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Retina (Philadelphia, Pa.) 2013To evaluate different therapies for choroidal neovascularization (CNV) due to angioid streaks (AS). (Review)
Review
PURPOSE
To evaluate different therapies for choroidal neovascularization (CNV) due to angioid streaks (AS).
METHODS
Studies were identified by a systematic literature search and were included in the analysis based on predefined criteria. Primary outcome measure was change in best-corrected visual acuity (BCVA).
RESULTS
Fifty-four relevant studies were identified and included mostly uncontrolled case series. No randomized controlled trials were available. Treatment with vascular endothelial growth factor inhibitors improved or stabilized BCVA in all case series. Photodynamic therapy slowed down disease progression with stabilization or decrease of BCVA. Individual BCVA and follow-up data for each treated eye were reported in >160 cases for both treatments, vascular endothelial growth factor inhibitors and photodynamic therapy. In a pooled analysis of those studies, the difference of mean change in BCVA between both treatment groups was estimated as approximately 6 lines (0.59 logMAR [95% confidence interval, 0.38-0.8; P < 0.0001]). A better baseline BCVA was associated with a better BCVA outcome (P < 0.0001). Laser photocoagulation yielded comparable results as photodynamic therapy but application was mostly restricted to extrafoveal lesions, was complicated by frequent recurrences, and led to more retinal damage with subsequent absolute scotomas. Combination therapies seem to be not superior to monotherapy.
CONCLUSION
Intravitreal vascular endothelial growth factor inhibitors are currently the most effective treatment of CNV due to angioid streaks.
Topics: Angiogenesis Inhibitors; Angioid Streaks; Choroidal Neovascularization; Humans; Light Coagulation; Photochemotherapy; Visual Acuity
PubMed: 23719398
DOI: 10.1097/IAE.0b013e3182914d2b -
JAMA Ophthalmology Dec 2019
Topics: Angioid Streaks; Ehlers-Danlos Syndrome; Fundus Oculi; Humans
PubMed: 31556922
DOI: 10.1001/jamaophthalmol.2019.2549 -
American Journal of Ophthalmology Dec 2020To investigate whether the extent of Bruch's membrane calcification is associated with choroidal neovascularization (CNV) and macular atrophy in patients with...
PURPOSE
To investigate whether the extent of Bruch's membrane calcification is associated with choroidal neovascularization (CNV) and macular atrophy in patients with pseudoxanthoma elasticum (PXE) by using the extent of angioid streaks as a surrogate marker for the degree of Bruch's membrane calcification.
DESIGN
Retrospective cross-sectional study.
METHODS
We investigated 301 patients with PXE (median age, 52 years; range, 9-79 years) in a tertiary referral center. For both eyes, we graded the extent of angioid streaks, that is, their distance from the optic disc, into 5 groups. Imaging was systematically assessed for signs of CNV and macular atrophy. Associations between the extent of angioid streaks and CNV or macular atrophy were investigated using regression analysis.
RESULTS
CNV was present in 148 patients (49%) and retinal atrophy in 71 patients (24%). The extent of angioid streaks was associated with older age (P for trend = 1.92 × 10) and a higher prevalence of CNV and/or macular atrophy (P for trend = 4.22 × 10 and P for trend = 5.17 × 10, respectively). In addition, the extent of angioid streaks was associated with the presence of CNV when adjusted for age and sex (odds ratio, 1.9; 95% confidence interval, 1.3-2.9) and with more severe macular atrophy (proportional odds ratio, 2.3; 95% confidence interval, 1.5-3.6).
CONCLUSIONS
In patients with PXE, longer angioid streaks are associated with an increased risk of CNV and macular atrophy, even after adjustment for age. These findings are relevant when counseling PXE patients on their visual prognosis.
Topics: Adolescent; Adult; Aged; Angioid Streaks; Bruch Membrane; Child; Cross-Sectional Studies; Female; Fluorescein Angiography; Fundus Oculi; Humans; Macular Degeneration; Male; Middle Aged; Pseudoxanthoma Elasticum; Retina; Retrospective Studies; Tomography, Optical Coherence; Visual Acuity; Young Adult
PubMed: 32702361
DOI: 10.1016/j.ajo.2020.07.022 -
Eye (London, England) Mar 2021To assess the short-term and long-term visual outcomes in patients with choroidal neovascularisation (CNV) secondary to angioid streaks treated with intravitreal...
PURPOSE
To assess the short-term and long-term visual outcomes in patients with choroidal neovascularisation (CNV) secondary to angioid streaks treated with intravitreal anti-vascular endothelial growth factor (VEGF).
METHODS
Retrospective, single-centre study.
RESULTS
Overall 66 eyes of 52 patients were analysed. Follow-up ranged from 1 to 10 years. BCVA was 62 ETDRS letters at baseline, 68 letters at 1 year, 60 ETDRS letters at 5 years and 58 letters at 7 years. At 2 years patients gained 5.7 ETDRS letters from baseline but this gain was lost at 5 years. At 5 years there was an average loss of ETDRS letters from baseline of 3.3 letters. Sub-group analysis of subfoveal CNV showed worse outcome compared with eyes with extrafoveal and juxtafoveal CNV. In subfoveal CNV, BCVA was 53 ETDRS letters at 1 year (p < 0.0001) and 39 ETDRS at 5 years (p = 0.0005).
CONCLUSION
Anti-VEGF therapy is effective at stabilising visual acuity in patients with choroidal neovascularisation secondary to angiod streaks, however there is a gradual decline in visual acuity observed with 5-10 years of follow-up. Furthermore, subfoveal CNV have worse visual outcome compared with extrafoveal and juxtafoveal CNV.
Topics: Angiogenesis Inhibitors; Angioid Streaks; Choroidal Neovascularization; Fluorescein Angiography; Follow-Up Studies; Humans; Intravitreal Injections; Retrospective Studies; Visual Acuity
PubMed: 32461565
DOI: 10.1038/s41433-020-0979-9 -
Journal Francais D'ophtalmologie Oct 2020
Topics: Anemia, Sickle Cell; Angioid Streaks; Fluorescein Angiography; Fundus Oculi; Hemoglobin, Sickle; Homozygote; Humans; Male; Middle Aged
PubMed: 32600990
DOI: 10.1016/j.jfo.2019.11.030 -
Ophthalmologica. Journal International... Feb 1948
Topics: Angioid Streaks; Eye; Humans
PubMed: 18910131
DOI: No ID Found -
JAMA Ophthalmology Oct 2019
Topics: Angioid Streaks; Female; Humans; Ophthalmoscopy; Pseudoxanthoma Elasticum; Young Adult
PubMed: 31600377
DOI: 10.1001/jamaophthalmol.2019.3129 -
Indian Journal of Ophthalmology Jul 2017Camurati-Engelmann disease (CED) is a rare autosomal dominant disease with various phenotypic expressions. The hallmark of the disease is bilateral symmetric diaphyseal...
Camurati-Engelmann disease (CED) is a rare autosomal dominant disease with various phenotypic expressions. The hallmark of the disease is bilateral symmetric diaphyseal hyperostosis of the long bones with progressive involvement of the metaphysis. Ocular manifestations occur rarely and mainly result from bony overgrowth of the orbit and optic canal stenosis. We report a case of CED showing angioid streaks (ASs) in both fundi with no macular involvement and discuss the possible theories of the pathogenesis of AS in this disease.
Topics: Adult; Angioid Streaks; Camurati-Engelmann Syndrome; Female; Fluorescein Angiography; Fundus Oculi; Humans; Retina; Tomography, Optical Coherence
PubMed: 28724827
DOI: 10.4103/ijo.IJO_910_16 -
Optometry and Vision Science : Official... Feb 2021Alagille syndrome is rare genetic disease, which affects liver and heart function. Cases are frequently diagnosed late, and a complete ocular examination aids in the...
SIGNIFICANCE
Alagille syndrome is rare genetic disease, which affects liver and heart function. Cases are frequently diagnosed late, and a complete ocular examination aids in the diagnosis. Although ophthalmic manifestations are usually benign, occasionally, sight-threatening complications occur.
PURPOSE
The case presented herein highlights a rare condition, which usually can be diagnosed by the ophthalmological findings. However, in this particular case report, the complication of angioid streaks and choroidal neovascularization is reported for the first time in Alagille syndrome.
CASE REPORT
Α 32-year-old woman diagnosed with Alagille syndrome presented with diminution of vision in the left eye. The cause was choroidal neovascularization related to angioid streaks. She was treated with intravitreal injections of ranibizumab but developed an extensive macular scar. A few years later, she developed the same complication in the right eye and was treated similarly.
CONCLUSIONS
Alagille syndrome has many ophthalmic manifestations, most of them benign with minimal threat to vision. Herein for the first time, we present a case of Alagille syndrome with angioid streak-related choroidal neovascularization, which resulted in severe vision loss.
Topics: Adult; Alagille Syndrome; Angiogenesis Inhibitors; Angioid Streaks; Choroidal Neovascularization; Female; Fluorescein Angiography; Humans; Intravitreal Injections; Ranibizumab; Tomography, Optical Coherence; Vascular Endothelial Growth Factor A
PubMed: 33617169
DOI: 10.1097/OPX.0000000000001641 -
Eye (London, England) Dec 2019To assess hyperreflective foci (HF) number in angioid streaks (AS) by means of spectral-domain optical coherence tomography (SD-OCT). (Observational Study)
Observational Study
AIMS
To assess hyperreflective foci (HF) number in angioid streaks (AS) by means of spectral-domain optical coherence tomography (SD-OCT).
METHODS
Observational and cross-sectional study. Sixty-two eyes with AS and 62 controls underwent best-corrected visual acuity (BCVA), fundoscopy, and SD-OCT. HF were assessed on the horizontal scan of a six-line radial OCT, in the fovea (1500-μm diameter) and parafovea (500 μm external to the fovea), and sub-classified as retinal or choroidal, small or large. Eyes were distributed in one of four groups, as carrying foveal AS without choroidal neovascularization (CNV) (14 eyes), extra-foveal AS without CNV (14), active CNV (20), and inactive CNV (14). Primary outcome was HF assessment in AS. Secondary outcomes included their correlations with BCVA.
RESULTS
AS-affected eyes had higher HF numbers. Our sub-analysis revealed that patients with active CNV had a larger number of retinal and choroidal HF than all the other groups, whereas retinal and choroidal foci were significantly increased in inactive CNV only with respect to controls. Interestingly, patients with foveal AS showed HF number increase in the choroid and fovea. BCVA deterioration positively correlated with the total HF number found in the fovea and the choroid.
CONCLUSION
HF are significantly increased in patients with AS. Despite being especially evident in active CNV, the increasing number in eyes without CNV might suggest new pathogenetic aspects of the disease.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Angioid Streaks; Choroid; Cross-Sectional Studies; Female; Fluorescein Angiography; Fovea Centralis; Fundus Oculi; Humans; Male; Middle Aged; Prospective Studies; Tomography, Optical Coherence; Visual Acuity; Young Adult
PubMed: 31278381
DOI: 10.1038/s41433-019-0483-2