Role of Tonsillectomy in PFAPA Syndrome: A Literature Review and Retrospective Study
DOI:
https://doi.org/10.34631/sporl.1059Keywords:
PFAPA Syndrome, Tonsillectomy, Corticotherapy, ColchicineAbstract
Objective: Literature review and compare the pharmacological therapeutics effectiveness with the tonsillectomy in the disease remission.
Material and methods: Retrospective analysis of clinical data (age, gender, clinic, family history), types of treatment (pharmacological and/or surgical), time of remission (after medical and surgical treatment) and remission in the 1st, 3rd and 6th month after tonsillectomy in children with PFAPA syndrome evaluated at Hospital Pedro Hispano between January 2016 and December 2021.
Results: Nine children were evaluated (56% female, 44% male) aged between 1 and 4 years old (mean age 1.9 ± 1.3 years). At diagnosis, the mean number of weeks of remission was 4.0 ± 1.8. After corticotherapy it was 3.88 ± 1.0 and after tonsillectomy it was 50.6 ± 33.8 weeks.
Conclusion: Tonsillectomy has demonstrated to be a very useful therapeutic option in the disease’s remission in children with PFAPA syndrome.
References
Thomas KT, Feder HM Jr, Lawton AR, Edwards KM. Periodic fever syndrome in children. J Pediatr. 1999 Jul;135(1):15-21. doi: 10.1016/s0022-3476(99)70321-5.
Forsvoll J, Kristoffersen EK, Oymar K. Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study. Acta Paediatr. 2013 Feb;102(2):187-92. doi: 10.1111/apa.12069.
Sparud-Lundin C, Berg S, Fasth A, Karlsson A, Wekell P. From uncertainty to gradually managing and awaiting recovery of a periodic condition—a qualitative study of parents’ experiences of PFAPA syndrome. BMC Pediatr. 2019 Apr 8;19(1):99. doi: 10.1186/s12887-019-1458-y.
Feder HM, Salazar JC. A clinical review of 105 patients with PFAPA (a periodic fever syndrome). Acta Paediatr. 2010 Feb;99(2):178-84. doi: 10.1111/j.1651-2227.2009.01554.x.
Marshall GS, Edwards KM, Butler J, Lawton AR. Syndromes of periodic fever pharyngitis and aphthous stomatitis. J Pediatr. 1987 Jan;110(1):43-6. doi: 10.1016/s0022-3476(87)80285-8.
Tasher D, Somekh E, Dalal I. PFAPA syndrome: new clinical aspects disclosed. Arch Dis Child. 2006 Dec;91(12):981-4. doi: 10.1136/adc.2005.084731.
Abramson JS, Givner LB, Thompson JN. Possible role of tonsillectomy and adenoidectomy in children with recurrent fever and tonsillopharyngitis. Pediatr Infect Dis J. 1989 Feb;8(2):119-20.
Gaggiano C, Rigante D, Sota J, Grosso S, Cantarini L. Treatment options for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome in children and adults: a narrative view. Clin Rheumatol. 2019 Jan;38(1):11-17. doi: 10.1007/s10067-018-4361-2.
Krol P, Bohm M, Sula V, Dytrych P, Katra R, Nemcova D. et al. PFAPA syndrome: clinical characteristics and treatment outcomes in a large single-centre cohort. Clin Exp Rheumatol. Nov-Dec 2013;31(6):980-7.
Steward DL, Welge JA, Myer CM. Steroids for improving recovery following tonsillectomy in children. Cochrane Database Syst Rev. 2003;(1):CD003997. doi: 10.1002/14651858.CD003997.
Dusser P, Hentgen V, Neven B, Koné-Paut I. Is colchicine an effective treatment in periodic fever, aphtous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome? Joint Bone Spine. 2016 Jul;83(4):406-11. doi: 10.1016/j.jbspin.2015.08.017.
Stojanov S, Lapidus S, Chitkara P, Feder H, Salazar JC, Fleisher TA. et al. Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade. Proc Natl Acad Sci U S A. 2011 Apr 26;108(17):7148-53. doi: 10.1073/pnas.1103681108.
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 Dec;159(6):958-64. doi: 10.1016/j.jpeds.2011.06.004.
Feder HM, Salazar JC. A clinical review of 105 patients with PFAPA (a periodic fever syndrome). Acta Paediatr. 2010 Feb;99(2):178-84. doi: 10.1111/j.1651-2227.2009.01554.x.
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 Nov;74(11):1203-8. doi: 10.1016/j.ijporl.2010.08.014.
Mitchell RB, Archer SM, Ishman SL, Rosenfeld RM, Coles S, Finestone SA. et al. Clinical practice guideline: tonsillectomy in children (update). Otolaryngol Head Neck Surg. 2019 Feb;160(1_suppl):S1-S42. doi: 10.1177/0194599818801757.
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 Sep;151(3):289-92. doi: 10.1016/j.jpeds.2007.03.015.
Garavello W, Romagnoli M, Gaini RM. Effectiveness of adenotonsillectomy in PFAPA syndrome: a randomized study. J Pediatr. 2009 Aug;155(2):250-3. doi: 10.1016/j.jpeds.2009.02.038.
Erdogan F, Kulak K, Ozturk O, İpek İO, Ceran O, Seven H. Surgery vs medical treatment in the management of PFAPA syndrome: a comparative trial. Paediatr Int Child Health. 2016 Nov;36(4):270-274. doi: 10.1179/2046905515Y.0000000051.
Forsvoll J, Oymar K. The role of tonsillectomy in the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a review of the literature. BMC Ear Nose Throat Disord. 2018 Feb 22;18:3. doi: 10.1186/s12901-017-0049-5.
Burton MJ, Pollard AJ, Ramsden JD, Chong LY, Venekamp RP. Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA). Cochrane Database Syst Rev. 2014 Sep 11;(9):CD008669. doi: 10.1002/14651858.CD008669.pub2.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2022 Portuguese Journal of Otorhinolaryngology and Head and Neck Surgery
![Creative Commons License](http://i.creativecommons.org/l/by-sa/4.0/88x31.png)
This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.