Vascular Tumors and Malformations of the Choroid

Choroidal vascular tumors; it may present as circumscribed and diffuse choroidal hemangiomas and rarely choroidal hemangiopericytoma and phacomatosis pigmentovascularis. Choroidal hemangiomas are benign vascular hamartomatous tumors that occur in two forms: circumscribed and diffuse. The circumscribed form is typically an isolated finding without systemic associations, while the diffuse form generally occurs in association with Sturge–Weber syndrome. Circumscribed choroidal hemangiomas are generally seen as well-circumscribed, round or oval, elevating dome-shaped orange or red masses located posterior to the equator in the fundus, whereas diffuse choroidal hemangiomas have a diffuse red-orange color appearance (tomatoketchup). Although the diagnosis of choroidal hemangioma is better investigated by means of multimodal imaging, indocyanine green angiography and ultrasonography are the most important tests in the differential diagnosis. For circumscribed choroidal hemangiomas with exudative retinal detachment or cystoid macular edema, a variety of treatment modalities are available. Symptomatic patients can be treated with oral propranolol, photodynamic therapy, transpupillary thermotherapy, plaque radiotherapy, external radiotherapy or intravitreal anti-vascular endothelial growth factors.In this review, the pathogenesis, clinical features and treatment methods in choroidal vascular tumors will be discussed.