Effectiveness Of Tonsillectomy In Periodic Fever, Aphthous Stomatitis, Pharyngitis, And Cervical Adenitis Syndrome: A Case Series

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by periodic high fever (febrile attacks) lasting for 3-6 days and recurring regularly. In this syndrome, fever is associated with aphthous stomatitis, pharyngitis, and cervical adenitis.(1-4) Almost all patients with PFAPA syndrome spontaneously resolve approximately 8 years after the onset of symptoms. However, the cause and etiology of PFAPA syndrome remain unclear. 4 Oral prednisolone reduces fever during febrile attacks.(5-7) Tonsillectomy has also been reported to induce remission of PFAPA syndrome.(8-12) However, tonsillectomy is not a popular treatment for PFAPA syndrome because its effectiveness is still unclear. Furthermore, tonsillectomy is generally recommended for patients aged 3 years and older to avoid post-operative complications.(13)We report here on four patients aged 3 years and older with PFAPA syndrome. Tonsillectomy completely eliminated febrile attacks and was safely performed without post-operative complications in all of our patients.