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2024

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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SURGICAL TREATMENT OF PFAPA SYNDROME – LONG TERM EFFECT

Mariana Cardoso de Oliveira1, Tiago Chantre1, Inês Alpoim Moreira1, Aníbal Eliseu1, Herédio Sousa1, Marta Conde2

1 - Serviço de Otorrinolaringologia - Centro Hospitalar Universitário Lisboa Central
2 - Unidade de Reumatologia Pediátrica - Centro Hospitalar Universitário Lisboa Central 

- 16th Congress of the European Society of Pediatric Otorhinolaryngology

Objective: To access the long-term effect of tonsillectomy on the clinical course of PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis) Syndrome.
Methods: The clinical files of all patients diagnosed with PFAPA Syndrome who underwent tonsillectomy between January 2008 and December 2021, in a Portuguese Tertiary Pediatric Hospital, were reviewed. The follow-up time after tonsillectomy was at least 1 year.
Results: Of 87 patients with PFAPA, 22 (25%) underwent tonsillectomy (N=22), 14 (16%) with associated adenoidectomy. The mean duration of PFAPA symptoms before surgery was 47 months. 12 (55%) of the patients had already been treated with colchicine and 8 (36%) with Vitamin D as primary prophylaxis. 18/22 (82%) patients responded to tonsillectomy, the majority (17/22, 77%) having no more fever attacks following surgery and 1 ceasing fever attacks within the year. In the 4 remaining cases, the frequency of fever attacks decreased. 5 (23%) had complete and 15 (68%) partial resolution of the other symptoms. Aphthous stomatitis was the symptom that persisted most (14, 64%). There is no statistically significant difference in resolution of fever attacks according to disease duration, age at onset, frequency of attacks and type of surgery (adenotonsillectomy vs tonsillectomy). There is also no difference between the resolution of aphthous stomatitis according to gender, disease duration and age at onset.
Conclusions: Tonsillectomy is a good option for PFAPA treatment but is more effective in resolving fever than other symptoms. There is no evidence of benefit in associating adenoidectomy.  

Palavras Chave: PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis) Syndrome, tonsillectomy