Optical coherence tomography angiography in Purtscher-like retinopathy associated with dermatomyositis : a case report

Purpose: To describe a multimodal imaging diagnosis of retinopathy in dermatomyositis. Case presentation: A 21-year-old white woman with a history of fatigue and a cutaneous rash complained of visual impairment in her left eye. A funduscopic examination showed multiple confluent cotton-wool spots in both eyes. Swept source-optical coherence tomography presented macular edema in both eyes; optical coherence tomography angiography revealed superficial and deep capillary occlusion in all areas affected by cotton-wool spots; and fluorescein angiography showed vascular walls enhancement, veins dilatation, and capillary leakage. After large doses of intravenously administered glucocorticoid therapy, followed by a cyclophosphamide regimen, best corrected visual acuity returned to 20/20 in both eyes. Conclusions: This case report presents optical coherence tomography angiography clinical findings in a rare case of dermatomyositis-associated retinopathy, remarking the importance of a multi-imaging approach for a correct diagnosis and treatment of eye injuries, in order to avoid serious complications and permanent sequelae

OCT Analysis of Retinal Pigment Epithelium in Myopic Choroidal Neovascularization: Correlation Analysis with Different Treatments

Objective: The objective of this study was to analyze the status of the retinal pigment epithelium (RPE) by means of the spectral domain optical coherence tomography (SD-OCT) overlying the myopic neovascular lesions in the involutive phase, looking for any correlations between the status of the RPE and the size of the lesions and the type and duration of the treatment. Methods: SD-OCT examinations of 83 consecutive patients with myopic choroidal neovascularization (CNV) were reviewed and divided into two groups: group A, patients with CNV characterized by uniformity of the overlying RPE, and group B, patients with CNV characterized by non-uniformity of the overlying RPE. Results: The median lesion area, major diameter, and minimum diameter were, respectively, 0.42 mm2 (0.30–1.01 mm2), 0.76 mm2 (0.54–1.28 mm2), and 0.47 mm2 (0.63–0.77 mm2) in group A, and 1.60 mm2 (0.72–2.67 mm2), 1.76 mm2 (1.13–2.23 mm2), and 0.98 mm2 (0.65–1.33 mm2) in group B. These values were lower in group A than in group B (p < 0.001). The number of treatments with a period free of disease recurrence for at least 6 months was greater (p < 0.010) in group B (6.54 ± 2.82) than in group A (3.67 ± 2.08), and treatments include intravitreal anti-vascular endothelial growth factor injection, photodynamic therapy, or both. Conclusions: Our results showed that the size of myopic neovascular lesion influences the development of a uniform RPE above the lesion and therefore the disease prognosis. The presence of uniform RPE was found to be extremely important in the follow-up of patients with myopic CNV, as it influences the duration of the disease and the number of treatments required

Choroidal abnormalities detected by near-infrared reflectance imaging as a new diagnostic criterion for neurofibromatosis 1

Objective: To investigate in a large sample of consecutive patients with neurofibromatosis type 1 (NF1) the possibility of including the presence of choroidal abnormalities detected by near-infrared reflectance (NIR) as a new diagnostic criterion for NF1. Design: Cross-sectional evaluation of a diagnostic test. Participants and Controls: Ninety-five consecutive adult and pediatric patients (190 eyes) with NF1, diagnosed based on the National Institutes of Health (NIH) criteria. Controls included 100 healthy age- and gender-matched control subjects. Methods: Confocal scanning laser ophthalmoscopy was performed for each subject, investigating the presence and the number of choroidal abnormalities. Main Outcome Measures: Sensitivity, specificity, and diagnostic accuracy for the different cutoff values of the criterion choroidal nodules detected by NIR compared with the NIH criteria. Results: Choroidal nodules detected by NIR imaging were present in 79 (82%) of 95 of the NF1 patients, including 15 (71%) of the 21 NF1 pediatric patients. Similar abnormalities were present in 7 (7%) of 100 healthy subjects, including 2 (8%) of the 25 healthy pediatric subjects. The highest accuracy was obtained at the cutoff value of 1.5 choroidal nodules detected by NIR imagery. Sensitivity and specificity of the examination at the optimal cutoff point were 83% and 96%, respectively. Diagnostic accuracy was 90% in the overall population and 83% in the pediatric population. Both of these values were in line with the most common NIH diagnostic criteria. Conclusions: Choroidal abnormalities appearing as bright patchy nodules detected by NIR imaging frequently occurred in NF1 patients. The present study shows that NIR examination to detect choroidal involvement should be considered as a new diagnostic criterion for NF1

Glacier features influencing the presence and abundance of supraglacial trees: the case study of the miage debris-covered glacier (Mont Blanc Massif, Italian Alps)

The number of debris-covered glaciers featuring supraglacial tree vegetation is increasing worldwide, as a response of high mountain environments to the current climate warming. At the debriscovered surface of these glaciers, trees can be found thus giving peculiar landscape and ecosystems. Their distribution is not homogeneous, thus suggesting that some glacier parameters influence germination and growth of trees. This study was performed on the widest Italian debris-covered glacier, the Miage Glacier in the Mont Blanc massif, where herbaceous and tree vegetation is present at the surface of the glacier tongue. We analyzed the ablation area in the range from 1730 m to 2400 m a.s.l., where a quite continuous debris coverage is present and colonized by trees (mainly Larix decidua Mill. and Picea abies Karst), also reaching an age of 60 years close to the terminus. By remote sensing investigations and through field surveys we obtained a record of glacier parameters (debris thickness, debris-surface temperature, slope, aspect, elevation, ablation rate, surface velocity, debris-NDMI, variation in ice thickness over several years) to be analyzed with respect to the presence and abundance of trees in 15 plots (plot size: 15 m x 15 m). Our results show that supraglacial trees are present at the Miage Glacier: 1) whenever exceeding a debris thickness threshold ( 6519 cm), 2) with a quite gentle slope ( 64 22\ub0), 3) with a low glacier surface velocity ( 64 7.0 m/year), 4) where the ice thinning due to surface ablation is moderate (ranging between -1.8 m/year and -0.7 m/year) and 5) where the vertical changes due to glacier dynamics are positive (i.e. prevalent increase due to both slow debris accumulation and then preservation of ice flow inputs that we found ranging from +7 m and +28 m over a period 28 years long). The analysis of the same parameters, conducted on other debris-covered glaciers featuring supraglacial trees, may provide new data in order to evaluate if such conditions are local ones or if they are actual and general factors driving germination and growth of trees

Assessing glacier features supporting supraglacial trees : a case study of the Miage debris-covered Glacier (Italian Alps)

The number of debris-covered glaciers featuring supraglacial trees is increasing worldwide as a response of high mountain environments to climate warming. Generally, their distribution on the glacier surface is not homogeneous, thus suggesting that some glacier parameters influence germination and growth of trees. In this study, we focused our attention on the widest Italian debris-covered glacier, the Miage Glacier (Mont Blanc massif). We analyzed the ablation area in the range from 1730 to 2400 m a.s.l. where continuous debris coverage is present and trees are found. Using data obtained by remote sensing investigations and field surveys, we defined a record of glacier parameters to be analyzed with respect to the presence and abundance of trees. We found that supraglacial trees are present at the Miage Glacier (1) whenever exceeding a debris thickness threshold (\u2a7e19 cm), (2) with a gentle slope (\u2a7d10\ub0), (3) with a low glacier surface velocity (\u2a7d7.0 m/yr), and (4) where the vertical changes due to glacier dynamics are positive (i.e. prevalent increase ranging between +7 and +28 m over 28 years due to both slow debris accumulation and preservation of ice flow inputs). The statistical analysis supports our findings. The analysis of the same parameters might be conducted on other debris-covered glaciers featuring supraglacial trees, in order to evaluate whether such conditions are local ones or whether they are general factors driving germination and growth of trees. By identifying the features supporting the presence and growth of trees in these environments, and their thresholds, a contribution is given for a better understanding of the importance of debris-covered glaciers and, in general, of debris-covered ice, as a refuge for trees during glacial and warm intervals of the Holocene

Purtscher-like retinopathy in septicemic disseminated intravascular coagulation associated with nephrotic syndrome

Purpose: To describe a case of severe Purtscher-like retinopathy during an episode of septicemic diffused intravascular coagulation (DIC) in a child with severe nephrotic syndrome. Methods: Case report. Results: A 5-year-old girl with a history of steroid-sensitive nephrotic syndrome was admitted for worsening symptoms of the systemic disease. Laboratory studies revealed evidence of DIC during an episode of septicemia. Ten days later, she had a sudden and severe bilateral visual loss. Her visual acuity was hand motion in either eye. Fundus examination showed ischemic retinal whitening and retinal hemorrhages. Fluorescein angiography revealed obstruction of arterioles and venules at the posterior pole. Three weeks later, ischemic retinal blanching and hemorrhages resolved in both eyes; visual acuity improved to 20/250 and 20/200 in right and left eye, respectively. No further functional improvement was noted after 3 months, due to diffuse thinning of the inner retina architecture as shown by optical coherence tomography. Conclusions: Purtscher-like retinopathy can occur in patients with septicemic DIC and nephrotic syndrome

MULTIMODAL IMAGING IN DEFEROXAMINE RETINOPATHY

PURPOSE: To describe macular lesions in patients with deferoxamine (DFO) retinopathy, and to follow their clinical course using multimodal imaging. METHODS: The authors retrospectively reviewed charts and multimodal imaging of 20 patients with \u3b2-thalassemia diagnosed with DFO retinopathy (40 eyes) after a minimum of 10 years of DFO treatment. Imaging included fundus photography, near-infrared reflectance and fundus autofluorescence imaging on confocal laser scanning ophthalmoscope, and spectral domain optical coherence tomography. RESULTS: Mean age of the 20 patients was 45 years, and mean duration of subcutaneous DFO therapy was 32 years (range, 20-52 years). Ten patients (50%) showed different types of pattern dystrophy-like fundus changes, including butterfly shaped-like (n = 3), fundus flavimaculatus-like (n = 3), fundus pulverulentus-like (n = 3), and vitelliform-like (n = 1) changes. Ten patients (50%) presented only minimal changes in the macula; these patients were significantly younger than patients presenting other patterns (P = 0.023). Confocal laser scanning ophthalmoscope and spectral domain optical coherence tomography showed that these abnormalities were more diverse and widespread than expected by ophthalmoscopy. Abnormal fundus autofluorescence and/or near-infrared reflectance signals corresponded to accumulation of material located within the outer retina or in the Bruch membrane-retinal pigment epithelium (RPE) complex on spectral domain optical coherence tomography. Follow-up examinations during a 40-month period revealed progressive development of RPE atrophy in areas of pattern dystrophy-like changes. CONCLUSION: DFO retinopathy included a variety of pattern dystrophy-like changes or minimal changes affecting the RPE-Bruch membrane-photoreceptor complex. Multimodal imaging demonstrated that fundus changes were more diverse and widespread than expected from ophthalmoscopy. Consistently with previous histologic description of DFO retinopathy, multimodal imaging confirmed that photoreceptor outer-derived retinoids, various fluorophores, and RPE displacement or clumping are involved in DFO retinopathy, finally leading to frank RPE atrophy in most cases of pattern dystrophy-like changes