University of Oulu

Lantto, U., Koivunen, P., Tapiainen, T. and Renko, M. (2021), Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: Relapse and Tonsillar Regrowth After Childhood Tonsillectomy. The Laryngoscope, 131: E2149-E2152.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome : relapse and tonsillar regrowth after childhood tonsillectomy

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Author: Lantto, Ulla1,2; Koivunen, Petri1,2; Tapiainen, Terhi1,3;
Organizations: 1PEDEGO Research Unit, Medical Research Center, University of Oulu, Oulu, Finland
2Department of Otorhinolaryngology, Head and Neck Surgery, Oulu University Hospital, Oulu, Finland
3Department of Pediatrics and Adolescent Medicine, Oulu University Hospital, Oulu, Finland
4Department of Pediatrics, University of Eastern Finland and Kuopio University Hospital, Kuopio, Finland
Format: article
Version: published version
Access: open
Online Access: PDF Full Text (PDF, 0.4 MB)
Persistent link:
Language: English
Published: John Wiley & Sons, 2021
Publish Date: 2021-09-16


Objectives/Hypothesis: Tonsillectomy is an effective treatment for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, but the role of adenoidectomy, as well as later tonsillar regrowth, is unclear. To find out if the volume of lymphoid tissue is pivotal to the efficacy, we analyzed the association between the relapse of the symptoms of PFAPA syndrome and regrowth of tonsillar tissue after tonsillectomy or adenotonsillectomy.

Study Design: Prospective cohort study of operated PFAPA pateints.

Methods: We invited all patients that had undergone tonsillectomy or adenotonsillectomy due to PFAPA syndrome at the Oulu University Hospital, Oulu, Finland, between the years 1990 and 2007, at the age of ≤12 years, to a follow-up visit, after an average period of 9.8 years after their diagnoses. Out of the 132 invited, 94 (71%) participated in the follow-up study.

Results: At the follow-up study visit, 5 (5%) of the 94 PFAPA syndrome cases experienced recurrent fevers. The regrowth of palatine tonsillar tissue was seen in four of them (80%) as compared to 19/89 (21%) of symptom-free patients (P = 0.006). Two of the patients with clear PFAPA relapse at the time of the study visit were reoperated with clear effect on the symptoms. At the time of the study visit, 59/63 (94%) of the patients who had undergone adenotonsillectomy and 30/31 of the patients (97%) who had undergone tonsillectomy earlier were free of fever flares (P = 0.99).

Conclusion: Palatine tonsil regrowth was associated with PFAPA syndrome relapse after tonsillectomy. Reoperation might be a treatment option in these patients.

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Series: Laryngoscope
ISSN: 0023-852X
ISSN-E: 1531-4995
ISSN-L: 0023-852X
Volume: 131
Issue: 7
Pages: E2149 - E2152
DOI: 10.1002/lary.29474
Type of Publication: A1 Journal article – refereed
Field of Science: 3123 Gynaecology and paediatrics
3125 Otorhinolaryngology, ophthalmology
Copyright information: © 2021 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.