title: Tonsillectomy in children with periodic fever with aphthous stomatitis, pharyngitis, and adenitis syndrome.
abstract: OBJECTIVE
  To seek evidence supporting a role for tonsillectomy or adenotonsillectomy in the management of affected children with periodic fever with aphthous stomatitis, pharnygitis, and adenitis (PFAPA) syndrome.
  
  
  METHODS
  A comprehensive literature search was conducted to identify all published English-language observational and randomized studies evaluating the efficacy of tonsillectomy or adenotonsillectomy on PFAPA syndrome. A combination of keywords was used to identify relevant articles.
  
  
  RESULTS
  A total of 15 studies including 149 treated children were found, including 13 observational noncomparative studies and 2 randomized controlled trials. The pooled rate of complete resolution emerging from the combined analysis of all treated children was 83% (95% CI, 77%-89%). A meta-analysis of the two randomized controlled trials showed homogeneity of the results (P=.37, Breslow-Day test) and a common odds ratio for complete resolution of 13 (95% CI, 4-43; P<.001).
  
  
  CONCLUSIONS
  Surgery appears to be a possible option for management of PFAPA syndrome. Available evidence is limited, however, and the precise role of surgery remains to be clarified. We suggest considering this option when symptoms markedly interfere with the child's quality of life and medical treatment has failed.
authors.affiliation: Department of Otorhinolaryngology, University of Milano-Bicocca, Monza, Italy. werner.garavello@unimib.it
authors.forename: Werner
authors.initials: W
authors.lastname: Garavello
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Pignataro
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Gaini
authors.forename: Sara
authors.initials: S
authors.lastname: Torretta
authors.forename: Edgardo
authors.initials: E
authors.lastname: Somigliana
authors.forename: Renato
authors.initials: R
authors.lastname: Gaini
mesh_terms: D000233:Adenoidectomy; D002648:Child; D005334:Fever / Q000601:surgery*; D006801:Humans; D008199:Lymphadenitis / Q000601:surgery*; D010507:Periodicity; D010612:Pharyngitis / Q000601:surgery*; D016032:Randomized Controlled Trials as Topic; D013281:Stomatitis, Aphthous / Q000601:surgery*; D013577:Syndrome; D014068:Tonsillectomy