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Review
. 2019 Jun;67(6):763-771.
doi: 10.4103/ijo.IJO_841_19.

Coats Disease: An Overview of Classification, Management and Outcomes

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Free PMC article
Review

Coats Disease: An Overview of Classification, Management and Outcomes

Mrittika Sen et al. Indian J Ophthalmol. .
Free PMC article

Abstract

Coats disease is an idiopathic retinal vascular disorder with retinal telangiectasia with intraretinal and/or subretinal exudation without appreciable retinal or vitreal traction. The condition is sporadic with no associated systemic abnormalities. Unilateral involvement in young males is the typical presentation with most cases being diagnosed in the first and second decade of life. Younger the patient, more severe is the presentation and poorer the visual outcome. The management varies with the stage of the disease. Over the years, we have shifted from enucleation to a more conservative approach for the treatment of Coats disease with laser photocoagulation, cryotherapy and surgery for retinal detachment achieving good outcomes. The anti-VEGF agents have come into the scene as important form of adjuvant treatment along with the traditional management options. This article describes the clinical features, underlying pathology, classification and staging, the complications and the management of Coats disease and gives an overview of the changing trends in treatment and outcomes spanning across five decades.

Keywords: Coats disease; exudation; retina; telangiectasia.

Conflict of interest statement

None

Figures

Figure 1
Figure 1
Retinal telangiectasia and subretinal exudation characteristic of Coats disease
Figure 2
Figure 2
Advanced Coats with total exudative retinal detachment and intraretinal hemorrhage around peripheral telangiectatic vessels
Figure 3
Figure 3
Pupillary reflex in Coats and retinoblastoma. (a) Xanthocoria in a child with Coats disease. (b) leukocoria in a child with endophytic retinoblastoma
Figure 4
Figure 4
Stage 3A2 Coats disease with (a) foveal exudation, (b) peripheral telangiectasia and leakage on fluorescein angiography, and (c) subfoveal fluid and exudation on macular optical coherence tomography
Figure 5
Figure 5
Stage 2B Coats disease (a and b) before and (c and d) after treatment with laser photocoagulation. (a) Peripheral telangiectasia with exudation (b) dense organised intra- and subretinal exudation at the macula. After treatment with diode laser photocoagulation (c) resolution of telangiectasia and minimal residual peripheral exudation (d) drastic reduction of posterior pole exudation
Figure 6
Figure 6
Stage 3A2 Coats disease (a-c) before and (d-f) after treatment with cryotherapy and laser photocoagulation. (a) Macular exudative retinopathy with (b) fluorescein angiography demonstrating temporal macular leakage and disc staining and (c) peripheral nonperfusion with telangiectasia. At 6 months following treatment, there was resolution of (d) macular exudation, (e) vascular and disc staining, and (f) peripheral telangiectasia. Visual acuity increased from 20/400 initially to 20/40 at 6 months
Figure 7
Figure 7
Stage 3B Coats disease with (a) massive retinal detachment up to the crystalline lens, (b) total detachment on ultrasonography, (c) peripheral confluent telangiectasia with intraretinal hemorrhage overlying subretinal exudation, and (d) irregularly dilated retinal vessels and light bulb telangiectasia with leakage on fluorescein angiography
Figure 8
Figure 8
Stage 4 Coats disease showing bullous exudative retinal detachment and irregular, telangiectatic vessels in right eye of a 27 month old male child, treated with enucleation

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