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Actas Dermo-sifiliograficas Sep 2020
Topics: Angioid Streaks; Breast Diseases; Elasticity Imaging Techniques; Fluorescein Angiography; Humans
PubMed: 32531239
DOI: 10.1016/j.ad.2018.09.028 -
The British Journal of Ophthalmology Dec 1981Forty-three additional members of a family described originally by Sorsby and Mason have been examined in order to typify pseudoinflammatory macular dystrophy more...
Forty-three additional members of a family described originally by Sorsby and Mason have been examined in order to typify pseudoinflammatory macular dystrophy more accurately. Six new affected members were identified by history alone and a further 7 were examined. Vision is lost from disciform macular degeneration generally during the 5th decade of life. Thereafter peripheral degeneration occurs in some affected members. Before loss of vision the fundus changes include fine drusen-like deposits at the level of Bruch's membrane, angioid streaks, and plaque-like deposits of yellow subretinal material in the macular region. These changes are different from those seen in dominant drusen.
Topics: Adult; Diagnosis, Differential; Female; Fluorescein Angiography; Humans; Macular Degeneration; Male; Middle Aged; Pedigree
PubMed: 7317334
DOI: 10.1136/bjo.65.12.859 -
Hippokratia 2021Ocular involvement in patients with transfusion-dependent β-thalassemia is quite common, and its frequency differs among studies. This case series aimed to describe the...
BACKGROUND
Ocular involvement in patients with transfusion-dependent β-thalassemia is quite common, and its frequency differs among studies. This case series aimed to describe the ocular abnormalities occurring in β-thalassemia patients who need regular blood transfusions and receive iron chelation therapy.
CASE SERIES
This is a case series prospectively studied 32 β-thalassemia patients from Northern Greece receiving regular blood transfusions and iron-chelating therapy. Patients' average age was 35.5 years. Eighteen patients with major phenotypes and fourteen patients with intermedia type underwent comprehensive ophthalmic examination at the time of enrolment, including visual acuity evaluation, refraction and color vision tests, Amsler grid test, slit-lamp, and dilated-pupil fundus examination. Additionally, we performed visual field testing and optical coherence tomography in all patients and fluorescein angiography only in selected cases. After six months, patients' complete ophthalmic examination was repeated for any new ocular findings due to the disease process and iron chelation therapy. Ocular involvement was detected in 46.87 % of the patients. Lesions were most frequently seen in elderly patients with thalassemia major. Lens opacities were present in 21.8 %, and degeneration of the retinal pigment epithelium was described in 15.6 % of the patients, representing the commonest fundus alteration observed, followed by fundus atrophy. The most severe and vision-threatening condition described in this study was the presence of angioid streaks with choroidal neovascularisation. Six months follow-up of patients did not reveal any new ocular findings.
CONCLUSION
Early detection of severe ocular abnormalities is important in patients with thalassemia; thus, an ophthalmologic examination should be included at regular check-ups. An annual examination is currently indicated for asymptomatic patients, while in symptomatic and complicated cases, patients should be closely followed-up. HIPPOKRATIA 2021, 25 (2):79-82.
PubMed: 35937508
DOI: No ID Found -
Transactions of the American... 1977A family with pseudoinflammatory macular dystrophy (PMD) is presented. This dominantly inherited macular dystrophy has its onset in the 3rd to 5th decades with the...
A family with pseudoinflammatory macular dystrophy (PMD) is presented. This dominantly inherited macular dystrophy has its onset in the 3rd to 5th decades with the earliest manifestation being a macular subretinal neovascular network. Visual function tests (ERG, EOG, visual fields, retinal sensitivity) in the early and late stages indicates this is local or geographic disease. This dystrophy should be differentiated from other hereditary causes for subretinal neovascularization (angioid streaks, vitelliform dystrophy, dominant drusen of Bruch's membrane, optic nerve drusen and myopia). It is suggested that treatment be directed at early obliteration of the subretinal neovascularization with intense photocoagulation since the outcome in virtually all cases of untreated PMD is legal blindness.
Topics: Adult; Color Vision Defects; Female; Fluorescein Angiography; Humans; Light Coagulation; Macular Degeneration; Male; Pedigree; Pigment Epithelium of Eye; Retinal Degeneration; Retinal Detachment; Retinal Hemorrhage; Retinal Vessels; Retinitis
PubMed: 306694
DOI: No ID Found -
Oman Journal of Ophthalmology 2018Pseudoxanthoma elasticum is a hereditary disorder that affects primarily the elastic tissues in the skin, the eyes and the blood vessels.
Pseudoxanthoma elasticum is a hereditary disorder that affects primarily the elastic tissues in the skin, the eyes and the blood vessels.
PubMed: 29563708
DOI: 10.4103/ojo.OJO_123_2016 -
The Pan African Medical Journal 2020This case series illustrates clinical features and treatment outcomes of angioid streak associated CNV (AS-CNV) in 3 consecutive patients. Mean age of patients was 43.2...
This case series illustrates clinical features and treatment outcomes of angioid streak associated CNV (AS-CNV) in 3 consecutive patients. Mean age of patients was 43.2 years with one female patient. Bilateral CNV was present in one patient. Comet-tail lesions were present in all cases. No underlying systemic association was found in any of the patients. All patients were treated with 3 loading doses of anti-VEGF injections (ranibizumab in two and aflibercept was used in one case). Subretinal fluid resolved in all cases with no recurrence of CNV activity at mean follow-up of 10.75 months. AS-CNV in Zambian eyes responds favourably to anti-VEGF injections.
Topics: Adult; Angiogenesis Inhibitors; Angioid Streaks; Choroidal Neovascularization; Female; Follow-Up Studies; Humans; Male; Ranibizumab; Receptors, Vascular Endothelial Growth Factor; Recombinant Fusion Proteins; Treatment Outcome; Zambia
PubMed: 33117488
DOI: 10.11604/pamj.2020.36.294.25065 -
Genes Aug 2021This study aimed to characterize Korean patients with pseudoxanthoma elasticum (PXE) presenting with angioid streaks. Retinal phenotypes were longitudinally evaluated by... (Observational Study)
Observational Study
This study aimed to characterize Korean patients with pseudoxanthoma elasticum (PXE) presenting with angioid streaks. Retinal phenotypes were longitudinally evaluated by multimodal ophthalmic imaging, and targeted gene panel sequencing for inherited retinal diseases was conducted. Seven subjects from unrelated families (median age, 51.2 years) were enrolled and followed for a median of 3.2 years. Four asymptomatic patients were significantly younger than three symptomatic patients with decreased visual acuity at presentation (mean age; 38.1 vs. 61.5 years, = 0.020). The asymptomatic patients maintained good vision (20/32 or better) and had no choroidal neovascularization (CNV) over the observation period. The symptomatic patients showed additional reduction in visual acuity and bilateral CNV occurrence during the longitudinal follow-up. Pathogenic variants were identified in all patients, leading to a diagnosis of PXE. Heterozygous monoallelic variants were identified in four patients and compound heterozygous variants were detected in three patients. Nine variants were identified, including one novel variant, c.2035G>T [p.Glu679Ter]. This is the first genetic study of Korean patients with PXE.
Topics: Adult; Angioid Streaks; Choroidal Neovascularization; Female; Fluorescein Angiography; Heterozygote; Humans; Male; Middle Aged; Multidrug Resistance-Associated Proteins; Pseudoxanthoma Elasticum; Republic of Korea; Retrospective Studies; Visual Acuity
PubMed: 34440381
DOI: 10.3390/genes12081207 -
Journal of Clinical Medicine Apr 2022Purpose: to quantitatively analyze choriocapillaris perfusion using swept-source optical coherence tomography angiography (SS-OCTA) in eyes presenting with angioid...
Purpose: to quantitatively analyze choriocapillaris perfusion using swept-source optical coherence tomography angiography (SS-OCTA) in eyes presenting with angioid streaks in comparison with control eyes. Methods: Macular 6 × 6 mm SS-OCTA scans were retrospectively analyzed in eyes with angioid streaks and in control eyes. En face choriocapillaris flow images were compensated with en face choriocapillaris structure images, followed by the Phansalkar local thresholding method (with a window radius of four and eight pixels). Quantitative analysis was performed in the four peripheral 1 × 1 mm corners of the 6 × 6 mm SS-OCTA image to include equidistant and comparable regions. The percentage of flow deficits (FD%), the number and size of the flow deficits (FDs) and the total area of FDs were then calculated. Results: 54 eyes of 31 patients were included in the study: 27 eyes diagnosed with angioid streaks and 27 controls. Analysis of the four 1 × 1 mm peripheral corners of the 6 × 6 mm SS-OCTA image showed that eyes with angioid streaks had a higher FD% compared to the control group (47.62 ± 8.06 versus 38.90 ± 6.38 using a radius of four pixels (p < 0.001); 48.37 ± 7.65 versus 39.66 ± 6.51 using a radius of eight pixels (p < 0.001). The average size of FDs as well as the total area size of the FDs were significantly higher in eyes with angioid streaks compared to control eyes (p < 0.001). Eyes with angioid streaks present reduced choriocapillaris flow compared to control eyes. Decreased choriocapillaris perfusion may contribute, among other factors, to the development of neovascularization and atrophy in patients with angioid streaks.
PubMed: 35456229
DOI: 10.3390/jcm11082134 -
The British Journal of Ophthalmology Oct 1990
Topics: Angioid Streaks; Humans; Osteitis Deformans
PubMed: 2285678
DOI: 10.1136/bjo.74.10.577 -
Eye (London, England) May 2022To inspect the inter-reader agreement of different diagnostic modalities in identifying choroidal neovascularization (CNV) activity secondary to angioid streaks (AS) and...
OBJECTIVES
To inspect the inter-reader agreement of different diagnostic modalities in identifying choroidal neovascularization (CNV) activity secondary to angioid streaks (AS) and to analyze the prevalence of subretinal hyper-reflective material (SHRM) in active CNV.
METHODS
Retrospective study of patients with AS with active CNV; optical coherence tomography (OCT), OCT angiography (OCTA), fundus fluorescein angiography (FFA), and indocyanine green angiography (ICGA) from each patient were collected. Agreement between two readers using different diagnostic modalities is presented as free-marginal kappa (k) and 95% confidence interval (CI).
RESULTS
This study included 19 eyes of 12 patients with active CNV (5 naive and 14 previously treated). Agreement among readers on CNV activity was excellent for OCT (k =0.88; 95% CI 0.71-1.00), good for FFA (k = 0.70; 95% CI 0.46-0.94) and ICGA (k = 0.58; 95% CI 0.31-0.84), and poor using OCTA (k = 0.39; 95% CI 0.11-0.68). SHRM was the most common OCT finding associated with active CNV (100%); fuzzy borders were present in 53% of SHRM cases at baseline.
CONCLUSIONS
Identification of CNV activity in AS is challenging; OCT was the best modality to inspect active CNV. The identification of SHRM contributed to recognizing active CNV. Further studies are needed to assess the role of SHRM in anticipating prognosis and guiding treatment of CNV secondary to AS.
Topics: Angioid Streaks; Choroidal Neovascularization; Coloring Agents; Fluorescein Angiography; Humans; Indocyanine Green; Retrospective Studies; Tomography, Optical Coherence
PubMed: 33972707
DOI: 10.1038/s41433-021-01555-5