Effect of Adenotonsillectomy in PFAPA Syndrome | Otolaryngology | JAMA Otolaryngology–Head & Neck Surgery | JAMA Network
[Skip to Navigation]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address 34.204.201.220. Please contact the publisher to request reinstatement.
1.
Marshall  GSEdwards  KMButler  JLawton  AR Syndrome of periodic fever, pharyngitis, and aphthous stomatitis.  J Pediatr 1987;110 (1) 43- 46PubMedGoogle ScholarCrossref
2.
Scholl  PR Periodic fever syndromes.  Curr Opin Pediatr 2000;12 (6) 563- 566PubMedGoogle ScholarCrossref
3.
Padeh  SBrezniak  NZemer  D  et al.  Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: clinical characteristics and outcome.  J Pediatr 1999;135 (1) 98- 101PubMedGoogle ScholarCrossref
4.
van der Meer  JWVossen  JMRadl  J  et al.  Hyperimmunoglobulinaemia D and periodic fever: a new syndrome.  Lancet 1984;1 (8386) 1087- 1090PubMedGoogle ScholarCrossref
5.
Padeh  S Periodic fever syndromes.  Pediatr Clin North Am 2005;52 (2) 577- 609, viiPubMedGoogle ScholarCrossref
6.
Schwan  TGPolicastro  PMiller  Z  et al.  Tick borne relapsing fever caused by Borrelia hermsii, Montana.  Emerg Infect Dis 2003;9 (9) 1151- 1154PubMedGoogle ScholarCrossref
7.
Ryan  MFerrigno  KO’Boyle  TLong  S Periodic fever and skin lesions caused by disseminated Mycobacterium chelonae infection in an immunocompetent child.  Pediatr Infect Dis 1996;15 (3) 270- 272PubMedGoogle ScholarCrossref
8.
Long  S Syndrome of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA): what it isn’t: what is it?  J Pediatr 1999;135 (1) 1- 5PubMedGoogle ScholarCrossref
9.
Stojanov  SHoffman  FKery  A  et al.  Cytokine profile in PFAPA suggests continuous inflammation and reduced anti-inflammatory response.  Eur Cytokine Netw 2006;17 (2) 90- 97PubMedGoogle Scholar
10.
Feder  HM  Jr Periodic fever, aphthous stomatitis, pharyngitis, adenitis: a clinical review of a new syndrome.  Curr Opin Pediatr 2000;12 (3) 253- 256PubMedGoogle ScholarCrossref
11.
Feder  HM  Jr Cimetidine treatment for periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis.  Pediatr Infect Dis J 1992;11 (4) 318- 321PubMedGoogle ScholarCrossref
12.
Thomas  KTFeder  HM  JrLawton  AREdwards  KM Periodic fever syndrome in children.  J Pediatr 1999;135 (1) 15- 21PubMedGoogle ScholarCrossref
13.
Abramson  JSGivner  LBThompson  JN Possible role of tonsillectomy and adenoidectomy in children with recurrent fever and tonsillopharyngitis.  Pediatr Infect Dis J 1989;8 (2) 119- 120PubMedGoogle Scholar
14.
Galanakis  EPapadakis  CEGiannoussi  EKaratzanis  ADBitsori  MHelidonis  ES PFAPA syndrome in children evaluated for tonsillectomy.  Arch Dis Child 2002;86 (6) 434- 435PubMedGoogle ScholarCrossref
15.
Dahn  KAGlode  MPChan  KH Periodic fever and pharyngitis in young children: a new disease for the otolaryngologist?  Arch Otolaryngol Head Neck Surg 2000;126 (9) 1146- 1149PubMedGoogle ScholarCrossref
16.
Berlucchi  MMeini  APlebani  ABonvini  MGLombardi  DNicolai  P Update on treatment of Marshall's syndrome (PFAPA syndrome): report of five cases with review of the literature.  Ann Otol Rhinol Laryngol 2003;112 (4) 365- 369PubMedGoogle Scholar
17.
Parikh  SRReiter  ERKenna  MARoberson  D Utility of tonsillectomy in 2 patients with the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis.  Arch Otolaryngol Head Neck Surg 2003;129 (6) 670- 673PubMedGoogle ScholarCrossref
18.
Leong  SCKarkos  PDApostolidou  MT Is there a role for the otolaryngologist in PFAPA syndrome? a systematic review.  Int J Pediatr Otorhinolaryngol 2006;70 (11) 1841- 1845PubMedGoogle ScholarCrossref
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
Abstract

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.

×