Faculty Bibliography

PURPOSE/OBJECTIVE:To describe the recovery timeline of spots and dots in multiple evanescent white dot syndrome. METHODS:Sequential multimodal retinal imaging including fundus autofluorescence and cross-sectional and en face optical coherence tomography was performed to track the development and resolution of spots and dots in a case of multiple evanescent white dot syndrome. RESULTS:En face optical coherence tomography showed that the spots are the result of ellipsoid zone loss and are hyperautofluorescent due to unmasking of the underlying retinal pigment epithelium autofluorescence. Conversely, the dots are hyperreflective with cross-sectional and en face optical coherence tomography and hyperautofluorescent, which we propose may be due to accumulation of degenerated photoreceptor material including fluorophores with autofluorescent capability such as precursors of A2E. The earlier resolution of the hyperautofluorescent spots allowed for later detection of the hyperautofluorescent dots. CONCLUSION/CONCLUSIONS:This case report illustrates the different recovery timelines of spots and dots in multiple evanescent white dot syndrome. Although both lesion types are hyperautofluorescent, the mechanism of autofluorescence is distinctive and may be explained by their contrasting pathoanatomy.

INTRODUCTION/UNASSIGNED:White dot syndromes are a heterogeneous group of diseases that affect different layers in the retina and choroid. Multimodal imaging is fundamental in the diagnosis, but also can be crucial in unveiling the pathogenesis of these entities. MATERIAL AND METHODS/UNASSIGNED:Literature review. RESULTS/UNASSIGNED:Optical coherence tomography (OCT) provides depth-resolved, histological grade images of the vitreous, retina, and choroid. This technology is very useful to localize the primary nature and level of pathology of the various white dot syndromes. En face OCT can provide additional information regarding the interrelationship of lesion types. Vascular involvement at the level of the retina, choriocapillaris or choroid can be assessed by en face OCT angiography (OCT-A) and is not limited by masking, leakage or staining as can occur with conventional angiography (fluorescein or indocyanine green angiography) which requires dye injection. CONCLUSION/UNASSIGNED:OCT and OCTA are fundamental in the diagnosis and follow-up of white dots syndromes.

PURPOSE/OBJECTIVE:To determine the prevalence and characteristics of multifocal choroiditis/punctate inner choroidopathy (MFC/PIC) in eyes with patchy atrophy due to pathologic myopia (PM). METHOD/METHODS:Five hundred eyes of 253 patients with patchy atrophy were examined between 2014 and 2020 at the Advanced Clinical Center for Myopia. The main outcome measures included the prevalence and characteristics of active MFC/PIC lesions diagnosed by optical coherence tomography (OCT). RESULTS:Fifty-five of the 500 eyes (11%) diagnosed with patchy atrophy had OCT features of active MFC/PIC lesions such as focal elevations of the RPE filled with medium hyperreflectivity material, curvilinear scars (Schlaegel lines), and/or areas of outer retinal atrophy. At the time when the MFC/PIC was diagnosed, the mean age was 57.3±12.0 years, and the mean axial length was 29.2±1.8 mm. Macular neovascularization (MNV) was found in 45 of eyes (81.8%) with MFC/PIC vs 151 eyes without such findings (33.9%; P <0.001). In 25 of the 55 eyes (45.5%), active MFC/PIC lesions were found before the development of the patchy atrophy. The Bruch's membrane defects were co-located with these lesions. CONCLUSIONS:Active MFC/PIC lesions were identified in a minority of eyes with PM, and a subset of these lesions were observed to progress to findings indistinguishable from myopic patchy atrophy. Evidence of MFC/PIC in eyes with PM appeared to be a risk factor for the development of MNV.

PURPOSE/OBJECTIVE:To investigate a possible microvascular component of poppers maculopathy (PMP) using optical coherence tomography angiography (OCTA). METHODS:Twelve patients suffering from poppers maculopathy were included. Health records, optical coherence tomography (OCT), and OCTA data was gathered and compared to a healthy control group (HC). PMP lesion type was determined by manifestation in OCT. OCTA-based evaluation of retinal vascular plexus and choriocapillaris (CC) was executed. Vessel density (VD) and vessel length density (VLD) in superficial and deep capillary plexus (SCP, DCP), as well as flow deficits (FD), within the foveal avascular zone (FAZ) in CC were assessed. RESULTS:Median age of PMP patients was 40 (min 24; max 64) years, all male. Eleven patients presented with ellipsoid zone-type lesions; one patient showed a vitelliform-type lesion. No qualitative microvascular changes between PMP patients and HC were identified. Quantitative values for VD and VLD of SCP and DCP did not differ in between the two groups. The analysis of FDs in CC showed no deviation from PMP patients to HC. CONCLUSIONS:No vascular anomalies in qualitative and quantitative analysis in OCTA were detected in PMP patients. The constitution of the CC within FAZ of PMP patients does not differ from HC when assessed as FD.

BACKGROUND/AIMS/OBJECTIVE:To analyse the long-term anatomic and visual outcomes of patients with peripapillary pachychoroid syndrome (PPS), a recently described entity in the pachychoroid disease spectrum. METHODS:This study retrospectively included patients from several retina centres worldwide. Visual acuity (VA), retinal thickness and choroidal thickness at baseline, 6 months and final follow-up were assessed. Temporal trends in VA and anatomic characteristics were evaluated. Visual and anatomic outcomes in eyes that were observed versus those that were treated were analysed. RESULTS:Fifty-six eyes of 35 patients were included with mean follow-up of 27±17 months. Median VA was 20/36 at baseline and remained stable through follow-up (p=0.77). Retinal thickness significantly decreased subfoveally (p=0.012), 1.5 mm nasal to the fovea (p=0.002) and 3.0 mm nasal to the fovea (p=0.0035) corresponding to areas of increased thickening at baseline. Choroidal thickness significantly decreased subfoveally (p=0.0030) and 1.5 mm nasal to the fovea (p=0.0030). Forty-three eyes were treated with modalities including antivascular endothelial growth factor injection, photodynamic therapy, and others. VA remained stable in treated eyes over follow-up (p=0.67). An isolated peripapillary fluid pocket in the outer nuclear layer was characteristic of PPS. CONCLUSION/CONCLUSIONS:Patients with PPS experienced decreased retinal oedema and decreased choroidal thickening throughout the course of disease. While some patients experienced visual decline, the overall visual outcome was relatively favourable and independent of trends in retinal or choroidal thickening.

PURPOSE/OBJECTIVE:To describe clinical features including multimodal imaging in a rare case of presumed embolic cilioretinal artery occlusion with long-term follow-up examination. METHODS:A 61-year-old man presented after noticing an acute-onset central scotoma in the left eye during coronary arteriography. Fundus color photography, red-free imaging, near-infrared reflectance, spectral domain optical coherence tomography (OCT), fluorescein angiography, and swept-source OCT angiography were performed. RESULTS:Color and red-free fundus imaging both showed retinal whitening in the territory corresponding to a cilioretinal artery in the left eye. Consistent with an ischemic event, the near-infrared reflectance showed a hyporeflective area in the same location. Spectral domain OCT showed hyperreflectivity and generalized thickening of the inner nuclear layer, ganglion cell layer, and retinal nerve fiber layer. Seven years later, color, red-free, and near-infrared reflectance imaging appeared normal, but the spectral domain OCT showed thinning of all retinal layers in the affected area. Furthermore, swept-source OCT angiography demonstrated markedly reduced capillary flow signal in the superficial and deep capillary plexuses, corresponding to a persistent scotoma described by the patient. CONCLUSION/CONCLUSIONS:This case report illustrates hallmark features of a rare presumed embolic cilioretinal artery occlusion and its long-term manifestations with thorough historical and multimodal imaging data. When standard fundus imaging with color, red-free, and near-infrared reflectance appears normal years after a retinal vascular insult, OCT and OCT angiography can corroborate a patient's persistent visual deficit and provide clarity to the diagnosis.

PURPOSE/OBJECTIVE:To report a very late recurrence of choroidal neovascularization (CNV) in elderly patients with noninfectious multifocal choroiditis (MFC). METHODS:Retrospective case series of patients with MFC with confirmed recurrence of CNV. Choroidal neovascularization was diagnosed with multimodal imaging, including optical coherence tomography angiography. Multifocal choroiditis-associated CNV eyes were treated with intravitreal injections of anti-vascular endothelial growth factor medication. RESULTS:Four eyes of three patients were included in our study, with a mean (range) age of 73 years (67-78). The period between the original CNV and the recurrence was 53 years, with a range of 48-60 years. The mean number (range) of injections given after the late recurrence per eye was 7 (5-11). The mean duration (range) of follow-up post-treatment initiation was 93 (40-122) weeks. All eyes improved to 20/30 visual acuity or better at 6 months after initial treatment. CONCLUSION/CONCLUSIONS:Patients with MFC are never exempt from recurrent CNV, warranting follow-up in perpetuity. Age-related factors are important to consider which may increase the susceptibility for activating MFC-associated CNV in elderly people. Macular neovascularization could respond to a standard approach to management, in these patients with MFC, by a judicious use of intravitreal injections of anti-vascular endothelial growth factor therapy.

PURPOSE/OBJECTIVE:To report the clinical presentation and imaging features in a case of bilateral self-inflicted handheld laser-induced maculopathy which masqueraded as progressive posterior uveitis in a patient with suspected IgG4-related disease. METHODS:Case report with clinical history, fundus photographs, fluorescein angiography, indocyanine green angiography, and swept-source optical coherence tomography. RESULTS:A young Asian Indian man presented with sudden progressive bilateral visual loss over the past 1 week. He was being treated with oral corticosteroids for multiple subcutaneous skin lesions believed to be due to IgG4-related disease. Findings included bilateral central areas of outer retinal disruption with eccentric linear and serpentine lesions showing hypoautofluorescence. Hyperreflective bands extending from the retinal pigment epithelium and interdigitation zone to the outer plexiform layer were present on swept-source optical coherence tomography. After careful history and evaluation of multimodal imaging, posterior uveitis was excluded, and a diagnosis of handheld laser-induced maculopathy was established. CONCLUSION/CONCLUSIONS:Pattern recognition is important in establishing a diagnosis of self-inflicted handheld laser-induced maculopathy which can masquerade as posterior uveitis. Increasing availability of powerful Class IIIb laser devices in both developed and developing countries will likely increase the incidence of this entity.