title: Outcome of tonsillectomy in selected patients with PFAPA syndrome.
abstract: 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.
authors.affiliation: 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
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Pignataro
authors.forename: Sara
authors.initials: S
authors.lastname: Torretta
authors.forename: Maria Cristina
authors.initials: MC
authors.lastname: Pietrogrande
authors.forename: Rosa Maria
authors.initials: RM
authors.lastname: Dellepiane
authors.forename: Paola
authors.initials: P
authors.lastname: Pavesi
authors.forename: Anna
authors.initials: A
authors.lastname: Bossi
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Drago
authors.forename: Pasquale
authors.initials: P
authors.lastname: Capaccio
mesh_terms: 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