Summary
Coats disease is an idiopathic disorder characterized by an abnormal development of retinal vessels (telangiectasia) with a progressive deposition of intraretinal or subretinal exudates, potentially leading to exudative retinal detachment. Coats disease is classically an isolated and unilateral condition affecting mainly young males. The onset of the clinical symptoms usually occurs at an early age, with an incidence peak between 6 and 8 years. The etiology of Coats disease remains largely unknown, but reported associations with different genetic syndromes emphasize the hypothesis of a genetic component. The abnormal permeability of the capillary endothelial cells in the retina is thought to be the underlying histopathological mechanism. The advanced stages of Coats disease (total retinal detachment, leukocoria, painful glaucoma secondary to angle closure) are difficult to differentiate from retinoblastoma. The diagnostic methods include indirect ophthalmoscopy, fluorescein angiography, ultrasonography, fine-needle aspiration biopsy, computerized tomography (CT) scanning and magnetic resonance imaging (MRI). The treatment is directed towards closure of the abnormal leaking retinal vessels to facilitate the resolution of exudation and retinal detachment. Available treatments include laser therapy and cryotherapy in the early stages. More advanced cases require surgical techniques for retinal reattachment, such as scleral buckling, pars plana vitrectomy and removal of the vitreous membrane. Stabilization of the disease course or clinical improvement can be currently achieved in 70% of the cases using a carefully selected therapy. *Author: Dr A. Del Longo (September 2004)*.
