Intraretinal calcification and osseous metaplasia in coats disease.
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OBJECTIVES: To review the frequency and nature of idiopathic macular telangiectasia and to classify the disorders based on new clinical and imaging observations. METHODS: A combined retrospective and prospective analysis of newly diagnosed patients seen over a period of 3 years. Patients were identified based on the Gass-Blodi classification and were studied with biomicroscopy, fluorescein angiography, and optical coherence tomography. RESULTS: Ten patients associated with aneurysmal telangiectasia (Gass-Blodi group 1) and 26 patients with perifoveal telangiectasia (Gass-Blodi group 2) were recruited. None with occlusive telangiectasia (Gass-Blodi group 3) were identified. New observations based on clinical, fluorescein angiographic, and optical coherence tomographic findings were made. CONCLUSIONS: Our series was similar to that in the Gass-Blodi study in terms of frequency. New observations in groups 1 and 2 have expanded our knowledge of the clinical spectrum of these disorders. A simplified classification termed idiopathic macular telangiectasia with 2 distinct types (type I, or aneurysmal telangiectasia, and type II, or perifoveal telangiectasia) was proposed to produce a better understanding of the entities and to enhance teaching and research. The third type, occlusive telangiectasia, has been omitted from our classification based on its rarity and presence of capillary nonperfusion rather than macular telangiectasia as the primary abnormality.
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Perforating diathermy coagulation can be used efficiently to destroy large angiomas of the retina. Five patients who had developed large angiomas (greater than 5 disc diameters) in association with various retinal diseases were treated with this technique. The follow-up period ranged from 1 1/2 to 7 years, except for one case in which the patient died five days after the surgical procedure. Several techniques of perforating diathermy are described, and the indications, surgical precautions, and complications of each method are discussed.
Three of four siblings of normal parents had a combination of Coats disease and retinitis pigmentosa bilaterally. Our report of their respective cases is, to our knowledge, the first report of a familial incidence of the combination of the two diseases in an individual and supports the implication that Coats disease may be a genetic abnormality. The pathological process was essentially the same in all three patients, although it occurred differently in one of the three siblings and gave rise to an altered functional and clinical picture. The similarities may indicate that the convenient categories of "tapeto-retinal degenerations" are clinical variations of the same genetic abnormality.
An 8-week-old infant had advanced Coats' disease. Six months after the Coats' disease was noted, a severe, secondary orbital cellulitis developed.
Acute disc swelling was documented in 21 eyes of 12 patients with long-standing juvenile diabetes. All but one patient were in the second or third decade of life, with a 13-year average duration of diabetes. Seventeen eyes had initial acuity of 20/50 or better, including nine eyes with 20/25 or better; disc swelling was asymptomatic in six eyes. Simultaneous bilateral disc swelling occurred in seven patients. With no specific therapy, vision generally recovered to normal levels within a few weeks, but a few patients retained arcuate, nerve fiber bundle, field defects and optic atrophy. There was no positive correlation with the degree of diabetic retinopathy, and disc swelling did not seem to be a harbinger of progressive retinopathy or proliferation at the nerve head. Disc swelling in juvenile diabetics represents a distinct clinical entity that must be distinguished from other causes of acquired nerve head elevation, especially papilledema of increased intracranial pressure.
The diagnosis of cone-rod dysfunction is made with the electroretinogram (ERG). Characteristically, the photopic ERG is worse than the scotopic ERG, and both are abnormal. Of a larger group of patients with retinal dystrophy, 20 cases of cone-rod dysfunction were identified. All patients had progressive disease, and all three main modes of inheritance were represented. There was remarkable similarity of findings among all patients, including temporal disc atrophy, telangiectasia of disc vessels, and little to no pigmentary retinal changes. Patients were not night-blind unless advanced disease was present. These patients suggest that neither telangiectasia nor temporal optic atrophy is pathognomonic for Leber's optic neuropathy nor dominant optic atrophy, respectively. Using a combination of electrophysiologic testing, fundus changes and modes of inheritance may prove helpful in better classifying the different types of retinal dystrophies, including retinitis pigmentosa.
A man complaining of blurred vision had diffuse retinal capillary telangiectasia as a primary retinal finding. Evaluation of the carotid arteries by a noninvasive angiographic method revealed bilateral, silent occlusions of both common carotid arteries. Thus, some cases of "idiopathic retinal capillary telangiectasia" may in fact be harbingers of underlying carotid artery disease.
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