In Vivo Confocal Microscopy in Patients With Central Cloudy Dystrophyof François

Abstract

To report in vivo corneal confocal microscopic findings of patients with central cloudy dystrophy of François.Methods: Two unrelated patients, a 78-year-old man and a 75-year-old woman, with central cloudy dystrophy of François were examined using routine slitlamp biomicroscopy and confocal microscopy.

In Vivo Confocal Microscopy in Presumed Central Cloudy Dystrophy of François

“…In our case, unlike the previous report,3 the endothelium revealed prominent polymegathism and pleomorphism, bilaterally. No endothelial accumulations or guttae were seen.…”

“…Similarly in CCDF, when tension in Descemet's membrane is released, collagen lamellae inserting obliquely might create a reproducible polygonal ridge pattern. 3 In our case, unlike the previous report, 3 the endothelium revealed prominent polymegathism and pleomorphism, bilaterally. No endothelial accumulations or guttae were seen.…”

“…In our case, however, hyperreflective deposits extended towards posterior stroma, where they merged with multiple dark striae with increased intensities of extracellular matrices. Additionally, there were no stromal layers, where keratocytes could be observed; and this was found to be the most important difference when compared with the previous observations 3. It might be postulated that in more advanced stages of the dystropy, a large number of extracellular vacuoles filled with mucopolysaccharide and lipid-like material are present in full-thickness corneal stroma.…”

“…In this report we present the in vivo confocal microscopic findings in a patient with phenotypic CCDF, but without any history suggesting autosomal dominant inheritance. To the best of our knowledge, this is the second report of the in vivo confocal microscopy findings in presumed CCDF 3…”

In Vivo Confocal Microscopy in Presumed Central Cloudy Dystrophy of François

“…In our case, unlike the previous report,3 the endothelium revealed prominent polymegathism and pleomorphism, bilaterally. No endothelial accumulations or guttae were seen.…”

“…Similarly in CCDF, when tension in Descemet's membrane is released, collagen lamellae inserting obliquely might create a reproducible polygonal ridge pattern. 3 In our case, unlike the previous report, 3 the endothelium revealed prominent polymegathism and pleomorphism, bilaterally. No endothelial accumulations or guttae were seen.…”

“…In our case, however, hyperreflective deposits extended towards posterior stroma, where they merged with multiple dark striae with increased intensities of extracellular matrices. Additionally, there were no stromal layers, where keratocytes could be observed; and this was found to be the most important difference when compared with the previous observations 3. It might be postulated that in more advanced stages of the dystropy, a large number of extracellular vacuoles filled with mucopolysaccharide and lipid-like material are present in full-thickness corneal stroma.…”

“…In this report we present the in vivo confocal microscopic findings in a patient with phenotypic CCDF, but without any history suggesting autosomal dominant inheritance. To the best of our knowledge, this is the second report of the in vivo confocal microscopy findings in presumed CCDF 3…”

Terrien's Marginal Degeneration Accompanied by Latticed Stromal Opacities

“…Although the polygonal stromal opacities in the present case were somewhat similar to posterior crocodile shagreen, the corneal opacities of our case were located in the anterior but not in the posterior corneal stroma. 17,18 Thus, we could distinguish our case of branching stromal lattice figure keratopathy from posterior crocodile shagreen.…”

In Vivo Confocal Microscopy in Presumed Central Cloudy Dystrophy of François

“…In our case, unlike the previous report,3 the endothelium revealed prominent polymegathism and pleomorphism, bilaterally. No endothelial accumulations or guttae were seen.…”

“…Similarly in CCDF, when tension in Descemet's membrane is released, collagen lamellae inserting obliquely might create a reproducible polygonal ridge pattern. 3 In our case, unlike the previous report, 3 the endothelium revealed prominent polymegathism and pleomorphism, bilaterally. No endothelial accumulations or guttae were seen.…”

“…In our case, however, hyperreflective deposits extended towards posterior stroma, where they merged with multiple dark striae with increased intensities of extracellular matrices. Additionally, there were no stromal layers, where keratocytes could be observed; and this was found to be the most important difference when compared with the previous observations 3. It might be postulated that in more advanced stages of the dystropy, a large number of extracellular vacuoles filled with mucopolysaccharide and lipid-like material are present in full-thickness corneal stroma.…”

“…In this report we present the in vivo confocal microscopic findings in a patient with phenotypic CCDF, but without any history suggesting autosomal dominant inheritance. To the best of our knowledge, this is the second report of the in vivo confocal microscopy findings in presumed CCDF 3…”

Terrien's Marginal Degeneration Accompanied by Latticed Stromal Opacities

“…Although the polygonal stromal opacities in the present case were somewhat similar to posterior crocodile shagreen, the corneal opacities of our case were located in the anterior but not in the posterior corneal stroma. 17,18 Thus, we could distinguish our case of branching stromal lattice figure keratopathy from posterior crocodile shagreen.…”

In Vivo Confocal Microscopy in Presumed Central Cloudy Dystrophy of François

“…In our case, unlike the previous report,3 the endothelium revealed prominent polymegathism and pleomorphism, bilaterally. No endothelial accumulations or guttae were seen.…”

“…Similarly in CCDF, when tension in Descemet's membrane is released, collagen lamellae inserting obliquely might create a reproducible polygonal ridge pattern. 3 In our case, unlike the previous report, 3 the endothelium revealed prominent polymegathism and pleomorphism, bilaterally. No endothelial accumulations or guttae were seen.…”

“…In our case, however, hyperreflective deposits extended towards posterior stroma, where they merged with multiple dark striae with increased intensities of extracellular matrices. Additionally, there were no stromal layers, where keratocytes could be observed; and this was found to be the most important difference when compared with the previous observations 3. It might be postulated that in more advanced stages of the dystropy, a large number of extracellular vacuoles filled with mucopolysaccharide and lipid-like material are present in full-thickness corneal stroma.…”

“…In this report we present the in vivo confocal microscopic findings in a patient with phenotypic CCDF, but without any history suggesting autosomal dominant inheritance. To the best of our knowledge, this is the second report of the in vivo confocal microscopy findings in presumed CCDF 3…”

Terrien's Marginal Degeneration Accompanied by Latticed Stromal Opacities

“…Although the polygonal stromal opacities in the present case were somewhat similar to posterior crocodile shagreen, the corneal opacities of our case were located in the anterior but not in the posterior corneal stroma. 17,18 Thus, we could distinguish our case of branching stromal lattice figure keratopathy from posterior crocodile shagreen.…”