Considerations for the Identification and Management of Geographic Atrophy: Recommendations from an Expert Panel.

Newly approved treatments for patients with geographic atrophy are changing the treatment paradigm, highlighting the need for eye care providers (ECPs) to have a set of recommendations on how to best manage GA patients. Here, we outline how to identify various stages of age-related macular degeneration including geographic atrophy (GA) by examining optimal management scenarios implicating various ECPs and reviewing treatment considerations for patients with GA. Early identification of GA will lead to optimal patient outcomes, while a standardized management scenario will reduce clinical burden among ECPs treating patients with GA.

Geographic Atrophy Management Consensus (GA-MAC): a Delphi panel study on identification, diagnosis and treatment.

BACKGROUND/AIMS: With a paradigm shift in geographic atrophy (GA) treatments now available, establishing consensus on the identification and diagnosis of the disease along with considerations for management of patients with GA will assist eye care professionals (ECP) in their day-to-day practices, leading to improved patient outcomes. METHODS: A modified Delphi panel process (Geographic Atrophy Management Consensus) consisting of three total surveys and one virtual live meeting held between survey 2 and survey 3. Data were collected from July to October 2022. Participants included expert members of the eye care community that have demonstrated outstanding leadership among peers: a steering committee with three ECPs and a 15-member panel divided between five optometrists, five comprehensive ophthalmologists and five retina specialists. Consensus on statements related to the management of patients with GA was calculated using the RAND/UCLA Appropriateness Method. RESULTS: At the conclusion of the third survey, consensus was reached on 91% of the 77 statements. Critical consensus topics include: (1) optical coherence tomography as the favoured method to diagnose and monitor GA, (2) preferred practice patterns regarding referral of patients to retina specialists and (3) treatment criteria given the advent of emerging therapeutics for GA. CONCLUSIONS: Generating awareness of early signs of disease development, progression and identifying the best tools to evaluate GA establishes ideal management and referral strategies. Given the paradigm shift in GA management driven by approved therapies, coupled with the fact that the disease is progressive resulting in devastating vision loss, these strategies are critical to ensure best overall outcomes.

Outer Retinal Tubulation: A Case Series.

PURPOSE: The advent of spectral domain optical coherence tomography has led to superb imaging capabilities in addition to enhanced visualization of the retinal layers. Such advancements have led to the identification of a variety of new retinal conditions, including outer retinal tubulations (ORTs). ORTs are ovoid hyporeflective spaces located in the outer retina. The pathogenesis is unclear but seems to involve sublethal injury to the photoreceptors leading to a compensatory reorganization of the photoreceptor layer with the neighboring ellipsoid zone resulting in a hyperreflective border surrounding a central lumen. Most ORTs have been linked to wet age-related macular degeneration; however, these peculiar structures are now seen in a myriad of retinal disorders. CASE REPORTS: Our cases will highlight the wide variety of clinical presentations associated with outer retinal tubulations. The clinical presentations include two cases of wet age-related macular degeneration, a case of presumed ocular histoplasmosis syndrome, a case of central areolar choroidal dystrophy, and a case of pathological myopia. CONCLUSIONS: By correctly differentiating outer retinal tubulations from other masqueraders, unnecessary referrals and interventions can be minimized. Understanding the various disease entities associated with outer retinal tubulation could give further insight into the mechanism and formation of these structures.

Atypical Exophytic Retinal Capillary Hemangioma and Diagnostic Modalities.

PURPOSE: Retinal capillary hemangioma (RCH) can occur in isolation or may be associated with von Hippel-Lindau disease. The classic RCH is described as a globular reddish lesion with a dilated feeding artery and a tortuous draining vein, indicative of a common endophytic growth pattern. Exophytic patterns are far more rare and, because of its subtle appearance, often missed or misdiagnosed. CASE REPORT: A 24-year-old woman presented with complaints of a mid-peripheral superior field defect OS. She had no family history of von Hippel-Lindau disease. Best-corrected visual acuity was 20/20 OD, OS. Dilated fundus examination of the left eye revealed a peripheral large inferotemporal retinal lesion with an overlying vascular network. Corresponding feeding and draining vasculature was not clearly noted on dilated fundus examination. Optical coherence tomography, ultrasonography, and fluorescein angiography testing were used to confirm the diagnosis of an exophytic peripheral RCH. Anti-vascular endothelial growth factor injections and cryotherapy were initiated, and the patient was scheduled for radioactive plaque brachytherapy. Genetic testing and proper scans were also recommended. CONCLUSIONS: This case illustrates an atypical exophytic peripheral RCH, rarely reported in the literature. The utilization of a variety of diagnostic modalities was highly effective, aiding in the diagnosis of this condition.

The continuum of primary retinal telangiectasia.

BACKGROUND: Primary retinal telangiectasia is characterized by abnormalities in the retinal vasculature. Any alteration of the normal retinal vasculature may result in variable degrees of retinal leakage, hemorrhages, and exudates. The retinal telangiectatic conditions of Coats' disease, Leber's miliary aneurysms, and idiopathic macular telangiectasia (IMT), although historically considered separate entities, may in fact be variants of the same pathophysiologic vascular process. This is based on observations noting that they share similarities in pathogenesis, histology, and clinical presentation. These observations are controversial and are contested in the literature. Conversely, others have documented Coats' disease, Leber's miliary aneurysms, and IMT as unique and separate retinal conditions, each with specific features. METHODS: Three cases are presented spanning the spectrum of primary retinal telangiectasia. Clinical evaluation, dilated fundus examination, and auxiliary testing document both the similar characteristics that the entities share as well as the distinct features, which define each disease's nomenclature and categorization. CONCLUSION: Coats' disease, Leber's miliary aneurysms, and IMT may be part of a singular clinical spectrum sharing pathophysiologic and histopathologic features and similarities in clinical presentation.

The expanding spectrum of vitreomacular traction.

BACKGROUND: Vitreomacular traction (VMT) syndrome is characterized as a partial detachment of the posterior vitreous with persistent adherence to the macula. The dynamic process associated with macular traction may induce a variety of macular conditions including cystoid macular edema (CME), epiretinal membranes (ERM), and macular hole formation. METHODS: Dilated fundus evaluations as well as Stratus and Cirrus optical coherence tomography (OCT) (Zeiss-Humphrey, Dublin, California) imaging were used to evaluate patients with various maculopathies associated with VMT. CONCLUSION: The use of the OCT has enhanced the evaluation of the vitreal-retinal interface, leading to a better understanding of VMT. Once thought to be a rare distinct clinical entity, VMT is now considered a spectrum of macular diseases. The understanding of VMT and its role in the pathophysiology of various macular conditions may facilitate diagnosis and management of these conditions.

Review and update: current treatment trends for patients with retinitis pigmentosa.

BACKGROUND: Retinitis pigmentosa (RP) is a heterogeneous group of inherited retinal disorders characterized by progressive photoreceptor apoptosis. It is the leading cause of inherited retinal degeneration-associated blindness. RP has a unique set of clinical characteristics that make it a complex disease associated with distinct inheritance patterns. An understanding of the pathogenesis is essential in the process of the differential diagnosis and the development of treatment options. Recent developments in research are likely to expand the various therapeutic modalities to include gene therapy, pharmacologic treatment, cell transplantation, and neuro-prosthetic devices. METHODS: A literature search was performed to comprehensively review RP diagnosis, pathophysiology, and treatment. CONCLUSION: Advances in the understanding of the pathophysiology of RP are creating new opportunities for the treatment of this often visually debilitating eye condition. Optometrists, as primary eye care practitioners, should be aware of the inheritance, pathophysiology, and current treatment options for RP as well as treatments in development so that they can best care for their patients with inherited retinal disorders.

Pupil sparing incomplete third nerve palsy secondary to a cavernous sinus meningioma: challenges in management.

BACKGROUND: The diagnosis of incomplete third nerve palsy can be clinically challenging because the aetiologies, as well as presentations, can be variable and subtle. The optometric clinician should be familiar with the association of third nerve palsy with compressive lesions, including the clinical presentations and management of these patients. CASE REPORT: We present a 68-year-old hypertensive male complaining of intermittent diplopia for the previous six months. Neurological examination revealed a mild deficit in adduction, reduced range of vertical motility and questionable ptosis, all limited to the OD. No pupillary involvement was noted. MRI revealed a cavernous sinus meningioma. The patient elected against surgery, choosing MRI every six months. CONCLUSION: Due to the subtle clinical presentation of incomplete pupil sparing third nerve palsy, this ophthalmoplegia may be easily overlooked. The associated aetiologies of palsy of cranial nerve three (CN3) carry increased risk of morbidity and mortality. Management should include evaluation for mass lesions and/or vascular aetiologies, regardless of pupillary involvement.