Orbital ganglioneuroma in a patient with chronic progressive proptosis.

cur within a subretinal mass in Coats disease. To our knowledge, this is the first report of intraretinal calcification in Coats disease. Intraretinal calcification classically occurs in retinoblastoma; however, it may also be detected in retinocytoma, tuberous sclerosis, and epiretinal membranes. This case reinforces the importance of a complete ophthalmic history, examination, and ancillary tests to distinguish Coats disease from retinoblastoma because intraretinal calcification may be present in Coats disease. Nearly one third of patients with Coats disease are referred to ocular oncology specialists for presumptive retinoblastoma. Distinguishing Coats disease from retinoblastoma may be difficult given the overlap of symptoms in a pediatric population. Moreover, ophthalmological examination alone is often insufficient to establish a diagnosis. Ultrasonographic and computed tomographic scans are frequently used to further evaluate the posterior pole for masses, retinal detachment, and calcification. Early diagnosis and treatment are critical inCoatsdisease.Patientswith retinal telangiectasis and retinal exudates generally have good visual outcomeswith laserphotocoagulation. In cases of Coats disease with retinal detachment, the visual outcomes are generallypoor.However,drainageof subretinal fluid, reattachment of the retina, and cryotherapy of retinal telangiectasis may halt the progression to neovascular glaucoma. Enucleation is performed in approximately 16% of patients with Coats disease. Enucleation is typically indicated in blind, painful eyes secondary to neovascular glaucoma. Enucleation was performed in this case primarily because of profound visual deficit and pain with little potential for visual rehabilitation with surgical retinal reattachment. Moreover, the presence of intraretinal calcification limited our ability to definitively exclude retinoblastoma. In conclusion, we present a case of Coats disease with extensive retinal pigment epithelium hypertrophy, osseous metaplasia, and intraretinal calcification. This is the first report of intraretinal calcification in Coats disease, and it emphasizes the challenge of distinguishing advanced Coats disease from retinoblastoma.