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MULTIMODAL IMAGING IN DEFEROXAMINE RETINOPATHY

Viola, Francesco MD; Barteselli, Giulio MD; Dell'Arti, Laura MD; Vezzola, Diego MD; Mapelli, Chiara MD; Villani, Edoardo MD; Ratiglia, Roberto MD

doi: 10.1097/IAE.0000000000000073
Original Study
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Purpose: To describe macular lesions in patients with deferoxamine (DFO) retinopathy, and to follow their clinical course using multimodal imaging.

Methods: The authors retrospectively reviewed charts and multimodal imaging of 20 patients with β-thalassemia diagnosed with DFO retinopathy (40 eyes) after a minimum of 10 years of DFO treatment. Imaging included fundus photography, near-infrared reflectance and fundus autofluorescence imaging on confocal laser scanning ophthalmoscope, and spectral domain optical coherence tomography.

Results: Mean age of the 20 patients was 45 years, and mean duration of subcutaneous DFO therapy was 32 years (range, 20–52 years). Ten patients (50%) showed different types of pattern dystrophy–like fundus changes, including butterfly shaped–like (n = 3), fundus flavimaculatus–like (n = 3), fundus pulverulentus–like (n = 3), and vitelliform-like (n = 1) changes. Ten patients (50%) presented only minimal changes in the macula; these patients were significantly younger than patients presenting other patterns (P = 0.023). Confocal laser scanning ophthalmoscope and spectral domain optical coherence tomography showed that these abnormalities were more diverse and widespread than expected by ophthalmoscopy. Abnormal fundus autofluorescence and/or near-infrared reflectance signals corresponded to accumulation of material located within the outer retina or in the Bruch membrane–retinal pigment epithelium (RPE) complex on spectral domain optical coherence tomography. Follow-up examinations during a 40-month period revealed progressive development of RPE atrophy in areas of pattern dystrophy–like changes.

Conclusion: DFO retinopathy included a variety of pattern dystrophy–like changes or minimal changes affecting the RPE–Bruch membrane–photoreceptor complex. Multimodal imaging demonstrated that fundus changes were more diverse and widespread than expected from ophthalmoscopy. Consistently with previous histologic description of DFO retinopathy, multimodal imaging confirmed that photoreceptor outer-derived retinoids, various fluorophores, and RPE displacement or clumping are involved in DFO retinopathy, finally leading to frank RPE atrophy in most cases of pattern dystrophy–like changes.

This retrospective review of charts and multimodal imaging of 20 patients with β-thalassemia diagnosed with deferoxamine retinopathy demonstrated a variety of pattern dystrophy–like changes or minimal changes affecting the RPE–Bruch membrane–photoreceptor complex. Photoreceptor outer-derived retinoids, various fluorophores, and RPE displacement or clumping are involved in deferoxamine retinopathy, finally leading to RPE atrophy in cases of pattern dystrophy–like changes.

Department of Clinical Sciences and Community Health, University of Milan, Ophthalmological Unit, Cà Granda Foundation-Ospedale Maggiore Policlinico, Milan, Italy.

Reprint requests: Francesco Viola, MD, Department of Clinical Sciences and Community Health, University of Milan, Ophthalmological Unit, Cà Granda Foundation-Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy; e-mail: francesco.viola@unimi.it

Dr. F. Viola and Dr. G. Barteselli contributed equally as first authors.

Supported by “Dote ricerca” FSE Regione Lombardia.

None of the authors have any conflicting interests to disclose.

© 2014 by Ophthalmic Communications Society, Inc.