Margo C E, Hamed L M, Fang E, Dawson W W
Department of Ophthalmology, University of Florida College of Medicine, Gainesville.
Arch Ophthalmol. 1992 Nov;110(11):1610-3. doi: 10.1001/archopht.1992.01080230110033.
Optic nerve aplasia is a rare congenital defect invariably associated with other ocular or systemic disorders. We examined a 3-year-old girl with monocular microphthalmos who had optic nerve aplasia on histopathologic examination of the eye after enucleation. Magnetic resonance imaging verified the presence of unilateral optic nerve aplasia, and demonstrated hemichiasmal hypoplasia on the affected side and bilateral optic tracts. The visually evoked cortical response revealed increased signals over the occipital cortex ipsilateral to the aplastic nerve, suggesting misdirection of axons from the temporal retina of the normal eye. The visual pathway in unilateral optic nerve aplasia may assume a primitive form of neuronal organization characterized by an increase in contralateral retinogeniculostriate projection.
视神经发育不全是一种罕见的先天性缺陷，总是与其他眼部或全身疾病相关。我们检查了一名3岁单眼小眼球女童，其眼球摘除术后的眼部组织病理学检查显示存在视神经发育不全。磁共振成像证实存在单侧视神经发育不全，并显示患侧半侧视交叉发育不全及双侧视束。视觉诱发电位皮层反应显示，发育不全神经同侧枕叶皮层信号增强，提示正常眼颞侧视网膜轴突发生错向。单侧视神经发育不全时的视觉通路可能呈现一种原始形式的神经元组织，其特征为对侧视网膜-膝状体-视皮层投射增加。
