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Visual function tests including the role of optical coherence tomography in neurofibromatosis 1.
Child's Nervous System ( IF 1.4 ) Pub Date : 2020-08-04 , DOI: 10.1007/s00381-020-04706-3
Daphna Mezad-Koursh 1, 2 , Anat Bachar Zipori 1, 2 , Dinah Zur 1, 2 , Lior Degabli 1, 2 , Meital Ben-Dov 1, 2 , Ainat Klein 1, 2
Affiliation  

Optic pathway glioma (OPG) is a common and significant complication of neurofibromatosis 1 (NF-1) that might lead to vision loss. The main reason to treat OPG is to preserve vision. Tumor location along the visual pathway largely dictates the presenting signs and symptoms. Clinical ophthalmic evaluation is focused on optic nerve functions including evaluation of pupils’ reaction to light, visual acuity, color vision, and visual field, as well as optic nerve appearance. An important relatively new ancillary test is optic coherence tomography (OCT) that measures the volume of retinal nerve fiber layer around the optic nerve and the ganglion cell layer-inner plexiform layer (GCL-IPL) of the macula, both proved to be strongly associated with losing vision in OPG. Accurate evaluation of vision functions plays a critical role in the decision of treatment. In this review, we describe the ophthalmological assessment including new biomarkers in clinical use. We also outline prognostic factors and current recommendations for surveillance and indications for treatment.



中文翻译:

视觉功能测试,包括光学相干断层扫描在神经纤维瘤病中的作用1。

视神经胶质瘤(OPG)是神经纤维瘤病1(NF-1)的常见并发症,可能导致视力丧失。治疗OPG的主要原因是保持视力。沿视觉通路的肿瘤位置在很大程度上决定了出现的体征和症状。临床眼科评估的重点是视神经功能,包括评估学生对光,视敏度,色觉和视野以及视神经外观的反应。一项重要的相对较新的辅助检查是光学相干断层扫描(OCT),该技术可测量视神经周围的视网膜神经纤维层和黄斑的神经节细胞层-内丛状层(GCL-IPL)的体积,两者均被证明具有很强的相关性在OPG中失去视力。视觉功能的准确评估在治疗决策中起着至关重要的作用。在这篇综述中,我们描述了眼科评估,包括临床使用中的新生物标志物。我们还概述了预后因素以及当前的监测建议和治疗适应症。

更新日期:2020-08-04
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