Faculty Bibliography

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.

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: 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: 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: 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/OBJECTIVE:In an eye with macular atrophy due to age-related macular degeneration (AMD; complete retinal pigment epithelium (RPE) and outer retinal atrophy (cRORA)), we correlated in vivo imaging to histology to characterize the atrophy border and evaluate the utility of optical coherence tomography (OCT) metrics suggested by previous histology. DESIGN/METHODS:Case study with clinicopathologic correlation. METHOD/METHODS:In vivo eye-tracked cross-sectional OCT scans at 13 and 8 months before death were compared to post-mortem histopathology. On OCT, the atrophy border was identified as either the descent of the external limiting membrane (ELM) towards Bruch's membrane (BrM) (representing gliosis), or the presence of choroidal hypertransmission (representing lack of shadowing by RPE). Thicknesses of RPE, basal laminar deposit (BLamD), and BrM was measured at 500 and 100 μm on the non-atrophic and atrophic sides of these borders, on in vivo eye-tracked OCT and histology matched to the same location. RESULTS:In all OCT scans, the ELM descent was visible. The RPE-BLamD band significantly thickened towards it (p<0.005), over time (p=0.015 and 0.043, at 500 and 100 μm, respectively). On OCT, the ELM descent delineated a smaller atrophic area than did hypertransmission. RPE-BLamD thicknesses manually measured on OCT overestimated histological thicknesses. BrM visibility varied with RPE status. CONCLUSION/CONCLUSIONS:Visible on OCT, the ELM descent is a histopathologic atrophy border supporting new terminology of cRORA, whereas hypertransmission reveals RPE degeneration. RPE-BLamD thickening across the macula, towards the atrophy and over time is confirmed. The presence of gliosis and thick RPE-BLamD suggests that macular atrophy is a late stage in disease progression, encouraging anatomical endpoints at earlier AMD stages, rather than atrophy enlargement.

PURPOSE: To describe the features of peripapillary pachychoroid syndrome (PPS), a novel pachychoroid disease spectrum (PDS) entity. METHODS: Medical records of 31 eyes (16 patients) with choroidal thickening associated with intraretinal and/or subretinal fluid in the nasal macula extending from the disk were reviewed (patients with PPS). Choroidal thickness was compared with 2 age-matched cohorts: typical PDS (17 eyes with central serous chorioretinopathy or pachychoroid neovasculopathy) and 19 normal eyes. RESULTS: The patients with PPS were 81% men aged 71 +/- 7 years. Peripapillary pachychoroid syndrome eyes displayed thicker nasal versus temporal macular choroids, unlike PDS eyes with thicker temporal macular choroids (P < 0.0001). Peripapillary intraretinal and/or subretinal fluid was often overlying dilated Haller layer vessels (pachyvessels). Fundus autofluorescence and fluorescein angiography illustrated peripapillary pigmentary mottling without focal leakage. Most PPS eyes (70%) exhibited other PDS findings including serous pigment epithelial detachment or gravitational tracks. Indocyanine green angiography illustrated dilated peripapillary pachyvessels and choroidal hyperpermeability. The disk was usually crowded, with edema noted in 4/31 (13%) eyes and mild late fluorescein disk leakage identified in half of the cases. Choroidal folds (77%), short axial lengths (39% less than 23 mm), and hyperopia (86%) were common. CONCLUSION: Peripapillary pachychoroid syndrome is a distinct PDS variant, in which peripapillary choroidal thickening is associated with nasal macular intraretinal and/or subretinal fluid and occasional disk edema. Recognition of PPS is important to distinguish it from disorders with overlapping features such as posterior uveitis and neuro-ophthalmologic conditions.

PURPOSE/OBJECTIVE:To investigate the utility of optical coherence tomography angiography (OCTA) for detecting pathologic vascularization within pigment epithelial detachments (PEDs). METHODS:This was a retrospective, cross-sectional, consecutive case series. Multimodal imaging (structural OCT, fluorescein, and indocyanine green angiography) was used as the gold standard to classify PEDs as nonvascularized or vascularized. Optical coherence tomography angiography imaging of the PED was subsequently and independently evaluated to classify PEDs as vascularized or nonvascularized. Specifically, OCTA images were evaluated for the presence of abnormal flow on cross-sectional OCTA and the presence of a vascular complex on en face OCTA. Comparisons between OCTA and the gold standard were determined. RESULTS:Sixty-four eyes of 49 patients were evaluated. A total of 18 eyes were classified as nonvascularized PED, and 46 eyes were classified as vascularized PED using the gold standard. Optical coherence tomography angiography was found to have a sensitivity of 76%, specificity of 61%, positive predictive value of 83%, and negative predictive value of 50% for detecting vascularized PEDs. False positive cases in the nonvascularized PED group were due to projection or flow artifacts from hyperreflective material overlying the PED. False negative cases were seen in eyes with minimal exudation on structural OCT and also those manifesting retinal pigment epithelial tears. CONCLUSION/CONCLUSIONS:Our proposed two-step approach of OCTA interpretation, first using cross-sectional OCTA and then en face OCTA, may allow the detection of vascularization within PEDs and, in some cases, reduce the need for conventional angiography. Increased awareness about potential artifacts and limitations of OCTA may help clinicians interpret OCTA more accurately.

Optical coherence tomography (OCT) images semi-transparent tissues noninvasively. Relying on backscatter and interferometry to calculate spatial relationships, OCT shares similarities with other pulse-echo modalities. There is considerable interest in using machine learning techniques for automated image classification, particularly among ophthalmologists who rely heavily on diagnostic OCT. Artificial neural networks (ANN) consist of interconnected nodes and can be employed as classifiers after training on large datasets. Conventionally, OCT scans are rendered as 2D or 3D human-readable images of which the smallest depth-resolved unit is the amplitude-scan reflectivity-function profile which is difficult for humans to interpret. We set out to determine whether amplitude-scan reflectivity-function profiles representing disease signatures could be distinguished and classified by a feed-forward ANN. Our classifier achieved high accuracies after training on only 24 eyes, with evidence of good generalization on unseen data. The repertoire of our classifier can now be expanded to include rare and unseen diseases and can be extended to other disciplines and industries.