Café-au-lait spots of the fundus in neurofibromatosis

Mild pigmentary changes found in the fundi of two patients with neurofibromatosis, resembled café-au-lait spots homologous with skin manifestations of the disease. In one, associated retinal hamartomas were present. The café-au-lait lesions were clinically distinct from previously reported uveal melanomas and may represent a hitherto unrecognized sign of ocular neurofibromatosis.