Purpose Patients with non-proliferative macular telangiectasia type 2 (MacTel) have ganglion cell layer (GCL) and nerve fibre layer (NFL) loss, but it is unclear whether the thinning is progressive. We quantified the change in retinal layer thickness over time in MacTel with and without diabetes. Methods In this retrospective, multicentre, comparative case series, subjects with MacTel with at least two optical coherence tomographic (OCT) scans separated by >9 months OCTs were segmented using the Iowa Reference Algorithms. Mean NFL and GCL thickness was computed across the total area of the early treatment diabetic retinopathy study grid and for the inner temporal region to determine the rate of thinning over time. Mixed effects models were fit to each layer and region to determine retinal thinning for each sublayer over time. Results 115 patients with MacTel were included; 57 patients (50%) had diabetes and 21 (18%) had a history of carbonic anhydrase inhibitor (CAI) treatment. MacTel patients with and without diabetes had similar rates of thinning. In patients without diabetes and untreated with CAIs, the temporal parafoveal NFL thinned at a rate of −0.25±0.09 µm/year (95% CI [−0.42 to –0.09]; p=0.003). The GCL in subfield 4 thinned faster in the eyes treated with CAI (−1.23±0.21 µm/year; 95% CI [−1.64 to –0.82]) than in untreated eyes (−0.19±0.16; 95% CI [−0.50, 0.11]; p<0.001), an effect also seen for the inner nuclear layer. Progressive outer retinal thinning was observed. Conclusions Patients with MacTel sustain progressive inner retinal neurodegeneration similar to those with diabetes without diabetic retinopathy. Further research is needed to understand the consequences of retinal thinning in MacTel. All data relevant to the study are included in the article or uploaded as supplementary information.

中文翻译：

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2 型非增殖性黄斑毛细血管扩张症的进行性视网膜内神经变性


目的 2 型非增殖性黄斑毛细血管扩张症 (MacTel) 患者存在神经节细胞层 (GCL) 和神经纤维层 (NFL) 损失，但尚不清楚这种变薄是否是进行性的。我们量化了患有和不患有糖尿病的 MacTel 视网膜层厚度随时间的变化。方法 在这个回顾性、多中心、比较病例系列中，使用爱荷华州参考算法对至少两次光学相干断层扫描 (OCT) 扫描（间隔 >9 个月的 OCT）的 MacTel 受试者进行分段。计算早期治疗糖尿病视网膜病变研究网格的整个区域以及内部颞区的平均 NFL 和 GCL 厚度，以确定随时间推移的变薄率。混合效应模型适合每个层和区域，以确定每个子层随时间的视网膜变薄情况。结果 纳入MacTel患者115例； 57 名患者 (50%) 患有糖尿病，21 名患者 (18%) 有碳酸酐酶抑制剂 (CAI) 治疗史。患有和不患有糖尿病的 MacTel 患者的瘦削率相似。在没有糖尿病且未接受 CAI 治疗的患者中，颞中心凹旁 NFL 以每年 -0.25±0.09 µm 的速度变薄（95% CI [-0.42 至 –0.09]；p=0.003）。在接受 CAI 治疗的眼睛中，子区域 4 中的 GCL 变薄得更快（−1.23±0.21 µm/年；95% CI [−1.64 至 –0.82]），比未治疗的眼睛（−0.19±0.16；95% CI [−0.50， 0.11]；p<0.001)，内核层也出现这种效应。观察到外层视网膜逐渐变薄。结论 MacTel 患者持续进行性视网膜内神经变性，与没有糖尿病视网膜病变的糖尿病患者相似。需要进一步的研究来了解 MacTel 视网膜变薄的后果。 与研究相关的所有数据都包含在文章中或作为补充信息上传。