Effect of adenotonsillectomy in PFAPA syndrome

Objective: To assess the benefits of adenotonsillectomy in the treatment of pediatric patients with PFAPA (periodic fever, aphthous ulcers, pharyngitis, and adenitis) syndrome. Design: Prospective case series. Setting: Tertiary care pediatric hospital. Patients: Pediatric patients meeting criteria for PFAPA syndrome. Intervention: Tonsillectomy with or without adenoidectomy. Main Outcome Measure: Resolution of PFAPA symptoms. Results: Twenty-seven (14 female, 13 male) children with PFAPA syndrome underwent tonsillectomy with or without adenoidectomy from 2004 through 2006. The length of follow-up for all patients ranged from 8 to 41 months. A total of 26 patients experienced a complete resolution of their symptoms. The 1 child who continued to have febrile episodes had fever cycles that were not regular in duration or interval and in hindsight was not likely a patient with PFAPA syndrome. Conclusions: Our findings showed complete resolution of symptoms in 26 of 27 patients with PFAPA syndrome treated surgically. Patients who meet clinical criteria for PFAPA syndrome should be considered for tonsillectomy and adenoidectomy if they do not respond to medical management.