Retinal Vasculitis in a Patient with Hansen's Disease.

PURPOSE: Hansen's disease is endemic in over 140 countries worldwide and a potentially blinding condition. We describe a case of retinal vasculitis in a patient with Hansen's disease with concomitant positive antiphospholipid antibody serology, a potentially under-reported complication in this setting. METHODS: A 37-year-old Brazilian man systemically stable on triple therapy (clofazimine, rifampin, dapsone) for Hansen's disease presented for a comprehensive ophthalmic evaluation. RESULTS: Dilated exam revealed diffuse peripheral intraretinal hemorrhages in his right eye. Fluorescein angiography showed peripheral non-perfusion, abnormal shunt vessels and leakage from the retinal veins in the right eye and peripheral non-perfusion and vascular leakage in the fellow eye, consistent with vasculitis. Laboratory workup was notable for positive antiphospholipid antibodies (lupus anticoagulant, anticardiolipin IgM, anti-beta-2 glycoprotein 1 IgM) and normocytic anemia. CONCLUSION: As antiphospholipid antibodies are present in a large proportion of patients with Hansen's disease, it is possible that retinal vasculitis may be more common than previously thought. The extent to which retinal vasculitis occurs in Hansen's disease remains uncertain and possibly underestimated due to the frequency of anterior segment scarring, which impedes retinal evaluation. Heightened surveillance for potential retinal vascular complications is warranted.

Severe Recurrence and Retinal Inflammatory Infiltration After Cessation of Immunosuppression for Multifocal Choroiditis and Panuveitis.

PURPOSE: To describe a severe recurrence of intraocular inflammation following the cessation of immunosuppression, previously administered for multifocal choroiditis and panuveitis (MCP). METHODS: Retrospective chart review. RESULTS: A 27-year-old woman with MCP initially was treated with intravenous and oral corticosteroids and photodynamic therapy because of an active macular neovascularization in both eyes. Mycophenolate was soon started and the recurrences during tapering of the oral corticosteroids in the first months were treated with periocular corticosteroids and anti-vascular endothelial growth factor injections as they became available. After a decade of immunosuppression without recurrences, the patient, having relocated, discontinued mycophenolate upon the advice of a new ophthalmologist who diagnosed her with punctate inner choroidopathy. This led to a severe recurrence in both eyes, characterized by new inflammatory lesions, ellipsoid zone loss, and widespread inflammatory cell infiltration into the outer retina. Intravitreal triamcinolone injections resulted in the resolution of sub- and intraretinal inflammatory lesions and ellipsoid zone defects. CONCLUSION: The abrupt discontinuation of immunosuppression in a patient with MCP was associated with a rebound phenomenon, characterized by multi-level inflammatory activity in the posterior pole. This rebound phenomenon may offer clues as to the inflammatory targets in MCP.

Retinal vessel volume reference database derived from volume-rendered optical coherence tomography angiography.

Optical coherence tomography angiography (OCTA) enables three-dimensional reconstruction of the functional blood vessels in the retina. Therefore, it enables the quantification of 3D retinal vessel parameters such as surface area and vessel volume. In spite of the widespread use of OCTA, no representative volume-rendered vessel volume (VV) data are published to date. In this study, OCTA 3 × 3 mm macular cubes were processed with volume-rendering techniques to measure VV in 203 eyes from 107 healthy volunteers. Generalized linear models (GLM) were constructed to assess the impact of age, gender, visual acuity (VA), spherical equivalent (SE), and axial length (AL) on VV. Overall mean VV was 0.23 ± 0.05mm3. Age and axial length showed a negative correlation with VV. However, GLM model analysis found that AL exerted the most pronounced influence on VV. No statistically significant associations were identified between gender or between left and right eyes. This is the first study to assess 3D OCTA VV and its naturally occurring variations in a large series of healthy subjects. It offers novel insights into the characterization of normal retinal vascular anatomy in healthy individuals, contributing to a valuable reference for future research in this field.

PRIMARY OCCLUSIVE PANVASCULITIS WITH SEGMENTAL PERIARTERIAL PLAQUES.

PURPOSE: The purpose of this study was to describe a new clinical entity of bilateral occlusive panvasculitis with segmental periarterial plaques and its clinical course in two patients. METHODS: This was a retrospective chart review. RESULTS: Two patients with no medical history of any systemic inflammatory diseases presented with bilateral segmental periarterial plaques (Kyrieleis plaques), cotton wool spots, and microaneurysms. Segmental leakage, staining, and vascular occlusions involved the arterioles, venules, and capillaries. Leakage from the superficial capillary plexus in some areas bordering deep capillary plexus nonperfusion was observed. Both had recurrent episodes of vascular occlusions, normal brain MRI, and audiology tests. Complete workup including serology for infections, inflammatory markers, and antibody titers was unremarkable. They were started on with corticosteroids and immunosuppressant, and there were no further vascular occlusions. Both developed neovascularization adjacent to the areas of capillary nonperfusion. The second patient also developed vitreomacular traction and cystoid macular edema. He required intravitreal anti-VEGF injection, sector laser photocoagulation, and underwent a vitrectomy with membrane peeling. At the last visit, the visual acuity was 20/30 in both eyes for the first patient and 20/20 in the right eye and 20/40 in the left eye for the second patient, 12 and 6.5 years after initial presentation, respectively. CONCLUSION: Both patients presented with findings of bilateral panvasculitis with prominent segmental periarterial plaques and had repeated episodes of vascular occlusions before corticosteroid and immunosuppressants treatment, after which no additional occlusions were observed. We propose that the constellation of findings constitutes a novel clinical entity, occlusive panvasculitis with segmental periarterial plaques.

RECONSTITUTION OF THE CHORIOCAPILLARIS IN ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY.

PURPOSE: The purpose of this study was to describe findings derived from extensions of optical coherence tomography, including volume rendering and frame-averaged optical coherence tomography angiography (OCT-A), in a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE). METHODS: This is a case report of a patient with APMPPE imaged during the acute and convalescent stages. RESULTS: A 20-year-old man presented with an acute change in vision. He had multiple yellow-white placoid lesions at the level of the retinal pigment epithelium in the macula and nasal to the optic nerve in both eyes. Within 2 weeks, his visual acuity worsened to 20/80 and 20/400 in the right and left eyes, respectively. Spectral domain OCT showed focal defects in the ellipsoid and retinal pigment epithelium layers. Volume-rendering OCT-A showed inflammatory cells in the outer nuclear layer above the APMPPE lesion. Frame-averaged OCT-A revealed significant loss of capillary flow signal within capillary segments of the choriocapillaris. Ten weeks after presentation, there was resolution of the placoid changes, discontinuance of the inflammatory infiltrate in the outer nuclear layer, and significant reconstitution of flow in the choriocapillaris. The visual acuity was 20/20 in both eyes. CONCLUSION: Novel volume-rendered and frame-averaged OCT-A images in a patient with APMPPE allowed detection of inflammatory cell infiltrate in the outer nuclear layer and reversible capillary segment nonperfusion of associated APMPPE lesions. The findings suggest short-term insults to choriocapillaris function may be reversible and can be tracked with modern imaging techniques.

Retinal Pigment Epithelial Detachments Devoid of Retinal Pigment Epithelium.

PURPOSE: To describe two patients with chronic central serous chorioretinopathy (CSC) showing what appeared to be retinal pigment epithelium detachments (PED) lacking imaging findings consistent with retinal pigment epithelium (RPE) over the elevation. METHOD: The patients underwent comprehensive ophthalmic examination, including multicolor fundus photography, fundus autofluorescence (FAF), and spectral-domain optical coherence tomography (SD-OCT). RESULTS: A 70-year-old male and a 58-year-old male, both diagnosed with chronic CSC, showed PED-like lesions that were hypoautofluorescent, suggesting an absence of RPE. SD-OCT B-scans showed serous, dome-shaped elevations composed of a narrow, mildly hyperreflective band (9-10 µm thick) that demonstrated hypertransmission of light. The material that constituted the elevation was contiguous with the outer portion of the RPE band at the lesion borders. CONCLUSION: Based on the multimodal imaging findings we hypothesize that these elevations of the retina have lost their overlying RPE. A thin layer of material that could represent a residual layer of basal laminar deposit produced by the RPE remains overlying the detachments, possibly accounting for their dome shape and structural stability.

Machine Learning to Predict Response to Ranibizumab in Neovascular Age-Related Macular Degeneration.

Purpose: Neovascular age-related macular degeneration (nAMD) shows variable treatment response to intravitreal anti-VEGF. This analysis compared the potential of different artificial intelligence (AI)-based machine learning models using OCT and clinical variables to accurately predict at baseline the best-corrected visual acuity (BCVA) at 9 months in response to ranibizumab in patients with nAMD. Design: Retrospective analysis. Participants: Baseline and imaging data from patients with subfoveal choroidal neovascularization secondary to age-related macular dengeration. Methods: Baseline data from 502 study eyes from the HARBOR (NCT00891735) prospective clinical trial (monthly ranibizumab 0.5 and 2.0 mg arms) were pooled; 432 baseline OCT volume scans were included in the analysis. Seven models, based on baseline quantitative OCT features (Least absolute shrinkage and selection operator [Lasso] OCT minimum [min], Lasso OCT 1 standard error [SE]); on quantitative OCT features and clinical variables at baseline (Lasso min, Lasso 1SE, CatBoost, RF [random forest]); or on baseline OCT images only (deep learning [DL] model), were systematically compared with a benchmark linear model of baseline age and BCVA. Quantitative OCT features were derived by a DL segmentation model on the volume images, including retinal layer volumes and thicknesses, and retinal fluid biomarkers, including statistics on fluid volume and distribution. Main Outcome Measures: Prognostic ability of the models was evaluated using coefficient of determination (R2) and median absolute error (MAE; letters). Results: In the first cross-validation split, mean R2 (MAE) of the Lasso min, Lasso 1SE, CatBoost, and RF models was 0.46 (7.87), 0.42 (8.43), 0.45 (7.75), and 0.43 (7.60), respectively. These models ranked higher than or similar to the benchmark model (mean R2, 0.41; mean MAE, 8.20 letters) and better than OCT-only models (mean R2: Lasso OCT min, 0.20; Lasso OCT 1SE, 0.16; DL, 0.34). The Lasso min model was selected for detailed analysis; mean R2 (MAE) of the Lasso min and benchmark models for 1000 repeated cross-validation splits were 0.46 (7.7) and 0.42 (8.0), respectively. Conclusions: Machine learning models based on AI-segmented OCT features and clinical variables at baseline may predict future response to ranibizumab treatment in patients with nAMD. However, further developments will be needed to realize the clinical utility of such AI-based tools. Financial Disclosures: Proprietary or commercial disclosure may be found after the references.

VENOUS OVERLOAD CHOROIDOPATHY ASSOCIATED WITH IDIOPATHIC INTRACRANIAL HYPERTENSION.

PURPOSE: To report a case of venous overload choroidopathy associated with idiopathic intracranial hypertension in a 41-year-old man. METHODS: History and clinical examination, fluorescein angiography, ultra-widefield indocyanine green angiography, swept-source optical coherence tomography, and contrast-enhanced magnetic resonance imaging. RESULTS: The patient was diagnosed as having idiopathic intracranial hypertension 2 years ago, was being managed on oral acetazolamide, and retained 20/20 visual acuity in both eyes until now when he presented with a complaint of visual loss in the right eye. Ophthalmoscopy revealed serous detachment in the fovea of the right eye. Swept-source optical coherence tomography showed the presence of subretinal fluid and detachments of the retinal pigment epithelium in both eyes. Fundus fluorescein angiography showed multiple dot leaks at the level of the retinal pigment epithelium in both eyes. Indocyanine green angiography revealed vortex vein anastomoses and choroidal vascular hyperpermeability in both eyes. Contrast-enhanced magnetic resonance imaging revealed dilated optic nerve sheath diameter and a partial empty sella and magnetic resonance venography showed bilateral stenosis of the transverse sinus. CONCLUSION: Transverse sinus stenosis is a common finding in "idiopathic" intracranial hypertension and contributes to the intracranial pressure through intracranial venous hypertension. Increased venous back pressure seemed to have overloaded the choroid resulting in choroidal vascular congestion and hyperpermeability, leaks from the level of the retinal pigment epithelium, and accumulation of subretinal fluid.

IMI-Nonpathological Human Ocular Tissue Changes With Axial Myopia.

Purpose: To describe nonpathological myopia-related characteristics of the human eye. Methods: Based on histomorphometric and clinical studies, qualitative and quantitative findings associated with myopic axial elongation are presented. Results: In axial myopia, the eye changes from a spherical shape to a prolate ellipsoid, photoreceptor, and retinal pigment epithelium cell density and total retinal thickness decrease, most marked in the retroequatorial region, followed by the equator. The choroid and sclera are thin, most markedly at the posterior pole and least markedly at the ora serrata. The sclera undergoes alterations in fibroblast activity, changes in extracellular matrix content, and remodeling. Bruch's membrane (BM) thickness is unrelated to axial length, although the BM volume increases. In moderate myopia, the BM opening shifts, usually toward the fovea, leading to the BM overhanging into the nasal intrapapillary compartment. Subsequently, the BM is absent in the temporal region (such as parapapillary gamma zone), the optic disc takes on a vertically oval shape, the fovea-optic disc distance elongates without macular BM elongation, the angle kappa reduces, and the papillomacular retinal vessels and nerve fibers straighten and stretch. In high myopia, the BM opening and the optic disc enlarge, the lamina cribrosa, the peripapillary scleral flange (such as parapapillary delta zone) and the peripapillary choroidal border tissue lengthen and thin, and a circular gamma and delta zone develop. Conclusions: A thorough characterization of ocular changes in nonpathological myopia are of importance to better understand the mechanisms of myopic axial elongation, pathological structural changes, and psychophysical sequelae of myopia on visual function.

Scleral Thickness in Autosomal Dominant Best Vitelliform Macular Dystrophy.

PURPOSE: To investigate the posterior and equatorial scleral thickness in patients with autosomal dominant Best disease, a condition that has chronic subretinal fluid. METHODS: Retrospective study involving patients with Best disease and age-matched controls. Participants were evaluated with contact B-scan ultrasonography and enhanced depth imaging optical coherence tomography to evaluate scleral thickness in the posterior pole and equator. Univariate analysis and generalized estimating equations were used. RESULTS: Of 9 patients with genetically proven Best disease and 23 age-matched controls, there was no significant difference in the age or the gender proportion between groups. Subfoveal choroidal thickness and axial length were not significantly different between groups. Both posterior scleral (OD; 1.38mm vs. 0.89mm, P<.001 and OS; 1.39mm vs. 0.83mm, P<.001) and equatorial scleral (OD; 0.61mm vs. 0.42mm, P=.003, and OS; 0.55mm vs. 0.41mm, P=.017) thicknesses were much greater in cases as compared with controls. Multivariate analysis showed male sex and having Best disease were each significant predictors of posterior scleral thickness and Best disease was the sole significant predictor for equatorial scleral thickness. CONCLUSION: BEST1 gene may have a developmental role leading to having a thicker sclera, influencing disease manifestation, and contributing to the accumulation of subretinal fluid in Best disease.

COMPLEMENT INHIBITION FOR GEOGRAPHIC ATROPHY: Review of Salient Functional Outcomes and Perspective.

PURPOSE: To evaluate available rationale and outcomes of randomized trial results for complement inhibition for geographic atrophy. METHODS: Data from recently completed randomized trials of complement inhibition, particularly for pegcetacoplan and avacincaptad pegol, were evaluated for both the outcome, area of autofluorescence loss, and functional vision tests. RESULTS: Pegcetacoplan 2 mg showed statistically significant reduction in expansion of the area of autofluorescence loss with monthly, but not every-other-month dosing, in a 12-month phase two trial. Nearly 40% of patients recruited for the monthly arm did not complete the treatment. In two parallel phase 3 studies there was a statistically significant reduction in the area of atrophy in one but not both studies as compared with untreated controls. Data released at 24 months follow-up showed statistically significant reduction in the area of autofluorescence-detected atrophy in both studies compared with sham. Patients did not show functional difference in best-corrected visual acuity, maximum reading speed, Functional Reading Independence Index, and mean microperimetry threshold sensitivities in the treatment versus sham arms. Avacincaptad pegol was evaluated in two randomized pivotal studies and showed a statistically significant reduction in the expansion of autofluorescence loss at 12 months. Patients in the treatment arms did not show any difference as compared with sham in the best-corrected visual acuity or low luminance visual acuity, the only functional outcomes mentioned. Both drugs increased the risk of macular neovascularization. CONCLUSION: Both avacincaptad pegol and pegcetacoplan show significant differences compared with sham in autofluorescence imaging but no benefit in visual function at 12 and 24 months, respectively.

THE WAY PATIENTS SEE FLOATERS: Widefield Dynamic Scanning Laser Ophthalmoscopy Imaging of Vitreous Abnormalities.

PURPOSE: To investigate the use of dynamic widefield scanning laser ophthalmoscopy (SLO) and B-scan ultrasonography in imaging vitreous abnormalities in patients with complaints of floaters. METHODS: Twenty-one patients underwent both dynamic SLO and B-scan ultrasonography to image their vitreous abnormalities. After reviewing these videos, patients graded each imaging technique on a scale of 1 to 10, based on how closely it represented their visual perception of floaters. RESULTS: The mean age of the patients (12 women and nine men) was 47.7 ± 18.5 years. The patients graded a median score of nine for SLO imaging (mean = 8.43) compared with a median score of 5 (mean = 4.95) for ultrasound ( P = 0.001). Widefield SLO imaging demonstrated three-dimensional interconnectivity within the condensations of the formed vitreous that exhibited translational and rotational movements with eye saccades. CONCLUSION: Floaters are a common complaint, but it is difficult to know whether imaging findings of the vitreous correlate to what patients perceive. Widefield SLO seems to image vitreous abnormalities related to how patients perceive their own floaters better than B-scan ultrasonography. Despite the term "floaters", the vitreous abnormalities in the videos seemed to be manifestations of a complex three-dimensional degeneration of the vitreous framework.

Bulbosities and Intervortex Venous Anastomosis in Venous Overload Choroidopathy Masquerading as Polypoidal Choroidal Vasculopathy.

PURPOSE: To describe a patient with venous overload choroidopathy in whom venous bulbosities masqueraded as polyps and intervortex venous anastomosis mimicked a branching vascular network, giving the appearance of polypoidal choroidal vasculopathy (PCV). METHODS: The patient had complete ophthalmic examination including indocyanine green angiography (ICGA) and optical coherence tomography (OCT). Venous bulbosities were defined on ICGA as focal dilations in which the diameter of the dilation is 2 times that of the host vessel. RESULTS: A 75-year-old female presented with combined subretinal and sub-retinal pigment epithelium (RPE) hemorrhages in the right eye. During ICGA, focal nodular hyperfluorescent lesions connected to a network of vessels were observed, which looked like polyps and branching vascular network in PCV. In both eyes, the mid-phase angiogram had multifocal choroidal vascular hyperpermeability. There was late-phase placoid staining nasal to the nerve in the right eye. During EDI-OCT evaluation, there were no RPE elevations that would be expected with polyps or branching vascular network in the right eye. A double layer sign was seen corresponding to the placoid area of staining. Diagnosis of venous overload choroidopathy and choroidal neovascularization membrane was made. She was treated with intravitreal anti-vascular endothelial growth factor injections for the choroidal neovascularization membrane. CONCLUSION: ICGA findings in venous overload choroidopathy may mimic PCV, but differentiation is essential as it has implications for treatment. Similar findings may have been misinterpreted in the past and may have previously contributed to conflicting clinical and histopathologic descriptions of PCV.

HEALING MECHANISMS AFTER MACULAR HOLE REPAIR SUGGESTS PROCESS OF FOVEATION.

PURPOSE: To evaluate the long-term healing of the fovea following macular hole surgery in light of the processes known to occur during the maturation of the fovea during childhood, in which there is migration of the inner retinal layers away from and the photoreceptors toward the center of the macula. METHODS: Consecutive patients with longer than 1 year of follow-up were evaluated with spectral-domain optical coherence tomography. The interinner nuclear layer distance, which was the separation across the fovea of the midpoints of the inner nuclear layers, and the ONL (outer nuclear layer) to RPE (retinal pigment epithelium) thicknesses were measured. RESULTS: There were 22 eyes of 21 patients, 16 of whom were female; the mean age was 65.6 years. Initially, after the surgery, there was closure of the hole with continuity of the inner and middle layers of the retina across the previous macular hole. Over time, there was a migration of the inner retinal layers away from and movement of the ONL toward the center of the macula. The mean interinner nuclear layer distance increased from 352 µ m to 549 µ m ( P = 0.001), and the ONL-RPE thickness increased from 77 µ m to 146 µ m ( P < 0.001). The mean logMAR acuity improved 6.5 lines. CONCLUSION: Healing of a macular hole after surgery seems to recapitulate the development of the macula in childhood. The process of foveation may have evolutionary benefit of optimizing macular function after injury despite the absence of effective replication of the highly specialized, postmitotic photoreceptors.

Describing The Location and Morphology of The Dots in White Dot Fovea Using Adaptive Optics.

PURPOSE: To characterize the white dots in white dot fovea, an entity chiefly described in Japan, using adaptive optics (AO) imaging and propose a hypothesis regarding their origin. METHOD: The patient underwent comprehensive ophthalmic examination, including color and green-monochromatic fundus photography, fluorescein angiography (FA), spectral-domain optical coherence tomography (SD-OCT), and AO imaging. RESULTS: An asymptomatic 66-year-old Korean woman presented with bilateral white, glistening deposits in a ring pattern around the fovea of both eyes. The dots were highly reflective on green monochromatic images, were not visible on FA, and appeared hyperreflective in the inner retina using SD-OCT. AO showed polygonal granules ranging from 1-10 microns in size. The patient denied any systemic disorder or the use of any medications related to crystalline retinopathy. The patient had lived in Japan for 35 years prior to just moving to the United States. CONCLUSION: The location, small size, and polygonal shape of the dots in white dot fovea are suggestive of intraretinal deposition of crystals, and not cells on the surface of the fovea as has been previously proposed. Carotenoid deposition related to dietary habits is a plausible cause because of the similarity to other retinopathies associated to these compounds.

Deep Learning to Predict Geographic Atrophy Area and Growth Rate from Multimodal Imaging.

OBJECTIVE: To develop deep learning models for annualized geographic atrophy (GA) growth rate prediction using fundus autofluorescence (FAF) images and spectral-domain OCT volumes from baseline visits, which can be used for prognostic covariate adjustment to increase power of clinical trials. DESIGN: This retrospective analysis estimated GA growth rate as the slope of a linear fit on all available measurements of lesion area over a 2-year period. Three multitask deep learning models-FAF-only, OCT-only, and multimodal (FAF and OCT)-were developed to predict concurrent GA area and annualized growth rate. PARTICIPANTS: Patients were from prospective and observational lampalizumab clinical trials. METHODS: The 3 models were trained on the development data set, tested on the holdout set, and further evaluated on the independent test sets. Baseline FAF images and OCT volumes from study eyes of patients with bilateral GA (NCT02247479; NCT02247531; and NCT02479386) were split into development (1279 patients/eyes) and holdout (443 patients/eyes) sets. Baseline FAF images from study eyes of NCT01229215 (106 patients/eyes) and NCT02399072 (169 patients/eyes) were used as independent test sets. MAIN OUTCOME MEASURES: Model performance was evaluated using squared Pearson correlation coefficient (r2) between observed and predicted lesion areas/growth rates. Confidence intervals were calculated by bootstrap resampling (B = 10 000). RESULTS: On the holdout data set, r2 (95% confidence interval) of the FAF-only, OCT-only, and multimodal models for GA lesion area prediction was 0.96 (0.95-0.97), 0.91 (0.87-0.95), and 0.94 (0.92-0.96), respectively, and for GA growth rate prediction was 0.48 (0.41-0.55), 0.36 (0.29-0.43), and 0.47 (0.40-0.54), respectively. On the 2 independent test sets, r2 of the FAF-only model for GA lesion area was 0.98 (0.97-0.99) and 0.95 (0.93-0.96), and for GA growth rate was 0.65 (0.52-0.75) and 0.47 (0.34-0.60). CONCLUSIONS: We show the feasibility of using baseline FAF images and OCT volumes to predict individual GA area and growth rates using a multitask deep learning approach. The deep learning-based growth rate predictions could be used for covariate adjustment to increase power of clinical trials. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

HIGH-RESOLUTION SPECTRAL-DOMAIN OPTICAL COHERENCE TOMOGRAPHY OF MULTIPLE EVANESCENT WHITE DOT SYNDROME.

PURPOSE: To evaluate a patient with multiple evanescent white dot syndrome with multimodal imaging including high-resolution spectral-domain optical coherence tomography. METHODS: The patient was evaluated with wide-field color and autofluorescence imaging, microperimetry, and near-infrared imaging. Spectral-domain optical coherence tomography was performed using an instrument capable of 3- µ m axial resolution, the high-resolution Heidelberg Spectralis. RESULTS: A 28-year-old woman developed photopsias and a scotoma in the field of vision of her left eye. She had multiple whitish spots with granularity in her fovea, consistent with the diagnosis of multiple evanescent white dot syndrome. She had supportive fluorescein angiographic and autofluorescence findings. Because of the high resolution and good layer contrast, it was possible to create en face slab images of the external limiting membrane, ellipsoid zone, interdigitation zone, and retinal pigment epithelium. The external limiting membrane showed no abnormalities. There were multiple regions of decreased reflectance in the ellipsoid zone slab but even more prominent changes in the interdigitation zone. The retinal pigment epithelium showed nearly no variation in layer reflectivity. With resolution of symptoms, the color and autofluorescence images returned to normal, the defects in the ellipsoid zone almost completely resolved, and the interdigitation zone continued to show abnormalities. CONCLUSION: Although past studies concluded that the ellipsoid zone was the main region of involvement in multiple evanescent white dot syndrome, high-resolution spectral-domain optical coherence tomography suggests the interdigitation zone was more prominently affected in this case.