Long-term Surgical Outcomes of Adenotonsillectomy for PFAPA Syndrome | Otolaryngology | JAMA Otolaryngology–Head & Neck Surgery | JAMA Network
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Original Article
Oct 2012

Long-term Surgical Outcomes of Adenotonsillectomy for PFAPA Syndrome

Author Affiliations

Author Affiliations: Department of Otolaryngology and Communication Disorders (Drs Licameli and Kenna and Ms Lawton) and Division of Immunology/Rheumatology (Dr Dedeoglu), Children's Hospital, and Department of Otology and Laryngology, Harvard Medical School (Drs Licameli and Kenna), Boston, Massachusetts.

Arch Otolaryngol Head Neck Surg. 2012;138(10):902-906. doi:10.1001/2013.jamaoto.313

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.