This healthy 6YO girl’s parents noted that her right eye had been deviating outward for the past several weeks. Vision was light perception OD and 20/20 OS.
Color photography shows fairly dense subfoveal lipid with more diffuse specks of subretinal lipid radiating outwards from the disc. Telangiectatic vessels are noted temporally OD and also in the left inferotemporal periphery. Fluorescein angiography shows diffuse leakage from these vessels OD, with distal retinal ischemia OS.
Scatter laser to the ischemic retina, along with focal treatment of the larger telangiectatic vessels, was applied. Nine months later, vision was unchanged, with consolidation of the subfoveal lipid scar OD along with persistent telangiectasia OS.
Learning Points:
Described by George Coats in 1908, Coats disease is an idiopathic disorder typically unilateral in males. It is characterized by retinal vascular telangiectasia involving capillaries, arterioles, and venules. Patients can present with a wide range of findings, from minimal exudation to total exudative retinal detachment. The leaking vessels are usually treated to prevent exudative complications. Thermal laser photocoagulation is usually the initial modality, but anti-VEGF injections can also be used to stabilize the vascular leakage. Patients will often require multiple rounds of treatment. See Sigler et al for a review of the current management (Survey Ophthalmology 2014;59:30-46).
Dyskeratosis congenita or familial exudative vitreoretinopathy should be considered for patients with frank bilateral disease (Teixeira et al, Arch Ophthalmol 2008;126:134-135), in women with Coats-like findings (Johnson et al, Genetics 2009;30:181-184), or in patients with a family history of similar findings. Our patient’s family history was negative, and her nail beds were normal. Genetic testing was not possible due to cost concerns.
