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Invited Commentary
April 2018

Association of Retinal Macrovessels With Venous Malformations of the Brain

Author Affiliations
  • 1Department of Ophthalmology, Northwestern University, Feinberg School of Medicine, Chicago, Illinois
JAMA Ophthalmol. 2018;136(4):380-381. doi:10.1001/jamaophthalmol.2018.0149

The retina is functionally and embryologically part of the brain. As a result, tumors, vascular anomalies, metabolic disorders, and other diseases will manifest in both sites. To our knowledge, the first series of patients with congenital retinal macrovessels was reported by Brown et al1 and included 7 patients with abnormal vessels crossing the macula. Congenital retinal macrovessel is a rare entity, with an estimated prevalence of 1 in 200 000 in the United States, but this figure is likely an underestimate because most patients are asymptomatic and are discovered on routine examination or when the patient presents with an unrelated condition. To our knowledge, only a single case has been previously reported in which congenital retinal macrovessels were associated with an intracranial venous malformation.2