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

Diffuse choroidal hemangioma is a congenital vascular hamartoma often assoc iated with hemangiomatous lesions of the brain, orbit, and periocular skin (nevus flammeus) in the Sturge-Weber syndrome. Visual loss from diffuse cho roidal hemangioma may result from chronic serous retinal detachments causin g retinal pigment epithelial, photoreceptor or cystoid degeneration, and gl aucomatous optic atrophy. Low-dose external beam irradiation has successful ly resolved exudative retinal detachment and caused shrinkage of the choroi dal hemangioma.(1-3) Visual loss in Sturge-Weber syndrome with diffuse chor oidal hemangioma often begins during amblyogenic years. Visual rehabilitati on may thus require not only therapeutic intervention to address organic di sease but also amblyopia therapy. Though many of the previously reported ca ses address treatment of the diffuse choroidal hemangioma with laser or rad iotherapy, none advocate or emphasize treatment of nonorganic amblyopia ass ociated with this condition. We report the case of a child with Sturge-Webe r syndrome and unilateral diffuse submacular choroidal hemangioma who devel oped an exudative retinal detachment that responded to the combination of l ow-dose external beam irradiation and aggressive amblyopia therapy.