International Consensuses and Guidelines on Diagnosing and Managing Coats Disease by the Academia Retina Internationalis (ARI), the Asia-Pacific Vitreo-retina Society (APVRS), and the Academy of the Asia-Pacific Professors of Ophthalmology (AAPPO)

Abstract

PURPOSE: Coats disease is a rare, retinal vascular disorder characterized by telangiectasias, aneurysmal dilations, and progressive exudative retinal detachment. Limited understanding of the disease warranted the need to identify controversial issues through an extensive literature search and a debate and discussion among international panels of experts. METHODS: Extensive literature search was done on multiple aspects of the disease-classification patterns, disease, Coats-plus, and Coats-like response. Other key factors included the etiology, possible genetic patterns, relationship with other vascular disorders, the role of inflammatory factors and vascular endothelial growth factor (VEGF) in the pathogenesis, diagnostic features, and complications. Considering the varying treatment patterns followed, imaging modalities, clinical findings, differential diagnosis, treatment options, prognostic factors, and emerging concepts in management were all covered in the search. Eighteen experts were included to opine on questions spanning classification, pathogenesis, diagnostic and treatment methods, prognostic controversies, and newer concepts in disease management. RESULTS: Of the 52 questions in 7 sections, the experts arrived at a consensus for 48 (92.3%) statements (with 75% voted as strong agreement or agreement). Most experts agreed on the suggested classification, diagnosis, and prognosis. The controversy, however, remained in questions regarding association with other vascular disorders, treatment of stages 3 and 4, possible benefits of newer anti-VEGF agents, and the role of artificial intelligence. CONCLUSIONS: These debates reflect the rarity of the condition, complex pathophysiology, and the challenges of treating a progressive blinding disease primarily affecting children. Hence, further studies are warranted, especially in areas that have not reached a consensus. (Am J Ophthalmol 2026;282: 162-186. (c) 2025 The Author(s). Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/))