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.

Background and Objective: Limbal stem cell deficiency (LSCD) describes the clinical condition when there is dysfunction of the corneal epithelial stem/progenitor cells and the inability to sustain the normal homeostasis of the corneal epithelium. The limbal stem cells are located in a specialized area of the eye called the palisades of Vogt (POV). There have been significant advances in the diagnosis and management of LSCD over the past decade and this review focuses on the pathophysiology of LSCD, its clinical manifestations, diagnosis, and causes.

Methods: Papers regarding LSCD were searched using PubMed to identify the current state of diagnosis and causes of LSCD published through to June 2022. 
Key Content and Findings: LSCD is clinically demonstrated by a whorl-epitheliopathy, loss of the POV, and conjunctivalization of the cornea. The diagnosis of this condition is based on clinical examination and aided by the use of impression cytology, in vivo confocal microscopy, and anterior segment optical coherence tomography (asOCT). There are many causes of LSCD, but those which are most common include chemical injuries, aniridia, contact lens wear, and Stevens-Johnson syndrome (SJS).

Conclusions: While this condition is most commonly encountered by corneal specialists, it is important that other ophthalmologists recognize the possibility of LSCD as it may arise in other co-morbid eye conditions.

Conjunctival flaps have previously proven to be effective in preserving the globe for individuals with severe ocular surface disease. Infectious keratitis, neurotrophic keratitis, nontraumatic corneal melts, descemetoceles, perforations, and corneal burns are all indications for this procedure. The flaps promote nutrition, metabolism, structure, and vascularity, as well as reduce pain, irritation, inflammation, and infection. Furthermore, patients avoid the emotional and psychological repercussions of enucleation or evisceration, while requiring fewer postoperative medications and office visits. Currently, fewer flaps are performed due to the emergence of additional therapeutic techniques, such as serum tears, bandage lenses, corneal grafting, Oxervate, amniotic membrane, and umbilical cord grafting. However, despite newer conservative medical methods, conjunctival flaps have been demonstrated to be useful and advantageous. Moreover, future technologies and approaches for globe preservation and sight restoration after prior conjunctival flaps are anticipated. Herein, we review the history, advantages, and disadvantages of various surgical techniques: Gundersen’s bipedicle flap, partial limbal advancement flap, selective pedunculated conjunctival flap with or without Tenon’s capsule, and Mekonnen’s modified inferior palpebral-bulbar conjunctival flap. The surgical pearls and recommendations offered by the innovators are also reviewed, including restrictions and potential complications. Procedures for visual rehabilitation in selective cases after conjunctival flap are reviewed as well.

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.

Background: To report a new simplified surgical technique to manage small iris coloboma or traumatic iris defect.

Methods: A new surgical technique in which simplified pupilloplasty technique through only a clear corneal paracentesis to manage the iris coloboma or traumatic iris defect within the 120° range was designed. A retrospective revision of the medical records of patients treated with this technique between the years 2013 and 2016 was made. Six eyes of six patients with iris coloboma or traumatic iris defect treated with this new technique were included.

Results: All the operated eyes quickly recovered with central round pupil, negligible complications, inessential symptoms of photophobia and glare, and mild inflammation after a median follow-up time of 22 months (range: 6–34 months).

Conclusions: The simplified pupilloplasty technique presented here could be a good alternative for the management of small iris coloboma or traumatic iris defect.

Background: To settle the fundamentals of a numerical procedure that relates retinal ganglion-cell density and threshold sensitivity in the visual field. The sensitivity of a generated retina and visual pathways to virtual stimuli are simulated, and the conditions required to reproduce glaucoma-type defects both in the optic-nerve head (ONH) and visual fields are explored.

Methods: A definition of selected structural elements of the optic pathways is a requisite to a translation of clinical knowledge to computer programs for visual field exploration. The program is able to generate a database of normalized visual fields. The relationship between the number of extant receptive fields and threshold sensitivity is plotted for background sensitivity and corresponding automated perimetry. A solution in two planes to the 3D distribution of axons in the ONH is proposed. Visual fields with induced damage in the optic disc are comparable in pattern and quantity to glaucomatous records.

Results: The two-level simulation of the ONH facilitates the analysis of optic-cup/retinal defects. We can generate the virtual optic pathways tailored to the age and morphology of the patient’s eye, and it is possible to reproduce glaucomatous damage by “reverse engineering” engineering. The virtual cortical model renders a quantitative relationship between visual defect and neural damage.

Conclusions: A two-level computing of the retina/optic nerve facilitates the analysis of neuroretinal defects and can be incorporated to automatic perimeters to facilitate visual field analysis.