Dagi L R, Leys M J, Hansen R M, Fulton A B
Department of Ophthalmology, Harvard Medical School, Boston, Mass 02115.
Arch Ophthalmol. 1990 May;108(5):709-12. doi: 10.1001/archopht.1990.01070070095043.
We studied the refractive status of 13 children with Leber's congenital amaurosis. Seven had the disease complicated by neurological or other systemic abnormalities, while the other 6 patients had only ophthalmic abnormalities. All 13 patients were hyperopic. The magnitude of hyperopia did not differ significantly between the complicated and uncomplicated groups. Therefore, one cannot, as previously suggested, use the presence of high hyperopia to differentiate an uncomplicated form of Leber's congenital amaurosis from one complicated by neurologic or other systemic abnormalities. The concurrence of hyperopia with Leber's congenital amaurosis should not steer the physician away from careful neurologic systemic or biochemical evaluation of the child.
我们研究了13例莱伯先天性黑矇患儿的屈光状态。其中7例患儿的疾病合并有神经或其他全身异常，而另外6例患儿仅有眼部异常。所有13例患者均为远视。复杂组和非复杂组之间的远视程度无显著差异。因此，正如之前所建议的，不能用高度远视的存在来区分单纯型莱伯先天性黑矇与合并神经或其他全身异常的类型。远视与莱伯先天性黑矇的并存不应使医生忽视对患儿进行仔细的神经、全身或生化评估。
