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Lorenzo Pignataro, MD; Sara Torretta, MD; Maria Cristina Pietrogrande, MD; Rosa Maria Dellepiane, MD; Paola Pavesi, MD; Anna Bossi, MD; Lorenzo Drago, MD; Pasquale Capaccio, MD

Arch Otolaryngol Head Neck Surg. 2009;135(6):548-553.

Objective  To assess the practicability of integrated medical and surgical management and the effectiveness of tonsillectomy in children with PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and cervical lymphadenopathy).

Design  A prospective study.

Setting  Secondary pediatric and otolaryngological university center.

Patients  Of 30 patients evaluated for periodic fever, 18 children with PFAPA syndrome were included in the study.

Interventions  Patients underwent long-term pediatric and otolaryngological assessments, and their parents were asked to keep monthly diaries with reports of any subsequent episodes, symptom, and related sign. Patients received traditional medical therapies, and 9 patients underwent tonsillectomy for the lack of lasting recovery.

Main Outcomes Measures  The association between postoperative outcomes and age at tonsillectomy and the differences in the patients' condition before and after tonsillectomy were statistically tested. In addition, the removed tonsillar tissue was analyzed molecularly to evaluate concomitant infections.

Results  All of the surgical patients reported a symptomatic improvement, with complete clinical recovery in 5 cases (56%) and significant reduction in number (P = .005) and duration (P = .03) of recurrences in the remaining 4 (44%). Results of molecular analysis of tonsillar specimens were negative for bacteria in all but 1 patient.

Conclusion  Otolaryngologists should be trained to recognize PFAPA syndrome, for which management consists of a regular and prolonged second-level pediatric and otolaryngological follow-up, with surgery only after the failure of traditional medical therapy.

Author Affiliations: Department of Otorhinolaryngological and Ophthalmological Sciences (Drs Pignataro, Torretta, and Capaccio), Department of Pediatrics, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS)–Ospedale Maggiore Policlinico, Mangiagalli and Regina Elena (Drs Pietrogrande, Dellepiane, and Pavesi), G. A. Maccacaro Institute of Medical Statistics and Biometrics (Dr Bossi), and Division of Clinical Microbiology, Department of Preclinical Science (DISP) LITA (Dr Drago), University of Milan, Milan, Italy.

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