OBJECTIVE: To investigate whether the ganglion cell layer assessed by OCT is a reliable measure to identify and detect relapses of symptomatic OPGs in children with NF1.
METHODS: Children (3-6 years) with NF1, with and without known OPG and children with sporadic OPG (S-OPG) resident in the Stockholm area, were invited and followed in a prospective study during a three-year period. Brain magnetic resonance tomography (MRI) had been performed in children with symptoms of OPG. Outcome measures were VA in logMAR, visual field index (VFI), average thicknesses of the ganglion cell-inner plexiform layer (GC-IPL), and peripapillary retinal nerve fiber layer (pRNFL).
RESULTS: There were 25 children with MRI-verified OPG and 52 with NF1 without symptomatic OPG. Eyes from NF1 patients without symptoms of OPG showed significantly better results in all four analyzed parameters compared to eyes with NF1-associated OPG. Mean GC-IPL measurements seemed stable and reliable, significantly correlated to pRNFL (correlation coefficient (r) = 0.662, confidence interval (CI) = .507 to .773 p<0.001), VA (r = -0.661, CI = -7.45 to -.551, p<0.001) and VFI (r = 0.644, CI = .452 to .774, p<0.001). GC-IPL measurements were easy to obtain and acquired at considerably younger age than pRNFL (5.6±1.5 vs 6.8±1.3; p<0.001).
CONCLUSIONS: The mean GC-IPL thickness could distinguish well between eyes with OPG and eyes without symptomatic OPG in children with NF1. As thinning of GC-IPL assessed with OCT could indicate underlying OPG, it should be included in the screening protocol of children with questionable VA measurements and in particular in children with NF1.
目的:研究通过OCT评估的神经节细胞层是否是鉴定和检测NF1患儿症状性OPG复发的可靠指标。
方法:患有NF1的儿童(3-6岁),有或没有已知的OPG以及居住在斯德哥尔摩地区的零星OPG(S-OPG)儿童,在为期三年的前瞻性研究中被邀请和随访。对有OPG症状的儿童进行了脑磁共振断层扫描(MRI)。结果指标是logMAR中的VA,视野指数(VFI),神经节细胞内丛状层的平均厚度(GC-IPL),和乳头周围视网膜神经纤维层(pRNFL)。
结果:有25名MRI证实OPG的儿童和52名NF1无症状OPG的儿童。与具有NF1相关OPG的眼睛相比,没有OPG症状的NF1患者的眼睛在所有四个分析参数中显示出明显更好的结果。平均GC-IPL测量结果似乎稳定可靠,与pRNFL显著相关(相关系数(r)=0.662,置信区间(CI)=.507至.773p<0.001),VA(r=-0.661,CI=-7.45至-.551,p<0.001)和VFI(r=0.644,CI=.452至.774,p<0.001)。GC-IPL测量易于获得,并且比pRNFL年龄小得多(5.6±1.5vs6.8±1.3;p<0.001)。
结论:在NF1患儿中,平均GC-IPL厚度可以很好地区分患有OPG的眼睛和没有症状性OPG的眼睛。由于用OCT评估的GC-IPL变薄可能表明潜在的OPG,应将其纳入VA测量有问题的儿童,特别是NF1儿童的筛查方案.