Retinal Angiomatosis

RETINAL ANGIOMATOSIS, a rarely occurring and little studied disease, described by Hippel and Tschermak, initially treated as an arteriovenous aneurysm. The artery and vein supplying a particular area of the retina give enormous dilation and empty into a spherical tumor, in the form of a vascular node, patho-anatomically resembling an angioma, without any strong opacity of the retina, but with the addition of sharp reactive phenomena from the retinal tissue, expressed in a gliotic nature hyperplasia. This gave some authors (Meller) reason to consider the process as a diffuse gliosis of the retina with subsequent formation of angiomas. Later, retinal detachment forms. A. s. is not always strictly differentiated from another rare disease of the retina - retinitis exsudativa externa Coats, which may subsequently join A. s. The disease is often bilateral, familial. Cases of its combination with brain tumors (cerebellar cysts) are known, which gives reason to think of a congenital predisposition to multiple angiomas. Most cases of A. s. are observed in adolescence, and possibly this disease has its roots in early childhood. Some authors consider this precisely a feature of Coats' disease, not Hippel's. Treatment is currently unproductive.