Topographical retinal function in oculocutaneous albinism
Topographical retinal function in oculocutaneous albinism
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DOI：
10.1016/j.ajo.2006.01.045
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发表时间：
2006-06-01
影响因子：
4.2
通讯作者：
Weiss, AH
中科院分区：
文献类型：
--
作者：
Kelly, JP;Weiss, AH
黄斑发育不全是白化病患者的主要眼部表现，因为它是视力下降和先天性眼球震颤的基础。解剖学研究表明缺乏中央凹分化。然而，在白化病黄斑的功能解剖是未知的。设计：观察性病例reports.METHODS：我们测量的拓扑结构的视网膜反应在两名患者眼皮肤白化病，同时使用眼底监测和多焦视网膜电图（ERG）。刺激由以黄斑为中心的103六边形阵列组成。结果：与成人正常值相比，ERG振幅仅在中央5~10 °处降低。当反应按刺激面积缩放时，多焦ERG振幅在所有视网膜eccentricities. CONCLUSIONS：结果表明，白化病的中央视网膜的锥体光感受器的均匀密度，与解剖学研究一致，显示逮捕出生后的黄斑发展。需要进一步的规范性数据来充分量化幼儿黄斑发育。
PURPOSE: Macular hypoplasia is the major ocular finding in albinism because it underlies reduced acuity and congenital nystagmus. Anatomic studies have shown a lack of foveal differentiation. However, functional anatomy of the macula in albinism is not known.DESIGN: Observational case reports.METHODS: We measured the topology of the retinal response in two patients with oculocutaneous albinism by using simultaneous fundus monitoring and multifocal electroretinographs (ERGs). Stimuli consisted of a 103-hexagon array centered on the macula. Recording conditions eliminated nystagmus artifact.RESULTS: ERG amplitudes were reduced only in the central 5 to 10 degrees compared with adult norms. When the response was scaled by stimulation area, multifocal ERG amplitudes were constant across all retinal eccentricities.CONCLUSIONS: The results suggest a homogeneous density of cone photoreceptors across the central retina in albinism, consistent with anatomic studies showing arrest of postnatal macular development. Further normative data are needed to fully quantify macular development in young children.