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: To explore the safety and effectiveness of Sclera patch grafts in the management of scleral defects.

Methods: This is a retrospective uncontrolled study. Medical records were retrospectively reviewed for 8 eyes of 8 patients with sclera patch grafts. Two patients had necrotizing scleritis, 2 patients had scleral melting/perforation secondary to thermal burns, 4 patients had scleral staphyloma secondary to surgery. Sclera was reconstructed with allogenic sclera patch grafts, 6 in 8 patients combined autologous conjunctival pedicle flap, 1 patient combined partial medial rectus translocation, 1 patient combined autologous pedicle tenon graft, simultaneously. Treatment outcomes were evaluated using structural integrity, best corrected visual acuity (BCVA), scleritis remission, sclera rejection and melt, and ocular symptoms.

Results: Eight patients were reviewed. In all of these cases, satisfactory anatomic and functional outcomes were achieved. In the at least half a year follow-up, the BCVA of all the eight patients were no worse than that of preoperative. No eye pain, foreign body sensation and other discomforts showed in all the patients, except one woman, who showed sclera rejection and melt 1 month postoperative. In addition, one patient showed high intraocular pressure (28 mmHg), which can be controlled by a kind of medicine.

Conclusions: In this series, sclera patch grafts is an effective method for management scleral defects in the at least half a year following-up. Attention should be paid to the sclera patch rejection and melt post operatively.

Abstract: Acute retinal necrosis (ARN) is a devastating syndrome characterized by panuveitis, retinal necrosis, and a high rate of retinal detachment that may result in poor visual outcomes if not promptly diagnosed and treated. ARN is most commonly caused by viruses within the herpesvirus family. Etiologies include varicella-zoster virus, herpes simplex virus, and cytomegalovirus, and may be promptly diagnosed by polymerase chain reaction testing of aqueous or vitreous fluid. The true incidence of ARN is not known due to its rarity; as a result, clinical treatment is often guided by retrospective case series, case reports, and expert opinion. Standard of care has evolved over time but currently includes a combination of systemic and intravitreal antiviral in conjunction with topical or oral steroids and surgical therapy as needed. Combination therapy may reduce the rate of severe vision loss and increase the rate of visual acuity gain, although further studies are needed in this area. In particular for patients with mild to moderate disease, combination therapy may reduce the rate of retinal detachment. Adjunctive therapies including oral corticosteroid and prophylactic laser barricade are incompletely studied, but corticosteroid in particular, may reduce inflammation, which also is involved in the severe disease pathogenesis observed in ARN. This review discusses the advances in diagnosis and treatment of ARN, including management with combination antiviral medication and surgical interventions.

Abstract: Uveitis can cause significant visual morbidity and often affects younger adults of working age. Anterior uveitis, or inflammation limited to the anterior chamber (AC), iris, and/or ciliary body comprises the majority of uveitis cases. Current clinical biomarkers and conventional grading scales for intraocular inflammation are mostly subjective and have only a moderate degree of interobserver reliability, and as such they have significant limitations when used in either clinical practice or research related to uveitis. In recent years, novel imaging techniques and applications have emerged that can supplement exam findings to detect subclinical disease, monitor quantitative biomarkers of disease progression or treatment effect, and provide overall a more nuanced understanding of disease entities. The first part of this review discusses automated algorithms for optical coherence tomography (OCT) image processing and analysis as a means to assess and describe intraocular inflammation with higher resolution than that afforded by conventional AC and vitreous cell ordinal grading scales. The second half of the review focuses on anterior segment OCT and OCT angiography (OCTA) in scleritis and iritis, especially with regards to their ability to directly image and characterize the pathologic structures and vasculature underlying these diseases. Finally, we briefly review experimental animal research with promising but more distant human clinical applications, including in vivo molecular microscopy of inflammatory markers and investigation of gold nanoparticles as a potential contrast agent in OCT imaging. Imaging modalities are discussed in the broader context of trends within the field of uveitis towards greater objectivity and quantifiable outcome measures and biomarkers.

Abstract: Our increase in knowledge of the pathophysiology of non-infectious uveitis (NIU) and other immune-mediated diseases has been mirrored over the last two decades by the expansion of therapeutic options in the realm of immunosuppressive medications. Principal among these advances is the emergence of biologics, which offer the promise of targeted therapy and the hope of reduced toxicity when compared to corticosteroids and “standard” immunosuppression. Among the biologics, monoclonal antibodies blocking tumor necrosis factor alpha (TNF-α) have been shown to be a very effective therapeutic target for uveitis and many associated systemic inflammatory diseases. Multiple TNF blockers have shown benefit for uveitis, and in 2016, adalimumab became the first biologic and non-corticosteroid immunosuppressive to obtain Food and Drug Administration (FDA) approval in the treatment of NIU. Although effective, TNF blockers are not universally so, and safety concerns such as infection and demyelinating disease must be carefully considered and ruled out prior to their use, especially in patients with intermediate uveitis with which multiple sclerosis is a known association. Ongoing study has identified novel targets for regulation in the treatment of immune-mediated and inflammatory diseases. Interferons, interleukin and Janus kinase inhibitors in addition to antibodies targeting T cell and B cell activation highlight the expanding field of treatment modalities in NIU. Ongoing study will be required to better determine the safety and efficacy of biologics in the armamentarium of immunosuppressive treatments for NIU.

Abstract: Red eye is common in our daily practice. It ranges from non-inflammatory to inflammatory causes. An extended course of disease should prompt suspicion and the possibility of diagnosis revision. A prolonged conjunctivitis mimicking nodular episcleritis can be presented as a manifestation of granulomatosis with polyangiitis (GPA). A 57-year-old woman complained of eye redness and tearing for two weeks which partially resolved with antibiotics. She was subsequently commenced on topical and oral non-steroidal anti-inflammatory drugs (NSAIDs) and topical anti-allergic. However, in the following reviews she developed cornea thinning and her systemic examination revealed an injected uvula with absence of upper respiratory tract infection. She was investigated for connective tissue disease and found to have raised anti-inflammatory markers and her antinuclear antibody and C-ANCA tests were positive. She was diagnosed with GPA. Her conditions improved followed by the commencement of topical corticosteroid with high dose of systemic corticosteroid, which followed by a tapering regime with oral corticosteroid. Although red eye is common, it is associated with a variety of diseases. GPA manifestation can be as subtle as a red eye. Any prolonged partially treated red eye should prompt suspicion of a more sinister cause. Sensitive detection of other subtle systemic signs is very important.

Background: Bacillary layer detachment (BALAD) is a phenomenon characterized by fluid accumulation at the myoid region of the inner photoreceptor segments identifiable on optical coherence tomography (OCT) imaging. This finding has been recently described in patients with diverse primary diagnoses which share the common feature of serous exudation in the posterior pole. However, thus far there have been very few reports in the literature of BALAD in patients with posterior scleritis.

Case Description: A 16-year-old male presented with unilateral vision changes that acutely worsened overnight to significant unilateral vision loss. He was eventually diagnosed with idiopathic posterior scleritis with associated BALAD on OCT. Similar to other reported cases of BALAD, he experienced anatomic restoration of the outer retina followed by good visual recovery after treatment with high dose steroid, ultimately with complete recovery of both retinal anatomy and vision within 4 months.

Conclusions: This case provides further evidence that posterior scleritis can be a cause of BALAD. The rapid presentation and excellent visual and anatomical outcome of this case is entirely consistent with known descriptions of BALAD in a variety of other conditions, further supporting the categorization of BALAD as an entity which retinal specialists should be able to recognize as distinct from other forms of intraretinal fluid, retinal detachment, and retinoschisis.