Tonsillectomy for PFAPA Syndrome

From Medscape Pediatrics > Viewpoints Tonsillectomy in Children With Periodic Fever With Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome Garavello W, Pignataro L, Gaini L, Torretta S, Somigliana E, Gaini R J Pediatr. 2011;159:138-142 Study Summary William T. Basco, Jr., MD Posted: 09/19/2011 The clinical entity that includes periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA syndrome) typically occurs in children 5 years of age and under. These children experience 3-4 days of illness that recur approximately every 4 weeks. Although the cause of this syndrome has been difficult to identify, the effects on these children and their families can be notable. Previous studies have shown that antibiotics do not alter symptoms from this condition, but corticosteroid therapy has been shown to quickly reduce fever. This study was a review of the literature on the utility of tonsillectomy in treating PFAPA syndrome. The investigators identified 15 studies published between 1999 and 2010 for this review, identifying articles that evaluated surgical tonsillectomy for PFAPA syndrome. The primary outcome of interest was whether the children experienced resolution of symptoms after tonsillectomy. Only 2 of the studies were randomized controlled trials. Of the 13 other case series, only 2 collected data prospectively. In 8 of the studies, the children underwent tonsillectomy alone, whereas the other 7 studies reported combinations of tonsillectomy with or without adenoidectomy. The 2 randomized trials were considered to be of better methodologic quality than the observational studies. The number of children in the studies varied from 2 to 27. The 2 randomized controlled trials had 14 and 19 children, respectively. After pooling outcomes from all the studies, 83% of the children experienced complete resolution of their symptoms after surgery. Looking at the 2 randomized trials individually, 100% of the children in one of the trials improved and 63% of the children in the other improved. The researchers were able to combine the data of the 2 randomized controlled trials for analysis, and the combined analysis demonstrated improved resolution after surgery with an odds ratio of 13 (95% confidence interval, 4-43). The investigators concluded that the evidence is insufficient that tonsillectomy is effective for treatment of PFAPA syndrome. They mentioned that limitations in the published literature, as well as varying definitions for enrollment among the trials, make true meta-analyses difficult. Viewpoint I have to concur with Garavello and colleagues that the state of the literature is still at the hypothesis-generating point. These studies contained a grand total of 149 children, and the randomized, controlled trials contained a total of 29 patients. As the investigators state in their discussion, the combined results of the 2 randomized, controlled trials suggests a marked improvement after surgery, providing justification for a well designed prospective study. However, it appears that care should be taken during future trials in both patient selection and surgical approach so that the results are more generalizable. Abstract