Intraretinal calcification and osseous metaplasia in coats disease.

We report a case of Coats disease in a 9-year-old boy who had a profound visual deficit and increasing pain in his right eye. Funduscopic examination revealed a complex, exudative retinal detachment with a subretinal mass and peripheral retinal telangiectasis. B-scan ultrasonography revealed a heterogeneously echogenic subretinal mass with several highly reflective foci consistent with calcification. Enucleation was performed and confirmed the diagnosis of Coats disease. Histopathological examination revealed the heretofore unreported finding of intraretinal calcification. In 1908, George Coats described a clinical entity characterized by telangiectasis, aneurysmal retinal vessels, and intraretinal and/or subretinal exudates in young males. In 1912, Leber reported a similar condition with telangiectasis and multiple retinal aneurysms but without the massive subretinal exudation found in Coats’ series. In 1956, Reese 3 observed that patients with multiple miliary aneurysms progressed to massive subretinal exudation and proposed that Leber’s and Coats’ cases represented a continuum of a single pathologic process. A recent review has further classified the criteria for Coats disease as idiopathic retinal telangiectasis, intraretinal and/or subretinal exudation, and frequent exudative retinal detachment without retinal or vitreal traction. Coats disease may be manifested at any age but is most often diagnosed between the ages of 5 and 11 years. It is typically unilateral, and approximately three fourths of the affected patients are male. The majority of patients have decreased vision, strabismus, or leukocoria. Retinoblastoma is the most commonprimary intraocularmalignancy in childhood and may be difficult to distinguish from Coats disease based onhistoryandophthalmologicexamination.Thepresenceofintraretinalcalcificationwithinaretinalmassstrongly supports the diagnosis of retinoblastoma. Herein, we present the clinicopathologic correlation of a patient with Coats disease consisting of a complex, exudative retinal detachment and subretinal mass with retinal telangiectasis. Although the history and clinical examination supported the diagnosis of Coats disease, retinoblastoma could not be definitively excluded because of the detection of apparent foci of intraretinal calcification with ultrasonography.