Table 1.
Case series reporting the outcome after tonsillectomy and adenotonsillectomy in children with the PFAPA syndrome
| Author/year (reference) | Number of children | Curative | Partly effective | Not effective | Observation post surgery (months, mean) | Adeno-tonsillectomya | Diagnosis set prior to surgery | Criteria for PFAPA diagnosisb | Quality | |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | Abramson et al. 1989 [14] | 4 | 4 | – | – | 15 | 3 (75%) | No | Marshall | Low |
| 2 | Padeh et al. 1999 [3] | 3 | 3 | – | – | Not clear | 0 | Yes | Marshall | Low |
| 3 | Dahn et al. 2000 [30] | 5 | 5 | – | – | 3 | 5 (100%) | No | Adapted | Low |
| 4 | Galanakis et al. 2002 [31] | 15 | 15 | – | – | Not clear | 0 | No | Thomas | Low |
| 5 | Berlucchi et al. 2003 [32] | 5 | 5 | – | – | 10 | 2 (40%) | Yes | Adapted | Low |
| 6 | Parikh et al. 2003 [33] | 2 | 0 | – | 2 | Not clear | 0 | No | Marshall | Low |
| 7 | Tasher et al. 2006 [5] | 6 | 6 | – | – | 19 | 0 | Yes | Thomas | Low |
| 8 | Wong et al. 2008 [34] | 9 | 8 | 1 | – | 24 | 0 | Yes | Marshall | Medium |
| 9 | Pignataro et al. 2009 [35] | 9 | 5 | 4 | – | 26 | 0 | Yes | Marshall | Medium |
| 10 | Fedrer et al. 2010 [36] | 11 | 11 | – | – | 18 | 0 | Yes | Thomas | Low |
| 11 | Peridis et al. 2010 [37] | 9 | 8 | – | 1 | 12 | 2 (22%) | Yes | Thomas | Low |
| 12 | Wurster et al. 2011 [7] | 12 | 6 | 3 | 3 | Not clear | 10 (83%) | Yes | Thomas | Low |
| 13 | Licameli et al. 2012 [38] c | 102 | 99 | – | 3 | 43 | 102 (100%) | Yes | Marshall | High |
| 14 | Førsvoll et al. 2013 [29] | 17 | 17 | – | – | Not clear | 7 (41%) | Yes | Thomas | Low |
| 15 | Krol et al. 2013 [39] | 18 | 18 | – | – | Not clear | 0 | Yes | Thomas | Low |
| 16 | Ter Haar et al. 2013 [40] | 8 | 4 | 3 | 1 | Not clear | Not clear | Yes | Marshall | Low |
| 17 | Valenzuela et al. 2013 [41] | 9 | 9 | – | – | 10 | 0 | Yes | Thomas | Low |
| 18 | Kubota et al. 2014 [42] | 5 | 4 | – | 1 | Not clear | 0 | Yes | Thomas | Low |
| 19 | Vigo et al. 2014 [43] | 41 | 27 | – | 14 | 69 | 0 | Yes | Marshall | High |
| 20 | Dytrych et al. 2015 [44] | 10 | 10 | – | – | 19 | 0 | Yes | Thomas | Low |
| 21 | Førsvoll et al. 2015 [45] | 4 | 4 | – | – | 27 | 1 (25%) | Yes | Thomas | Medium |
| 22 | Lantto et al. 2015 [46]d | 31 | 31 | – | – | 6 | 11 (35%) | No | Adapted | Low |
| 23 | Perko et al. 2015 [47] | 28 | 26 | 2 | – | Not clear | 0 | Yes | Thomas | Medium |
| 24 | Batu et al. 2016 [48]c | 53 | 50 | – | 3 | Not clear | 0 | Yes | Thomas | Medium |
| 25 | Dusser et al. 2016 [49] | 4 | 3 | – | 1 | Not clear | Not clear | Yes | Thomas | Low |
| 26 | Erdogan et al. 2016 [50] | 75 | 74 | 1 | – | 24 | Not clear | Yes | Thomas | High |
| 27 | Lantto et al. 2016 [22]d | 58 | 56 | – | 2 | 107 | 0 | No | Thomas | Medium |
| 28 | Rigante et al. 2016 [51] | 2 | 1 | – | 1 | Not clear | 0 | Yes | Thomas | Low |
| Total | 555 | 509 | 14 | 32 | – | 143 (26%) | – |
PFAPA Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis
a:Adenotonsillectomy performed instead of tonsillectomy alone
b:PFAPA diagnosis was set according to criteria by Marshall [13], Thomas [4] or adapted criteria not clearly based on either of the two
cand d:Possible overlap between study populations