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Adenotonsillectomy in Children With von Willebrand Disease
Gregory C. Allen, MD;
Derek R. Armfield, MD;
Franklin A. Bontempo, MD;
Lawrence A. Kingsley, DrPH;
Nira A. Goldstein, MD;
J. Christopher Post, MD
Arch Otolaryngol Head Neck Surg. 1999;125:547-551.
Objective To review the effectiveness of a perioperative management protocol and our experience with a large population of patients with von Willebrand disease (vWD) who require adenotonsillar surgery (T&A).
Design A retrospective review of the medical records of all patients having 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 single intravenous dose of desmopressin acetate, 0.3 µg/kg, approximately 20 minutes before the induction of anesthesia. Beginning January 15, 1994, a standard management protocol involving the postoperative administration of fluids and electrolytes was followed.
Main Outcome Measures Operative blood loss and the incidence of postoperative 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 preoperatively received desmopressin; 2 were identified by postoperative workup as a result of excessive surgical bleeding. Minimal immediate postoperative bleeding was noted in 7 patients (10%), but none required intervention. Delayed bleeding occurred in 9 patients (13%); all were readmitted to the hospital for observation, 4 (6%) requiring operative cauterization. Substantial postoperative hyponatremia occurred in 3 patients, and 1 patient had seizure activity. Symptomatic hyponatremia has been avoided since a protocol of 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 administration 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.
From the Department of Pediatric Otolaryngology, Children's Hospital of Pittsburgh, and the Department of Otolaryngology (Drs Allen, Armfield, Goldstein, and Post); the Coagulation Laboratory, Institute for Transfusion Medicine, and the Department of Medicine (Dr Bontempo); and the Departments of Infectious Disease and Microbiology and Epidemiology, Graduate School of Public Health (Dr Kingsley); University of Pittsburgh School of Medicine, Pittsburgh, Pa. Dr Allen is now with the Department of Pediatric Otolaryngology, Children's Hospital, and the Department of Otolaryngology–Head and Neck Surgery, University of Colorado School of Medicine, Denver. Dr Goldstein is now with the Department of Otolaryngology, State University of New York, Health Sciences Center at Brooklyn. Dr Post is now with the Department of Otolaryngology, Allegheny University Health Sciences; Pediatric Otolaryngology at Allegheny General Hospital; and the Center for Genomic Sciences, Pittsburgh.
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