Amram Alec L, Whitmore S Scott, Wang Cheryl, Clavell Christine, Lyons Lance J, Rusakevich Alexander M, Han Ian, Folk James, Boldt H Culver, Stone Edwin M, Russell Stephen R, Lee Kyungmoo, Abramoff Michael, Wykoff Charles, Sohn Elliott H
Department of Ophthalmology & Visual Sciences, University of Iowa Health Care, Iowa City, Iowa, USA.
Retina Consultants of Houston, Houston, Texas, USA.
Br J Ophthalmol. 2025 Feb 24;109(3):401-407. doi: 10.1136/bjo-2023-325115.
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.
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.
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.
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.
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）变薄，但尚不清楚这种变薄是否会进展。我们对患有和未患有糖尿病的MacTel患者视网膜层厚度随时间的变化进行了量化。
在这项回顾性、多中心、比较性病例系列研究中，对具有至少两次间隔超过9个月的光学相干断层扫描（OCT）的MacTel患者，使用爱荷华参考算法对OCT图像进行分层。计算早期治疗糖尿病性视网膜病变研究网格总面积以及颞侧内部区域的平均NFL和GCL厚度，以确定随时间的变薄速率。对每一层和区域拟合混合效应模型，以确定每个子层随时间的视网膜变薄情况。
纳入115例MacTel患者；57例（50%）患有糖尿病，21例（18%）有碳酸酐酶抑制剂（CAI）治疗史。患有和未患有糖尿病的MacTel患者变薄速率相似。在未患有糖尿病且未接受CAI治疗的患者中，颞侧旁黄斑NFL以-0.25±0.09 µm/年的速率变薄（95%置信区间[-0.42至-0.09]；p = 0.003）。在接受CAI治疗的眼中，4区的GCL变薄速度更快（-1.23±0.21 µm/年；95%置信区间[-1.64至-0.82]），高于未治疗的眼（-0.19±0.16；95%置信区间[-0.50, 0.11]；p < 0.001），内核层也观察到这种效应。观察到视网膜外层逐渐变薄。
MacTel患者存在与无糖尿病视网膜病变的糖尿病患者相似的进行性视网膜内层神经退行性变。需要进一步研究以了解MacTel患者视网膜变薄的后果。
