Clinical-histopathological correlation in a case of Coats' disease

Abstract

Background: Coats' disease is a non-hereditary ocular disease, with no systemic manifestation, first described by Coats in 1908. It occurs more commonly in children and has a clear male predominance. Most patients present clinically with unilateral decreased vision, strabismus or leukocoria. The most important differential diagnosis is unilateral retinoblastoma, which occurs in the same age group and has some overlapping clinical manifestations.

Coats‐like retinopathy in a Young Indian Rhesus Macaque (Macaca mulatta)

“…These exudates induce retinal degeneration and infiltration of phagocytic, lipid‐laden ‘ghost’ cells . Our immunohistochemistry of CD68 revealed that these inflammatory cells within the retina and subretinal space were macrophages, which was consistent with the results in human Coats disease .…”

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

“…The first consists of a breakdown of the blood-retinal barrier at the endothelial level, which causes plasma leakage into the vessel wall and thickening of parts of the vessel wall, becoming necrotic and disorganized and producing what Egbert et al [12 ]have described as a ‘sausage-like’ shape of the vessel. The second concerns the presence of abnormal pericytes and endothelial cells in retinal blood vessels, which subsequently degenerate, causing abnormal retinal vasculature and formation of aneurysms, as well as closure of vessels, leading to ischaemia [13]. Noticeably, Jones et al [1] describe the process as being similar to that of diabetic retinopathy.…”

“…Noticeably, Jones et al [1] describe the process as being similar to that of diabetic retinopathy. The loss of endothelial cells and pericytes from the capillaries and the dilated telangiectasic arterioles causes leakage of a lipid-rich exudate into the retina, which can lead to changes in the retina, including thickening, cyst formation or retinal detachment [13,14]. …”

Pearls and Pitfalls in Diagnosis and Management of Coats Disease

“…The second concerns the presence of abnormal pericytes and endothelial cells in retinal blood vessels that subsequently degenerate, causing abnormal retinal vasculature and formation of aneurysms, as well as closure of vessels resulting in ischemia. 43 In contrast to diabetic microaneurysms, these arteriolar aneurysms do not leak lipid or fluorescein. We hypothesize that Coats disease may be a defect in midcapillary angiogenesis, and that aneurysmal dilations are a secondary abortive feature.…”

“…George Coats described macular fibrosis as "located in the choroid," and multiple histologic investigations have demonstrated this in the early 1900s. 43,45 In fact, early histologic investigations referred to "retinochoroidal synechiae." This point has been lost, however, likely because of the more recent recognition of choroidal neovascularization as an ocular pathologic process and the paucity of enucleations performed during current clinical management of Coats disease.…”