Introduction: The medical syndrome clinically characterized by the association of periodic fever, aphthous stomatitis, pharyngitis, and cervical lymphadenopathy. Renamed it PFAPA syndrome to underline the distinctiveness of the clockwork periodicity of events and thus include it in the differential diagnosis of periodic fever syndromes in children.
Objective: To assess the effectiveness of tonsillectomy in the management of children with PFAPA syndrome.
Patients and Methods: A prospective study carried out in collaboration between Pediatrics and Otolaryngology Departments, Qena Faculty of Medicine, South Valley University. Egypt in the period between January (2010) to December (2013) after approval from Qena Faculty of Medicine ethics committee. Thirty-six patients with age range from 20-52 months evaluated for periodic fever were included in the study PFAPA syndrome, who attended Qena University Hospital, South Valley University. Patients underwent tonsillectomy long-term pediatrics and otorhinolaryngological assessments. Their parents were asked to keep monthly diaries with reports of any subsequent episodes, symptoms, and related signs. Patients received traditional medical therapies, and 36 patients underwent tonsillectomy due to the lack of lasting recovery. The associations between postoperative outcomes and age at tonsillectomy and the differences in the patients' condition before and after tonsillectomy were statistically tested. In addition, the removed tonsillar tissue was analyzed molecularly to evaluate concomitant infections.
Results: All of the surgical patients reported a symptomatic improvement, with complete clinical recovery in 35 cases (97.22%), follow up were done to all patients at 3,6 and 12 months after tonsillectomy (36 patients,32cases and 32 cases respectively) show complete clinical recovery.
Conclusions: Tonsillectomy operation is effective and appropriate in the management of PFAPA in children who do not improve spontaneously or do not respond to traditional medical therapy. Recommendations: Otolaryngologists should be trained to recognize PFAPA syndrome, for which management consists of a regular and prolonged second level pediatric and otolaryngological follow-up, with surgery only after the failure of traditional medical therapy.