Natural course of the vitelliform stage in best vitelliform macular dystrophy: a five-year follow-up study.

PURPOSE: The vitelliform stage is the typical phenotypic manifestation of Best vitelliform macular dystrophy (BVMD). As yet, no study has focused specifically on the clinical changes occurring in the vitelliform stage over the follow-up. METHODS: The survey takes the form of a prospective observational study with a 5-year follow-up. Twenty-one eyes of 11 patients in the vitelliform stage were examined annually. The primary outcome was the identification of the changes in the vitelliform lesion over a 5-year follow-up. Secondary outcomes included changes in structural optical coherence tomography (OCT) parameters and the correlation with the BCVA variation over the follow-up. RESULTS: Spectral domain OCT at baseline showed one subform characterized by solid vitelliform deposition, in 81% of eyes, and another subform characterized by a combination of solid deposition and subretinal fluid, in 19% of eyes. Overall, 62% of eyes showed an increase in the area of vitelliform deposition. Once the maximal area was reached, a progressive flattening of the vitelliform deposition took place, with subsequent flattening of the vitelliform lesion and formation of subretinal fluid. Hyperreflective foci (HF) increased in number as long as the vitelliform area continued to expand, with no variation in HF when the vitelliform lesion flattened or the subretinal fluid formed. CONCLUSIONS: The vitelliform stage reveals more subforms with clinical variations over the follow-up. Our data suggest that the substage before the flattening of the lesion, thus before the so-called subretinal fluid accumulates and when the visual acuity is still high, might offer the best opportunity for an optimal therapeutic approach.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY CAN CATEGORIZE DIFFERENT SUBGROUPS OF CHOROIDAL NEOVASCULARIZATION SECONDARY TO AGE-RELATED MACULAR DEGENERATION.

PURPOSE: Choroidal neovascularization (CNV) is a common complication of patients affected by age-related macular degeneration, showing a highly variable visual outcome. The main aim of the study was, at baseline, to perform a quantitative optical coherence tomography angiography assessment of CNV secondary to age-related macular degeneration and to assess posttreatment outcomes. METHODS: Seventy-eight naïve age-related macular degeneration-related CNV patients (39 men, mean age 78 ± 8 years) were recruited and underwent complete ophthalmologic evaluation and multimodal imaging. Several OCT and optical coherence tomography angiography parameters were collected, including vessel tortuosity and vessel dispersion (VDisp), measured for each segmented CNV. All patients underwent anti-vascular endothelial growth factor PRN treatment. Vessel tortuosity and VDisp values of CNVs were tested at baseline to establish a cutoff able to distinguish clinically different patient subgroups. RESULTS: Mean best-corrected visual acuity was 0.49 ± 0.57 (20/62) at baseline, improving to 0.31 ± 0.29 (20/41) at the 1-year follow-up (P < 0.01), with a mean number of 6.4 ± 1.9 injections. Our cohort included the following CNV types: occult (45 eyes; 58%), classic (14 eyes; 18%), and mixed (19 eyes; 24%). Observing optical coherence tomography angiography parameters, classic, mixed, and occult CNV revealed significantly different values of VDisp, with classic forms showing the highest values and the occult CNVs showing the lowest (P < 0.01); mixed forms displayed intermediate VDisp values. The ROC analysis revealed that a CNV vessel tortuosity cut-off of 8.40, calculated at baseline, enabled two patient subgroups differing significantly in visual outcomes after anti-vascular endothelial growth factor treatment to be distinguished. CONCLUSION: A baseline quantitative optical coherence tomography angiography-based parameter could provide information regarding both clinical and functional outcomes after anti-vascular endothelial growth factor treatment in age-related macular degeneration-related CNV.

Quantitative Optical Coherence Tomography Angiography Parameters in Type 1 Macular Neovascularization Secondary to Age-Related Macular Degeneration.

Purpose: The purpose of this paper was to study type 1 macular neovascularization (MNV) quantitative optical coherence tomography (OCT) angiography (OCTA) features by means of advanced postprocessing analyses. Methods: We recruited patients affected by naïve type 1 MNV secondary to age-related macular degeneration (AMD) and age-matched controls. All patients underwent ophthalmologic examination and multimodal imaging. They were treated with pro-re-nata anti-VEGF injections. The ensuing follow-up lasted 24 months. Quantitative OCT and OCTA parameters were statistically analyzed to obtain cutoff values able to distinguish two clinically different patient subgroups. Main outcome measures were best-corrected visual acuity (BCVA), central macular thickness, vessel density of superficial, deep and choriocapillaris plexa, vessel tortuosity (VT) of MNV, vessel dispersion of MNV, number of injections, blooding, pigment epithelium detachment, subretinal fluid, photoreceptor elongation, subretinal fibrosis, and outer retinal atrophy. Results: Ninety-one eyes (91 patients; 49 men; mean age 78 ± 7 years) and 91 control eyes were included. Mean logarithm of the minimum angle of resolution (logMAR) BCVA was 0.46 ± 0.56 at baseline, increasing up to 0.29 ± 0.30 after 2 years of treatment (P < 0.01). The mean number of intravitreal injections was 7.1 ± 2.0 during the first year and 4.5 ± 1.4 during the second year. A baseline VT cutoff of 8.40 detected two patients' subgroups differing significantly in terms of BCVA improvement after 2 years of treatment. Conclusions: OCTA-based classification of type 1 MNV, performed at baseline, provided useful information in terms of the functional outcome achievable after 24 months of anti-VEGF treatment. Translational Relevance: Quantitative OCTA-based classification of type 1 MNV, performed at baseline, provided useful information in terms of the functional outcome achievable after 24 months of anti-VEGF treatment.

Multimodal evaluation of central and peripheral alterations in Stargardt disease: a pilot study.

BACKGROUND: The clinical phenotype of Stargardt disease (STGD) can be extremely heterogeneous, with variable macular and peripheral retinal involvement. The study aim was to correlate peripheral ultrawide field (UWF) involvement with macular alterations, as assessed by structural optical coherence tomography (OCT) and OCT angiography (OCTA), in order to identify potentially different phenotypes. METHODS: The study involved patients with STGD and healthy controls. We performed a complete ophthalmologic assessment and multimodal imaging, including OCT, OCTA, fundus autofluorescence and UWF imaging. Patients with STGD were subdivided according to the peripheral involvement. OCT and OCTA quantitative parameters were analysed. The main outcome of the study was the classification of UWF subtypes and the correlation between UWF subtypes and macular involvement. RESULTS: Seventy STGD eyes (19 male; mean age 41.3±13.2 years) and 70 healthy eyes (35 male; 50%; mean age 41.2±9.8 years) were included in the analyses. Mean best-corrected visual acuity was 0.60±0.45 LogMAR for the STGD group and 0.0±0.0 LogMAR for controls (p<0.01). All clinical and imaging findings proved to be statistically worse in patients with STGD than in the control subjects (p<0.01). UWF types were distributed as follows: type I (49%), type II (34%), type III (17%). Type III patients proved to be significantly worse in terms of visual function and OCT and OCTA imaging parameters. CONCLUSIONS: The UWF autofluorescence performed in the present study suggests that there exist three different STGD phenotypes. Each phenotype is associated with variable OCT and OCTA impairment. Further studies providing a better assessment of the peripheral retinal involvement in STGD are warranted.

Choroidal Patterns in Stargardt Disease: Correlations with Visual Acuity and Disease Progression.

BACKGROUND: To identify different choroidal patterns in Stargardt disease (STGD) and to assess their clinical correlates. METHODS: 100 STGD eyes (29 males; mean age 42.6 ± 16.5 years) and 100 control eyes (29 males; mean age 43.2 ± 8.5 years) were included. Optical coherence tomography (OCT) and OCT angiography (OCTA) images were obtained. Four different choroidal patterns, quantitative OCT and OCTA parameters were assessed and statistically analyzed. The main outcome was the correlation between each choroidal pattern and anatomical and functional retinal status. Furthermore, we assessed structural and best corrected visual acuity (BCVA) changes of each STGD subgroup after one-year. RESULTS: Mean BCVA was 0.63 ± 0.44 LogMAR for STGD patients and 0.0 ± 0.0 LogMAR for controls (p < 0.01). All quantitative parameters appeared deteriorated in STGD compared to controls (p < 0.01). Choroidal patterns were distributed as follows: Pattern 1 (normal appearing choroid) (15%), Pattern 2 (reduced Sattler or Haller layer) (29%), Pattern 3 (reduced Sattler and Haller layers) (26%), Pattern 4 (Pattern 3 + choroidal caverns) (30%). More advanced patterns significantly correlated with a more severe loss of retinal structural integrity. Furthermore, only Pattern 3 and Pattern 4 showed remarkable signs of progression after one year. CONCLUSIONS: Choroidal patterns were related with retinal structural status and BCVA loss, and with different disease progression.

OCTA-Based Identification of Different Vascular Patterns in Stargardt Disease.

PURPOSE: The aim of the present study was to analyze quantitative optical coherence tomography (OCT) and OCT angiography (OCTA) parameters to identify clinically relevant cutoff values able to detect clinically different Stargardt's disease (STGD) subgroups. METHODS: Consecutive STGD patients were recruited and underwent complete ophthalmologic examination, including multimodal imaging. Several quantitative parameters were extracted both from structural OCT and OCTA images and were statistically analyzed. A post hoc analysis was performed to identify a quantitative cutoff able to distinguish two clinically different STGD subgroups. Main outcome measures were total retinal thickness, central macular thickness (CMT), retinal layers thickness, retinal and choroidal hyperreflective foci (HF) number, vessel density (VD), vessel tortuosity (VT), vessel dispersion (Vdisp), and vessel rarefaction (VR) of macular and optic nerve head plexa. RESULTS: Overall, 54 eyes of 54 STGD patients (18 males) and 54 eyes of 54 healthy age- and sex-matched controls were included in the analysis. All quantitative parameters resulted significantly worse in STGD than controls (P < 0.01). Moreover, a VT cutoff of 5 allowed to distinguish the following two categories: a functionally and anatomically better STGD group and a worse group. BCVA resulted 0.42 ± 0.28 logMAR in the best group versus 1.09 ± 0.36 logMAR in the worst (P < 0.01). Structural OCT and OCTA parameters significantly differed between the two STGD groups. CONCLUSIONS: Quantitative OCTA was able to detect different morphofunctional STGD phenotypes. TRANSLATIONAL RELEVANCE: OCTA-based classification of STGD patients detected different patients' subgroups, differing in terms of morphologic and functional features, with a potential impact on clinical and research settings.