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Published on: November 6, 2019
Effect of adenotonsillectomy in PFAPA syndrome
Greg Licameli1, Jessica Jeffrey, Jennifer Luz
1Department of Otolaryngology and Communication Disorders, Children's Hospital, 300 Longwood Ave, LO 367, Boston, MA 02115. greg.licameli@childrens.harvard.edu
Insights
Adenotonsillectomy effectively treats pediatric PFAPA syndrome (periodic fever, aphthous ulcers, pharyngitis, and adenitis), with 26 of 27 patients experiencing symptom resolution. Surgical intervention is recommended for PFAPA patients unresponsive to medical management.
Area of Science:
- Pediatric Otolaryngology
- Pediatric Immunology
- Pediatric Infectious Diseases
Background:
- PFAPA syndrome (periodic fever, aphthous ulcers, pharyngitis, and adenitis) is a common autoinflammatory disorder in children.
- Recurrent symptoms significantly impact quality of life and necessitate effective treatment strategies.
Purpose of the Study:
- To evaluate the efficacy of adenotonsillectomy in resolving PFAPA syndrome symptoms in pediatric patients.
- To determine the long-term benefits of surgical intervention for PFAPA.
Main Methods:
- A prospective case series was conducted at a tertiary care pediatric hospital.
- Twenty-seven pediatric patients diagnosed with PFAPA syndrome underwent tonsillectomy with or without adenoidectomy.
- Symptom resolution was the primary outcome measure, with follow-up ranging from 8 to 41 months.
Main Results:
- Complete resolution of PFAPA symptoms was observed in 26 out of 27 treated patients (96.3%).
- The single patient with persistent symptoms was deemed unlikely to have had PFAPA syndrome upon retrospective review.
- Surgical treatment demonstrated a high success rate in managing this condition.
Conclusions:
- Adenotonsillectomy is a highly effective treatment for PFAPA syndrome, leading to complete symptom resolution in the vast majority of cases.
- Surgical intervention, including tonsillectomy and adenoidectomy, should be strongly considered for pediatric PFAPA patients who do not respond to medical therapies.
Objective:
To assess the benefits of adenotonsillectomy in the treatment of pediatric patients with PFAPA (periodic fever, aphthous ulcers, pharyngitis, and adenitis) syndrome.
Design:
Prospective case series.
Setting:
Tertiary care pediatric hospital.
Patients:
Pediatric patients meeting criteria for PFAPA syndrome.
Intervention:
Tonsillectomy with or without adenoidectomy.
Main Outcome Measure:
Resolution of PFAPA symptoms.
Results:
Twenty-seven (14 female, 13 male) children with PFAPA syndrome underwent tonsillectomy with or without adenoidectomy from 2004 through 2006. The length of follow-up for all patients ranged from 8 to 41 months. A total of 26 patients experienced a complete resolution of their symptoms. The 1 child who continued to have febrile episodes had fever cycles that were not regular in duration or interval and in hindsight was not likely a patient with PFAPA syndrome.
Conclusions:
Our findings showed complete resolution of symptoms in 26 of 27 patients with PFAPA syndrome treated surgically. Patients who meet clinical criteria for PFAPA syndrome should be considered for tonsillectomy and adenoidectomy if they do not respond to medical management.
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