Atypical presentation of Coats disease.

Abstract

To highlight the possibility of intraocular calcification in Coats disease and evaluate the ultrasound and computed tomographic findings. A 7.5-year-old boy had a 2-week history of unilateral glaucoma and leukocoria with retinal detachment, suggestive of Coats disease. The possibility of retinoblastoma, however, could not be excluded by ultrasound or computed tomographic examination, which revealed a retinal detachment overlying a subretinal mass with calcification. The blind, painful eye was subsequently enucleated. Histopathologically, there were telangiectatic retinal vessels in a fold of the detached retina peripherally and proteinaceous exudate in the subretinal space containing cholesterol clefts and foamy histiocytes, characteristic of Coats disease. There was also a fibro-osseous nodule in the macular area that correlated with intraocular calcification clinically. This case provides the first documentation of ancillary corroboration of intraocular bone formation in Coats disease, which, although rare, is an important consideration in the differential diagnosis of retinoblastoma.