Isolated Racemose Angioma—A Rare Retinal Condition

The patient is a 42-year-old man who was noted to have tangles of dilated, intertwined, and tortuous retinal vessels of his right eye on fundus examination. On examination, Snellen visual acuity was 20/20 OU, and intraocular pressure was within normal limits. Fluorescein angiography (Figure) demonstrated retinal arteriovenous malformation with no evidence of leakage of dye from the vessels. A diagnosis of racemose angioma was made. Magnetic resonance imaging of the brain and orbits did not demonstrate any underlying arteriovenous malformations. Multiple follow-up evaluations have revealed a stable course, and the patient continues to remain otherwise asymptomatic. Congenital retinal arteriovenous malformations (racemose angioma) are associated with ipsilateral intracranial or interosseous vascular malformations that can spontaneously hemorrhage, in a condition called Wyburn-Mason syndrome.¹ In the eye, the findings are usually stable and benign.²