CENTRAL RETINAL ARTERY-OCCLUSION IN A PATIENT WITH FABRYS-DISEASE DOCUMENTED BY SCANNING LASER OPHTHALMOSCOPY

Fabry's disease, angiokeratoma corporis diffusum, is an X-linked inbor n error of glycosphingolipid metabolism due to lack of activity of the lysosomal enzyme, alpha-galactosidase A, resulting in progressive int racellular deposition of neutral glycosphingolipids in various tissues , including vascular endothelial - and smooth muscle cells. Occlusions of the retinal vessels are rare. We present a case of central retinal artery occlusion in a 25-year-old male with Fabry's disease, document ed by fluorescein- and indocyanine green angiography performed by scan ning laser opththalmoscopy.