Classification and management of Coats disease: the 2000 Proctor Lecture

A population-based study of Coats disease in the United Kingdom I: epidemiology and clinical features at diagnosis

“…Eyes having stage 1 at initial assessment are very rare, just 1% in Shields series 6 and none at all in our population-based study. Severe end stage eyes (stage 4 and 5) were rare in our series with just four cases (7%) compared with 17% for Shields.…”

“…1,5,6 While our series is smaller it is significantly different in several respects and because it is population based, more likely to be representative of the typical spectrum of Coats. Shields reported a mean age at presentation of 11 years (132 months), comparable to our finding of 146 months.…”

“…The staging of Coats disease followed that described by Shields. 6 Stage 1 has telangiectasia only; stage 2 is characterised by telangiectasia plus exudation (2A extrafoveal exudation; 2B foveal exudation); stage 3 is characterised by exudative retinal detachment (3A subtotal; 3B total). Stage 4 has total retinal detachment with secondary glaucoma, and stage 5 is advanced end stage disease.…”

“…Stage 4 has total retinal detachment with secondary glaucoma, and stage 5 is advanced end stage disease. 6 We enquired about investigations carried out and the initial treatments employed. Finally, we enquired whether there was a family history of eye disease.…”

“…1,[5][6][7] While there are a number of published case series of Coats disease, these mostly originate from large tertiary referrals centres and perhaps give a skewed view of the severity of Coats disease. 1,5,6 We conducted a prospective study of Coats disease in the United Kingdom, which would provide a more representative picture of the full clinical spectrum of Coats disease, current treatment preferences and their anatomic and visual outcomes. This information will hopefully be very informative to clinicians and should allow them to plan treatment for patients with Coats and to counsel patients and parents.…”