Effectiveness of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome: a case series

  • Kazuto Taniguchi Tara Town Hospital, Tara, Saga, Japan
  • Sakumo Kii Tara Town Hospital, Tara, Saga, Japan
  • Kazuyasu Uemichi Tara Town Hospital, Tara, Saga, Japan
DOI: https://doi.org/10.14238/pi61.3.2021.171-4
Keywords: tonsillectomy, PFAPA syndrome, febrile attack, remission, therapeutic strategy

Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by periodic high fever (febrile attacks) lasting for 3–6 days and recurring regularly. In this syndrome, fever is associated with aphthous stomatitis, pharyngitis, and cervical adenitis.1-4 Almost all patients with PFAPA syndrome spontaneously resolve approximately 8 years after the onset of symptoms. However, the cause and etiology of PFAPA syndrome remain unclear.4 Oral prednisolone reduces fever during febrile attacks.5-7 Tonsillectomy has also been reported to induce remission of PFAPA syndrome.8-12 However, tonsillectomy is not a popular treatment for PFAPA syndrome because its effectiveness is still unclear. Furthermore, tonsillectomy is generally recommended for patients aged 3 years and older to avoid post-operative complications.13

We report here on four patients aged 3 years and older with PFAPA syndrome. Tonsillectomy completely eliminated febrile attacks and was safely performed without post-operative complications in all of our patients.

 

References

1. Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr. 1987;110:43-6. DOI: 10.1016/s0022-3476(87)80285-8.
2. Marshall GS, Edwards KM, Lawton AR. PFAPA syndrome. Pediatr Infect Dis J. 1989;8:658-9. DOI: 10.1097/00006454-198909000-00026.
3. Thomas KT, Feder HM Jr, Lawton AR, Edwards KM. Periodic fever syndrome in children. J Pediatr. 1999;135:15-21. DOI: 10.1016/s0022-3476(99)70321-5.
4. Feder HM, Salazar JC. A clinical review of 105 patients with PFAPA (a periodic fever syndrome). Acta Paediatr. 2010;99:178-84. DOI: 10.1111/j.1651-2227.2009.01554.x.
5. Feder HM Jr. Cimetidine treatment for periodic fever associated with aphthous stomatitis, pharyngitis, and cervical adenitis. Pediatr Infect Dis J. 1992;11:318-21. DOI: 10.1097/00006454-199204000-00011.
6. Pinto A, Lindemeyer RG, Sollecito TP. The PFAPA syndrome in oral medicine: differential diagnosis and treatment. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006;102:35-9. DOI: 10.1016/j.tripleo.2005.12.023.
7. Wurster VM, Carlucci JG, Feder HM Jr, Edwards KM. Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. J Pediatr. 2011;159:958-64. DOI: 10.1016/j.jpeds.2011.06.004.
8. Galanakis E, Papadakis CE, Giannoussi E, Karatzanis AD, Bitsori M, Helidonis ES. PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child. 2002;86:434-5. DOI: 10.1136/adc.86.6.434.
9. Renko M, Salo E, Putto-Laurila A, Saxen H, Mattila PS, Luotonen J, et al. A randomized, controlled trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. J Pediatr. 2007;151:289-92. DOI: 10.1016/j.jpeds.2007.03.015.
10. Licameli G, Jeffrey J, Luz J, Jones D, Kenna M. Effect of adenotonsillectomy in PFAPA syndrome. Arch Otolaryngol Head Neck Surg. 2008;134:136-40. DOI: 10.1001/archoto.2007.7.
11. Garavello W, Romagnoli M, Gaini RM. Effectiveness of adenotonsillectomy in PFAPA syndrome: a randomized study. J Pediatr. 2009;155:250-3. DOI: 10.1016/j.jpeds.2009.02.038.
12. Peridis S, Pilgrim G, Koudoumnakis E, Athanasopoulos I, Houlakis M, Parpounas K. PFAPA syndrome in children: a meta-analysis on surgical versus medical treatment. Int J Pediatr Otorhinolaryngol. 2010;74:1203-8. DOI: 10.1016/j.ijporl.2010.08.014.
13. Baugh RF, Archer SM, Mitchell RB, Rosenfeld RM, Amin R, Burns JJ, et al. Clinical practice guideline: tonsillectomy in children. Otolaryngol Head Neck Surg. 2011;144:S1-30. DOI: 10.1177/0194599810389949.
14. Murata T, Okamoto N, Shimizu T, Tamai H. Diagnosis and management of periodic fever with aphthous pharyngitis and adenitis (PFAPA). Nihon Rinsho Meneki Gakkai Kaishi. 2007;30:101-7. DOI: 10.2177/jsci.30.101.
15. Vanoni F, Theodoropoulou K, Hofer M. PFAPA syndrome: a review on treatment and outcome. Pediatr Rheumatol Online J. 2016;14:38. DOI: 10.1186/s12969-016-0101-9.
16. Tasher D, Somekh E, Dalal I. PFAPA syndrome: new clinical aspects disclosed. Arch Dis Child. 2006;91:981-4. DOI: 10.1136/adc.2005.084731.
Published
2021-04-29
How to Cite
1.
Taniguchi K, Kii S, Uemichi K. Effectiveness of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome: a case series. PI [Internet]. 29Apr.2021 [cited 17Apr.2024];61(3):171-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/2349
Issue
Vol 61 No 3 (2021): May 2021
Section
Case Report

Copyright (c) 2021 Kazuto Taniguchi, Sakumo Kii, Kazuyasu Uemichi

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Authors who publish with this journal agree to the following terms:

Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.

Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Received 2019-12-02
Accepted 2021-04-29
Published 2021-04-29