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

PURPOSE: To describe the structural characteristics of retinal angiomatous proliferation and chorioretinal anastomosis in childhood Coats disease and redefine the previously described macular fibrosis.

METHODS: 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.

RESULTS: 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.

CONCLUSION: 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."