Coats' disease diagnosed in adulthood.

PURPOSE

To investigate the manifestations and clinical course of Coats' disease diagnosed in adulthood and to describe the characteristics of patients who develop it.

DESIGN

Retrospective observational case series.

PARTICIPANTS

Thirteen eyes of 13 patients with Coats' disease diagnosed after age 35 years.

METHODS

All patients examined in the authors' referral practices for Coats' disease diagnosed after the age of 35 years were identified. Patients with a history of radiation exposure, intraocular inflammation, retinal vascular occlusion, or Coats' response were excluded. A review of their historical, clinical, and fluorescein angiographic features and demographics was performed.

RESULTS

There were 13 patients with a mean age at diagnosis of 50 years (range, 36-79) and a mean follow-up of 5.8 years (range, 0-17). These patients manifested many findings typical of Coats' disease, including the unilateral nature of the disease (13/13 patients), male predominance (12/13), vascular telangiectasis, lipid exudation, macular edema, and areas of capillary nonperfusion with adjacent webs of filigreelike capillaries. Disease was limited to < or =6 clock hours in 10 of 13 patients (76.9%), did not generally involve the retinal vasculature anterior to the equator, and showed limited potential to expand during the follow-up period. A localized hemorrhage was noted in 10 of 13 patients (76.9%), almost always near larger aneurysmal dilatations. Limited exudative detachment of the retina was seen in 4 eyes, 3 of which responded to localized laser photocoagulation. One patient had subretinal fluid seen on ocular coherence tomography that did not require treatment. On average, patients lost 2.1 lines of visual acuity during the follow-up period; 6 patients had a decline in vision, 2 patients had improved vision, and 3 patients had stable vision. No patient had end-stage findings of Coats' disease, such as iris neovascularization or total exudative detachment.

CONCLUSIONS

Coats' disease can first be diagnosed in adulthood with retinal vascular abnormalities similar to those seen in younger patients. There are a number of important differences in disease manifestation in adults, including limited area of involvement, slower apparent progression of disease, and hemorrhage near larger vascular dilatations.