fioptic nerve and retina, resulting in postnatal myelination continued growth, and crossed the lamina cribrosa level, reaching the retina, and even more peripheral fundus. the typical fundus changes around the optic disc for visible opaque white haze, from the edge of the optic nerve outward feathery. conclusion: retinal lesions limited to around the optic disc and retinal periphery are generally no obvious clinical symptoms, vision is generally no effect, and don’t need treatment. a large area of the retina myelinated nerve fibers in patients or lesions involving the macula, more associated with high myopia, amblyopia or strabismus and other defects. typical retinal nerve fibers need to be identified with the diseases of the choroid retina, Jensen’s disease, and infiammatory exudates of the optic papilla.%目的：总结视网膜有髓神经纤维（retinal Medullated nerve fibers）的特征，供同道借鉴。方法通过临床发现的视网膜有髓神经纤维（retinal Medullated nerve fibers）两例罕见疾病，分析发病原因、特征、病情、鉴别诊断。结果视网膜有髓神经纤维，是一种发育异常性疾病。正常情况下，视神经髓鞘纤维在胚胎7个月时，开始由中枢神经向周围生长，在出生3个月后达到并终止于视盘筛板后端。若存在发育异常，神经纤维髓鞘的少突细胞从视神经异位至视网膜上，导致出生后髓鞘仍持续性生长，并越过筛板水平，达到视网膜甚至更周边的眼底。典型眼底改变为视盘周围可见不透明白色混浊，从视盘边缘向外扩张呈羽毛状。结论病变累及视网膜仅限于视盘周围或视网膜周边部者一般无明显临床症状，视力一般无影响，无需治疗。大面积的视网膜有髓神经纤维或病变累及黄斑的患者，多伴发高度近视、弱视甚至斜视等缺陷。典型的视网膜有髓神经纤维需与脉络膜视网膜炎、Jensen病、视乳头旁炎性渗出斑等疾病相鉴别。
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