Correlation of fundus autofluorescence, spectral-domain optical coherence tomography, and microperimetry in late deferoxamine maculopathy.

Abstract

To describe long-term functional and morphologic retinal changes by deferoxamine, a chelating agent used to treat systemic iron overload. Ophthalmologic examination of acute deferoxamine maculopathy and follow-up of 5 years, using ophthalmologic examination, fluorescence angiography, electrophysiology, color vision testing, fundus image-correlated microperimetry, time-domain and spectral-domain optical coherence tomographies. The patient is a 53-year old woman who presented with acute visual deterioration to 20/50 in the right eye, 20/60 in the left eye, and reduced color vision. She had been treated with deferoxamine for 6 months. The diagnosis of deferoxamine maculopathy was made, and the drug discontinued immediately. After 5 months, visual acuity was 20/25 bilaterally, but mottling of the retinal pigment epithelium (RPE) had developed. Five years later, visual acuity was 20/20 in both eyes. Fundus autofluorescence showed progressively clumped hyper- and hypofluorescence with larger areas of total RPE atrophy in the left eye. Spectral-domain optical coherence tomography visualized areas of RPE thickening, photoreceptor outer segment elongation, outer retinal tubulation, and outer retinal and RPE atrophy. Fundus image-correlated microperimetry showed relative central scotomas at the beginning, some of which recovered to normal light sensitivity, while others deteriorated to absolute scotomas. Although visual acuity had recovered after cessation of deferoxamine, functional and morphologic residues remained, which included disturbed color vision, central microscotomas, and progressive RPE and outer retinal degeneration.