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Tonsillectomy in children with periodic fever with aphthous stomatitis, pharyngitis, and adenitis syndrome

Werner Garavello1, Lorenzo Pignataro, Lorenzo Gaini

  • 1Department of Otorhinolaryngology, University of Milano-Bicocca, Monza, Italy. werner.garavello@unimib.it

The Journal of Pediatrics
|February 9, 2011
PubMed

Insights

Tonsillectomy or adenotonsillectomy may effectively treat periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in children. Surgery offers an 83% resolution rate, particularly when other treatments fail and symptoms impact quality of life.

Area of Science:

  • Pediatric Otolaryngology
  • Pediatric Immunology
  • Evidence-Based Medicine

Background:

  • Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a common autoinflammatory disorder in children.
  • Management strategies for PFAPA syndrome often include medical therapies, but surgical interventions are also considered.
  • Tonsillectomy and adenotonsillectomy are surgical procedures involving the removal of tonsils and adenoids.

Purpose of the Study:

  • To evaluate the efficacy of tonsillectomy or adenotonsillectomy in managing children diagnosed with PFAPA syndrome.
  • To synthesize existing evidence regarding surgical outcomes for PFAPA syndrome.
  • To determine the role of surgical intervention in the treatment algorithm for PFAPA syndrome.

Main Methods:

  • A systematic literature search was performed to identify relevant English-language studies.
  • Included studies encompassed observational and randomized controlled trials assessing tonsillectomy or adenotonsillectomy for PFAPA syndrome.
  • Data from 15 studies involving 149 children were analyzed.

Main Results:

  • The pooled complete resolution rate following surgery for PFAPA syndrome was 83% (95% CI, 77%-89%).
  • A meta-analysis of two randomized controlled trials indicated a significant odds ratio of 13 (95% CI, 4-43; P<.001) for complete resolution after surgery.
  • The results demonstrated homogeneity across the included randomized controlled trials.

Conclusions:

  • Tonsillectomy or adenotonsillectomy is a viable surgical option for managing PFAPA syndrome in children.
  • While evidence suggests high efficacy, further research is needed to fully clarify the precise role of surgery.
  • Surgery should be considered for children with PFAPA syndrome when symptoms severely affect quality of life and medical treatments have been unsuccessful.
Abstract

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