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A randomized, controlled trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis
M Renko1, E Salo, A Putto-Laurila
1Department of Pediatrics, University of Oulu, Finland. marjo.renko@oulu.fi
Insights
Tonsillectomy effectively treats periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in children. While some PFAPA cases resolve spontaneously, surgery offers a significant benefit for persistent symptoms.
Area of Science:
- Pediatric Otolaryngology
- Pediatric Immunology
- Clinical Trial Research
Background:
- Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome is a common autoinflammatory disorder in children.
- The clinical course and optimal management of PFAPA syndrome remain subjects of investigation.
Purpose of the Study:
- To evaluate the efficacy of tonsillectomy in managing PFAPA syndrome through a prospective, randomized, controlled trial.
- To compare tonsillectomy versus conservative management for PFAPA syndrome in pediatric patients.
Main Methods:
- A randomized controlled trial involving 26 children diagnosed with PFAPA syndrome.
- Participants were allocated to either tonsillectomy or watchful waiting, with the option for tonsillectomy after 6 months if symptoms persisted.
- Symptom tracking was conducted using diaries over a 12-month follow-up period.
Main Results:
- After 6 months, 100% of children who underwent tonsillectomy were symptom-free, compared to 50% in the control group (P < .001).
- Tonsillectomy was subsequently performed on 5 out of 6 children in the control group who did not achieve remission.
- One child in the control group experienced less severe, recurrent symptoms but did not opt for surgery.
Conclusions:
- Tonsillectomy is an effective intervention for children diagnosed with PFAPA syndrome.
- While spontaneous remission occurs in some cases, tonsillectomy provides a significant therapeutic option for persistent PFAPA symptoms.
- The underlying mechanisms of PFAPA syndrome require further elucidation, but surgical intervention is a viable treatment strategy.
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.
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