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Miriam Habiel, Gerald W Zaidman, Kenneth Juechter; Corneal Histopathological Findings In Lecithin-Cholesterol Acyltransferase (LCAT) Deficiency. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):1942.
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<br /> Previous cases have described the histopathological findings of corneas of patients with LCAT deficiency. They have demonstrated mucopolysaccharide-filled vacuoles deposited extracellularly in Bowman’s layer, corneal stroma and predescemet's membrane. In this case-report, we will describe new light and electron microscopy findings on a penetrating keratopasty specimen from a patient with LCAT deficiency.
<br /> A penetrating keratoplasty was performed on a 38 year old male with LCAT deficiency. The sample was examined under light and electron microscopy.
<br /> A few vacuoles containing granular deposits were seen in the epithelium and Bowman’s layer on light microscopy and on Toluidine blue stain. They were most numerous in the anterior stroma but extended into the posterior stroma. They also were located intracellularly within keratocytes and extracellularly. The vacuoles were found to disrupt the stromal lamellar collagen bundles. Vacuoles contacting fine granular material, some membrane bound, were seen to extend down to descement's membrane and to the corneal endothelium on electron microscopy.
<br /> To our knowledge, this is the first description in the literature of a patient with LCAT deficiency with granular material intracellularly within keratocytes and within vacuoles extending down to and involving corneal endothelium.
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