title: Microstructural assessment of rare corneal dystrophies using real-time in vivo confocal microscopy.
abstract: OBJECTIVE
  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.affiliation: Discipline of Ophthalmology, Faculty of Medical and Health Sciences, University of Auckland, New Zealand.
authors.forename: C N
authors.initials: CN
authors.lastname: Grupcheva
authors.forename: T Y
authors.initials: TY
authors.lastname: Malik
authors.forename: J P
authors.initials: JP
authors.lastname: Craig
authors.forename: T
authors.initials: T
authors.lastname: Sherwin
authors.forename: C N
authors.initials: CN
authors.lastname: McGhee
mesh_terms: D000328:Adult; D002648:Child; D003317:Corneal Dystrophies, Hereditary / Q000175:diagnosis* / Q000503:physiopathology; D003319:Corneal Stroma / Q000473:pathology* / Q000503:physiopathology; D003886:Descemet Membrane / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D018613:Microscopy, Confocal / Q000379:methods*