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Evidence summaries

Tonsillectomy for Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome (Pfapa)

Tonsillectomy may be a useful treatment option in the management of children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. Level of evidence: "C"

The quality of evidence is downgraded and by inconsistency (statistical heterogeneity), and by imprecise results (few patients and outcome events).

Summary

A Cochrane review [Abstract] 1 included 2 studies with a total of 67 children. One study (n=39) included children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome diagnosed according to rigid, standard criteria, and compared adenotonsillectomy to watchful waiting with a follow-up of 18 months. The other study (n=28) applied less stringent criteria for diagnosing PFAPA and probably also included participants with alternative types of recurrent pharyngitis. This study compared tonsillectomy alone to no treatment with a follow-up of 6 months.

Children with PFAPA experienced less fever and less severe episodes after surgery compared to those receiving no surgery (table T1). The number of patients who had "immediate and persistent resolution of PFAPA syndrome" after the surgery/randomisation, which was sustained until the end of follow-up (at 18 months) was 12/19 in the surgery group and 1/20 in the control group in one study. In the other study, corresponding numbers for the six-month follow-up period (their longest available follow-up) were 10/14 in the surgery group and 4/12 in the control group. Both studies reported that there were no complications of surgery but the numbers of patients randomly allocated to surgery (19 and 14 patients respectively) were too small to detect potentially important complications such as haemorrhage. Quality of life, number of days with pain after surgery and absence from school were not measured or reported.

Surgery (tonsillectomy +/- adenoidectomy) for children with PFAPA

OutcomeRelative effect (95% CI)Assumed risk (control)Corresponding risk (surgery, 95% CI)Participants (studies)
* statistical heterogeneity, I2 = 71%
Complete resolution of symptomsRR 4.38 (0.64 to 30.11)*156 per 1000684 per 1000(100 to 1000)65 (2 studies)
Number of episodes of fever and associated symptomsRate ratio 0.08 (0.05 to 0.13)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)65 (2 studies)
Severity of episodes: number of days with fever and the associated symptoms (per episode)MD 1.8 days per episodeMean 3.5 (range of 2 to 6) days per episodeMean 1.7 (range of 2 to 4) days per episode39 (1 study)
Use of corticosteroidsRR 0.58 (0.37 to 0.92)900 per 1000522 per 1000(333 to 828)39 (1 study)

Comments

It is uncertain whether adenoidectomy combined with tonsillectomy adds any additional benefit to tonsillectomy alone. One study performed adenotonsillectomy while the other only removed the tonsils.

Note

Date of latest search:

    References

    • Burton MJ, Pollard AJ, Ramsden JD et al. Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA). Cochrane Database Syst Rev 2019;(12):CD008669. [PubMed]

Primary/Secondary Keywords