Microstructural assessment of rare corneal dystrophies using real-time in vivo confocal microscopy


  • Presented in part as a scientific poster at the Royal Australian College of Ophthalmologists 32nd Annual Scientific Congress, Sydney, 19–23 November 2000.

Correspondence: Prof CharlesNj McGhee, Discipline of Ophthalmology, The University of Auckland, Private Bag 92019, Auckland 1, New Zealand. Email: c.mcghee@auckland.ac.nz


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 μm in diameter in the mid and posterior stroma as well as smaller hyperreflective dot-like intracellular deposits, of less than 1 μm 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.