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Minerva Oftalmologica 2012 September;54(3):135-47

language: Italian

Coats’ disease

Schiemer S. 1, Velotti N. 2, Soda M. 3, Chiariello Vecchio E. 4

1 Dipartimento di Scienze Oftalmologiche, Università degli Studi di Napoli, “Federico II”, Napoli, Italia;
2 Dipartimento di Scienze Oftalmologiche, Università degli Studi di Napoli, “Federico II”, Napoli, Italia;
3 Dipartimento di Scienze Oftalmologiche, Università degli Studi di Napoli, “Federico II”, Napoli, Italia;
4 Dipartimento di Scienze Oftalmologiche, Università degli Studi di Napoli , Federico II”, Napoli, Italia


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Coats’ disease is a non-hereditary disorder characterized by idiopathic retinal telangiectasia associated with intraretinal and/or subretinal exudation, frequently leading to exudative retinal detachment without signs of appreciable retinal or vitreal traction. Coats’ disease is classically isolated, unilateral and affects mainly young males. Most of the diagnosis is made within the first two decades of life, with a peak incidence between ages 5 and 8. When the disease appears in adults it usually exhibits a more benign clinical course. The etiology of Coats’ disease remains almost unknown, even if some reports describe associations with different genetic syndromes, which emphasize the hypothesis of a genetic component. The increased vascular permeability of retinal capillaries, caused by loss of vascular endothelial cells and pericytes, is thought to be the underlying histopathological mechanism. The clinical course of Coats’ disease is variable, but mainly progressive; the disease is often diagnosed by chance and it is important to differentiate the advanced stages from retinoblastoma. The diagnostic methods include indirect ophthalmoscopy, fluorescein angiography, ultrasonography, CT scan, MR imaging, fine-needle aspiration biopsy, and recently OCT. The standard treatment is directed towards obliteration of the abnormal leaking retinal vessels in the early stages through laser photocoagulation or cryotherapy. Some recent reports have demonstrated the efficacy of intravitreal antivascular endothelial growth factor (VEGF) agents, in association with standard therapy, in reducing macular edema and promote resorption of exudation; more advanced stages require a surgical approach.

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