Bernstein Aryeh, Sunness Janet S, Applegate Carol A, Tegins Elizabeth O
*Hoover Low Vision Rehabilitation Services and the Department of Ophthalmology, Greater Baltimore Medical Center, Baltimore, Maryland; †Department of Medicine, Technion Institute of Technology, Haifa, Israel; ‡Department of Ophthalmology and Visual Sciences, University of Maryland School of Medicine, Baltimore, Maryland; and §North Carolina Retina Associates, Raleigh, North Carolina.
Retina. 2016 Sep;36(9):1741-50. doi: 10.1097/IAE.0000000000001003.
To describe the enlargement of the dense scotoma over time in Stargardt disease and to highlight methodologic issues in tracking enlargement.
Retrospective study of patients with full mapping of the border of the dense scotoma using the MP-1 for at least two visits.
14 eyes of 7 patients met this criterion. Patients had median of 3 visits (range 2-5), with median total follow-up of 4.5 years (range 1.5-8). Mean baseline visual acuity was 20/56 (range 20/25-20/200), mean baseline dense scotoma area was 2.23 mm (range 0.41-5.48), and mean dense scotoma enlargement rate was 1.36 mm/year (range 0.22-2.91). The younger patients tended to have more rapid loss of visual acuity, which tended to plateau when the visual acuity was 20/100 or worse. The patients who developed Stargardt before age 20 years, and the single patient who developed Stargardt disease after age 40 years, had more rapid enlargement rates, with preservation of central vision in the oldest patient. The ability to precisely define the dense scotoma area was dependent on the density location of the points tested; this led to significant variability in the assessment of the scotoma enlargement rate in three of the seven patients. The dense scotoma was not described adequately by the extent of the homogeneous dark area on fundus autofluorescence imaging.
Microperimetry is necessary for mapping the scotoma in patients with Stargardt disease, because current imaging is not adequate. Standardized grid testing, plus a standardized procedure for refining the border of the dense scotoma, should allow more precise testing and longitudinal assessment of enlargement rates.
描述斯塔加特病中致密暗点随时间的扩大情况，并强调追踪扩大情况时的方法学问题。
对使用MP-1至少进行两次随访以完整绘制致密暗点边界的患者进行回顾性研究。
7例患者的14只眼符合该标准。患者的中位随访次数为3次（范围2 - 5次），中位总随访时间为4.5年（范围1.5 - 8年）。平均基线视力为20/56（范围20/25 - 20/200），平均基线致密暗点面积为2.23平方毫米（范围0.41 - 5.48），平均致密暗点扩大率为1.36平方毫米/年（范围0.22 - 2.91）。较年轻的患者往往视力丧失更快，当视力为20/100或更差时趋于平稳。20岁之前患斯塔加特病的患者以及40岁之后患斯塔加特病的唯一患者，其扩大率更快，最年长的患者保留了中心视力。精确界定致密暗点面积的能力取决于所测试点的密度位置；这导致7例患者中有3例在暗点扩大率评估上存在显著差异。眼底自发荧光成像上均匀暗区的范围并不能充分描述致密暗点。
微视野检查对于绘制斯塔加特病患者的暗点是必要的，因为目前的成像方法并不充分。标准化的网格测试，加上完善致密暗点边界的标准化程序，应能实现更精确的测试和扩大率的纵向评估。
