A Randomized, Controlled Trial of Tonsillectomy in Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome

Renko, M.; Salo, E.; Putto-Laurila, A.; Saxen, H.; Mattila, P.S.; Luotonen, J.; Ruuskanen, O.; Uhari, M.

doi:10.1016/j.jpeds.2007.03.015

« Previous Next »The Journal of Pediatrics
Volume 151, Issue 3 , Pages 289-292, September 2007

A Randomized, Controlled Trial of Tonsillectomy in Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome

M. Renko, MD, PhD
- Department of Pediatrics, University of Oulu, Finland
- Reprint requests: Marjo Renko, MD, PhD, Department of Pediatrics, University of Oulu, Box 5000, FIN-90014 University of Oulu, Finland.
E. Salo, MD, PhD
- Department of Pediatrics, University of Helsinki, Finland
A. Putto-Laurila, MD, PhD
- Department of Pediatrics, University of Turku, Finland.
H. Saxen, MD, PhD
- Department of Pediatrics, University of Helsinki, Finland
P.S. Mattila, MD, PhD
- Department of Otorhinolaryngology, University of Helsinki, Finland
J. Luotonen, MD, PhD
- Department of Otorhinolaryngology, University of Oulu, Finland
O. Ruuskanen, MD, PhD
- Department of Pediatrics, University of Turku, Finland.
M. Uhari, MD, PhD
- Department of Pediatrics, University of Oulu, Finland

Received 17 October 2006; received in revised form 29 December 2006; accepted 6 March 2007. published online 16 June 2007.

Objective

We carried out a prospective, randomized, controlled trial to clarify the effect of tonsillectomy on the clinical course of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome.

Study design

Twenty-six consecutive children (mean age 4.1 years) with at least 5 PFAPA attacks were recruited from 3 tertiary care pediatric hospitals during 1999-2003 and randomly allocated to tonsillectomy or follow-up alone. They were all followed up with symptom diaries for 12 months. Tonsillectomy was allowed after 6 months in the control group if the attacks recurred.

Results

Six months after randomization all 14 children in the tonsillectomy group and 6/12 children in the control group (50%) were free of symptoms (difference 50%, 95% confidence interval 23% to 75%, P < .001). Tonsillectomy was performed on 5/6 of the patients in the control group who still had symptoms after 6 months. The remaining unoperated child in the control group had recurrences of the fever episodes throughout the follow-up, but the symptoms became less severe, and the parents did not choose tonsillectomy.

Conclusion

Tonsillectomy appeared to be effective for treating PFAPA syndrome. The fever episodes ceased without any intervention in half of the control subjects. We conclude that although the mechanisms behind this syndrome are unknown, tonsillectomy can be offered as an effective intervention for children with PFAPA.

Abbreviations: HIDS, Hyperimmunoglobulinemia D syndrome, MEFV, Mediterranean fever, PFAPA, Periodic fever, aphthous stomatitis, pharyngitis and adenitis, TRAPS, Tumor necrosis factor receptor–associated periodic syndrome