FP1460 : Epidemiology & Features of Inflammatory Retinal Vascular Occlusions: Data of 2 Tertiary Institutions.

FP1460 : Epidemiology & Features of Inflammatory Retinal Vascular Occlusions: Data of 2 Tertiary Institutions.
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By SiteAdmin Uvea June 8, 2017

Dr. Aniruddha Agarwal, A15367, Dr. Vishali Gupta, Dr. Gupta Amod Kumar,Dr. Mangat R Dogra

Authors: Aniruddha Agarwal,1,2 MD, Samendra Karkhur,2 MS, Kanika Aggarwal,2 MS, Alessandro Invernizzi,3,4 MD, Ramandeep Singh, MS,2 Mangat Ram Dogra, MD,2 Vishali Gupta,2 MD, Amod Gupta,2 MS, Diana V. Do,1 MD, Quan Dong Nguyen,1 MD, MSc

1: Ocular Imaging Research and Reading Center (OIRRC), Omaha, USA

2: Advanced Eye Center, Department of Ophthalmology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

3: Ophthalmological Unit, Ca’ Granda Foundation-Ospedale Maggiore Policlinico, Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy

4: Eye Clinic, Department of Biomedical and Clinical Science, Luigi Sacco Hospital, University of Milan, Milan, Italy

Short Title: Inflammatory Retinal Vascular Occlusion

Address for Correspondence:

Quan Dong Nguyen, MD, MSc

Omaha, Nebraska 68198-5540

Phone: (402) 559-4276

Fax: (402) 559-5514

Email: quan.nguyen@unmc.edu

Keywords: Retinal vasculitis; vitreous hemorrhage; occlusive vasculitis; uveitis; laser photocoagulation; immunomodulatory therapy; fluorescein angiography

Word Count: 3336

Abstract

Purpose:

To characterize the epidemiology, clinical course, imaging features, treatment, and outcomes of inflammatory retinal artery and vein occlusion (IRAO and IRVO).

Methods:

In this retrospective study, records of 2438 uveitis patients at two large tertiary-care institutions (USA and India) were reviewed. Out of 2438 patients, 346 patients were diagnosed with retinal vasculitis of which 77 patients (96 eyes) were diagnosed with IRAO and/or IRVO. Patients with occlusive vasculitis (capillary, arteriolar and/or venular) were further analyzed. Diagnostic criteria for occlusive vasculitis included: (1) absence of blood flow in the vessels (arterioles, venules and/or capillaries) identified clinically and confirmed using fluorescein angiography, (2) capillary non-perfusion areas and/or arteriolar-venous anastomosis, (3) intraretinal hemorrhages, cotton-wool spots or vitreous hemorrhage. The outcome measures were final best-corrected visual acuity (BCVA), treatment and complications.

Results:

The mean age of the patients was 32.09±13.51 years. Most common etiologies were tuberculosis and Adamantiades-Behçet’s disease in India and systemic lupus erythematosus in USA. BCVA improved from 0.38±0.30 LogMAR units (20/48 Snellen’s equivalent) at presentation to 0.25±0.30 (20/35 Snellen’s equivalent) at the final visit (p<0.0001). 45.94% eyes required scatter laser photocoagulation for neovascularization. Vitreous hemorrhage was seen in 31.08% eyes. Pars plana vitrectomy was performed in 12.16% eyes. Three eyes developed inoperable retinal detachment. 78.48% patients required therapy with systemic steroids. In addition, 46.75% patients required aggressive therapy with immunomodulators and/or biologics.

Conclusions

: IRAO and/or IRVO are caused by heterogeneous group of uveitides depending upon the geographic location. Eyes with occlusive retinal vasculitis are predisposed to complications requiring aggressive therapy.

Introduction

Retinal vasculitis is a common sight-threatening manifestation of various ocular inflammatory diseases. The Standardization of Uveitis Nomenclature (SUN) has described retinal vasculitis as a conglomerate of changes observed clinically and/or on fluorescein angiography (FA), such as sheathing, retinal hemorrhage, cotton wool spots, leakage or occlusion.1

There are a number of ocular and systemic disorders associated with retinal vasculitis.2 The heterogeneity in the diagnosis has led to limited information on the course and outcome of this condition.3,4 In a subset of patients with retinal vasculitis, occlusion of blood flow through the inflamed retinal vessels may occur. Previous series have reported that certain uveitic entities such as Adamantiades-Behçet’s disease (ABD) and systemic lupus erythematosus (SLE) may present with vascular occlusion.5 It has been proposed that the pathogenesis of vasculitis in these eyes may result in higher levels of ischemia compared to non-occlusive vasculitis.6

Identification of the vascular occlusion among patients with ocular inflammation is of great concern since available literature suggests these eyes may have a poor prognosis.5 Attempts have been made by various authors to identify clinical entities associated with occlusion of retinal blood flow. However, large cohort studies reporting inflammatory retinal artery (or vein) occlusion (IRAO or IRVO) have not been conducted thus far. This nomenclature may help segregate these patients from the umbrella term, retinal vasculitis, in which additional therapy for retinal ischemia may not be required. Separate analysis of patients with IRAO and IRVO may help in identifying the clinical course and morphological features indicative of future prognosis, since the etiologies may vary depending upon the geographic location, ethnicity and genetic composition. This information may help to develop treatment guidelines for such challenging cases.6

In the index study, we report the epidemiological characters, clinical and imaging features, course of the disease, and outcomes in patients with IRAO and IRVO from two countries having diverse causative etiologies.

Materials and Methods

This retrospective cohort study was conducted at two large tertiary-care academic institutes, i.e. Stanley M. Truhlsen Eye Institute, University of Nebraska Medical Center, USA, and the Retina and Uveitis Services at the Advanced Eye Center, Post Graduate Institute of Medical Education and Research, Chandigarh, India. The institutional review board (IRB)/ethics committee approval was obtained for the protocol of the study at both centers. The study adhered to the tenets of the declaration of Helsinki and the rules laid down by Health Insurance Portability and Accountability Act of 1996 (HIPAA).

Charts of consecutive patients attending the uveitis clinic from January 2012 to July 2015 at both centers were retrospectively reviewed. Patients diagnosed with retinal vasculitis, either at presentation, or during the course of their follow-up, were identified. Among them, patients demonstrating features of retinal vascular occlusion were included in the study. The diagnosis of retinal vascular occlusion was defined in this study as: (1) absence of blood flow in the vessels (arterioles, venules and/or capillaries) identified clinically and confirmed using FA, (2) presence of capillary non-perfusion areas (CNP) and/or arteriolar-venous anastomosis, (3) presence of intraretinal hemorrhages, cotton-wool spots or vitreous hemorrhage (along with criteria 1 and/or 2). No minimum follow-up time was needed to enroll patients in the study and all the available clinic visits were reviewed. Patients diagnosed with other confounding retinal vascular diseases, such as concomitant diabetic retinopathy, hypertensive retinopathy, hypercoagulable states, and others were excluded from the study.

All the patients underwent complete ocular and systemic examination along with tailored laboratory investigations. Clinical data including history, demographic features such as age, gender and race, laterality of uveitis, best-corrected visual acuity (BCVA) (measured using Snellen’s chart and converted to LogMAR units for analysis) at presentation and final follow-up visit, and intraocular pressure (IOP) obtained using Goldman applanation tonometry was noted for each patient. Ocular examination details such as anterior chamber and vitreous inflammation, macular edema, neovascularization, and presence of chorioretinal lesions were retrieved using standard grading forms. A systematic review of all the available laboratory reports, including blood, serum and ocular fluid analysis was performed. Details of the treatment including systemic and local ocular therapies were noted for all the patients included in the study.

Morphologic characterization of retinal vascular occlusion during active and inactive stages of uveitis was performed by analyzing the digital fundus photographs and FA images (Zeiss FF450, Carl Zeiss Meditec, Jena, Germany, and Optos P200Tx, Optos Inc., Scotland, UK) of the patients included in the study. Patients with both gradable fundus photographs and FAs were included in the study. Ultra-wide field imaging was available since January 2013 at both centers. Complications occurring during the course of follow-up, if any, were noted. Total duration of follow-up and final visual outcomes was obtained.

All the data were captured using a standardized matrix at both sites. The SUN guidelines for reporting clinical data was employed.1 The statistical analysis was performed using GraphPad Prism 6 ® (GraphPad Software, Inc., La Jolla, CA, USA). The data was entered in an excel sheet (Microsoft Excel 2007 ®, Redmond, WA, USA) and coded for analysis. Descriptive analysis was used to assess the characteristics of IRAO and IRVO in the study population. Quantitative data was analyzed using non-parametric tests (Wilcoxon Signed Rank test). The value of significance was set at 5% and a p value of < 0.05 was considered to be statistically significant. Since a number of patients with occlusive vasculitis were diagnosed with tuberculosis (TB), patients were divided into two groups: TB and non-TB. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and positive likelihood ratio (LR) (with 95% confidence intervals) were calculated for TB and non-TB patients. All the tests were performed using 2-tailed analysis.

Results

A thorough review of clinical records of 2438 patients diagnosed with uveitis in the past three years at both institutions was performed. 346 patients were diagnosed with retinal vasculitis, either at presentation, or during the course of their follow-up (14.19%). Out of these 346 patients, retinal vascular occlusion (as defined in the inclusion criteria) was noted in 77 patients (96 eyes) (22.25%). The mean duration of follow-up was 13.65 ± 21.05 months. 15 patients had a follow-up of less than 6 months from starting therapy. Baseline demographic details of the patients are listed in Table 1.

The mean age of the patients was 32.34 ± 14.01 years. 60 patients were males (77.92%). The disease was bilateral in 19 patients (24.68%). Posterior segment of fellow eyes could not be assessed due to occlusio pupillae and complicated cataract in 2 eyes and inoperable retinal detachment in 1 eye. Of the 77 patients, 57 were Asian Indians and 20 were Americans (Table 1). Right eye was involved in 55% cases. The mean duration of the disease was 9.63 ± 12.24 months (range: 3 weeks to 5 years).

The most common presenting symptom was decreased visual acuity/blurring of vision in eyes (81.25%). The mean BCVA at the initial visit was 0.38 ± 0.32 LogMAR units (20/48 Snellen’s equivalent). The BCVA at the final visit was 0.25 ± 0.30 LogMAR units (20/35 Snellen’s equivalent) (p < 0.0001) (≥20/40 in 66 eyes, 68.75%; 20/40-20/100 in 28 eyes, 29.17%; >20/100 in 1 eye). The clinical characteristics of the patients are listed in Table 2.

Overall, the most common etiology for retinal vascular occlusion in our study was intraocular TB (33 patients, 41 eyes). Other etiologies included ABD (6 patients), SLE (5 patients), and idiopathic (18 patients), among others. The most common etiology for retinal vascular occlusion was TB in the Asian Indian population (Figure 1) and SLE (Figure 2) in the American cohort. Among patients with TB, the tuberculin skin test (Mantoux test) was positive (≥10 mm induration at 48-72 hours) in 24 patients (24/33; 72.73%) (Possible intraocular TB). Among the remaining 9 patients: QuantiFERON TB Gold was positive in 7 patients (probable intraocular TB) and 2 eyes tested positive for TB using the multi-targeted polymerase chain reaction (PCR) assay (Gene Xpert MTB/RIF assay, Cepheid, Sunnyvale, CA, USA) (confirmed intraocular TB).7 Patients with SLE were already diagnosed prior to ophthalmic complaints by the internists on the basis of clinical and laboratory data (American College of Rheumatology guidelines).8 One patient was diagnosed with discoid lupus erythematosus on the basis of characteristic skin lesions. Three patients were positive for anti-phospholipid antibody (APLA). Diagnosis of ABD was made based on the International Team for the Revision of the International Criteria for Behçet’s disease (ITR-ICBD), 2014 scoring system (Figure 3).9 Out of the 6 patients diagnosed with ABD, 1 was Caucasian. Oral aphthous ulcers were present in 4 patients and 1 patient had associated neurological disease. Three patients were diagnosed with viral uveitis (2 with herpes simplex virus and 1 with varicella zoster virus) based on serum IgM values. Two patients were diagnosed with Susac’s syndrome based on neuroimaging and audiology evaluation (Figure 4). Other diagnoses of retinal vascular occlusion in our study population included syphilis, masquerades such as leukemia (Figure 5), and sarcoidosis, among others (Table 3).

Active retinal vasculitis (associated with vascular sheathing and leakage on FA) was noted in 84 eyes (87.5%) at the time of presentation. Clinical signs associated with inflammation included: anterior chamber inflammation (27 eyes), vitreous cells (40 eyes) and snow balls (7 eyes) (Table 2). The most common type of iris nodules was Busacca’s nodules (2 eyes – diagnosed with TB). Iris atrophy was observed in both patients diagnosed with herpetic viral uveitis. Choroidal granulomas were seen in 1 eye with TB. In one patient with herpes simplex virus-associated uveitis, a patch of outer retinal necrosis was seen near the superior arcade (Figure 6). Retinal vasculitis presented as frosted branch angiitis in 4 eyes (Table 4) (Figure 7).

Retinal vascular occlusion affected the arterioles alone in 11 eyes. Common causes of IRAO included ABD and viral uveitis. IRVO was diagnosed in 66 eyes. IRVO mimicked non-inflammatory central retinal vein occlusion (CRVO) in 6 eyes (4 diagnosed with TB). 19 patients presented with both arteriolar and venular occlusions. SLE and ABD were the most common causes of combined IRAO and IRVO. The most common location of retinal vascular occlusion was in the superotemporal quadrant. IRAOs showed a predilection to involve the macular area. Table 5 lists the characteristics of retinal vascular occlusions and the prevalence of complications among patients with IRAO, IRVO and combined occlusions.

Details of the systemic and ocular treatment of patients with occlusive vasculitis are provided in Table 6. Vitrectomy was performed for various indications including diagnostic (5 eyes) and therapeutic (4 eyes) (Figure 8). 75.32% patients received therapy with oral steroids. Therapy with steroid-sparing long-term systemic immunosuppressants or biological agents was required in 36 patients (46.75%). 71.43% patients required pan-retinal/scatter laser therapy due to complications of retinal or optic disc neovascularization. 38.96% patients received intravitreal anti-vascular endothelial growth factor therapy for macular edema and retinal neovascularization. One eye that developed neovascular glaucoma required diode laser cyclophotocoagulation for IOP and pain control.

Since intraocular TB was the overall most common etiology of occlusive vasculitis (presumed and confirmed cases), clinical features of TB were compared to the non-TB cohort. The constellation of perivascular choroiditis and capillary non-perfusion were highly suggestive of TB with a PPV of 94.58 and NPV of 95.09 (Table 7). Findings of perivascular choroiditis and retinal neovascularization and/or vitreous hemorrhage were more likely to occur among patients with TB compared to the non-TB group with likelihood ratios varying from 2.7 to 17.45 (Table 7).

Discussion

Retinal vasculitis has been reported to be associated with a wide range of uveitic entities resulting in significant visual morbidity and potential for causing blinding complications in various population-based studies.5,10-14 While macular edema may be responsible for visual morbidity in a significant number of patients with retinal vasculitis,5,15 of serious concern are cases that present with retinal ischemia due to inflammatory vascular occlusion.6 While various authors have previously recognized the importance of diagnosing and treating occlusive retinal vasculitis,6,15,16 prospective natural history series have not been conducted thus far.

In a subset analysis of patients with retinal vasculitis from Oregon, USA, the prevalence of occlusive retinal vasculitis was observed to be rare (5.9% eyes).5 However, the authors excluded a number of patients with ocular conditions such as infections from the original cohort.3 Thus, this study may have underestimated the true prevalence of occlusive vasculitis. In a retrospective study from Eastern India, capillary non-perfusion was observed in 40% cases with retinal vasculitis.14 The results of our study indicate that features of retinal vascular occlusion may be observed in over 22% cases of retinal vasculitis. Thus, it is imperative for ophthalmologists to carefully assess the clinical and imaging features of patients presenting with retinal vasculitis for subtle and early signs of vascular occlusion, such as capillary non-perfusion areas and cotton-wool spots. The most commonly affected population is young-to-middle aged adults (mean age of 32.34 years with male:female ratio of 3.5:1). A significant number of patients (58/77) in our cohort were Asian Indians from TB-endemic areas. Unilateral occlusive disease was found to be more common (24.68%), though majority of the fellow eyes had evidence of non-ischemic retinal vasculitis (Table 1).

Analysis of clinical features revealed that a significant number of patients (34.37%) had initial BCVA ≤ 20/100 (Table 2). On the other hand, clinical features of inflammation, such as anterior chamber cells and flare, and vitritis were present in less than 50% of patients in the cohort. Similar to a previous report,4 systemic vasculitis was rarely associated with IRVO and IRAO and was observed only in 3 patients with SLE. An important observation of the study was that more than 27% eyes had extensive, large vessel occlusion resulting in hemorrhages and loss of retinal architecture, which may mask the fundoscopic features of the underlying uveitic entity (Table 4). Occasionally, the occlusive disease manifested as CRVO or CRAO, misleading the clinician (7 eyes), resulting in a diagnostic and management challenge.

The major highlight of our study results is that unlike non-ischemic retinal vasculitis, IRAO and IRVOs were associated with fairly limited range of uveitic entities depending upon the geographic location (Table 3). In endemic countries such as India, intraocular TB appears to be the major cause of occlusive vasculitis, whereas in developed countries, SLE may be the most common cause. ABD, a common cause of retinal vasculitis,11 was less common etiology in our cohort possibly due to its lower incidence in the Midwestern US.17 Intraocular TB has been reported to cause retinal periphlebitis and neovascularization in 35% and 29% of the eyes, respectively, in previous series.18,19 Subsequently, using genome detection techniques, it was recognized that Eales’ disease, an inflammatory veno-occlusive disease typically found in the Indian subcontinent,20 largely represented intraocular TB, and is thus referred to as presumed tubercular retinal vasculitis.21 As observed in our cohort, patients with TB-related retinal vasculitis are young males with retinal periphlebitis, and high incidence of capillary-non perfusion, perivascular choroiditis, and retinal neovascularization with or without vitreous hemorrhage (Figure 1 and 8). The constellation of perivascular choroiditis and capillary non-perfusion has a high PPV of 94.58 and NPV of 95.09, strongly suggestive of TB-related uveitis (Table 7). Thus, one must suspect intraocular TB, even if the patient with occlusive vasculitis does not have clinical features such as broad-based posterior synechiae, serpiginous-like choroiditis, or choroidal tubercles, which typify the disease.22

In a previous series, 21% patients with ocular ABD presented with occlusive retinal vasculitis.23 ABD can be recognized as the etiology among patients with occlusive retinal vasculitis in the presence of extraocular systemic features such as aphthous ulcers, and ocular features such as retinitis and venular inflammation, as seen in our cohort (Figure 3). While both venular and arteriolar occlusion may occur in patients with ABD, our results indicate that the presence of predominant arteriolar occlusion makes systemic vasculitides such as SLE, and entities such as Susac’s syndrome, very likely (Figure 4, Table 5). SLE has been recognized as an important cause of occlusive vasculitis in literature, involving both arterioles and venules.24,25 Ischemic vasculitis among patients with SLE may be very severe leading to extensive neovascularization as observed in our series (Figure 2).26-29 It is imperative to note that SLE-related retinal vascular manifestations may be secondary to fibrinoid necrosis, thrombus formation and immune complex deposition leading to occlusive disease with only mild inflammation. Thus, it may truly represent mainly vasculopathy rather than vasculitis.30 We have included SLE-related vasculitis in our manuscript because previous studies have considered this entity to be secondary retinal vasculitis associated with systemic disease in which the inflammation is not primarily directed towards the vessels.31,32 In our study, IRAO was also associated with viral uveitis (Figure 6), as reported in literature.33-35

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) is often associated with retinal vascular occlusion.36-38 We identified 2 cases of IRVAN at the two centers during the study period with typical knot-like aneurysms at arteriolar bifurcations. While occlusive retinal vasculitis may be rarely associated with etiologies such as Sweet syndrome,39 relapsing polychondritis,40 Kikuchi-Fujimoto disease,41  cytomegalovirus infection,42 H1N1 influenza infection,43 syphilis44 and cat-scratch disease,45 we did not identify such cases in our cohort. IRVO has been also associated with West Nile Virus (WNV) fever in literature.46-48 Two patients were diagnosed with WNV-associated retinopathy in our cohort. However, both patients did not show evidence of IRVO and hence, were not included in the analysis. Therefore, occlusive retinal vasculitis may be caused by rare entities beyond those observed in our study.

High rates of complications were observed among the patients with IRVO and IRAO, similar to previous reports.5,6,15,29 The prevalence of retinal neovascularization and macular leakage was high in our cohort of occlusive vasculitis (38.54% and 54.17% eyes, respectively) (Table 4). Similarly, the sequelae of ischemic changes resulted in development of fibrovascular proliferation, epiretinal membranes and tractional retinal detachment in a significant number of eyes. Seven eyes developed secondary glaucoma during the follow-up (Table 5).

Patients with severe retinal ischemia due to vascular occlusion often require aggressive therapy in order to prevent debilitating visual complications. More than 75% patients in our study cohort required long-term therapy with oral steroids. Six patients also required pulse therapy with intravenous methylprednisolone. Systemic immunomodulatory agents as well as biological therapy were required in approximately half of the patients (46.75%). Anti-VEGF agents were employed for complications such as retinal neovascularization in more than one-third patients in the study cohort (Table 6).

The limitations of our study include the retrospective nature of the analysis. The visual acuity analysis has been performed using the values reported at the final visit. The patients may, however, continue to gain or lose vision leading to a potential bias. As mentioned earlier, our cohort does not include all potential etiologies of occlusive retinal vasculitis, since rare entities may present with vascular occlusion as an unusual manifestation. Given the wide spectrum of the clinical and pathological manifestations of retinal vasculitis, and lack of standardized diagnostic criteria,2 it is likely that some cases of IRAO and/or IRVO may have been missed. In addition, some patients in our cohort were imaged using conventional field fundus cameras (15/77 patients diagnosed with occlusive vasculitis were imaged using conventional camera). Thus, it is likely that patients with anterior peripheral vascular occlusion that may be otherwise diagnosed using ultra-wide field fundus cameras were missed on conventional camera. Development of vasculitis over time was not assessed, i.e. limiting the potential to identify the rate at which vasculitis was diagnosed in our cohort of patients.

In conclusion, to our knowledge, this is the first large epidemiological study of patients with occlusive retinal vasculitis in ethnically and geographically diverse group. Among patients presenting with capillary non-perfusion, and neovascularization/vitreous hemorrhage, especially in an endemic country, intraocular TB must be strongly suspected. The diagnosis may often require techniques such as vitrectomy, PCR, and specialized laboratory assays. Early identification of features of retinal vascular occlusion, such as cotton-wool spots and retinal hemorrhages, is of major clinical relevance. Such findings must alert the treating ophthalmologist to order tailored investigations and initiate early, aggressive therapy which may help to prevent serious complications such as vitreous hemorrhage and tractional retinal detachment. Often, identification of limited clinical features may aid the clinician in arriving at the diagnosis and determining the etiology.

A.Funding/Support

This work was partly supported by a grant from Department of Science and Technology, India for the development of Centre of Excellence at the Advanced Eye Centre, PGIMER Chandigarh.

B.Financial Disclosures

The authors have no financial disclosure/proprietary interest. No conflicting relationship exists for any author.

The authors report no conflicts of interest. The authors alone are responsible for the content and preparation of this manuscript.

C.Other Acknowledgements

None

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Figure Legends

Figure 1: Classical presentation of occlusive retinal vasculitis in a patient with intraocular tuberculosis. Color fundus photography (only right eye shown) demonstrates capillary washout in the temporal periphery along with retinal hemorrhages. There is exudation at the macula (A) and significant sheathing of large retinal vessels (B). Fluorescein angiography reveals large capillary non-perfusion areas and blocked fluorescence due to the retinal hemorrhages. There is a neovascular frond at the superotemporal arcade (C) and significant peripheral retinal ischemia (D).

Figure 2: Multimodal imaging of a patient with discoid lupus erythematosus (DLE). Color fundus photography and fluorescein angiogram of the right eyes (A-B) at initial presentation reveals multiple retinal hemorrhages in all four quadrants along with capillary washout in the posterior pole and periphery (A). On fluorescein angiography (FA), there is active vascular leakage and filling of only major vessels. The patient developed significant retinal neovascularization and underwent pan-retinal photocoagulation which resulted in decreased hemorrhages (C). However, the patient continued to have leakage from fragile new vessels on FA (D) requiring intravitreal injections of ranibizumab. The skin lesions are shown in (E). In addition, the patient had macular edema with large cystoid spaces (yellow asterisks) in both eyes as seen on optical coherence tomography (F) (only right eye shown).

Figure 3: Occlusive retinal vasculitis in a patient with Adamantiades-Behçet’s disease. On color fundus photography, sheathing of inferotemporal vessels is observed along with a patch of retinitis involving the inferior macula (marked by a yellow dotted rectangle). Fluorescein angiography (B-D) shows presence of retinal venous occlusion along with peripheral ischemic changes (C) and neovascularization (D).

Figure 4: Imaging of a patient with Susac’s syndrome. There is presence of active retinal vasculitis with sheathing involving the superotemporal retinal arteriole (white arrows) (A). In addition, an area of grayish white retinal opacification suggestive of retinal nerve fiber layer (RNFL) edema is observed. Fluorescein angiography (FA) confirms the site of vascular occlusion (B). The infrared image (C) captured using spectral-domain optical coherence tomography is able to demarcate the area of RNFL edema more clearly (white arrowheads) (D).

Figure 5: Color fundus photography of both the eyes of a patient with acute lymphoid leukemia. There are multiple retinal hemorrhages at different levels in both the eyes (A and B) in all four quadrants. The right eye (A) has sub-internal limiting membrane bleed along with sheathing of inferior vessels. Focal areas of retinal vascular sheathing are observed inferiorly (yellow arrows) (A and B).

Figure 6: Imaging of a patient with herpes simplex virus-related acute retinal necrosis and occlusive vasculitis. The color fundus photography of the right eye (A) shows an area of acute retinal necrosis near the superior arcade (black arrowheads) with peripapillary hemorrhage. Retinal vessels appear attenuated with perivascular sheathing (white arrows). There are retinal nerve fiber layer (RNFL) infarcts near the inferior arcade. The left eye (B) shows multiple areas of focal periphlebitis (white arrows) and RNFL infarcts. Fluorescein angiography (FA) (C and D) in the peak phase (approximately 1.5 minutes) shows bilateral occlusive vasculitis and vascular leakage (left eye more than the right). There is optic nerve head hyperfluorescence in the FA of the left eye (D) suggestive of optic neuritis.

Figure 7: Tuberculosis-related retinal vasculitis presenting as bilateral frosted branch angiitis (only left eye shown). Color fundus photography using ultra-wide field imaging shows severe vascular sheathing involving multiple vessels 360°. There is dense macular exudation (A). Fluorescein angiography (B) reveals leakage at the optic disc, near-complete capillary washout in the retina beyond the equator, and peripheral vascular telangiectasia. There is subtle leakage of the dye involving the macula and a small neovascular frond in the superotemporal retina.

Figure 8: Tractional retinal detachment and vitreous hemorrhage in a patient with retinal vascular occlusion due to intraocular tuberculosis (A). The view of the fundus is limited on fluorescein angiography (FA) (B) due to the presence of vitreous hemorrhage. The patient underwent pars plana vitrectomy and endolaser to treat the areas of retinal ischemia. Four weeks after surgery, color fundus photography (C) reveals attached retina, pale optic nerve head, and attenuated vessels in the nasal and temporal periphery. On FA (D), the areas of capillary non-perfusion are clearly demonstrated.

FP1482 : Role of Computerised Tomography (Ct) in Finding the Etiology of Posterior Uveitis
FP1581 : Case Series of Ocular Toxocariasis (Ot) in a Tertiary Eye Care Centre in South India

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