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The aim of this study was to determine the ophthalmologic involvement in patients with hereditary transthyretin amyloidosis and its correlation with the mutations described in the literature.Cross-sectional, noninterventional study. Fifty-two eyes of 26 consecutive patients diagnosed with hereditary transthyretin amyloidosis who visited the Puerta de Hierro-Majadahonda University Hospital from September 2019 to March 2022. All patients underwent complete ophthalmologic examination and multimodal imaging. Cardiologic, neurologic, digestive, and renal examinations were also recorded.Eighteen eyes of the total (34.61%) showed amyloid-related ocular involvement, vitreous amyloid deposits being the most common ocular manifestation (18/52). Statistically significant differences were found for the presence of vitreous amyloid deposits ( P0.01), crystalline amyloid deposits ( P0.05), parenchymal amyloid deposits ( P0.01), and vascular alterations ( P0.01) when comparing affected and unaffected eyes. Moreover, affected eyes showed worse best-corrected visual acuity ( P0.01).Ocular manifestations are present in a substantial number of patients with ATTR that could potentially lead to devastating consequences to patients' best-corrected visual acuity and quality of life. Therefore, it is important to emphasize the importance of multidisciplinary management and ophthalmologic assessment, follow-up and surgical treatment when necessary. To the best of our knowledge, this represents the largest series in Spain of amyloidosis' ophthalmologic involvement.

The results of this study show that the ATN grading system has a high intraobserver and interobserver correlation, suggesting that the system is reliable and highly reproducible. This system is simple yet comprehensive, taking into account the three main components (atrophy, traction, and neovascularization) of the disease., Purpose: To validate the recently developed ATN grading system for myopic maculopathy to classify eyes with pathologic myopia. Methods: Cross-sectional study. A series of consecutive eyes diagnosed with pathologic myopia and signs of myopic maculopathy (grade ≥1 for atrophic, tractional, or neovascular components of the ATN), with a refractive error > −6.0 diopters (D), were included. All patients underwent complete ophthalmological examination including fundus photography and swept-source optical coherence tomography. Six observers graded each eye twice using the ATN system (≥15 days between assessments) based only on the aforementioned data. Results: Sixty eyes from 47 patients (61.7% female) were graded. Mean patient age was 63.2 ± 11.7 years. The mean spherical equivalent was −13.8 ± 6.5 D. Mean axial length was 28.6 ± 2.16 mm. Overall, the mean intraobserver agreement (%) for the same image was 92.0%, and the mean interobserver agreement for the second image was 77.5%. The weighted Fleiss k showed excellent correlation (k > 0.8) for the traction and neovascularization components and good correlation (0.75) for atrophy. Interobserver agreement for each of these three components was 95.2%, 98.4%, 95.0%, respectively. Conclusion: Application of the ATN resulted in high intraobserver and interobserver correlation, underscoring the reproducibility of the system.

To analyze the vascular density of the choroid in a healthy population using swept-source optical coherence tomography.A cross-sectional, noninterventional study.best-corrected visual acuity between 20/20 and 20/25, spherical equivalent between ±3 diopters, no systemic or ocular diseases, and ages ranging between 3 and 85 years. One hundred and thirty-six eyes from 136 subjects were analyzed, 86 eyes (63.2%) were from male and 50 eyes (36.8%) from female subjects. The eyes were divided into different age groups to analyze the possible age-related changes. Twelve-millimeter horizontal, fovea-centered B-scans were used. Choroidal stroma and vessel area analysis involved automated segmentation and binarization using validated algorithms.Mean age was 33.1 ± 24.5 years. Mean choroidal area was 0.5554 ± 0.1377 mm. Mean stromal area was 0.2524 ± 0.0762 mm, and mean vascular region area was 0.3029 ± 0.0893 mm. The percentage of choroidal vascularity (vascular area/total area) was 54.40 ± 8.35%. Choroid area, vascular region, and percentage of choroidal vascular density were statistically higher in the18-year-old group versus the18-year-old group (P0.001). The stromal region was not different (P = 0.46). In the same way, choroid area, vascular region, and percentage of choroidal vascular density between the 5 age groups were statistically different (P0.001), showing larger figures in the 0 to 10-year-old group, but not stromal region (P = 0.71). There were no gender-related differences.The luminal area and the percentage of vascular/total area decrease with increasing age, while the stromal area remains stable.