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SYMPTOMS
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Leukocoria, Decrease in vision, Eye turn
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SIGNS
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Typically unilateral (95% of the time)
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Retinal telangiectasias, Dilated capillaries with microaneurysms, Extensive retinal exudates, Venous sheathing, Retinal detachments, Rubeosis iridis, Secondary angle closure glaucoma
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WORK-UP
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Pupils, EOMs, Cover test, Gonioscopy, Full eye exam with dilated retinal exam, OCT analysis of the macula, OCT-Angiography, Fundus Autofluorescence, Fluorescein Angiography, Fundus photos, B-scan ultrasound, Amsler grid
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TREATMENT
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If there is minimal telangiectatic retinal vessels with little to no exudation in the peripheral retina and no symptoms, the patient can be monitored closely. If this is not the case, the patient needs to be referred to a retinal specialist or glaucoma specialist or pediatric ophthalmologist ASAP for further evaluation and treatment
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FOLLOW-UP
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If monitoring, the patient needs to be watched closely and seen back every 3-4 months
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The patient most likely will continue to be followed up by a retinal specialist/pediatric ophthalmologist/glaucoma specialist on a regular basis if the patient has extensive retinal telangiectasias, extensive exudation, and/or complications secondary to Coats disease
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ETIOLOGY
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Idiopathic retinal vascular disorder that occurs due to increased permeability of retinal capillaries and abnormal pericytes. May be related to a mutation in the NDP gene (Somatic mutation that is noninheritable)
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DIFFERENTIAL DX
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Retinopathy of prematurity, Familial exudative vitreoretinopathy, Retinal capillary hemangioma, Retinoblastoma
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NOTES
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Typically affects male patients (85% of the time)
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