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Purpose: To compare AngioTool (AT) vascular parameters (VP) between MacTel2 eyes and normal eyes. Secondary outcome measures were to correlate VP with BCVA and to analyze VP between various grades of Simple MacTel Classification., Methods: This is a retrospective study. SD OCTA images of the superficial vascular complex (SVC) and deep capillary complex (DVC) were exported into Image J and AT. The explant area (EA), vessel area (VA), vessel percentage area (VPA), total number of junctions (TNJ), junction density (JD), total vessel length (TVL), average vessel length (AVL), total number of endpoints (TNE) and mean E lacunarity (MEL) were studied., Results: Group 1 had 120 MacTel2 eyes. Group 2 had 60 age-matched normal eyes. All VP were significantly different between the two groups except EA and TNE in both complexes. None of the VP had a correlation with BCVA. Interquadrant analysis (IQA) in SVC and DVC showed statistical significance in VPA, AVL and JD and in AVL, TNE, JD, VPA respectively. Post hoc analysis in SVC and DVC showed statistical significance in TNJ, JD, TVL and AVL between grade 1 and grade 3, and in VA, VPA, TNJ, JD, TVL and MEL between grade 0 and grade 3 respectively., Conclusion: VP were affected in MacTel2 eyes. VP did not correlate with BCVA. Occurrence of pigmentation is an important event in the progression of disease. AT may provide quantitative markers to measure disease progression., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To describe imaging features of macular telangiectasia type 2 eyes experiencing ellipsoid zone (EZ) recovery., Methods: Macular telangiectasia type 2 patients with EZ recovery were identified from the Natural History and Observational Registry study and underwent retinal imaging including optical coherence tomography and fundus photography. Eyes were graded according to the classification system by Gass and Blodi, the EZ-loss area was measured, and optical coherence tomography parameters were assessed by two independent readers. Parameters were analyzed for their presence before EZ recovery., Results: Twenty-four eyes of 21 patients (12 female, 57.12%; mean age 68 ± 8.54 years) were included in this study and followed for 21.25 months ± 12.79 months. At baseline, mean EZ-loss area was 0.036 mm 2 ± 0.028 mm 2 and 0.01 mm 2 ± 0.013 mm 2 at follow-up ( P < 0.001). A persisting external limiting membrane overlaying the EZ-loss was detected in 16 cases (66%), and hyperreflective changes in the outer retina were present in 18 cases (75%). Best-corrected visual acuity was 0.23 (20/32) ± 0.33 logMAR at baseline and 0.34 (20/40) ± 0.34 logMAR at follow-up ( P = 0.3)., Conclusion: Distinct optical coherence tomography features precede ellipsoid zone recovery in macular telangiectasia type 2 and warrant further studies investigating implications for patient care and clinical trial interpretation.

We analysed the imaging findings of macular telangiectasia (MacTel) type 2 in Korea using spectral domain optical coherence tomography (SD-OCT) and investigated their relationship with visual acuity and clinical stages. A retrospective multicentre cross-sectional study was conducted in six tertiary hospitals in Korea and included 129 patients. We analysed all the SD-OCT images encompassing the macular area. Hyporeflective cavities (77.7%) were the most frequently detected abnormalities in SD-OCT. Disruption of the external limiting membrane, ellipsoid zone, and interdigitation zone were found in 67 (40.4%), 87 (52.4%), and 94 eyes (56.6%), respectively. Four eyes (2.4%) had lamellar macular hole, and five eyes (3.0%) full-thickness macular hole. Neovascularisation, either subretinal or intraretinal, was found in 14 eyes (8.4%). Eyes with outer retinal hyperreflective band disruption had lower visual acuity than those without them. The presented characteristic clinical features of OCT in MacTel type 2 can not only aid in differentiating this disease from others but are also helpful for better judgement of the disease stage in daily clinical practice. Inner retinal hyporeflective cavities without outer retinal abnormalities on SD-OCT, although classified as severity scale 3, could be considered a relatively early stage in the disease process in terms of vision.

Purpose: To assess visual outcomes of Coats disease by classification stage., Methods: A retrospective review was conducted on consecutive patients with Coats disease, classified according to Shields classification and with available Snellen visual acuity before and after treatment., Results: There were 160 eyes with Coats disease (stage 1 (n=2) vs stage 2A (n=17) vs stage 2B (n=22) vs stage 3A1 (n=26) vs stage 3A2 (n=40) vs stage 3B (n=42) vs stage 4 (n=9) vs stage 5 (n=2)). By comparison, more advanced stage showed greater frequency of poor presenting visual acuity (<20/200) (0% vs 0% vs 50% vs 35% vs 38% vs 83% vs 100% vs 100%, p<0.001) and higher mean intraocular pressure (17 vs 15 vs 15 vs 15 vs 15 vs 15 vs 37 vs 26, p<0.001). More advanced stage was less likely managed with laser photocoagulation (100% vs 87% vs 48% vs 62% vs 74% vs 35% vs 0% vs 0%, p<0.001) and more likely with cryotherapy (0% vs 47% vs 81% vs 81% vs 82% vs 88% vs 50% vs 100%, p=0.001). More advanced stage was associated with lower frequency of visual acuity ≥20/40 (100% vs 71% vs 5% vs 42% vs 23% vs 5% vs 0% vs 0%, p<0.001) and greater frequency of visual acuity <20/200 (0% vs 12% vs 36% vs31 % vs 45% vs 88% vs 0% vs 100%, p<0.001)., Conclusion: Visual acuity in eyes with Coats disease parallels staging with more advanced stage demonstrating poorer visual acuity at presentation and final visit., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To evaluate changes in foveal and parafoveal thickness measured using optical coherence tomography across five stages of macular telangiectasia 2 (MacTel 2) and to correlate the foveal slope angle with best-corrected visual acuity (BCVA)., Methods: A multicentered, cross-sectional study of 90 patients with bilateral MacTel 2 was carried out. We reviewed medical records of patients who had undergone optical coherence tomography. Mean retinal thickness foveal and subfoveal were noted from ETDRS (Early Treatment Diabetic Retinopathy Study) mapping and later used for calculating foveal slope angle. The change in retinal thickness and foveal angle across five stages of MacTel 2 was assessed. Central foveal thickness and foveal slope angle were correlated with BCVA., Results: The foveal slope angles were calculated quadrantwise and stagewise. The mean slope angle was minimum in temporal quadrant (6.29°) followed by inferior (7.13°), superior (7.54°), and nasal (7.93°). The slope angles were 9.27°, 7.95°, 6.70°, 6.10°, and 6.31° in Stages 1, 2, 3, 4, and 5, respectively. Statistically significant correlation noted between the temporal, superior, inferior slope angles and BCVA (in logarithm of the minimum angle of resolution) with r = -0.18, -0.19, and -0.25, respectively (P < 0.05). No statistically significant correlation was noted between central retinal thickness and BCVA., Conclusion: The BCVA correlates better with slope angles than central retinal thickness. This implies that, change in both foveal and parafoveal regions accounts for vision change. Greater the foveal slope angle, the better the vision.

Purpose: To determine the prevalence, clinical characteristics and nature of subfoveal nodules in Coats' disease and the associated impact on the long-term visual outcome., Methods: Consecutive cases of Coats' disease with foveal exudation were retrospectively reviewed. The presence of a subfoveal nodule or macular fibrosis was recorded. Clinical characteristics, retinal imaging, and outcome were analyzed by comparative analysis. The histopathological description of an enucleated eye with subfoveal nodule was performed., Results: Among 40 patients presenting unilateral Stage 2B or 3A1 Coats' disease, a subfoveal nodule was detected in 21 patients (52.5%). The median follow-up was 4.7 years. Nineteen patients (47.5%) did not present a subfoveal nodule. Three patients (15.8%) without subfoveal nodule and 21 patients (100%) with subfoveal nodule progressed to a macular fibrotic scar (P < 0.0001), and the mean time of macular fibrosis onset was 11.0 ± 2.6 months. Final visual acuity was significantly worse in patients who presented a subfoveal nodule at diagnosis (P = 0.01). Of 18 cases with subfoveal nodule who underwent fluorescein angiography, retinal-retinal anastomosis and neovascularization were detected in 13 (72.2%) and 2 eyes (11.1%), respectively. Histopathological analysis of a subfoveal nodule revealed an aggregate of proteinaceous material including fibrin, spindle cells, macrophages, and pigmented cells., Conclusion: The presence of a subfoveal nodule at presentation is a predictive factor for macular fibrosis development and worse visual outcome in patients with Coats' disease. These observations suggest an updated classification introducing two subcategories within Stage 2B: without subfoveal nodule (Stage 2B1) and with subfoveal nodule (Stage 2B2).

Purpose: To describe the natural course of Type 2 idiopathic macular telangiectasia in terms of visual outcomes, causes of visual loss, and incidence of subretinal neovascular membranes (SRNV)., Methods: This retrospective observational case series consisted of chart review of 104 outpatients (203 eyes; 66 women, 38 men) who were diagnosed to have Type 2 idiopathic macular telangiectasia by clinical examination and fluorescein angiography between January 2000 and December 2008. Visual and anatomic outcomes were analyzed during a minimum follow-up of 1 year., Results: The mean age of the patients was 57 years (range, 40-74 years). Nineteen eyes (18 patients) presented with SRNV; the number increased to 29 eyes (14%; 23 patients) by the final visit (mean follow-up, 31 months). Diabetes was common (59%) though retinopathy was initially absent or mild to moderate in 99% patients. Mean logarithm of the minimum angle of resolution best-corrected visual acuity declined from 0.35 to 0.43 by the last visit (P < 0.0001) overall; final mean logarithm of the minimum angle of resolution best-corrected visual acuity was 0.61 (20/80) in the eyes with SRNV and 0.40 (20/50) in eyes without SRNV. The latter group started with better best-corrected visual acuity than SRNV group and remained better at 1-year, 2-year, and final follow-ups (P ≤ 0.0002). Overall, 30 eyes (15%; 24 patients) lost ≥ 2 Snellen lines, the main causes being SRNV and intraretinal pigment migration. Of 128 eyes (including SRNV) with best-corrected visual acuity ≥ 20/40 at baseline, 98 (77%) retained stable visual status; 74 (71%) patients retained best-corrected visual acuity of 20/40 or better at least in 1 eye., Conclusion: Over a follow-up of approximately 3 years, most eyes with Type 2 idiopathic macular telangiectasia starting with good vision were found to retain status quo; sight-threatening complications developed in a minority of eyes; most patients retained good vision at least in 1 eye.