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: Necrotising fasciitis (NF) is a rare but severe necrotising infection of the subcutaneous tissues. We report a case of periocular NF associated with a concurrent COVID-19 infection and explore potential mechanisms of pathogenesis of COVID-19 infection and necrotising superinfections.
Case Description: A 33-year-old previously healthy female presented with right-sided progressive periocular swelling, erythema, pain and fever, two days after sustaining a laceration to the right superolateral brow from a clenched fist. She had a concurrent COVID-19 infection, detected on nasopharyngeal polymerase chain reaction swab thirteen days prior to presentation and again at presentation. She did not have an oxygen requirement. There was a large bulbous collection of the right upper lid with fluctuance and overlying erythema, and a communicating sinus drained frank pus from the superolateral brow. Pre-operative T2-weighted MRI demonstrated fascial hyperintensity involving the pre-septal tissues and extending to the anterior temporal fossa. She was commenced on intravenous meropenem, clindamycin and vancomycin, and underwent early surgical debridement. Initial debridement demonstrated right upper lid necrosis involving the dermal and pre-septal layers, including the orbicularis, but sparing the tarsus. Streptococcus pyogenes was isolated, and she was continued on a prolonged course of intravenous antibiotic. Periocular defects were repaired with a right-sided brow adipo-fascial flap based on the supratrochlear artery, browpexy and dual full thickness skin grafts on the right upper lid and flap.
Conclusions: NF is an acute fulminant infection rarely affecting the periocular tissues. This represents a unique case of periocular NF associated with a concurrent COVID-19 infection.
Background: Pterygium is a sun-related ocular surface disease secondary to ultraviolet (UV) radiation exposure. Outdoor occupational UV exposure is known to occur secondary to sun exposure. We present a unique case of pterygium associated with indoor occupational light-emitting diode (LED) exposure not previously described in the literature.
Case Description: A mobile phone repairer presented with blurred vision and a superotemporal pterygium of his dominant left eye associated with a magnifying glass LED work lamp was diagnosed. This was excised routinely with conjunctival autografting to the defect. Histopathology confirmed benign pterygium and recovery was uncomplicated with resolution of blur.
Conclusions: The development of pterygium in our patient may have arisen due to the LED lamp’s wavelengths possibly falling within the UV as well as the upper end of the visible light radiation spectrum. Given the increasing reliance on LED light sources in modern life, ocular conditions arising from exposure to these radiation sources may now need to be listed in the differential diagnoses of patients with pterygium. Appropriate UV protection counselling for these types of lights may also now need to be considered.
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: The disease burden of diabetic retinopathy (DR) is tremendous around the world. While DR is correlated with hemoglobin A1c (HbA1c) and duration of diabetes, genetic differences likely account for variation in susceptibility to DR. DR is a polygenic disorder with demonstrated heritability. However, linkage and admixture analyses, candidate gene association studies, and genome-wide association studies (GWAS) have not identified many loci for DR that can be consistently replicated. Larger, collaborative, multi-ethnic GWAS are needed to identify common variants with small effects. Rigorous defining of controls groups as patients with a long duration of diabetes without DR, and case groups as patients with severe DR will also aid in finding genes associated with DR. Replication in independent cohorts will be key to establishing associated loci for DR. Investigations of mitochondrial DNA and epigenetics in DR are ongoing. Whole exome sequencing presents new opportunities to identify rare variants that might be implicated in DR development. Continued research in the genetic epidemiology of DR is needed, with the potential to elucidate pathogenesis and treatment of an important disease.
Abstract: Retinopathy of prematurity (ROP) is a proliferative disorder of the developing retina in premature and low birth weight infants. Recently, the role of vascular endothelial growth factor (VEGF) in the pathophysiology of ROP has been well studied and anti-VEGF drugs have been used in phase 2 to treat ROP patients in many ways. At first, ophthalmologists began to give intravitreal bevacizumab (IVB) or ranibizumab off-label to treat ROP as a salvage treatment after failure in laser photocoagulation or in combination with laser as an adjuvant treatment for patients had media opacity or rigid pupil. Now anti-VEGF drugs are also used as monotherapy in type I ROP or perioperative use in stage 4/5 ROP. Questions remain regarding long-term safety, dose, timing, visual outcomes and long-term effects, including systemically.