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BMC Ear, Nose and Throat Disorders
Published in: BMC Ear, Nose and Throat Disorders 1/2018

Open Access 01-12-2018 | Research article

The role of tonsillectomy in the Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome; a literature review

Authors: Jostein Førsvoll, Knut Øymar

Published in: BMC Ear, Nose and Throat Disorders | Issue 1/2018

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Abstract

Background

Tonsillectomy (TE) or adenotonsillectomy (ATE) may have a beneficial effect on the clinical course in children with the Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis (PFAPA) syndrome. However, an immunological reason for this effect remains unknown. This literature review summarizes the current knowledge of the effect of TE or ATE in the PFAPA syndrome.

Methods

A search of PubMed, Medline, EMBASE and Cochrane was conducted for papers written in English dated from 1 January 1987 to 31 December 2016. The search included all studies reporting outcomes after TE or ATE from children aged 0 to 18 years with PFAPA.

Results

Two randomized controlled trials reported significantly faster resolution of febrile episodes after TE or ATE in children with PFAPA compared to controls (non-surgery groups). We identified 28 case series including 555 children with PFAPA. The diagnosis was set prospectively before surgery in 440 children and retrospectively after surgery in 115 of the children. TE or ATE had a curative effect in 509 of the 555 children with PFAPA (92%), but few studies were of high quality.

Conclusion

TE or ATE may have a curative effect on children with PFAPA, but the evidence is of moderate quality. Further high-quality randomized controlled studies are still needed.
Appendix
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Metadata
Title
The role of tonsillectomy in the Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome; a literature review
Authors
Jostein Førsvoll
Knut Øymar
Publication date
01-12-2018
Publisher
BioMed Central
Published in
BMC Ear, Nose and Throat Disorders / Issue 1/2018
Electronic ISSN: 1472-6815
DOI
https://doi.org/10.1186/s12901-017-0049-5

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