Effect of fenestrated sterile drape and face mask on bacterial dispersion toward the periocular area during intravitreal injection.

This experimental crossover study was performed to investigate whether fenestrated surgical drapes (covering the nose and mouth but with an opening over the periorbital area) with or without patients' surgical face masks increase periorbital bacterial dispersion during simulated intravitreal injection conditions. Each of the 16 healthy volunteers performed 14 scenarios involving different mask and drape conditions in both silent and speaking situations. In each scenario, the subject lay down flat on the back with a blood agar plate being held at the inferior orbital rim perpendicular to the face to capture airflow from breathing/speaking. Another blood agar plate placed 50 cm away from the subject served as an experimental control. A total of 224 experiments were performed. Speaking situations significantly showed more colony forming units (CFUs) compared with their controls (P = 0.014). There were no significant differences in CFUs between wearing vs not wearing the masks (P = 0.887 for speaking and P = 0.219 for silent) and using vs not using the drapes (P = 0.941 for speaking and P = 0.687 for silent). Reusable and disposable drapes were also not significantly different (P = 1.00 for speaking and P = 0.625 for silent). Streptococcus spp., the oropharyngeal microbiota, were only cultivated from speaking scenarios. While refraining from speaking (for both practitioners and patients) is the mainstay of reducing bacterial dispersion and risks of post-injection endophthalmitis, the use of fenestrated surgical drapes or patients' face masks did not significantly affect the amount of bacterial dispersion toward the periorbital area.

Klebsiella pneumoniae Endophthalmitis with Subretinal Abscess: A Case Series and Review of the Literature.

This study assessed prognostic factors and the role of vitrectomy in patients with subretinal abscesses secondary to K. pneumoniae endophthalmitis. We reviewed published studies, including three cases from our cohort. Among 50 eyes, 26 had poor visual outcomes (final visual acuity <20/800, eyeball removal, or phthisis bulbi). Poor outcomes correlated with delayed ocular symptom-to-diagnosis time, initial visual acuity <20/800, severe vitritis, and macular involvement of abscesses (p < 0.001, p = 0.008, p < 0.001, and p = 0.033, respectively). Vitrectomy had a trend towards reducing eyeball removal and phthisis bulbi rates compared with non-vitrectomy (10.8% vs 30.8%, p = 0.181). However, the final visual acuity was not different and the rate of retinal detachment tended to be higher in vitrectomized eyes (45.9% vs 15.4%, p = 0.095). The study suggested that vitrectomy and drainage of K. pneumoniae subretinal abscesses could be avoided in patients with a mild degree of vitritis.

Myopic macular schisis: Insights into distinct morphological subtypes and novel biomechanical hypothesis.

PURPOSE: To analyze the features of myopic macular schisis (MMS) in different retinal layers and to explore the role of Müller cells in the pathophysiology of such condition. METHODS: Spectral-domain optical coherence tomography (SD-OCT) images of myopic eyes with staphyloma and macular schisis were reviewed. The morphological features of MMS were analyzed and correlated with their geographical location in the parafoveal and perifoveal region. A biomechanical model was adopted to explain MMS morphological differences. The effect of the different schisis subtypes with best corrected visual acuity (BCVA) was also explored. RESULTS: A total of 36 eyes from 26 patients were included in this study. MMS was classified into inner, middle and outer retinal subtypes. The prevalence of middle retinal schisis was significantly lower in the parafovea, within a central 3 mm-diameter circle (p < 0.001) centered at the fovea . The prevalence of inner retinal schisis was significantly higher outside the central 3-mm diameter circle, in the perifoveal region (p < 0.001). No significant differences were noted in the prevalence of outer retinal schisis for these two locations (p = 0.475). The presence of middle retinal schisis within the central 3-mm diameter circle showed a weak association with lower BCVA (p = 0.058). The presence of outer retinal schisis within the central 3-mm diameter circle was significantly related with lower BCVA (p = 0.024). CONCLUSION: Three major forms of MMS are distinguished: inner, middle and outer retinal schisis. This classification may have clinical importance as only the outer grade of schisis was associated with vision loss.

Optical Coherence Tomography Angiography Biomarkers in Thai Patients With Diabetic Nephropathy: A Diabetic Eye and Kidney Diseases (DEK-D) Study.

Purpose: To identify optical coherence tomography angiography (OCTA) biomarkers to predict the diabetic nephropathy (DN) and their associations with 24-hour urine albumin levels in diabetic patients. Methods: This cross-sectional, observational study examined 186 eyes from 93 individuals subdivided into three groups according to 24-hour urine albumin levels: no DN, early DN, and late DN. Vessel density (VD), fractal dimension, foveal avascular zone area, intercapillary area, central retinal thickness, and subfoveal choroidal thickness were measured from OCTA images to determine their association with the DN stages. Results: VD values of the superficial capillary plexus, deep capillary plexus, and whole retina were significantly lower in the early DN group compared to the no DN group (adjusted P = 0.042, 0.016, and 0.008, respectively). VD values for the deep capillary plexus and whole retina were significantly decreased in the late DN group compared to the no DN group (adjusted P = 0.025 and 0.021, respectively). Mean fractal dimension, intercapillary area, foveal avascular zone area, central retinal thickness, and subfoveal choroidal thickness were not statistically different among the three groups. Conclusions: VD may be a useful parameter for the early non-invasive screening of DN. Further studies in larger populations are needed to establish a cutoff value for detection. Translational Relevance: This study investigated the association of each retinal vasculature measurement by OCTA and diabetic nephropathy status which could serve as an alternative way to screen for albuminuria.

Optical Coherence Tomography Features for Identifying Posttreatment Complete Polypoidal Regression in Polypoidal Choroidal Vasculopathy.

PURPOSE: To determine accuracy and relative risk (RR) of posttreatment optical coherence tomography (OCT) features in identifying complete or incomplete polypoidal regression in polypoidal choroidal vasculopathy (PCV). DESIGN: Validity analysis. METHODS: Treatment-naive PCV eyes undergoing OCT and indocyanine green angiography (ICGA) at baseline and posttreatment were included. Two graders confirmed diagnosis and identified posttreatment complete or incomplete regression on ICGA. Two other graders classified OCT characteristics of pigment epithelial detachment (PED) (polypoidal lesion) based on 5 prespecified features: "A," no PED; "B," PED with internal homogeneous reflectivity with predominant "BUN" (blended retinal pigment epithelium with underlying structure) sign; "C," PED with internal homogeneous reflectivity with minimal "BUN"; "D," heterogeneous PED; and "E," PED with hyporeflectivity. RESULTS: Among 130 polypoidal lesions (65 pretreatment and 65 posttreatment) of 39 PCV eyes (39 patients; 54% female; mean age±SD: 64.6±8.2), all pretreatment lesions showed feature D on OCT. Posttreatment lesions with complete regression (31 lesions) showed OCT features A, B, C, D, and E in 32%, 45%, 13%, 10%, and 0%, respectively. Posttreatment lesions with incomplete regression (34 lesions) showed OCT features A, B, C, D, and E in 0%, 6%, 15%, 79%, and 0%, respectively. Presence of either feature A or B had highest accuracy (86%; 95% confidence interval: 75%-93%); 77% sensitivity; 94% specificity; RR 5.0 (3.5-7.1, P<0.001) for complete regression. Presence of feature D had highest accuracy (85%; 95% confidence interval: 74%-92%); 79% sensitivity; 90% specificity; RR 4.6 (3.0-6.9, P<0.001) for incomplete regression. CONCLUSIONS: Without ICGA, OCT features could provide high accuracy in identifying posttreatment complete or incomplete polypoidal regression in PCV.

Subretinal gnathostomiasis: A successful focal laser photocoagulation for a living parasite.

PURPOSE: To report a case of subretinal gnathostomiasis presenting with progressive subretinal tracts of a living parasite and successfully treated with focal laser photocoagulation. METHOD: Observational case report. PATIENT: A 29-year-old Thai male complained of blurred vision and floaters in his left eye for two weeks. An ocular examination showed multiple, whitish, subretinal tracks at the superotemporal retina. After 5 days of oral albendazole, a moving parasite was confirmed by multimodal retinal imaging. An immunoblotting analysis was positive for Gnathostoma species. RESULT: The patient was treated by laser photocoagulation with frequency-doubled Nd:YAG laser around and over the parasite. Oral albendozole was continued and naproxen was prescribed for four weeks. His vision improved to 20/20 and the inflammation subsided completely within three months. The patient has been followed for five years without local and systemic complications. CONCLUSIONS: Focal laser photocoagulation without systemic steroids could be a successful treatment for active subretinal gnathostomiasis with a satisfactory safety profile in a long-term follow-up.

Long-term visual and anatomic outcomes of patients with peripapillary pachychoroid syndrome.

BACKGROUND/AIMS: To analyse the long-term anatomic and visual outcomes of patients with peripapillary pachychoroid syndrome (PPS), a recently described entity in the pachychoroid disease spectrum. METHODS: This study retrospectively included patients from several retina centres worldwide. Visual acuity (VA), retinal thickness and choroidal thickness at baseline, 6 months and final follow-up were assessed. Temporal trends in VA and anatomic characteristics were evaluated. Visual and anatomic outcomes in eyes that were observed versus those that were treated were analysed. RESULTS: Fifty-six eyes of 35 patients were included with mean follow-up of 27±17 months. Median VA was 20/36 at baseline and remained stable through follow-up (p=0.77). Retinal thickness significantly decreased subfoveally (p=0.012), 1.5 mm nasal to the fovea (p=0.002) and 3.0 mm nasal to the fovea (p=0.0035) corresponding to areas of increased thickening at baseline. Choroidal thickness significantly decreased subfoveally (p=0.0030) and 1.5 mm nasal to the fovea (p=0.0030). Forty-three eyes were treated with modalities including antivascular endothelial growth factor injection, photodynamic therapy, and others. VA remained stable in treated eyes over follow-up (p=0.67). An isolated peripapillary fluid pocket in the outer nuclear layer was characteristic of PPS. CONCLUSION: Patients with PPS experienced decreased retinal oedema and decreased choroidal thickening throughout the course of disease. While some patients experienced visual decline, the overall visual outcome was relatively favourable and independent of trends in retinal or choroidal thickening.

In-Person Verification of Deep Learning Algorithm for Diabetic Retinopathy Screening Using Different Techniques Across Fundus Image Devices.

Purpose: To evaluate the clinical performance of an automated diabetic retinopathy (DR) screening model to detect referable cases at Siriraj Hospital, Bangkok, Thailand. Methods: A retrospective review of two sets of fundus photographs (Eidon and Nidek) was undertaken. The images were classified by DR staging prior to the development of a DR screening model. In a prospective cross-sectional enrollment of patients with diabetes, automated detection of referable DR was compared with the results of the gold standard, a dilated fundus examination. Results: The study analyzed 2533 Nidek fundus images and 1989 Eidon images. The sensitivities calculated for the Nidek and Eidon images were 0.93 and 0.88 and the specificities were 0.91 and 0.85, respectively. In a clinical verification phase using 982 Nidek and 674 Eidon photographs, the calculated sensitivities and specificities were 0.86 and 0.92 for Nidek along with 0.92 and 0.84 for Eidon, respectively. The 60°-field images from the Eidon yielded a more desirable performance in differentiating referable DR than did the corresponding images from the Nidek. Conclusions: A conventional fundus examination requires intense healthcare resources. It is time consuming and possibly leads to unavoidable human errors. The deep learning algorithm for the detection of referable DR exhibited a favorable performance and is a promising alternative for DR screening. However, variations in the color and pixels of photographs can cause differences in sensitivity and specificity. The image angle and poor quality of fundus photographs were the main limitations of the automated method. Translational Relevance: The deep learning algorithm, developed from basic research of image processing, was applied to detect referable DR in a real-word clinical care setting.

Non-Neovascular Pachychoroid Disease Mimicking Exudative Age-Related Macular Degeneration.

PURPOSE: To describe a case of pachychoroid disease most compatible with central serous chorioretinopathy (CSC) presented with a large relapsing retinal pigment epithelial detachment (PED) associated with only a small amount of subretinal fluid (SRF) in the background of macular drusen in an elderly patient mimicking neovascular age-related macular degeneration (AMD). METHODS: This was a review of 32 months of the clinical course and findings on multimodal imaging including fundus photography, optical coherence tomography, fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and optical coherence tomography angiography (OCTA). RESULTS: A large relapsing PED sometimes with small amount of SRF at its apex was found in the background of macular drusen in the right eye of a 63-year-old Thai woman. The relapses of the PED showed an apparent association with recent steroid exposure. Multimodal imaging demonstrated the characteristics of pachychoroid diseases with pachyvessels and choroidal hyperpermeability. The PED and SRF responded well to anti-vascular endothelial growth factor (anti-VEGF) therapy, especially aflibercept, but could also be spontaneously resolved without anti-VEGF. No evidence of choroidal neovascularization or polyps could be identified by serial FFA, ICGA, and OCTA even when the retinal pigment epithelium was completely flat with no obscuration by PED. CONCLUSIONS: A large PED with little SRF could present signs of CSC. This clinical presentation could be confused with neovascular AMD, and multimodal retinal imaging was crucial to guide correct diagnosis and management.

Recent advances in wide field and ultrawide field optical coherence tomography angiography in retinochoroidal pathologies.

Introduction: With the advent of wide field (WF) and ultra-wide field (UWF) imaging in color fundus photography and fluorescein angiography (FA), the importance of an expanded field-of-view (FOV) has been illustrated for early detection of several chorioretinal disorders. The need to bring similar utility to optical coherence tomography angiography (OCTA) is imperative.Areas covered: For this review, we explored all research items with WF or UWF on Pubmed Central and Google Scholar. Software-based methods included algorithms for motion correction, artifact reduction, image pre-processing, post-processing and montage protocols while hardware methods included swept-source OCTA, faster scanning sources, hardware-based motion tracking and image registration, scanning sources with faster speeds and lower resolution. For various disorders such as diabetic retinopathy, uveitis, vascular disorders, among others, the increased FOV showed demonstrably increased detection rates and diagnostic yield.Expert opinion: Increased FOV in OCTA imaging is a meaningful and impactful step in ophthalmic imaging. Overcoming the hardware-based limitations can potentially yield higher FOV without loss of important details. In addition to improvements in the hardware and available equipment, better software tools and image processing algorithms may greatly enhance the utility of UWF OCTA in care of patients with various retinochoroidal diseases.

Extralesional microvascular and structural macular abnormalities in cytomegalovirus retinitis.

To evaluate extralesional microvascular and structural changes of the macula using optical coherence tomography angiography (OCTA) and structural OCT in cytomegalovirus retinitis (CMVR). An observational study of CMVR patients were performed. Complete ophthalmic examination, serial color fundus photography, structural OCT and OCTA were performed at baseline and follow-up visits for up to 12 months. The structural OCT was analyzed to evaluate macular areas within, bordering and beyond the CMVR lesions. Extralesional retinal capillary plexus of the macula were evaluated by OCT angiography and compared with the unaffected fellow eyes. Thirteen eyes from 13 patients were enrolled. At baseline, macular areas without CMVR lesions showed decreased vessel density (VD) of both the superficial (P = 0.0002) and deep (P < 0.0001) retinal capillary plexus in eyes with CMVR as compared with the corresponding macular areas of the unaffected fellow eyes. The decrease of VD persisted through the follow-up period for up to 12 months after adjusting for degree of vitreous haze. Structural macular OCT characteristics at the borders and beyond the lesions included intraretinal hyperreflective dots, cystoid macular edema, subretinal fluid and selective ellipsoid zone (EZ) loss. The selective EZ loss found in 6 of 12 eyes showed recovery in 4 eyes after receiving anti-viral treatment. In CMVR eyes, there were microvascular and microstructural abnormalities in the macular area without clinically visible CMVR lesions. Our results provided interesting insights into CMV infection of the retina.

Unusual non-nanophthalmic uveal effusion syndrome with histologically normal scleral architecture: a case report.

BACKGROUND: To report an unusual case of non-nanophthalmic uveal effusion syndrome (UES) with histologically normal sclera but responsive to scleral resection. CASE PRESENTATION: A73-year-old man presented with a bullous retinal detachment without ciliochoroidal detachment on funduscopic examination of the right eye. The axial length of both eyes was normal. Extensive investigations for possible causes of exudative retinal detachment were performed with unremarkable results except for choroidal hyperpermeability on indocyanine green angiography (ICGA). Ultrasound biomicroscopy (UBM) revealed scleral thickening with peripheral choroidal elevation leading to the diagnosis of UES. Partial thickness sclerectomy and sclerotomy was performed resulting in complete retinal reattachment, reduction of choroidal hyperpermeability on ICGA and improvement of visual acuity. However, histological studies of the excised sclera revealed no scleral architectural changes or abnormal deposits. CONCLUSIONS: The diagnosis of UES in non-nanophthalmic eyes is challenging. Thorough systemic and ocular investigations are critical to rule out other etiologies. UBM can be helpful to evaluate scleral thickness and anterior choroid in equivocal cases. Our case was unique in that, although the sclera was thick, no abnormal microscopic scleral architecture could be identified. Misdiagnosis may lead to different surgical procedures such as vitrectomy resulting in unfavorable outcomes.

Relationship Between Nerve Fiber Layer Hemorrhages and Outcomes in Central Retinal Vein Occlusion.

Purpose: To evaluate the depth and pattern of retinal hemorrhage in acute central retinal vein occlusion (CRVO) and to correlate these with visual and anatomic outcomes. Methods: Retinal hemorrhages were evaluated with color fundus photography and fluorescein angiography at baseline and follow-up. Snellen visual acuity (VA), central foveal thickness (CFT), extent of retinal ischemia, and development of neovascularization were analyzed. Results: 108 eyes from 108 patients were evaluated. Mean age was 63.6 ± 16.1 years with a predilection for the right eye (73.1%). Average follow-up was 17.2 ± 19.2 months. Mean VA at baseline was 20/126 and 20/80 at final follow-up. Baseline (P = 0.005) and final VA (P = 0.02) in eyes with perivascular nerve fiber layer (NFL) hemorrhages were significantly worse than in eyes with deep hemorrhages alone. Baseline CFT was greater in the group with perivascular hemorrhages (826 ± 394 µm) compared to the group with deep hemorrhages alone (455 ± 273 µm, P < 0.001). The 10 disc areas of retinal ischemia was more common in patients with perivascular (80.0%) and peripapillary (31.3%) versus deep hemorrhages alone (16.1%, P < 0.001). Neovascularization of the iris was more common, although this differrence was not significant, in the groups with peripapillary (14.3%) and perivascular (2.0%) NFL versus deep hemorrhages alone (0.0%). Conclusions: NFL retinal hemorrhages at baseline correlate with more severe forms of CRVO, with greater macular edema, poorer visual outcomes, and greater risk of ischemia and neovascularization. This may be related to the organization of the retinal capillary plexus. The depth and pattern of distribution of retinal hemorrhages in CRVO may provide an easily identifiable early biomarker of CRVO prognosis.

PERIVASCULAR GRANULOMATA IN THE RETINA DEMONSTRATED BY EN FACE OPTICAL COHERENCE TOMOGRAPHY IN A PATIENT WITH BLAU SYNDROME.

PURPOSE: To describe the multimodal retinal imaging findings of a case of Blau syndrome. METHODS: Case report. The multimodal retinal imaging findings, including widefield angiography, cross-sectional and en face spectral domain optical coherence tomography, and optical coherence tomography angiography, of a case of Blau syndrome are described. RESULTS: A 12-year-old Hispanic girl with polyarticular arthritis, skin rash, and panuveitis was diagnosed with a rare condition referred to as Blau syndrome. Genetic testing identified an NOD2 mutation. Cross-sectional and en face optical coherence tomography illustrated presumed perivascular granulomata in the middle retina. Optical coherence tomography angiography failed to display flow within these lesions. CONCLUSION: This report illustrates the presence of a remarkable distribution of perivascular, presumably granulomatous, retinal lesions in the middle retina. Similar perivascular granulomata have been noted in the histopathological analysis of ocular sarcoidosis.

BIOMARKERS OF NEOVASCULAR ACTIVITY IN AGE-RELATED MACULAR DEGENERATION USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To study the qualitative and quantitative features of choroidal neovascular (NV) membranes in age-related macular degeneration using optical coherence tomography angiography in patients with active and quiescent NV lesions before and after treatment with anti-vascular endothelial growth factor. METHODS: Macular optical coherence tomography angiography images were obtained using RTVue XR Avanti with AngioVue. Morphologic features and quantitative measurements of the NV lesion were analyzed using en face projection images. The NV lesion was subdivided into inner segment and outer fringe for further fractal dimension analysis. RESULTS: In a series of 31 eyes, 11 eyes with active NV lesions at baseline and after consecutive follow-up after treatment with anti-vascular endothelial growth factor therapy and 20 eyes with quiescent NV lesions were included in this study. Morphologically, all the quiescent NV lesions versus 63.6% of the active NV lesions demonstrated a prominent central vessel and active leasions demonstrated a greater rate of small vessels branching (82%) and peripheral arcades (82%) than quiescent lesions (30% and 40% respectively) and this was statistically significant. The lesion area and vessel density was not statistically significantly different after treatment or versus quiescent lesions although the latter lesions were reduced in area. Lesion pattern complexity measured by the fractal dimension was statistically significantly lower in the inner part of the lesion after treatment and statistically significantly lower in the total lesion of the quiescent NV compared with the active NV. CONCLUSION: Optical coherence tomography angiography is a new, noninvasive imaging modality that can be used to perform qualitative and quantitative analyses of NV lesions. In the future, OCT angiography may provide biomarkers of activity and guide the evaluation and treatment and monitoring of neovascularization in age-related macular degeneration.

PROGRESSION OF MACULAR ATROPHY IN EYES WITH TYPE 1 NEOVASCULARIZATION AND AGE-RELATED MACULAR DEGENERATION RECEIVING LONG-TERM INTRAVITREAL ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR THERAPY: An Optical Coherence Tomographic Angiography Analysis.

PURPOSE: To evaluate the size and location of macular atrophy in eyes with Type-1 neovascularization (NV) and age-related macular degeneration receiving chronic intravitreal anti-vascular endothelial growth factor therapy. METHODS: A retrospective review of a case series of 27 eyes with Type-1 NV and retinal pigment epithelial detachment (PED) having a minimum of 12 months follow-up was performed. Demographic information and visual acuity at baseline and the final follow-up were collected. Spectral-domain optical coherence tomography (OCT) and near-infrared reflectance were analyzed at 6-month intervals to detect and measure macular atrophy. Location and area (in square millimeter) of macular atrophy were measured using Heidelberg software tools. Also, OCT angiography was used to colocalize the area of Type-1 NV flow versus the location of atrophy. RESULTS: Twenty-seven eyes of 27 patients were included in this analysis. The median visual acuity was 20/50, mean age was 82.7 years, and mean number of injections was 29.5. A larger percentage of eyes (59.3%) developed atrophy predominantly eccentric to the PED versus predominantly overlying the PED (11.1%) when measured with spectral-domain OCT and near-infrared imaging. At the final follow-up, there was a larger area of atrophy surrounding the fibrovascular PED (mean, 3.326 mm) than overlying it (mean, 0.542 mm), and this was statistically significant (P = 0.0118). En-face OCT images were overlaid with OCT angiography in 11 eyes, and a predominantly eccentric pattern of atrophy was identified in 9 of 11 eyes. Using this method, the mean area of atrophy predominantly overlying the Type-1 NV was 1.652 mm (range of 0-10.464 mm), whereas the area of atrophy predominantly eccentric to the neovascular complex was 4.345 mm (range of 0.705-13.758 mm), and this was statistically significant (P = 0.0465). The average rate of atrophy progression was 1.04 mm/year (SD 0.938). CONCLUSION: With long-term anti-vascular endothelial growth factor therapy for eyes with Type-1 NV secondary to age-related macular degeneration, macular atrophy tends to develop predominantly eccentric to the PED and the neovascular flow imaged on OCT angiography. With chronic vascular endothelial growth factor suppression, Type-1 NV may evolve into a multilayered PED that may confer a protective effect to the overlying retinal pigment epithelium and outer retina.

PERIPAPILLARY PACHYCHOROID SYNDROME.

PURPOSE: To describe the features of peripapillary pachychoroid syndrome (PPS), a novel pachychoroid disease spectrum (PDS) entity. METHODS: Medical records of 31 eyes (16 patients) with choroidal thickening associated with intraretinal and/or subretinal fluid in the nasal macula extending from the disk were reviewed (patients with PPS). Choroidal thickness was compared with 2 age-matched cohorts: typical PDS (17 eyes with central serous chorioretinopathy or pachychoroid neovasculopathy) and 19 normal eyes. RESULTS: The patients with PPS were 81% men aged 71 ± 7 years. Peripapillary pachychoroid syndrome eyes displayed thicker nasal versus temporal macular choroids, unlike PDS eyes with thicker temporal macular choroids (P < 0.0001). Peripapillary intraretinal and/or subretinal fluid was often overlying dilated Haller layer vessels (pachyvessels). Fundus autofluorescence and fluorescein angiography illustrated peripapillary pigmentary mottling without focal leakage. Most PPS eyes (70%) exhibited other PDS findings including serous pigment epithelial detachment or gravitational tracks. Indocyanine green angiography illustrated dilated peripapillary pachyvessels and choroidal hyperpermeability. The disk was usually crowded, with edema noted in 4/31 (13%) eyes and mild late fluorescein disk leakage identified in half of the cases. Choroidal folds (77%), short axial lengths (39% less than 23 mm), and hyperopia (86%) were common. CONCLUSION: Peripapillary pachychoroid syndrome is a distinct PDS variant, in which peripapillary choroidal thickening is associated with nasal macular intraretinal and/or subretinal fluid and occasional disk edema. Recognition of PPS is important to distinguish it from disorders with overlapping features such as posterior uveitis and neuro-ophthalmologic conditions.

Foveal and Peripapillary Vascular Decrement in Migraine With Aura Demonstrated by Optical Coherence Tomography Angiography.

Purpose: Migraine, particularly with aura, has been associated with ocular and systemic ischemic complications, but there are limited data on the ocular vasculature in migraine. We used optical coherence tomography angiography (OCTA) to assess perfusion of the macula and optic nerve in migraine patients, with (MA) and without (MO) aura, compared to healthy controls (HC). Methods: We recruited 15 MA (mean age 42 years), 12 MO (mean age 46 years), and 22 HC (mean age 39 years) participants from neurology and neuro-ophthalmology clinics. Participants underwent optical coherence tomography and 3 × 3 mm OCTA of the macula and optic nerve. Foveal avascular zone area was automatically measured using AngioVue software, and vessel density was calculated as blood vessel length divided by scan area (mm-1) after skeletonization of OCTA images. Results: On macular OCTA, MA participants had an enlarged foveal avascular zone area when compared with HC (0.300 ± 0.019 vs. 0.220 ± 0.066 mm2, P = 0.006). In addition, superficial foveal vessel density was decreased in MA participants when compared with MO participants (7.8 ± 0.31 vs. 9.3 ± 0.44, P = 0.04) and HC (7.8 ± 0.31 vs. 9.4 ± 0.21 mm-1, P = 0.002). On optic nerve OCTA, the MA participants had reduced superior peripapillary vessel density when compared with the MO participants (12.0 ± 0.45 vs. 14.0 ± 0.38 mm-1, P = 0.031) and HC (12.0 ± 0.45 vs. 14.1 ± 0.53 mm-1, P = 0.035). There were no significant differences between the MO and HC groups. Conclusions: Migraine with, but not without, aura was associated with foveal and peripapillary vascular decrements, which may possibly mediate increased risk of ocular and systemic vascular complications in these patients. OCTA could potentially be useful as a biomarker for migraine with aura.

Quantitative Analysis of Three Distinct Retinal Capillary Plexuses in Healthy Eyes Using Optical Coherence Tomography Angiography.

Purpose: To identify and quantify the three distinct retinal capillary plexuses and the foveal avascular zone (FAZ) in healthy subjects according to age using optical coherence tomography angiography (OCTA) with novel projection artifact removal (PAR) software and improved segmentation. Methods: All eyes in this cross-sectional study underwent OCTA imaging using RTVue XR Avanti with novel PAR AngioVue software. OCTA scans were analyzed and the three main parafoveal retinal capillary plexuses were segmented and vessel density and FAZ area were calculated. Results: A total of 152 normal eyes from 95 subjects (39 males, 56 females, mean age 42 ± 25 years) were included. The mean vessel density was 15.48 ± 2.04 mm-1 in the superficial retinal capillary plexus (SCP), 15.28 ± 1.82 mm-1 in the intermediate retinal capillary plexus (ICP), and 16.33 ± 2.32 mm-1 in the deep retinal capillary plexus (DCP) for 3 × 3-mm OCTA images. Analysis of 3 × 3-mm scans yielded a mean FAZ area of 0.270 ± 0.101 mm2. The average reduction in vessel density per year of age with 3 × 3-mm OCTA scans was 0.04 mm-1 (0.22%) in the SCP, 0.05 mm-1 (0.27%) in the ICP, and 0.06 mm-1 (0.30%) in the DCP. The average increase in FAZ area per year of age was 0.0015 mm2 (0.72%). Conclusions: Novel PAR software may provide improved visualization of all three major parafoveal retinal capillary plexuses including the ICP. Using this technology, SCP, ICP, and DCP vessel density decreased with increasing age while FAZ area increased with age.

The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex.

PURPOSE: To expand our understanding of the uncommon entity, referred to as perifoveal exudative vascular anomalous complex (PEVAC) by describing multimodal imaging findings, including optical coherence tomography angiography (OCT-A). DESIGN: Retrospective cohort study. METHODS: Patients diagnosed with PEVAC were identified at 4 retina referral centers worldwide and underwent complete ophthalmologic examination including structural OCT, OCT-A, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Demographics and clinical findings were analyzed at baseline and at available follow-ups. RESULTS: Fifteen eyes (15 patients, mean age 73 ± 13 years) were included. Six of 15 eyes were diagnosed with coincident age-related macular degeneration (AMD) and 2 with myopic macular degeneration. On fundus examination PEVAC presented as a large perifoveal isolated aneurysm, unifocal in 12 of 15 eyes, associated with small retinal hemorrhages and intraretinal exudation. On structural OCT, PEVAC appeared as a round hyperreflective lesion with hyporeflective lumen, typically surrounded by intraretinal cystic spaces. Dye angiography demonstrated a well-defined hyperfluorescent lesion with variable leakage on FA and without leakage on ICGA. OCT-A showed flow signal correlating with the aneurysmal lesion connecting to retinal capillary plexuses. Seven patients were followed for 13.0 ± 10.5 months with no evidence of functional/anatomic changes. Three patients underwent anti-vascular endothelial growth factor (VEGF) intravitreal injections without improvement. Two eyes were associated with a type 3 neovascularization eccentric to PEVAC. CONCLUSIONS: PEVAC is an isolated, perifoveal, aneurysmal abnormality, occurring in otherwise healthy patients who may manifest other macular disease including AMD and myopic macular degeneration. PEVAC did not typically respond to anti-VEGF therapy, and may be associated with type 3 neovascularization.