FUNDUS AUTOFLUORESCENCE IN EXTENSIVE MACULAR ATROPHY WITH PSEUDODRUSEN AND DIFFUSE TRICKLING GEOGRAPHIC ATROPHY.

PURPOSE: To establish whether extensive macular atrophy with pseudodrusen (EMAP) can be distinguished from the diffuse-trickling phenotype of geographic atrophy (DTGA) secondary to age-related macular degeneration on the basis of its features on blue-light autofluorescence. METHODS: The authors reviewed our prospectively maintained database to enroll patients with a diagnosis of EMAP, DTGA, and non-DTGA with a minimum follow-up of 1 year. Atrophic areas and growth rates were measured on blue-light autofluorescence images, using the Heidelberg Region Finder tool. Circularity and roundness were chosen as atrophy shape descriptors, extracted using ImageJ, and compared between disease groups. RESULTS: A total of 28 EMAP, 27 DTGA, and 30 non-DTGA eyes were included in the analysis. The median follow-up time was around 3.5 years. Extensive macular atrophy with pseudodrusen was characterized by an irregular and elongated shape (low circularity and low roundness) and associated with a fast atrophy growth rate (3.6 mm 2 /year), compared with non-DTGA. However, these parameters were not significantly different between EMAP and DTGA. CONCLUSION: Our study found that EMAP and DTGA cannot be effectively differentiated on fundus autofluorescence. In both diseases, the macular atrophic area has a major vertical axis, fringed borders, and fast progression.

RISK FACTORS OF VISION LOSS AND MULTIPLE RECURRENCES IN MYOPIC MACULAR NEOVASCULARIZATION.

PURPOSE: To investigate the factors associated with maximum visual improvement (peak vision) gain and the risk factors of peak vision loss and multiple recurrences in myopic macular neovascularization undergoing antivascular endothelial growth factor therapy. METHODS: Retrospective study of 310 eyes with active myopic macular neovascularization and median follow-up of 3.5 years. We defined peak vision gain as the maximum best-corrected visual acuity value reached under treatment and peak vision loss as best-corrected visual acuity never scoring as peak vision. We used multiple-event Prentice, Williams, and Peterson models to compute recurrences' incidence and Cox regression to identify risk factors for peak vision gain, peak vision loss, and multiple recurrences. RESULTS: Eyes with worse baseline best-corrected visual acuity {hazard ratio (HR) = 2.59 (95% confidence interval [CI]: 1.63-4.11) for 0.1 logMAR increase, P < 0.001} had higher chance to achieve peak vision. Peak vision was lost in 162 eyes (52%). Older age (HR = 1.22 [95% CI: 1.02-1.43] for 10-year increase, P = 0.02) and recurrences (HR = 1.10 [95% CI: 1.01-1.22] for event, P = 0.04) predicted nonsustained peak vision. Older age (HR = 1.13 [95% CI: 1.04-1.27] for 10-year increase, P = 0.006), larger myopic macular neovascularization (HR = 1.06 [95% CI: 1.01-1.13] for 1-mm 2 increase, P = 0.04), and juxtafoveal location (HR = 1.88 [95% CI: 1.28-2.77] vs. extrafoveal, P = 0.001) predicted multiple recurrences. CONCLUSION: Myopic macular neovascularization eyes lose vision mainly because of multiple recurrences. Patients at risk for recurrences should undergo more attentive monitoring to avoid vision loss.

HYPERREFLECTIVE BAND IN THE GANGLION CELL LAYER IN RETINITIS PIGMENTOSA.

PURPOSE: To describe a sign that takes the form of a continuous hyperreflective band within the thickness of the ganglion cell layer (GCL), thus dubbed the "hyperreflective ganglion cell layer band" (HGB), which the authors detected in a fraction of patients affected by retinitis pigmentosa (RP). METHODS: Retrospective, cross-sectional, observational study. Optical coherence tomography (OCT) images of patients with RP examined between May 2015 and June 2021 were retrospectively reviewed for the presence of HGB, epiretinal membrane (ERM), macular hole, and cystoid macular edema (CME). The ellipsoid zone (EZ) width was also measured. A subgroup of patients underwent microperimetry in the central 2°, 4°, and 10°. RESULTS: One hundred and fifty-four eyes from 77 subjects were included in the study. The HGB was present in 39 (25.3%) eyes with RP. Mean best-corrected visual acuity (BCVA) was 0.39 ± 0.05 logMAR (approximately 20/50 Snellen equivalent) and 0.18 ± 0.03 logMAR (approximately 20/32 Snellen equivalent) in eyes with and without HGB, respectively ( P < 0.001). The two groups did not differ regarding EZ width; mean 2°, 4°, and 10° retinal sensitivity; and prevalence of CME, ERM, and macular hole. The multivariable analysis showed the presence of HGB to be a predictor of poorer BCVA ( P < 0.001). CONCLUSION: HGB is an OCT finding detectable in approximately a quarter of eyes with RP and is associated with a poorer visual function. In the discussion, the authors speculate about possible morphogenetic scenarios to explain this observation.

BASELINE SATTLER LAYER-CHORIOCAPILLARIS COMPLEX THICKNESS CUTOFFS ASSOCIATED WITH AGE-RELATED MACULAR DEGENERATION PROGRESSION.

PURPOSE: To assess the relationship between choroidal overall and sublayer thickness and age-related macular degeneration (AMD) stage progression. METHODS: A prospective, observational case series was performed. Two hundred and sixty-two eyes of 262 patients with different stages of AMD were imaged by optical coherence tomography. Age-related macular degeneration stage, choroidal thickness, Sattler layer-choriocapillaris complex thickness (SLCCT), and Haller layer thickness were determined at the baseline visit, at a 1-year follow-up visit, at a 2-year follow up visit, and at a final visit (performed after a mean of 5 ± 1 year from the baseline visit). RESULTS: Baseline AMD stages were distributed as follows: early AMD (30 eyes; 12%), intermediate AMD (97 eyes; 39%), and late AMD (126 eyes; 49%). At the final follow-up, AMD stages were so distributed: early AMD (14 eyes; 6%), intermediate AMD (83 eyes; 33%), and late AMD (156 eyes; 61%). Each group showed a statistically significant decrease in choroidal thickness values over the entire follow-up ( P < 0.001), and SLCCT reduction was associated with AMD progression ( P < 0.001). Moreover, SLCCT quantitative cutoffs of <20.50 µ m and <10.5 µ m were associated with a moderate and high probability of AMD progression, respectively, and SLCCT quantitative cutoffs of <18.50 µ m and <8.50 µ m implied a moderate and high probability of macular neovascularization onset, respectively. CONCLUSION: Progressive choroidal impairment contributes to AMD progression. Among choroidal layers, a reduced SLCCT is a promising biomarker of disease worsening, and its quantitative evaluation could help to identify patients at higher risk of stage advancement.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FINDINGS IN PIGMENTED PARAVENOUS CHORIORETINAL ATROPHY.

PURPOSE: To analyze the retino-choroidal vascular characteristics of patients affected by pigmented paravenous chorio-retinal atrophy by means of optical coherence tomography (OCT) angiography. METHODS: This study was designed as an observational, cross-sectional case series. Multimodal imaging included fundus autofluorescence, structural OCT, and OCT angiography. The quantitative OCT angiography analyses included the calculation of the vessel density and choriocapillaris porosity. RESULTS: Overall, 12 patients (24 eyes) affected by pigmented paravenous chorio-retinal atrophy were recruited. Structural OCT of the areas involved by pigmented paravenous chorio-retinal atrophy as visualized on the fundus autofluorescence showed a complete ellipsoid zone and external limiting membrane absence, with thinning of ganglion cell complex, outer nuclear layer, and outer plexiform layer, but associated with the optical partial preservation of the retinal pigment epithelium. Optical coherence tomography angiography quantitative assessment of the retinal regions affected by PPRCA, as visualized by fundus autofluorescence, was characterized by normal vessel density at the level of superficial capillary plexus but significantly altered vessel density of deep capillary plexus and choriocapillaris, with higher choriocapillaris porosity. The presence of macular atrophy was significantly correlated with worse deep capillary plexus and choriocapillaris vessel density values. Furthermore, a statistically significant correlation between the fundus autofluorescence patterns and the retinal vascular status was found. CONCLUSION: Optical coherence tomography angiography quantitative analyses in pigmented paravenous chorio-retinal atrophy demonstrate a specific impairment at the level of the deep capillary plexus, which could in turn bring about a thinning of ganglion cell complex and outer nuclear layer. The alterations at the level of the choriocapillaris and the choroid, in general, could then represent a secondary effect.

PUNCTATE INNER CHOROIDOPATHY-LIKE REACTIONS IN UNRELATED RETINAL DISEASES.

PURPOSE: To report a cohort of patients with a punctate inner choroidopathy (PIC)-like reaction in concurrent, unrelated, chorioretinal disorders. METHODS: This was a retrospective observational study of patients seen at two referral centers with lesions consistent with PIC on multimodal imaging; patients with lesions resembling idiopathic multifocal choroiditis were also included. Active PIC-like lesions appeared as focal hyperreflective lesions splitting the retinal pigment epithelium/Bruch membrane (RPE/BrM) complex on optical coherence tomography. Chronic PIC-like lesions included subretinal fibrosis, multifocal punched-out chorioretinal atrophy, and curvilinear streaks. Patients' demographics, additional imaging features, and treatment responses were collected and summarized. RESULTS: Twenty-two eyes of 16 patients with a PIC-like reaction were included (75% females; median age 40 years). Underlying diagnoses included hereditary retinal conditions (10 patients, 63%) and acquired etiologies, all characterized by the RPE/BrM or outer retinal disruption. Fifteen eyes (68%) had active PIC-like lesions; seven eyes (32%) had chronic PIC-like lesions. Active PIC-like lesions regressed with time and responded to systemic steroids. Subretinal fibrosis (3 eyes, 20%), macular atrophy (3 eyes, 20%), and concomitant subretinal fibrosis and macular atrophy (5 eyes, 33%) developed on follow-up. Recurrences occurred in five eyes (23%). CONCLUSION: RPE/BrM or outer retina disruption may trigger a PIC-like reaction in susceptible patients, presumably because of the loss of immune privilege. A PIC-like reaction may influence the clinical progression and the visual prognosis of the primary chorioretinal disease.

SHORT-TERM MODIFICATIONS OF ELLIPSOID ZONE IN BEST VITELLIFORM MACULAR DYSTROPHY.

PURPOSE: To assess ellipsoid zone (EZ) alterations in Best vitelliform macular dystrophy using spectral-domain optical coherence tomography. METHODS: Prospective, observational case series. Forty-three patients (43 eyes) underwent complete ophthalmological examination at baseline and at 24 months: best-corrected visual acuity (BCVA), biomicroscopy, fundus photography, and spectral-domain optical coherence tomography were performed. Acquisition protocol included 19-line raster scan. Alterations in EZ were marked on spectral-domain optical coherence tomography, and the area was manually calculated on a near-infrared reflectance image. Three patterns were identified: A (decrease >0.25 mm2), B (±0.25 mm2), and C (increase >0.25 mm2). Primary outcome was to describe different patterns of EZ alteration. Secondary outcomes included their correlation with BCVA and the description of a central optically preserved islet. RESULTS: At baseline, altered EZ was identified in 40 eyes. Worse BCVA significantly correlated with larger EZ alterations but not with lesion extension on fundus photograph. Only "pattern-C" eyes unveiled BCVA worsening at follow-up. Optically preserved islet was detected in 16 eyes (37%), disclosing significantly better vision; its disappearance at follow-up (n = 7; 44% of 16 eyes) correlated with a decrease in BCVA. CONCLUSION: The assessment of EZ status might represent a valuable functional marker in Best vitelliform macular dystrophy because stable alterations and the maintenance of a central optically preserved islet are associated with better visual acuity.

Extrafoveal Müller cells detection in vivo in the human retina: A pilot study based on optical coherence tomography.

Müller cells (MC) represent a key element for the metabolic and functional regulation of the vertebrate retina. The aim of the present study was to test the feasibility of a new method for the in-vivo detection and quantification of extrafoveal MC in human retina. We developed a new approach to isolate and analyse extrafoveal MC in vivo, starting from structural optical coherence tomography data. Our pilot investigation was based on the optical properties of MC, which are known to not interfere with the light reaching the outer retinal structures. We reconstructed MC in the macular region of 18 healthy subjects and the quantitative analyses revealed ~42,000/9 mm2 cells detected. Furthermore, we included 2 patients affected by peripheral intraocular melanoma, with macular sparing, needing surgical enucleation. We used these two eyes to perform a qualitative comparison between our reconstructions and histological findings. Our study represents the first pilot investigation dedicated on the non-invasive isolation and quantification of MC, in-vivo, in human retina. Although we are aware that our study has several limitations, first of all related with the proper detection of foveal MC, because of the peculiar z-shape morphology, this approach may open new opportunities for the non-invasive in vivo analysis of MC, providing also potential useful perspectives in retinal diseases.

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.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FEATURES OF FOCAL CHOROIDAL EXCAVATION AND THE CHOROIDAL STROMA VARIATIONS WITH OCCURRENCE OF EXCAVATION.

PURPOSE: To describe retinal and choroidal vascular changes, and choroidal stroma variations occurring in focal choroidal excavation (FCE). METHODS: Study design was a cross-sectional case series. Consecutive patients affected by FCE and healthy controls were recruited. All patients underwent complete ophthalmologic assessment and multimodal imaging, including structural optical coherence tomography and optical coherence tomography angiography. Choroidal thickness and stromal index were calculated from structural optical coherence tomography images. Moreover, we measured vessel density values of the superficial capillary plexus, deep capillary plexus and choriocapillaris at the level of the macula. RESULTS: Twenty-two patients (28 eyes; mean age 57.2 ± 16.4) and 28 control eyes (mean age of 56.5 ± 9.8) were included. Five patients (23%) were asymptomatic, whereas 17 patients (77%) complained of visual symptoms. FCE was associated with choroidal neovascularization in 10 eyes (35%). Choroidal stromal component was lower in FCE patients than controls, whereas choroidal thickness was unremarkable. Stromal index values calculated in the region proximal to the FCE was significantly lower than the values obtained from the external region. Deep capillary plexus vessel density was lower in FCE than controls. Choriocapillaris was altered in the region surrounding the FCE, whereas it was normal in the external region. CONCLUSION: Deep capillary plexus and choriocapillaris plexus were significantly altered in FCE patients. Moreover, choroidal stroma was significantly reduced in the areas closer to FCE compared to the surrounding choroid in patients, as well as compared to healthy controls, suggesting the hypothesis of weakening of the architectural support, creating a more friable point, which can favor FCE development.

HYPERREFLECTIVE FOCI AS A PATHOGENETIC BIOMARKER IN CHOROIDEREMIA.

PURPOSE: To assess hyperreflective foci (HF) number and distribution in choroideremia (CHM) using spectral domain optical coherence tomography. METHODS: Observational, cross-sectional case series. Consecutive patients and matched controls (20 eyes each) underwent best-corrected visual acuity measurement, fundoscopy, blue-light autofluorescence (BL-FAF) and spectral domain optical coherence tomography. Hyperreflective foci were assessed on a horizontal spectral domain optical coherence tomography scan, in the 500-µm area centered on the umbo, and in the 500-µm-wide areas internal (preserved border) and external (pathologic border) to the chorioretinal atrophy of CHM patients, and in the parafovea of controls. Hyperreflective foci were subclassified as retinal or choroidal. The spared central islet was measured on BL-FAF. Primary outcome was HF quantification in CHM. Secondary outcomes included their relationships with atrophy extent. RESULTS: Choroideremia eyes disclosed a significantly higher HF number across the pathologic border and in the fovea when compared with controls; in particular, these HF were primarily located in the choroid (59-87%). Moreover, choroidal HF in the pathologic border inversely correlated with the area of the preserved central islet. CONCLUSION: Hyperreflective foci might turn out to be a potential biomarker of CHM activity or severity. In this regard, we hypothesize that HF may be related to macrophages activation or progressive retinal pigment epithelium degeneration.

Hyperreflective foci in Stargardt disease: 1-year follow-up.

PURPOSE: To describe the hyperreflective foci (HF) characteristics in eyes affected by Stargardt disease (STGD), correlating HF with the atrophy progression at 1-year follow-up. METHODS: Prospective observational case series with 1-year follow-up. Twenty-eight eyes (14 patients) affected by STGD and 28 eyes (14 age- and sex-matched healthy patients) used as control group were recruited. All patients underwent a complete ophthalmologic examination including fundus autofluorescence and spectral-domain optical coherence tomography. The primary outcome was the identification of HF specific location in STGD and their modification over a 1-year follow-up. Secondary outcome included the correlation between the number and the location of HF and atrophic changes. RESULTS: HF turned out to be more frequent in STGD patients compared with healthy controls (p < 0.001). In particular, mean number of HF in the pathological edge was significantly higher than in the healthy edge of the atrophy (p < 0.001) and in the foveal area (p < 0.001). A negative correlation was found between the total HF number in the pathological edge and the atrophic area at baseline. HF number in the outer retina of the pathological edge significantly decreased between the baseline and the final follow-up examination (p = 0.011). The enlargement of the atrophic area in eyes with more than five outer retinal HF in the pathological edge at baseline was significantly less than that found in the eyes with fewer than five HF (p = 0.010). CONCLUSIONS: HF are most common at the pathological margin of the central atrophy, with outer retina foci being more frequently found in smaller atrophic lesions.

Anti-VEGF treatment for choroidal neovascularization complicating pattern dystrophy-like deposit associated with pseudoxanthoma elasticum.

PURPOSE: To evaluate the efficacy of intravitreal anti-VEGF injections in choroidal neovascularization (CNV) related to pattern dystrophy-like deposit in pseudoxanthoma elasticum (PXE). METHODS: One-year prospective, interventional study. Nine eyes were recruited in the ophthalmology departments of San Raffaele University and University of Barcelona. Each patient underwent best corrected visual acuity (BCVA) measurement on ETDRS chart, slit-lamp biomicroscopy, fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT). The protocol included a first anti-VEGF injection, followed by monthly evaluations with re-treatments based on new funduscopic hemorrhages, fluid on OCT or leakage on FA and/or ICGA. Primary outcome measures were the mean BCVA changes. Secondary outcomes included central macular thickness (CMT) variations and the number of injections needed. RESULTS: At month 12, mean BCVA significantly improved from 20/45 to 20/35 Snellen equivalent, with 3 eyes gaining at least 3 ETDRS lines. Mean CMT decreased from 297 ± 22 to 262 ± 13 µm, after 5.5 ± 4.0 injections. No leakage was observed at the end of follow-up. CONCLUSIONS: Intravitreal anti-VEGF injections represent an effective treatment for CNV related to pattern dystrophy-like deposit in PXE, with an improvement of BCVA and CMT. Mean injection number is in line with other studies performed in CNV secondary to angioid streaks.

CAPILLARY NETWORK ALTERATIONS IN X-LINKED RETINOSCHISIS IMAGED ON OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To assess foveal and parafoveal vasculature at the superficial capillary plexus, deep capillary plexus, and choriocapillaris of patients with X-linked retinoschisis by means of optical coherence tomography angiography. METHODS: Six patients with X-linked retinoschisis (12 eyes) and seven healthy controls (14 eyes) were recruited and underwent complete ophthalmologic examination, including best-corrected visual acuity, dilated fundoscopy, and 3 × 3-mm optical coherence tomography angiography macular scans (DRI OCT Triton; Topcon Corp). After segmentation and quality review, optical coherence tomography angiography slabs were imported into ImageJ 1.50 (NIH; Bethesda) and digitally binarized. Quantification of vessel density was performed after foveal avascular zone area measurement and exclusion. Patients were additionally divided into "responders" and "nonresponders" to dorzolamide therapy. RESULTS: Foveal avascular zone area resulted markedly enlarged at the deep capillary plexus (P < 0.001), particularly in nonresponders. Moreover, patients disclosed a significant deep capillary plexus rarefaction, when compared with controls (P: 0.04); however, a subanalysis revealed that this damage was limited to the fovea (P: 0.006). Finally, the enlargement of foveal avascular zone area positively correlated with a decline in best-corrected visual acuity (P: 0.01). CONCLUSION: Prominent foveal vascular impairment is detectable in the deep capillary plexus of patients with X-linked retinoschisis. Our results correlate with functional outcomes, suggesting a possible vascular role in X-linked retinoschisis clinical manifestations.

VASCULAR ALTERATIONS REVEALED WITH OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN PATIENTS WITH CHOROIDEREMIA.

PURPOSE: Choroideremia is a rare degenerative retinal disease that causes incurable blindness. It occurs as a result of the deficiency of the X-linked CHM gene, which encodes the Rab escort protein 1 (REP1). Gene therapy has been developed to treat CHM using adeno-associated viral vectors and is currently undergoing clinical trials. Expression of the CHM gene is ubiquitous throughout the retina, and it is therefore important to identify which retinal layers are affected in the disease process. The purpose of this study was to assess in particular the choriocapillaris using optical coherence tomography angiography because this layer is difficult to see with conventional imaging techniques. METHODS: Six men with choroideremia were identified and underwent standardized optical coherence tomography angiography as part of an ethics-approved clinical study and were compared with age-matched control subjects. RESULTS: The choriocapillaris appeared normal in regions where the retinal pigment epithelium remained intact, but it was deficient elsewhere. The outer retinal vasculature showed significant changes peripherally but also some changes centrally. The inner retinal vasculature appeared unaffected by the disease process. CONCLUSION: Choroideremia is a disease in which the choriocapillaris maintains a normal structure until the loss of the overlying retinal pigment epithelium. The inner retina also appears not to be affected at the vascular level. Although this study is limited by the small number of patients eligible for inclusion in the study, the observations support the concept of targeting gene therapy to the retinal pigment epithelium and outer retina because there is no evidence of independent degeneration of the choriocapillaris.

SUBTHRESHOLD LASER TREATMENT FOR SEROUS RETINAL DETACHMENT IN DOME-SHAPED MACULA ASSOCIATED WITH PATHOLOGIC MYOPIA.

PURPOSE: To evaluate the effects of the subthreshold laser treatment as a therapeutic option in the treatment of serous retinal detachment secondary to dome-shaped macula. METHODS: Prospective pilot study with 12-month follow-up. Each patient underwent a complete ophthalmologic examination, including best-corrected visual acuity, fluorescein angiography, indocyanine green angiography, and spectral domain optical coherence tomography. Eyes with persistent serous retinal detachment lasting for a minimum of 12 months and absence of best-corrected visual acuity improvement underwent subthreshold laser treatment in a single session. The laser spots were delivered in a contiguous mode, covering the area of hyperfluorescence recognized on indocyanine green angiography. The primary outcome measure was the best-corrected visual acuity changes at the 12-month examination. Secondary outcomes included changes in central foveal thickness (CFT) and number of eyes achieving a complete serous retinal detachment resolution. RESULTS: Twelve eyes (8 patients) were included in the study. Best-corrected visual acuity changed from a baseline value of 0.8 ± 0.2 (±SD, logarithm of the minimum angle of resolution [Snellen equivalent: 20/125]) to 0.48 ± 0.1 (Snellen equivalent: 20/60, P = 0.001). Central foveal thickness decreased from 320 ± 52 µm to a final value of 266 ± 41 µm (P = 0.001). Serous retinal detachment reduced in all cases; however, just one eye (8.3%) showed a complete resolution at the 12-month examination. Fluorescein angiography and indocyanine green angiography showed that the hyperfluorescence had disappeared after subthreshold laser treatment. No adverse event was registered, including enlargement of atrophic alterations and choroidal neovascularization. CONCLUSION: Subthreshold laser treatment can lead to improvements in visual acuity and central foveal thickness in myopic eyes with serous retinal detachment secondary to dome-shaped macula. Further studies are required to confirm our preliminary results.

SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FEATURES IN DIFFERENT STAGES OF BEST VITELLIFORM MACULAR DYSTROPHY.

PURPOSE: To provide a systematic classification of findings regarding the different stages of vitelliform macular dystrophy on spectral domain optical coherence tomography (SD-OCT). METHODS: Ninety-four eyes of 47 patients were recruited in a prospective cross-sectional study. All patients underwent a complete ophthalmologic examination, including best-corrected visual acuity using Early Treatment Diabetic Retinopathy Study (ETDRS) charts, biomicroscopy, and SD-OCT. The findings assessed included vitelliform material, neurosensory detachment, status of external limiting membrane, ellipsoid zone and retinal pigment epithelium, choroidal excavation, foveal cavitation, choroidal neovascularization, vitreomacular traction, and macular hole. The primary outcome measure was the identification of SD-OCT findings in each vitelliform macular dystrophy stage. Secondary outcomes included the correlations between SD-OCT features and visual acuity changes. RESULTS: The outer retinal layers (external limiting membrane, ellipsoid zone, and retinal pigment epithelium) were found to be more commonly disrupted in Stages 2 to 4 (range: 86%-100%), whereas their absence was more typical of Stage 5 (71%-86%). Vitelliform material was found in 100% of Stages 2 and 3, 93% of Stage 4, and interestingly in 43% of Stage 5. Eyes characterized by vitelliform material showed a greater correlation with higher best-corrected visual acuity than eyes without it (0.35 logarithm of the minimum angle of resolution vs. 0.80 ± 0.36 logarithm of the minimum angle of resolution, approximately 20/45 and 20/125 Snellen equivalent, respectively) (t = 3.726, P < 0.05). Moreover, its absence was associated with a best-corrected visual acuity of 0.5 logarithm of the minimum angle of resolution or worse (approximately 20/63 Snellen equivalent; P < 0.05). Subretinal fluid was more common in Stages 3 and 4 (72.7% and 75%, respectively) than Stages 2 and 5 (P = 0.004). Eyes with subretinal fluid were significantly associated with a visual acuity of 0.2 logarithm of the minimum angle of resolution or worse (approximately 20/32 Snellen equivalent; P = 0.04). CONCLUSION: Spectral domain optical coherence tomography assessment primarily indicates an outer retinal layer disruption in Stages 2 to 4, along with the presence of vitelliform material extending into the more advanced clinical stages too. Eyes characterized by the persistence of vitelliform material show better best-corrected visual acuity. Future investigations based on a longitudinal follow-up are warranted to correlate SD-OCT modifications with functional responses to identify SD-OCT indicators for prognostic and therapeutic purposes.

HOW VITREOMACULAR INTERFACE MODIFIES THE EFFICACY OF ANTI-VEGF THERAPY FOR MYOPIC CHOROIDAL NEOVASCULARIZATION.

PURPOSE: To evaluate the efficacy of intravitreal ranibizumab in the treatment of myopic choroidal neovascularization (mCNV) complicated by vitreoretinal interface alterations. METHODS: Thirty-two patients affected by mCNV and concurrent vitreoretinal interface disorders, including macular epiretinal membrane (18 patients), lamellar macular hole (4 patients), full-thickness macular hole (1 patient), broad/focal vitreomacular traction (3 patients), broad/focal vitreomacular adhesion (4 patients), and myopic foveoschisis (2 patients), were enrolled in a prospective study. After a comprehensive ophthalmologic examination, including best-corrected visual acuity (BCVA), fluorescein angiography, and spectral-domain optical coherence tomography, each patient received a first intravitreal ranibizumab. Further re-treatments were performed in the presence of choroidal neovascularization activity (new hemorrhages, leakage on fluorescein angiography, intraretinal/subretinal fluid on spectral-domain optical coherence tomography, visual acuity loss of five letters). Main outcome measure was the change in the BCVA and in the central foveal thickness. Data were compared with the historical control group with uncomplicated mCNV. RESULTS: The median BCVA in the epiretinal membrane-myopic choroidal neovascularization subgroup showed a stabilization from the baseline value of 0.30 logarithm of minimal angle resolution (20/40) to 0.40 (20/50, P: 0.49) at the last visit (30 ± 13 months). Median BCVA significantly improved from 0.30 (20/40) to 0.10 (20/25, P: 0.0005) in the mCNV group and was better than the epiretinal membrane-myopic choroidal neovascularization subgroup (0.008). Central foveal thickness reduced significantly within both groups, with no difference between the groups at the final examination. Considering the vitreoretinal alterations with lower prevalence, BCVA stabilization was registered after a follow-up of 28.9 ± 13 months, with a median BCVA of 0.3 logarithm of minimal angle resolution (20/40) at the baseline and at the final examination. A nonstatistically significant reduction in the median central foveal thickness was registered at the final examination (P: 0.12). CONCLUSION: The data show that ranibizumab is effective in controlling mCNV activity when associated with vitreoretinal interface alterations. However, a visual recovery was observed only in patients with uncomplicated mCNV.

THE EXPANDING CLINICAL SPECTRUM OF CHOROIDAL EXCAVATION IN MACULAR DYSTROPHIES.

PURPOSE: To assess the prevalence and the clinical course of focal choroidal excavation (FCE) in patients affected by macular dystrophies. METHODS: Prospective case series. All the patients underwent a complete ophthalmologic examination, including best-corrected visual acuity and spectral domain optical coherence tomography. The presence of choroidal neovascularization (CNV) was assessed on the basis of the leakage detected on fluorescein angiography. RESULTS: A total of 162 eyes from 81 patients with macular dystrophy were included in the study. FCE was diagnosed in seven eyes (4.3% of the eyes), including four eyes with Best vitelliform dystrophy, two eyes with pattern dystrophy associated with pseudoxanthoma elasticum, and one case of Stargardt disease. In eyes with FCE and macular dystrophy, the mean best-corrected visual acuity was 0.4 ± 0.1 logarithm of the minimum angle of resolution (approximately corresponding to 20/50 Snellen equivalent) at baseline and was stable to 0.41 ± 0.1 logarithm of the minimum angle of resolution (approximately corresponding to 20/50 Snellen equivalent) at the final visit. In four of these seven eyes, FCE was associated with a subfoveal CNV. The CNV was managed with one intravitreal anti-vascular endothelial growth factor injection, achieving the complete anatomical stabilization of the CNV and recovery of the best-corrected visual acuity. CONCLUSION: Focal choroidal excavation can be infrequently encountered in patients with macular dystrophies. The presence of CNV may complicate FCE in these patients, and anti-vascular endothelial growth factor seems to be an effective treatment with no progression of FCE over time.