结论:这项多中心研究评估了婴儿眼震综合征(INS)的临床和心理方面,重点是其管理和非手术治疗。
目的:本研究旨在评估临床特征,管理,关系生活,和心理影响一组在儿童年龄发生眼球震颤的患者。
方法:这项观察性研究包括2017年1月1日至2020年12月31日在意大利两个中心转诊的INS患者。眼科和矫正特征以及视觉功能对生活质量的影响,根据眼球震颤特异性眼球震颤生活质量问卷,在整个样本和任何INS亚组中进行了分析。
结果:纳入43例患者;其中65.1%患有特发性INS(IINS),34.9%的人患有与眼部疾病相关的INSOD。中位年龄为15.4岁(四分位距[IQR],10.4至17.3年),组间差异显著(中位数,在IINSvs.的人中15.8年INSOD患者为12.3年;p<0.001)。在INSOD亚组中,斜视明显更普遍(93.3vs.57.1%;p=0.017)。在IINS子样本中,主要位置的双眼距离最佳矫正视力明显更高(p<0.001)。在异常头部位置评估中进一步证实了这种行为(p<0.001)。在接近最佳矫正视力评估时,两组间的差异在初始位置(p<0.001)比在异常头部位置更为显著.对比敏感度显示IINS亚组的值显著较高(p<0.001)。眼球震颤生活质量问卷显示,与INSOD相比,IINS的得分显着降低(中位数总分,90.5[IQR,84to97]vs.94[IQR,83.0至96.5];p<0.001)。
结论:与INSOD组相比,IINS组的眼科和直视结果明显更好。相反,在IINS组中,心理和生活质量的影响明显更大。据我们所知,这是第一项多中心研究,调查IIN的临床特征并比较两个主要亚组,INS和INSOD。
CONCLUSIONS: This multicenter study assessed clinical and psychological aspects of infantile nystagmus syndrome (INS) focusing on its management and nonsurgical treatment.
OBJECTIVE: This study aimed to assess clinical features, management, relationship life, and psychological impact in a group of patients with nystagmus onset in pediatric age.
METHODS: This observational study included patients diagnosed with INS referred to two Italian centers from January 1, 2017, to December 31, 2020. Ophthalmologic and orthoptic features and impact of visual function on quality of life, according to nystagmus-specific nystagmus quality of life questionnaire, were analyzed within the overall sample and in any of INS subgroups.
RESULTS: Forty-three patients were included; 65.1% of them had idiopathic INS (IINS), and 34.9% had INS associated with ocular diseases (INSOD). The median age was 15.4 years (interquartile range [IQR], 10.4 to 17.3 years), significantly different between groups (median, 15.8 years among those with IINS vs. 12.3 years among those with INSOD; p<0.001). In the INSOD subgroup, strabismus was significantly more prevalent (93.3 vs. 57.1%; p=0.017). Binocular distance best-corrected visual acuity in primary position was significantly higher in the IINS subsample (p<0.001). Such behavior was further confirmed at anomalous head position evaluation (p<0.001). At near best-corrected visual acuity assessment, differences between groups were more remarkable in primary position (p<0.001) than in anomalous head position. Contrast sensitivity showed significantly higher values in the IINS subgroup (p<0.001). The nystagmus quality of life questionnaire disclosed a significantly lower score in IINS as compared with INSOD (median total score, 90.5 [IQR, 84 to 97] vs. 94 [IQR, 83.0 to 96.5]; p<0.001).
CONCLUSIONS: The IINS group showed significantly better ophthalmologic and orthoptic outcomes than the INSOD group. The psychological and quality-of-life impact was instead significantly greater in the IINS group. To the best of our knowledge, this is the first multicenter study investigating the clinical features of IIN and comparing the two main subgroups, IINS and INSOD.