Methods: Patients with PFAPA seen at Vanderbilt Children’s Hospital from 2000-2015 were identified by chart review. Parents/guardians were contacted by phone and administered a structured questionnaire to identify patients who underwent palatine tonsillectomy and determine their subsequent outcome. Episode characteristics of those with and without complete resolution of episodes following tonsillectomy were compared with the chi-square or Mann-Whitney U test.
Results: A total of 37 patients with PFAPA who underwent tonsillectomy were identified. Of these, 70% had complete resolution of episodes, 16% had less severe or less frequent episodes, 5% reported “feverless” episodes with symptoms of PFAPA episodes without fever, and 8% had a period of remission followed by recurrence of episodes 9 months to 9 years following tonsillectomy. Over 80% of respondents considered tonsillectomy “very effective” in treating PFAPA. Those with complete resolution began having episodes at a later age than those without (23 months vs. 15 months of age, p=0.04). Only one symptom was associated with poor response; 47% with abdominal pain had complete resolution, whereas 90% without abdominal pain had complete resolution (p=0.01). Other symptoms during flares and the patient’s age at tonsillectomy were not significantly associated with outcome.
Conclusion: Tonsillectomy was curative in the majority of patients with PFAPA, and those with continued flares had significant improvement in the severity of episodes. Those with abdominal pain during episodes and early age of episode onset were less likely to have complete resolution of episodes after tonsillectomy. Further studies are needed to determine whether additional sites of lymph tissue involvement like the abdomen might correlate with an early age of episode onset and abdominal pain during PFAPA flares.