Management of children with von Willebrand disease undergoing adenotonsillectomy.

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.