Hodes B L, Feiner L A, Sherman S H, Cunningham D
Arch Ophthalmol. 1984 Mar;102(3):381-3. doi: 10.1001/archopht.1984.01040030299023.
Pseudovitelliform macular dystrophy is a dominantly inherited macular disorder. It is characterized by the presence of horizontally oval yellowish deposits within the maculas that are approximately one-third disc diameter in size. As classically described, progression of the lesions over a follow-up period of as long as six years has not been described and the prognosis for retention of good visual function is excellent. We report the ophthalmoscopic appearance of six eyes of three patients from a very subtle alteration of macular pigmentation to severe disruption of macular architecture, and demonstrate in one of our patients drastic deterioration in both ophthalmoscopic appearance and visual function over a three-year period of follow-up. The prognosis for retention of good vision in this disorder must include the possibility of the eventual development of severe visual impairment.
假性卵黄样黄斑营养不良是一种常染色体显性遗传性黄斑疾病。其特征为黄斑区内存在水平椭圆形淡黄色沉积物,大小约为视盘直径的三分之一。如经典描述的那样,病变在长达六年的随访期内的进展情况尚未见报道,且保留良好视功能的预后极佳。我们报告了三名患者六只眼的眼底镜检查表现,从黄斑色素的非常细微改变到黄斑结构的严重破坏,并在我们的一名患者中显示,在三年的随访期内,眼底镜检查表现和视功能均急剧恶化。这种疾病保留良好视力的预后必须考虑到最终发展为严重视力损害的可能性。