Management of children with inherited mild bleeding disorders undergoing adenotonsillar procedures

Practical aspects of DDAVP use in patients with von Willebrand Disease undergoing invasive procedures: a European survey

“…The eight studies using DDAVP to cover surgical interventions [10][11][12][13][14][15][16][17] were mainly in type 1 VWD, although some included patients with 'low VWD', were 'possible type 1', mild/moderate VWD or had unspecified bleeding disorders (Table 1). Four retrospective [10,11,14,15], three prospective [12,16,17] and one case report [13] included a total of 609 patients, of whom 225 underwent 232 procedures, predominantly adenotonsillectomy (108), otolaryngological procedures (41) or dental procedures (35). Other procedures could not be identified.…”

Posttonsillectomy Hemorrhage in Children With von Willebrand Disease or Hemophilia

“…These protocols vary widely in drug selection, dosing, and patient monitoring. [4][5][6][7]24 There is even older literature about hemophilia management in tonsillectomy patients. However, there does seem to be greater consensus on general perioperative hemophilia evaluation and treatment, reinforced by the recent publication of US-based best-practice guidelines.…”

“…According to recent case series, bleeding rates for children with known coagulopathies are between 2% and 17%. [4][5][6][7] This wide range may be attributed to the highly unpredictable presentation of certain coagulopathies, especially von Willebrand disease (VWD), as well as variation in perioperative management of these disorders due to a lack of standard guidelines. Because VWD is the most common hereditary bleeding disorder with a prevalence of about 1%, 8,9 and the hemophilia disorders are the second most common with an incidence of 1:50 000 males in the United States, 10 the burden of posttonsillectomy hemorrhage attributable to known coagulopathies is potentially quite substantial.…”

Perioperative management of a pediatric patient with suspected type 1 von Willebrand disease undergoing tonsillectomy: a case report

“…Tonsillectomy is not a major surgery, but there is a high risk of bleeding during and after the procedure [4]. Previous reports indicated that bleeding rates in children with known coagulopathies are between 2% and 17% [5][6][7][8]. Sun et al [7] reported that the rate of delayed hemorrhage after tonsillectomy in children with VWD or hemophilia was much higher, especially in older children.…”

“…Post-tonsillectomy hemorrhage is a common complication, with an overall frequency of approximately 5% [4]. Coagulopathies are evidently risk factors for post-tonsillectomy hemorrhage [5][6][7][8]. Accordingly, perioperative management of patients with VWD is a major clinical concern.…”