Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: Relapse and Tonsillar Regrowth After Childhood Tonsillectomy

Lantto, Ulla; Koivunen, Petri; Tapiainen, Terhi; Renko, Marjo

doi:10.1002/lary.29474

Cited by 9 publications

(6 citation statements)

References 21 publications

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“…It has been suggested that the volume of the removed tonsillar tissue could be crucial in the efficacy of operative treatments in PFAPA syndrome. 26,27 In this trial, the amount of removed tonsillar tissue was often small, adenoids were left untouched, and the effect on PFAPA symptoms was still evident. In addition to the volume of the tonsils, localization of the removed tissue may be important.…”

Section: Discussionmentioning

confidence: 84%

“…The mechanisms of the effectiveness of tonsil surgery in PFAPA syndrome are not known. It has been suggested that the volume of the removed tonsillar tissue could be crucial in the efficacy of operative treatments in PFAPA syndrome 26,27 . In this trial, the amount of removed tonsillar tissue was often small, adenoids were left untouched, and the effect on PFAPA symptoms was still evident.…”

Section: Discussionmentioning

confidence: 91%

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Tonsillotomy for Periodic Fever Syndrome: A Randomized and Controlled Trial

et al. 2023

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ObjectiveTonsillectomy is an effective treatment for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Tonsillotomy has a milder operative risk profile and postoperative morbidity in children than tonsillectomy. We aimed to compare the efficacy of tonsillotomy to observation‐only in children with PFAPA syndrome at a 3‐month follow‐up.MethodsThis was a randomized multicenter trial with sequential design. Participants were randomized into a tonsillotomy group and a control group that was only observed. The trial started in 1/2017 and was accomplished in 12/2021 with 16 patients (10 boys, six girls, the mean age 4.2 years). The symptoms were monitored with daily symptom diaries.ResultsAfter the 3‐month follow‐up, 7/8 patients (87.5%) in the tonsillotomy group and 2/8 (25%) patients in the control group were free from PFAPA symptoms (95% CI 13% to 87%; p = 0.0021). The mean number of days with fever was 2.6 (SD 3.7) in the tonsillotomy group and 8.0 (SD 6.5) days in the control group (n = 8) (p = 0.06). Mean number of fever days compatible with PFAPA syndrome was 0.8 (SD 1.4) in the tonsillotomy group and 6.5 (SD 6.0) in the control group (95%CI −10% to −1%; p = 0.007). Rescue tonsillectomy was needed for all patients in the control group and none of the patients in the tonsillotomy group.ConclusionsTonsillotomy might be an effective treatment option for children with PFAPA syndrome. Further studies are needed to clarify the long‐term efficacy of tonsillotomy for treating PFAPA.Level of Evidence2 Laryngoscope, 2023

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Section: Discussionmentioning

confidence: 84%

Section: Discussionmentioning

confidence: 91%

Tonsillotomy for Periodic Fever Syndrome: A Randomized and Controlled Trial

et al. 2023

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“…Regrowth of the tonsillar tissue is also proposed to play a role in those with continued symptoms after tonsillectomy [42]. Five (5%) of 94 children in Finnish cohort had continued flares after tonsillectomy; most of these five patients had relapse of symptoms years after tonsillectomy.…”

Section: New Data On Treatment Strategiesmentioning

confidence: 99%

“…Characterizing risk variants for both PFAPA and SURF and understanding whether particular risk variants may predict response to tonsillectomy may shed light on differences and similarities in their pathogenesis. Interestingly, tonsillectomy alone led to complete episode resolution in two cohorts of patients with PFAPA [42,44 ▪ ], suggesting that adenoidectomy may not been needed and that the adenoids do not play as significant of a role in disease pathogenesis.…”

Section: New Data On Treatment Strategiesmentioning

confidence: 99%

What is PFAPA syndrome? Genetic clues about the pathogenesis

Manthiram

2023

Current Opinion in Rheumatology

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Purpose of review Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in childhood. Recent studies report genetic susceptibility variants for PFAPA syndrome and the efficacy of tonsillectomy in a broader cohort of patients with recurrent stereotypical fever. In this review, we highlight the findings of these studies and what they may reveal about the pathogenesis of PFAPA. Recent findings Newly identified genetic susceptibility loci for PFAPA suggest that it is a complex genetic disorder linked to Behçet's disease and recurrent aphthous ulcers. Patients who have PFAPA with some features of Behçet's disease have been reported. Moreover, the efficacy of tonsillectomy has now been described in patients who do not meet the full diagnostic criteria for PFAPA, although the immunologic profile in the tonsils is different from those with PFAPA. Factors that predict response to tonsillectomy are also reported. Summary These findings highlight the heterogeneous phenotypes that may be related to PFAPA due to common genetic susceptibility or response to therapy. These relationships raise questions about how to define PFAPA and highlight the importance of understanding of the genetic architecture of PFAPA and related diseases.

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“…Aside from traditional tonsillitis, intracapsular tonsillectomy has been used for symptomatic relief for active mononucleosis with massive tonsillar hypertrophy [23]. In addition, it has very recently been introduced for successful management of periodic fever, aphthous stomatitis, pharyngitis, and adenitis, or PFAPA [24,25 ▪ ].…”

Section: Expanding Indicationsmentioning

confidence: 99%

Intracapsular tonsillectomy: setting a new standard

Bassett

2023

Current Opinion in Otolaryngology &Amp; Head &Amp; Neck Surgery

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Purpose of review Tonsillectomy is one of the most common surgical procedures performed on children in the United States. Since 2002, the intracapsular technique has been studied as a safer and less painful alternative to total tonsillectomy. Concerns have been raised, however, as to the potential for regrowth and long-term outcomes regarding this technique. Recent findings Studies support the use of intracapsular tonsillectomy in the management of sleep disordered breathing, including in syndromic populations, as well as for tonsillitis. In addition, safety profiles continue to be improved over that of extracapsular dissection. While the incidence of regrowth ranges depending on the study and duration of follow up, it remains acceptably low. The most consistent independent risk factor for revision surgery includes young age. Summary While total tonsillectomy is more thoroughly studied historically, an important absence in the literature is a definitive superiority over the intracapsular technique. With continued high-level studies, as well as additional examination of long-term outcomes, we should continue to see greater acceptance of intracapsular tonsillectomy as a standard of practice in a vulnerable population.

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Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: Relapse and Tonsillar Regrowth After Childhood Tonsillectomy

Cited by 9 publications

References 21 publications

Tonsillotomy for Periodic Fever Syndrome: A Randomized and Controlled Trial

Tonsillotomy for Periodic Fever Syndrome: A Randomized and Controlled Trial

What is PFAPA syndrome? Genetic clues about the pathogenesis

Intracapsular tonsillectomy: setting a new standard

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