Rahi J S, Botting B
Department of Paediatric Epidemiology, Institute of Child Health, Great Ormond Street Hospital NHS Trust, London, UK.
Br J Ophthalmol. 2001 Sep;85(9):1049-51. doi: 10.1136/bjo.85.9.1049.
BACKGROUND/AIMS: Congenital ocular anomalies contribute significantly to childhood visual morbidity, with congenital cataract being a major cause of visual impairment throughout the world. As in many other countries, a National Congenital Anomaly System (NCAS) exists in England and Wales to monitor the frequency of ocular and other anomalies in order to identify new public health hazards and inform aetiological research. The aim of this study was to assess level of ascertainment by the NCAS of children with congenital cataract.
Using independent ophthalmic and paediatric national active surveillance schemes, all infants (< or =1 year) newly diagnosed with congenital and infantile cataract in England and Wales in 1 year from September 1995 were identified. These notifications were compared with those made independently to the NCAS during the same period. The proportion of cases identified by the active surveillance schemes and also notified to the NCAS was determined.
10% (15/149) of eligible children with newly diagnosed congenital or infantile cataract were actually notified to the NCAS. A higher proportion of those diagnosed as neonates (16%, 14/85) than in later infancy (2%, 1/64) was ascertained through the NCAS. There is a need for better verification of notifications and reported information in the NCAS.
Currently, ascertainment of congenital cataract through the NCAS is low and the system is likely to be insensitive to small but important changes in risk factors for this disorder. This limits its use for monitoring secular and other trends in ocular anomalies. Strategies to improve its future use are discussed, including enhancing the awareness and participation of ophthalmic professionals involved in managing children with anomalies.
背景/目的：先天性眼部异常是儿童视力低下的重要原因，先天性白内障是全球视力损害的主要原因。与许多其他国家一样，英格兰和威尔士设有国家先天性异常系统（NCAS），以监测眼部及其他异常的发生频率，从而识别新的公共卫生危害并为病因学研究提供信息。本研究的目的是评估NCAS对先天性白内障患儿的确诊水平。
采用独立的眼科和儿科国家主动监测方案，确定1995年9月起1年内英格兰和威尔士所有新诊断为先天性和婴儿性白内障的婴儿（≤1岁）。将这些报告与同期独立向NCAS报告的情况进行比较。确定主动监测方案识别并同时报告给NCAS的病例比例。
新诊断为先天性或婴儿性白内障的合格儿童中，仅有10%（15/149）实际报告给了NCAS。通过NCAS确诊的新生儿比例（16%，14/85）高于婴儿后期（2%，1/64）。NCAS需要更好地核实报告及所报告的信息。
目前，通过NCAS确诊先天性白内障的比例较低，该系统可能对这种疾病危险因素的微小但重要的变化不敏感。这限制了其在监测眼部异常的长期趋势和其他趋势方面的应用。文中讨论了改善其未来应用的策略，包括提高参与管理异常儿童的眼科专业人员的意识和参与度。
