1/35. A preliminary study of photodynamic therapy using verteporfin for choroidal neovascularization in pathologic myopia, ocular histoplasmosis syndrome, angioid streaks, and idiopathic causes.OBJECTIVE: To evaluate short-term safety and the effects on visual acuity and fluorescein angiography of single or multiple sessions of photodynamic therapy with verteporfin for choroidal neovascularization (CNV) not related to age-related macular degeneration (AMD), including pathologic myopia, the ocular histoplasmosis syndrome, angioid streaks, and idiopathic causes. DESIGN: A nonrandomized, multicenter, open-label, dose-escalation phase 1 and 2 clinical trial. SETTING: Four ophthalmic centers in europe and north america providing retinal care. PARTICIPANTS: Thirteen patients with subfoveal CNV due to pathologic myopia, the ocular histoplasmosis syndrome, angioid streaks, or idiopathic causes. methods: Standardized protocol refraction, visual acuity testing, ophthalmic examinations, color photographs, and fluorescein angiograms were used to evaluate the results of photodynamic therapy treatments with verteporfin. Follow-up ranged from 12 weeks for patients who were treated once to 43 weeks for patients who were treated up to 4 times. RESULTS: Verteporfin therapy was well tolerated in patients with CNV not related to AMD. No deterioration in visual acuity was observed; most patients gained at least 1 line of vision. Reduction in the size of leakage area from classic CNV was noted in all patients as early as 1 week after verteporfin therapy, with complete absence of leakage from classic CNV in almost half of the patients. Improvement in visual acuity after verteporfin therapy was greatest ( 6, 8, and 9 lines) in 3 patients with relatively poor initial visual acuity (between 20/200 and 20/800). Up to 4 treatments were found to have short-term safety even with retreatment intervals as short as 4 weeks. CONCLUSIONS: Treatment of CNV not related to AMD with verteporfin therapy achieves short-term cessation of fluorescein leakage from CNV in a small number of patients without loss of vision. Further randomized clinical trials including a larger number of patients are under way to confirm whether verteporfin therapy is beneficial for subfoveal CNV not related to AMD.- - - - - - - - - - ranking = 1keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
2/35. Macular translocation for subfoveal choroidal neovascularization in angioid streaks.PURPOSE: To report a case of visual improvement after macular translocation performed for a subfoveal choroidal neovascular membrane in a patient with pseudoxanthoma elasticum and angioid streaks. methods: The fovea was translocated inferiorly by scleral imbrication, intentional retinal detachment with a small posterior retinotomy, and partial fluid-air exchange. The choroidal neovascular membrane was photocoagulated 1 week later. RESULTS: The visual acuity of the patient improved from 20/125 to 20/40. The center of the foveal avascular zone was moved inferiorly 844 microm. The choroidal neovascular membrane was extrafoveal after translocation and was treated with laser photocoagulation. CONCLUSION: Macular translocation may be considered in the management of subfoveal choroidal neovascular membrane in patients with pseudoxanthoma elasticum and angioid streaks.- - - - - - - - - - ranking = 2.0054534866722keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
3/35. Optic disc drusen, angioid streaks, and mottled fundus in various combinations in a Sicilian family.BACKGROUND: We describe a Sicilian family in which optic disc drusen, angioid streaks, and mottled fundus--without dermatological signs of pseudoxanthoma elasticum (PXE)--are present in various combinations and segregate as an autosomal dominant trait. Since these ocular manifestations can be part of the clinical signs of PXE, we examined the possible involvement of a mutation in the ABCC6 gene, which is known to be responsible for PXE. methods: Linkage analysis was performed with both intragenic and flanking markers. We used marker D16B9722 and a single-nucleotide polymorphism located in exon 15 of the ABCC6 gene. lod score values were calculated on the assumption of a gene frequency of 0.0001 and both complete penetrance and reduced penetrance (90%), with theta values between 0.0 and 0.4. RESULTS: lod score values excluded the involvement of the ABCC6 gene. CONCLUSIONS: The dominant transmission of optic disc drusen, mottled fundus, and angioid streaks in this family is not due to alterations in the ABCC6 gene.- - - - - - - - - - ranking = 2.0054534866722keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
4/35. angioid streaks and sickle haemoglobinopathies.Five patients had angioid streaks associated with sickle cell haemoglobinopathy. Other diseases associated with angioid streaks were ruled out, as was elastic tissue degenegation in sickle cell patients. After studying over 350 patients, we believe the incidence of angioid streaks in sickle cell disease to be between 1 and 2 per cent.- - - - - - - - - - ranking = 1.0230665671521keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
5/35. Bruch's membrane in pseudoxanthoma elasticum. Histochemical, ultrastructural, and x-ray microanalytical study of the membrane and angioid streak areas.In a case of pseudoxanthoma elasticum (PXE), Bruch's membrane was studied histochemically, ultrastructurally and by element analysis. Two kinds of calcifications were observed, one composed of hydroxyapatite and the other of CaHPO4. They were seen in the scar tissue of angioid streak areas and in the membrane outside these areas, respectively. Furthermore, a thready material was found in the membrane as well as an increased amount of acid mucopolysaccharides. The genesis of the calcifications and the pathogenesis of angioid streaks are discussed. The author is in favor of a malformed collagen as the basic defect in PXE.- - - - - - - - - - ranking = 2.0054534866722keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
6/35. Novel mutation in ABCC6 gene in a Japanese pedigree with pseudoxanthoma elasticum and retinitis pigmentosa.PURPOSE: To report a novel mutation of the ABCC6 gene in a Japanese family that had a case of pseudoxanthoma elasticum (PXE) another with PXE and retinitis pigmentosa. methods: Ophthalmologic examinations were performed, and the ABCC6 gene was analysed by direct genomic sequencing. RESULTS: Fundus examinations of the 48-year-old proband disclosed angioid streaks and a peud'orange appearance of the retina of the both eyes, whereas both of his 25- and 20-year-old daughters had pigmentary degeneration and angioid streaks. In the sibilings, the mixed cone-rod ERG was almost nondetectable, whereas that of the proband was well-preserved. Molecular genetic analysis revealed that the proband has a homozygous nonsense mutation at the 595 bp in the ABCC6, and the siblings were heterozygous for the same mutation. This mutation was not detected in Japanese subjects in the JSNP database (http://snp.ims.u-tokyo.ac.jp/). CONCLUSIONS: Our results demonstrated an association between a novel mutation in the ABCC6 gene and PXE in a Japanese family.- - - - - - - - - - ranking = 0.66848449555739keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
7/35. Development of angioid streaks in association with pseudoxanthoma elasticum.angioid streaks were noted 6 years after the diagnosis of pseudoxanthoma elasticum in a young girl with peau d'orange discolouration of the fundi. The author discusses the evolution of angioid streaks.- - - - - - - - - - ranking = 1.6762961948475keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
8/35. Disk drusen and angioid streaks in pseudoxanthoma elasticum.Visual field loss secondary to optic disk drusen became evident before the development of angioid streaks in a patient with pseudoxanthoma elasticum. The incidence of optic disk drusen in cases of pseudoxanthoma elasticum is 20 to 50 times greater than that in the healthy population. We postulate that the abnormal aggregation of macromolecules with a high affinity for calcium (resulting in abnormalities in elastin in cases of pseudoxanthoma elasticum) also develops at the cribriform plate, disrupting axonal flow and leading to disk drusen formation. pseudoxanthoma elasticum is associated with marked cardiovascular and gastrointestinal morbidity. Moreover, macular hemorrhage and precipitation of angioid streaks have frequently been noted after trauma. Prompt diagnosis of pseudoxanthoma elasticum will allow necessary prophylaxis and must be considered in patients with optic disk drusen.- - - - - - - - - - ranking = 2.0054534866722keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
9/35. Groenblad Strandberg syndrome--a case report.A rare case of Groenblad Strandberg syndrome in which angioid streaks are associated with pseudoxanthoma elasticum is being reported. The pathology, clinical features, complications and treatment of angioid streaks are discussed in detail.- - - - - - - - - - ranking = 0.66848449555739keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
10/35. Juxtapapillary choroidal neovascular membrane in a patient with Paget's disease and lattice corneal dystrophy.Paget's disease (osteitis deformans) is a relatively common metabolic bone disease characterized by the gradual development of bony deformities. These deformities lead to cranial and peripheral neuropathies from nerve and blood vessel impingement. angioid streaks are associated with Paget's disease and are the result of linear breaks in Bruch's membrane caused by the calcification and degeneration of this elastic tissue layer. A case report of a patient with Paget's disease with angioid streaks and a secondary choroidal neovascular membrane is presented. The membrane was treated with argon laser photocoagulation. The patient also had an incidental finding of lattice corneal dystrophy which required penetrating keratoplasty.- - - - - - - - - - ranking = 0.3393272037327keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
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