Cases reported "Macular Degeneration"

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1/38. Detection of beta-A4 amyloid and its precursor protein in the muscle of a patient with juvenile neuronal ceroid lipofuscinosis (Spielmeyer-Vogt-Sjogren).

    Muscle biopsy tissue from a patient affected by the juvenile form of neuronal ceroid lipofuscinosis (NCL) was studied immunohistochemically using antibodies to beta-amyloid peptide and amyloid precursor protein. Positive reaction in muscle was specifically localized to autophagic vacuoles and blood vessel walls. Increased acid phosphatase reaction suggested enhanced lysosomal activity. We hypothesize that beta-amyloid is deposited in NCL muscle by a lysosomal mechanism similar to that proposed in other disorders involving beta-amyloid.
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2/38. ICGA-guided laser photocoagulation of feeder vessels of choroidal neovascular membranes in age-related macular degeneration. indocyanine green angiography.

    PURPOSE: To report the ability of indocyanine green angiography (ICGA) with a confocal scanning laser ophthalmoscope (SLO) to identify feeder vessels of choroidal neovascular membranes (CNVM) secondary to age-related macular degeneration (ARMD) and to show the feasibility of inducing complete closure of the CNVM by photocoagulation targeted exclusively to the feeder vessels. methods: Five consecutive patients with exudative ARMD in whom ICGA with the confocal SLO showed extrafoveal feeder vessels supplying choroidal neovascular nets had laser photocoagulation done only to the feeder vessels. In two patients, two separate membranes were seen. RESULTS: Laser photocoagulation resulted in closure of the feeder vessels and the CNVM in four patients. Complete closure was achieved with one treatment in one patient and with two treatments in three patients. In one patient, two treatments failed to close the feeder vessel and the CNVM, but a third, more intense laser treatment resulted in temporary closure of the feeder vessel and CNVM, which recanalized 2 to 4 weeks later with development of a large rip in the retinal pigment epithelium. In one patient, two separate CNVMs grew from the edge of the laser scars, but they were not directly related to the original CNVM and its feeder vessel, and were treated successfully. The same eye later developed subfoveal occult CNVM with gradual deterioration of visual acuity. In the other four patients, visual acuity improved in two and was unchanged in two. CONCLUSIONS: indocyanine green angiography with the confocal SLO can identify choroidal feeder vessels supplying CNVM secondary to ARMD. Laser treatment to such extrafoveal feeder vessels, particularly in membranes that are large or subfoveal, may be effective in closing the feeder vessel and CNVM with preservation of the fovea and central vision. More than one treatment may be required, however, and failures and complications may be expected with this treatment modality.
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3/38. Polypoidal choroidal vasculopathy pattern in age-related macular degeneration: a clinicopathologic correlation.

    PURPOSE: To report the histopathologic features of surgically removed submacular tissue from an elderly patient with a pattern of polypoidal choroidal vasculopathy on indocyanine green angiography. methods: Clinical examination including fluorescein and indocyanine green angiography and light microscopy of surgical specimen. RESULTS: A thick yellow proteinaceous subretinal fluid was seen in the right macula of an 81-year-old white man. fluorescein angiography indicated progressive leakage from undetermined source apart from a few focal hyperfluorescent points. indocyanine green angiography showed several polyps as well as dilated choroidal vessels in the macula and along the superior temporal arcade. A large plaque was visualized in the late phase. Microscopically, the specimen consisted of a thick fibrovascular membrane located on the choroidal side of the retinal pigment epithelium (RPE). The RPE layer was discontinuous whereas on its choroidal side an almost intact layer of diffuse drusen was observed. A group of dilated thin-walled vessels were found that appeared to be saccular on serial sections. Some of these were located almost immediately under the diffuse drusen. CONCLUSION: Histologic examination of submacular tissue removed from an eye with polypoidal choroidal vasculopathy showed several aneurysmal dilatations located directly under diffuse drusen within a sub-RPE, intra-Bruch's fibrovascular membrane.
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4/38. indocyanine green angiographic findings of chorioretinal folds.

    PURPOSE: To analyze indocyanine green (ICG) angiographic findings of chorioretinal folds. methods: Eight patients (9 eyes) in whom chorioretinal folds had been diagnosed were enrolled in this study. color photography, fluorescein angiography (FA) and ICG angiography (IA) were performed. RESULTS: indocyanine green angiography demonstrated choroidal venous congestion and a filling delay of the choroidal vessels in one case with an orbital tumor. In one posterior scleritis case, IA showed a filling delay of choroidal vessels in the early phase and multiple patchy hypofluorescent lesions scattered in the posterior pole during the late phase. Idiopathic cases showed choroidal venous dilatation. No abnormalities of the choroidal vasculature in the form of radial folds, were revealed in two cases of AMD. Linear hyperfluorescent areas suggestive of chorioretinal folds seen on IA were less numerous and wider than those observed on FA in some eyes. On the other hand, they were equally numerous and wider on IA than those on FA in other eyes. CONCLUSION: indocyanine green angiography is useful for evaluating both pathological conditions of the choroidal vasculature and the width of chorioretinal folds at the level of the choroidal vasculature.
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5/38. radiotherapy of exudative age-related macular degeneration; a clinical and pathologic study.

    BACKGROUND: radiotherapy has recently been employed to treat patients with exudative macular degeneration in order to prevent severe visual loss. radiotherapy affects the evolution of exudative macular degeneration directly by endothelial toxicity, leading to capillary closure, and/or indirectly through its attenuating effects on the inflammatory response, mediated by macrophages and other inflammatory cells. methods: In this study we describe the histopathologic findings in a patient with exudative age-related macular degeneration (AMD) in both eyes whose right eye was treated with radiotherapy (5 times 2 Gy) 3 years before he died. The eyes were enucleated post mortem and investigated by light microscopy. Additionally, immunohistochemical investigation with antibodies against CD34 and CD68 was performed to identify patent endothelial cells and macrophages. RESULTS: Both eyes showed neovascular AMD consisting of mixed fibrocellular and fibrovascular membranes. capillaries in both the choriocapillaris and the neovascular membrane were patent in both eyes. macrophages were present in the choroidal neovascularization of both eyes. Neither preexistent choroidal, intraretinal, nor neovascular vessels showed increased wall thickness as sign of radiation damage. CONCLUSION: No radiation-related histopathologic effect could be demonstrated 3 years after radiation therapy in this patient with AMD.
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6/38. Clinicopathologic studies of age-related macular degeneration with classic subfoveal choroidal neovascularization treated with photodynamic therapy.

    BACKGROUND: Photodynamic therapy (PDT) is a relatively new modality that is currently under clinical and experimental evaluation for treatment of subfoveal choroidal neovascularization (CNV). The authors report the case of an 82-year-old woman who underwent verteporfin-mediated PDT for classic subfoveal CNV. fluorescein angiography performed 2 weeks after treatment disclosed reduction of the initial area of neovascularization and leakage by approximately 60%. Three weeks after PDT, however, the area of leakage was almost the same size as that before treatment. The patient underwent submacular membranectomy almost 4 weeks after treatment. The authors describe the ultrastructural vascular changes after PDT and a clinicopathologic study of classic CNV. methods: The submacular membrane was studied by light and electron microscopy and immunohistochemical techniques. RESULTS: Ultrastructural examination of the peripheral vessels showed evidence of endothelial cell degeneration with platelet aggregation and thrombus formation. Occasional occluded vessels were surrounded by macrophages, a phenomenon previously reported to describe the process of resorption of such blood vessels. The vessels in the center of the membrane were unremarkable. CONCLUSION: Photodynamic therapy causes endothelial cell damage, thrombus formation, and vascular occlusion of classic CNV in age-related macular degeneration.
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7/38. Quantifying changes in RPE and choroidal vasculature in eyes with age-related macular degeneration.

    PURPOSE: An image-analysis technique was developed to quantify changes in the retinal pigment epithelium (RPE) and choriocapillaris in eyes of deceased donors with age-related macular degeneration (AMD). methods: Both eyes of two donors with AMD and of one normal control donor were used to develop this technique. After removal of the anterior segments, the eyecups were hemisected through the macula, with the disc included in one half of the eyecup. The choroid with RPE cells was dissected from the sclera and incubated for alkaline phosphatase (APase) activity, and the pigment was partially bleached with H2O2. The APase-incubated choroid was flat embedded and sectioned after image and morphometric analyses. Quantitative computer-assisted morphometric analyses of the two AMD-affected eyes (cases 1 and 2) were compared with analysis of the normal eye of a 70-year-old control subject (case 3). RESULTS: The right eye in case 1 had geographic atrophy (GA) and demonstrated a large area in the posterior pole with very few RPE cells (90% loss of RPE), but the border of the area of RPE atrophy was not well defined. The density of choroidal blood vessels in this area was reduced 30% to 50%, compared with the same regions in the control eye. No area was completely devoid of choriocapillaris. Clinically undetected choroidal neovascularization (CNV) was observed in the right eye in case 1 in both the periphery and the macula and was generally associated with surviving RPE cells. The right eye in case 2 had GA (areolar RPE atrophy) and demonstrated a reduction in vascular density in the area from disc to macula that was even greater than that in the eye in case 1 (53% reduction in the submacular region). RPE atrophy between the disc and macula was almost complete. The border of the RPE defect was clearly delineated and coincided closely with the area of decreased choroidal vascular density. Surviving choriocapillaris in the area of RPE atrophy was significantly narrower than choriocapillaris in the control subject and in normal areas of the eyes with GA (P < 0.0001). CONCLUSIONS: In these eyes with GA, RPE atrophy was more severe than loss of choriocapillaris. Surviving choriocapillaris in areas with complete RPE loss was highly constricted. The association of surviving RPE cells with CNV suggests that RPE cells may furnish a stimulus for new vessel formation or stabilization.
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8/38. Clinicopathological correlation of polypoidal choroidal vasculopathy revealed by ultrastructural study.

    AIMS: To describe the clinical and histopathological findings in a patient with polypoidal choroidal vasculopathy. methods: A 76 year old Japanese man had a discrete, orange-red lesion of 1 disc diameter in the macula, with the fluorescein and indocyanine green angiographic and optical coherence tomographic findings compatible with polypoidal choroidal vasculopathy. He underwent a surgical removal of the macular lesion, followed by light and electron microscopic examinations. RESULTS: The histopathological examination revealed that the specimen consisted of degenerated retinal pigment epithelium-Bruch's membrane-choriocapillaris complex and inner choroid. A tortuous, unusually dilated venule was present adjacent to an arteriole with marked sclerotic changes, appearing to form arteriovenous crossing. These vessels seemed to represent native inner choroidal vessels, and had haemorrhage per diapedesis. blood cells and fibrin filled the lumina of the vessels and accumulated in the extravascular spaces, indicating vascular stasis. CONCLUSION: Hyperpermeability and haemorrhage due to stasis of a dilated venule and an arteriole involved by sclerosis at the site where they cross in the inner choroid might cause oedema and degeneration of the tissue. Voluminous accumulation of blood cells and fibrin might generate elevation of tissue pressure sufficient to displace the weakened lesion anteriorly. The result suggests that the polypoidal vessels in this case represent abnormality in the inner choroidal vasculature.
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9/38. Ultrastructural immunocytochemistry of subretinal neovascular membranes in age-related macular degeneration.

    BACKGROUND: The authors studied various cellular and extracellular matrix components of subretinal neovascular membranes (SRNVM) from patients with age-related macular degeneration (ARMD). methods: Electron microscopic immunocytochemistry was used on the subfoveal neovascular membranes surgically removed from three patients with disciform lesions due to ARMD. FINDINGS: The SRNVMs always contained large "feeder" vessels along with many new capillaries in different stages of maturation. capillaries were sparse and embedded in an abundant stroma. The majority of the nonvascular cells were either retinal pigment epithelial (RPE) cells or fibroblast-like cells. The RPE cells formed single or multiple layers on one side of the membranes. The stroma was composed mainly of collagen types I and IV and fibronectin, with small amounts of collagen types III, V, and VI. The absence of Bruch's membrane suggests that a splitting may occur between the RPE cells and Bruch's membrane with the new vessels growing into this cleft. A thickened layer of collagen type iv was often present under the RPE cells. The basement membranes of the newly formed capillaries were morphologically ill-defined, and contained substantial amounts of collagen type iv and fibronectin, but, unlike the basement membranes of normal capillaries, they lacked laminin or heparan sulfate proteoglycan. CONCLUSION: These results on the ultrastructural components of the SRNVMs may be useful in clarifying the nature of the disciform process in ARMD.
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10/38. Clinicopathological findings of choroidal neovascularisation following verteporfin photodynamic therapy.

    AIMS: To report the clinicopathologic findings of surgically excised choroidal neovascularisation (CNV) three days after verteporfin photodynamic therapy (PDT). methods: In three patients (three eyes) with age related macular degeneration, the CNV was surgically removed three days after PDT. The CNV specimens were examined by light microscopy. RESULTS: The patients had subfoveal classic CNV. fluorescein angiography revealed non-perfusion of the CNV after PDT and before surgery in all eyes. The light microscopy of the CNV membranes showed swollen and damaged endothelium. Thrombus formation or vascular occlusion in the CNV vessels was not detected. CONCLUSION: PDT did not cause a thrombosis of the vessels within the CNV three days after PDT. Severe endothelial damage of the CNV was observed and is likely a primary effect of PDT. Non-perfusion of the CNV at this stage is possibly secondary to occlusion at a deeper level, namely the underlying feeding choroid.
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