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患有初级先天性玻璃眼患者的长期功能和结构结果-丹麦全国性研究

Troels Brynskov¹, Daniella Bach-Holm¹, Per Kappelgaard¹

¹Department of Ophthalmology, Copenhagen University Hospital Rigshospitalet-Glostrup, Copenhagen, Denmark.

Acta ophthalmologica

|October 5, 2023

概括

大多数双侧原发性先天性玻璃眼 (PCG) 患者至少在一只眼睛保持良好的视力. 然而,延迟诊断和多次手术与PCG患者的结果更差有关.

科学领域:

眼科医生眼科眼科
遗传学遗传学是一个
儿科医学儿科医学

背景情况:

初级先天性玻璃眼 (PCG) 是一种罕见的遗传性疾病,导致眼内压力升高和视神经受损.
早期诊断和干预对于保护PCG患者的视力至关重要.

研究的目的:

评估初级先天性玻璃眼 (PCG) 患者的长期功能和结构结果.
在PCG患者中评估视觉敏度 (VA),视野 (VF) 和周状视网膜神经纤维层 (pRNFL) 厚度.

主要方法:

在丹麦 (1977-2016) 诊断的PCG患者的医疗记录的回顾性审查.
定义严重的视力丧失为VA <6/60和/或VF>20分贝 (dB).
使用相关性矩阵评估预后因素.

主要成果:

在94名患者中 (153个PCG眼睛),62%的VA≥6/18,但22%的VA<6/60.
在视力较好的眼睛中,90%的VA≥6/18,只有5%的VA<6/60.
在29%的测量眼睛中观察到减少的prnfl;差的va,vf和prnfl是相关的 (p<0.0001).
更多的手术和更长的诊断延迟与较差的视力和VF结局有关.

结论:

在丹麦,大多数双边PCG患者在较好的眼睛中保持VA≥6/18.
视力敏度差与视野受损有显著的关联.
较长的诊断延迟和增加的手术干预与PCG的较差长期预后相关.

关键词:

长期的结果是长期的结果.光学连贯性断层扫描 (optical coherence tomography) 是一种光学连贯性断层扫描技术.一次性先天性玻璃眼.主要的儿科玻璃眼.视网膜神经纤维层厚度视网膜神经纤维层厚度标准自动化周边测量系统

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