Automated Pupillometry as an Adjunct to Clinical Examination in Patients With Acute Vision Loss.

BACKGROUND: Despite automated pupillometry's (AP) improved detection of relative afferent pupillary defects (RAPDs) compared with the Swinging Flashlight Test (SFT), AP remains uncommon in clinical practice. This study examined barriers to routine use of AP in evaluation of acute vision loss. METHODS: (1) Ophthalmologists and optometrists' perceptions of AP were captured via electronic survey. (2) Ophthalmologists were presented with clinical vignettes to assess their use of AP in clinical decision-making. (3) Patients presenting with decreased vision to an ophthalmology urgent care clinic underwent manual SFT and AP screening to evaluate ophthalmologists' perceptions of the device. RESULTS: Surveys indicated that clinicians were "neutral" to "somewhat likely" to use AP. In clinical vignettes, more physicians proceeded with workup for optic nerve pathology when presented with an RAPD by AP than SFT (77% vs 26%, P = 0.003). When SFT and AP results were discordant, more physicians proceeded with workup for optic nerve disease when AP was positive and SFT was negative than vice versa (61% vs 18%, P = 0.008). In the clinical study of 21 patients, 50% of RAPDs detected by AP were not detected by SFT, although ophthalmologists rated AP's usefulness as only "neutral" to "somewhat useful." CONCLUSION: Clinicians value pupillary examination and trust AP over SFT; however, widespread adoption and perceived value of AP may depend on its impact on clinical outcomes. Within a comprehensive diagnostic device, AP may be an important tool, but is not necessary to screen for optic nerve disease or evaluate acute vision loss.

Sarcoidosis in the national veteran population: association of ocular inflammation and mortality.

PURPOSE: To describe the distribution of ocular sarcoidosis in the veteran population and to determine the association between ocular disease and all-cause mortality. DESIGN: Retrospective review. METHODS: The Veterans Health Administration National Patient Care Database information on medical diagnoses, date of diagnosis, age, race, gender, and Veterans Administration medical center station number for site-specific calculations for fiscal years 2010 through 2012 was collected. Mortality data were obtained from the Beneficiary Identification Records Locator Subsystem. The patient cohort was identified with a primary diagnosis of sarcoidosis using International Classification of Disease, ninth edition, code of 135 in outpatient treatment files for the study period. The sarcoidosis patients were divided into those with uveitis or orbital inflammation (defined as ocular inflammation for this study) and those without uveitis or orbital inflammation. Survival analysis was performed using the Cox proportional hazard method. MAIN OUTCOME MEASURE: Association between ocular inflammation and 1-year mortality. RESULTS: Of 15 130 subjects with sarcoidosis, 3364 (22.2%) were evaluated in an eye clinic within a Veterans Administration Medical Center. Most patients were diagnosed with anterior uveitis (n = 1013; 80.7% of ocular inflammation), and the least common diagnosis was orbital granuloma (n = 28; 2.2% of ocular inflammation). Male gender was protective to the development of uveitis (estimate, 0.76; 95% confidence interval, 0.65-0.88; P = 0.0005). The overall 1-year all-cause mortality for all patients with a diagnosis of sarcoidosis was 2.0%. Ocular inflammation was associated with a decrease in 1-year all-cause mortality (simple model: hazard ratio, 0.36; P = 0.0015; complex model: hazard ratio, 0.35; P = 0.013). CONCLUSIONS: Veterans with ocular inflammation had significantly lower 1-year all-cause mortality than those without documented ocular inflammation. The reason for this finding remains to be established.

Update on sarcoidosis.

PURPOSE OF REVIEW: Sarcoidosis is a multisystem inflammatory disease, characterized by the presence of noncaseating granulomas. Ocular inflammation is often the first manifestation of the disease, and uveitis can be the driving force for treatment. The goal of this review was to provide an update on the relationship between ocular and systemic disease, with a particular focus on cardiac sarcoidosis. RECENT FINDINGS: Chest radiograph remains the best imaging tool for sarcoidosis, although newer modalities, such as whole-body PET scan, cardiac MRI, and chest computed tomography (CT), may provide additional valuable information in select populations. Ocular sarcoidosis is a marker for vascular endothelial dysfunction and increased arterial rigidity. Choroidal involvement is associated with an increased risk of cardiac disease requiring intervention. Cardiac disease continues to be underdiagnosed in patients with sarcoidosis, although it remains a leading cause of death. SUMMARY: Sarcoidosis is a systemic disease, and ophthalmologists should continually assess patients for extraocular manifestations. Although no screening guidelines exist, baseline ECGs on asymptomatic patients might identify those at risk for adverse cardiac events. Patients with symptoms of cardiac disease, including palpitations, chest pain, and dyspnea, should have an evaluation by a cardiologist.

Patient Surgical Outcomes When Surgery Residents Are the Primary Surgeon by Intensity of Surgical Attending Supervision in Veterans Affairs Medical Centers.

Objective: Using health records from the Department of Veterans Affairs (VA), the largest healthcare training platform in the United States, we estimated independent associations between the intensity of attending supervision of surgical residents and 30-day postoperation patient outcomes. Background: Academic leaders do not agree on the level of autonomy from supervision to grant surgery residents to best prepare them to enter independent practice without risking patient outcomes. Methods: Secondary data came from a national, systematic 1:8 sample of n = 862,425 teaching encounters where residents were listed as primary surgeon at 122 VA medical centers from July 1, 2004, through September 30, 2019. Independent associations between whether attendings had scrubbed or not scrubbed on patient 30-day all-cause mortality, complications, and 30-day readmission were estimated using generalized linear-mixed models. Estimates were tested for any residual confounding biases, robustness to different regression models, stability over time, and validated using moderator and secondary factors analyses. Results: After accounting for potential confounding factors, residents supervised by scrubbed attendings in 733,997 nonemergency surgery encounters had fewer deaths within 30 days of the operation by 14.2% [0.3%, 29.9%], fewer case complications by 7.9% [2.0%, 14.0%], and fewer readmissions by 17.5% [11.2%, 24.2%] than had attendings not scrubbed. Over the 15 study years, scrubbed surgery attendings may have averted an estimated 13,700 deaths, 43,600 cases with complications, and 73,800 readmissions. Conclusions: VA policies on attending surgeon supervision have protected patient safety while allowing residents in selected teaching encounters to have limited autonomy from supervision.

75 Years of the Historic Partnership Between the VA and Academic Medical Centers.

Background: The US Department of Veterans Affairs (VA) conducts the largest health professions education program in the country in partnership with academic medical, nursing, and associated health programs across the nation. After World War II, the VA was pressed to meet the increasing population of veterans needing health care and faced challenges in recruiting clinicians. Observations: The passage of 2 legislative actions, the Servicemen's Readjustment Act and Public Law 79-293, and a key policy memorandum set the foundation for the partnership between the VA and academic medical centers that led to improved medical care for veterans and expansion of health professions education for the VA and the nation. Conclusions: Since passage of these actions, the VA-academic health professions education partnership has grown to involve 113,000 trainees rotating through 150 VA medical centers annually from more than 1400 colleges and universities.

The Role of Screening for Asymptomatic Ocular Inflammation in Sarcoidosis.

PURPOSE: To determine the utility of routine screening ophthalmic exam in patients with systemic sarcoidosis and no history of uveitis. METHODS: Prospective, single-center, observational study conducted at Northwestern University from October 11, 2012 to October 1, 2020 of new patients with biopsy-proven systemic sarcoidosis and no history of uveitis, referred by medical subspecialists for screening ophthalmic exam. RESULTS: Forty-nine patients, with mean age of 51 ± 8.7 years, 59% female, 47% African American, 43% Caucasian, were enrolled. The majority (55%) had no ocular symptoms. The most common location of ocular involvement was the adnexa, in the form of conjunctival nodules (62%) and aqueous tear deficiency (23%). Intraocular inflammation was detected in 6 patients (13%); only 2 had active disease requiring treatment (4%). No asymptomatic patient had ocular involvement necessitating treatment. CONCLUSION: Screening exams are indicated in sarcoidosis patients with ocular symptoms. No benefit of screening was demonstrated in asymptomatic patients.

Presentation and outcome of patients with punctate inner choroidopathy at a tertiary referral center.

PURPOSE: To describe the clinical course of patients with punctate inner choroidopathy seen at the University of Illinois, with emphasis on development of choroidal neovascularization (CNV). METHODS: Patients with a diagnosis of punctate inner choroidopathy were identified retrospectively. The diagnosis was made clinically based on findings of multiple, small "punched-out" lesions in the posterior pole without intraocular inflammation. Medical records were evaluated for evidence of CNV. RESULTS: Twelve patients with a diagnosis of punctate inner choroidopathy were identified. Average age at presentation was 32 years (range, 24-52 years). Eleven were women, and 11 were white. Eleven patients had available refractive data: 10 were myopic. Eight had CNV at initial presentation, and 1 later developed CNV. Eight had follow-up averaging 4 years (range, 1.4-9.6 years). Of these, five had CNV at initial presentation, and five developed new CNV. Four had multiple CNV membranes. All 6 patients with follow-up of ≥ 3 years had visual acuity at 3 years postpresentation of >20/40 in at least 1 eye. Four had visual acuity >20/40 bilaterally. CONCLUSION: Of punctate inner choroidopathy patients in this series, 75% had CNV, and more than 30% developed multiple neovascular membranes. With treatment, all patients with follow-up of ≥ 3 years had >20/40 vision in at least 1 eye.

Subretinal fluid in acute posterior multifocal placoid pigment epitheliopathy.

PURPOSE: The purpose of this study was to describe the clinical finding of macular subretinal fluid by optical coherence tomography in patients with acute posterior multifocal placoid pigment epitheliopathy. METHODS: Patients with acute posterior multifocal placoid pigment epitheliopathy were identified, and those with macular serous retinal detachment noted clinically and confirmed by optical coherence tomography are described. RESULTS: Of 8 patients with acute posterior multifocal placoid pigment epitheliopathy evaluated by the uveitis service at the Illinois Eye and Ear Infirmary between 2003 and 2008, 4 eyes of 3 patients presented with macular subretinal fluid. Confirmatory optical coherence tomography was performed in two patients. CONCLUSION: Acute posterior multifocal placoid pigment epitheliopathy may present clinically with macular subretinal fluid. This finding can be confirmed and monitored with optical coherence tomography.

Ocular sarcoidosis misdiagnosed as primary intraocular lymphoma.

PURPOSE: The purpose of this study was to describe patients initially carrying a diagnosis of primary intraocular lymphoma who were ultimately diagnosed with ocular sarcoidosis. METHODS: The medical records of patients evaluated between 1995 and 2007 fitting the criteria described earlier were identified, and pertinent clinical findings allowing for the diagnosis of sarcoidosis are described. RESULTS: Nine patients between the ages of 52 and 83 were referred with a diagnosis of primary intraocular lymphoma but were ultimately diagnosed with sarcoidosis. The most common clinical signs found in these patients that are atypical for primary intraocular lymphoma but common in sarcoidosis were multifocal choroiditis (n = 7) and cystoid macular edema (n = 6). Additional findings included keratic precipitates, posterior synechiae, and Koeppe nodules. Chest computerized tomography was consistent with sarcoidosis in seven of eight tested patients, and five of these patients had normal chest x-rays. Other findings included elevated angiotensin-converting enzyme and/or lysozyme, and biopsy revealing noncaseating granulomas. CONCLUSION: Although primary intraocular lymphoma should always be in the differential diagnosis of older patients who present with signs of ocular inflammation, ophthalmologists must also consider other etiologies, including sarcoidosis. A chest computerized tomography may be helpful in the diagnosis, particularly when laboratory findings are supportive of sarcoidosis.

Treating uveitis-associated hypotony with pars plana vitrectomy and silicone oil injection.

PURPOSE: The purpose of this study was to determine the effect on intraocular pressure (IOP) and visual acuity of treating uveitis-related hypotony in patients with vitrectomy and intravitreal silicone oil injection. METHODS: Patients who underwent pars plana vitrectomy and silicone oil injection for uveitis-associated hypotony treatment were identified retrospectively. The primary outcome was maintaining an IOP of > or =5 mmHg. Visual acuity improvement was defined as an increase in > or =2 lines of acuity. RESULTS: Twelve eyes of 10 patients were identified. Median preoperative IOP was 2 mmHg (range: 0-7 mmHg). Two of 12 eyes had an IOP of > or =5 mmHg at presentation. The number of eyes with an IOP of > or =5 mmHg was 7 of 12 eyes (58%) at 1 month, 4 of 12 eyes (33%) at 3 months, 6 of 12 eyes (50%) at 6 months, and 3 of 9 eyes (33%) at 1 year. Five of 12 eyes (42%) were reinjected between 1 and 3 times with silicone oil for recurring hypotony. Median presenting Snellen visual acuity was counting fingers (range: 20/125 to light perception). Seven of 9 eyes (78%) maintained their preoperative vision at 1 year. CONCLUSION: Intraocular pressure elevated modestly in most patients in this series. However, results were often transient, and some eyes required repeated silicone oil injections. Although silicone oil is reasonable to consider for the treatment and maintenance of IOP in patients with ocular hypotony secondary to uveitis, better treatments are needed.

Pharmacological characterization, localization, and regulation of ionotropic glutamate receptors in skate horizontal cells.

Glutamate is believed to be the primary excitatory neurotransmitter in the vertebrate retina, and its fast postsynaptic effects are elicited by activating NMDA-, kainate-, or AMPA-type glutamate receptors. We have characterized the ionotropic glutamate receptors present on retinal horizontal cells of the skate, which possess a unique all-rod retina simplifying synaptic circuitry within the outer plexiform layer (OPL). Isolated external horizontal cells were examined using whole-cell voltage-clamp techniques. Glutamate and its analogues kainate and AMPA, but not NMDA, elicited dose-dependent currents. The AMPA receptor antagonist GYKI 52466 at 100 microm abolished glutamate-elicited currents. Desensitization of glutamate currents was removed upon coapplication of cyclothiazide, known to potentiate AMPA receptor responses, but not by concanavalin A, which potentiates kainate receptor responses. The dose-response curve to glutamate was significantly broader in the presence of the desensitization inhibitor cyclothiazide. Polyclonal antibodies directed against AMPA receptor subunits revealed prominent labeling of isolated external horizontal cells with the GluR2/3 and GluR4 antibodies. 1-Naphthylacetyl spermine, known to block calcium-permeable AMPA receptors, significantly reduced glutamate-gated currents of horizontal cells. Downregulation of glutamate responses was induced by increasing extracellular ion concentrations of Zn2+ and H+. The present study suggests that Ca2+-permeable AMPA receptors likely play an important role in shaping the synaptic responses of skate horizontal cells and that alterations in extracellular concentrations of calcium, zinc, and hydrogen ions have the potential to regulate the strength of postsynaptic signals mediated by AMPA receptors within the OPL.

Epidemiologic relationship between fuchs heterochromic iridocyclitis and the United States rubella vaccination program.

PURPOSE: To investigate an epidemiologic association between Fuchs heterochromic iridocyclitis (FHI) and the rubella vaccination program in the United States. DESIGN: Observational case series. METHODS: The percentages of patients with FHI, idiopathic chronic iridocyclitis, and idiopathic chronic granulomatous iridocyclitis at the University of Illinois were compared over time. Analysis of country of origin (United States vs foreign-born) was also performed. Findings were correlated with implementation of the rubella vaccination program in the United States in 1969. RESULTS: A total of 3,856 patients were seen between 1985 and 2005. Percentages of patients with FHI and idiopathic chronic granulomatous iridocyclitis born between 1919 and 1958 were similar (FHI 3.51% to 5.19%; idiopathic chronic granulomatous iridocyclitis 3.51% to 4.72%), with more variability in the idiopathic chronic iridocyclitis group (6.63% to 11.32%). A 64.8% reduction in FHI subjects was seen for those born the following decade (1959 to 1968). An additional 39.7% drop in FHI occurred in patients born between 1969 and 1978. Only one patient with FHI was born during the decade 1979 to 1988. The trend over time for FHI differed significantly from idiopathic chronic iridocyclitis (P = .0007) and idiopathic chronic granulomatous iridocyclitis (P = .0002), with no difference between the controls (P = .5437). The percentage of foreign-born patients with FHI increased after the institution of the rubella vaccination program (42% to 55%) compared with those born in previous decades (24% to 25%). This was not observed in the controls. CONCLUSION: FHI is less common in patients born since the introduction of the US rubella vaccination program, with a corresponding increase in percentage of foreign-born cases. This epidemiologic study supports a relationship between the rubella virus and FHI.

Bilateral eyelid ecchymoses and corneal crystals: an unusual presentation of multiple myeloma.

PURPOSE: This report describes a case of multiple myeloma that presented as bilateral eyelid ecchymoses and corneal crystals in the absence of widespread signs of systemic disease. METHODS: A 55-year-old man was found to have the sudden appearance of bilateral eyelid ecchymoses after he flexed 90 degrees at the waist. On examination, amyloid deposition was found in the area of the lid ecchymoses, and corneal crystals were dispersed centrally and peripherally throughout all levels of the cornea on slit lamp examination. Urinalysis revealed Bence-Jones proteins and free kappa light chains. Bone marrow plasmacytosis suggested multiple myeloma. RESULT: Ophthalmic and systemic findings led to a workup, which resulted in a diagnosis of multiple myeloma. CONCLUSION: Our case demonstrates that multiple myeloma may present clinically with ophthalmic findings such as bilateral eyelid ecchymoses and corneal crystals in the absence of widespread signs of disease. The ocular findings may be the first manifestations of disease. Therefore, a systemic workup should be performed on patients with this presentation, including urine and bone marrow analyses.

A case of hypopyon uveitis nonresponsive to steroid therapy and a review of anterior segment masquerade syndromes in childhood.

We report a case of hypopyon uveitis referred as inflammatory disease nonresponsive to corticosteroids. A detailed history revealed that the patient had been treated previously for acute lymphoblastic leukemia (ALL). Anterior chamber tap was consistent with recurrence of ALL. A review of pseudouveitis in the pediatric population is also presented.

Sarcoidosis: sex-dependent variations in presentation and management.

Sarcoidosis is an inflammatory disease with a wide range of clinical presentations. The manifestations and prognosis in sarcoidosis are dependent upon not only organ involvement but also age and sex. The purpose of this review is to describe the systemic and ocular manifestations of sarcoidosis with a specific focus on sex-dependent difference in presentation and management. Sarcoidosis is more common in women, particularly in patients who present after age of 50 years. Women with sarcoidosis are more likely to develop cystoid macular edema and the mortality rate is higher than that of men.

Correlation between clinical signs and optical coherence tomography with enhanced depth imaging findings in patients with birdshot chorioretinopathy.

IMPORTANCE: Birdshot chorioretinopathy (BCR) is a bilateral posterior uveitis that typically requires aggressive therapy to prevent loss of vision. Clinical signs of disease activity may be subtle and visual acuity is often preserved despite significant loss of visual function. Optical coherence tomography with enhanced depth imaging (OCT-EDI), a new technology that allows visualization of structures posterior to the retinal pigment epithelium, may be a useful tool to monitor disease activity in these patients. OBJECTIVE: To determine the correlation between symptoms and signs of disease activity in BCR and specific findings on OCT-EDI. DESIGN, SETTING, AND PARTICIPANTS: Retrospective medical record review of 14 patients treated for BCR in the uveitis clinic at Northwestern University. All patients underwent OCT-EDI (58 scans). Clinical symptoms of photopsias/vibrating vision and signs of macular edema, vitreous haze, and retinal vasculitis were graded; a second grading scale was developed for the evaluation of OCT-EDI. Individual scans of each eye of each patient at each point were graded in a masked fashion. EXPOSURE: Optical coherence tomography with EDI in BCR. MAIN OUTCOMES AND MEASURES: Spearman rank correlation of clinical measures to OCT-EDI measures. RESULTS: The most frequent score in each clinical category was 0 (inactive). In those BCR patients with symptoms (21 eye examinations), the subjective complaint of photopsias/vibrating vision was associated with the objective finding of suprachoroidal fluid on OCT-EDI (P = .003), and the frequency and severity of photopsias correlated with the thickness of the fluid band (Pearson product moment correlation, 0.39). Two of the clinical markers of disease activity measured in this study (vasculitis and vitreous haze) also showed a significant Spearman rank correlation with the presence and amount of suprachoroidal fluid on OCT-EDI (vasculitis, 0.45 [P < .001]; vitreous haze, 0.59 [P < .001]). CONCLUSIONS AND RELEVANCE: The presence of suprachoroidal fluid on OCT-EDI appears to correlate with the subjective complaints of photopsias in patients with BCR and other more easily assessed clinical features such as vasculitis and vitreous haze. Optical coherence tomography with EDI may be a useful tool for objective monitoring of BCR.

TNF inhibition for ophthalmic indications: current status and outlook.

BACKGROUND: Tumor necrosis factors (TNF) are a group of cytokines that play a role in systemic inflammation, stimulating the acute phase reaction. They are involved in systemic rheumatologic conditions such as rheumatoid arthritis and juvenile idiopathic arthritis, as well as ocular inflammatory conditions in the uveitis spectrum. Several drugs were developed to inhibit the action of TNF, thereby reducing inflammation. The three most commonly used TNF inhibitors in the US are etanercept, infliximab, and adalimumab. Newer drugs include certolizumab and golimumab. In this review, we discuss the differences in the mechanism of action, route of administration, indication, and efficacy of TNF inhibitors used in the treatment of ocular inflammation. METHODS: A review of the literature in the PubMed, MEDLINE, and Cochrane databases was conducted to identify clinical trials, comparative studies, case series, and case reports describing the use of tumor necrosis factor inhibitors in uveitis therapy. The search was limited to primary reports published in English with human subjects from 1990 to the present, yielding 5,238 manuscripts. In addition, referenced articles from the initial searches were hand searched to identify additional relevant reports. After title and abstract selection, duplicate elimination, and manual search, 69 papers were selected for analysis. Exclusion criteria included review articles and case reports on the efficacy of etanercept, infliximab, and adalimumab. Manuscripts with fewer than 20 study subjects were excluded if other larger studies existed on the use of the same drug for a particular indication. Studies with <6 months of patient follow-up were also excluded, except in the case where no other data were available. Articles meeting these criteria were then reviewed by the three authors for inclusion in this review. RESULTS: Tumor necrosis factor inhibitors have been shown to decrease inflammation associated with a number of rheumatologic conditions. Three of the five commercially available TNF inhibitors-etanercept, infliximab, and adalimumab-have been studied for their efficacy in treatment of ocular inflammation. Etanercept appears to be inadequate in controlling ocular inflammation and is not recommended for the treatment of uveitis. Infliximab and adalimumab, however, have shown encouraging results in multiple trials. Serious potential side effects such as infection, including reactivation of latent tuberculosis, malignancy, and demyelinating disease, may limit the use of TNF inhibitors in uveitis. Proper screening of patients prior to initiating these therapies may decrease these risks. DISCUSSION: Early success with infliximab and adalimumab has paved the way for new TNF inhibitors and other corticosteroid-sparing drugs to emerge in the treatment of ocular inflammation. Future studies are on the horizon to determine the long-term safety and efficacy of newer TNF inhibitors such as certolizumab and golimumab.

Behçet uveitis in the American midwest.

PURPOSE: Behçet disease is most common in Asia, the Middle East, and North Africa (Silk Road). The authors compare clinical presentation and course of Behçet uveitis in patients of Silk Road and non-Silk Road descent. METHODS: Retrospective review of patients evaluated at the University of Illinois January 1983-July 2010. RESULTS: Of 6134 new uveitis patients, 36 with available medical records met diagnostic criteria for Behçet disease. Ten (28%) were of Silk Road origin. Retinal vasculitis was the most common ocular manifestation, followed by panuveitis and retinitis. Ocular and systemic manifestations were similar between groups. Seventy-eight percent were treated with systemic immunosuppression, which reduced inflammatory attack rate. CONCLUSIONS: Behçet disease is uncommon in the midwestern United States. Three-quarters of patients in this series were not of Silk Road descent. Physicians should consider the diagnosis of Behçet disease in any patient with retinal vasculitis or uveitis regardless of ethnicity.