The fundus photos show parafoveal microaneurysms in the right eye and superotemporal microaneurysms in the left eye. OCT reveals juxtafoveal edema in the right eye only. OCTA of the right eye reveals microaneurysms and capillary nonperfusion that is most pronounced in the middle capillary layer and is less severe in the inner capillary layer. OCTA of the left eye reveals less capillary nonperfusion, and it is also greatest in the middle capillary layer. Fluorescein angiography of the right eye reveals microaneurysms with late leakage. Fluorescein angiography of the left eye shows fewer microaneurysms, but interestingly there is subtle telangiectasia of capillaries in the temporal macula and temporal to the macula as well as in the temporal periphery.
This patient’s clinical presentation is consistent with idiopathic macular telangiectasia (IMT) type 1, which is likely a variant of Coats’ disease. The bilateral presentation is very unusual, but it has been reported. There were other diagnostic considerations. Diabetic retinopathy can present with microaneurysms and capillary nonperfusion, but there are typically hemorrhages and the dilated, telangiectatic capillaries in the left eye would be very atypical. Our patient was recently tested for diabetes, and results were negative. Other causes of microaneurysms and capillary nonperfusion include hypertension and radiation retinopathy.
In terms of treatment, laser photocoagulation can be effective. However, the juxtafoveal location of the vasculopathy in our patient discourages this treatment. Anti-VEGF treatment has a variable response in Coats’ disease, and it would be reasonable to treat this patient if the edema worsened or if there were changes in visual acuity or visual function.
References
Gass JD, Blodi BA. Idiopathic juxtafoveal retinal telangiectasias. Update of classification and follow-up study. Ophthalmology 1993;100:1536-1546.
Christakis PG, Fine HF, Wiley HE. The diagnosis and management of macular telangiectasia. Ophthalmic Surgery, Lasers and Imaging Retina. 2019;5);139-144.
Rishi E, Rishi P, Appukuttan B, Uparkar M, Sharma T, Gopal L. Coats’ disease of adult-onset in 48 eyes. Indian Journal of Ophthalmology 2016;64:518-523.
