Diagnosis and Treatment of Coats’ Disease: A Review of the Literature

Abstract

Coats’ disease is an idiopathic, ophthalmic condition characterized by retinal telangiectasis, intraretinal and subretinal exudation, which can lead to retinal detachment. It is mostly unilateral, progressive and affects mainly males during childhood, although adult cases have also been described. In this review, we make an update of the literature about Coats’ disease, emphasizing on diagnosis and treatment, including the most recent treatment modalities, i.e. anti-vascular endothelial growth factor agents.

Doença de Coats em Estadio Avançado: O Desafio do Diagnóstico

“…Na fase avançada em que se encontrava a doença, não existia indicação nem para o tratamento com fotocoagulação ou crioterapia, preconizado para estadios mais precoces de forma a destruir os vasos anómalos e reduzir a exsudação, 8 nem para cirurgia vítreo-retiniana do descolamento de retina. Por outro lado, afastada a hipótese de retinoblastoma e não sendo um olho doloroso, descartou-se a necessidade de uma enucleação, optando-se apenas pela observação e controlo da pressão intraocular elevada associada ao glaucoma neovascular.…”

Ranibizumab in the Management of Advanced Coats Disease Stages 3b and 4

“…3 As an alternative to these only partially successful surgical approaches, and following analysis of subretinal fluid and aqueous humor that revealed an increased VEGF level in eyes with Coats disease, 14,15 the use of anti-VEGF has already been reported to be effective in lowering VEGF levels and reducing exudates in pediatric Coats disease. 16 This prompted us to test the role of ranibizumab as neoadjuvent or adjuvant to potentialize the effectiveness of classic treatment in advanced disease Stages 3B and 4 to reduce exudation by transient restoration of the hemato-retinal barrier and suppression of neovascularization.…”

George Coats and His Disease

“…Ghorbanian et al [1] revert to Coat’s midway in their otherwise fluent review of this intriguing condition in Ophthalmologica .…”

“…Moreover, the authors state that ‘it was first described in 1908 by George Coats, a Scottish medical student, as a unilateral condition with retinal exudation and telangiectasis in male children’ [1]. Coats graduated from Glasgow University in 1897 and received his doctorate in 1901 while a resident at its Eye Infirmary [2].…”

“…Regarding current diagnostics, one would like to add to the succinct review of Ghorbanian et al [1] that magnetic resonance imaging is valuable not only in ruling out retinoblastoma. When Coats’ reaction is bilateral or occurs in a girl, it is also valuable in excluding leukoencephalopathy with intracranial calcifications and cysts, which in addition to an exudative retinopathy characterize two autosomal recessive multisystem disorders, cerebroretinal microangiopathy with calcifications and cysts [5], also known as Coats plus [6], and Revesz syndrome [7], which apparently result from impaired telomere maintenance [8,9] and carry significant morbidity and mortality from gastrointestinal bleeding and aplastic anemia, respectively.…”