Stromal, descemet's and endothelial changes in macular corneal dystrophy type II

A. J. Quantock, N. J. Fullwood, E. J M A Thonar, S. R. Waltman, M. S. Cape, M. C. Kincaid, M. Ito, S. M. Verity, D. J. Schanzlin

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Abstract

Purpose. A multi-faceted investigation of an individual macular corneal dystrophy (MCD) type II cornea from a 42-year-old female. Methods. Quantification of antigenic keratan sulphate in serum (ELISA with 5-D-4 antibody), synchrotron X-ray diffraction, energy dispersive X-ray microanalysis, and electron microscopic histochemistry (cuprolinic blue) and immunohistochemistry (5-D-4). Results. The level of antigenic keratan sulphate in our patient's serum was well below normal (19ng/ml compared to 251±78ng/ml). A characteristic 4.6Å X-ray reflection was evident, and the mid-stroma contained 30% less sulphur than normal. Close-packing of collagen was restricted to the superficial stroma. Abnormally-large proteoglycan filaments were noted throughout the extracellular matrix and Descemet's membrane's posterior non-banded zone, but not its anterior banded zone. Small, collagen-associated stromal proteoglycans were susceptible to digestion with chondroitinase ABC, but not keratanase I or N-glycanase. On occasion, collagen fibrils ranged in size from 20nm to 58nm, with preferential diameters of 34nm and 42nm. The endothelium expressed reduced anti-keratan sulphate labelling. Numerous endothelial inclusions were most likely due to intracellular fibrillogranular vacuoles similar to those found in the stroma. Conclusion. Based on our immunochemical findings it seems that a heterogeneity may exist within the MCD type II subgroup. Moreover, our patient's histopathology reveals pockets of large collagen fibrils, and proteoglycan abnormalities that involve Descemet's membrane and the endothelial cells.

Original languageEnglish (US)
Pages (from-to)S1019
JournalInvestigative Ophthalmology and Visual Science
Volume37
Issue number3
StatePublished - Feb 15 1996

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems
  • Cellular and Molecular Neuroscience

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    Quantock, A. J., Fullwood, N. J., Thonar, E. J. M. A., Waltman, S. R., Cape, M. S., Kincaid, M. C., Ito, M., Verity, S. M., & Schanzlin, D. J. (1996). Stromal, descemet's and endothelial changes in macular corneal dystrophy type II. Investigative Ophthalmology and Visual Science, 37(3), S1019.