Letter to the editorCerebroretinal microangiopathy with calcifications and cysts-1 (CRMCC1), also known as Coats plus syndrome, is an autosomal recessive, multisystem disorder characterized by obliterative angiopathy of small vessels, primarily in the brain, eyes, bone, and gastrointestinal tract. The clinical features of this condition include prenatal and postnatal growth restriction, bilateral retinal telangiectasias and exudates, intracranial calcification, leukoencephalopathy sometimes associated with parenchymal cysts, osteopenia with a tendency for fracture, bone marrow suppression, and gastrointestinal bleeding with cirrhosis. Less frequently, patients demonstrate sparse, gray hair, dystrophic nails, and café au lait patches [1]. It has no known effective treatment to date. Bevacizumab, a vascular endothelial growth factor (VEGF) inhibitor, has been successfully used in the treatment of two disorders that share similar pathologies with a more limited distribution: Coats disease and leukoencephalopathy with calcifications and cysts (LCC), also known as Labrune syndrome [2,3]. We report the case of a patient who was diagnosed with Coats plus syndrome and demonstrated almost complete free-1 www.annchildneurol.org
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