The syndrome of optic nerve hypoplasia

被引:44
作者
Borchert, Mark [1 ]
Garcia-Filion, Pamela [1 ]
机构
[1] Childrens Hosp Los Angeles, Vis Ctr, Los Angeles, CA 90027 USA
基金
美国国家卫生研究院;
关键词
D O I
10.1007/s11910-008-0061-7
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
The congenital malformation known as optic nerve hypoplasia (ONH) has been recognized in the past 30 years as an epidemic cause of congenital blindness. It was believed to occur either as an isolated anomaly or as a component of the syndrome of septo-optic dysplasia, which has evolved to include midline brain malformations and hypopituitarism. Evidence now suggests that ONH infrequently occurs in isolation. Most afflicted children will have hypothalamic dysfunction and/or neurodevelopmental impairment, regardless of MRI findings or severity of ONH. Adverse outcomes can often be ameliorated with early intervention. Thus, the syndrome of ONH should be suspected in all infants with signs of hypothalamic dysfunction or vision impairment.
引用
收藏
页码:395 / 403
页数:9
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