Clinically, Coats disease presents with:
- decreased vision.
- strabismus.
- leukocoria.
(white pupillary reflex or cats eye reflex), and redness.
It may, however, be detected incidentally
It is not generally associated with pain, except for in advanced cases with secondary associated neovascular glaucoma.
It has not been associated with infectious or inflammatory conditions, and the great majority of patients have no associated systemic medical problems.
The clinical presentation shows gr
eat variability.
Typically, the ophthalmoscopic examination
reveals:
- A localized area of subretinal exudates associated with vascular abnormalities.
- Vascular changes may include peripheral retinal telangiectasia, capillary and small vessel dilatation and tortuosity, sheathing, capillary nonperfusion, and small aneurysms located at the equator of the eye and the ora serrata (most commonly, inferotemporal).
- The posterior pole is less frequently affected.
- Exudation is a common feature that presents in most cases as flat intraretinal and subretinal exudates. These exudates initially appear in areas of telangiectasia and progress to become more widespread.
- Macular protein accumulation can occur directly from macular telangiectasia or indirectly from peripheral disease.
- A dense exudate or white nodule in the macula can progress to a disciform lesion that indicates
- a poor visual prognosis.
- Retinal hemorrhages may be seen.
- The vitreous remains clear until the advanced stages.
- Retinal cysts may be seen and are common in chronic retinal detachments of different etiologies.
The diagnosis may be suspected clinically by:
- Ancillary testing is extremely helpful to differentiate Coats disease from retinoblastoma.
- Intravenous fluorescein angiography helps to visualize the telangiectatic vessels as irregular dilated tortuous vessels filling in the late arterial and early venous phases. Microaneurysms are seen as "lightbulb aneurysms". The angiogram will also show progressive leakage from abnormal vessels, adjacent areas of capillary dropout, and late staining of intraretinal exudates.
Based on the clinical appearance and progression, Coats disease may be classified in 5 grades, as follows:
- Grade I: Isolated focal exudates.
- Grade II: Massive elevated exudation.
- Grade III: Partial retinal detachment.
- Grade IV: Total retinal detachment.
- Grade V: Secondary complications.
A more recent classification system has emerged, which is based on the prognosis. It grades the disease in the following manner:
- Grade I: Telangiectasias only.
- Grade II: Telangiectasias and exudation.
- Grade III: Exudative retinal detachment.
- Grade IV: Total retinal detachment with secondary glaucoma.
- Grade V: End-stage disease.
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