Efficacy of partial tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome: a STROBE retrospective observational study.

Introduction: PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis) or Marshall syndrome is the most frequent cause of recurrent auto-inflammatory fever in children. Frequent episodes impair the child's quality of life and family life. Total tonsillectomy demonstrated efficacy in improving symptoms, but few studies assessed partial tonsillectomy in this indication. The aim of the present study was to assess postoperative course after partial tonsillectomy for PFAPA syndrome, with comparison to total tonsillectomy.

Materials and methods: This retrospective cohort study adhered to STROBE guidelines. It included children with PFAPA syndrome on EUROFEVER criteria, treated by partial or total tonsillectomy between January 1, 2011 and December 31, 2022 in our university hospital center. For comparisons, the significance threshold was set at P<0.005.

Results: Thirty-six children were included: 16 with partial and 20 with total tonsillectomy. With partial tonsillectomy, the number of episodes decreased by 10 per year (range, 5-21) (P<0.005) over 6 years' follow-up. The decrease was 50% with partial tonsillectomy and 93% with total tonsillectomy (P=0.056). The decrease in number was statistically suggestive (P=0.028). There were no complications with partial tonsillectomy and 2 patients with complications (10%) with total tonsillectomy. Two of the 16 patients with partial tonsillectomy (12.5%) required totalization, achieving remission in both cases.

Conclusion: Partial tonsillectomy significantly reduced the frequency, duration and intensity of postoperative episodes in PFAPA syndrome. It may be less effective than total tonsillectomy, but has a lower risk of complications awaiting remission in adolescence.