Otolaryngologic surgery in children with von Willebrand disease.

OBJECTIVE

To assess the efficacy, safety, and complications of otolaryngologic surgery in children with von Willebrand disease (vWD) undergoing surgery.

DESIGN

A prospective, controlled study of 41 children with vWD who underwent surgery between June 1, 1999, and January 31, 2001.

SETTING

A tertiary care, university-based children's hospital.

INTERVENTIONS: All children had a preoperative diagnosis of vWD. The patients were treated with either a protocol that includes the use of desmopressin acetate and tranexamic acid (37 children) or factor VIII concentrate in children with a positive history of seizures (4 children).

MAIN OUTCOME MEASURES: Immediate and delayed postoperative bleeding, hyponatremia, seizures, and urine output.

RESULTS

Two adenotonsillectomy patients (5%) had an immediate postoperative hemorrhage. Delayed postoperative bleeding was not detected in our patients. Severe hyponatremia occurred in 2 patients (1 of them with clinical manifestations).

CONCLUSIONS

Our management of children with vWD was efficacious in otolaryngologic surgery. One child had important adverse effects with the use of desmopressin (seizure). Thus, the use of desmopressin should be weighed and closely monitored.