Features
Coats disease, first described in 1908, is a sporadic vascular disorder of the retina. The mean age of presentation is 5 years and is most commonly seen unilaterally in males. No direct genetic or racial association has been found in idiopathic Coats disease. A retinal–blood barrier disruption exists in pathological vessels. Inflammatory mediators are released, including vascular endothelial growth factor (VEGF), increasing vascular permeability that leads to exudative retinal detachment. As the disease progresses, hypoperfusion, ischemia, vitreoretinal pathological organization (preretinal fibrosis, subretinal fibrosis, or a combination), and retinoschisis or “retinal macrocysts” occur. It was classified in 2001 into the following stages depending on the severity.
Stage 1: Retinal telangiectasia.
Stage 2: Retinal telangiectasia and exudates (2A extrafoveal, 2B foveal).
Stage 3: Exudative retinal detachment (3A partial [extrafoveal or fovea] or 3B total).
Stage 4: Total retinal detachment and neovascular glaucoma.
Stage 5: End-stage disease with or without phthisis bulbi.
99.1.1 Common Symptoms
Photopsia, and floaters common; decreased vision; disease progression can result in severe vision loss.
99.1.2 Exam Findings
Retinal vascular telangiectatic vessels, mild to extensive exudates, and “light bulb” aneurysms may be present (▶ Fig. 99.1, ▶ Fig. 99.2). In more severe cases, exudative retinal detachments may be seen (▶ Fig. 99.3). Deprivational strabismus can occur in early life. Leukocoria can be seen with a total retinal detachment, cataract, and/or cholesterol in anterior chamber. Less frequent, retinoschisis (retinal “macrocyst”), or vasoproliferative tumors (retinal angiomas or pseudoangiomatous retinal gliosis) can appear.
99.2 Key Diagnostic Tests and Findings
99.2.1 Optical Coherence Tomography
Hyperreflective cystic changes consistent with cystoid macular edema or schisis-like changes in the retina may be present. Subretinal fluid may be present. Hyperreflective lesions are exudates, subretinal fibrosis, sub-RPE fibrosis, and preretinal membranes. Optical coherence tomography is useful to guide treatment of cystoid macular edema, retinal detachments, and for surgical planning.
99.2.2 Fluorescein Angiography or Ultra-Widefield Fluorescein Angiography
Ultra-widefield fluorescein angiography is often instrumental for diagnosis and guiding treatment. Telangiectatic/aneurysmal hyperfluorescent lesions are typically present. Vascular leakage, pooling in the subretinal space, staining of fibrosis or scarring tissue, and leakage from neovascularization may all be present. Hypofluorescence indicative of capillary nonperfusion and retinal hemorrhages may be present. The fellow eye can present also vascular anomalies (e.g., aneurysms, peripheral avascular retina; ▶ Fig. 99.1, ▶ Fig. 99.2, ▶ Fig. 99.3).
Fig. 99.1 (a) Widefield fundus photograph demonstrating yellow exudates that resemble a mass with overlying vascular abnormalities. (b) Widefield fluorescein angiography reveals vascular filling abnormalities and pooling. (c) Following 2 years of therapy and quiescence, widefield fundus photography document reactivation is noted as a yellow lesion posterior to the laser scars and temporal to the fovea.
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