Search

Purpose: To investigate changes to the vitreoretinal interface in uveitis with multimodal imaging including adaptive optics., Methods: Four eyes (four patients) affected by fovea-attached (subtype 1A) or fovea-sparing epiretinal membranes (ERMs) on spectral-domain optical coherence tomography or visible internal limiting membrane (ILM) on infrared scanning laser ophthalmoscope (SLO) fundus imaging were recruited in this pilot study. The microstructure of the vitreoretinal interface was imaged using flood-illumination adaptive optics (FIAO), and the images were compared with the cross-sectional spectral-domain optical coherence tomography data., Results: Adaptive optics images revealed multiple abnormalities of the vitreoretinal interface, such as deep linear striae in ERM, and hyperreflective microstructures at the location of ERMs and ILMs. The cone mosaic was imaged by FIAO and was found altered in the four eyes with ERMs or visible ILM. The same four eyes presented alteration of photopic 30 Hz flicker that was reduced in amplitude indicating cone inner retinal layer dysfunction., Conclusion: FIAO imaging can identify specific patterns associated with ERMs and ILMs. Correlating FIAO imaging of the vitreomacular interface with the structural alterations seen in FIAO at the level of the outer retinal structures can help understand the cause of significant macular dysfunction associated with ERM.

The pachychoroid disease spectrum is a phenotype characterized by alterations in choroidal vasculature which result in outer retinal and choriocapillaris damage and visual loss. The presence of pachyvessels is one of the key features of the pachychoroid phenotype. Recent imaging studies suggest that pachyvessels may form because of choroidal venous congestion in one or more quadrants. The formation of intervortex anastomosis may function as a compensatory mechanism to dissipate the increased venous pressure, while outflow obstruction has been hypothesized to occur at the site of the vortex vein exiting the sclera. This review aims to summarize recent imaging findings and discuss evolution in the understanding of pathogenesis of the pachychoroid disease spectrum. We have summarized notable treatment trials in central serous chorioretinopathy and polypoidal choroidal vasculopathy and included an update of the current diagnostic and management strategies of the entities that are part of the pachychoroid disease spectrum., (© 2024. The Author(s).)

Objective: To identify characteristics and visual outcomes of coagulase-negative staphylococcal (CoNS) endophthalmitis in the era after the Endophthalmitis Vitrectomy Study., Design: Single-centre retrospective analysis., Participants: Forty-two samples from 40 patients with documented CoNS endophthalmitis., Methods: Visual acuity outcomes of CoNS endophthalmitis were assessed in relation to species and type of treatment instituted (i.e., pars plana vitrectomy [PPV] versus vitreous tap and injection of intravitreal antibiotics [T&I]) on 42 samples from 40 patients., Results: Staphylococcus epidermidis was the most prevalent CoNS in our study. Cataract surgery and intravitreal injections were the most common sources for acute CoNS endophthalmitis. Eyes presenting with hand motion or better vision had similar mean final vision after either intravitreal antibiotics or PPV, whereas those with light perception or worse vision at onset had better outcomes after PPV only. Subanalysis showed that patients with S. epidermidis endophthalmitis (n = 39 eyes) had similar visual outcomes with either intravitreal injections or PPV regardless of visual acuity. Hypopyon and vitritis are not always present., Conclusions: Patients with S. epidermidis endophthalmitis may benefit similarly from either early vitrectomy or intravitreal antibiotic injections regardless of visual acuity. This finding may be a supplement to the complements the management standards set forth by the Endophthalmitis Vitrectomy Study., (Copyright © 2023. Published by Elsevier Inc.)

Background: Anterior uveitis, inflammation of the anterior chamber and related structures, is a cohort of diseases that can present to almost any general or sub-specialty Ophthalmology practice. Its features classically involve anterior chamber cell and flare. Below the surface of these two signs exist a panoply of diagnoses. BODY: The purpose of this review is to provide a general framework for diagnoses of anterior uveitis that are often missed as well as non-uveitic pathologies that often mimic anterior uveitis. Diagnostic deviation in either direction can have vision-threatening and rarely life-threatening consequences for patients. Using a comprehensive literature review we have collected a broad spectrum of etiologies of anterior uveitis that are easily missed and non-uveitic pathologies that can masquerade as anterior uveitis., Conclusions: We present a focused review on specific misdiagnosed anterior uveitis pathologies and some of the conditions that can masquerade as anterior uveitis and scleritis., (© 2024. The Author(s).)

Background: Optical coherence tomography angiography (OCTA) is a noninvasive imaging modality used to analyze the retinochoroidal vasculature and detect vascular flow. The resulting images can be segmented to view each vascular plexus individually. While fluorescein angiography is still the gold standard for the diagnosis of posterior uveitis, it has limitations, and can be replaced by OCTA in some cases., Methods: This case series describes five patients with posterior noninfectious uveitis and their description by OCTA., Results: Cases included lupus retinopathy ( n = 1) for which OCTA showed ischemic maculopathy as areas of flow deficit at the superficial and deep capillary plexus; choroidal granulomas ( n = 1) with a non-detectable flow signal in the choroid; active punctate inner choroiditis and multifocal choroiditis ( n = 1) with OCTA that showed active inflammatory chorioretinal lesions as non-detectable flow signals in choriocapillaris and choroid; dense type 2 inflammatory secondary neovascularization ( n = 1) associated with active choroiditis; and acute posterior multifocal placoid pigment epitheliopathy (APMPPE) ( n = 1) without flow abnormalities at the superficial and deep retinal plexuses but non-detectable flow at the levels of the choriocapillaris and choroid., Conclusions: Ophthalmologists can use OCTA to identify inflammatory changes in retinal and choroidal vasculature, aiding in the diagnosis, management, and monitoring of posterior uveitis.

Purpose: To describe longitudinal, anatomical, and functional alterations caused by inflammatory and neovascular lesions of idiopathic multifocal choroiditis/punctate inner choroidopathy using adaptive optics imaging and microperimetry., Methods: Longitudinal case study using multiple imaging modalities, including spectral-domain optical coherence tomography, fluorescein angiography, indocyanine green angiography, optical coherence tomography angiography, flood illumination adaptive optics, and microperimetry., Results: A 21-year-old myopic Asian man presented with blurred vision in the right eye. Clinical examination was notable for an isolated hypopigmented, perifoveal lesion in each eye. Multimodal imaging showed inflammatory lesions in the outer retina, retina pigment epithelium, and inner choroid lesions of both eyes. The right eye additionally exhibited active Type-2 macular neovascularization with loss of cone mosaic regularity that was associated with reduced sensitivity on microperimetry. The clinical picture was consistent with multifocal choroiditis/punctate inner choroidopathy. The patient was treated with oral steroids and three injections of intravitreal bevacizumab in the right eye. After therapy, imaging showed reestablishment of the cone mosaic on flood illumination adaptive optics and improvement in sensitivity on microperimetry., Conclusion: Adaptive optics imaging and microperimetry may detect biomarkers that help to characterize the nature and activity of multifocal choroiditis lesions and to help monitor response to therapy. With timely intervention, structural abnormalities in the outer retina and choroid can be treated, and anatomical improvements precede improvements in visual function.

Purpose: To test the hypothesis that hyperreflective foci in central serous chorioretinopathy (CSCR) are autofluorescent and may represent macrophages that have engulfed outer retinal fluorophores from the retinal pigment epithelium (RPE) and photoreceptors., Methods: Enrolled subjects underwent spectral domain and swept-source optical coherence tomography, adaptive optics flood-illumination, and adaptive optics scanning laser ophthalmoscopy (AOSLO), including near-infrared autofluorescence (AO-IRAF). For the AO-IRAF imaging, retinal fluorophores were excited using 795 nm light and collected in an emission band from 814 to 850 nm., Results: In 2 of 3 eyes, a hyperautofluorescent signal was detected with an elliptical shape and punctate, granular aspects surrounded by a hypoautofluorescent halo. The size of these structures in the active case was measured to be 17 ± 4 μm in diameter, with at least 45 individual hyperautofluorescent foci identified from the AO-IRAF montage in the active stage of patient 2. In the asymptomatic case there were fewer structures visible (∼10) and their size was smaller (11 ± 4 μm). These hyper-AF foci were colocalized with hyperreflective foci on OCT and visible in simultaneously acquired confocal AOSLO images in active stage. The hyperautofluorescent foci in the patient with active CSCR disappeared coincident with clinical resolution., Conclusion and Importance: We show here the first AO-IRAF images from patients with CSCR, demonstrating hyper-autofluorescent punctate foci, colocalized with hyper-reflective foci on confocal AOSLO images and in OCT. The autofluorescence of these foci may be driven by the accumulation of photoreceptor and RPE fluorophores within macrophages during the active stage of the disease., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2022 The Authors.)

Purpose: Fundus autofluorescence (AF) using adaptive optics scanning laser ophthalmoscopy (AOSLO) enables morphometric analysis of individual retinal pigmented epithelial (RPE) cells. However, only a few excitation wavelengths in the visible and near-infrared have been evaluated. Visible light excitation (<600 nm) presents additional safety hazards and is uncomfortable for patients. Near-infrared excitation (>700 nm) overcomes those problems but introduces others, including decreased AF signal and cone signatures that obscure RPE structure. Here we investigated the use of an intermediate wavelength, 663 nm, for excitation and compared it to 795 nm., Methods: Subjects were imaged using AOSLO equipped with a detection channel to collect AF emission between 814 and 850 nm. Two light sources (663 and 795 nm) were used to excite the retinal fluorophores. We recorded 90 s videos and registered them with custom software to integrate AF images for analysis., Results: We imaged healthy eyes and an eye with pattern dystrophy. Similar AF microstructures were detected with each excitation source, despite ~4 times lower excitation power with 663 nm. The signal-to-noise values showed no meaningful difference between 663 nm and 795 nm excitation and a similar trend was observed for image contrast between the two excitation wavelengths., Conclusions: Lower light levels can be used with shorter wavelength excitation to achieve comparable images of the microstructure of the RPE as have been obtained using higher light levels at longer wavelengths. Further experiments are needed to fully characterize AF across spectrum and determine the optimal excitation and emission bandwidths that balance efficiency, patient comfort, and efficacy., (© 2021. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Purpose: To characterize an unusual presentation of infectious posterior uveitis using multimodal imaging, and discuss the clinical decision-making involved in diagnosis and treatment., Methods: Wide-field fundus photography, swept-source optical coherence tomography (OCT), swept-source OCT angiography, fluorescein angiography, and indocyanine green angiography., Results: This patient presented with cyclical fevers and blurry vision. Fundus examination revealed bilateral optic disc edema, macular intraretinal white spots and many scattered yellow-white chorioretinal lesions. Multimodal imaging characteristics suggested that many of these lesions represent choroidal granulomas. Extensive systemic workup was only notable for borderline elevated Bartonella henselae IgG titers (1:128), however convalescent IgG titers were elevated at 38 days (1:512) supporting the diagnosis of Bartonella chorioretinitis., Conclusion: Ocular manifestations of Bartonella henselae infection are varied and may include choroidal granulomas. Multimodal imaging characteristics may help identify etiologies of infectious uveitis. Convalescent titers are important when evaluating patients with suspected Bartonellosis, especially patients with atypical presentations.

Objective: To develop and validate a novel convolutional neural network (CNN) termed "Super U-Net" for medical image segmentation., Methods: Super U-Net integrates a dynamic receptive field module and a fusion upsampling module into the classical U-Net architecture. The model was developed and tested to segment retinal vessels, gastrointestinal (GI) polyps, skin lesions on several image types (i.e., fundus images, endoscopic images, dermoscopic images). We also trained and tested the traditional U-Net architecture, seven U-Net variants, and two non-U-Net segmentation architectures. K-fold cross-validation was used to evaluate performance. The performance metrics included Dice similarity coefficient (DSC), accuracy, positive predictive value (PPV), and sensitivity., Results: Super U-Net achieved average DSCs of 0.808±0.0210, 0.752±0.019, 0.804±0.239, and 0.877±0.135 for segmenting retinal vessels, pediatric retinal vessels, GI polyps, and skin lesions, respectively. The Super U-net consistently outperformed U-Net, seven U-Net variants, and two non-U-Net segmentation architectures (p < 0.05)., Conclusion: Dynamic receptive fields and fusion upsampling can significantly improve image segmentation performance., Competing Interests: Declaration of Interests The authors have no conflicts of interest to declare.

The choriocapillaris is the innermost structure of the choroid that directly nourishes the retinal pigment epithelium and photoreceptors. This article provides an overview of its hemovasculogenesis development to achieve its final architecture as a lobular vasculature, and also summarizes the current histological and molecular knowledge about choriocapillaris and its dysfunction. After describing the existing state-of-the-art tools to image the choriocapillaris, we report the findings in the choriocapillaris encountered in the most frequent retinochoroidal diseases including vascular diseases, inflammatory diseases, myopia, pachychoroid disease spectrum disorders, and glaucoma. The final section focuses on the development of imaging technology to optimize visualization of the choriocapillaris as well as current treatments of retinochoroidal disorders that specifically target the choriocapillaris. We conclude the article with pertinent unanswered questions and future directions in research for the choriocapillaris., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Purpose: To describe the application of OCT-A in various posterior uveitis disorders in our experience and to compare it with the available literature., Methods: Eighteen eyes with the diagnoses of multifocal choroiditis (MFC), multifocal placoid pigment epitheliopathy (APMPPE), multiple evanescent white dot syndrome (MEWDS), tuberculous serpiginous-like choroiditis (SLC), serpiginous choroiditis (SC), and birdshot chorioretinopathy (BSCR) were studied., Results: We found flow void of the choriocapillaris in patients with APMPPE, SC, MFC, BSCR, and in SLC. In contrast, perfusion of the choriocapillaris seemed normal in patients with MEWDS., Conclusions: We confirmed that OCT-A contributes new information on the physiopathology of white dot syndromes and inflammatory chorioretinopathies, notably on whether or not the choriocapillaris is involved. Comparing the OCT-A features allowed us to suggest that both APMPPE and SLC might be part of the same spectrum of inflammatory disease with primary involvement at the level of the choriocapillaris and secondary RPE damage.

Torpedo maculopathy (TM) is a rare congenital defect of the retinal pigment epithelium (RPE). The RPE is often evaluated clinically using fundus autofluorescence (AF), a technique that visualizes RPE structure at the tissue level from the intrinsic AF of RPE fluorophores. TM lesions typically emit little or no AF, but this macroscopic assessment is unable to resolve the RPE cells, leaving the organization of the RPE cell mosaic in TM unknown. We used fluorescence adaptive optics scanning laser ophthalmoscopy (AOSLO) to show here for the first time the microscopic cellular-level structural alterations to the RPE cell mosaic in TM that underlie the tissue-level changes seen in conventional clinical imaging. We evaluated two patients with TM using conventional clinical imaging techniques and adaptive optics (AO) infrared autofluorescence (IRAF) in AOSLO. Confocal AOSLO revealed relatively normal cones outside the TM lesion but altered cone appearance within it and along its margins in both patients. We quantified cone topography and RPE cell morphometry from the fovea to the margin of the lesion in case 1 and found cone density to be within the normal range across the locations imaged. However, RPE morphometric analysis revealed disrupted RPE cells outside the margin of the lesion; the mean RPE cell area was greater than two standard deviations above the normative range up to approximately 1.5 mm from the lesion margin. Similar morphometric changes were seen to individual RPE cells in case 2. Multi-modal imaging with AOSLO reveals that RPE cells are abnormal in TM well beyond the margins of the characteristic TM lesion boundary defined with conventional clinical imaging. Since the TM fovea appears to be fully formed, with normal cone packing, it is possible that the congenital RPE defect in TM occurs relatively late in retinal development. This work demonstrates how cellular level imaging of the RPE can provide new insight into RPE pathologies, particularly for rare conditions such as TM., Competing Interests: Some aspects of this work include technologies that ER is an inventor on for patents that are owned by the University of Rochester (US Patent No.: US 10,123,697 and US 10,092,181). The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Vienola, Dansingani, Eller, Martel, Snyder and Rossi.)

Purpose: Drusen are dynamic sub-RPE deposits that are risk factors for late-stage age-related macular degeneration (AMD). Here we show a new imaging method using flood-illumination adaptive optics (FIAO) that reveal drusen with high contrast and resolution., Methods: A fovea-centered 4° × 4° FIAO image and eight surrounding images with gaze displaced by ±2° vertically and horizontally were acquired. Clinical color fundus and spectral-domain optical coherence tomography were acquired for clinical grading and comparison. Custom software registered overlapping FIAO images and fused the data statistically to generate a fovea-centered 4° × 4° gaze-dependent image. Our dataset included 15 controls (aged 31-72) and 182 eyes from 104 AMD patients (aged 56-92), graded as either normal aging (n = 7), and early (n = 12), intermediate (n = 108) and late AMD (n = 42); 27 had subretinal drusenoid deposits (SDDs), and 83 were imaged longitudinally., Results: No gaze varying structures were detected in young eyes. In aging eyes with no evidence of age-related changes, putative drusen <20 µm in diameter were visible. Gaze-dependent images revealed more drusen and many smaller drusen than visible in color fundus images. Longitudinal images showed expansion and fusion of drusen. SDDs were lower contrast, and RPE atrophy did not yield a consistent signal., Conclusions: Gaze-dependent imaging in a commercially available FIAO fundus camera combined with image registration and postprocessing permits visualization of drusen and their progression with high contrast and resolution., Translational Relevance: This new technique offers promise as a robust and sensitive method to detect, map, quantify, and monitor the dynamics of drusen in aging and AMD.

To study the topographic distribution of the short posterior ciliary arteries (SPCA) entry sites into the choroid in normal eyes using structural en-face swept source optical coherence tomography (SS-OCT). Retrospective analysis of SS-OCT scans (wide-field structural SS-OCT 12 × 12 mm) of 13 healthy subjects was performed. Cross-sectional swept-source OCT scans derived from a volume scan were represented as en-face image display following the Choroid-Scleral Interface to obtain en-face OCT. SPCAs in their last scleral location before choroidal entrance were identified manually, counted and localized by two masked observers. Correlations between two masked observers were analyzed using inter- and intra-class correlation. Accuracy for the choroidal inner and outer border segmentation was 95-99%. Eighteen eyes from 13 normal subjects were included for SPCA analysis. The mean number of arteries was 13.8 ± 3.5 per eye. Thirty-six percent were in the center of the posterior pole image; however, 21% were in the temporal part of the posterior pole. Median accuracy of the detection is 0.94. The correlation between the two observers was fair (0.54). Our algorithm allows visualization of the SPCA at the posterior pole of the eye using wide-field en-face SS-OCT. It can also help the clinicians to study the SPCAs in numerous ocular diseases, particularly its relationship with focal choroidal diseases.

Aims: To characterise the epidemiology of eye trauma in the event of falls presenting to the emergency departments (ED) in the USA., Method: Retrospective cohort study. Nationwide Emergency Department Sample was used to analyse fall encounters presenting to the ED with eye trauma from 2006 to 2015. National estimates of the leading diagnoses were determined, and multivariable regression was used to determine the relationship between factors involved in fall encounters presenting with eye trauma., Results: From 2006 to 2015, an estimated 87 991 036 fall encounters presented to the ED, of which 952 781 encounters had eye trauma as either a primary or secondary diagnosis. The overall incidence of fall encounters with eye trauma per 100 000 US population increased from 30.7 encounters in 2006 to 33.8 encounters per 100 000 population in 2014 with a decrease seen in 2015. Eye trauma, including vision-threatening type, was highest in females (n=500 520, 52.5%), elderly (n=400 209, 42%) and children (n=2 06 741, 21.7%). Elderly were more likely to have eye trauma in the setting of falls (adjusted OR (aOR) 2.06, 95% CI 2.02 to 2.11) and be admitted (aOR 1.89, 95% CI 1.86 to 1.91) than adults (reference). The leading types of eye trauma were contusion of orbital tissues (n=174 292, 18.3%), laceration of eyelid and periocular area (n=172 361, 18.1%) and orbital fractures (n=151 013, 15.8%)., Conclusions: Falls are preventable, yet the incidence of falls and resulting eye trauma are increasing despite our best efforts. As ophthalmologists, we should not only develop guidelines to recognise and counsel at-risk groups under our care but also strategies for prevention of eye trauma secondary to falls., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To describe the epidemiology of Emergency Department (ED) visits related to opioid abuse with primary ophthalmic diagnoses in the United States (US)., Methods: This retrospective cross-sectional study used National ED Sample (NEDS) (2006-2015), a representative sample of all US EDs, to analyze and compare the epidemiology of primary ophthalmic diagnoses in opioid abusers and a control group of non-opioid users. National incidence and descriptive statistics were calculated for demographics and prevalent diagnoses. Multivariable logistic regression was used to compare outcomes between primary ophthalmic diagnoses in opioid and non-opioid abusers., Results: An estimated 10,617 visits had a primary ophthalmic diagnosis and an accompanying opioid abuse diagnosis, and the incidence increased from 0.2 in 2006 to 0.6 per 100,000 US population in 2015. Opioid abuse group had more adults (6,747:63.5%) and middle-aged (3,361:31.7%) patients, while in controls adults (7,905,003:40.4%) and children (4,068,534:20.8%) were affected more. Leading etiologies were similar: traumatic and infectious etiologies were most common; however, opioid abuse patients had more severe ophthalmic diagnoses such as orbital fractures (8.4%), orbital cellulitis (7.4%), globe injury (3.4%) and endophthalmitis (3.2%) compared to controls. Patients in the opioid abuse group were also more likely to be admitted (adjusted Odds Ratio [aOR], 28.38 [95% CI, 24.50-32.87])., Conclusions: In the era of opioid crisis, an increase in ED visits with ophthalmic complaints is seen, with increasing direct and indirect costs on the healthcare system. More research is needed to establish causality and devise strategies to lower this burden.

Retinal pigmented epithelial (RPE) cells are essential for maintaining normal visual function, especially in their role in the visual cycle, and are thought to be one of the first cell classes affected by age-related macular degeneration (AMD). Clinical imaging systems routinely evaluate the structure of the RPE at the tissue level, but cellular level information may provide valuable RPE biomarkers of health, aging and disease. In this exploratory study, participants were imaged with 795 nm excitation in adaptive optics scanning laser ophthalmoscopy (AOSLO) to observe the microstructure of the near-infrared autofluorescence (AO-IRAF) from the RPE layer in healthy retinas and patients with AMD. The expected hexagonal mosaic of RPE cells was only sometimes seen in normal eyes, while AMD patients exhibited highly variable patterns of altered AO-IRAF. In some participants, AO-IRAF structure corresponding to cones was observed, as we have demonstrated previously. In some AMD patients, marked alterations in the pattern of AO-IRAF could be seen even in areas where the RPE appeared relatively normal in clinical imaging modalities, such as spectral domain optical coherence tomography (SD-OCT). AO-IRAF imaging using AOSLO offers promise for better detection and understanding of early RPE changes in the course of AMD, potentially before clinical signs appear.

Purpose: This study analyzes a subset of patients with peripapillary polypoidal choroidal vasculopathy (PCV) to determine whether quantifiable pachychoroid features colocalize with disease foci., Methods: Patients with PCV diagnosed by indocyanine green angiography were identified for the analysis of medical records and multimodal imaging and classified as having peripapillary or macular PCV. The ratio of Haller layer thickness to total choroidal thickness was calculated at the fovea and at the site of dilated Haller vessels that showed spatial correlation with the origin of neovascularization. Choroidal thickness was measured horizontally across the fovea and circumferentially around the temporal side of the disk to study its relationship to neovascularization., Results: Three hundred and fourteen eyes of 299 patients with PCV were identified, of which 17 eyes (5%) had peripapillary disease. Although eyes with peripapillary PCV exhibited thinner subfoveal choroids than those with macular PCV, at the extrafoveal disease foci, choroidal thickness, Haller's layer thickness, and its ratio to total choroidal thickness were relatively high., Conclusion: Quantitative indices of choroidal structure previously identified in macular PCV performed consistently when applied to a peripapillary PCV cohort, thus supporting the hypothesis that inner choroidal thinning and Haller vessel enlargement are mechanistically relevant to these related entities.

The automatic segmentation of fluid spaces in optical coherence tomography (OCT) imaging facilitates clinically relevant quantification and monitoring of eye disorders over time. Eyes with florid disease are particularly challenging to segment, as the anatomy is often highly distorted from normal. In this context, we propose an end-to-end machine learning method consisting of near perfect detection of retinal fluid using random forest classifier and an efficient DeepLab algorithm for quantification and labeling of the target fluid compartments. In particular, we achieve an average Dice score of 86.23% with reference to manual delineations made by a trained expert.

The choroid is the vascular coat of the eye, and its role has been studied in multiple chorioretinal disorders. Recent advancements in choroidal imaging techniques, including enhanced depth imaging optical coherence tomography, swept source optical coherence tomography, en face optical coherence tomography, and optical coherence tomography angiography have facilitated an in-depth analysis of the choroid. The gradual shift from manual to automated segmentation and binarization methods have led to precise and reproducible measurements of choroidal parameters. These qualitative and quantitative parameters, called choroidal imaging biomarkers, have evolved over the past decade from a simple linear subfoveal choroidal thickness to more complex 3D choroidal reconstruction, thus widening the spectrum encompassing multiple parameters. These biomarkers have provided a better understanding of the pathogenesis, are helpful in diagnostic dilemmas, and, in the future may also help to devise treatment options. The lack of normative data, absence of standardized parameters, and limitations of the imaging techniques, however, have led to ambiguity and difficulty in the interpretation of these variables. We attempt to address these lacunae in the literature and provide a basic understanding of the choroid in both health and disease using these choroidal biomarkers., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Posterior column ataxia retinitis pigmentosa (PCARP) with feline leukemia virus subgroup C cellular receptor 1 (FLVCR1) gene mutation is a rare disorder with significant ophthalmic features., Materials and Methods: We conducted a retrospective case series study of patients diagnosed with PCARP and genetic testing positive for FLVCR1 mutation between 1 January 2015 and 1 October 2017 at the Children's Hospital of Pittsburgh. Clinical charts, visual fields, fundus autofluorescence, and spectral-domain optical coherence tomography (SD-OCT) were reviewed., Results: Seven patients from three families were identified to have PCARP and FLVCR1 mutation. The median age at presentation was 13 years (range, 7-28 years). Common clinical exam findings were astigmatism, cataracts, and vitreous syneresis. Funduscopy on all patients revealed bull's eye maculopathy, retinal vessels attenuation, and bone spicule changes in the peripheral retina. Fundus autofluorescence showed bilateral hyperautofluorescent rings. SD-OCT demonstrated morphological changes, which differed based on age. The youngest sibling family exhibited peripheral loss, but subfoveal preservation of the outer retinal layers. These layers were lost in the oldest sibling family. Visual fields loss paralleled SD-OCT findings., Conclusion: There is limited published ophthalmic data on FLVCR1-related PCARP. We describe clinical and retinal imaging features in the one of the largest cohorts of affected patients in the literature. Given the availability of genetic testing for this phenotype, testing for FLVCR1 mutations should be considered in pediatric and adult patients with sensory ataxia and retinitis pigmentosa.

Importance: Analysis of collateral vessel formation following retinal vein occlusion may advance our understanding of the venous outflow anatomy in the macula., Objective: To determine the location of collateral vessels with optical coherence tomography (OCT) angiography imaging., Design, Setting, and Participants: Observational retrospective cohort study. Collateral vessel formation was studied with OCT angiography (OCTA) in patients with retinal vein occlusion (RVO). The study took place at 2 retinal practices (Vitreous Retina Macula Consultants of New York and Stein Eye Institute, University of California, Los Angeles), with patient records retrieved from March 2015 to August 2017. Data analysis was completed in November 2017., Exposures: Collaterals identified with fundus photography and/or fluorescein angiography were analyzed with OCTA to determine their course through the superficial vascular plexus (SVP) and the deep vascular complex (DVC)., Main Outcomes and Measures: Collateral vessel pathways through the SVP and DVC were analyzed with cross-sectional and en face OCT and OCTA segmentation and color-coded volume renderings prepared from raw OCTA voxel data., Results: From 23 eyes (22 branch and 1 hemispheric retinal vein occlusion ) of 23 patients (mean [SD] age, 73 [11] years), 101 collateral vessels were identified and analyzed (mean [SD], 4.4 [2.0]; range, 2-9 collateral per eye). On OCTA, the collaterals appeared as curvilinear dilated flow signals that connected veins across the horizontal raphe or veins on opposite sides of an occluded venous segment within the same retinal hemisphere. Of the 101 collaterals analyzed, all showed greater flow signal in the DVC, and all had some portion of their course identified within the DVC. No collaterals were found exclusively in the SVP. Volume renderings for 3 cases confirmed qualitatively that retinal collateral vessels course through the retina predominantly at the level of the DVC., Conclusions and Relevance: Based on a limited number of cases, all collateral vessels associated with retinal vein occlusion were found to course through the DVC. The absence of collaterals isolated to the SVP supports a serial arrangement of the SVP and DVC, with venous drainage predominantly coursing through the DVC.

Purpose: To study zones of reduced inner choroidal flow signal, foci of reduced inner choroidal thickness, and pathologically dilated Haller layer vessels (pachyvessels) in eyes with pachychoroid disease using optical coherence tomography (OCT) and OCT angiography., Methods: Patients with treatment-naive pachychoroid disease were recruited. All patients prospectively underwent swept-source OCT and OCT angiography. Zones of reduced choriocapillaris flow were labeled and enumerated. Areas where reduced flow signal was attributable to masking/artifacts were excluded. Regions of inner choroidal thinning were identified on structural OCT and labeled. Overlap between reduced choriocapillaris flow and structural inner choroidal attenuation was quantified using Jaccard indices. The relationship of reduced flow to pachyvessels was recorded., Results: Twenty-four eyes of 19 patients were identified. All eyes exhibited at least one zone of reduced flow. A total of 146 flow signal attenuation zones were identified. Sixty-two (42%) of 146 zones showed overlap or proximity with structural inner choroidal thinning. The mean Jaccard index per eye was 0.10 (SD = 0.08). Pachyvessels were spatially related to 100 (68%) of 146 zones of flow attenuation., Conclusion: Zones of reduced choriocapillaris flow are prevalent in eyes with pachychoroid disease. Approximately 60% of these zones anatomically correlate with pachyvessels. Inner choroidal ischemia seems related to the pathogenesis of pachychoroid diseases.

Purpose: To report the posterior segment and retinal vascular manifestations of calcific uremic arteriolopathy (calciphylaxis). Clinical findings are correlated with multimodal imaging results., Methods: Observational case report., Results: A 65-year-old white woman on hemodialysis was referred for assessment of poor vision bilaterally. Clinical examination demonstrated a crystalline retinopathy with stigma of previous retinal arterial occlusion. Fluorescein angiography revealed delayed retinal arterial filling bilaterally, sheathing of vessels, and peripheral nonperfusion. The crystals were hyperautofluorescent. Spectral domain and enhanced depth imaging optical coherence tomography localized the crystals within the retina with a predilection for the retinal arterial vasculature. The choriocapillaris was not involved. Two years prior, the patient developed necrotic skin lesions which were biopsied and confirmed the diagnosis of calciphylaxis., Conclusion: Calcific uremic arteriolopathy is an extremely rare cause of thrombogenic microangiopathy in end-stage renal disease patients. Retinal arterial occlusion appears to be a rare but significant cause of visual loss in this disease and is likely to be consequent to crystalline deposition in the retinal vasculature.

Optical coherence tomography (OCT) images semi-transparent tissues noninvasively. Relying on backscatter and interferometry to calculate spatial relationships, OCT shares similarities with other pulse-echo modalities. There is considerable interest in using machine learning techniques for automated image classification, particularly among ophthalmologists who rely heavily on diagnostic OCT. Artificial neural networks (ANN) consist of interconnected nodes and can be employed as classifiers after training on large datasets. Conventionally, OCT scans are rendered as 2D or 3D human-readable images of which the smallest depth-resolved unit is the amplitude-scan reflectivity-function profile which is difficult for humans to interpret. We set out to determine whether amplitude-scan reflectivity-function profiles representing disease signatures could be distinguished and classified by a feed-forward ANN. Our classifier achieved high accuracies after training on only 24 eyes, with evidence of good generalization on unseen data. The repertoire of our classifier can now be expanded to include rare and unseen diseases and can be extended to other disciplines and industries.

Significance: This study compares foveal avascular zone (FAZ) geometry in healthy eyes as imaged by two commercially available optical coherence tomography angiography (OCTA) devices. Foveal avascular zone measurements are repeatable and reproducible with each OCTA device, but interdevice agreement was poor. We provide conversion factors between devices., Purpose: The purpose of this study was to perform comparative evaluation of FAZ geometry in healthy eyes as imaged by two commercially available OCTA devices., Methods: Ninety-six eyes of 48 healthy subjects were imaged prospectively on each of two OCTA devices (DRI-OCT [Topcon Corporation, Tokyo, Japan]; Cirrus 5000 [Carl Zeiss Meditec Inc., Dublin, CA]). The FAZ was evaluated in the superficial capillary plexus layer of 6 × 6-mm foveal scans by two masked observers. Intraobserver and interobserver agreement was determined using intraclass correlation by using linear mixed models and Bland-Altman plots. K-means clustering was used to provide conversion values between two devices. Foveal avascular zone acircularity was calculated using scans from each device and compared., Results: The intraobserver repeatability for DRI-OCT was 0.95 (95% confidence interval [CI], 0.90 to 0.98) for observer A and 0.92 (95% CI, 0.83 to 0.96) for observer B. Intraobserver repeatability for Cirrus 5000 was 0.988 (95% CI, 0.972 to 0.995) for observer A and 0.993 (95% CI, 0.983 to 0.997) for observer B. The interobserver variability between observers A and B for DRI-OCT was 0.87 (0.73 to 0.94) and for Cirrus 5000 was 0.984 (95% CI, 0.964 to 0.993). Poor interdevice agreement (0.205 [95% CI, -0.202 to 0.554]) was noted, and conversion formulas were devised to convert FAZ area measurements from one device to another. No significant correlation was found when comparing FAZ acircularity indices between devices (P = .39)., Conclusions: Repeatable and reproducible FAZ area measurements were obtained with each respective OCTA device, but interdevice agreement was poor, yet quantifiable and systematic with calculable conversion factors between devices.

Purpose: To compare the prevailing patterns of Haller vessel arrangements at the posterior pole between healthy eyes and those with central serous chorioretinopathy (CSC) using en face optical coherence tomography., Methods: Eyes of normal subjects and patients with acute or chronic CSC underwent optical coherence tomography imaging (RTVue 100; Optovue Inc, Fremont, CA). En face sections at the level of the Haller layer were classified by two masked graders into five mutually exclusive morphologic categories (temporal herringbone, branched from below, laterally diagonal, double arcuate, and reticular). The relative prevalence of each Haller vessel arrangement pattern was determined for each phenotype., Results: Numbers of eyes examined were as follows: 154 eyes of 77 normal subjects; 41 eyes of 31 patients with acute CSC; and 39 eyes of 33 patients with chronic CSC. The mean age of participants was 44.4 ± 14.6 years for healthy subjects (M:F = 37:40), 48.5 ± 8.2 years (M:F = 24:7) for acute CSC, and 65.3 ± 13.1 years (M:F = 28:5) for chronic CSC. The relative prevalence of each Haller vessel arrangement pattern differed by phenotype. The temporal herringbone pattern was most prevalent in healthy eyes (49.2%), whereas a reticular pattern was most prevalent in eyes with acute and/or chronic CSC (combined, 48.8%)., Conclusion: A significant difference was observed in the prevalence of respective Haller vessel arrangement patterns between eyes of normal subjects and those of patients with either acute or chronic CSC. Although further study is needed to determine the mechanistic factors underlying these differences, and the hemodynamic implications, our data suggest that en face optical coherence tomography may find a formal role in choroidal disease classification.

Conventionally rendered optical coherence tomography (OCT) images of the posterior segment contain shadows which influence the visualization of deep structures such as the choroid. The purpose of this study was to determine whether OCT shadow compensation (SC) alters the appearance of the choroid and the apparent choroidal vascularity index (CVI), an OCT-derived estimated ratio of luminal to total choroidal volume. All scans were shadow compensated using a previously published algorithm, binarized using a novel validated algorithm and extracted binarized choroid to estimate CVI. On 27 raw swept-source OCT volume-scans of healthy subjects, the effect of SC on CVI was established both qualitatively and quantitatively. In shadow compensated scans, the choroid was visualized with greater brightness than the neurosensory retina and the masking of deep tissues by retinal blood vessels was greatly reduced. Among study subjects, significant mean difference in CVI of -0.13 was observed between raw and shadow compensated scans. Conventionally acquired OCT underestimates both choroidal reflectivity and calculated CVI. Quantitative analysis based on subjective grading demonstrated that SC increased the contrast between stromal and luminal regions and are in agreement with true tissue regions. This study is warranted to determine the effects of SC on CVI in diseased eyes.

The term aneurysmal type 1 neovascularization is derived from terminology, which is established in the literature but has fallen out of use. We believe that aneurysmal type 1 neovascularization accurately describes the lesions which define the entity known as polypoidal choroidal vasculopathy (PCV). Over the last three decades, the clinical spectrum of PCV has expanded to recognize the occurrence of the aneurysmal (polypoidal) lesions in different contexts, resulting in a complex and unwieldy taxonomy based sometimes on circumstantial findings rather than mechanistic considerations. Advances in multimodal imaging provides increasingly convincing evidence that the lesions which define various forms of PCV are indeed vascular and arise from type 1 neovascular networks. The understanding of PCV as type 1 neovascularization with aneurysms renews focus on the question as to why some patients with type 1 neovascularization develop aneurysms while others do not. Conceptual themes and potential for further study are discussed., (© 2017 The Authors Clinical & Experimental Ophthalmology published by John Wiley & Sons Australia, Ltd on behalf of Royal Australian and New Zealand College of Ophthalmologists.)

Purpose: To describe clinical findings in patients with acute exudative polymorphous vitelliform maculopathy (AEPVM)., Design: Retrospective, observational, multicenter case series review., Participants: Consecutive patients diagnosed with idiopathic AEPVM., Methods: Review of clinical charts, multimodal imaging, electrophysiologic findings, and genetic findings in previously unpublished patients and review of the literature., Main Outcome Measures: Clinical features of idiopathic AEPVM and differential diagnosis., Results: Eighteen patients (age range, 21-74 years) with typical features of AEPVM, including initial localized, serous detachments followed by the development of characteristic yellow-white deposits in the vitelliform space. Over time, this hyperautofluorescent material gravitated within the larger lesions, resulting in typical curvilinear deposits characteristic of later stages. Symptoms and clinical findings lasted from weeks to several years. Some patients showed previously undescribed features such as fluorescein-negative intraretinal cystic changes, choroidal neovascularization, serous retinal elevations mimicking retinal folds, increased choroidal thickness, lack of rapid visual recovery, and recurrence years after complete resolution of initial manifestations., Conclusions: Acute exudative polymorphous vitelliform maculopathy can present with a more variable natural course than previously described. Paraneoplastic retinopathy and autosomal recessive bestrophinopathy closely resemble AEPVM, necessitating medical and hereditary evaluation to exclude these clinical possibilities. This series of patients with AEPVM expands the clinical spectrum of the disorder, including demographics, clinical manifestations, imaging features, natural course, and visual prognosis., (Copyright © 2017 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To study the relationship of choroidal abnormalities with serous retinal detachment (SRD) in eyes with staphyloma, dome-shaped macula, or tilted disk syndrome., Methods: Group 1, 28 eyes of 20 patients with staphyloma/dome-shaped macula/tilted disk syndrome associated with SRD was compared with Group 2, 30 eyes of 20 patients, with staphyloma/dome-shaped macula/tilted disk syndrome but without SRD. Radial and en-face optical coherence tomography and choroidal analysis were performed., Results: Group 1 had a thicker mean subfoveal choroidal thickness (161 μm vs. 92 μm, P < 0.05) and a greater variation in choroidal thickness (112 μm vs. 76 μm, P > 0.05) compared with eyes of Group 2. Focal abrupt changes in choroidal thickness were more commonly seen in Group 1 versus eyes in Group 2 (90% vs. 30%, P < 0.05) and this area of abrupt change was located within or at the edge of the SRD in 64% of eyes. Large choroidal vessels (pachyvessels) (82% located within the area of SRD) were always associated with the presence of SRD., Conclusion: An abrupt transition in choroidal thickness may be involved in the pathogenesis of SRD. In some cases, a radial scan pattern may better demonstrate mild SRD, choroidal contours and the focal choroidal variations than horizontal or vertical raster scan patterns.

Purpose: To describe anatomical relationships of retinal neovascular complexes (NVCs) and the posterior vitreous in proliferative diabetic retinopathy using spectral domain optical coherence tomography., Methods: Cross-sectional study. Neovascular complexes were imaged using spectral domain optical coherence tomography in 51 eyes of 37 patients. The relationship of NVCs to the posterior vitreous cortex and posterior vitreous spaces, such as the premacular bursa, prevascular vitreous fissures, and perimacular cisterns, was analyzed., Results: In the 77 NVCs evaluated, 61 (79%) had grown along the outer surface of the posterior hyaloid face, and vitreoschisis was present in 37 (48%). The "wolf's jaw" configuration was present in 9% and resulted from NVC arising from the arcades and proliferating along the posterior hyaloid face. By contrast, NVCs that invaded the bursa originated from smaller venous tributaries more distant from the arcades. The premacular bursa and prevascular vitreous fissure/perimacular cistern were invaded infrequently, respectively, in 15% and 38% (P = 0.137)., Conclusion: Tomographic analysis of diabetic NVCs showed that most NVCs arise and grow along the posterior hyaloid face and that vitreoschisis is more prevalent than what has been found in ultrasound studies. The wolf's jaw configuration does not seem to result from the invasion of the bursa, as previously suggested.

Purpose: Histologic details of progression routes to geographic atrophy (GA) in AMD are becoming available through optical coherence tomography (OCT). We studied the origins and evolution of an OCT signature called plateau in eyes with GA and suggested a histologic correlate., Methods: Serial eye-tracked OCT scans and multimodal imaging were acquired from eight eyes of seven patients with GA and plateau signatures over a mean follow-up of 7.7 years (range, 3.7-11.6). The histology of unrelated donor eyes with AMD was reviewed., Results: Drusenoid pigment epithelial detachment (PED) on OCT imaging progressed into wide-based mound-like signatures with flattened apices characterized by a hyporeflective yet heterogeneous interior and an overlying hyperreflective exterior, similar to outer retinal corrugations previously ascribed to persistent basal laminar deposit (BLamD) but larger. These new signatures are described as "plateaus." An initial increase of the PED volume and hyporeflectivity of its contents was followed by a decrease in PED volume and thinning of an overlying hyperreflective band attributable to the loss of the overlying RPE leaving persistent BLamD. Both imaging and histology revealed persistent BLamD with defects through which gliotic Müller cell processes pass., Conclusions: Plateaus can be traced back to drusenoid PEDs on OCT imaging. We hypothesize that during progressive RPE atrophy, Müller cell extension through focal defects in the residual persistent BLamD may contribute to the heterogeneous internal reflectivity of these entities. The role of Müller cell activation and extension in the pathogenesis of AMD should be explored in future studies.

Purpose: To study the cross-sectional and en face optical coherence tomography angiography (OCTA) findings in Type 3 neovascularization (NV)., Methods: Optical coherence tomography angiography imaging of 27 eyes of 23 patients with Type 3 NV was analyzed with 9 eyes having consecutive follow-up OCTA studies., Results: Type 3 NV appeared as a linear high-flow structure on cross-sectional OCTA corresponding to a high-flow tuft of vessels seen on en face OCTA. Cross-sectional OCTA seemed to enable the distinction between vascular and nonvascular intraretinal hyperreflective foci. Two patterns of flow were observed; Pattern 1 (11%): a flow signal confined to the neurosensory retina and Pattern 2 (74%): a flow signal extending through the retinal pigment epithelium. No definitive retinal-choroidal anastomosis was observed; however, projection artifacts confounded the interpretation of deeper structures. An increase in the intensity of the high-flow tuft was seen during the progression or recurrence of Type 3 NV. Intravitreal anti-vascular endothelial growth factor therapy caused a reduction in the intensity of the high-flow tuft which was not sustained., Conclusion: Compared with conventional imaging, OCTA may improve detection and delineation of vascular changes occurring in Type 3 NV. Cross-sectional and en face OCTA may prove useful in studying the pathogenesis and guiding the management of these lesions.

Purpose: To subsegment the choroid in patients with polypoidal choroidal vasculopathy and to determine whether the ratio of choriocapillaris/Sattler layer thickness to total choroidal thickness is decreased at sites of polypoidal pathology., Methods: Retrospective, observational, cross-sectional study. A total of 320 eyes of 305 patients with polypoidal choroidal vasculopathy were studied with optical coherence tomography and dye angiography. The ratio of choriocapillaris/Sattler layer thickness to total choroidal thickness was calculated at polypoidal lesion sites in eyes with subfoveal choroidal thickness (SFCT) ≤200 μm., Results: Mean SFCT was 267.7 ± 118.5 μm for the entire cohort. Mean SFCT was 151.2 ± 35.0 μm in eyes with SFCT ≤200 μm (n = 124, 39%). In this subgroup, dilated Haller vessels (pachyvessels) were identified under the site of neovascular ingrowth in 117 eyes (94%). Choroidal thickness in the pachyvessel zone was greater (213.3 ± 52.2 μm) than SFCT (P < 0.001) with a significantly lower choriocapillaris/Sattler layer to total thickness ratio (P < 0.001). Qualitative alterations of the retinal pigment epithelium were observed in 60 eyes (51%)., Conclusion: Eyes with normal or subnormal SFCT exhibited extrafoveal choroidal thickening at sites of polypoidal disease. The choriocapillaris and Sattler layers were attenuated at these locations, but Haller vessels were markedly dilated. These changes were topographically associated with sites of neovascular ingrowth and support the classification of polypoidal choroidal vasculopathy as a pachychoroid disorder.

Purpose: To describe and compare the clinical and imaging characteristics of pigment epithelial detachments (PEDs) in age-related macular degeneration (AMD), polypoidal choroidal vasculopathy (PCV), and central serous chorioretinopathy (CSC) as seen in a clinical setting of a tertiary retinal practice., Design: A perspective supported by clinical and imaging characteristics of a consecutive cohort of patients with strictly defined PEDs., Results: One hundred seventy-four eyes of 113 patients with PEDs were studied with comprehensive clinical retinal examination and multimodal imaging; PEDs were differentiated into nonvascularized and vascularized forms with 3 main underlying etiologies: AMD (76%), PCV (9%), and CSC (3%). AMD was the most common diagnosis, with both nonvascularized PEDs (drusenoid and serous) and vascularized PEDs (type 1 and type 3 neovascularization) associated with drusen and a thin choroid. PCV patients had large, vascularized, peaked PEDs associated with polyps and a variable choroidal thickness, while CSC patients had a thick choroid and predominantly nonvascularized, serous PEDs with an overlying neurosensory detachment. The combined clinical and imaging characteristics form a profile for each PED subtype related to their underlying disease. However, atypical features noted in 11% of patients may complicate the underlying diagnosis., Conclusion: Typical phenotypic manifestations of PEDs and other features seen with multimodal imaging were associated with specific underlying etiologies. As suggested by our study, identification of these features help clinicians to determine the precise underlying etiology and manage both vascularized PEDs, where evidence-based treatment exists, and nonvascularized PEDs, where current treatment is not supported by convincing evidence., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To describe a patient who showed progression of age-related macular degeneration along the course of an underlying large choroidal vessel., Methods: Retrospective case report., Results: An 84-year-old woman was diagnosed with age-related macular degeneration and followed for more than 6 years. Retinal pigment epithelial changes were more advanced in her right eye which had a thinner choroid than the fellow left eye. Multimodal imaging with swept-source optical coherence tomography showed that the progression of outer retinal and retinal pigment epithelial changes appeared to follow the course of a large choroidal vessel., Conclusion: The authors hypothesize that large choroidal vessels can compress the overlying choriocapillaris, possibly leading to outer retinal ischemia, which subsequently could focally accelerate the course of age-related macular degeneration.

Purpose: The range of subretinal hyperreflective material (SHRM) seen in macular disease includes type 2 macular neovascularization, fibrosis, exudation, vitelliform material, and hemorrhage. The prognostic significance of SHRM has been evaluated retrospectively in clinical trials, but discriminating SHRM subtypes traditionally requires multiple imaging modalities. The purpose of this study is to describe optical coherence tomography angiography (OCTA) flow characteristics and artifacts that might help to distinguish SHRM subtypes., Design: Validity analysis., Methods: Patients with age-related macular degeneration (AMD), myopia, pachychoroid disease, and macular dystrophy, manifesting SHRM on optical coherence tomography (OCT), were recruited. Clinical chart review and multimodal imaging established the SHRM subtype. All patients underwent OCTA. OCT and OCTA images were examined together for (1) intrinsic flow, (2) retinal projection onto the anterior SHRM surface (strong, weak, absent), (3) retinal projection through SHRM onto retinal pigment epithelium (RPE), and (4) masking of choriocapillaris flow., Results: Thirty-three eyes of 25 patients were included (type 2 neovascularization ×3; fibrosis ×4; exudation ×10; hemorrhage ×5; vitelliform ×17). Mean age per eye was 76 years (standard deviation: 12). Intrinsic flow was strongest in type 2 neovascularization. Subretinal fibrosis showed limited flow in residual large-caliber vessels and branches. Flow was not detected within foci of exudation, hemorrhage, or vitelliform lesions. Retina-SHRM surface projection was strongest onto smooth-surfaced SHRM and weaker onto exudation. Retinal projection was weakest on the surface of vitelliform lesions. Retina-RPE projection was masked by dense hemorrhage and vitelliform material. In compound SHRM, OCTA distinguished between vascular and avascular components., Conclusion: Optical coherence tomography angiography can distinguish vascular from avascular SHRM components. OCTA artifacts may distinguish certain avascular SHRM components., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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.

Purpose: To determine the sensitivity of the combination of optical coherence tomography angiography (OCTA) and structural optical coherence tomography (OCT) for detecting type 1 neovascularization (NV) and to determine significant factors that preclude visualization of type 1 NV using OCTA., Methods: Multicenter, retrospective cohort study of 115 eyes from 100 patients with type 1 NV. A retrospective review of fluorescein (FA), OCT, and OCTA imaging was performed on a consecutive series of eyes with type 1 NV from five institutions. Unmasked graders utilized FA and structural OCT data to determine the diagnosis of type 1 NV. Masked graders evaluated FA data alone, en face OCTA data alone and combined en face OCTA and structural OCT data to determine the presence of type 1 NV. Sensitivity analyses were performed using combined FA and OCT data as the reference standard., Results: A total of 105 eyes were diagnosed with type 1 NV using the reference. Of these, 90 (85.7%) could be detected using en face OCTA and structural OCT. The sensitivities of FA data alone and en face OCTA data alone for visualizing type 1 NV were the same (66.7%). Significant factors that precluded visualization of NV using en face OCTA included the height of pigment epithelial detachment, low signal strength, and treatment-naïve disease (P < 0.05, respectively)., Conclusions: En face OCTA and structural OCT showed better detection of type 1 NV than either FA alone or en face OCTA alone. Combining en face OCTA and structural OCT information may therefore be a useful way to noninvasively diagnose and monitor the treatment of type 1 NV.

Suprachoroidal hemorrhage is typically observed in patients with hypotony in the context of intraocular surgery and trauma. We report a rare presentation of suprachoroidal hemorrhage associated with emesis (Valsalva maneuver) in a myopic patient with few other identifiable risk factors. This diagnosis was confirmed with wide-field fundus photography and B-scan ultrasound and was localized to the suprachoroidal space using both enhanced depth and swept-source optical coherence tomography imaging. This case is unique in its presentation and demonstrates the utility of newer imaging techniques to localize hemorrhages using a multimodal approach. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:674-676.]., (Copyright 2016, SLACK Incorporated.)