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L. N. Santos, B. F. Fernandes, L. R. M. Santos, D. P. Cheema, S. Maloney, M. N. Burnier, Jr.; The Histopathological Diagnosis of Corneal Stromal Dystrophies: A 10-Year Single Center Analysis. Invest. Ophthalmol. Vis. Sci. 2007;48(13):5855.
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Corneal dystrophies are inherited disorders characterized by bilateral, symmetrical or asymmetrical opacities. The corneal opacities are caused by progressive accumulation of deposits affecting the transparency or the refractive power of the cornea. They are primary corneal lesions and are not associated with prior inflammation or trauma. Onset is usually around puberty, progressing slowly throughout the lifetime of the patient, and may or may not affect vision. The aim of this study was to determine the frequency and describe the histopathological features of stromal corneal dystrophies over a 10-year period.
A single-center, retrospective analysis was performed on twenty-six corneal specimens previously diagnosed as stromal dystrophies retrieved from an archive of 885 corneal specimens from The Henry C. Witelson Ophthalmic Pathology Laboratory and Registry (Montreal, Canada) over a period of 10 years. Corneal specimens obtained during keratoplasty were stained with H&E, PAS, Masson trichrome (granular dystrophy), Alcian blue (macular dystrophy) and Congo red (lattice dystrophy) for light microscopic examination. All cases of combined "Avellino" dystrophy (ACD) were stained with Masson trichrome and Congo red.
During the period of the study out of the 885 corneal specimens from penetrating keratoplasties, 26 (2,9%) specimens from a total of 22 patients were diagnosed as corneal stromal dystrophies. The distribution of these dystrophic stromal diseases was: 17 (65.4%) eyes with lattice dystrophy, 5 (19.2%) eyes with ACD, 3 (11.5%) eyes with granular dystrophy and 1 (3.9%) eye with macular dystrophy.
Corneal stromal dystrophies have distinct histopathological features, which are highlighted using special stains. Although lattice dystrophy was the most frequent stromal dystrophy in this population, the importance of the histo-pathological diagnosis of "Avellino" should be emphasized. Clinically this dystrophy may present as lattice or granular type making the histo-pathological study crucial for confirming the final diagnosis.
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