported bleeding.
Conclusion:
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.
