The purpose of this review is to provide a comprehensive and updated overview of the clinical features, imaging modalities, differential diagnosis, diagnostic criteria, and treatment options for Vogt-Koyanagi-Harada (VKH) syndrome, a rare progressive inflammatory condition characterized by bilateral granulomatous panuveitis and systemic manifestations. While the clinical features and disease course of VKH syndrome are well-characterized in the literature, its diagnosis is challenging due to a broad differential that include infectious and noninfectious causes of uveitis and rare inflammatory conditions, as well as a lack of a single diagnostic finding on exam, laboratory testing, or imaging. The evolution of the diagnostic criteria for VKH syndrome reflects the growing understanding of the disease by the ophthalmic community and advancement of imaging technology. Findings on enhanced depth imaging (EDI) optical coherence tomography (OCT) and indocyanine green angiography (ICGA) help detect subtle inflammation of the choroid and were incorporated into new diagnostic criteria developed in the last few years. There is limited research on the treatment for acute VKH, but results of studies to date support the early initiation of immunomodulatory therapy (IMT) due to a high recurrence rate and progression to chronic disease in patients treated with monotherapy with high-dose systemic corticosteroids. This review will provide an in-depth summary of recent literature on advanced imaging modality and IMT to guide clinicians in their management of patients with VKH syndrome.

The prevalence of diabetic retinopathy (DR) continues to increase in pregnant females; these individuals are also at a higher risk of disease progression. The lack of evidence regarding the safety and efficacy of current treatment options in pregnancy makes disease management particularly challenging.All pregnant women with diabetes should have a prenatal DR screening, as well as receive counseling regarding the progression and management of DR during pregnancy. Optimal blood glucose and blood pressure control should be encouraged. For patients with proliferative diabetic retinopathy (PDR) in the absence of visually significant diabetic macular edema (DME), panretinal photocoagulation (PRP) remains a safe and effective treatment option. Visually significant DME can be treated with focal laser if areas of focal leakage are identified in the macula on fluorescein angiogram, intravitreal steroids or anti-vascular endothelial growth factor (VEGF) agents, The theoretical risk of anti-VEGF agents to the fetus should be considered and the patients should be extensively counselled regarding the risks and benefits of initiating anti-VEGF therapy before initiating treatment. When the decision is made to treat with anti-VEGF agents, Ranibizumab should be the agent of choice. In conclusion, ophthalmologists should make treatment decisions in pregnant patients with DR on a case-by-case basis taking into consideration disease severity, risk of permanent threat to vision, gestational age, and patient preferences.

Abstract: Inherited retinal diseases (IRD) are a leading cause of blindness in the working age population. The advances in ocular genetics, retinal imaging and molecular biology, have conspired to create the ideal environment for establishing treatments for IRD, with the first approved gene therapy and the commencement of multiple therapy trials. The scope of this review is to familiarize clinicians and scientists with the current landscape of retinal imaging in IRD. Herein we present in a comprehensive and concise manner the imaging findings of: (I) macular dystrophies (MD) [Stargardt disease (ABCA4), X-linked retinoschisis (RS1), Best disease (BEST1), pattern dystrophy (PRPH2), Sorsby fundus dystrophy (TIMP3), and autosomal dominant drusen (EFEMP1)], (II) cone and cone-rod dystrophies (GUCA1A, PRPH2, ABCA4 and RPGR), (III) cone dysfunction syndromes [achromatopsia (CNGA3, CNGB3, PDE6C, PDE6H, GNAT2, ATF6], blue-cone monochromatism (OPN1LW/OPN1MW array), oligocone trichromacy, bradyopsia (RGS9/R9AP) and Bornholm eye disease (OPN1LW/OPN1MW), (IV) Leber congenital amaurosis (GUCY2D, CEP290, CRB1, RDH12, RPE65, TULP1, AIPL1 and NMNAT1), (V) rod-cone dystrophies [retinitis pigmentosa, enhanced S-Cone syndrome (NR2E3), Bietti crystalline corneoretinal dystrophy (CYP4V2)], (VI) rod dysfunction syndromes (congenital stationary night blindness, fundus albipunctatus (RDH5), Oguchi disease (SAG, GRK1), and (VII) chorioretinal dystrophies [choroideremia (CHM), gyrate atrophy (OAT)].

Abstract: Optical coherence tomography angiography (OCTA) is a fast, non-invasive imaging modality that provides detailed information on retinal and choroidal vascular flow and macular structure. OCTA offers an accurate three-dimensional view of the individual retinal vascular plexuses and the choriocapillaris which facilitates the detection of the microvascular abnormalities in a variety of macular diseases. The perfusion indices (vessel density and flow index) are valuable parameters evaluated by OCTA that allow a quantitative interpretation of changes in the retinal vasculature that can reflect the severity of disease. Crystalline retinopathy encompasses a group of conditions whose distinctive feature is the presence of retinal crystals often located in the posterior pole. Select crystalline retinopathies also demonstrate retinal vascular abnormalities as well. Considering that the OCTA is a novel imaging modality and crystalline retinopathies are relatively rare conditions, there are currently few reports of OCTA findings associated with crystalline retinopathy. The advent of OCTA allows visualization of vascular and structural changes in crystalline retinopathies that are unique and cannot be appreciated on other imaging modalities, including fluorescein angiography (FA). This article reviews novel OCTA findings which provide new insights in the pathogenesis of crystalline retinopathies, including Bietti crystalline retinopathy, talc retinopathy, macular telangiectasia type 2, tamoxifen retinopathy, and Sj?gren-Larsson Syndrome maculopathy.

Abstract: Dramatic advances in retinal imaging technology over the last two decades have significantly improved our understanding of the natural history and pathophysiology of non-neovascular age-related macular degeneration (AMD). Currently, aside from micronutrient supplements, there are no proven treatments for non-neovascular or dry AMD. Recently, a number of pharmacological agents have been evaluated or are under evaluation for treatment of patients with end-stage dry AMD manifesting as geographic atrophy (GA). It may preferable, however, to intervene earlier in the disease before the development of irreversible loss of visual function. Earlier intervention would require a more precise understanding of biomarkers which may increase the risk of progression from early and intermediate stages to the late stage of the disease. The development of optical coherence tomography angiography (OCTA) has allowed the layers of the retinal microcirculation and choriocapillaris (CC) to be visualized and quantified. Flow deficits in the CC have been observed to increase with age, particularly centrally, and these flow deficits appear to worsen with development and progression of AMD. As such, OCTA-based CC assessment appears to be a valuable new biomarker in our assessment and risk-stratification of AMD. Alterations in the CC may also provide new insights into the pathophysiology of the disease. Enhancement of choriocapillaris function may also prove to be a target of future therapeutic strategies or as a biomarker to monitor the effectiveness of therapy. As such, CC imaging may be anticipated to be an integral tool in the management of dry AMD.

Background: To evaluate a fully automated vascular density (VD), skeletal density (SD) and fractal dimension (FD) method for the longitudinal analysis of retinal vein occlusion (RVO) eyes using projection-resolved optical coherence tomography angiography (OCTA) images and to evaluate the association between these quantitative variables and the visual prognosis in RVO eyes.

Methods: Retrospective longitudinal observational case series. Patients presenting with RVO to Creteil University Eye Clinic between October 2014 and December 2018 and healthy controls were retrospectively evaluated. Group 1 consisted of central RVO (CRVO) eyes, group 2 consisted of eyes with branch RVO (BRVO) and group 3 of healthy control eyes. OCTA acquisitions (AngioVue RTVue XR Avanti, Optovue, Inc., Freemont, CA) were performed at baseline and last follow up visit. VD, SD, and FD analysis were computed on OCTA superficial and deep vascular complex (SVC, DVC) images at baseline and final follow up using an automated algorithm. Logistic regression was performed to find if and which variable (VD, SD, FD) was predictive for the visual outcome.

Results: Forty-one eyes, of which 21 consecutive eyes of 20 RVO patients (13 CRVO in group 1, 8 BRVO in group 2), and 20 eyes of 20 healthy controls were included. At the level of SVC, VD and FD were significantly lower in RVO eyes compared to controls (P<0.0001 and P=0.0008 respectively). Best-corrected visual acuity (BCVA) at last follow-up visit was associated with baseline VD (P=0.013), FD (P=0.016), and SD (P=0.01) at the level of the SVC, as well as with baseline FD at the DVC level (P=0.046).

Conclusions: Baseline VD, SD, and FD are associated with the visual outcome in RVO eyes. These parameters seem valuable biomarkers and may help improve the evaluation and management of RVO patients.