OPHTHALMOLOGIC INVOLVEMENT IN PATIENTS WITH HEREDITARY TRANSTHYRETIN AMYLOIDOSIS.

PURPOSE: 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. METHODS: 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. RESULTS: 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 ( P < 0.01), crystalline amyloid deposits ( P < 0.05), parenchymal amyloid deposits ( P < 0.01), and vascular alterations ( P < 0.01) when comparing affected and unaffected eyes. Moreover, affected eyes showed worse best-corrected visual acuity ( P < 0.01). CONCLUSION: 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.

ASSESSMENT AND ROLE OF ARTERY-VEIN COMPLEX IN MYOPIC CHOROIDAL NEOVASCULARIZATION USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To analyze the presence of an artery-vein complex (AVC) underneath myopic choroidal neovascularization (mCNV) and to determine its relationship with neovascular activity. METHODS: Retrospective analysis of 681 eyes from 362 patients with high myopia defined by an axial length of >26 mm using optical coherence tomography (OCT) and OCT angiography imaging. Patients with clinical diagnosis of mCNV and good quality OCT angiography images were then selected. An AVC was defined by the identification of both perforating scleral vessels and dilated choroidal veins under or in contact with the mCNV in the same case. Swept source OCT (SS-OCT) and SS-OCT angiography images (TRITON; Topcon Corporation, Tokyo, Japan) were reviewed to detect AVC in the mCNV area. RESULTS: Fifty eyes of 49 highly myopic patients with mCNV were analyzed. Eyes with AVC were statistically older (69.95 ± 13.53 vs. 60.83 ± 10.47 years old; P < 0.01), needed less intravitreal injections/year along the follow-up period (0.80 ± 0.62 vs. 1.92 ± 0.17; P < 0.01), and showed less relapses/year (0.58 ± 0.75 vs. 0.46 ± 0.42; P < 0.05) when compared with eyes without AVC. Moreover, eyes with AVC were less likely to relapse during the first year from mCNV activation (n = 5/14 vs. n = 14/16; P < 0.01; P < 0.01). No significant differences were found regarding either axial length (30.55 ± 2.31 vs. 29.65 ± 2.24, P > 0.05) or best-corrected visual acuity (0.4 ± 0.5 vs. 0.4 ± 0.5 Logarithm of the Minimum Angle of Resolution (logMAR), P > 0.05) between groups. CONCLUSION: AVC complex has an influence over myopic choroidal neovascularization activity resulting in less aggressive neovascular lesions than those with perforating scleral vessels only.

Atrophic, Tractional, and Neovascular Grading System in a Dome-Shaped Macula and Ridge-Shaped Macula Highly Myopic Cohort.

INTRODUCTION: The aim of this study was to analyze the atrophic, tractional, and neovascular (ATN) components grading in highly myopic patients with dome-shaped macula (DSM) and ridge-shaped macula (RSM). METHODS: This was a cross-sectional, noninterventional study. 57 eyes of 38 different patients were included. They were classified as DSM or RSM based on the number of radial scans that showed an inward protrusion ≥50 µm in the swept-source optical coherence tomography (SS-OCT) (12 = DSM; <12 = RSM). All patients underwent a complete ophthalmological examination in addition to SS-OCT. They were graded using the ATN system for myopic maculopathy by 2 masked retina specialists that assessed the atrophic (A), tractional (T), and neovascular (N) components in order to analyze the differences between the groups. As complementary measurements, age, axial length, and best-corrected visual acuity were collected. Height and orientation of the macular bulge and the presence of Bruch's membrane defects, scleral perforating vessels, and staphyloma were recorded. RESULTS: Out of total 57 eyes, 13 eyes (22.8%) were classified as DSM. Regarding the atrophic component (A), there were statistically significant differences between groups, with DSM group showing a greater stage of atrophy (predominantly stage A3 in 69.2% of the sample) compared to the RSM group (predominantly stage A2 in 61.3% of the sample) (p < 0.05). For the T and N components, there were no significant differences between groups. The presence of Bruch's membrane defects was more frequently seen in DSM (p < 0.05). CONCLUSIONS: DSM group showed more Bruch's membrane defects and a greater stage of the atrophy component, based on the ATN grading system, compared with RSM group. As Bruch's membrane may have biomechanical properties in terms of strength, the defects found around the macula, added to the major atrophic component, may be a cause of a local relaxation that induce a central bulge forming the dome.

Pathologic myopia and severe pathologic myopia: correlation with axial length.

PURPOSE: This study had three aims: (1) correlate axial length (AL), age and best-corrected visual acuity in high myopic patients scored on the ATN grading system; (2) determine AL cut-off values to distinguish between pathologic myopia (PM) and severe PM; and (3) identify clinical differences between PM and severe PM. METHODS: This is a cross-sectional, non-interventional study. All patients underwent complete ophthalmologic examination, ATN grading and multimodal imaging (colour fundus photography, swept-source OCT, fundus autofluorescence, OCT angiography and fluorescein angiography). RESULTS: Six hundred forty-four eyes from 345 high myopic patients were included. The eyes were graded on the ATN system and classified as PM (≥ A2) or severe PM (≥ A3, ≥ T3 and/or N2). Significant between-group (PM vs. severe PM) differences (p < 0.05) were observed on the individual ATN components (atrophic [A], tractional [T] and neovascular [N]), age, BCVA and AL. AL was also linearly correlated with the A, T and N components (r = 0.53, p < 0.01; r = 0.24, p < 0.01; r = 0.20, p < 0.01; respectively). ROC curve analysis showed the optimal AL cut-off value to distinguish between PM at 28 mm (AUC ROC curve: 0.813, specificity: 75%, sensitivity: 75%) and severe PM at 29.50 mm (AUC ROC curve: 0.760, specificity: 75%, sensitivity: 70%). CONCLUSION: AL is the main variable associated with myopic maculopathy. Due to the clinical differences found between PM and severe PM, there is need to create an objective cut-off point to distinguish these two different entities being the optimal cut-off points for AL 28 mm and 29.5 mm, respectively. These objective AL cut-off values should be taken into account for determining a correct follow-up, ophthalmic management and treatment.

RELATIONSHIP BETWEEN MYOPIC CHOROIDAL NEOVASCULARIZATION ACTIVITY AND PERFORATING SCLERAL VESSELS IN HIGH MYOPIA.

PURPOSE: To study perforating scleral vessels (PSVs) in patients with high myopia using swept-source optical coherence tomography and to determine their relationship with myopic choroidal neovascularization (mCNV) and its activity. METHODS: Retrospective analysis of patients with high myopia (≥-6 D or ≥26 mm of axial length) using multimodal imaging. The presence of PSVs and mCNV was assessed using swept-source optical coherence tomography images (TRITON; Topcon Corporation, Japan). RESULTS: Five hundred sixty-four eyes from 297 highly myopic patients were studied. One hundred fifty-five eyes (27.5%) showed signs of mCNV while PSVs were found in 500 eyes (88.6%). Perforating scleral vessels were found in 93.5% (145/155) of eyes with mCNV, and they were under or in contact with the mCNV in 80.6% (117/145). The mean number of intravitreal injections received by patients with mCNV was 4.06 ± 4.17 along 66.9 ± 4.1 months of follow-up. The number of injections per year was 1.32 ± 1.56, the mean number of relapses was 1.11 ± 1.83, and the mean number of relapses per year was 0.25 ± 0.41. CONCLUSION: Perforating scleral vessels are more common among highly myopic patients suffering from neovascular complications. Myopic CNV complexes that are coincident with PSVs on optical coherence tomography show higher rates of activity, needing more injections to control them and being more prone to relapses.

Evolution of Macular Bruch Membrane Defects of Patchy Chorioretinal Atrophy in Pathologic Myopia Based on a Recent Classification System.

PURPOSE: The purpose of this study was to analyze the progression of macular Bruch membrane defects (BMD) in highly myopic patients with patchy atrophy (PA); and study its correlation with the enlargement of PA and ATN grading. Setting/Venue: Puerta de Hierro-Majadahonda University Hospital, Madrid, Spain. PATIENTS AND METHODS: This was a cross-sectional, noninterventional study. A series of 451 highly myopic eyes with spherical equivalent > -6.0 D and/or >26 mm of axial length (AL) were included. All patients underwent a complete ophthalmological examination and swept-source optical coherence tomography (SS-OCT), and were graded using the ATN system by 2 masked retina experts that assessed the atrophic (A), tractional (T), and neovascular (N) components. SS-OCT b-scans were employed to study PA and macular BMD at baseline and at a 1-year follow-up, in patients with good foveal fixation. RESULTS: Out of total 451 eyes, 126 eyes (27.9%) had PA (53 patients; 75.4% women). Mean T and N in eyes with PA were 1.1 ± 1.3 and 0.08 ± 0.2, respectively. Sixty-eight of them had >1-year follow-up with a good foveal fixation and enough image quality. From them, BMD were found in 44 eyes (64.7%) at baseline and increased to 59 eyes (86.7%) at a 1-year follow-up. The mean great linear dimension of PA and macular BMD increased with a median of 384.5 ± 462.5 µm (IR 68.0-660.2) and 265.6 ± 418.1 µm (IR 0-331.7), respectively. At 1-year, PA and BMD sizes increase, and were statistically significant (p < 0.001). There was a positive correlation between the growth of macular BMD and the growth of PA (r = 0.490, p < 0.00). T grading correlated significantly with PA growth (p < 0.05). CONCLUSIONS: Macular BMD increase its prevalence and its size over time in highly myopic patients with PA. There is a positive correlation between BMD and PA area growth. New studies with a larger sample size, longer follow-up, and AL elongation correlation are necessary to corroborate our findings.

Influence of posterior staphyloma in myopic maculopathy and visual prognosis.

BACKGROUND/OBJECTIVES: Posterior staphyloma is a hallmark of high myopia and its presence associates to greater degrees of myopic maculopathy. Nonetheless, its development, repercussion on visual function and relationship with maculopathy components, is still unclear. The objective was to analyze the impact of posterior staphyloma on the incidence and severity of myopic maculopathy and its repercussion on visual prognosis. SUBJECTS/METHODS: Cross-sectional study conducted on 473 consecutive eyes of 259 highly myopic patients examined at Puerta de Hierro-Majadahonda University Hospital (Madrid, Spain). All patients underwent complete ophthalmologic examination including best corrected visual acuity (BCVA), axial length (AL), myopic maculopathy classification according to ATN system (atrophic/traction/neovascularization), determined the presence of posterior staphyloma, pathologic myopia (PM) and severe PM. Multimodal imaging were performed including fundus photography, optical coherence tomography (OCT), OCT-angiography, fundus autofluorescence and/ or fluorescein angiography. RESULTS: Out of the total, 70.65% were female patients (n = 173/259), mean BCVA was 0.41 ± 0.54 logMAR units and mean AL was 29.3 ± 2.6 mm (26-37.6). Posterior staphyloma was present in 69.4% of eyes. Eyes with posterior staphyloma compared to non-staphyloma were older (p < 0.05), had greater AL (p < 0.01), worse BCVA (p < 0.01) and higher stage in ATN components (p < 0.01). Moreover, compound subgroup showed worse BCVA (p < 0.01) and greater stage in each of the ATN components (p < 0.01). Staphylomas with macular involvement presented worse BCVA (p < 0.01), higher AL (p < 0.01), and greater ATN (p < 0.05). The risk of posterior staphyloma presence in eyes with PM and severe PM eyes was 89.8% and 96.7%, respectively. Posterior staphyloma was the best predictor for BCVA in myopic patients (p < 0.01). CONCLUSIONS: Posterior staphyloma's presence determines high risk of myopic maculopathy and therefore worse visual prognosis, especially those with macular involvement. Posterior staphyloma represented the best predictor for BCVA in highly myopic patients.

Anterior Scleral Thickness and Anterior Segment Biometrics Measured with Swept Source Ocular Coherence Tomography in High Myopic Eyes with and without Glaucoma: A Comparative Study.

Background: To assess the anterior scleral thickness (AST), Schlemm's canal diameter (SCD), trabecular meshwork diameter (TMD) and conjunctiva tenon capsule thickness (CTT) in high myopic (HM) subjects and HM subjects with glaucoma (HMG) compared to control eyes. Methods: One hundred and twenty eyes were included, and AST at 0, 1, 2 and 3 mm from the scleral spur, SCD, TMD and CTT were measured. Results: Mean age was 64.2 ± 11.0 years, and the temporal SCD and temporal TMD were significantly longer in the HMG subjects compared to the controls (380.0 ± 62 µm vs. 316.7 ± 72 µm, p = 0.001) and (637.6 ± 113 µm vs. 512.1 ± 97 µm, p = 0.000), respectively. There were no significant differences between the HM and HMG subjects in SCD and TMD (all p > 0.025). Compared to the HM subjects, the temporal AST0 (432.5 ± 79 µm vs. 532.8 ± 99 µm, p = 0.000), temporal AST1 (383.9 ± 64 µm vs. 460.5 ± 80 µm, p = 0.000), temporal AST2 (404.0 ± 68 µm vs. 464.0 ± 88 µm, p = 0.006) and temporal AST3 (403.0 ± 80 µm vs. 458.1 ± 91 µm, p = 0.014) were significantly thinner in the HMG group. No differences were found between the CTT in the three groups (all p > 0.025). Conclusions: Our data indicate a thinner AST in HMG subjects and no differences in SCD and TMD between HM and HMG subjects.

Posterior Staphyloma as Determining Factor for Myopic Maculopathy.

PURPOSE: The aim of this study was to determine the influence of posterior staphyloma (PS) on the development of myopic maculopathy. DESIGN: Cross-sectional study. METHODS: A total of 467 highly myopic eyes (axial length [AL] ≥26 mm) of 246 patients were included. Patients underwent a complete ophthalmological examination, including multimodal imaging. Presence of PS was defined as the main variable analyzed between groups (PS vs non-PS): age, AL, best-corrected visual acuity (BCVA), atrophy/traction/neovascularization (ATN) components, and presence of severe pathologic myopia (PM). Two different cohorts were studied (age-matched and AL-matched) comparing PS vs non-PS eyes. RESULTS: In all, 325 eyes (69.59%) presented with PS. Eyes without PS were younger and had lower AL, ATN components, and prevalence of severe PM than those with PS (P < .001). Moreover, non-PS eyes had better BCVA (P < .001). Analyzing the age-matched cohort (P = .96); mean AL, A, and T components, and severe PM were significantly higher in the PS group (P < .001), as well as the N component (P < .005), showing worse BCVA (P < .001). Regarding the AL-matched cohort (P = .93), the PS group showed worse BCVA (P < .01), older age (P < .001), A (P < .001), and T components (P < .01), and severe PM (P < .01). The risk of PS increased by 10% per year of age (odds ratio = 1.109, P < .001) and by 132% per each millimeter of growth of AL (odds ratio = 2.318, P < .001). CONCLUSIONS: Posterior staphyloma is associated with myopic maculopathy, worse visual acuity, and higher prevalence of severe PM. AL and age, in this order, constitute the main factors associated with the onset of PS.

Analysis of Bilaterality and Symmetry of Posterior Staphyloma in High Myopia.

The purpose of this study was to examine bilaterality and symmetry of posterior staphyloma (PS) in high myopic eyes. Methods: This cross-sectional and non-interventional study assessed 473 high myopic eyes [axial length (AL) ≥ 26 mm] of 259 patients. Patients underwent an ophthalmological examination including multimodal-imaging and myopic maculopathy grading according to Atrophic/Tractional/Neovascular (ATN) system, presence and subtype of PS, and severe pathologic myopia (PM). Bilaterality of PS and subtype's symmetry between eyes of the same patient was assessed. Four groups were analyzed: (1) bilateral vs. unilateral PS's eyes. Within bilateral group, symmetric vs. asymmetric subtypes according to (2) Curtin's classification, (3) Ohno-Matsui's classification, and (4) primary/compound subtypes. Results: Out of the total, 334 myopic eyes of 167 patients were included. The 92.8% (n = 310/334) of the eyes presented PS and was bilateral in 85.6% (n = 143/167) of the patients. Bilateral eyes showed significantly (p < 0.01) greater AL, severe PM, A and N components vs. unilateral PS. AL-difference between both eyes was greater in unilateral PS (p < 0.01). Among bilateral PS, the subtype was symmetric in 79 (55.2%), 84 (58.7%), and 115 (80.4%) patients according to Curtin's classification, Ohno-Matsui's classification, and primary/compound; respectively. The asymmetric group presented worse best-corrected visual acuity (p < 0.01), higher AL (p < 0.01), incidence of PM, and severe PM (p < 0.05). Conclusions: PS was bilateral in most of the patients without clinical differences between both eyes, being symmetrical in more than half of bilateral cases. Patients with bilateral PS showed higher myopic maculopathy, AL, and incidence of severe PM than unilateral PS.

Dome-Shaped Macula versus Ridge-Shaped Macula Eyes in High Myopia Based on the 12-line Radial Optical Coherence Tomography Scan Pattern. Differences in Clinical Features.

PURPOSE: To study clinical features in patients with ridge-shaped macula (RSM) compared with those with dome-shaped macula (DSM) having been previously classified by the number of swept-source optical coherence tomography (SS-OCT) radial scans affected. METHODS: Retrospective observational study including 49 highly myopic eyes from 31 patients who underwent SS-OCT. DSM eyes were defined as those that showed a complete round inward convexity in all their axes, presenting an inward convexity ≥50 µm in the 12-line radial OCT scans. Eyes that did not meet this criterion and had at least one flat radial scan were grouped into the RSM group, defined as a macular inward convexity in some meridians across the fovea, whereas the opposite perpendicularly oriented meridians were flat. Age, spherical equivalent, axial length (AL), and best-corrected visual acuity (BCVA) were collected. Height of the bulge, scleral and choroidal thicknesses, Bruch´s membrane defects, and presence of perforating scleral vessels were recorded. RESULTS: Thirty-seven (75.5%) eyes were classified into the RSM group and 12 (24.5%) into the DSM group. Twenty-six (53.0%) eyes showed macular elevation only in the horizontal direction. Mean AL showed statistically significant differences (28.8 ± 2.7 vs. 30.5 ± 1.5 mm in the RMS vs. DSM group, respectively) and the presence of Bruch´s membrane defects was more frequently seen in DSM (p < 0.001). Mean age, spherical equivalent, BCVA, height of the inward convexity, retinal foveal thickness, foveal scleral thickness, subfoveal choroidal thickness, and the presence of perforating scleral vessels did not show significant differences between groups. CONCLUSION: This study shows the reliability of using the 12 equal radial OCT scans as an objective method to define and differentiate DSM versus RSM. Patients with RSM showed differences in AL compared with those with DSM, being longer in DSM, and regarding the presence of Bruch´s membrane defects, being more common in DSM. This may contribute to identifying those patients that, in daily clinical practice, have a higher risk of developing complications due to their myopia.