Burton Martin J, Pollard Andrew J, Ramsden James D
Department of Otolaryngology - Head and Neck Surgery, Oxford Radcliffe Hospitals NHS Trust, Level LG1, West Wing, John Radcliffe Hospital, Oxford, UK, OX3 9DU.
Cochrane Database Syst Rev. 2010 Sep 8(9):CD008669. doi: 10.1002/14651858.CD008669.
PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome) is a rare clinical syndrome of unknown cause usually identified in children.
To assess the efficacy of tonsillectomy (with or without adenoidectomy) in children with PFAPA.
We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library, 2010 Issue 1); MEDLINE (PubMed); EMBASE; CINAHL; mRCT (metaRegister of clinical trials, including ClinicalTrials.gov); NRR (National Research Register); LILACS; KoreaMed; IndMed; PakMediNet; China Knowledge Network; CAB Abstracts; Web of Science; BIOSIS Previews; ICTRP (International Clinical Trials Registry Platform) and Google. The date of the last search was 21 January 2010.
Randomised studies comparing adeno-/tonsillectomy with non-surgical treatment.
Two authors independently assessed trial quality and extracted data.
Two trials involving 67 children were included. One high quality study demonstrated a dramatic benefit of adenotonsillectomy in children with PFAPA diagnosed according to rigid, standard criteria with a relative 'risk' (RR) of symptom resolution after 18 months of 12.63 (95% CI 1.81 to 87.98) and a lower rate of episodes per patient-month (rate ratio 0.07; 95% CI 0.04 to 0.13). A less methodologically rigorous study enrolled some children with PFAPA, but probably included others with alternative types of recurrent pharyngitis, and performed tonsillectomy alone. This also demonstrated a significant benefit for surgery at six months: RR 1.93 (95% CI 1.11 to 3.36); rate ratio episodes per patient-month 0.10 (95% CI 0.04 to 0.28). The pooled relative risk of symptom resolution was 3.25 (95% CI 1.78 to 5.92) and the resulting number needed to treat (NNT) 2 (95% CI 1 to 3).
AUTHORS' CONCLUSIONS: The trials included in this review reported follow up at 18 and six months respectively but it is well-established that children with PFAPA recover spontaneously and treatment can be administered to try and reduce the severity of individual episodes. Therefore, the parents and carers of children with PFAPA must weigh the risks and consequences of surgery (hospitalisation, a predictable period of time postoperatively away from school/nursery, the risks of surgery) against the alternative of a finite period of recurrent episodes of disease at predictable intervals, potentially requiring time off school and the regular use of medication. It is uncertain whether adenoidectomy combined with tonsillectomy adds any additional benefit to tonsillectomy alone.
PFAPA综合征（周期性发热、阿弗他口炎、咽炎和颈淋巴结炎综合征）是一种病因不明的罕见临床综合征，通常在儿童中发现。
评估扁桃体切除术（伴或不伴腺样体切除术）对PFAPA患儿的疗效。
我们检索了Cochrane对照试验中心注册库（CENTRAL）（《Cochrane图书馆》，2010年第1期）；MEDLINE（PubMed）；EMBASE；CINAHL；mRCT（临床试验元注册库，包括ClinicalTrials.gov）；NRR（国家研究注册库）；LILACS；KoreaMed；IndMed；PakMediNet；中国知网；CAB文摘数据库；科学引文索引；生物学文摘数据库；ICTRP（国际临床试验注册平台）以及谷歌。最后一次检索日期为2010年1月21日。
比较腺样体/扁桃体切除术与非手术治疗的随机研究。
两位作者独立评估试验质量并提取数据。
纳入了两项涉及67名儿童的试验。一项高质量研究表明，对于根据严格标准确诊的PFAPA患儿，腺样体扁桃体切除术有显著益处，18个月后症状缓解的相对“风险”（RR）为12.63（95%可信区间1.81至87.98），且每位患者每月发作次数较低（发作率比为0.07；95%可信区间0.04至0.13）。一项方法学严谨性稍差的研究纳入了一些PFAPA患儿，但可能也包括其他类型复发性咽炎患儿，且仅进行了扁桃体切除术。该研究也表明手术在6个月时具有显著益处：RR为1.93（95%可信区间为1.11至3.36）；每位患者每月发作率比为0.10（95%可信区间0.04至0.28）。症状缓解的合并相对风险为3.25（95%可信区间1.78至5.92），所需治疗人数（NNT）为2（95%可信区间1至3）。
本综述纳入的试验分别报告了18个月和6个月的随访情况，但众所周知PFAPA患儿可自发恢复，可进行治疗以试图减轻个体发作的严重程度。因此，PFAPA患儿的家长和护理人员必须权衡手术的风险和后果（住院、术后一段可预见的缺课/缺托时间、手术风险）与疾病以可预见的间隔反复发作为期有限的另一种选择，后者可能需要缺课以及定期用药。腺样体切除术联合扁桃体切除术是否比单纯扁桃体切除术有任何额外益处尚不确定。
