Multimodality imaging for diagnosis of subclinical hereditary transthyretin cardiac amyloidosis.

We present a case of a patient with worsening visual acuity and dense vitreal debris who was found to have vitreal transthyretin amyloid (ATTR) infiltration. Cardiac workup, performed to identify systemic amyloidosis, demonstrated focal myocardial amyloid infiltration on pyrophosphate (PYP) scintigraphy and cardiac magnetic resonance (CMR), resulting in a diagnosis of subclinical ATTR cardiac amyloidosis (ATTR-CA). Patient was identified as a carrier of p.S70R mutation which results in an aggressive ATTR phenotype. Patient is tolerating transthyretin silencer therapy well. Through this case, we discuss the role of a multimodality imaging approach for the diagnosis of subclinical ATTR-CA.

Frequency of Urgent or Emergent Vitreoretinal Surgical Procedures in the United States During the COVID-19 Pandemic.

Importance: The American Academy of Ophthalmology (AAO) indicated that urgent or emergent vitreoretinal surgical procedures should continue during the coronavirus disease 2019 (COVID-19) pandemic. Although decreases in the frequency of critical procedures have been reported outside the field of ophthalmology, analyses are limited by volume, geography, and time. Objective: To evaluate whether the frequency of ophthalmic surgical procedures deemed urgent or emergent by the AAO changed across the United States during the COVID-19 pandemic. Design, Setting, and Participants: Vitreoretinal practices from 17 institutions throughout the US participated in this multicenter cross-sectional study. The frequency of 11 billed vitreoretinal Current Procedural Terminology (CPT) codes across respective weeks was obtained from each practice between January 1, 2019, and May 31, 2020. Data were clustered into intravitreal injections (code 67028), lasers and cryotherapy (codes 67141, 67145, and 67228), retinal detachment (RD) repairs (codes 67107, 67108, 67110, and 67113), and other vitrectomies (codes 67036, 67039, and 67040). Institutions were categorized by region (Northeast, Midwest, South, and West Coast), practice setting (academic [tax-exempt] or private [non-tax-exempt]), and date of respective statewide stay-at-home orders. Main Outcomes and Measures: Nationwide changes in the frequency of billing for urgent or emergent vitreoretinal surgical procedures during the COVID-19 pandemic. Results: A total of 526 536 CPT codes were ascertained: 483 313 injections, 19 257 lasers or cryotherapy, 14 949 RD repairs, and 9017 other vitrectomies. Relative to 2019, a weekly institutional decrease in injections was observed from March 30 to May 2, 2020, with a maximal 38.6% decrease (from a mean [SD] of 437.8 [436.3] to 273.8 [269.0] injections) from April 6 to 12, 2020 (95% CI, -259 to -69 injections; P = .002). A weekly decrease was also identified that spanned a longer interval, at least until study conclusion (March 16 to May 31, 2020), for lasers and cryotherapy, with a maximal 79.6% decrease (from a mean [SD] of 6.6 [7.7] to 1.5 [2.0] procedures) from April 6 to 12, 2020 (95% CI, -6.8 to -3.3 procedures; P < .001), for RD repairs, with a maximal 59.4% decrease (from a mean [SD] of 3.5 [4.0] to 1.6 [2.2] repairs) from April 13 to 19, 2020 (95% CI, -2.7 to -1.4 repairs; P < .001), and for other vitrectomies, with a maximal 84.3% decrease (from a mean [SD] of 3.0 [3.1] to 0.4 [0.8] other vitrectomies) from April 6 to 12, 2020 (95% CI, -3.3 to -1.8 other vitrectomies; P < .001). No differences were identified by region, setting, or state-level stay-at-home order adjustment. Conclusions and Relevance: Although the AAO endorsed the continued performance of urgent or emergent vitreoretinal surgical procedures, the frequency of such procedures throughout the country experienced a substantial decrease that may persist after the COVID-19 pandemic's initial exponential growth phase. This decrease appears independent of region, setting, and state-level stay-at-home orders. It is unknown to what extent vitreoretinal intervention would have decreased without AAO recommendations, and how the decrease is associated with outcomes. Although safety is paramount during the COVID-19 pandemic, practices should consider prioritizing availability for managing high-acuity conditions until underlying reasons for the reduction are fully appreciated.

13. Tractional retinal detachments.

Tractional retinal detachments (TRD) occur as a consequence of various retinal pathologies but is most commonly associated with proliferative diabetic retinopathy (PDR). Monitoring for diabetic eye disease and early identification of TRD are crucial for preventing vision loss.

BARTONELLA HENSELAE NEURORETINITIS ASSOCIATED WITH CENTRAL RETINAL VEIN OCCLUSION, CHOROIDAL ISCHEMIA, AND ISCHEMIC OPTIC NEUROPATHY.

PURPOSE: We describe a healthy 37-year-old man with Bartonella henselae (B. henselae) neuroretinitis with concurrent central retinal vein occlusion and ischemic optic neuropathy resulting in optic atrophy and choroidal ischemia. METHODS: Case report. RESULTS: A 37-year-old man presented with unilateral decreased vision and a fundus examination consistent with neuroretinitis. Further imaging review supported a concurrent diagnosis of central retinal vein occlusion. Although initially negative, repeat serological testing for B. henselae infection was positive. Multimodal imaging displayed severe outer retinal disruption, ischemic optic neuropathy, and choroidal ischemia. The patient demonstrated near complete resolution of fundus findings and restoration of outer retinal architecture. Residual findings included optic disk pallor and ischemic choroidopathy. DISCUSSION: B. henselae neuroretinitis may be associated with concurrent retinal vascular occlusive disease and ischemic optic neuropathy. Central retinal vein occlusion and choroidal ischemia leading to optic nerve atrophy are additional sequelae further expanding the clinical spectrum of this entity.

In vivo imaging of the fibrillar architecture of the posterior vitreous and its relationship to the premacular bursa, Cloquet's canal, prevascular vitreous fissures, and cisterns.

PURPOSE: To describe the fibrillar architecture of the posterior cortical vitreous and identify variations across eyes of different axial lengths in vivo. METHODS: Sixty-four eyes of 32 subjects were examined with swept-source optical coherence tomography (SS-OCT). Grading of vitreous degeneration, presence of vitreous cisterns/lacunae, posterior hyaloid status, directionality of vitreous fibers and their relations to vitreous spaces, and lamellar reflectivity of the posterior vitreous were assessed. RESULTS: A consistent pattern of fibrillar organization was discovered. Eyewall parallel fibers formed a dense meshwork over the retinal surface and fibers oriented in a perpendicular fashion to this meshwork were found to envelop the various vitreous spaces, intersecting at variable angles of insertion to the eyewall parallel fibers. Lamellar reflectivity suggestive of splitting of the cortical fibrillar meshwork was detected in 27 eyes (42%) with 56% of these eyes demonstrating perpendicularly oriented intersecting fibers. Fifty-six percent of eyes with lamellar reflectivity had an axial length > 25 mm. CONCLUSION: SS-OCT imaging revealed fibrillar organization of the posterior vitreous. Eye wall parallel hyperreflectivity of cortical vitreous was a universal finding. This pattern is suggestive of a splitting of cortical vitreous tissue and may represent a precursor to vitreoschisis. Perpendicular fibers appear to be important constituents of the walls of the various liquid vitreous spaces.

Unilateral Ocular Manifestations of Vogt-Koyanagi-Harada Disease.

PURPOSE: To describe a case of unilateral Vogt-Koyanagi-Harada (VKH) disease and associated multimodal imaging. METHODS: Retrospective case report. RESULTS: A 50-year-old Hispanic male presented with three days of painless decreased vision in his left eye, headache, and decreased hearing. His visual acuity was 20/20 in the right eye and counting fingers in the left eye. Examination of his right eye was unremarkable. Funduscopic examination of his left eye revealed multiple serous retinal detachments. Fluorescein angiography demonstrated late multifocal pinpoint hyperfluorescence in his left eye and a diagnosis of VKH disease was made. He was treated with oral prednisone. Serial re-examination demonstrated resolution of the serous retinal detachments and a taper of his oral prednisone was initiated with improvement of his visual acuity to 20/25. CONCLUSIONS: Our patient had imaging and a clinical course that was consistent with VKH disease. This unilateral presentation may represent a clinical variant of VKH disease.

MULTIMODAL IMAGING ADDS NEW INSIGHTS INTO ACUTE SYPHILITIC POSTERIOR PLACOID CHORIORETINITIS.

PURPOSE: Acute syphilitic posterior placoid chorioretinitis (ASPPC) is an uncommon manifestation of ocular syphilis with distinct clinical features. We describe new multimodal imaging findings in a patient with ASPPC. METHODS: Observational case report with multimodal imaging. RESULTS: A 44-year-old woman presented with 5 days of decreased vision in her right eye. Visual acuity was counting fingers in her right eye and 20/20 in her left eye. Funduscopic examination of the right eye showed a yellow placoid macular lesion with extension beyond the equator, which was encircled by an annular ring of outer retinal whitening. Ultra-widefield fundus autofluorescence demonstrated hyperautofluorescence corresponding to the placoid lesion. Examination of the left eye appeared unremarkable, but ultra-widefield fundus autofluorescence showed an area of hyperautofluorescence located superonasal to the optic nerve. Optical coherence tomography of the right eye demonstrated subretinal fluid and overlying disruption of the ellipsoid zone. Fluorescein angiography demonstrated early hypofluorescent and hyperfluorescent spots and late staining within the placoid lesion. Optical coherence tomography angiography showed several areas of decreased flow signal within the placoid lesion at the level of the choriocapillaris. Laboratory testing revealed a rapid plasma reagin titer of 1:1,024. Two months after treatment with intravenous penicillin G, visual acuity had improved to 20/25 in her right eye, and optical coherence tomography showed partial restoration of the ellipsoid zone. The annular ring resolved with near normalization of fundus autofluorescence and optical coherence tomography angiography demonstrated resolution of flow. CONCLUSION: Multimodal imaging provides further insight into the pathogenesis of ASPPC. Ultra-widefield fundus autofluorescence may show evidence of ellipsoid zone disruption in areas that clinically appear normal. Flow voids within the choriocapillaris in ASPPC appear to resolve with appropriate treatment, a finding that suggests a transient disruption of choriocapillaris flow in ASPPC.

CONTRALATERAL ANESTHESIA IN TWO PATIENTS AFTER RETROBULBAR BLOCK.

PURPOSE: To report a case series of two patients with contralateral anesthesia after retrobulbar block. METHODS: Retrospective review of two cases and review of the literature. RESULTS: Two patients of one practitioner received contralateral anesthesia after retrobulbar block for posterior segment surgery. Patient 1 suffered from transient contralateral akinesia, whereas Patient 2 experienced transient contralateral amaurosis. CONCLUSION: Posterior spread of anesthetics is a rare but potentially serious complication of retrobulbar anesthesia caused by spread of anesthetics along the optic nerve sheath. Modification of injection technique can decrease the risk of this complication.

IDIOPATHIC MULTIFOCAL CHOROIDITIS PRESENTING WITH A TRANSIENT PERIPAPILLARY WHITE RING.

PURPOSE: We describe with multimodal imaging the presentation and follow-up for a patient with idiopathic multifocal choroiditis and a transient peripapillary white ring. METHODS: Case report. RESULTS: A 39-year-old Asian woman was initially seen for an evaluation of lattice degeneration in 2015. Her medical history included Graves disease and psoriasis. Best-corrected visual acuity was 20/25 in her right eye and 20/25 in her left eye. Ultra-widefield fundus autofluorescence imaging showed a curvilinear hyperautofluorescent line in her right eye. One year later, the patient returned complaining of floaters in her right eye for 1 month. Her visual acuity was unchanged. Funduscopic examination showed new inflammatory yellowish lesions in the right eye corresponding to hyperreflective sub-retinal pigment epithelium lesions on structural spectral domain optical coherence tomography. Fluorescein angiography showed corresponding late staining of these active lesions. Late-phase indocyanine green angiography showed multiple nummular hypocyanescent dots. Ultra-widefield fundus autofluorescence showed large areas of hyperautofluorescence. The patient was started on a 60-mg oral prednisone taper and demonstrated subsequent regression of the inflammatory lesions. Ten months later, the patient returned emergently with complaints of floaters in both eyes for 2 days and a new temporal scotoma in her left eye. Funduscopic examination demonstrated a white ring around the optic nerve of the left eye corresponding to a hyperautofluorescent lesion. Ultra-widefield fundus autofluorescence showed new areas of hyperautofluorescence in both eyes. Structural spectral domain optical coherence tomography showed new sub-retinal pigment epithelium inflammatory lesions and a disruption of the ellipsoid zone in both eyes. The patient was again treated with a 60-mg oral prednisone taper and demonstrated subsequent restoration of the ellipsoid zone. CONCLUSION: To our knowledge, this is the first report of a transient annular white ring occurring in a case of multifocal choroiditis. There was marked restoration of the disrupted ellipsoid zone after treatment with oral corticosteroids.

An Assessment of Vitreous Degeneration in Eyes with Vitreomacular Traction and Macular Holes.

Purpose. To compare the stages of vitreous degeneration in patients with vitreomacular traction (VMT) and macular holes (MH). Methods. A retrospective study was performed analyzing stages of vitreous degeneration of eyes with VMT or MH using swept-source optical coherence tomography (SS-OCT) and spectral-domain optical coherence tomography (SD-OCT). An analogous review was performed on a control group of eyes with contralateral posterior vitreous detachments. Thirty-four eyes with VMT/MH and 39 control eyes were reviewed. Results. Twenty-seven VMT/MH eyes and 31 control eyes were included. Eyes with VMT/MH demonstrated significantly earlier stages of vitreous degeneration when compared to the control group (p = 0.048) despite significantly greater age (p = 0.032). Conclusions. Vitreoretinal interface disease is more often associated with a formed vitreous and an intact premacular bursa. This is contrary to previous assumptions implicating degeneration of vitreous as a precipitating factor of interface disease when in conjunction with abnormal vitreomacular separation.

UNUSUAL POSTERIOR VARIX OF A VORTEX VEIN AMPULLA.

PURPOSE: We describe a patient with an unusually posterior varix of a vortex vein ampulla. METHODS: Case report. RESULTS: An unusually posterior varix of a vortex vein ampulla mimicking a choroidal neoplasm was identified. The diagnosis was obscured by the presence of overlying drusen. Through a combination of indocyanine green angiography and enhanced depth imaging optical coherence tomography, the varix was identified as an engorged vascular process correlating to ophthalmoscopic and tomographic examination. Collapse of the varix after direct digital pressure on the globe was observed on enhanced depth imaging optical coherence tomography further confirming our diagnosis. CONCLUSION: Varices of vortex vein ampullas may be incorrectly diagnosed as choroidal neoplasms. Using a combination of multimodal imaging and dynamic maneuvering, varices can be correctly identified.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY OF CHORIORETINAL LESIONS DUE TO IDIOPATHIC MULTIFOCAL CHOROIDITIS.

PURPOSE: To evaluate the spectrum of macular chorioretinal lesions occurring in idiopathic multifocal choroiditis using optical coherence tomography angiography (OCTA) to evaluate those showing neovascular flow. METHODS: This was a descriptive, retrospective study of 18 eyes of 14 patients with multifocal choroiditis. Macular lesions were characterized as subretinal pigment epithelium, subretinal, or mixed and evaluated during active and presumed inactive states of multifocal choroiditis. Correlations between structural optical coherence tomography and OCTA were performed. In select cases, correlations between OCTA, fluorescein angiography, and fundus autofluorescence were evaluated. In 5 eyes, quantitative measurements of neovascular lesions were compared at baseline and following intravitreal anti-vascular endothelial growth factor therapy. RESULTS: Mean patient age was 48 years (SD: 13.8; 86% women). Optical coherence tomography angiography flow signatures consistent with neovascularization were identified in 83% of eyes, including in 0% of subretinal pigment epithelium, 91% of subretinal, and 100% of mixed lesions. Lesions that did not demonstrate definitive signs of fluorescein angiography leakage were frequently found to have neovascularization using OCTA. There was no change in quantitative measurements of neovascular lesions after anti-vascular endothelial growth factor therapy (all tested variables P > 0.05). CONCLUSION: Optical coherence tomography angiography may be a useful imaging modality for understanding the pathophysiology of multifocal choroiditis and monitoring its clinical course.

FOCAL CHOROIDAL EXCAVATION AND CHOROIDAL NEOVASCULARIZATION WITH ASSOCIATED PACHYCHOROID.

PURPOSE: To report a case of focal choroidal excavation and choroidal neovascularization in the setting of pachychoroid disease. METHODS: Multimodal imaging, including fundus photography, fundus autofluorescence, enhanced depth imaging optical coherence tomography, indocyanine green angiography, and en face structural and angiographic optical coherence tomography. RESULTS: The authors describe a 39-year-old female with moderately high myopia presenting with focal choroidal excavation and associated choroidal neovascularization. Multimodal imaging demonstrated pachychoroid features with dilated choroidal vessels surrounding the lesion. Optical coherence tomography angiography showed Type 2 neovascularization. CONCLUSION: Some cases of focal choroidal excavation and associated neovascularization may be related to structural abnormalities of the associated choroidal vasculature.

CENTRAL SEROUS CHORIORETINOPATHY TREATED WITH MINERALOCORTICOID ANTAGONISTS: A ONE-YEAR PILOT STUDY.

PURPOSE: To assess the treatment response to mineralocorticoid antagonists in a pilot study of patients diagnosed with central serous chorioretinopathy using multimodal imaging. METHODS: This retrospective observational case series included 23 eyes of 14 patients with central serous chorioretinopathy treated by a single physician (L.A.Y.) with either spironolactone, eplerenone, or both consecutively over a 12-month period. Choroidal thickness, central macular thickness, and best-corrected visual acuity were measured and compared with baseline values. Twelve eyes of 11 patients demonstrated subretinal fluid before or during the initiated treatment course. Subretinal fluid was measured and compared with baseline values in this subgroup. RESULTS: In all eyes (n = 23), best-corrected visual acuity improved at 12 months of treatment; however, central macular thickness and choroidal thickness showed no improvement. In the subgroup with subretinal fluid (n = 12), subretinal fluid was significantly decreased at 6 months and 12 months of treatment; however, central macular thickness, choroidal thickness, and best-corrected visual acuity showed no significant change. CONCLUSION: Mineralocorticoid antagonists may improve best-corrected visual acuity and decrease subretinal fluid in patients with central serous chorioretinopathy, but do not affect the choroidal or macular thickness. This pilot study demonstrates that mineralocorticoid receptor antagonists may be effective in treating central serous chorioretinopathy but warrants consideration for future research within a randomized clinical trial.

Aflibercept: a review of its use in the treatment of choroidal neovascularization due to age-related macular degeneration.

Choroidal neovascularization (CNV) due to age-related macular degeneration (AMD) is an important cause of visual morbidity globally. Modern treatment strategies for neovascular AMD achieve regression of CNV by suppressing the activity of key growth factors that mediate angiogenesis. Vascular endothelial growth factor (VEGF) has been the major target of neovascular AMD therapy for almost two decades, and there have been several intravitreally-administered agents that have enabled anatomical restitution and improvement in visual function with continual dosing. Aflibercept (EYLEA(®)), initially named VEGF Trap-eye, is the most recent anti-VEGF agent to be granted US Food and Drug Administration approval for the treatment of neovascular AMD. Biologic advantages of aflibercept include its greater binding affinity for VEGF, a longer intravitreal half-life relative to other anti-VEGF agents, and the capacity to antagonize growth factors other than VEGF. This paper provides an up-to-date summary of the molecular mechanisms mediating CNV. The structural, pharmacodynamic, and pharmacokinetic advantages of aflibercept are also reviewed to rationalize the utility of this agent for treating CNV. Results of landmark clinical investigations, including VIEW 1 and 2 trials, and other important studies are then summarized and used to illustrate the efficacy of aflibercept for managing treatment-naïve CNV, recalcitrant CNV, and CNV due to polypoidal choroidal vasculopathy. Safety profile, patient tolerability, and quality of life measures related to aflibercept are also provided. The evidence provided in this paper suggests aflibercept to be a promising agent that can be used to reduce the treatment burden of neovascular AMD.

Measurement of local retinal ganglion cell layer thickness in patients with glaucoma using frequency-domain optical coherence tomography.

OBJECTIVE: To explore the feasibility of obtaining a local measurement of the thickness of the retinal ganglion cell layer in patients with glaucoma using frequency-domain optical coherence tomography (fdOCT) and a computer-aided manual segmentation procedure. METHODS: The fdOCT scans were obtained from the horizontal midline for 1 eye of 26 patients with glaucoma and 20 control subjects. The thickness of various layers was measured with a manual segmentation procedure aided by a computer program. The patients were divided into low- and high-sensitivity groups based on their foveal sensitivity on standard automated perimetry. RESULTS: The RGC plus inner plexiform and the retinal nerve fiber layers of the low-sensitivity group were significantly thinner than those of the high-sensitivity group. While these layers were thinner in the patients than the controls, the thicknesses of inner nuclear layer and receptor layer were similar in all 3 groups. Further, the thinning of the retinal ganglion cell plus inner plexiform layer in 1 glaucoma-affected eye showed qualitative correspondence to the loss in 10-2 visual field sensitivity. CONCLUSIONS: Local measures of RGC layer thickness can be obtained from fdOCT scans using a manual segmentation procedure, and these measures show qualitative agreement with visual field sensitivity.

An analysis of normal variations in retinal nerve fiber layer thickness profiles measured with optical coherence tomography.

PURPOSE: To assess the normal variations in retinal nerve fiber layer (RNFL) thickness measured with optical coherence tomography (OCT). SUBJECTS AND METHODS: Both eyes of 48 individuals (age 56.4+/-9.5 y) with normal vision and refractive errors between +/-6.0 D were tested with the fast RNFL scan protocol of the OCT3 (Zeiss Meditech). Their 256-point RNFL profiles were exported for analysis. The location and peak amplitude of the maxima of the RNFL profiles were measured. Intersubject and interocular variations were assessed with a coefficient of determination, R2. An R2 of 1.0 indicated that the average profile from all 48 individuals (or of the 2 eyes) accounted for 100% of the variation of an individual eye's profile. RESULTS: The R2 for the interocular comparison was good, with averages of 0.91+/-0.07 (right eye) and 0.92+/-0.05 (left eye). The R2 for the comparison of the individual's profile to the mean group profile was only 0.61+/-0.29 (right eye) and 0.65+/-0.24 (left eye), with 27% of the R2 values below 0.5. Even after normalizing each individual's profile by its mean, R2 was only 0.75+/-0.16 (0.75+/-0.16) for the right (left) eye. The location of the peaks for the right (left) eye ranged over 91 degrees (88 degrees) for the superior peak and over 64 degrees (66 degrees) for the inferior peak. The range of peak amplitudes for the right (left) eye spanned a factor of 1.7 (1.8) and 2.0 (1.7) for the superior and inferior peaks, respectively. CONCLUSIONS: There was a wide variation in the amplitude and shape of the individual RNFL profiles. However, the RNFL profiles of the 2 eyes of an individual were extremely similar. Adding an interocular comparison with OCT RNFL tests should help identify some false positives.

A comparison of multifocal and conventional visual evoked potential techniques in patients with optic neuritis/multiple sclerosis.

PURPOSE: To compare conventional visual evoked potential (cVEP) and multifocal visual evoked potential (mfVEP) methods in patients with optic neuritis/multiple sclerosis (ON/MS). METHODS: mfVEPs and cVEPs were obtained from eyes of the 19 patients with multiple sclerosis confirmed on MRI scans, and from eyes of 40 normal controls. For the mfVEP, the display was a pattern-reversal dartboard array, 48 degrees in diameter, which contained 60 sectors. Monocular cVEPs were obtained using a checkerboard stimulus with check sizes of 15' and 60'. For the cVEP, the latency of P100 for both check sizes were measured, while for the mfVEP, the mean latency, percent of locations with abnormal latency, and clusters of contiguous abnormal locations were obtained. RESULTS: For a specificity of 95%, the mfVEP(interocular cluster criterion) showed the highest sensitivity (89.5%) of the 5 monocular or interocular tests. Similarly, when a combined monocular/interocular criterion was employed, the mfVEP(cluster criterion) had the highest sensitivity (94.7%)/specificity (90%), missing only one patient. The combined monocular/interocular cVEP(60') test had a sensitivity (84.2%)/specificity (90%), missing 3 patients, 2 more than did the monocular/interocular mfVEP(cluster) test. CONCLUSION: As the cVEP is more readily available and currently a shorter test, it should be used to screen patients for ON/MS with mfVEP testing added when the cVEP test is negative and the damage is local.