2020–2021 BCSC Basic and Clinical Science Course™
6 Pediatric Ophthalmology and Strabismus
Part II: Pediatric Ophthalmology
Chapter 28: Ocular Manifestations of Systemic Disease
Wyburn-Mason syndrome (racemose angioma) is a nonhereditary arteriovenous malformation of the eye and brain. Extraocular features are summarized in Table 28-9.
Ocular manifestations are unilateral and congenital, and they may progress during childhood. The typical lesion consists of markedly dilated and tortuous vessels that shunt blood directly from arteries to veins (Fig 28-23). These vessels do not leak fluid. Vision ranges from normal to markedly reduced. Intraocular hemorrhage and secondary neovascular glaucoma are possible complications. More than half of affected eyes are blind, and an additional one-quarter have severe visual impairment.
Table 28-9 Extraocular Features in Wyburn-Mason Syndrome
Figure 28-23 Wyburn-Mason syndrome, or racemose angioma of the retina, left eye.
No treatment is indicated for primary lesions. Treatment may be considered for associated complications, such as scatter photocoagulation for ischemic venous occlusive disease, vitrectomy for nonclearing vitreous hemorrhage, and cyclodestructive treatment for neovascular glaucoma.
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.