Visual rehabilitation in a child with diffuse choroidal hemangioma by using aggressive amblyopia therapy with low-dose external beam irradiation

Abstract Diffuse choroidal hemangioma is a congenital vascular hamartoma often associated with hemangiomatous lesions of the brain, orbit, and periocular skin (nevus flammeus) in the Sturge-Weber syndrome. Visual loss from diffuse choroidal hemangioma may result from chronic serous retinal detachments causing retinal pigment epithelial, photoreceptor or cystoid degeneration, and glaucomatous optic atrophy. Low-dose external beam irradiation has successfully resolved exudative retinal detachment and caused shrinkage of the choroidal hemangioma. 1-3 Visual loss in Sturge-Weber syndrome with diffuse choroidal hemangioma often begins during amblyogenic years. Visual rehabilitation may thus require not only therapeutic intervention to address organic disease but also amblyopia therapy. Though many of the previously reported cases address treatment of the diffuse choroidal hemangioma with laser or radiotherapy, none advocate or emphasize treatment of nonorganic amblyopia associated with this condition. We report the case of a child with Sturge-Weber syndrome and unilateral diffuse submacular choroidal hemangioma who developed an exudative retinal detachment that responded to the combination of low-dose external beam irradiation and aggressive amblyopia therapy. JAAPOS 2000;4:321-2.