Pattern Dystrophy of the Macula in a Case of Steinert Disease
A 41-year-old Hispanic female diagnosed with type 1 myotonic dystrophy (Steinert disease) was referred for ophthalmologic examination with the chief complaint of progressive vision loss. Her best-corrected visual acuity was 20/32 in her right eye and 20/40 in her left eye. Ocular motility was normal. She had bilateral ptosis with abnormal elevator function. Slit lamp examination revealed the presence a bilateral polychromatic cataract with posterior subcapsular component.\nDilated fundus examination was remarkable for macular pigmentary changes with multifocal yellowish depigmented patches within the macular area in both eyes (fig. ). Fundus autofluorescence highlighted a branching linear pattern of mixed hyper- and hypoautofluorescence limited to the macular area in both eyes (fig. ).\nOptical coherence tomography images showed diffuse subretinal hyperreflective material between the photoreceptor layer and the RPE in both eyes. Larger focal vitelliform lesions were also evidenced. No subretinal or intraretinal fluid was found in association with the deposits (fig. ).