Retinal angiomatous proliferation with chorioretinal anastomosis in childhood Coats disease: a reappraisal of macular fibrosis using multimodal imaging.

Abstract

To describe the structural characteristics of retinal angiomatous proliferation and chorioretinal anastomosis in childhood Coats disease and redefine the previously described macular fibrosis. Prospective observational case series of consecutive patients with Coats disease examined over a 1-year study period. Multimodal imaging, including color fundus photography, wide-field fluorescein angiography, and spectral domain optical coherence tomography, was used to identify the features of macular retinal angiomatous proliferation and chorioretinal anastomosis. Retinal angiomatous proliferation and chorioretinal anastomosis were present in 5 of 21 patients with Coats disease (24%). The lesions appeared as well demarcated, nodular retinal pigment epithelial detachments surrounded by exudate, with retinal vessels continuous with the underlying choroidal neovascularization. Retinal angiomatous proliferation and chorioretinal anastomoses are features observed in a number of children (24% in the present series) with Coats disease and macular involvement. This lesion represents a distinct macular variant of Coats disease that underlies at least a proportion (all in the present series) of the previously described "macular fibrosis" and "subretinal mounds."