PURPOSE: To analyse and describe three cases of rare corneal dystrophy and highlight their in vivo microstructural features. METHODS: Subject 1 was diagnosed with a posterior stromal fleck corneal dystrophy Two of her three children were also affected. Subjects 2 and 3 exhibited an almost identical clinical appearance on biomicroscopic examination, such that both clinically were diagnosed as having pre-Descemet's dystrophies. All subjects underwent in vivo confocal microscopy and approximately 300 sequential digital images were obtained and analysed for each cornea. RESULTS: In vivo confocal microscopy of subject 1 demonstrated an abnormal appearance of numerous large ovoid particles, measuring 50-70 microm in diameter in the mid and posterior stroma as well as smaller hyperreflective dot-like intracellular deposits, of less than 1 microm diameter. Despite the near-identical clinical appearance, subjects 2 and 3 could be clearly differentiated by in vivo confocal microscopy. Subject 2 exhibited small, irregular, optically dense particles, mainly in the anterior stroma, whereas subject 3 possessed classical involvement of the stroma immediately adjacent to Descemet's membrane, with numerous regular small, hyperreflective particles. CONCLUSIONS: The ability of in vivo confocal microscopy to localize and accurately measure various elements in different corneal layers may help to resolve whether abnormalities are intra- or extracellular, and aid clearer differentiation of rare corneal disorders.
PURPOSE: To analyse and describe three cases of rare corneal dystrophy and highlight their in vivo microstructural features. METHODS: Subject 1 was diagnosed with a posterior stromal fleck corneal dystrophy Two of her three children were also affected. Subjects 2 and 3 exhibited an almost identical clinical appearance on biomicroscopic examination, such that both clinically were diagnosed as having pre-Descemet's dystrophies. All subjects underwent in vivo confocal microscopy and approximately 300 sequential digital images were obtained and analysed for each cornea. RESULTS: In vivo confocal microscopy of subject 1 demonstrated an abnormal appearance of numerous large ovoid particles, measuring 50-70 microm in diameter in the mid and posterior stroma as well as smaller hyperreflective dot-like intracellular deposits, of less than 1 microm diameter. Despite the near-identical clinical appearance, subjects 2 and 3 could be clearly differentiated by in vivo confocal microscopy. Subject 2 exhibited small, irregular, optically dense particles, mainly in the anterior stroma, whereas subject 3 possessed classical involvement of the stroma immediately adjacent to Descemet's membrane, with numerous regular small, hyperreflective particles. CONCLUSIONS: The ability of in vivo confocal microscopy to localize and accurately measure various elements in different corneal layers may help to resolve whether abnormalities are intra- or extracellular, and aid clearer differentiation of rare corneal disorders.
Authors: María Angélica Henríquez-Recine; Kelly Sonia Marquina-Lima; Elena Vallespín-García; Sixto García-Miñaur; José Manuel Benitez Del Castillo; Ana Boto de Los Bueis Journal: Graefes Arch Clin Exp Ophthalmol Date: 2018-05-04 Impact factor: 3.117
Authors: Andrea L Vincent; David M Markie; Betina De Karolyi; Catherine E Wheeldon; Dipika V Patel; Christina N Grupcheva; Charles N J McGhee Journal: Mol Vis Date: 2009-08-26 Impact factor: 2.367