Mehta M, Katsumi O, Tetsuka S, Hirose T, Tolentino F I
Department of Ophthalmology, Eye Research Institute of Retina Foundation, Boston.
Acta Ophthalmol (Copenh). 1991 Feb;69(1):131-4. doi: 10.1111/j.1755-3768.1991.tb02010.x.
The occurrence of a macular hole with Best's dystrophy is an extremely rare finding with only one reported case in the literature. We wish to report two cases with typical Best's dystrophy in one eye and a macular hole in the contralateral eye. In one case the Pattern Reversal VER (PVER) was also recorded and graphed as an amplitude-check size function curve. The PVER changes in this case were grossly subnormal in both the eyes, suggesting a significant degree of bilateral macular impairment. This implies that a significant amount of functional impairment occurs at the vitelliform stage itself, though how this stage progresses to a macular hole is unclear.
黄斑裂孔合并Best氏营养不良极为罕见，文献中仅报道过一例。我们希望报告两例病例，其中一例眼睛患有典型的Best氏营养不良，对侧眼睛有黄斑裂孔。在其中一例中，还记录了图形视网膜电图（PVER），并绘制为振幅-检查大小函数曲线。该病例中双眼的PVER变化严重低于正常水平，提示双眼黄斑存在明显程度的损害。这意味着在卵黄样病变阶段就已出现显著的功能损害，尽管该阶段如何发展为黄斑裂孔尚不清楚。
