Department of Ophthalmology, Faculty of Medical and Health Sciences, New Zealand National Eye Centre, University of Auckland, Auckland, New Zealand.
Br J Ophthalmol. 2014 Oct;98(10):1364-7. doi: 10.1136/bjophthalmol-2013-304605. Epub 2014 May 13.
To identify the clinical features of optic nerve hypoplasia (ONH) and prevalence within a population of New Zealand children with severe visual impairment.
Retrospective review of medical records of children with severe visual impairment registered with Blind and Low Vision Educational Network New Zealand.
Of 1500 children with severe visual impairment, 94 (6.3%) exhibited ONH, and 91 (97%) cases were bilateral. Of these 94 cases, 52 (55%) were males and ethnicities were European Caucasian (52%), Maori (40%), Pasifika (6%) and other (2%). Most children with ONH had poor vision, with 60% having ≤ 6/60 Snellen visual acuity equivalent. The median maternal age was 20.0 years old with 52% ≤ 20 years. There was a statistically significant over-representation of Maori ethnicity (40%) and young maternal age with age less than 20 years old (44%) in our cohort compared to the general population (14.6% and 7.4%, respectively; p<0.0001). Half had hypopituitarism, while neuroimaging abnormalities were detected in 60% cases. Cerebral neuroradiographic abnormalities were found to be associated with higher rate of developmental delay (OR 9.764 95% CI 3.246 to 29.373).
This is the first major study of visual impairment in New Zealand children, and it demonstrates that ONH is an important cause of severe visual disability; with an over-representation of Maori children and younger maternal age.
确定新西兰重度视力损伤儿童群体中视神经发育不全（ONH）的临床特征和患病率。
对新西兰盲与低视力教育网络注册的重度视力损伤儿童的病历进行回顾性研究。
在 1500 名重度视力损伤儿童中，94 名（6.3%）患有 ONH，且 91 名（97%）为双侧。这 94 名患者中，52 名（55%）为男性，种族为欧洲白种人（52%）、毛利人（40%）、太平洋岛民（6%）和其他（2%）。大多数患有 ONH 的儿童视力较差，60%的儿童等效 Snellen 视力≤6/60。母亲的中位年龄为 20.0 岁，其中 52%的母亲年龄小于 20 岁。与普通人群相比（分别为 14.6%和 7.4%；p<0.0001），我们的队列中毛利人（40%）和年轻母亲（年龄小于 20 岁）的比例明显偏高。50%的儿童存在垂体功能减退症，60%的儿童存在神经影像学异常。脑部神经影像学异常与发育迟缓发生率较高相关（OR 9.764 95% CI 3.246 至 29.373）。
这是新西兰儿童视力损伤的首次重要研究，表明 ONH 是导致严重视力障碍的重要原因；毛利儿童和年轻母亲的比例偏高。
