Faculty Bibliography

PURPOSE/OBJECTIVE:To report a case of a full thickness macular hole (FTMH) after exposure to an extremely powerful handheld laser pointer. METHODS:We evaluated a 14-year-old male with a laser induced FTMH one month after a momentary exposure to a 5000 mW blue laser pointer. Imaging modalities including fundus color, autofluorescence, and spectral domain optical coherence tomography (SD-OCT), acquired both at our clinic and by the referring physician soon after the injury, are used to describe the clinical evolution of the case. RESULTS:Soon after the injury an intensely white, circular opacification of the retina approximately 400 µm in diameter was seen in the fovea. Early SD-OCT images showed full thickness hyperreflectivity, likely representing tissue necrosis. One month later, a FTMH and eradication of the retinal pigment epithelium at its base were evident in the fundus color, autofluorescence and SD-OCT images. CONCLUSION/CONCLUSIONS:High power laser pointers have become easily available online. The presenting findings after exposure to such high-power devices are distinct from those reported after exposure to weaker laser pointers. While long exposure to weaker lasers typically produces extensive, calligraphic figures and yellow placoid lesions involving only the outer retina, in our case a very brief exposure led to focal, full-thickness injury of the fovea.

PURPOSE/OBJECTIVE:To describe a patient with recurrent acute retinal necrosis (ARN), her treatment, and propose a possible pathophysiologic mechanism. METHOD/METHODS:Case report. RESULTS:A four-year-old girl presented elsewhere with bilateral ARN, was treated, but developed a retinal detachment in the left eye that failed vitrectomy surgery. She was referred 10 years later with recurrent ARN. The infection was difficult to get under control, but eventually responded to intravenous acyclovir and foscarnet. She was given laser photocoagulation. She was placed on oral valacyclovir prophylaxis and was disease free for 10 years at which point she decided to go to South America on vacation and stop her valacyclovir. Within a few days she developed a recurrence of ARN and flew back for treatment. She had discrete areas of retinal necrosis, vasculitis, and the laser photocoagulation lesions appeared to be ringed by a retinal change suggestive of retinitis. She responded to antiviral treatment, but developed a retinal detachment that was successfully treated. Her visual acuity was 20/20 six years later, and she was using antiviral prophylaxis. CONCLUSIONS:Recurrent ARN can respond to aggressive treatment. Chorioretinal scars, such as from photocoagulation, may be potential sites of viral invasion during recurrences. Anti-viral prophylaxis may be indicated for at risk patients.

PURPOSE/OBJECTIVE:To develop a consensus nomenclature for reporting optical coherence tomography angiography (OCTA) findings in retinal vascular disease (e.g., diabetic retinopathy, retinal vein occlusion) by international experts. DESIGN/METHODS:Delphi-based survey SUBJECTS, PARTICIPANTS AND/OR CONTROLS: Twenty-five retinal vascular disease and OCTA imaging experts METHODS, INTERVENTION, OR TESTING: A Delphi method of consensus development was used, comprising two rounds of online questionnaires, followed by a face-to-face meeting conducted virtually. Twenty-five experts in retinal vascular disease and retinal OCTA imaging were selected to constitute the OCTA Nomenclature in Delphi Study Group for retinal vascular disease. The four main areas of consensus were: definition of parameters of "widefield (WF)" OCTA, measurement of decreased vascular flow on conventional and WF-OCTA, nomenclature of OCTA findings, and OCTA in retinal vascular disease management and staging. The study endpoint was defined by the degree of consensus for each question: "strong consensus" was defined as ≥ 85% agreement, "consensus" as 80-84% and "near consensus" as 70-79%. MAIN OUTCOME MEASURES/METHODS:Consensus and near-consensus on OCTA nomenclature in retinal vascular disease RESULTS: A consensus was reached that a meaningful change in percentage of flow on WF-OCTA imaging should be an increase or decrease ≥30% of the absolute imaged area of flow signal and that a "large area" of WF-OCTA reduced flow signal should also be defined as ≥ 30% of absolute imaged area. The presence of new vessels (NV) and intra-retinal microvascular abnormalities (IRMAs), the foveal avascular zone (FAZ) parameters, the presence and amount of "no flow" area and the assessment of vessel density in various retinal layers should be added for the staging and classification of DR. Decreased flow ≥ 30% of the absolute imaged area should define an ischemic central retinal vein occlusion (CRVO). Several other items did not meet consensus requirements or were rejected in the final discussion round. CONCLUSIONS:This study provides international consensus recommendations for reporting OCTA findings in retinal vascular disease, which may help to improve the interpretability and description in clinic and clinical trials. Further validation in these settings is warranted and ongoing. Efforts are continuing to address unresolved questions.

PURPOSE/OBJECTIVE:To present a patient with a subhyaloid hemorrhage from proliferative diabetic retinopathy who showed a unique contrast between the fundus autofluorescent patterns of yellow with red blood. METHODS:Analysis of color and autofluorescence fundus photographs of a patient with an aging subhyaloid hemorrhage. RESULTS:The remnants of a resorbing subhyaloid hemorrhage had two layers, a superior yellow portion that was intensely hyperautofluorescent and an inferior relatively hypoautofluorescent red portion. We argue that the yellow appearance of the devitalized blood and fluorophores imaged are related to free base porphyrins. CONCLUSION/CONCLUSIONS:Fundus autofluorescence is a useful modality to image subhyaloid blood and may lend important insights into the fluorophores that hyperfluoresce. The blood breakdown products are potentially toxic and autofluorescence imaging may offer clues to their presence.

PURPOSE/OBJECTIVE:To describe the clinical, optical coherence tomography (OCT), and OCT angiography findings of a patient with a foveal disturbance from the acute phase to the resolution of the visual disturbances. METHOD/METHODS:The patient had a comprehensive ophthalmic examination to include OCT and OCT angiography. RESULTS:A 36-year-old man presented with decreased vision and distortion in the right eye. The right eye showed yellow-white punctate opacities in the central fovea. OCT showed numerous, well-defined, globular, aggregated, hyperreflective lesions that corresponded to the visible opacities along with a focal discontinuity of the outer retinal layers. Over 3 weeks, the patient's findings resolved, and the VA improved to 20/20. No abnormalities of the choriocapillaris flow were detected using OCT angiography. The lesion resolved without pigmentary change. CONCLUSION/CONCLUSIONS:The configuration of the hyperreflective deposits, the lack of pigmentary change, and the absence of OCT angiographic findings of flow problems in the choriocapillaris argue against a primary retinal pigment epithelial or choriocapillaris abnormality as the fundamental cause of the disease. The name acute fovealitis is suggested.

PURPOSE/OBJECTIVE:To describe the long-term findings of a patient with Sorsby fundus dystrophy treated with corticosteroids and propose a mechanism by which the results were obtained. METHODS:Comprehensive ophthalmologic examination with multimodal imaging to include optical coherence tomography and optical coherence tomography angiography was used to evaluate a patient with Sorsby fundus dystrophy treated with intravitreal triamcinolone. RESULTS:A 35-year-old woman presented in 2003 with aggressive macular neovascularization in both eyes; her visual acuity was 20/25 in the right and 20/400 in the left eye. She previously had photodynamic therapy without apparent benefit. She was then treated with photodynamic therapy and an intravitreal injection of 4 mg of triamcinolone, which caused the neovascularization to become inactive. She was eventually switched to an intravitreal injection of triamcinolone 4 mg every 3 to 4 months in the right eye. She had no further treatment in the left eye because of extensive scarring. After 15 1/2 years of treatment, her visual acuity in the right eye was 20/20. Optical coherence tomography showed a large, low-level, irregular elevation of the retinal pigment epithelium. optical coherence tomography angiography revealed widespread macular neovascularization, and the choriocapillaris showed extensive loss. The patient had a TIMP-3 mutation, c.610A>T (p.Ser204Cys). CONCLUSION/CONCLUSIONS:TIMP3 has numerous effects including controlling vascular endothelial growth factor signaling and tumor necrosis factor alpha production. Corticosteroids have the potential to modulate both cytokines. This is the longest reported treatment follow-up of Sorsby fundus dystrophy with macular neovascularization, and the patient retained excellent visual acuity.

PURPOSE/OBJECTIVE:To describe a transient positive scotoma and corresponding optical coherence tomography (OCT) structural and angiographic findings. METHODS:The patient was evaluated with a comprehensive ophthalmic examination to include OCT structural and angiographic imaging with two different instruments, the Zeiss Plex Elite and the Optovue RTVue XR Avanti. RESULTS:A 45-year-old man had a sudden onset of a positive scotoma in the visual field of the left eye. No abnormalities were noted by ophthalmoscopy or fundus photography. Optical coherence tomography angiography was performed to evaluate the macular perfusion status. With each instrument, a small hyperreflective area, 175 μm in diameter, was imaged in the inner nuclear layer. The OCT angiographic images suggested a small area of decreased perfusion in the deep capillary plexus. Except for the diminutive size, the lesion had an appearance suggestive of paracentral acute middle maculopathy. The symptoms lessened rapidly, and when examined 4 days later, the lesion was less hyperreflective. Two weeks after presentation, the positive scotoma was not present and there was no longer any hyperreflectivity in the inner nuclear layer. CONCLUSION/CONCLUSIONS:Detection of the lesion was aided by using OCT angiographic scans, which have a much higher scan density than conventional OCT evaluations. The diminutive abnormality was consistent with a paracentral acute middle maculopathy lesion, although smaller than those previously reported. Micro-paracentral acute middle maculopathy lesions should be considered in the differential diagnosis of positive scotomas.

PURPOSE/OBJECTIVE:To evaluate the various patterns of subretinal fluid (SRF) in eyes with age-related macular degeneration (AMD) in the absence of macular neovascularisation (MNV) and to assess the long-term outcomes in these eyes. METHODS:This retrospective study included only eyes with non-neovascular AMD and associated SRF. Eyes with evidence of MNV were excluded. Spectral-domain optical coherence tomography (SD-OCT) was obtained at baseline and at follow-up, and qualitative and quantitative SD-OCT analysis of macular drusen including drusenoid pigment epithelial detachment (PED) and associated SRF was performed to determine anatomic outcomes. RESULTS:Forty-five eyes (45 patients) were included in this analysis. Mean duration of follow-up was 49.7±36.7 months. SRF exhibited three different morphologies: crest of fluid over the apex of the drusenoid PED, pocket of fluid at the angle of a large druse or in the crypt of confluent drusen or drape of low-lying fluid over confluent drusen. Twenty-seven (60%) of the 45 eyes with fluid displayed collapse of the associated druse or drusenoid PED and 24 (53%) of the 45 eyes developed evidence of complete or incomplete retinal pigment epithelial and outer retinal atrophy. CONCLUSION/CONCLUSIONS:Non-neovascular AMD with SRF is an important clinical entity to recognise to avoid unnecessary anti-vascular endothelial growth factor therapy. Clinicians should be aware that SRF can be associated with drusen or drusenoid PED in the absence of MNV and may be the result of retinal pigment epithelial (RPE) decompensation and RPE pump failure.

PURPOSE/OBJECTIVE:To quantify retinal vasculature changes in Stargardt disease1 with volume-rendered optical coherence tomography angiography (OCTA). METHODS:OCTA volumes from heathy subjects and two subgroups of STGD1 patients with the presence/absence of definitely decreased autofluorescence (DDAF) areas were compared. OCTA vessel surface area (VSA) and vessel volume (VV) were measured in central zones (Z) of 1, 2 and 3mm diameter. RESULTS:29 eyes of 15 STGD1 patients (20/9 eyes with/without DDAF) and 30 eyes of 15 controls contributed data. An enlarged foveal avascular zone was found in STGD1 patients without and even more with DDAF associated with a vessel rarefication in central and also paracentral zones with unnoticeable autofluorescence. VSA and VV were reduced in both STGD1 subgroups for all zones (P<0.0001). STGD1 eyes with compared to without DDAF showed reduced VSA and VV in Z2+3 (both P<0.05). CONCLUSION/CONCLUSIONS:Volume-rendering of OCTA in STGD1 shows a reduced retinal flow in the central macula. This is most likely secondary to loss of neurosensory tissue with disease progression and therefore not likely be favorably influenced by gene transfer and retinal pigment epithelial transplantation. Retinal blood flow assessed by 3D-volume rendered OCTA could serve as surrogate marker for vascular changes of the central retina.

PURPOSE/OBJECTIVE:To provide an image-based description of retinal features associated with risk for development of geographic atrophy (GA) in eyes with age-related macular degeneration (AMD), as visualized with multimodal imaging anchored by structural optical coherence tomography. DESIGN/METHODS:Consensus meeting METHODS: As part of the Classification of Atrophy Meeting program, an international group of experts analyzed and discussed retinal multimodal imaging features in eyes with AMD associated with GA and/or risk of progression to GA. Attendees undertook pre-meeting grading exercises that were reviewed during the meeting sessions. Meeting presentations illustrated established and investigational multimodal imaging features and associated histology. These different features were then each discussed openly by the entire group to arrive at consensus definitions. These definitions were applied to 40 additional images that were graded independently by attendees, to further refine the consensus definitions and descriptions. RESULTS:Consensus was reached on images with descriptors for 12 features. These features included components of outer retinal atrophy (e.g., ellipsoid zone disruption), components of complete retinal pigment epithelium (RPE) and outer retinal atrophy (e.g., RPE perturbation with associated hypo- or hyper-transmission), features frequently seen in eyes with atrophy (e.g., refractile drusen) and features conferring risk for atrophy development (e.g., hyperreflective foci, drusen, and subretinal drusenoid deposits). CONCLUSIONS:An International consensus on terms and descriptions was reached on multimodal imaging features associated GA and with risk for GA progression in eyes with AMD. We believe this information will be useful to clinicians who manage patients with AMD, researchers who study AMD disease interventions and pathogenesis, and those who design clinical trials for therapies targeting earlier AMD stages than GA expansion.