Cases reported "Corneal Opacity"

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1/70. Double anterior chamber deep lamellar keratoplasty: case report.

    PURPOSE AND methods: We report a case in which, although we planned to perform a penetrating keratoplasty for corneal stromal opacity with normal corneal endothelium, the host's Descemet's membrane became inadvertently detached and the operation resulted in double anterior chamber deep lamellar keratoplasty (DLKP). RESULT: After surgery, the patient's corrected visual acuity was 20/30. CONCLUSION: Double anterior chamber DLKP is safe and valuable.
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2/70. corneal opacity and congenital glaucoma associated with massive heparan sulfaturia: report of one case.

    A four month-old male infant was noted to have had severe corneal opacity since birth. Buphthalmos, increased intraocular pressure and corneal opacity with neovascularization were noted during physical examination. There was neither dysmorphic face nor hirsutism and the liver and spleen were impalpable. In addition, hypotonia, poor head control, and absence of Moro and grasping reflexes were also noted. There was no evidence of congenital infection by TORCH study. Tests of both urine and plasma amino acids were within normal limits. However, excessive urinary excretion of heparan sulfate was detected by thin-layer chromatography. corneal transplantation was performed at 6 months old. Histopathological examination of the corneal button showed homogeneous thickening of Bowmen's membrane and intracytoplasmic pinkish substances in corneal stroma. The alcian blue stain was positive, which was consistent with mucopolysaccharidosis of cornea. The manifestation of this case may be a clinical variant of Sanfilippo's syndrome (Mucopolysaccharidosis type III).
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3/70. Amniotic membrane transplantation in acute chemical and thermal injury.

    PURPOSE: To present a case of chemical injury and a case of thermal injury treated by amniotic membrane transplantation in acute phase. methods: case reports. An eye with sodium hydroxide injury, opaque cornea, and limbal ischemia of more than 180 degrees and an eye with hot tea injury, opaque cornea, stromal edema, and scarring were treated by amniotic membrane transplantation within the first few weeks of injury. RESULTS: In the eye with sodium hydroxide injury, 4 months after amniotic membrane transplantation, the ocular surface is stable, superficial corneal scarring with vascularization is present, and visual acuity is 20/25. In the eye with thermal injury, 6 months after amniotic membrane transplantation, the ocular surface is stable, but there is superficial scarring and vascularization, and visual acuity is 20/20. CONCLUSIONS: Amniotic membrane transplantation can be considered in chemical injury with severe limbal ischemia and in severe thermal injury in acute phase. Long-term studies are warranted to evaluate further the efficacy of amniotic membrane transplantation in these clinical situations.
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4/70. Corneal manifestations of X-linked ichthyosis in two brothers.

    PURPOSE: To report the unusual manifestation of X-linked ichthyosis in two brothers. methods: Leukocyte separation and sterylsulfatase assay are performed to show the deficiency of sterylsulfatase. RESULTS: Two brothers presented in our clinic with cutaneous alterations consistent with X-linked ichthyosis. Ocular examination disclosed fine, flour-like, punctate, evenly, and diffusely distributed opacities of the posterior corneal stroma, close to descemet membrane in both patients. In one patient, superficial, small, granular opacities were detected. They were gray in color and seemed to involve the epithelium and the subepithelial and anterior stromal layers. In both patients, the deficiency of sterylsulfatase could be shown and confirmed the diagnosis. CONCLUSIONS: flour-like opacities in the posterior stroma have been shown to be a common manifestation of X-linked ichthyosis. Though the underlying biochemical defect in X-linked ichthyosis has been discovered, the question of how these opacities develop is still a subject of debate. Subepithelial stromal keratopathies or epithelial irregularities are uncommon and are rarely described in the literature. The superficial corneal changes seen in one of our patients are unusual and are not similar to those reported by other authors.
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5/70. Ocular findings in Fryns syndrome.

    PURPOSE: To demonstrate the ocular histopathologic findings in Fryns syndrome, a multiple congenital anomaly syndrome, with characteristic features including Dandy-Walker malformation, cleft palate, diaphragmatic hernia, lung hypoplasia, distal limb anomalies and polyhydramnios. The prevalence is about 0.7 per 10,000 births. Reported ocular features include microphthalmus, "cloudy cornea", irregularities of Bowman's layer, thickened posterior lens capsule and retinal dysplasia. methods: Case report. The ocular histopathologic and ultrastructural findings in a male fetus with Fryns syndrome who died immediately after his birth at 26th week of gestation are shown. RESULTS: An abnormal Descemet's membrane was found in addition to "cloudy corneae". Electron microscopy demonstrated absence of the banded collagen fibrils in Descemet's membrane, indicating corneal endothelial dysfunction. Otherwise, the eye was morphologically normal for its age; none of the other reported ocular features of Fryns syndrome were found. CONCLUSION: Corneal endothelial dysfunction might cause abnormal composition of anterior Descemet's membrane and could contribute to the "cloudy cornea" known to occur in Fryns syndrome.
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6/70. corneal opacity after repeated photorefractive keratectomy.

    corneal opacity developed in an eye that had photorefractive keratectomy (PRK) with a 193 nm excimer laser 5 times over 3 years. Six months after the last PRK, a partial penetrating keratoplasty was performed. The cornea was stained and immunohistochemically evaluated for collagen types. light microscopy showed thickening of epithelial layers, proliferation of subepithelial fibroblasts, and the absence of Bowman's membrane. Transmission electron microscopy showed irregular collagen lamellae and electron-dense deposits adjacent to keratocytes. The staining was positive for alcian blue, and immunohistochemistry was positive for type IV and VI collagen. This case suggests that corneal opacity after repeated PRK is the result of deposits of type IV and VI collagen and acidic mucoprotein in the extracellular matrix.
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7/70. Deep lamellar keratoplasty as surgical management of anterior and posterior segment injuries to the eye.

    PURPOSE: To report on the feasibility of combined deep lamellar keratoplasty and vitreoretinal surgery in one patient with corneal opacity associated with retinal detachment. methods: A 35-year-old man presented with a major hematocornea and retinal detachment after experiencing a right ocular trauma with corneoscleral wound 1 month earlier. We elected to perform deep lamellar keratoplasty to perform vitreoretinal surgery through the bared Descemet's membrane within the same surgical procedure. RESULTS: Deep lamellar keratoplasty offered perfect visibility of the anterior and posterior segments of the eye through the bared Descemet's membrane during the 4-hour operation. Descemet's membrane was resilient enough to maintain remarkable tightness of the anterior chamber throughout vitreoretinal surgery procedures (vitrectomy, peeling of epiretinal membranes, encircling scleral buckling). Unfortunately, despite our efforts and extended operative time, the retina could not be restored to its position because of the high baseline level of ocular impairment. CONCLUSION: The combined procedure (deep lamellar keratoplasty and pars plana vitrectomy) appeared to be a good and feasible alternative to the temporary keratoprosthesis usually applied in that situation.
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8/70. The histopathology of Peters' anomaly.

    Corneal, iris, and trabecular meshwork tissue in a 23-month-old boy with Peters' anomaly were studied with light and electron microscopy. In the cornea, both Descemet's membrane and endothelium were present beneath the area of the corneal opacity except at the point of iris adhesion. However, Descemet's membrane was thin and embryonal in nature, and the endothelium markedly attenuated. The trabecular meshwork demonstrated changes characteristic of old age such as wide-banded collagen and the presence of phagocytosed pigment granules in the endothelium. The iris appeared normal except for anterior synechiae to the corneal opacity. Developmentally, the primary fault appeared to be a failure in the normal differentiation of endothelial cells of the cornea and trabecular meshwork.
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9/70. Congenital central corneal leukoma (Peters' anomaly).

    Corneal buttons from three patients with congenital central corneal leukoma (Peters' anomaly) were examined by light and electron microscopy. All cases exhibited a central absence of Bowman's membrane and iris synechiae to the periphery of the leukoma. In the first patient, extensive keratolenticular adhesion with retrocorneal fibrous tissue filling the central defect of endothelium and Descemet's membrane implicated late anterior displacement of the normally developed lens as the cause of secondary endothelial degeneration. In the second patient, attenuated endothelium and abnormally composed Descemet's membrane indicated primary dysgenesis of the endothelium. In the third patient, the extensive defect of posterior stroma with anterior stromal disorganization and endothelial metaplasia suggested dysgenesis of both the keratocytic and endothelial mesoderm. Thus, although no unified pathogenic mechanism was consistently applicable, either primary or secondary dysgenesis of the corneal mesoderm must be responsible.
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10/70. Cornea with Peters' anomaly: perturbed differentiation of corneal cells and abnormal extracellular matrix in the corneal stroma.

    PURPOSE: We examined histopathologically the anterior ocular segment including the cornea and lens of an eye which had been enucleated in a patient with Peters' anomaly because of untreatable corneal perforation. Special effort was made to differentiate the corneal stromal and endothelial cells, and the stromal extracellular matrix.methods: light microscopy, with hematoxylin and eosin staining, and transmission electron microscopy were employed.RESULTS: Corneal endothelial cells and Descemet's membrane were not detected in the central cornea, where there were immature cells with a fibroblastic configuration. The inner surface of the peripheral cornea was covered with cells containing pigment granules in the cytoplasm. Cell density in the central corneal stroma was relatively high. The diameter of the stromal collagen fibrils was not uniform. A mature collagen fibril-free area was also seen in the central corneal stroma.CONCLUSIONS: Differentiation of neural crest-derived cells in corneal stroma and endothelium might have been perturbed in the cornea of this patient with Peters' anomaly, inducing the defect in the corneal endothelium and the qualitative and quantitative abnormalities of the extracellular matrix.
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