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What is the best way to prepare children with von Willebrand disease for tonsillectomy to reduce the risk of post-operative hemorrhage?

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Do you routinely use anti-fibrinolytic agents post-tonsillectomy for children with von Willebrand disease?

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2 Answers
Mednet MemberInvited Expert
Hematology · UPMC
Answered on

I am an adult provider, but the treatment is similar regardless of age. The exact treatment plan will vary depending on the type and severity of VWD (i.e., mild type 1 VWD vs type 2B VWD); however, for tonsillectomies, I provide VWF concentrate 40-80 IU/kg 5-30 min preoperatively, followed by postoperative treatment 20-40 IU/kg BID-daily for 3-5 days - again, exact specifics depending on clinical circumstances. TXA 1,300 mg TID can be used as an adjunct postoperatively.

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Mednet Member
Pediatric Hematology/Oncology · Western Michigan HTC
Answered on

I'm a pediatric hematologist at the Western Michigan HTC. I do give replacement VWB factor beforehand, keep them overnight, and provide a dose the next day. I then see them in clinic about a week later for another dose (since the eschar tends to fall off about that time), but don't give factor days 3-6 unless they have a severe bleeding phenotype. We give IV antifibrinolytics pre-op and I continue oral antifibrinolytics until day 10.

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