Related Experiment Video
Updated: Jun 22, 2026
CO2-Lasertonsillotomy Under Local Anesthesia in Adults
Published on: November 6, 2019
Outcome of tonsillectomy in selected patients with PFAPA syndrome
Lorenzo Pignataro1, Sara Torretta, Maria Cristina Pietrogrande
1Department of Otorhinolaryngological and Ophthalmological Sciences, University of Milan, Fondazione IRCCS-Ospedale Maggiore Policlinico, Mangiagalli and Regina Elena, Via F. Sforza 35, Milan, Italy. lorenzo.pignataro@unimi.it
Insights
Tonsillectomy can be an effective treatment for children with PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and cervical lymphadenopathy) when medical therapies fail. This surgical intervention significantly reduces symptom recurrence and improves overall patient condition.
Area of Science:
- Pediatric Otolaryngology
- Immunology
- Infectious Diseases
Background:
- PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and cervical lymphadenopathy) is a common autoinflammatory disorder in children.
- Current management often involves medical therapies, but surgical options like tonsillectomy are considered for refractory cases.
Purpose of the Study:
- To evaluate the feasibility of combined medical and surgical management for PFAPA syndrome.
- To determine the efficacy of tonsillectomy in improving symptoms and reducing recurrences in pediatric patients with PFAPA syndrome.
Main Methods:
- A prospective study was conducted at a secondary pediatric and otolaryngological university center.
- 18 children diagnosed with PFAPA syndrome were included, undergoing long-term pediatric and otolaryngological assessments.
- 9 patients underwent tonsillectomy after failure of traditional medical therapies; outcomes were compared pre- and post-surgery.
Main Results:
- All patients who underwent tonsillectomy experienced symptomatic improvement.
- Complete clinical recovery was observed in 56% of surgical cases, with significant reductions in recurrence frequency and duration for the remaining 44%.
- Molecular analysis of tonsillar tissue was negative for bacterial infections in most patients.
Conclusions:
- Tonsillectomy is a viable and effective treatment option for children with PFAPA syndrome unresponsive to medical management.
- Otolaryngologists should be proficient in diagnosing PFAPA syndrome, emphasizing prolonged follow-up and considering surgery after exhausting medical treatments.
Objective:
To assess the practicability of integrated medical and surgical management and the effectiveness of tonsillectomy in children with PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and cervical lymphadenopathy).
Design:
A prospective study.
Setting:
Secondary pediatric and otolaryngological university center.
Patients:
Of 30 patients evaluated for periodic fever, 18 children with PFAPA syndrome were included in the study.
Interventions:
Patients underwent long-term pediatric and otolaryngological assessments, and their parents were asked to keep monthly diaries with reports of any subsequent episodes, symptom, and related sign. Patients received traditional medical therapies, and 9 patients underwent tonsillectomy for the lack of lasting recovery.
Main Outcome Measures:
The association between postoperative outcomes and age at tonsillectomy and the differences in the patients' condition before and after tonsillectomy were statistically tested. In addition, the removed tonsillar tissue was analyzed molecularly to evaluate concomitant infections.
Results:
All of the surgical patients reported a symptomatic improvement, with complete clinical recovery in 5 cases (56%) and significant reduction in number (P = .005) and duration (P = .03) of recurrences in the remaining 4 (44%). Results of molecular analysis of tonsillar specimens were negative for bacteria in all but 1 patient.
Conclusion:
Otolaryngologists should be trained to recognize PFAPA syndrome, for which management consists of a regular and prolonged second-level pediatric and otolaryngological follow-up, with surgery only after the failure of traditional medical therapy.
Related Concept Videos
01:23Chronic Pharyngitis
Etiology
It often arises from persistent viral or bacterial infections affecting sinuses and tonsils.
Additional contributing factors include inadequate dental hygiene, mouth breathing, recurring tonsillitis, allergic rhinitis, laryngopharyngeal reflux, and exposure to smoke, chemicals, and other environmental pollutants. Allergic reactions to pollen, mold, and pet dander, chronic cough, excessive voice usage,...
01:26Tonsillitis II: Management
01:30Tonsillitis I: Introduction
Etiology
Three primary contributing factors have been identified.
01:30Acute Pharyngitis
Acute pharyngitis is the inflammation of the back of the throat (pharynx), commonly resulting in a sore throat. It is a frequently encountered condition that prompts individuals to seek medical advice.
Classification
Acute pharyngitis can be categorized based on its underlying cause: