In Wyburn-Mason syndrome, congenital retinal arteriovenous malformations occur in conjunction with similar ipsilateral vascular malformations in the brain, face, orbit, and mandible. The lesions are composed of blood vessels without an intervening capillary bed (racemose hemangioma). The abnormalities may range from a single arteriovenous communication to a complex anastomotic system. In the eye, the lesions are usually asymptomatic, unilateral, and located in the retina and optic nerve. Typically, they do not show leakage on fluorescein angiography. Intraosseous vascular malformations that may occur in the maxilla and mandible can lead to unexpected hemorrhaging during dental extractions. Most commonly, racemose hemangiomas in the retina are isolated and are not part of the full syndrome.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.