Background/Purpose: Periodic Fevers with Aphthous Ulcers, Pharyngitis, and Adenitis Syndrome (PFAPA Syndrome) is the most common pediatric periodic fever syndrome. The most recent diagnostic criteria was described by Thomas et al. in 1999 and defines PFAPA as; regular periodic fevers lasting 3 to 7 days, occurring every 3 to 8 weeks, accompanied by at least one of the following symptoms: aphthous stomatitis, pharyngitis, and cervical adenitis. Between febrile episodes the patient is well and demonstrates normal growth and development. Treatment of PFAPA has considerable heterogenicity between practitioners, and there is no standard of care given a lack of clinical trials. Fortunately, PFAPA is relatively benign and will usually resolve spontaneously over time. In 1989, Abramson and colleagues described successful fever resolution post-tonsillectomy. Post-tonsillectomy, patients show a complete response with immediate cessation of fevers, a partial response with fever cessation followed by recurrence, or a failure with no fever cessation. The role of tonsillectomy in PFAPA treatment remains controversial. Our study aimed to determine the effectiveness of tonsillectomy as curative treatment of PFAPA compared to conservative non-surgical management.

Methods: We conducted a single center retrospective cohort study of children with PFAPA who were seen at the Alberta Childrens Hospital Rheumatology Clinic from January 2015 to July 2020. Inclusion criteria was based on the modified Thomas criteria for PFAPA. Exclusion criteria included patient with the Familial Mediterranean Fever gene variants, monogenic autoinflammatory diseases, and those that underwent tonsillectomy for another reason. We compared the time to fever resolution between the patients who underwent tonsillectomy and those who did not.

Results: We reviewed 113 charts of potential PFAPA patients and identified 34 patients who met inclusion criteria for typical PFAPA syndrome. 13 (38.2%) underwent tonsillectomy and 21 did not. In the tonsillectomy group, 10 were found to have complete response with immediate cessation of their fevers following tonsillectomy, 2 had a partial response, and 1 had no response. Patient characteristics were comparable; average age of fever onset was 2.9 and 3 years in the tonsillectomy and non-tonsillectomy groups and the average duration of PFAPA symptoms was 2.6 years and 3.2 years respectively.

Conclusion: Our study demonstrates that tonsillectomy may be beneficial in shorting the duration of PFAPA symptoms by approximately 6 months. Given the associated surgical risks, careful consideration should be given prior to recommending tonsillectomy as curative treatment.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-effectiveness-of-tonsillectomy-in-periodic-fever-aphthous-ulcer-pharyngitis-and-adenitis-syndrome-in-pediatric-patients/