Ultra Widefield Swept Source OCTA Findings in Coats Plus Syndrome

Coats plus syndrome (CPS), also referred to as cerebroretinal microangiopathy with calcifications and cysts (CMCC), is a rare autosomal recessive disease that primarily targets the microvasculature of the retina, brain, bones, and gastrointestinal system. This study reports the case of a 24-year-old female patient who was initially diagnosed with familial exudative vitreoretinopathy (FEVR) and was lost to follow-up. The patient underwent multimodal retinal imaging, including ultra-widefield swept-source optical coherence tomography angiography (UWF SS-OCTA) (DREAM OCT, Intalight). Examination revealed areas of peripheral avascular retina with limited exudation and telangiectasis. Ultra-widefield SS-OCTA provided a detailed view of the peripheral retinal changes, including temporal retinal ischemia, vessel tortuosity, dilated intercapillary spaces, and vessel shunting. Genetic testing was positive for CTC1 mutation and the diagnosis of CPS was made. Mild phenotypes of Coats plus can mimic FEVR and there is a need to maintain a level of suspicion in patients with any systemic symptoms. Ultra-widefield OCTA can be used to assess peripheral avascularity and telangiectasias to aid in the diagnosis and management. [ Ophthalmic Surg Lasers Imaging Retina 2025;56:XX–XX.]