1/93. Recurrent macular corneal dystrophy type II 49 years after penetrating keratoplasty.recurrence of macular corneal dystrophy after keratoplasty is rare. We report light microscopic, immunohistochemical, electron microscopic, and serologic findings in a 78-year-old woman who underwent regrafting 49 years following the first penetrating keratoplasty. Examination of the corneal button revealed deposits of glycosaminoglycans in the graft beneath the Bowman layer, throughout the stroma, and in the endothelium with positive staining for antigenic keratan sulfate. By transmission electron microscopy, intracellular and extracellular deposits of a fibrillogranular material were detected in the stroma, descemet membrane, and endothelium. The serum level of antigenic keratan sulfate was normal. Our findings indicate that macular corneal dystrophy type II may show late recurrence after penetrating keratoplasty with intense deposition of antigenic keratan sulfate in all corneal layers.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
2/93. Confocal microscopy in posterior polymorphous corneal dystrophy.PURPOSE: To report the distinguishing characteristics of posterior polymorphous corneal dystrophy (PPMD) using confocal microscopy. MATERIAL AND methods: Two consecutive patients with PPMD were prospectively examined using a white-light tandem scanning confocal microscope with a 24x/0.60 contact objective. RESULTS: At the level of Descement's membrane, roundish hyporeflective images were found in 1 patient. In the other patient, hyporeflective bands were detected. In both patients, patchy hyperreflective areas were identified. CONCLUSION: Confocal microscopy may allow diagnosis of PPMD by demonstrating the alterations in Descement's membrane. This technique is especially valuable in cases of endothelial decompensation, where slit-lamp and specular microscopy may fail to demonstrate changes in Descement's membrane.- - - - - - - - - - ranking = 3keywords = membrane (Clic here for more details about this article) |
3/93. Terrien's marginal degeneration associated with posterior polymorphous dystrophy.PURPOSE: To document an association between Terrien's marginal degeneration and posterior polymorphous dystrophy. methods: A 23-year-old Saudi man presented with decreased vision, peripheral corneal thinning with vascularization and scarring, and abnormalities of the posterior stroma and Descemet's membrane. RESULTS: Clinical examination, corneal topography, and specular microscopy were consistent with a diagnosis of Terrien's marginal degeneration and posterior polymorphous dystrophy. CONCLUSION: We report the first case, to our knowledge, of the simultaneous occurrence of Terrien's marginal degeneration with posterior polymorphous dystrophy.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
4/93. Endothelial alterations in congenital corneal dystrophies.We studied the clinical and ultrastructural findings in three different types of congenital endothelial dystrophies: hereditary posterior polymorphous dystrophy, congenital hereditary corneal dystrophy, and a nonhereditary congenital endothelial dystrophy. In the first patient, with hereditary posterior polymorphous dystrophy, a layer of epithelial-like cells was observed adjacent to endothelial cells on the posterior corneal surface. Descemet's membrane displayed a multilaminar pattern and consisted of an anterior, thin (3 mu), PAS-positive layer and a posterior, thicker (25 to 30 mu) zone of abnormal collagen. The second patient, with congenital hereditary endothelial dystrophy, showed a thickened multilaminar Descemet's membrane and scant endothelial cells. In the third patient with nonhereditary congenital endothelial dystrophy, the thickened Descemet's membrane was lined posteriorly by a retrocorneal fibrous membrane. A few degenerated endothelial cells were present. All three cases showed 100- to 110-nm banding posteriorly. In these three clinically distinct entities, electron microscopy was useful in demonstrating the unusual form of endothelial transformation to epithelial-like cells in one patient, in contrast to the more common fibroblast-like metaplasia of endothelial cells seen in the other two patients.- - - - - - - - - - ranking = 4keywords = membrane (Clic here for more details about this article) |
5/93. Recurrent corneal granular dystrophy: a clinicopathologic study.recurrence of granular corneal dystrophy occurred in two patients following two successive penetrating corneal grafts. In the first patient, the deposits involved the central stroma of the donor cornea, almost to the level of Descemet's membrane. The second patient had predominantly subepithelial deposits in bilateral recurrences. Electron microscopy revealed rod-shaped, electron dense material typical of granular dystrophy in both patients.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
6/93. Fleck (Mouchetee) dystrophy of the cornea.Five families, four Caucasian and one Negro (14 patients) with fleck (speckled or Mouchetee) dystrophy of the cornea are presented. In each, the typical presentation of fine scattered fleck-like dystrophic lesions was found throughout all layers of the central and peripheral corneal stroma but not affecting the epithelium, Bowman's membrane, Descemet's membrane or the endothelium. Clear stroma was noted between each lesion. visual acuity in all patients was normal or only slightly affected. Except for an occasional patient with minor photophobia, almost all patients found affected with this dystrophy were asymptomatic in regard to their corneal condition. There was no decrease in corneal sensitivity noted in any patient. All families displayed evidence of an autosomal dominant hereditary pattern and progression of the dystrophy is slow and benign in character. With the exception of one family with atopic disease, no systemic organic illness was noted by histroy in all of the families. Laboratory screening of 2 of the 5 families showed no abnormalities of any systemic metabolic disorders. The characteristic clinical picture and favorable prognosis of this condition enables one to easily differentiate this condition from other known parenchymatous corneal dystrophies. The incidence of this condition is probably much more common than the reported cases in the literature might indicate.- - - - - - - - - - ranking = 2keywords = membrane (Clic here for more details about this article) |
7/93. Sloughing of corneal epithelium and wound healing complications associated with laser in situ keratomileusis in patients with epithelial basement membrane dystrophy.PURPOSE: To report sloughing of corneal epithelium during laser in situ keratomileusis and subsequent wound healing complications in patients with epithelial basement membrane dystrophy. methods: In a retrospective study, the surgical procedures, postoperative course, and visual acuities of 16 eyes of nine patients with epithelial basement membrane dystrophy who underwent laser in situ keratomileusis complicated with epithelial sloughing at three centers were reviewed. The mean follow-up period was 23 weeks (range, 4 to 52 weeks). RESULTS: In 13 (81%) of 16 eyes with epithelial basement membrane dystrophy, epithelial sloughing occurred during laser in situ keratomileusis. In eight of the 13 eyes, epithelial growth beneath the flap was observed. The flap was lifted and the interface epithelium scraped in six eyes. Flap melt or keratolysis occurred in four eyes. At the last follow-up visit, 13 of 16 eyes had an uncorrected visual acuity of 20/30 or better, and all eyes had a best-corrected visual acuity of 20/30 or better. CONCLUSIONS: patients with epithelial basement membrane dystrophy have poorly adherent corneal epithelium and are predisposed to epithelial sloughing during the microkeratome pass of laser in situ keratomileusis. This may lead to flap distortion, interface epithelial growth, flap keratolysis, and corneal scarring. It is not recommended that laser in situ keratomileusis be performed in patients with classic, symptomatic epithelial basement membrane dystrophy. In patients who present with mild and asymptomatic epithelial basement membrane dystrophy, laser in situ keratomileusis should be performed with caution, or photorefractive keratectomy may be the preferred refractive procedure.- - - - - - - - - - ranking = 10keywords = membrane (Clic here for more details about this article) |
8/93. Triple anterior chamber after full-thickness lamellar keratoplasty for lattice corneal dystrophy.PURPOSE: To report a patient with lattice corneal dystrophy type I (LCDI) who developed a triple anterior chamber after full-thickness lamellar keratoplasty (LKP). methods: A 46-year-old woman underwent a full-thickness LKP in her right eye for visual disturbances caused by LCDI. Her visual acuity was 20/200 OD before surgery. A complete ophthalmic examination, including slit lamp biomicroscopy and optical coherence tomography (OCT), was performed before and after surgery. Molecular genetic analysis was performed on dna extracted from the peripheral leukocytes. RESULTS: The surgery was performed uneventfully; however, extra spaces posterior to the graft, along with the severe graft edema, were observed to form a triple anterior chamber a few days after surgery. The extra spaces resolved in 3 weeks with no surgical treatment, and her visual acuity improved to 20/20 OD without correction 3 months after surgery. The triple anterior chamber was clearly demonstrated by OCT, but not by slit lamp biomicroscopy. A heterozygous single base-pair transition (CGC to TGC, arginin to cysteine) was detected in codon 124 of the TGFBIgene in the patient. CONCLUSION: The separation of the graft and the host's deep corneal tissue and a Descemet's membrane detachment in the host's cornea caused the triple anterior chamber. The Descemet's membrane detachment demonstrated the weak adhesion of the stroma and the Descemet's membrane, probably resulting from a dysfunction of the TGFBI protein caused by the mutation of the TGFBIgene. OCT is useful for the objective documentation of the posterior corneal region even with severe corneal edema.- - - - - - - - - - ranking = 3keywords = membrane (Clic here for more details about this article) |
9/93. Congenital hereditary endothelial dystrophy and band keratopathy in an infant with corpus callosum agenesis.PURPOSE: To report the features of a syndrome of endothelial failure and band-shaped keratopathy in an infant with corpus callosum agenesis. methods: The clinical and histopathologic features of an infant presenting with bilateral corneal clouding and corpus callosum agenesis are reported. The patient underwent bilateral penetrating keratoplasty at ages 28 months and 4 years. light and electron microscopy were used to characterize the structural changes. RESULTS: The epithelium was thin and degenerate. Bowman's membrane contained spherical aggregates that were present also within a connective tissue pannus. The midstroma was normal, but there were floral and rope-like aggregations of collagen in the pre-Descemet's membrane region. Under electron microscopy, the spherules formed target-shaped lesions with a central focus of alternating electron-dense and lucent material. Numerous microfilaments in the pannus and anterior stroma labeled with fibrillin-1 antibody. Microfilaments within fibroblasts were stained with vimentin antibody. Posteriorly, the endothelium was mainly absent and Descemet's membrane showed a fetal layer and a posterior collagenous layer. CONCLUSION: Corneal appearances in this patient were in keeping with those of congenital hereditary endothelial corneal dystrophy. However, there was no family history and neither parent showed a clinical endothelial abnormality. The presence of fetal, banded material in Descemet's membrane suggested that endothelial loss began at or near the time of birth. The band keratopathy was regarded as a secondary change. The association with corpus callosum agenesis does not appear to have been described previously.- - - - - - - - - - ranking = 4keywords = membrane (Clic here for more details about this article) |
10/93. Microstructural assessment of rare corneal dystrophies using real-time in vivo confocal microscopy.PURPOSE: To analyse and describe three cases of rare corneal dystrophy and highlight their in vivo microstructural features. methods: Subject 1 was diagnosed with a posterior stromal fleck corneal dystrophy Two of her three children were also affected. Subjects 2 and 3 exhibited an almost identical clinical appearance on biomicroscopic examination, such that both clinically were diagnosed as having pre-Descemet's dystrophies. All subjects underwent in vivo confocal microscopy and approximately 300 sequential digital images were obtained and analysed for each cornea. RESULTS: In vivo confocal microscopy of subject 1 demonstrated an abnormal appearance of numerous large ovoid particles, measuring 50-70 microm in diameter in the mid and posterior stroma as well as smaller hyperreflective dot-like intracellular deposits, of less than 1 microm diameter. Despite the near-identical clinical appearance, subjects 2 and 3 could be clearly differentiated by in vivo confocal microscopy. Subject 2 exhibited small, irregular, optically dense particles, mainly in the anterior stroma, whereas subject 3 possessed classical involvement of the stroma immediately adjacent to Descemet's membrane, with numerous regular small, hyperreflective particles. CONCLUSIONS: The ability of in vivo confocal microscopy to localize and accurately measure various elements in different corneal layers may help to resolve whether abnormalities are intra- or extracellular, and aid clearer differentiation of rare corneal disorders.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
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