Faculty Bibliography

A major cause of poor visual prognosis in Coats disease is the formation of fibrovascular changes following dense foveal lipid deposition. The authors document the multimodal imaging findings of a 38-year-old woman and a 23-year-old man with adult-onset Coats disease who presented with macular edema and foveal lipid accumulation. Thermal laser targeting individual capillary macroaneurysms combined with intravitreal anti-vascular endothelial growth factor (VEGF) therapy was performed. Although there was a subsequent increase in foveal lipid immediately following the resolution of macular edema, these lipids largely resolved, leaving behind no evidence macular neovascular fibrosis. This report highlights the potential protective effect of combination therapy with thermal laser and intravitreal anti-VEGF therapy for macular exudation associated with Coats disease. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:396-399.].

A panel of imaging experts was assembled to review neovascular age-related macular degeneration optical coherence tomography angiography descriptors published to date, and test agreement on use of these terms, which was found to be low. Optical coherence tomography angiography (OCTA) has been used to identify and characterize macular neovascularization (MNV) secondary to age-related macular degeneration (AMD).^1-4 Many studies have explored OCTA morphological features of MNV that might serve as biomarkers to assess disease activity and response to treatment.^1-6 The proliferation of studies however has resulted in an OCTA terminology that has been variable and inconsistent. To address inconsistency of nomenclature and allow harmonization, a multidisciplinary panel of retinal imaging experts with a history of relevant research contributions to the field was assembled with the purpose of reviewing published terminology and to recommend a reduced list of key terms pertinent to OCTA. The group was called UNICORN, because of its ultimate goal of generating a UNIfied COmmentary of the committee of inteRnational experts on the nomenclature for Neovascular AMD in OCTA. In this report we describe the first steps, which included a review of OCTA descriptors of neovascular AMD (nAMD) published to date, and an exercise that tested agreement of these terms among retinal imaging experts. Prior to the first UNICORN meeting, a non-systematic review of the literature was performed, using the search terms "optical coherence tomography angiography" or "OCT angiography" or "OCT-A", AND "neovascular macular degeneration" or "neovascular age-related macular degeneration" or "neovascular AMD" or "nAMD" or "wet age-related macular degeneration" or "wet AMD" or "wet ARMD". A dictionary of OCTA descriptors relating to the features of MNV in AMD was generated and circulated to the panel.

PURPOSE/OBJECTIVE:To study the effect of the suspended scattering particles in motion (SSPiM) on optical coherence tomography angiography (OCTA) vessel density metrics in eyes with diabetic macular edema (DME) METHODS:: Thirty-four eyes with DME from 27 patients (16 males and 11 females, 61.4 ± 9.6 years) with DME were included in this retrospective cohort study. Among these eyes, 19 (55.9%) showed the SSPiM artifact on OCTA. All participants received 3-mm and 6-mm optical coherence tomography angiography (OCTA) imaging. Perfusion density and skeletonized vessel density were calculated for the superficial capillary plexus (SCP) and the deep capillary plexus (DCP), and these were compared between eyes with and without SSPiM. Additionally, foveal vessel density in a 300-µm-wide region around the foveal avascular zone (FVD) was evaluated on 3-mm OCTA scans. The main outcome measures were vessel density in the SCP and the DCP. RESULTS:Among the 3-mm OCTA images, there was no statistically significant difference in SCP vessel density in eyes with and without SSPiM (p = 0.98). Vessel density in the DCP (p = 0.001 and p = 0.028 for perfusion and skeletonized vessel density, respectively) and FVD (p = 0.03) on 3-mm OCTA scans were significantly higher in DME eyes with SSPiM than in those without SSPiM. There were no statistically significant differences in vessel density in SCP and DCP between eyes with and without SSPiM based on 6-mm OCTA scans. CONCLUSION/CONCLUSIONS:The presence of SSPiM may lead to an overestimation of DCP vessel density in eyes with DME when 3-mm OCTA scans are used for analysis.

To describe a peculiar hyperreflective optical coherence tomography finding of nodular epiretinal gliosis at the fovea that may be a manifestation of Müller response to retinal injury. The central fovea is stabilized by the Müller cell cone but is at risk of foveal dehiscence due to various insults including vitreomacular traction which can lead to lamellar or full thickness macular hole formation.¹ Degenerative lamellar macular holes can be associated with epiretinal proliferation which has been attributed to activated Muller cells.² In this report, we describe a very unusual optical coherence tomography (OCT) finding of nodular epiretinal gliosis which may be the result of Müller cell activation and expansion.

PURPOSE/OBJECTIVE:To demonstrate the clinical and research value of a simplified technique enabling alignment of functional microperimetry data with retinal structure imaged by eye-tracked optical coherence tomography (OCT) in eyes with macular disease. METHODS:Normal and diseased eyes underwent sequential Spectralis OCT macular raster scans and Macular Integrity Assessment (MAIA) microperimetry using both standard central 10° analysis and custom scan patterns. The microperimetry data were imported into Spectralis research software which was automatically registered to the scanning laser ophthalmoscopy near-infrared reflectance image obtained during OCT acquisition. The OCT B-scans were directly correlated to the microperimetry data so that retina sensitivity (RS) and retinal microstructure at corresponding points could be evaluated simultaneously. RESULTS:Seventy eyes of 41 patients (110 studies) aged 22-95 years (mean 63.5 ± 18.0 years) with both normal and pathologic macular changes were included. The mean MAIA RS of all 110 studies was 22.2 ± 4.9 (range: 0.1 - 30.3). Retinal sensitivity showed good correspondence structural changes seen on OCT B-scans. CONCLUSIONS:We demonstrate a practical method to align RS data to anatomic tomographic findings. This technique provides data not obtainable with standard visual acuity measures.

PURPOSE/OBJECTIVE:To report a retinal pigment epithelium (RPE) tumor with exudative maculopathy, originating from an atypical RPE lesion presumed to represent congenital hypertrophy of the RPE or RPE hyperplasia. METHODS:Multimodal imaging including fundus autofluorescence, optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography. RESULTS:A 76-year-old West African man noted visual acuity reduction to count fingers in the right eye and 20/400 in the left eye. Features of chronic glaucoma were noted. In addition, there was a fairly well-circumscribed darkly pigmented RPE lesion in the paramacular region in the right eye, measuring 4 mm in diameter and flat and consistent with atypical congenital hypertrophy of the RPE or RPE hyperplasia. On the posterior margin of this mass was an RPE tumor, presumed to represent RPE adenoma, producing exudative maculopathy and cystoid macular edema. Multimodal imaging was used to distinguish the RPE tumor from macular neovascularization. A similar atypical congenital hypertrophy of the RPE without retinopathy measuring 3.5mm in diameter was noted in the temporal macular region in the left eye. After six monthly doses of intravitreal bevacizumab (1.25 mg/0.05 mL) in the right eye, the maculopathy resolved and the RPE mass showed partial involution with visual acuity return to baseline 20/200. CONCLUSION/CONCLUSIONS:Congenital hypertrophy of the RPE and RPE hyperplasia can produce RPE adenoma with related exudative maculopathy. In this case, the maculopathy responded to bevacizumab.

PURPOSE/OBJECTIVE:To describe the occurrence of an acquired choroidal nevus in a 73-year-old white man. METHODS:Case report. RESULTS:A 73-year-old white man was referred for an evaluation and treatment of macular changes in his left eye consistent with pachychoroid neovasculopathy. Baseline funduscopic examination and color fundus photographs showed two small peripheral choroidal nevi in the right eye and a single small choroidal nevus in the far temporal macula of the left eye. Treatment with intravitreal aflibercept was initiated in the left eye on a treat-and-extend dosing regimen. Approximately 1 year later, a new pigmented choroidal lesion was detected in the left macula in an area where previous high-resolution color fundus photographs had shown no abnormal pigmentation. Swept-source optical coherence tomography of the new pigmented lesion showed flat hyperreflectivity within the inner choroid consistent with a small choroidal nevus. The patient was referred to his internist who found no evidence of an occult malignancy. Over the course of more than 4 additional years of continuous follow-up, the new choroidal nevus remained stable, no new fundus abnormalities were detected in either eye, and the patient remained medically stable. CONCLUSION/CONCLUSIONS:To the best of our knowledge, this is the first documented case of a new choroidal nevus. Multimodal imaging performed before lesion detection and over the ensuing 4 years showed its stability, thus allowing for the conclusion that it was a benign choroidal nevus rather than a neoplastic or paraneoplastic process.

PURPOSE/OBJECTIVE:To describe the clinical and multimodal imaging (MMI) features of age-related macular degeneration (AMD) eyes presenting with intraretinal exudation and no evidence of neovascularization or structural alterations of native retinal vessels. METHODS:This was a retrospective review of the MMI and electronic health records for 3 consecutive patients presenting with unilateral exudative non-neovascular age-related macular degeneration. MMI included confocal color fundus photography (CFP), fundus autofluorescence (FAF), fluorescein angiography (FA), spectral domain optical coherence tomography (SD-OCT), swept-source optical coherence tomography angiography (SS-OCTA), and spectral domain optical coherence tomography angiography (SD-OCTA). Dense B-scan OCTA (DB-OCTA) patterns and implemented image post-processing were used to improve spatial resolution in the OCTA analysis and remove projection artifacts. RESULTS:Three eyes of 3 patients (1 male and 2 females, ages 72-87) developed intraretinal fluid (IRF) producing retinal edema during regular follow-up for non-neovascular AMD. FA, SS-OCTA, and DB-OCTA demonstrated no evidence of macular neovascularization or discrete retinal vascular abnormalities that could explain the IRF accumulation. Two eyes received intravitreal anti-VEGF therapy and demonstrated prompt resolution of IRF with periodic recurrences over time. CONCLUSION/CONCLUSIONS:Exudative non-neovascular AMD is a novel clinical phenotype characterized by the presence of non-neovascular intraretinal exudation producing macular edema. Differentiating this condition from other manifestations of AMD requires appropriate use of MMI. Further study is needed to assess the clinical impact and optimal management of exudative non-neovascular AMD.

PURPOSE/OBJECTIVE:To describe the differential response of two distinct inflammatory signs occurring in eyes with punctate inner choroidopathy (PIC). METHODS:Retrospective, observational case series utilizing multimodal imaging (MMI). RESULTS:Four eyes of 4 myopic female patients (mean age 35 years, range 31-42) presenting with retinal manifestations of PIC. All study eyes had 2 distinct signs of active disease: 1) acute focal hyperreflective lesions splitting the retinal pigment epithelium/Bruch's membrane (RPE/BrM) complex on optical coherence tomography (OCT) which appeared hypoautofluorescent on fundus autofluorescence (FAF), and 2) more diffuse areas of outer retinal disruption (ORD) limited to the ellipsoid zone and interdigitation zone on OCT and corresponding to hyperautofluorescence on FAF. All patients were treated with oral prednisone and demonstrated prompt regression of the RPE/BrM complex lesions with a concurrent, paradoxical centrifugal expansion of ORD. The ORD eventually resolved in all eyes (mean time 6 weeks, range 4-10 weeks). CONCLUSIONS:In patients with PIC, two distinct inflammatory signs observed with MMI display a differential response to systemic corticosteroids. Whereas focal inflammatory lesions splitting the RPE/BrM complex appear to respond rapidly, the more diffuse, transient ORD shows little response. This difference in treatment response may reflect different immunological phenomena with independent natural history.