Olivia Zambrowski

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 adult-onset foveomacular vitelliform dystrophy

Purpose To describe structural features of eyes with adult-onset foveomacular vitelliform dystrophy (AFVD) on optical coherence tomography angiography (OCT-A) and to evaluate the ability to detect the presence of choroidal neovascularisation (CNV). Methods Consecutive patients presenting at the University Eye Clinic of Creteil with diagnosis of AFVD were included. All patients underwent a complete ophthalmological examination, including fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography and OCT-A by Optovue RTVue XR Avanti. Results Twenty-two eyes of 18 consecutive patients (8 women and 10 men; 68±12.8 years) were included. On OCT-A the presence of subretinal material leads to displacement of blood vessels at both the superficial and deep capillary plexuses of the retina. In one case, these vascular abnormalities were associated with long filamentous vessels running thorough the foveal avascular area. In all cases, a rarefaction of the choriocapillaris was also observed. In two eyes OCT-A distinctly disclosed the presence of a CNV secondary to AFVD while conventional imaging did not show clearly the neovascularisation due to masking effect of the subretinal vitelliform material. Conclusions In patients with AFVD, OCT-A showed vascular network rarefaction with less blood vessels at the superficial and deep capillary plexuses, and the choriocapillaris layer. These vascular abnormalities may play a role in the pathogenesis or simply represent a consequence of material accumulation and reabsorption in AFVD. In two cases, the conventional imaging did not show clearly the neovascularisation due to masking effect of the subretinal vitelliform material, while OCT-A showed distinctly the CNV.

Optical Coherence Tomography Angiography Changes In Early Type 3 Neovascularization After Anti-vascular Endothelial Growth Factor Treatment.

Purpose: To investigate the morphologic changes on optical coherence tomography angiography (OCTA) of treatment-naive Type 3 neovascularization secondary to exudative age-related macular degeneration after 1 year of anti–vascular endothelial growth factor therapy. Methods: Consecutive patients diagnosed with treatment-naive early-stage Type 3 neovascularization were enrolled in this retrospective study. All patients underwent color fundus photographs/MultiColor (Heidelberg Engineering) imaging, fluorescein angiography, indocyanine green angiography, structural spectral domain OCT, and OCTA Optovue RTVue XR Avanti (Optovue) at baseline, and repeated OCTA and structural spectral domain OCT at Month 12. Qualitative analysis of the 3 × 3 OCTA examinations at baseline and Month 12 was then compared, to assess changes after anti–vascular endothelial growth factor therapy. Results: A total of 15 treatment-naive eyes of 15 consecutive patients were included in the analysis. At 12-month follow-up after pro-re-data anti–vascular endothelial growth factor therapy (5.75 ± 1.48 injections of ranibizumab, and injections of 6.33 ± 1.21 of aflibercept), OCTA demonstrated persistence of the deep capillary plexus abnormalities in 13/15 eyes. In the outer retina and choriocapillaris, the initial lesion became undetectable in 7/15 cases, accompanied by choriocapillaris atrophy. The abnormal vascular complex persisted in the form of a tuft-shaped lesion in the outer retinal segmentation in 9/15 eyes, which in the choriocapillaris segmentation was associated with sub–retinal pigment epithelium neovascularization in 8 cases. Conclusion: Optical coherence tomography angiography showed that the tuft-shaped abnormal outer retinal lesion, frequently associated with a small clew-like flow signal in the choriocapillaris, after 1 year of anti–vascular endothelial growth factor therapy, either becomes undetectable or develops sub–retinal pigment epithelium neovascularization.

Visual Function in Asymptomatic Patients With Homozygous Sickle Cell Disease and Temporal Macular Atrophy

Importance Temporal macular involvement in sickle cell disease can now easily be detected by optical coherence tomography (OCT). However, while recent studies have demonstrated its high prevalence, little is known about its potential consequences on visual function. Objective To assess the visual function of patients with sickle cell disease with no visual symptoms despite temporal macular atrophy. Design, Setting, and Participants This retrospective case series included data collection and explorations made in a single referral center for sickle cell disease in 2016. Three patients with sickle cell disease exhibiting preserved visual acuity but showing temporal macular retinal atrophy were included. Exposures Patients underwent the following explorations: best-corrected distance and near visual acuity evaluation; dilated fundus examination; OCT with 12 × 6-mm thickness map; horizontal, vertical, and en face sections; OCT angiography of the 6 × 6-mm perifoveal retina; 30° and 12° central visual fields; Lanthony 15-hue color vision test; automated static contrast sensitivity test; and global electroretinography. Main Outcomes and Measures The OCT thickness maps were checked for areas of retinal thinning, appearing as blue patches. When present, these areas were compared with the areas of superficial and deep capillary flow loss on OCT angiography and with the scotomas on visual fields. Contrast sensitivity and color vision loss were quantified. Results All 3 patients included had homozygous sickle cell disease. They presented with a 20/20 distance visual acuity, and Parinaud 1,5 near visual acuity in both eyes. They were all followed up for a severe cerebral vasculopathy related to sickle cell disease. The areas of atrophy involved the inner retinal layers and were associated with an absence of signal in the deep capillary plexuses in OCT angiography. These patches of retinal thinning were also matching with scotomas in the automated visual fields. Color vision ability and contrast sensitivity were impaired in all patients. Global electroretinography findings were normal. Conclusions and Relevance Temporal macular atrophy in sickle cell disease may have direct consequences on visual function, including in children, even when visual acuity is preserved. Optical coherence tomographic imaging may be warranted when evaluating patients with sickle cell disease, even if asymptomatic with 20/20 visual acuity.

Electroretinogram Findings in Early-Stage Sickle Cell Retinopathy According to Hemoglobin Type

Purpose Although extensive clinical research has been performed on structural analysis of sickle cell (SC) retinopathy, functional aspects have been poorly investigated. Our purpose was to report full-field electroretinogram (ffERG) findings in patients with early SC retinopathy according to the following hemoglobin types: HbSS or HbSC (homozygous or heterozygous mutations, respectively). Methods In this monocentric retrospective observational study, patients affected by nonproliferative SC retinopathy were included from November 2014 to April 2016. Patients were separated into one of the following three groups: HbSS, HbSC, and control. All groups underwent full ophthalmologic examination (fundus examination) and ffERG. For SC patients, additional imaging testing was also performed (fluorescein angiography and spectral domain optical coherence tomography). Results A total of 24 eyes from 12 patients (6 HbSS and 6 HbSC) and 12 eyes from 6 controls were included. The HbSS group exhibited a dramatic decrease of the b-wave amplitudes for all dark-adapted (DA) ffERG responses when compared with the control group (P = 0.02, P = 0.003, P = 0.005, respectively, after DA 0.01, DA 3.0, and DA 10.0 cd.s.m-2 stimulations) and decreased a-wave amplitudes for light-adapted responses (P = 0.03 after light-adapted 3.0 cd.s.m-2 stimulations). The a-Wave amplitudes were significantly reduced for all dark-adapted and light-adapted responses in HbSC group compared to the control group (P = 0.03, P = 0.01, P = 0.03, respectively, after DA 3.0, DA 10.0, and light-adapted 3.0 cd.s.m-2 stimulations). The HbSS+HbSC groups presented decreased a-wave amplitudes for DA and light-adapted responses and decreased b-wave amplitude after DA 0.01 and 10.0 cd.s.m-2 stimulations when compared to the control group. Conclusions These results could suggest an early involvement of the inner retinal cells in the disease process in HbSS patients and of the outer retinal cells in HbSC patients. This could provide new insights on the pathophysiology of the retinal affection in HbSS/HbSC SC disease.

Circadian disturbance and idiopathic central serous chorioretinopathy.

BackgroundThis present retrospective case control study was designed to evaluate circadian disturbance in patients with chronic idiopathic central serous chorioretinopathy (ICSC).MethodsBetween January 1st, 2012, and November 30th, 2014, 29 consecutive patients with chronic ICSC examined in a referral setting were compared with a gender-matched and age-matched control group of 29 patients. A history of pharmacologic medication (including corticosteroid treatment), sleep disturbance, irregular working hours, cardiovascular risk factors, and depressive anxiety disorders was noted.ResultsThe median age of the patients was 52, and in the control subjects it was 50. The male–female ratio for both groups was 4.8:1. Patients with chronic ISCS were more likely to be exposed to irregular working hours (p < 0.01, OR 9.3 [2.29–37.6]) and to present with overweight than the control subjects (p = 0.016). No significant differences were found for sleeping disturbances, pharmacological medication, cardiovascular risk factors, or depressive anxiety disorders.ConclusionsIn this preliminary study, the exposition of irregular working hours as a risk factor for chronic ICSC was identified, which had not been previously reported. If further studies confirm these findings, then employment with regular working hours could be recommended for chronic ICSC patients.

Dietary, environmental, and genetic risk factors of Extensive Macular Atrophy with Pseudodrusen, a severe bilateral macular atrophy of middle-aged patients

EMAP (Extensive Macular Atrophy with Pseudodrusen) is a maculopathy we recently described that shares pseudodrusen and geographic atrophy with Age-related Macular Disease (AMD). EMAP differs from AMD by an earlier age of onset (50-55 years) and a characteristic natural history comprising a night blindness followed by a severe visual loss. In a prospective case-control study, ten referral centers included 115 EMAP (70 women, 45 men) patients and 345 matched controls to appraise dietary, environmental, and genetic risk factors. The incidence of EMAP (mean 2.95/1.106) was lower in Provence-Côte d’Azur with a Mediterranean diet (1.9/1.106), and higher in regions with intensive farming or industrialized activities (5 to 20/1.106). EMAP patients reported toxic exposure during professional activities (OR 2.29). The frequencies of common AMD complement factor risk alleles were comparable in EMAP. By contrast, only one EMAP patient had a rare AMD variant. This study suggests that EMAP could be a neurodegenerative disorder caused by lifelong toxic exposure and that it is associated with a chronic inflammation and abnormal complement pathway regulation. This leads to diffuse subretinal deposits with rod dysfunction and cone apoptosis around the age of 50 with characteristic extensive macular atrophy and paving stones in the far peripheral retina.

DIFFUSE LARGE B-CELL LYMPHOMA-ASSOCIATED-RETINOPATHY CHARACTERIZED BY MINIMAL MORPHOLOGIC CHANGES AND SEVERE FUNCTIONAL IMPAIRMENT.

Purpose: To report the case of a patient whose retinal disease was found to be associated with a diffuse large B-cell lymphoma found 30 years after the apparent successful treatment of a classical Hodgkin lymphoma. Methods: Observational case report. Results: The authors describe the case of a 69-year-old man referred to their Department because of progressive, bilateral vision loss over the last few months. Deterioration in color vision and intense photophobia were also present. His best-corrected visual acuity was 20/400 in the right eye (RE) and 20/800 in the left eye (LE). Slit lamp and fundus examination failed to show any abnormalities. Spectral domain optical coherence tomography (SD-OCT) detected diffuse attenuation of the ellipsoid layers in addition to a focal subfoveal defect in both eyes. Both fluorescein and indocyanine angiographies (FA and ICGA) were normal. Full flash electroretinogram (ERG) revealed bilateral cone rod dysfunction with decreased amplitudes of both a and b waves. Conclusion: Because of the late onset of the disease, poor visual acuity compared with a small macular anatomical lesion and a history of Hodgkin lymphoma 30 years ago, a neoplastic etiology was investigated. Poor performance status and chest pain led to a thoracic CT scan, which identified a massive mediastinal tumor. Serum analysis found an abnormal amount of antibody activity within the 40 kD region of Western blot of retina. The diagnosis of diffuse large B-cell lymphoma was established. Systemic examinations found a Stage IV non-Hodgkin lymphoma.

Origins of ghost drusen: a follow-up analysis

Purpose Ghost drusen (GD) are pyramidal or dome-shaped retinal pigment epithelium elevations observed in some geographic atrophy (GA) areas in the context of age-related macular degeneration (AMD). The purpose was to investigate the first morphologic features preceding GD on spectral-domain optical coherence tomography (SD-OCT) on patients with GA associated with AMD. Methods A retrospective observational study was performed on a series of patients with GA that had at least 3 years of follow-up. Using the follow-up tool of SD-OCT, we tracked the initial lesions that could lead to GD. Results Among 442 patients with GA, 37 had well defined GD (8%). We included the 17/37 patients (31 eyes) with at least 3 years of follow-up for analysis, which led to a total of 582 counted GD. Most GD were already present at the first visit, and remained stable. However, on 13 of the 582 analyzed GD (2.2%), soft drusen were shown as the initial lesion, which progressively turned into GD. Conclusions GD were observed in less than 10% of eyes with GA. None of the ghost drusen turned into another shaped lesion, suggesting that GD is a possible final stage of evolution. In a few cases, large drusen were shown as the primary lesion that progressed into GD.