Long-Term Outcome of Classic and Incomplete PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) Syndrome after Tonsillectomy

Ulla Lantto, Petri Koivunen, Terhi Tapiainen, Marjo Renko

To compare the effectiveness of tonsillectomy and the long-term outcome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in patients fulfilling the classic diagnostic criteria and in those with regularly recurring fever as the only symptom or with onset of symptoms after age 5 years or both. We reviewed the medical records of 3852 children who underwent tonsillectomy between 1990 and 2007 and identified 108 children who did so because of regularly recurring fevers. The patients were invited to an outpatient visit and were classified into 2 groups: those who met (N = 58) and those who did not meet (N = 50) Thomas diagnostic criteria. We then compared the clinical profile and outcome of PFAPA symptoms after tonsillectomy between the 2 groups. In the group that met Thomas criteria, 97% (56/58) had complete resolution of fever episodes after tonsillectomy; in the group that did not meet Thomas criteria (50/50) had complete resolution of fever episodes after tonsillectomy (P = .25). The clinical profile of the periodic fevers and the occurrence of other illnesses during follow-up were similar in both groups. Thomas criteria identified 56 of 106 patients responding to tonsillectomy. Tonsillectomy was an effective treatment for patients with regularly recurring fever episodes who failed to meet the classic Thomas criteria. We suggest that PFAPA syndrome should be suspected and tonsillectomy considered in children with a late onset of symptoms (>5 years of age) or when fever is the only symptom during the episodes.