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Purpose: To report eight cases of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) or persistent placoid maculopathy (PPM) initially masquerading as age-related macular degeneration in elderly individuals., Methods: APMPPE or PPM eyes in patients above age 55 years with macular retinal pigment epithelium disruption including drusenoid lesions on macular examination and/or with multimodal imaging were included. At least one method of multimodal imaging including fluorescein angiography (FA), indocyanine green angiography, optical coherence tomography (OCT), and OCT angiography (OCTA) was performed in all eyes for diagnosis and to monitor for macular neovascularization., Results: Eight elderly male patients presented with vision loss and were all initially diagnosed with non-neovascular or neovascular age-related macular degeneration. With the aid of multimodal retinal imaging, a final diagnosis of either APMPPE or PPM was rendered. With FA and indocyanine green angiography, choroidal hypoperfusion was detected in all but one eye. With OCT, the angular sign of Henle fiber layer hyperreflectivity was identified in >50% of eyes. With OCTA, inner choroidal flow deficits were detected in all eyes. Macular neovascularization requiring anti-vascular endothelial growth factor injection therapy complicated three of eight cases., Conclusion: Both APMPPE and PPM may develop in elderly individuals and may masquerade as age-related macular degeneration on presentation. Multimodal imaging including FA, indocyanine green angiography, and OCTA are important diagnostic modalities to assess for inner choroidal hypoperfusion to arrive at an accurate diagnosis and to detect macular neovascularization, which frequently complicates APMPPE and PPM. In these patients, serial anti-vascular endothelial growth factor intravitreal injections are essential in treating macular neovascularization and in preventing significant vision loss.

Purpose: To expand the multimodal imaging correlation of the concentric macular rings (CMR) sign seen on ultra-widefield fundus photography in patients with foveal hypoplasia., Design: Retrospective case series., Participants: Thirty-two patients with foveal hypoplasia who demonstrated the CMR sign on ultra-widefield fundus photography., Methods: Inclusion criterion was the presence of a CMR sign detected on ultra-widefield fundus photography., Main Outcome Measures: Noninvasive multimodal retinal imaging, including ultra-widefield fundus photography, structural OCT, near-infrared reflectance, and blue fundus autofluorescence, were investigated. Horizontal dense B-scans and en face OCT images were acquired., Results: Evaluation of all patients (n = 32 patients) demonstrated a CMR sign on ultra-widefield fundus photography. Structural OCT scans were consistent with foveal hypoplasia in all patients. En face OCT images acquired at the level of the Henle fiber layer highlighted similar concentric rings around the location of the incipient fovea. The series of concentric rings was not visible at any other level of the macula. A significant correlation was found between the horizontal diameter of the largest outer ring and foveal hypoplasia grades (P < 0.0001)., Conclusions: The CMR sign seen on ultra-widefield fundus imaging may be a distinctive feature of foveal hypoplasia and can support this diagnosis, especially in patients in whom OCT cannot be performed (patients with poor fixation or nystagmus or young children). Multimodal imaging correlation suggests that these concentric rings occur exclusively at the level of the Henle fiber layer. This distinctive Henle fiber layer geometry may reflect an arrested development stage in the timeline of foveal maturation., (Copyright © 2020 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to report the multimodal imaging features of a cavitary choroidal nevus showing thickness fluctuations that mirrored the response of diabetic macular edema (DME) to intravitreal antivascular endothelial growth factor (VEGF) therapy. Methods: This is a retrospective case report. Multimodal imaging findings including fundus photography, optical coherence tomography (OCT), fluorescein and indocyanine green angiography, OCT-angiography (OCTA), and B-scan ultrasonography were analyzed. Results: A woman in her 80s with a cavitary choroidal nevus and DME was treated with intravitreal anti-VEGF therapy using a pro re nata regimen over 5 years. The choroidal nevus showed thickness fluctuations paralleling the response of DME to anti-VEGF therapy. Worsening of the DME was associated with marked increased choroidal lesion thickness on OCT. Conversely, resolution of DME after intravitreal anti-VEGF injections was followed by choroidal lesion flattening on OCT. Variations of the choroidal lesion thickness were mainly dependent on changes of intralesional hyporeflective caverns on OCT. Conclusion: Our report shows thickness variations of a cavitary choroidal nevus that paralleled the clinical course of DME treated with intravitreal anti-VEGF therapy. To the best of our knowledge, this is the first report on volume variations of a cavitary choroidal nevus after anti-VEGF therapy. [ABSTRACT FROM AUTHOR]

Purpose: To report the long-term (23 years) clinical and multimodal imaging features of acquired vitelliform lesions (AVLs) associated with nonneovascular age-related macular degeneration. Methods: Retrospective case report. Color and red-free fundus photographs, highresolution optical coherence tomography, fluorescein and indocyanine green angiography, and optical coherence tomography-angiography were performed. Results: A 58-year-old man presented with bilateral AVLs in the setting of nonneovascular age-related macular degeneration. At baseline, his best-corrected visual acuity was 20/30 in his right eye and 20/20 in his left eye. Red-free fundus photographs showed AVLs with cuticular drusen in both eyes corresponding to a "stars-in-the-sky" pattern on fluorescein. Indocyanine green angiography showed no evidence of macular neovascularization. Throughout the 23-year follow-up, the patient reported consuming 20 mg/day of lutein supplement. At the end of follow-up, his best-corrected visual acuity was 20/20 in both eyes. Color fundus photographs showed resorption of the AVLs in both eyes and High-Res optical coherence tomography showed relative preservation of the outer retinal bands in the fovea. Optical coherence tomography-angiography confirmed the absence of macular neovascularization. Conclusion: In nonneovascular age-related macular degeneration, spontaneous resorption of AVLs may be associated with long-term maintenance of visual acuity and relative preservation of the outer retinal morphology. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of recurrent acute retinopathy associated with pseudoxanthoma elasticum and to propose a reappraisal of this entity based on multimodal imaging analysis. Methods: Retrospective case report. High-resolution optical coherence tomography (high-res OCT), ultra-widefield imaging, and widefield swept-source OCT angiography and en face OCT were performed. Results: A man in his 40s diagnosed with pseudoxanthoma elasticum and angioid streaks presented with two distinct episodes of acute retinopathy in his right eye during a one-year follow-up period. Acute retinopathy was characterized by rapid vision loss. High-res OCT showed multifocal hyperreflective lesions splitting the retinal pigment epithelium/Bruch membrane complex and associated with focal choroidal thickening. After the first episode, OCT angiography confirmed the development of macular neovascularization at the site of a previous inflammatory lesion. During the second episode, multimodal images showed findings consistent with epiphenomenon multiple evanescent white dot syndrome (EpiMEWDS). On en face widefield OCT, acute retinopathy was characterized by multiple hyperreflective spots scattered at the posterior pole. Conclusion: Recurrence of acute retinopathy can be observed in patients with pseudoxanthoma elasticum and angioid streaks. Multimodal imaging shows that some lesions of pseudoxanthoma elasticum-associated acute retinopathy closely resemble those of punctate inner choroidopathy/idiopathic multifocal choroiditis. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of bilateral paracentral acute middle maculopathy lesions on spectral domain-optical coherence tomography(OCT) secondary to severe Plasmodium falciparum malaria. Methods: Retrospective case report. Spectral domain-OCT, ultra-widefield fluorescein angiography, and OCT angiography were performed and analyzed. Results: A 54-year-old healthy man presented with acute vision loss in both eyes few days after being diagnosed with severe Plasmodium falciparum malaria. Ophthalmoscopic examination was unremarkable, but near-infrared reflectance imaging showed patchy hyporeflective areas located at the terminal tips of the venous branches. Corresponding spectral-domain OCT demonstrated alternating bands of hyperreflectivity involving the inner nuclear layer, consistent with skip paracentral acute middle maculopathy lesions. Optical coherance tomography angiography illustrated corresponding flow signal loss at the level of the deep capillary plexus. Ultra-widefield fluorescein angiography showed peripheral retinal vein staining and capillary nonperfusion. Conclusion: Paracentral acute middle maculopathy may be an OCT manifestation of malarial retinopathy associated with severe Plasmodium falciparum infection. [ABSTRACT FROM AUTHOR]

A 21-year-old healthy man presented with branch retinal artery occlusion and paracentral acute middle maculopathy on optical coherence tomography. Subsequent workup for systemic associations was unremarkable, except for heavy cannabis consumption. His clinical presentation and multimodal imaging findings are described. Purpose: To report a case of branch retinal artery occlusion associated with paracentral acute middle maculopathy on spectral-domain optical coherence tomography presumably related to heavy cannabis consumption. Methods: Retrospective case report. Spectral-domain optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography were performed. Results: A 21-year-old healthy man described the acute onset of superior visual field loss in his right eye. He admitted smoking approximately 15 g daily of cannabis for several weeks during COVID-19 confinement. Ophthalmoscopic examination of the right eye showed inferotemporal retinal whitening. Spectral-domain optical coherence tomography illustrated evidence of the ischemic cascade with diffuse hyperreflectivity of the inner and middle retinal layers within the central region of the retinal infarct and paracentral acute middle maculopathy at the border of the infarct. Optical coherence tomography angiography demonstrated predominant flow signal loss at the level of the deep retinal capillary plexus. Fluorescein angiography and complete systemic workup were unremarkable. Conclusion: Branch retinal artery occlusion and paracentral acute middle maculopathy may be related to heavy cannabis use as the result of transient arterial vasospasm. [ABSTRACT FROM AUTHOR]

The Henle fiber layer (HFL) is comprised of bundles of unmyelinated photoreceptor axons intermingled with outer Müller cell processes. The photoreceptor axons extend from the cell bodies located in the outer nuclear layer and radially project toward the outer plexiform layer, the inner third of which includes the synaptic junctional complexes and the outer two-thirds of which includes the HFL. The oblique path of the HFL provides unique structural and reflectance properties and this radial anatomy is highlighted in many macular disorders including those with macular star exudation and HFL hemorrhage. Recent investigations using multimodal imaging techniques, especially cross sectional and en face optical coherence tomography (OCT), have provided new perspectives regarding HFL disruption in retinal diseases. The aim of this review is to highlight the pathoanatomy and multimodal imaging, especially OCT, associated with HFL disruption that is present in various macular diseases. After describing the current knowledge of the embryology, anatomy, and physiology of the HFL, we review the existing imaging modalities that allow in vivo visualization of the HFL in the healthy and diseased retina. Finally, we report the clinical and imaging findings of acute HFL alteration in various macular disorders, including degenerative, inflammatory, and vascular conditions. Also, we propose a novel and signature OCT biomarker indicative of acute photoreceptor disruption involving the HFL, termed the "angular sign of HFL hyperreflectivity" (ASHH) of macular disease, to unify the pathoanatomy common to these various macular disorders and to provide clarity regarding the underlying pathogenesis. [ABSTRACT FROM AUTHOR]