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.

Background: A variety of experimental animal models are used in basic ophthalmological research to elucidate physiological mechanisms of vision and disease pathogenesis. The choice of animal model is based on the measurability of specific parameters or structures, the applicability of clinical measurement technologies, and the similarity to human eye function. Studies of eye pathology usually compare optical parameters between a healthy and altered state, so accurate baseline assessments are critical, but few reports have comprehensively examined the normal anatomical structures and physiological functions in these models.

Methods: Three cynomolgus monkeys, six New Zealand rabbits, ten Sprague Dawley (SD) rats, and BALB/c mice were examined by fundus photography (FP), fundus fluorescein angiography (FFA), and optical coherence tomography (OCT).

Results: Most retinal structures of cynomolgus monkey were anatomically similar to the corresponding human structures as revealed by FP, FFA, and OCT. New Zealand rabbits have large eyeballs, but they have large optic disc and myelinated retinal nerve fibers in their retinas, and the growth pattern of retinal vessels were also different to the human retinas. Unlike monkeys and rabbits, the retinal vessels of SD rats and BALB/c mice were widely distributed and clear. The OCT performance of them were similar with human beings except the macular.

Conclusions: Monkey is a good model to study changes in retinal structure associated with fundus disease, rabbits are not suitable for studies on retinal vessel diseases and optic nerve diseases, and rats and mice are good models for retinal vascular diseases. These measures will help guide the choice of model and measurement technology and reduce the number of experimental animals required.

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.

Background and Objective: Nearly 30 years have passed since limbal stem cell deficiency (LSCD) was first identified by pioneers and given clinical attention. LSCD remains a difficult disease to treat. It can potentially lead to blinding. At present, understanding of limbal stem cells (LSCs) has deepened and various treatment options for LSCD have been devised. The objective of this review is to summarize basic knowledge of LSCD and current treatment strategies.

Methods: PubMed search was performed to find studies published in English on LSCs and LSCD including original reports and reviews. Literatures published from 1989 to 2022 were reviewed.
Key Content and Findings: LSCs are enigmatic stem cells for which no specific marker has been discovered yet. Although LSCD is not difficult to diagnose, it is still challenging to treat. An important advancement in the treatment of LSCD is the provision of guidelines for selecting systematic surgical treatment according to the patient’s condition. It is also encouraging that stem cell technologies are being actively investigated for their potential usefulness in the treatment of LSCD.

Conclusions: Although various treatment options for LSCD have been developed, it should be kept in mind that the best chance of treatment for LSCD is in the early stage of the disease. Every effort should be made to preserve as many LSCs as possible in the early treatment of LSCD.

Abstract: Four challenging and unusual retinal cases: (I) 11-year follow-up for retinal hemangioblastoma with von Hippel-Lindau (VHL) disease; (II) treatment for central serous chorioretinopathy (CSC)—observation, half does photodynamic therapy (PDT) or micropulse laser photocoagulation; (III) diagnosis and treatment for a child with optic nerve defect; (IV) the optional treatment for retinal detachment (RD) with iridolenticular choroidal coloboma, were presented and discussed by three international retinal specialists at a retinal clinical round in Fundus Diseases Center of Zhongshan Ophthalmic Center (ZOC). The discussion helps us a better understanding of the pathogenesis and managements of these four retinal diseases and their association with systemic conditions.

Abstract: Primary vitreoretinal lymphoma (PVRL), as a subset of primary central nervous system lymphoma (PCNSL), is a rare and fatal ocular malignancy. Most PVRL masquerades as chronic posterior uveitis, which makes the clinical diagnosis challenging. Vitreous cells, subretinal lesions and imaging techniques are essential for clinical diagnosis. Importantly, cytopathology/histopathology identification of malignant cells is the gold standard for the diagnosis of PVRL. In addition, molecular detection of immunoglobulin heavy chain (IgH) or T cell receptor (TCR) gene rearrangements, immunophenotyping for cell markers, and cytokine analysis of interleukine-10 elevation are often used as adjunct procedures. Current management of PVRL involves local radiation, intravitreal chemotherapy (methotrexate and rituximab), with or without systemic chemotherapy depending on the involvement of non-ocular tissues. In cases with concomitant PCNSL, systemic high-dose methotrexate/rituximab based therapy in conjunction with local therapy, whole brain radiotherapy and/or autologous stem cell transplantation is considered. Although PVRL normally responds well to initial treatment, high rates of relapse and CNS involvement usually lead to poor prognosis and limited survival. A professional team of medical experts in ophthalmologists, ocular pathologists, neuro-oncologists and hemato-oncologists is essential for optimizing patient management.

Abstract: We reviewed randomized controlled trials associated with the intravitreal use of aflibercept for this article. These studies proved that aflibercept is an effective anti-vascular endothelial growth factor agent for the treatment of neovascular age-related macular degeneration (nAMD), myopic choroidal neovascularization (mCNV), diabetic macular edema (DME), and macular edema associated with retinal vein occlusion. The incidence of severe ocular or systemic complications after intravitreal administration of aflibercept was low.