Zahavi Alon, Toledano Helen, Cohen Rony, Sella Sara, Luckman Judith, Michowiz Shalom, Goldenberg-Cohen Nitza
Department of Ophthalmology, Rabin Medical Center-Beilinson Hospital, Petah Tikva, Israel.
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Front Neurol. 2018 Dec 20;9:1102. doi: 10.3389/fneur.2018.01102. eCollection 2018.
Optic pathway glioma (OPG) presents in childhood and can cause significant morbidity and visual loss. Magnetic resonance imaging (MRI) is the current imaging modality of choice for evaluation of OPG progression, but it is a relatively limited resource often requiring sedation in the pediatric age group. Additionally, OPG progression on MRI does not always correlate with clinical progression. As a result, several other modalities for evaluating OPG are being investigated, including optical coherence tomography (OCT), a readily available imaging technique in ophthalmic practice. The purpose of the present study was to examine the association between retinal nerve fiber layer (RNFL) thickness measured using OCT and optic nerve function in children with OPG with and without neurofibromatosis-1 (NF-1). A retrospective chart review was conducted to identify children diagnosed with OPG from 2001 to 2015 at a tertiary pediatric medical center. The correlation between OCT measurements and clinical visual parameters was statistically analyzed. Included were 23 children with imaging-confirmed OPG and spectral domain OCT: 10 with NF-1 (mean age at diagnosis 5.8 years) and 13 without (mean age at diagnosis 5.9 years). The glioma involved the chiasma-hypothalamus in 19 patients, optic nerve in 11, and optic tract in 7; more than one anatomic site was affected in 15. Symptoms were reported in 2 patients with NF-1 and most patients without NF-1. Visual field defects included monocular, bitemporal, nasal, and homonymous hemianopia. Initial mean RNFL was 85.4 μm in the NF-1 group and 65 μm in the non-NF-1 group. Visual acuity deteriorated in 1/10 patients and 5/13 patients, respectively. Repeated OCT showed continued RNFL thinning in 3 patients (5 eyes) in the NF-1 group and in 8 patients (11 eyes) in the non-NF-1 group, often associated with a decrease in optic nerve function. In conclusion, visual function in children with OPG is correlated with repeated OCT measurements and weakly with neuroimaging. Children without NF-1 are usually symptomatic and have a worse clinical outcome. These findings may have important implications when considering initiating, continuing or stopping chemotherapy for OPG. The application of OCT in the assessment of OPG and the correlation of the findings to clinical progression can have a significant impact on OPG patient management.
视路胶质瘤（OPG）多在儿童期发病，可导致严重的发病情况及视力丧失。磁共振成像（MRI）是目前评估OPG进展的首选影像学检查方法，但它是一种相对有限的资源，在儿科年龄组中通常需要镇静。此外，MRI上OPG的进展并不总是与临床进展相关。因此，正在研究其他几种评估OPG的方法，包括光学相干断层扫描（OCT），这是眼科实践中一种易于获得的成像技术。本研究的目的是检查在患有和未患有1型神经纤维瘤病（NF-1）的OPG儿童中，使用OCT测量的视网膜神经纤维层（RNFL）厚度与视神经功能之间的关联。进行了一项回顾性病历审查，以确定2001年至2015年在一家三级儿科医疗中心被诊断为OPG的儿童。对OCT测量值与临床视觉参数之间的相关性进行了统计分析。纳入了23例经影像学证实患有OPG并接受了光谱域OCT检查的儿童：10例患有NF-1（诊断时平均年龄5.8岁），13例未患有NF-1（诊断时平均年龄5.9岁）。胶质瘤累及视交叉-下丘脑的有19例患者，累及视神经的有11例，累及视束的有7例；15例患者有一个以上的解剖部位受累。10例患有NF-1的患者中有2例报告有症状，大多数未患有NF-1的患者有症状。视野缺损包括单眼、双颞侧、鼻侧和同向性偏盲。NF-1组初始平均RNFL为85.4μm，非NF-1组为65μm。视力分别在1/10例和5/13例患者中恶化。重复OCT检查显示NF-1组3例患者（5只眼）和非NF-1组8例患者（11只眼）的RNFL持续变薄，这通常与视神经功能下降有关。总之，OPG儿童的视觉功能与重复的OCT测量值相关，与神经影像学的相关性较弱。未患有NF-1的儿童通常有症状，临床结局较差。在考虑开始、继续或停止OPG化疗时，这些发现可能具有重要意义。OCT在OPG评估中的应用以及检查结果与临床进展的相关性可能会对OPG患者的管理产生重大影响。
