Assessment of retinal arteriolar tortuosity in patients with COL4A1 or COL4A2 mutations.

PURPOSE: Qualitative and quantitative analyzes of retinal arteriolar tortuosity (RAT) in patients with COL4A1 and COL4A2 mutations to identify a tortuosity index (TI) threshold for detecting increased RAT. METHODS: Fifty-two eyes of 28 patients were included. Group 1 included eyes with a normal arteriolar pattern (n=19, 37%), group 2 included eyes with moderately increased arteriolar tortuosity (n=13, 25%), and group 3 included eyes with typical abnormal arteriolar tortuosity (n=20, 38%). The TI was measured by calculating the arc-to-chord ratio of arterioles and venules in the posterior pole. RESULTS: The mean arteriolar TI was significantly higher in all groups with a COL4A1/A2 mutation compared to controls: 1.19 ± 0.03, 1.24 ±0.05, and 1.57 ± 0.23 in groups 1, 2 and 3, respectively, vs. 1.12 ± 0.01 (all p <0.0001). The TI threshold was 1.13, with a sensitivity of 98.1% and a specificity of 100%. The area under the curve was 0.995. CONCLUSION: Measuring the arteriolar TI allowed diagnosing increased RAT in all eyes with a COL4A1/A2 mutation, whereas the subjective assessment suspected or detected it in only 62% of eyes. In adult patients with cerebral microangiopathy, detecting increased RAT, even when mild, directs the diagnosis towards COL4A1/A2-related cerebroretinal angiopathy.

Multilayer Retinal Correspondence of the Structural and Vascular Anomalies in Eyes With Early Macular Telangiectasia Type 2.

Purpose: To assess the correspondence between interdigitation zone (IZ) reflectivity, ellipsoid zone (EZ) loss, inner retinal layer reflectivity, patterns of capillary dilation, and telangiectasia in eyes with early macular telangiectasia type 2 (MacTel). Patients and Methods: Twenty-eight eyes of 22 patients with grade 0-2 MacTel (according to the MacTel project classification) and 28 healthy control eyes were included in this study. Multimodal imaging, including optical coherence tomography (OCT) angiography, adaptive optics flood illumination ophthalmoscopy (AO-FIO) and blue light reflectance (BLR), was performed. The EZ, IZ, and outer plexiform layer (OPL) were analyzed on the structural OCT C-scans. The vascular density (VD) was measured on the binarized and skeletonized angiograms of the superficial vascular plexus and deep capillary complex. The vascular diameter index (VDI) was calculated by dividing the binarized VD by the skeletonized VD. Results: On AO-FIO, cone density in the MacTel zone was significantly lower in MacTel eyes than in controls, even in areas located outside the EZ loss (P < 0.001). A distinctive pattern of IZ reflectivity attenuation extended beyond the area of EZ attenuation. The shape and size of a strong OPL hyper-reflectivity corresponded to the MacTel white area (MacTel zone) seen on BLR. Capillary dilation and rarefaction were colocalized with this area, extending beyond visible telangiectasia. The VDI was higher in MacTel eyes than in controls (P < 0.001). Conclusions: These findings suggest that in early MacTel eyes, photoreceptor signal alteration, OPL hyper-reflectivity, and capillary dilation, potentially associated with Müller cell dysfunction, precede the EZ loss.

Internal Limiting Membrane Peeling for Large Macular Holes Induces Only Structural Remodeling without Functional Impairment Over 12 Years.

PURPOSE: To evaluate the very long-term functional and structural outcomes of internal limiting membrane (ILM) peeling for full-thickness macular holes (FTMH). DESIGN: Observational case series nested within a multicenter, randomized, controlled clinical trial (RCT) (ClinicalTrials.gov: NCT00190190). SUBJECTS: Patients who underwent vitrectomy with or without ILM peeling for an idiopathic large FTMH in a tertiary ophthalmology center, with a minimum follow-up of 10 years after surgery. METHODS: Review of charts, spectral-domain OCT (SD-OCT) scans, OCT angiography (OCTA) scans, and microperimetry of patients originally enrolled in the RCT. MAIN OUTCOME MEASURES: Primary outcome was functional assessment in both groups (ILM peeling or not) including the retinal sensitivity (RS), distance and near best-corrected visual acuity (BCVA), and number of eyes achieving ≥0.3 logarithm of the minimum angle of resolution >10 years after surgery. Secondary outcomes were structural assessment in the entire 3 × 3-mm and 6 × 6-mm areas, and regionally in the different areas of the ETDRS grid: OCT and OCTA biomarkers in both groups and fellow eyes. RESULTS: Thirteen eyes of 13 patients with a mean follow-up of 12 ± 0.73 years were included. The mean RS and BCVA, or visual improvement did not differ between ILM peeling (n = 8) and no peeling (n = 5) (all P > 0.05). The dissociated optic nerve fiber layers on en face OCT were only observed in eyes with ILM peeling, predominantly in temporal parafoveal (20%) and perifoveal (19%) rings. The mean total retinal thickness and inner retinal thickness in the parafoveal ring were significantly lower in peeled eyes (309 ± 11 µm and 94 ± 9 µm respectively) versus nonpeeled eyes (330 ± 21 µm and 108 ± 11 µm respectively; P = 0.037 and P = 0.040), without significant difference in ganglion cell or retinal nerve fiber layers. Accordingly, the mean superficial capillary plexus density in the parafoveal ring was significantly lower in eyes with peeling versus without (39.65 ± 3.76 % versus 47.22 ± 4.00; P = 0.005). The mean foveal avascular zone area was smaller in eyes with peeling versus without (0.24 ± 0.05 mm2 vs. 0.42 ± 0.13 mm2, respectively, P = 0.005). CONCLUSIONS: Despite persistent structural changes especially in the parafoveal ring, ILM peeling for idiopathic large FTMH did not seem to impact long-term RS or BCVA over 12 years. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Retinal and Vitreous Changes Associated with Spontaneous Improvement in Myopic Macular Schisis.

PURPOSE: To describe the retinal and vitreous changes in eyes showing myopic macular schisis (MMS) improvement when vitrectomy was not performed and identify triggering factors. DESIGN: Retrospective observational study. SUBJECTS: Patients with nonoperated MMS. METHODS: The records of patients with MMS who were followed without performing surgery for >6 months were retrospectively reviewed, and the eyes showing an anatomic improvement were included. Myopic macular schisis evolution was analyzed quantitatively (central foveal thickness [CFT], parafoveal thickness, maximum height) and qualitatively (presence/absence of foveal detachment, lamellar hole, epiretinal membrane, choroidal neovascularization, inner and outer retinoschisis, vitreous status) at baseline and at the final visit. An anatomic improvement was defined as a decrease in CFT by ≥50 µm. MAIN OUTCOME MEASURES: The rate of anatomic improvement of MMS without performing vitrectomy and the morphological changes observed in these cases. RESULTS: In a cohort of 74 nonoperated eyes with MMS, MMS improved in 14 eyes (19%) after a mean follow-up of 55 ± 38 months (range, 8-138). In these improved cases, the mean decrease in CFT was 153 ± 166 µm (range, 24-635; P = 0.005) and a complete resolution of MMS was observed in 9 eyes (64%). In 9 eyes (64%), the improvement was associated with visible vitreous changes in the macular area on the OCT scans. The mean visual acuity, which was already good at baseline (20/50, 0.4 ± 0.2 logarithm of the minimum angle of resolution), increased at the last visit (20/40, 0.3 ± 0.3 logarithm of the minimum angle of resolution) but without reaching significance. CONCLUSIONS: This long-term follow-up analysis showed that almost 20% of MMS in eyes without indication for surgery could improve over time. In most cases, the improvement was associated with an apparent resolution of vitreous tensions. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

FROM OUTER RETINAL NEOVACULARIZATION TO EXUDATIVE SUBRETINAL NEOVASCULARIZATION IN MACULAR TELANGIECTASIA TYPE 2.

PURPOSE: To describe the progression from outer retinal neovascularization (ORNV) to exudative subretinal new vessels (SRNVs) in idiopathic macular telangiectasia type 2. METHODS: A total of 135 patients (270 eyes) imaged with optical coherence tomography angiography were included. MAIN OUTCOME MEASURES: Ellipsoid zone loss, outer retinal hyperreflectivity, ORNV, and SRNVs. Outer retinal neovascularization was defined as a flow signal passing through the outer plexiform layer, with or without vertical linear outer retinal hyperreflectivity on the optical coherence tomography B-scan. Subretinal new vessels were defined as an abnormal capillary network with a peripheral anastomotic arcade seen on en face optical coherence tomography angiography and a convex hyperreflectivity at the retinal pigment epithelium. RESULTS: Subretinal new vessels were observed in 38/270 eyes (14%). Subretinal new vessels were at a fibrotic stage in 24/38 eyes and at an exudative stage in 6/38 eyes, and a progression from ORNV to SRNVs was documented in 8/38 eyes. All cases showed an ellipsoid zone loss. In seven eyes (2.5%), SRNVs were also associated with subepithelial neovascularization. No retinochoroidal anastomosis was detected. The visual acuity dropped when SRNVs were present. CONCLUSION: In this case series, SRNVs were found in 14% of eyes. In all cases, they were associated with an ellipsoid zone loss and with outer retinal hyperreflectivity. A progression from ORNV to SRNVs was observed.

Hemorrhagic unilateral retinopathy: a report of multimodal imaging in three cases.

PURPOSE: To report three cases of hemorrhagic unilateral retinopathy, diagnosed by multimodal imaging. METHODS: Case report of 3 patients, 2 women and one man, aged 51, 74, and 52, respectively. RESULTS: Symptoms were acute floaters, blurred vision, or central scotoma, unilateral in all cases. The best-corrected visual acuity was 20/20 in the affected eye in 2 patients with a paracentral scotoma, and 20/160 in the third patient. Funduscopic examination revealed multiple unilateral posterior hemorrhages located in the Henle fiber layer in the macula and beneath the internal limiting membrane around the optic disc on spectral-domain optical coherence tomography (SD-OCT). Fluorescein angiography and indocyanine green angiography (ICGA) did not show any vascular abnormalities. SD-OCT angiography (SD-OCT-A) did not show any capillary drop-out or choroidal abnormalities. In all patients, the visual symptoms completely disappeared within a few weeks, with spontaneous regression of the hemorrhages. CONCLUSION: Hemorrhagic unilateral retinopathy is a rarely reported and poorly understood disorder. ICGA and SD-OCT-A did not allow better understanding the condition. No etiology has been associated with this entity so far. The spontaneous resolution of the present cases confirmed the favorable visual prognosis of the condition.

Value and Significance of Hypofluorescent Lesions Seen on Late-Phase Indocyanine Green Angiography.

Background and Purpose: The hypofluorescence of fundus lesions observed during the late phase of indocyanine green angiography (ICGA) in various diseases has often been overlooked or misinterpreted. This article explores the significance of fundus lesions that are initially isofluorescent during the early phase of ICGA but become hypofluorescent later in the examination. Findings: Pathologies such as multiple evanescent white spot syndrome, acute posterior placoid syphilitic chorioretinitis, chronic central serous chorioretinopathy, choroidal hemangioma, and some fundus with drusen, present this phenomenon of late hypofluorescence. Interpretation: The interpretation of ICGA images and the role of indocyanine green (ICG) uptake by the retinal pigment epithelium (RPE) in late fundus fluorescence is debated. Experimental evidence suggests that ICG accumulates progressively in the RPE after intravenous injection of the dye or after direct contact in vitro, making it a potential marker of RPE activity. Although the exact mechanisms of ICG diffusion through the choroid and its binding to the RPE require further investigation, the late hypofluorescence observed in certain ICGA diseases provides information on different modalities of RPE dysfunction. Financial Disclosures: The author has no proprietary or commercial interest in any materials discussed in this article.

Natural History and Surgical Outcomes of Lamellar Macular Holes.

PURPOSE: To assess the natural history and surgical outcomes of lamellar macular holes (LMHs). DESIGN: Retrospective and consecutive case series. SUBJECTS: Patients with LMHs from multiple tertiary care centers. METHODS: Clinical charts and OCT scans were reviewed. MAIN OUTCOME MEASURES: The visual acuity (VA) changes and the occurrence rate of full-thickness macular hole (FTMH) were studied in both groups. Within the operated group, factors associated with 6-month VA and development of FTMH were explored. RESULTS: One hundred seventy-eight eyes were included, of which 89 were monitored and 89 underwent surgery. In the observation group, the mean VA decreased from 0.25 ± 0.18 to 0.28 ± 0.18 logarithm of the minimum angle of resolution (logMAR; P = 0.13), with 14 eyes (15.7%) that lost ≥ 0.2 logMAR VA, after 45.7 ± 33.3 months. Nine eyes (10.1%) spontaneously developed an FTMH. In the operated group, the mean VA increased from 0.47 ± 0.23 to 0.35 ± 0.25 logMAR at 6 months (P < 0.001) and 0.36 ± 0.28 logMAR (P = 0.001) after 24.1 ± 30.1 months. By multivariate analysis, better baseline VA (P < 0.001), the presence of an epiretinal membrane (P = 0.03), and the peeling of the internal limiting membrane (ILM; P = 0.02), with a greater effect of ILM perihole sparing, were associated with a greater 6-month VA. Perihole epiretinal proliferation sparing was associated with a better postoperative VA by univariate analysis (P = 0.03), but this was not significant by multivariate analysis. Eight eyes (9.0%) developed a postoperative FTMH. Using Cox proportional hazard ratios [HRs], pseudophakia at baseline (HR, 0.06; 95% confidence interval [CI], 0.00-0.75; P = 0.03) and peeling of the ILM (HR, 0.05; 95% CI, 0.01-0.39; P = 0.004) were protective factors, while ellipsoid zone disruption (HR, 10.5; 95% CI, 1.04-105; P = 0.05) was associated with an increased risk of FTMH. CONCLUSION: Observed eyes with LMH experienced, on average, progressive VA loss. Patients with LMH and altered vision may benefit from surgery. Internal limiting membrane peeling, with perihole ILM sparing, represents a crucial step of the surgery associated with a greater VA and a lower risk of postoperative FTMH. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

PARACENTRAL ACUTE MIDDLE MACULOPATHY ASSOCIATED WITH SEVERE PLASMODIUM FALCIPARUM MALARIA.

PURPOSE: To report a case of bilateral paracentral acute middle maculopathy lesions on spectral domain-optical coherence tomography(OCT) secondary to severe Plasmodium falciparum malaria. METHODS: Retrospective case report. Spectral domain-OCT, ultra-widefield fluorescein angiography, and OCT angiography were performed and analyzed. RESULTS: A 54-year-old healthy man presented with acute vision loss in both eyes few days after being diagnosed with severe Plasmodium falciparum malaria. Ophthalmoscopic examination was unremarkable, but near-infrared reflectance imaging showed patchy hyporeflective areas located at the terminal tips of the venous branches. Corresponding spectral-domain OCT demonstrated alternating bands of hyperreflectivity involving the inner nuclear layer, consistent with skip paracentral acute middle maculopathy lesions. Optical coherance tomography angiography illustrated corresponding flow signal loss at the level of the deep capillary plexus. Ultra-widefield fluorescein angiography showed peripheral retinal vein staining and capillary nonperfusion. CONCLUSION: Paracentral acute middle maculopathy may be an OCT manifestation of malarial retinopathy associated with severe Plasmodium falciparum infection.

Is There a Nonperfusion Threshold on OCT Angiography Associated With New Vessels Detected on Ultra-Wide-Field Imaging in Diabetic Retinopathy?

Purpose: To determine whether the nonperfusion index (NPI) measured on widefield (WF) optical coherence tomography angiography (OCTA) could be used as an alternative method for the diagnosis of proliferative diabetic retinopathy (PDR) and to study the relationship between the NPI and the location of new vessels (NV) in eyes with PDR. Methods: Fifty-one treatment-naïve eyes with either severe nonproliferative DR (NPDR) or PDR were imaged using ultra-wide-field imaging and wide-field OCTA. Results: The NPI was significantly higher in eyes with PDR (18.94% vs. 7.51%; P < 0.01). Using the NPI on the whole image to assess PDR status, the area under the curve was 0.770, but the area under the curve increased when the NPI of the most peripheral circle was used (area under the curve of 0.792). Four eyes with PDR (17%) had NV outside the OCTA image field, and their mean NPI (6.15 %) did not differ from that measured in severe NPDR eyes (7.51%; P = 0.67) and was lower than in other eyes with PDR (21.49%; P = 0.023). The presence of NV in a sector was associated with a higher NPI in the same sector (29.2% vs. 6.0%; P < 10-15). Conclusions: Although the NPI was significantly higher in eyes with PDR compared with severe NPDR eyes, its measurement on the whole wide-field OCTA image was not sensitive enough to replace the detection of NV for the diagnosis of PDR. Translational Relevance: Because the presence of new vessels was related to the local nonperfusion index in the same sector, the assessment of nonperfusion outside the optical coherence tomography angiography field is important in diabetic retinopathy.

Subretinal autofluorescent deposits: A review and proposal for clinical classification.

Subretinal autofluorescent deposits (SADs) may be found in the posterior pole, associated with very various conditions. These disorders usually present a typical pattern of autofluorescent lesions seen on short-wavelength fundus autofluorescence. We describe SADs according to their putative pathophysiological origin and also according to their clinical pattern, i.e., number, shape, and usual location. Five main putative pathophysiological origins of SADs were identified in disorders associated with an intrinsic impairment of phagocytosis and protein transportation, with excess of retinal pigment epithelium phagocytic capacity, with direct or indirect retinal pigment epithelium injury, and/or disorders associated with long-standing serous retinal detachment with mechanical separation between the retinal pigment epithelium and the photoreceptor outer segments. Clinically, however, they could be classified into eight subclasses of SADs, as observed on fundus autofluorescence as follows: single vitelliform macular lesion, multiple roundish or vitelliform lesions, multiple peripapillary lesions, flecked lesions, leopard-spot lesions, macular patterned lesions, patterned lesions located in the same area as the causal disorder, or nonpatterned lesions. Thus, if multimodal imaging may be required to diagnose the cause of SADs, the proposed classification based on noninvasive, widely available short-wavelength fundus autofluorescence could guide clinicians in making their diagnosis decision tree before considering the use of more invasive tools.

ABNORMALITIES IN THE RETINAL CAPILLARY PLEXUSES IN COATS DISEASE IN ADULTHOOD ON OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To describe and quantify the abnormalities of the retinal capillary plexuses using optical coherence tomography angiography in Coats disease. METHODS: Retrospective study. Eleven eyes of 11 patients with Coats disease (9 men and two women aged 32-80 years) compared with nine fellow eyes and 11 healthy control eyes. Horizontal bands of contiguous 3 × 3 mm optical coherence tomography angiograms of the superficial vascular plexus and deep capillary complex were acquired from the optic disk to 6 mm temporal to the fovea, through areas with telangiectasia visible on fluorescein angiography in 9 cases. RESULTS: The vascular density was significantly decreased in both plexuses in eyes with Coats disease compared with normal and fellow eyes within the 6 mm temporal to the fovea (superficial vascular plexus: 21.5 vs. 29.4%, P = 0.00004 and vs. 30.3%, P = 0.00008; deep capillary complex, 16.5 vs. 23.9%, P = 0.00004 and vs. 24.7%, P = 0.00008, respectively). The fractal dimension was also significantly decreased in eyes with Coats disease (superficial vascular plexus: 1.796 vs. 1.848 P = 0.001 and vs. 1.833, P = 0.003; deep capillary complex: 1.762 vs. 1.853, P = 0.003 and vs. 1.838, P = 0.004, respectively). CONCLUSION: Retinal plexuses' vascular density was decreased in Coats disease, including in areas with no visible telangiectasia.

Macular Telangiectasia Type 2: A Classification System Using MultiModal Imaging MacTel Project Report Number 10.

Purpose: To develop a severity classification for macular telangiectasia type 2 (MacTel) disease using multimodal imaging. Design: An algorithm was used on data from a prospective natural history study of MacTel for classification development. Subjects: A total of 1733 participants enrolled in an international natural history study of MacTel. Methods: The Classification and Regression Trees (CART), a predictive nonparametric algorithm used in machine learning, analyzed the features of the multimodal imaging important for the development of a classification, including reading center gradings of the following digital images: stereoscopic color and red-free fundus photographs, fluorescein angiographic images, fundus autofluorescence images, and spectral-domain (SD)-OCT images. Regression models that used least square method created a decision tree using features of the ocular images into different categories of disease severity. Main Outcome Measures: The primary target of interest for the algorithm development by CART was the change in best-corrected visual acuity (BCVA) at baseline for the right and left eyes. These analyses using the algorithm were repeated for the BCVA obtained at the last study visit of the natural history study for the right and left eyes. Results: The CART analyses demonstrated 3 important features from the multimodal imaging for the classification: OCT hyper-reflectivity, pigment, and ellipsoid zone loss. By combining these 3 features (as absent, present, noncentral involvement, and central involvement of the macula), a 7-step scale was created, ranging from excellent to poor visual acuity. At grade 0, 3 features are not present. At the most severe grade, pigment and exudative neovascularization are present. To further validate the classification, using the Generalized Estimating Equation regression models, analyses for the annual relative risk of progression over a period of 5 years for vision loss and for progression along the scale were performed. Conclusions: This analysis using the data from current imaging modalities in participants followed in the MacTel natural history study informed a classification for MacTel disease severity featuring variables from SD-OCT. This classification is designed to provide better communications to other clinicians, researchers, and patients. Financial Disclosures: Proprietary or commercial disclosure may be found after the references.

CENTRAL SEROUS CHORIORETINOPATHY MIMICKING IDIOPATHIC UVEAL EFFUSION SYNDROME.

PURPOSE: To describe central serous chorioretinopathy cases presenting as uveal effusion syndrome, providing new insights into "pachychoroid spectrum" diseases. METHODS: Clinical charts, color fundus photographs, fluorescein angiography, indocyanine green angiography, optical coherence tomography, ultrasound imaging, cerebral magnetic resonance imaging, and biometry of four eyes of three patients were assessed. A literature review was conducted. RESULTS: The three patients had peripheral choroidal detachment and inferior bullous retinal detachment associated with central serous chorioretinopathy features detected using multimodal imaging, including fluorescein and indocyanine green angiography. The choroid was thick in the three patients, and uveal effusion occurred after steroid treatment in all cases. Subretinal fluid drainage and deep sclerectomy with flaps of 4 × 4 mm in both inferior quadrants were performed in three eyes of two patients with good outcomes. One patient was treated with photodynamic therapy. All three patients developed a typical leopard-spot pigmentary pattern in the fundus. CONCLUSION: A severe presentation of highly exudative central serous chorioretinopathy may occur in rare cases with a peripheral choroidal detachment mimicking uveal effusion syndrome. These severe cases highlighted the role of choroidal thickening and hyperpermeability, choroidal vein dilation, and possible scleral thickening in both entities.

Non-vasogenic cystoid maculopathies.

Besides cystoid macular edema due to a blood-retinal barrier breakdown, another type of macular cystoid spaces referred to as non-vasogenic cystoid maculopathies (NVCM) may be detected on optical coherence tomography but not on fluorescein angiography. Various causes may disrupt retinal cell cohesion or impair retinal pigment epithelium (RPE) and Müller cell functions in the maintenance of retinal dehydration, resulting in cystoid spaces formation. Tractional causes include vitreomacular traction, epiretinal membranes and myopic foveoschisis. Surgical treatment does not always allow cystoid space resorption. In inherited retinal dystrophies, cystoid spaces may be part of the disease as in X-linked retinoschisis or enhanced S-cone syndrome, or occur occasionally as in bestrophinopathies, retinitis pigmentosa and allied diseases, congenital microphthalmia, choroideremia, gyrate atrophy and Bietti crystalline dystrophy. In macular telangiectasia type 2, cystoid spaces and cavitations do not depend on the fluid leakage from telangiectasia. Various causes affecting RPE function may result in NVCM such as chronic central serous chorioretinopathy and paraneoplastic syndromes. Non-exudative age macular degeneration may also be complicated by intraretinal cystoid spaces in the absence of fluorescein leakage. In these diseases, cystoid spaces occur in a context of retinal cell loss. Various causes of optic atrophy, including open-angle glaucoma, result in microcystoid spaces in the inner nuclear layer due to a retrograde transsynaptic degeneration. Lastly, drug toxicity may also induce cystoid maculopathy. Identifying NVCM on multimodal imaging, including fluorescein angiography if needed, allows guiding the diagnosis of the causative disease and choosing adequate treatment when available.

Myopic Foveoschisis Completely Resolves within 12 Months after Vitrectomy.

PURPOSE: To assess the sequence of anatomical resolution of myopic foveoschisis (MFS) after vitrectomy. DESIGN: Monocentric retrospective observational case series. SUBJECTS: The files of consecutive patients with MFS who underwent vitreoretinal surgery and were followed postoperatively for at least 6 months were reviewed. METHODS: Patients underwent pars plana vitrectomy for MFS. The central foveal thickness (CFT) was measured. The presence of a foveal involvement, and/or outer retinoschisis (ORS), with or without inner retinoschisis (IRS), and foveal detachment (FD) were analyzed. Anatomical success was defined as the resolution of foveal ORS and FD. MAIN OUTCOME MEASURES: The main outcome was the time to resolution of the different morphologic features of MFS after surgery. RESULTS: Thirty-nine eyes of 36 patients were included in the analysis. The mean follow-up was 14.8 ± 12.9 months (range, 6-84 months). Anatomical success was achieved in 82% of cases at the end of the follow-up and in > 80% of cases during the first year. The CFT was significantly decreased in 79% of cases at 3 months. Inner retinoschisis, present in 18 eyes (46%), resolved in all cases after a median time of 1 month. Foveal ORS, present in all cases, resolved in 82% of cases after a median time of 3 months. The FD, present in 23 eyes (59%), resolved in 91% of cases after a median time of 6 months. Extrafoveal ORS resolved in 59% of cases after a median time of 12 months. The mean best-corrected visual acuity significantly improved from 0.80 ± 0.64 logarithm of the minimum angle of resolution (logMAR) (Snellen Eq 20/148) to 0.48 ± 0.52 logMAR (Snellen Eq 20/70). CONCLUSIONS: Most MFS (80%) are completely resolved during the first year. The decrease in CFT and early resolution of IRS could be used as early biomarkers of surgical success.

Indocyanine Green Angiography Features in Acute Syphilitic Posterior Placoid Chorioretinitis.

PURPOSE: Acute syphilitic posterior placoid chorioretinitis (ASPPC) is a rare clinical manifestation of ocular syphilis. The cause of the placoid lesion is still up for debate but could be caused by an impaired choriocapillaris perfusion. However, less attention has been paid to the hypofluorescence of the plaque on late-phase indocyanine green angiography (ICGA). The aim of this study was to comprehensively analyze multimodal imaging findings in patients with ASPPC and to highlight the value of ICGA for the diagnosis of ASPPC. DESIGN: Retrospective observational case study. METHODS: The medical records of patients with uveitis who consulted our tertiary center between 2012 and December 2015 were reviewed. Patients who were diagnosed with uveitis related to syphilis infection with posterior placoid lesions seen on multimodal imaging were included. We compared the aspect of ASPPC on fundus color photography, blue autofluorescence, fluorescein angiography, optical coherence tomography, and early-, mid- and late-phase ICGA. RESULTS: Fifteen eyes of 12 patients were included in the study. Hypofluorescent plaques were seen on late-phase ICGA in all eyes, corresponding to the placoid lesions visible on blue autofluorescence, while the choriocapillaris filling was normal on fluorescein angiography and ICGA. Within the plaques, optical coherence tomography showed ellipsoid zone disruptions, outer retinal disruptions, and retinal pigment epithelium granulations. CONCLUSION: ASPPC could be caused by retinal pigment epithelium dysfunction secondary to an infectious or inflammatory disorder, characterized by a hypofluorescence visible only on late-phase ICGA, and resulting in photoreceptor disruptions. The RPE impairment was reversible after prompt antibiotic treatment.

Recommendations for OCT Angiography Reporting in Retinal Vascular Disease: A Delphi Approach by International Experts.

PURPOSE: To develop a consensus nomenclature for reporting OCT angiography (OCTA) findings in retinal vascular disease (e.g., diabetic retinopathy, retinal vein occlusion) by international experts. DESIGN: Delphi-based survey. SUBJECTS, PARTICIPANTS, AND/OR CONTROLS: Twenty-five retinal vascular disease and OCTA imaging experts. METHODS, INTERVENTION, OR TESTING: A Delphi method of consensus development was used, comprising 2 rounds of online questionnaires, followed by a face-to-face meeting conducted virtually. Twenty-five experts in retinal vascular disease and retinal OCTA imaging were selected to constitute the OCTA Nomenclature in Delphi Study Group for retinal vascular disease. The 4 main areas of consensus were: definition of the parameters of "wide-field (WF)" OCTA, measurement of decreased vascular flow on conventional and WF-OCTA, nomenclature of OCTA findings, and OCTA in retinal vascular disease management and staging. The study end point was defined by the degree of consensus for each question: "strong consensus" was defined as ≥85% agreement, "consensus" as 80% to 84%, and "near consensus" as 70% to 79%. MAIN OUTCOME MEASURES: Consensus and near consensus on OCTA nomenclature in retinal vascular disease. RESULTS: A consensus was reached that a meaningful change in percentage of flow on WF-OCTA imaging should be an increase or decrease ≥30% of the absolute imaged area of flow signal and that a "large area" of WF-OCTA reduced flow signal should also be defined as ≥30% of the absolute imaged area. The presence of new vessels and intraretinal microvascular abnormalities, the foveal avascular zone parameters, the presence and amount of "no-flow areas," and the assessment of vessel density in various retinal layers should be added for the staging and classification of diabetic retinopathy. Decreased flow ≥30% of the absolute imaged area should define an ischemic central retinal vein occlusion. Several other items did not meet consensus requirements or were rejected in the final discussion round. CONCLUSIONS: This study provides international consensus recommendations for reporting OCTA findings in retinal vascular disease, which may help to improve the interpretability and description in clinic and clinical trials. Further validation in these settings is warranted and ongoing. Efforts are continuing to address unresolved questions.

Review of the Current Literature and Our Experience on the Value of OCT-angiography in White Dot Syndromes.

PURPOSE: To describe the application of OCT-A in various posterior uveitis disorders in our experience and to compare it with the available literature. METHODS: Eighteen eyes with the diagnoses of multifocal choroiditis (MFC), multifocal placoid pigment epitheliopathy (APMPPE), multiple evanescent white dot syndrome (MEWDS), tuberculous serpiginous-like choroiditis (SLC), serpiginous choroiditis (SC), and birdshot chorioretinopathy (BSCR) were studied. RESULTS: We found flow void of the choriocapillaris in patients with APMPPE, SC, MFC, BSCR, and in SLC. In contrast, perfusion of the choriocapillaris seemed normal in patients with MEWDS. CONCLUSIONS: We confirmed that OCT-A contributes new information on the physiopathology of white dot syndromes and inflammatory chorioretinopathies, notably on whether or not the choriocapillaris is involved. Comparing the OCT-A features allowed us to suggest that both APMPPE and SLC might be part of the same spectrum of inflammatory disease with primary involvement at the level of the choriocapillaris and secondary RPE damage.

Type one macular neovascularization in central serous chorioretinopathy: Short-term response to anti-vascular endothelial growth factor therapy.

PURPOSE: The aim of this study was to assess the short-term effect of anti-vascular endothelial growth factor (VEGF) treatment on type 1 macular neovascularization (MNV) secondary to central serous chorioretinopathy (CSCR) and to identify potential predictive factors for treatment response using multimodal imaging. METHODS: Retrospective, multicentre study in CSCR patients with MNV detected by OCT-angiography and treated with anti-VEGF injections. Clinical and multimodal imaging data before and after anti-VEGF injections was reviewed. Univariate and multivariate linear regression analyses were performed to evaluate associations between the change in central macular thickness (CMT) after anti-VEGF therapy and other factors. RESULTS: Forty patients were included. One month after receiving a mean number of 2.7 anti-VEGF intravitreal injections, visual acuity increased significantly from 0.46 ± 0.3 logMAR at baseline to 0.38 ± 0.4 logMAR (p = 0.04). The CMT and foveal serous retinal detachment (SRD) decreased significantly from 330 ± 81.9 µm at baseline to 261.7 ± 63.1 µm after treatment (p < 0.001) and from 145.1 ± 98.8 µm at baseline to 52.6 ± 71.3 µm (p < 0.001), respectively. Subretinal fluid and/or intraretinal fluid were still present in 18 eyes (45%) one month after treatment. In the multivariate analysis, a higher SRD height was associated with a greater CMT change (p = 0.002) and a lower CMT change with the presence of subretinal hyperreflective material (SHRM) (p = 0.04). CONCLUSION: Fluid resorption was incomplete in about half of the patients with MNV secondary to CSCR after anti-VEGF injections. Shallower SRD or the presence of SHRM were predictors of poor response to anti-VEGF.