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

Jostein Førsvoll,Knut Øymar

doi:10.1186/s12901-017-0049-5

Jostein Førsvoll, Knut Øymar

Open Access

https://doi.org/10.1186/s12901-017-0049-5

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Abstract

BackgroundTonsillectomy (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.MethodsA 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.ResultsTwo 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.ConclusionTE 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.

Highlights

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
After a review of the titles, abstracts, and reference lists of the relevant manuscripts, two randomized controlled trials (RCTs) and 28 case series reporting the outcome after TE or ATE in children with PFAPA were identified
The outcome of PFAPA after tonsillectomy with or without adenoidectomy The first RCT performed by Renko et al included 26 children with PFAPA [17]

Summary

Introduction

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. The Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis (PFAPA) syndrome is the most common paediatric periodic fever syndrome [1, 2], with a cumulative incidence of 2.2 per 10.000 children up to the age of 5 years in a Nordic population [2]. The hallmarks of the disease are short (3–5 days), regularly occurring episodes of high fever accompanied by at least one of the following major symptoms: pharyngitis, cervical adenitis and aphthous stomatitis [3,4,5]. The clinical entity was first described by Marshall et al in 1987 [12], and in 1989 they presented the acronym PFAPA and suggested a set of diagnostic criteria for the syndrome [13].

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