- 1.
What is Coats disease?
Exudation, retinal telangiectasia, and retinal aneurysms are hallmarks of the disorder, named for the British ophthalmologist George Coats, who first described this condition in 1908. It comes on painlessly and may be slow and insidious in its development. In many instances, Coats disease is not discovered until the patient is beyond childhood.
- 2.
List the clinical characteristics of Coats disease.
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It is a lifetime disease.
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It occurs 80% to 90% of the time in young boys.
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It is usually unilateral.
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It is not familial.
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Characteristic retinal vascular lesions are telangiectatic-like “light bulb” aneurysms that are associated with capillary dropout in the fundus periphery ( Fig. 41-1, B and D ).
Figure 41-1
A, Pretreatment photograph of exudate in the posterior pole of a 10-year-old male with Coats disease. B, Peripheral vascular retinal changes of patient shown in A. C, Although Coats disease predominantly affects males, females may also develop the disease, as seen in this 9-month-old girl. D, Peripheral retinal vascular changes are present in the temporal periphery of the patient shown in C. E, The 9-month-old baby girl is now 22 years of age and has not had a recurrence. F, After resorption of the exudate in the patient shown in E the OCT shows a normal macular appearance, but best corrected vision is only 20/200 despite patching in childhood. G, Exudate disappearing after laser treatment. H, Microaneurysmal changes in a patient with Coats disease. I, Histopathologic section of the retina in a patient with Coats disease with bullous retinal detachment almost touching the posterior lens capsule secondary. Aneurysmal changes can be seen in the nerve fiber layer. (Courtesy of Dr. Ralph Eagle.) J, Recurrent Coats in a 26-year-old male diagnosed at 5 years of age. Exudate is beginning to diminish 3 months after retreatment. K, 27-year-old male with retinitis pigmentosa and Coats disease. L, The patient in J has reduced and delayed hertz (hz) cone electroretinogram both eyes compatible with retinitis pigmentosa.
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Intraretinal and subretinal exudation, a prominent feature, has a predilection to accumulate in the macular area ( Fig. 41-1, A and C ); the exudate contains cholesterol crystals.
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Coats disease may lead to exudative retinal detachment, cataract, neovascular glaucoma, and phthisis bulbi.
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- 3.
What percentage of patients are girls?
Between 8% and 10% of patients are girls.
- 4.
What is the most common age at which Coats disease becomes apparent?
Coats disease usually becomes apparent between 8 and 10 years of age. However, it can present in infancy and later in life. It is often much more severe when noted in infancy.
- 5.
What percentage of cases are unilateral versus bilateral?
Approximately 80% to 90% of the cases are unilateral. When bilateral cases do develop, there is usually asymmetry, with one eye being much more involved than the other.
- 6.
Are the retinal vascular changes easy to detect?
If the patient is cooperative, it is not hard to diagnose the peripheral retinal vascular changes. However, examination under general anesthesia may be necessary in younger patients.
- 7.
How does this condition differ from Leber’s miliary aneurysms?
In 1912, Leber described retinal miliary aneurysms. He suggested that the conditions were one and the same as that reported by Coats, and that is the generally accepted thinking at the present time.
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Do we know the etiology of Coats disease?
The precise etiology for Coats disease has not been determined.
- 9.
Are there any conditions with which Coats disease can be confused?
When there is exudation in the macula and peripheral telangiectasia, and no retinal detachment, the Coats disease can be diagnosed with confidence. There are a number of conditions to rule out, most notably retinoblastoma, the malignant intraocular tumor that occurs in infancy and childhood. It has been estimated that approximately 3.9% of eyes originally diagnosed as harboring retinoblastoma were subsequently discovered to have Coats disease. See Table 41-1 .
Table 41-1
Differential Diagnosis of Coats Disease
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Retinoblastoma
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Familial exudative vitreoretinopathy
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Von Hippel-Lindau disease
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Retinopathy of prematurity
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Persistent fetal vasculature
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