RETINAL RACEMOSE HEMANGIOMA (RETINAL ARTERIOVENOUS COMMUNICATION) DIAGNOSED AND MANAGED WITH MULTIMODAL IMAGING.

BACKGROUND/PURPOSE: Retinal racemose hemangioma is a rare congenital abnormality of the retinal vasculature with a variety of secondary manifestations that can cause vision loss, including macular edema. This report aims to demonstrate the use of swept-source optical coherence tomography angiography in further characterizing this abnormality. METHODS: Case report with multimodal imaging including swept-source optical coherence tomography angiography. RESULTS: A 56-year-old woman with blurred vision was diagnosed macular edema secondary to retinal racemose hemangioma. Localization of the arterial-venous connection was identified with swept-source optical coherence tomography angiography at the deep capillary plexus. Conservative management of the associated foveal exudation ultimately led to a favorable outcome. CONCLUSION: Observation or topical therapy may be useful and warranted in select cases of retinal racemose hemangioma. The anomalous vascular connection in retinal racemose hemangioma seems to originate at the level of the deep capillary plexus in this case; however, larger studies are necessary for corroboration. Evolving angiographic modalities such as swept-source optical coherence tomography angiography may continue to provide insights for this rare disease.

ARE THERE TWO FORMS OF MULTIPLE EVANESCENT WHITE DOT SYNDROME?

PURPOSE: To analyze the nature of multiple evanescent white dot syndrome (MEWDS) and differentiate an idiopathic or primary form of MEWDS from a secondary form that is seen in association with other clinical conditions affecting the posterior segment of the eye. METHODS: Clinical and multimodal imaging findings including color fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography of patients with secondary MEWDS are presented. RESULTS: Twenty consecutive patients with secondary MEWDS were evaluated. Fifteen patients were female. Most were young adults aged between 20 to 40 years with myopia (less than -6 diopters). Pathologic conditions associated with the secondary MEWDS reaction were high myopia (greater than -6 diopters) in two eyes, previous vitreoretinal surgery for rhegmatogenous retinal detachment in 2 eyes, and manifestations of multifocal choroiditis in 18 eyes. In all eyes, the MEWDS lesions followed a course of progression and resolution independent from the underlying condition. CONCLUSION: Secondary MEWDS seems to be an epiphenomenon ("EpiMEWDS") that may be seen in association with clinical manifestations disruptive to the choriocapillaris-Bruch membrane-retinal pigment epithelium complex.

Resolution of Foveal Lipid Deposition in Adult-Onset Coats Disease With Combined Focal Laser Photocoagulation and Anti-VEGF Therapy.

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.].

Evaluation of non-exudative microcystoid macular abnormalities secondary to retinal vein occlusion.

PURPOSE: We aimed to investigate non-exudative microcystoid macular abnormalities for visual and anatomical outcome in patients with retinal vein occlusion (RVO) with and without glaucomatous optic neuropathy (GON). METHODS: Medical records of 124 eyes (105 patients) with RVO were reviewed and analyzed. Eyes demonstrating microcystoid macular abnormalities were divided into 2 groups, those with evidence of glaucoma (group A) and those without glaucoma (group B). Best-corrected visual acuity (BCVA), the prevalence and number of microcystoid macular abnormalities, and number of intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections were compared at baseline and follow-up. RESULTS: Seventy-one out of 105 eyes (67.6%) with RVO displayed microcystoid macular abnormalities. Thirty-eight out of 71 eyes (53.5%) presented with concomitant glaucoma (group A), while the remaining 33 eyes (42.6%) had no history of glaucoma (group B). At the end of the follow-up period, mean BCVA was worse in group A versus group B (20/80 versus 20/40, respectively; p = .003). The mean number of anti-VEGF injections was 10.1 ± 9.2 in group A versus 5.9 ± 6.9 in group B (p = .03). CONCLUSION: Eyes with RVO and concomitant glaucoma exhibited a significantly higher number of microcystoid macular abnormalities and worse BCVA versus eyes with RVO without glaucoma.

Presumed Natural History of Combined Hamartoma of the Retina and Retinal Pigment Epithelium.

PURPOSE: To correlate structural changes of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) with patient age. DESIGN: Retrospective study. PARTICIPANTS: Fifty eyes of 49 patients (age range, 1-74 years) with CHRRPE studied at 9 tertiary vitreoretinal institutions. METHODS: We analyzed the clinical findings with respect to lesion topography and pigmentation as well as investigated the OCT findings regarding the thickness, vitreoretinal interface, outer plexiform layer distortion, ellipsoid zone disruption, and retinal pigment epithelium-Bruch's membrane complex involvement of CHRRPE. MAIN OUTCOME MEASURES: Clinical and imaging findings of CHRRPE at different ages. RESULTS: Analysis of 50 CHRRPE patients revealed that younger patients were more likely to demonstrate partial thickness involvement of the retina (P = 0.009) with predominantly inner retinal layer involvement (P = 0.04). The inverse was true for older patients with CHRRPE. In addition, older patients more commonly showed pigmentary changes. Eyes with CHRRPE were more likely to show an increase in central macular thickness independently of tumor location. CONCLUSIONS: Based on these findings, we believe that CHRRPE typically begins in the inner retina and continues toward the outer retina over time, with increase in central macular thickness, despite the location of the tumor.

A CASE OF INTRARETINAL PERIPAPILLARY NEOVASCULARIZATION IN ABCA4-RELATED RETINOPATHY.

PURPOSE: To report a case of ABCA4-related retinopathy and potential complications. METHODS: The authors describe a case report of intraretinal neovascularization in a patient with ABCA4-related retinopathy and describe the multimodal retinal imaging findings. RESULTS: A 49-year-old woman presents with cystoid macular edema and diffuse intraretinal and perivascular hyperpigmentation in both eyes. Genetic testing confirmed ABCA4-related retinopathy. Fluorescein angiography revealed peripapillary intraretinal neovascularization in the absence of any other identifiable retinal pathology. CONCLUSION: To the authors' knowledge, this is the first reported case of intraretinal neovascularization-associated ABCA4-related retinopathy. Ancillary testing of ABCA4-related retinopathy with fluorescein angiography or optical coherence tomography angiography may be helpful in identifying this rare complication.

EFFECT OF LASER PHOTOCOAGULATION ON MACULAR EDEMA ASSOCIATED WITH MACULAR HOLES.

PURPOSE: To report the outcomes of laser therapy to barricade eccentric full-thickness macular hole with associated cystoid macular edema. METHODS: We report two patients who developed an eccentric full-thickness macular hole with persistent cystoid macular edema after pars plan vitrectomy with and without internal limiting membrane peel for epiretinal membrane and the results of argon laser therapy. RESULTS: Barricade argon laser therapy was applied concentric to the full-thickness macular hole. Associated cystoid macular edema was noted to resolve within 1 to 3 months of therapy in both cases. CONCLUSION: Barricade laser therapy surrounding a macular hole can lead to resolution of associated cystoid macular edema. Pathogenic mechanisms to explain this favorable outcome are discussed.

Exudative non-neovascular age-related macular degeneration.

PURPOSE: 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: 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.

Presumed retinal pericapillary astrocytic hamartoma: multimodal imaging findings of a novel hamartomatous lesion.

PURPOSE: To describe the multimodal imaging findings of retinal lesions that clinically resemble retinal astrocytic hamartomas (RAHs), but also have unique characteristics that we believe represent a novel variant. METHODS: Observational study. Five eyes in five patients with solitary retinal lesion evaluated at the retina division of three institutions. We describe the multimodal imaging findings including fundus photography, fundus autofluorescence, fluorescein angiography, spectral-domain optical coherence tomography (OCT), swept-source OCT, swept-source OCT angiography and ultrasonography. RESULTS: The retinal lesions described shared similar appearance to RAHs but demonstrated unique features such as glistening granular appearance on fundus photographs with perivascular hyperreflectivity with OCT and OCT angiography. CONCLUSION: The lesions described herein appear to have unique characteristics that warrant a designation as a novel RAH variant. The name presumed retinal pericapillary astrocytic hamartoma is suggested.

Detection of Occult Arteriovenous Malformation With Annular Array Ultrasonography.

Retinal vascular tortuosity may occur in a wide range of ocular disorders. When retinal vascular tortuosity involves both arteries and veins, and presents unilaterally and without hemorrhage, a diagnosis of Wyburn Mason syndrome (WMS) should be considered due to the potential morbidity and mortality associated with cerebral involvement. Magnetic resonance imaging (MRI) and MRI angiography (MRA) are important tools for identifying cerebral arteriovenous malformations (AVMs), but these imaging modalities have limited spatial resolution to detect very small vascular lesions. Annular array contact ocular ultrasound is a new imaging modality capable of detecting small intraorbital AVMs. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:239-243.].

Cross-Sectional Soft-Tissue Composition and the Distribution of Blue Cutaneous Discoloration in the Lower Eyelid after Hyaluronic Acid Injection.

The soft-tissue composition of the eyelid varies from lateral to medial, in that the orbicularis area (in square millimeters) is greatest medially and least laterally, whereas the reverse is true for the preauricularis and postorbicularis fat. The purpose of this study was to describe the relative contribution of tissue types to the volume of the lower eyelid-midface junction. This is an observational cohort study of 11 subjects (20 eyes) without a history of orbital or periorbital surgery or abnormality. Quasi-sagittal surface coil magnetic resonance imaging scans in planes parallel to the long axis of each orbit were obtained during central gaze fixation. Measurements of the tissue area (in square millimeters) of the skin, preorbicularis fat, orbicularis, and postorbicularis fat were obtained in the center, medial, and lateral eyelid regions. Differences in tissue area across the eyelid were assessed. The segmental cross-sectional area and percentage of total segment represented by the orbicularis muscle increased from the lateral (9.4 mm; 21.8 percent) to the central (14.1 mm; 35.0 percent) and to the medial (18.5 mm; 47.0 percent) eyelid (p < 0.01). The inverse pattern was noted for both preorbicularis and postorbicularis fat, each occupying less area (in square millimeters) from lateral to medial (p < 0.01). Skin thickness did not vary significantly across the eyelid. The soft-tissue composition of the eyelid varies from lateral to medial in that the orbicularis increases in area, whereas the reverse is true for the preorbicularis and postorbicularis fat. This anatomical progression may help to explain the stereotypical location of blue discoloration in some patients after hyaluronic acid filler injection.

BULL'S EYE MACULOPATHY WITH MUTATIONS IN RDS/PRPH2 AND ROM-1.

PURPOSE: To report a case of bull's eye maculopathy associated with mutations in RDS/PRPH2 and ROM-1 genes. METHODS: We present a case report of a patient with a characteristic maculopathy and describe the multimodal retinal imaging findings including spectral domain optical coherence tomography and fundus autofluorescence and full-field electrophysiology. The results of genetic testing are also reported. RESULTS: A 60-year-old woman presented with decreased vision and a remarkable bull's eye maculopathy with retinal examination. Fundus autofluorescence illustrated a striking pattern of speckled hyperautofluorescence and hypoautofluorescence that highlighted the bull's eye maculopathy in each eye and guided genetic testing, which confirmed a mutation of the RDS/PRPH2 gene and a novel mutation of the ROM-1 gene. CONCLUSION: Multimodal imaging including fundus autofluorescence may guide genetic testing in patients with a characteristic maculopathy. RDS/PRPH2 genetic mutation can be associated with a bull's eye maculopathy with a signature fundus autofluorescence presentation.