1/32. 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 = 1keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
2/32. pseudoxanthoma elasticum diagnosed 25 years after the onset of cardiovascular disease.A 63-year-old man who had experienced cerebral infarction and myocardial infarction at an early age, and repeatedly underwent coronary angioplasty was admitted to our hospital for cardiac evaluation. A coronary angiography showed complex multi-vessel disease with significant stenosis in all major vessels and coronary-artery bypass grafts. A funduscopic examination to evaluate hypertensive and diabetic changes revealed angioid streaks. Therefore, a skin biopsy was performed despite the absence of characteristic skin lesions. Histopathologic examinations revealed calcification and fragmentation of elastic fibers. Therefore, he was finally diagnosed as having pseudoxanthoma elasticum 25 years after the onset of cardiovascular disease.- - - - - - - - - - ranking = 0.16666666666667keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
3/32. Involvement of central nervous system in pseudoxanthoma elasticum.A 24- year-old female with typical pseudoxanthoma elasticum of the skin, angioid streaks of the optic fundi and cardiovascular involvement developed neurasthenic symptoms, epilepsy, progressive mental deterioration and focal neurological deficits. cerebral angiography revealed marked generalized arteriosclerotic change and occlusion of the left internal carotid artery. The literature of pseudoxanthoma elasticum is reviewed with special reference to central nervous system (CNS) manifestations. The possible role of the involvement of cerebral arteries in the genesis of CNS manifectations is discussed. And the importance of neurasthenic syndrome as an initial sign, which suggests that CNS involvement has already started in this disease, is stressed.- - - - - - - - - - ranking = 0.036685912989592keywords = angioid, streak (Clic here for more details about this article) |
4/32. 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 = 1keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
5/32. pseudoxanthoma elasticum with abnormal nailfold microcirculatory findings.pseudoxanthoma elasticum (PXE) is an inherited disorder of elastic tissue. Here we report a 34-year-old male patient who developed multiple symptomless yellowish papules over his neck for several months. He visited our dermatologic out-patient-clinic because his sister had similar skin lesions and mild visual impairment. The pathologic features of the skin biopsy showed fragmented calcified elastic fibers in the mid-to-lower dermis under the H&E, Verhoeff-van Gieson and Von Kossa stains. Under electron microscopy, calcified degenerated elastic fibers were noted. No other internal organ involvement was found except angioid streaks on the fundus. In addition, morphological changes of the nailfold capillaries, including increased tortuosity, dilated venous limbs of capillary loops, and decreased red-blood-cell velocity, were observed under the capillaroscopy. Though former reports have indicated that cardiovascular manifestations are caused by degeneration of elastic fibers of blood vessels, this study is the first to emphasize the microcirculatory disturbance of nailfold capillary, including morphology and blood-cell velocity, in PXE.- - - - - - - - - - ranking = 0.16666666666667keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
6/32. Central retinal artery occlusion in a patient with pseudoxanthoma elasticum.We examined a patient with central retinal artery occlusion (CRAO) who was diagnosed as having pseudoxanthoma elasticum and exhibited angioid streaks. echocardiography revealed stenosis and plaques of the right carotid artery. magnetic resonance imaging (MRI) showed multiple cerebral infarctions, which were considered to be the result of vascular-endothelial abnormalities associated with pseudoxanthoma elasticum. Systemic examination of any plaque which may cause CRAO is recommended.- - - - - - - - - - ranking = 0.16666666666667keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
7/32. 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 = 1keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
8/32. Visual loss associated with pseudoxanthoma elasticum.An 18-year-old woman with pseudoxanthoma elasticum had sudden profound bilateral visual loss, presumably as a result of optic nerve infarction caused by anemia and hypotension from massive gastrointestinal bleeding. Her central visual acuity largely recovered in her right eye, but her left eye had only hand motions acuity. During the subsequent 24 years, she underwent three separate surgical procedures for gastrointestinal bleeding. Abdominal arterial angiography showed numerous aneurysms of the gastric arteries. A subtotal gastrectomy was done, and histopathologic examination found advanced degenerative changes of the elastic lamina of the gastric arteries. At age 42 years, the patient lost residual vision in her right eye. This was caused by an extrafoveal subretinal choroidal neovascular membrane, which was obliterated with laser photocoagulation. Degeneration of elastic tissue, the primary defect in this syndrome, may cause cardiovascular complications and formation of breaks in the bruch membrane, which are visible as angioid streaks. Ingrowth of fibrovascular tissue through angioid streaks may cause disturbances of macular function. The optic nerve ischemia and infarction associated with the profound visual loss in this patient reminds the ophthalmologist of the systemic nature of this disorder and the possibility that systemic vascular complications with massive blood loss may occur.- - - - - - - - - - ranking = 0.20335257965626keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
9/32. 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 = 0.33333333333333keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
10/32. 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 = 0.83545655414158keywords = angioid streak, angioid, streak (Clic here for more details about this article) |
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