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July 1951

ANGIOMA OF THE CHOROID: A Clinicopathologic Report of Two Cases of Partial and Complete Encephalotrigeminal Angiomatosis

AMA Arch Ophthalmol. 1951;46(1):39-48. doi:10.1001/archopht.1951.01700020044006

FOR MANY years the existence of an angioma of the choroid has been consistently noted in the relatively few eyes examined histologically from cases of the Sturge-Weber syndrome. The features of this syndrome are well known and offer no diagnostic problems. In fully developed form, they are (1) nevus flammeus of the face, involving the cutaneous and conjunctival distribution of the trigeminal nerve; (2) glaucoma, of either infantile (buphthalmic) or adult type; (3) neurologic signs—Jacksonian epilepsy affecting the opposite half of the body, hemiplegia, dyspituitarism, and meningeal telangiectasia, which may be calcific.

Less well known are the cases of choroidal angioma in which cutaneous stigmas are lacking and glaucoma may or may not be exhibited at the time of examination. Most of these tumors are found in eyes enucleated for absolute glaucoma (Lindenmeyer,1 1932; Hill and Dart,2 1936). Of particular interest and importance are the tumors which are

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