Optical coherence tomography of peripheral pigmented lesions in Stargardt’s disease

A 14-year-old girl with ABCA4-positive Stargardt’s disease was reviewed for ocular examination. Fundus examination revealed a beaten-bronze appearance of the macula, yellowish-white flecks, and a well-defined, pigmented lesion in the temporal periphery [Fig. 1a]. Fundus autofluorescence findings were typical of Stargardt’s disease, and the peripheral lesions were hypoautofluorescent [Fig. 1b]. Ultra-wide field imaging (Optos Silverstone) was used to obtain optical coherence tomography (OCT) sections through this peripheral pigmented lesion [Fig. 1c and d], which revealed it to be irregular thickening of the retinal pigment epithelium.Figure 1: (a) Ultra-wide field fundus photo showing beaten-bronze appearance of the macula with an irregular, well-defined pigmented lesion in the superotemporal periphery. (b) Fundus autofluorescence showing hypofluorescence of the lesion. (c and d) Optos OCT section through the peripheral lesion showing irregular thickening of the retinal pigment epithelium. OCT = optical coherence tomographyPeripheral pigmented lesions resembling congenital hypertrophy of the retinal pigment epithelium have been previously described in Stargardt’s disease,[1] but have not been characterized via OCT. The exact pathogenesis of these lesions is yet to be elucidated. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.