Tonsillectomy in Children with Periodic Fever with Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome

Long-term Surgical Outcomes of Adenotonsillectomy for PFAPA Syndrome

“…Garavello et al 14 reviewed the available literature through 2010 to identify English-language studies evaluating the efficacy of tonsillectomy or adenotonsillectomy on PFAPA syndrome. In their analysis of 13 case studies and 2 randomized controlled trials, complete resolution of symptoms occurred in 83% of 149 children who underwent surgical intervention.…”

“…Furthermore, the financial costs to the family caring for a child with a chronic illness has not been quantified. 14 Surgical intervention can be considered based on the presumption of a chronic tonsillar infection triggering an autoinflammatory-type response. Whether adenoid removal in addition to tonsillectomy is necessary for positive outcomes is unclear.…”

Successful Treatment of PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) Syndrome with Shinbuto*

“…Nonetheless, some treatments have been tried. Garavello et al reported that the most effective treatment is corticosteroid therapy, which is associated with cessation of the fever cycle within 12 -24 h [5]. Stojanov et al suggested that the syndrome results from a disturbance of the immune response with continuous pro-inflammatory response that occurs both during and between febrile attacks [6].…”

“…The reason why corticosteroid therapy succeeds may be a reduction in cytokines. Furthermore, Garavello et al suggested that tonsillectomy should be considered when symptoms markedly interfered with the child's quality of life and medical treatment failed [5]. On the other hand, Peridis et al reported that tonsillectomy is an effective treatment for PFAPA syndrome [7].…”

Procalcitonin Levels in PFAPA Syndrome

“…The non-responsive group (six cases) underwent tonsillectomy. Tonsillectomy should be reserved only for selected indications (24,25) . …”