Faculty Bibliography

PURPOSE/OBJECTIVE:To analyze the spectrum of ischemia associated with paracentral acute middle maculopathy (PAMM) in eyes with acute retinal vascular occlusion and to describe an ischemic cascade. DESIGN/METHODS:A retrospective observational case series. METHODS:Patients presenting with PAMM secondary to acute retinal vascular occlusion were identified. Analysis of multimodal imaging was performed at baseline and at follow up visits to elucidate the patterns and progression of ischemia within the retinal layers. RESULTS:Multimodal retinal imaging from 16 eyes of 16 patients with acute retinal vascular occlusion associated with PAMM was studied. Analysis of en face OCT segmentation of the inner nuclear layer (INL) identified distinct patterns of PAMM correlating with the severity of ischemia and not the type of occlusion. A perivenular fern-like PAMM pattern was associated with better visual outcomes (average final visual acuity was 20/25). This pattern was noted to sequentially progress in 2 cases to a diffuse globular PAMM pattern in the INL, or to a pattern of ischemia involving both the middle and inner retinal layers with commensurate vision loss. Globular patterns of PAMM or ischemia involving both the middle and inner retina correlated with poorer visual outcomes (average final visual acuity was counting fingers at 5.5 ft). These various patterns of ischemia developed in eyes with retinal vascular occlusions in which blood flow through the retinal capillary plexuses was present but was significantly reduced and delayed. CONCLUSIONS:This study describes OCT findings suggestive of an ischemic cascade in eyes with retinal vascular occlusion. The middle retina at the level of the deep capillary plexus, especially at the venular pole, may be more vulnerable to ischemic injury.

The recent introduction of optical coherence tomography angiography (OCTA) has remarkably expanded our knowledge of the choroid through in vivo investigation of the anatomical and pathological features of this important vascular layer. New insights elucidating the morphological features of the choroid, in both physiological and pathological conditions, indicate that this vascular structure plays a crucial role in many chorioretinal disorders. In this article, a review of the salient histological and anatomical features of the choroid, essential for the proper interpretation of in vivo imaging, is followed by a discussion of the fundamental principles of OCTA and the application of this advanced imaging modality to study and understand the choroid. The current limitations of OCTA and potential advancements that may improve the widespread adoption of this tool are also discussed. A detailed review of the OCTA features of the choroid in the healthy eye is followed by relevant findings in major chorioretinal diseases, including age-related macular degeneration, central serous chorioretinopathy, uveitis, and inherited retinal disorders.

BACKGROUND AND OBJECTIVE/OBJECTIVE:To use quantitative fundus autofluorescence (qAF) to analyze different stages of non-neovascular age-related macular degeneration (AMD). PATIENTS AND METHODS/METHODS:In this cohort study, 38 pseudophakic patients and 36 age-matched controls participated. We performed near-infrared, spectral-domain optical coherence tomography and qAF imaging on 31 pseudophakic eyes and controls of participants older than 60 years with non-neovascular AMD phenotypes using the Spectralis HRA + OCT (Heidelberg Engineering, Heidelberg, Germany). RESULTS:The patients included in this study had a mean age of 83.9 years, and 35.7% patients were men. Mean qAF was higher in control participants than in all patients with AMD (P < .001). According to non-neovascular AMD phenotype, mean qAF levels were significantly lower in eyes with subretinal drusenoid deposits than in control eyes (P < .05). The lowest mean qAF was in patients with geographic atrophy. CONCLUSION/CONCLUSIONS:Quantitative fundus autofluorescence of non-neovascular AMD decreases from normal to early to late AMD, suggesting that loss of lipofuscin fluorophores, not increase, signifies AMD progression. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:S34-S42.].

PURPOSE/OBJECTIVE:To systematically characterize histologic features of multiple chorioretinal layers in eyes with geographic atrophy, or complete retinal pigment epithelium (RPE) and outer retinal atrophy, secondary to age-related macular degeneration, including Henle fiber layer and outer nuclear layer; and to compare these changes to those in the underlying RPE-Bruch membrane-choriocapillaris complex and associated extracellular deposits. METHODS:Geographic atrophy was delimited by the external limiting membrane (ELM) descent towards Bruch membrane. In 13 eyes, histologic phenotypes and/or thicknesses of Henle fiber layer, outer nuclear layer, underlying supporting tissues, and extracellular deposits at four defined locations on the non-atrophic and atrophic sides of the ELM descent were assessed and compared across other tissue layers, with generalized estimating equations and logit models. RESULTS:On the non-atrophic side of the ELM descent, distinct Henle fiber layer and outer nuclear layer became dyslaminated, cone photoreceptor inner segment myoids shortened, photoreceptor nuclei and mitochondria translocated inward, and RPE was dysmorphic. On the atrophic side of the ELM descent, all measures of photoreceptor health declined to zero. Henle fiber layer/outer nuclear layer thickness halved, and only Müller cells remained, in the absence of photoreceptors. Sub-RPE deposits remained, Bruch membrane thinned, and choriocapillaris density decreased. CONCLUSION/CONCLUSIONS:The ELM descent sharply delimits an area of marked gliosis and near-total photoreceptor depletion clinically defined as Geographic atrophy (or outer retinal atrophy), indicating severe and potentially irreversible tissue damage. Degeneration of supporting tissues across this boundary is gradual, consistent with steady age-related change and suggesting that RPE and Müller cells subsequently respond to a threshold of stress. Novel clinical trial endpoints should be sought at age-related macular degeneration stages before intense gliosis and thick deposits impede therapeutic intervention.This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

BACKGROUND/PURPOSE/OBJECTIVE:To report a case of persistent retinal toxicity associated with a high dose of sildenafil citrate intake. METHODS:Single retrospective case report. RESULTS:A 31-year-old white man with no medical history presented with complaints of bilateral multicolored photopsias and erythropsia (red-tinted vision), shortly after taking sildenafil citrate-purchased through the internet. Patient was found to have cone photoreceptor damage, demonstrated using electroretinogram, optical coherence tomography, and adaptive optics imaging. The patient's symptoms and the photoreceptor structural changes persisted for several months. CONCLUSION/CONCLUSIONS:Sildenafil citrate is a widely used erectile dysfunction medication that is typically associated with transient visual symptoms in normal dosage. At high dosage, sildenafil citrate can lead to persistent retinal toxicity in certain individuals.

PURPOSE/OBJECTIVE:To study zones of reduced inner choroidal flow signal, foci of reduced inner choroidal thickness, and pathologically dilated Haller layer vessels (pachyvessels) in eyes with pachychoroid disease using optical coherence tomography (OCT) and OCT angiography. METHODS:Patients with treatment-naive pachychoroid disease were recruited. All patients prospectively underwent swept-source OCT and OCT angiography. Zones of reduced choriocapillaris flow were labeled and enumerated. Areas where reduced flow signal was attributable to masking/artifacts were excluded. Regions of inner choroidal thinning were identified on structural OCT and labeled. Overlap between reduced choriocapillaris flow and structural inner choroidal attenuation was quantified using Jaccard indices. The relationship of reduced flow to pachyvessels was recorded. RESULTS:Twenty-four eyes of 19 patients were identified. All eyes exhibited at least one zone of reduced flow. A total of 146 flow signal attenuation zones were identified. Sixty-two (42%) of 146 zones showed overlap or proximity with structural inner choroidal thinning. The mean Jaccard index per eye was 0.10 (SD = 0.08). Pachyvessels were spatially related to 100 (68%) of 146 zones of flow attenuation. CONCLUSION/CONCLUSIONS:Zones of reduced choriocapillaris flow are prevalent in eyes with pachychoroid disease. Approximately 60% of these zones anatomically correlate with pachyvessels. Inner choroidal ischemia seems related to the pathogenesis of pachychoroid diseases.

PURPOSE: We describe with multimodal imaging the presentation and follow-up for a patient with idiopathic multifocal choroiditis and a transient peripapillary white ring. METHODS: Case report. RESULTS: A 39-year-old Asian woman was initially seen for an evaluation of lattice degeneration in 2015. Her medical history included Graves disease and psoriasis. Best-corrected visual acuity was 20/25 in her right eye and 20/25 in her left eye. Ultra-widefield fundus autofluorescence imaging showed a curvilinear hyperautofluorescent line in her right eye. One year later, the patient returned complaining of floaters in her right eye for 1 month. Her visual acuity was unchanged. Funduscopic examination showed new inflammatory yellowish lesions in the right eye corresponding to hyperreflective sub-retinal pigment epithelium lesions on structural spectral domain optical coherence tomography. Fluorescein angiography showed corresponding late staining of these active lesions. Late-phase indocyanine green angiography showed multiple nummular hypocyanescent dots. Ultra-widefield fundus autofluorescence showed large areas of hyperautofluorescence. The patient was started on a 60-mg oral prednisone taper and demonstrated subsequent regression of the inflammatory lesions. Ten months later, the patient returned emergently with complaints of floaters in both eyes for 2 days and a new temporal scotoma in her left eye. Funduscopic examination demonstrated a white ring around the optic nerve of the left eye corresponding to a hyperautofluorescent lesion. Ultra-widefield fundus autofluorescence showed new areas of hyperautofluorescence in both eyes. Structural spectral domain optical coherence tomography showed new sub-retinal pigment epithelium inflammatory lesions and a disruption of the ellipsoid zone in both eyes. The patient was again treated with a 60-mg oral prednisone taper and demonstrated subsequent restoration of the ellipsoid zone. CONCLUSION: To our knowledge, this is the first report of a transient annular white ring occurring in a case of multifocal choroiditis. There was marked restoration of the disrupted ellipsoid zone after treatment with oral corticosteroids.

PURPOSE: Acute syphilitic posterior placoid chorioretinitis (ASPPC) is an uncommon manifestation of ocular syphilis with distinct clinical features. We describe new multimodal imaging findings in a patient with ASPPC. METHODS: Observational case report with multimodal imaging. RESULTS: A 44-year-old woman presented with 5 days of decreased vision in her right eye. Visual acuity was counting fingers in her right eye and 20/20 in her left eye. Funduscopic examination of the right eye showed a yellow placoid macular lesion with extension beyond the equator, which was encircled by an annular ring of outer retinal whitening. Ultra-widefield fundus autofluorescence demonstrated hyperautofluorescence corresponding to the placoid lesion. Examination of the left eye appeared unremarkable, but ultra-widefield fundus autofluorescence showed an area of hyperautofluorescence located superonasal to the optic nerve. Optical coherence tomography of the right eye demonstrated subretinal fluid and overlying disruption of the ellipsoid zone. Fluorescein angiography demonstrated early hypofluorescent and hyperfluorescent spots and late staining within the placoid lesion. Optical coherence tomography angiography showed several areas of decreased flow signal within the placoid lesion at the level of the choriocapillaris. Laboratory testing revealed a rapid plasma reagin titer of 1:1,024. Two months after treatment with intravenous penicillin G, visual acuity had improved to 20/25 in her right eye, and optical coherence tomography showed partial restoration of the ellipsoid zone. The annular ring resolved with near normalization of fundus autofluorescence and optical coherence tomography angiography demonstrated resolution of flow. CONCLUSION: Multimodal imaging provides further insight into the pathogenesis of ASPPC. Ultra-widefield fundus autofluorescence may show evidence of ellipsoid zone disruption in areas that clinically appear normal. Flow voids within the choriocapillaris in ASPPC appear to resolve with appropriate treatment, a finding that suggests a transient disruption of choriocapillaris flow in ASPPC.

PURPOSE: To report a case of factitious self-inflicted handheld laser-induced maculopathy. METHODS: A 29-year-old man presented to our clinic complaining of a step-wise progressive loss of vision that abruptly began in his left eye but then became bilateral. He underwent comprehensive ocular examination, including visual acuity testing, biomicroscopic, dilated funduscopic examination, structural optical coherence tomography, en face structural optical coherence tomography, optical coherence tomography angiography, fundus autofluorescence, fluorescein angiography, and indocyanine green angiography. RESULTS: Visual acuity was 20/200 in both eyes. Although the multimodal imaging was highly consistent with handheld laser-induced maculopathy, the patient continued to deny this behavior. CONCLUSION: Self-inflicted handheld laser-induced maculopathy may be a novel manifestation of factitious disorder. Clinical suspicion for this should remain high in the presence of the signature multimodal retinal findings despite denial by the patient. This category of patients could benefit from psychiatric referral, to prevent further ocular or extra-ocular self-injury.

PURPOSE/OBJECTIVE:To investigate the association between choroidal caverns, choroidal vascular hyperpermeability (CVH), and pachyvessels in eyes with pachychoroid disease. METHODS:This was a retrospective review of swept-source optical coherence tomography and indocyanine green angiography imaging performed on eyes with pachychoroid disease. RESULTS:Imaging from 21 eyes with pachychoroid disease entities (8 eyes with pachychoroid pigment epitheliopathy, 11 eyes with central serous chorioretinopathy, and 3 eyes with pachychoroid neovasculopathy) from 11 patients (mean 49.5 years, male/female: 10/1, all white) was available for review. In all study eyes, pachyvessels traversed the areas of CVH visible in mid- and late-phase indocyanine green angiography. A total of 504 choroidal caverns were identified in 11 study eyes (52%). Of the 504 choroidal caverns, 445 (88%) were seen within the areas of CVH compared with 59 (12%), which were detected outside the areas of CVH (P < 0.001). Eyes with multiple caverns had an increased choroidal thickness when compared with eyes with ≤1 cavern (P < 0.001). CONCLUSION/CONCLUSIONS:Choroidal caverns, found primarily in the areas of indocyanine green angiography CVH traversed by pachyvessels, were detected in 52% of eyes with pachychoroid disease. The presence of choroidal caverns in these cases may indicate a loss of normal choroidal architecture associated with dilated Haller layer veins and increased choroidal thickness.