Cerebral tuberous sclerosis and Coats disease

1994 
: A 10-year-old boy with known tuberous sclerosis since early childhood suffered from unilateral Coats' disease. Initially his parents noticed a divergent squint. On examination, well-advanced Coats' disease involving almost the entire retina including the macula was found. Visual acuity had decreased to 20/300. Intrascleral diathermy was successfully performed, and after painful secondary glaucoma, cryotherapy of the ciliary body became necessary. Coats' disease has been reported in a number of genetic diseases, most of them quite variable in their clinical manifestations. To the best of our knowledge this is the first description of Coats' disease associated with tuberous sclerosis, which is known to be of genetic origin. Although in Coats' disease no genetic predisposition has been proven to date, the growing list of genetic diseases associated with Coats' disease should be considered in patients presenting with this retinopathy.
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