Stargardt’s macular dystrophy: clinical case

Abstract

Stargardt's Macular Dystrophy was described in 1909 by Karl Stargardt, a German ophthalmologist. It is the most common autosomal recessive macular dystrophy, it’s prevalence 1:10000. It is characterized by central visual loss, atrophy of the retinal pigment epithelium. In this clinical case, we are presenting 13-year-old boy complaining of reduced visual acuity in both eyes for several months. His best-corrected visual acuity in the right eye was 0.1, and 0.1 in the left eye. Ophthalmoscopy of the both eyes revealed the onset of disease, and showed a mild scattering of pigment and loss of macular reflex. Colour vision test showed the damage of red and green colours vision. Severe patern ERG abnormality with normal photopic and scotopic a- and b-waves, during Ganzfeld ERG and loss of photopic a- and b-waves were found. Fluorescein angiography revealed a dark/masked choroid. As Stargardt’s macular dystrophy is the most common autosomal recessive heriditary macular dystrophy, but it takes very long time till true diagnosis, that‘s why we are presenting a case report with a mild changes of the eyes fundus, which will be helpful for early recognizing of Stargardt’s macular dystrophy.