Surgical management for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome: a systematic review and meta-analysis

Abstract

Objective  This systematic review and meta-analysis aimed to evaluate the efficacy of surgical management, specifically total and partial tonsillectomy, for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) syndrome, focusing on symptom resolution and recurrence reduction.

Data sources  A systematic search of MEDLINE, Scopus, and Cochrane databases identified randomized controlled trials (RCTs) comparing surgical to non-surgical management in children with PFAPA. Data extraction and quality assessment adhered to Cochrane guidelines and PRISMA protocols. Risk ratios (RR) and confidence intervals (CI) were calculated using random-effects models.

Summary of findings  After the removal of duplicates, 31 studies were screened and 3 studies were included. Pooled analysis revealed a 72% reduction in persistent symptoms following surgical interventions compared to non-surgical management (RR: 0.28, 95% CI: 0.12-0.68, I² = 22%, p = 0.005).

Conclusion  The findings confirm that surgical management, including total and partial tonsillectomy, is an effective therapeutic option for PFAPA, with substantial benefits in symptom resolution and quality of life improvement. While surgery offers long-term benefits, its risks, and broader immunological implications require careful consideration. The study underscores the necessity for larger, multicenter trials to validate these findings across diverse populations and optimize treatment strategies.

Keywords
Syndrome of periodic fever; Aphthous stomatitis, pharyngitis, and cervical adenitis; Tonsillectomy; Tonsillotomy; Randomized controlled trial; Meta-analysis