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

ABSTRACT

PURPOSE: To describe long-term functional and morphologic retinal changes by deferoxamine, a chelating agent used to treat systemic iron overload. METHODS: 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. RESULTS: 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. CONCLUSION: 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.