Is there a role for the otolaryngologist in PFAPA syndrome? A systematic review

To define the role of medical or surgical treatment in patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA syndrome).

A Medline search was performed using the terms PFAPA, periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis, treatment. A systematic review of the English literature was performed. Papers on pyrexia of unknown origin in the pediatric population were excluded; this was because the clinical entities included in the differential diagnosis of pyrexia of unknown origin share many clinical characteristics with PFAPA, and may be ill defined.

Twenty-seven papers have been published since 1989, of which 20 were in English language. There were five single case reports and two papers involving two patients each. There were 6 retrospective reviews of case notes, involving 5-94 patients over a 3-10 year review period. Given that current evidence on the effectiveness of tonsillectomy in PFAPA is extremely weak (level of evidence V), tonsillectomy should not be performed.

PFAPA usually resolves without any long-term adverse effect, and as such, there is no role for tonsillectomy in these patients.