Tonsillotomy for Periodic Fever Syndrome: A Randomized and Controlled Trial

Laryngoscope. 2024 Feb;134(2):968-972. doi: 10.1002/lary.30863. Epub 2023 Jul 21.

Abstract

Objective: Tonsillectomy is an effective treatment for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Tonsillotomy has a milder operative risk profile and postoperative morbidity in children than tonsillectomy. We aimed to compare the efficacy of tonsillotomy to observation-only in children with PFAPA syndrome at a 3-month follow-up.

Methods: This was a randomized multicenter trial with sequential design. Participants were randomized into a tonsillotomy group and a control group that was only observed. The trial started in 1/2017 and was accomplished in 12/2021 with 16 patients (10 boys, six girls, the mean age 4.2 years). The symptoms were monitored with daily symptom diaries.

Results: After the 3-month follow-up, 7/8 patients (87.5%) in the tonsillotomy group and 2/8 (25%) patients in the control group were free from PFAPA symptoms (95% CI 13% to 87%; p = 0.0021). The mean number of days with fever was 2.6 (SD 3.7) in the tonsillotomy group and 8.0 (SD 6.5) days in the control group (n = 8) (p = 0.06). Mean number of fever days compatible with PFAPA syndrome was 0.8 (SD 1.4) in the tonsillotomy group and 6.5 (SD 6.0) in the control group (95%CI -10% to -1%; p = 0.007). Rescue tonsillectomy was needed for all patients in the control group and none of the patients in the tonsillotomy group.

Conclusions: Tonsillotomy might be an effective treatment option for children with PFAPA syndrome. Further studies are needed to clarify the long-term efficacy of tonsillotomy for treating PFAPA.

Level of evidence: 2 Laryngoscope, 134:968-972, 2024.

Keywords: children; lymphoid tissue; periodic fever; tonsillotomy; tonsills.

Publication types

  • Randomized Controlled Trial
  • Multicenter Study

MeSH terms

  • Child
  • Child, Preschool
  • Female
  • Fever / diagnosis
  • Fever / surgery
  • Humans
  • Lymphadenitis* / surgery
  • Lymphadenopathy*
  • Male
  • Pharyngitis* / surgery
  • Stomatitis, Aphthous* / surgery
  • Syndrome
  • Tonsillectomy*