Blanc Sibylle, Bourrier Thierry, Boralevi Franck, Sabouraud-Leclerc Dominique, Pham-Thi Nhân, Couderc Laure, Deschildre Antoine, Dutau Guy, Albertini Marc, Tran Antoine, Giovannini-Chami Lisa
Pediatric Pulmonology and Allergy Department, Hôpitaux pédiatriques de Nice University Hospital-Lenval, Nice, France.
Pediatric Dermatology Unit, Hôpital Pellegrin-enfants, Bordeaux University Hospital, Bordeaux, France.
J Pediatr. 2016 Jul;174:211-217.e2. doi: 10.1016/j.jpeds.2016.03.070. Epub 2016 May 2.
To describe the features of Frey syndrome (auriculotemporal nerve dysfunction with gustatory flushing) in childhood.
A multicenter, retrospective, descriptive observational national case series study was conducted with the help of French academic societies. Diagnostic criteria were based on clinical history, and sometimes also on photographs or provocation tests.
Forty-eight cases were identified, with 2 subtypes: 35 unilateral and 13 bilateral. Associated sweating was reported in only 10% of cases. Diagnosis was made in only 20% of children at the first consultation and inappropriate dietary restriction was prescribed for 21%. Instrumented vaginal delivery was significantly associated with unilateral forms (OR [unilateral vs bilateral] = 29; 95% CI 3.99-311.58; P < .001). The outcome was favorable overall with 57% regression, 20% recovery, and only 23% persistence of initial symptoms. Regression was more frequent in unilateral forms (OR = 6.60; 95% CI 1.23-44.04; P = .016), observed in 69% of unilateral forms at a median age of 27 (24-48) months. Recovery predominated in bilateral forms (OR = 0.05; 95% CI 0-0.38; P = .001), observed in 58% of bilateral cases at a median age of 8 (7-9) months.
Frey syndrome in childhood is a rare but benign condition with mild symptoms and a favorable outcome in most cases. Unilateral forms are mostly associated with instrumented delivery. Pediatricians should be familiar with this disorder in order to avoid misdiagnosis, mainly as food allergy, and unnecessary referrals and tests.
描述儿童弗雷综合征（伴有味觉性潮红的耳颞神经功能障碍）的特征。
在法国学术团体的帮助下开展了一项多中心、回顾性、描述性观察性全国病例系列研究。诊断标准基于临床病史，有时也基于照片或激发试验。
共识别出48例病例，分为2种亚型：35例单侧型和13例双侧型。仅10%的病例报告有相关出汗症状。初诊时仅20%的儿童得以确诊，21%的儿童被给予了不恰当的饮食限制。器械助产与单侧型显著相关（比值比[单侧型与双侧型] = 29；95%置信区间3.99 - 311.58；P <.001）。总体预后良好，57%症状消退，20%恢复，仅23%持续存在初始症状。单侧型症状消退更为常见（比值比 = 6.60；95%置信区间1.23 - 44.04；P = 0.016），69%的单侧型病例在中位年龄27（24 - 48）个月时症状消退。双侧型以恢复为主（比值比 = 0.05；95%置信区间0 - 0.38；P = 0.001），58%的双侧型病例在中位年龄8（7 - 9）个月时恢复。
儿童弗雷综合征是一种罕见但良性的疾病，症状较轻，多数病例预后良好。单侧型大多与器械助产有关。儿科医生应熟悉这种疾病，以避免误诊，主要是误诊为食物过敏，以及不必要的转诊和检查。
