Original Article
Effectiveness of Adenotonsillectomy in PFAPA Syndrome: A Randomized Study

https://doi.org/10.1016/j.jpeds.2009.02.038Get rights and content

Objective

To evaluate whether adenotonsillectomy leads to complete resolution in children with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome.

Study design

Thirty-nine children with PFAPA syndrome were randomized to either adenotonsillectomy (surgery group; n = 19) or expectant management (control group; n = 20). All patients were then invited prospectively to record all PFAPA episodes, and were evaluated clinically every 3 months for 18 months after randomization.

Results

The proportion of patients experiencing complete resolution was 63% in the surgery group and 5% in the control group (P < .001). The mean (± standard deviation) number of episodes recorded during the study period was 0.7 ± 1.2 in the surgery group and 8.1 ± 3.9 in the control group (P < .001). The episodes were less severe in the surgery group.

Conclusions

Adenotonsillectomy is an effective treatment strategy for children with PFAPA syndrome.

Section snippets

Methods

Between February 2003 and December 2006, all children affected by PFAPA referred to the Department of Otorhinolaryngology of the University of Milano-Bicocca were offered participation in a randomized study. Children were eligible if they met the following diagnostic criteria for PFAPA syndrome2, 4: (1) onset of disease in early childhood (before age 5 years); (2) regularly recurring, abrupt episodes of fever lasting approximately 5 days associated with constitutional symptoms and at least 1 of

Results

A total of 41 patients were eligible; 2 parents declined to participate. Thirty-nine patients were randomized. Nineteen patients underwent surgery, and 20 patients were allocated to expectant management. All randomized patients completed the study. Baseline clinical characteristics of the recruited children according to treatment allocation are given in Table I.

No major surgical complication was reported in the surgery group. Pathological diagnoses were reactive lymphoid hyperplasia in 14

Discussion

Adenoidectomy and tonsillectomy is a highly effective treatment strategy for children with PFAPA syndrome. In our study, the majority of patients experienced complete resolution immediately after surgery. Noteworthy benefit also was documented in those without resolution. Indeed, none of them had more than 3 episodes during the study period, complete resolution was achieved in all cases within 1 year, and episodes that occurred after surgery were less severe.

Our trial was not blinded and we did

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    Citation Excerpt :

    A number of case series have reported a high rate of remission of PFAPA symptoms after tonsillectomy, and a recently conducted review of these series found that surgery was curative in 509 out of 555 (92%) patients.69 However, to date, there have only been two randomized controlled trials investigating the efficacy of tonsillectomy in treating PFAPA, by Renko et al70 in 2007 and Garavello et al71 in 2009. A recent Cochrane review of these two studies found that children with PFAPA are likely to benefit from tonsillectomy and experience a significant reduction (or resolution) of symptoms, but that the level of evidence to support surgical intervention was only moderate.72

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The authors declare no conflicts of interest.

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