Fundus imaging features of congenital rubella retinopathy.

PURPOSE: To evaluate the clinical characteristics of congenital rubella retinopathy (CRR) with modern fundus imaging. METHODS: Single-center case series. Eleven patients (2005-2020) at the Emory Eye Center with known or presumed CRR. Trained image readers reviewed fundus imaging (color fundus photography, widefield pseudocolor imaging, near-infrared reflectance imaging, autofluorescence imaging, and spectral-domain optical coherence tomography) for pre-specified features suggestive of CRR. RESULTS: Eleven patients with confirmed (63.6%) or presumed (36.3%) CRR were identified. All were female with median (range) age of 53 (35-67) years. Six (54.5%) were born during the 1964-1965 United States rubella epidemic. All had congenital hearing loss. Two (18.2%) had a congenital heart defect. Eleven (50.0%) eyes had salt-and-pepper retinal pigmentary changes. Twenty-two eyes (100.0%) had irregularly distributed regions of speckled hypoautofluorescence. One eye (4.5%) had a presumed macular neovascularization. CONCLUSION: Modern fundus imaging demonstrates characteristic features of CRR, even when pigmentary changes are not readily apparent on examination. Widefield autofluorescence findings of irregularly distributed speckled hypoautofluorescence are particularly revealing. This series of newly diagnosed adults with CRR may represent the milder end of the phenotypic spectrum of this condition, highlighting imaging findings that may aid in diagnostically challenging cases of CRR.

Surgical Outcomes of Progressive Retinoschisis-Related Retinal Detachments: A 17-Year Survey From a Large Academic Center.

BACKGROUND AND OBJECTIVE: To provide an overview of progressive retinoschisis-related retinal detachment (RSRD) management at a tertiary referral center. MATERIALS AND METHODS: Single-institution retrospective case series from January 1, 2003, to May 1, 2020. RESULTS: Progressive RSRD occurred in 0.9% of patients with retinoschisis. Mean (range) age at time of surgery was 58.7 years (40.0 to 74.0). Ten eyes were initially treated with scleral buckle, three eyes with vitrectomy, and three eyes with combined scleral buckle and vitrectomy. Overall reattachment rate was 100.0%; single-surgery success was 56.2%. Proliferative vitreoretinopathy developed in 10.0% of scleral buckles, 33.3% of vitrectomies, and 33.3% of combined surgeries. CONCLUSIONS: Progressive RSRD is rare and poses surgical management challenges. Final retinal attachment can be achieved successfully but often requires secondary and staged surgeries. Localization of outer retinal breaks may help guide surgical management. Further research-such as a large-scale, prospective, multicenter, randomized trial-would be needed to determine the optimal surgical technique. [Ophthalmic Surg Lasers Imaging Retina. 2022;53:132-138.].

Novel Detection and Restorative Levodopa Treatment for Preclinical Diabetic Retinopathy.

Diabetic retinopathy (DR) is diagnosed clinically by directly viewing retinal vascular changes during ophthalmoscopy or through fundus photographs. However, electroretinography (ERG) studies in humans and rodents have revealed that retinal dysfunction is demonstrable prior to the development of visible vascular defects. Specifically, delays in dark-adapted ERG oscillatory potential (OP) implicit times in response to dim-flash stimuli (<-1.8 log cd · s/m2) occur prior to clinically recognized DR. Animal studies suggest that retinal dopamine deficiency underlies these early functional deficits. In this study, we randomized individuals with diabetes, without clinically detectable retinopathy, to treatment with either low- or high-dose Sinemet (levodopa plus carbidopa) for 2 weeks and compared their ERG findings with those of control subjects (no diabetes). We assessed dim-flash-stimulated OP delays using a novel handheld ERG system (RETeval) at baseline and 2 and 4 weeks. RETeval recordings identified significant OP implicit time delays in individuals with diabetes without retinopathy compared with age-matched control subjects (P < 0.001). After 2 weeks of Sinemet treatment, OP implicit times were restored to control values, and these improvements persisted even after a 2-week washout. We conclude that detection of dim-flash OP delays could provide early detection of DR and that Sinemet treatment may reverse retinal dysfunction.

EFFECT OF LUBRICANTS ON CORNEAL THICKNESS AFTER VITRECTOMY.

PURPOSE: This study examines the impact of corneal surface lubricants used during pars plana vitrectomy on corneal edema. METHODS: This prospective, observational, clinical study occurred at an academic institution. Participants were individuals aged 18 years and older who had already consented to undergo pars plana vitrectomy, without pre-existing corneal pathology. A corneal lubricant was chosen by the surgeon. Corneal thickness was measured preoperatively and postoperatively using pachymetry and anterior segment optical coherence tomography (AS-OCT). Main outcome measure was change in corneal thickness as measured by pachymetry. RESULTS: Forty-one patients completed the study protocol. The 23 subjects in the SHCS group had a significantly smaller increase in corneal thickness as measured by pachymetry compared with the 18 subjects in the HPMC group (29.9 µm vs. 58.1 µm, P value 0.02). When measured by anterior segment optical coherence tomography, the SHCS group had a smaller increase in corneal thickness compared with the HPMC group (0.04 mm vs. 0.06 mm, P value 0.09) but did not reach significance. CONCLUSION: SHCS is associated with reduced postoperative increase in corneal pachymetry as compared to HPMC.

Necrotic uveal melanoma presenting as orbital cellulitis with intraocular hemorrhage: A case report.

PURPOSE: To report a case of necrotic uveal melanoma presenting as orbital cellulitis with an intraocular hemorrhage. OBSERVATIONS: A 61 year-old non-verbal male presented with a two-week history of right eyelid swelling and erythema unresponsive to antibiotics. In addition to these signs of orbital cellulitis, he presented with an opaque media precluding fundus visualization. He was later found to have a collar-button shaped mass consistent with uveal melanoma on B scan ultrasonography during an exam under anesthesia. The patient underwent enucleation with histopathology confirming a necrotic uveal melanoma. CONCLUSION AND IMPORTANCE: This case demonstrates how necrotic uveal melanoma can present as orbital cellulitis and the importance of keeping the diagnosis on the differential.

Rhegmatogenous Retinal Detachment in Children: Clinical Factors Predictive of Successful Surgical Repair.

PURPOSE: To describe presenting clinical features and surgical techniques that are associated with successful surgical repair of pediatric rhegmatogenous retinal detachment (RRD). DESIGN: Retrospective interventional case series. PARTICIPANTS: Two hundred twelve eyes of 191 patients 0 to 18 years of age undergoing surgical repair for RRD between 2001 and 2015 with a minimum follow-up of 3 months. METHODS: Patients were divided into 3 age groups (0-6 years, 7-12 years, and 13-18 years) and comparisons were made using bivariate and multivariate generalized estimating equation models. A mixed means model was used to examine visual acuity in each age group over time. MAIN OUTCOME MEASURES: Complete reattachment of the retina at final follow-up. RESULTS: Of 212 eyes, 166 (78%) achieved total reattachment at final follow-up. Mean follow-up was 36.3 months. Rhegmatogenous retinal detachment associated with Stickler syndrome was more likely to occur in the younger cohorts (odds ratio [OR], 0.45; 95% confidence interval [CI], 0.22-0.91), whereas RRD associated with blunt trauma was more likely to occur in the oldest cohort (OR, 2.3; 95% CI, 1.2-4.4). Subtotal RRD was more likely to be repaired successfully than total RRD (OR, 3.6; 95% CI, 1.5-8.4; P = 0.0100), and eyes with previous vitreoretinal surgery were less likely to undergo successful repair (OR, 0.30; 95% CI, 0.12-0.78; P = 0.0258). There was no significant difference between age groups in the rate of surgical success (P = 0.55). There was a significantly higher success rate with primary scleral buckle (SB; 63%; OR, 2.2; 95% CI, 1.1-4.5) and combined SB plus pars plana vitrectomy (PPV; 68%; OR, 2.3; 95% CI, 1.1-5.1) compared with PPV alone (51%). CONCLUSIONS: Most pediatric patients with RRD achieved complete reattachment with surgery. Success was more common in patients with a subtotal RRD at presentation. Previous vitreoretinal surgery was a risk factor for failure. Younger patients were more likely to demonstrate RRD involving the macula, but there was no difference between age groups in successful reattachment at final follow-up. Primary PPV showed a lower rate of success than SB or combined SB plus PPV.

Association of serum vitamin D levels and diabetic retinopathy in Asian Indians with type 2 diabetes.

BACKGROUND: Vitamin D deficiency (VDD) is a condition that has been associated with diabetic retinopathy (DR) in various populations, but has not been studied in Asian Indians. AIMS: To evaluate the association of serum 25-hydroxyvitamin D (25(OH)D) levels with presence and severity of DR among Asian Indians with type 2 diabetes. METHODS: We collected information on individuals with type 2 diabetes that received care at a tertiary diabetes centre in India, between 2012 and 2015. Patients were 18 years of age or older, underwent retinal examinations with DR severity grading and had serum 25(OH)D measurements. RESULTS: Serum 25(OH)D levels were lower in patients with retinopathy compared to those without (11.9 ±â€¯2.2 ng/ml vs. 13.7 ±â€¯2.1 ng/ml, p < 0.001). Stratifying patients by DR grade, reduced geometric means of 25(OH)D levels were associated with increased retinopathy severity. After adjusting for six key covariates, VDD was associated with increased rates of proliferative DR (OR 2.05; 95% CI 1.35-3.11; p = 0.001). CONCLUSIONS: In Asian Indians with type 2 diabetes, lower serum 25(OH)D was associated with increased severity of DR and the presence of VDD was associated with a two-fold increased risk for proliferative DR.

Propranolol for Proliferative Diabetic Retinopathy.

BACKGROUND AND OBJECTIVE: To determine the effect of propranolol on retinal neovascularization due to proliferative diabetic retinopathy (PDR). PATIENTS AND METHODS: For this prospective pilot, interventional, case series, patients with diabetes with PDR (n = 10 subjects; 12 eyes) were recruited at the ophthalmology clinic of the University of Wisconsin - Madison. Subjects were administered oral propranolol for 12 weeks and retinopathy and area of retinal neovascularization were monitored with fundus photography and fluorescein angiography (FA). The study's main outcome measures were photographic area of retinal neovascularization and degree of leakage on FA. RESULTS: All eyes demonstrated stable degrees of retinal neovascularization by the end of 12 weeks. CONCLUSION: This dose of oral propranolol during a period of 12 weeks did not demonstrate significant effect on retinal neovascularization due to PDR. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:35-40.].

Current and Next Generation Portable Screening Devices for Diabetic Retinopathy.

Diabetic retinopathy (DR) is the leading cause of legal blindness in the United States, and with the growing epidemic of diabetes, a global increase in the incidence of DR is inevitable, so it is of utmost importance to identify the most cost-effective tools for DR screening. Emerging technology may provide advancements to offset the burden of care, simplify the process, and provide financially responsible methods to safely and effectively optimize care for patients with diabetes mellitus (DM). We review here currently available technology, both in production and under development, for DR screening. Preliminary results of smartphone-based devices, "all-in-one" devices, and alternative technologies are encouraging, but are largely pending verification of utility when used by nonophthalmic personnel. Further research comparing these devices to current nonportable telemedicine strategies and clinical fundus examination is necessary to validate these techniques and to potentially overcome the poor compliance around the globe of current strategies for DR screening.

Diabetic Retinopathy.

The prevalence of diabetes is on the rise globally as are the consequences, such as diabetic retinopathy. Diabetic retinopathy is a leading cause of vision loss in working-age adults in developed countries. Visual impairment as a result of diabetic retinopathy has a significant negative impact on the patient's quality of life and their ability to successfully manage their disease. Glycemic control, blood pressure normalization, and lipid management form the basis for long-term diabetes management and protection from worsening eye disease.

Comparison Among Methods of Retinopathy Assessment (CAMRA) Study: Smartphone, Nonmydriatic, and Mydriatic Photography.

PURPOSE: We compared smartphone fundus photography, nonmydriatic fundus photography, and 7-field mydriatic fundus photography for their abilities to detect and grade diabetic retinopathy (DR). DESIGN: This was a prospective, comparative study of 3 photography modalities. PARTICIPANTS: Diabetic patients (n = 300) were recruited at the ophthalmology clinic of a tertiary diabetes care center in Chennai, India. METHODS: Patients underwent photography by all 3 modalities, and photographs were evaluated by 2 retina specialists. MAIN OUTCOME MEASURES: The sensitivity and specificity in the detection of DR for both smartphone and nonmydriatic photography were determined by comparison with the standard method, 7-field mydriatic fundus photography. RESULTS: The sensitivity and specificity of smartphone fundus photography, compared with 7-field mydriatic fundus photography, for the detection of any DR were 50% (95% confidence interval [CI], 43-56) and 94% (95% CI, 92-97), respectively, and of nonmydriatic fundus photography were 81% (95% CI, 75-86) and 94% (95% CI, 92-96%), respectively. The sensitivity and specificity of smartphone fundus photography for the detection of vision-threatening DR were 59% (95% CI, 46-72) and 100% (95% CI, 99-100), respectively, and of nonmydriatic fundus photography were 54% (95% CI, 40-67) and 99% (95% CI, 98-100), respectively. CONCLUSIONS: Smartphone and nonmydriatic fundus photography are each able to detect DR and sight-threatening disease. However, the nonmydriatic camera is more sensitive at detecting DR than the smartphone. At this time, the benefits of the smartphone (connectivity, portability, and reduced cost) are not offset by the lack of sufficient sensitivity for detection of DR in most clinical circumstances.

Toxoplasma chorioretinitis diagnosed by histopathology in a patient with AIDS.

PURPOSE: To demonstrate the diagnostic difficulties in cases of retinal necrosis in immunocompromised patients including the potential for false-negative anterior segment sampling and also to emphasize the utility of diagnostic vitrectomy with histopathologic examination. METHODS: This patient's chart was thoroughly reviewed to present salient features that are relevant to any ophthalmologist attempting to diagnose and treat chorioretinitis. A 38-year-old man with HIV/AIDS who presented with bilateral retinal necrosis. Thorough workup, including multiple samples of anterior chamber fluid for polymerase chain reaction, was negative. RESULTS: Diagnostic vitrectomy revealed a toxoplasma cyst. Triple therapy stabilized retinitis, although vision did not improve. CONCLUSION: This case reminds the clinician to consider a broad differential diagnosis for retinal necrosis in immunocompromised hosts and, when serologic and anterior chamber samples are negative, to consider diagnostic vitrectomy for polymerase chain reaction and histopathologic examination.

Cost-effectiveness of Different Diabetic Retinopathy Screening Modalities.

Current screening strategies aimed at detection of diabetic retinopathy (DR) historically have poor compliance, but advancements in technology can enable improved access to care. Nearly 80% of all persons with diabetes live in low- and middle-income countries (LMICs), highlighting the importance of a cost effective screening program. Establishing mechanisms to reach populations with geographic and financial barriers to access is essential to prevent visual disability. Teleretinal programs leverage technology to improve access and reduce cost. The quality of currently employed screening modalities depends on many variables including the instrument used, use of pupillary mydriasis, number of photographic fields, and the qualifications of the photographer and image interpreter. Recent telemedicine and newer technological approaches have been introduced, but data for these technologies is yet limited. We present results of a systematic review of studies evaluating cost-effectiveness of DR screening, and discuss potential relevance for LMICs.

Ophthalmic manifestations of endocrine disorders: approaches and medical management.

PURPOSE OF REVIEW: Disorders of the endocrine system may present with various ophthalmic signs, symptoms, and pathology. Early detection, prevention, and monitoring of these manifestations can significantly benefit morbidity associated with such disease states including permanent visual dysfunction. RECENT FINDINGS: Several studies highlight the importance of early diagnosis in reducing the long-term complications associated with these endocrine conditions, although these observations are mostly based on case reports and case series. SUMMARY: This review will focus on the connection of endocrine-related disease states with the development of specific ophthalmic pathology and is geared towards the general medical practitioner. The focus of the treating physician should be on facilitating early diagnosis to prevent potentially blinding complications.

Primary open-angle glaucoma: diagnostic approaches and management.

Glaucoma represents the second most common cause of blindness in the United States affecting approximately 2 million Americans, with many others unaware of being afflicted. Primary open-angle glaucoma (POAG), which is the most common of all types of glaucoma, leads to progressive optic nerve damage. If left untreated, POAG can lead to irreversible blindness. Since POAG is a relatively asymptomatic disease, prevention of long-term complications must be accomplished with regular screening for early detection and appropriate treatment. Primary care physicians play a critical role in screening at-risk populations and, therefore, reducing the risk of visual loss that characterizes advanced stages of POAG.

Ex-PRESS mini glaucoma shunt: surgical technique and review of clinical experience.

Glaucoma is a leading cause of worldwide blindness. Treatment of this chronic disease, centered on lowering intraocular pressure (IOP), may require surgical intervention when medical and/or laser therapy fail to control IOP. Traditional glaucoma surgery, such as trabeculectomy, has been performed for decades and has a proven track record of success. Unfortunately, many complications such as hypotony and slow visual recovery are known to occur and can lead to significant patient morbidity. New devices are being developed to allow surgeons increased control with more predictable postoperative results. One such device, the Ex-PRESS mini glaucoma shunt, has undergone changes in design and method of insertion making it more appealing for use in patients requiring IOP-lowering surgery. We summarize pertinent literature and expand on the future role of this device in glaucoma surgery.

Functional coupling of simultaneous electrical and metabolic activity in the human brain.

The relationships between brain electrical and metabolic activity are being uncovered currently in animal models using invasive methods; however, in the human brain this relationship remains not well understood. In particular, the relationship between noninvasive measurements of electrical activity and metabolism remains largely undefined. To understand better these relations, cerebral activity was measured simultaneously with electroencephalography (EEG) and positron emission tomography using [(18)f]-fluoro-2-deoxy-D-glucose (PET-FDG) in 12 normal human subjects during rest. Intracerebral distributions of current density were estimated, yielding tomographic maps for seven standard EEG frequency bands. The PET and EEG data were registered to the same space and voxel dimensions, and correlational maps were created on a voxel-by-voxel basis across all subjects. For each band, significant positive and negative correlations were found that are generally consistent with extant understanding of EEG band power function. With increasing EEG frequency, there was an increase in the number of positively correlated voxels, whereas the lower alpha band (8.5-10.0 Hz) was associated with the highest number of negative correlations. This work presents a method for comparing EEG signals with other more traditionally tomographic functional imaging data on a 3-D basis. This method will be useful in the future when it is applied to functional imaging methods with faster time resolution, such as short half-life PET blood flow tracers and functional magnetic resonance imaging.

Affective judgments of faces modulate early activity (approximately 160 ms) within the fusiform gyri.

Functional neuroimaging studies have implicated the fusiform gyri (FG) in structural encoding of faces, while event-related potential (ERP) and magnetoencephalography studies have shown that such encoding occurs approximately 170 ms poststimulus. Behavioral and functional neuroimaging studies suggest that processes involved in face recognition may be strongly modulated by socially relevant information conveyed by faces. To test the hypothesis that affective information indeed modulates early stages of face processing, ERPs were recorded to individually assessed liked, neutral, and disliked faces and checkerboard-reversal stimuli. At the N170 latency, the cortical three-dimensional distribution of current density was computed in stereotactic space using a tomographic source localization technique. Mean activity was extracted from the FG, defined by structure-probability maps, and a meta-cluster delineated by the coordinates of the voxel with the strongest face-sensitive response from five published functional magnetic resonance imaging studies. In the FG, approximately 160 ms poststimulus, liked faces elicited stronger activation than disliked and neutral faces and checkerboard-reversal stimuli. Further, confirming recent results, affect-modulated brain electrical activity started very early in the human brain (approximately 112 ms). These findings suggest that affective features conveyed by faces modulate structural face encoding. Behavioral results from an independent study revealed that the stimuli were not biased toward particular facial expressions and confirmed that liked faces were rated as more attractive. Increased FG activation for liked faces may thus be interpreted as reflecting enhanced attention due to their saliency.

Brain electrical tomography in depression: the importance of symptom severity, anxiety, and melancholic features.

BACKGROUND: The frontal lobe has been crucially involved in the neurobiology of major depression, but inconsistencies among studies exist, in part due to a failure of considering modulatory variables such as symptom severity, comorbidity with anxiety, and distinct subtypes, as codeterminants for patterns of brain activation in depression. METHODS: Resting electroencephalogram was recorded in 38 unmedicated subjects with major depressive disorder and 18 normal comparison subjects, and analyzed with a tomographic source localization method that computes the cortical three-dimensional distribution of current density for standard electroencephalogram frequency bands. Symptom severity and anxiety were measured via self-report and melancholic features via clinical interview. RESULTS: Depressed subjects showed more excitatory (beta3, 21.5-30.0 Hz) activity in the right superior and inferior frontal lobe (Brodmann's area 9/10/11) than comparison subjects. In melancholic subjects, this effect was particularly pronounced for severe depression, and right frontal activity correlated positively with anxiety. Depressed subjects showed posterior cingulate and precuneus hypoactivity. CONCLUSIONS: While confirming prior results implicating right frontal and posterior cingulate regions, this study highlights the importance of depression severity, anxiety, and melancholic features in patterns of brain activity accompanying depression.