In vivo visualization of variable photoreceptor alteration in a case of peripapillary congenital hypertrophy of the retinal pigment epithelium using spectralis ® high magnification module.

PURPOSE: To analyze using the high magnification module (HMM) a case of peripapillary congenital hypertrophy of retinal pigment epithelium (CHRPE) and to correlate the findings to multimodal imaging and swept-source optical coherence tomography angiography (SS-OCTA, PLEX® Elite 9000, Carl Zeiss Meditec, Dublin, California, USA) imaging. OBSERVATIONS: A 57-year-old Caucasian woman presenting a peripapillary CHRPE of the left eye (LE) was examined using HMM and SS-OCTA, in addition to multimodal imaging. SS-OCTA disclosed the gradual changes, with four distinguishable zones: Zone 1 with complete outer retinal and retinal pigment epithelium (RPE) atrophy, Zones 2 and 3, corresponding to incomplete outer retinal (and RPE) atrophy presenting increased flow deficits, and normal choriocapillaris outside the lesion (Zone 4). High Magnification Module (HMM, Spectralis ®, Heidelberg Engineering) showed small polygonal hyperreflective outlines over the pigmented parts of the lesion (Zone 2), and partly over the narrow halo surrounding the lesion (Zone 3), with an absence of these outlines over the lacunae (Zone 1). CONCLUSIONS AND IMPORTANCE: HMM is a non-invasive imaging modality, allowing the in vivo visualization of a mosaic pattern, corresponding to the hyperreflective polygonal outlines, or absence thereof, in different zones of CHRPE.

Subretinal pigment epithelium fibrotic tissue morphological changes after a single anti-vascular endothelial growth factor injection in age-related macular degeneration.

AIMS: To demonstrate and evaluate the morphological changes of multilayered fibrovascular pigment epithelial detachment (PED) to a single anti-vascular endothelial growth factor (VEGF) injection in age-related macular degeneration (AMD). METHODS: We retrospectively analysed the morphological changes of 30 eyes with exudative AMD showing fibrotic multilayered PED, between two consecutive visits. All patients had one anti-VEGF intravitreal injection at the first visit. We quantitatively analysed the different compartments within the PED and their morphological response. RESULTS: The mean follow-up time interval between the first and the second visit was 32.46±4.64 days. We defined three optical coherence tomography zones within the PED: a subretinal pigment epithelium inhomogeneous hyporeflective space (layer 1), a hyper-reflective band beneath layer 1 (layer 2), and a hyporeflective space between the Bruch's membrane and layer 2 (layer 3). The mean height of layer 1 was 142±44.63 and 99.30±39.79 µm at visits 1 and 2, respectively. The mean thickness of layer 2 was 101.42±46.66 and 82.76±35.24 µm at visits 1 and 2, respectively. The mean height of layer 3 was 35.77±32.77 and 5.66±8.68 µm at visits 1 and 2, respectively (p=0.009). The mean height change for layer 1 was statistically significantly higher than for layer 2 (p=0.0002). CONCLUSIONS: Fibrovascular PED was compartmented into three layers with different reflectivities that morphologically responded differently to a single anti-VEGF injection. Layer 2 had a statistically significantly lower response compared with layer 1, suggesting the hypothesis of a fibrotic component in layer 2.

Vascular remodeling of choroidal neovascularization in older myopic patients treated with ranibizumab.

PURPOSE: To investigate morphological changes in myopic choroidal neovascularization (mCNV) using optical coherence tomography-angiography (OCT-A) after treatment with ranibizumab. METHODS: Retrospective analysis of consecutive patients over a 24-month period. All treatment-naïve mCNV were imaged at baseline with color pictures, spectral-domain OCT and OCT-A, and fluorescein angiography in selected cases. CNV morphology was classified at baseline and at 6 months. The CNV lesion surface was also compared. RESULTS: Twenty-nine patients with a mean age of 70.3 ± 10.1 years were included. They received a mean number of 2.65 injections over 6 months. Best-corrected visual acuity improved from 62.2 to 68.5 letters (p = 0.004), with regression of exudation in 24 eyes (82.7%). Baseline CNV was classified into tree-in-bud (16 eyes), medusa (9 eyes), or sea-fan (4 eyes) pattern. At 6 months, no abnormal blood flow was observed in CNV in 13 eyes. Eyes with complete regression or evolution towards an indistinct pattern showed more often a complete regression of exudation than eyes with unchanged pattern (p = 0.007). The mean CNV surface significantly decreased from 0.19 to 0.08 mm2 (p < 0.0001). CONCLUSION: An unchanged pattern was more often associated with exudation persistence, while a complete regression or evolution towards indistinct pattern was always associated with vascular inactivity. However, variable changes in mCNV were observed after anti-VEGF. Thus, OCT-A could be more useful in the diagnosis than in the follow-up of mCNV.

Comparison of Retinal and Choroidal Involvement in Sarcoidosis-related Chorioretinitis Using Fluorescein and Indocyanine Green Angiography.

PURPOSE: To compare the involvement of the retina with that of the choroid in ocular sarcoidosis (OS) using dual fluorescein angiography (FA)/indocyanine green angiography (ICGA). METHODS: A retrospective study of 23 patients with the diagnosis of OS was performed. Angiographic signs were quantified following the established FA/ICGA scoring system for uveitis. RESULTS: The choroid was predominantly involved in 19 (82.6%) patients or 87% (40/46) of the eyes, and the retina in 2 (8.7%) patients or 13% (6/46) of the eyes. The mean angiographic score was 7.15 ± 4.5 for the retina (FA) compared to 14.02 ± 4.86 for the choroid (ICGA) (P < 0.0001). In 13% (3/23) of patients, FA did not show retinal inflammation, whereas ICGA was strongly positive, revealing occult choroidal lesions. CONCLUSION: The choroid is preferentially involved in OS, for which ICGA is the examination of choice. There is a risk of underestimating the global ocular involvement and of missing choroidal involvement if only FA is used. FA/ICGA scoring system allows for quantitative assessment of inflammation in the posterior uvea that occurs in OS; therefore, the system can be useful to quantitatively monitor outcomes in clinical trials.

Optical Coherence Tomography Angiography of Type 2 Neovascularization in Age-Related Macular Degeneration.

Well-defined choroidal neovascularization, known as type 2 neovascularization (NV) or classic NV, is the least representative phenotype of exudative age-related macular degeneration. Clinical aspects of type 2 NV have been widely described in the literature, and to date fluorescein angiography remains the gold standard for imaging age-related macular degeneration at initial presentation. Optical coherence tomography angiography (OCT-A) can be used to image vessels based on flow characteristics without any dye injection. Type 2 NV can be visualized using OCT-A with very typical patterns. A neovascular membrane appears as either a medusa-shaped complex or a glomerulus-shaped lesion in the outer retina and the choriocapillaris layer. Furthermore, in the choriocapillaris layer, the external borders of the lesion appear as a dark ring in most cases, and one or more central feeder vessels that extend deeply into the more profound choroidal layers are visible. Identification of type 2 NV is easily feasible for any clinician using OCT-A, especially in areas where there are normally no vessels, like in subretinal space, if the interpretation rules are respected.

Optical coherence tomography angiography characteristics of polypoidal choroidal vasculopathy.

PURPOSE: To analyse the morphological characteristics of polypoidal choroidal vasculopathy (PCV) on optical coherence tomography angiography (OCT-A). METHODS: Prospective study with consecutive patients affected with PCV were included. All patients underwent a complete ophthalmological examination including fundus photography, fluorescein angiography, indocyanine green angiography, spectral-domain OCT and OCT-A. RESULTS: Twelve eyes of 12 patients (mean age 72.6±10.5 years; 4 men and 8 women) were included for analysis. In all eyes (12/12) the segmentation of the choriocapillaris layer on OCT-A revealed the branching vascular network (BVN) as a hyperflow lesion. OCT-A segmentation of the choriocapillaris layer in correspondence of the polypoidal lesion showed in 3/12 eyes (25%) a hyperflow round structure, surrounded by a hypointense halo, and in 9/12 eyes (75%) a hypoflow round structure. CONCLUSIONS: The OCT-A is a non-invasive imaging modality allowing the visualisation of different structures in PCV. The BVN is constantly clearly detected. The hypoflow round structure appearance of the polyp in OCT-A, is probably due to an unusual blood flow inside the polypoidal lesions, contrasting with the BVN. Further improvement in OCT-A knowledge will provide information on the specificity of the different intensity characteristics in PCV.

Optical Coherence Tomography Angiography in Central Serous Chorioretinopathy.

Purpose. To analyze optical coherence tomography angiography (OCTA) findings in eyes with central serous chorioretinopathy (CSC) and to compare them with those obtained with multimodal imaging. Methods. A series of consecutive patients diagnosed with CSC, underwent OCTA and multimodal imaging, including spectral domain OCT, fluorescein, and indocyanine green angiography. OCTA images were performed at three main depth intervals: automatically segmented outer retina, manually adjusted outer retina, and automatically segmented choriocapillaris. Results. Thirty-three eyes of 32 consecutive patients were analyzed. OCTA showed 3 main anomalies at the choriocapillaris: the presence of dark areas (19/33 eyes) which were frequently associated with serous retinal detachment, presence of dark spots (7/33 eyes) which were frequently associated with retinal pigment epithelium detachment, and presence of abnormal vessels (12/33 eyes) which were frequently, but not systematically, associated with choroidal neovascularization, as confirmed by multimodal imaging. Conclusions. OCTA revealed dark areas and dark spots, which were commonly observed. An abnormal choroidal pattern was also observed in one-third of cases, even when multimodal imaging did not evidence any choroidal neovascularization. Abnormal choroidal vessels should be interpreted with caution, and we could assume that this pathological choroidal vascular pattern observed in many CSC cases could be distinct from CNV.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FEATURES OF SUBRETINAL FIBROSIS IN AGE-RELATED MACULAR DEGENERATION.

PURPOSE: To report the imaging features of subretinal fibrosis secondary to exudative age-related macular degeneration (AMD) on optical coherence tomography angiography. METHODS: All consecutive patients diagnosed with subretinal fibrosis complicating exudative AMD were imaged by color retinal photographs or multicolor imaging, fluorescein angiography, spectral domain optical coherence tomography, and optical coherence tomography angiography. Eyes with active exudative features observed during the last 6 months were compared with those without any sign of exudation >6 months. RESULTS: Forty-nine eyes of 47 consecutive patients were included. A blood flow inside the fibrotic scar could be detected in 46 of 49 cases (93.8%). Three patterns of vascular networks could be distinguished, that were described as pruned vascular tree (26 of 49 eyes; 53.1%), tangled network (14 of 49; 28.6%), and/or vascular loop (25 of 49; 51.0%). Furthermore, 2 types of hyporeflective structures, large flow void, and/or dark halo were observed in 63% and in 65% of eyes, respectively. The observed patterns did not differ between eyes with active or inactive lesions. CONCLUSION: Optical coherence tomography angiography of subretinal fibrosis showed almost constantly a perfused, abnormal vascular network and collateral architectural changes in the outer retina and the choriocapillaris layer. These features were associated with both active and inactive fibrotic choroidal neovessels.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN EARLY TYPE 3 NEOVASCULARIZATION.

PURPOSE: To report the imaging features of Type 3 neovascularization secondary to exudative age-related macular degeneration on optical coherence tomography angiography (OCTA). METHODS: All consecutive treatment-naive patients diagnosed with early-stage Type 3 neovascularization underwent imaging by color retinal photographs or multicolor imaging, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography, and OCTA. The OCTA features were analyzed and correlated with the findings of conventional angiography and spectral domain optical coherence tomography. RESULTS: A total of 18 treatment-naive eyes of 18 consecutive patients (13 females and 5 males; mean age 81.3 ± 6.0) were included in the analysis. Optical coherence tomography angiography showed lesions characterized by a retinal-retinal anastomosis that emerged from the deep capillary plexus, forming in all 18 eyes a clear tuft-shaped high-flow network in the outer retinal segmentation, finally abutting in the subretinal pigment epithelium space. In 15 of 18 eyes, in the choriocapillaris segmentation, there appeared a small clew-like lesion, which in 2 cases seemed connected with the choroid through a small caliber vessel. CONCLUSION: Optical coherence tomography angiography of treatment-naive Type 3 neovascularization showed almost constantly a high-flow, tuft-shaped abnormal outer retinal proliferation, frequently associated to a small clew-like lesion in the choriocapillaris layer.

TYPE 2 NEOVASCULARIZATION SECONDARY TO AGE-RELATED MACULAR DEGENERATION IMAGED BY OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: Optical coherence tomography angiography is a novel and noninvasive technique for imaging retinal microvasculature by detecting changes in reflectivity that is related to blood flow. The purpose of this study was to describe Type 2 neovascularization characteristics in age-related macular degeneration using optical coherence tomography angiography. METHODS: Fourteen eyes of 14 consecutive patients with Type 2 neovascularization were prospectively included. All patients underwent a complete ophthalmological examination, including color and infrared fundus photography, fluorescein and indocyanine green angiography, spectral domain optical coherence tomography angiography, and optical coherence tomography angiography. RESULTS: In all cases, Type 2 lesions could be detected by optical coherence tomography angiography, presenting as a hyperflow lesion in the outer retina, with a glomerulus (4/14) or medusa shape (10/14), surrounded by a dark halo. The superficial layer and the deep retina showed no abnormal flow. Surprisingly, the Type 2 lesions could also be observed in the presumed choriocapillaris layer. These glomerulus- or medusa-shaped lesions were connected, in 10/14 eyes, to a thicker main branch, which seemed to continue deep into the choroidal layers. CONCLUSION: Optical coherence tomography angiography may be a new imaging method for the diagnosis of Type 2 neovascularization in clinical routine. However, the specificity of the features needs to be investigated in further studies.