Tonsillectomy as treatment of PFAPA syndrome

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Periodic-Fever-Aphthous stomatitis-Pharyngitis and cervical Adenitis (PFAPA) syndrome is a curious, troublesome and not uncommon pediatric diagnosis. Diagnosis is entirely clinical, with the primary feature of clockwork periodicity of fever every 3-6 weeks for 3-5 days with little else. The cause is unknown, but the course is known likely to be persistent over years before spontaneous, full resolution. Case series have all reported a curious, apparent, immediate, curative effect of tonsillectomy in many affected children. In this issue of The Journal, Renko et al report results of a randomized controlled trial of tonsillectomy versus follow-up alone in 26 children with PFAPA enrolled at mean age of 4.1 years after at least 5 predictable, periodic episodes of fever. Six months after randomization, all 14 children who underwent tonsillectomy were free of symptoms, whereas 6 of 12 children randomized to follow-up alone continued to have periodic fever; 5 of these 6 children then underwent tonsillectomy and were promptly “cured.” With caveats of small sample size, inability to blind and inability to ascertain specificity of diagnosis of PFAPA (and noting that 29% had exudative tonsillitis, which is distinctly unusual in case series from the United States), these results are impressive, and “cure” is as curious as is the problem.