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: Benign essential blepharospasm (BEB), aberrant facial nerve degeneration and hemifacial spasm (HFS) are all examples dystonia which, though not life-threatening, can have a significant impact on patient quality of life. The need for reliable self-rating surveys to monitor functional disability is fundamental. The Blepharospasm Disability Index (BSDI) is already a widely utilised and validated self-rating score for blepharospasm whilst the functional disability score (FDS) requires further validation. The principle aim of this study is to repeat validation of the FDS against the BSDI, which has been validated by several groups since its original description but only in patients with BEB.

Methods: A randomised blinded prospective cohort study was conducted at a single unit on 38 patients with BEB, aberrant facial nerve degeneration and HFS. Patients were blinded to complete the FDS followed by the BSDI or the BSDI followed by the FDS with a 30-minute interval.

Results: Both the FDS and BSDI were found to be reliable with high internal consistency and test-retest reliability. Both scales were also found to be moderately correlated with the Jankovic disease severity score.

Conclusions: This study is the first to use the FDS as a rating scale in patients with HFS and aberrant facial nerve degeneration. It is also the first study to formally validate the FDS as an acceptable rating scale for patients with dystonia and in particular it provides validation for its use in patients with HFS and aberrant facial nerve degeneration.

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.

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.

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: Retinopathy of prematurity (ROP) is an emerging cause of childhood blindness in the developing countries. The low and middle-income countries are facing common challenges in the midst of the ‘third epidemic’ of ROP. Improvement in neonatal care facilities has increased survival of preterm babies. Lack of awareness and non-uniform standards of care in the ever-increasing number of neonatal intensive care units (NICUs) and special newborn care units (SNCUs) has resulted in this surge of ROP. Apart from low birth weight and the degree of prematurity, use of unblended supplemental oxygen, sepsis, anemia and blood transfusion are important risk factors associated with ROP in developing countries. Atypical forms of aggressive posterior ROP (APROP) are seen in heavier birth weight babies in the developing countries. Prevention of ROP by good quality neonatal care, timely diagnosis by mandatory ROP screening in NICUs and training manpower for laser treatment of ROP requires close collaboration between the neonatologists, ophthalmologists and the policy makers. Team approach and inter-disciplinary co-ordination are keys in a nation’s drive to fight this preventable cause of blindness.