Pierre-Robin综合征儿童临床特征及睡眠呼吸紊乱分析

Pierre-Robin综合征儿童临床特征及睡眠呼吸紊乱分析
林晶;张依纯;叶楚远;蔡慧琳;俞晨艺;蔡晓红
【期刊名称】《温州医学院学报》
【年(卷),期】2018(048)001
【摘要】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 clini-cal 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.%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.
【总页数】4页(P34-37)
【作者】林晶;张依纯;叶楚远;蔡慧琳;俞晨艺;蔡晓红
【作者单位】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;杭州市儿童医院儿童内科,浙江杭州 310000;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;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;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;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;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
【正文语种】中文
【中图分类】R725.6
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