Characteristics, Etiology, and Clinical Outcome of Retinal Vasculitis in Tertiary Hospital in Thailand.

OBJECTIVE: To analyze characteristics, etiology, and outcome of retinal vasculitis in Central Thailand. METHODS: A retrospective cohort study. RESULTS: Retinal vasculitis was found in 10% of uveitis, 74 from 741 uveitis, noninfectious (64.9%) and infectious group (35.1%). The most common cause was Behcet's disease (48.6%). Behcet's disease was the most common cause of all types of vascular leakage on angiography, including capillary (80.4%), venous (56.3%), and arterial leakage (56%). Final visual acuity was 0.86 ± 0.97 logMAR. Cataract was the most frequent complication (42.5%). Acute clinical course (p = .025) and retinal neovascularization (p = .031) were associated with infectious group. Forty-three percent of vasculitis complicated by ischemia required photocoagulation (33%) and anti-VEGF injection (17%). Furthermore, 17% of vasculitis underwent vitrectomy. CONCLUSION: One-half of the retinal vasculitis in Central Thailand were Behcet's disease. Acute onset and retinal neovascularization may suggest infectious etiology. Retinal ischemia should be cautious and undergo early interventions to prevent sight-threatening complications.

Clinical Correlation of Retinal Fluid Fluctuation Represented by Fluctuation Index in Wet Age-Related Macular Degeneration: TOWER Study Report 2.

Purpose: To explore outcomes and biomarkers associated with retinal fluid instability represented by a new parameter in neovascular age-related macular degeneration (nAMD). Methods: Patients with treatment-naïve nAMD receiving anti-vascular endothelial growth factor (VEGF) injections for a duration of 1 to 3 years were consecutively reviewed. Fluctuation Index (FI) of each eye, calculated by averaging the sum of differences in 1-mm central subfield thickness between each follow-up from months 3 to 24, was arranged into ascending order from the lowest to the highest and split equally into low, moderate, and high fluctuation groups. Outcomes were analyzed at 24 months. Results: Of 558 eyes, FI values showed a negative correlation with a degree-response gradient with 24-month visual improvement. After controlling for baseline best-corrected visual acuity and potential confounders, eyes with low fluctuation gained more Early Treatment Diabetic Retinopathy Study letters than those in the moderate and high fluctuation group (Δ, 10.1 and 14.0 letters, respectively). Significant best-corrected visual acuity improvement from baseline to month 24 (11.8 letters) was observed exclusively in the low fluctuation group despite the indifference in the number of injections and types of anti-VEGF drug used among groups. Patients presenting with central subfield thickness of ≥405 µm or intraretinal fluid coinciding with subretinal fluid showed a significant association with foveal thickness instability during the maintenance phase. Conclusions: Apart from the central subfield thickness values, unstable macular thickening represented by the FI was associated with some baseline features and may contribute to substandard visual outcomes. Translational Relevance: FI may be a valuable tool for assessing therapeutic adequacy in the treatment of nAMD.

Pigmentary retinopathy and nodular granuloma associated with acute retinal necrosis from varicella zoster virus and human herpes virus type 6: Case report.

RATIONALE: Acute retinal necrosis (ARN) caused by human herpes virus type 6 (HHV-6) is uncommon. We described a case of consecutive bilateral ARN, which was found to be a coinfection of varicella zoster virus (VZV) and HHV-6 in a 50-year-old woman, not well responded with systemic acyclovir. We showed the atypical findings with corresponding fundus and optical coherence tomography imaging. PATIENT CONCERNS: She presented with anterior segment inflammation with peripheral retinitis and vasculitis in the left eye with disease progression despite of initial antiviral treatment, end up with retinal detachment. The right eye, subsequently, developed focal retinitis. DIAGNOSIS: ARN was diagnosed by clinical fundus picture, confirmed by polymerase chain reaction (PCR). INTERVENTIONS: Initially, she was treated with intravenous acyclovir and intravitreal ganciclovir for left eye. Retinal necrosis progressed, followed by retinal detachment. Pars plana vitrectomy with silicone oil was performed. The right eye, subsequently, developed focal retinitis. Medication was switched to intravenous ganciclovir and then oral valganciclovir. OUTCOMES: Retinitis was resolved, generalized hyperpigmentation appeared as a salt-and-pepper appearance in the right eye. The left eye presented preretinal deposits on silicone-retina interphase along retinal vessels. Spectral-domain optical coherence tomography (SD-OCT) showed multiple hyperreflective nodules on retinal surface. LESSONS: ARN from coinfection of VZV and HHV-6 is rare. Preretinal granulomas and generalized hyperpigmentation could be one of the HHV-6 features. HHV-6 should be in the differential diagnosis for ARN. It responds well to systemic ganciclovir.

Treatment Outcome of Wet Age-Related Macular Degeneration Management in Thailand: A Retrospective Real-World Study (TOWER Study).

INTRODUCTION: To present real-world outcomes of neovascular age-related macular degeneration (nAMD) management in Thailand. METHODS: This multicenter retrospective study reviewed medical records of naive nAMD patients diagnosed from 1 January 2016 until 31 December 2018. The patients received at least one intravitreal anti-vascular endothelial growth factor (VEGF) treatment and had captured visual acuity (VA) at baseline and at month 12. Treatment outcomes were assessed at month 12, 24, and 36. The primary outcome was a mean change in VA from baseline to month 12. RESULTS: Five hundred seventy-two (572) eyes were included in this study and of these eyes, 222 and 96 had 2- and 3-year follow-up periods, respectively. At month 12, the mean improvement of VA (ETDRS letter) was six letters (P < 0.0001), and central retinal thickness (CRT) decreased on average by 104 microns (P < 0.0001). However, visual improvement by 0.1 letters at month 36 did not show statistical significance. The presence of fluid was found in approximately half of patients throughout the study period (45.98%, 48.85%, and 50.91% at month 12, 24, and 36, respectively). Mean number of injections (SD) was 6.06 (3.00), 3.44 (2.94), and 2.71 (3.07) for years 1, 2, and 3, respectively. The mean number of visits (SD) in year 1 was 9.01 (2.60) and declined to 5.67 (2.69) in year 2 and 4.93 (2.49) in year 3. Patients who had an average injection interval of ≤ 8 weeks were 74.46% in year 1, 51.28% in year 2, and 45.24 in year 3; 35.31% of patients were lost to follow-up. CONCLUSIONS: This analysis reflects real-world nAMD management with significant improvement of outcomes. At the same time, the study reveals unmet needs in anti-VEGF therapy in nAMD including persistent disease activities, inadequacy of available treatment, and lack of treatment adherence leading to visual deterioration in the long-term.

Fluorescein angiographic findings and Behcet's disease ocular attack score 24 (BOS24) as prognostic factors for visual outcome in patients with ocular Behcet's disease.

PURPOSE: To determine the application of fluorescein angiographic (FA) findings and Behcet's disease ocular attack score 24 (BOS24) scoring system in predicting poor visual outcome in patients with ocular Behcet's disease. STUDY DESIGN: Retrospective cohort study. METHODS: We included 73 eyes of 38 patients with ocular Behcet's disease who underwent FA and reviewed FA images, anterior chamber cells, vitreous opacity, retinal and optic disc lesions, which are parameters in BOS24. The correlation between FA findings, BOS24, and visual acuity was assessed. RESULTS: Optic disc hyperfluoresence (74%), diffuse posterior pole leakage (52%) and diffuse peripheral leakage (52%) were the three most common findings. Common complications were peripheral capillary nonperfusion (29%), arterial narrowing (22%), and macular ischemia (19%). BOS24 scores of ≥ 6 (p < 0.0001), arterial narrowing (p < 0.0001), and severe posterior pole leakage (p = 0.004) were significantly associated with poor visual outcome. Combining significant FA findings: arterial narrowing and severe posterior pole leakage, to BOS24 ≥ 6 results in an increased relative risk of developing poor visual acuity from 7.30 to 10.43 and 1.89 to 2.02 respectively. CONCLUSION: Fluorescein angiography is an important investigation for predicting poor visual outcome. BOS24 may be a useful alternative when fluorescein angiographic is unavailable.

Combined central retinal vein, central retinal artery and cilioretinal artery occlusion with ischemic macular hole secondary to severe orbital cellulitis after black fly bite.

Background: Orbital cellulitis is an infection of the ocular adnexal and orbital tissues behind the orbital septum. The complications are poor prognosis. This paper is to describe some severe rare complications of orbital cellulitis. Case presentation: A case of 32-year-old female presented with acute severe progressive orbital cellulitis on the right eye after a black fly (Simuliidae spp.) bite. Orbital imaging with computed tomography and magnetic resonance imaging showed eyelid abscess with orbital abscess formation and superior ophthalmic vein thrombosis. She was promptly treated with intravenous vancomycin, piperacillin and had surgical procedures to release orbital pressure including lateral canthotomy, lateral cantholysis, and eyelid with orbital abscess drainage. Pus collection culture was positive for Staphylococcus aureus. Even though she responded well to antibiotics treatment, progression involved severe orbital inflammation with marked increased orbital pressure, and her visual acuity was such that she had no light perception. Combined central retinal vein, central retinal artery and cilioretinal artery occlusion were identified. Fundus examination showed retinal whitening at the posterior pole and a few scattered flame-shaped retinal hemorrhages. The cilioretinal artery presented with perivascular retinal whitening. A fundus fluorescein angiogram revealed cilioretinal and retinal artery filling delay and also delayed arteriovenous transit time. She also developed an ischemic macular hole after a week of treatment. We demonstrate an optical coherence tomography image showing the intact posterior hyaloid membrane that represents no traction on the macula to support the ischemic cause. Conclusion: Multiple retinal vascular occlusion and ischemic macular hole could be an early complication of severe orbital cellulitis from increased orbital pressure; therefore, close monitoring of visual acuity with IOP, and prompt treatment when indicated might reduce the risk of consequent blindness.

Assessment of Vascular Change Using Swept-Source Optical Coherence Tomography Angiography: A New Theory Explains Central Visual Loss in Behcet's Disease.

OBJECTIVE: To evaluate retinal vascular structural change in ocular Behcet's using optical coherence tomography angiography (OCTA) and fluorescein angiography (FA). METHODS: An analytic cross-sectional study of 37 eyes of 21 Behcet's uveitic patients was performed. Foveal retinal thickness (FRT), perifoveal hypoperfusion areas in superficial capillary plexus (SCP), and deep capillary plexus (DCP) were measured with swept-source optical coherence tomography and OCTA. FA images were used for assessing the vascular features and correlation. RESULTS: Twenty-one patients were enrolled (52.4% males). The average age at onset was 36.7 ± 12.93 years. The median of disease duration was 5 years (1-25). FRT was 118.1 ± 52.35 µm, which correlated with visual acuity (95% CI -60.47, -13.92). Using OCTA, the area of hypoperfusion in SCP (0.47 ± 0.17 mm2) was smaller than that in DCP (1.94 ± 3.87 mm2) (p < 0.001). Superficial to deep capillary plexus nonperfusion (SCP : DCP) ratio was 0.57 ± 0.27 which had the positive coefficient correlation with visual acuity (95% CI -0.644, -0.015). CONCLUSIONS: OCTA is an alternative noninvasive method to monitor macular ischemia in Behcet. Behcet's uveitis affects DCP more than SCP. Decreasing SCP : DCP ratio and decrease FRT correlates with poor visual acuity. Macular ischemia and DCP loss can be found early and can explain vision loss in Behcet.

Idiopathic polypoidal choroidal vasculopathy in Thai patients with clinical and angiographic choroidal neovascularization.

OBJECTIVE: This study aimed to study the prevalence and characteristics of idiopathic polypoidal choroidal vasculopathy (IPCV) in Thai patients with clinical and angiographic choroidal neovascularization (CNV). PATIENTS AND METHODS: A consecutive case study of 140 patients presenting with CNV was conducted in nine large referral eye centers throughout Thailand. The demographic data, fundus photographs, fundus fluorescein angiography and indocyanine green angiography of the patients were analyzed. RESULTS: Of 129 patients with clinical and angiographic CNV, IPCV was diagnosed in 100 patients (77.52%), idiopathic CNVs in 16 patients (12.40%) and age-related macular degeneration (AMD) in 12 patients (9.30%). Of the 107 eyes with IPCV, 90 eyes (84.11%) had both branching venous networks (BVNs) and polypoidal lesions. Most IPCV patients (93%) had unilateral involvement and were at a younger age than AMD patients. In all, 79 eyes (73.83%) had lesions found in the macular area, 14 eyes (13.08%) in the temporal to vascular arcades, ten eyes (9.35%) in the peripapillary area and four eyes (3.74%) in both macular and peripapillary areas. The clinical manifestations of IPCV at presentation were categorized into two patterns. There were 95 eyes (88.79%) of a hemorrhagic pattern and 12 eyes (11.21%) of an exudative pattern. CONCLUSION: IPCV is the most common macular disease in Thai patients with CNV. Most IPCVs have both BVNs and polypoidal lesions located in the macular area and present with a hemorrhagic pattern.

MULTIMODAL VISUAL FUNCTION TESTING IN EYES WITH NONEXUDATIVE AGE-RELATED MACULAR DEGENERATION.

PURPOSE: To investigate the interactions among drusen type and multimodal vision testing in eyes with nonexudative age-related macular degeneration. METHODS: Fifty-one eyes of 39 patients with nonexudative age-related macular degeneration underwent fundus imaging including spectral domain optical coherence tomography, color fundus photograph, and autofluorescence imaging, each of which was graded by 2 masked readers. Multimodal vision testing included visual acuity using the Early Treatment Diabetic Retinopathy Study protocol refraction, contrast sensitivity, and microperimetry. RESULTS: Generalized estimating equation modeling showed that the significant predictors of contrast sensitivity was the presence of pseudodrusen (P = 0.012) and refractive error (P = 0.028). The presence of pseudodrusen inversely correlated with contrast sensitivity. The significant predictors of parafoveal microperimetry score were area of confluent hypoautofluorescence (P = 0.026) and the presence of pseudodrusen (P = 0.027). Both of them showed an inverse correlation with microperimetry score. The only significant predictor of macular microperimetry score was the presence of pseudodrusen (P = 0.004), which showed an inverse correlation with microperimetry score. CONCLUSION: The analysis of predictors of the visual function highlights the importance of pseudodrusen. Pseudodrusen are not only the risk factor of late age-related macular degeneration but also affect visual function. Recognition of this problem is important for low-vision rehabilitation and therapeutic strategies for late age-related macular degeneration.

Colocalization error between the scanning laser ophthalmoscope infrared reflectance and optical coherence tomography images of the heidelberg spectralis.

PURPOSE: To examine the colocalization error between the infrared reflectance (IR) scanning laser ophthalmoscope (SLO) and spectral domain optical coherence tomography (SD-OCT) images of the Heidelberg Spectralis. METHODS: The IR and corresponding horizontal raster SD-OCT images were compared in 10 healthy volunteers examined with 3 Heidelberg Spectralis + OCT instruments. The center points of retinal vessels selected by random uniform sampling in scanning laser ophthalmoscope-IR images were compared with colocalizing points in corresponding SD-OCT images by two masked readers. The error of colocalization was measured in the SD-OCT image. The point positions were recorded using Cartesian coordinates measured in microns. The error of colocalization was evaluated using a fixed-effects generalized least squares regression model with location and instrument as predictor variables. RESULTS: A total of 1,617 points in the IR and SD-OCT images were analyzed, and the mean error of colocalization was 42.2 ± 32.4 µm. The Heidelberg Spectralis instrument used was not a significant predictor of colocalization error (P = 0.15). The colocalization errors were negatively correlated with x-coordinate position (P < 0.001). CONCLUSION: There is a mean colocalization error between the IR and SD-OCT images produced by the Heidelberg Spectralis that is on the scale of many features being evaluated in the fundus. The variability in these measurements means the confidence interval for the exact colocalization is much larger. Because of the magnitude and variability of the error, the colocalization feature of the Heidelberg Spectralis should be used as a rough guide, not an absolute determinant.

The lack of concordance between subretinal drusenoid deposits and large choroidal blood vessels.

PURPOSE: To evaluate the concordance between pseudodrusen as manifested by subretinal drusenoid deposits and large choroidal blood vessels using stereological analysis of spectral-domain optical coherence tomography (SD OCT) images. DESIGN: Retrospective, observational case series. METHODS: The SD OCT images of 31 consecutive patients with the clinical appearance of pseudodrusen from a private-referral retinal clinic were retrospectively reviewed. A grid of 19 evenly spaced vertical lines was randomly superimposed on each SD OCT image using ImageJ to perform systematic uniform random sampling. The main outcome measure was the likelihood of association between subretinal drusenoid deposits and large choroidal vessels. RESULTS: Uniform random systematic sampling of 589 samples found the proportion of geometric probes intersecting subretinal drusenoid deposits to be 0.28, large choroidal vessel 0.65, and both 0.19. This value was nearly identical to the product of the joint probabilities and was within the 95% confidence interval (0.15-0.21) of the point estimate as calculated by the binomial theorem, indicating mutual independence. The subretinal drusenoid deposits were associated with neither large choroidal vessels nor the intervals in between. CONCLUSIONS: Our results demonstrate that there is no concordance between subretinal drusenoid deposits and large choroidal vessels or the stroma in between. As a consequence, hypotheses postulating that subretinal drusenoid deposits are associated with large choroidal vessels or the choroidal stromal spaces should be abandoned. Stereological techniques are powerful methods used in image evaluation in other fields of study and appear to have utility in analyzing OCT findings of the retina and choroid.

Outer retinal corrugations in age-related macular degeneration.

IMPORTANCE: Optical coherence tomography (OCT) abnormalities of age-related macular degeneration (AMD) have not been fully characterized because of the complex morphology and a lack of correlative histologic studies. Expansion of our ability to interpret increasing attributes brings us closer to the goal of in vivo histologic analysis of the eye by OCT. OBJECTIVE: To describe a new outer retinal finding of AMD using spectral-domain (SD) OCT and suggest histopathologic correlates. DESIGN, SETTING, AND PARTICIPANTS: Twenty-five eyes of 16 patients with AMD with severe atrophy due to either choroidal neovascularization (CNV) or geographic atrophy (GA) and 53 donor eyes of 53 patients with late AMD were included. Imaging studies were conducted at a referral retinal practice and histopathology was done at a university research laboratory. EXPOSURES: Findings in the outer retina were evaluated in SD-OCT images in eyes with atrophy of the retinal pigment epithelium (RPE) and compared with histopathologic findings in eyes with GA or CNV that also showed loss of the RPE. MAIN OUTCOMES AND MEASURES: Spectral-domain OCT and histologic characteristics of the outer retina. RESULTS: The mean (SD) age of the 16 patients was 82.7 (7.9) years. Twenty eyes had CNV and 5 eyes had GA. The mean best-corrected visual acuity was 0.800 logMAR (interquartile range, 0.350-1.000 logMAR), a Snellen equivalent of 20/126. A curvilinear hyperreflective density was identified above the Bruch membrane line within the atrophic area in the SD-OCT images. At the internal border, the material was contiguous with the outer portion of the RPE band. Below the material was a relatively hyporeflective space. The material was thrown into folds in cases with atrophy following CNV or was seen as a sheet with numerous bumps in eyes with GA. Review of histopathologic findings of eyes with advanced GA and CNV revealed a rippled layer of basal laminar deposits in an area of RPE atrophy that was located in the same level as the curvilinear line seen in the OCT images. CONCLUSIONS AND RELEVANCE: We have described a new entity, termed outer retinal corrugations, which may correspond to histological findings of basal laminar deposits, extracellular deposits that persist in eyes with late AMD. Observation of this undulating band does not necessarily mean there is exudation or leakage; as a consequence, these patients do not need treatment based on this solitary finding.