Macular arteriolar occlusions in sickle cell beta-thalassemia.
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Biomedical subjects
Publications and source records attributed to S S Feman.
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Thirteen patients who underwent radial keratotomy developed complications leading to visual impairment. Three eyes were legally blind. Two groups of complications were seen: those common to surgical procedures involving the eye--optic atrophy, infections, cataract and retinal detachment, and those unique to radial keratotomy--complete failure of the procedure, marked undercorrection, marked overcorrection, and induced astigmatism. Symptoms due to anisometropia were prominent in the latter group who considered themselves visually disabled by the surgery. Radial keratotomy, like all surgical procedures, is liable to complications that may lead to visual impairment, blindness, or loss of an eye.
Presumed ocular histoplasmosis syndrome is a multifocal choroiditis; it is not an uncommon cause of visual loss in patients who have positive cutaneous responses to histoplasmin tests. However, Histoplasma capsulatum infection is, most often, a pulmonary disease, or a systemically disseminated disorder, without visual symptoms. For this reason, a correspondence between the nonocular and ocular aspects of this disorder has been difficult to ascertain. In a series of eight patients with histoplasmosis, we found asymptomatic ocular lesions in six. We believe that the presence of asymptomatic lesions may have prevented earlier investigators from recognizing this correlation.
Sclerocornea is a primary anomaly in which scleralization of a peripheral part of the cornea, or the entire corneal tissue, occurs. In the peripheral type of sclerocornea, the affected area is vascularized with regular arcades of superficial scleral vessels. In total sclerocornea, the entire cornea is opaque and vascularized. To our knowledge, 97 cases of all types of sclerocornea have been reported in the world literature, either as a primary anomaly or in association with cornea plana. Peripheral sclerocornea in association with cornea plana was found in nine members of one family, in four of five generations studied. To our knowledge, this is the largest pedigree of hereditary peripheral sclerocornea identified. Our pedigree suggests the autosomal-dominant transmission of this entity but doesn't rule out phenocopies or other modes of inheritance in other cases of sclerocornea. Chromosomal analyses of representative family members revealed normal karyotypes.
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In the past 20 years, the survival rates for premature infants have improved. To reassess the incidence of cicatricial retinopathy of prematurity, 197 consecutive premature infants were examined. Each child was seen at intervals until age 6 months. Changes that could influence visual acuity were found in 22% of those with birth weight less than 1,000 g; 10% of those between 1,001 and 1,250 g; and 0% of those greater than 1,250 g. These findings were compared to a similar study performed at our university a decade earlier. A significant reduction in the incidence of premature retinopathy was demonstrated. However, because greater numbers of profoundly premature infants are surviving, the number of children with retinopathy of prematurity appears to be increasing.
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Two patients with histories of migraine had ischemic optic neuropathy develop during an episode of migraine. Clinical and radiologic studies excluded other causes of visual loss. Fluorescein angiography confirmed as ischemic process involving the optic dis. In both cases, visual loss was permanent. No further visual disturbance has occurred in either patient during a two-year period of follow-up while taking oral doses of propranolol hydrochloride.
Tennessee has the highest histoplasmosis infection incidence in the United States. Over 60% of the population reacts positively to skin tests and more than 60 new patients require hospitalization for the treatment of this disorder each year. However, the presumed ocular histoplasmosis syndrome (POHS) remains an uncommon cause of blindness in this state. A retrospective analysis performed with the assistance of 173 Tennessee Ophthalmologists identified 98 new POHS cases in 1980. A similar study, performed in a prospective manner, found 44 new POHS cases in the first six months of 1981. An analysis of recipients of Aid to the Blind revealed 0.5% with POHS; while 2.8% of new applicants for Aid to the Blind had POHS.
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Degenerative retinoschisis and rhegmatogenous retinal detachment are disorders that originate in the peripheral part of the retina. Both of these disorders are associated with biochemical processes that result in the finding of metabolic products within fluid-filled spaces. An attempt at biochemical differentiation of these two retinal disorders is described. Cholesterol, phospholipids, and proteins were present in the fluid samples, but low-density lipoproteins were not found. It is suggested that the cholesterol-to-phospholipid ratio may reflect the degree of retinal cellular destruction.
Waldenstrom's macroglobulinemia is characterized by hyperviscosity. This is usually the result of an increased serum concentration of IgM molecules; this paraprotein has a high molecular weight and unusual shape. The changes in shear rate associated with increased IgM concentrations produces maximal viscosity in the venous circulation. One of the major causes of retinal venous occlusion is venous stasis retinopathy. The typical patient with this disorder is a young individual with a periphlebitis.. However, the hyperviscosity in Waldenstrom's macroglobulinemia is an ideal representative of a venous stasis that is without associated vascular pathology. To illustrate this situation, two cases are described and their etiologic features are discussed.
A 16-year-old boy with Fabry's disease had sudden onset of diplopia unassociated with any other neurologic symptoms. A right internuclear ophthalmoplegia characterized by optokinetic phenomena and ocular dysmetria was demonstrated. The diplopia resolved spontaneously in six weeks. This is the first reported case of internuclear ophthalmoplegia occurring with Fabry's disease and represents one of the youngest patients to have unilateral internuclear ophthalmoplegia presumably caused by vascular occlusive disease.
Twenty-four eyes with macular holes have been followed for periods ranging from one to four years. Each hole was demonstrated, clinically, to be full thickness and associated with a small, localized, retinal detachment and a posterior vitreous separation. With one exception, all eyes were within three diopters of emmetropia, and only one eye developed progression of the retinal detachment. A high incidence of systemic estrogen therapy was noted in these patients.
A 71-year-old woman had von Willebrand's disease, an inheritable abnormality of platelet activity, and developed diabetic retinal neovascularization. A 59-year-old man had a myeoloproliferative disorder with thrombocythemia but may have had retinal changes before the development of his platelet dysfunction. The occurrence of diabetic proliferative retinopathy in patients with reduced platelet activity suggests that platelet induced microcirculatory abnormalities may not be required for neovascularization.
During rhegmatogenous retinal detachment, two major phagocytic cell populations can be identified in the subretinal fluid. Routine light and electron microscopic studies may be inadequate to assess quantitatively the free-floating retinal pigment epithelial cells and the macrophagic retinal pigment-laden cells. Enzyme histochemical staining permits a simple, rapid, reproducible method of identifying each cell type. This cytologic biochemical differentiation is an important tool for reexamination of the theories associated with subretinal fluid cell activity.
We measured immunoreactive insulin concentrations in ocular fluids and serum of 19 patients. In rhegmatogenous retinal detachment patients without diabetes mellitus, the serum insulin level was 7 +/- 2 (SE) microunits/ml and the subretinal fluid insulin levels were mostly undetectable. By contrast, in rhegmatogenous retinal detachment patients with diabetes mellitus, serum insulin was 35 +/- 5 microunits/ml and the subretinal fluid always contained low but detectable amojnts of insulin. The data may suggest a small leak of insulin into the subretinal fluid of rhegmatogenous retinal detachment patients with diabetic retinopathy. In vitrectomy patients with diabetes mellitus there was no insulin in the vitreous in eight out of nine cases.
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