Adenotonsillectomy in children with von Willebrand disease

Objective: To review the effectiveness of a perioperative management protoc ol and our experience with a large population of patients with von Willebra nd disease (vWD) who require adenotonsillar surgery (T&A). Design: A retrospective review of the medical records of all patients havin g the diagnosis of vWD who underwent T&A between January 1, 1992, and July 31, 1996. Setting: A tertiary care, university-based children's hospital. Interventions: Patients having a preoperative diagnosis of vWD received a s ingle intravenous dose of desmopressin acetate, 0.3 mu g/kg, approximately 20 minutes before the induction of anesthesia. Beginning January 15, 1994, a standard management protocol involving the postoperative administration o f fluids and electrolytes was followed. Main Outcome Measures: Operative blood loss and the incidence of postoperat ive bleeding and of hyponatremia. Results: Of approximately 4800 patients who underwent T&A during the study period, 69 patients had a diagnosis of vWD. All 67 patients identified preo peratively received desmopressin; 2 were identified by postoperative workup as a result of excessive surgical bleeding. Minimal immediate postoperativ e bleeding was noted in 7 patients (10%), but none required intervention. D elayed bleeding occurred in 9 patients (13%); all were readmitted to the ho spital for observation, 4 (6%) requiring operative cauterization. Substanti al postoperative hyponatremia occurred in 3 patients, and 1 patient had sei zure activity. Symptomatic hyponatremia has been avoided since a protocol o f fluid and electrolyte administration was instituted. Conclusions: Although T&A can be performed safely in patients with vWD, it is not without an increased risk of postoperative hemorrhage. The administr ation 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.