An interesting case of Coats' disease

Abstract

Coats' disease is an idiopathic exudative retinopathy characterized by abnormal retinal vascular development (telangiectasia) that results in massive intraretinal and subretinal lipid accumulation. This disease entity was first described by a Scottish ophthalmologist, George Coats, in 1908.1 It is a relatively rare disease, with a reported incidence of 0.09 per 100,000 of the population.2 Coats' disease is classically a unilateral process, usually affecting young males in (85%) cases, with a mean age of diagnosis at 6–16 years of age,3 without any racial predominance. It is sporadic and nonhereditary.4 An adult variant of the disease is less commonly seen and is frequently associated with hypercholesterolemia. Coats' disease mimics retinoblastoma, and may present with leucocoria and strabismus, which makes it that much more important for an early and accurate diagnosis.5 If left untreated the disease shows progressive worsening which can lead to neovascular glaucoma and phthisis bulbi, sometimes even requiring enucleation.3 Here we report an interesting case of Coats' disease, presenting to us with a severe diminution of vision. He was managed aggressively with intra-vitreal long acting steroids (triamcinolone), laser photocoagulation and finally with intra-vitreal anti-vascular endothelial growth factor (VEGF) agents, with a successful restoration of useful functional vision.