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Cochrane Database of Systematic Reviews

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)

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Information

DOI:
https://doi.org/10.1002/14651858.CD008669.pub2Copy DOI
Database:
  1. Cochrane Database of Systematic Reviews
Version published:
  1. 11 September 2014see what's new
Type:
  1. Intervention
Stage:
  1. Review
Cochrane Editorial Group:
  1. Cochrane ENT Group

Copyright:
  1. Copyright © 2014 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.

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Authors

  • Martin J Burton

    Correspondence to: UK Cochrane Centre, Oxford, UK

    [email protected]

  • Andrew J Pollard

    Department of Paediatrics, University of Oxford, Children's Hospital, Oxford, UK

  • James D Ramsden

    Department of Otolaryngology ‐ Head and Neck Surgery, Oxford Radcliffe Hospitals NHS Trust, Oxford, UK

  • Lee Yee Chong

    UK Cochrane Centre, Oxford, UK

  • Roderick P Venekamp

    Department of Otorhinolaryngology & Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, Netherlands

Contributions of authors

Martin Burton: conceived protocol, extracted data, conducted analysis, reported results, contributed to final paper.
Andrew Pollard: conceived protocol, contributed to final paper.
James Ramsden: conceived protocol, extracted data, conducted analysis, reported results, contributed to final paper.
Roderick Venekamp: conducted searches, identified studies, extracted data, conducted analysis and prepared manuscript for the updated review in 2014.
Lee Yee Chong: identified studies, contacted authors, extracted data, conducted analysis and prepared manuscript for the update review in 2014.
All authors have reviewed and provided comment on the updated version of the review.

Sources of support

Internal sources

  • None, Other.

External sources

  • None, Other.

Declarations of interest

None known.

Acknowledgements

We gratefully acknowledge the assistance received from the staff at the Cochrane ENT Disorders Group editorial base in Oxford and gratefully thank Samantha Faulkner and Gemma Sandberg for their support with the search strategy and searches. We would also like to thank the peer reviewers, Mr William McKerrow and Dr Andrew Riordan, and editors for their valuable feedback, and the study authors (Marjo Renko) for responding to our queries.

Version history

Published

Title

Stage

Authors

Version

2019 Dec 30

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)

Review

Martin J Burton, Andrew J Pollard, James D Ramsden, Lee‐Yee Chong, Roderick P Venekamp

https://doi.org/10.1002/14651858.CD008669.pub3

2014 Sep 11

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)

Review

Martin J Burton, Andrew J Pollard, James D Ramsden, Lee Yee Chong, Roderick P Venekamp

https://doi.org/10.1002/14651858.CD008669.pub2

2010 Sep 08

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)

Review

Martin J Burton, Andrew J Pollard, James D Ramsden

https://doi.org/10.1002/14651858.CD008669

Differences between protocol and review

In the 2014 update, we expanded the objective of the review to include evaluation of both clinical effectiveness and safety. We added additional outcomes to the review protocol, to ensure that both potential harms and benefits that are important to patients were captured in the review. These were complications of surgery (haemorrhage and number of days with postoperative pain), number of courses of corticosteroids, absence or time off school and quality of life.

Keywords

MeSH

PICOs

Population
Intervention
Comparison
Outcome

The PICO model is widely used and taught in evidence-based health care as a strategy for formulating questions and search strategies and for characterizing clinical studies or meta-analyses. PICO stands for four different potential components of a clinical question: Patient, Population or Problem; Intervention; Comparison; Outcome.

See more on using PICO in the Cochrane Handbook.

Process for sifting search results and selecting studies for inclusion
Figures and Tables -
Figure 1

Process for sifting search results and selecting studies for inclusion

'Risk of bias' graph: review authors' judgements about each methodological quality item presented as percentages across all included studies.
Figures and Tables -
Figure 2

'Risk of bias' graph: review authors' judgements about each methodological quality item presented as percentages across all included studies.

'Risk of bias' summary: review authors' judgements about each methodological quality item for each included study.
Figures and Tables -
Figure 3

'Risk of bias' summary: review authors' judgements about each methodological quality item for each included study.

Comparison 1 Surgery (tonsillectomy +/‐ adenoidectomy) versus control, Outcome 1 Proportion of patients whose symptoms have completely resolved.
Figures and Tables -
Analysis 1.1

Comparison 1 Surgery (tonsillectomy +/‐ adenoidectomy) versus control, Outcome 1 Proportion of patients whose symptoms have completely resolved.

Comparison 1 Surgery (tonsillectomy +/‐ adenoidectomy) versus control, Outcome 2 Number of episodes of fever and associated symptoms (per person‐month).
Figures and Tables -
Analysis 1.2

Comparison 1 Surgery (tonsillectomy +/‐ adenoidectomy) versus control, Outcome 2 Number of episodes of fever and associated symptoms (per person‐month).

Comparison 1 Surgery (tonsillectomy +/‐ adenoidectomy) versus control, Outcome 3 Use of corticosteroids.
Figures and Tables -
Analysis 1.3

Comparison 1 Surgery (tonsillectomy +/‐ adenoidectomy) versus control, Outcome 3 Use of corticosteroids.

Summary of findings for the main comparison. Surgery (tonsillectomy +/‐ adenoidectomy) for children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)

Surgery (tonsillectomy +/‐ adenoidectomy) for children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)

Patient or population: children with PFAPA
Settings: Europe (Finland/Italy) in tertiary hospitals
Intervention: surgery (tonsillectomy +/‐ adenoidectomy)

Outcomes

Illustrative comparative risks* (95% CI)

Relative effect
(95% CI)

No of participants
(studies)

Quality of the evidence
(GRADE)

Comments

Assumed risk

Corresponding risk

Control

Surgery (tonsillectomy +/‐ adenoidectomy)

Complete resolution of symptoms

(proportion of patients with 'immediate and persistent resolution of symptoms' from the point of surgery/randomisation up to end of study follow‐up)

Study population (average)

RR 4.38
(0.64 to 30.11)

65
(2 studies)

⊕⊕⊕⊝
moderate1

The NNTB based on the study population risk was 1/(684‐156)*1000 = 1.89

1 study followed up patients up to 6 months; another up to 18 months

156 per 1000

684 per 1000
(100 to 1000)

Medium risk population

192 per 1000

841 per 1000
(123 to 1000)

Complications of surgery

(haemorrhage and days with pain)

Both studies reported no complications from surgery

Days of pain not reported as an outcome

Number of episodes of fever and associated symptoms

(episodes per person per month)

Mean 0.5 episode per person per month (1 episode every 2 months)

Mean 0.04 episode per person per month (1 episode every 25 months)

Rate ratio: 0.08 (0.05 to 0.13)

65
(2 studies)

⊕⊕⊕⊝
moderate1

Severity of episodes

(number of days with fever and the associated symptoms (per episode))

Mean 3.5 (range of 2 to 6) days per episode

Mean 1.7 (range of 2 to 4) days per episode

Mean difference: 1.8 days per episode

39

(1 study)

⊕⊕⊕⊝
moderate1

Study reported statistical significance. Standard deviations were not reported in the study for average number of days per episode

Use of corticosteroids

(proportion of patients using corticosteroids)

Study population (average)

RR 0.58

(0.37 to 0.92)

39

(1 study)

⊕⊕⊝⊝
low1,2

Review intended to report number of courses/patient, but these data were not available in the studies

900 per 1000

522 per 1000
(333 to 828)

Absence or time off school

None of the studies reported this

Quality of life

None of the studies reported this

*The basis for the assumed risk was the median risk in the control groups for "medium risk population" and for "study population", this was the average (i.e. total number of participants with events divided by total number of participants included in the meta‐analysis). The corresponding risk (and its 95% confidence interval) is based on the assumed risk in the comparison group and the relative effect of the intervention (and its 95% CI).
CI: confidence interval; NNTB: number needed to treat to benefit; RR: risk ratio

GRADE Working Group grades of evidence
High quality: Further research is very unlikely to change our confidence in the estimate of effect.
Moderate quality: Further research is likely to have an important impact on our confidence in the estimate of effect and may change the estimate.
Low quality: Further research is very likely to have an important impact on our confidence in the estimate of effect and is likely to change the estimate.
Very low quality: We are very uncertain about the estimate.

1Quality (confidence in the evidence) was reduced because of the small size of the studies and broad confidence intervals. There was also concern as to whether the treatment received by the control group reflects current practice. The criteria for recruitment of patients into one of the studies was not stringent and could have included patients who did not have PFAPA (Renko 2007).
2Additional concerns about the applicability of this outcome. Measuring the proportion of patients who had received steroids might not reflect the potential harms from this alternative treatment, which increases with the number of courses used. The numbers used in our analysis were estimations from the percentages reported in the paper, with the assumption that all patients randomised were included in the analysis. However, it is possible that not all participants were included in the study's analysis (i.e. some loss of data).

Figures and Tables -
Summary of findings for the main comparison. Surgery (tonsillectomy +/‐ adenoidectomy) for children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)
Comparison 1. Surgery (tonsillectomy +/‐ adenoidectomy) versus control

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

1 Proportion of patients whose symptoms have completely resolved Show forest plot

2

65

Risk Ratio (M‐H, Random, 95% CI)

4.38 [0.64, 30.11]

2 Number of episodes of fever and associated symptoms (per person‐month) Show forest plot

2

65

Rate Ratio (Fixed, 95% CI)

0.08 [0.05, 0.13]

3 Use of corticosteroids Show forest plot

1

39

Risk Ratio (M‐H, Fixed, 95% CI)

0.58 [0.37, 0.92]

Figures and Tables -
Comparison 1. Surgery (tonsillectomy +/‐ adenoidectomy) versus control