[Dystrophia albipunctata retinae and fundus albipunctatus].
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Biomedical subjects
Publications and source records attributed to T Streicher.
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The authors describe a family with autosomal-dominant inheritance of hereditary Bruch's membrane drusen. After the death of the proband who was kept under clinical observation with fluorescein-angiographic examination over a period of seven years, the authors obtained the bulbus for histopathologic study. Light microscopic changes in the boundary-line structures of the retina and choroid and the occurrence of an intraretinal membrane in the maculopapillary bunch area are described. This membrane is thought to represent a "predisciform stage" in the development of this hereditary dystrophy.
Examination of a bulbus from a patient with hereditary drusen of Bruch's membrane revealed gradual development of drusen from fingerlike and fungous projections of the basal membrane of the retinal pigment epithelium culminating in large deposits with a conspicuous radial and laminar arrangement. The granular material which formed the drusen contained cellular debris as well as fragments of the fibrous long-spacing collagen. Cells of the pigment epithelium proliferated as metaplastic glial cells with formation of a new stratum separating the layer of cones and rods from Bruch's membrane. Transmembranous fibrovascular proliferation from the choroid into the periretinal space was not encountered and does not seem to play a decisive role in the pathogenesis of the disease.
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The authors present five patients with dominant hereditary colloid bodies of the retinal centre from two family trees. Their fluoroangiographical pictures confirm the variable clinical manifestations and ophthalmological findings which because of the special colloid bodies arrangement show a resemblance to the Hutchinson-Tay, Holthouse-Batton, Doyne and Klaingutti cases, also within the framework of a single family tree. With the help of the angiogram the type and development stage can be followed, while this disease passes from functionally and ophthalmologically unimportant lesions into the final stage of atrophy and the fibrous conversion of the whole rear eye pole.
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