New optical coherence tomography fundus findings in a case of beta-thalassemia

MI Eleftheriadou,¹ PG Theodossiadis,¹ A Rouvas,¹ D Alonistiotis,¹ GP Theodossiadis<sup>2

1</sup>2nd Department of Ophthalmology,”Attikon” University Hospital, University of Athens, ²Department of Ophthalmology, “Henry Dunant” Hospital, Athens, Greece

Abstract: Patients with beta-thalassemia may present with an acquired diffuse elastic tissue defect due to degeneration of elastic tissue along with vaso-occlusive findings in the retinal microvasculature. Here we report the case of a patient with granular-like accumulation presenting as black sunburst lesions detected by optical coherence tomography (OCT). A 38-year-old man with beta-thalassemia intermedia associated with angioid streaks complained of deterioration of vision in both eyes. Funduscopic examination revealed small, round, hyperpigmented lesions bilaterally. During the early and late phases of fluorescein angiography, granular hyperfluoresence was present, associated with pigment decompensation and mottled-like hypofluorescence. The main OCT finding was the presence of granuloid-like accumulations at the retinal pigment epithelium level. Granule penetration was also noticed at the photoreceptor layer, while isolated granuloid-like accumulations were found in the inner layers of the macula and choroid. In this case, the new OCT finding was the granular-like hyperpigmented accumulations in the macula located at the level of the retinal pigment epithelium. To the best of our knowledge, our OCT findings show for the first time granuloid-like accumulations representing black sunburst lesions.

Keywords: beta-thalassemia, angioid streaks, optical coherence tomography, granuloid-like accumulation, retinal pigment epithelium