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Management of children with von Willebrand disease undergoing adenotonsillectomy
C S Derkay1, E Werner, E Plotnick
1Department of Otolaryngology-Head and Neck Surgery and Pediatrics, Eastern Virginia Medical School, Norfolk, USA.
Insights
Children with von Willebrand disease (vWD) undergoing adenotonsillar surgery can be managed safely with desmopressin (DDAVP) and antifibrinolytic agents, minimizing hemorrhage risk and blood product use.
Area of Science:
- Pediatric Otolaryngology
- Hematology
Background:
- Children with von Willebrand disease (vWD) face increased bleeding risks after adenotonsillar surgery.
- Effective management strategies are crucial to prevent intraoperative and postoperative hemorrhage.
Purpose of the Study:
- To report favorable outcomes of 12 consecutive adenotonsillar procedures in pediatric patients with vWD.
- To present a prospective treatment protocol for managing vWD patients undergoing adenotonsillar surgery.
Main Methods:
- Prospective management involving local hemostasis, desmopressin (DDAVP), and antifibrinolytic agents.
- Use of cryoprecipitate or factor VIII concentrates for patients intolerant to DDAVP.
Main Results:
- Successful completion of 12 adenotonsillar procedures with minimal blood product utilization.
- Favorable outcomes achieved with the described management protocol.
Conclusions:
- A practical screening algorithm and treatment protocol for managing pediatric vWD patients undergoing adenotonsillar surgery.
- Review of literature supports the use of DDAVP in managing vWD-related bleeding risks.
Purpose:
Children with von Willebrand disease (vWD) are at greater risk for hemorrhage following adenotonsillar surgery. We report the favorable results of 12 consecutive adenotonsillar procedures performed since 1989 in children with vWD, with minimal use of blood products.
Materials And Methods:
Patients were prospectively managed with careful local control, desmopressin (DDAVP), and antifibrinolytic agents. Cryoprecipitate or intermediate purity factor VIII concentrates containing von Willebrand factor (vWF) were used for three patients who did not tolerate DDAVP.
Results:
Based on this experience, we propose a prospective treatment protocol.
Conclusions:
The literature regarding the use of DDAVP is reviewed, and our screening algorithm and treatment protocol for managing these challenging cases is presented.