Coats' disease of adult-onset in 48 eyes

Abstract

Background: Coats' disease diagnosed in adulthood is an idiopathic, retinal exudative vascular disease without an inciting factor and has retinal features different from the childhood disease.

Aim: To describe clinical features, treatment, and outcomes of eyes with Coats' disease first diagnosed in patients 35 years or older.

Materials and methods: Retrospective chart review of patients first diagnosed with Coats' disease at the age of 35 years or more at a tertiary eye care center between January 1995 and 2012. Eyes with retinal exudation or Coats'-like response from secondary causes were excluded.

Results: Forty-five of 646 patients (7%) diagnosed with Coats' disease had adult-onset disease. Mean age at presentation was 47 years. Systemic hypertension was the most common (22%) systemic association and decreased vision the predominant presenting feature (83%). Localized (<6 clock h) presentation (74%) was unique to adults as against diffuse involvement (69%) in children (P < 0.001). Eyes were treated with laser photocoagulation 29 (60%), cryotherapy (4%), or both (2%) with surgical intervention in three (6%) eyes. Following treatment eight (35%) eyes improved, 11 (48%) eyes were stable while four (12%) eyes worsened due to complications.

Conclusion: Adult-onset Coats' disease has less extensive involvement, more benign natural course, and a more favorable treatment outcome as against the childhood-onset disease. The bilateral presentation emphasizes the need for regular follow-up to detect possible future involvement of the fellow eye.

Figure 1

(a) Color fundus montage picture revealing telangiectasias in the temporal periphery with the presence of exudates and subretinal fluid. (b) Fundus fluorescein angiogram (arteriovenous phase) of the corresponding area depicting telangiectasias with typical light bulb appearance with areas of capillary nonperfusion

Figure 2

Bar diagram depicting the pre- and post-treatment visual acuity of eyes treated with laser photocoagulation

Figure 3

(a) Diffuse retinal thickness with subfoveal fluid (b) following treatment foveal thickness reduced with a resolution of fluid and subretinal scarring (c) near normal macula at presentation (d) lamellar macular hole and chronic cystoid changes developed despite laser to peripheral lesions. (e) Diffuse and spongiform retinal thickness with massive schitic changes (f) line scan through telangiectatic vessel shows area of signal void with shadowing (g) subfoveal scarring and adjacent diffuse retinal thickening (h) line scan through organized retinal exudation reveals increased retinal thickening with shadowing and back-scattering