Macular fibrosis in Coats disease

The purpose of this study was to describe the angiographic features and visual impact of macular fibrosis in patients with Coats disease.

This is a single institution retrospective case series. Charts of patients diagnosed with Coats disease between 1973 and 2007 were reviewed. Data collected included patient demographics, treatment method, initial and final logarithm of the minimum angle of resolution visual acuity, photographic findings, angiographic characteristics, and anatomical outcome.

Forty-seven patients were identified with adequate imaging and posttreatment follow-up. Average age at presentation was 38 years (4-82 years). Average follow-up was 4.9 years (0-17 years). Macular fibrosis was identified in 11 patients (23%). At presentation, the average logarithm of the minimum angle of resolution visual acuity was as follows: all patients, 0.67; patients with macular fibrosis, 1.14; and patients without macular fibrosis, 0.50 (P = 0.01, 2-tailed Student's t-test). The average posttreatment logarithm of the minimum angle of resolution visual acuity was as follows: all patients, 0.78; patients with macular fibrosis, 0.97; and patients without macular fibrosis, 0.70 (P = 0.26). Macular fibrosis was associated with a pigmented spot at the point of apparent intraretinal vascular anastamosis. Fluorescein angiography showed leakage consistent with neovascularization that appeared to be intraretinal.

Macular fibrosis is a common finding in Coats disease, occurring in 23% of our patients. It may be a result of intraretinal neovascularization and is associated with a worse vision outcome.