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AAP Grand Rounds 18:66-67 (2007)
© 2007 American Academy of Pediatrics

Tonsillectomy as a Therapeutic Option for PFAPA

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, first described by Marshall¹ in 1987, is the commonest type of periodic fever among children. Episodes of PFAPA are characterized by periodic high fever lasting three to six days and recurring every three to eight weeks, often accompanied by aphthous stomatitis, pharyngitis, and adenitis. Treatment with antipyretics and antibiotics has been ineffective.² A single high dose of corticosteroid (prednisone 1–2 mg/kg) frequently leads to rapid resolution of the fever³ but does not prevent subsequent episodes. The only therapies reported to reduce recurrence of episodes are continuous cimetidine therapy⁴ and tonsillectomy,⁵ but no controlled studies of either have been published.

Investigators from the Universities of Oulu, Helsinki, and Turku in Finland carried out a prospective, randomized, controlled trial to clarify the effect of tonsillectomy on the clinical course of PFAPA syndrome. Consecutive children with at least five PFAPA attacks were recruited from three tertiary care pediatric hospitals between 1999 and 2003. Criteria for an attack included high fever (>38.5°C) of unknown origin recurring in a typical regular pattern and asymptomatic intervals of two to five weeks. Participants were randomly assigned to either the treatment group (tonsillectomy performed within one month of enrollment) or the control group. They were all followed up with symptom diaries to be completed by parents. Accompanying signs of aphthous stomatitis, pharyngitis, and adenitis were recorded.

Twenty-eight children (mean age of four years) were initially enrolled and found to have an average of nine febrile episodes (range 4–20) before recruitment, with fever lasting a mean of 3.6 days (range 2–6) and a mean interval between episodes of 25.6 days (range 18–28). In 41% of the children, fever was the only finding during each recurrent episode. Twenty-nine percent had exudative tonsillitis during at least one of the episodes, and 21% had either aphthous stomatitis, cervical adenitis, or pain in the mouth or throat during at least one episode. Two children were excluded from analysis: one developed leukemia during follow-up and the second was lost to follow-up.

All 14 children in the tonsillectomy group and six of 12 (50%) in the control group were free of symptoms (as defined by disappearance of fever episodes) six months after randomization (P<.001). Tonsillectomy was performed on five of the 12 children in the control group after the follow-up period of six months because symptoms persisted, after which the symptoms disappeared in all five cases for the duration of the one-year follow-up period. The six children in the control group who were better without tonsillectomy did not differ from the six in whom the attacks recurred with regard to age, sex, or features of the PFAPA attacks. The authors conclude that tonsillectomy is an effective therapeutic option for children with PFAPA.

Commentary by Donald Schiff, MD, FAAP1 and Leslie L. Barton, MD, FAAP2

1 University of Colorado School of Medicine and The Children’s Hospital, Denver, CO 2 Pediatrics, University of Arizona School of Medicine, Tucson, AZ

 
Drs. Schiff and Barton have disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

As previously discussed in these pages (see AAP Grand Rounds, October 1999;2:39–40[Free Full Text] ⁶), the diagnosis of PFAPA syndrome is based upon a clinical picture of recurrent, stereotyped, febrile episodes and a constellation of associated findings with no currently discernable etiology. The etiology of PFAPA is unknown, and affected children appear to be healthy between episodes. The prognosis is excellent, even when episodes recur for many years, and no complications of this disease have been reported. The spectrum of symptoms which may accompany the characteristic periodic fever requires careful observation and diagnostic studies to prevent other entities from being missed. This is well-illustrated by the child in the control group in whom acute leukemia was diagnosed during his six-month follow-up visit. The authors enumerate other causes of familial periodic fever syndromes, including familial Mediterranean Fever (FMF), hyper-IgD syndrome, tumor necrosis factor receptor-associated periodic syndrome (TRAPS), and cyclic neutropenia. However, the only investigations performed in the patients enrolled in this study were measurements of serum immunoglobulins, sedimentation rate or CRP, and blood leukocyte counts. Although repeated courses of corticosteroids are not without side effects, the potential benefit of tonsillectomy for the treatment of PFAPA must be balanced against the recognized risks of tonsillectomy. We await a larger study with more rigorous etiologic investigations and longer follow-up before we recommend surgery for a disease in which resolution without sequelae is common.

References

1. Marshall GS, et al. J Pediatr. 1987;110:43–46.[CrossRef][Medline]
2. Galanakis E, et al. Arch Dis Child. 2002;86:434–435.[Abstract/Free Full Text]
3. Thomas KT, et al. J Pediatr. 1999;135:15–21.[CrossRef][Medline]
4. Feder HM. Pediatr Infect Dis J. 1992;11:318–321.[Medline]
5. Galanakis E. Arch Dis Child. 2002; 86:434–435.[Abstract/Free Full Text]
6. Thomas KT, et al. J Pediatr. 1999;135:15–21.[CrossRef][Medline]

This Article Extract Full Text (PDF) Take the CME quiz: Vol. 18 No. 6, December 2007 Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services E-mail this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to My File Cabinet Download to citation manager Citing Articles Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Schiff, D. Articles by Barton, L. L. Search for Related Content PubMed Articles by Schiff, D. Articles by Barton, L. L.