Surgical outcomes and histology findings after tonsillectomy in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome

Abstract 

Purpose

This study aimed to evaluate (a) specific histologic findings in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome who had tonsillectomy and (b) to assess any improvement of symptoms after tonsillectomy with or without adenoidectomy.

Materials and Methods

This project is a retrospective study performed at “Aghia Sophia” Children's Hospital (Athens, Greece), between May 2007 and July 2008. Nine children were recruited into this study. Patients with defined diagnostic criteria for PFAPA syndrome till 14 years of age undergoing tonsillectomy with or without adenoidectomy were included in the study.

Results

Nine children met our inclusion criteria in the PFAPA group, of which 5 were male (55.56%) and 4 were female (44.44%), with ages ranging between 2.5 and 5 years at the age of surgery (mean, 3.4 years). The length of follow-up ranged from 6 to 19 months. Eight (88.89%) of 9 patients had complete remission of symptoms immediately after surgery. The histologic and immunohistochemical examination showed features of chronic tonsillar inflammation.

Conclusions

(a) No specific findings were observed in the tonsils of PFAPA patients; (b) we could not find any difference in tonsillar histology between PFAPA and chronic tonsillar inflammation; and (c) tonsillectomy is an effective treatment for PFAPA syndrome, improving patients and parental quality of life.