The Retina Reference

Sturge-Weber Syndrome with Thick Choroid

This is a multimedia montage of photographs taken from an 11-year-old male patient with Sturge- Weber syndrome. Notice the difference in color of the fundus between right and left eyes. The right fundus is more vermillion due to a diffuse choroidal hemangioma ipsilateral to the port wine stain. In this case a secondary serous retinal detachment has developed as shown in the OCT (red arrows). Notice how thick the choroid is in the right eye on the OCT scans. The choroidal-scleral junction is well seen on the left (purple arrow) but is not visible on the right (orange arrow) because it is deeper than the region shown in the OCT frame. The serous retinal detachment has been present for some time as witnessed by the reactive subretinal pigment hyperplasia (turquoise arrow). The port wine stain on the boy’s right cheek has been treated recently with laser in an attempt to make the lesions less noticeable (green arrow). This patient has glaucoma in his right eye which has been treated with a filtering operation and for which he takes Cosopt, Alphagan, Latanoprost, and Trusopt. Secondary glaucoma is common in Sturge-Weber syndrome, and is often refractory to treatment.