Background: PFAPA syndrome is characterized by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Tonsillectomy and adenotonsillectomy are surgical treatment options for this periodic fever syndrome, the significance of which will be discussed.
Material and methods: Between 2004 and 2010 we collected data of 36 patients with the diagnosis of PFAPA syndrome. Data analysis was carried out on the basis of structured questionnairs, patients' files as well as a systematic evaluation of international literature up to April 2011.
Results: The average age for the appearance of PFAPA episodes was 22 months and they recurred for an average duration of 3-5 days every 14-33 days. During a PFAPA attack, aphthous stomatitis was present in 70% of the patients, pharyngitis was present in 93% and cervical adenitis in 96%. The family history for recurrent fever was positive in 4 of the patients. In 85% cortikosteroids were the only effective medicative treatment with no further symptoms until the next attack. Surgery (tonsillectomy±adenoidectomy) aborted the PFAPA episodes in 10 of 16 patients, in 2 patients the frequency of episodes decreased, 3 patients had no noticeable change and 1 patient died as a result of postsurgical bleeding at another institution. After an average duration of illness of 4 years the PFAPA syndrome was in spontaneous remission in 8 patients.
Conclusion: Tonsillectomy is an effective treatment option for the PFAPA syndrome. Contrary to a general indication the decision should be personalized considering the benefit vs. the risk of operation with the advice of an ENT specialist.
Georg Thieme Verlag KG Stuttgart · New York.