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.

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.

Postoperative outcomes of idiopathic epiretinal membrane associated with foveoschisis.

AIMS: To analyse the prevalence and postoperative outcomes of a particular form of epiretinal membrane (ERM) with foveoschisis-like stretched hyporeflective spaces in emmetropic eyes. METHODS: A retrospective study of all consecutive eyes operated for primary ERM over a 46-month period was conducted. The presence of foveoschisis-like stretched hyporeflective spaces was assessed on the preoperative optical coherence tomography B-scan in all eyes. Highly myopic eyes were excluded. Preoperative and postoperative characteristics of eyes with foveoschisis were compared with those of a control group of 100 consecutive eyes with primary ERM without cystoid spaces. RESULTS: Of 544 eyes with primary ERM, 17 had foveoschisis, corresponding to a prevalence of 3.1%. After a mean postoperative follow-up of 17.9±10.9 months, the foveoschisis had completely resolved in 76.5% of eyes (n=13/17). In the four eyes (23.5%) with persistent foveoschisis, the remaining hyporeflective cystoid spaces were located exclusively in the inner nuclear layer. The postoperative visual acuity and central macular thickness did not differ between both groups at the final visit. However, an acute postoperative macular oedema occurred in 24% (n=4/17) of cases (vs 3% in the control group; p=0.0084). CONCLUSION: Foveoschisis was found in about 3% of eyes with idiopathic ERM. After peeling, the foveoschisis usually resolves completely with functional outcomes similar to those achieved with other primary ERM. However, this feature was associated with a higher risk of postoperative macular oedema (in 1/4 of cases).

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.

Surgical outcomes in patients with lamellar macular holes selected based on the optical coherence tomography consensus definition.

PURPOSE: The surgical indication for lamellar macular holes (LMH) is controversial due to a misclassification of different macular diseases. A consensus based on an optical coherence tomography (OCT) definition has recently been suggested. The aim of this study was to investigate the surgical outcomes of patients with LMH selected based on this OCT-based consensus definition. METHODS: Retrospective review of patients who underwent surgery for LMH with a follow-up of at least 3 months. Anatomical OCT criteria for the diagnosis of LMH were the presence of an irregular foveal contour with foveal cavitation and a loss of retinal tissue. Cases of macular pseudoholes and epiretinal membrane foveoschisis were excluded. Surgery consisted in pars plana vitrectomy with centripetal peri-hole peeling of epiretinal proliferation and internal limiting membrane. Pre- and postoperative visual acuities (VA) were compared, and changes in OCT anatomical features, including the restoration of the foveal profile and outer retinal layers, were assessed. RESULTS: Eleven eyes of 11 patients were included, of which 9 eyes (81.8%) showed proliferation on preoperative OCT. The mean VA improved from 0.44 ± 0.19 LogMAR (20/55 Snellen equivalent) to 0.16 ± 0.08 LogMAR (20/28 Snellen equivalent), after a mean follow-up of 7.2 ± 2.9 months (P = 0.02). Postoperatively, all eyes showed a restored foveal profile. The mean central foveal thickness increased from 127.6 ± 29.9 µm to 209.0 ± 44.0 µm (P = 0.001). At baseline, ellipsoid zone disruption and external limiting membrane disruption were found in 9 and 7 eyes, respectively. Postoperatively, the ellipsoid zone and external limiting membrane were restored in respectively 6/9 eyes (66.7%) and 5/7 eyes (71.4%). No cases of postoperative full-thickness macular hole were found. CONCLUSION: In patients with LMH carefully selected based on the recent OCT-based criteria and showing a loss of retinal tissue, the foveal architecture was restored and the VA was improved after vitrectomy with peri-hole peeling for epiretinal proliferation.

IMI Pathologic Myopia.

Pathologic myopia is a major cause of visual impairment worldwide. Pathologic myopia is distinctly different from high myopia. High myopia is a high degree of myopic refractive error, whereas pathologic myopia is defined by a presence of typical complications in the fundus (posterior staphyloma or myopic maculopathy equal to or more serious than diffuse choroidal atrophy). Pathologic myopia often occurs in eyes with high myopia, however its complications especially posterior staphyloma can also occur in eyes without high myopia. Owing to a recent advance in ocular imaging, an objective and accurate diagnosis of pathologic myopia has become possible. Especially, optical coherence tomography has revealed novel lesions like dome-shaped macula and myopic traction maculopathy. Wide-field optical coherence tomography has succeeded in visualizing the entire extent of large staphylomas. The effectiveness of new therapies for complications have been shown, such as anti-VEGF therapies for myopic macular neovascularization and vitreoretinal surgery for myopic traction maculopathy. Myopia, especially childhood myopia, has been increasing rapidly in the world. In parallel with an increase in myopia, the prevalence of high myopia has also been increasing. However, it remains unclear whether or not pathologic myopia will increase in parallel with an increase of myopia itself. In addition, it has remained unclear whether genes responsible for pathologic myopia are the same as those for myopia in general, or whether pathologic myopia is genetically different from other myopia.

Spontaneous Conversion of Lamellar Macular Holes to Full-Thickness Macular Holes: Clinical Features and Surgical Outcomes.

PURPOSE: To describe the clinical features and surgical outcomes of patients experiencing a spontaneous conversion of a lamellar macular hole (LMH) to a full-thickness macular hole (FTMH). DESIGN: Retrospective, multicenter, observational case series. PARTICIPANTS: Patients with LMH who experienced a spontaneous conversion to FTMH and underwent FTMH surgery. METHODS: Clinical charts and OCT features of 20 eyes of 20 patients were reviewed. MAIN OUTCOME MEASURES: OCT features and surgical outcomes of FTMH derived from LMH. RESULTS: The mean baseline visual acuity (VA) was 0.21 ± 0.19 logarithm of the minimum angle of resolution (logMAR) (20/32 Snellen equivalent [SE]). Epiretinal proliferation was noted in 18 eyes (90%), and 14 eyes (75%) had an epiretinal membrane. At the diagnosis of FTMH, the mean VA decreased to 0.61 ± 0.50 logMAR (20/81 SE) (P = 0.001). The mean FTMH diameter was 224.4 ± 194.8 µm, with 15 (75%) small (≤250 µm), 2 (10%) medium (>250-≤400 µm), and 3 (15%) large (>400 µm) FTMHs. Eighteen (90%) FTMHs were sealed after 1 surgery, and 2 (10%) required an additional procedure. At the last follow-up, the mean VA was increased to 0.29 ± 0.23 logMAR (20/38 SE) (P = 0.003), but did not significantly differ from the baseline VA (P = 0.071). CONCLUSIONS: Patients with LMH may develop an FTMH with no evidence of vitreomacular traction. A tangential traction from an epiretinal membrane may contribute to its genesis, but a progressive loss of retinal tissue and an inherent weakness of the foveal architecture in LMH eyes could be sufficient. Most FTMHs derived from LMH had a small diameter, showed epiretinal proliferation, showed limited retinal hydration, and were associated with relatively poor surgical outcomes compared with idiopathic FTMH.

Henle fibre layer haemorrhage: clinical features and pathogenesis.

BACKGROUND: To describe the clinical presentation and characteristic imaging features of deep retinal haemorrhages primarily located in the Henle fibre layer (HFL) of the macula. The spectrum of aetiologies and a comprehensive theory of pathogenesis are presented. METHODS: This is a retrospective, multicentre case series evaluating eyes with retinal haemorrhage in HFL. Clinical features, underlying aetiology, systemic and ocular risk factors, visual acuity, and multimodal imaging including fundus photography and cross-sectional and en face optical coherence tomography (OCT) are presented. RESULTS: Retinal haemorrhages localised to HFL in 33 eyes from 23 patients were secondary to acute blunt trauma to the head (n=2), eye (n=1) and trunk (n=1), ruptured intracranial aneurysm (Terson's syndrome, n=3), general anaesthesia (n=1), epidural anaesthesia (n=1), hypertension with anaemia (n=1), decompression retinopathy (n=1), postvitrectomy with intraocular gas (n=1), retinal vein occlusion (n=7), myopic degeneration (n=2), macular telangiectasia type 2 (n=1), and polypoidal choroidal vasculopathy (n=1). Defining clinical features included deep retinal haemorrhage with feathery margin and petaloid pattern radiating from the fovea. OCT demonstrated characteristic hyper-reflectivity from the haemorrhage delineated by obliquely oriented fibres in the Henle layer. Spontaneous resolution of HFL haemorrhage occurred after 3 months in 15 patients with follow-up. CONCLUSION: The characteristic petaloid-shaped, deep intraretinal haemorrhage with a feathery margin localised to HFL is associated with various disorders. The terminology 'Henle fiber layer hemorrhage (HH)' is proposed to describe the clinical and OCT findings, which may result from abnormal retinal venous pressure from systemic or local retinovascular disorders affecting the deep capillary plexus or from choroidal vascular abnormalities.

Widefield OCT-Angiography and Fluorescein Angiography Assessments of Nonperfusion in Diabetic Retinopathy and Edema Treated with Anti-Vascular Endothelial Growth Factor.

PURPOSE: To assess change in retinal nonperfusion (NP) after anti-vascular endothelial growth factor (VEGF) therapy for diabetic macular edema (DME) using 2 different imaging modalities: swept-source widefield (SS-WF) OCT angiography (OCTA) and ultra-widefield (UWF) fluorescein angiography (FA). DESIGN: Observational case series. PARTICIPANTS: Ten eyes of 9 patients with severe nonproliferative diabetic retinopathy (NPDR) or proliferative DR (PDR) initiating 3 monthly anti-VEGF intravitreal injections for DME. METHODS: All eyes were imaged with UWF color fundus photographs (CFPs), UWF FA, and SS-WF OCTA at baseline (M0) and 1 month after the third anti-VEGF injection (M3). All images were aligned and divided into 16 boxes for analysis of NP areas by 2 blinded retina specialists. MAIN OUTCOME MEASURES: The number of discrepancies between SS-WF OCTA and UWF FA regarding the detection of NP areas and small vessels passing through NP areas; assessment of DR severity on UWF CFP; and change in each NP area between M0 and M3: number of boxes/eye with presence of at least 1 NP area, number of arterioles or venules that disappeared or reappeared, and number of NP areas in which capillaries disappeared or reappeared. RESULTS: The diabetic retinopathy (DR) severity score improved by at least 1 stage in 8 of 10 eyes, with a significant decrease in the mean number of microaneurysms and retinal hemorrhages on UWF CFP at M3 versus M0 (n = 40±28 vs. 121±57; P = 0.0020) and regression of fundus neovascularization when present. All NP areas detected on FA were seen on SS-WF OCTA, but additional NP areas were detected on SS-WF OCTA at M0 in 29% (46/160) of boxes. No reperfusion of arterioles or venules was observed at M3 on FA or SS-WF OCTA. Retinal capillaries were only visible on OCTA, and no reperfusion in NP areas was observed even when a reduction in dark areas was visible on FA. CONCLUSIONS: No reperfusion of vessels or capillary network was detected in NP areas using 2 imaging techniques, UWF FA and SS-WF OCTA, in eyes with DR after 3 anti-VEGF injections. The detection rate of NP areas was higher with SS-WF OCTA than with UWF FA.

MANAGEMENT OF RETINAL HEMANGIOBLASTOMA IN VON HIPPEL-LINDAU DISEASE.

PURPOSE: To review the current state of diagnosis and management of retinal hemangioblastoma and retinal vascular proliferation arising from von Hippel-Lindau (VHL) disease. METHODS: A review of the literature was performed. Consensus was reached among authors regarding current practice, with reference to published data where possible. RESULTS: von Hippel-Lindau disease and its ocular manifestations are relatively rare, and there is limited evidence in the literature on which to base management. There was consensus on core principles, including 1) recognition and diagnosis of von Hippel-Lindau disease when present, with appropriate referral for care of this potentially lethal systemic condition; 2) regular ophthalmic evaluation for individuals with von Hippel-Lindau disease, to identify and offer timely treatment for new or active retinal hemangioblastomas; 3) ablative treatment of retinal hemangioblastomas that can be safely destroyed, to lower risk of vision loss; 4) observation or consideration of nonablative treatments for retinal hemangioblastomas that cannot be safely destroyed; and 5) observation of asymptomatic retinal vascular proliferation, with consideration of vitrectomy for lesions exerting effects on vision. CONCLUSION: Ocular outcomes can be gratifying in many cases with appropriate management. Improved understanding of the molecular basis for the disease creates an opportunity for rational design of better therapies.

VON HIPPEL-LINDAU DISEASE: Update on Pathogenesis and Systemic Aspects.

PURPOSE: To provide an update summarizing the biologic pathways governing von Hippel-Lindau (VHL) disease pathogenesis and to provide an overview of systemic manifestations as well as screening recommendations. METHODS: A PubMed search of the English language literature was reviewed using the following search terms: von Hippel-Lindau, von Hippel-Lindau disease, and VHL. Of 6,696 publications, the most current and pertinent information related to the pathogenesis and systemic aspects of VHL disease were included in this review. RESULTS: von Hippel-Lindau disease is one of the most frequently occurring multisystem familial cancer syndromes. The disease results from germline mutation in the VHL tumor suppressor gene on the short arm of chromosome 3. Mutation in the VHL gene affects multiple cellular processes including transcriptional regulation, extracellular matrix formation, apoptosis, and, in particular, the cellular adaptive response to hypoxia. As a result, there is widespread development of vascular tumors affecting the retina, brain, and spine, as well as a spectrum of benign and malignant tumors and/or cysts in visceral organs. CONCLUSION: The ophthalmologist plays a key role in VHL disease diagnosis, as retinal hemangioblastoma is frequently the first disease manifestation. Screening guidelines for individuals with known VHL disease, and those at risk of VHL disease, help to ensure early detection of potentially vision-threatening and life-threatening disease.

Neoplasia and intraocular inflammation: From masquerade syndromes to immunotherapy-induced uveitis.

Masquerade syndromes represent a large set of ophthalmological entities that mimic inflammatory conditions. Any delay in their diagnosis may be correlated with systemic dissemination or worsening of the causal disease and, therefore, with poor prognosis. One of the disadvantages of the new potent treatments of uveitis is the delay that they can induce in the diagnosis of neoplastic intraocular infiltrations. Thorough and careful clinical examination of all patients referred for uveitis, especially when they are Caucasian, over 50 years of age, and with posterior segment involvement, is of paramount importance in this context. Ancillary investigations and often-invasive histo-pathologic evaluation of tissue specimens or ocular fluids are regularly required in these situations. The most common masquerade syndrome is primary vitreoretinal lymphoma (PVRL). New molecular diagnostic tools may be helpful in challenging cases lacking cytological confirmation. Therapeutic strategies targeting tumoral cells in the eye and also in the central nervous system can improve the life expectancy of affected patients. In this review, we discuss diagnostic strategies and current therapies in PVRL and provide an overview of other conditions that can mimic primary ocular inflammation, especially in the field of oncology and its new therapeutic armamentarium.

ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR THERAPY CAN IMPROVE DIABETIC RETINOPATHY SCORE WITHOUT CHANGE IN RETINAL PERFUSION.

PURPOSE: To compare the changes in retinal perfusion on ultra-wide-field fluorescein angiography with the changes in diabetic retinopathy lesions observed on ultra-wide-field fundus color photographs after 3 monthly anti-vascular endothelial growth factor injections. METHODS: Retrospective interventional cohort study analyzing the files of 14 patients with DR (18 eyes). UWF color photos and FA were analyzed at baseline (M0) and 1 month after the third anti-VEGF injection (M3). The main outcomes included the count of the number of red dots (microaneurysms, hemorrhages) and assessment of DR severity score (DRSS); the analysis of non-perfusion areas and disappearance or reappearance of arterioles or venules in the non-perfusion areas on FA. RESULTS: Eighteen eyes of 14 diabetic patients, with mean age of 63 ± 5 years, were included. The DRSS score improved by at least one stage in 11/18 (61%) eyes. The mean number of red dots significantly decreased at M3 (n = 80 ± 85) compared with M0 (n = 139 ± 130) (P < 0.0001). No reperfusion of arterioles or venules was observed in or around nonperfusion areas. CONCLUSION: After anti-vascular endothelial growth factor injections, the improvement in the DRSS score based on color fundus photographs can occur without retinal reperfusion on ultra-wide-field fluorescein angiography.

Optical Coherence Tomography Angiography of Familial Retinal Arteriolar Tortuosity.

BACKGROUND AND OBJECTIVE: To analyze the location of familial retinal arterial tortuosity (fRAT) in the three-dimensional structure of retinal capillaries. PATIENTS AND METHODS: Retrospective observational study. Twelve eyes of six patients (two of whom were brothers) were imaged by optical coherence tomography angiography (OCTA). The data from their ocular and systemic examinations were recorded. RESULTS: OCTA imaging clearly showed increased tortuosity of second- and third-order retinal arteries in all cases, visible in the superficial vascular plexus (SVP) up to the arteriole termination in the capillaries. No change was visible in the deep capillary plexus (DCP). CONCLUSIONS: OCTA shows that fRAT affects all the course of the arterioles up to the capillaries in the SVP. The DCP does not show arteriolar tortuosity because it does not contain arterioles. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:397-401.].

Association Between Vessel Density and Visual Acuity in Patients With Diabetic Retinopathy and Poorly Controlled Type 1 Diabetes.

Importance: Capillary dropout is a hallmark of diabetic retinopathy, but its role in visual loss remains unclear. Objective: To examine how macular vessel density is correlated with visual acuity (VA) in patients younger than 40 years who have type 1 diabetes without macular edema but who have diabetic retinopathy requiring panretinal photocoagulation. Design, Settings, and Participants: Retrospective cohort study of VA and optical coherence tomography angiography data collected from consecutive patients during a single visit to Lariboisière Hospital, a tertiary referral center in Paris, France. The cohort included 22 eyes of 22 patients with type 1 diabetes without macular edema but with bilateral rapidly progressive diabetic retinopathy that was treated with panretinal photocoagulation between August 15, 2015, and December 30, 2016. Eyes were classified into 2 groups by VA: normal (logMAR, 0; Snellen equivalent, 20/20) and decreased (logMAR, >0; Snellen equivalent, <20/20). The control group included 12 eyes from age-matched healthy participants with normal vision. Main Outcomes and Measures: Visual acuity and mean vessel density in 4 retinal vascular plexuses: the superficial vascular plexus and the deep capillary complex, which comprises the intermediate capillary plexus and the deep capillary plexus. Results: Of the 22 participants, 11 (50%) were men, mean (SD) age was 30 (6) years, and mean (SD) hemoglobin A1c level was 8.9% (1.6%). Of the 22 eyes with diabetic retinopathy, 13 (59%) had normal VA and 9 (41%) had decreased VA (mean [SD]: logMAR, 0.12 [0.04]; Snellen equivalent, 20/25). Mean [SE] vessel density was lower for eyes with diabetic retinopathy and normal VA compared with the control group in the superficial vascular plexus (44.1% [0.9%] vs 49.1% [0.9%]; difference, -5.0% [1.3%]; 95% CI, -7.5% to -2.4%; P < .001), in the deep capillary complex (44.3% [1.2%] vs 50.6% [1.3%]; difference, -6.3% [1.8%]; 95% CI, -9.9% to -2.7%; P = .001), in the intermediate capillary plexus (43.8% [1.2%] vs 49.3% [1.2%]; difference, -5.5% [1.7%]; 95% CI, -9.0% to -2.0%; P = .003), and in the deep capillary plexus (24.5% [1.0%] vs 30.5% [1.0%]; difference, -6.1% [1.4%]; 95% CI, -8.9% to -3.2%; P < .001). Mean vessel density was lower in eyes with diabetic retinopathy and decreased VA compared with eyes with diabetic retinopathy and normal VA; the mean (SE) loss was more pronounced in the deep capillary complex (34.6% [1.5%] vs 44.3% [1.2%]; difference, -9.6% [1.9%]; 95% CI, -13.6% to -5.7%; P < .001), especially in the deep capillary plexus (15.2% [1.2%] vs 24.5% [1.0%]; difference, -9.3% [1.5%]; 95% CI, -12.4% to -6.1%; P < .001), than in the superficial vascular plexus (39.6% [1.1%] vs 44.1% [0.9%]; difference, -4.5% [1.4%]; 95% CI, -7.3% to -1.7%; P = .002). Conclusions and Relevance: These data suggest that in patients with type 1 diabetes without macular edema but with severe nonproliferative or proliferative diabetic retinopathy, decreased VA may be associated with the degree of capillary loss in the deep capillary complex.

ACUTE PSEUDOPHAKIC CYSTOID MACULAR EDEMA IMAGED BY OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To study macular capillary changes and vessel density in acute pseudophakic cystoid macular edema (PCME) before and after treatment using optical coherence tomography angiography. METHODS: Retrospective observational case-control study of seven consecutive patients (eight eyes) with PCME and eight age-matched control eyes imaged with optical coherence tomography angiography (RTVue XR Avanti; Optovue, Inc, Fremont, CA) using Projection Removal Artifacts software. Vessel density was calculated. RESULTS: The mean time to diagnosis of PCME was 2.3 ± 0.9 months after surgery. At initial examination, the superficial capillary plexus pattern was near-normal in all PCME eyes, although it was attenuated in the deep capillary plexus. The mean vessel density of the superficial capillary plexus in PCME eyes was slightly but significantly lower than in control eyes (47.8 ± 3.8% vs. 52.9 ± 4.0%, P = 0.01), the difference being greater in the deep capillary plexus (44.1 ± 7.4% vs. 54.2 ± 3.2%, P = 0.007). After resolution of the edema, the deep capillary plexus completely recovered its normal pattern and the vessel density in both plexuses was no longer different from that observed in control eyes. CONCLUSION: Macular vessel density after resolution of an acute PCME did not differ from that of normal control eyes in both the superficial capillary plexus and deep capillary plexus, unlike macular edema in retinal vaso-occlusive diseases.

Epiretinal Membrane in Dome-Shaped Macula Complicated with Serous Retinal Detachment: Transient Efficacy of Surgery.

Dome-shaped macula (DSM) is an entity recently described as a convex anterior protrusion of the macular area within a posterior myopic staphyloma. Specific complications were associated with DSM, like serous retinal detachment (SRD). We describe a woman presenting with a decreased vision at 20/50. SD-OCT scans were performed, showing a macular bulge. SRD was present and an epiretinal membrane could also be observed. Fluorescein angiography and indocyanin green angiography did not show any leakage nor choroidal neovascularization. Epiretinal membrane peeling was performed, and 3 months after surgery, SRD completely disappeared. However, SRD reappeared 1 year after surgery and enlarged within 2 years following surgery. In conclusion, two mechanisms could be considered for physiopathology of SRD: first, the epiretinal membrane may have exerted traction on the macular retina, second, vitreous body might constitute a tank for cytokines and/or other factors, triggering subretinal fluid accumulation, leading to SRD.

Comparing Parafoveal Cone Photoreceptor Mosaic Metrics in Younger and Older Age Groups Using an Adaptive Optics Retinal Camera.

BACKGROUND AND OBJECTIVE: To analyze cone mosaic metrics on adaptive optics (AO) images as a function of retinal eccentricity in two different age groups using a commercial flood illumination AO device. PATIENTS AND METHODS: Fifty-three eyes of 28 healthy subjects divided into two age groups were imaged using an AO flood-illumination camera (rtx1; Imagine Eyes, Orsay, France). A 16° × 4° field was obtained horizontally. Cone-packing metrics were determined in five neighboring 50 µm × 50 µm regions. Both retinal (cones/mm2 and µm) and visual (cones/degrees2 and arcmin) units were computed. RESULTS: Results for cone mosaic metrics at 2°, 2.5°, 3°, 4°, and 5° eccentricity were compatible with previous AO scanning laser ophthalmoscopy and histology data. No significant difference was observed between the two age groups. CONCLUSIONS: The rtx1 camera enabled reproducible measurements of cone-packing metrics across the extrafoveal retina. These findings may contribute to the development of normative data and act as a reference for future research. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:45-50.].