Effect of Adenotonsillectomy in PFAPA Syndrome | Otolaryngology | JAMA Otolaryngology–Head & Neck Surgery | JAMA Network
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Original Article
February 1, 2008

Effect of Adenotonsillectomy in PFAPA Syndrome

Author Affiliations

Author Affiliations: Department of Otolaryngology and Communication Disorders, Children's Hospital (Drs Licameli, Jones, and Kenna and Mss Jeffrey and Luz), and Department of Otology and Laryngology, Harvard Medical School (Drs Licameli, Jones, and Kenna), Boston, Massachusetts.

Arch Otolaryngol Head Neck Surg. 2008;134(2):136-140. doi:10.1001/archoto.2007.7

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.