Paracentral Acute Middle Maculopathy in Pregnancy.

ABSTRACT: A 31-year-old previously healthy, pregnant woman presented with acute, unilateral, painless paracentral vision loss in the left eye. The results of neuroimaging studies were normal. Hypercoagulable workup was negative, and fundus examination showed no retinal emboli and no retinal vascular abnormalities. The patient had well-controlled blood pressure and did not have eclampsia/preeclampsia. Although a presumptive referral diagnosis of "optic neuritis" was made, optical coherence tomography (OCT) of the macula showed a hyperreflective band involving the inner nuclear layer, consistent with paracentral acute middle maculopathy (PAMM). Furthermore, OCT angiography (OCT-A) showed flow attenuation of the outer retinal capillary plexus, further supporting a diagnosis of PAMM in pregnancy. Clinicians should be aware of the benefit of OCT-A as a complement to macular OCT in the evaluation of acute monocular vision loss mimicking retrobulbar optic neuropathy, particularly when signs and findings of other retinal vasculopathy are absent. PAMM should be considered in pregnant patients with acute visual changes.

Paracentral Acute Middle Maculopathy Mimicking Retrobulbar Optic Neuropathy.

ABSTRACT: A 70-year-old woman presented with acute awareness of decreased color vision in her right eye. Fundus examination and a flash electroretinography (ERG) were both normal. A multifocal ERG (mfERG) however revealed foveal depression, and thinning of inner nuclear layer was noted on macular optical coherence tomography (OCT), and a diagnosis of resolved paracentral acute middle maculopathy was made. Clinicians should be aware of the complementary role of OCT and mfERG in unexplained acute central visual loss to distinguish retinal from neuro-ophthalmic etiologies. Structural ocular imaging with OCT shows features of inner, middle, and outer retinal localizations to the visual loss.

Necrotizing Fasciitis of the Periorbital Region Complicated by Combined Central Retinal Artery Occlusion, Central Retinal Vein Occlusion, and Posterior Ciliary Occlusion.

A 50 year-old man on immunosuppressive agents presented with left eye vision loss, periorbital swelling, pain, and ophthalmoplegia. The patient was clinically found to have a central retinal artery and vein occlusion. A CT scan was performed which demonstrated intraorbital fat stranding, however the patient lacked sinus disease. The etiology of the orbital infection was held in question. The area was debrided in the operating room, and the specimen demonstrated group A streptococcal species consistent with necrotizing fasciitis. Periorbital necrotizing fasciitis should be suspected in patients with rapidly progressive orbital symptoms without sinus disease as lack of surgical intervention can result in poor outcomes. The unusual aspect to this case is the mechanism of vision loss, as the authors hypothesize that there was vascular infiltration of the infection resulting in the central retinal artery occlusion and central retinal vein occlusion which have not been previously reported secondary to necrotizing fasciitis of the orbit.

Multimodal imaging of white and dark without pressure fundus lesions.

PURPOSE: To describe multimodal imaging findings in patients with dark or white without pressure lesions of the fundus. METHODS: Retrospective observational case series of 10 patients with white or dark without pressure lesions. We analyzed multimodal imaging using spectral domain optical coherence tomography, color and near-infrared fundus photography, and fundus autofluorescence imaging to explore the findings associated with these lesions. RESULTS: All patients had geographic dark or white lesions on clinical examination and color photography, which were either hyporeflective or hyperreflective on near-infrared reflectance imaging, respectively. On optical coherence tomography, these lesions correlated with an abrupt change of the photoreceptor reflectivity, with relative hyporeflectivity of photoreceptor zones (ellipsoid and interdigitation zones, as well as outer segments) within the dark, and relative hyperreflectivity within white lesions. Ten patients underwent fundus autofluorescence, which showed well-defined zones of relative hypo-autofluorescence within the lesion, compared with neighboring uninvolved regions, whether dark or white without pressure. In two patients who had a lesion combining white and dark without pressure, we observed the transition in photoreceptor reflectivity from the dark lesion (hyporeflective) to the white lesion (hyperreflective), relative to the surrounding retina. CONCLUSION: Both white and dark without pressure lesions are associated with changes in outer retinal reflectivity on optical coherence tomography, which occur in opposite directions compared with the surrounding unaffected areas. In the face of normal visual field testing to date, the clinical significance of this finding remains uncertain. Recognition of the optical coherence tomography appearance will help clinicians avoid unnecessary workup of these patients for outer retinal dystrophy or degeneration.

Automated analysis of diabetic retinopathy images: principles, recent developments, and emerging trends.

Diabetic retinopathy (DR) is a vision-threatening complication of diabetes. Timely diagnosis and intervention are essential for treatment that reduces the risk of vision loss. A good color retinal (fundus) photograph can be used as a surrogate for face-to-face evaluation of DR. The use of computers to assist or fully automate DR evaluation from retinal images has been studied for many years. Early work showed promising results for algorithms in detecting and classifying DR pathology. Newer techniques include those that adapt machine learning technology to DR image analysis. Challenges remain, however, that must be overcome before fully automatic DR detection and analysis systems become practical clinical tools.

Post-traumatic amaurosis secondary to paraophthalmic internal carotid artery pseudoaneurysm treated with pipeline embolization device.

During evaluation for monocular visual loss, a 48-year-old woman was found to have a posttraumatic paraophthalmic internal carotid artery (ICA) pseudoaneurysm. She underwent reconstruction of the ophthalmic segment of the right ICA with a Pipeline embolization device but her vision did not return.

Mosaics versus Early Treatment Diabetic Retinopathy seven standard fields for evaluation of diabetic retinopathy severity.

PURPOSE: To compare agreement between mosaicked and seven field photographs for classification of the diabetic retinopathy (DR) severity. METHODS: Mosaic digital (MosD) images were compared with seven field stereo film (7FF) and stereo digital (7FD) photographs from a 152-eye cohort with full-spectrum Early Treatment of Diabetic Retinopathy severity levels for agreement on severity level, DR presence with ascending severity thresholds, DR index lesion presence, and classification repeatability. RESULTS: There was a substantial agreement classifying the Early Treatment Diabetic Retinopathy Study DR severity level between MosD and 7FF (kunweighted = 0.59, klinear weighted = 0.83), MosD and 7FD (κ = 0.62, κ weighted = 0.86), and 7FD and 7FF (κ = 0.62, κ weighted = 0.86) images. Marginal homogeneity analyses found no significant difference between MosD and 7FF (P = 0.44, Bhapkar's test). Kappa between MosD and 7FF ranged from 0.75 to 0.91 for the presence or absence of DR at 8 ascending severity thresholds. Repeatability among readers using MosD images was similar to repeatability among those using 7FF or 7FD. Repeatability among readers using MosD and 7FF images at various severity thresholds was similar. Kappa between MosD and 7FF grading for identifying DR lesions ranged from 0.61 to 1.00. CONCLUSION: Mosaic images are generally comparable with standard seven-field photographs for classifying DR severity.

Telehealth practice recommendations for diabetic retinopathy, second edition.

Ocular telemedicine and telehealth have the potential to decrease vision loss from DR. Planning, execution, and follow-up are key factors for success. Telemedicine is complex, requiring the services of expert teams working collaboratively to provide care matching the quality of conventional clinical settings. Improving access and outcomes, however, makes telemedicine a valuable tool for our diabetic patients. Programs that focus on patient needs, consider available resources, define clear goals, promote informed expectations, appropriately train personnel, and adhere to regulatory and statutory requirements have the highest chance of achieving success.

Giant choroidal nevus clinical features and natural course in 322 cases.

PURPOSE: Evaluation of clinical features and natural course of giant choroidal nevi (diameter >or=10 mm). DESIGN: Retrospective observational case series. PARTICIPANTS: We included 322 eyes of 322 patients. METHODS: Clinic-based study of tumor features, tumor outcome, and vision outcome. Kaplan-Meier analysis was used to assess time to transformation into melanoma. Cox proportional hazards regressions evaluated clinical factors predictive of nevus transformation into melanoma and nevus-related decreased vision (defined as <20/20 and unrelated to other eye pathology). MAIN OUTCOME MEASURES: Transformation of giant choroidal nevus into melanoma and nevus-related decreased vision. RESULTS: A medical record review of 4100 patients diagnosed with choroidal nevus identified 322 (8%) giant choroidal nevi. Median nevus basal diameter was 11 mm (range, 10-24). Median thickness was 1.9 mm (range, 0-4.4). Related retinal findings included drusen overlying nevus (n = 261 [81%]), subretinal fluid (n = 26 [8%]), orange pigment (n = 4 [1%]), retinal pigment epithelial (RPE) detachment (n = 6 [2%]), hyperplasia (n = 48 [15%]), fibrous metaplasia (n = 48 [15%]), atrophy (n = 63 [20%]), or trough (n = 6 [2%]). Kaplan-Meier analysis estimated transformation into melanoma in 13% at 5 years and 18% at 10 years. Multivariate analyses revealed factors predictive of transformation into melanoma including involvement or close proximity to the foveola (P = 0.017) and acoustic hollowness (P = 0.052). Nevus-related decreased vision was found in 2.2% of eyes at initial visit and 3.7% at final visit (median 41 and mean 61 months follow-up). Factors associated with nevus-related decreased vision at initial visit included subretinal fluid (P = 0.001), involvement or close proximity to foveola (P = 0.005), RPE detachment (P = 0.033), and nevus-related choroidal neovascular membrane (P = 0.044). Factors predictive of nevus-related decreased vision at final visit included involvement or close proximity to the foveola (P = 0.001) and presence of symptoms at the initial visit (P = 0.032). CONCLUSIONS: Giant choroidal nevi can clinically resemble choroidal melanoma but show features of chronicity, such as overlying drusen and RPE alterations. Over time, 18% transformed into melanoma, underscoring the importance of life-long surveillance.

Grading diabetic retinopathy severity from compressed digital retinal images compared with uncompressed images and film.

PURPOSE: To compare research grading of diabetic retinopathy (DR) severity level from compressed digital images versus uncompressed images and film. METHODS: Compressed (JPEG2000, 37:1) digital images (C) were compared with uncompressed digital (U) and film (F) stereoscopic photographs from a 152-eye cohort with full-spectrum Early Treatment Diabetic Retinopathy Study severity levels for agreement on severity level, DR presence with ascending severity threshold, presence of DR index lesions, and repeatability of grading. RESULTS: Classification of Early Treatment Diabetic Retinopathy Study severity levels from C images agreed substantially with results from F images (κ = 0.60, κ(w) [linear weighted] = 0.86) and uncompressed digital images (κ = 0.76, κ(w) = 0.92). For agreement of uncompressed digital versus F images, κ = 0.62 and κ(w) = 0.86. Distribution of Early Treatment Diabetic Retinopathy Study levels was not significantly different between C and F images (P = 0.09, Bhapkar's test for marginal homogeneity). For presence/absence of DR at 8 ascending severity thresholds, agreement between C and F was "almost perfect" (κ ≥ 0.8). Agreement on severity level between readers with C images was at least as good as that with uncompressed digital image or F. Repeatability of severity threshold grading between readers was similar using C or F images. For identifying individual DR lesions, agreement between C and F ranged from "moderate" to "perfect." Agreement of grading venous beading from C was slightly lower than from F. CONCLUSION: Full Early Treatment Diabetic Retinopathy Study scale DR severity level grading using C images is comparable to that using U images or film.

Comparison of time-domain OCT and fundus photographic assessments of retinal thickening in eyes with diabetic macular edema.

PURPOSE: To explore the correlation between optical coherence tomography (OCT) and stereoscopic fundus photographs (FP) for the assessment of retinal thickening (RT) in diabetic macular edema (DME) within a clinical trial. METHODS: OCT, FP, and best corrected visual acuity (VA) measurements were obtained in both eyes of 263 participants in a trial comparing two photocoagulation techniques for DME. Correlation coefficients (r) were calculated comparing RT measured by OCT, RT estimated from FP, and VA. Principal variables were central subfield retinal thickness (CSRT) obtained from the OCT fast macular map and DME severity assessed by a reading center using a seven-step photographic scale combining the area of thickened retina within 1 disc diameter of the foveal center and thickening at the center. RESULTS: Medians (quartiles) for retinal thickness within the center subfield by OCT at baseline increased from 236 (214, 264) microm in the lowest level of the photographic scale to 517 (455, 598) microm in the highest level (r = 0.67). However, CSRT interquartile ranges were broad and overlapping between FP scale levels, and there were many outliers. Correlations between either modality and VA were weaker (r = 0.57 for CSRT, and r = 0.47 for the FP scale). OCT appeared to be more reproducible and more sensitive to change in RT between baseline and 1 year than was FP. CONCLUSIONS: There was a moderate correlation between OCT and FP assessments of RT in patients with DME and slightly less correlation of either measure with VA. OCT and FP provide complementary information but neither is a reliable surrogate for VA.

Retinal thickness on Stratus optical coherence tomography in people with diabetes and minimal or no diabetic retinopathy.

PURPOSE: To evaluate optical coherence tomography (OCT) thickness of the macula in people with diabetes but minimal or no retinopathy and to compare these findings with published normative data in the literature from subjects reported to have no retinal disease. DESIGN: Cross-sectional study. METHODS: In a multicenter community- and university-based practices setting, 97 subjects with diabetes with no or minimal diabetic retinopathy and no central retinal thickening on clinical examination and a center point thickness of 225 microm or less on OCT (Stratus OCT; Carl Zeiss Meditec, Dublin, California, USA) were recruited. Electronic Early Treatment of Diabetic Retinopathy Study best-corrected visual acuity, seven-field stereoscopic color fundus photographs, and Stratus OCT fast macular scan were noted. Main outcome measures were central subfield (CSF) thickness measured on Stratus OCT. RESULTS: On average, CSF thickness was 201 +/- 22 microm. CSF thickness was significantly greater in retinas from men than retinas from women (mean +/- standard deviation, 209 +/- 18 microm vs 194 +/- 23 microm; P < .001). After adjusting for gender, no additional factors were found to be associated significantly with CSF thickness (P > .10). CONCLUSIONS: CSF thicknesses on Stratus OCT in people with diabetes and minimal or no retinopathy are similar to thicknesses reported from a normative database of people without diabetes. CSF thickness is greater in men than in women, consistent with many, but not all, previous reports. Studies involving comparisons of retinal thickness with expected norms should consider different mean values for women and men.

Cost-effectiveness analysis of telemedicine to evaluate diabetic retinopathy in a prison population.

OBJECTIVE: A cost-effectiveness analysis was conducted to investigate the clinical and economic impact of teleophthalmology in evaluating diabetic retinopathy in prison inmates with type 2 diabetes. RESEARCH DESIGN AND METHODS: Based on a hypothetical teleophthalmology system to evaluate diabetic retinopathy patients with type 2 diabetes in a prison care setting, a Markov decision model was developed with probability and cost data derived primarily from published epidemiological and outcome studies. A 40-year-old African-American man with type 2 diabetes was used as a reference case subject. The number of quality-adjusted life-years (QALYs) gained was used as the clinical outcome, and the cost in U.S. dollars from the year 2003 was used as the economic outcome. Teleophthalmology and nonteleophthalmology strategies were compared using an expected QALYs calculation and two types of sensitivity analyses: probabilistic and traditional n-way sensitivity analyses. RESULTS: The teleophthalmology strategy dominates in the cost-effectiveness analysis for the reference case subject: 16,514/18.73 dollars QALYs for teleophthalmology and 17,590/18.58 dollars QALYs for nonteleophthalmology. Ninety percent of the Monte Carlo simulations showed cost effectiveness (annual cost/QALYs < or = 50,000 dollars) in the teleophthalmology strategy based on an assumed inmate population. Teleophthalmology is the better strategy if the number of diabetic inmates in the prison community is >500. CONCLUSIONS: Our cost-effectiveness analysis demonstrates that teleophthalmology holds great promise to reduce the cost of inmate care and reduce blindness caused by diabetic retinopathy in type 2 diabetic patients.

Course of cytomegalovirus retinitis in the era of highly active antiretroviral therapy: 2. Second eye involvement and retinal detachment.

PURPOSE: To describe the course of cytomegalovirus (CMV) retinitis in patients with AIDS in the era of highly active antiretroviral therapy (HAART). DESIGN: Multicenter, prospective, observational study. PARTICIPANTS: Two hundred seventy-one patients with AIDS and CMV retinitis. METHODS: Follow-up every 3 months with medical history, ophthalmologic examination, laboratory testing, and fundus photographs. MAIN OUTCOME MEASURE: Second (contralateral) eye involvement among patients with unilateral disease and retinal detachment (RD). RESULTS: The overall rate of second eye involvement among patients with unilateral CMV retinitis was 0.07 per person-year (PY); among those with CD4+ T-cell counts of <50/microl it was 0.34/PY, compared with 0.02/PY among those with CD4+ T-cell counts of > or =200/microl (P<0.0001). Risk factors for contralateral eye involvement included low CD4+ T-cell count and detectable CMV load. The overall rate of RD was 0.06/PY; among those with CD4+ T-cell counts of <50/microl it was 0.30/PY, compared with 0.02/PY among those with CD4+ T-cell counts of > or =200/microl (P<0.0001). Risk factors for RD included a low CD4+ T-cell count and larger area of CMV retinitis. CONCLUSIONS: Compared with the rates reported in the pre-HAART era of second eye involvement (approximately 0.40/PY) and RD (approximately 0.50/PY), the rates of these events were reduced among patients in the HAART era. However, among patients with CD4+ T-cell counts of <50/microl, the rates were more similar to those from the pre-HAART era.

Course of cytomegalovirus retinitis in the era of highly active antiretroviral therapy: 1. Retinitis progression.

PURPOSE: To describe the course of cytomegalovirus (CMV) retinitis in patients with AIDS in the era of highly active antiretroviral therapy (HAART). DESIGN: Multicenter, prospective, observational study. PARTICIPANTS: Two hundred seventy-one patients with AIDS and CMV retinitis. METHODS: Follow-up every 3 months with medical history, ophthalmologic examination, laboratory testing, and fundus photographs. Photographs were evaluated for relapse of the retinitis (progression) by graders at a centralized reading center. MAIN OUTCOME MEASURE: Retinitis progression (movement of the border of a CMV lesion > or =750 microm over a > or =750-microm front or occurrence of a new lesion one-quarter disc area or more in size). RESULTS: The overall rate of retinitis progression was 0.10/person-year (PY); among those with CD4+ T-cell counts of <50/microl, it was 0.58/PY, compared to 0.02/PY among those with CD4+ T-cell counts of > or =200/microl (P<0.0001). In the multivariate analysis, significant risk factors for retinitis progression included a low CD4+ T-cell count, positive CMV load, longer time from AIDS diagnosis, and low Karnofsky score. CONCLUSIONS: Compared with the rate of retinitis progression (approximately 3.0/PY) reported in the pre-HAART era, the rate of retinitis progression was reduced among patients in the HAART era, even among those with low CD4+ T-cell counts, who might be expected to behave most like patients from the pre-HAART era. However, these events also occurred among patients with high CD4+ T-cell counts and presumed immune recovery. Continued ophthalmologic follow-up of patients with immune recovery is recommended to detect early retinitis progression.