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Effective hemostasis in children with Von Willebrand factor defects undergoing adenotonsillar procedures

Rosa Diaz1, MaryFrances Musso2, Donald Mahoney1

  • 1Department of Pediatrics, Section of Hematology and Oncology, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA.

Pediatric Hematology and Oncology
|August 18, 2020
PubMed

Insights

Children with low von Willebrand factor (VWF) activity or type 1 von Willebrand disease (VWD) undergoing adenotonsillar procedures face bleeding risks. An institutional protocol using Desmopressin acetate (DDAVP) or VWF concentrate and epsilon aminocaproic acid (EACA) effectively prevented major bleeding but noted safety concerns.

Area of Science:

  • Pediatric Hematology
  • Otolaryngology
  • Pharmacology

Background:

  • Children with low von Willebrand factor (VWF) activity or type 1 von Willebrand disease (VWD) have an elevated risk of bleeding post-adenotonsillar procedures.
  • Optimal perioperative management strategies to mitigate this bleeding risk remain undetermined.

Purpose of the Study:

  • To evaluate the efficacy and safety of an institutional protocol designed to minimize postoperative bleeding in pediatric patients with type 1 VWD or low VWF activity.

Main Methods:

  • A retrospective chart review was conducted on 13 children with type 1 VWD or low VWF activity who underwent adenotonsillar procedures.
  • The protocol involved repeated doses of Desmopressin acetate (DDAVP) or VWF concentrate, brief hospitalization for observation, and extended use of oral epsilon aminocaproic acid (EACA).

Main Results:

  • No patients experienced major bleeding, required transfusions, or underwent additional surgery.
  • Minor immediate bleeding occurred in 7.6% and minor delayed bleeding in 23% of patients.
  • Mild hyponatremia was observed in 80% of patients who received DDAVP.

Conclusions:

  • The institutional protocol, utilizing repeated DDAVP or VWF concentrate and extended EACA, effectively prevents major bleeding after adenotonsillar procedures in children with VWD.
  • Observed safety concerns, particularly hyponatremia with DDAVP, necessitated protocol modifications.
  • Further prospective studies are required to establish optimal and safe strategies for managing bleeding risks in these patients.
Abstract

Keywords:
AdenotonsillectomyDDAVPaminocaproic acidlow VWFvon Willebrand disease

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