This 36YO male presented with asymptomatic findings on fundus examination in his right eye. Vision was 20/25 in his right eye and 20/20 in his normal left eye.
Optos color RG imaging shows telangiectatic vessels in the temporal and superotemporal midperipheries. Subretinal lipid is most dense superotemporally.
Fluorescein angiography beautifully shows the telangiectasia, including capillary loss, microaneurysms, and macroaneurysms. He was referred to a retinal specialist for further management.
Learning Points:
Described by George Coats in 1908, Coats disease is an idiopathic disorder that is usually unilateral in males. It is characterized by retinal vascular telangiectasia involving capillaries, arterioles, and venules. Patients can present with a wide variety of findings ranging 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).
Atypical cases, including positive family history, bilateral findings, or in females, should be considered for genetic testing to rule out inherited telomere disorders, including Coats plus syndrome (Teixeira et al, Arch Ophthalmology 2008;126:134-135) and dyskeratosis congenita (Johnson et al, Ophthalmic Genetics 2009;30:181-184).
