Butterfly-shaped pigment dystrophy of the fovea associated with subretinal neovascularization.

BACKGROUND: The association of butterfly-shaped pigment dystrophy of the fovea, an uncommon inherited macular disease, with subretinal neovascularization has rarely been reported in the literature. CASE REPORT: We describe the clinical history of a patient affected with butterfly-shaped pigment dystrophy of the fovea, myopia, and optic nerve head dysplasia. She was followed up for 23 years. During the course of the disease, bilateral subretinal neovascularization in the macular area occurred. Fluorescein angiography confirmed the diagnosis. Recently, indocyanine green (CG) videoangiography was also performed. Because of the bilateral subfoveal localization no laser treatment was advised. DISCUSSION: Usually, good visual acuity is maintained in this uncommon inherited macular disease. However, acute visual loss can be caused by the ingrowth of subretinal new vessels. Therefore, if visual acuity decreases or metamorphopsia develops in these patients, careful biomicroscopic examination and fluorescein/ICG angiography is advisable.