Abstract
A patient with Sturge-Weber syndrome presented with over 1.5 years of blurry vision of the right eye. On exam, they were found to have a visual acuity of 20/40 in the right eye and 20/20 in the left. External exam was notable for a large cutaneous capillary malformation involving the majority of the right face and engorged conjunctival/episcleral vessels of the right eye. Fundus exam of the right eye revealed a diffusely thickened, reddish fundus. Autofluorescence and OCT imaging demonstrated subretinal fluid in the macula, and B-scan ultrasonography revealed a diffuse choroidal tumor with uniformly high internal reflectivity. A diagnosis of diffuse choroidal hemangioma was made. At 2-month follow-up, the subretinal fluid was increased and visual acuity was reduced to 20/400. In order to treat the subretinal fluid, photodynamic therapy was performed, but the subretinal fluid further worsened despite this. Therefore, photodynamic therapy was repeated, this time together with intravitreal bevacizumab. 2 months later, the subretinal fluid was significantly decreased, and it nearly resolved at subsequent visits with administration of intravitreal metoprolol every 1-2 months. However, the tumor eventually became resistant to these treatments, and the subretinal fluid recurred. Therefore, I-125 plaque brachytherapy with a dose of 35 Gy to the apex of the thickest portion of the tumor was performed. The subretinal fluid was fully resolved 3 months later, and visual acuity improved to 20/30-2. The patient has remained stable since, with no recurrence of subretinal fluid in over 2 years and a final visual acuity of 20/25-1.
Presentation Date: 04/09/2026
Issue Date: 04/10/2026
