Laura Kuehlewein

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY OF TYPE 3 NEOVASCULARIZATION SECONDARY TO AGE-RELATED MACULAR DEGENERATION.

Purpose: To characterize the vascular structure of Type 3 neovascularization secondary to age-related macular degeneration using optical coherence tomography angiography. Methods: Optical coherence tomography angiography cube scans (3 mm × 3 mm) were acquired in 29 eyes of 24 patients with Type 3 lesions secondary to age-related macular degeneration using the RTVue XR Avanti with AngioVue, Split-spectrum amplitude-decorrelation, and motion correction technology. Automated layer segmentation boundaries were adjusted to best visualize the neovascular complex on en face projection images. Results: A distinct neovascular complex could be identified in 10 (34%) eyes, all of which were active on optical coherence tomography imaging. In all 10 eyes, the neovascular complex appeared as a small tuft of bright, high-flow tiny vessels with curvilinear morphology located in the outer retinal layers with a feeder vessel communicating with the inner retinal circulation (i.e., deep retinal capillary plexus). The mean (SD) size of the neovascular complex measured 0.07 (± 0.07) mm2. Conclusion: With optical coherence tomography angiography, it is possible to identify small intraretinal neovascular complexes communicating with the deep retinal capillary plexus in eyes with Type 3 neovascularization secondary to age-related macular degeneration. Qualitative and quantitative analyses of Type 3 neovascular complexes can be performed using optical coherence tomography angiography.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN RETINAL ARTERY OCCLUSION.

Purpose: To describe the retinal microvasculature of the eyes with nonarteritic retinal artery occlusion (RAO) based on optical coherence tomography angiography. Methods: Cross-sectional, prospective, observational study performed from September 2014 through February 2015. En face projection of optical coherence tomography angiography images centered at the macula and optic disk of the eyes presenting with RAO were acquired using the RTVue XR Avanti with AngioVue software. Qualitative analysis of the morphology of the superficial and deep retinal capillary plexuses, and radial peripapillary capillaries was performed. Retinal vasculature images using optical coherence tomography angiography were correlated with fluorescein angiography images. Results: Seven patients (seven eyes) were enrolled in the study, including three eyes with central RAO and four eyes with branch RAO. Distinct differences in the distribution of zones of decreased vascular perfusion between the superficial and deep retinal capillary plexus corresponding to areas of delayed dye perfusion on fluorescein angiography were demonstrated in 6 of 7 (86.5%) eyes. Conclusion: This small series suggests that optical coherence tomography angiography imaging can accurately discern retinal capillary plexuses at different levels in the eyes with RAO and may be sensitive for more precisely characterizing the extent of macular ischemia and monitoring vascular flow changes during the course of the disease.

Retinal Capillary Network and Foveal Avascular Zone in Eyes with Vein Occlusion and Fellow Eyes Analyzed With Optical Coherence Tomography Angiography.

PURPOSE To evaluate the perifoveolar retinal capillary network at different depths and to quantify the foveal avascular zone (FAZ) in eyes with retinal vein occlusion (RVO) compared with their fellow eyes and healthy controls using spectral-domain optical coherence tomography angiography (SD-OCTA). METHODS We prospectively recruited 23 patients with RVO including 15 eyes with central RVO (CRVO) and 8 eyes with branch RVO (BRVO), their fellow eyes, and 8 age-matched healthy controls (8 eyes) for imaging on prototype OCTA software within RTVue-XR Avanti. The 3 × 3 mm and 6 × 6 mm en face angiograms of superficial and deep retinal capillary plexuses were segmented. Perifoveolar retinal capillary network was analyzed and FAZ was quantified. RESULTS Decrease in vascular perfusion at the deep plexus was observed in all eyes with CRVO (8/8, 100%) and BRVO (6/6, 100%) without cystoid macular edema, and in 8 of 15 (53%) and 2 of 8 (25%) of the fellow eyes, respectively. Vascular tortuosity was observed in 13 of 15 (87%) CRVO and 5 of 8 (63%) BRVO eyes. Collaterals were seen in 10 of 15 (67%) CRVO and 5 of 8 (63%) BRVO eyes. Mean FAZ area was larger in eyes with RVO than their fellow eyes (1.13 ± 0.25 mm2 versus 0.58 ± 0.28 mm2; P = 0.007) and controls (1.13 ± 0.25 mm2 versus 0.30 ± 0.09 mm2; P < 0.0001), and in fellow eyes of RVO patients when compared to controls (0.58 ± 0.28 mm2 versus 0.30 ± 0.09 mm2; P = 0.01). CONCLUSIONS Spectral-domain OCTA reveals abnormalities at different levels of perifoveolar retinal capillary network and is able to quantify the FAZ in RVO. Longitudinal studies may be considered to evaluate the clinical utility of OCTA in RVO and other retinal vascular diseases.

Paracentral Acute Middle Maculopathy: What We Knew Then and What We Know Now.

What We Knew Then and What We Know Now In 2013, we characterized a novel presentation of hyperreflective band-like spectral-domain optical coherence tomography (SD-OCT) lesions at the level of the inner nuclear layer (INL) in a clinical series of 6 eyes from 5 patients. We descriptively termed this finding paracentral acute middle maculopathy (PAMM) due to the parafoveal position of the causative gray lesion with near-infrared reflectance imaging, the acute onset of the resulting scotoma, and the SD-OCT localization of involvement to the middle layer (INL) of the retina. Localized retinal capillary ischemia was proposed as the mechanism underlying the development of these lesions. Since then, a growing body of knowledge regarding PAMM has led to a greater understanding of this entity, and this review will serve to update the clinical associations and pathogenic mechanisms of this abnormality while distinguishing it from similar disorders.

Imaging Areas of Retinal Nonperfusion in Ischemic Branch Retinal Vein Occlusion With Swept-Source OCT Microangiography

The authors present the case of a patient with a history of ischemic branch vein occlusion and multimodal imaging of the retinal vasculature by fluorescein angiography (FA) and ultrahigh-speed swept-source optical coherence tomography (SS-OCT) microangiography (SS-OCT laser prototype; 1,050 nm, 100,000 A-scans/second). Multiple images across the macula were acquired (3 × 3 mm cubes in clusters of four repeated B-scans). En face images of the vasculature were generated by implementing an intensity differentiation algorithm. The retinal vasculature as well areas of nonperfusion could be identified precisely at multiple retinal levels. Ultrahigh-speed SS-OCT microangiography provides noninvasive, three-dimensional, high-resolution images of the retinal vasculature including the capillaries.

Predictors of Macular Atrophy Detected by Fundus Autofluorescence in Patients With Neovascular Age-Related Macular Degeneration After Long-Term Ranibizumab Treatment.

BACKGROUND AND OBJECTIVE To study the relationship between baseline morphologic characteristics of the choroidal neovascular (CNV) lesion and long-term development of macular atrophy in eyes with neovascular age-related macular degeneration (AMD) treated with ranibizumab (Lucentis; Genentech, South San Francisco, CA). PATIENTS AND METHODS Certified graders evaluated baseline and 7-year follow-up (SEVEN-UP study) images of 41 eyes from the MARINA/ANCHOR and HORIZON trials. Using GRADOR software and stepwise linear regression, graders correlated lesion characteristics on fluorescein angiography (FA) at both visits with areas of definite decreased autofluorescence (DDAF) on fundus autofluorescence (FAF) imaging at the SEVEN-UP visit. RESULTS Three of 41 eyes (7.3%) had macular atrophy on FA at baseline (mean ± standard deviation [SD] size: 0.29 mm(2) ± 1.50 mm(2)), 29 (70.7%) at SEVEN-UP (mean ± standard deviation [SD] area: 7.42 mm(2) ± 7.97 mm(2)). On FAF imaging at the SEVEN-UP visit, all 41 eyes (100%) had DDAF (mean ± SD size: 10.29 mm(2) ± 8.07 mm(2)). Variables significantly associated with area of DDAF at the SEVEN-UP visit were the area of leaking CNV lesion components (coefficient: 0.953; P < .001), the area of other lesion components (coefficient: 1.094; P = .038), and the area of retinal pigment epithelial (RPE) atrophy (coefficient: 1.334; P = .040) on baseline FA imaging. CONCLUSION The area of DDAF at more than 7 years after initiation of ranibizumab therapy was 35% larger than the original CNV lesion. The baseline area of leaking CNV and other components of the CNV lesion and the baseline area of RPE atrophy were important predictors of the area of definite decreased autofluorescence, presumably corresponding to areas of photoreceptor and RPE loss. The findings from this study may guide hypothesis generation for future AMD trials.

SUBRETINAL NEOVASCULARIZATION IN MACULAR TELANGIECTASIA TYPE 2: OPTICAL COHERENCE TOMOGRAPHIC ANGIOGRAPHY AND TREATMENT RESPONSE.

PURPOSE To report the optical coherence tomographic angiography findings and response to treatment in a case of macular telangiectasia Type 2 with subretinal neovascularization. METHODS Case report. RESULTS A 64-year-old man with macular telangiectasia Type 2 developed subretinal neovascularization, which was imaged on optical coherence tomographic angiography. He was treated with intravitreal aflibercept, and there was a remarkable reduction of flow in the subretinal neovascular network on optical coherence tomographic angiography. CONCLUSION Optical coherence tomographic angiography provides detailed information on the retinal microvasculature and subretinal neovascularization in macular telangiectasia Type 2. It can be used to assess response to treatment.

FUNDUS ALBIPUNCTATUS ASSOCIATED WITH CONE DYSFUNCTION.

Purpose: To describe a case of cone dysfunction associated with fundus albipunctatus. Methods: This report is an observational case report. The examination included multimodal imaging, electrophysiological recordings after standard and prolonged dark adaption, and disease targeted gene panel sequencing. Results: In this report, the authors present a 55-year-old Chinese male with findings on fundus examination, optical coherence tomography, and full-field electroretinography after standard and prolonged dark adaption consistent with fundus albipunctatus associated with cone dysfunction. Disease targeted gene panel sequencing revealed two heterozygous mutations in RDH5 (c.124C>T; p.Arg42Cys and c.500G>A; p.Arg167His). Conclusion: The authors report the case of a patient with ophthalmic findings characteristic for cone dysfunction in the setting of genetically confirmed fundus albipunctatus.

EN FACE OPTICAL COHERENCE TOMOGRAPHY OF MACULAR MICROCYSTS DUE TO OPTIC NEUROPATHY FROM NEUROMYELITIS OPTICA.

PURPOSE To describe the multimodal imaging, including en face optical coherence tomography (OCT) and OCT angiography, findings of a case of macular microcysts associated with neuromyelitis optica. METHODS Findings on clinical examination, color fundus photography, fluorescein angiography, fundus autofluorescence, visual fields, and OCT including en face OCT and OCT angiography are presented. RESULTS A 12-year-old African American boy presented with bilateral optic atrophy from neuromyelitis optica. Clinical examination was notable for bilateral optic nerve head pallor. Visual fields of both eyes showed generalized depression. Fluorescein angiography and fundus autofluorescence were unremarkable. Spectral domain OCT B-scan images showed characteristic paracentral, hyporeflective, microcystic lesions in the inner nuclear layer of both eyes, and en face OCT images demonstrated a corresponding pattern of large paracentral cysts radiating peripherally into smaller diffuse cysts. Optical coherence tomographic angiography of the superficial and deep retinal capillary plexuses was unremarkable. CONCLUSION Macular microcysts have been associated with various forms of optic atrophy, including neuromyelitis optica. Spectral domain and en face OCT imaging of the microcysts demonstrated a very characteristic pattern. Normal fluorescein and OCT angiography suggest that nonvascular causes, such as Müller cell degeneration, might contribute to the etiologic mechanism.

Development of Methodology and Study Protocol: Safety and Efficacy of a Single Subretinal Injection of rAAV.hCNGA3 in Patients with CNGA3-Linked Achromatopsia Investigated in an Exploratory Dose-Escalation Trial

Achromatopsia is an autosomal recessively inherited congenital defect characterized by a lack of cone photoreceptor function, leading to severely impaired vision. In this clinical study, achromatopsia patients were treated with a single subretinal injection of rAAV.hCNGA3 to restore cone function. The focus of this trial was on the safety of the treatment. After surgery, patients were monitored in eight extensive visits during the first year, followed by a 4-year follow-up period with annual visits. For essential complementation of the standard ophthalmological and systemic examinations, disease-specific methods were developed to assess the safety, efficacy, and patient-reported outcomes in this trial.