Blake K D, Russell-Eggitt I M, Morgan D W, Ratcliffe J M, Wyse R K
Department of Paediatric Cardiology, Institute of Child Health, London.
Arch Dis Child. 1990 Feb;65(2):217-23. doi: 10.1136/adc.65.2.217.
Clinical experience of 50 patients with the CHARGE association is reviewed and problems with management of children born with multiple system involvement is highlighted. It was found that the outlook for survival was poor if more than one of the following three features was present: cyanotic cardiac lesions, bilateral posterior choanal atresia, or tracheo-oesophageal fistula. Mortality was largely due not to the structural heart defects or choanal abnormalities, but reflected underlying pharyngeal and laryngeal incoordination, which resulted in aspiration of secretions. Outcome is likely to be improved if collaboration between specialist surgical teams allows necessary procedures to be performed using the minimum number of anaesthetics. Examination of both the short and long term management of these children has stressed the importance of a multidisciplinary approach to their care.
回顾了50例患有CHARGE综合征患者的临床经验,并强调了患有多系统受累的患儿管理方面的问题。研究发现,如果出现以下三种特征中的一种以上,生存前景就很差:紫绀型心脏病变、双侧后鼻孔闭锁或气管食管瘘。死亡率主要不是由于结构性心脏缺陷或后鼻孔异常,而是反映了潜在的咽喉协调障碍,这导致分泌物误吸。如果专科手术团队之间的合作能够允许使用最少的麻醉剂进行必要的手术,那么预后可能会得到改善。对这些患儿短期和长期管理的研究强调了多学科护理方法的重要性。