Coats’ Disease

In 1908, George Coats, curator of the Royal London Ophthalmic Hospital, described an ophthalmic disease that was typically unilateral, had a predilection for healthy males, and resulted in focal deposition of exudates within the fundus and “peculiar” retinal vascular findings. Four years later, Coats classified his cases of “exudative retinitis” into three groups. Group I manifested massive exudation but no discernable vascular abnormalities. Group II had marked vascular disease, intraretinal hemorrhage, and exudation. Group III presented with obvious arteriovenous malformations and exudation. Group III was later considered a retinal hemangioma. During this same time, Theodor Leber described a nonexudative retinal vascular degeneration characterized by “multiple miliary aneurysms.” Leber’s multiple miliary aneurysms are now believed to represent an early stage of Coats’ disease. In this chapter, we provide a comprehensive review of the pathogenesis, clinical findings, treatment options, and prognosis of Coats’ disease.