Role of tonsillectomy in PFAPA syndrome

objective: To examine the efficacy of tonsillectomy in ameliorating symptoms and preventing recurrence of episodes in children with PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and adenitis). Design: Retrospective case series. Setting: Tertiary care children's hospital. Patients: Patients who presented to a major tertiary teaching hospital in Vancouver, British Columbia, Canada, between 2000 and 2004 with the diagnosis of PFAPA syndrome or for whom the diagnosis was made on their initial consultation. Intervention: Tonsillectomy. Main Outcome Measures: Resolution of symptoms at 3, 12, and 24 months after tonsillectomy. Results: Eight of the 9 patients achieved complete remission within 3 months. In the remaining patient, the frequency of episodes decreased from every 2 weeks to once every 3 to 4 months. This patient eventually had resolution of symptoms at 2 years after tonsillectomy. No complications resulted from the tonsillectomy. Conclusion: Tonsillectomy is a viable treatment option for patients with PFAPA syndrome.