By J.J. de Laey, M. Hanssens

This record for the Belgian Ophthalmological Society issues a couple of fundus ailments, which so much ophthalmologists in simple terms hardly come upon. nonetheless it's of value that they need to be capable of realize them, not just simply because almost all these eye ailments are treatable after they are clinically determined early adequate, but in addition simply because they're occasionally linked to significant visceral and neurological difficulties. The ophthalmologist could be the first practitioner to suspect a von Hippel-Lindau's sickness or a neurofibromatosis. He may still observe at that second that his function is not just to regard the eyes but additionally to suggest his sufferer, refer him to different experts and think about the genetical implications of his analysis. Clinicopathologic correlations are of serious significance and we felt that it was once essential to emphasize the histopathological point of those ailments. A record has to assessment the literature on a particular subject. The literature on vascular tumors of the fundus is intensely immense or even if we attempted to be as whole as attainable, we needs to confess that we didn't cite each writer who released on such circumstances. a 3rd element, which was once regarded as vital, was once to supply enough medical and histopathologic examples of a number of the disorder, that are reviewed. we're indebted to plenty of associates who kindly approved the book in their fabric. particularly we want thank Prof. A. Brini (Strasbourg), Dr. D. de Wolff-Rouendaal (Leiden), Prof. P.

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Elliot [20] in 1957 found less than 170 cases in the literature, whereas Go et al. [29] compared in 1981 their cases with 384 other reported cases. Retinal angiomatosis has no race nor sex predilection; women are as commonly affected as men. The ocular lesions usually become symptomatic in the third decade. 2 years. Systematic family investigation however allows an earlier diagnosis even in children or in babies. The youngest patient with retinal angiomatosis was one day old [56]. On the other hand patients of over 80 years of age may still be asymptomatic [29].

3. Nests of round to polyhedral cells Nests of round to polyhedral cells (described as interstitial cells [64], stromal cells [44, 61, 92], foam cells [89] or pseudo-xanthoma cells [5, 69]) can be found between the vascular elements. They have a clear [64, 92], foamy [44, 69,43,92], vacuolated [64], honeycombed [69] or heavily lipidized cytoplasm [5, 44, 69, 89] with positive fat-staining of the vacuoles [64]. On electron microscopy the vacuoles contain either lipid consistent with saturated, neutral lipid [64] or nonosmophilic lipid [61].

Schmidt D and Neumann HPH (1987): Atypische retinale Veranderungen bei HippelLindau Syndrom. Fortschr. 84: 187-189. Seidel E (1932): Anatomische Friihstadien der AngiomatOSIs retinae (v. Hippelsche Erkrankung) Ber Dtsche Ophthalmol Ges, 49: 535-541. , 89: 529-543. Shields JF (1983): Vascular tumors of the retina and optic disc. in Diagnosis and management of intra-ocular tumors, the CV Mosby Co, St Louis, Toronto, London, 534-568. Spence AH and Rubinstein LJ (1975): Cerebellar capillary hemangioblastoma: its histogenesis studied by organ culture and electron microscopy.

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