Adenotonsillectomy in Children With von Willebrand Disease

Abstract

Although T&A can be performed safely in patients with vWD, it is not without an increased risk of postoperative hemorrhage. The administration of desmopressin has been reported to reduce the risk of bleeding, but it is not without risk. A protocol for fluid and electrolyte management is recommended.

Post‐tonsillectomy bleeding in children with von Willebrand disease: A single‐institution experience

“…There was no difference in the pre- and post-DDAVP challenge of aPTT, factor VIII:c levels, vWF antigen levels, or ristocetin cofactor activity among bleeding and non-bleeding vWD patients, which may be due to our small bleeding rate. This marks a change in comparison to a prior study published in 1999 from this institution, in which initial factor VIII levels were lower in vWD patients who had post-tonsillectomy bleeding, and lower vWF antigen levels approached statistical significance 6. However, the data from this study do suggest that children with known vWD who responded well to the DDAVP challenge and were treated preoperatively with DDAVP and other appropriate medications have an overall post-tonsillectomy bleeding rate lower than expected in comparison to prior studies evaluating children with vWD who underwent tonsillectomy 6,19.…”

“…Desmopressin acetate (DDAVP), a synthetic analogue of antidiuretic hormone, is the treatment of choice for type 1 vWD and many patients with other forms of vWF/factor VIII-deficient vWD because it induces the secretion of autologous vWF and factor VIII from endothelial cell compartments into the plasma 4,6,17. This relatively inexpensive and widely available medication is diluted in 50 mL of isotonic saline solution and administered to children and adults at a dose of 0.3 μg/kg of body weight via intravenous infusion over 30 minutes, typically resulting in a transient increase in plasma vWF and factor VIII three to five times baseline levels 4,17.…”

“…Second, vWF binds to coagulation factor VIII, a critical component of the hemostatic pathway, preventing its inactivation and clearance from plasma 4,5. vWD clinically presents as epistaxis, menorrhagia, gastrointestinal bleeding, excessive bleeding following soft tissue trauma, or other mucocutaneous bleeding 5,6. This disorder also commonly presents with disproportionate perioperative bleeding following otherwise benign surgical procedures, such as dental work 1.…”

“…Seventy to 80 percent of individuals with vWD are categorized as type 1, having an autosomal dominant-inherited abnormality that results in mildly to moderately decreased levels of normally functioning vWF in the plasma 5,6. Type 2 vWD, which is composed of four subtypes, comprises various qualitative defects in the vWF molecule 4,5.…”

“…Type 3 vWD, a variant seen in 1 percent to 5 percent of all cases, is usually inherited in an autosomal recessive manner and is characterized by nearly undetectable or absent levels of plasma vWF and below-normal levels of factor VIII 4. Of these categories, type 3 vWD is the most likely to cause severe, even life-threatening post-tonsillectomy hemorrhage 4,6…”