Pierre-Robin综合征儿童临床特征及睡眠呼吸紊乱分析

摘要
图/表
参考文献
相关文章 (15)

全文: PDF (1235 KB) HTML (1 KB)
输出: BibTeX | EndNote (RIS)

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。

	服务
	把本文推荐给朋友
	加入我的书架
	加入引用管理器
	E-mail Alert
	RSS
	作者相关文章
	林晶
	张依纯
	叶楚远
	蔡慧琳
	俞晨艺
	蔡晓红

关键词 ： Pierre-Robin综合征, 小下颌, 睡眠呼吸紊乱, 儿童

Abstract：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.

Key words： Pierre-Robin syndrome micrognathia sleep-disordered breathing child

PACS:

基金资助: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

通讯作者: 蔡晓红，教授，主任医师，Email：caixh839@sina.com

作者简介: 林晶（1991-），女，浙江台州人，硕士生

链接本文:

https://xb.wmu.edu.cn/CN/ 或 https://xb.wmu.edu.cn/CN/Y2018/V48/I1/34

[1] 程琪. Pierre Robin序列征的研究进展[J]. 国际儿科学杂志,

2010, 37(6): 565-567.

[2] COLE A, LYNCH P, SLATOR R. A new grading of Pierre Robin sequence[J]. Cleft Palate Craniofac J, 2008, 45(6): 603-606.

[3] 蔡晓红, 李昌崇, 张海邻, 等. 温州地区2~12岁儿童睡眠障碍流行病学调查[J]. 中国实用儿科杂志, 2004, 19(12): 728-730.

[4] 苏苗赏, 俞晨艺, 张渊博, 等. 儿童OSAHS危险因素和微觉醒预警机制探讨[J]. 温州医科大学学报, 2015, 45(3): 180-184.

[5] 中华耳鼻咽喉头颈外科杂志编委会, 中华医学会耳鼻咽喉科学分会. 儿童阻塞性睡眠呼吸暂停低通气综合征诊疗指南草案(乌鲁木齐)[J]. 中华耳鼻咽喉头颈外科杂志, 2007, 42(2): 83-84.

[6] 江载芳, 申昆玲, 沈颖. 诸福棠实用儿科学[M]. 8版. 北京:人民卫生出版社, 2015: 28-66.

[7] 周波. Pierre-Robin综合征病例报告1例及其文献回顾[J]. 医药前沿, 2017, 7(34): 233-234.

[8] 李光荣, 郭斌, 张新荣. Pierre-Robin综合征的CT表现及分型[J]. 中国医学计算机成像杂志, 2014, 20(6): 544-547.

[9] KIELY J L, DEEGAN P C, MCNICHOLAS W T. Resolution of obstructive sleep apnoea with growth in the Robin sequence[J]. Eur Respir J, 1998, 12(2): 499-501.

[10] RACHMIEL A, EMODI O, RACHMIEL D, et al. Internal mandibular distraction to relieve airway obstruction in children with severe micrognathia[J]. Int J Oral Maxillofac Surg, 2014, 43(10): 1176-1181.

[11] 赵竟伊, 靳小雷. 下颌骨牵引成骨技术在婴幼儿Pierre Robin序列征治疗中的应用进展[J]. 中华整形外科杂志, 2014, 30(2): 157-160.

[12] VERLINDEN C R, van de VIJFEIJKEN S E, JANSMA

E P, et al. Complications of mandibular distraction osteogenesis for congenital deformities: a systematic review of the literature and proposal of a new classification for complica-tions[J]. Int J Oral Maxillofac Surg, 2015, 44(1): 37-43.

[13] BREIK O, TIVEY D, UMAPATHYSIVAM K, et al. Mandibular distraction osteogenesis for the management of upper airway obstruction in children with micrognathia: a systematic review[J]. Int J Oral Maxillofac Surg, 2016, 45(6): 769-782.

[14] 万腾, 王国民, 王科, 等. Robin序列征37例临床研究[J]. 口腔颌面外科杂志, 2010, 20(5): 327-330.

[15] DANIEL M, BAILEY S, WALKER K, et al. Airway, feeding and growth in infants with Robin sequence and sleep ap-noea[J]. Int J Pediatr Otorhinolaryngol, 2013, 77(4): 499-503.