Coats disease

Coats disease is a rare, non-hereditary eye disorder characterized by abnormal development of the blood vessels in the retina (retinal telangiectasia). The condition typically affects only one eye (unilateral) and predominantly occurs in young males. In Coats disease, the retinal blood vessels are malformed and leaky, leading to the accumulation of lipid-rich fluid (exudate) beneath and within the retina. Over time, this progressive exudation can cause retinal detachment, vision loss, and in severe cases, secondary complications such as neovascular glaucoma, cataracts, and phthisis bulbi (shrinkage of the eye). The disease is sometimes referred to as retinal telangiectasia or exudative retinitis. The condition primarily affects the eye and visual system. Early stages may be asymptomatic or present with decreased visual acuity, strabismus (misalignment of the eyes), or leukocoria (a white pupillary reflex), which can mimic retinoblastoma and must be carefully differentiated from it. The disease is classified into five stages based on severity, ranging from retinal telangiectasia alone (Stage 1) to advanced end-stage disease with total retinal detachment and secondary complications (Stage 5). Treatment depends on the stage of the disease and aims to destroy the abnormal blood vessels and reduce exudation. Options include laser photocoagulation, cryotherapy (freezing treatment), and intravitreal injections of anti-VEGF (vascular endothelial growth factor) agents. In advanced cases, vitreoretinal surgery may be necessary to reattach the retina. Early detection and treatment are critical for preserving vision, though the prognosis varies widely depending on the stage at diagnosis. There is currently no cure, and some patients may ultimately lose vision in the affected eye despite treatment.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Coats disease