Tonsillectomy for Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome (Pfapa)

Summary

A Cochrane review [Abstract] 1 included 2 studies with a total of 67 children. One study (n=39) included children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome diagnosed according to rigid, standard criteria, and compared adenotonsillectomy to watchful waiting with a follow-up of 18 months. The other study (n=28) applied less stringent criteria for diagnosing PFAPA and probably also included participants with alternative types of recurrent pharyngitis. This study compared tonsillectomy alone to no treatment with a follow-up of 6 months.

Children with PFAPA experienced less fever and less severe episodes after surgery compared to those receiving no surgery (table T1). The number of patients who had "immediate and persistent resolution of PFAPA syndrome" after the surgery/randomisation, which was sustained until the end of follow-up (at 18 months) was 12/19 in the surgery group and 1/20 in the control group in one study. In the other study, corresponding numbers for the six-month follow-up period (their longest available follow-up) were 10/14 in the surgery group and 4/12 in the control group. Both studies reported that there were no complications of surgery but the numbers of patients randomly allocated to surgery (19 and 14 patients respectively) were too small to detect potentially important complications such as haemorrhage. Quality of life, number of days with pain after surgery and absence from school were not measured or reported.