Abstract：
BACKGROUND: To investigate best-corrected visual acuity (BCVA) outcomes in patients with optic nerve hypoplasia (ONH)/septo-optic-pituitary dysplasia (SOD). Our primary hypothesis was that BCVA in patients with ONH/SOD does not change significantly over time.
METHODS: A chart review was undertaken in patients with a confirmed diagnosis of ONH/SOD. Demographic and clinical ophthalmologic data were extracted. Quantitative BCVA data were investigated across clinic visits after converting acuities to the logarithm of the minimum angle of resolution (logMAR).
RESULTS: There were 102 patients (56 males). Median age at the end of the study was 12.7 years. Median duration of follow-up was 4.5 years. BCVA significantly worsened slightly in the most affected eyes (0.056 average increase in logMAR/year, 95% confidence interval [CI]: 0.037 to 0.075) and significantly improved mildly in the lesser or equally affected eyes (0.014 average decrease in logMAR/year, 95% CI: 0.009 to 0.019) (P < 0.0001).
CONCLUSIONS: Although the overall BCVA data showed a statistically significant change with time, the actual changes were small and are of doubtful meaningful clinical significance (less than one line change on a Snellen chart). Our data suggest that ONH/SOD are nonprogressive neurodevelopmental disorders. The mild worsening of BCVA in the most affected eyes may be caused by amblyopia, whereas the small improvement in the lesser or equally affected eyes may be caused by developmental maturation. In addition, the changes in BCVA may also be due to increasing reliability of visual assessments with increasing age.
METHODS: A chart review was undertaken in patients with a confirmed diagnosis of ONH/SOD. Demographic and clinical ophthalmologic data were extracted. Quantitative BCVA data were investigated across clinic visits after converting acuities to the logarithm of the minimum angle of resolution (logMAR).
RESULTS: There were 102 patients (56 males). Median age at the end of the study was 12.7 years. Median duration of follow-up was 4.5 years. BCVA significantly worsened slightly in the most affected eyes (0.056 average increase in logMAR/year, 95% confidence interval [CI]: 0.037 to 0.075) and significantly improved mildly in the lesser or equally affected eyes (0.014 average decrease in logMAR/year, 95% CI: 0.009 to 0.019) (P < 0.0001).
CONCLUSIONS: Although the overall BCVA data showed a statistically significant change with time, the actual changes were small and are of doubtful meaningful clinical significance (less than one line change on a Snellen chart). Our data suggest that ONH/SOD are nonprogressive neurodevelopmental disorders. The mild worsening of BCVA in the most affected eyes may be caused by amblyopia, whereas the small improvement in the lesser or equally affected eyes may be caused by developmental maturation. In addition, the changes in BCVA may also be due to increasing reliability of visual assessments with increasing age.
摘要：
背景：探讨视神经发育不良（ONH）/隔视神经-垂体发育不良（SOD）患者的最佳矫正视力（BCVA）结局。我们的主要假设是ONH/SOD患者的BCVA随时间没有显着变化。
方法：对确诊为ONH/SOD的患者进行图表回顾。提取人口统计学和临床眼科数据。在将视力转换为最小分辨角（logMAR）的对数后，在整个临床就诊中研究了定量BCVA数据。
结果：共102例（男56例）。研究结束时的中位年龄为12.7岁。中位随访时间为4.5年。受影响最严重的眼睛的BCVA显着轻度恶化（logMAR/年平均增加0.056，95％置信区间[CI]：0.037至0.075），并且在受影响较小或同等程度的眼睛中显着轻度改善（logMAR/年平均下降0.014，95%CI：0.009~0.019(P<0.0001)。
结论：尽管总体BCVA数据显示出随时间的统计学显着变化，实际变化很小,具有值得怀疑的有意义的临床意义(Snellen图表上的变化小于一行).我们的数据表明ONH/SOD是非进行性神经发育障碍。受影响最严重的眼睛中BCVA的轻度恶化可能是由弱视引起的，而受影响较小或同等程度的眼睛的微小改善可能是由发育成熟引起的。此外,BCVA的变化也可能是由于视觉评估的可靠性随着年龄的增长而增加.
方法：对确诊为ONH/SOD的患者进行图表回顾。提取人口统计学和临床眼科数据。在将视力转换为最小分辨角（logMAR）的对数后，在整个临床就诊中研究了定量BCVA数据。
结果：共102例（男56例）。研究结束时的中位年龄为12.7岁。中位随访时间为4.5年。受影响最严重的眼睛的BCVA显着轻度恶化（logMAR/年平均增加0.056，95％置信区间[CI]：0.037至0.075），并且在受影响较小或同等程度的眼睛中显着轻度改善（logMAR/年平均下降0.014，95%CI：0.009~0.019(P<0.0001)。
结论：尽管总体BCVA数据显示出随时间的统计学显着变化，实际变化很小,具有值得怀疑的有意义的临床意义(Snellen图表上的变化小于一行).我们的数据表明ONH/SOD是非进行性神经发育障碍。受影响最严重的眼睛中BCVA的轻度恶化可能是由弱视引起的，而受影响较小或同等程度的眼睛的微小改善可能是由发育成熟引起的。此外,BCVA的变化也可能是由于视觉评估的可靠性随着年龄的增长而增加.
