Tonsillectomy for management of PFAPA

Abstract

Since the first report by Abramson in 1989 of unexpected complete resolution of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) after tonsillectomy in 4 children (Ped Infect Dis J 1989;8:119-20), many case series reported the effectiveness in halting PFAPA in 64% to 100% of patients. In the only previously published randomized controlled trial from Finland, of management of PFAPA, tonsillectomy was curative within 6 months in all 14 patients in whom it was performed versus spontaneous resolution in 6 of 12 control patients (J Pediatr 2007;151:289-92). In the prospective study reported in this issue of The Journal by Garavello et al from Italy, 19 children with PFAPA were randomized to adenotonsillectomy (A&T) and 20 children were managed medically, with 18 months of follow-up in both groups. In the surgical group, 60% of children had immediate resolution compared with 5% in the non-surgical group. Although 40% of children in the surgical group did not have immediate resolution of PFAPA, over the ensuing 18 months, those who had surgery compared with those who had medical management had significantly fewer episodes (12 vs 179), shorter episodes (1.7 vs 3.5 days) (P values < .001), and all were “cured” of febrile episodes 12 months after surgery. The two major stumbling blocks in testing treatments for PFAPA are the small numbers of patients available for study and inability to confirm the diagnosis of PFAPA, or to know whether PFAPA indeed is a single disease. The ages of patients in the current study and delineation of other clinical characteristics convinces the reader that they are a good “fit” for a PFAPA diagnosis. The immediate “cure” in about one-half of the patients and waning of symptoms over time in the other half of the patients who underwent A&T may be a reasonable expectation for other patients with PFAPA. Shorter follow-up may explain the lower “cure” rates reported in some studies, and “contamination” with children with recurring tonsillitis (in whom tonsillectomy would yield rapid benefit) could explain higher “cure” rates in still other studies. Although adenoidectomy was performed in addition to tonsillectomy in this study, this study does not address the need for adenoidectomy. Finally, although the observation of rapid defervescence of an episode with administration of a corticosteroid was used as a diagnostic criterion for PFAPA in the study by Garavello et al, caution is warranted as the specificity of such a response has not been tested. Corticosteroid can cause rapid defervescence in febrile infections as well as periodic fevers due to other autoinflammatory diseases (such as tumor necrosis factor receptor-associated periodic syndrome or hyperimmunoglobulinemia D syndrome). Article page 250▸