Pierre-Robin综合征儿童临床特征及睡眠呼吸紊乱分析  

Clinical features and sleep-disordered breathing in the Pierre-Robin syndrome children

作　　者：林晶[1,2] 张依纯 叶楚远 蔡慧琳 俞晨艺 蔡晓红[1] 

机构地区：[1]温州医科大学附属第二医院育英儿童医院儿童睡眠医学科,浙江温州325027 [2]杭州市儿童医院儿童内科,浙江杭州310000

出　　处：《温州医科大学学报》2018年第1期34-37,共4页Journal of Wenzhou Medical University

基　　金：浙江省自然科学基金资助项目(LY17H010004);国家科技支撑计划项目(2015BAI12B09);浙江省医药卫生科技平台项目(2018ZD010);温州市公益性科技计划项目(Y20170133);浙江省医学重点儿童睡眠医学创新学科

摘　　要：目的:探讨Pierre-Robin综合征(PRS)儿童的临床特征和睡眠呼吸紊乱(SDB)情况,为合理诊治提供依据。方法:收集2014年12月至2016年6月温州医科大学附属第二医院育英儿童医院收治的PRS儿童15例,回顾性分析临床资料,总结临床特征,并随访转归。8例和7例PRS儿童分别在3月龄和12月龄时完成睡眠问卷调查。1例行多导睡眠监测(PSG)。结果:15例均有小下颌畸形、舌后坠及高腭弓,其中3例伴腭裂。13例有呼吸困难,8例合并肺部感染或吸入性肺炎,6例有喂养困难。睡眠问卷调查显示3月龄时8例有SDB,12月龄时5例有SDB。1例PSG结果示中度阻塞性睡眠呼吸暂停低通气综合征。1例4月龄时行下颌牵引成骨术,12例保守治疗,2例失访。13例随访儿童中,5例死亡。8例于12月龄时平均体质量为(8.0±1.5)kg,平均身高为(72.0±3.0)cm。结论:PRS儿童是SDB的高发人群,临床上需提高对该病的认识,选择合适的时机和方法及早治疗。Objective： To study the clinical features and sleep-disordered breathing （SDB） in children with Pierre-Robin syndrome （PRS） to provide accurate diagnosis and treatment of PRS. Methods： Fifteen infants with Pierre-Robin syndrome admitted to the Second Affiliated Hospital ＆ Yuying Children＇s Hospital of Wenzhou Medical University from December 2014 to June 2016 were recruited in this study. A summary of the clinical characteristics, complications, and investigation of the prognosis by out-patient review or telephone follow-up was performed. Seven PRS children finished pediatric sleep questionnaires at 3 months old and eight children did it at 12 months old. Polysomnography （PSG） was monitored in 1 child. Results： Fifteen cases had typical clinical features of PRS including micrognathia, high arch palate, glossoptosis, and 3 had cleft palate. Thirteen cases developed dyspnea, 8 developed lung infection or aspiration pneumonia, and 6 developed feeding difficulties. The results of questionnaire survey show that 8 cases were screened positive for SDB at 3 months old, while 5 at 12 months old. Overnight PSG was performed in 1 case at 3 months and showed evidence of moderate OSAHS. The child with moderate obstructive sleep apnea underwent mandibular distraction osteogenesis at 4 months old. Twelve cases chose conservative treatment. Two cases failed to follow-up. Among 13 follow-up children, 5 died. In the 12th month of follow-up, the average weight of 8 cases was （8.0±1.5） kg, the average height was （72.0±3.0） cm. Conclusion： The PRS in children is associated with high risk for sdb. Timely diagnosis and proper treatment in children with PRS may help prevent long-term adverse sequelae.

关 键 词：Pierre-Robin综合征  小下颌  睡眠呼吸紊乱  儿童 

分 类 号：R725.6[医药卫生—儿科]