Outcome of tonsillectomy in selected patients with PFAPA syndrome.
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).


METHODS
A prospective study.


METHODS
Secondary pediatric and otolaryngological university center.


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


METHODS
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.


METHODS
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.


CONCLUSIONS
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.
Pignataro
Lorenzo
L
Department of Otorhinolaryngological and Ophthalmological Sciences, University of Milan, Fondazione IRCCS-Ospedale Maggiore Policlinico, Mangiagalli and Regina Elena, Via F. Sforza 35, Milan, Italy. lorenzo.pignataro@unimi.it
Torretta
Sara
S
Pietrogrande
Maria Cristina
MC
Dellepiane
Rosa Maria
RM
Pavesi
Paola
P
Bossi
Anna
A
Drago
Lorenzo
L
Capaccio
Pasquale
P
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D005260:Female; D006801:Humans; D008199:Lymphadenitis / Q000601:surgery / Q000628:therapy*; D008297:Male; D010612:Pharyngitis / Q000628:therapy*; D013281:Stomatitis, Aphthous / Q000628:therapy*; D013577:Syndrome; D014068:Tonsillectomy; D016896:Treatment Outcome; D055815:Young Adult