Current status of retrolental fibroplasia. The retinopathy of prematurity.
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Biomedical subjects
Publications and source records attributed to A Patz.
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The ocular clinicopathologic features of this unique patient were congenital stationary night blindness, drusen of the optic nerve head, cilioretinal artery, intraretinal pigmented lesion, and branch retinal vein occlusion. Photocoagulation therapy led to total disappearance of the neovascular tissue, clinically and histopathologically. Histopathologic examination showed an occluded branch vein associated with a sclerotic retinal arteriole. Peripheral to the site of venous occlusion, inner ischemic retinal atrophy was present. The normal complement of rod and cone photoreceptors supports the view that the night blindness in this case was an abnormality in the neural transmission and not on a morphological basis. The pigmented intraretinal lesion proved to be a localized area of retinal and choroidal neovascularization with anastomosis and secondary retinal pigment epithelial hyperplasia. This lesion was identical to Fuchs' dot of myopia but out patient was hyperopic.
We performed argon laser photocoagulation on 31 eyes of 30 patients with presumed ocular histoplasmosis. Nineteen of 25 eyes (76%) with neovascular membranes 0.25 disk diameter or further from the fovea had 6/12 (20/40) or better vision after an average follow-up period of 20 months. Twenty-two of these eyes (88%) had the same or improved visual acuity during this period. When compared to a similar group of untreated eyes, the apparent advantage of photocoagulation in preserving visual acuity was not statistically significant.
Tumor cells introduced into the rabbit vitreous produced a form of retinal neovascularization, but only when the tumor cells were in direct contact with the vascularized retina. This contrasted with the production of neovascularization at a distance of several millimeters in other angiogenesis models. An extract of adult rabbit vitreous was found to inhibit the growth of limbal neovascularization induced by tumors implanted in the rabbit corneal stroma. The experimental model of retrolental fibroplasia provides a bioassay to investigate further the apparent inhibitory substances in the vitreous.
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The demonstration of an inhibitory effect on the potent source of corneal neovascularization which is elicited by the stromal implantation of solid tumours raises the possibility that similar inhibition may be obtained in the animal model of retinal neovascularization. A major program has been initiated to extend the inhibitor studies observed in cornea to the retina.
Adult rabbit retinal vessels underwent neovascularization in response to tumor implantation within the vitreous body. The neovascular response was presumably elicited by the tumor angiogenesis factor (TAF). The response of adult retinal vessels to an angiogenic stimulus raises the possibility that a similar substance may cause retinal neovascularization in humans, and that in normal conditions the vitreous may be able to suppress angiogenic activity.
Twenty-three members of one white family were studied for a new form of dominant slowly progressive macular dystrophy in which visual acuity remained good until the seventh decade. Ten patients had positive signs of this entity. Eight patients had possible early forms. Five had no signs. Several patients had visual acuity fluctuations, documented by their ophthalmologists who saw associated pigment epithelial alterations in some cases. Obvious macular changes included perifoveal pigment epithelial atrophy, posterior pole flecks, and fundus lesions resembling an atrophic form of senile macular degeneration. We suggest a possible hereditary predisposition to senile macular degeneration in our patients.
Sixty-four eyes of 60 patients with ocular histoplasmosis, and choroidal neovascularization documented by fluorescein angiography, and not treated with photocoagulation, were followed for an average of 29 months. The primary determinant of visual outcome was the proximity of the neovascular membrane to the fovea. Sixty-four percent of eyes with membranes 0.25 disk diameter or greater from the fovea had a final visual acuity of 6/12 (20/40) or better, and 71% of such eyes maintained or improved initial visual acuity. All eyes with subfoveal neovascularization had a poor visual result. Other factors related to visual prognosis included initial visual acuity, size of the neovascular membrane, size of sensory retinal detachment, and presence of hemorrhage.
A 71-year-old woman complained of "smoky" vision, which was found to be caused by a hyphema with blood dripping from a vascular tuft located in the 12 o'clock meridian of the iris. Fluorescein angiography delineated vascular tufts and argon laser photocoagulation eradicated one of the tufts that bled. Histopathologic studies of iris obtained at the time of cataract extraction showed an aggregate of small vessels at the pupillary margin. Most patients with vascular tufts of the pupillary margin have no systemic disease but they are also observed in diabetes mellitus and myotonic dystrophy.
There are two barriers between the blood and retina--one in the retinal circulation at the endothelial cell lining with its tight junctions in the retinal capillaries, and the second involving the choroidal circulation at the zonula occludens between adjacent retinal pigment epithelial cells. Damage to either of these barriers results in a leakage of fluid into or beneath the sensory retina. Serous detachment of the retinal pigment epithelium is a special situation in which both barriers remain intact but fluid accumulates in the space between the detached pigment epithelial cells and Bruch's membrane. It is convenient to analyze the fluorescein angiographic findings in relation to these two barriers, as a large number of retinal disorders affect either the retinal vascular system or the choriocapillaris-Bruch's membrane-pigment epithelium layers.
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The relation between PaO2 and retrolental fibroplasia (RLF) was studied prospectively in 719 premature infants born in or treated in the intensive care units of a group of university hospitals. Blood gas studies were performed on 589 of these infants, 66 of whom had a diagnosis of RLF; in 27 of these 66, some grade of mostly nonblinding cicatricial disease developed. The frequency of RLF was highest among infants of lowest birth weight. A multivariate statistical method was used to analyze simultaneously the effect of possible etiologic factors associated with RLF. The occurrence of RLF was found to be unrelated to PaO2, as determined by the limited information available from intermittent sampling. RLF is associated with concentration of oxygen administered in the lightest birth weight group, but the strongest association, aside from birth weight, was with time in oxygen. None of the other variables involving blood chemical values appeared to be associated with RLF. The severity of cicatricial RLF is clearly greater in infants weighing less than 1,200 g at birth. Conservative administration of oxygen may have been responsible for failure to demonstrate quantitative association between PaO2 levels and disease. Agreement between the observed and predicted numbers of infants with RLF demonstrate the strength of the multivariate technique employed in making the statistical analyses.
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