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Related Concept Videos

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Epithelial phenotype in total sclerocornea.

David Hui-Kang Ma1, Lung-Kung Yeh2, Hung-Chi Chen2

  • 1Limbal Stem Cell Laboratory, Department of Ophthalmology, Chang Gung Memorial Hospital, Taoyuan, Taiwan ; Department of Chinese Medicine, College of Medicine, Chang Gung University, Taoyuan, Taiwan.

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Summary

The epithelial cells in total sclerocornea are primarily corneal in origin. However, conjunctival epithelium invasion can occur with significant corneal neovascularization.

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Area of Science:

  • Ophthalmology
  • Corneal Diseases
  • Histopathology

Background:

  • Total sclerocornea is a rare congenital condition characterized by opacification of the cornea.
  • Understanding the cellular origin of the corneal epithelium in sclerocornea is crucial for diagnosis and treatment.

Purpose of the Study:

  • To determine if the epithelial phenotype in total sclerocornea originates from corneal or conjunctival tissue.

Main Methods:

  • Analysis of corneal buttons from four patients with total sclerocornea who underwent penetrating keratoplasty.
  • Utilized transmission electron microscopy (TEM) and immunoconfocal microscopy.
  • Examined expression of cytokeratins (3, 12, 13), stem cell markers (p63, ABCG2), and extracellular matrix proteins (laminin-5, fibronectin).

Main Results:

  • TEM revealed attenuated Bowman's layer and disorganized collagen fibrils in sclerocorneas.
  • Immunoconfocal microscopy showed expression of cornea-specific keratin 3 and keratin 12 in most patients.
  • Absence of stem cell markers (p63, ABCG2) and conjunctival marker MUC5AC was noted, except for a unique keratin 12/13 expression pattern in one patient.

Conclusions:

  • The corneal epithelium in total sclerocornea predominantly exhibits a corneal phenotype.
  • Conjunctival epithelial invasion is a possibility in cases with extensive corneal angiogenesis.