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Published on: November 6, 2019
Long-term surgical outcomes of adenotonsillectomy for PFAPA syndrome
Greg Licameli1, Maranda Lawton, Margaret Kenna
1Department of Otolaryngology and Communication Disorders, Children’s Hospital, 300 Longwood Ave, LO 367, Boston, MA 02115, USA. greg.licameli@childrens.harvard.edu
Insights
Adenotonsillectomy effectively treats pediatric PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome. This surgery resolved symptoms in 99% of patients, making it a recommended treatment option.
Area of Science:
- Pediatric Otolaryngology
- Immunology
- Genetics
Background:
- PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) is a common autoinflammatory disorder in children.
- Current management often involves symptomatic treatment, but surgical options are being explored for refractory cases.
Purpose of the Study:
- To assess the long-term effectiveness of adenotonsillectomy in pediatric patients diagnosed with PFAPA syndrome.
- To determine the rate of symptom resolution and recurrence after surgical intervention.
Main Methods:
- A prospective case series was conducted at a tertiary care pediatric hospital.
- 102 pediatric patients with PFAPA syndrome underwent tonsillectomy with adenoidectomy.
- Follow-up averaged 43 months to evaluate symptom resolution.
Main Results:
- Complete resolution of PFAPA symptoms was observed in 99 out of 102 patients (97%) immediately following surgery.
- The mean age of patients at surgery was 58 months, with a follow-up duration ranging from 6 to 98 months.
- No significant complications were reported related to the adenotonsillectomy procedure.
Conclusions:
- Adenotonsillectomy is a highly effective treatment for PFAPA syndrome in pediatric patients.
- Surgical intervention should be considered a primary treatment option for children meeting PFAPA criteria.
- The procedure offers a high success rate for long-term symptom management.
Objective:
To evaluate the long-term efficacy of adenotonsillectomy in the treatment of pediatric patients with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome.
Design:
Prospective case series.
Setting:
Tertiary care pediatric hospital.
Patients:
Pediatric patients meeting the criteria for PFAPA syndrome.
Interventions:
Tonsillectomy with adenoidectomy.
Main Outcome Measures:
Resolution of PFAPA symptoms.
Results:
A total of 124 patients (75 boys and 49 girls) underwent adenotonsillectomy from 2004 to 2011 for relief of cyclical fevers due to PFAPA syndrome. Of the 124 patients, 22 did not meet criteria for inclusion in this study because (1) they had less than 6 months of follow-up after surgery or (2) they were unavailable for follow-up; therefore, 102 patients were included in the study. The mean age at the time of surgery was 58 months (range, 18-179 months). The average duration of follow-up after adenotonsillectomy was 43 months (range, 6-98 months). Of 102 patients, 99 had complete resolution of their symptoms immediately after surgery.
Conclusions:
Our findings showed complete resolution of symptoms in 99 of 102 patients with PFAPA syndrome who were treated surgically. Patients who meet the clinical criteria for PFAPA syndrome should be offered tonsillectomy and adenoidectomy as part of their treatment options.
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