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Sample records for schnyder corneal dystrophy

  1. Distrofia corneal de Schnyder

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    Michel Guerra Almaguer

    Full Text Available La principal entidad hereditaria con depósitos de lípidos en el estroma corneal es la distrofia cristalina central, conocida como distrofia de Schnyder, quien la describió en Suiza en 1927. Se caracteriza por depósitos blanco-amarillentos en el estroma corneal central y superficial. Se presenta un paciente de 28 años, del sexo masculino y piel negra, con antecedente de salud anterior. Acudió a consulta y refirió una disminución de la visión y cambio de coloración progresiva de ambos ojos, de años de evolución. En la exploración oftalmológica de ambos ojos se apreciaron lesiones blanquecinas anulares a nivel del estroma corneal, con ligera turbidez corneal central. Los estudios refractivos realizados constataron un astigmatismo hipermetrópico simple. El resto del examen oftalmológico fue negativo. Para el diagnóstico de certeza se empleó el microscopio confocal. Se concluye que el caso presenta una distrofia corneal estromal de tipo cristalina, de Schnyder.

  2. UBIAD1 mutation alters a mitochondrial prenyltransferase to cause Schnyder corneal dystrophy.

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    Michael L Nickerson

    2010-05-01

    Full Text Available Mutations in a novel gene, UBIAD1, were recently found to cause the autosomal dominant eye disease Schnyder corneal dystrophy (SCD. SCD is characterized by an abnormal deposition of cholesterol and phospholipids in the cornea resulting in progressive corneal opacification and visual loss. We characterized lesions in the UBIAD1 gene in new SCD families and examined protein homology, localization, and structure.We characterized five novel mutations in the UBIAD1 gene in ten SCD families, including a first SCD family of Native American ethnicity. Examination of protein homology revealed that SCD altered amino acids which were highly conserved across species. Cell lines were established from patients including keratocytes obtained after corneal transplant surgery and lymphoblastoid cell lines from Epstein-Barr virus immortalized peripheral blood mononuclear cells. These were used to determine the subcellular localization of mutant and wild type protein, and to examine cholesterol metabolite ratios. Immunohistochemistry using antibodies specific for UBIAD1 protein in keratocytes revealed that both wild type and N102S protein were localized sub-cellularly to mitochondria. Analysis of cholesterol metabolites in patient cell line extracts showed no significant alteration in the presence of mutant protein indicating a potentially novel function of the UBIAD1 protein in cholesterol biochemistry. Molecular modeling was used to develop a model of human UBIAD1 protein in a membrane and revealed potentially critical roles for amino acids mutated in SCD. Potential primary and secondary substrate binding sites were identified and docking simulations indicated likely substrates including prenyl and phenolic molecules.Accumulating evidence from the SCD familial mutation spectrum, protein homology across species, and molecular modeling suggest that protein function is likely down-regulated by SCD mutations. Mitochondrial UBIAD1 protein appears to have a highly

  3. Corneal dystrophies

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    Klintworth Gordon K

    2009-02-01

    Full Text Available Abstract The term corneal dystrophy embraces a heterogenous group of bilateral genetically determined non-inflammatory corneal diseases that are restricted to the cornea. The designation is imprecise but remains in vogue because of its clinical value. Clinically, the corneal dystrophies can be divided into three groups based on the sole or predominant anatomical location of the abnormalities. Some affect primarily the corneal epithelium and its basement membrane or Bowman layer and the superficial corneal stroma (anterior corneal dystrophies, the corneal stroma (stromal corneal dystrophies, or Descemet membrane and the corneal endothelium (posterior corneal dystrophies. Most corneal dystrophies have no systemic manifestations and present with variable shaped corneal opacities in a clear or cloudy cornea and they affect visual acuity to different degrees. Corneal dystrophies may have a simple autosomal dominant, autosomal recessive or X-linked recessive Mendelian mode of inheritance. Different corneal dystrophies are caused by mutations in the CHST6, KRT3, KRT12, PIP5K3, SLC4A11, TACSTD2, TGFBI, and UBIAD1 genes. Knowledge about the responsible genetic mutations responsible for these disorders has led to a better understanding of their basic defect and to molecular tests for their precise diagnosis. Genes for other corneal dystrophies have been mapped to specific chromosomal loci, but have not yet been identified. As clinical manifestations widely vary with the different entities, corneal dystrophies should be suspected when corneal transparency is lost or corneal opacities occur spontaneously, particularly in both corneas, and especially in the presence of a positive family history or in the offspring of consanguineous parents. Main differential diagnoses include various causes of monoclonal gammopathy, lecithin-cholesterol-acyltransferase deficiency, Fabry disease, cystinosis, tyrosine transaminase deficiency, systemic lysosomal storage

  4. Corneal stromal dystrophies: a clinical pathologic study

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    Elvira Barbosa Abreu

    2012-12-01

    Full Text Available INTRODUCTION: Corneal dystrophy is defined as bilateral and symmetric primary corneal disease, without previous associated ocular inflammation. Corneal dystrophies are classified according to the involved corneal layer in superficial, stromal, and posterior dystrophy. Incidence of each dystrophy varies according to the geographic region studied. PURPOSE: To evaluate the prevalence of stromal corneal dystrophies among corneal buttons specimens obtained by penetrating keratoplasty (PK in an ocular pathology laboratory and to correlate the diagnosis with patient age and gender. METHODS: Corneal button cases of penetrating keratoplasty from January-1996 to May-2009 were retrieved from the archives of The Henry C. Witelson Ophthalmic Pathology Laboratory and Registry, Montreal, Canada. The cases with histopathological diagnosis of stromal corneal dystrophies were stained with special stains (Peroxid acid Schiff, Masson trichrome, Congo red analyzed under polarized light, and alcian blue for classification and correlated with epidemiological information (age at time of PK and gender from patients' file. RESULTS: 1,300 corneal buttons cases with clinical diagnose of corneal dystrophy were retrieved. Stromal corneal dystrophy was found in 40 (3.1% cases. Lattice corneal dystrophy was the most prevalent with 26 cases (65%. Nineteen were female (73.07% and the PK was performed at average age of 59.3 years old. Combined corneal dystrophy was found in 8 (20% cases, 5 (62.5% of them were female and the average age of the penetrating keratoplasty was 54.8 years old. Granular corneal dystrophy was represented by 5 (12.5% cases, and 2 (40% of them were female. Penetrating keratoplasty was performed at average age of 39.5 years old in granular corneal dystrophy cases. Macular corneal dystrophy was present in only 1 (2.5% case, in a 36 years old female. CONCLUSION: Systematic histopathological approach and evaluation, including special stains in all stromal

  5. Genetics Home Reference: lattice corneal dystrophy type I

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    ... corneal dystrophy type I lattice corneal dystrophy type I Printable PDF Open All Close All Enable Javascript ... expand/collapse boxes. Description Lattice corneal dystrophy type I is an eye disorder that affects the clear, ...

  6. Immunoglobulins in granular corneal dystrophy Groenouw type I

    DEFF Research Database (Denmark)

    Møller, H U; Bojsen-Møller, M; Schrøder, H D

    1993-01-01

    Three patients with granular corneal dystrophy Groenouw type I underwent corneal grafting, and cryostat sections of the corneal buttons were examined immunohistochemically for immunoglobulins. Positive results were obtained for IgG, Kappa-, and Lambda chains with immunofluorescence technique. The...

  7. Posterior amorphous corneal dystrophy caused by a de novo deletion.

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    Odent, S; Casteels, I; Cassiman, C; Dieltiëns, M; Hua, M-T; Devriendt, K

    2017-01-01

    We present a newborn diagnosed with posterior amorphous corneal dystrophy (PACD). PACD is a rare disorder with partial or complete posterior lamellar corneal opacification. Genetic screening showed a deletion of chromosome 12q21.33-q22 containing the identified four small leucine-rich proteoglycans (SLRP's) associated with this particular dystrophy. Neither parents were carrier of the deletion. To our knowledge, this is the first report of a de novo mutation causing PACD.

  8. Intraocular pressure, corneal thickness, and corneal hysteresis in Steinert's myotonic dystrophy

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    Carlos Alexandre de A. Garcia Filho

    2011-06-01

    Full Text Available PURPOSE: Low intraocular pressure (IOP measured by Goldmann applanation tonometry (GAT is one of the ocular manifestations of Steinert's myotonic dystrophy. The goal of this study was to evaluate the corneal-compensated IOP as well as corneal properties (central corneal thickness and corneal hysteresis in patients with myotonic dystrophy. METHODS: A total of 12 eyes of 6 patients with Steinert's myotonic dystrophy (dystrophy group and 12 eyes of 6 age-, race-, and gender-matched healthy volunteers (control group were included in the study. GAT, Dynamic Contour Tonometry (DCT-Pascal and Ocular Response Analyzer (ORA were used to assess the IOP. Central corneal thickness was obtained by ultrasound pachymetry, and corneal hysteresis was analyzed using the ORA device. In light of the multiplicity of tests performed, the significance level was set at 0.01 rather than 0.05. RESULTS: The mean (standard deviation [SD] GAT, DCT, and corneal-compensated ORA IOP in the dystrophy group were 5.4 (1.4 mmHg, 9.7 (1.5 mmHg, and 10.1 (2.6 mmHg, respectively. The mean (SD GAT, DCT, and corneal-compensated ORA IOP in the control group was 12.6 (2.9 mmHg, 15.5 (2.7 mmHg, and 15.8 (3.4 mmHg, respectively. There were significant differences in IOP values between dystrophy and control groups obtained by GAT (mean, -7.2 mmHg; 99% confidence interval [CI], -10.5 to -3.9 mmHg; P<0.001, DCT (mean, -5.9 mmHg; 99% CI, -8.9 to -2.8 mmHg; P<0.001, and corneal-compensated ORA measurements (mean, -5.7 mmHg; 99% CI, -10.4 to -1.0 mmHg; P=0.003. The mean (SD central corneal thickness was similar in the dystrophy (542 [31] µm and control (537 [11] µm groups (P=0.65. The mean (SD corneal hysteresis in the dystrophy and control groups were 11.2 (1.5 mmHg and 9.7 (1.2 mmHg, respectively (P=0.04. CONCLUSIONS: Patients with Steinert's myotonic dystrophy showed lower Goldmann and corneal-compensated IOP in comparison with healthy individuals. Since central corneal thickness and

  9. Intraocular pressure, corneal thickness, and corneal hysteresis in Steinert's myotonic dystrophy.

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    Garcia Filho, Carlos Alexandre de A; Prata, Tiago Santos; Sousa, Aline Katia Siqueira; Doi, Larissa Morimoto; Melo Jr, Luiz Alberto Soares

    2011-01-01

    Low intraocular pressure (IOP) measured by Goldmann applanation tonometry (GAT) is one of the ocular manifestations of Steinert's myotonic dystrophy. The goal of this study was to evaluate the corneal-compensated IOP as well as corneal properties (central corneal thickness and corneal hysteresis) in patients with myotonic dystrophy. A total of 12 eyes of 6 patients with Steinert's myotonic dystrophy (dystrophy group) and 12 eyes of 6 age-, race-, and gender-matched healthy volunteers (control group) were included in the study. GAT, Dynamic Contour Tonometry (DCT-Pascal) and Ocular Response Analyzer (ORA) were used to assess the IOP. Central corneal thickness was obtained by ultrasound pachymetry, and corneal hysteresis was analyzed using the ORA device. In light of the multiplicity of tests performed, the significance level was set at 0.01 rather than 0.05. The mean (standard deviation [SD]) GAT, DCT, and corneal-compensated ORA IOP in the dystrophy group were 5.4 (1.4) mmHg, 9.7 (1.5) mmHg, and 10.1 (2.6) mmHg, respectively. The mean (SD) GAT, DCT, and corneal-compensated ORA IOP in the control group was 12.6 (2.9) mmHg, 15.5 (2.7) mmHg, and 15.8 (3.4) mmHg, respectively. There were significant differences in IOP values between dystrophy and control groups obtained by GAT (mean, -7.2 mmHg; 99% confidence interval [CI], -10.5 to -3.9 mmHg; Pcorneal-compensated ORA measurements (mean, -5.7 mmHg; 99% CI, -10.4 to -1.0 mmHg; P=0.003). The mean (SD) central corneal thickness was similar in the dystrophy (542 [31] µm) and control (537 [11] µm) groups (P=0.65). The mean (SD) corneal hysteresis in the dystrophy and control groups were 11.2 (1.5) mmHg and 9.7 (1.2) mmHg, respectively (P=0.04). Patients with Steinert's myotonic dystrophy showed lower Goldmann and corneal-compensated IOP in comparison with healthy individuals. Since central corneal thickness and corneal hysteresis did not differ significantly between groups, the lower IOP readings documented in this

  10. Mutation-Induced Deamidation of Corneal Dystrophy-Related Transforming Growth Factor β-Induced Protein

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    Nielsen, Nadia Sukusu; Juhl, Dennis Wilkens; Poulsen, Ebbe Toftgaard

    2017-01-01

    Mutations in the transforming growth factor β-induced protein (TGFBIp) cause phenotypically diverse corneal dystrophies, where protein aggregation in the cornea leads to severe visual impairment. Previous studies have shown a relationship between mutant-specific corneal dystrophy phenotypes...

  11. Delayed epithelial healing after keratoplasty for lattice corneal dystrophy.

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    Foerster, Claudia G; Langenbucher, Achim; Cursiefen, Claus; Kruse, Friedrich E; Seitz, Berthold

    2007-12-01

    To compare the time necessary for complete epithelial healing after penetrating keratoplasty carried out for various corneal dystrophies. In a retrospective single-center study, 679 eyes that underwent nonmechanical keratoplasty were evaluated concerning postoperative epithelial healing time. On the basis of corneal pathology, the eyes were divided into 5 groups: group 1, lattice dystrophy (n = 9); group 2, macular dystrophy (n = 16); group 3, Fuchs dystrophy (n = 207); group 4, granular dystrophy (n = 13); group 5, keratoconus (n = 434). After keratoplasty, the time necessary for complete healing of the epithelial defect was compared among the groups. In lattice dystrophy, 22% showed delayed healing, in contrast to 0% in granular dystrophy. Mean time necessary for healing in group 1 (8.8 +/- 9.4 days) was significantly longer than in group 2 (2.5 +/- 2.5 days, P = 0.003), group 3 (4.4 +/- 4.1 days, P = 0.09), group 4 (2.2 +/- 1.4 days, P = 0.003), and group 5 (3.1 +/- 2.7 days, P = 0.005). In patients with lattice dystrophy, delayed epithelial healing after penetrating keratoplasty may be anticipated. Patients should be counseled accordingly.

  12. Granular corneal dystrophy in 830-nm spectral optical coherence tomography.

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    Kaluzny, Bartlomiej J; Szkulmowska, Anna; Szkulmowski, Maciej; Bajraszewski, Tomasz; Wawrocka, Anna; Krawczynski, Maciej R; Kowalczyk, Andrzej; Wojtkowski, Maciej

    2008-08-01

    Spectral optical coherence tomography (SOCT) is a new imaging technique that can provide high-resolution tomograms much faster and with higher sensitivity than conventional Time domain (TdOCT) systems. Its usefulness in producing cross-sectional imaging of different corneal pathologies in vivo has already been presented. The aim of this case report is to show 830-nm SOCT findings in granular corneal dystrophy. A 48-year-old woman with granular corneal dystrophy was examined with a slit-lamp, confocal microscope (Confoscan 4) and a prototype SOCT instrument constructed at the Institute of Physics, Nicolaus Copernicus University, Torun, Poland. A genetic examination showed a mutation of arginine 555-to-tryptophan (Arg555Trp) in the TGFBI gene that confirmed the clinical diagnosis. SOCT tomograms showed multiple hyperreflective changes throughout the corneal stroma that corresponded to hyaline deposits. Precise and objective assessment of the localization, size, shape, and light scattering properties of the pathologic changes was possible. Three-dimensional rendering of the acquired data allowed a comprehensive evaluation of the deposits in the central cornea. SOCT (830 nm) provides clinically valuable 2- and 3-dimensional assessments of pathomorphologic changes in granular corneal dystrophy in vivo.

  13. Delayed corneal epithelial wound healing after penetrating keratoplasty in individuals with lattice corneal dystrophy.

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    Kawamoto, Koji; Morishige, Naoyuki; Yamada, Naoyuki; Chikama, Tai-Ichiro; Nishida, Teruo

    2006-07-01

    To investigate whether corneal epithelial cells of individuals with lattice corneal dystrophy (LCD) possess an intrinsic defect. Retrospective case-control study. The medical charts of nine individuals with LCD and those of 14 patients with keratoconus and 11 patients with corneal leukoma (controls), all of whom underwent penetrating keratoplasty (PKP) in one eye at Yamaguchi University Hospital between February 1998 and November 2001, were examined for the time for epithelial resurfacing after surgery. The time required for resurfacing of the corneal epithelium after PKP was significantly greater in LCD patients (8.56 +/- 4.95 days, mean +/- SD) than in patients with either keratoconus (1.71 +/- 0.91 days, P = .006) or corneal leukoma (3.00 +/- 1.95 days, P = .03). Corneal epithelial wound healing was delayed in LCD patients after PKP, suggesting that the keratoepithelin gene mutations responsible for this condition affect corneal epithelial cells.

  14. Punctiform and Polychromatophilic Dominant Pre-Descemet Corneal Dystrophy.

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    Lagrou, Lisa; Midgley, Julian; Romanchuk, Kenneth Gerald

    2016-04-01

    To describe the slit-lamp appearance and corneal confocal microscopy of autosomal dominant punctiform and polychromatophilic pre-Descemet corneal dystrophy in 3 members of the same family. Slit-lamp examination of a 9-year-old boy showed bilateral polychromatophilic corneal opacities in a pre-Descemet membrane location evenly deposited limbus to limbus, both horizontally and vertically, with an intervening clear cornea. The corneal endothelium was normal on corneal confocal microscopy, with hyperreflective opacities of various sizes located pre-Descemet membrane. Slit-lamp examination of the patient's father and brother revealed identical crystalline deposition in the pre-Descemet corneal stroma. The remainders of the eye examinations were otherwise normal in all 3 individuals, and all were asymptomatic. The general physical examination and laboratory investigations of the patient were all normal, as were the laboratory investigations of the other 2 family members. There was no progression in the corneal findings over 6 months of follow-up. These patients likely illustrate a rare autosomal dominant pre-Descemet crystalline keratopathy that has been reported only once previously.

  15. Clinical findings and treatments of granular corneal dystrophy type 2 (avellino corneal dystrophy): a review of the literature.

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    Han, Kyung Eun; Kim, Tae-im; Chung, Woo Suk; Choi, Seung-il; Kim, Bong-yoon; Kim, Eung Kweon

    2010-09-01

    To review the literature about clinical findings and treatments of granular corneal dystrophy type 2 (GCD2). Various literatures on clinical findings, exacerbations after refractive corneal surgery, and treatment modalities of GCD2 were reviewed. GCD2 is an autosomal dominant disease. Mutation of transforming growth factor beta-induced gene, TGFBI, or keratoepithelin gene in human chromosome 5 (5q31) is the key pathogenic process in patient with GCD2. Corneal trauma activates TGFBI and then it overproduces transforming growth factor beta-induced gene protein (TGFBIp), which is main component of the corneal opacity. Refractive corneal surgery is a popular procedure to correct refractive error worldwide. However, several cases about exacerbation of GCD2 after corneal refractive surgery such as photorefractive keratectomy, laser in situ keratomileusis, and laser epithelial keratomileusis have been reported. The opacities deteriorate patient's best-corrected visual acuity. Recurrence-free interval varies many factors such as the type of procedure the patient had received and the genotype of the patient. To treat the opacities in GCD2, phototherapeutic keratectomy, lamellar keratoplasty, deep lamellar keratoplasty, and penetrating keratoplasty (PKP) were used. However, the recurrence is still an unsolved problem. Perfect treatment of exacerbation after corneal surface ablation does not exist until now. To prevent exacerbation, refractive surgeons must do a careful preoperative examination of candidates in refractive surgeries.

  16. Meretoja’s Syndrome: Lattice Corneal Dystrophy, Gelsolin Type

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    I. Casal

    2017-01-01

    Full Text Available Lattice corneal dystrophy gelsolin type was first described in 1969 by Jouko Meretoja, a Finnish ophthalmologist. It is caused by an autosomal dominant mutation in gelsolin gene resulting in unstable protein fragments and amyloid deposition in various organs. The age of onset is usually after the third decade of life and typical diagnostic triad includes progressive bilateral facial paralysis, loose skin, and lattice corneal dystrophy. We report a case of a 53-year-old female patient referred to our Department of Ophthalmology by severe dry eye and incomplete eyelid closure. She had severe bilateral facial paresis, significant orbicularis, and perioral sagging as well as hypoesthesia of extremities and was diagnosed with Meretoja’s syndrome at the age of 50, confirmed by the presence of gelsolin mutation. At our observation she had bilateral diminished tear film break-up time and Schirmer test, diffuse keratitis, corneal opacification, and neovascularization in the left eye. She was treated with preservative-free lubricants and topical cyclosporine, associated with nocturnal complete occlusion of both eyes, and underwent placement of lacrimal punctal plugs. Ocular symptoms are the first to appear and our role as ophthalmologists is essential for the diagnosis, treatment, and monitoring of ocular alterations in these patients.

  17. [Imaging of corneal dystrophies: Correlations between en face anterior segment OCT and in vivo confocal microscopy].

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    Ghouali, W; Tahiri Joutei Hassani, R; Liang, H; Dupont-Monod, S; Auclin, F; Baudouin, C; Labbé, A

    2015-05-01

    To evaluate the usefulness of en face Optical Coherence Tomography (OCT) for evaluation of corneal dystrophies and to describe correlations with in vivo confocal microscopy (IVCM). Thirty-two eyes of 16 patients with 4 types of corneal dystrophies (epithelial basement membrane dystrophy, Fuchs dystrophy, Reis-Bücklers corneal dystrophy and Crocodile Shagreen dystrophy) were enrolled in this study. Axial and reconstructed en face scans were acquired using OCT. Images were then correlated to IVCM findings. En face OCT provided new insights into the structure, size and depth of corneal tissue alterations in various corneal dystrophies. OCT en face images were well correlated with IVCM features. Despite lower resolution than IVCM, en face OCT offers the advantages of being non-invasive and allowing the analysis of larger corneal areas. En face OCT provides useful new information in corneal dystrophies. This imaging technique will probably increase in popularity in the near future for the assessment of various anterior segment diseases. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  18. Corneal Astigmatism Stability in Descemet Membrane Endothelial Keratoplasty for Fuchs Corneal Dystrophy.

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    Yokogawa, Hideaki; Sanchez, P James; Mayko, Zachary M; Straiko, Michael D; Terry, Mark A

    2016-07-01

    To calculate the magnitude and angle of the shift in corneal astigmatism associated with Descemet membrane endothelial keratoplasty (DMEK) surgery to determine the feasibility of concurrent astigmatism correction at the time of DMEK triple procedures. Retrospective study. Forty-seven eyes that previously underwent the DMEK procedure for Fuchs endothelial corneal dystrophy and that had more than 1.0 diopter (D) of front corneal astigmatism preoperatively were identified. All DMEK surgeries used a clear corneal temporal incision of 3.2 mm. Surgically induced astigmatism (SIA) was evaluated 6 months postsurgery with vector analysis using Scheimpflug image reading. We did not find a difference between pre- and postoperative magnitude of front astigmatism (P = 0.88; paired t test). The magnitude of the SIA front surface was 0.77 ± 0.63 D (range, 0.10-3.14 D). The centroid vector of the SIA front surface was 0.14 at 89.3°. A hyperopic corneal power shift was noted in both the front surface by 0.26 ± 0.74 D (range, 0.45-3.05 D) (P = 0.018; paired t test) and back surface by 0.56 ± 0.55 D (range, 0.25-2.40 D) (P astigmatism that is a with-the-rule shift associated with a temporal clear corneal incision. The stability of these data from preop to postop supports the plausibility of incorporating astigmatism correction with the cautious use of toric intraocular lenses for patients with Fuchs corneal dystrophy and cataract.

  19. Biomechanical properties of the cornea in Fuchs' corneal dystrophy.

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    del Buey, María A; Cristóbal, José A; Ascaso, Francisco J; Lavilla, Laura; Lanchares, Elena

    2009-07-01

    To investigate the effects of Fuchs' corneal dystrophy (FCD) on corneal biomechanical properties and the results of IOP readings in relation to changes in corneal hysteresis (CH) and central corneal thickness (CCT). Corneal biomechanical properties, including CH, corneal resistance factor (CRF), and CCT, were measured with the ocular response analyzer (ORA) in 11 eyes of 11 patients with clinically confirmed FCD and 12 eyes of 12 healthy subjects. The ORA was also used to determine the values of intraocular pressure (IOP(g)) and corneal compensated IOP (IOP(cc)). Goldmann applanation tonometry (GAT) was also measured. CH measured 10.3 +/- 1.6 mm Hg (range, 8.7-13.8) in normal eyes and 6.9 +/- 1.8 mm Hg (range, 4.6-11.7) in FCD eyes (P = 0.001). CRF in the normal and FCD eyes was 10.5 +/- 1.5 mm Hg (range, 8.5-13.3) and 8.1 +/- 1.9 (range, 4.5-11.2), respectively (P = 0.005). CCT was higher in FCD eyes (606 +/- 20 microm; range, 578-635) than in normal eyes (538.4 +/- 24.9 microm; range, 495-575; P = 0.0001). IOP(g) was 16.2 +/- 2.2 mm Hg (range, 13.5-18.7) in control eyes compared with 17.6 +/- 2.7 mm Hg (range, 12.8-18.6) in FCD eyes (P = 0.201). However, IOP(cc) in the FCD group (21.8 +/- 4.6 mm Hg; range, 12.8-29.0) was higher than in the control group (16.5 +/- 3.4 mm Hg; range, 11.9-23.9; P = 0.006). GAT in the normal and FCD eyes was 16.7 +/- 2.1 mm Hg (range, 12.8-18.6) and 16.9 +/- 2.3 mm Hg (range, 13.1-19.0), respectively (P = 0.205). FCD led to a change of corneal biomechanical properties. CH and CRF were significantly lower in FCD eyes than in normal eyes. IOP(cc) was significantly higher in FCD eyes than in control eyes. These values may be useful in addition to CCT when assessing corneal rigidity. Thus, FCD may cause an underestimation error in IOP measurement.

  20. Corneal Backscatters as an Objective Index for Assessing Fuchs’ Endothelial Corneal Dystrophy: A Pilot Study

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    Hsueh-Yen Chu

    2017-01-01

    Full Text Available Purpose. To provide an objective, quantitative approach for monitoring Fuchs’ endothelial corneal dystrophy (FECD, with Scheimpflug imaging. Design. This is a retrospective case-control pilot study. Methods. The study group consisted of 53 eyes in 27 patients diagnosed with FECD, with normal subjects paired as control. Main outcome measures were corneal thickness, morphological patterns on densitograms, and indices of corneal density including the average area density (mean AD and the average ratio of Descemet’s membrane density versus area density (DM/AD in Pentacam Scheimpflug images. Results. There were no significant differences in age and corneal thickness between FECD and normal groups. Morphologically, hanging-hammock patterns were noted on the densitograms of FECD patients, which were different from the high-back chair patterns in normal subjects. Quantitatively, mean AD and DM/AD were both elevated in FECD patients as compared with normal subjects (P=0.01 and 0.025, resp.. In addition, FECD patients with corneal edema had significantly higher mean AD (P=0.018 than those without corneal edema. Conclusions. This pilot study shows that Pentacam system provides an objective, quantitative way to approach FECD corneas. It can assist ophthalmologists in detecting the early change and in monitoring disease progression of FECD. Further studies are needed to consolidate the findings.

  1. Relationship between Corneal Guttae and Quality of Vision in Patients with Mild Fuchs' Endothelial Corneal Dystrophy.

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    Watanabe, Shinya; Oie, Yoshinori; Fujimoto, Hisataka; Soma, Takeshi; Koh, Shizuka; Tsujikawa, Motokazu; Maeda, Naoyuki; Nishida, Kohji

    2015-10-01

    To investigate the effect of the severity of corneal guttae on quality of vision (QOV) in patients with mild Fuchs' endothelial corneal dystrophy (FECD). Cross-sectional study. Twenty-three eyes of 14 patients with mild FECD without corneal edema on slit-lamp examination (5 pseudophakic eyes and 18 phakic eyes with mild lens opacity; grade 1.0-2.0 nuclear opalescence, grade 1.0-2.0 nuclear color, grade 1.0 cortical cataract, and grade 1.0 posterior subcapsular cataract on the Lens Opacities Classification System, version III). The area ratio of the corneal guttae (ARCG) in the endothelial cells was measured by multifocal specular microscopy. The QOV parameters, that is, corrected distance visual acuity (CDVA), letter contrast sensitivity (LCS), and intraocular straylight, also were measured. The correlations were assessed between the ARCG and QOV parameters and between the straylight and CDVA and LCS. The ARCG, logarithm of the minimum angle of resolution CDVA, LCS, and straylight. Univariate analysis showed that the ARCG was correlated significantly with the CDVA, LCS, and straylight (R(2) = 0.41, P = 0.001; R(2) = 0.55, P = 0.001; and R(2) = 0.39, P = 0.002, respectively). Univariate analysis also showed that straylight was correlated significantly with the CDVA and LCS (R(2) = 0.47, P = 0.001 and R(2) = 0.41, P = 0.001, respectively). Corneal guttae without edema caused the QOV to deteriorate in eyes with FECD. Patients with higher straylight had worse CDVA or LCS. Intraocular forward light scatter caused by corneal guttae may result in visual disturbances. Quantification of corneal guttae can be useful to evaluate the effect of guttae on the QOV and determine the surgical indications of endothelial keratoplasty for eyes with mild FECD. Copyright © 2015 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

  2. Disease: H00959 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00959 Schnyder corneal dystrophy (SCD); Schnyder crystalline corneal dystrophy (SC...CD); Crystalline stromal dystrophy; Schnyder crystalline dystrophy sine crystals Schnyder corneal dystrophy (SCD) is a corneal... dystrophy that is characterized by deposition of cholesterol in the corneal stroma. SCD u...sually becomes apparent early in life with corneal clouding or with crystals within the corneal... stroma. Over time, an initially unremarkable corneal stroma acquires small white opacities an

  3. Deep anterior lamellar keratoplasty for the treatment of stromal corneal dystrophies.

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    Unal, Mustafa; Arslan, Osman S; Atalay, Eray; Mangan, Mehmet S; Bilgin, Ahmet B

    2013-03-01

    To report the perioperative complications and clinical outcomes after deep anterior lamellar keratoplasty (DALK) using the big bubble technique in eyes with stromal corneal dystrophies. Seventy-four eyes of 65 patients who underwent DALK for stromal corneal dystrophies were evaluated in this retrospective interventional case series study. Main outcome measures were intraoperative and postoperative complications, postoperative uncorrected visual acuity, best spectacle-corrected visual acuity, spherical equivalent refraction, and topographic astigmatism. There were 44 eyes with macular corneal dystrophy, 18 eyes with lattice dystrophy, and 12 eyes with granular dystrophy. DALK was completed in 69 cases (94.6%). Descemet membrane microperforations occurred in 6 eyes (8.7%). The mean follow-up period was 43.5 ± 23.9 months, ranging from 12 to 96 months. Postoperative best spectacle-corrected visual acuity of 0.5 or better was present in 52 of 69 eyes (75.4%). There were 3 episodes of stromal graft rejection, which responded to topical therapy. Lattice dystrophy recurred in 6 eyes (35.3%). DALK using the big bubble technique is an effective procedure in the treatment of patients with corneal stromal dystrophies. Recurrence of lattice dystrophy was relatively high.

  4. Extracellular matrix alterations in late-onset Fuchs' corneal dystrophy.

    Science.gov (United States)

    Weller, Julia M; Zenkel, Matthias; Schlötzer-Schrehardt, Ursula; Bachmann, Bjoern O; Tourtas, Theofilos; Kruse, Friedrich E

    2014-05-15

    To characterize the alterations of extracellular matrix proteins in Descemet's membranes (DM) of patients with late-onset Fuchs' corneal dystrophy (FCD) and to differentiate them from nonspecific alterations in pseudophakic bullous keratopathy (PBK). Human DM-endothelial cell complexes were obtained from patients with late-onset FCD (n = 40), PBK (n = 6), and control eyes (n = 5). Gene expression profiles of endothelial cells were compared using a commercial real-time PCR array and quantitative real-time PCR assays for confirmation of differentially expressed genes. A total of 24 extracellular matrix proteins were also localized in cryosections of corneal specimens from FCD (n = 10), PBK (n = 4), and control eyes (n = 5) by immunohistochemistry. Polymerase chain reaction array analysis revealed a significant upregulation of 27 out of 84 extracellular matrix-related genes including collagens, proteoglycans, glycoproteins, cell adhesion molecules, and matrix metalloproteinases in FCD specimens as compared to normal controls, which could be partly confirmed and quantified by real-time PCR. Comparative analysis of FCD and PBK specimens showed a significant and consistent FCD-specific upregulation of collagen types I, III, and XVI; fibronectin; agrin; clusterin; transforming growth factor beta-induced (TGFBI); and integrin α4 (3- to 18-fold, P matrix alterations in the pathophysiology of FCD. Copyright 2014 The Association for Research in Vision and Ophthalmology, Inc.

  5. Bilateral Atypical Granular Corneal Dystrophy Associated with Unilateral Keratoconus in a Male Child.

    Science.gov (United States)

    Dangra, Kavita Lohiya; Das, Manoranjan; Periasamy, Sundersan; Prajna, N Venkatesh

    2016-01-01

    A 14-year-old male presented with decreased vision. Slit lamp examination indicated multiple anterior corneal stromal opacities with clear intervening spaces accompanied with superficial subepithelial lines arranged in a quasi-whorl-like fashion bilateral with greater prominence in the right eye. Corneal steepening associated with thinning was noted only in the right eye. Genetic analysis confirmed a mutation suggestive of granular corneal dystrophy. Here, we describe a rare case of an atypical granular dystrophy associated with unilateral keratoconus in a male child.

  6. Intraocular pressure and corneal biomechanics in Fuchs' endothelial dystrophy and after posterior lamellar keratoplasty.

    Science.gov (United States)

    Clemmensen, Kåre; Hjortdal, Jesper

    2014-06-01

      To evaluate the precision of techniques for measuring intraocular pressure (IOP) in corneas with presumably altered biomechanical properties.   Intraocular pressure was measured with a Goldmann applanation tonometer (GAT), ocular response analyzer (ORA) and dynamic contour tonometer (DCT) in 70 eyes. Thirty-five eyes were normal corneas, 18 eyes had Fuchs' endothelial dystrophy, and 17 eyes had undergone Descemet's stripping automated endothelial keratoplasty (DSAEK) surgery. Corneal hysteresis (CH), corneal resistance factor (CRF) as well as central corneal thickness (CCT) were recorded with the ORA.   The measured cornea-corrected IOP using ORA was significantly higher than GAT in all three groups (pkeratoplasty. GAT and DCT seem to measure IOP correctly in patients with Fuchs' endothelial dystrophy as well as after posterior lamellar keratoplasty. Corneal-corrected IOP as measured with the ORA appears to overestimate IOP in patients with Fuchs' endothelial dystrophy as well as after posterior lamellar keratoplasty. © 2013 Acta Ophthalmologica Scandinavica Foundation. Published by Blackwell Publishing Ltd.

  7. Optical coherence tomography image in gelatinous drop-like corneal dystrophy: case report

    OpenAIRE

    Magalhães,Otávio de Azevedo; Rymer,Samuel; Marinho,Diane Ruschel; Kwitko,Sérgio; Cardoso,Isabel Habeyche; Kliemann,Lúcia

    2012-01-01

    Gelatinous drop-like corneal dystrophy is a rare disorder with few cases described in the present literature. The following report will show how difficult it is to diagnose this disease in early stages. Modern image exams, such as optical coherence tomography helps to diagnose and can be crucial to establish the best treatment. We will present the histopathological changes and clinical features in this unusual dystrophy.

  8. Spontaneous corneal dystrophy and generalized basement membrane changes in Fischer-344 rats.

    Science.gov (United States)

    Bruner, R H; Keller, W F; Stitzel, K A; Sauers, L J; Reer, P J; Long, P H; Bruce, R D; Alden, C L

    1992-01-01

    Groups of young, sexually mature Fischer-344 rats (n = 25/sex) obtained from commercial breeders were examined ophthalmologically and histopathologically to determine the prevalence and severity of corneal basement membrane lesions (corneal dystrophy) and basement membrane changes in select nonocular tissues. Results disclosed a high incidence of corneal basement membrane dystrophy in rats of both sexes from all breeders; however, severity levels were significantly increased in rats obtained from one breeder when compared to others. Furthermore, rats that displayed the most advanced corneal lesions also exhibited more severe basement membrane changes in other organs, especially renal tubules and vascular internal laminae. These findings suggest that both ocular and nonocular dystrophic changes may have been linked through common physiologic (or genetic) mechanisms. Animals that displayed basement membrane lesions were not considered to represent compromised biologic test systems.

  9. Keratoconus associated with corneal macular dystrophy: in vivo confocal microscopic evaluation.

    Science.gov (United States)

    Balestrazzi, A; Martone, G; Traversi, C; Haka, G; Toti, P; Caporossi, A

    2006-01-01

    The authors present a case, studied through in vivo confocal microscopy, of concomitant keratoconus and macular corneal dystrophy (MCD). A 29-year-old man underwent a penetrating keratoplasty in the right eye in May 2005. Confocal microscopy was performed to examine the cornea of the right eye. A diagnosis of concomitant keratoconus and MCD was suspected, due to the simultaneous findings of corneal ectasia and stromal opacities. In this case, using in vivo confocal microscopy, morphologic changes were detected in many corneal layers and compared with the histopathologic findings. The morphologic alterations were found mainly in the area of the cornea apex.

  10. Hearing disability in patients with Fuchs' endothelial corneal dystrophy: unrecognized co-pathology?

    NARCIS (Netherlands)

    Stehouwer, Marilette; Bijlsma, Ward R.; van der Lelij, Allegonda

    2011-01-01

    To investigate a possible association between Fuchs' endothelial corneal dystrophy (FECD) and hearing disability. A cross-sectional observational study was performed at the University Medical Center Utrecht. Cases and controls were patients who were treated by a cornea specialist between 2004 and

  11. Outcomes of Penetrating Keratoplasty for Macular Corneal Dystrophy

    Directory of Open Access Journals (Sweden)

    Farid Karimian

    2009-01-01

    Full Text Available

    PURPOSE: To report the outcomes of penetrating keratoplasty (PKP in patients with macular corneal dystrophy (MCD. METHODS: This retrospective case series includes consecutive patients with MCD who underwent PKP from 1986 to 2006 with at least 6 months' follow-up. Main outcome measures included best spectacle-corrected visual acuity (BSCVA, postoperative astigmatism and graft survival. RESULTS: Sixty-two eyes of 39 patients with mean age of 34.0±10.5 (range 13-58 years at the time of keratoplasty were included for analysis. After a mean follow-up period of 52.0±47.3 (range 6-190 months, BSCVA improved from 1.4±0.4 logMAR (4/100 preoperatively to 0.2±0.3 logMAR (20/32 at final follow-up (P < 0.001. Mean postoperative BSCVA was 0.15±0.40 logMAR in patients (36 eyes aged less than 35 years at the time of surgery as compared to 0.26±0.25 logMAR in subjects (26 eyes older than 35 years (P=0.005. Final astigmatism was comparable with different suturing techniques including separate, continuous, and combined sutures (P=0.9. All grafts were clear at final follow-up except a single case of MCD with visually insignificant recurrence. Episodes of immunologic graft rejection occurred in 12 eyes (19.4% but none led to graft failure. CONCLUSION: PKP for MCD entails favorable outcomes in terms of graft survival and visual improvement

  12. Genotype-phenotype correlation of TGFBI corneal dystrophies in Polish patients.

    Science.gov (United States)

    Nowińska, Anna K; Wylegala, Edward; Janiszewska, Dominika A; Dobrowolski, Dariusz; Aragona, Pasquale; Roszkowska, Anna M; Puzzolo, Domenico

    2011-01-01

    To analyze genotype-phenotype correlation in patients originating from Polish population with the transforming growth factor beta induced (TGFBI) corneal dystrophies. Sixty affected and 31 unaffected individuals from 15 unrelated Polish families were included in the study. The clinical diagnosis was based on the slit-lamp exam, 1310 nm time domain and 1310 nm swept source spectral domain optical coherence tomography (OCT). Histopathologic analysis was performed on 10 available corneal buttons. Exons of the TGFBI gene were screened for mutations with polymerase chain reaction (PCR) and direct DNA sequencing. We found the lattice phenotype dominant compared to the granular one in the Polish population (41:16 patients; lattice:granular). We identified five distinct mutations responsible for TGFBI corneal dystrophies (R124R, R124H, R555W, R555Q, and H626R). There was a strong genotype-phenotype correlation in the case of R124R and R555W mutations, while there was a distinct phenotypic heterogeneity in the case of the H626R mutation. OCT analysis revealed that the reflectivity, location and pattern of the corneal deposits were different among the TGFBI corneal dystrophies. The advantage of spectral swept source OCT over time-domain OCT scans is a more distinct visualization of the Bowman's layer area and deposits located under the epithelium. This study underlines the role of comprehensive phenotype-genotype analysis in TGFBI corneal dystrophies, describes for the first time the TGFBI mutation spectrum in a Polish population and reveals phenotypic heterogeneity in the case of the H626R mutation.

  13. Simultaneous Transplantation of Limbal Stem Cells May Reduce Recurrences of Granular Dystrophy After Corneal Transplantation

    Science.gov (United States)

    Lang, Stefan J.; Eberwein, Philipp; Reinshagen, Helga; Reinhard, Thomas; Sundmacher, Rainer

    2015-01-01

    Abstract To present 2 cases with long-term relapse-free intervals only after limbo-keratoplasty but not after conventional penetrating keratoplasty in granular dystrophy. Retrospective review of the patient charts and photographs taken during long-term follow-up of 2 cases with granular dystrophy, in which 1 eye received penetrating keratoplasty and the fellow eye received penetrating limbo-keratoplasty. In the first patient, 1 eye showed extensive recurrence of granular deposits 17 years after penetrating keratoplasty was performed while in the second eye two-thirds of the corneal transplant adjacent to the transplanted limbal area remained clear 12 years after the limbo-corneal transplant. In the second patient, 1 eye showed no signs of recurrence 5 years after limbo-keratoplasty, whereas a recurrence of granular corneal deposits occurred 18 months after surgery in the fellow eye. These cases show that the simultaneous transplantation of healthy donor limbus when performing penetrating keratoplasty may prolong recurrence in granular corneal dystrophy. Although we were unable to prove it on the molecular level, these clinical courses may support the hypothesis that a limbal transplant helps prevent a recurrence. PMID:25997048

  14. Mutational spectrum of the ZEB1 gene in corneal dystrophies supports a genotype-phenotype correlation.

    Science.gov (United States)

    Lechner, Judith; Dash, Durga P; Muszynska, Dorota; Hosseini, Mohsen; Segev, Fani; George, Sonia; Frazer, David G; Moore, Jonathan E; Kaye, Stephen B; Young, Terri; Simpson, David A; Churchill, Amanda J; Héon, Elise; Willoughby, Colin E

    2013-05-03

    Mutations in ZEB1 have been reported in posterior polymorphous corneal dystrophy (PPCD3; MIM #609141) and Fuchs' endothelial corneal dystrophy (FECD6; MIM #613270). Although PPCD and keratoconus are clinically and pathologically distinct, PPCD has been associated with keratoconus, suggesting a common genetic basis. The purpose of our study was to perform mutational screening of the ZEB1 gene in patients affected with keratoconus or PPCD. Sanger sequencing of ZEB1 was performed in 70 unrelated patients with keratoconus and 18 unrelated patients with PPCD. Real-time quantitative PCR (RT-qPCR) was performed on RNA from cultured corneal keratocytes obtained from a keratoconic patient harboring a missense ZEB1 mutation (p.Gln640His) undergoing corneal transplantation. Mutational analysis of ZEB1 in PPCD identified a previously reported frameshift mutation (C.1578_1579INSG) and a novel nonsense mutation (C.2249C A) in exon 7 of ZEB1 causing the insertion of a stop codon: p.Ser750X. In the keratoconus cohort, a novel heterozygous pathogenic mutation in exon 7 (c.1920G > T; p.Gln640His) of ZEB1 was identified in a family affected with keratoconus and Fuchs' endothelial corneal dystrophy. RT-qPCR performed on cultured corneal keratocytes harboring the missense ZEB1 mutation (p.Gln640His) demonstrated that COL4A1 and COL4A2 were markedly downregulated, and COL4A3, COL4A4, and COL8A2 were moderately downregulated. Our data combined with the previously reported mutational spectrum of ZEB1 support a genotypephenotype correlation: missense substitutions in the ZEB1 protein are associated with FECD6 and keratoconus, whereas protein truncating ZEB1 mutations result in PPCD3. The dysregulation of α-type IV collagens represents a common link between ZEB1 mutation and the clinical phenotypes (PPCD3, FECD, and keratoconus).

  15. The TGFBI A546D mutation causes an atypical type of lattice corneal dystrophy.

    Science.gov (United States)

    Correa-Gomez, Vicente; Villalvazo-Cordero, Leonardo; Zenteno, Juan Carlos

    2007-09-17

    To report the clinical, molecular, and histopathological features of a distinct transforming growth factor-beta-induced (TGFBI) gene-linked amyloidotic corneal dystrophy exhibiting an unusual lattice pattern. A complete ophthalmologic examination was performed in 10 individuals of a Mexican family in which autosomal dominant transmission of the disease was observed. DNA was obtained from peripheral blood leukocytes of each participating subject. Genetic analyses included TGFBI polymerase chain reaction (PCR) amplification and automated nucleotidic sequencing of exons 4, 11, 12, 13, and 14 from genomic DNA. Histological analysis of corneal tissue from an affected individual who underwent a penetrating keratoplasty was also performed. The corneal phenotype in this pedigree was characterized by multiple bilateral round opacities in the central part of the cornea combined with a conspicuous central and peripheral lattice pattern. TGFBI analysis revealed a heterozygous point mutation at exon 12 (1637 C>A) in all affected individuals, predicting an A546D missense change. The lattice phenotype resulting from the TGFBI A546D mutation in this family is distinct from that observed in a previously described pedigree carrying the A546D mutation and exhibiting a phenotype designated "polymorphic corneal amyloidosis". We propose this particular disorder to be classified as an atypical type of lattice stromal corneal dystrophy.

  16. Proteomics of Fuchs' Endothelial Corneal Dystrophy Support That the Extracellular Matrix of Descemet's Membrane is Disordered

    DEFF Research Database (Denmark)

    Poulsen, Ebbe Toftgaard; Dyrlund, Thomas Franck; Runager, Kasper

    2014-01-01

    Fuchs' endothelial corneal dystrophy (FECD) is a major corneal disorder affecting the innermost part of the cornea, leading to visual impairment. As the morphological changes in FECD are mainly observed in the extracellular matrix of the Descemet's membrane/endothelial layer we determined...... that the morphological changes observed in FECD is caused in part by an aberrant assembly of the extracellular matrix within the Descemet's membrane/endothelial layer....... differentially regulated proteins, many of which are extracellular proteins known to be involved in proper assembly of the basement membrane in other tissues. In total 26 differentially regulated proteins were identified, of which 6 proteins were regulated by both methods. These results support...

  17. Structural and Functional Implications of Human Transforming Growth Factor β-Induced Protein, TGFBIp, in Corneal Dystrophies

    DEFF Research Database (Denmark)

    García-Castellanos, Raquel; Nielsen, Nadia Sukusu; Runager, Kasper

    2017-01-01

    A major cause of visual impairment, corneal dystrophies result from accumulation of protein deposits in the cornea. One of the proteins involved is transforming growth factor β-induced protein (TGFBIp), an extracellular matrix component that interacts with integrins but also produces corneal depo...

  18. Intraocular pressure and corneal biomechanical properties in patients with myotonic dystrophy.

    Science.gov (United States)

    Rosa, Nicola; Lanza, Michele; Borrelli, Maria; Palladino, Alberto; Di Gregorio, M Grazia; Politano, Luisa

    2009-02-01

    To compare intraocular pressure (IOP) between patients with myotonic dystrophy (DM1) and normal subjects, taking into account corneal characteristics. To determine whether lower IOP measurements in patients with DM1 are due to thinner corneas. Comparative case series. Fifty-three eyes of patients with DM1 and 53 eyes of normal age- and sex-matched subjects. Corneal biomechanical properties and corneal compensated intraocular pressure (IOPcc) measured with the Ocular Response Analyzer (Reichert Inc., Depew, NY), central corneal thickness measured with the Oculus Pentacam (Oculus, Wetzlar, Germany), and IOP were evaluated in patients with DM1 and compared with age- and sex-matched healthy subjects. Goldmann applanation tonometry, central corneal thickness, corneal hysteresis (CH), corneal resistance factor (CRF), and IOPcc. Compared with the healthy subjects, patients with DM1 showed lower IOP (12.4+/-3.6 mm Hg vs. 14.9+/-3.4 mmHg) (Pcornea (575.9+/-35.02 mum vs. 556.3+/-33.2 microm) (Pbiomechanical properties. The author(s) have no proprietary or commercial interest in any materials discussed in this article.

  19. Hearing disability in patients with Fuchs’ endothelial corneal dystrophy: unrecognized co-pathology?

    OpenAIRE

    Stehouwer M; Bijlsma WR; Van der Lelij A

    2011-01-01

    Marilette Stehouwer, Ward R Bijlsma, Allegonda Van der LelijDepartment of Ophthalmology, University Medical Center Utrecht, Utrecht, The NetherlandsPurpose: To investigate a possible association between Fuchs’ endothelial corneal dystrophy (FECD) and hearing disability.Methods: A cross-sectional observational study was performed at the University Medical Center Utrecht. Cases and controls were patients who were treated by a cornea specialist between 2004 and 2008. FECD patients had ...

  20. Differing roles for TCF4 and COL8A2 in central corneal thickness and fuchs endothelial corneal dystrophy.

    Directory of Open Access Journals (Sweden)

    Robert P Igo

    Full Text Available Fuchs endothelial corneal dystrophy (FECD is the most common late-onset, vision-threatening corneal dystrophy in the United States, affecting about 4% of the population. Advanced FECD involves a thickening of the cornea from stromal edema and changes in Descemet membrane. To understand the relationship between FECD and central corneal thickness (CCT, we characterized common genetic variation in COL8A2 and TCF4, genes previously implicated in CCT and/or FECD. Other genes previously associated with FECD (PITX2, ZEB1, SLC4A11, and genes only known to affect CCT (COL5A1, FOXO1, AVGR8, ZNF469 were also interrogated. FECD probands, relatives and controls were recruited from 32 clinical sites; a total of 532 cases and 204 controls were genotyped and tested for association of FECD case/control status, a 7-step FECD severity scale and CCT, adjusting for age and sex. Association of FECD grade with TCF4 was highly significant (OR= 6.01 at rs613872; p = 4.8×10(-25, and remained significant when adjusted for changes in CCT (OR= 4.84; p = 2.2×10(-16. Association of CCT with TCF4 was also significant (p = 6.1×10(-7, but was abolished with adjustment for FECD grade (p = 0.92. After adjusting for FECD grade, markers in other genes examined were modestly associated (p ∼ 0.001 with FECD and/or CCT. Thus, common variants in TCF4 appear to influence FECD directly, and CCT secondarily via FECD. Additionally, changes in corneal thickness due to the effect of other loci may modify disease severity, age-at-onset, or other biomechanical characteristics.

  1. Descemet membrane endothelial keratoplasty with a stromal rim in the treatment of posterior polymorphous corneal dystrophy

    Directory of Open Access Journals (Sweden)

    Pavel Studeny

    2012-01-01

    Full Text Available A 20-year-old patient, diagnosed with posterior polymorphous corneal dystrophy, developed corneal edema for which he underwent Descemet membrane endothelial keratoplasty with a stromal rim (DMEK-S in the right eye. No intra- or postoperative complications were noted. At the last follow-up 2 years and 9 months after the procedure, the best corrected visual acuity was 1.0 and endothelial cell density declined from 3533 cells/mm 2 to 1012 cells/mm 2 . Despite the endothelial cell loss, DMEK-S appears to be a good alternative to other surgical techniques for the treatment of corneal endotheliopathies, and it may be of benefit to young patients.

  2. Granular and lattice deposits in corneal dystrophy caused by R124C mutation of TGFBIp.

    Science.gov (United States)

    Patel, Dhara A; Chang, Shu-Hong; Harocopos, George J; Vora, Smita C; Thang, Diep Huu; Huang, Andrew J W

    2010-11-01

    Both granular and lattice deposits are present in Avellino corneal dystrophy (ACD), primarily associated with the R124H mutation of transforming growth factor-β-induced (TGFBIp). We investigated the presence of these deposits in other TGFBI mutations and the use of Thioflavin-T (ThT), a fluorescent amyloid stain for characterizing corneal amyloid deposits. Surgical corneal specimens of 3 unrelated patients clinically diagnosed with ACD were studied. Corneal sections from normal individuals and patients with prior lattice corneal dystrophy (LCD) were used as controls. Histochemical studies were performed with Congo red and Masson trichrome stains, and fluorescent imaging with scanning laser confocal microscopy was performed for ThT and anti-TGFBIp antibody staining. Clinical and histopathological findings supported the diagnoses of ACD in these 3 cases in whom granular deposits stained with Masson trichrome and lattice deposits stained with ThT and Congo red showed birefringence and dichroism as expected. However, genotyping revealed a heterozygous R124C mutation in each case. In addition to classical stromal deposits, unique subepithelial TGFBIp aggregates, which stain with neither ThT nor trichrome, were observed. In control LCD sections, stromal deposits were stained with ThT but not with trichrome, confirming lack of granular deposits. Our results demonstrate that both granular and lattice corneal deposits can be associated with R124C mutation in addition to the more common R124H mutation. An additional feature of nonhyaline, nonamyloid, TGFBIp subepithelial deposits might substantiate the categorization of such cases as a variant form of ACD. This study further validates ThT staining for detection of amyloid TGFBIp deposits.

  3. Macular corneal dystrophy: mutational spectrum in German patients, novel mutations and therapeutic options.

    Science.gov (United States)

    Gruenauer-Kloevekorn, Claudia; Braeutigam, Saskia; Heinritz, Wolfram; Froster, Ursula G; Duncker, Gernot I W

    2008-10-01

    The objective of this study was to investigate genotype-phenotype correlations, the consequences for surgical treatment, and the therapeutical options in patients with macular corneal dystrophy (MCD). We investigated MCD genotype by using polymerase chain reaction followed by direct sequencing in one family and four patients with MCD. Results were confirmed by restriction analysis. Clinical phenotypes, histopathological findings, and therapeutical proceedings of each patient were reported and compared with the molecular genetic results. Five mutations, four missense mutations, and one frameshift mutation, from which three were novel, and one single-nucleotide polymorphism, were identified within the coding region of the CHST6 gene. In three patients, two with a homozygous mutation within the start codon (Met1Leu) and one with a heterozygous mutation (Leu200Arg) and a polymorphism (Arg162Gly), with irregular corneal surface and recurrent erosions a phototherapeutic keratectomy lead to a transient success. An additional fitting of rigid gas permeable contact lenses in one patient could further improve irregular astigmatism. In two patients, one with a frameshift mutation (1734_1735delTG; Arg211Gln) and one with two compound heterozygous mutations (Leu200Arg; Leu173Phe) and an additional polymorphism (Arg162Gly) a penetrating keratoplasty improved BCVA without any recurrence of the opacities within the follow-up time. Different genotypes imply several phenotypes, which influence therapeutical proceedings in MCD patients. Our study shows the wide range of diagnostic findings and therapeutical options in patients suffering from macular corneal dystrophy depending on the genotype.

  4. Fuchs endothelial corneal dystrophy: clinical characteristics of surgical and nonsurgical patients

    Directory of Open Access Journals (Sweden)

    Goldberg RA

    2014-09-01

    Full Text Available Roger A Goldberg,1,2 Sabri Raza,1 Eric Walford,1 William J Feuer,1 Jeffrey L Goldberg1,3 1Bascom Palmer Eye Institute, University of Miami, Miami, FL, USA; 2Tufts-New England Eye Center/Ophthalmic Consultants of Boston, Boston, MA, USA; 3Shiley Eye Center, University of California San Diego, San Diego, CA, USA Purpose: To review the patient and clinical characteristics of patients with Fuchs endothelial corneal dystrophy (FECD. Methods: Review of records for every patient who presented to the Bascom Palmer Eye Institute between 2003 and 2009 whose visit was coded for endothelial corneal dystrophy (International Classification of Diseases, Ninth Revision [ICD9] 371.57, bullous keratopathy (ICD9 371.23, or who underwent a corneal surgery with or without cataract extraction. Demographic, clinical, and ancillary testing data were collected from the time of presentation, diagnosis, and follow-up, and the use, timing, and type of surgical interventions was documented, with 6-month and final visual acuities recorded. Results: A total of 2,370 charts were included in this study, of which 966 patients had a diagnosis of FECD. Of these, 197 patients (21% received a corneal transplantation procedure. The surgery most often performed was penetrating keratoplasty with or without cataract extraction (66%, followed by endothelial keratoplasty with or without cataract extraction (34%. The risk factors for surgery include worse visual acuity at presentation (20/60 Snellen visual acuity in surgical patients versus 20/40 Snellen visual acuity in nonsurgical patients, P<0.001, greater average central corneal thickness (635 µm versus 592 µm, P<0.001, loss of visual acuity over time (two lines lost versus zero lines lost, P<0.001, increasing age (P<0.001, and male sex (P=0.008. Over half of patients (52% did not receive surgery despite poor vision. Conclusion: During this time period, FECD did not have a consistent pattern for management or treatment, and despite

  5. Keratolimbal autograft transplantation as a possible new treatment of Lisch epithelial corneal dystrophy.

    Science.gov (United States)

    Celis Sánchez, J; Mesa Varona, D V; Avendaño Cantos, E; López-Romero Moraleda, S; Cebrian Rosado, E; González Del Valle, F

    2016-07-01

    The case concerns 64-year-old woman with visual acuity of 20/40 in the right eye. Slit-lamp examination revealed a grey, feathery corneal opacification with intraepithelial microcysts compatible with Lisch epithelial corneal dystrophy (LECD). It was treated with epithelial debridements, contact lenses and mitomycin C, but the opacification recurred within months. The removal of limbus sector and autologous limbal transplantation (KLAT) were used successfully without recurrence. After removal of damaged limbus, KLAT should be considered as a treatment option for asymmetric LECD when other treatments have failed. Copyright © 2016 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  6. TGFBI, CHST6, and GSN gene analysis in Mexican patients with stromal corneal dystrophies.

    Science.gov (United States)

    Gonzalez-Rodriguez, Johanna; Ramirez-Miranda, Arturo; Hernandez-Da Mota, Sergio E; Zenteno, Juan C

    2014-08-01

    The purpose of our study was to describe the results of molecular screening of TGFBI, CHST6, and GSN genes in a group of Mexican patients with different stromal corneal dystrophies (CD). A total of 16 CD Mexican patients pertaining to nine different pedigrees were subjected to a complete ophthalmological investigation. A clinical diagnosis of lattice CD was performed in 10 patients from five pedigrees. Three patients from two pedigrees were diagnosed with granular CD type 2, two patients with unrelated probands had Finnish-type corneal amyloidosis, and one patient had macular CD. Genetic analysis included DNA isolation from blood leukocytes and polymerase chain reaction (PCR) amplification and direct nucleotide sequencing of TGFBI, CHST6, and GSN genes. Seven lattice CD patients from four unrelated families had an identical p.H626R mutation in TGFBI, three patients from a single lattice CD family carried a p.R124C substitution in TGFBI, and a granular type 2 CD pedigree was demonstrated to carry a heterozygous TGFBI p.M619K substitution. A patient having Finnish-type corneal amyloidosis had a p.D187N mutation in GSN. Finally, molecular analysis of CHST6 in a patient with macular CD disclosed the presence of a homozygous p.Y110C change. This study improves the knowledge of the genetic features of Mexican patients with corneal stromal dystrophies by identifying mutations in the TGFBI, CHST6, and GSN genes. Genetic screening of larger samples of patients from distinct ethnic groups would be of great importance for a better understanding of the mutational spectrum of stromal CD.

  7. Role of Decorin Core Protein in Collagen Organisation in Congenital Stromal Corneal Dystrophy (CSCD)

    Science.gov (United States)

    Kamma-Lorger, Christina S.; Pinali, Christian; Martínez, Juan Carlos; Harris, Jon; Young, Robert D.; Bredrup, Cecilie; Crosas, Eva; Malfois, Marc; Rødahl, Eyvind

    2016-01-01

    The role of Decorin in organising the extracellular matrix was examined in normal human corneas and in corneas from patients with Congenital Stromal Corneal Dystrophy (CSCD). In CSCD, corneal clouding occurs due to a truncating mutation (c.967delT) in the decorin (DCN) gene. Normal human Decorin protein and the truncated one were reconstructed in silico using homology modelling techniques to explore structural changes in the diseased protein. Corneal CSCD specimens were also examined using 3-D electron tomography and Small Angle X-ray diffraction (SAXS), to image the collagen-proteoglycan arrangement and to quantify fibrillar diameters, respectively. Homology modelling showed that truncated Decorin had a different spatial geometry to the normal one, with the truncation removing a major part of the site that interacts with collagen, compromising its ability to bind effectively. Electron tomography showed regions of abnormal stroma, where collagen fibrils came together to form thicker fibrillar structures, showing that Decorin plays a key role in the maintenance of the order in the normal corneal extracellular matrix. Average diameter of individual fibrils throughout the thickness of the cornea however remained normal. PMID:26828927

  8. Role of Decorin Core Protein in Collagen Organisation in Congenital Stromal Corneal Dystrophy (CSCD.

    Directory of Open Access Journals (Sweden)

    Christina S Kamma-Lorger

    Full Text Available The role of Decorin in organising the extracellular matrix was examined in normal human corneas and in corneas from patients with Congenital Stromal Corneal Dystrophy (CSCD. In CSCD, corneal clouding occurs due to a truncating mutation (c.967delT in the decorin (DCN gene. Normal human Decorin protein and the truncated one were reconstructed in silico using homology modelling techniques to explore structural changes in the diseased protein. Corneal CSCD specimens were also examined using 3-D electron tomography and Small Angle X-ray diffraction (SAXS, to image the collagen-proteoglycan arrangement and to quantify fibrillar diameters, respectively. Homology modelling showed that truncated Decorin had a different spatial geometry to the normal one, with the truncation removing a major part of the site that interacts with collagen, compromising its ability to bind effectively. Electron tomography showed regions of abnormal stroma, where collagen fibrils came together to form thicker fibrillar structures, showing that Decorin plays a key role in the maintenance of the order in the normal corneal extracellular matrix. Average diameter of individual fibrils throughout the thickness of the cornea however remained normal.

  9. A multicenter study to map genes for Fuchs endothelial corneal dystrophy: baseline characteristics and heritability.

    Science.gov (United States)

    Louttit, Megan D; Kopplin, Laura J; Igo, Robert P; Fondran, Jeremy R; Tagliaferri, Angela; Bardenstein, David; Aldave, Anthony J; Croasdale, Christopher R; Price, Marianne O; Rosenwasser, George O; Lass, Jonathan H; Iyengar, Sudha K

    2012-01-01

    To describe the methods for family and case-control recruitment for a multicenter genetic and associated heritability analyses of Fuchs endothelial corneal dystrophy (FECD). Twenty-nine enrolling sites with 62 trained investigators and coordinators gathered individual and family information, graded the phenotype, and collected blood and/or saliva for genetic analysis on all individuals with and without FECD. The degree of FECD was assessed in a 0 to 6 semiquantitative scale using standardized clinical methods with pathological verification of FECD on at least 1 member of each family. Central corneal thickness was measured by ultrasonic pachymetry. Three hundred twenty-two families with 330 affected sibling pairs with FECD were enrolled and included a total of 650 sibling pairs of all disease grades. Using the entire 7-step FECD grading scale or a dichotomous definition of severe disease, heritability was assessed in families via sib-sib correlations. Both binary indicators of severe disease and semiquantitative measures of disease severity were significantly heritable, with heritability estimates of 30% for severe disease, 37% to 39% for FECD score, and 47% for central corneal thickness. Genetic risk factors have a strong role in the severity of the FECD phenotype and corneal thickness. Genotyping this cohort with high-density genetic markers followed by appropriate statistical analyses should lead to novel loci for disease susceptibility.

  10. Polymorphism of the Flap Endonuclease 1 Gene in Keratoconus and Fuchs Endothelial Corneal Dystrophy

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    Katarzyna A. Wojcik

    2014-08-01

    Full Text Available Oxidative stress is implicated in the pathogenesis of many diseases, including serious ocular diseases, keratoconus (KC and Fuchs endothelial corneal dystrophy (FECD. Flap endonuclease 1 (FEN1 plays an important role in the repair of oxidative DNA damage in the base excision repair pathway. We determined the association between two single nucleotide polymorphisms (SNPs, c.–441G>A (rs174538 and g.61564299G>T (rs4246215, in the FEN1 gene and the occurrence of KC and FECD. This study involved 279 patients with KC, 225 patients with FECD and 322 control individuals. Polymerase chain reaction (PCR and length polymorphism restriction fragment analysis (RFLP were applied. The T/T genotype of the g.61564299G>T polymorphism was associated with an increased occurrence of KC and FECD. There was no association between the c.–441G>A polymorphism and either disease. However, the GG haplotype of both polymorphisms was observed more frequently and the GT haplotype less frequently in the KC group than the control. The AG haplotype was associated with increased FECD occurrence. Our findings suggest that the g.61564299G>T and c.–441G>A polymorphisms in the FEN1 gene may modulate the risk of keratoconus and Fuchs endothelial corneal dystrophy.

  11. Corneal stromal dystrophies: a clinical pathologic study Distrofia corneana estromal: um estudo clínicopatológico

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    Elvira Barbosa Abreu

    2012-12-01

    Full Text Available INTRODUCTION: Corneal dystrophy is defined as bilateral and symmetric primary corneal disease, without previous associated ocular inflammation. Corneal dystrophies are classified according to the involved corneal layer in superficial, stromal, and posterior dystrophy. Incidence of each dystrophy varies according to the geographic region studied. PURPOSE: To evaluate the prevalence of stromal corneal dystrophies among corneal buttons specimens obtained by penetrating keratoplasty (PK in an ocular pathology laboratory and to correlate the diagnosis with patient age and gender. METHODS: Corneal button cases of penetrating keratoplasty from January-1996 to May-2009 were retrieved from the archives of The Henry C. Witelson Ophthalmic Pathology Laboratory and Registry, Montreal, Canada. The cases with histopathological diagnosis of stromal corneal dystrophies were stained with special stains (Peroxid acid Schiff, Masson trichrome, Congo red analyzed under polarized light, and alcian blue for classification and correlated with epidemiological information (age at time of PK and gender from patients' file. RESULTS: 1,300 corneal buttons cases with clinical diagnose of corneal dystrophy were retrieved. Stromal corneal dystrophy was found in 40 (3.1% cases. Lattice corneal dystrophy was the most prevalent with 26 cases (65%. Nineteen were female (73.07% and the PK was performed at average age of 59.3 years old. Combined corneal dystrophy was found in 8 (20% cases, 5 (62.5% of them were female and the average age of the penetrating keratoplasty was 54.8 years old. Granular corneal dystrophy was represented by 5 (12.5% cases, and 2 (40% of them were female. Penetrating keratoplasty was performed at average age of 39.5 years old in granular corneal dystrophy cases. Macular corneal dystrophy was present in only 1 (2.5% case, in a 36 years old female. CONCLUSION: Systematic histopathological approach and evaluation, including special stains in all stromal

  12. Alterations of epithelial adhesion molecules and basement membrane components in lattice corneal dystrophy (LCD).

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    Resch, Miklós D; Schlötzer-Schrehardt, Ursula; Hofmann-Rummelt, Carmen; Kruse, Friedrich E; Seitz, Berthold

    2009-08-01

    The aim of the study was to investigate the histopathological and ultrastructural correlate of delayed epithelial healing in eyes with lattice corneal dystrophy (LCD). Corneal buttons from 4 patients with LCD (two with subepithelial, two with stromal amyloid deposits) and 2 control corneas were examined. Cell-matrix adhesion molecules and basement membrane components of the corneal epithelium were analyzed by immunohistochemistry and hemidesmosomes between epithelium and stroma were quantified by transmission electron microscopy (TEM). By TEM well-developed hemidesmosomes anchored the basal epithelial cells to the underlying basement membrane in all normal and LCD corneas. Hemidesmosome density was not significantly different in subepithelial (224.7 +/- 34.1/100 microm) and stromal (234.3 +/- 36.3/100 microm) LCD compared to controls (241.3 +/- 26.8/100 microm). The basement membrane was interrupted in subepithelial, but continuous in stromal LCD. Integrin alpha6 and beta4 staining formed a continuous line along the basal surface of the corneal epithelium in control corneas, whereas it appeared discontinuous and patchy both in subepithelial and stromal forms of LCD. Staining for alphaV integrin showed irregular staining patterns, i.e. enhanced labelling intensity in subepithelial and interrupted pattern in stromal LCD, respectively. Integrins alpha3, beta1, beta2, and beta5, dystroglycan, and plectin were not markedly different in dystrophic corneas. Type VII collagen showed a discontinuous staining in subepithelial forms of LCD. In stromal forms of LCD, type VII collagen staining occurred in additional patches underneath the epithelial basement membrane zone. Type XVII collagen staining was reduced in subepithelial LCD. Laminin-1, laminin-5 and laminin gamma2 showed variable irregular staining patterns in dystrophic corneas with focal interruptions, focal thickenings, and reduplications of basement membrane. Some irregularities in corneas with subepithelial

  13. In vivo confocal microscopy of pre-Descemet corneal dystrophy associated with X-linked ichthyosis: a case report.

    Science.gov (United States)

    Shi, Hui; Qi, Xiao-Feng; Liu, Tao-Tao; Hao, Qian; Li, Xiao-Hong; Liang, Ling-Ling; Wang, Yi-Miao; Cui, Zhi-Hua

    2017-03-16

    Pre-Descemet corneal dystrophy (PDCD) is characterized by the presence of numerous, tiny, polymorphic opacities immediately anterior to Descemet membrane, which is a rare form of corneal stromal dystrophy and hard to be diagnosed. In vivo confocal microscopy (IVCM) is a useful tool to examine the minimal lesions of the cornea at the cellular level. In this article, we report a rare case of PDCD associated with X-linked ichthyosis and evaluate IVCM findings. We present a 34-year-old male Chinese patient with PDCD associated with X-linked ichthyosis. Slit-lamp biomicroscopy showed the presence of tiny and pleomorphic opacities in the posterior stroma immediately anterior to Descemet membrane bilaterally. IVCM revealed regular distributed hyperreflective particles inside the enlarged and activated keratocytes in the posterior stroma. Hyperreflective particles were also observed dispersedly outside the keratocytes in the anterior stroma. Dermatological examination revealed that the skin over the patient's entire body was dry and coarse, with thickening and scaling of the skin in the extensor side of the extremities. PCR results demonstrated that all ten exons and part flanking sequences of STS gene failed to produce any amplicons in the patient. IVCM is useful for analyzing the living corneal structural changes in rare corneal dystrophies. We first reported the IVCM characteristics of PDCD associated with X-linked ichthyosis, which was caused by a deletion of the steroid sulfatase (STS) gene, confirmed by gene analysis.

  14. Proteoglycan biosynthesis by human corneas from patients with types 1 and 2 macular corneal dystrophy

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    Midura, R.J.; Hascall, V.C.; MacCallum, D.K.; Meyer, R.F.; Thonar, E.J.; Hassell, J.R.; Smith, C.F.; Klintworth, G.K. (National Institute of Dental Research, Bethesda, MD (USA))

    1990-09-15

    Corneal buttons were obtained from patients with types 1 and 2 macular corneal dystrophy (MCD) and from control patients with Fuchs' dystrophy or keratoconus. Buttons were incubated for 20 h in the presence of (3H)glucosamine or (2-3H)mannose. Radiolabeled proteoglycans and lactosaminoglycan-glycoproteins (L-GPs) were purified using chromatography on Q-Sepharose, Superose 6, and octyl-Sepharose. They were identified using chondroitinase ABC, keratanase or endo-beta-galactosidase digestion, and sodium dodecyl sulfate-polyacrylamide gel electrophoresis or Superose 6 chromatography. This study confirms previous reports that type 1 MCD corneas synthesize a normal dermatan sulfate-proteoglycan (DS-PG) and an abnormal keratan sulfate-proteoglycan (KS-PG). The data indicate that typ 1 MCD corneas synthesize L-GP instead of KS-PG. This L-GP has a core protein of similar hydrophobicity (elution from octyl-Sepharose) and nearly similar mass (42 kDa) as the core protein of the KS-PG. It has identical glycoconjugates as those of the KS-PG except that they lack sulfate. Thus, type 1 MCD fails to synthesize keratan sulfate as a result of a defect in a sulfotransferase specific for sulfating lactosaminoglycans. Further, proteoglycans synthesized by a cornea from a patient with type 2 MCD were studied. This cornea synthesized a normal ratio of KS-PG to DS-PG although net synthesis of proteoglycans was approximately 30% below normal. The KS-PG appeared normal whereas the DS-PG had dermatan sulfate chains that were approximately 40% shorter than normal.

  15. Establishment of a transgenic mouse model of corneal dystrophy overexpressing human BIGH3.

    Science.gov (United States)

    Liao, Xin; Cui, Hongping; Wang, Fang

    2013-11-01

    This study aimed to establish a transgenic mouse model of corneal dystrophy (CD) overexpressing the human transforming growth factor, β-induced, 68 kDa (TGFBI, also known as BIGH3) gene. A purified and linearized recombinant plasmid carrying the expression cassette BIGH3‑IRES‑EGFP was microinjected into the pronuclei of C57BL/6J mouse fertilized eggs under the control of the phosphoglycerate kinase (PGK) promoter. The expression of human BIGH3 in the transgenic mice was confirmed by PCR using DNA extracted from tail tissue. Four founder transgenic mice were identified by PCR and the increased expression of BIGH3 was observed in the corneas of the transgenic mice by RT-PCR and western blot analysis. The abnormal corneas with central opacity were observed in the transgenic mice by corneal photography. We concluded that the exogenous gene, BIGH3, was integrated successfully into the mouse genome through microinjection. In addition, the phenotype observed in this BIGH3 transgenic mouse model was similar to CD. Therefore, this transgenic model may prove useful in the investigation of the pathogenesis of CD.

  16. Development of a Transgenic Mouse with R124H Human TGFBI Mutation Associated with Granular Corneal Dystrophy Type 2.

    Science.gov (United States)

    Yamazoe, Katsuya; Yoshida, Satoru; Yasuda, Miyuki; Hatou, Shin; Inagaki, Emi; Ogawa, Yoko; Tsubota, Kazuo; Shimmura, Shigeto

    2015-01-01

    To investigate the phenotype and predisposing factors of a granular corneal dystrophy type 2 transgenic mouse model. Human TGFBI cDNA with R124H mutation was used to make a transgenic mouse expressing human protein (TGFBIR124H mouse). Reverse transcription PCR (RT-PCR) was performed to analyze TGFBIR124H expression. A total of 226 mice including 23 homozygotes, 106 heterozygotes and 97 wild-type mice were examined for phenotype. Affected mice were also examined by histology, immunohistochemistry and electron microcopy. RT-PCR confirmed the expression of TGFBIR124H in transgenic mice. Corneal opacity defined as granular and lattice deposits was observed in 45.0% of homozygotes, 19.4% of heterozygotes. The incidence of corneal opacity was significantly higher in homozygotes than in heterozygotes (p = 0.02). Histology of affected mice was similar to histology of human disease. Lesions were Congo red and Masson Trichrome positive, and were observed as a deposit of amorphous material by electron microscopy. Subepithelial stroma was also stained with thioflavin T and LC3, a marker of autophagy activation. The incidence of corneal opacity was higher in aged mice in each group. Homozygotes were not necessarily more severe than heterozygotes, which deffers from human cases. We established a granular corneal dystrophy type 2 mouse model caused by R124H mutation of human TGFBI. Although the phenotype of this mouse model is not equivalent to that in humans, further studies using this model may help elucidate the pathophysiology of this disease.

  17. Posterior amorphous corneal dystrophy is associated with a deletion of small leucine-rich proteoglycans on chromosome 12.

    Science.gov (United States)

    Kim, Michelle J; Frausto, Ricardo F; Rosenwasser, George O D; Bui, Tina; Le, Derek J; Stone, Edwin M; Aldave, Anthony J

    2014-01-01

    Posterior amorphous corneal dystrophy (PACD) is a rare, autosomal dominant disorder affecting the cornea and iris. Next-generation sequencing of the previously identified PACD linkage interval on chromosome 12q21.33 failed to yield a pathogenic mutation. However, array-based copy number analysis and qPCR were used to detect a hemizygous deletion in the PACD linkage interval containing 4 genes encoding small leucine-rich proteoglycans (SLRPs): KERA, LUM, DCN, and EPYC. Two other unrelated families with PACD also demonstrated deletion of these SLRPs, which play important roles in collagen fibrillogenesis and matrix assembly. Given that these genes are essential to the maintenance of corneal clarity and the observation that knockout murine models display corneal phenotypic similarities to PACD, we provide convincing evidence that PACD is associated with haploinsufficiency of these SLRPs.

  18. Intraocular lens power calculations for cataract surgery after phototherapeutic keratectomy in granular corneal dystrophy type 2.

    Science.gov (United States)

    Jung, Se Hwan; Han, Kyung Eun; Sgrignoli, Bradford; Kim, Tae-Im; Lee, Hyung Keun; Kim, Eung Kweon

    2012-10-01

    To investigate the predictability of various intraocular lens (IOL) power calculation methods in granular corneal dystrophy type 2 (GCD2) with prior phototherapeutic keratectomy (PTK) and to suggest the more predictable IOL power calculation method. Medical records of 20 eyes from 16 patients with GCD2, all having undergone cataract surgery after PTK, were retrospectively evaluated. Postoperative cataract refractive errors were compared with target diopters (D) using IOL power calculation methods as follows: 1) myopic and 2) hyperopic Haigis-L formula in IOLMaster (Carl Zeiss Meditec); 3) SRK/T formula using 4.5-mm zone Holladay equivalent keratometry readings (EKRs) (single-K Holladay EKRs method); 4) central keratometry power of true net power map in the Pentacam system (Oculus Optikgeräte GmbH); and 5) clinical history, Aramberri double-K, and double-K Holladay EKRs methods. Topographic status of corneal curvature after PTK was evaluated. Fourteen (70%) of 20 eyes showed central island formation after PTK. When central island was present, the mean absolute error (MAE) using the hyperopic Haigis-L formula was 0.25±0.15 D. When central island was not present, the myopic Haigis-L formula showed MAE of 0.33±0.16 D. When central island formation and IOLMaster keratometry underestimation were present, the hyperopic Haigis-L formula showed the least MAE of 0.26±0.08 D when switching the IOL-Master keratometry values equal to 4.5-mm zone Holladay EKRs. In planning for cataract surgery after PTK in GCD2, topographic analysis for central island formation is necessary. With or without central island formation, the hyperopic or myopic Haigis-L formula can be applied. When IOLMaster keratometry shows underestimation, the Haigis-L formula using 4.5-mm zone Holladay EKRs can be considered. Copyright 2012, SLACK Incorporated.

  19. Boston Type 1 Keratoprosthesis for Gelatinous Drop-Like Corneal Dystrophy.

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    Lekhanont, Kaevalin; Jongkhajornpong, Passara; Chuephanich, Pichaya; Inatomi, Tsutomu; Kinoshita, Shigeru

    2016-06-01

    To report the outcomes of Boston type 1 keratoprosthesis in the management of advanced gelatinous drop-like corneal dystrophy (GDLD). A retrospective, noncomparative, interventional case series was conducted at Ramathibodi Hospital, Bangkok, Thailand. Four eyes of three siblings with molecularly and histologically confirmed GDLD from a Thai family underwent an uneventful Boston type 1 keratoprosthesis implantation for visual rehabilitation. Clinical data were obtained from a review of the medical records. Visual acuity, device retention, and postoperative complications were the main outcome measures. The follow-up ranged from 8 to 96 months. One eye received keratoprosthesis surgery as a primary penetrating procedure. The other three eyes had the surgery as a secondary procedure after graft failure. Best-corrected visual acuity was favorably improved from counting fingers to 20/25 in two eyes, from hand movement to 20/20 in one eye, and from hand movement to counting fingers at 2 feet in one eye caused by severe amblyopia. The improved vision was maintained for 8 months to 6.2 years after surgery. Postoperative complications included disease recurrence in the donor graft (N = 3), manageable retroprosthetic membrane (N = 3), intraocular pressure elevation responded to antiglaucoma drugs (N = 2), and Pseudomonas keratitis with severe corneal melting requiring device removal (N = 1). All of our patients failed to have a comfortably well-fitting contact lens after surgery. Boston type 1 keratoprosthesis could be considered as a reasonable option in the management of advanced GDLD. However, patients remain at risk for sight-threatening postoperative complications as long as the keratoprosthesis is retained. The use of Boston keratoprosthesis implantation needed to be individualized on a case-by-case basis.

  20. Novel CHST6 gene mutations in 2 unrelated cases of macular corneal dystrophy.

    Science.gov (United States)

    Patel, Dhara A; Harocopos, George J; Chang, Shu-Hong; Vora, Smita C; Lubniewski, Anthony J; Huang, Andrew Jw

    2011-06-01

    To investigate the possible mutations in the carbohydrate sulfotransferase 6 (CHST6) gene of 2 unrelated cases of macular corneal dystrophy (MCD) and to report atypical stromal deposits in one of them. Corneal tissues were stained with antisulfated keratan sulfate (KS), antitransforming growth factor beta 1-induced protein (TGFBIp), thioflavin-T, alcian blue, and Masson trichrome. Sequencing was performed to identify potential mutations in the CHST6 gene and the fourth and twelfth exons of the TGFBI gene. Alcian blue staining revealed the presence of multiple subepithelial and intrastromal mucopolysaccharide deposits, confirming the diagnosis of MCD in both cases. Immunofluorescence staining in case 1 revealed the presence of sulfated KS only in the keratocytes and select endothelial cells, consistent with MCD type IA. Preferential expression of sulfated KS was observed in keratocytes and extracellular stromal matrix in case 2, consistent with MCD type II. Atypical subepithelial and superficial stromal deposits were observed in case 1, which stained positively with alcian blue, eosin, Masson trichrome, and thioflavin-T indicating the presence of hyaline and amyloid materials. CHST6 gene sequencing revealed 2 heterozygous mutations in case 1 (a p.Arg211Gln and a novel mutation of p.Arg177Gly) and a novel homozygous mutation of p.Pro186Arg in case 2. No mutations were found in exons 4 or 12 of the TGFBI gene in case 1. Secondary hyalinosis and amyloidosis occur in a case of MCD type IA with a novel p.Arg177Gly mutation in CHST6. A novel p.Pro186Arg mutation in CHST6 is associated with MCD type II in an African American.

  1. Surgical outcome after phototherapeutic keratectomy in patients with TGFBI-linked corneal dystrophies in relation to molecular genetic findings.

    Science.gov (United States)

    Gruenauer-Kloevekorn, Claudia; Braeutigam, Saskia; Froster, Ursula G; Duncker, Gernot I W

    2009-01-01

    To evaluate the correlation between surgical outcome after phototherapeutic keratectomy in patients with autosomal dominant transforming growth factor, beta-induced (TGFBI)-linked corneal dystrophies (CD) and molecular genetic findings regarding the TGFBI gene. Twelve patients were examined to investigate genotype by direct sequencing of the TGFBI gene. Twenty eyes of 12 patients were treated with phototherapeutic keratektomy (PTK) to remove superficial corneal opacifications and to decrease recurrent erosions. Surgical outcome, including visual improvement, recurrence of opacifications, postoperative complications, and additional therapeutic proceedings were reported and compared with the molecular genetic results. Four different missense mutations were identified within the coding region of the TGFBI gene: Arg124Cys in one eye, Arg555Trp in nine eyes, Arg124His in four eyes and Gly623Arg in six eyes. In all eyes the PTK was successful without clinically significant recurrent opacifications after a mean follow-up time of 17.6 months (min 3 months, max 42 months). The best corrected visual acuity (BCVA) improved with an average increase of 3.1 lines (minimum 2 lines, maximum 5 lines). In one eye (Arg124Cys), we observed delayed wound healing and a delayed increase in BCVA, in two eyes we performed an Epilasik to correct remaining hyperopia, and in four eyes we fitted rigid gas-permeable tricurve contact lenses to correct the remaining irregular astigmatism. The variable genotypes in patients with TGFBI-linked corneal dystrophies lead to significantly different results after surgical treatment. The Gly623Arg mutation seems to be an optimum genotype on which to perform PTK even in older patients. It is essential to determine the genotype in order to standardize the PTK treatment and to evaluate the success in TGFBI-linked corneal dystrophies.

  2. Polymorphism of the Transferrin Gene in Eye Diseases: Keratoconus and Fuchs Endothelial Corneal Dystrophy

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    Katarzyna A. Wójcik

    2013-01-01

    Full Text Available Oxidative stress may play a role in the pathogenesis of keratoconus (KC and Fuchs endothelial corneal dystrophy (FECD. Iron may promote the stress by the Fenton reaction, so its homeostasis should be strictly controlled. Transferrin is essential for iron homeostasis because it transports iron from plasma into cells. The malfunction of transferrin, which may be caused by variation in its gene (TF variation, may contribute to oxidative stress and change KC and FECD risk. To verify this hypothesis we investigated the association between three polymorphisms of the TF gene, g.3296G>A (rs8177178, g.3481A>G (rs8177179, and c.–2G>A (rs1130459, and KC and FECD occurrence. Genotyping was performed in blood lymphocytes in 216 patients with KC, 130 patients with FECD and 228 controls by PCR-RFLP. We studied also the influence of other risk factors. The A/A genotype and the A allele of the g.3296G>A polymorphism were associated with KC occurrence, while the G allele was negatively correlated with it. We observed a decrease in KC occurrence associated with the A/G genotype of the g.3481A>G polymorphism. We did not find any association between the c.–2G>A polymorphism and KC. No association was found between all three polymorphisms and FECD occurrence.

  3. Analysis and documentation of progression of Fuchs corneal dystrophy with retroillumination photography.

    Science.gov (United States)

    Gottsch, John D; Sundin, Olof H; Rencs, Erik V; Emmert, David G; Stark, Walter J; Cheng, Clement J; Schmidt, Gregory W

    2006-05-01

    Fuchs corneal dystrophy (FCD) is a degenerative disorder of the cornea that is characterized by the progressive accumulation of guttae, which are small excrescences of Descemet's membrane. We describe a method for documenting the location and number of guttae, and ask whether disease progression can be observed during relatively short periods. Patients with FCD were imaged by standard retroillumination photography with a slit lamp. Scanned photographs were analyzed by using NIH ImageJ software to determine the number of individual guttae and areas of confluence. In 4 FCD patients, photographs taken 23 to 30 months apart revealed that, once formed, individual guttae and their relative positions persisted during this period. Very few guttae disappeared, and the emergence of many new guttae was observed. Determination of the area with confluent guttae was used to quantify disease stage. Computer-assisted analysis of retroillumination photographs is proposed as an effective way to document the number and distribution of individual guttae. Although the disease typically progresses slowly during decades, we have been able to detect the formation of new guttae within only 2 years. This rapid assessment of disease progression could be used to measure phenotypic differences between genetic subtypes of FCD. It also could provide important baseline information and methodology for clinical trials of therapeutic options, should these become available.

  4. Automated Retroillumination Photography Analysis for Objective Assessment of Fuchs Corneal Dystrophy.

    Science.gov (United States)

    Eghrari, Allen O; Mumtaz, Aisha A; Garrett, Brian; Rezaei, Mahsa; Akhavan, Mina S; Riazuddin, S Amer; Gottsch, John D

    2017-01-01

    Retroillumination photography analysis is an objective tool for the assessment of the number and distribution of guttae in eyes affected with Fuchs corneal dystrophy (FCD). Current protocols include manual processing of images; here, we assess validity and interrater reliability of automated analysis across various levels of FCD severity. Retroillumination photographs of 97 FCD-affected corneas were acquired, and total counts of guttae were previously summated manually. For each cornea, a single image was loaded into ImageJ software. We reduced color variability and subtracted background noise. Reflection of light from each gutta was identified as a local area of maximum intensity and counted automatically. Noise tolerance level was titrated for each cornea by examining a small region of each image with automated overlay to ensure appropriate coverage of individual guttae. We tested interrater reliability of automated counts of guttae across a spectrum of clinical and educational experience. A set of 97 retroillumination photographs was analyzed. Clinical severity as measured by a modified Krachmer scale ranged from a severity level of 1 to 5 in the set of analyzed corneas. Automated counts by an ophthalmologist correlated strongly with Krachmer grading (R = 0.79) and manual counts (R = 0.88). Intraclass correlation coefficients demonstrated strong correlation at 0.924 (95% CI, 0.870-0.958) among cases analyzed by 3 students, and 0.869 (95% CI, 0.797-0.918) among cases for which images were analyzed by an ophthalmologist and 2 students. Automated retroillumination photography analysis allows for grading of FCD severity with high resolution across a spectrum of disease severity.

  5. Polymorphisms of the Homologous Recombination Gene RAD51 in Keratoconus and Fuchs Endothelial Corneal Dystrophy

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    Ewelina Synowiec

    2013-01-01

    Full Text Available Purpose. We investigated the association between genotypes and haplotypes of the c.-61G>T (rs 1801320 and c.-98G>C (rs 1801321 polymorphisms of the RAD51 gene and the occurrence of keratoconus (KC and Fuchs endothelial corneal dystrophy (FECD in dependence on some environmental factors. Methods. The polymorphisms were genotyped in peripheral blood lymphocytes of 100 KC and 100 FECD patients as well as 150 controls with PCR-RFLP. Results. The G/T genotype of the c.-61G>T polymorphism was associated with significantly increased frequency occurrence of KC (crude OR 2.99, 95% CI 1.75–5.13. On the other hand, the G/G genotype of this polymorphism was positively correlated with a decreased occurrence of this disease (crude OR 0.52, 95% CI 0.31–0.88. We did not find any correlation between genotypes/alleles of the c.-98G>C polymorphism and the occurrence of KC. We also found that the G/G genotype and G allele of the c.-98G>C polymorphism had a protective effect against FECD (crude OR 0.51, 95% CI 0.28–0.92; crude OR 0.53, 95% CI 0.30–0.92, resp., while the G/C genotype and the C allele increased FECD occurrence (crude OR 1.85, 95% CI 1.01–3.36; crude OR 1.90, 95% CI 1.09–3.29, resp.. Conclusions. The c.-61T/T and c.-98G>C polymorphisms of the RAD51 gene may have a role in the KC and FECD pathogenesis and can be considered as markers in these diseases.

  6. Automated retroillumination photography analysis for objective assessment of Fuchs Corneal Dystrophy severity

    Science.gov (United States)

    Eghrari, Allen O.; Mumtaz, Aisha A.; Garrett, Brian; Rezaei, Mahsa; Akhavan, Mina S.; Riazuddin, S. Amer; Gottsch, John D.

    2016-01-01

    Purpose Retroillumination photography analysis (RPA) is an objective tool for assessment of the number and distribution of guttae in eyes affected with Fuchs Corneal Dystrophy (FCD). Current protocols include manual processing of images; here we assess validity and interrater reliability of automated analysis across various levels of FCD severity. Methods Retroillumination photographs of 97 FCD-affected corneas were acquired and total counts of guttae previously summated manually. For each cornea, a single image was loaded into ImageJ software. We reduced color variability and subtracted background noise. Reflection of light from each gutta was identified as a local area of maximum intensity and counted automatically. Noise tolerance level was titrated for each cornea by examining a small region of each image with automated overlay to ensure appropriate coverage of individual guttae. We tested interrater reliability of automated counts of guttae across a spectrum of clinical and educational experience. Results A set of 97 retroillumination photographs were analyzed. Clinical severity as measured by a modified Krachmer scale ranged from a severity level of 1 to 5 in the set of analyzed corneas. Automated counts by an ophthalmologist correlated strongly with Krachmer grading (R2=0.79) and manual counts (R2=0.88). Intraclass correlation coefficient demonstrated strong correlation, at 0.924 (95% CI, 0.870- 0.958) among cases analyzed by three students, and 0.869 (95% CI, 0.797- 0.918) among cases for which images was analyzed by an ophthalmologist and two students. Conclusions Automated RPA allows for grading of FCD severity with high resolution across a spectrum of disease severity. PMID:27811565

  7. Retroillumination photography analysis enhances clinical definition of severe Fuchs Corneal Dystrophy

    Science.gov (United States)

    Eghrari, Allen O.; Garrett, Brian S.; Mumtaz, Aisha A.; Edalati, Armand E.; Meadows, Danielle N.; McGlumphy, Elyse J.; Iliff, Benjamin W.; Gottsch, John D.

    2015-01-01

    Purpose Retroillumination photography analysis (RPA) provides objective assessment of the number and distribution of guttae in Fuchs Corneal Dystrophy. Here, we assess its correlation with clinical grading using slit-lamp biomicroscopy across varying levels of severity. Methods Retroillumination photographs were conducted of 95 affected corneas with slit-lamp flash photography after pupillary dilation. Individual guttae were counted manually and the position of individual points recorded. Clinical grading using the Krachmer scale was documented for each eye during examination, and regression analyses were performed to identify the strength of association with number of guttae. We assessed range at each stage of clinical grading, and utilized the Mann-Whitney U test to assess whether clinical grading levels demonstrated successively higher numbers of guttae. Results Krachmer score ranged from 1 to 5, with mean of 2.6. Mean numbers of guttae at each level of severity were 289 (1+), 999 (2+), 2669 (3+), 5474 (4+), and 7133 (5+). Each stage demonstrated significantly higher numbers of guttae than its preceding level except from 4+ to 5+ (p=0.30), consistent with the definition of 4+ as the highest level defined by presence of guttae. Higher levels of clinical grading were associated with larger ranges of guttae (p<0.01). A linear regression model resulted in a strong fit between RPA and Krachmer score (r=0.81). Conclusion In this largest study of RPA data and comparison with subjective clinical grading of FCD severity, RPA correlates strongly and demonstrates enhanced definition of severity at advanced stages of disease. PMID:26488628

  8. Boston type I keratoprosthesis for treatment of gelatinous drop-like corneal dystrophy after repeated graft failure.

    Science.gov (United States)

    Fadlallah, A; Jakobiec, F A; Mendoza, P R; Zalloua, P A; Melki, S A

    2015-03-01

    To describe the use of a Boston type I keratoprosthesis as a secondary penetrating procedure to treat gelatinous drop-like corneal dystrophy (GDLD), with presentation of pathologic findings, genetic analysis, and discussion of other surgical options. A 43-year-old woman with GDLD in both eyes, best corrected visual acuity (BCVA) of counting fingers in both eyes, and recurrent corneal opacification following two penetrating keratoplasties presented for visual rehabilitation. A Boston type I keratoprosthesis was implanted in her left eye after extracapsular clear lens extraction. The surgery was uneventful and one month after surgery, best corrected vision improved to 20/30, which has been maintained for a period of more than nine months. At the 12-month visit, her vision was noted to be diminished to 20/200 due to a retroprosthetic membrane and improved to 20/25 two weeks after a Yag capsulotomy. Histopathologic examination of the corneal specimen disclosed predominantly subepithelial amyloid deposition. Genetic analysis is presented. GDLD is a rare disorder of primary corneal amyloidosis. Recurrence of this condition following surgery is very common. Boston type I keratoprosthesis as a secondary procedure can be successful in restoring vision in affected patients.

  9. The PPCD1 mouse: characterization of a mouse model for posterior polymorphous corneal dystrophy and identification of a candidate gene.

    Directory of Open Access Journals (Sweden)

    Anna L Shen

    2010-08-01

    Full Text Available The PPCD1 mouse, a spontaneous mutant that arose in our mouse colony, is characterized by an enlarged anterior chamber resulting from metaplasia of the corneal endothelium and blockage of the iridocorneal angle by epithelialized corneal endothelial cells. The presence of stratified multilayered corneal endothelial cells with abnormal patterns of cytokeratin expression are remarkably similar to those observed in human posterior polymorphous corneal dystrophy (PPCD and the sporadic condition, iridocorneal endothelial syndrome. Affected eyes exhibit epithelialized corneal endothelial cells, with inappropriate cytokeratin expression and proliferation over the iridocorneal angle and posterior cornea. We have termed this the "mouse PPCD1" phenotype and mapped the mouse locus for this phenotype, designated "Ppcd1", to a 6.1 Mbp interval on Chromosome 2, which is syntenic to the human Chromosome 20 PPCD1 interval. Inheritance of the mouse PPCD1 phenotype is autosomal dominant, with complete penetrance on the sensitive DBA/2J background and decreased penetrance on the C57BL/6J background. Comparative genome hybridization has identified a hemizygous 78 Kbp duplication in the mapped interval. The endpoints of the duplication are located in positions that disrupt the genes Csrp2bp and 6330439K17Rik and lead to duplication of the pseudogene LOC100043552. Quantitative reverse transcriptase-PCR indicates that expression levels of Csrp2bp and 6330439K17Rik are decreased in eyes of PPCD1 mice. Based on the observations of decreased gene expression levels, association with ZEB1-related pathways, and the report of corneal opacities in Csrp2bp(tm1a(KOMPWtsi heterozygotes and embryonic lethality in nulls, we postulate that duplication of the 78 Kbp segment leading to haploinsufficiency of Csrp2bp is responsible for the mouse PPCD1 phenotype. Similarly, CSRP2BP haploinsufficiency may lead to human PPCD.

  10. Combined Excimer Laser Photoablation and Amniotic Membrane Overlay for Relief of Symptomatic Discomfort in Gelatinous Drop-like Corneal Dystrophy.

    Science.gov (United States)

    Alex, Anne F; Eter, Nicole; Uhlig, Constantin E

    2015-10-01

    To describe the efficacy of combined excimer laser photoablation and amniotic overlay membrane in the relief of symptomatic discomfort in a 17-year-old patient who had gelatinous drop-like corneal dystrophy. The best-corrected visual acuity (BCVA) was measured with Snellen letters. Slit-lamp examination of the ocular surface and anterior chamber was performed at baseline. Results were photodocumented. Excimer laser photoablation was performed and subsequently 2 amniotic membranes were transconjunctivally fixated with 10.0 nylon sutures. Investigations and documentation were performed at baseline, every 2 months in the first year, and then every 6 months. The duration of follow-up was 22 months. At baseline, the BCVA was 20/70 in the right eye and 20/200 in the left eye. The patient reported distinct photophobia. Slit-lamp examination was difficult because of blepharospasm. Although gelatinous drops developed again and the BCVA decreased to 2/200, the patient reported significant relief after both microsurgical treatments and remained comfortable at 20 and 22 months. Excimer laser photocoagulation combined with amniotic membrane overlay does not stop the development of gelatinous drop-like corneal dystrophy but may improve subjective comfort. Such treatment does not hinder subsequent lamellar or penetrating grafts and is helpful in providing the necessary time for preparation of matched keratoplasties.

  11. Proteomic profiling of TGFBI-null mouse corneas reveals only minor changes in matrix composition supportive of TGFBI knockdown as therapy against TGFBI-linked corneal dystrophies

    DEFF Research Database (Denmark)

    Poulsen, Ebbe Toftgaard; Runager, Kasper; Nielsen, Nadia Sukusu

    2017-01-01

    role in maintaining structural integrity and possibly corneal transparency as well. Significantly, more than 60 point mutations within the TGFBI gene have been reported to result in aberrant TGFBIp folding and aggregation in the cornea, resulting in severe visual impairment and blindness. Several...... studies have focused on targeting TGFBIp in the cornea as a therapeutic approach to treat TGFBI-linked corneal dystrophies, but the effect of this approach on corneal homeostasis and matrix integrity remained unknown. In the current study, we evaluated the histological and proteomic profiles of corneas...

  12. Active transforming growth factor-β2 in the aqueous humor of posterior polymorphous corneal dystrophy patients.

    Directory of Open Access Journals (Sweden)

    Andrea Stadnikova

    Full Text Available Posterior polymorphous corneal dystrophy (PPCD is characterized by abnormal proliferation of corneal endothelial cells. It was shown that TGF-β2 present in aqueous humor (AH could help maintaining the corneal endothelium in a G1-phase-arrest state. We wanted to determine whether the levels of this protein are changed in AH of PPCD patients.We determined the concentrations of active TGF-β2 in the AH of 29 PPCD patients (42 samples and 40 cadaver controls (44 samples by ELISA. For data analysis the PPCD patients were divided based on either the molecular genetic cause of their disease as PPCD1 (37 samples, PPCD3 (1 sample and PPCDx (not linked to a known PPCD loci, 4 samples or on the presence (17 samples or absence (25 samples of secondary glaucoma or on whether they had undergone penetrating keratoplasty (PK, 32 samples or repeated PK (rePK, 7 samples.The level of active TGF-β2 in the AH of all PPCD patients (mean ± SD; 386.98 ± 114.88 pg/ml in comparison to the control group (260.95 ± 112.43 pg/ml was significantly higher (P = 0.0001. Compared to the control group, a significantly higher level of active TGF-β2 was found in the PPCD1 (P = 0.0005 and PPCDx (P = 0.0022 groups. Among patients the levels of active TGF-β2 were not significantly affected by gender, age, secondary glaucoma or by the progression of dystrophy when one or repeated PK were performed.The levels of active TGF-β2 in the AH of PPCD patients are significantly higher than control values, and thus the increased levels of TGF-β2 could be a consequence of the PPCD phenotype and can be considered as another feature characterizing this disease.

  13. Menadione-Induced DNA Damage Leads to Mitochondrial Dysfunction and Fragmentation During Rosette Formation in Fuchs Endothelial Corneal Dystrophy.

    Science.gov (United States)

    Halilovic, Adna; Schmedt, Thore; Benischke, Anne-Sophie; Hamill, Cecily; Chen, Yuming; Santos, Janine Hertzog; Jurkunas, Ula V

    2016-06-20

    Fuchs endothelial corneal dystrophy (FECD), a leading cause of age-related corneal edema requiring transplantation, is characterized by rosette formation of corneal endothelium with ensuing apoptosis. We sought to determine whether excess of mitochondrial reactive oxygen species leads to chronic accumulation of oxidative DNA damage and mitochondrial dysfunction, instigating cell death. We modeled the pathognomonic rosette formation of postmitotic corneal cells by increasing endogenous cellular oxidative stress with menadione (MN) and performed a temporal analysis of its effect in normal (HCEnC, HCECi) and FECD (FECDi) cells and ex vivo specimens. FECDi and FECD ex vivo specimens exhibited extensive mtDNA and nDNA damage as detected by quantitative PCR. Exposure to MN triggered an increase in mitochondrial superoxide levels and led to mtDNA and nDNA damage, while DNA amplification was restored with NAC pretreatment. Furthermore, MN exposure led to a decrease in ΔΨm and adenosine triphosphate levels in normal cells, while FECDi exhibited mitochondrial dysfunction at baseline. Mitochondrial fragmentation and cytochrome c release were detected in FECD tissue and after MN treatment of HCEnCs. Furthermore, cleavage of caspase-9 and caspase-3 followed MN-induced cytochrome c release in HCEnCs. This study provides the first line of evidence that accumulation of oxidative DNA damage leads to rosette formation, loss of functionally intact mitochondria via fragmentation, and subsequent cell death during postmitotic cell degeneration of ocular tissue. MN induced rosette formation, along with mtDNA and nDNA damage, mitochondrial dysfunction, and fragmentation, leading to activation of the intrinsic apoptosis via caspase cleavage and cytochrome c release. Antioxid. Redox Signal. 24, 1072-1083.

  14. Simultaneous Transplantation of Limbal Stem Cells May Reduce Recurrences of Granular Dystrophy After Corneal Transplantation

    OpenAIRE

    Lang, Stefan J.; Eberwein, Philipp; Reinshagen, Helga; Reinhard, Thomas; Sundmacher, Rainer

    2015-01-01

    Abstract To present 2 cases with long-term relapse-free intervals only after limbo-keratoplasty but not after conventional penetrating keratoplasty in granular dystrophy. Retrospective review of the patient charts and photographs taken during long-term follow-up of 2 cases with granular dystrophy, in which 1 eye received penetrating keratoplasty and the fellow eye received penetrating limbo-keratoplasty. In the first patient, 1 eye showed extensive recurrence of granular deposits 17 years aft...

  15. Simultaneous transplantation of limbal stem cells may reduce recurrences of granular dystrophy after corneal transplantation: 2 long-term case reports.

    Science.gov (United States)

    Lang, Stefan J; Eberwein, Philipp; Reinshagen, Helga; Reinhard, Thomas; Sundmacher, Rainer

    2015-05-01

    To present 2 cases with long-term relapse-free intervals only after limbo-keratoplasty but not after conventional penetrating keratoplasty in granular dystrophy.Retrospective review of the patient charts and photographs taken during long-term follow-up of 2 cases with granular dystrophy, in which 1 eye received penetrating keratoplasty and the fellow eye received penetrating limbo-keratoplasty.In the first patient, 1 eye showed extensive recurrence of granular deposits 17 years after penetrating keratoplasty was performed while in the second eye two-thirds of the corneal transplant adjacent to the transplanted limbal area remained clear 12 years after the limbo-corneal transplant. In the second patient, 1 eye showed no signs of recurrence 5 years after limbo-keratoplasty, whereas a recurrence of granular corneal deposits occurred 18 months after surgery in the fellow eye.These cases show that the simultaneous transplantation of healthy donor limbus when performing penetrating keratoplasty may prolong recurrence in granular corneal dystrophy. Although we were unable to prove it on the molecular level, these clinical courses may support the hypothesis that a limbal transplant helps prevent a recurrence.

  16. Clinical outcomes and risk factors for graft failure after deep anterior lamellar keratoplasty and penetrating keratoplasty for macular corneal dystrophy.

    Science.gov (United States)

    Reddy, Jagadesh C; Murthy, Somasheila I; Vaddavalli, Pravin K; Garg, Prashant; Ramappa, Muralidhar; Chaurasia, Sunita; Rathi, Varsha; Sangwan, Virender S

    2015-02-01

    The aim of this study was to compare visual acuity, clinical outcomes, complications, and risk factors for graft failure after deep anterior lamellar keratoplasty (DALK) and penetrating keratoplasty (PK) for macular corneal dystrophy. Retrospective comparative case series. The PK group consisted of 109 eyes of 84 patients and the DALK group consisted of 21 eyes of 20 patients. The mean logarithm of the minimum angle of resolution best-corrected visual acuity at 3 and 12 months was 0.5 versus 0.5 (P = 0.285) and 0.4 versus 0.4 (P = 0.67) in the DALK and PK groups, respectively. There was no significant statistical difference in astigmatism and spherical equivalent between the 2 groups at 12 months. In the PK group, graft rejection that was the most common cause of graft failure was seen in 27 eyes (25%), of which 55% occurred within 1 year. In the DALK group, Descemet membrane microperforation occurred in 5 eyes (24%) intraoperatively, and early postoperative Descemet membrane detachment with double anterior chamber occurred in 9 eyes (43%). Kaplan-Meier estimate of graft survival in PK versus DALK groups were 93% versus 80% at 1 year and 78% versus 70% at 4 years, respectively. Visual and refractive outcomes are comparable between DALK and PK groups. DALK was superior to PK in its safety against postoperative complications such as endothelial rejection and secondary glaucoma. Graft failure in DALK was mostly associated with either intraoperative or early postoperative complications. DALK is a viable surgical option in cases with macular corneal dystrophy.

  17. Corneal Laceration

    Medline Plus

    Full Text Available ... inflammatory drugs. These drugs thin the blood and may increase bleeding after you have finished protecting the ... a hair dryer to treat my Fuchs’ dystrophy? May 06, 2017 I lost sight from a corneal ...

  18. Comparative Study of Anterior Eye Segment Measurements with Spectral Swept-Source and Time-Domain Optical Coherence Tomography in Eyes with Corneal Dystrophies

    Directory of Open Access Journals (Sweden)

    Anna K. Nowinska

    2015-01-01

    Full Text Available Purpose. To compare anterior eye segment measurements and morphology obtained with two optical coherence tomography systems (TD OCT, SS OCT in eyes with corneal dystrophies (CDs. Methods. Fifty healthy volunteers (50 eyes and 54 patients (96 eyes diagnosed with CD (epithelial basement membrane dystrophy, EBMD = 12 eyes; Thiel-Behnke CD = 6 eyes; lattice CD TGFBI type = 15 eyes; granular CD type 1 = 7 eyes, granular CD type 2 = 2 eyes; macular CD = 23 eyes; and Fuchs endothelial CD = 31 eyes were recruited for the study. Automated and manual central corneal thickness (aCCT, mCCT, anterior chamber depth (ACD, and nasal and temporal trabecular iris angle (nTIA, tTIA were measured and compared with Bland-Altman plots. Results. Good agreement between the TD and SS OCT measurements was demonstrated for mCCT and aCCT in normal individuals and for mCCT in the CDs group. The ACD, nTIA, and tTIA measurements differed significantly in both groups. TBCD, LCD, and FECD caused increased CCT. MCD caused significant corneal thinning. FECD affected all analyzed parameters. Conclusions. Better agreement between SS OCT and TD OCT measurements was demonstrated in normal individuals compared to the CDs group. OCT provides comprehensive corneal deposits analysis and demonstrates the association of CD with CCT, ACD, and TIA measurements.

  19. Corneal Laceration

    Medline Plus

    Full Text Available ... Answers How often and for how long should I use a hair dryer to treat my Fuchs’ dystrophy? May 06, 2017 I lost sight from a corneal scar as a child. Now that I’m older, will a corneal transplant help me? ...

  20. Evaluating the association of VSX1 mutation with keratoconus and the granular corneal dystrophy in an Iranian family

    Directory of Open Access Journals (Sweden)

    Hossein Aghamollaei

    2017-06-01

    Full Text Available AIM: To evaluate association between mutations in the visual system homeobox 1(VSX1gene and keratoconus(KCNcomplicated with granular corneal dystrophy(GCD, direct sequencing was performed in an Iranian family affected by KCN and GCD in four generations. METHODS: An Iranian pedigree with keratoconus spanning four generations along with GCD was identified. Whole blood sample was used for genomic DNA extraction. The molecular analysis by using polymerase chain reaction(PCRof the entire coding region and intron-exon boundaries of VSX1 gene was preformed to investigate the possible linkage between KCN and GCD. Subsequently, direct sequencing was used for PCR products and mutation analysis was conducted in the patients and controls. RESULTS: Mutation analysis in VSX1 gene did not detect evidence for association between KCN and GCD diseases and VSX1 gene. Our data excluded VSX1 as the disease-causing gene for KCN/GCD in this specific pedigree.CONCLUSION: Despite of no association between KCN patients with GCD and VSX1 gene variations, other probable genes involved in pathogenesis of the KCN and GCD diseases need to be investigated in the patients.

  1. Pre-Descemet corneal dystrophy and X-linked ichthyosis associated with deletion of Xp22.31 containing the STS gene.

    Science.gov (United States)

    Hung, Crystal; Ayabe, Reed I; Wang, Cynthia; Frausto, Ricardo F; Aldave, Anthony J

    2013-09-01

    To report the association of X-linked ichthyosis and pre-Descemet corneal dystrophy with a deletion of the steroid sulfatase gene (STS) detected with microarray-based comparative genomic hybridization (aCGH). A slit-lamp biomicroscopic examination and cutaneous examination were performed, after which a saliva sample was collected as a source of genomic DNA. Polymerase chain reaction amplification of each of the 10 exons of STS was performed, as was aCGH on genomic DNA to detect copy number variation. The slit-lamp examination revealed punctate opacities in the posterior corneal stroma of each eye. The cutaneous examination demonstrated scaling and flaking skin of the arms and legs. Polymerase chain reaction amplification using primers designed to amplify each of the 10 exons of STS failed to produce any amplicons. Subsequently, aCGH performed on genomic DNA revealed a microdeletion in the Xp22.31 cytoband of approximately 1.7 megabases, containing STS. The identification of a microdeletion within Xp22.3 containing STS with aCGH in an individual with suspected pre-Descemet corneal dystrophy and X-linked ichthyosis demonstrates the clinical utility of copy number variation analysis in confirming a presumptive clinical diagnosis.

  2. Alcohol epitheliectomy with mechanical debridement in a case of granular corneal dystrophy with r555w homozygous mutation of TGF B1 gene

    Directory of Open Access Journals (Sweden)

    Garg Prashant

    2010-01-01

    Full Text Available An eight-year-old girl, an offspring of a consanguineous marriage presented with multiple anterior stromal geographic corneal opacities in both eyes. She was diagnosed to have superficial variant of granular dystrophy based on the family history, clinical features and mutation of TGF B1 gene. She was treated by alcohol-assisted removal of epithelium followed by mechanical debridement of abnormal deposits. Postoperatively, the cornea in both eyes was clear with no trace of opacity and the patient had an unaided visual acuity of 20/20 partial.

  3. Corneal Transplantation in Children

    OpenAIRE

    Gabrić, N.; Dekaris, I.; Vojniković, B.; Karaman, Ž.; Mravičić, I.; Katušić, J.

    2001-01-01

    The main purpose of the study was to describe the surgical success rate and visual results of penetrating keratoplasty in children. This retrospective study included children that underwent corneal transplantation at the Department of Ophthalmology, General Hospital »Sveti Duh«, in the period 1994–1999. Patients’ age ranged from 6 to 16 years. Twenty-five corneal transplants were performed in 24 eyes. Corneal pathologies were corneal leucoma, congenital dystrophy, corneal combu...

  4. High prevalence of posterior polymorphous corneal dystrophy in the Czech Republic; linkage disequilibrium mapping and dating an ancestral mutation.

    Science.gov (United States)

    Liskova, Petra; Gwilliam, Rhian; Filipec, Martin; Jirsova, Katerina; Reinstein Merjava, Stanislava; Deloukas, Panos; Webb, Tom R; Bhattacharya, Shomi S; Ebenezer, Neil D; Morris, Alex G; Hardcastle, Alison J

    2012-01-01

    Posterior polymorphous corneal dystrophy (PPCD) is a rare autosomal dominant genetically heterogeneous disorder. Nineteen Czech PPCD pedigrees with 113 affected family members were identified, and 17 of these kindreds were genotyped for markers on chromosome 20p12.1- 20q12. Comparison of haplotypes in 81 affected members, 20 unaffected first degree relatives and 13 spouses, as well as 55 unrelated controls, supported the hypothesis of a shared ancestor in 12 families originating from one geographic location. In 38 affected individuals from nine of these pedigrees, a common haplotype was observed between D20S48 and D20S107 spanning approximately 23 Mb, demonstrating segregation of disease with the PPCD1 locus. This haplotype was not detected in 110 ethnically matched control chromosomes. Within the common founder haplotype, a core mini-haplotype was detected for D20S605, D20S182 and M189K2 in all 67 affected members from families 1-12, however alleles representing the core mini-haplotype were also detected in population matched controls. The most likely location of the responsible gene within the disease interval, and estimated mutational age, were inferred by linkage disequilibrium mapping (DMLE+2.3). The appearance of a disease-causing mutation was dated between 64-133 generations. The inferred ancestral locus carrying a PPCD1 disease-causing variant within the disease interval spans 60 Kb on 20p11.23, which contains a single known protein coding gene, ZNF133. However, direct sequence analysis of coding and untranslated exons did not reveal a potential pathogenic mutation. Microdeletion or duplication was also excluded by comparative genomic hybridization using a dense chromosome 20 specific array. Geographical origin, haplotype and statistical analysis suggest that in 14 unrelated families an as yet undiscovered mutation on 20p11.23 was inherited from a common ancestor. Prevalence of PPCD in the Czech Republic appears to be the highest worldwide and our data

  5. High prevalence of posterior polymorphous corneal dystrophy in the Czech Republic; linkage disequilibrium mapping and dating an ancestral mutation.

    Directory of Open Access Journals (Sweden)

    Petra Liskova

    Full Text Available Posterior polymorphous corneal dystrophy (PPCD is a rare autosomal dominant genetically heterogeneous disorder. Nineteen Czech PPCD pedigrees with 113 affected family members were identified, and 17 of these kindreds were genotyped for markers on chromosome 20p12.1- 20q12. Comparison of haplotypes in 81 affected members, 20 unaffected first degree relatives and 13 spouses, as well as 55 unrelated controls, supported the hypothesis of a shared ancestor in 12 families originating from one geographic location. In 38 affected individuals from nine of these pedigrees, a common haplotype was observed between D20S48 and D20S107 spanning approximately 23 Mb, demonstrating segregation of disease with the PPCD1 locus. This haplotype was not detected in 110 ethnically matched control chromosomes. Within the common founder haplotype, a core mini-haplotype was detected for D20S605, D20S182 and M189K2 in all 67 affected members from families 1-12, however alleles representing the core mini-haplotype were also detected in population matched controls. The most likely location of the responsible gene within the disease interval, and estimated mutational age, were inferred by linkage disequilibrium mapping (DMLE+2.3. The appearance of a disease-causing mutation was dated between 64-133 generations. The inferred ancestral locus carrying a PPCD1 disease-causing variant within the disease interval spans 60 Kb on 20p11.23, which contains a single known protein coding gene, ZNF133. However, direct sequence analysis of coding and untranslated exons did not reveal a potential pathogenic mutation. Microdeletion or duplication was also excluded by comparative genomic hybridization using a dense chromosome 20 specific array. Geographical origin, haplotype and statistical analysis suggest that in 14 unrelated families an as yet undiscovered mutation on 20p11.23 was inherited from a common ancestor. Prevalence of PPCD in the Czech Republic appears to be the highest worldwide

  6. Association of a Chromosomal Rearrangement Event with Mouse Posterior Polymorphous Corneal Dystrophy and Alterations in Csrp2bp, Dzank1, and Ovol2 Gene Expression.

    Directory of Open Access Journals (Sweden)

    Anna L Shen

    Full Text Available We have previously described a mouse model of human posterior polymorphous corneal dystrophy (PPCD and localized the causative mutation to a 6.2 Mbp region of chromosome 2, termed Ppcd1. We now show that the gene rearrangement linked to mouse Ppcd1 is a 3.9 Mbp chromosomal inversion flanked by 81 Kbp and 542 bp deletions. This recombination event leads to deletion of Csrp2bp Exons 8 through 11, Dzank1 Exons 20 and 21, and the pseudogene Znf133. In addition, we identified translocation of novel downstream sequences to positions adjacent to Csrp2bp Exon 7 and Dzank1 Exon 20. Twelve novel fusion transcripts involving Csrp2bp or Dzank1 linked to downstream sequences have been identified. Eight are expressed at detectable levels in PPCD1 but not wildtype eyes. Upregulation of two Csrp2bp fusion transcripts, as well as upregulation of the adjacent gene, Ovol2, was observed. Absence of the PPCD1 phenotype in animals haploinsufficient for Csrp2bp or both Csrp2bp and Dzank1 rules out haploinsufficiency of these genes as a cause of mouse PPCD1. Complementation experiments confirm that PPCD1 embryonic lethality is due to disruption of Csrp2bp expression. The ocular expression pattern of Csrp2bp is consistent with a role for this protein in corneal development and pathogenesis of PPCD1.

  7. Inheritance of a Novel COL8A2 Mutation Defines a Distinct Early-Onset Subtype of Fuchs Corneal Dystrophy

    National Research Council Canada - National Science Library

    Gottsch, John D; Sundin, Olof H; Liu, Sammy H; Jun, Albert S; Broman, Karl W; Stark, Walter J; Vito, Elizabeth C. L; Narang, Amol K; Thompson, John M; Magovern, Malcolm

    2005-01-01

    1 From the Center for Corneal Genetics, Cornea and External Disease Service, the 2 Laboratory of Developmental Genetics, The Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland...

  8. Corneal transplantation in children.

    Science.gov (United States)

    Gabrić, N; Dekaris, I; Vojniković, B; Karaman, Z; Mravicić, I; Katusić, J

    2001-01-01

    The main purpose of the study was to describe the surgical success rate and visual results of penetrating keratoplasty in children. This retrospective study included children that underwent corneal transplantation at the Department of Ophthalmology, General Hospital "Sveti Duh", in the period 1994-1999. Patients' age ranged from 6 to 16 years. Twenty-five corneal transplants were performed in 24 eyes. Corneal pathologies were corneal leucoma, congenital dystrophy, corneal combustion, corneal scar after perforating injury, keratoconus, corneal melting, hematocornea and rekeratoplasty. The follow-up period was at least 6 months. The rate of graft survival was 1 year in 75% of eyes with congenital dystrophy and keratoconus. Hematocornea and rekeratoplasty ended with graft failure. Postoperative visual acuity improvement was recorded in 14 out of 25 eyes. Penetrating keratoplasty in children showed very good surgical success. The final visual outcome was affected by irreversible amblyopia.

  9. Keratoconus in Patients with Macular Stromal Dystrophy.

    Science.gov (United States)

    Kosrirukvongs, Panida; Ngowyutagon, Panotsom; Booranapong, Wipawee

    2016-01-01

    To show the association between keratoconus and macular dystrophy. All patients with macular dystrophy and associated clinical findings leading to a diagnosis of keratoconus by corneal topography were retrospectively reviewed during a 10-year period. Uncorrected and best-corrected visual acuity, automated refraction, manifest refraction, corneal thickness, and corneal curvature by corneal topography were evaluated Three patients with macular dystrophy exhibiting decreased vision, multifocal white dense deposits, and haze surrounding the deposits in the corneal stroma were evaluated. All had a steep corneal curvature of >47 diopters and a thin cornea consistent with keratoconus. Penetrating keratoplasty was performed in one patient with severely decreased vision. Macular dystrophy was diagnosed based on an Alcian blue-stained pathological specimen. Keratoconus may develop as a result of changes associated with macular dystrophy. Therefore, patients with severely decreased vision should be evaluated for keratoconus to ensure proper management.

  10. Clinical and genetic features of TGFBI-linked corneal dystrophies in Mexican population: description of novel mutations and novel genotype-phenotype correlations.

    Science.gov (United States)

    Zenteno, Juan Carlos; Correa-Gomez, Vicente; Santacruz-Valdez, Concepción; Suarez-Sanchez, Raul; Villanueva-Mendoza, Cristina

    2009-08-01

    Corneal dystrophies (CDS) are inherited disorders characterized by an altered corneal transparency and refractive index which may be caused by a progressive accumulation of deposits within the different corneal layers. Most CDs are inherited in an autosomal dominant fashion and mutations in the TGFBI gene at chromosome 5q31 cause the majority of CDs affecting the stromal layer. A genotype-phenotype correlation has been identified in most analyzed populations as specific amino acid changes in TGFBI protein cause specific stromal phenotypes. However, analysis of additional populations will help to broaden the mutational spectrum ultimately allowing a better clinical-molecular classification of patients with this group of diseases. In this work, eighteen unrelated Mexican probands suffering from stromal CDs were clinically assessed and their TGFBI gene status investigated. Complete ophthalmologic evaluation, including biomicroscopic inspection and dilated fundus examination, was performed. In addition, detailed genealogical analyses as well as automated DNA sequencing of the entire TGFBI gene were done in all probands. Mutation-carrying exons were examined in 50 first and second degree relatives. Phenotypic analysis disclosed the occurrence of 6 cases of lattice CD, 6 of granular CD, 2 of granular type 2 (Avellino CD), 2 of polymorphic corneal amyloidosis, 1 of Reis-Bucklers CD, and 1 of an unclassifiable phenotype. TGFBI mutations were identified in all 18 probands. A total of six different mutations were observed: p.V113I, p.M502V, p.A546D, p.L550P, p.R555W, and p.H626R. Of these, mutations p.L550P (originated by the change c.1649T>C at exon 12), p.M502V (c.1504A>G, at exon 11), and p.V113I (c.337G>A, at exon 4), are novel TGFBI mutations. All subjects with lattice CD in our sample carried the p.H626R mutation. No instances of defects at codon 124, one of the two most frequently mutated sites in TGFBI-linked CDs, were detected. A distinct TGFBI mutational pattern

  11. Corneal collagens.

    Science.gov (United States)

    Robert, L; Legeais, J M; Robert, A M; Renard, G

    2001-05-01

    Cornea is a highly differentiated tissue rich in extracellular matrix (ECM) specifically distributed in space in order to insure its dual role--transparency and protection of inner eye-tissues. Corneal ECM is especially rich in collagens. Since the characterisation of a number of distinct collagen types it appeared that most of them are present in the cornea. Their synthesis follows a specific program of sequential expression of the different collagen types to be synthesised during the development and maturation of the cornea. The precise regulation of the diameter and orientation of fibers, and of the interfibrillar spaces is partially at least attributed to interactions between glycosaminoglycans and collagens. The 'program' of vectorial collagen synthesis and GAG-collagen interactions changes also with age and in several pathological conditions as corneal dystrophies and wound healing. The Maillard reaction, especially in diabetes, is one of these important factors involved in age-dependent modifications of corneal structure and function. Far from being inert, corneal collagens were shown to have relatively short half-lives. The biosynthesis of corneal collagens was studied also during wound healing. The refibrillation of wounded corneas does not follow the original 'program' of ECM-synthesis as shown by the comparative study of wound healing using biochemical and morphometric methods. This review recapitulates briefly previous and recent studies on corneal collagens in order to present to clinicians and scientists an overview of the state of the art of this important field at the intersection of eye research and matrix biology.

  12. Corneal Laceration

    Medline Plus

    Full Text Available ... What Is Corneal Laceration? Corneal Laceration Symptoms What Causes Corneal Laceration? Corneal Laceration Diagnosis Corneal Laceration Treatment ... the corneal laceration is deep enough it can cause a full thickness laceration. This is when the ...

  13. Corneal Laceration

    Medline Plus

    Full Text Available ... What Is Corneal Laceration? Corneal Laceration Symptoms What Causes Corneal Laceration? Corneal Laceration Diagnosis Corneal Laceration Treatment What Is Corneal Laceration? Leer en Español: ¿Qué ...

  14. Genetics of corneal endothelial dystrophies

    Indian Academy of Sciences (India)

    2009-12-31

    Dec 31, 2009 ... additional extracellular matrix known as the posterior col- lagenous layer, posterior to the normal DM, as a response to different types of diseases including ... clude clusters of vesicles or blister-like lesions, band-shaped irregularities, or more extensive irregularity involving the en- tire DM and the variable ...

  15. L450W and Q455K Col8a2 knock-in mouse models of Fuchs endothelial corneal dystrophy show distinct phenotypes and evidence for altered autophagy.

    Science.gov (United States)

    Meng, Huan; Matthaei, Mario; Ramanan, Narendrakumar; Grebe, Rhonda; Chakravarti, Shukti; Speck, Caroline L; Kimos, Martha; Vij, Neeraj; Eberhart, Charles G; Jun, Albert S

    2013-03-28

    We compared the cellular phenotypes and studied the role of autophagy in the pathogenesis of Fuchs endothelial corneal dystrophy (FECD) using two α2 collagen VIII (Col8a2) knock-in mouse models and human FECD tissues. In vivo corneal endothelial cell (CEC) counts and morphology were analyzed by clinical confocal microscopy. Ultrastructural analysis of CECs was performed by transmission electron microscopy. Real-time PCR and Western blotting were performed using total RNA, and protein extracted from mouse CECs and human CECs obtained from FECD and autopsy patients. Both Col8a2 mouse models exhibited hallmarks of FECD; however, the Col8a2(L450W/L450W) mice exhibited a milder phenotype compared to the Col8a2(Q455K/Q455K) mice. Both models exhibited upregulation of the unfolded protein response (UPR) as evidenced by dilated rough endoplasmic reticulum (RER), and upregulation of UPR-associated genes and proteins. Real-time PCR of Col8a2(L450W/L450W) and Col8a2(Q455K/Q455K) CECs at 40 weeks revealed a 2.1-fold (P human FECD endothelium revealed a 10.4-fold upregulation of DRAM1 (P human FECD endothelial cells suggested a role for altered autophagy in this disease.

  16. Corneal Diseases in Children in The Gambia | Onabolu | Nigerian ...

    African Journals Online (AJOL)

    trauma in 23 (32.4%) vernal kerato-conjunctivitis in 16 (22.54%), congenital eye diseases in 12 (16.9%), corneal infections in 12 (16.9%), corneal scarring from unknown causes in 5 (7.04%) and corneal dystrophy/degenerations in 3 patients. Ten patients (14%) became bilaterally blind while 22 patients (31%) suffered ...

  17. Corneal Laceration

    Medline Plus

    Full Text Available ... Corneal Laceration? Corneal Laceration Diagnosis Corneal Laceration Treatment What Is Corneal Laceration? Leer en Español: ¿Qué Es una Laceración de la Córnea? Written By: Daniel ...

  18. Contribuição da tomografia de coerência óptica de 820 nm na distrofia granular corneana: relato de caso Contribution of 820 nm optical coherence tomography in granular corneal dystrophy: case report

    Directory of Open Access Journals (Sweden)

    Beatriz de Abreu Fiuza Gomes

    2008-10-01

    Full Text Available O objetivo deste trabalho é correlacionar os dados clínicos de um caso de distrofia granular com o exame de tomografia de coerência óptica (OCT de 820 nm de modo a determinar o nível intra-estromal dos depósitos corneanos. É relatado um caso de distrofia granular em paciente de 38 anos do sexo masculino. Foi realizado exame oftalmológico completo e OCT para documentação do caso. O aparelho utilizado foi o Stratus OCT III, originalmente desenvolvido para avaliação do segmento posterior. A acuidade visual corrigida foi de 20/50 em ambos os olhos. À biomicroscopia, foram observadas opacidades em "floco de neve" em diferentes níveis do estroma corneano bilateral e de forma simétrica. A avaliação pelo OCT revelou múltiplos pontos hiperreflectivos nas camadas superficiais e profundas do estroma. Foi possível acessar a anatomia morfológica da córnea através de cortes seriados realizados pelo Stratus OCT III e confirmar a profundidade dos depósitos corneanos.The purpose of this case report is to correlate clinical features of granular dystrophy using 820 nm optical coherence tomography (OCT in order to determine the depth of intrastromal corneal deposits. We report a case of a 38-year-old man with granular dystrophy. After complete ophthalmologic examination the patient was submitted to OCT, using the Stratus OCT III, originally proposed for studying disorders of the posterior segment. Best-corrected visual acuity was 20/50 in both eyes. Biomicroscopy revealed bilateral "snowflake"opacities in different levels of the stromal layer. OCT disclosed multiple hiperreflective dots in anterior and deeper stromal layers. It was possible to assess the morphology of the cornea and to determine the depth of the corneal deposits with serial cross-sectional OCT scans using the Stratus OCT III.

  19. Muscular Dystrophy

    Science.gov (United States)

    ... Devices The Search for a Cure en español Distrofia muscular About MD Muscular dystrophy (MD) is a genetic ... muscles and cause different degrees of muscle weakness. Duchenne muscular dystrophy is the most common and the most ...

  20. Muscular Dystrophy

    Science.gov (United States)

    Muscular dystrophy (MD) is a group of more than 30 inherited diseases. They all cause muscle weakness and ... ability to walk. There is no cure for muscular dystrophy. Treatments can help with the symptoms and prevent ...

  1. CORNEAL BLINDNESS AND XENOTRANSPLANTATION

    Science.gov (United States)

    Lamm, Vladimir; Hara, Hidetaka; Mammen, Alex; Dhaliwal, Deepinder; Cooper, David K.C.

    2014-01-01

    Approximately 39 million people are blind worldwide, with an estimated 285 million visually impaired. The developing world shoulders 90% of the world’s blindness, with 80% of causative diseases being preventable or treatable. Blindness has a major detrimental impact on the patient, community, and healthcare spending. Corneal diseases are significant causes of blindness, affecting at least 4 million people worldwide. The prevalence of corneal disease varies among parts of the world. Trachoma, for instance, is the second leading cause of blindness in Africa, after cataracts, but is rarely found today in developed nations. When preventive strategies have failed, corneal transplantation is the most effective treatment for advanced corneal disease. The major surgical techniques for corneal transplantation include penetrating keratoplasty (PK), anterior lamellar keratoplasty (ALK), and endothelial keratoplasty (EK). Indications for corneal transplantation vary among countries, with Fuchs’ dystrophy being the leading indication in the U.S. and keratoconus in Australia. With the exception of the US, where EK will soon overtake PK as the most common surgical procedure, PK is the overwhelming procedure of choice. Success using corneal grafts in developing nations, such as Nepal, demonstrates the feasibility of corneal transplantation on a global scale. The number of suitable corneas from deceased human donors that becomes available will never be sufficient, and so research into various alternatives, e.g., stem cells, amniotic membrane transplantation, synthetic and biosynthetic corneas, and xenotransplantation, is progressing. While each of these has potential, we suggest that xenotransplantation holds the greatest potential for a corneal replacement. With the increasing availability of genetically-engineered pigs, pig corneas may alleviate the global shortage of corneas in the near future. PMID:25268248

  2. Clinical applications of corneal confocal microscopy

    Directory of Open Access Journals (Sweden)

    Mitra Tavakoli

    2008-06-01

    Full Text Available Mitra Tavakoli1, Parwez Hossain2, Rayaz A Malik11Division of Cardiovascular Medicine, University of Manchester and Manchester Royal Infirmary, Manchester, UK; 2University of Southampton, Southampton Eye Unit, Southampton General Hospital, Southampton, UKAbstract: Corneal confocal microscopy is a novel clinical technique for the study of corneal cellular structure. It provides images which are comparable to in-vitro histochemical techniques delineating corneal epithelium, Bowman’s layer, stroma, Descemet’s membrane and the corneal endothelium. Because, corneal confocal microscopy is a non invasive technique for in vivo imaging of the living cornea it has huge clinical potential to investigate numerous corneal diseases. Thus far it has been used in the detection and management of pathologic and infectious conditions, corneal dystrophies and ecstasies, monitoring contact lens induced corneal changes and for pre and post surgical evaluation (PRK, LASIK and LASEK, flap evaluations and Radial Keratotomy, and penetrating keratoplasty. Most recently it has been used as a surrogate for peripheral nerve damage in a variety of peripheral neuropathies and may have potential in acting as a surrogate marker for endothelial abnormalities.Keywords: corneal confocal microscopy, cornea, infective keratitis, corneal dystrophy, neuropathy

  3. Corneal Ulcer

    Science.gov (United States)

    ... Español Eye Health / Eye Health A-Z Corneal Ulcer Sections What Is a Corneal Ulcer? Corneal Ulcer ... Diagnosis Corneal Ulcer Treatment What Is a Corneal Ulcer? Leer en Español: ¿Qué es una Úlcera de ...

  4. Fuchs' Dystrophy

    Science.gov (United States)

    ... and can cause your vision to gradually worsen over years. But most people with Fuchs' dystrophy don't develop symptoms until they reach their 50s or 60s. Some medications and self-care steps may help relieve your Fuchs' dystrophy ...

  5. Muscular Dystrophy

    Science.gov (United States)

    ... Inheritance patterns Muscular dystrophy Symptoms & causes Diagnosis & treatment Advertisement Mayo Clinic does not endorse companies or products. ... a Job Site Map About This Site Twitter Facebook Google YouTube Pinterest Mayo Clinic is a not- ...

  6. Phototherapeutic Keratectomy Outcomes in Superficial Corneal Opacities

    Directory of Open Access Journals (Sweden)

    Khalid Al Arfaj

    2011-01-01

    Full Text Available Purpose Compare the effectiveness of Phototherapeutic keratectomy (PTK in treatment corneal dystrophies versus superficial corneal scars: visual outcomes, recurrence rate and safety profile. Methods PTK was performed in 51 eyes of 51 patients. Data regarding the indications for PTK, ablation depth, symptomatic relief, pre-and postoperative best spectacle-corrected visual acuity (BSCVA, spherical equivalent changes, recurrence and complications were analyzed. The indications for PTK in our study were classified into two categories – group A: patients with corneal dystrophies (n = 23 and the other group B (n = 28 with other indications. Results The average age of the patients was 47 years (±16.4. The mean follow up period was 15.16 months (±10.01 months. Post operatively, there were no significant complications. While the overall BSCVA in the patients improved from 20/41 (0.484 to 20/32 (0.645, group A showed improvement from 20/35 (0.561 to 20/29 (0.687, as compared to group B in which BSCVA improved from 20/47 (0.421 to 20/33 (0.611. The most common indication in group A was granular corneal dystrophy (n = 10 and the most common indication in group B was post traumatic/infectious corneal scar or opacity (n = 10. Eighty-six percent (n = 44 of all patients had alleviation of symptoms. Recurrence of symptoms was seen in 3 eyes of recurrent corneal erosions which required retreatment. Conclusion PTK is a safe and effective procedure. The outcome of this study suggests that PTK improves BSCVA. PTK appears to improve ocular surface health. Furthermore, PTK can be recommended to most patients with corneal dystrophies as a treatment modality prior to other more invasive procedure (viz. penetrating keratoplasty.

  7. Corneal Laceration

    Medline Plus

    Full Text Available ... or apply pressure to eye avoid giving aspirin, ibuprofen or other non-steroidal, anti-inflammatory drugs. These ... lost sight from a corneal scar as a child. Now that I’m older, will a corneal ...

  8. Corneal Laceration

    Medline Plus

    Full Text Available ... rub or apply pressure to eye avoid giving aspirin, ibuprofen or other non-steroidal, anti-inflammatory drugs. ... lost sight from a corneal scar as a child. Now that I’m older, will a corneal ...

  9. Corneal Laceration

    Medline Plus

    Full Text Available ... Now that I’m older, will a corneal transplant help me? May 15, 2015 Why Do My ... May Have A Future Alternative to Full Corneal Transplantation Nov 29, 2016 Combating Eye Injuries from Air ...

  10. Unusual early recurrence of granular dystrophy after deep anterior lamellar keratoplasty: case report

    Directory of Open Access Journals (Sweden)

    Paolo Rama

    2013-04-01

    Full Text Available We report an atypical case of granular corneal dystrophy recurrence after deep anterior lamellar keratoplasty. We describe clinical features, histopathological analysis of the lamellar graft specimen and DNA analysis results. The slit-lamp examination and histopathological findings from the graft specimen indicated the confinement of the typical deposits of granular corneal dystrophy deep in the graft interface area. This localization is atypical, since in most cases recurrences in grafts tend to be initially superficial and situated in the epithelial or subepithelial corneal layers. Molecular genetic analysis revealed an already described mutation and a new intronic variant. The unusual localization and timing of this recurrence of granular corneal dystrophy after deep anterior lamellar keratoplasty suggests that corneal stromal keratocytes may play a role in the formation of granular deposits.

  11. Muscular Dystrophy

    Science.gov (United States)

    ... Tawil R, Van Der Maarel SM. Facioscapulohumeral muscular dystrophy . Muscle Nerve. Jul 2006;34(1):1-15. Congenital ( ... affected? Throat References: 1. Emery AEH. The muscular ... V. Prevalence of genetic muscle disease in Northern England: in-depth analysis of ...

  12. Learning about Myotonic Dystrophy

    Science.gov (United States)

    ... and other body systems. People who have myotonic dystrophy have muscle wasting and weakness in their lower legs, hands, ... symptoms of myotonic dystrophy? People who have myotonic dystrophy have progressive muscle wasting and weakness beginning in their 20's or ...

  13. Genetics Home Reference: congenital stromal corneal dystrophy

    Science.gov (United States)

    ... ligaments. In the cornea, well-organized bundles of collagen make the cornea transparent. Decorin ensures that collagen fibrils in the cornea are uniformly sized and regularly spaced. Mutations in ...

  14. [Corneal biomechanics].

    Science.gov (United States)

    Torres, R M; Merayo-Lloves, J; Jaramillo, M A; Galvis, V

    2005-04-01

    To review the corneal biomechanic concepts and to analyse, clarify and understand their relevance in refractive surgery. A literature review has been done using different databases. Corneal biomechanic concepts are not new and are applied implicitly in numerous surgical procedures. Their origin is related to tonometry studies, but they gained in popularity when they were linked to the treatment of keratoconus, a pathology in which the mechanical properties of the cornea are altered. Factors determining corneal stability were thus defined. Corneal biomechanics have also been used following refractive surgery to study post-operative keratectasia and to improve ablation patterns, which ignores the corneal response. The new ablation systems need to include the biomechanical factors, which motivate research conducted in physical-mathematical models and in corneal wound healing, improving our knowledge about the corneal biomechanical response. The corneal biomechanic concepts have gained in popularity with the advent of refractive surgery, although they did exist previously. Their relevance is linked to improvements in the ablation systems used in an attempt to obtain more accurate and reliable results.

  15. Corneal Laceration

    Medline Plus

    Full Text Available ... Tips & Prevention News Ask an Ophthalmologist Patient Stories Español Eye Health / Eye Health A-Z Corneal Laceration ... Laceration Treatment What Is Corneal Laceration? Leer en Español: ¿Qué Es una Laceración de la Córnea? Written ...

  16. Corneal Laceration

    Medline Plus

    Full Text Available ... Health Find an Ophthalmologist Academy Store Eye Health A-Z Symptoms Glasses & Contacts Tips & Prevention News Ask ... Ophthalmologist Patient Stories Español Eye Health / Eye Health A-Z Corneal Laceration Sections What Is Corneal Laceration? ...

  17. Corneal topography.

    Science.gov (United States)

    Seitz, B; Behrens, A; Langenbucher, A

    1997-08-01

    In the review period, limitations of individual Placido disk-based topography systems have been studied and new principles, such as raster photogrammetry, pancorneal slit topography, laser holographic interferometry, and confocal laser scanning topography, have been introduced for laboratory or clinical work. Both Fourier analysis and Zernike decomposition of topographic height data seem to be powerful new tools for cross-sectional analysis of complex topographic corneal images, such as after cataract surgery, penetrating keratoplasty, and refractive surgery, as well as for longitudinal studies of corneal changes, such as in schoolchildren. Subdividing into rational optical components may improve consistency and standardization of topography data from different systems. Topography-based flying-spot-mode excimer laser photoablation after Zernike decomposition of topography height data has been proposed for correction of irregular corneal astigmatism. Topography-based central power measurements are of increasing value for intraocular lens power calculation before cataract surgery in eyes with irregular corneal surfaces, such as in keratoconus or after refractive surgery procedures. Quantitative and qualitative classification of corneal topography maps after corneal transplantation following conventional mechanical and nonmechanical trephination or after refractive surgery may lead to a better understanding of impaired visual acuity despite a clear graft or despite significantly reduced ametropia or corneal astigmatism.

  18. Novel CYP4V2 gene mutation in a Mexican patient with Bietti's crystalline corneoretinal dystrophy.

    Science.gov (United States)

    Zenteno, Juan C; Ayala-Ramirez, Raul; Graue-Wiechers, Federico

    2008-04-01

    To report the clinical and genetic analysis of a Mexican female patient with a sporadic Bietti's crystalline corneoretinal dystrophy. Ophthalmological examination included best-corrected visual acuity, slit lamp examination, applanation tonometry, fundus photography, fluorescein retinal angiography, Goldmann kinetic perimetry, corneal rotating Scheimpflug imaging, and anterior segment optical coherence tomography (Visante OCT). Genetic analysis included PCR amplification and direct nucleotide sequencing of the entire CYP4V2 gene in DNA from the propositus and her relatives. A late-stage retinal dystrophy was established in the patient. No retinal or corneal crystalline deposits were evident during clinical evaluation. Retrospective analysis of fundus imaging disclosed the presence of retinal crystalline deposits, suggesting the diagnosis of Bietti's crystalline corneoretinal dystrophy. Molecular analysis of the CYP4V2 gene revealed the presence of a novel C to T mutation at nucleotide position 974 (exon 7), predicting a threonine to isoleucine replacement at amino acid position 325. Corneal deposits were not seen by biomicroscopy, corneal OCT, or specular microscopy but were evidenced by means of the corneal rotating Scheimpflug imaging. Our results expand the allelic heterogeneity of Bietti's crystalline corneoretinal dystrophy. This is the first patient of Latin-American origin in which a molecular analysis of the disease has been performed. Our results suggest that the use of corneal rotating Scheimpflug imaging can evidence corneal deposits that are not apparent by other methods.

  19. [Posterior polymorphous dystrophy, case report and literature review].

    Science.gov (United States)

    Mendoza-Adam, G; Hernandez-Camarena, J C; Valdez-García, J E

    2015-09-01

    Posterior Polymorphous Dystrophy (DPP) is a rare posterior corneal dystrophy that is genetically transmitted as autosomal dominant. Corneal structures affected in this dystrophy are Descemet membrane and the endothelium. A case is presented on a 47 years old woman with no relevant history, with typical findings of DPP (vesicular and band lesions at the endothelium and posterior Descemet). To our knowledge there are no reported cases of DPP in Latin-American patients in the literature. The clinical manifestations in our patient were found to be very similar to the cases reported in other populations. Copyright © 2014 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  20. Biomechanical relationships between the corneal endothelium and Descemet's membrane.

    Science.gov (United States)

    Ali, Maryam; Raghunathan, VijayKrishna; Li, Jennifer Y; Murphy, Christopher J; Thomasy, Sara M

    2016-11-01

    The posterior face of the cornea consists of the corneal endothelium, a monolayer of cuboidal cells that secrete and attach to Descemet's membrane, an exaggerated basement membrane. Dysfunction of the endothelium compromises the barrier and pump functions of this layer that maintain corneal deturgesence. A large number of corneal endothelial dystrophies feature irregularities in Descemet's membrane, suggesting that cells create and respond to the biophysical signals offered by their underlying matrix. This review provides an overview of the bidirectional relationship between Descemet's membrane and the corneal endothelium. Several experimental methods have characterized a richly topographic and compliant biophysical microenvironment presented by the posterior surface of Descemet's membrane, as well as the ultrastructure and composition of the membrane as it builds during a lifetime. We highlight the signaling pathways involved in the mechanotransduction of biophysical cues that influence cell behavior. We present the specific example of Fuchs' corneal endothelial dystrophy as a condition in which a dysregulated Descemet's membrane may influence the progression of disease. Finally, we discuss some disease models and regenerative strategies that may facilitate improved treatments for corneal dystrophies. Copyright © 2016 Elsevier Ltd. All rights reserved.

  1. Corneal Laceration

    Medline Plus

    Full Text Available ... by something sharp flying into the eye. It can also be caused by something striking the eye ... If the corneal laceration is deep enough it can cause a full thickness laceration. This is when ...

  2. Corneal Laceration

    Medline Plus

    Full Text Available ... itself. A corneal laceration is a very serious injury and requires immediate medical attention to avoid severe ... 27, 2015 Dark Spot in Vision After Blunt Trauma Dec 21, 2014 Pain a Year After Eyelid ...

  3. Corneal Laceration

    Medline Plus

    Full Text Available ... the blood and may increase bleeding after you have finished protecting the eye, see a physician immediately ... Jun 30, 2017 People with Advanced Keratoconus May Have A Future Alternative to Full Corneal Transplantation Nov ...

  4. Corneal Laceration

    Medline Plus

    Full Text Available ... People with Advanced Keratoconus May Have A Future Alternative to Full Corneal Transplantation Nov 29, 2016 Combating Eye Injuries from ... of Service For Advertisers For Media Ophthalmology Job Center © American Academy of Ophthalmology 2017 ...

  5. Corneal Laceration

    Medline Plus

    Full Text Available ... By: Devin A Harrison MD Sep. 01, 2017 The cornea is the clear front window of the eye . A corneal laceration is a cut on the cornea. It is usually caused by something sharp ...

  6. Corneal Laceration

    Medline Plus

    Full Text Available ... itself. A corneal laceration is a very serious injury and requires immediate medical attention to avoid severe ... Ask an Ophthalmologist Answers Did my traumatic brain injury cause early cataracts? Jan 21, 2018 Did I ...

  7. Corneal Laceration

    Medline Plus

    Full Text Available ... caused by something striking the eye with significant force, like a metallic hand tool. A corneal laceration ... and preserving your vision. Privacy Policy Related Top 5 Eye Health Stories of 2017 Dec 21, 2017 ...

  8. Corneal Laceration

    Medline Plus

    Full Text Available ... itself. A corneal laceration is a very serious injury and requires immediate medical attention to avoid severe ... Dangerous for Your Eyes Sep 20, 2017 Eye Injuries from Laundry Packets On the Rise Jun 30, ...

  9. Deep corneal stromal opacities associated with long term contact lens wear.

    OpenAIRE

    Pimenides, D; Steele, C F; McGhee, C N; Bryce, I G

    1996-01-01

    BACKGROUND: One male and three female long term contact lens wearers (mean age 30.3 years; range 26-33) demonstrated unusual deep corneal stromal opacities which were predominantly just anterior to Descemet's membrane. None had any history of corneal dystrophy. These opacities were more common centrally, but were also identified in the corneal periphery. METHODS: All patients underwent routine ophthalmic examinations and, where appropriate, slit-lamp photography and specular microscopy. RESUL...

  10. Duchenne muscular dystrophy

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000705.htm Duchenne muscular dystrophy To use the sharing features on this page, please enable JavaScript. Duchenne muscular dystrophy is an inherited disorder. It involves muscle weakness , ...

  11. Corneal Transplantation

    DEFF Research Database (Denmark)

    Hjortdal, Jesper Østergaard

    Corneal transplantation has been performed for more than 100 years. Until 15 years ago the state-of-the art type of transplantation was penetrating keratoplasty, but since the start of this millennium, newly designed surgical techniques have developed considerably. Today, the vast majority...... with less risk of rejection episodes. Besides covering updated chapters on penetrating keratoplasty, and anterior and posterior lamellar procedures, this textbook also gives a thorough overview of the history of corneal transplantation and a detailed presentation of the microstructural components....... Economic considerations on cost and benefi t of medical treatment and surgical procedures are today an integrated part of the health system in many countries, and a chapter covers these aspects of corneal transplantation. This textbook is aimed at presenting an updated review of the new techniques...

  12. Corneal Intelligence

    African Journals Online (AJOL)

    Murdoch3

    the damping effect of the tissue to an applied force) or corneal compliance being the major risk factor. Figure 1. The percentage of participants in the observation group who developed primary open-angle glaucoma (median follow-up, 72 months) ...

  13. Corneal ulcers

    African Journals Online (AJOL)

    visual acuity chart. • fluorescein strips. • topical anaesthetic eye drops. • direct ophthalmoscope. Examination. Assessing vision with a Snellen visual acuity chart gives a clue as to the extent of the problem (e.g. a corneal abrasion with good vision is unlikely to require specialist intervention). Each eye should be tested ...

  14. Corneal Intelligence

    African Journals Online (AJOL)

    Murdoch3

    Corneal Intelligence. Ian Murdoch. Institute of Ophthalmology, Bath Street, London. In 2002, the ocular hypertension treatment study (OHTS) published their results. This study had taken 1636 ocular hypertensives. 1, 2. (IOP 24-32mmHg) and randomized them to receive therapy or no therapy. The primary outcome of the ...

  15. Genetics Home Reference: myotonic dystrophy

    Science.gov (United States)

    ... SJ. Myotonic dystrophy: emerging mechanisms for DM1 and DM2. Biochim Biophys Acta. 2007 Feb;1772(2):195- ... Moxley R. 140th ENMC International Workshop: Myotonic Dystrophy DM2/PROMM and other myotonic dystrophies with guidelines on ...

  16. "Tuck In" Lamellar Keratoplasty (TILK) for corneal ectasias involving corneal periphery.

    Science.gov (United States)

    Kaushal, S; Jhanji, V; Sharma, N; Tandon, R; Titiyal, J S; Vajpayee, R B

    2008-02-01

    Evaluation of a new surgical technique for the management of corneal ectasia with peripheral corneal involvement. Twelve eyes of 12 patients with corneal ectasias and peripheral corneal thinning requiring surgical intervention, including eight patients with combined keratoconus and PMD and four patients of keratoglobus, were enrolled for the study in a tertiary care hospital. All patients were contact lens intolerant and had a best corrected visual acuity (BCVA) Keratoplasty (TILK) that included a central lamellar keratoplasty with intrastromal tucking of the peripheral flange was performed in these patients. The main outcome measures analysed were uncorrected visual acuity (UCVA), BCVA, keratometry, refractive status and time for epithelial healing. At the last follow-up (mean: 1.7 years (range 13-48 months)), six patients (50.0%) had BCVA >/=20/60, and all patients had BCVA >/=20/80. The mean keratometry decreased from 57.54 (SD 6.89) D preoperatively to 46.36 (2.39) D (p = 0.003), and the mean spherical equivalent (SEQ) refractive error decreased from -7.8 (4.6) D preoperatively to 1.23 (1.88) D (p = 0.007). A significant decrease was also seen in mean refractive astigmatism which decreased from 5.93 (3.06) D preoperatively to 3.23 (1.14) D (p = 0.037). Our technique of TILK is an effective surgical modality for the management of ectatic corneal dystrophies with peripheral corneal thinning.

  17. Effect of Donor and Recipient Factors on Corneal Graft Rejection

    Science.gov (United States)

    Stulting, R. Doyle; Sugar, Alan; Beck, Roy; Belin, Michael; Dontchev, Mariya; Feder, Robert S.; Gal, Robin L.; Holland, Edward J.; Kollman, Craig; Mannis, Mark J.; Price, Francis; Stark, Walter; Verdier, David D.

    2014-01-01

    Purpose To assess the relationship between donor and recipient factors and corneal allograft rejection in eyes that underwent penetrating keratoplasty (PK) in the Cornea Donor Study. Methods 1090 subjects undergoing corneal transplantation for a moderate risk condition (principally Fuchs’ dystrophy or pseudophakic corneal edema) were followed for up to 5 years. Associations of baseline recipient and donor factors with the occurrence of a probable or definite rejection event were assessed in univariate and multivariate proportional hazards models. Results Eyes with pseudophakic or aphakic corneal edema (N=369) were more likely to experience a rejection event than eyes with Fuchs’ dystrophy (N=676) (34% ± 6% versus 22% ± 4%; hazard ratio = 1.56; 95% confidence interval 1.21 to 2.03). Among eyes with Fuchs’dystrophy, a higher probability of a rejection event was observed in phakic post-transplant eyes compared with eyes that underwent cataract extraction with or without intraocular lens implantation during PK (29% vs. 19%; hazard ratio = 0.54; 95% confidence interval 0.36 to 0.82). Female recipients had a higher probability of a rejection event than males (29% vs. 21%; hazard ratio=1.42; 95% confidence interval 1.08 to 1.87), after controlling for the effect of preoperative diagnosis and lens status. Donor age and donor recipient ABO compatibility were not associated with rejection. Conclusions There was a substantially higher graft rejection rate in eyes with pseudophakic or aphakic corneal edema compared with eyes with Fuchs’ dystrophy. Female recipients were more likely to have a rejection event than males. Graft rejection was not associated with donor age. PMID:22488114

  18. Idiopathic 20-nail dystrophy

    DEFF Research Database (Denmark)

    Larsen, Camilla; Bygum, Anette

    2016-01-01

    Twenty-nail dystrophy is a rare disease, typically with all 20 nails affected, which normally occurs as an idiopathic condition in childhood but can be linked to other diseases. We report a case of a 7-year-old girl with a 3-year history of 20-nail dystrophy and no associated diseases or family...... history of skin or nail diseases. She was followed and treated conservatively and, after 6 years of follow-up, we found a marked improvement and almost full resolution of her nail dystrophy. The aim of this report is to show that idiopathic 20-nail dystrophy in children is a self-limiting condition...

  19. Management of acute hydrops with perforation in a patient with keratoconus and cone dystrophy: case report and literature review.

    Science.gov (United States)

    Yeh, Steven; Smith, Janine A

    2008-10-01

    To describe a patient with cone dystrophy who presented with acute hydrops and perforation, leading to the diagnosis of keratoconus. Case report and literature review. A 21 -year-old male patient with a history of cone dystrophy presented with a flat anterior chamber, diffuse corneal stromal edema with an intrastromal cleft, and ruptured Descemet membrane, findings consistent with acute hydrops with corneal perforation. After bandage contact lens placement and instillation of a cycloplegic agent, the anterior chamber reformed within 24 hours. Over the next week, conservative management with a bandage lens, pressure patching, topical fluoroquinolone antibiotic, and topical cycloplegic led to the reformation and maintenance of anterior chamber stability. Corneal topography of the unaffected eye showed global corneal thinning and steep sim K readings suspicious for early keratoconus. Although the association between keratoconus and cone dystrophy is extremely rare, our patient's vision-threatening complication of acute hydrops with corneal perforation highlights the importance of corneal evaluation including topography in cone dystrophy. Conservative management was successful in the restoration of anatomic integrity in this situation.

  20. Meaning of Muscular Dystrophy

    Science.gov (United States)

    ... MD Living With MD en español Qué significa distrofia muscular What Is Muscular Dystrophy? Muscular dystrophy (say: MUS- ... blood test if a kid has Becker or Duchenne MD. Or the doctor might take a small piece of the muscle and look at it under a microscope to ...

  1. Facioscapulohumeral muscular dystrophy

    Science.gov (United States)

    ... on this page, please enable JavaScript. Facioscapulohumeral muscular dystrophy is muscle weakness and loss of muscle tissue that gets ... to have children. Alternative Names Landouzy-Dejerine muscular dystrophy Images ... Superficial anterior muscles References Preston DC, Shapiro BE. Proximal, distal, and ...

  2. Global Survey of Corneal Transplantation and Eye Banking.

    Science.gov (United States)

    Gain, Philippe; Jullienne, Rémy; He, Zhiguo; Aldossary, Mansour; Acquart, Sophie; Cognasse, Fabrice; Thuret, Gilles

    2016-02-01

    Corneal transplantation restores visual function when visual impairment caused by a corneal disease becomes too severe. It is considered the world's most frequent type of transplantation, but, to our knowledge, there are no exhaustive data allowing measurement of supply and demand, although such data are essential in defining local, national, and global strategies to fight corneal blindness. To describe the worldwide situation of corneal transplantation supply and demand. Data were collected between August 2012 and August 2013 from a systematic review of published literature in parallel with national and international reports on corneal transplantation and eye banking. In a second step, eye bank staff and/or corneal surgeons were interviewed on their local activities. Interviews were performed during international ophthalmology or eye-banking congresses or by telephone or email. Countries' national supply/demand status was classified using a 7-grade system. Data were collected from 148 countries. Corneal transplantation and corneal procurements per capita in each country. In 2012, we identified 184,576 corneal transplants performed in 116 countries. These were procured from 283,530 corneas and stored in 742 eye banks. The top indications were Fuchs dystrophy (39% of all corneal transplants performed), a primary corneal edema mostly affecting elderly individuals; keratoconus (27%), a corneal disease that slowly deforms the cornea in young people; and sequellae of infectious keratitis (20%). The United States, with 199.10-6 corneal transplants per capita, had the highest transplantation rate, followed by Lebanon (122.10-6) and Canada (117.10-6), while the median of the 116 transplanting countries was 19.10-6. Corneas were procured in only 82 countries. Only the United States and Sri Lanka exported large numbers of donor corneas. About 53% of the world's population had no access to corneal transplantation. Our survey globally quantified the considerable shortage of

  3. Long-Term Keratometric Changes after Penetrating Keratoplasty for Keratoconus and Fuchs Endothelial Dystrophy

    Science.gov (United States)

    Raecker, Matthew E.; Erie, Jay C.; Patel, Sanjay V.; Bourne, William M.

    2008-01-01

    Purpose To compare long-term keratometric changes after penetrating keratoplasty (PK) for keratoconus and Fuchs endothelial dystrophy. Methods We retrospectively analyzed 168 corneas after PK for keratoconus (85 eyes of 63 subjects) and Fuchs dystrophy (83 eyes of 60 subjects). Patients were examined after final suture removal at 12 months after PK to 30 years after surgery. Operations were performed by one surgeon using the same suturing technique in all cases. Eyes were excluded from further analysis after regrafting or after relaxing incisions. Mean keratometric corneal power and astigmatism were measured by manual keratometry. Data were assessed by using generalized estimating equation models to determine change over time. Results Mean keratometric corneal power and keratometric astigmatism increased through 30 years after PK for keratoconus (P astigmatism after PK in keratoconus patients differed from the change in Fuchs dystrophy patients only at 10 or more years after PK (P = .002 and P = .003). Conclusions Corneal curvature and regular astigmatism increase progressively after PK for keratoconus but remain stable after PK for Fuchs dystrophy. This keratometric instability after PK for keratoconus may lead to delayed corneal ectasia. PMID:19277234

  4. Influence of corneal astigmatism, corneal curvature and meridional differences on corneal hysteresis and corneal resistance factor.

    Science.gov (United States)

    Wong, Yin-zhi; Lam, Andrew K C

    2011-09-01

    This study investigated the influence of corneal astigmatism, corneal curvature and meridional differences on corneal hysteresis (CH) and the corneal resistance factor (CRF) in a group of normal Chinese persons. Ninety-five participants were recruited and data from the eye with higher corneal astigmatism were analysed. The anterior corneal curvature was measured by corneal topography. The Goldmann-correlated intraocular pressure (IOPg), corneal-compensated intraocular pressure (IOPcc), CH and CRF at different meridians (default horizontal position, 10°, 20° and 30° along the superotemporal and inferonasal meridians) were obtained from an ocular response analyser. The corneal powers at these specific meridians also were calculated. At the default position, the IOPg and CRF had weak correlations with corneal astigmatism, while the IOPcc and CH were not significantly correlated with corneal astigmatism. Both the IOPg and IOPcc were measured significantly higher at the default position. The CH and CRF were lower at the default position but the difference in the CRF from obliquity could not reach statistical significance. The CH was not significantly correlated with the corneal power at all meridians. The CRF correlated with the corneal power only at 30° superotemporal. Corneal astigmatism and head tilt did not have much effect on the measurement of CH and the CRF, both of which were lowest along the horizontal meridian. Clinically, the difference was small. The influence of corneal power on CH and the CRF was minimal. © 2011 The Authors. Clinical and Experimental Optometry © 2011 Optometrists Association Australia.

  5. Study of light scattering using C-Quant® in patients with Fuchs' endothelial dystrophy: A pilot study.

    Science.gov (United States)

    Castaño-Martín, B; Gros-Otero, J; Martínez, J; Teus, M

    2017-11-01

    The purpose of this study was to determine the light scattering in patients with Fuchs' endothelial dystrophy without clinically significant corneal oedema, and evaluate its relationship with endothelial cell count, corneal thickness, and corneal biomechanical parameters. The values of light scattering were measured by C-Quant® (Oculus Optikgeräte GmbH, Germany) in 32 eyes of 17 patients diagnosed with Fuchs' endothelial dystrophy without clinically significant corneal oedema. Corneal biomechanical properties were determined using ORA (ocular response) and Corvis ST® (tonometry). A light scattering value outside the normal range was observed in 93.8% of eyes studied. No statistically significant association (P>.05) was found between the values of the measured light scattering by C-Quant® and endothelial count, pachymetry, or corneal biomechanical properties. In this study, changes were found in the values of light scattering values of patients with corneal Fuchs' endothelial dystrophy. This change does not appear to correlate significantly with disease severity. Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  6. Regulation of Corneal Stroma Extracellular Matrix Assembly

    Science.gov (United States)

    Chen, Shoujun; Mienaltowski, Michael J.; Birk, David E.

    2014-01-01

    The transparent cornea is the major refractive element of the eye. A finely controlled assembly of the stromal extracellular matrix is critical to corneal function, as well as in establishing the appropriate mechanical stability required to maintain corneal shape and curvature. In the stroma, homogeneous, small diameter collagen fibrils, regularly packed with a highly ordered hierarchical organization, are essential for function. This review focuses on corneal stroma assembly and the regulation of collagen fibrillogenesis. Corneal collagen fibrillogenesis involves multiple molecules interacting in sequential steps, as well as interactions between keratocytes and stroma matrix components. The stroma has the highest collagen V:I ratio in the body. Collagen V regulates the nucleation of protofibril assembly, thus controlling the number of fibrils and assembly of smaller diameter fibrils in the stroma. The corneal stroma is also enriched in small leucine-rich proteoglycans (SLRPs) that cooperate in a temporal and spatial manner to regulate linear and lateral collagen fibril growth. In addition, the fibril-associated collagens (FACITs) such as collagen XII and collagen XIV have roles in the regulation of fibril packing and inter-lamellar interactions. A communicating keratocyte network contributes to the overall and long-range regulation of stromal extracellular matrix assembly, by creating micro-domains where the sequential steps in stromal matrix assembly are controlled. Keratocytes control the synthesis of extracellular matrix components, which interact with the keratocytes dynamically to coordinate the regulatory steps into a cohesive process. Mutations or deficiencies in stromal regulatory molecules result in altered interactions and deficiencies in both transparency and refraction, leading to corneal stroma pathobiology such as stromal dystrophies, cornea plana and keratoconus. PMID:25819456

  7. Evaluation of Limb-Girdle Muscular Dystrophy

    Science.gov (United States)

    2014-03-06

    Becker Muscular Dystrophy; Limb-Girdle Muscular Dystrophy, Type 2A (Calpain-3 Deficiency); Limb-Girdle Muscular Dystrophy, Type 2B (Miyoshi Myopathy, Dysferlin Deficiency); Limb-Girdle Muscular Dystrophy, Type 2I (FKRP-deficiency)

  8. Changing Indications and Surgical Techniques for Corneal Transplantation Between 2004 and 2009 at a Tertiary Referral Center

    Science.gov (United States)

    Zare, Mohammad; Javadi, Mohammad A.; Einollahi, Bahram; Karimian, Farid; Rafie, Ali R. B.; Feizi, Sepehr; Azimzadeh, Ahmad

    2012-01-01

    Purpose: The aim of this study is to report the indications, techniques, and clinical outcomes of corneal transplantation and investigate any changing trends in surgical techniques over a 6 year period. Materials and Methods: Records of patients who had undergone any kind of corneal transplantation at Labbafinejad Medical Center, Tehran, Iran, from January 2004 to December 2009 were reviewed to determine the indications and types of corneal transplantation. Postoperative best-corrected visual acuity, refractive error, graft clarity, and complications were reported. Results: During this period, 1859 eyes of 1624 patients with a mean age of 41.3 ± 21.3 years underwent corneal transplantation. The most common indication was keratoconus (38.4%) followed by aphakic/pseudophakic bullous keratopathy (11.7%), previous failed grafts (10.6%), infectious corneal ulcers (10.1%), non-herpetic corneal scars (7.6%), trachoma keratopathy (4.7%), stromal corneal dystrophies (4.6%), post-herpetic corneal scar (3.7%), Fuchs’ endothelial dystrophy (0.8%), and congenital hereditary endothelial dystrophy (0.4%). Techniques of corneal transplantation included penetrating keratoplasty (PKP; 70.9%), deep anterior lamellar keratoplasty (DALK; 20.1%), conventional lamellar keratoplasty (LKP; 4.4%), and Descemet's stripping automated endothelial keratoplasty (DSAEK; 2.3%). Over the study period, there was a significant increase in the relative frequency of infectious corneal ulcers, failed grafts, and trachoma keratopathy. Additionally, a significant reduction was observed in PKP and LKP procedures, and volume of DALK and DSAEK increased significantly. At final follow-up, 69.0% of grafts were clear in the PKP group. This figure was 82.6%, 82.7%, and 97.6% in the DALK, LKP, and DSAEK groups, respectively. Conclusion: Keratoconus was the most common indication and PKP was the most prevalent technique used for corneal transplantation. However, significant changes in the indications and

  9. Spider dystrophy as an ocular manifestation of myotonic dystrophy.

    Science.gov (United States)

    Louprasong, Amber C; Light, Dennis J; Diller, Rebecca S

    2010-04-01

    Myotonic dystrophy is the most common adult-onset muscular dystrophy. It is an autosomal dominant inherited neuromuscular disease that is characterized by myotonia, muscle weakness, and atrophy. It affects multiple systems including skeletal muscular, gastrointestinal, cardiac, respiratory, central nervous, endocrine, and ocular. Ocular manifestations of myotonic dystrophy include cataract, ocular muscle changes, hypotony, and retinal pigmentary changes in the periphery or in the macula (known as pigment pattern dystrophy). This report presents and discusses the case of a pigmented pattern dystrophy known as spider dystrophy as an ocular manifestation of myotonic dystrophy. A 44-year-old man with myotonic dystrophy presented to the eye clinic for routine examination. Ocular history included previous bilateral cataract surgery and mild bilateral ptosis for the last "few years." Dilated fundus examination was remarkable for bilateral macular pigmentary changes in an irregular "spider"-shaped pattern. The patient was asymptomatic without decrease in vision or Amsler grid defects. Optical coherence tomography was normal. A retinal consult concurred with the diagnosis of spider dystrophy. Photo documentation was obtained, and the patient is being monitored annually. Pigmented pattern dystrophies, including spider dystrophy, have been associated with myotonic dystrophy. They are set apart from other retinal dystrophies because they rarely affect visual acuity, and the majority of patients are asymptomatic. Progression may lead to reduced vision and in rare cases choroidal neovascularization. Annual dilated examinations, photo documentation, optical coherence tomography, and home Amsler grid monitoring are recommended for follow-up care. Published by Elsevier Inc.

  10. In Vivo Confocal Microscopy of Corneal Nerves in Health and Disease.

    Science.gov (United States)

    Cruzat, Andrea; Qazi, Yureeda; Hamrah, Pedram

    2017-01-01

    In vivo confocal microscopy (IVCM) is becoming an indispensable tool for studying corneal physiology and disease. Enabling the dissection of corneal architecture at a cellular level, this technique offers fast and noninvasive in vivo imaging of the cornea with images comparable to those of ex vivo histochemical techniques. Corneal nerves bear substantial relevance to clinicians and scientists alike, given their pivotal roles in regulation of corneal sensation, maintenance of epithelial integrity, as well as proliferation and promotion of wound healing. Thus, IVCM offers a unique method to study corneal nerve alterations in a myriad of conditions, such as ocular and systemic diseases and following corneal surgery, without altering the tissue microenvironment. Of particular interest has been the correlation of corneal subbasal nerves to their function, which has been studied in normal eyes, contact lens wearers, and patients with keratoconus, infectious keratitis, corneal dystrophies, and neurotrophic keratopathy. Longitudinal studies have applied IVCM to investigate the effects of corneal surgery on nerves, demonstrating their regenerative capacity. IVCM is increasingly important in the diagnosis and management of systemic conditions such as peripheral diabetic neuropathy and, more recently, in ocular diseases. In this review, we outline the principles and applications of IVCM in the study of corneal nerves in various ocular and systemic diseases. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Comparison of confocal biomicroscopy and noncontact specular microscopy for evaluation of the corneal endothelium.

    Science.gov (United States)

    Hara, Makiko; Morishige, Naoyuki; Chikama, Tai-Ichiro; Nishida, Teruo

    2003-08-01

    To compare the clinical efficacy of confocal biomicroscopy with that of noncontact specular microscopy for the evaluation of the corneal endothelium. The corneal endothelium was examined in 14 normal subjects (28 eyes) and in 6 patients (11 eyes) with Fuchs corneal endothelial dystrophy using a noncontact specular microscope (SP-2000P, Topcon, Japan) and a confocal biomicroscope (ConfoScan, Tomey, Japan). The images and the calculated densities of corneal endothelial cells obtained by the 2 techniques were compared. For normal subjects, the images of corneal endothelial cells obtained by the 2 techniques were almost identical, although the density of these cells determined by confocal biomicroscopy (2916 +/- 334 cells/mm2) was slightly higher than that determined by noncontact specular microscopy (2765 +/- 323 cells/mm2). In contrast, whereas clear images of corneal endothelial cells, allowing the determination of cell density, were obtained for all 11 eyes of the patient group by confocal biomicroscopy, clear images were obtained for only 4 of these 11 eyes (36.4%) by noncontact specular microscopy. Both noncontact specular microscopy and confocal biomicroscopy revealed the shapes and number of endothelial cells in the normal cornea. However, for corneas with Fuchs dystrophy, clear images were obtained only by confocal biomicroscopy. Confocal biomicroscopy is thus an effective tool for evaluation of the diseased corneal endothelium.

  12. Targeted corneal transplantation.

    Science.gov (United States)

    Jhanji, Vishal; Mehta, Jod S; Sharma, Namrata; Sharma, Bhavana; Vajpayee, Rasik B

    2012-07-01

    Corneal transplantation surgery has moved from an era of conventional penetrating keratoplasty to selective replacement of the diseased corneal layer with complementary healthy donor corneal tissue. Anterior lamellar transplantation surgeries do not involve replacement of corneal endothelium, consequently eliminating the occurrence of endothelial rejection. Similarly, in diseases affecting the corneal endothelium, selective replacement with a lamellar lenticule bearing healthy endothelium provides better outcomes in terms of ocular surface, lesser astigmatism and quick visual recovery. In addition to the advantages of enhanced surgical outcomes, targeted corneal transplantation allows the use of one donor cornea for more than one recipient, thereby offering a viable solution to the problem of paucity of donor corneas. Evolving techniques of corneal transplantation have enabled better utilization of donor corneal tissue. Anterior lamellar as well as endothelial keratoplasty surgeries have become first-choice surgeries in appropriately selected cases. This review briefly discusses some of these novel surgical techniques. A better understanding of targeted corneal transplantation would lead to adaptation of the concept of component corneal surgery. This would further enable the corneal surgeons to circumvent the problem of donor corneal shortage especially in the developing world.

  13. Corneal collagen crosslinking for keratoconus. A review

    Directory of Open Access Journals (Sweden)

    M. M. Bikbov

    2014-01-01

    Full Text Available Photochemical crosslinking is widely applied in ophthalmology. Its biochemical effect is due to the release of singlet oxygen that promotes anaerobic photochemical reaction. Keratoconus is one of the most common corneal ectasia affecting 1 in 250 to 250 000 persons. Currently, the rate of iatrogenic ectasia following eximer laser refractive surgery increases due to biomechanical weakening of the cornea. Morphologically and biochemically, ectasia is characterized by corneal layers thinning, contact between the stroma and epithelium resulting from Bowman’s membrane rupture, chromatin fragmentation in keratocyte nuclei, phagocytosis, abnormal staining and arrangement of collagen fibers, enzyme system disorders, and keratocyte apoptosis. In corneal ectasia, altered enzymatic processes result in the synthesis of abnormal collagen. Collagen packing is determined by the activity of various extracellular matrix enzymes which bind amines and aldehydes of collagen fiber amino acids. In the late stage, morphological changes of Descemet’s membrane (i.e., rupture and detachment develop. Abnormal hexagonal-shaped keratocytes and their apoptosis are the signs of endothelial dystrophy. The lack of analogs in domestic ophthalmology encouraged the scientists of Ufa Eye Research Institute to develop a device for corneal collagen crosslinking. The parameters of ultraviolet (i.e., wavelength, exposure time, power to achieve the desired effect were identified. The specifics of some photosensitizers in the course of the procedure were studied. UFalink, a device for UV irradiation of cornea, and photosensitizer Dextralink were developed and adopted. Due to the high risk of endothelial damage, this treatment is contraindicated in severe keratoconus (CCT less than 400 microns. Major effects of corneal collagen crosslinking are the following: Young’s modulus (modulus of elasticity increase by 328.9 % (on average, temperature tolerance increase by 5

  14. Corneal collagen crosslinking for keratoconus. A review

    Directory of Open Access Journals (Sweden)

    M. M. Bikbov

    2014-10-01

    Full Text Available Photochemical crosslinking is widely applied in ophthalmology. Its biochemical effect is due to the release of singlet oxygen that promotes anaerobic photochemical reaction. Keratoconus is one of the most common corneal ectasia affecting 1 in 250 to 250 000 persons. Currently, the rate of iatrogenic ectasia following eximer laser refractive surgery increases due to biomechanical weakening of the cornea. Morphologically and biochemically, ectasia is characterized by corneal layers thinning, contact between the stroma and epithelium resulting from Bowman’s membrane rupture, chromatin fragmentation in keratocyte nuclei, phagocytosis, abnormal staining and arrangement of collagen fibers, enzyme system disorders, and keratocyte apoptosis. In corneal ectasia, altered enzymatic processes result in the synthesis of abnormal collagen. Collagen packing is determined by the activity of various extracellular matrix enzymes which bind amines and aldehydes of collagen fiber amino acids. In the late stage, morphological changes of Descemet’s membrane (i.e., rupture and detachment develop. Abnormal hexagonal-shaped keratocytes and their apoptosis are the signs of endothelial dystrophy. The lack of analogs in domestic ophthalmology encouraged the scientists of Ufa Eye Research Institute to develop a device for corneal collagen crosslinking. The parameters of ultraviolet (i.e., wavelength, exposure time, power to achieve the desired effect were identified. The specifics of some photosensitizers in the course of the procedure were studied. UFalink, a device for UV irradiation of cornea, and photosensitizer Dextralink were developed and adopted. Due to the high risk of endothelial damage, this treatment is contraindicated in severe keratoconus (CCT less than 400 microns. Major effects of corneal collagen crosslinking are the following: Young’s modulus (modulus of elasticity increase by 328.9 % (on average, temperature tolerance increase by 5

  15. Duchenne and Becker Muscular Dystrophies

    Science.gov (United States)

    ... that our son Mike, then age 4, has Duchenne muscular dystrophy, we were devastated. Immediately, our hopes and dreams ... over time, the per- son’s muscle strength declines. Duchenne muscular dystrophy (DMD) was first described by the French neurologist ...

  16. One donor cornea for 3 recipients: a new concept for corneal transplantation surgery.

    Science.gov (United States)

    Vajpayee, Rasik B; Sharma, Namrata; Jhanji, Vishal; Titiyal, Jeewan S; Tandon, Radhika

    2007-04-01

    To describe the use of a single donor corneal tissue in 3 patients with corneal pathologic conditions. A donor corneal tissue was divided into 3 parts using a microkeratome and a trephine. The anterior lamellar disc was transplanted into a patient with macular corneal dystrophy using the automated lamellar therapeutic keratoplasty technique. The posterior lamellar disc was transplanted into a patient with pseudophakic bullous keratopathy using the Descemet stripping automated endothelial keratoplasty technique. The peripheral corneoscleral rim was used for limbal stem cell transplantation in a child with limbal stem cell deficiency. All surgical procedures were performed successfully. At 3 months, the best-corrected visual acuities achieved following automated lamellar therapeutic keratoplasty, Descemet stripping automated endothelial keratoplasty, and limbal stem cell transplantation were 20/60, 20/40, and 20/200, respectively. The advent of customized component corneal transplantation techniques may allow the use of 1 donor cornea to treat multiple patients.

  17. Cornea stress test--evaluation of corneal endothelial function in vivo by contact lens induced stress

    Directory of Open Access Journals (Sweden)

    Saini Jagjit

    1997-01-01

    Full Text Available Reliable and valid assessment of corneal endothelial function is a critical input for diagnosing, prognosticating and monitoring progression of disorders affecting corneal endothelium. In 123 eyes, corneal endothelial function was assessed employing data from the corneal hydration recovery dynamics. Serial pachometric readings were recorded on Haag-Striet pachometer with Mishima-Hedbys modification before and after two hours of thick soft contact lens wear. Percentage Recovery Per Hour (PRPH was derived from raw data as an index of endothelial function. Assessed PRPH in pseudophakic corneal oedema and Fuchs′ endothelial dystrophy eyes (35.9 +/- 9.8% was significantly lower than normal controls (61.9 +/- 10.5%. On employing receiver operation characteristics curve analysis the tested results demonstrated high sensitivity (87% and specificity (92% for detection of low endothelial function at PRPH cut off of 47.5%. Using this PRPH cut off, 80% of Fuchs′ endothelial dystrophy and 93.3% of pseudophakic corneal oedema eyes could be demonstrated to have low endothelial function. A total of 66.7% of diabetic eyes also demonstrated PRPH of lower than 47.5%. Clear corneal grafts demonstrated PRPH values of 24.6% to 73.0%. Of 6 corneal grafts that demonstrated initial PRPH of lower than 47.5%, 4 failed within 4 to 6 months. Our data demonstrated high sensitivity and specificity of this corneal stress test. PRPH index was useful in quantifying endothelial function in clinical disorders including diabetes mellitus. The index PRPH was demonstrated to be useful in monitoring and prognosticating outcome of corneal grafts.

  18. Equine corneal stromal abscesses

    DEFF Research Database (Denmark)

    Henriksen, M. D. L.; Andersen, P. H.; Plummer, C. E.

    2013-01-01

    The last 30 years have seen many changes in the understanding of the pathogenesis and treatment of equine corneal stromal abscesses (SAs). Stromal abscesses were previously considered an eye problem related to corneal bacterial infection, equine recurrent uveitis, corneal microtrauma and corneal...... foreign bodies in horses. They were more commonly diagnosed in horses living in subtropical climatic areas of the world. Therapeutic recommendations to treat equine SAs were historically nearly always a medical approach directed at bacteria and the often associated severe iridocyclitis. Today...... the pathogenesis of most equine SAs appears to be more often related to fungal inoculation of the anterior corneal stroma followed by posterior migration of the fungi deeper into the corneal stroma. There is also now an increased incidence of diagnosis of corneal SAs in horses living in more temperate climates...

  19. Ways to Improve (Visual) Outcome in Corneal Transplantation, Corneal Pathology

    NARCIS (Netherlands)

    M.C. Bartels (Marjolijn)

    2005-01-01

    textabstractThe normally transparent cornea can lose its ability to refract light regularly from various conditions. Among these conditions are corneal opacities and corneal diseases leading to a distortion of the corneal contour. Vision might be restored by a corneal transplantation. Corneal

  20. Effects of aberrant Pax6 gene dosage on mouse corneal pathophysiology and corneal epithelial homeostasis.

    Directory of Open Access Journals (Sweden)

    Richard L Mort

    Full Text Available Altered dosage of the transcription factor PAX6 causes multiple human eye pathophysiologies. PAX6⁺/⁻ heterozygotes suffer from aniridia and aniridia-related keratopathy (ARK, a corneal deterioration that probably involves a limbal epithelial stem cell (LESC deficiency. Heterozygous Pax6(+/Sey-Neu (Pax6⁺/⁻ mice recapitulate the human disease and are a good model of ARK. Corneal pathologies also occur in other mouse Pax6 mutants and in PAX77(Tg/- transgenics, which over-express Pax6 and model human PAX6 duplication.We used electron microscopy to investigate ocular defects in Pax6⁺/⁻ heterozygotes (low Pax6 levels and PAX77(Tg/- transgenics (high Pax6 levels. As well as the well-documented epithelial defects, aberrant Pax6 dosage had profound effects on the corneal stroma and endothelium in both genotypes, including cellular vacuolation, similar to that reported for human macular corneal dystrophy. We used mosaic expression of an X-linked LacZ transgene in X-inactivation mosaic female (XLacZ(Tg/- mice to investigate corneal epithelial maintenance by LESC clones in Pax6⁺/⁻ and PAX77(Tg/- mosaic mice. PAX77(Tg/- mosaics, over-expressing Pax6, produced normal corneal epithelial radial striped patterns (despite other corneal defects, suggesting that centripetal cell movement was unaffected. Moderately disrupted patterns in Pax6⁺/⁻ mosaics were corrected by introducing the PAX77 transgene (in Pax6⁺/⁻, PAX77(Tg/- mosaics. Pax6(Leca4/+, XLacZ(Tg/- mosaic mice (heterozygous for the Pax6(Leca4 missense mutation showed more severely disrupted mosaic patterns. Corrected corneal epithelial stripe numbers (an indirect estimate of active LESC clone numbers declined with age (between 15 and 30 weeks in wild-type XLacZ(Tg/- mosaics. In contrast, corrected stripe numbers were already low at 15 weeks in Pax6⁺/⁻ and PAX77(Tg/- mosaic corneas, suggesting Pax6 under- and over-expression both affect LESC clones.Pax6⁺/⁻ and PAX77(Tg

  1. Vanishing corneal vessels

    Science.gov (United States)

    Nicholson, Luke; Chana, Rupinder

    2013-01-01

    We wish to highlight the importance of acknowledging the accompanying effects of topical phenylephrine drops on the eye other than its intended mydriasis. We reported a case of a 92-year-old woman with a corneal graft who was noted to have superficial corneal vascularisation which was not documented previously. After the instillation of topical tropicamide 1% and phenylephrine 2.5%, for funduscopy, the corneal vascularisation was not visible. When reassessed on another visit, tropicamide had no effect on the vessels and only phenylephrine did. We wish to highlight that when reviewing patients in cornea clinics, instilling phenylephrine prior to being seen may mask important corneal vascularisation. PMID:24121816

  2. Limb girdle muscular dystrophies

    DEFF Research Database (Denmark)

    Vissing, John

    2016-01-01

    PURPOSE OF REVIEW: The aim of the study was to describe the clinical spectrum of limb girdle muscular dystrophies (LGMDs), the pitfalls of the current classification system for LGMDs, and emerging therapies for these conditions. RECENT FINDINGS: Close to half of all LGMD subtypes have been...

  3. RECURRENT CORNEAL EROSION SYNDROME (a review

    Directory of Open Access Journals (Sweden)

    S. V. Trufanov

    2015-01-01

    Full Text Available Recurrent corneal erosion (RCE syndrome is characterized by episodes of recurrent spontaneous epithelial defects. Main clinical symptoms (pain, redness, photophobia, lacrimation occurred at night. Corneal lesions revealed by slit lamp exam vary depending on the presence of corneal epithelium raise, epithelial microcysts or epithelial erosions, stromal infiltrates and opacities. Microtraumas, anterior corneal dystrophies, and herpesvirus give rise to RCE. Other causes or factors which increase the risk of RCE syndrome include meibomian gland dysfunction, keratoconjunctivitis sicca, diabetes, and post-LASIK conditions. Basal membrane abnormalities and instability of epithelial adhesion to stroma play a key role in RCE pathogenesis. Ultrastructural changes in RCE include abnormalities of basal epithelial cells and epithelial basal membrane, absence or deficiency of semi-desmosomes, loss of anchor fibrils. Increase in matrix metalloproteinases and collagenases which contribute to basal membrane destruction results in recurrent erosions and further development of abnormal basal membrane. The goals of RCE therapy are to reduce pain (in acute stage, to stimulate re-epithelization, and to restore «adhesion complex» of basal membrane. In most cases, RCE responds to simple conservative treatment that includes lubricants, healing agents, and eye patches. RCEs that are resistant to simple treatment, require complex approach. Non-invasive methods include long-term contact lens use, instillations of autologous serum (eye drops, injections of botulinum toxin (induces ptosis, antiviral agent use or oral intake of metalloproteinase inhibitors. Cell membrane stabilizers, i.e., antioxidants, should be included into treatment approaches as well. Antioxidant effect of Emoxipine promotes tissue reparation due to the prevention of cell membrane lipid peroxidation as well as due to its anti-hypoxic, angioprotective, and antiplatelet effects. If conservative therapy

  4. Limb-Girdle Muscular Dystrophy (LGMD)

    Science.gov (United States)

    ... Blog Donate Search MDA.org Close Limb-Girdle Muscular Dystrophy (LGMD) Share print email share facebook twitter google plus linkedin Limb-Girdle Muscular Dystrophy (LGMD) What is limb-girdle muscular dystrophy? Limb- ...

  5. Genetics Home Reference: Fukuyama congenital muscular dystrophy

    Science.gov (United States)

    ... Health Conditions Fukuyama congenital muscular dystrophy Fukuyama congenital muscular dystrophy Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Fukuyama congenital muscular dystrophy is an inherited condition that predominantly affects the ...

  6. Genetics Home Reference: tibial muscular dystrophy

    Science.gov (United States)

    ... Twitter Home Health Conditions Tibial muscular dystrophy Tibial muscular dystrophy Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Tibial muscular dystrophy is a condition that affects the muscles at ...

  7. The Molecular Basis for TGFBIp-Related Corneal Dystrophies

    DEFF Research Database (Denmark)

    Stenvang, Marcel Renè; Andreasen, Maria; Otzen, Daniel

    2014-01-01

    granular to lattice and rod-like deposits. Biophysical methods have begun to help us elucidate how and why these mutations lead to polymorphic aggregates. Most CD-inducing mutations are found in the fourth fasciclin-1 domain of TGFBIp, and this domain also controls the stability of the entire TGFBIp...

  8. Characterization of Corneal Indentation Hysteresis.

    Science.gov (United States)

    Ko, Match W L; Dongming Wei; Leung, Christopher K S

    2015-01-01

    Corneal indentation is adapted for the design and development of a characterization method for corneal hysteresis behavior - Corneal Indentation Hysteresis (CIH). Fourteen porcine eyes were tested using the corneal indentation method. The CIH measured in enucleated porcine eyes showed indentation rate and intraocular pressure (IOP) dependences. The CIH increased with indentation rate at lower IOP ( 25 mmHg). The CIH was linear proportional to the IOP within an individual eye. The CIH was positively correlated with the IOP, corneal in-plane tensile stress and corneal tangent modulus (E). A new method based on corneal indentation for the measurement of Corneal Indentation Hysteresis in vivo is developed. To our knowledge, this is the first study to introduce the corneal indentation hysteresis and correlate the corneal indentation hysteresis and corneal tangent modulus.

  9. corneal pyogenic granuloma

    African Journals Online (AJOL)

    GB

    2012-09-14

    Sep 14, 2012 ... Figure 3: A child with pyogenic cornea granuloma intra operatively. After excision of the mass, central corneal stromal defect developed. The patient was followed up for more than a month with topical antibiotic and cycloplegic. Subsequently, the defect healed and leucoma corneal opacity (figure.

  10. Therapeutic advances in muscular dystrophy

    OpenAIRE

    Leung, Doris G; Wagner, Kathryn R

    2013-01-01

    The muscular dystrophies comprise a heterogeneous group of genetic disorders that produce progressive skeletal muscle weakness and wasting. There has been rapid growth and change in our understanding of these disorders in recent years, and advances in basic science are being translated into increasing numbers of clinical trials. This review will discuss therapeutic developments in 3 of the most common forms of muscular dystrophy: Duchenne muscular dystrophy, facioscapulohumeral muscular dystr...

  11. Corneal biomechanics - a review.

    Science.gov (United States)

    Kling, Sabine; Hafezi, Farhad

    2017-05-01

    In recent years, the interest in corneal biomechanics has strongly increased. The material properties of the cornea determine its shape and therefore play an important role in corneal ectasia and related pathologies. This review addresses the molecular origin of biomechanical properties, models for their description, methods for their characterisation, techniques for their modification, and computational simulation approaches. Recent research has focused on developing non-contact techniques to measure the biomechanical properties in vivo, on determining structural and molecular abnormalities in pathological corneas, on developing and optimising techniques to reinforce the corneal tissue and on the computational simulation of surgical interventions. A better understanding of corneal biomechanics will help to improve current refractive surgeries, allow an earlier diagnosis of ectatic disorders and a better quantification of treatments aiming at reinforcing the corneal tissue. © 2017 The Authors Ophthalmic & Physiological Optics © 2017 The College of Optometrists.

  12. Duchenne muscular dystrophy.

    Science.gov (United States)

    Yiu, Eppie M; Kornberg, Andrew J

    2015-08-01

    Duchenne muscular dystrophy, an X-linked disorder, has an incidence of one in 5000 boys and presents in early childhood with proximal muscle weakness. Untreated boys become wheelchair bound by the age of 12 years and die of cardiorespiratory complications in their late teens to early 20s. The use of corticosteroids, non-invasive respiratory support, and active surveillance and management of associated complications have improved ambulation, function, quality of life and life expectancy. The clinical features, investigations and management of Duchenne muscular dystrophy are reviewed, as well as the latest in some of the novel therapies. © 2015 The Authors. Journal of Paediatrics and Child Health © 2015 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  13. Late occurrence of granular dystrophy in bilateral keratoconus: Penetrating keratoplasty and long-term follow-up

    Directory of Open Access Journals (Sweden)

    Varsha M Rathi

    2011-01-01

    Full Text Available We report a rare case of keratoconus with granular dystrophy with a follow-up of two decades, documenting the sequential presentation of two diseases confirmed by histology and genetic studies. A 13-year-old boy was diagnosed in 1988 with keratoconus in both eyes (BE based on slit-lamp biomicroscopy findings of corneal ectasia in BE accompanied by Fleischer′s ring, Vogt′s striae, a small, old, healed hydrops. The left eye (LE had central corneal thinning and scar in the central area involving the mid and posterior stroma secondary to healed hydrops. Penetrating keratoplasty (PKP was advised. The boy was lost to follow-up till 1991 and presented with white, dot-like opacities in the central cornea in the RE only, suggestive of granular corneal dystrophy. Similar findings of white, dot-like opacities were noted in the LE in 1995 and the patient subsequently underwent PKP in BE. Histopathology of corneal buttons confirmed the presence of patchy, crystal-like orange deposits, which stained bright red with Masson′s trichrome. Mutational analysis of the TGFBI gene in patient′s DNA revealed a heterozygous mutation corresponding to a change in Arg555Trp in the keratoepithelin protein. Granular dystrophy recurred after 8 years in the RE.

  14. Corneal transplant - slideshow

    Science.gov (United States)

    ... ency/presentations/100082.htm Corneal transplant - series—Normal anatomy To use the sharing features on this page, ... Bethesda, MD 20894 U.S. Department of Health and Human Services National Institutes of Health Page last updated: ...

  15. Eckardt Keratoprosthesis for Tectonic Repair of a Large Corneal Perforation.

    Science.gov (United States)

    Helsen, Silke; Ní Dhubhghaill, Sorcha; Zakaria, Nadia; Koppen, Carina

    2016-08-01

    To report on the use of the Eckardt keratoprosthesis as an emergency temporary tectonic seal for a full-thickness, large decentered corneal perforation. Case report and review of the literature. A 47-year-old male patient with myotonic dystrophy presented with a large corneal perforation as a complication of chronic ulceration caused by lagophthalmia and recurrent herpetic keratitis. The perforation was triggered by a superinfection with Gram-positive bacteria. In an emergency setting where no donor cornea was available, the Eckardt keratoprosthesis was sutured into the debrided corneal defect as a tectonic measure. A secondary procedure, consisting of open sky cataract extraction combined with penetrating keratoplasty was performed 3 weeks later. During this period, the prosthesis was well tolerated and the anterior chamber stayed well formed. The Eckardt keratoprosthesis allowed us to convert what would have been an emergency à chaud keratoplasty into a well-controlled elective procedure. In our case, the silicone prosthesis was well tolerated during the 3-week period while awaiting final repair with a corneal donor button.

  16. Indications for Corneal Transplantation at a Tertiary Referral Center in Tehran

    Directory of Open Access Journals (Sweden)

    Mohammad Zare

    2010-01-01

    Full Text Available Purpose: To report the indications and techniques of corneal transplantation at a tertiary referral center in Tehran over a 3-year period. Methods: Records of patients who had undergone any kind of corneal transplantation at Labbafinejad Medical Center, Tehran, Iran from March 2004 to March 2007 were reviewed to determine the indications and types of corneal transplantation. Results: During this period, 776 eyes of 756 patients (including 504 male subjects with mean age of 41.3±21.3 years underwent corneal transplantation. The most common indication was keratoconus (n=317, 40.8% followed by bullous keratopathy (n=90, 11.6%, non-herpetic corneal scars (n=62, 8.0%, infectious corneal ulcers (n=61, 7.9%, previously failed grafts (n=61, 7.9%, endothelial and stromal corneal dystrophies (n=28, 3.6%, and trachoma keratopathy (n=26, 3.3%. Other indications including Terrien′s marginal degeneration, post-LASIK keratectasia, trauma, chemical burns, and peripheral ulcerative keratitis constituted the rest of cases. Techniques of corneal transplantation included penetrating keratoplasty (n=607, 78.2%, deep anterior lamellar keratoplasty (n=108, 13.9%, conventional lamellar keratoplasty (n=44, 5.7%, automated lamellar therapeutic keratoplasty (n=8, 1.0%, and Descemet stripping endothelial keratoplasty (n=6, 0.8% in descending order. The remaining cases were endothelial keratoplasty and sclerokeratoplasty. Conclusion: In this study, keratoconus was the most common indication for penetrating keratoplasty which was the most prevalent technique of corneal transplantation. However, deep anterior lamellar keratoplasty is emerging as a growing alternative for corneal pathologies not involving the endothelium.

  17. Cone rod dystrophies

    Directory of Open Access Journals (Sweden)

    Hamel Christian P

    2007-02-01

    Full Text Available Abstract Cone rod dystrophies (CRDs (prevalence 1/40,000 are inherited retinal dystrophies that belong to the group of pigmentary retinopathies. CRDs are characterized by retinal pigment deposits visible on fundus examination, predominantly localized to the macular region. In contrast to typical retinitis pigmentosa (RP, also called the rod cone dystrophies (RCDs resulting from the primary loss in rod photoreceptors and later followed by the secondary loss in cone photoreceptors, CRDs reflect the opposite sequence of events. CRD is characterized by primary cone involvement, or, sometimes, by concomitant loss of both cones and rods that explains the predominant symptoms of CRDs: decreased visual acuity, color vision defects, photoaversion and decreased sensitivity in the central visual field, later followed by progressive loss in peripheral vision and night blindness. The clinical course of CRDs is generally more severe and rapid than that of RCDs, leading to earlier legal blindness and disability. At end stage, however, CRDs do not differ from RCDs. CRDs are most frequently non syndromic, but they may also be part of several syndromes, such as Bardet Biedl syndrome and Spinocerebellar Ataxia Type 7 (SCA7. Non syndromic CRDs are genetically heterogeneous (ten cloned genes and three loci have been identified so far. The four major causative genes involved in the pathogenesis of CRDs are ABCA4 (which causes Stargardt disease and also 30 to 60% of autosomal recessive CRDs, CRX and GUCY2D (which are responsible for many reported cases of autosomal dominant CRDs, and RPGR (which causes about 2/3 of X-linked RP and also an undetermined percentage of X-linked CRDs. It is likely that highly deleterious mutations in genes that otherwise cause RP or macular dystrophy may also lead to CRDs. The diagnosis of CRDs is based on clinical history, fundus examination and electroretinogram. Molecular diagnosis can be made for some genes, genetic counseling is

  18. Congenital muscular dystrophy in Jordanian children.

    Science.gov (United States)

    Al-Qudah, A A; Tarawneh, M

    1998-08-01

    This is a consecutive study on 28 patients who have been diagnosed as having congenital muscular dystrophy at Jordan University Hospital in the period from January 1990 to February 1997. Of 75 patients diagnosed as having muscle disease, 55 (73.3%) had muscular dystrophy. Of 55 muscular dystrophy patients, 28 (50.9%) had congenital muscular dystrophy, 11 (20%) had Duchenne muscular dystrophy, 9 (16.4%) had Becker muscular dystrophy, 4 (7.3%) had myotonic dystrophy, 2 (3.6%) had limb-girdle dystrophy, and 1 (1.8%) patient had facioscapulohumeral dystrophy. Age of onset of symptoms of congenital muscular dystrophy (hypotonia and weakness) was documented antenatally or in the first few months in the majority (92.9%) of patients. Parental consanguinity was documented in 21 (75%) of congenital muscular dystrophy cases, and family history of possible similar cases in 15 (53.6%). Congenital muscular dystrophy patients with normal cognitive milestones (n = 16; 57.1%) were slightly more common than patients with cognitive delay. In contrast to previous reports, congenital muscular dystrophy is probably more common in communities with high rates of parental consanguinity than other dystrophies. Our study adds significant support to the most recent literature on this finding.

  19. Research progress of corneal transplantation

    OpenAIRE

    Bing-Jie Zhang; Heng Sun; Yuan-Ping Zhang; Lin-Kun Ma

    2015-01-01

    Corneal transplantation is an ophthalmology treatment technique for corneal disease to help restore vision or control the development of corneal diseases by removing a scarred or damaged host cornea and replacing it with a clear and healthy donor cornea. Traditional corneal transplantation includes penetrating keratoplasty and lamellar keratoplasty. In recent ten years, deep lamellar keratoplasty and endothelial keratoplasty have gradually developed. At present, the development of keratoprost...

  20. Muscular Dystrophy: Data and Statistics

    Science.gov (United States)

    ... listing of Medicaid or Children’s Health Insurance Program (CHIP) insurance in the medical record. Age in Years ( ... Oleszek J; Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STAR net ). Trends with corticosteroid use in ...

  1. Wasting Mechanisms in Muscular Dystrophy

    Science.gov (United States)

    Shin, Jonghyun; Tajrishi, Marjan M.; Ogura, Yuji; Kumar, Ashok

    2013-01-01

    Muscular dystrophy is a group of more than 30 different clinical genetic disorders that are characterized by progressive skeletal muscle wasting and degeneration. Primary deficiency of specific extracellular matrix, sarcoplasmic, cytoskeletal, or nuclear membrane protein results in several secondary changes such as sarcolemmal instability, calcium influx, fiber necrosis, oxidative stress, inflammatory response, breakdown of extracellular matrix, and eventually fibrosis which leads to loss of ambulance and cardiac and respiratory failure. A number of molecular processes have now been identified which hasten disease progression in human patients and animal models of muscular dystrophy. Accumulating evidence further suggests that aberrant activation of several signaling pathways aggravate pathological cascades in dystrophic muscle. Although replacement of defective gene with wild-type is paramount to cure, management of secondary pathological changes has enormous potential to improving the quality of life and extending lifespan of muscular dystrophy patients. In this article, we have reviewed major cellular and molecular mechanisms leading to muscle wasting in muscular dystrophy. PMID:23669245

  2. Learning about Duchenne Muscular Dystrophy

    Science.gov (United States)

    Skip to main content Learning About Duchenne Muscular Dystrophy Enter Search Term(s): Español Research Funding An Overview Bioinformatics Current Grants Education and Training Funding Extramural Research News Features Funding Divisions Funding ...

  3. Latest progress of BIGH3 gene in corneal diseases and diabetic retinopathy

    Directory of Open Access Journals (Sweden)

    Fan-Qian Song

    2017-03-01

    Full Text Available BIGH3 gene plays an important role in ocular diseases. On the one hand, it is closely related to the occurrence of corneal diseases. BIGH3 gene can inhibit corneal neovascularization, lead to corneal dystrophy, participate in keratoconus formation. On the other hand, it can lead to the formation of neovascularization in diabetic retinopathy. The latest experiments show that TGF beta secreted by macrophages can promote the expression of BIGH3 mRNA and BIGH3 protein, and promote apoptosis of retinal endothelial cells and pericytes, which leads to the formation of neovascularization in diabetic retinopathy. This article will describe the new progress of BIGH3 gene in ocular diseases from several aspects as mentioned above.

  4. "Double bubble" deep anterior lamellar keratoplasty for management of corneal stromal pathologies.

    Science.gov (United States)

    Jhanji, Vishal; Beltz, Jacqueline; Sharma, Namrata; Graue, Enrique; Vajpayee, Rasik B

    2011-08-01

    'Big Bubble' deep anterior lamellar keratoplasty (DALK) is becoming an accepted corneal transplantation technique for keratoconus and other anterior stromal corneal pathologies that spare the Descemet's membrane (DM) and endothelium. However, it is not always possible to conclusively recognise formation and identification of the 'Big Bubble'. We describe the surgical technique of DALK called 'Double Bubble' technique that allows the surgeon to definitely and immediately identify the formation of an adequate big bubble. DALK was performed using the 'Double Bubble' technique in twelve eyes of twelve patients with corneal stromal pathologies (keratoconus, 9 eyes; macular corneal dystrophy, 2 eyes; postinfectious keratitis corneal stromal scar, 1 eye) at the Royal Victorian Eye and Ear Hospital, Melbourne. Big bubble was successfully formed in 10 eyes. Maximum-depth deep lamellar keratoplasty was performed in two eyes. There were no instances of intraoperative perforation of the DM. All grafts were clear at last follow-up. Best-corrected visual acuity of ≥20/40 was achieved in all the cases at last follow-up (6-12 months). 'Double Bubble' DALK helps in identification of the big bubble and has the potential to increase the success of standard 'Big Bubble' DALK in patients with corneal stromal pathologies sparing the DM and endothelium.

  5. Corneal Cross-Linking: An Example of Photoinduced Polymerization as a Treatment Modality in Keratoconus.

    Science.gov (United States)

    Kubrak-Kisza, Magdalena; Kisza, Krystian Jerzy; Misiuk-Hojło, Marta

    2016-01-01

    The cornea is one of the principal refractive elements in the human eye and plays a crucial role in the process of vision. Keratoconus is the most common corneal dystrophy, found mostly among young adults. It is characterized by a reduced number of collagen cross-links in the corneal stroma, resulting in reduced biomechanical stability and an abnormal shape of the cornea. These changes lead to progressive myopia, corneal thinning, central scarring and irregular astigmatism, causing severely impaired vision. Hard contact lenses, photorefractive keratectomy or intracorneal rings are the most common treatment options for refractive error caused by keratoconus. However, these techniques do not treat the underlying cause of the corneal ectasia and therefore are not able to stop the progression of the disease. Riboflavin photoinduced polymerization of corneal collagen, also known as corneal cross-linking (CXL), has been introduced as the first therapy which, by stabilizing the structure of the cornea, prevents the progression of keratoconus. It stiffens the cornea using the photo-sensitizer riboflavin in combination with ultraviolet irradiation. This is a current review of the CXL procedure as a therapy for keratoconus, which relies on photoinduced polymerization of human tissue. We have focused on its biomechanical and physiological influences on the human cornea and have reviewed the previous and current biochemical theories behind cross-linking reactions in the cornea.

  6. Airbag induced corneal ectasia.

    Science.gov (United States)

    Mearza, Ali A; Koufaki, Fedra N; Aslanides, Ioannis M

    2008-02-01

    To report a case of airbag induced corneal ectasia. Case report. A patient 3 years post-LASIK developed bilateral corneal ectasia worse in the right eye following airbag deployment in a road traffic accident. At last follow up, best corrected vision was 20/40 with -4.00/-4.00 x 25 in the right eye and 20/25 with -1.25/-0.50 x 135 in the left eye. This is a rare presentation of trauma induced ectasia in a patient post-LASIK. It is possible that reduction in biomechanical integrity of the cornea from prior refractive surgery contributed to this presentation.

  7. Research progress of corneal transplantation

    Directory of Open Access Journals (Sweden)

    Bing-Jie Zhang

    2015-06-01

    Full Text Available Corneal transplantation is an ophthalmology treatment technique for corneal disease to help restore vision or control the development of corneal diseases by removing a scarred or damaged host cornea and replacing it with a clear and healthy donor cornea. Traditional corneal transplantation includes penetrating keratoplasty and lamellar keratoplasty. In recent ten years, deep lamellar keratoplasty and endothelial keratoplasty have gradually developed. At present, the development of keratoprosthesis provides a new choice for the patients no suitable for traditional. The review describes current surgical techniques in the field of corneal transplantation about indications, postoperative complications, and so on.

  8. Congenital Hereditary Endothelial Dystrophy Caused by SLC4A11 Mutations Progresses to Harboyan Syndrome

    Science.gov (United States)

    Siddiqui, Salina; Zenteno, Juan Carlos; Rice, Aine; Chacón-Camacho, Oscar; Naylor, Steven G.; Rivera-de la Parra, David; Spokes, David M.; James, Nigel; Toomes, Carmel; Inglehearn, Chris F.

    2013-01-01

    Purpose: Homozygous mutations in SLC4A11 cause 2 rare recessive conditions: congenital hereditary endothelial dystrophy (CHED), affecting the cornea alone, and Harboyan syndrome consisting of corneal dystrophy and sensorineural hearing loss. In addition, adult-onset Fuchs endothelial corneal dystrophy (FECD) is associated with dominant mutations in SLC4A11. In this report, we investigate whether patients with CHED go on to develop hearing loss and whether their parents, who are carriers of an SLC4A11 mutation, show signs of having FECD. Methods: Patients with CHED were screened for mutations in the SLC4A11 gene and underwent audiometric testing. The patients and their parents underwent a clinical examination and specular microscopy. Results: Molecular analyses confirmed SLC4A11 mutations in 4 affected individuals from 3 families. All the patients were found to have varying degrees of sensorineural hearing loss at a higher frequency range. Guttate lesions were seen in 2 of the 4 parents who were available for examination. Conclusions: Our observations suggest that CHED caused by homozygous SLC4A11 mutations progresses to Harboyan syndrome, but the severity of this may vary considerably. Patients with CHED should therefore be monitored for progressive hearing loss. We could not determine conclusively whether the parents of the patients with CHED were at increased risk of developing late-onset FECD. PMID:24351571

  9. Alternatives to allograft corneal transplantation.

    Science.gov (United States)

    Jhanji, Vishal; Sharma, Namrata; Agarwal, Tushar; Vajpayee, Rasik B

    2010-07-01

    Corneal transplantation is the most commonly performed solid organ transplantation in the world. Despite a glorious history of more than a 100 years, the success of conventional corneal transplantation surgery is marred by problems like graft rejection,graft infection and associated glaucoma due to long-term use of topical corticosteroids.In addition there is a dearth of donor corneal tissue in some parts of the world which subsequently adds on to the existing burden on the eye banks every year. We propose alternatives to the conventional corneal transplantation surgery for the management of corneal scarring. The potential use of alternatives to allograft corneal transplantation surgery has been described by corneal surgeons around the world. These techniques consist of nonsurgical interventions like contact lens fitting. Surgical alternatives include excimer laser phototherapeutic keratectomy, optical iridectomy, rotational autokeratoplasty and contralateral autokeratoplasty. Although these techniques are not practiced routinely, however, their appropriate utilization would clearly help the corneal surgeons to get rid of certain problems associated with allograft corneal transplantation. Careful selection of patients can yield encouraging results with the use of these alternative techniques. Visual outcomes may not be as good as after a routine keratoplasty; nevertheless, this setback is outweighed by advantages such as absence of corneal graft rejection. We also believe that the use of these techniques would at least partially resolve the issue of scarcity of donor corneal tissue in the developing world.

  10. How Do People Cope with Muscular Dystrophy?

    Science.gov (United States)

    ... Other FAQs Share Facebook Twitter Pinterest Email Print Muscular Dystrophy: Other FAQs Basic information for topics, such as “ ... in this section. How do people cope with muscular dystrophy (MD)? Although MD presents many challenges in many ...

  11. What Are the Treatments for Muscular Dystrophy?

    Science.gov (United States)

    ... Pinterest Email Print What are the treatments for muscular dystrophy? No treatment is currently available to stop or reverse any form of muscular dystrophy (MD). Instead, certain therapies and medications aim to ...

  12. Delayed onset of congenital hereditary endothelial dystrophy due to compound heterozygous SLC4A11 mutations

    Directory of Open Access Journals (Sweden)

    Babu Lal Kumawat

    2016-01-01

    Full Text Available Background: Congenital hereditary endothelial dystrophy (CHED is an autosomal recessive disorder characterized by bilateral, symmetrical, noninflammatory corneal clouding (edema present at birth or shortly thereafter. This study reports on an unusual delayed presentation of CHED with compound heterozygous SLC4A11 mutations. Materials and Methods: A 45-year-old female, presenting with bilateral decreased vision since childhood that deteriorated in the last 5 years, was evaluated to rule out trauma, viral illness, chemical injury, glaucoma, and corneal endothelial dystrophies. Tear sample was sent for herpes simplex viral (HSV antigen testing. Genomic DNA from peripheral blood was screened for mutations in all exons of SLC4A11 by direct sequencing. Full-thickness penetrating keratoplasty was done and corneal button was sent for histopathological examination. Results: Slit-lamp findings revealed bilateral diffuse corneal edema and left eye spheroidal degeneration with scarring. Increased corneal thickness (762 μm and 854 μm in the right and left eyes, respectively, normal intraocular pressure (12 mmHg and 16 mmHg in the right and left eyes, respectively, inconclusive confocal scan, and specular microscopy, near normal tear film parameters, were the other clinical features. HSV-polymerase chain reaction was negative. Histopathological examination revealed markedly thickened Descemet′s membrane with subepithelial spheroidal degeneration. SLC4A11 screening showed a novel variant p.Ser415Asn, reported mutation p.Cys386Arg and two polymorphisms, all in the heterozygous state and not identified in 100 controls. Conclusions: The study shows, for the first time, compound heterozygous SLC4A11 mutations impair protein function leading to delayed onset of the disease.

  13. Fuchs' dystrophy associated with radial keratotomy: Lamellar or perforating keratoplasty?

    Science.gov (United States)

    Rodriguez-Ausin, P; Antolin-Garcia, D; Santamaria Garcia, L; Blazquez-Fernandez, A-B

    2017-05-01

    A 70 year-old male patient with a history of radial keratotomy suffering from Fuchs' dystrophy and a cataract. The patient received a two-step surgery: lens phacoemulsification and intraocular lens implant, followed by descemet stripping automated endothelial keratoplasty in both eyes, four months later. There were no complications apart from a recurrent cystoid macular oedema in both eyes. The best corrected visual acuity was 20/40 both eyes, and the patient was satisfied. Descemet stripping automated endothelial keratoplasty may be considered as an alternative to penetrating keratoplasty in the case of endothelial dysfunction and radial keratotomy in patients with no corneal ectasia or significant stromal opacity. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  14. Spontaneous Corneal Hydrops in a Patient with a Corneal Ulcer

    Directory of Open Access Journals (Sweden)

    Hatim Batawi

    2016-01-01

    Full Text Available Purpose: We report the case of a 77-year-old man with no history of keratoconus or other ectatic disorders who presented with corneal hydrops in the setting of a corneal ulcer. The risk factors, pathogenesis and treatment options of corneal hydrops are discussed. Method: This is an observational case report study. Results: A 77-year-old man presented with a 1-day history of severe pain, redness, mucous discharge and photophobia in the right eye. A slit-lamp examination of the right eye showed an area of focal corneal edema and protrusion. Within the area of edema and protrusion, there was an infiltrate with an overlying epithelial defect consistent with an infectious corneal ulcer. The Seidel test showed no leakage, so a clinical diagnosis of corneal hydrops associated with nonperforated corneal ulcer was made. With appropriate antibiotic treatment, the corneal ulcer and hydrops both resolved over a 1-month period. Conclusion: Corneal hydrops can occur in the setting of corneal infections.

  15. Massive corneal edema treated with corneal cross-linking.

    Science.gov (United States)

    Laborante, A; Buzzonetti, L; Longo, C

    2012-01-01

    Massive corneal edema disrupts the fine architecture of corneal stroma that guarantees its transparency, causing opacities that seriously impair clear vision and are usually solved by corneal transplant. Corneal cross-linking, a treatment developed to halt keratoconus progression, results in a loss of water and a compaction of corneal stroma. It might therefore be useful to improve the pathologic edematous condition of some corneas, ameliorating visual acuity and allowing more time for a surgical procedure of keratoplasty. Six patients with visual impairing corneal edemas further to lens phacoemulsification, penetrating keratoplasty, or post-infective neovascularization were treated with corneal cross-linking alone, or in combination with amniotic membrane apposition with or without anti-angiogenic therapy. All patients partly resolved the edematous condition, improving both corneal transparency and visual acuity. Corneal cross-linking appears to be a useful method to treat massive corneal edemas, so that keratoplasty can be at least delayed, and need not to be an emergency treatment in these cases.

  16. Corneal biomechanical properties from air-puff corneal deformation imaging

    Science.gov (United States)

    Marcos, Susana; Kling, Sabine; Bekesi, Nandor; Dorronsoro, Carlos

    2014-02-01

    The combination of air-puff systems with real-time corneal imaging (i.e. Optical Coherence Tomography (OCT), or Scheimpflug) is a promising approach to assess the dynamic biomechanical properties of the corneal tissue in vivo. In this study we present an experimental system which, together with finite element modeling, allows measurements of corneal biomechanical properties from corneal deformation imaging, both ex vivo and in vivo. A spectral OCT instrument combined with an air puff from a non-contact tonometer in a non-collinear configuration was used to image the corneal deformation over full corneal cross-sections, as well as to obtain high speed measurements of the temporal deformation of the corneal apex. Quantitative analysis allows direct extraction of several deformation parameters, such as apex indentation across time, maximal indentation depth, temporal symmetry and peak distance at maximal deformation. The potential of the technique is demonstrated and compared to air-puff imaging with Scheimpflug. Measurements ex vivo were performed on 14 freshly enucleated porcine eyes and five human donor eyes. Measurements in vivo were performed on nine human eyes. Corneal deformation was studied as a function of Intraocular Pressure (IOP, 15-45 mmHg), dehydration, changes in corneal rigidity (produced by UV corneal cross-linking, CXL), and different boundary conditions (sclera, ocular muscles). Geometrical deformation parameters were used as input for inverse finite element simulation to retrieve the corneal dynamic elastic and viscoelastic parameters. Temporal and spatial deformation profiles were very sensitive to the IOP. CXL produced a significant reduction of the cornea indentation (1.41x), and a change in the temporal symmetry of the corneal deformation profile (1.65x), indicating a change in the viscoelastic properties with treatment. Combining air-puff with dynamic imaging and finite element modeling allows characterizing the corneal biomechanics in-vivo.

  17. Infrastructure for Clinical Trials in Duchenne Dystrophy

    Science.gov (United States)

    2010-09-13

    sites. Review of Potential Grant submissions: Treatment of early cardiac systolic dysfunction in Duchenne muscular dystrophy with lisinopril or...revamped and implemented for two studies in Duchenne Muscular Dystrophy (DMD). CINRG’s public website has been revamped. One measurement clinical...SUBJECT TERMS Duchenne Muscular Dystrophy , CINRG, CQMS, coordinating center, electronic data capture 16. SECURITY CLASSIFICATION OF: 17. LIMITATION

  18. Inhibition of TGF-β signaling enables human corneal endothelial cell expansion in vitro for use in regenerative medicine.

    Directory of Open Access Journals (Sweden)

    Naoki Okumura

    Full Text Available Corneal endothelial dysfunctions occurring in patients with Fuchs' endothelial corneal dystrophy, pseudoexfoliation syndrome, corneal endotheliitis, and surgically induced corneal endothelial damage cause blindness due to the loss of endothelial function that maintains corneal transparency. Transplantation of cultivated corneal endothelial cells (CECs has been researched to repair endothelial dysfunction in animal models, though the in vitro expansion of human CECs (HCECs is a pivotal practical issue. In this study we established an optimum condition for the cultivation of HCECs. When exposed to culture conditions, both primate and human CECs showed two distinct phenotypes: contact-inhibited polygonal monolayer and fibroblastic phenotypes. The use of SB431542, a selective inhibitor of the transforming growth factor-beta (TGF-β receptor, counteracted the fibroblastic phenotypes to the normal contact-inhibited monolayer, and these polygonal cells maintained endothelial physiological functions. Expression of ZO-1 and Na(+/K(+-ATPase maintained their subcellular localization at the plasma membrane. Furthermore, expression of type I collagen and fibronectin was greatly reduced. This present study may prove to be the substantial protocol to provide the efficient in vitro expansion of HCECs with an inhibitor to the TGF-β receptor, and may ultimately provide clinicians with a new therapeutic modality in regenerative medicine for the treatment of corneal endothelial dysfunctions.

  19. Computational Model for Corneal Transplantation

    Science.gov (United States)

    Cabrera, Delia

    2003-10-01

    We evaluated the refractive consequences of corneal transplants using a biomechanical model with homogeneous and inhomogeneous Young's modulus distributions within the cornea, taking into account ablation of some stromal tissue. A FEM model was used to simulate corneal transplants in diseased cornea. The diseased cornea was modeled as an axisymmetric structure taking into account a nonlinearly elastic, isotropic formulation. The model simulating the penetrating keratoplasty procedure gives more change in the postoperative corneal curvature when compared to the models simulating the anterior and posterior lamellar graft procedures. When a lenticle shaped tissue was ablated in the graft during the anterior and posterior keratoplasty, the models provided an additional correction of about -3.85 and -4.45 diopters, respectively. Despite the controversy around the corneal thinning disorders treatment with volume removal procedures, results indicate that significant changes in corneal refractive power could be introduced by a corneal transplantation combined with myopic laser ablation.

  20. Human corneal epithelial subpopulations

    DEFF Research Database (Denmark)

    Søndergaard, Chris Bath

    2013-01-01

    subpopulations in human corneal epithelium using a combination of laser capture microdissection and RNA sequencing for global transcriptomic profiling. We compared dissociation cultures, using either expansion on γ-irradiated NIH/3T3 feeder cells in serum-rich medium or expansion directly on plastic in serum......-free EpiLife medium, using a range of physiologically relevant oxygen concentrations (2%, 5%, 10%, 15% and 20%). Using immunocytochemistry and advanced fluorescence microscopy, cells were characterized regarding growth, cell cycle distribution, colony-forming efficiency (CFE), phenotypes...... was not dependent on the system used for propagation (Bath et al. 2013a). Laser capture microdissection was used to isolate cellular subpopulations in situ from the spatially defined differentiation pathway in human corneal epithelium according to an optimized protocol for maintenance of expression profiles...

  1. Biomechanics of Corneal Ring Implants

    OpenAIRE

    Daxer, Albert

    2015-01-01

    Purpose: To evaluate the biomechanics of corneal ring implants by providing a related mathematical theory and biomechanical model for the treatment of myopia and keratoconus. Methods: The spherical dome model considers the inhomogeneity of the tunica of the eye, dimensions of the cornea, lamellar structure of the corneal stroma, and asphericity of the cornea. It is used in this study for calculating a strengthening factor sf for the characterization of different ring-shaped corneal implant de...

  2. Corneal biomechanics: a review

    OpenAIRE

    Piñero Llorens, David Pablo; Alcón, Natividad

    2014-01-01

    Biomechanics is often defined as ‘mechanics applied to biology’. Due to the variety and complexity of the behaviour of biological structures and materials, biomechanics is better defined as the development, extension and application of mechanics for a better understanding of physiology and physiopathology and consequently for a better diagnosis and treatment of disease and injury. Different methods for the characterisation of corneal biomechanics are reviewed in detail, including those that a...

  3. Airbag-induced corneal flap.

    Science.gov (United States)

    Liyanage, Sidath E; Mearza, Ali A

    2009-02-01

    To describe a case of airbag-induced corneal flap in a previously normal cornea. Case report. A 27-year-old woman presented with complete loss of vision in her left eye following a road traffic accident which involved airbag deployment. There was no previous ocular history. Examination revealed a large corneal flap of 6mm in diameter, extending to the depth of anterior stroma. This was accompanied by a traumatic optic neuropathy. One month follow-up revealed complete reattachment of the corneal flap. This is the first reported case of a corneal flap induced by airbag deployment in a cornea with previously normal architecture.

  4. Congenital hereditary endothelial dystrophy with progressive sensorineural deafness (Harboyan syndrome

    Directory of Open Access Journals (Sweden)

    Abramowicz Marc

    2008-10-01

    Full Text Available Abstract Harboyan syndrome is a degenerative corneal disorder defined as congenital hereditary endothelial dystrophy (CHED accompanied by progressive, postlingual sensorineural hearing loss. To date, 24 cases from 11 families of various origin (Asian Indian, South American Indian, Sephardi Jewish, Brazilian Portuguese, Dutch, Gypsy, Moroccan, Dominican have been reported. More than 50% of the reported cases have been associated with parental consanguinity. The ocular manifestations in Harboyan syndrome include diffuse bilateral corneal edema occurring with severe corneal clouding, blurred vision, visual loss and nystagmus. They are apparent at birth or within the neonatal period and are indistinguishable from those characteristic of the autosomal recessive CHED (CHED2. Hearing deficit in Harboyan is slowly progressive and typically found in patients 10–25 years old. There are no reported cases with prelinglual deafness, however, a significant hearing loss in children as young as 4 years old has been detected by audiometry, suggesting that hearing may be affected earlier, even at birth. Harboyan syndrome is caused by mutations in the SLC4A11 gene located at the CHED2 locus on chromosome 20p13-p12, indicating that CHED2 and Harboyan syndrome are allelic disorders. A total of 62 different SLC4A11 mutations have been reported in 98 families (92 CHED2 and 6 Harboyan. All reported cases have been consistent with autosomal recessive transmission. Diagnosis is based on clinical criteria, detailed ophthalmological assessment and audiometry. A molecular confirmation of the clinical diagnosis is feasible. A variety of genetic, metabolic, developmental and acquired diseases presenting with clouding of the cornea should be considered in the differential diagnosis (Peters anomaly, sclerocornea, limbal dermoids, congenital glaucoma. Audiometry must be performed to differentiate Harboyan syndrome from CHED2. Autosomal recessive types of CHED (CHED2 and

  5. Correlations between corneal hysteresis, intraocular pressure, and corneal central pachymetry.

    Science.gov (United States)

    Touboul, David; Roberts, Cynthia; Kérautret, Julien; Garra, Caroline; Maurice-Tison, Sylvie; Saubusse, Elodie; Colin, Joseph

    2008-04-01

    To analyze the correlation between corneal hysteresis (CH) measured with the Ocular Response Analyzer (ORA, Reichert) and ultrasonic corneal central thickness (CCT US) and intraocular pressure measured with Goldmann applanation tonometry (IOP GA). Bordeaux 2 University, Ophthalmology Department, Bordeaux, France. This study comprised 498 eyes of 258 patients. Corneal hysteresis, corneal resistance factor (CRF), and IOP corneal-compensated (IOPcc) were provided by the ORA device; CCT US and IOP GA were also measured in each eye. The study population was divided into 5 groups: normal (n = 122), glaucoma (n = 159), keratoconus (n = 88), laser in situ keratomileusis (LASIK) (n = 78), and photorefractive keratectomy (n = 39). The Pearson correlation was used for statistical analysis. Corneal hysteresis was not strongly correlated with IOP or CCT US. The mean CH in the LASIK (8.87 mm Hg) and keratoconus (8.34 mm Hg) groups was lower than in the glaucoma (9.48 mm Hg) and normal (10.26 mm Hg) groups. The lower the CH, the lower its correlation with IOPcc and IOP GA. A CH higher than the CRF was significantly associated with the keratoconus and post-LASIK groups. Corneal hysteresis, a new corneal parameter, had a moderate dependence on IOP and CCT US. Weaker corneas could be screened with ORA parameters, and low CH could be considered a risk factor for underestimation of IOP. The CCT US should continue to be considered a useful parameter.

  6. Changes in corneal hysteresis after clear corneal cataract surgery.

    Science.gov (United States)

    Hager, Annette; Loge, Kristina; Füllhas, Marc-Oliver; Schroeder, Bernd; Grossherr, Martin; Wiegand, Wolfgang

    2007-09-01

    To assess the changes in corneal hysteresis (CH) as measured by the Ocular Response Analyzer (ORA; Reichert Ophthalmic Instruments, Buffalo, New York, USA) to describe the influence of clear corneal cataract surgery on corneal viscoelastic properties and intraocular pressure (IOP) measured by noncontact tonometry (NCT) and Goldmann applanation tonometry (GAT). Retrospective, interventional, comparative study. One hundred and one eyes of 101 consecutive patients who underwent routine clear corneal cataract surgery were evaluated. CH, NCT, and central corneal thickness (CCT) were measured by ORA before surgery and at postoperative day 1. A control group of 48 pseudophakic eyes (surgery >3 months previously) was included. CCT increased from 556.82 +/- 32.5 microm before surgery to 580.26 +/- 45.5 microm after surgery (P corneal cataract surgery, CH is diminished, whereas CCT is increased significantly. Postoperative corneal edema leads to a change of corneal viscoelastic properties, resulting in a lower damping capacity of the cornea. It is supposed that GAT and NCT measurements are significantly different because of postoperative changes in viscoelastic properties of the cornea.

  7. Donor Age and Corneal Endothelial Cell Loss 5 Years after Successful Corneal Transplantation: Specular Microscopy Ancillary Study Results

    Science.gov (United States)

    2010-01-01

    Objective To determine whether endothelial cell loss 5 years after successful corneal transplantation is related to the age of the donor. Design Multicenter, prospective, double-masked clinical trial. Participants Three hundred forty-seven subjects participating in the Cornea Donor Study who had not experienced graft failure 5 years after corneal transplantation for a moderate-risk condition (principally Fuchs’ dystrophy or pseudophakic corneal edema). Testing Specular microscopic images of donor corneas obtained before surgery and postoperatively at 6 months, 12 months, and then annually through 5 years were submitted to a central reading center to measure endothelial cell density (ECD). Main Outcome Measure Endothelial cell density at 5 years. Results At 5 years, there was a substantial decrease in ECD from baseline for all donor ages. Subjects who received a cornea from a donor 12 to 65 years old experienced a median cell loss of 69% in the study eye, resulting in a 5-year median ECD of 824 cells/mm2 (interquartile range, 613–1342), whereas subjects who received a cornea from a donor 66 to 75 years old experienced a cell loss of 75%, resulting in a median 5-year ECD of 654 cells/mm2 (interquartile range, 538–986) (P [adjusted for baseline ECD] = 0.04). Statistically, there was a weak negative association between ECD and donor age analyzed as a continuous variable (r [adjusted for baseline ECD] = −0.19; 95% confidence interval, −0.29 to −0.08). Conclusions Endothelial cell loss is substantial in the 5 years after corneal transplantation. There is a slight association between cell loss and donor age. This finding emphasizes the importance of longer-term follow-up of this cohort to determine if this relationship affects graft survival. PMID:18387408

  8. Survival analysis and visual outcome in a large series of corneal transplants in India.

    Science.gov (United States)

    Dandona, L; Naduvilath, T J; Janarthanan, M; Ragu, K; Rao, G N

    1997-09-01

    keratoconus (9.99 (6.10-16.36)) or corneal dystrophies (1.77 (1.21-2.58)) than for the other preoperative diagnoses. Reasonable success with corneal transplantation is possible in the developing world if data from this part of the world regarding the different survival rates for the various preoperative diagnoses and the influence of risk factors on transplant survival and visual outcome are taken into account while determining priority for transplant cases in the present situation of limited availability of donor corneas.

  9. Porcine models of muscular dystrophy

    Science.gov (United States)

    Duchenne muscular dystrophy is a progressive, fatal, X-linked disease caused by a failure to accumulate the cytoskeletal protein, dystrophin. This disease is modeled by a variety of animal models including several fish models, mice, rats, and dogs. While these models have contributed substantially t...

  10. Benign concentric annular macular dystrophy

    Directory of Open Access Journals (Sweden)

    Luísa Salles de Moura Mendonça

    2015-06-01

    Full Text Available The purpose of the authors is to show clinical findings of a patient with benign concentric annular macular dystrophy, which is an unusual condition, and part of the "bull’s eye" maculopathy differential diagnosis. An ophthalmologic examination with color perception, fluorescein angiography, and ocular electrophysiology was performed.

  11. Myotonic Dystrophy: An Anaesthetic Dilemma

    Directory of Open Access Journals (Sweden)

    N Gupta

    2009-01-01

    Full Text Available Myotonic dystrophy (dystrophia myotonica, DM is a chronic, slowly progressing, highly variable inherited multisystemic disease that can manifest at any age from birth to old age. We present a 32-year-old female with adenexal mass posted for exploratory laparotomy. She was a known case of dilated cardiomyopathy (DCMP.The ECG suggested incomplete RBBB& LAHB& the ECHO revealed mild mitral regurgitation, tricuspid regurgitation, pulmonary artery hypertension with severe left ventricular dysfunction (ejection fraction of 30-35 %. General anaes-thesia (GA with epidural anaesthesia was planned. The patient was haemodynamically stable through out the surgi-cal procedure. The patient was reversed and shifted to post anaesthesia care unit. On the 2nd postoperative day patient developed respiratory distress and hypotension. ABG revealed Type 1 respiratory failure. Since the patient didn′t improve with oxygen therapy and nebulisation, she was intubated and shifted to ICU. Patient was tolerating the tube without sedation and relaxants so, consultant anaesthesiologist asked for neurologist referral to rule out myotonic dystrophy. Subsequent muscle biopsy and genetic analysis was suggestive of myotonic dystrophy. Despite all possible efforts we were unable to wean her off the ventilator for 390 days. Patients with myotonic dystrophy are a challenge to the attending anaesthesiologist. These patients can be very well managed with preoperative optimized medical treatment and well-planned perioperative care.

  12. Prednisone Therapy for Duchenne Dystrophy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2005-02-01

    Full Text Available The effects of prednisone on muscle function and the extent of steroid-related adverse effects were studied in 17 ambulant children with Duchenne muscular dystrophy (DMD at University Hospital, Groningen; Rehabilitation Centre, Utrecht; and Leiden University Medical Centre, the Netherlands.

  13. Glucocorticoids for Duchenne Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2013-07-01

    Full Text Available Investigators at the Dubowitz Neuromuscular Centre, Great Ormond Street Hospital, and other centers in the UK, conducted a prospective longitudinal study across 17 neuromuscular centers in the UK of 360 boys aged 3-15 years with Duchenne muscular dystrophy who were treated with daily or intermittent (10 days on/10 days off prednisolone for a mean duration of 4 years.

  14. AMPUTATION AND REFLEX SYMPATHETIC DYSTROPHY

    NARCIS (Netherlands)

    GEERTZEN, JHB; EISMA, WH

    Reflex sympathetic dystrophy is a chronic pain syndrome characterized by chronic burning pain, restricted range of motion, oedema and vasolability. Patients are difficult to treat and the prognosis is very often poor. This report emphasizes that an amputation in case of a reflex sympathetic

  15. Genetics Home Reference: Emery-Dreifuss muscular dystrophy

    Science.gov (United States)

    ... Health Conditions Emery-Dreifuss muscular dystrophy Emery-Dreifuss muscular dystrophy Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Emery-Dreifuss muscular dystrophy is a condition that primarily affects muscles used ...

  16. Genetics Home Reference: limb-girdle muscular dystrophy

    Science.gov (United States)

    ... Health Conditions Limb-girdle muscular dystrophy Limb-girdle muscular dystrophy Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Limb-girdle muscular dystrophy is a term for a group of diseases ...

  17. Genetics Home Reference: LAMA2-related muscular dystrophy

    Science.gov (United States)

    ... Health Conditions LAMA2-related muscular dystrophy LAMA2-related muscular dystrophy Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description LAMA2 -related muscular dystrophy is a disorder that causes weakness and wasting ( ...

  18. Role of corneal collagen fibrils in corneal disorders and related pathological conditions

    Directory of Open Access Journals (Sweden)

    Hong-Yan Zhou

    2017-05-01

    Full Text Available The cornea is a soft tissue located at the front of the eye with the principal function of transmitting and refracting light rays to precisely sense visual information. Corneal shape, refraction, and stromal stiffness are to a large part determined by corneal fibrils, the arrangements of which define the corneal cells and their functional behaviour. However, the modality and alignment of native corneal collagen lamellae are altered in various corneal pathological states such as infection, injury, keratoconus, corneal scar formation, and keratoprosthesis. Furthermore, corneal recuperation after corneal pathological change is dependent on the balance of corneal collagen degradation and contraction. A thorough understanding of the characteristics of corneal collagen is thus necessary to develop viable therapies using the outcome of strategies using engineered corneas. In this review, we discuss the composition and distribution of corneal collagens as well as their degradation and contraction, and address the current status of corneal tissue engineering and the progress of corneal cross-linking.

  19. Genetics Home Reference: infantile neuroaxonal dystrophy

    Science.gov (United States)

    ... optic nerve) often occur in infantile neuroaxonal dystrophy . Hearing loss may also develop. Children with this disorder experience progressive deterioration of cognitive functions (dementia), and ...

  20. Corneal structure and transparency

    Science.gov (United States)

    Meek, Keith M.; Knupp, Carlo

    2015-01-01

    The corneal stroma plays several pivotal roles within the eye. Optically, it is the main refracting lens and thus has to combine almost perfect transmission of visible light with precise shape, in order to focus incoming light. Furthermore, mechanically it has to be extremely tough to protect the inner contents of the eye. These functions are governed by its structure at all hierarchical levels. The basic principles of corneal structure and transparency have been known for some time, but in recent years X-ray scattering and other methods have revealed that the details of this structure are far more complex than previously thought and that the intricacy of the arrangement of the collagenous lamellae provides the shape and the mechanical properties of the tissue. At the molecular level, modern technologies and theoretical modelling have started to explain exactly how the collagen fibrils are arranged within the stromal lamellae and how proteoglycans maintain this ultrastructure. In this review we describe the current state of knowledge about the three-dimensional stromal architecture at the microscopic level, and about the control mechanisms at the nanoscopic level that lead to optical transparency. PMID:26145225

  1. CLINICAL OUTCOME OF PENETRATING KERATOPLASTY IN CORNEAL OPACITIES OF DIFFERENT AETIOLOGY- A CLINICAL STUDY

    Directory of Open Access Journals (Sweden)

    Dipak Bhuyan

    2017-01-01

    Full Text Available BACKGROUND Corneal transplantation or grafting is an operation in which abnormal corneal host tissue is replaced by healthy donor cornea. According to the World Health Organization (WHO, corneal diseases are among the major causes of vision loss and blindness in the world today after cataract and glaucoma. The aim of the study is to- 1. Evaluate the different aetiology of corneal opacity including active infective aetiology as indicated for penetrating keratoplasty. 2. Determine the clinical outcome of penetrating keratoplasty in relation to graft survival, graft rejection and peri-operative complications in different aetiology groups. 3. Determine the final visual outcome. MATERIALS AND METHODS Candidates for keratoplasty were selected from- 1. Eye Bank of Regional Institute of Ophthalmology (R.I.O. 2. R.I.O OPD. The study period was from September 2014 to August 2015. 30 cases were taken in the study. Descriptive statistics were applied to analyse the data wherever necessary. RESULTS 34.6±19.73 yrs. (mean±SD was the mean age at which transplants were done in the study. Out of total 30 cases, 13 (43.33% and 17 (56.66% were male and female, respectively. The different indications for penetrating keratoplasty are- Post ulcer corneal opacity in 14 cases (46.66%, posttraumatic corneal opacity 9 cases (30%, pseudophakic bullous keratopathy 4 cases (13.33%, corneal dystrophy in 2 cases (6.66% and non-healing corneal ulcer in 1 case (3.33%. 16 cases (53.33% showed clear graft till the last follow up while 11 (33.33% cases showed partially clear graft resulting in improved visual outcome while 3 cases (10.00% of the grafts were opaque due to graft failure. CONCLUSION The major indications for penetrating keratoplasty in this part of the world are post ulcer and posttraumatic corneal opacity and majority of them are illiterate agricultural workers who failed to get adequate treatment on time. Graft survival rate is high, which can be attributed to the

  2. Tissue Engineering of Corneal Endothelium

    Directory of Open Access Journals (Sweden)

    Satoru Yamagami

    2012-10-01

    Full Text Available Human corneal endothelial cells (HCECs do not replicate after wounding. Therefore, corneal endothelial deficiency can result in irreversible corneal edema. Descemet stripping automated endothelial keratoplasty (DSAEK allows selective replacement of the diseased corneal endothelium. However, DSAEK requires a donor cornea and the worldwide shortage of corneas limits its application. This review presents current knowledge on the tissue engineering of corneal endothelium using cultured HCECs. We also provide our recent work on tissue engineering for DSAEK grafts using cultured HCECs. We reconstructed DSAEK grafts by seeding cultured DiI-labelled HCECs on collagen sheets. Then HCEC sheets were transplanted onto the posterior stroma after descemetorhexis in the DSAEK group. Severe stromal edema was detected in the control group, but not in the DSAEK group throughout the observation period. Fluorescein microscopy one month after surgery showed numerous DiI-labelled cells on the posterior corneal surface in the DSAEK group. Frozen sections showed a monolayer of DiI-labelled cells on Descemet’s membrane. These findings indicate that cultured adult HCECs, transplanted with DSAEK surgery, maintain corneal transparency after transplantation and suggest the feasibility of performing DSAEK with HCECs to treat endothelial dysfunction.

  3. Corneal Confocal Microscopy Detects Corneal Nerve Damage in Patients Admitted With Acute Ischemic Stroke.

    Science.gov (United States)

    Khan, Adnan; Akhtar, Naveed; Kamran, Saadat; Ponirakis, Georgios; Petropoulos, Ioannis N; Tunio, Nahel A; Dargham, Soha R; Imam, Yahia; Sartaj, Faheem; Parray, Aijaz; Bourke, Paula; Khan, Rabia; Santos, Mark; Joseph, Sujatha; Shuaib, Ashfaq; Malik, Rayaz A

    2017-11-01

    Corneal confocal microscopy can identify corneal nerve damage in patients with peripheral and central neurodegeneration. However, the use of corneal confocal microscopy in patients presenting with acute ischemic stroke is unknown. One hundred thirty patients (57 without diabetes mellitus [normal glucose tolerance], 32 with impaired glucose tolerance, and 41 with type 2 diabetes mellitus) admitted with acute ischemic stroke, and 28 age-matched healthy control participants underwent corneal confocal microscopy to quantify corneal nerve fiber density, corneal nerve branch density, and corneal nerve fiber length. There was a significant reduction in corneal nerve fiber density, corneal nerve branch density, and corneal nerve fiber length in stroke patients with normal glucose tolerance ( P stroke. Corneal confocal microscopy is a rapid noninvasive ophthalmic imaging technique that identifies corneal nerve fiber loss in patients with acute ischemic stroke. © 2017 American Heart Association, Inc.

  4. Morbidity in reflex sympathetic dystrophy

    OpenAIRE

    Murray, C; Cohen, A.; Perkins, T.; Davidson, J; Sills, J

    2000-01-01

    Reflex sympathetic dystrophy (RSD), an unusual diagnosis in general paediatrics, is well recognised by paediatric rheumatologists. This study reports the presentation and the clinical course of 46 patients (35 female, age range 8-15.2) with RSD. The patients saw professionals from an average of 2.3 specialties (range 1-5). Twenty five (54%) had a history of trauma. Median time to diagnosis was 12 weeks (range 1-130). Many children had multiple investigations and treatments. Once d...

  5. Myotonic dystrophy in Ancient Egypt.

    Science.gov (United States)

    Cattaino, G; Vicario, L

    1999-01-01

    Amenhotep IV, better known as Akhenaton, the heretical pharaoh, was a king of the New Kingdom of Ancient Egypt. Statues and reliefs of him show an unhealthy man whose body has abnormal features. By studying the pictures of Akhenaton (the mummy has not yet been found), we conclude that he may have been affected by myotonic dystrophy (MD). Moreover, the available data on his family suggest that MD may have caused the end of the royal bloodline of the Eighteenth Dynasty.

  6. Corneal Biomechanical Findings in Contact Lens Induced Corneal Warpage

    OpenAIRE

    Fateme Alipour; Mojgan Letafatnejad; Amir Hooshang Beheshtnejad; Seyed-Farzad Mohammadi; Seyed Reza Ghaffary; Narges Hassanpoor; Mehdi Yaseri

    2016-01-01

    Purpose. To evaluate the difference in biomechanical properties between contact lens induced corneal warpage and normal and keratoconic eyes. Method. Prospective observational case control study, where 94 eyes of 47 warpage suspicious and 46 eyes of 23 keratoconic patients were included. Warpage suspected cases were followed until a definite diagnosis was made (warpage, normal, or keratoconus). Results. 44 eyes of 22 patients had contact lens related corneal warpage. 46 eyes of 23 people were...

  7. Intrastromal Corneal Ring Implants for Corneal Thinning Disorders

    Science.gov (United States)

    2009-01-01

    Executive Summary Objective The purpose of this project was to determine the role of corneal implants in the management of corneal thinning disease conditions. An evidence-based review was conducted to determine the safety, effectiveness and durability of corneal implants for the management of corneal thinning disorders. The evolving directions of research in this area were also reviewed. Subject of the Evidence-Based Analysis The primary treatment objectives for corneal implants are to normalize corneal surface topography, improve contact lens tolerability, and restore visual acuity in order to delay or defer the need for corneal transplant. Implant placement is a minimally invasive procedure that is purported to be safe and effective. The procedure is also claimed to be adjustable, reversible, and both eyes can be treated at the same time. Further, implants do not limit the performance of subsequent surgical approaches or interfere with corneal transplant. The evidence for these claims is the focus of this review. The specific research questions for the evidence review were as follows: Safety Corneal Surface Topographic Effects: Effects on corneal surface remodelling Impact of these changes on subsequent interventions, particularly corneal transplantation (penetrating keratoplasty [PKP]) Visual Acuity Refractive Outcomes Visual Quality (Symptoms): such as contrast vision or decreased visual symptoms (halos, fluctuating vision) Contact lens tolerance Functional visual rehabilitation and quality of life Patient satisfaction: Disease Process: Impact on corneal thinning process Effect on delaying or deferring the need for corneal transplantation Clinical Need: Target Population and Condition Corneal ectasia (thinning) comprises a range of disorders involving either primary disease conditions such as keratoconus and pellucid marginal corneal degeneration or secondary iatrogenic conditions such as corneal thinning occurring after LASIK refractive surgery. The condition

  8. [Transplantation of corneal endothelial cells].

    Science.gov (United States)

    Amano, Shiro

    2002-12-01

    Though conventional corneal transplantation has achieved great success, it still has several drawbacks including limited availability of donor corneas, recurrent allograft rejection, and subsequent graft failure in certain cases. Reconstructing clinically usable corneas by applying the technology of regenerative medicine can offer a solution to these problems, as well as making corneal transplantation a non-emergency surgery and enabling the usage of banked corneal cells. In the present study, we focused on corneal endothelium that is critical for corneal transparency and investigated the reconstruction of cornea utilizing cultured human corneal endothelial cells (HCECs). We succeeded in steadily culturing HCECs by using culture dishes pre-coated with extracellular matrix produced by calf corneal endothelial cells and culture media that contained basic fibroblast growth factor and fetal bovine serum. We performed the following analysis utilizing these cultured HCECs. The older the donor was, the more frequently large senescent cells appeared in the passaged HCECs. The telomeres of HCECs were measured as terminal restriction fragments (TRF) by Southern blotting. HCECs, in vivo from donors in their seventies had a long TRFs of over 12 kilobases. Passaging shortened the TRFs but there was no difference in TRFs among donors of various ages. These results indicated that shortening of telomere length is not related to senescence of HCECs. We investigated the role of advanced glycation end products (AGEs) in the senescence of in vivo HCECs. The results indicated that AGE-protein in the aqueous humor is endocytosed into HCECs via AGE receptors expressed on the surface of HCECs and damages HCECs by producing reactive oxygen species and inducing apoptosis, suggesting that AGEs, at least partly, cause the senescence of HECEs. HCECs were cultured using adult human serum instead of bovine serum to get rid of bovine material that can be infected with prions. Primary and passage

  9. Myotonic dystrophy: a retrospective diagnosis | Jain | Southern ...

    African Journals Online (AJOL)

    Myotonic dystrophy is not commonly encountered in anaesthetic practice and its existence in a patient can easily go undetected, leading to intraoperative and postoperative complications. We report a case of a 45-year-old female without any typical features of myotonic dystrophy, who presented at our hospital for a ...

  10. Corneal epithelium following penetrating keratoplasty.

    OpenAIRE

    Tsubota, K; Mashima, Y; Murata, H; Yamada, M.; Sato, N.

    1995-01-01

    AIMS--This study was designed to observe any changes to the corneal epithelium after penetrating keratoplasty. METHODS--The corneal epithelia of 26 patients were observed by specular microscopy 1 week, 1 month, 3 months, and 6 months following penetrating keratoplasty. RESULTS--After re-epithelialisation was confirmed by biomicroscopy 1 week after surgery, specular microscopy revealed many abnormal cells, including spindle shaped cells, nucleated cells, large cells, as well as irregular cell ...

  11. Corneal Topographic Changes After Eyelid Ptosis Surgery.

    Science.gov (United States)

    Savino, Gustavo; Battendieri, Remo; Riso, Monica; Traina, Salvatore; Poscia, Andrea; DʼAmico, Giovanni; Caporossi, Aldo

    2016-04-01

    To evaluate the corneal topography and the topographic changes after ptosis surgery on patients affected by congenital and acquired blepharoptosis. Twenty eyes of 17 patients affected by acquired and congenital ptosis underwent surgical correction through anterior levator complex tightening. Computerized tomography (Syrius Sistem; CSO) was used to analyze any change in corneal astigmatism (CYL), simulated keratometry, anterior corneal symmetry index front, apical keratometry front, and central corneal thickness. Visual acuity, margin reflex distance, and levator function were also measured. After surgical ptosis repair, corneal topography demonstrated a reduction in average keratometry of 0.15 ± 0.47 diopters (D) and in corneal astigmatism of 0.26 ± 1.12 D. Significant differences were found in apical keratometry front (-1.84 ± 1.76 D) and in best-corrected visual acuity (-0.18 ± 0.06 logMAR) in the postoperative examinations. Central corneal thickness did not show significant differences between preoperative and postoperative examinations. Postoperative topographic maps showed a reduction of symmetry index front (0.10 ± 0.64 D). Eyelid ptosis modifies anterior corneal surface inducing refractive errors and modifying corneal astigmatism in patients, thus affecting the quality of vision. The surgical correction of blepharoptosis induces anterior corneal surface modification, restoring corneal symmetry and regular corneal astigmatism. Postoperative corneal topography showed normal corneal contours.

  12. Biomechanical model of corneal transplantation.

    Science.gov (United States)

    Cabrera Fernández, D; Niazy, A M; Kurtz, R M; Djotyan, G P; Juhasz, T

    2006-03-01

    Refractive consequences of corneal transplants are analyzed using corneal biomechanical models assuming homogeneous and inhomogeneous stiffness distributions across the cornea. Additionally, refractive effects of grafts combined with volume removal procedures are also evaluated to develop methods to reduce postoperative refractive management of patients. Refinements of a two-dimensional finite element model are applied to simulate the biomechanical and refractive effects of different corneal transplant procedures: anterior lamellar keratoplasty, posterior lamellar keratoplasty, and penetrating keratoplasty. The models are based on a nonlinearly elastic, isotropic formulation. Predictions are compared with published clinical data. The model simulating the penetrating keratoplasty procedure predicts more change in the postoperative corneal curvature than models simulating anterior lamellar keratoplasty or posterior lamellar keratoplasty procedures. When a lenticle-shaped tissue with a central thickness of 50 microns and a diameter of 4 mm is removed from the anterior corneal surface along with the anterior lamellar keratoplasty or posterior lamellar keratoplasty, the models predict a refractive correction of -8.6 and -8.9 diopters, respectively. Simulations indicate that a posterior lamellar keratoplasty procedure is preferable for obtaining a better corneal curvature profile, eliminating the need for specific secondary treatments.

  13. Paradigm shifts in corneal transplantation.

    Science.gov (United States)

    Tan, Donald T H; Anshu, Arundhati; Mehta, Jodhbir S

    2009-04-01

    Conventional corneal transplantation, in the form of penetrating keratoplasty (PK), involves full-thickness replacement of the cornea, and is a highly successful procedure. However, the cornea is anatomically a multi-layered structure. Pathology may only affect individual layers of the cornea, hence selective lamellar surgical replacement of only the diseased corneal layers whilst retaining unaffected layers represents a new paradigm shift in the field. Recent advancements in surgical techniques and instrumentation have resulted in several forms of manual, microkeratome and femto-second laser-assisted lamellar transplantation procedures. Anterior lamellar keratoplasty (ALK) aims at replacing only diseased or scarred corneal stroma, whilst retaining the unaffected corneal endothelial layer, thus obviating the risk of endothelial allograft rejection. Posterior lamellar keratoplasty/endothelial keratoplasty (PLK/EK) involves the replacement of the dysfunctional endothelial cell layer only. Whilst significant technical and surgical challenges are involved in performing lamellar micro-dissection of a tissue which is only 0.5 mm thick, the benefits of a more controlled surgical procedure and improved graft survival rates have resulted in a shift away from conventional PK. This review details the current advances in emerging lamellar corneal surgical procedures and highlights the main advantages and disadvantages of these new lamellar corneal procedures.

  14. Corneal topography in cataract surgery.

    Science.gov (United States)

    Martinez, C E; Klyce, S D

    1996-02-01

    Keratometry and corneal topography remain the most important means of evaluating induced corneal changes after surgery and have comparable sensitivities in the paracentral region of the cornea. However, keratometry gives no information about the peripheral cornea or about asymmetry of the cornea. Videokeratography should be performed after cataract surgery in cases in which best-corrected visual acuity is not adequate and there are no other obvious causes for poor vision to determine whether corneal irregularities are present. The recent literature on corneal topographic evaluation of induced astigmatism after cataract surgery suggests that in general, smaller, temporal incisions result in less astigmatism. Preoperatively, corneal topography can be used in the calculation of intraocular lens power as well as incision planning. Postoperatively, it can be used to detect tight sutures, torsion of the wound, internal wound gape, and irregular astigmatism, as well as to guide suture removal. In the future, corneal topography will become increasingly important in the determination of intraocular lens power in difficult cases such as patients undergoing combined cataract extraction and penetrating keratoplasty as well as patients with a history of radial keratotomy or photorefractive surgery.

  15. CONTACT LENS RELATED CORNEAL ULCER

    Directory of Open Access Journals (Sweden)

    AGARWAL P

    2010-01-01

    Full Text Available A corneal ulcer caused by infection is one of the major causes of blindness worldwide. One of the recent health concerns is the increasing incidence of corneal ulcers associated with contact lens user especially if the users fail to follow specific instruction in using their contact lenses. Risk factors associated with increased risk of contact lens related corneal ulcers are:overnight wear, long duration of continuous wear, lower socio-economic classes, smoking, dry eye and poor hygiene. The presenting symptoms of contact lens related corneal ulcers include eye discomfort, foreign body sensation and lacrimation. More serious symptoms are redness (especially circum-corneal injection, severe pain, photophobia, eye discharge and blurring of vision. The diagnosis is established by a thorough slit lamp microscopic examination with fluorescein staining and corneal scraping for Gram stain and culture of the infective organism. Delay in diagnosing and treatment can cause permanent blindness, therefore an early referral to ophthalmologist and commencing of antimicrobial therapy can prevent visual loss.

  16. Corneal wound healing after excimer laser keratectomy.

    Science.gov (United States)

    Kaji, Yuichi; Yamashita, Hidetoshi; Oshika, Tetsuro

    2003-03-01

    Excimer laser keratectomy is widely used to correct refractive errors. Several complications of excimer laser keratectomy are reported including corneal infection, regression, corneal haze formation, glare and halo. Most of the complications are closely related to the corneal stromal wound healing process. In order to perform the excimer laser keratectomy with minimum complications, we should understand the mechanism of the corneal stroma wound healing process. In addition, such knowledge will help us to regulate the corneal stromal wound healing process in the future. In the present article, we discuss the molecular mechanism of the corneal stromal wound healing process after excimer laser keratectomy and its regulation by anti-inflammatory agents.

  17. Outcome of corneal transplantation in a private institution in Saudi Arabia

    Directory of Open Access Journals (Sweden)

    Omar N

    2013-06-01

    Full Text Available Nazri Omar,1,2 Charbel T Bou Chacra,1 Khalid F Tabbara1,3,4 1The Eye Center and The Eye Foundation for Research in Ophthalmology, Riyadh, Saudi Arabia; 2Department of Ophthalmology, Universiti Putra Malaysia, Serdang, Malaysia; 3Department of Ophthalmology, King Saud University, Riyadh, Saudi Arabia; 4The Wilmer Ophthalmological Institute of Johns Hopkins University School of Medicine, Baltimore, MD, USA Background: The aim of this work was to describe the indications, complications, and outcomes of penetrating keratoplasty (PKP in Saudi Arabia. Methods: In a retrospective, noncomparative interventional case series, the medical records of patients who underwent PKP from January 2000 to December 2008 and had a minimum follow-up of 6 months were reviewed. All corneas were obtained from eye banks in the US. Indications, complications, and outcomes of surgery were recorded. This study was approved by the institutional review board. Results: Eighty-five consecutive eyes were included in this study. There were 52 (61.2% males and 33 (38.8% females. The median age was 35.0 years (range 3–85 years, and the median follow-up period was 24 months (range 6–108 months. The indications for PKP were keratoconus, bullous keratopathy, corneal scars, corneal dystrophy, and corneal regraft. The overall graft survival time was 88.9 months ± 4.9 months (mean ± standard error of mean, 95% confidence interval [CI] 79.4 months -98.4 months while the 3-year and 5-year cumulative survival rates were 90.7% and 84.3%, respectively. Surgical indication (P = 0.038, immune rejection (P < 0.001, preoperative corneal vascularization (P = 0.022, and perioperative high intraocular pressure (P = 0.032 were associated significantly with corneal graft failure in univariate analysis. Multivariate analysis reduced these significant associations to rejection (P < 0.001 and vascularization (P = 0.009. Relative risk for failure in rejected cornea was 16.22 (95% CI 4.99–52.69 and

  18. Corneal thickness changes after corneal collagen crosslinking for keratoconus and corneal ectasia: one-year results.

    Science.gov (United States)

    Greenstein, Steven A; Shah, Vinnie P; Fry, Kristen L; Hersh, Peter S

    2011-04-01

    To determine the changes in corneal thickness over time after corneal collagen crosslinking (CXL) for keratoconus and corneal ectasia. Cornea and refractive surgery subspecialty practice. Prospective randomized controlled clinical trial. Corneal thickness at the apex, thinnest point, and pupil center were measured using Scheimpflug imaging (Pentacam) at baseline and 1, 3, 6, and 12 months after CXL. The treatment group was compared with both a sham-procedure control group and a fellow-eye control group. Associations with clinical outcomes (uncorrected and corrected distance visual acuities and maximum keratometry) were analyzed. The study comprised 82 eyes, 54 with keratoconus and 28 with ectasia after laser in situ keratomileusis. The mean preoperative thinnest pachymetry was 440.7 μm ± 52.9 (SD). After CXL, the cornea thinned at 1 month (mean change -23.8 ± 28.7 μm; Pdegree of corneal thinning at 3 months and clinical outcomes after CXL. After CXL, the cornea thins and then recovers toward baseline thickness. The cause and implications of corneal thickness changes after CXL remain to be elucidated. No author has a financial or proprietary interest in any material or method mentioned. Additional disclosure is found in the footnotes. Copyright © 2011 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.

  19. Corneal Sparing Conjunctival Abrasion

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    Hamid Ehsani-Nia

    2017-07-01

    Full Text Available History of present illness: A 15-year-old male was transported to the emergency department via ambulance as a trauma activation after being struck by an automobile while jogging. Patient was alert and oriented, with no focal neurological deficits and no loss of consciousness. The patient complained of right eye foreign body sensation. Significant findings: Physical exam was significant for multiple broken teeth, multiple minor abrasions on the face, and fine shards of shattered glass on his face and hair. His right eye had conjunctival injection, with no signs of subconjunctival hemorrhage or ocular penetration. Vision, extraocular movement, and pupillary exam were grossly intact. Fluorescein staining with slit lamp exam with cobalt blue filter examination of the right eye revealed superficial bulbar conjunctival uptake of fluorescein dye staining an area of the conjunctiva inferior to the limbus 5 mm vertical by 2 mm horizontal (estimation by photo provided. No foreign bodies were visualized in the inferior fornix. These findings were consistent with superficial conjunctival abrasion. The exam noted sparing of the corneal epithelium. Discussion: The conjunctiva is a thin, transparent membrane covering the ocular surface from the corneal limbus to the posterior eyelid margin.1 When damaged, the patient will classically have a “foreign body” sensation. It is important to identify the extent of the injury as not extending over the cornea, and also to search for lodged foreign bodies that damage the conjunctiva further with each blink. Classically retained foreign bodies will form a linear and vertical pattern of staining with fluorescein.2,3 Fluorescein stains expose basement membrane and fluoresces bright green under ultraviolet light, thus indicating areas of damage in contrast to its surrounding tissue.4,5 In the setting of acute trauma, urgent Ophthalmologic consultation is indicated if there is anterior chamber hemorrhage, a ruptured or

  20. Central corneal thickness and corneal hysteresis associated with glaucoma damage.

    Science.gov (United States)

    Congdon, Nathan G; Broman, Aimee T; Bandeen-Roche, Karen; Grover, Davinder; Quigley, Harry A

    2006-05-01

    We sought to measure the impact of central corneal thickness (CCT), a possible risk factor for glaucoma damage, and corneal hysteresis, a proposed measure of corneal resistance to deformation, on various indicators of glaucoma damage. Observational study. Adult patients of the Wilmer Glaucoma Service underwent measurement of hysteresis on the Reichert Ocular Response Analyzer and measurement of CCT by ultrasonic pachymetry. Two glaucoma specialists (H.A.Q., N.G.C.) reviewed the chart to determine highest known intraocular pressure (IOP), target IOP, diagnosis, years with glaucoma, cup-to-disk ratio (CDR), mean defect (MD), pattern standard deviation (PSD), glaucoma hemifield test (GHT), and presence or absence of visual field progression. Among 230 subjects, the mean age was 65 +/- 14 years, 127 (55%) were female, 161 (70%) were white, and 194 (85%) had a diagnosis of primary open-angle glaucoma (POAG) or suspected POAG. In multivariate generalized estimating equation models, lower corneal hysteresis value (P = .03), but not CCT, was associated with visual field progression. When axial length was included in the model, hysteresis was not a significant risk factor (P = .09). A thinner CCT (P = .02), but not hysteresis, was associated with a higher CDR at the most recent examination. Neither CCT nor hysteresis was associated with MD, PSD, or GHT "outside normal limits." Thinner CCT was associated with the state of glaucoma damage as indicated by CDR. Axial length and corneal hysteresis were associated with progressive field worsening.

  1. Rehabilitation of the muscular dystrophies.

    Science.gov (United States)

    Pangilinan, Percival H; Hornyak, Joseph E

    2013-01-01

    The muscular dystrophies (MD) are a heterogeneous group of inherited disorders characterized by findings on muscle biopsy. In general, they feature progressive muscle wasting and weakness. In addition to the musculoskeletal system, direct and indirect effects can be seen in a variety of organ systems. These issues create challenges in patients with MD for ambulation and mobility, self-care, pain, fatigue, and community involvement. Because of its progressive nature and wide variety of pathophysiological mechanisms, patients with MD require individualized rehabilitation care. This chapter reviews specific rehabilitation needs and treatment of patients with MD. Copyright © 2013 Elsevier B.V. All rights reserved.

  2. Substrates for Expansion of Corneal Endothelial Cells towards Bioengineering of Human Corneal Endothelium

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    Jesintha Navaratnam

    2015-09-01

    Full Text Available Corneal endothelium is a single layer of specialized cells that lines the posterior surface of cornea and maintains corneal hydration and corneal transparency essential for vision. Currently, transplantation is the only therapeutic option for diseases affecting the corneal endothelium. Transplantation of corneal endothelium, called endothelial keratoplasty, is widely used for corneal endothelial diseases. However, corneal transplantation is limited by global donor shortage. Therefore, there is a need to overcome the deficiency of sufficient donor corneal tissue. New approaches are being explored to engineer corneal tissues such that sufficient amount of corneal endothelium becomes available to offset the present shortage of functional cornea. Although human corneal endothelial cells have limited proliferative capacity in vivo, several laboratories have been successful in in vitro expansion of human corneal endothelial cells. Here we provide a comprehensive analysis of different substrates employed for in vitro cultivation of human corneal endothelial cells. Advances and emerging challenges with ex vivo cultured corneal endothelial layer for the ultimate goal of therapeutic replacement of dysfunctional corneal endothelium in humans with functional corneal endothelium are also presented.

  3. Quiste dermoide corneal bilateral

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    Michel Guerra Almaguer

    Full Text Available El dermoide es un tipo de coristoma (tejido embrionario normal en una localización anormal que afecta con frecuencia la córnea. Aparece como una masa sólida, blanca, redonda y elevada. Suele localizarse en el limbo inferotemporal, aunque puede hacerlo en cualquier lugar del globo ocular o de la órbita. Se presenta un paciente masculino, de 8 años de edad, quien desde su nacimiento muestra una mancha blanca en ambos ojos, agudeza visual sin corrección de movimiento de mano a 33 centímetros en el ojo derecho y percepción luminosa en el ojo izquierdo. En la exploración oftalmológica de ambos ojos se apreciaron lesiones blanquecinas sobre la córnea. Se le realizó exéresis del quiste y queratoplastia lamelar de ambos ojos, con resultados visuales satisfactorios. Se concluye que el caso presenta un quiste dermoide corneal bilateral.

  4. Corneal biomechanics: a review.

    Science.gov (United States)

    Piñero, David P; Alcón, Natividad

    2015-03-01

    Biomechanics is often defined as 'mechanics applied to biology'. Due to the variety and complexity of the behaviour of biological structures and materials, biomechanics is better defined as the development, extension and application of mechanics for a better understanding of physiology and physiopathology and consequently for a better diagnosis and treatment of disease and injury. Different methods for the characterisation of corneal biomechanics are reviewed in detail, including those that are currently commercially available (Ocular Response Analyzer and CorVis ST). The clinical applicability of the parameters provided by these devices are discussed, especially in the fields of glaucoma, detection of ectatic disorders and orthokeratology. Likewise, other methods are also reviewed, such as Brillouin microscopy or dynamic optical coherence tomography and others with potential application to clinical practice but not validated for in vivo measurements, such as ultrasonic elastography. Advantages and disadvantages of all these techniques are described. Finally, the concept of biomechanical modelling is revised as well as the requirements for developing biomechanical models, with special emphasis on finite element modelling. © 2014 The Authors. Clinical and Experimental Optometry © 2014 Optometry Australia.

  5. Use of ultra-high-resolution optical coherence tomography to detect in vivo characteristics of Descemet's membrane in Fuchs' dystrophy.

    Science.gov (United States)

    Shousha, Mohamed Abou; Perez, Victor L; Wang, Jianhua; Ide, Takeshi; Jiao, Shuliang; Chen, Qi; Chang, Victoria; Buchser, Nancy; Dubovy, Sander R; Feuer, William; Yoo, Sonia H

    2010-06-01

    To demonstrate the capability of ultra-high-resolution (UHR) anterior segment optical coherence tomography (OCT) to image Descemet's membrane (DM) and measure its thickness in vivo. (2) To evaluate the use of DM characteristics and thickness in the diagnosis of Fuchs' dystrophy. Case-control study. Twenty eyes of 12 Fuchs' dystrophy patients, 20 eyes of 13 young normal, and 20 eyes of 15 elderly normal subjects. Subjects were imaged using novel, custom-built UHR-OCT. Images were used to describe the characteristics of DM. Custom-made software was used to measure DM thickness and central corneal thickness (CCT). Specimens of DM obtained from Fuchs' dystrophy patients who underwent endothelial keratoplasty (EK) were histopathologically examined. Regression analyses were used to assess the correlation of DM thickness measured by UHR-OCT in vivo and by light microscopy and to determine the intergroup correlations between age, CCT, and DM thickness. We assessed DM characteristics and thickness, CCT, and age. Using UHR-OCT, the DM seemed in normal young subjects as a single, opaque, smooth line and in normal elderly subjects as a band of 2 smooth opaque lines with a translucent space in between. In Fuchs' dystrophy, DM appeared as a thickened band of 2 opaque lines; the anterior line was smooth whereas the posterior line had a wavy and irregular appearance with areas of localized thickenings. The DM thickness measured in vivo by UHR-OCT correlated significantly with that measured by light microscopy in 5 Fuchs' dystrophy eyes that underwent EK. The average central thicknesses of DM in normal young, in normal elderly and in Fuchs' dystrophy eyes were 10+/-3, 16+/-2, and 34+/-11 microm, respectively (P<0.001). There was a significant correlation between age and DM thickness only in normal groups. In Fuchs' dystrophy patients, there was a significant correlation between CCT and DM thickness that was not significant for normal groups. Ultra-high-resolution OCT is an

  6. Femtosecond laser corneal refractive surgery

    Science.gov (United States)

    Kurtz, Ron M.; Spooner, Greg J. R.; Sletten, Karin R.; Yen, Kimberly G.; Sayegh, Samir I.; Loesel, Frieder H.; Horvath, Christopher; Liu, HsiaoHua; Elner, Victor; Cabrera, Delia; Muenier, Marie-Helene; Sacks, Zachary S.; Juhasz, Tibor

    1999-06-01

    We evaluated the efficacy, safety, and stability of femtosecond laser intrastromal refractive procedures in ex vivo and in vivo models. When compared with longer pulsewidth nanosecond or picosecond laser pulses, femtosecond laser-tissue interactions are characterized by significantly smaller and more deterministic photodisruptive energy thresholds, as well as reduced shock waves and smaller cavitation bubbles. We utilized a highly reliable, all-solid-state femtosecond laser system for all studies to demonstrate clinical practicality. Contiguous tissue effects were achieved by scanning a 5 μm focused laser spot below the corneal surface at pulse energies of approximately 2 - 4 microjoules. A variety of scanning patterns was used to perform three prototype procedures in animal eyes; corneal flap cutting, keratomileusis, and intrastromal vision correction. Superior dissection and surface quality results were obtained for lamellar procedures (corneal flap cutting and keratomileusis). Preliminary in vivo evaluation of intrastromal vision correction in a rabbit model revealed consistent and stable pachymetry changes, without significant inflammation or loss of corneal transparency. We conclude that femtosecond laser technology may be able to perform a variety of corneal refractive procedures with high precision, offering advantages over current mechanical and laser devices and techniques.

  7. Corneal complications of vernal keratoconjunctivitis.

    Science.gov (United States)

    Solomon, Abraham

    2015-10-01

    Vernal keratoconjunctivitis (VKC) is a severe bilateral chronic allergic inflammatory disease of the ocular surface. In most of the cases, the disease is limited to the tarsal conjunctiva and to the limbus. However, in the more severe cases, the cornea may be involved, leading to potentially sight threatening complications. Prompt recognition of these complications is crucial in the management of VKC, which is one of the most severe ocular allergic diseases. A vicious cycle of inflammation occurs as a result of a set of reciprocal interactions between the conjunctiva and the cornea, which results in damage to the corneal epithelium and corneal stoma, and to the formation of shield ulcers and plaques, infectious keratitis, keratoconus, scarring, and limbal stem cell deficiency. These corneal complications can cause permanent decrease or loss of vision in children suffering from VKC. Corneal complications in VKC are the result of an on-going process of uncontrolled inflammation. Proper recognition of the corneal complications in VKC is crucial, as most of these can be managed or prevented by a combination of medical and surgical measures.

  8. Duchenne muscular dystrophy: current cell therapies.

    Science.gov (United States)

    Sienkiewicz, Dorota; Kulak, Wojciech; Okurowska-Zawada, Bożena; Paszko-Patej, Grażyna; Kawnik, Katarzyna

    2015-07-01

    Duchenne muscular dystrophy is a genetically determined X-linked disease and the most common, progressive pediatric muscle disorder. For decades, research has been conducted to find an effective therapy. This review presents current therapeutic methods for Duchenne muscular dystrophy, based on scientific articles in English published mainly in the period 2000 to 2014. We used the PubMed database to identify and review the most important studies. An analysis of contemporary studies of stem cell therapy and the use of granulocyte colony-stimulating factor (G-CSF) in muscular dystrophy was performed.

  9. An accessible approach for corneal topography

    OpenAIRE

    André Luís Beling da Rosa

    2013-01-01

    Corneal topography consists of measuring the corneal shape, which is a key factor for visual acuity. The exam is used, for instance, in keratoconus detection, personalized contact lens fitting, in pre- and post-procedures associated with refractive surgery and corneal transplants. This thesis presents an accessible, inexpensive and portable approach to perform corneal topographies. The results obtained with our prototype show a mean difference of about 0.02 millimeters, equivalent to 0.5% of ...

  10. Progress of research on corneal collagen cross-linking for corneal melting

    Directory of Open Access Journals (Sweden)

    Ke-Ren Xiao

    2016-06-01

    Full Text Available Corneal collagen cross-linking(CXLcould increase the mechanical strength, biological stability and halt ectasia progression due to covalent bond formed by photochemical reaction between ultraviolet-A and emulsion of riboflavin between collagen fibers in corneal stroma. Corneal melting is an autoimmune related noninfectious corneal ulcer. The mechanism of corneal melting, major treatment, the basic fundamental of ultraviolet-A riboflavin induced CXL and the clinical researches status and experiment in CXL were summarized in the study.

  11. Ocular dimensions, corneal thickness, and corneal curvature in quarter horses with hereditary equine regional dermal asthenia.

    Science.gov (United States)

    Badial, Peres R; Cisneros-Àlvarez, Luis Emiliano; Brandão, Cláudia Valéria S; Ranzani, José Joaquim T; Tomaz, Mayana A R V; Machado, Vania M; Borges, Alexandre S

    2015-09-01

    The aim of this study was to compare ocular dimensions, corneal curvature, and corneal thickness between horses affected with hereditary equine regional dermal asthenia (HERDA) and unaffected horses. Five HERDA-affected quarter horses and five healthy control quarter horses were used. Schirmer's tear test, tonometry, and corneal diameter measurements were performed in both eyes of all horses prior to ophthalmologic examinations. Ultrasonic pachymetry was performed to measure the central, temporal, nasal, dorsal, and ventral corneal thicknesses in all horses. B-mode ultrasound scanning was performed on both eyes of each horse to determine the dimensions of the ocular structures and to calculate the corneal curvature. Each corneal region examined in this study was thinner in the affected group compared with the healthy control group. However, significant differences in corneal thickness were only observed for the central and dorsal regions. HERDA-affected horses exhibited significant increases in corneal curvature and corneal diameter compared with unaffected animals. The ophthalmologic examinations revealed mild corneal opacity in one eye of one affected horse and in both eyes of three affected horses. No significant between-group differences were observed for Schirmer's tear test, intraocular pressure, or ocular dimensions. Hereditary equine regional dermal asthenia-affected horses exhibit decreased corneal thickness in several regions of the cornea, increased corneal curvature, increased corneal diameter, and mild corneal opacity. Additional research is required to determine whether the increased corneal curvature significantly impacts the visual accuracy of horses with HERDA. © 2014 American College of Veterinary Ophthalmologists.

  12. Acute corneal hydrops in keratoconus

    Directory of Open Access Journals (Sweden)

    Prafulla K Maharana

    2013-01-01

    Full Text Available Acute corneal hydrops is a condition characterized by stromal edema due to leakage of aqueous through a tear in descemet membrane. The patient presents with sudden onset decrease in vision, photophobia, and pain. Corneal thinning and ectasias combined with trivial trauma to the eye mostly by eye rubbing is considered as the underlying cause. With conservative approach self-resolution takes around 2 to 3 months. Surgical intervention is required in cases of non-resolution of corneal edema to avoid complications and for early visual rehabilitation. Intracameral injection of air or gas such as perflouropropane is the most common surgical procedure done. Recent investigative modality such as anterior segment optical coherence tomography is an extremely useful tool for diagnosis, surgical planning, and postoperative follow up. Resolution of hydrops may improve the contact lens tolerance and visual acuity but most cases require keratoplasty for visual rehabilitation.

  13. [Corneal toxicity due to amantadine].

    Science.gov (United States)

    Avendaño-Cantos, E M; Celis-Sánchez, J; Mesa-Varona, D; Gálvez-Martínez, J; López-Arroquia, E; González Del Valle, F

    2012-09-01

    A 64 year-old female with Parkinson disease treated with amantadine for two years who suddenly suffered bilateral corneal oedema. It was initially treated as herpetic endotheliitis without improvement as we lacked information on her chronic treatment. The corneal oedema finally resolved after withdrawing the drug. Amantadine hydrochloride may produce endothelial dysfunction. Once the amantadine treatment is stopped, the corneal oedema may be reversible but endothelial density remains low. An ophthalmologist examination should be performed before the initiation of amantadine treatment in order to establish a risk: benefit ratio, especially in those patients with low endothelial density or any endothelial anomaly. Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

  14. Posterior Corneal Surface Stability after Femtosecond Laser-Assisted Keratomileusis

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    Carlo Cagini

    2015-01-01

    Full Text Available The purpose of this study was to evaluate posterior corneal surface variation after femtosecond laser-assisted keratomileusis in patients with myopia and myopic astigmatism. Patients were evaluated by corneal tomography preoperatively and at 1, 6, and 12 months. We analyzed changes in the posterior corneal curvature, posterior corneal elevation, and anterior chamber depth. Moreover, we explored correlation between corneal ablation depth, residual corneal thickness, percentage of ablated corneal tissue, and preoperative corneal thickness. During follow-up, the posterior corneal surface did not have a significant forward corneal shift: no significant linear relationships emerged between the anterior displacement of the posterior corneal surface and corneal ablation depth, residual corneal thickness, or percentage of ablated corneal tissue.

  15. Changes in Technique and Indications for Keratoplasty in Poland, 1989 to 2014: An Analysis of Corneal Transplantations Performed at Saint Barbara Hospital, Trauma Center, Sosnowiec, Poland.

    Science.gov (United States)

    Jankowska-Szmul, J; Dobrowolski, D; Krysik, K; Kwas, J; Nejman, M; Wylegala, E

    2016-06-01

    The purpose of this work was to study the evolving trends in techniques and indications for corneal transplantation in Poland. This retrospective, descriptive analysis of corneal transplantations was performed at the Ophthalmology Department of Saint Barbara Hospital, Trauma Center, Sosnowiec, Poland, between 1988 and 2014. Structure of indications and surgery type rates over 26 years were tabulated by means of 5-year intervals. Between 1989 and 2014, 1762 corneal transplantations were performed, including 1375 (78%) cases of penetrating keratoplasty, 137 (8%) lamellar keratoplasty, 112 (6%) patch grafts, and 138 (8%) keratolimbal allografts. The major indications and their respective overall percentage were corneal leucoma (24%), pseudophakic/aphakic bullous keratopathy (22%), Fuchs dystrophy (13%), keratoconus (13%), re-graft (11%), keratitis (9%), and limbal stem cell deficiency (8%), with changes in relative frequency of the leading indications over the following time intervals. The number of corneal transplantations gradually increased during the years reviewed. The rates of lamellar, keratolimbal, and patch grafts have grown dynamically since 2010, reaching 20%, 17%, and 12%, respectively, of procedures performed during 2010 to 2014. Over the past quarter of a century, there has been evolution in corneal transplantation service, from the first penetrating keratoplasty to the wide spectrum of procedures, including femtosecond laser-assisted keratoplasty. Although, since 2010, the rates of lamellar and keratolimbal allografts have grown rapidly, penetrating keratoplasty has remained the leading technique. Copyright © 2016 Elsevier Inc. All rights reserved.

  16. Changing trends in corneal graft surgery: a ten-year review

    Science.gov (United States)

    de Sanctis, Ugo; Alovisi, Camilla; Bauchiero, Luigi; Caramello, Guido; Girotto, Gianfranco; Panico, Claudio; Vinai, Luisa; Genzano, Federico; Amoroso, Antonio; Grignolo, Federico

    2016-01-01

    AIM To review indications and corneal tissue use for penetrating and lamellar surgery between 2002 and 2011. METHODS The surgical reports of corneal grafts performed during 2002-2011, using tissues supplied by the Eye Bank of Piedmont (Italy), were reviewed retrospectively. Patient demographic data, date of intervention, indication for surgery, and surgical technique used were recorded. Surgical techniques included penetrating keratoplasty (PK), deep anterior lamellar keratoplasty (DALK) and endothelial keratoplasty (EK). The χ2 test was used to compare the distribution of indications and types of surgical technique used, for corneal grafts done during 2002-2006 versus those done during 2007-2011. RESULTS The number of corneal grafts increased by 30.7% from 2002-2006 to 2007-2011 (from 1567 to 2048). Comparing the two periods, both main indications and surgical techniques changed significantly. In 2007-2011, the proportion of interventions for aphakic/pseudophakic bullous keratopathy (from 16.8% to 21.3%), graft failure (from 16.4% to 19.1%) and Fuchs endothelial dystrophy (from 12.8% to 16.7%) all increased significantly (P<0.05), while those for keratoconus decreased significantly (from 35.6% to 27.3%; P<0.001). In 2007-2011, the proportion of PK decreased significantly (from 92.4% to 57.2%; P<0.001) while that of EK and DALK went from 0.4% to 30.2% (P<0.001) and from 7.2% to 12.6% (P<0.001) respectively. CONCLUSION During 2002-2011 the number of interventions increased significantly for corneal endothelial diseases and graft failure. The growing demand for interventions for these diseases corresponded to the widespread adoption of EK techniques. The use of DALK also increased, but more moderately than EK procedures. PMID:26949609

  17. Altered corneal stromal matrix organization is associated with mucopolysaccharidosis I, III and VI.

    Science.gov (United States)

    Alroy, J; Haskins, M; Birk, D E

    1999-05-01

    The presence of cloudy corneas is a prominent feature of mucopolysaccharidosis (MPS) types I and VI, but not MPS IIIA or IIIB. The cause of corneal cloudiness in MPS I and VI is speculative. Transparency of the cornea is dependent on the uniform diameter and the regular spacing and arrangement of the collagen fibrils within the stroma. Alterations in the spacing of collagen fibrils in a variety of conditions including corneal edema, scars, and macular corneal dystrophy is clinically manifested as corneal opacity. The purpose of this study was to compare the structural organization of the stromal extracellular matrix of normal corneas with that of MPS corneas. The size and arrangement of collagen fibrils in cloudy corneas from patients with MPS I were examined. The alterations observed were an increased mean fibril diameter with a broader distribution in the MPS corneas. The MPS I corneas also had altered fibril spacing and more irregular packing compared with normal control corneas. The clear corneas of patients with MPS IIIA and IIIB also showed increases in mean fibril diameter and fibril spacing. However, there was less variation indicating more regularity than seen in MPS I. In addition, corneas from cat models of certain MPS were compared to the human corneas. Cats with MPS I and VI, as well as normal control cats, were examined. Structural alterations comparable to those seen in human MPS corneas were seen in MPS I and VI cats relative to normal clear corneas. The findings suggest that cloudy corneas in MPS I and VI are in part a consequence of structural alterations in the corneal stroma, including abnormal spacing, size, and arrangement of collagen fibrils. Copyright 1999 Academic Press.

  18. Progress in corneal wound healing

    Science.gov (United States)

    Ljubimov, Alexander V.; Saghizadeh, Mehrnoosh

    2015-01-01

    Corneal wound healing is a complex process involving cell death, migration, proliferation, differentiation, and extracellular matrix remodeling. Many similarities are observed in the healing processes of corneal epithelial, stromal and endothelial cells, as well as cell-specific differences. Corneal epithelial healing largely depends on limbal stem cells and remodeling of the basement membrane. During stromal healing, keratocytes get transformed to motile and contractile myofibroblasts largely due to activation of transforming growth factor-β system. Endothelial cells heal mostly by migration and spreading, with cell proliferation playing a secondary role. In the last decade, many aspects of wound healing process in different parts of the cornea have been elucidated, and some new therapeutic approaches have emerged. The concept of limbal stem cells received rigorous experimental corroboration, with new markers uncovered and new treatment options including gene and microRNA therapy tested in experimental systems. Transplantation of limbal stem cell-enriched cultures for efficient re-epithelialization in stem cell deficiency and corneal injuries has become reality in clinical setting. Mediators and course of events during stromal healing have been detailed, and new treatment regimens including gene (decorin) and stem cell therapy for excessive healing have been designed. This is a very important advance given the popularity of various refractive surgeries entailing stromal wound healing. Successful surgical ways of replacing the diseased endothelium have been clinically tested, and new approaches to accelerate endothelial healing and suppress endothelial-mesenchymal transformation have been proposed including Rho kinase (ROCK) inhibitor eye drops and gene therapy to activate TGF-β inhibitor SMAD7. Promising new technologies with potential for corneal wound healing manipulation including microRNA, induced pluripotent stem cells to generate corneal epithelium, and

  19. What Are the Treatments for Muscular Dystrophy?

    Science.gov (United States)

    ... NICHD Research Information Find a Study More Information Preeclampsia and Eclampsia About NICHD Research Information Find a ... Facebook Twitter Pinterest Email Print What are the treatments for muscular dystrophy? No treatment is currently available ...

  20. Physical Therapy and Facioscapulohumeral Muscular Dystrophy (FSHD)

    Science.gov (United States)

    Physical Therapy & FSHD Facioscapulohumeral Muscular Dystrophy A Guide for Patients & Physical Therapists Authors: Wendy M. King, P.T., ... expertise and patient preferences. The goals of any physical therapy plan of care are to assist patients to:  ...

  1. An unusual variant of Becker muscular dystrophy

    NARCIS (Netherlands)

    de Visser, M.; Bakker, E.; Defesche, J. C.; Bolhuis, P. A.; van Ommen, G. J.

    1990-01-01

    We report on 5 brothers with slowly progressive limbgirdle weakness. Calf hypertrophy was absent. The levels of creatine kinase, electromyography, and findings from a muscle biopsy specimen were compatible with muscular dystrophy. The propositus's biopsy specimen also showed numerous rimmed

  2. Brain MRI Findings in Congenital Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2006-03-01

    Full Text Available Brain magnetic resonance imaging (MRI findings in 13 patients with congenital muscular dystrophy (MDCIC and Fukutin-related protein (FKRP gene mutations were retrospectively reviewed in a study at Hammersmith Hospital, London, UK, and European centers.

  3. Non-Coding RNAs in Muscle Dystrophies

    Directory of Open Access Journals (Sweden)

    Alessandra Ferlini

    2013-09-01

    Full Text Available ncRNAs are the most recently identified class of regulatory RNAs with vital functions in gene expression regulation and cell development. Among the variety of roles they play, their involvement in human diseases has opened new avenues of research towards the discovery and development of novel therapeutic approaches. Important data come from the field of hereditary muscle dystrophies, like Duchenne muscle dystrophy and Myotonic dystrophies, rare diseases affecting 1 in 7000–15,000 newborns and is characterized by severe to mild muscle weakness associated with cardiac involvement. Novel therapeutic approaches are now ongoing for these diseases, also based on splicing modulation. In this review we provide an overview about ncRNAs and their behavior in muscular dystrophy and explore their links with diagnosis, prognosis and treatments, highlighting the role of regulatory RNAs in these pathologies.

  4. Duchenne muscular dystrophy: current cell therapies

    OpenAIRE

    Sienkiewicz, Dorota; Kulak, Wojciech; Okurowska-Zawada, Bożena; Paszko-Patej, Grażyna; Kawnik, Katarzyna

    2015-01-01

    Duchenne muscular dystrophy is a genetically determined X-linked disease and the most common, progressive pediatric muscle disorder. For decades, research has been conducted to find an effective therapy. This review presents current therapeutic methods for Duchenne muscular dystrophy, based on scientific articles in English published mainly in the period 2000 to 2014. We used the PubMed database to identify and review the most important studies. An analysis of contemporary studies of stem cel...

  5. Duchenne muscular dystrophy: the management of scoliosis.

    Science.gov (United States)

    Archer, James E; Gardner, Adrian C; Roper, Helen P; Chikermane, Ashish A; Tatman, Andrew J

    2016-09-01

    This study summaries the current management of scoliosis in patients with Duchenne Muscular Dystrophy. A literature review of Medline was performed and the collected articles critically appraised. This literature is discussed to give an overview of the current management of scoliosis within Duchenne Muscular Dystrophy. Importantly, improvements in respiratory care, the use of steroids and improving surgical techniques have allowed patients to maintain quality of life and improved life expectancy in this patient group.

  6. Corneal Thickness as a Predictor of Corneal Transplant Outcome

    Science.gov (United States)

    Verdier, David D.; Sugar, Alan; Baratz, Keith; Beck, Roy; Dontchev, Mariya; Dunn, Steven; Gal, Robin L.; Holland, Edward J.; Kollman, Craig; Lass, Jonathan H.; Mannis, Mark J.; Penta, Jeffrey

    2013-01-01

    Purpose Assess corneal thickness (CT) and correlation with graft outcome after penetrating keratoplasty in the Cornea Donor Study. Methods 887 subjects with a corneal transplant for a moderate risk condition (principally Fuchs or pseudophakic corneal edema) had post-operative CT measurements throughout a 5 year follow up time. Relationships between baseline (recipient, donor, and operative) factors and CT were explored. Proportional hazards models were used to assess association between CT and graft failure. Relationship between CT and cell density was assessed with a longitudinal repeated measures model and Spearman correlation estimates. Results Higher longitudinal CT measurements were associated with diagnosis of pseudophakic or aphakic corneal edema (P 25mmHg during the first post-operative month (P=0.003), white (non-Hispanic) donor race (P=0.002) and respiratory causes of donor death (P600μm. In multivariate analysis, both 1 year CT and cell density were associated with subsequent graft failure (P=0.002 and 0.009). CT increase was modestly associated with endothelial cell loss during follow up (r=-0.29). Conclusion During the first 5 years following penetrating keratoplasty, CT can serve as a predictor of graft survival. However, CT is not a substitute for cell density measurement as both measures were independently predictive of graft failure. PMID:23343949

  7. Metalloproteinases in corneal diseases: degradation and processing.

    Science.gov (United States)

    Sakimoto, Tohru; Sawa, Mitsuru

    2012-11-01

    Matrix metalloproteinases (MMPs) are zinc-dependent endopeptidases with the potential to degrade all types of extracellular matrix. The ADAM (a disintegrin and metalloproteinase) family of peptidases was recently identified as cleaving the extracellular domain of transmembrane proteins. This was termed ectodomain shedding. We investigated the MMP expression in patients with corneal diseases and the potential role of ADAMs in corneal pathophysiology. We detected upregulation of the active form of MMP-2 and MMP-9 in the tear fluid from patients with corneal melting or recurrent corneal erosion. Using human corneal epithelial cells, we observed ADAM17-dependent ectodomain shedding of soluble tumor necrosis factor receptor 1 and soluble interleukin-6 (IL-6) receptor (sIL-6R). The production of sIL-6R was also induced by messenger RNA splicing in the human corneal epithelial cells. IL-6/sIL-6R-induced signal transducer and activator of transcription 3 phosphorylation was observed in cultured human corneal fibroblasts, suggesting that IL-6 trans-signaling induced inflammatory cellular signaling in the human corneal fibroblasts. We demonstrated that MMPs are significantly upregulated in collagen-destructive disorders of the cornea. Additionally, we observed that ectodomain shedding by ADAMs in corneal epithelial cells mediated the production of soluble cytokine receptors. Trans-signaling of IL-6 can induce an inflammatory response in corneal stroma, indicating the significance of IL-6 trans-signaling in ocular surface inflammation. Thus, MMPs and ADAMs play an important role in the pathophysiology of corneal diseases.

  8. Corneal Regeneration After Photorefractive Keratectomy: A Review☆

    Science.gov (United States)

    Tomás-Juan, Javier; Murueta-Goyena Larrañaga, Ane; Hanneken, Ludger

    2014-01-01

    Photorefractive keratectomy (PRK) remodels corneal stroma to compensate refractive errors. The removal of epithelium and the ablation of stroma provoke the disruption of corneal nerves and a release of several peptides from tears, epithelium, stroma and nerves. A myriad of cytokines, growth factors, and matrix metalloproteases participate in the process of corneal wound healing. Their balance will determine if reepithelization and stromal remodeling are appropriate. The final aim is to achieve corneal transparency for restoring corneal function, and a proper visual quality. Therefore, wound-healing response is critical for a successful refractive surgery. Our goal is to provide an overview into how corneal wounding develops following PRK. We will also review the influence of intraoperative application of mitomycin C, bandage contact lenses, anti-inflammatory and other drugs in preventing corneal haze and post-PRK pain. PMID:25444646

  9. Corneal Regeneration After Photorefractive Keratectomy: A Review.

    Science.gov (United States)

    Tomás-Juan, Javier; Murueta-Goyena Larrañaga, Ane; Hanneken, Ludger

    2015-01-01

    Photorefractive keratectomy (PRK) remodels corneal stroma to compensate refractive errors. The removal of epithelium and the ablation of stroma provoke the disruption of corneal nerves and a release of several peptides from tears, epithelium, stroma and nerves. A myriad of cytokines, growth factors, and matrix metalloproteases participate in the process of corneal wound healing. Their balance will determine if reepithelization and stromal remodeling are appropriate. The final aim is to achieve corneal transparency for restoring corneal function, and a proper visual quality. Therefore, wound-healing response is critical for a successful refractive surgery. Our goal is to provide an overview into how corneal wounding develops following PRK. We will also review the influence of intraoperative application of mitomycin C, bandage contact lenses, anti-inflammatory and other drugs in preventing corneal haze and post-PRK pain. Copyright © 2014 Spanish General Council of Optometry. Published by Elsevier Espana. All rights reserved.

  10. History of corneal transplantation in Australia.

    Science.gov (United States)

    Coster, Douglas J

    2015-04-01

    Corneal transplantation is a triumph of modern ophthalmology. The possibility of corneal transplantation was first raised in 1797 but a century passed before Zirm achieved the first successful penetrating graft in 1905. Gibson reported the first corneal graft in Australia from Brisbane in 1940 and English established the first eye bank there a few years later. Corneal transplantation evolved steadily over the twentieth century. In the second half of the century, developments in microsurgery, including surgical materials such as monofilament nylon and strong topical steroid drops, accounted for improvements in outcomes. In 2013, approximately 1500 corneal transplants were done in Australia. Eye banking has evolved to cope with the rising demands for donor corneas. Australian corneal surgeons collaborated to establish and support the Australian Corneal Graft Registry in 1985. It follows the outcomes of their surgery and has become an important international resource for surgeons seeking further improvement with the procedure. © 2014 Royal Australian and New Zealand College of Ophthalmologists.

  11. Corneal biomechanical properties in floppy eyelid syndrome.

    Science.gov (United States)

    Muniesa, MaJesús; Muniesa Royo, MaJesús; March, Ana; March de Ribot, Ana; Sánchez-de-la-Torre, Manuel; Huerva, Valetín; Huerva Escanilla, Valetín; Jurjo, Carmen; Jurjo Campo, Carmen; Barbé, Ferran; Barbé Illa, Ferran

    2015-05-01

    To determine corneal biomechanical properties in patients with floppy eyelid syndrome (FES) and to compare them with eyes of controls. This case-control study included 208 eyes (72 eyes with FES and 136 without FES) of 107 patients (37 patients with FES and 70 without FES). Patients underwent a complete clinical eye examination that included corneal biomechanical evaluation carried out with the Reichert Ocular Response Analyzer. Corneal hysteresis (CH), corneal resistance factor (CRF), central corneal thickness (CCT), Goldmann-correlated intraocular pressure (IOPg), and corneal-compensated intraocular pressure (IOPcc) were evaluated. Mean CH was significantly lower in patients with FES than in those without FES (9.51 ± 1.56 vs. 11.66 ± 9.11; P corneal biomechanical properties could be changed in patients with FES, reflecting additional structural changes in FES.

  12. The heart in Becker muscular dystrophy, facioscapulohumeral dystrophy, and Bethlem myopathy

    NARCIS (Netherlands)

    de Visser, M.; de Voogt, W. G.; la Rivière, G. V.

    1992-01-01

    We report a study, assessing involvement of the heart in 33 familial cases of Becker muscular dystrophy (BMD), 31 familiar cases of facioscapulohumeral (FSH) dystrophy, and 27 familial cases of Bethlem myopathy. In the patients with BMD, correlations of myocardial involvement with age and extent of

  13. Morbidity in reflex sympathetic dystrophy.

    Science.gov (United States)

    Murray, C S; Cohen, A; Perkins, T; Davidson, J E; Sills, J A

    2000-03-01

    Reflex sympathetic dystrophy (RSD), an unusual diagnosis in general paediatrics, is well recognised by paediatric rheumatologists. This study reports the presentation and the clinical course of 46 patients (35 female, age range 8-15.2) with RSD. The patients saw professionals from an average of 2.3 specialties (range 1-5). Twenty five (54%) had a history of trauma. Median time to diagnosis was 12 weeks (range 1-130). Many children had multiple investigations and treatments. Once diagnosis was made, treatment followed with physiotherapy and analgesics. Median time to recovery was seven weeks (range 1-140), with 27.5% relapsing. Nine children required assessment by the child and adolescent psychiatry team. This disease, though rare, has significant morbidity and it is therefore important to raise clinicians' awareness of RSD in childhood. Children with the condition may then be recognised and referred for appropriate management earlier, and spared unnecessary investigations and treatments which may exacerbate the condition.

  14. Reflex sympathetic dystrophy in childhood.

    Science.gov (United States)

    Tekgül, Hasan; Serdaroglu, Guil; Uyar, Meltem; Tütüncüoglu, Sarenur

    2002-04-01

    Reflex sympathetic dystrophy is characterized by constant burning pain and hyperesthesia in an extremity. Lower extremities are usually affected. Pain is accompanied by swelling, sweating, vasomotor instability and sometimes trophic changes. There may be a history of minor injury or not. Muscle spasms, myoclonus or focal dystonia may occur. Diffuse pain, loss of function and autonomic dysfunction are three main criteria suggested for diagnosis. Symptoms can last a few days to as long as a year. In this report we present a girl with multiple limb involvement of stage I RSD. The sympathetic skin responses were tested during a remission period. She had milder attacks with a recurrence rate of 4 per year in the following three years from onset.

  15. Corneal stroma microfibrils

    KAUST Repository

    Hanlon, Samuel D.

    2015-03-01

    Elastic tissue was first described well over a hundred years ago and has since been identified in nearly every part of the body. In this review, we examine elastic tissue in the corneal stroma with some mention of other ocular structures which have been more thoroughly described in the past. True elastic fibers consist of an elastin core surrounded by fibrillin microfibrils. However, the presence of elastin fibers is not a requirement and some elastic tissue is comprised of non-elastin-containing bundles of microfibrils. Fibers containing a higher relative amount of elastin are associated with greater elasticity and those without elastin, with structural support. Recently it has been shown that the microfibrils, not only serve mechanical roles, but are also involved in cell signaling through force transduction and the release of TGF-β. A well characterized example of elastin-free microfibril bundles (EFMBs) is found in the ciliary zonules which suspend the crystalline lens in the eye. Through contraction of the ciliary muscle they exert enough force to reshape the lens and thereby change its focal point. It is believed that the molecules comprising these fibers do not turn-over and yet retain their tensile strength for the life of the animal. The mechanical properties of the cornea (strength, elasticity, resiliency) would suggest that EFMBs are present there as well. However, many authors have reported that, although present during embryonic and early postnatal development, EFMBs are generally not present in adults. Serial-block-face imaging with a scanning electron microscope enabled 3D reconstruction of elements in murine corneas. Among these elements were found fibers that formed an extensive network throughout the cornea. In single sections these fibers appeared as electron dense patches. Transmission electron microscopy provided additional detail of these patches and showed them to be composed of fibrils (~10nm diameter). Immunogold evidence clearly

  16. Biomechanics of Corneal Ring Implants

    Science.gov (United States)

    2015-01-01

    Purpose: To evaluate the biomechanics of corneal ring implants by providing a related mathematical theory and biomechanical model for the treatment of myopia and keratoconus. Methods: The spherical dome model considers the inhomogeneity of the tunica of the eye, dimensions of the cornea, lamellar structure of the corneal stroma, and asphericity of the cornea. It is used in this study for calculating a strengthening factor sf for the characterization of different ring-shaped corneal implant designs. The strengthening factor is a measure of the amount of strengthening of the cornea induced by the implant. Results: For ring segments and incomplete rings, sf = 1.0, which indicates that these implants are not able to strengthen the cornea. The intracorneal continuous complete ring (MyoRing) has a strengthening factor of up to sf = 3.2. The MyoRing is, therefore, able to strengthen the cornea significantly. Conclusions: The result of the presented biomechanical analysis of different ring-shaped corneal implant designs can explain the different postoperative clinical results of different implant types in myopia and keratoconus. PMID:26312619

  17. Corneal Protection for Burn Patients

    Science.gov (United States)

    2013-10-01

    report, Figs. 2-7. Rose Bengal (RB) photosensitization was tested because RB is FDA-allowed as a diagnostic for corneal abrasions...incisions. J Cataract Refract Surg, 30, 2420-4. Personnel supported by this grant All personnel are employed by the Massachusetts General Hospital

  18. Biomechanics of Corneal Ring Implants.

    Science.gov (United States)

    Daxer, Albert

    2015-11-01

    To evaluate the biomechanics of corneal ring implants by providing a related mathematical theory and biomechanical model for the treatment of myopia and keratoconus. The spherical dome model considers the inhomogeneity of the tunica of the eye, dimensions of the cornea, lamellar structure of the corneal stroma, and asphericity of the cornea. It is used in this study for calculating a strengthening factor sf for the characterization of different ring-shaped corneal implant designs. The strengthening factor is a measure of the amount of strengthening of the cornea induced by the implant. For ring segments and incomplete rings, sf = 1.0, which indicates that these implants are not able to strengthen the cornea. The intracorneal continuous complete ring (MyoRing) has a strengthening factor of up to sf = 3.2. The MyoRing is, therefore, able to strengthen the cornea significantly. The result of the presented biomechanical analysis of different ring-shaped corneal implant designs can explain the different postoperative clinical results of different implant types in myopia and keratoconus.

  19. Corneale crosslinking voor progressieve keratoconus

    NARCIS (Netherlands)

    Wisse, Robert P L; Soeters, Nienke; Godefrooij, Daniel A.; De Koning-Tahzib, Nayyirih G.

    2016-01-01

    Keratoconus is a corneal disease with onset typically occurring during puberty or early adulthood. The cornea progressively thins and acquires a cone-like shape which negatively affects visual acuity. In the early stages, visual acuity can be corrected with glasses or contact lenses. In more

  20. Glutaminolysis is Essential for Energy Production and Ion Transport in Human Corneal Endothelium

    Directory of Open Access Journals (Sweden)

    Wenlin Zhang

    2017-02-01

    Full Text Available Corneal endothelium (CE is among the most metabolically active tissues in the body. This elevated metabolic rate helps the CE maintain corneal transparency by its ion and fluid transport properties, which when disrupted, leads to visual impairment. Here we demonstrate that glutamine catabolism (glutaminolysis through TCA cycle generates a large fraction of the ATP needed to maintain CE function, and this glutaminolysis is severely disrupted in cells deficient in NH3:H+ cotransporter Solute Carrier Family 4 Member 11 (SLC4A11. Considering SLC4A11 mutations leads to corneal endothelial dystrophy and sensorineural deafness, our results indicate that SLC4A11-associated developmental and degenerative disorders result from altered glutamine catabolism. Overall, our results describe an important metabolic mechanism that provides CE cells with the energy required to maintain high level transport activity, reveal a direct link between glutamine metabolism and developmental and degenerative neuronal diseases, and suggest an approach for protecting the CE during ophthalmic surgeries.

  1. Genetics Home Reference: autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions APECED Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy Printable PDF Open All Close All ... view the expand/collapse boxes. Description Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy ( APECED ) is an inherited condition that ...

  2. Corneal biomechanical parameters during pregnancy.

    Science.gov (United States)

    Sen, Emine; Onaran, Yüksel; Nalcacioglu-Yuksekkaya, Pinar; Elgin, Ufuk; Ozturk, Faruk

    2014-01-01

    To evaluate the variation in biomechanical properties and central corneal thickness (CCT) for each trimester during pregnancy and to compare the values with those in nonpregnant women. We prospectively studied the eyes of 32 pregnant and 34 age-matched non-pregnant women. The parameters included corneal hysteresis (CH), corneal resistance factor (CRF), Goldmann-correlated intraocular pressure (IOP), and corneal-compensated IOP measured by the Ocular Response Analyzer (ORA). The CCT was also measured with an ultrasonic pachymeter attached to the ORA. The mean age was 27.0 ± 3.8 years in the study group and 28.0 ± 4.1 years in the control group. The mean CH measurement was 10.6 ± 1.4 mmHg in the study group and 10.1 ± 1.3 mmHg in the control group. The mean CRF value was 9.6 ± 1.7 mmHg in the study group and 10.0 ± 1.4 mmHg in the control group. The mean CCT value was 541.1 ± 22.4 µm in the study group and 536.5 ± 27.1 µm in the control group. No statistically significant differences were found regarding CH, CRF, or CCT values between the 2 groups (independent t test, p = 0.160, p = 0.355, p = 0.450, respectively). Hormonal changes during pregnancy may not affect corneal biomechanics. This may be due to the balanced effect of the various hormones on the cornea during pregnancy.

  3. Management of corneal bee sting

    Directory of Open Access Journals (Sweden)

    Razmjoo H

    2011-12-01

    Full Text Available Hassan Razmjoo1,2, Mohammad-Ali Abtahi1,2,4, Peyman Roomizadeh1,3, Zahra Mohammadi1,2, Seyed-Hossein Abtahi1,3,41Medical School, Isfahan University of Medical Sciences (IUMS; 2Ophthalmology Ward, Feiz Hospital, IUMS; 3Isfahan Medical Students Research Center (IMSRC, IUMS; 4Isfahan Ophthalmology Research Center (IORC, Feiz Hospital, IUMS, Isfahan, IranAbstract: Corneal bee sting is an uncommon environmental eye injury that can result in various ocular complications with an etiology of penetrating, immunologic, and toxic effects of the stinger and its injected venom. In this study we present our experience in the management of a middle-aged male with a right-sided deep corneal bee sting. On arrival, the patient was complaining of severe pain, blurry vision with acuity of 160/200, and tearing, which he had experienced soon after the injury. Firstly, we administered conventional drugs for eye injuries, including topical antibiotic, corticosteroid, and cycloplegic agents. After 2 days, corneal stromal infiltration and edema developed around the site of the sting, and visual acuity decreased to 100/200. These conditions led us to remove the stinger surgically. Within 25 days of follow-up, the corneal infiltration decreased gradually, and visual acuity improved to 180/200. We suggest a two-stage management approach for cases of corneal sting. For the first stage, if the stinger is readily accessible or primary dramatic reactions, including infiltration, especially on the visual axis, exist, manual or surgical removal would be indicated. Otherwise, we recommend conventional treatments for eye injuries. Given this situation, patients should be closely monitored for detection of any worsening. If the condition does not resolve or even deteriorates, for the second stage, surgical removal of the stinger under local or generalized anesthesia is indicated.Keywords: bee sting, stinger, cornea, removal, management, surgery

  4. Changes in lipidomic profile of aqueous humour in Fuchs endothelial dystrophy

    DEFF Research Database (Denmark)

    Nuñez, Francisco Javier Cabrerizo; Urcola, Javier Aritz; Vecino, Elena

    2017-01-01

    surgery, anterior segment pathology or intraocular injections were excluded. Topical ocular medications within the last 6 months were reported. Aqueous humour (AH) was obtained during the first step of Descemet membrane endothelial keratoplasty in FECD patients and during refractive lensectomy......PURPOSE: To identify and determine differences in lipid profile of aqueous humour (AH) in patients with Fuchs endothelial corneal dystrophy (FECD). METHODS: Lipidomic profile of eight AH samples of FECD patients and 10 control samples was analysed. Patients with previous history of anterior segment....... In addition, eight sphingomyelins and up to two long-chain highly unsaturated cholesteryl esters present higher levels in FECD samples when compared to controls. CONCLUSION: The lipid composition of AH in FECD patients differs from that of healthy subjects. Those changes may reflect oxidative stress...

  5. Improvement in corneal scarring following bacterial keratitis.

    Science.gov (United States)

    McClintic, S M; Srinivasan, M; Mascarenhas, J; Greninger, D A; Acharya, N R; Lietman, T M; Keenan, J D

    2013-03-01

    Bacterial keratitis results in corneal scarring and subsequent visual impairment. The long-term evolution of corneal scars has not been well described. In this case series, we identified patients who had improvement in corneal scarring and visual acuity from a clinical trial for bacterial keratitis. We searched the records of the Steroids for Corneal Ulcers Trial (SCUT) for patients who had improvement in vision between the 3-month and 12-month visits and reviewed their clinical photographs. Of the 500 patients enrolled in SCUT, five patients with large central corneal scars due to bacterial keratitis are presented. All experienced improvement in rigid contact lens-corrected visual acuity from months 3 to 12. All patients also had marked improvement in corneal opacity during the same time period. None of the patients opted to have penetrating keratoplasty. Corneal scars may continue to improve even many months after a bacterial corneal ulcer has healed. The corneal remodeling can be accompanied by considerable improvement in visual acuity, such that corneal transplantation may not be necessary.

  6. Imaging, Reconstruction, And Display Of Corneal Topography

    Science.gov (United States)

    Klyce, Stephen D.; Wilson, Steven E.

    1989-12-01

    The cornea is the major refractive element in the eye; even minor surface distortions can produce a significant reduction in visual acuity. Standard clinical methods used to evaluate corneal shape include keratometry, which assumes the cornea is ellipsoidal in shape, and photokeratoscopy, which images a series of concentric light rings on the corneal surface. These methods fail to document many of the corneal distortions that can degrade visual acuity. Algorithms have been developed to reconstruct the three dimensional shape of the cornea from keratoscope images, and to present these data in the clinically useful display of color-coded contour maps of corneal surface power. This approach has been implemented on a new generation video keratoscope system (Computed Anatomy, Inc.) with rapid automatic digitization of the image rings by a rule-based approach. The system has found clinical use in the early diagnosis of corneal shape anomalies such as keratoconus and contact lens-induced corneal warpage, in the evaluation of cataract and corneal transplant procedures, and in the assessment of corneal refractive surgical procedures. Currently, ray tracing techniques are being used to correlate corneal surface topography with potential visual acuity in an effort to more fully understand the tolerances of corneal shape consistent with good vision and to help determine the site of dysfunction in the visually impaired.

  7. Corneal biomechanical properties in thyroid eye disease.

    Science.gov (United States)

    Karabulut, Gamze Ozturk; Kaynak, Pelin; Altan, Cıgdem; Ozturker, Can; Aksoy, Ebru Funda; Demirok, Ahmet; Yılmaz, Omer Faruk

    2014-06-01

    The purpose of this study is to investigate the effect of thyroid eye disease (TED) on the measurement of corneal biomechanical properties and the relationship between these parameters and disease manifestations. A total of 54 eyes of 27 individuals with TED and 52 eyes of 30 healthy control participants were enrolled. Thyroid ophthalmopathy activity was defined using the VISA (vision, inflammation, strabismus, and appearance/exposure) classification for TED. The intraocular pressure (IOP) measurement with Goldmann applanation tonometer (GAT), axial length (AL), keratometry, and central corneal thickness (CCT) measurements were taken from each patient. Corneal biomechanical properties, including corneal hysteresis (CH) and corneal resistance factor (CRF) and noncontact IOP measurements, Goldmann-correlated IOP (IOPg) and corneal-compensated IOP (IOPcc) were measured with the Ocular Response Analyzer (ORA) using the standard technique. Parameters such as best corrected visual acuity, axial length, central corneal thickness, and corneal curvature were not statistically significant between the two groups (p > 0.05). IOP measured with GAT was higher in participants with TED (p corneal resistance factor between groups. However, IOPg and IOPcc were significantly higher in TED patients. CH and VISA grading of TED patients showed a negative correlation (p = 0.007). In conclusion, TED affects the corneal biomechanical properties by decreasing CH. IOP with GAT and IOPg is found to be increased in these patients. As the severity of TED increases, CH decreases in these patients. Copyright © 2014. Published by Elsevier B.V.

  8. Turning the tide of corneal blindness

    Directory of Open Access Journals (Sweden)

    Matthew S Oliva

    2012-01-01

    Full Text Available Corneal diseases represent the second leading cause of blindness in most developing world countries. Worldwide, major investments in public health infrastructure and primary eye care services have built a strong foundation for preventing future corneal blindness. However, there are an estimated 4.9 million bilaterally corneal blind persons worldwide who could potentially have their sight restored through corneal transplantation. Traditionally, barriers to increased corneal transplantation have been daunting, with limited tissue availability and lack of trained corneal surgeons making widespread keratoplasty services cost prohibitive and logistically unfeasible. The ascendancy of cataract surgical rates and more robust eye care infrastructure of several Asian and African countries now provide a solid base from which to dramatically expand corneal transplantation rates. India emerges as a clear global priority as it has the world′s largest corneal blind population and strong infrastructural readiness to rapidly scale its keratoplasty numbers. Technological modernization of the eye bank infrastructure must follow suit. Two key factors are the development of professional eye bank managers and the establishment of Hospital Cornea Recovery Programs. Recent adaptation of these modern eye banking models in India have led to corresponding high growth rates in the procurement of transplantable tissues, improved utilization rates, operating efficiency realization, and increased financial sustainability. The widespread adaptation of lamellar keratoplasty techniques also holds promise to improve corneal transplant success rates. The global ophthalmic community is now poised to scale up widespread access to corneal transplantation to meet the needs of the millions who are currently blind.

  9. Changes of corneal biomechanics with keratoconus.

    Science.gov (United States)

    Wolffsohn, James S; Safeen, Saima; Shah, Sunil; Laiquzzaman, Mohammad

    2012-08-01

    To perform advanced analysis of the corneal deformation response to air pressure in keratoconics compared with age- and sex-matched controls. The ocular response analyzer was used to measure the air pressure-corneal deformation relationship of 37 patients with keratoconus and 37 age (mean 36 ± 10 years)- and sex-matched controls with healthy corneas. Four repeat air pressure-corneal deformation profiles were averaged, and 42 separate parameters relating to each element of the profiles were extracted. Corneal topography and pachymetry were performed with the Orbscan II. The severity of the keratoconus was graded based on a single metric derived from anterior corneal curvatures, difference in astigmatism in each meridian, anterior best-fit sphere, and posterior best-fit sphere. Most of the biomechanical characteristics of keratoconic eyes were significantly different from normal eyes (P corneal applanation. With increasing keratoconus severity, the cornea was thinner (r = -0.407, P corneal concave deformation past applanation was quicker (dive; r2 = -0.314, P = 0.01), and the tear film index was lower (r = -0.319, P = 0.01). The variance in keratoconus severity could be accounted for by the corneal curvature and central corneal thickness (r = 0.80) with biomechanical characteristics contributing an additional 4% (total r = 0.84). The area under the receiver operating characteristic curve was 0.919 ± 0.025 for keratometry alone, 0.965 ± 0.014 with the addition of pachymetry, and 0.972 ± 0.012 combined with ocular response analyzer biomechanical parameters. Characteristics of the air pressure-corneal deformation profile are more affected by keratoconus than the traditionally extracted corneal hysteresis and corneal resistance factors. These biomechanical metrics slightly improved the detection and severity prediction of keratoconus above traditional keratometric and pachymetric assessment of corneal shape.

  10. Evaluation of corneal symmetry after UV corneal crosslinking for keratoconus.

    Science.gov (United States)

    Mofty, Hanan; Alzahrani, Khaled; Carley, Fiona; Harper, Sophie; Brahma, Arun; Au, Leon; Morley, Debbie; Hillarby, M Chantal

    2017-01-01

    The purpose of this study was to assess UV corneal crosslinking (CXL) treatment outcomes for keratoconus by evaluating the corneal regularity in patients through follow-up using the Oculus Pentacam. A total of 18 eyes from CXL patients with keratoconus were studied before and after CXL treatment, and six eyes from six patients who were not treated with CXL served as controls. Treated patients had Pentacam images taken before CXL treatment and regularly 3 months post treatment up to the 12th month. Controls were imaged during their first appointment and after 12 months. Symmetry and asphericity were evaluated and correlated with both best-corrected visual acuity (BCVA) and maximum K -readings. In the CXL-treated group, there was a significant improvement in the index of symmetrical variation (ISV) and keratoconus index (KI) at 3 months and in the index of height asymmetry (IHA) and minimum radius of curvature ( R min ) at 9 months post treatment. On the contrary, the untreated group's indices showed some significant worsening in ISV, KI, central keratoconus index (CKI), and R min . A novel finding in our study was a slight positive shift of anterior asphericity in the 6 mm, 7 mm, and 8 mm 3 months after treatment, which had a correlation with BCVA ( R 2 =0.390, p =0.053) and a strong correlation with maximum K -reading ( R 2 =0.690, p =0.005). However, the untreated group had no significant changes after 1 year. The corneal asymmetrical shape is associated with the spherical aberration alteration influenced by temporal evolution of surface ablation and increased corneal haze. However, insignificant changes in symmetry attest the stabilization effect on cornea postoperatively as compared with controls.

  11. Posterior corneal shape : Comparison of height data from 3 corneal topographers

    NARCIS (Netherlands)

    de Jong, Tim; Sheehan, Matthew T.; Koopmans, Steven A.; Jansonius, Nomdo M.

    Purpose: To compare the ability of 3 clinical corneal topographers to describe the posterior corneal shape. Setting: University Medical Center Groningen, the Netherlands. Design: Prospective observational study. Methods: Corneas of healthy participants were measured twice with a dual Scheimpflug

  12. Value of recombinant human epidermal growth factor in corneal wound repair after corneal foreign body elimination

    OpenAIRE

    Hong-Jie Han

    2013-01-01

    AIM: To investigate the repair efficacy of recombinant human epidermal growth factor on corneal epithelium after corneal foreign body eliminating operation. METHODS: There were 102 patients with corneal foreign body(188 affected eyes)chosen for the study. All patients were divided into treatment group and control group according to the random number table. Both groups received corneal foreign body elimination by slit lamp. Postoperatively, the treatment group was given eye drops containing ep...

  13. Variation of corneal refractive index with hydration

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young L; Walsh, Joseph T Jr.; Goldstick, Thomas K; Glucksberg, Matthew R [Biomedical Engineering Department, Northwestern University, Evanston, IL 60208 (United States)

    2004-03-07

    We report the effect of changes in the corneal hydration on the refractive index of the cornea. Using optical coherence tomography (OCT), the geometrical thickness and the group refractive index of the bovine cornea were derived simultaneously as the corneal hydration was varied. The corneal hydration was then calculated from the corneal thickness. The group refractive index of the cornea increased non-linearly as the cornea dehydrated. In addition, a simple mathematical model was developed, based on the assumption that changes in corneal hydration occur only in the interfibrilar space with constant water content within the collagen fibrils. Good agreement between the experimental results and the mathematical model supports the assumption. The results also demonstrate that the measurement of refractive index is a quantitative indicator of corneal hydration.

  14. Corneal Toxicity Following Exposure to Asclepias Tuberosa

    DEFF Research Database (Denmark)

    Mikkelsen, Lauge Hjorth; Hamoudi, Hassan; Gül, Cigdem Altuntas

    2017-01-01

    PURPOSE: To present a case of corneal toxicity following exposure to milky plant latex from Asclepias tuberosa. METHODS: A 70-year-old female presented with blurred vision and pain in her left eye after handling an Ascepias tuberosa. Clinical examination revealed a corneal stromal oedema with small...... that inhibit the Na+/ K+-ATPase in the corneal endothelium. The oedema resolved after 96 hours. After nine months the best corrected visual acuity was 20/20. CONCLUSION: Corneal toxicity has previously been reported for plants of the Asclepias family. This is a rare case describing severe corneal toxicity...... caused by exposure to latex from Asclepias tuberosa. Handling of plants of the Asclepias family should be kept as a differential diagnosis in cases of acute corneal toxicity....

  15. Corneal epithelium in penetrating keratoplasty.

    Science.gov (United States)

    Meyer, R F; Bobb, K C

    1980-08-01

    We studied corneal epithelium in 66 patients with bullous keratopathy treated with penetrating keratoplasty using McCarey-Kaufman stored donor corneas. Epithelium was evaluated at times of storage, surgery, and postoperative dressing changes. Epithelium was intact in 43 of the donor corneas at storage, and 23 had 5 to 100% (median, 50%) epithelium missing. At the end of the keratoplasty procedure, 16 grafts had epithelium intact, and 50 had 5 to 100% (median, 20%) epithelium missing. Postoperative epithelial healing time ranged from one to 12 days, with a median of two days. Postoperative healing was significantly prolonged when donor corneal epithelium was missing at keratoplasty. As the amount of epithelium intact at the end of surgery decreased, the number of days to heal postoperatively increased. We found that donor corneas could be stored as long as 79 hours, with 63 hours in McCarey-Kaufman medium, and still have epithelium intact at the end of the keratoplasty procedure.

  16. Corneal astigmatism following cataract extraction.

    Science.gov (United States)

    Wishart, M S; Wishart, P K; Gregor, Z J

    1986-01-01

    The changes in corneal curvature in the first six months after cataract extraction were studied by performing sequential keratometry on a group of 57 patients. 8/0 Virgin silk interrupted sutures were used for the closure of corneoscleral incisions, and 10/0 monofilament tied in double running (bootlace) or single running (continuous) fashion was used for corneal wound closure. A high degree of with-the-rule astigmatism was evident in all patients two weeks postoperatively, but thereafter the character of the astigmatism produced by 8/0 virgin silk and 10/0 monofilament closure was quite different: in the 8/0 virgin silk group there was an early and pronounced shift in the axis of astigmatism to against-the-rule, whereas in the 10/0 monofilament group there was little further change in the astigmatism unless the sutures were removed. Wound compression and wound gape as factors responsible for these changes are discussed. PMID:3539177

  17. Glaucoma and Corneal Transplant Procedures

    Directory of Open Access Journals (Sweden)

    Ammar M. Al-Mahmood

    2012-01-01

    Full Text Available Glaucoma after corneal transplantation is a leading cause of ocular morbidity after penetrating keratoplasty. The incidence reported is highly variable and a number of etiologic factors have been identified. A number of treatment options are available; surgical intervention for IOP control is associated with a high incidence of graft failure. IOP elevation is less frequently seen following deep anterior lamellar keratoplasty. Descemet's striping-automated endothelial keratoplasty is also associated with postprocedure intraocular pressure elevation and secondary glaucoma and presents unique surgical challenges in patients with preexisting glaucoma surgeries. Glaucoma exists in up to three-quarters of patients who undergo keratoprosthesis surgery and the management if often challenging. The aim of this paper is to highlight the incidence, etiology, and management of glaucoma following different corneal transplant procedures. It also focuses on the challenges in the diagnosis of glaucoma and intraocular pressure monitoring in this group of patients.

  18. Corneal hemangiosarcoma in a cat.

    Science.gov (United States)

    Cazalot, G; Regnier, A; Deviers, A; Serra, F; Lucas, M N; Etienne, C L; Letron, I Raymond

    2011-09-01

    A 10 year-old castrated male Domestic Short-hair cat with a history of chronic bilateral keratitis was referred for assessment of a red, elevated mass involving the left cornea. The rapid growth of the mass, over a month period in combination with pronounced vascularization and invasion of the corneal surface suggested an aggressive inflammatory or neoplastic process. Following keratectomy, the lesion was diagnosed histopathologically as a hemangiosarcoma. The tumor recurred locally within 3 weeks and enucleation was performed. Histopathologic examination of the globe confirmed the diagnosis and did not reveal infiltration of the limbus and conjunctiva. No signs of local recurrence or metastatic disease have been observed 18 months following enucleation. To the authors' knowledge this is the first case of primary corneal hemangiosarcoma described in the feline species. © 2011 American College of Veterinary Ophthalmologists.

  19. Evaluation of corneal symmetry after UV corneal crosslinking for keratoconus

    Directory of Open Access Journals (Sweden)

    Mofty H

    2017-11-01

    Full Text Available Hanan Mofty,1,2 Khaled Alzahrani,2 Fiona Carley,3 Sophie Harper,3 Arun Brahma,3 Leon Au,3 Debbie Morley,3 M Chantal Hillarby2 1Optometry Department, College of Applied Medical Science, King Saud University, Riyadh, Kingdom of Saudi Arabia; 2Division of Pharmacy and Optometry, School of Health Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, 3Manchester Royal Eye Hospital, Manchester, UK Purpose: The purpose of this study was to assess UV corneal crosslinking (CXL treatment outcomes for keratoconus by evaluating the corneal regularity in patients through follow-up using the Oculus Pentacam.Patients and methods: A total of 18 eyes from CXL patients with keratoconus were studied before and after CXL treatment, and six eyes from six patients who were not treated with CXL served as controls. Treated patients had Pentacam images taken before CXL treatment and regularly 3 months post treatment up to the 12th month. Controls were imaged during their first appointment and after 12 months. Symmetry and asphericity were evaluated and correlated with both best-corrected visual acuity (BCVA and maximum K-readings.Results: In the CXL-treated group, there was a significant improvement in the index of symmetrical variation (ISV and keratoconus index (KI at 3 months and in the index of height asymmetry (IHA and minimum radius of curvature (Rmin at 9 months post treatment. On the contrary, the untreated group’s indices showed some significant worsening in ISV, KI, central keratoconus index (CKI, and Rmin. A novel finding in our study was a slight positive shift of anterior asphericity in the 6 mm, 7 mm, and 8 mm 3 months after treatment, which had a correlation with BCVA (R2=0.390, p=0.053 and a strong correlation with maximum K-reading (R2=0.690, p=0.005. However, the untreated group had no significant changes after 1 year.Conclusion: The corneal asymmetrical shape is associated with the spherical aberration alteration

  20. Corneal Donor Tissue Preparation for Endothelial Keratoplasty

    OpenAIRE

    Woodward, Maria A.; Titus, Michael; Mavin, Kyle; Shtein, Roni M.

    2012-01-01

    Over the past ten years, corneal transplantation surgical techniques have undergone revolutionary changes1,2. Since its inception, traditional full thickness corneal transplantation has been the treatment to restore sight in those limited by corneal disease. Some disadvantages to this approach include a high degree of post-operative astigmatism, lack of predictable refractive outcome, and disturbance to the ocular surface. The development of Descemet's stripping endothelial keratoplasty (DSEK...

  1. Corneal Biomechanics Determination in Healthy Myopic Subjects

    OpenAIRE

    Kunliang Qiu; Xuehui Lu; Riping Zhang; Geng Wang; Mingzhi Zhang

    2016-01-01

    Purpose. To determine the corneal biomechanical properties by using the Ocular Response Analyzer? and to investigate potential factors associated with the corneal biomechanics in healthy myopic subjects. Methods. 135 eyes from 135 healthy myopic subjects were included in this cross-sectional observational study. Cornea hysteresis (CH), corneal resistance factor (CRF), cornea-compensated intraocular pressure (IOPcc), and Goldmann-correlated intraocular pressure (IOPg) were determined with the ...

  2. Duchenne muscular dystrophy: Case report and review

    Directory of Open Access Journals (Sweden)

    Rupam Sinha

    2017-01-01

    Full Text Available Muscular dystrophies are a clinically and heterogeneous group of disorders that all share clinical characteristics of progressive muscular weakness. Duchenne muscular dystrophy (DMD is the most common X-linked disorder muscular dystrophy in children, presenting in early childhood and characterized by proximal muscle weakness and calf hypertrophy in affected boys. There is usually delay in motor development and eventually wheelchair confinement followed by premature death from cardiac or respiratory complications. Treatment modalities such as corticosteroid therapy and use of intermittent positive pressure ventilation have provided improvements in function, ambulation, quality of life, and life expectancy, although novel therapies still aim to provide a cure for this devastating disorder. Here, we present a case of DMD in a 12-year-old male with remarkable clinical and oral manifestations.

  3. Mitochondrial disorders in progressive muscular dystrophies

    Directory of Open Access Journals (Sweden)

    D. A. Kharlamov

    2014-01-01

    Full Text Available The literature review gives data on the role of mitochondrial disorders in the pathogenesis of different progressive muscular dystrophies. It describes changes in Duchenne, limb-girdle, facial scapulohumeral (Landuzi—Degerina muscular dystrophies. The review is based on both clinical and experimental animal studies. Along with the implication of mitochondria in the pathogenesis of the diseases, it describes muscular dystrophy treatment options compensating for energy disorders and overcoming oxidative stress and mitochondrial dysfunction. Mitochondrial studies in different muscle diseases hand physicians treatment modalities that fail to lead to recovery, but compensate for disorders caused by mutations in the genetic apparatus. 

  4. Duchenne muscular dystrophy: Case report and review.

    Science.gov (United States)

    Sinha, Rupam; Sarkar, Soumyabrata; Khaitan, Tanya; Dutta, Soumyajit

    2017-01-01

    Muscular dystrophies are a clinically and heterogeneous group of disorders that all share clinical characteristics of progressive muscular weakness. Duchenne muscular dystrophy (DMD) is the most common X-linked disorder muscular dystrophy in children, presenting in early childhood and characterized by proximal muscle weakness and calf hypertrophy in affected boys. There is usually delay in motor development and eventually wheelchair confinement followed by premature death from cardiac or respiratory complications. Treatment modalities such as corticosteroid therapy and use of intermittent positive pressure ventilation have provided improvements in function, ambulation, quality of life, and life expectancy, although novel therapies still aim to provide a cure for this devastating disorder. Here, we present a case of DMD in a 12-year-old male with remarkable clinical and oral manifestations.

  5. Circulating Biomarkers for Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Aartsma-Rus, Annemieke; Spitali, Pietro

    2015-07-22

    Duchenne muscular dystrophy is the most common form of muscular dystrophy. Genetic and biochemical research over the years has characterized the cause, pathophysiology and development of the disease providing several potential therapeutic targets and/or biomarkers. High throughput - omic technologies have provided a comprehensive understanding of the changes occurring in dystrophic muscles. Murine and canine animal models have been a valuable source to profile muscles and body fluids, thus providing candidate biomarkers that can be evaluated in patients. This review will illustrate known circulating biomarkers that could track disease progression and response to therapy in patients affected by Duchenne muscular dystrophy. We present an overview of the transcriptomic, proteomic, metabolomics and lipidomic biomarkers described in literature. We show how studies in muscle tissue have led to the identification of serum and urine biomarkers and we highlight the importance of evaluating biomarkers as possible surrogate endpoints to facilitate regulatory processes for new medicinal products.

  6. Refractive surgery following corneal graft.

    Science.gov (United States)

    Alió, Jorge L; Abdou, Ahmed A; Abdelghany, Ahmed A; Zein, Ghassam

    2015-07-01

    To review the different surgical procedures for management of postkeratoplasty refractive errors after total suture removal. There are different surgical options to address residual refractive errors that frequently occur after corneal transplantation. The correction can be done on the corneal surface or intraocular with intraocular lens (IOL) implantation which requires complete tectonic and refractive stability after suture removal. The most commonly used procedures are photorefractive keratectomy, laser in-situ keratomileusis and Phakic IOLs. Keratoplasty has been profited by recent advances in refractive surgery. Custom excimer laser ablation is an alternative way to treat irregular errors. New IOL modalities are good practical options for a wide range of errors. Femtosecond laser, as a new option in the toolbox, can modify corneal grafting refractive results and assist corrective refractive procedures. Although being the most successful organ transplantation, keratoplasty is usually followed by significant ametropia. Different corrective modalities exist and the choice should fit ocular conditions, patient requirements, surgeon skills and the available technologies. Recent advances in ophthalmic surgery have improved the outcomes.

  7. Refractive surgery after corneal transplantation.

    Science.gov (United States)

    Chang, Daniel H; Hardten, David R

    2005-08-01

    Many patients who have undergone corneal transplantation are unable to achieve satisfactory visual acuity with spectacle and contact lens correction alone. For these patients, refractive surgery becomes a viable option to reduce the post-keratoplasty ametropia. With the many recent advances in refractive surgery for naturally occurring refractive error, new possibilities arise for application to this complicated set of patients. This review discusses key recent developments in refractive surgery after corneal transplantation. The biomechanical effects of incisional keratotomy on post-keratoplasty corneas continue to be studied, and these techniques remain a common and simple method of reducing astigmatism. Photorefractive keratectomy, previously problematic for regression and haze formation, is gaining new prominence as early experience with the adjunctive use of mitomycin C has demonstrated good results. Long-term studies with laser in-situ keratomileusis (LASIK) have continued to show good safety and efficacy. Modern developments in cataract surgery appear to have lower incidences of graft rejection and failure. Developments in lens implantation technology continue to offer expanding options for intraocular refractive surgery. Although visual rehabilitation after corneal transplantation remains a formidable challenge, developments in refractive surgery for naturally occurring ametropias directly translate into an improved ability to help these most challenging refractive cases. Continued research will bring about improved efficacy while maintaining a high level of safety.

  8. Experimental assessment of corneal anisotropy.

    Science.gov (United States)

    Elsheikh, Ahmed; Brown, Michael; Alhasso, Daad; Rama, Paolo; Campanelli, Marino; Garway-Heath, David

    2008-02-01

    To determine the variation of corneal biomechanical properties with anatomical orientation. Strip specimens extracted from fresh porcine corneas were tested under uniaxial tension with strain rates representing static and dynamic loading conditions. The specimens were extracted from the vertical, horizontal, and 45 degrees diagonal directions. The load elongation results were used to derive the stress-strain behavior of each specimen. The average behavior for specimens taken in each anatomical direction was determined along with the effect of strain rate. Specimens from a small number of human corneas were included in the study to verify the findings. Specimens extracted from the vertical direction of porcine and human corneas demonstrated the highest strength (fracture stress) followed by horizontal then diagonal specimens. Vertical specimens were 10% to 20% stronger than horizontal specimens in porcine and human corneas. At low strain rates (1%/min), vertical specimens displayed similar stiffness (resistance to deformation) to horizontal specimens but greater stiffness than diagonal specimens. On increasing the strain rate to 500%/min, the stiffness behavior matched that of strength with vertical specimens being 10% to 20% stiffer than horizontal specimens in porcine and human corneas. The corneal anisotropic behavior is compatible with the preferential orientation of stromal fibrils in the vertical and horizontal directions. Quantifying the effect of this nonuniform fibril organization on corneal anisotropic behavior will be useful in developing numerical models of the cornea for applications where its integrity is compromised such as in simulating refractive surgery procedures.

  9. Obtaining corneal tissue for keratoplasty.

    Science.gov (United States)

    Navarro Martínez-Cantullera, A; Calatayud Pinuaga, M

    2016-10-01

    Cornea transplant is the most common tissue transplant in the world. In Spain, tissue donation activities depend upon transplant coordinator activities and the well-known Spanish model for organ and tissue donation. Tissue donor detection system and tissue donor evaluation is performed mainly by transplant coordinators using the Spanish model on donation. The evaluation of a potential tissue donor from detection until recovery is based on an exhaustive review of the medical and social history, physical examination, family interview to determine will of the deceased, and a laboratory screening test. Corneal acceptance criteria for transplantation have a wider spectrum than other tissues, as donors with active malignancies and infections are accepted for kearatoplasty in most tissue banks. Corneal evaluation during the whole process is performed to ensure the safety of the donor and the recipient, as well as an effective transplant. Last step before processing, corneal recovery, must be performed under standard operating procedures and in a correct environment. Copyright © 2016 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  10. Central corneal thickness in glaucoma.

    Science.gov (United States)

    Sng, Chelvin C A; Ang, Marcus; Barton, Keith

    2017-03-01

    The purpose of this review is to summarize the role of central corneal thickness (CCT) in the clinical management of a glaucoma patient. The prognostic value of CCT is well recognized in patients with ocular hypertension. However, its predictive value in other glaucoma suspects and patients with established glaucoma is less certain. Tonometry artefacts can result from variations in CCT. However, an adequately validated correction algorithm for Goldmann applanation tonometry measurements does not exist. Newer methods of tonometry are potentially less influenced by CCT but are limited in their clinical use. There may also be biological and genetic associations between corneal thickness and glaucoma. Demographics, environmental factors, glaucoma treatment and the measurement device used have a significant influence on CCT, and should be considered when interpreting the effect of cornea thickness in patients with glaucoma. New measurements of the biomechanical properties of the cornea are likely to be better approximations of the globe biomechanics than CCT, but these require further evaluation. The clinical significance of CCT is well recognized in the context of glaucoma diagnosis and management, though the extent of its importance remains debatable. Corneal biomechanical properties may be more significantly associated with glaucoma than CCT.

  11. Relationship between Corneal Sensation, Blinking, and Tear Film Quality

    National Research Council Canada - National Science Library

    Nosch, Daniela Sonja; Pult, Heiko; Albon, Julie; Purslow, Christine; Murphy, Paul John

    2016-01-01

    PURPOSETo examine the possible role of corneal sensitivity and tear film quality in triggering a blink by investigating the relationship between blink rate, central corneal sensitivity threshold (CST...

  12. Response of corneal hysteresis and central corneal thickness following clear corneal cataract surgery.

    Science.gov (United States)

    Kandarakis, Artemios; Soumplis, Vasileios; Karampelas, Michalis; Koutroumanos, Ioannis; Panos, Christos; Kandarakis, Stylianos; Karagiannis, Dimitrios

    2012-09-01

    To evaluate the effect of routine phacoemulsification in corneal viscoelastic properties determined by corneal hysteresis (CH) and central corneal thickness (CCT) and to explore the impact of phaco energy on the above parameters. Forty-one eyes of 41 patients undergoing cataract surgery were enrolled in this prospective study. CH and CCT were measured preoperatively, 1 day and 1 week postoperatively. CCT measurement was performed using a non-contact optical pachymeter followed by ocular response analyzer (ORA) examination. Intraoperatively ultrasound time, average phaco power and effective phaco time (EPT) were recorded. Mean CH was 10.05±1.86 mmHg preoperatively, 8.25±1.85 mmHg 1 day and 9.12±1.37 mmHg 1 week postoperatively (pcorneal alterations following cataract surgery resulted in a statistical change in CH and CCT. These two parameters responded in a different manner that clearly demarcates their different nature. On the first postoperative day, CCT increase was correlated at a statistically significant level with intraoperative EPT. This correlation was not found with CH reduction. Other factors, besides cornea oedema or phacoemulsification energy, could be responsible for this CH modification. © 2011 The Authors. Acta Ophthalmologica © 2011 Acta Ophthalmologica Scandinavica Foundation.

  13. Corneal donor tissue preparation for endothelial keratoplasty.

    Science.gov (United States)

    Woodward, Maria A; Titus, Michael; Mavin, Kyle; Shtein, Roni M

    2012-06-12

    Over the past ten years, corneal transplantation surgical techniques have undergone revolutionary changes. Since its inception, traditional full thickness corneal transplantation has been the treatment to restore sight in those limited by corneal disease. Some disadvantages to this approach include a high degree of post-operative astigmatism, lack of predictable refractive outcome, and disturbance to the ocular surface. The development of Descemet's stripping endothelial keratoplasty (DSEK), transplanting only the posterior corneal stroma, Descemet's membrane, and endothelium, has dramatically changed treatment of corneal endothelial disease. DSEK is performed through a smaller incision; this technique avoids 'open sky' surgery with its risk of hemorrhage or expulsion, decreases the incidence of postoperative wound dehiscence, reduces unpredictable refractive outcomes, and may decrease the rate of transplant rejection. Initially, cornea donor posterior lamellar dissection for DSEK was performed manually resulting in variable graft thickness and damage to the delicate corneal endothelial tissue during tissue processing. Automated lamellar dissection (Descemet's stripping automated endothelial keratoplasty, DSAEK) was developed to address these issues. Automated dissection utilizes the same technology as LASIK corneal flap creation with a mechanical microkeratome blade that helps to create uniform and thin tissue grafts for DSAEK surgery with minimal corneal endothelial cell loss in tissue processing. Eye banks have been providing full thickness corneas for surgical transplantation for many years. In 2006, eye banks began to develop methodologies for supplying precut corneal tissue for endothelial keratoplasty. With the input of corneal surgeons, eye banks have developed thorough protocols to safely and effectively prepare posterior lamellar tissue for DSAEK surgery. This can be performed preoperatively at the eye bank. Research shows no significant difference in

  14. Optic disc planimetry, corneal hysteresis, central corneal thickness, and intraocular pressure as risk factors for glaucoma.

    Science.gov (United States)

    Carbonaro, Francis; Hysi, Pirro G; Fahy, Samantha J; Nag, Abhishek; Hammond, Christopher J

    2014-02-01

    To determine whether corneal hysteresis and central corneal thickness are independent risk factors for glaucoma. A cross-sectional population-based cohort study. Associations were tested between corneal hysteresis, measured in 1754 population-based subjects from the TwinsUK cohort, and glaucoma-related endophenotypes, including intraocular pressure (IOP), vertical cup-to-disc ratio, optic disc area, and optic disc cup area. Corneal hysteresis, IOP, and central corneal thickness (CCT) were measured; optic disc photographs were analyzed; and multivariable linear regression analysis was performed. Data were available on 1645 individuals. Multiple regression analysis showed corneal hysteresis to be significantly negatively associated with age (beta coefficient = -0.03, P Corneal hysteresis was also found to be associated with CCT (beta coefficient = 0.02, P corneal hysteresis and optic disc area (P = .6), cup area (P = .77), vertical cup-to-disc ratio (P = .51), or spherical equivalent (P = .08). CCT was also found to be significantly associated with IOP (beta coefficient = 3.3, P corneal hysteresis (beta coefficient = 9.4, P corneal hysteresis or CCT and quantitative measures of optic disc cupping, suggesting that corneal hysteresis and CCT are not independent risk factors for glaucoma. Copyright © 2014 Elsevier Inc. All rights reserved.

  15. Association between corneal hysteresis and central corneal thickness in glaucomatous and non-glaucomatous eyes.

    Science.gov (United States)

    Mangouritsas, George; Morphis, George; Mourtzoukos, Spyridon; Feretis, Elias

    2009-11-01

    We aimed to determine corneal hysteresis values (CH) using the ocular response analyser (ORA) in non-glaucomatous and glaucomatous eyes and their relationship with central corneal thickness (CCT). Corneal hysteresis, intraocular pressure (IOP) as measured by Goldmann applanation tonometry (GAT) and CCT were prospectively evaluated in 74 non-glaucoma subjects with IOP Corneal hysteresis was significantly lower in eyes with treated POAG than in non-glaucomatous eyes. The corneal biomechanical response was strongly associated with CCT in non-glaucoma subjects, but only moderately so in glaucoma patients. It can be assumed that diverse structural factors, in addition to thickness, determine the differences in the corneal biomechanical profile between non-glaucomatous and glaucomatous eyes. Corneal hysteresis could be a useful tool in the diagnosis of glaucoma.

  16. Finite element modeling of corneal strip extensometry

    CSIR Research Space (South Africa)

    Botha, N

    2012-12-01

    Full Text Available symmetric conicoid [19]: (x xo) 2+(y yo) 2+(1+Q)(z zo) 2 2R(z zo) 2 = 0; (2) c SACAM 2012 25 Top view Isometric view Initial corneal curvature z y x x y z Fig. 3: Finite element model of the vertical corneal strip, including the orthogonal...

  17. Corneal staining after treatment with topical tetracycline

    NARCIS (Netherlands)

    Lapid-Gortzak, Ruth; Nieuwendaal, Carla P.; Slomovic, Allan R.; Spanjaard, Lodewijk

    2006-01-01

    PURPOSE: The purpose of this paper is to report a case of corneal staining after treatment with topical tetracycline. METHODS: A patient with crystalline keratopathy caused by Streptococcus viridans after corneal transplantation was treated topically with tetracycline eye drops, based on results of

  18. Corynebacterium macginleyi isolated from a corneal ulcer

    Directory of Open Access Journals (Sweden)

    Kathryn Ruoff

    2010-02-01

    Full Text Available We report the isolation of Corynebacterium macginleyi from the corneal ulcer culture of a patient, later enrolled in the Steroids for Corneal Ulcer Trial (SCUT. To our knowledge this is the first published report from North America of the recovery of C. macginleyi from a serious ocular infection.

  19. Corneal hysteresis and its relevance to glaucoma

    Science.gov (United States)

    Deol, Madhvi; Taylor, David A.; Radcliffe, Nathan M.

    2015-01-01

    Purpose of review Glaucoma is a leading cause of irreversible blindness worldwide. It is estimated that roughly 60.5 million people had glaucoma in 2010 and that this number is increasing. Many patients continue to lose vision despite apparent disease control according to traditional risk factors. The purpose of this review is to discuss the recent findings with regard to corneal hysteresis, a variable that is thought to be associated with the risk and progression of glaucoma. Recent findings Low corneal hysteresis is associated with optic nerve and visual field damage in glaucoma and the risk of structural and functional glaucoma progression. In addition, hysteresis may enhance intraocular pressure (IOP) interpretation: low corneal hysteresis is associated with a larger magnitude of IOP reduction following various glaucoma therapies. Corneal hysteresis is dynamic and may increase in eyes after IOP-lowering interventions are implemented. Summary It is widely accepted that central corneal thickness is a predictive factor for the risk of glaucoma progression. Recent evidence shows that corneal hysteresis also provides valuable information for several aspects of glaucoma management. In fact, corneal hysteresis may be more strongly associated with glaucoma presence, risk of progression, and effectiveness of glaucoma treatments than central corneal thickness. PMID:25611166

  20. Corneal Biomechanics Determination in Healthy Myopic Subjects

    Science.gov (United States)

    Qiu, Kunliang; Lu, Xuehui; Zhang, Riping; Wang, Geng

    2016-01-01

    Purpose. To determine the corneal biomechanical properties by using the Ocular Response Analyzer™ and to investigate potential factors associated with the corneal biomechanics in healthy myopic subjects. Methods. 135 eyes from 135 healthy myopic subjects were included in this cross-sectional observational study. Cornea hysteresis (CH), corneal resistance factor (CRF), cornea-compensated intraocular pressure (IOPcc), and Goldmann-correlated intraocular pressure (IOPg) were determined with the Reichert Ocular Response Analyzer (ORA). Univariate and multivariate regression analyses were performed to investigate factors associated with corneal biomechanics. Results. The mean CH and CRF were 9.82 ± 1.34 mmHg and 9.64 ± 1.57 mmHg, respectively. In univariate regression analysis, CH was significantly correlated with axial length, refraction, central corneal thickness (CCT), and IOPg (r = −0.27, 0.23, 0.45, and 0.21, resp.; all with p ≤ 0.015), but not with corneal curvature or age; CRF was significantly correlated with CCT and IOPg (r = 0.52 and 0.70, resp.; all with p corneal curvature, or age. In multivariate regression analysis, axial length, IOPcc, and CCT were found to be independently associated with CH, while CCT and IOPg were associated with CRF. Conclusions. Both CH and CRF were positively correlated with CCT. Lower CH but not CRF was associated with increasing degree of myopia. Evaluation of corneal biomechanical properties should take CCT and myopic status into consideration. PMID:27525109

  1. Corneal hysteresis and its relevance to glaucoma.

    Science.gov (United States)

    Deol, Madhvi; Taylor, David A; Radcliffe, Nathan M

    2015-03-01

    Glaucoma is a leading cause of irreversible blindness worldwide. It is estimated that roughly 60.5 million people had glaucoma in 2010 and that this number is increasing. Many patients continue to lose vision despite apparent disease control according to traditional risk factors. The purpose of this review is to discuss the recent findings with regard to corneal hysteresis, a variable that is thought to be associated with the risk and progression of glaucoma. Low corneal hysteresis is associated with optic nerve and visual field damage in glaucoma and the risk of structural and functional glaucoma progression. In addition, hysteresis may enhance intraocular pressure (IOP) interpretation: low corneal hysteresis is associated with a larger magnitude of IOP reduction following various glaucoma therapies. Corneal hysteresis is dynamic and may increase in eyes after IOP-lowering interventions are implemented. It is widely accepted that central corneal thickness is a predictive factor for the risk of glaucoma progression. Recent evidence shows that corneal hysteresis also provides valuable information for several aspects of glaucoma management. In fact, corneal hysteresis may be more strongly associated with glaucoma presence, risk of progression, and effectiveness of glaucoma treatments than central corneal thickness.

  2. Inhibition of Corneal Neovascularization by Hydrazinocurcumin ...

    African Journals Online (AJOL)

    Purpose: To investigate the effect of hydrazinocurcumin on a human vascular endothelial growth factor (VEGF)-induced corneal neovascularization in rabbit model. Methods: Murine corneal neovascularization (CorNV) was induced via two intrastromal implantations of VEGF polymer 2 mm from the limbus.

  3. Corneal plaque containing levofloxacin in a dog.

    Science.gov (United States)

    Park, Young-Woo; Kang, Byung-Jae; Lim, Jae Hyun; Ahn, Jung-Mo; Lim, Hyun Sook

    2015-11-01

    A 13-year-old castrated male Yorkshire terrier developed a corneal ulcer 2 weeks after intracapsular lens extraction (ICLE) in the right eye. The corneal ulcer was treated with levofloxacin eye drops. A plaque with a white luster developed in the central cornea 2 weeks after treatment with levofloxacin eye drops. The corneal plaque was surgically removed under inhalant anesthesia. The corneal plaque displayed antimicrobial activity against Escherichia coli. Furthermore, levofloxacin content in the plaque was confirmed by matrix-assisted laser desorption/ionization time-of-flight/time-of-flight (MALDI-TOF/TOF) mass spectrometry (MS). The corneal ulcer completely resolved 2 weeks after the surgical removal of the corneal lesion and replacement of levofloxacin eye drops with tobramycin eye drops. Although the topical use of levofloxacin is unlikely to lead to corneal chemical deposits due to the high water solubility of the drug compared to other topical fluoroquinolones, this patient developed corneal plaque of the antibiotic drop. © 2015 American College of Veterinary Ophthalmologists.

  4. Corneal laceration caused by river crab

    Directory of Open Access Journals (Sweden)

    Vinuthinee N

    2015-01-01

    Full Text Available Naidu Vinuthinee,1,2 Anuar Azreen-Redzal,1 Jaafar Juanarita,1 Embong Zunaina2 1Department of Ophthalmology, Hospital Sultanah Bahiyah, Alor Setar, 2Department of Ophthalmology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Malaysia Abstract: A 5-year-old boy presented with right eye pain associated with tearing and photophobia of 1-day duration. He gave a history of playing with a river crab when suddenly the crab clamped his fingers. He attempted to fling the crab off, but the crab flew and hit his right eye. Ocular examination revealed a right eye corneal ulcer with clumps of fibrin located beneath the corneal ulcer and 1.6 mm level of hypopyon. At presentation, the Seidel test was negative, with a deep anterior chamber. Culture from the corneal scrapping specimen grew Citrobacter diversus and Proteus vulgaris, and the boy was treated with topical gentamicin and ceftazidime eyedrops. Fibrin clumps beneath the corneal ulcer subsequently dislodged, and revealed a full-thickness corneal laceration wound with a positive Seidel test and shallow anterior chamber. The patient underwent emergency corneal toileting and suturing. Postoperatively, he was treated with oral ciprofloxacin 250 mg 12-hourly for 1 week, topical gentamicin, ceftazidime, and dexamethasone eyedrops for 4 weeks. Right eye vision improved to 6/9 and 6/6 with pinhole at the 2-week follow-up following corneal suture removal. Keywords: corneal ulcer, pediatric trauma, ocular injury

  5. Complications from Infective Corneal Conditions Treated by ...

    African Journals Online (AJOL)

    Background: Complications from infective corneal conditions are an important cause of blindness in adults and children. The eyelids play a major role in the pathogenesis of staphyloma and the perforation of descemetoceles. Tarsorrhaphy could, therefore, be beneficial in treating serious complications from infective corneal ...

  6. Facts about the Cornea and Corneal Disease

    Science.gov (United States)

    ... the Cornea and Corneal Disease Facts About the Cornea and Corneal Disease What is the cornea? The cornea is the eye’s outermost layer. It ... your vision. What are the parts of the cornea? Although the cornea may look clear and seem ...

  7. Corneal ulcers: For the general practitioner

    African Journals Online (AJOL)

    153 CME April 2013 Vol. 31 No. 4. Corneal ulcers: For the general practitioner. A corneal ulcer is a defect in the epithelial layer of the cornea. e general practitioner may play an important role in early management and appropriate referral. Incidence varies and depends on aetiology. S Ballim, MB ChB, Dip Ophth (SA), FC ...

  8. Inhibition of Corneal Neovascularization by Hydrazinocurcumin

    African Journals Online (AJOL)

    Purpose: To investigate the effect of hydrazinocurcumin on a human vascular endothelial growth factor. (VEGF)-induced corneal neovascularization in rabbit model. Methods: Murine corneal neovascularization (CorNV) was induced via two intrastromal implantations of. VEGF polymer 2 mm from the limbus.

  9. The heart in limb girdle muscular dystrophy

    NARCIS (Netherlands)

    van der Kooi, A. J.; de Voogt, W. G.; Barth, P. G.; Busch, H. F.; Jennekens, F. G.; Jongen, P. J.; de Visser, M.

    1998-01-01

    OBJECTIVE: To assess the frequency, nature, and severity of cardiac abnormalities in limb girdle muscular dystrophy, and its relation to age and weakness in various genotypes. DESIGN: In 26 autosomal dominant, 38 autosomal recessive, and 33 sporadic strictly defined patients with limb girdle

  10. Clinical features of facioscapulohumeral muscular dystrophy 2.

    NARCIS (Netherlands)

    Greef, J.C. de; Lemmers, R.J.; Camano, P.; Day, J.W.; Sacconi, S.; Dunand, M.; Engelen, B.G.M. van; Kiuru-Enari, S.; Padberg, G.W.A.M.; Rosa, A.L.; Desnuelle, C.; Spuler, S.; Tarnopolsky, M.; Venance, S.L.; Frants, R.R.; Maarel, S.M. van der; Tawil, R.

    2010-01-01

    OBJECTIVE: In some 5% of patients with facioscapulohumeral muscular dystrophy (FSHD), no D4Z4 repeat contraction on chromosome 4q35 is observed. Such patients, termed patients with FSHD2, show loss of DNA methylation and heterochromatin markers at the D4Z4 repeat that are similar to patients with

  11. Aberrant Myokine Signaling in Congenital Myotonic Dystrophy

    Directory of Open Access Journals (Sweden)

    Masayuki Nakamori

    2017-10-01

    Full Text Available Summary: Myotonic dystrophy types 1 (DM1 and 2 (DM2 are dominantly inherited neuromuscular disorders caused by a toxic gain of function of expanded CUG and CCUG repeats, respectively. Although both disorders are clinically similar, congenital myotonic dystrophy (CDM, a severe DM form, is found only in DM1. CDM is also characterized by muscle fiber immaturity not observed in adult DM, suggesting specific pathological mechanisms. Here, we revealed upregulation of the interleukin-6 (IL-6 myokine signaling pathway in CDM muscles. We also found a correlation between muscle immaturity and not only IL-6 expression but also expanded CTG repeat length and CpG methylation status upstream of the repeats. Aberrant CpG methylation was associated with transcriptional dysregulation at the repeat locus, increasing the toxic RNA burden that upregulates IL-6. Because the IL-6 pathway is involved in myocyte maturation and muscle atrophy, our results indicate that enhanced RNA toxicity contributes to severe CDM phenotypes through aberrant IL-6 signaling. : Congenital myotonic dystrophy (CDM manifests characteristic genetic (very large CTG repeat expansions, epigenetic (CpG hypermethylation upstream of the repeat, and phenotypic (muscle immaturity features not seen in adult DM. Nakamori et al. find phenotype-genotype and epigenotype correlation in CDM muscle and reveal involvement of the IL-6 myokine signaling pathway in the disease process. Keywords: CTCF, ER stress, IL-6, muscular dystrophy, NF-κB, trinucleotide, cytokine, splicing

  12. Hereditary muscular dystrophies and the heart

    NARCIS (Netherlands)

    Hermans, M. C. E.; Pinto, Y. M.; Merkies, I. S. J.; de Die-Smulders, C. E. M.; Crijns, H. J. G. M.; Faber, C. G.

    2010-01-01

    Cardiac disease is a common clinical manifestation of neuromuscular disorders, particularly of muscular dystrophies. Heart muscle cells as well as specialized conducting myocardial fibres may be affected by the dystrophic process. The incidence and nature of cardiac involvement vary with different

  13. Brain MRI in Congenital Muscular Dystrophies

    OpenAIRE

    J Gordon Millichap

    1997-01-01

    Magnetic resonance imaging (MRI) findings in 21 patients with congenital muscular dystrophy (CMD) complicated by cerebral anomalies were analysed from data collected at several Departments of Child Neurology, The Netherlands: Free University Hospital, and Emma Children’s Hospital, Amsterdam; Sophia Children’s Hospital, Rotterdam; Leiden University Hospital; Groningen University Hospital; and St Radboud University Hospital, Nijmegen.

  14. Prevalence of congenital muscular dystrophy in Italy

    Science.gov (United States)

    Graziano, Alessandra; Bianco, Flaviana; D'Amico, Adele; Moroni, Isabella; Messina, Sonia; Bruno, Claudio; Pegoraro, Elena; Mora, Marina; Astrea, Guja; Magri, Francesca; Comi, Giacomo P.; Berardinelli, Angela; Moggio, Maurizio; Morandi, Lucia; Pini, Antonella; Petillo, Roberta; Tasca, Giorgio; Monforte, Mauro; Minetti, Carlo; Mongini, Tiziana; Ricci, Enzo; Gorni, Ksenija; Battini, Roberta; Villanova, Marcello; Politano, Luisa; Gualandi, Francesca; Ferlini, Alessandra; Muntoni, Francesco; Santorelli, Filippo Maria; Bertini, Enrico; Pane, Marika

    2015-01-01

    Objective: We provide a nationwide population study of patients with congenital muscular dystrophy in Italy. Methods: Cases were ascertained from the databases in all the tertiary referral centers for pediatric neuromuscular disorders and from all the genetic diagnostic centers in which diagnostic tests for these forms are performed. Results: The study includes 336 patients with a point prevalence of 0.563 per 100,000. Mutations were identified in 220 of the 336 (65.5%). The cohort was subdivided into diagnostic categories based on the most recent classifications on congenital muscular dystrophies. The most common forms were those with α-dystroglycan glycosylation deficiency (40.18%) followed by those with laminin α2 deficiency (24.11%) and collagen VI deficiency (20.24%). The forms of congenital muscular dystrophy related to mutations in SEPN1 and LMNA were less frequent (6.25% and 5.95%, respectively). Conclusions: Our study provides for the first time comprehensive epidemiologic information and point prevalence figures for each of the major diagnostic categories on a large cohort of congenital muscular dystrophies. The study also reflects the diagnostic progress in this field with an accurate classification of the cases according to the most recent gene discoveries. PMID:25653289

  15. A Drosophila model for Duchenne muscular dystrophy

    NARCIS (Netherlands)

    Plas, Mariska Cathelijne van der

    2008-01-01

    Duchenne Muscular Dystrophy (DMD) is a severe X-linked disease characterized by progressive muscle wasting and sometimes mild mental retardation. The disease is caused by mutations in the dystrophin gene. DMD is correlated with the absence of Dp427, which is located along the sarcolemma in skeletal

  16. Prevalence of generalized retinal dystrophy in Denmark

    DEFF Research Database (Denmark)

    Bertelsen, Mette; Jensen, Hanne; Bregnhøj, Jesper F

    2014-01-01

    . RESULTS: Of the 5,602,628 Danish citizens on January 1, 2013, 1622 patients were registered as having a generalized retinal dystrophy and were alive and living in Denmark, corresponding to a prevalence of 1:3,454. In 28% of cases the eye condition was part of a syndrome, while the remaining 72% had eye...

  17. Visuospatial Attention Disturbance in Duchenne Muscular Dystrophy

    Science.gov (United States)

    De Moura, Maria Clara Drummond Soares; do Valle, Luiz Eduardo Ribeiro; Resende, Maria Bernadete Dutra; Pinto, Katia Osternack

    2010-01-01

    Aim: The cognitive deficits present in the Duchenne muscular dystrophy (DMD) are not yet well characterized. Attention, considered to be the brain mechanism responsible for the selection of sensory stimuli, could be disturbed in DMD, contributing, at least partially, to the observed global cognitive deficit. The aim of this study was to…

  18. Duchenne muscular dystrophy - a molecular service

    African Journals Online (AJOL)

    In 1987 a carrier detection and prenatal diagnostic service for. Duchenne muscular dystrophy using molecular technology was instituted at the Department of Human Genetics, Uni- versity of Cape Town, to serve affe.cted families in southern. Africa. DNA samples from 100 affected male subjects and. 350 of their relatives ...

  19. Brain Function in Duchenne Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    J. Gordon Millichap

    2002-02-01

    Full Text Available The role of dystrophin disorders in the CNS function of boys with Duchenne muscular dystrophy (DMD and the dystrophin-deficient mdx mouse, an animal model of DMD, is reviewed at the University of New South Wales, University of Sydney, Australia.

  20. Antisense Oligonucleotide Therapy for Inherited Retinal Dystrophies

    NARCIS (Netherlands)

    Gerard, X.; Garanto Iglesias, A.; Rozet, J.M.; Collin, R.W.J.

    2016-01-01

    Inherited retinal dystrophies (IRDs) are an extremely heterogeneous group of genetic diseases for which currently no effective treatment strategies exist. Over the last decade, significant progress has been made utilizing gene augmentation therapy for a few genetic subtypes of IRD, although several

  1. Corneal thickness and intraocular pressure in edematous corneas before and after Descemet stripping with automated endothelial keratoplasty.

    Science.gov (United States)

    Chang, Diane T W; Pantcheva, Mina B; Noecker, Robert J

    2010-10-01

    It is important to accurately measure intraocular pressure (IOP) in eyes with corneal endothelial dysfunction both before and after Descemet stripping with automated endothelial keratoplasty (DSAEK). Glaucoma is a common comorbidity in this population, and IOP elevation can worsen corneal edema. Additionally, preexisting glaucoma and steroid-responsive ocular hypertension are significant risk factors for graft rejection after DSAEK. Accurate tonometry is limited by variations in central corneal thickness (CCT) and corneal hydration that may affect corneal biomechanical properties. We analyzed CCT and IOP in eyes before and after DSAEK to determine whether changes in corneal biomechanics because of edema, grafted tissue, and subsequent stromal deturgescence affect IOP measurement. A retrospective chart review was performed on 32 eyes from 31 patients with corneal edema secondary to Fuchs endothelial dystrophy, bullous keratopathy, or prior graft failure, or rejection that received uncomplicated DSAEK with no evidence of persistent corneal edema or steroid-induced ocular hypertension. IOP was measured by Tono-Pen XL, and CCT was measured by ultrasound pachymetry before and approximately 3 months after surgery. We used paired t tests to evaluate changes in CCT and IOP after DSAEK and linear regression to determine the relationship between CCT and IOP before and after surgery. CCT significantly decreased from 703 ± 82 to 650 ± 52 μm after DSAEK (P = 0.0026), but there was no significant change in measured IOP (16.7 ± 3.4 mm Hg preoperatively and 16.3 ± 4.1 mm Hg postoperatively; P = 0.61). There was no significant relationship between CCT and IOP before (slope = 0.10 ± 0.07 mm Hg/10 μm; r = 0.062; P = 0.17) or after (slope = 0.21 ± 0.14 mm Hg/10 μm; r = 0.072; P = 0.14) DSAEK. CCT is significantly reduced by DSAEK but remains well above the normal range. IOP remains near the preoperative level 3 months after DSAEK. Furthermore, no correction is required for

  2. Molecular expression in transfected corneal endothelial cells

    Science.gov (United States)

    Wang, Fan; Miao, Zhuang; Lu, Chengwei; Hao, Jilong

    2017-10-01

    To investigate the capability of human corneal endothelial cells serving as immunological cells. Expression of HLA-DP, -DQ, -DR, CD40, CD80, and CD86 was determined by immunohistochemical methods. Meanwhile, purified peripheral blood mononuclear cells were cocultured with human corneal endothelial cells which were pre-treated with and without -IFN respectively, activation of lymphocytes was determined by FACS analysis. In coculture system, T lymphocyte was activated by corneal endothelial cells, HLA-DP, -DQ, -DR and CD40 expression were increased by - IFN induction. Costimulatory molecular CD80 was shown on the endothelial cells. Human corneal endothelial cells were assumed to be involved in the corneal transplantation rejection process as potential antigen presenting cells.

  3. Genetics Home Reference: T-cell immunodeficiency, congenital alopecia, and nail dystrophy

    Science.gov (United States)

    ... alopecia, and nail dystrophy T-cell immunodeficiency, congenital alopecia, and nail dystrophy Printable PDF Open All Close ... expand/collapse boxes. Description T-cell immunodeficiency, congenital alopecia, and nail dystrophy is a type of severe ...

  4. Corneal biomechanical properties in thyroid eye disease

    Directory of Open Access Journals (Sweden)

    Gamze Ozturk Karabulut

    2014-06-01

    Full Text Available The purpose of this study is to investigate the effect of thyroid eye disease (TED on the measurement of corneal biomechanical properties and the relationship between these parameters and disease manifestations. A total of 54 eyes of 27 individuals with TED and 52 eyes of 30 healthy control participants were enrolled. Thyroid ophthalmopathy activity was defined using the VISA (vision, inflammation, strabismus, and appearance/exposure classification for TED. The intraocular pressure (IOP measurement with Goldmann applanation tonometer (GAT, axial length (AL, keratometry, and central corneal thickness (CCT measurements were taken from each patient. Corneal biomechanical properties, including corneal hysteresis (CH and corneal resistance factor (CRF and noncontact IOP measurements, Goldmann-correlated IOP (IOPg and corneal-compensated IOP (IOPcc were measured with the Ocular Response Analyzer (ORA using the standard technique. Parameters such as best corrected visual acuity, axial length, central corneal thickness, and corneal curvature were not statistically significant between the two groups (p > 0.05. IOP measured with GAT was higher in participants with TED (p < 0.001. The CH of TED patients was significantly lower than that of the control group. There was no significant difference in the corneal resistance factor between groups. However, IOPg and IOPcc were significantly higher in TED patients. CH and VISA grading of TED patients showed a negative correlation (p = 0.007. In conclusion, TED affects the corneal biomechanical properties by decreasing CH. IOP with GAT and IOPg is found to be increased in these patients. As the severity of TED increases, CH decreases in these patients.

  5. Corneal manifestations of ocular demodex infestation.

    Science.gov (United States)

    Kheirkhah, Ahmad; Casas, Victoria; Li, Wei; Raju, Vadrevu K; Tseng, Scheffer C G

    2007-05-01

    To report the corneal manifestations in eyes with Demodex infestation of the eyelids. Noncomparative, interventional case series. This retrospective review included six patients with Demodex blepharitis who also exhibited corneal abnormalities, which led to suspicion of limbal stem cell deficiency in three cases. All patients received weekly lid scrubs with 50% tea tree oil and a daily lid scrubs with tea tree shampoo for a minimum of six weeks. Improvement of symptoms and corneal and conjunctival signs were evaluated. All six patients exhibited ocular irritation and conjunctival inflammation, while meibomian gland dysfunction (n = 5), rosacea (n = 4), and decreased vision (n = 3) also were noted despite prior treatments with oral tetracycline, topical steroids with antibiotics, and lid scrub with baby shampoo. These patients were proven to have Demodex folliculorum (n = 6) and Demodex brevis (n = 3) by microscopic examination of epilated lashes. Their corneal manifestation included superficial corneal vascularization (six eyes of five cases), marginal corneal infiltration (two eyes of two cases), phlyctenule-like lesion (one eye of one case), superficial corneal opacity (two eyes of two cases), and nodular corneal scar (two eyes of two cases). After treatment, the Demodex count was reduced from 6.8 +/- 2.8 to 1 +/- 0.9 (standard deviation; P = .001). All patients showed dramatic resolution of ocular irritation, conjunctival inflammation, and all inflammatory, but not scarred, corneal signs; three patients showed improved vision. A variety of corneal pathologic features together with conjunctival inflammation, commonly noted in rosacea, can be found in patients with Demodex infestation of the eyelids. When conventional treatments for rosacea fail, one may consider lid scrub with tea tree oil to eradicate mites as a new treatment.

  6. Influence of corneal hydration on optical coherence elastography

    Science.gov (United States)

    Twa, Michael D.; Vantipalli, Srilatha; Singh, Manmohan; Li, Jiasong; Larin, Kirill V.

    2016-03-01

    Corneal biomechanical properties are influenced by several factors, including intraocular pressure, corneal thickness, and viscoelastic responses. Corneal thickness is directly proportional to tissue hydration and can influence corneal stiffness, but there is no consensus on the magnitude or direction of this effect. We evaluated the influence of corneal hydration on dynamic surface deformation responses using optical coherence elastography (OCE). Fresh rabbit eyes (n=10) were prepared by removing the corneal epithelium and dropping with 0.9% saline every 5 minutes for 1 hour, followed by 20% dextran solution every 5 minutes for one hour. Corneal thickness was determined from structural OCT imaging and OCE measurements were performed at baseline and every 20 minutes thereafter. Micron-scale deformations were induced at the apex of the corneal tissue using a spatially-focused (150μm) short-duration (corneal thickness due to hydration process. Corneal thickness rapidly increased and remained constant following epithelium removal and changed little thereafter. Likewise, corneal stiffness changed little over the first hour and then decreased sharply after Dextran application (thickness: -46% [-315/682 μm] RR: - 24% [-0.7/2.88 ms-1]; GV: -19% [-0.6/3.2 m/s]). Corneal thickness and corneal stiffness (RR) were well correlated (R2 = .66). Corneal biomechanical properties are highly correlated with tissue hydration over a wide range of corneal thickness and these changes in corneal stiffness are quantifiable using OCE.

  7. Corneal Regeneration by Deep Anterior Lamellar Keratoplasty (DALK Using Decellularized Corneal Matrix.

    Directory of Open Access Journals (Sweden)

    Yoshihide Hashimoto

    Full Text Available The purpose of this study is to demonstrate the feasibility of DALK using a decellularized corneal matrix obtained by HHP methodology. Porcine corneas were hydrostatically pressurized at 980 MPa at 10°C for 10 minutes to destroy the cells, followed by washing with EGM-2 medium to remove the cell debris. The HHP-treated corneas were stained with H-E to assess the efficacy of decellularization. The decellularized corneal matrix of 300 μm thickness and 6.0 mm diameter was transplanted onto a 6.0 mm diameter keratectomy wound. The time course of regeneration on the decellularized corneal matrix was evaluated by haze grading score, fluorescein staining, and immunohistochemistry. H-E staining revealed that no cell nuclei were observed in the decellularized corneal matrix. The decellularized corneal matrices were opaque immediately after transplantation, but became completely transparent after 4 months. Fluorescein staining revealed that initial migration of epithelial cells over the grafts was slow, taking 3 months to completely cover the implant. Histological sections revealed that the implanted decellularized corneal matrix was completely integrated with the receptive rabbit cornea, and keratocytes infiltrated into the decellularized corneal matrix 6 months after transplantation. No inflammatory cells such as macrophages, or neovascularization, were observed during the implantation period. The decellularized corneal matrix improved corneal transparency, and remodelled the graft after being transplanted, demonstrating that the matrix obtained by HHP was a useful graft for corneal tissue regeneration.

  8. High interocular corneal symmetry in average simulated keratometry, central corneal thickness, and posterior elevation.

    Science.gov (United States)

    Myrowitz, Elliott H; Kouzis, Anthony C; O'Brien, Terrence P

    2005-05-01

    The purpose of this study was to assess interocular corneal symmetry in average simulated keratometry, corneal thickness, and posterior corneal elevation. This retrospective analysis included data from scanning slit topography (Orbscan II; Bausch and Lomb, Rochester, NY) on 242 eyes from 121 consecutive patients undergoing standard evaluation for consideration of elective laser vision correction. The symmetry between the right and left eye in average simulated keratometry, minimum central corneal thickness, and posterior corneal elevation was assessed by comparative data analysis. Simulated keratometry ranged from 39.9 to 48.6 D. The interocular difference in average simulated keratometry was 0.47 D (standard deviation [SD] 0.43). The interocular Pearson correlation coefficient for average simulated keratometry was 0.90 (p central corneal thickness was 0.95 (p symmetry in all these parameters was very high in this group of consecutive patients. Asymmetry of these interocular parameters may warrant repeat clinical testing for accuracy and may predict corneal abnormalities. Normative data on posterior cornea elevation is presented. This study points out potentially clinically important high interocular corneal symmetry data in simulated keratometry, corneal thickness, and posterior corneal elevation.

  9. Central nervous system involvement in progressive muscular dystrophy.

    Science.gov (United States)

    Yoshioka, M; Okuno, T; Honda, Y; Nakano, Y

    1980-01-01

    Several abnormalities in the central nervous system were shown in patients with progressive muscular dystrophy using computerised tomography (CT) scans, electroencephalograms, psychometry, and ophthalmological methods. In congenital muscular dystrophy, the most characteristic finding in the CT scan was a low density area in the white matter, seen in 14 (56%) out of 25 cases. In Duchenne dystrophy, slight cerebral atrophy was observed in 20 (67%) out of 30 cases. It was interesting that in the case of Duchenne dystrophy the older the patient, the more severe were the CT findings. In congenital muscular dystrophy half the patients with a low density area showed a spike or a spike-and-wave complex in the electroencephalogram, and optic atrophy was evident in several cases. It is concluded that progressive muscular dystrophy is not only a myogenic disorder but also one which affects the central nervous system. Images Fig. 1 Fig. 2 PMID:7436514

  10. Corneal epithelium following penetrating keratoplasty.

    Science.gov (United States)

    Tsubota, K; Mashima, Y; Murata, H; Yamada, M; Sato, N

    1995-03-01

    This study was designed to observe any changes to the corneal epithelium after penetrating keratoplasty. The corneal epithelia of 26 patients were observed by specular microscopy 1 week, 1 month, 3 months, and 6 months following penetrating keratoplasty. After re-epithelialisation was confirmed by biomicroscopy 1 week after surgery, specular microscopy revealed many abnormal cells, including spindle shaped cells, nucleated cells, large cells, as well as irregular cell configurations. Although these abnormal findings tended to decrease with time, they were still present in some cases as much as 6 months postoperatively. Computerised morphometric analysis yielded mean cell areas of 1121 (SD 168) microns 2, 1139 (675) microns 2, 1712 (496) microns 2, and 1400 (377) microns 2 at 1 week, 1 month, 3 months, and 6 months respectively, all significantly greater than that of age matched controls (710 (151) microns 2). The shape factor decreased with time, but was still greater than the control level at 6 months. This study demonstrates that epithelial abnormalities persist longer than expected after penetrating keratoplasty, and that these subtle changes can be detected by specular microscopic observation, potentially allowing for modification and enhancement of the wound healing process.

  11. Intrastromal corneal ring implants for corneal thinning disorders: an evidence-based analysis.

    Science.gov (United States)

    2009-01-01

    The purpose of this project was to determine the role of corneal implants in the management of corneal thinning disease conditions. An evidence-based review was conducted to determine the safety, effectiveness and durability of corneal implants for the management of corneal thinning disorders. The evolving directions of research in this area were also reviewed. SUBJECT OF THE EVIDENCE-BASED ANALYSIS: The primary treatment objectives for corneal implants are to normalize corneal surface topography, improve contact lens tolerability, and restore visual acuity in order to delay or defer the need for corneal transplant. Implant placement is a minimally invasive procedure that is purported to be safe and effective. The procedure is also claimed to be adjustable, reversible, and both eyes can be treated at the same time. Further, implants do not limit the performance of subsequent surgical approaches or interfere with corneal transplant. The evidence for these claims is the focus of this review. The specific research questions for the evidence review were as follows: SafetyCorneal Surface Topographic Effects:Effects on corneal surface remodellingImpact of these changes on subsequent interventions, particularly corneal transplantation (penetrating keratoplasty [PKP])Visual AcuityRefractive OutcomesVISUAL QUALITY (SYMPTOMS): such as contrast vision or decreased visual symptoms (halos, fluctuating vision)Contact lens toleranceFunctional visual rehabilitation and quality of lifePatient satisfaction:Disease Process:Impact on corneal thinning processEffect on delaying or deferring the need for corneal transplantation TARGET POPULATION AND CONDITION Corneal ectasia (thinning) comprises a range of disorders involving either primary disease conditions such as keratoconus and pellucid marginal corneal degeneration or secondary iatrogenic conditions such as corneal thinning occurring after LASIK refractive surgery. The condition occurs when the normally round dome-shaped cornea

  12. Retinal capillaritis in a CRB1-associated retinal dystrophy.

    Science.gov (United States)

    Murro, Vittoria; Mucciolo, Dario Pasquale; Sodi, Andrea; Vannozzi, Lorenzo; De Libero, Cinzia; Simonini, Gabriele; Rizzo, Stanislao

    2017-12-01

    To report a case of CRB1-associated retinal dystrophy characterized by vitritis, retinal capillaritis, and cystoid macular edema (CME). A case report. An 8-year-old boy was diagnosed with intermediate uveitis and treated with corticosteroids. He was subsequently diagnosed with retinal dystrophy and found to have two CRB1 mutations. Retinal capillaritis, vitritis, and CME could be inflammatory features of CRB1 retinal dystrophy in our young patient.

  13. Cognitive and Neurobehavioral Profile in Boys With Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Banihani, Rudaina; Smile, Sharon; Yoon, Grace; Dupuis, Annie; Mosleh, Maureen; Snider, Andrea; McAdam, Laura

    2015-10-01

    Duchenne muscular dystrophy is a progressive neuromuscular condition that has a high rate of cognitive and learning disabilities as well as neurobehavioral disorders, some of which have been associated with disruption of dystrophin isoforms. Retrospective cohort of 59 boys investigated the cognitive and neurobehavioral profile of boys with Duchenne muscular dystrophy. Full-scale IQ of Duchenne muscular dystrophy. © The Author(s) 2015.

  14. Current treatment options for corneal ectasia.

    Science.gov (United States)

    Tan, Donald T H; Por, Yong-Ming

    2007-07-01

    The approach to the management of various forms of corneal ectasia is changing, with the advent of new surgical and nonsurgical options. The purpose of this review is to summarize and evaluate relevant studies on new treatments for keratoconus, postrefractive surgery keratectasia, and peripheral ectatic corneal disorders. Various alternatives to corneal transplantation for the management of keratoconus aim to enhance corneal rigidity by means of nonsurgical collagen cross-linking, or with the use of intrastromal corneal ring segments, and studies suggest that these treatments may reduce astigmatism or ectatic progression to varying degrees. Recent developments in anterior lamellar keratoplasty enable targeted replacement or augmentation of corneal stroma without replacement of endothelium, and include procedures such as deep anterior lamellar keratoplasty, microkeratome or laser-assisted anterior lamellar surgery, and peripheral tectonic lamellar keratoplasty procedures demonstrate successful reinforcement of peripheral stroma to reduce astigmatism. These new forms of surgery are viable alternatives to conventional penetrating keratoplasty and bring added safety profiles for long-term visual rehabilitation and restoration of tectonic integrity in central and peripheral forms of corneal ectasia.

  15. Factors Affecting Corneal Hysteresis in Taiwanese Adults.

    Science.gov (United States)

    Wang, Jia-Kang; Huang, Tzu-Lun; Pei-Yuan Su; Chang, Pei-Yao

    2015-09-01

    To investigate the correlation of various corneal hysteresis (CH) factors in Chinese adults. From January 2009 to November 2011, the healthy right eyes of a total of 292 adults were recruited into the study. Goldmann-correlated intraocular pressure (IOPG) and CH were measured using an ocular response analyzer (ORA). Central corneal thickness was measured using the ORA's integrated handheld ultrasonic pachymeter. The IOLMaster was used to obtain the ocular biometric measurements including axial length, anterior chamber depth, and keratometric values. The Pearson correlation coefficient was used to test correlations between CH and quantitative factors. The chi-square test was used to detect differences in categorical values. Longer axial length (P = 0.0001), lower IOPG (P = 0.03), older age (P = 0.003), and thinner central corneal thickness (P = 0.0001) were significantly associated with lower CH. The anterior chamber depth (P = 0.34), gender (P = 0.23), and corneal curvature (P = 0.18) had no relationship to CH. Various factors including axial length, intraocular pressure, age, and central corneal thickness can affect measurement of corneal biomechanical properties in Chinese adults. But the anterior chamber depth, gender, and corneal curvature were irrelevant to CH.

  16. Analysis of ethanol effects on corneal epithelium.

    Science.gov (United States)

    Oh, Joo Youn; Yu, Ji Min; Ko, Jung Hwa

    2013-06-04

    Ethanol is widely used in ocular surface surgeries and for the treatment of corneal diseases. However, ethanol is a toxic agent that is related to the development of a number of alcohol-related diseases. Despite the common use of ethanol for therapeutic purposes in ophthalmology, effects of ethanol on the ocular surface have been poorly defined. Hence, we performed this study to investigate effects of ethanol on corneal epithelium from various aspects. We exposed corneal epithelial cells in culture to different concentrations of ethanol for 30 seconds and evaluated the cells for toxicity, survival, and expression of cell-specific markers and inflammatory cytokines at 24, 48, and 72 hours after ethanol exposure. We found that ethanol markedly decreased the viability of cells in a concentration-dependent manner by causing cell lysis, suppressing proliferation, and inducing apoptosis. Also, expression of corneal epithelial cell-specific markers, both stem cell and differentiation markers, was significantly reduced by ethanol exposure. Expression of proinflammatory cytokines and chemokines was highly increased in corneal epithelial and stromal cells that were exposed to ethanol. Together, data suggest that brief exposure of the corneal surface to ethanol may have long-term effects by disrupting the integrity of corneal epithelium and generating inflammation, both of which are precursors to a number of ocular surface diseases.

  17. Electrospun Scaffolds for Corneal Tissue Engineering: A Review

    OpenAIRE

    Bin Kong; Shengli Mi

    2016-01-01

    Corneal diseases constitute the second leading cause of vision loss and affect more than 10 million people globally. As there is a severe shortage of fresh donated corneas and an unknown risk of immune rejection with traditional heterografts, it is very important and urgent to construct a corneal equivalent to replace pathologic corneal tissue. Corneal tissue engineering has emerged as a practical strategy to develop corneal tissue substitutes, and the design of a scaffold with mechanical pro...

  18. Current concepts and techniques in corneal transplantation.

    Science.gov (United States)

    Laibson, Peter R

    2002-08-01

    The corneal endothelium is the most important single layer in corneal transplantation. In his Castroviejo Lecture, William Bourne, MD, summarizes his work on the corneal endothelium and its importance to corneal transplantation. Almost half the corneal transplants performed in the United States are done so because of malfunctioning, diseased, or absent endothelial cells. If just this layer could be transplanted, the long wait for better vision after keratoplasty (up to two years in some countries) can be eliminated, as well as the problems of epithelial and subepithelial graft rejection. The significant astigmatism after keratoplasty could also be reduced. Transplantation of the endothelium in deep lamellar keratoplasty is being done in limited fashion throughout the world and the first patients have now been done in the United States. In many countries where corneal tissue is difficult to obtain, keratoplasty is only performed on one eye, even though both eyes may need it. One article this year discusses binocular vision recovery in bilateral keratoplasty and the objective and subjective improvements after bilateral keratoplasty. Patients who are bilaterally blind from diseases such as Stevens-Johnson syndrome, and ocular pemphigoid have little hope of visual recovery from conventional corneal transplants. The use of a keratoprosthesis to bypass the totally abnormal conjunctival surface has helped many people in the past. The preoperative prognostic categories of patients who may benefit from keratoprosthesis has been carefully reviewed in a large number of keratoprosthesis patients and this information will help ophthalmologists decide who will benefit the most benefit from keratoprosthesis. This year, articles on corneal transplantation after conjunctival flaps, suture-related complications in keratoplasty, the implantation of an intraocular lens after penetrating keratoplasty, and long-term results of penetrating keratoplasty with glaucoma drainage tube

  19. Corneal Biomechanical Properties in Rheumatoid Arthritis.

    Science.gov (United States)

    Can, Mehmet Erol; Erten, Sukran; Can, Gamze Dereli; Cakmak, Hasan Basri; Sarac, Ozge; Cagil, Nurullah

    2015-11-01

    To investigate the variations in biomechanical properties of the cornea in rheumatoid arthritis (RA) patients. A total of 53 RA patients, and 25 healthy individuals (control group) were enrolled. Rheumatoid arthritis patients were classified as in active phase (group 1; n=24) or in remission phase (group 2; n=29). Corneal biomechanical parameters including corneal hysteresis (CH), corneal resistance factor (CRF), corneal compensated intraocular pressure (IOPcc), and Goldmann-correlated IOP (IOPg) were measured with the Reichert Ocular Response Analyzer. Topographical measurements, including central corneal thickness (CCT), anterior chamber depth, iridocorneal angle, and corneal volume were measured using a Sirius corneal topographer. The mean CH was 9.43±1.17 mm Hg in group 1, 9.42±1.84 mm Hg in group 2, and 10.47±1.68 mm Hg in the control group (P=0.03). The mean IOPcc was 17.85±3.2 mm Hg in group 1, 17.95±3.49 mm Hg in group 2, and 15.36±3.11 mm Hg in the control group (P=0.008). The CH showed a significant positive correlation with CRF (P=0.000, r=0.809) and CCT (P=0.000, r=0.461), and a significant negative correlation with IOPcc (P=0.000, r=-0.469). Decrease in the mean CH measurements indicates that ultrastructural changes in the cornea may occur in the active phase, and these changes persist in the remission period. In addition, IOPcc is significantly affected by the corneal biomechanical properties. In RA patients, it is important to control the corneal parameters and IOP measurements against the irreversible changes on the optic nerve.

  20. PABPN1 gene therapy for oculopharyngeal muscular dystrophy

    National Research Council Canada - National Science Library

    A Malerba; P Klein; H Bachtarzi; S A Jarmin; G Cordova; A Ferry; V Strings; M Polay Espinoza; K Mamchaoui; S C Blumen; J Lacau St Guily; V Mouly; M Graham; G Butler-browne; D A Suhy; C Trollet; G Dickson

    2017-01-01

      Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant, late-onset muscle disorder characterized by ptosis, swallowing difficulties, proximal limb weakness and nuclear aggregates in skeletal muscles...

  1. C.57 C > T Mutation in MIR 184 is Responsible for Congenital Cataracts and Corneal Abnormalities in a Five-generation Family from Galicia, Spain.

    Science.gov (United States)

    Bykhovskaya, Yelena; Caiado Canedo, Ana L; Wright, Kenneth W; Rabinowitz, Yaron S

    2015-01-01

    A c.57 C > T mutation in the seed region of MIR184 located at the 15q25.1 chromosomal region has been independently associated with autosomal dominant keratoconus with early-onset anterior polar cataract in the Northern Irish family and with autosomal dominant EDICT (Endothelial Dystrophy, Iris hypoplasia, Congenital cataracts, and stromal Thinning) syndrome. In this study we report a five-generation family originating in Galicia, Spain with early onset cataracts and variable corneal abnormalities which include non-ectatic corneal thinning and severe early-onset keratoconus. We identified a heterozygous c.57 C > T mutation in miR-184 in the proband and two additional affected relatives on the maternal side. This finding represents a third independent occurrence of this mutation in familiar ocular disease thus strengthening the link between miR-184 abnormalities and inherited eye defects.

  2. Technology needs for corneal transplant surgery

    Science.gov (United States)

    Vaddavalli, Pravin K.; Yoo, Sonia H.

    2011-03-01

    Corneal transplant surgery has undergone numerous modifications over the years with improvements in technique, instrumentation and eye banking. The main goals of corneal transplantation are achieving excellent optical clarity with long-term graft survival. Penetrating, anterior and posterior lamellar surgery along with femtosecond laser technology have partially met these goals, but outcomes are often unpredictable and surgeon dependent. Technology to predictably separate stroma from Descemet's membrane, techniques to minimize endothelial cell loss, improvements in imaging technology and emerging techniques like laser welding that might replace suturing, eventually making corneal transplantation a refractively predictable procedure are on the wish list of the cornea surgeon.

  3. The Effect of Smoking on Corneal Biomechanics.

    Science.gov (United States)

    Kilavuzoglu, Ayse Ebru; Celebi, Ali Riza Cenk; Altiparmak, Ugur Emrah; Cosar, Cemile Banu

    2017-01-01

    To determine the effect of smoking on corneal biomechanical behavior. The medical records of consecutive patients that presented to the ophthalmology department were reviewed. History of smoking and ophthalmological examination findings were recorded. The smoking group met the following criteria: a clear history of and present smoking habit, negative history of corneal disease and surgery, ocular response analyzer measurement at the time of examination, and a waveform score ≥3.7. Nonsmokers (never smoked or quit smoking ≥6 months earlier) that met the same criteria constituted the control group. Corneal biomechanical parameters were measured using ocular response analyzer. Data were analyzed using Pearson's χ(2) test, Mann-Whitney U test, and Spearman's correlation coefficient. The smoking group included 166 eyes of 166 patients with a mean age of 38.7 ± 11.95 years, and the control group consisted of 170 eyes of 170 patients with a mean age of 38.40 ± 12.2 years. Mean cumulative smoking dose in the smoking group was 9.59 ± 11.87 pack-years (0.04- 75.00). There was no significant correlation between cumulative smoking dose and corneal hysteresis and corneal resistance factor (P = 0.382 and 0.074, respectively). There were no significant differences in corneal hysteresis or the corneal resistance factor between the two groups (P > 0.05). There was no significant difference in corneal hysteresis between those in the smoking group aged 18-44 years and those aged 45-64 years (P = 0.258), whereas in the control group mean corneal hysteresis was significantly lower in the 45-64 year olds than in the 18-44 year olds (P = 0.034). Although there was no significant difference in corneal biomechanics between smoking and control groups, the decrease in corneal hysteresis with aging was less apparent in the smoking group, which may be due to the potential changes in the cornea's microstructure induced by smoking during aging and the effect of smoking in aged corneal

  4. Should nylon corneal sutures be routinely removed?

    Science.gov (United States)

    Jackson, H.; Bosanquet, R.

    1991-01-01

    Three groups of patients who had undergone cataract extraction through a corneal incision closed with 10/0 nylon sutures one, two, and three years previously were recalled to determine the incidence of suture related complications. Broken corneal sutures were found in 87.5% of patients after two years and 90% after three years and were causing symptoms in over half the patients. It is recommended that 10/0 nylon corneal sutures be routinely removed no later than one year after surgery. Images PMID:1751460

  5. Microkeratome-assisted superficial anterior lamellar keratoplasty for anterior stromal corneal opacities after penetrating keratoplasty.

    Science.gov (United States)

    Patel, Amit K; Scorcia, Vincenzo; Kadyan, Anju; Lapenna, Lucia; Ponzin, Diego; Busin, Massimo

    2012-01-01

    To describe the surgical technique and report the outcomes of patients treated with microkeratome-assisted superficial anterior lamellar keratoplasty for anterior stromal corneal opacities developing after penetrating keratoplasty (PK). All patients with post-penetrating keratoplasty anterior stromal opacities treated with microkeratome-assisted superficial anterior lamellar keratoplasty between July 2005 and June 2007 were reviewed. A 130-μm superficial keratectomy was performed, followed by the placement of an appropriately sized donor graft, which was secured with overlay sutures. Refraction, corneal topography, and uncorrected and best-corrected visual acuities (UCVA, BCVA, respectively) were noted at each examination. Nine eyes of 8 consecutive patients were identified. Causes of anterior stromal opacities included dystrophy recurrence (n = 3), post-photorefractive keratectomy haze (n = 2), and scarring after stromal melt (n = 4). BCVA improved in all 9 eyes at final follow-up, and 7 of 9 eyes achieved ≥20/40 within the first month. Average follow-up period was 28 ± 3.9 months. Refractive astigmatism also improved by an average of 0.7 diopters. Superficial anterior lamellar keratoplasty is a viable and effective alternative to repeat PK in treating anterior stromal scars. It avoids open-globe surgery and exposure to endothelial rejection associated with repeat PK, and visual rehabilitation is considerably quicker.

  6. Clinical study on human lamellar keratoplasty for fungal corneal ulcers with porcine acellular corneal stroma

    OpenAIRE

    Fu-Hong Liao; Zi-Zhong Yu; Bin Hu

    2017-01-01

    AIM: To observe the transplantation of acellular porcine corneal stroma on the treatment of superficial keratitis by drug-resistant fungal. METHODS: We performed a retrospective analysis of 16 cases of fungal keratitis received the transplantation of acellular porcine corneal matrix from June 2015 to March 2016 with a follow-up of 6mo. We analyzed on items as postoperative visual acuity, corneal graft status, postoperative recurrence and postoperative complications. RESULTS: We observed a hea...

  7. Corneal-Wavefront guided transepithelial photorefractive keratectomy after corneal collagen cross linking in keratoconus

    OpenAIRE

    Camellin, Massimo; Guidotti, Jacopo Maria; Arba Mosquera, Samuel

    2016-01-01

    Purpose To evaluate the efficacy and safety of Corneal-Wavefront guided transepithelial photorefractive keratectomy (TransPRK) after corneal collagen cross linking (CXL) in keratoconic patients. Methods In this retrospective, non-comparative, consecutive case series, 39 keratoconic eyes underwent Corneal-Wavefront guided TransPRK for the correction of aberrations at least 4 months after conventional CXL at SEKAL Rovigo Microsurgery Centre, Rovigo, Italy. Two eyes (5%) underwent a secondary la...

  8. Role of Corneal Epithelium in Riboflavin/Ultraviolet-A Mediated Corneal Cross-Linking Treatment in Rabbit Eyes

    OpenAIRE

    Xiangchen Tao; Haiqun Yu; Yong Zhang; Zhiwei Li; Vishal Jhanji; Shouxiang Ni; Ya Wang; Guoying Mu

    2013-01-01

    Purpose. To evaluate the role of corneal epithelium in riboflavin/ultraviolet-A (UVA) mediated corneal collagen cross-linking treatment. Methods. Fifty New Zealand rabbits were divided into 5 groups: UVA treatment with or without corneal epithelium, UVA+riboflavin treatment with or without corneal epithelium, and control without any treatment. All rabbits were sacrificed after irradiation and subsequently 4?mm???10?mm corneal strips were harvested for biomechanical evaluation. Results. UVA ir...

  9. Clinical assessment of the soft corneal contact lens on corneal epithelium defect after vitrectomy

    Directory of Open Access Journals (Sweden)

    Meng-Su Tang

    2017-03-01

    Full Text Available AIM: To investigate the effect of soft corneal contact lens on the recovery of corneal epithelial defects and the comfort in patients with diabetic retinopathy after vitrectomy. METHODS: Twenty patients(20 eyeswith diabetic retinopathy whose corneal epithelium defected after vitrectomy were randomly divided into 2 groups. The trial group(10 eyesreceived the combination treatment of wearing soft corneal contact lenses and eyedrops to promote corneal epithelial repair, and the control group(10 eyesonly received eyedrops. The corneal epithelial healing time was observed postoperatively. And the scores of the comfort in patients between two groups were compared. RESULTS: The average duration of corneal epithelial defects was 4.1±1.20d in the trial group, and 14.2±6.07d in the control group, which was statistically significant different between the two groups(PP>0.05. CONCLUSION: The soft corneal contact lens could effectively treat patients with corneal epithelial defects after vitrectomy, and improve their comfort.

  10. Correlation of both corneal surfaces in corneal ectasia after myopic LASIK.

    Science.gov (United States)

    Peinado, Teresa Fernández; Piñero, David P; López, Ignacio Alcaraz; Alio, Jorge L

    2011-04-01

    We report a case of corneal ectasia in a 25-year-old man after myopic laser in situ keratomileusis in which a complete characterization of the corneal structure was performed by means of a Scheimpflug photography-based system. The patient presented in the ectatic eye with a subjective refraction of +0.50 to 6.00 × 100°, which with correction gave a visual acuity of 20/25. With the topographic analysis, corneal shapes from both corneal surfaces at the four different quadrants were found to be complementary, maintaining the meniscus-shaped profile of the cornea. This correlation between the anterior and posterior corneal surfaces was also confirmed with an optical tomography evaluation. Corneal biomechanics was also evaluated by means of the Ocular Response Analyzer (Reichert), which confirmed the biomechanical alteration. In summary, biomechanical changes leading to corneal ectasia in this case affected the global corneal structure, inducing alterations in the shape of both anterior and posterior corneal surfaces.

  11. [The effect of cataract and vitreoretinal surgery on central corneal thickness and corneal hysteresis].

    Science.gov (United States)

    Hager, A; Loge, K; Kutschan, A; Wiegand, W

    2008-03-01

    Intraocular irrigating solutions remain for several hours beyond the actual time of surgery in the eye. The irrigating solution ought to resemble biochemically aqueous humor and vitreous and offer protection for sensitive structures of the eye, such as the corneal endothelium. Impairment of the corneal endothelium may lead to corneal oedema and biomechanical alterations of the cornea. 54 eyes after pars-plana vitrectomy (PPV) in elective macular surgery were evaluated by measuring corneal thickness (CCT) using ultrasound pachymetry (20 MHz) and corneal hysteresis (CH) using the ocular response analyser (Reichert Ophthalmic Instruments, Buffalo, NY, USA). Measurements were performed not earlier than 2 weeks prior to surgery and 1 to 3 days after surgery. Results were compared to a control group (n = 39) and to 101 eyes after clear cornea cataract extraction (KAT). The two groups (PPV and KAT) did not differ with respect to age (p = 0.555). Corneal thickness has increased significantly in both groups (p corneal hysteresis decreased significantly postoperatively (p corneal thickness but also by biomechanical parameters such as corneal hysteresis.

  12. Corneal hysteresis, resistance factor, topography, and pachymetry after corneal lamellar flap.

    Science.gov (United States)

    Gatinel, Damien; Chaabouni, Slim; Adam, Pierre-Alexandre; Munck, Jacques; Puech, Michel; Hoang-Xuan, Thanh

    2007-01-01

    To measure prospectively the early changes in corneal hysteresis, topography, and pachymetry after the creation of a stromal flap cut without laser photoablation. A 37-year-old man was referred for a bioptic procedure to correct for compound myopic astigmatism in the left eye. A 159-microm-thick 8x8.5-mm superior hinged flap was created with a mechanical microkeratome in the left cornea. Changes in the corneal hysteresis, corneal resistance factor, Goldmann correlated intraocular pressure (lOP), corneal compensated IOP, anterior and posterior topography, and optical and ultrasound pachymetry were monitored prospectively before and at 1 hour, 1 day, 5 days, and 25 days after flap creation. The right eye served as a control. In the left eye, corneal hysteresis and corneal resistance factor decreased immediately after the flap cut and remained lower than preoperatively at 1 hour, 1 day, 5 days, and 25 days. Corneal compensated IOP varied significantly less than Goldmann correlated IOP in both eyes. Central flattening of the horizontal meridians was observed on the difference topography maps. The values of the left eye posterior best fit sphere increased after the flap cut. Increased central corneal thickness occurred immediately after the flap cut and decreased over time without returning to its preoperative value. The creation of a stromal flap can modify the biomechanical properties of the cornea, including a reduction in corneal hysteresis. The topographic changes were consistent with previously reported cases of flap cut in normal corneas.

  13. Repair of corneal ulcer or perforation using the corneal stromal lenticule

    Directory of Open Access Journals (Sweden)

    Xiao-Feng Hao

    2018-01-01

    Full Text Available AIM: To describe the outcomes of corneal stromal lenticules in repairing of corneal ulcer and/or perforation. METHODS: This was a retrospective chart review of 6 eyes of 6 patients from January to June 2017,who underwent corneal ulcer repair with the corneal, stromal lenticules harvested from femtosecond laser refractive surgery and kept in pure glycerin for use. Three cases of infectious corneal ulcers were bacterial, fungal, and infection with foreign bodies in corneal deep layer, one each. The other 3 were corneal ulcer perforation. Making sure no air bubble between donor graft and Descemet membrane. The mean follow-up time was 3.71±1.56mo(range 1-6mo. RESULTS: All eyes were successfully treated under control of infection without intra-operative complications, and early postoperative evaluation showed a clear graft in all cases. The last follow-up visit showed the mean best corrected visual acuity(VAsignificantly improved after surgery. There was significant difference from 0.48±0.12 to 1.50±0.08(PCONCLUSION: The preliminary results suggest that the use of corneal stromal lenticules may be a safe and effective surgical alternative for corneal ulcer, even though the long-term outcome of the graft needs to be further observed.

  14. Disrupted cell cycle arrest and reduced proliferation in corneal fibroblasts from GCD2 patients: A potential role for altered autophagy flux

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Seung-il; Dadakhujaev, Shorafidinkhuja; Maeng, Yong-Sun; Ahn, So-yeon; Kim, Tae-im [Department of Ophthalmology, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Corneal Dystrophy Research Institute, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, Eung Kweon, E-mail: eungkkim@yuhs.ac [Department of Ophthalmology, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Corneal Dystrophy Research Institute, Yonsei University College of Medicine, Seoul (Korea, Republic of); BK21 Plus Project for Medical Science and Severance Biomedical Science Institute, Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2015-01-02

    Highlights: • Reduced cell proliferation in granular corneal dystrophy type 2. • Abnormal cell cycle arrest by defective autophagy. • Decreased Cyclin A1, B1, and D1 in Atg7 gene knockout cells. • Increase in p16 and p27 expressions were observed in Atg7 gene knockout cells. - Abstract: This study investigates the role of impaired proliferation, altered cell cycle arrest, and defective autophagy flux of corneal fibroblasts in granular corneal dystrophy type 2 (GCD2) pathogenesis. The proliferation rates of homozygous (HO) GCD2 corneal fibroblasts at 72 h, 96 h, and 120 h were significantly lower (1.102 ± 0.027, 1.397 ± 0.039, and 1.527 ± 0.056, respectively) than those observed for the wild-type (WT) controls (1.441 ± 0.029, 1.758 ± 0.043, and 2.003 ± 0.046, respectively). Flow cytometry indicated a decreased G{sub 1} cell cycle progression and the accumulation of cells in the S and G{sub 2}/M phases in GCD2 cells. These accumulations were associated with decreased levels of Cyclin A1, B1, and E1, and increased expression of p16 and p27. p21 and p53 expression was also significantly lower in GCD2 cells compared to the WT. Interestingly, treatment with the autophagy flux inhibitor, bafilomycin A{sub 1}, resulted in similarly decreased Cyclin A1, B1, D1, and p53 expression in WT fibroblasts. Furthermore, similar findings, including a decrease in Cyclin A1, B1, and D1 and an increase in p16 and p27 expression were observed in autophagy-related 7 (Atg7; known to be essential for autophagy) gene knockout cells. These data provide new insight concerning the role of autophagy in cell cycle arrest and cellular proliferation, uncovering a number of novel therapeutic possibilities for GCD2 treatment.

  15. Anoctamin 5 muscular dystrophy in Denmark

    DEFF Research Database (Denmark)

    Witting, Nanna; Duno, Morten; Petri, Helle

    2013-01-01

    Since the initial description in 2010 of anoctamin 5 deficiency as a cause of muscular dystrophy, a handful of papers have described this disease in cases of mixed populations. We report the first large regional study and present data on new aspects of prevalence, muscular and cardiac phenotypic...... characteristics, and muscle protein expression. All patients in our neuromuscular unit with genetically unclassified, recessive limb girdle muscular dystrophy (LGMD2), Miyoshi-type distal myopathy (MMD) or persistent asymptomatic hyperCK-emia (PACK) were assessed for mutations in the ANO5 gene. Genetically...... confirmed patients were evaluated with muscular and cardiopulmonary examination. Among 40 unclassified patients (28 LGMD2, 5 MMD, 7 PACK), 20 were homozygous or compound heterozygous for ANO5 mutations, (13 LGMD2, 5 MMD, 2 PACK). Prevalence of ANO5 deficiency in Denmark was estimated at 1:100.000 and ANO5...

  16. Pain characterization in Duchenne muscular dystrophy

    Directory of Open Access Journals (Sweden)

    Talita Dias da Silva

    Full Text Available ABSTRACT Duchenne muscular dystrophy (DMD is an X-linked recessive disorder, characterized by progressive muscle weakness. Historically, pain has not been considered to be a major symptom in DMD. Objective To investigate the relationship between DMD and pain. Methods We conducted a systematic review in Medline/PubMed and BVS (virtual library in health databases. We searched for articles that showed the terms “Muscular Dystrophy, Duchenne” and “Pain” in all fields. All studies included boys diagnosed with DMD and the occurrence/amount of pain on this population. Results Initially, there were 175 studies. 167 articles were excluded for not meeting the inclusion criteria. The remaining eight eligible studies, involving pain assessment in DMD, were analyzed. Conclusion Pain is a frequent problem in this population and this symptom is potentially tractable. Studies conclude that pain can directly influence the quality of life of this population.

  17. Limb Girdle Muscular Dystrophy (LGMD): Case Report.

    Science.gov (United States)

    Kanitkar, Shubhangi A; Kalyan, Meenakshi; Gaikwad, Anu N; Makadia, Ankit; Shah, Harshad

    2015-01-01

    We report a young male of autosomal recessive limb girdle muscular dystrophy (LGMD) with positive family history presented with gradual onset proximal muscle weakness in all four limbs since eight years and thinning of shoulders, arms and thighs. Neurological examination revealed atrophy of both shoulders with wasting of both deltoids thinning of thighs and pseudo hypertrophy of both calves, hypotonia in all four limbs. Gower's sign was positive. Winging of scapula was present. Power was 3/5 at both shoulders, 4/5 at both elbows, 5/5 at both wrists, 3/5 at both hip joints, 3/5 at both knees, 5/5 at both ankles. All deep tendon reflexes and superficial reflexes were present with plantars bilateral flexors. Electromyography (EMG) showed myopathic pattern. He had elevated creatinine phosphokinase levels and muscle biopsy findings consistent with muscular dystrophy.

  18. Association between culture results of corneal scrapings and culture and histopathology results of corneal tissues in therapeutic keratoplasty.

    Science.gov (United States)

    Das, Sujata; Sharma, Savitri; Priyadarshini, Omega; Sahu, Srikant K; Kar, Sarita; Vemuganti, Geeta K

    2011-09-01

    To correlate the culture results of corneal scrapings with culture and histopathology results of corneal tissues in therapeutic keratoplasty. A retrospective analysis of the culture results of corneal scrapings and corneal tissues of eyes that received therapeutic penetrating keratoplasty at a tertiary eye care center between December 2006 and November 2008 was conducted. As per the preferred practice, those cases that did not respond to appropriate antimicrobial therapy and/or presented with a large infiltrate/perforation received therapeutic keratoplasty. The microbiology and histopathology findings of the corneal tissues were compared. Thirty-eight therapeutic keratoplasties were performed on 36 patients. Although all cases had histopathology and culture of the corneal tissue, corneal scrapings were not performed in 4 cases. Corneal scrapings and corneal tissues were culture-positive in 76% (26 of 34) and 60% (23 of 38) of cases, respectively. In 8 cases, the corneal scrapings and corneal tissues yielded identical organisms, whereas different organisms grew in 4 cases. In 6 cases, the corneal tissues were culture-positive but the corneal scrapings were sterile. In 20 cases, the corneal tissues were culture-positive for fungus and also showed fungal filaments in their corresponding histopathology specimens. Corneal tissue culture can provide additional information in cases undergoing therapeutic keratoplasty. It helps to determine the management of patients after keratoplasty.

  19. Rehabilitation therapy of Duchenne muscular dystrophy

    OpenAIRE

    ZHANG Cheng; YANG Juan

    2012-01-01

    It is very important that the rehabilitation therapy of Duchenne muscular dystrophy (DMD) can improve the quality of life and delay the disease progression. There are the guidelines for DMD rehabilitation therapy in some countries, but it is not emphasized by clinical doctors in our country. According to our experiences to DMD rehabilitation therapy, we reviewed the progress of DMD rehabilitation therapy. It includes the clinical stages and characteristics of DMD, the general principle and th...

  20. The burden of Duchenne muscular dystrophy

    OpenAIRE

    Landfeldt, Erik; Lindgren, Peter; Bell, Christopher F.; Schmitt, Claude; Guglieri, Michela; Straub, Volker; Lochmüller, Hanns; Bushby, Katharine

    2014-01-01

    Objective: The objective of this study was to estimate the total cost of illness and economic burden of Duchenne muscular dystrophy (DMD). Methods: Patients with DMD from Germany, Italy, United Kingdom, and United States were identified through Translational Research in Europe–Assessment & Treatment of Neuromuscular Diseases registries and invited to complete a questionnaire online together with a caregiver. Data on health care use, quality of life, work status, informal care, and household e...

  1. Urological manifestations of Duchenne muscular dystrophy.

    Science.gov (United States)

    Askeland, Eric J; Arlen, Angela M; Erickson, Bradley A; Mathews, Katherine D; Cooper, Christopher S

    2013-10-01

    Duchenne muscular dystrophy is a dystrophinopathy affecting males that is associated with multiple organ system complications. To our knowledge urological complications of Duchenne muscular dystrophy have been described only anecdotally to date. We reviewed the medical charts of 135 patients with Duchenne or Duchenne-Becker muscular dystrophy for demographics and disease progression, urological diagnoses, intervention and followup. Of 135 patients 67 (50%) had at least 1 documented urological diagnosis and 38 (28%) had multiple manifestations. Lower urinary tract symptoms were the most common urological diagnosis (32% of patients). Survival analysis revealed a median age at onset of lower urinary tract symptoms of 23 years (95% CI 17.7-23.9). Intervention was required in 12 patients (9%), most commonly due to nephrolithiasis. Urological morbidity increased with Duchenne muscular dystrophy progression when stratified by clinical progression. Lower urinary tract symptoms were more common in nonambulatory patients (40.7% vs 19%, p = 0.007), those with a diagnosis of scoliosis (44% vs 19.7%, p = 0.003) and/or scoliosis spine surgery (60% vs 22%, p <0.001), and those on invasive respiratory support (53% vs 29%, p = 0.046). Likewise, nephrolithiasis was more common in nonambulatory patients (10% vs 0%, p = 0.017), those with scoliosis (12% vs 0%, p = 0.004) and/or scoliosis spine surgery (20% vs 1%, p <0.001), and those on invasive respiratory support (29% vs 3%, p <0.001). Only 28% of patients with a urological manifestation were referred to urology. As these patients transition into adolescence and adulthood, the increased prevalence of urological manifestations warrants increased awareness and referral to urologists. Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  2. Nutrition Considerations in Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Davis, Jillian; Samuels, Emily; Mullins, Lucille

    2015-08-01

    Duchenne muscular dystrophy (DMD) is a serious degenerative muscular disease affecting males. Diagnosis usually occurs in childhood and is confirmed through genetic testing and/or muscle biopsy. Accompanying the disease are several nutrition-related concerns: growth, body composition, energy and protein requirements, constipation, swallowing difficulties, bone health, and complementary medicine. This review article addresses the nutrition aspects of DMD. © 2015 American Society for Parenteral and Enteral Nutrition.

  3. The keratoconus corneal proteome: loss of epithelial integrity and stromal degeneration.

    Science.gov (United States)

    Chaerkady, Raghothama; Shao, Hanjuan; Scott, Sherri-Gae; Pandey, Akhilesh; Jun, Albert S; Chakravarti, Shukti

    2013-07-11

    Keratoconus is a thinning corneal dystrophy that begins in the early teenage years and ultimately requires cornea transplantation to restore vision. Here we conducted a highly sensitive mass spectrometric analysis of the epithelium and the stroma from keratoconus and normal donor corneas. We identified a total of 932 and 1157 proteins in the consolidated data of the epithelium and stroma, respectively. Technical replicates showed strong correlations (≥0.88) in levels of all common proteins, indicating very low technical variations in the data. Analysis of the most increased (≥1.5 fold) and decreased (≤0.8 fold) proteins in the keratoconus corneal epithelial protein extracts identified proteins related to dermal diseases, inflammation, epithelial stratification and mesenchymal changes. Increased proteins included keratins 6A, 16 and vimentin, while the iron transporter lactotransferrin was decreased. The keratoconus stromal proteome suggests endoplasmic reticular stress, oxidative stress and widespread decreases in many extracellular matrix proteoglycan core proteins, lumican and keratocan, collagen types I, III, V and XII. Marked increase in apoptosis and endocytosis-related proteins suggest degenerative changes in keratocytes, the resident cells of the stroma. This is the most comprehensive proteome analysis of the cornea that highlights similarities of keratoconus with other neurodegenerative diseases. This study provides, to our knowledge, the most comprehensive proteomic analysis of the vision threatening disease keratoconus, which affects a significant portion of the US and global populations. Using iTRAQ and LC/MS/MS, we have identified significant changes in the human corneal epithelium and stromal proteome that correlate to in vivo clinical findings. The protein changes identified will lead to molecular insights into disease pathogenesis and provide candidate genes for genetic studies of keratoconus. Copyright © 2013 Elsevier B.V. All rights reserved.

  4. Corneal Graft Rejection Ten Years after Penetrating Keratoplasty in the Cornea Donor Study

    Science.gov (United States)

    Dunn, Steven P.; Gal, Robin L.; Kollman, Craig; Raghinaru, Dan; Dontchev, Mariya; Blanton, Christopher L.; Holland, Edward J; Lass, Jonathan H.; Kenyon, Kenneth R.; Mannis, Mark J; Mian, Shahzad I.; Rapuano, Christopher J.; Stark, Walter J.; Beck, Roy W.

    2015-01-01

    Purpose To assess the effect of donor and recipient factors on corneal allograft rejection and evaluate whether a rejection event was associated with graft failure. Methods 1,090 subjects undergoing penetrating keratoplasty for a moderate risk condition (principally Fuchs’ dystrophy or pseudophakic corneal edema) were followed for up to 12 years. Associations of baseline recipient and donor factors with the occurrence of a rejection event were assessed in univariate and multivariate proportional hazards models. Results Among 651 eyes with a surviving graft at 5 years, the 10-year graft failure (± 99% CI) rates were 12% ± 4% among eyes with no rejection events in the first 5 years, 17% ± 12% in eyes with at least one probable, but no definite rejection event, and 22% ± 20% in eyes with at least one definite rejection event. The only baseline factor significantly associated with a higher risk of definite graft rejection was a preoperative history of glaucoma, particularly when prior glaucoma surgery had been performed and glaucoma medications were being used at time of transplant (10-year incidence 35% ± 23% compared with 14% ± 4% in eyes with no history of glaucoma/intraocular pressure treatment, p=0.008). Conclusion Those patients who experienced a definite rejection event frequently went on to graft failure raising important questions as to how we might change acute and long-term corneal graft management. Multivariate analysis indicated that the prior use of glaucoma medications and glaucoma filtering surgery was a significant risk factor related to a definite rejection event. PMID:25119961

  5. Split cornea transplantation for 2 recipients: a new strategy to reduce corneal tissue cost and shortage.

    Science.gov (United States)

    Heindl, Ludwig M; Riss, Stephan; Bachmann, Bjoern O; Laaser, Kathrin; Kruse, Friedrich E; Cursiefen, Claus

    2011-02-01

    To evaluate the feasibility of using a single donor cornea for 2 recipients by combining deep anterior lamellar keratoplasty (DALK) and Descemet's membrane endothelial keratoplasty (DMEK) surgeries on the same day. Single-center, nonrandomized, prospective, interventional case series. Twelve consecutive donor corneas were scheduled for split cornea transplantation combining DALK for a keratoconus patient and DMEK for a Fuchs' endothelial dystrophy patient on the same surgery day. First, a big-bubble DALK procedure was performed for the keratoconus eye. When bare Descemet's membrane was prepared successfully requiring no conversion to penetrating keratoplasty (PK), then during surgery the donor, endothelium-Descemet's membrane layer was removed and stored for subsequent DMEK in a second patient, and the remaining anterior lamella of the donor cornea was used to complete the DALK surgery. Afterward, a DMEK procedure was performed on the second patient with Fuchs' endothelial dystrophy, grafting the stored endothelium-Descemet's membrane layer of the original donor button. Success of using a single donor cornea for 2 recipient eyes, best spectacle-corrected visual acuity (BSCVA), and complication rates within 6 months follow-up. A single donor cornea could be used for 2 recipients in 10 of 12 donor buttons (83%). In 2 cases (17%), the DALK procedure had to be converted to PK requiring a full-thickness corneal graft. Therefore, 10 donor corneas (45%) could be saved. Six months after surgery, mean BSCVA was 20/35 (range, 20/50-20/25) in 10 eyes that underwent successful DALK, 20/50 (range, 20/63-20/40) in 2 eyes that underwent conversion from DALK to PK, and 20/31 (range, 20/50-20/16) in 10 eyes that underwent DMEK. Postoperative complications after DALK included Descemet's folds in 3 eyes (30%) and epitheliopathy in 2 eyes (20%). After DMEK, partial graft detachment occurred in 5 eyes (50%) and was managed successfully with intracameral air reinjection. All corneas

  6. CONGENITAL MYOTONIC DYSTROPHY – CASE REPORT

    Directory of Open Access Journals (Sweden)

    David Neubauer

    2001-07-01

    Full Text Available Background. Myotonic dystrophy is inherited as an autosomal dominant trait. It is characterized by myotonia, myopathy of voluntary and involuntary muscles, frontal baldness in men, cardiac conduction abnormalities, catharacts, intellectual deterioration and endocrinopathy. Men with this disorder have often gonadal atrophy and infertility. On the other hand women are generally fertile. During pregnancy their myopathy worsens, often causing severe obstetrical complications. Their children may develop congenital form of the disease with signs of myopathy in utero and have great difficulties in maintaining life functions after birth, together with other characteristical signs of this form: bilateral facial weakness, severe hypotonia, feeding difficulties, talipes equinovarus and mental retardation. The authors present a female newborn with such congenital form of myotonic dystrophy.Conclusions. The authors have emphasized the importance of medical history, regular updating of all the cases of neuromuscular diseases in the region and clinical characteristics for the recognition of congenital form of myotonic dystrophy because of possible prenatal diagnostics and better antenatal and postantal care.

  7. Gene therapy for Duchenne muscular dystrophy.

    Science.gov (United States)

    Verhaart, Ingrid E C; Aartsma-Rus, Annemieke

    2012-10-01

    Duchenne muscular dystrophy is a severe neuromuscular disorder for which there is currently no cure. Years of research have come to fruition during the past 18 months with publications on clinical trials for several gene therapy approaches for Duchenne muscular dystrophy. This review covers the present status of these approaches. The exon skipping approach is most advanced in the process of clinical application. Encouraging results have been obtained in two systemic clinical trials and further optimization has increased delivery to the heart in animal models. Limitations of the approach are the mutation-specificity and the anticipated requirement for lifelong treatment. Gene therapy by means of gene transfer holds the promise of more long-lasting effects. Results of a first, early-stage gene therapy trial, using viral vectors to deliver a minidystrophin gene, were reported. Animal studies suggest that it may be possible to overcome the main challenges currently facing gene therapy (immunogenicity of the vector and systemic body-wide delivery). Significant steps have been made in the development of gene therapy approaches for Duchenne muscular dystrophy. These approaches aim to slow down disease progression, requiring robust outcome measures to assess efficacy.

  8. Muscle MRI findings in facioscapulohumeral muscular dystrophy.

    Science.gov (United States)

    Gerevini, Simonetta; Scarlato, Marina; Maggi, Lorenzo; Cava, Mariangela; Caliendo, Giandomenico; Pasanisi, Barbara; Falini, Andrea; Previtali, Stefano Carlo; Morandi, Lucia

    2016-03-01

    Facioscapulohumeral muscular dystrophy (FSHD) is characterized by extremely variable degrees of facial, scapular and lower limb muscle involvement. Clinical and genetic determination can be difficult, as molecular analysis is not always definitive, and other similar muscle disorders may have overlapping clinical manifestations. Whole-body muscle MRI examination for fat infiltration, atrophy and oedema was performed to identify specific patterns of muscle involvement in FSHD patients (30 subjects), and compared to a group of control patients (23) affected by other myopathies (NFSHD). In FSHD patients, we detected a specific pattern of muscle fatty replacement and atrophy, particularly in upper girdle muscles. The most frequently affected muscles, including paucisymptomatic and severely affected FSHD patients, were trapezius, teres major and serratus anterior. Moreover, asymmetric muscle involvement was significantly higher in FSHD as compared to NFSHD patients. In conclusion, muscle MRI is very sensitive for identifying a specific pattern of involvement in FSHD patients and in detecting selective muscle involvement of non-clinically testable muscles. Muscle MRI constitutes a reliable tool for differentiating FSHD from other muscular dystrophies to direct diagnostic molecular analysis, as well as to investigate FSHD natural history and follow-up of the disease. Muscle MRI identifies a specific pattern of muscle involvement in FSHD patients. Muscle MRI may predict FSHD in asymptomatic and severely affected patients. Muscle MRI of upper girdle better predicts FSHD. Muscle MRI may differentiate FSHD from other forms of muscular dystrophy. Muscle MRI may show the involvement of non-clinical testable muscles.

  9. Thrombomodulin promotes corneal epithelial wound healing

    National Research Council Canada - National Science Library

    Huang, Yi-Hsun; I, Ching-Chang; Kuo, Cheng-Hsiang; Hsu, Yun-Yan; Lee, Fang-Tzu; Shi, Guey-Yueh; Tseng, Sung-Huei; Wu, Hua-Lin

    2015-01-01

    To determine the role of thrombomodulin (TM) in corneal epithelial wound healing, and to investigate whether recombinant TM epidermal growth factor-like domain plus serine/threonine-rich domain (rTMD23...

  10. Femtosecond Lasers and Corneal Surgical Procedures.

    Science.gov (United States)

    Marino, Gustavo K; Santhiago, Marcony R; Wilson, Steven E

    2017-01-01

    Our purpose is to present a broad review about the principles, early history, evolution, applications, and complications of femtosecond lasers used in refractive and nonrefractive corneal surgical procedures. Femtosecond laser technology added not only safety, precision, and reproducibility to established corneal surgical procedures such as laser in situ keratomileusis (LASIK) and astigmatic keratotomy, but it also introduced new promising concepts such as the intrastromal lenticule procedures with refractive lenticule extraction (ReLEx). Over time, the refinements in laser optics and the overall design of femtosecond laser platforms led to it becoming an essential tool for corneal surgeons. In conclusion, femtosecond laser is a heavily utilized tool in refractive and nonrefractive corneal surgical procedures, and further technological advances are likely to expand its applications. Copyright 2017 Asia-Pacific Academy of Ophthalmology.

  11. Clear corneal incision in cataract surgery.

    Science.gov (United States)

    Al Mahmood, Ammar M; Al-Swailem, Samar A; Behrens, Ashley

    2014-01-01

    Since the introduction of sutureless clear corneal cataract incisions, the procedure has gained increasing popularity worldwide because it offers several advantages over the traditional sutured scleral tunnels and limbal incisions. Some of these benefits include lack of conjunctival trauma, less discomfort and bleeding, absence of suture-induced astigmatism, and faster visual rehabilitation. However, an increasing incidence of postoperative endophthalmitis after clear corneal cataract surgery has been reported. Different authors have shown a significant increase up to 15-fold in the incidence of endophthalmitis following clear corneal incision compared to scleral tunnels. The aim of this report is to review the advantages and disadvantages of clear corneal incisions in cataract surgery, emphasizing on wound construction recommendations based on published literature.

  12. Corneal biomechanical properties in thyroid eye disease

    National Research Council Canada - National Science Library

    Karabulut, Gamze Ozturk; Kaynak, Pelin; Altan, Cıgdem; Ozturker, Can; Aksoy, Ebru Funda; Demirok, Ahmet; Yılmaz, Omer Faruk

    2014-01-01

    The purpose of this study is to investigate the effect of thyroid eye disease (TED) on the measurement of corneal biomechanical properties and the relationship between these parameters and disease manifestations...

  13. Conjunctival intraepithelial neoplasia with corneal furrow degeneration

    Directory of Open Access Journals (Sweden)

    Pukhraj Rishi

    2014-01-01

    Full Text Available A 68-year-old man presented with redness of left eye since six months. Examination revealed bilateral corneal furrow degeneration. Left eye lesion was suggestive of conjunctival squamous cell carcinoma, encroaching on to cornea. Anterior segment optical coherence tomography (AS-OCT confirmed peripheral corneal thinning. Fluorescein angiography confirmed intrinsic vascularity of lesion. Patient was managed with "no touch" surgical excision, dry keratectomy without alcohol, cryotherapy, and primary closure. Pathologic examination of removed tissue confirmed clinical diagnosis. Management of this particular case required modification of standard treatment protocol. Unlike the alcohol-assisted technique of tumor dissection described, ethyl alcohol was not used for risk of corneal perforation due to underlying peripheral corneal thinning. Likewise, topical steroids were withheld in the post-operative period. Three weeks post-operatively, left eye was healing well. Hence, per-operative usage of absolute alcohol and post-operative use of topical steroids may be best avoided in such eyes.

  14. Surgical compensation of presbyopia with corneal inlays.

    Science.gov (United States)

    Konstantopoulos, Aris; Mehta, Jodhbir S

    2015-05-01

    Presbyopia, the physiological change in near vision that develops with ageing, gradually affects individuals older than 40 years and is a growing cause of visual disability due to ageing demographics of the global population. The routine use of computers and 'smartphones', combined with the affluence of the 'baby boomers' generation has set high standards for near vision correction. Corneal inlays are a relatively new treatment modality that is effective at compensating for presbyopia. The dimensions of these devices vary from 2 to 3.8 mm in diameter and 5 to 32 μm in thickness. They are implanted in the anterior corneal stroma of the non-dominant eye, most commonly, in a femtosecond laser created corneal pocket. They improve near vision by increasing the depth of focus, creating a hyper-prolate region of increased central cornea power or providing a refractive add power. This article reviews the literature on the efficacy and safety of corneal inlays.

  15. [Nanostructured bioplastic material for traumatic corneal injuries].

    Science.gov (United States)

    Kanyukov, V N; Stadnikov, A A; Trubina, O M; Yakhina, O M

    2015-01-01

    To substantiate the use of nanostructured bioplastic material for the treatment of traumatic eye injuries. The study enrolled 96 eyes of 48 rabbits and was carried out in 3 series of experiments, different in the type of induced corneal trauma: mechanical erosion, alkaline or acid burn. The animals were clinically monitored and sacrificed for morphological investigation at days 3, 7, 14, 30, and 90. The size of mechanical corneal erosions was repeatedly evaluated with fluorescein eye stain test. In the experimental group, Hyamatrix biomaterial was topically administered according to an original technique. In the controls, soft contact lenses were inserted and sutured. Complete closure of the epithelial defect with no impact on corneal properties was achieved in 3 days in the experimental group and in4 days in the control group. As for alkaline and acid corneal burns, experimental and control groups received Hyamatrix biomaterial and Solcoseryl eye gel correspondingly. In the experimental group of alkaline burn the defect closed by day 7, in the controls--by day 10-11. Acid-induced corneal edema also resolved by day 7 in the experimental group and by day 14 in the control group. 1. The results of this experimental and morphological study prove the hyaluronic acid-derived nanostructured bioplastic material effective in accelerating corneal re-epithelialization after mechanical erosions as compared with the controls. 2. Topical application of the hyaluronic acid-derived nanostructured bioplastic material shortens the exudative phase of inflammation, promotes corneal defect closure with formation of a more subtle opacification, and stimulates corneal restoration after chemical burns.

  16. IOL Power Calculation after Corneal Refractive Surgery

    OpenAIRE

    Maddalena De Bernardo; Luigi Capasso; Luisa Caliendo; Francesco Paolercio; Nicola Rosa

    2014-01-01

    Purpose. To describe the different formulas that try to overcome the problem of calculating the intraocular lens (IOL) power in patients that underwent corneal refractive surgery (CRS). Methods. A Pubmed literature search review of all published articles, on keyword associated with IOL power calculation and corneal refractive surgery, as well as the reference lists of retrieved articles, was performed. Results. A total of 33 peer reviewed articles dealing with methods that try to overcome the...

  17. Corneal collagen crosslinking for keratoconus. A review

    OpenAIRE

    M. M. Bikbov; V. K. Surkova

    2014-01-01

    Photochemical crosslinking is widely applied in ophthalmology. Its biochemical effect is due to the release of singlet oxygen that promotes anaerobic photochemical reaction. Keratoconus is one of the most common corneal ectasia affecting 1 in 250 to 250 000 persons. Currently, the rate of iatrogenic ectasia following eximer laser refractive surgery increases due to biomechanical weakening of the cornea. Morphologically and biochemically, ectasia is characterized by corneal layers thinning, co...

  18. Corneal biomechanical properties of patients with acromegaly.

    Science.gov (United States)

    Ozkok, Ahmet; Hatipoglu, Esra; Tamcelik, Nevbahar; Balta, Burcu; Gundogdu, Ahmet Sadi; Ozdamar, Mehmet Akif; Kadioglu, Pinar

    2014-05-01

    Growth hormone (GH) and insulin-like growth factor-1 (IGF-1) excess in acromegaly have various effects on many organs. The ophthalmologic effects of GH and IGF-1 excess have not yet been investigated in detail. The aim of the current study is to compare the corneal biomechanical properties of patients with acromegaly and those of healthy subjects. 45 patients with acromegaly (F/M=27/18) and 42 age-matched and gender-matched healthy individuals (F/M=24/18) were enrolled in this cross-sectional study. Central corneal thickness (CCT), corneal resistance factor (CRF), corneal hysteresis (CH), corneal compensated intraocular pressure (IOPcc) and Goldmann correlated IOPG were measured in patients with acromegaly and in healthy individuals using the Ocular Response Analyser (ORA). GH and IGF1 values were also determined in the study group. The mean CH and CRF values were higher in acromegalic patients (12.1±2.2 and 12.3±2.4, respectively) than in healthy subjects (11.0±1.6 and 10.8±1.5, respectively; for CH, p=0.014; for CRF, p=0.001). Mean IOPG measurement was higher in the acromegaly group than in the control group (p=0.017). There was no statistically significant difference in measured CCT (p=0.117) and IOPcc (p=0.594) values between acromegalic patients and healthy subjects. These findings indicate that acromegaly has target organ effects on the eye. Consequently, it can change corneal biomechanical properties such as corneal hysteresis and the CRF. Corneal biomechanical properties are known to affect the accuracy of IOP measurements. These findings should be taken into account when measuring IOP values in acromegaly patients, as IOP readings may be overestimated.

  19. Corneal Biomechanics Determination in Healthy Myopic Subjects

    Directory of Open Access Journals (Sweden)

    Kunliang Qiu

    2016-01-01

    Full Text Available Purpose. To determine the corneal biomechanical properties by using the Ocular Response Analyzer™ and to investigate potential factors associated with the corneal biomechanics in healthy myopic subjects. Methods. 135 eyes from 135 healthy myopic subjects were included in this cross-sectional observational study. Cornea hysteresis (CH, corneal resistance factor (CRF, cornea-compensated intraocular pressure (IOPcc, and Goldmann-correlated intraocular pressure (IOPg were determined with the Reichert Ocular Response Analyzer (ORA. Univariate and multivariate regression analyses were performed to investigate factors associated with corneal biomechanics. Results. The mean CH and CRF were 9.82±1.34 mmHg and 9.64±1.57 mmHg, respectively. In univariate regression analysis, CH was significantly correlated with axial length, refraction, central corneal thickness (CCT, and IOPg (r=-0.27, 0.23, 0.45, and 0.21, resp.; all with p≤0.015, but not with corneal curvature or age; CRF was significantly correlated with CCT and IOPg (r=0.52 and 0.70, resp.; all with p<0.001, but not with axial length/refraction, corneal curvature, or age. In multivariate regression analysis, axial length, IOPcc, and CCT were found to be independently associated with CH, while CCT and IOPg were associated with CRF. Conclusions. Both CH and CRF were positively correlated with CCT. Lower CH but not CRF was associated with increasing degree of myopia. Evaluation of corneal biomechanical properties should take CCT and myopic status into consideration.

  20. Corneal hysteresis in mucopolysaccharidosis I and VI.

    Science.gov (United States)

    Fahnehjelm, Kristina Teär; Chen, Enping; Winiarski, Jacek

    2012-08-01

    High intraocular pressure (IOP) and glaucoma are often suspected in patients with mucopolysaccharidosis (MPS). To determine corneal hysteresis (CH) and IOP in children with mucopolysaccharidosis I-Hurler (MPS I-H) and MPS VI. Clinical measurements with ocular response analyzer (ORA). In seven patients, five with MPS I-H treated with stem cell transplantation (SCT), and two with MPS VI, one treated with SCT and the other with enzyme therapy, the IOP was examined with ORA. Ocular response analyzer measurements were made at a median age of 8.7 years in the patients with MPS I-H and at a median age of 9.3 years in the patients with MPS VI. Earlier measurements had raised suspicion of high IOP in one patient. The ORA showed an increased CH and a falsely high IOP values in all 14 eyes. The recalculated IOPs were normal in all 14 eyes. Mild to severe corneal opacities were present in all 14 eyes. Optic disc areas, borders and cupping were clinically normal in the 12 of 14 eyes that were possible to examine. Severe corneal opacities hampered optic disc evaluation in the older patient with MPS VI. Three eyes in two patients had normal thickness of the retinal nerve fibre layer measured with scanning laser polarimetry with corneal compensation (GDx VCC). No patient was diagnosed or treated for glaucoma. The IOPs are often falsely high because of an increased resistance of the cornea and correlate to the extent of corneal clouding. In this small, cross-sectional study, it appears that corneal resistance is directly correlated with corneal clouding, although a longitudinal study that evaluates resistance as the cornea clears with treatment would provide more direct evidence that corneal deposits are directly related to resistance. A correct measured IOP can avoid unnecessary medical or surgical hypotensive treatment. © 2011 The Authors. Acta Ophthalmologica © 2011 Acta Ophthalmologica Scandinavica Foundation.

  1. Corneal topography and soft contact lens fit.

    Science.gov (United States)

    Young, Graeme; Schnider, Cristina; Hunt, Chris; Efron, Suzanne

    2010-05-01

    To determine which ocular topography variables affect soft contact lens fit. Fifty subjects each wore three pairs of soft lenses in random succession (Vistakon Acuvue 2, Vistakon Acuvue Advance, Ciba Vision Night & Day), and various aspects of lens fit were evaluated. The steeper base curves of each type were worn in one eye and the flatter base curves in the other eye. Corneal topography data were collected using a Medmont E300 corneal topographer (Camberwell, Australia). Corneal curvature, shape factor (SF), and corneal height were measured over a 10 mm chord and also over the maximum measurable diameter. These were measured in the horizontal, vertical, steepest, and flattest meridians. With each lens type, the steeper base curve provided the best fit on the greatest proportion of eyes and the significant differences in various aspects of fit were noted between base curves. For each lens type, there was no significant difference in mean K-reading between those eyes best fit with the steeper base curve and those eyes best fit with the flatter base curve. Two of the lenses showed a positive correlation between centration and horizontal corneal height (maximum), whereas one lens showed a negative correlation between centration and horizontal SF (SF = e). Several lenses showed a positive correlation between post-blink movement and horizontal or vertical corneal SF. The measurement of corneal topography using current Placido disc instrumentation allows a better prediction of soft lens fit than by keratometry, but it is not reliable enough to enable accurate selection of the best fitting base curve. Some correlations are evident between corneal measurements; however, trial fitting remains the method of choice for selection of soft lens base curve.

  2. Corneal Collagen Cross-Linking Outcomes: Review

    OpenAIRE

    Jankov II,Mirko R.; Jovanovic,Vesna; Delevic, Sladjana; Coskunseven, Efekan

    2011-01-01

    Keratoconus is a condition characterized by biomechanical instability of the cornea, presenting in a progressive, asymmetric and bilateral way. Corneal collagen cross-linking with riboflavin and UVA (CXL) is a new technique of corneal tissue strengthening that combines the use of riboflavin as a photo sensitizer and UVA irradiation. The studies showed that CXL was effective in halting the progression of keratoconus over a period of up to four years. The published studies also revealed a reduc...

  3. Effects of axial length and corneal curvature on corneal biomechanics in elderly population

    Directory of Open Access Journals (Sweden)

    Sha-Sha Song

    2018-02-01

    Full Text Available AIM:To explore the corneal biomechanical properties of the elderly with different axial length(ALand corneal curvature by corneal visualization Scheimpflug Technology(Corvis ST. METHODS: Cross-sectional study. A total of 161 patients(297 eyesundergoing phacoemulsification were collected in this study. They were divided into 22-24mm, 24-26mm, more than 26mm groups according to axial length(190 eyes, 54 eyes and 53 eyes, respectively. Those of whom axial length was 22-24mm and the corneal curvature was 42-44D were divided into male and female groups(44 eyes and 49 eyes, respectively. Those of whom axial length was 22-24mm were divided into 42-44D group, more than 44D group according to corneal curvature(88 eyes, 102 eyes, respectively. Corvis ST was used to measure the biomechanical parameters of the cornea. The differences in the parameters between different groups were analyzed using the independent-samples t test or one-way analysis of variance and correlation analyses were performed using Pearson correlation analysis. RESULTS: When comparing the corneal biomechanical parameters, no statistically significant differences were found between male and female groups(P>0.05. The first applanation length and second applanation length among different corneal curvatures were statistically significant(PPr=0.429, 0.278; Pr=-0.291, -0.415; PCONCLUSION: The corneal curvature and ocular axial length may be the factors affecting the corneal biomechanical characteristics. The longer axial length, the thinner corneal thickness, the more easily the corneal is deformed, and with the increase of the axial length, intraocular pressure also increases.

  4. Intracorneal Ring Segments Implantation for Corneal Ectasia.

    Science.gov (United States)

    Giacomin, Natalia T; Mello, Glauco R; Medeiros, Carla S; Kiliç, Alyin; Serpe, Cristine C; Almeida, Hirlana G; Kara-Junior, Newton; Santhiago, Marcony R

    2016-12-01

    To provide an overview of the predictability, safety, and efficacy of intrastromal corneal ring segment (ICRS) implantation as a tool to improve visual acuity and its association with other techniques such as corneal collagen cross-linking (CXL), addressing biomechanical outcomes, models, surgical planning and technique, indications, contraindications, and complications in ectatic corneas. Literature review. ICRSs have been used to regularize the corneal shape and reduce corneal astigmatism and higher order aberrations, improve visual acuity to acceptable limits, and delay, or eventually prevent, a corneal keratoplasty in keratoconic eyes. Changes in ICRS thickness and size, combination of techniques, and the addition of femtosecond lasers to dissect more foreseeable channels represent an improvement toward more predictable results. Several studies have shown, over time, the long-term efficacy and safety of ICRS treatment for keratoconus, with variable predictability, maintaining the early satisfactory outcomes regarding visual acuity, keratometry, and corneal thickness. It is just as important to ensure that the disease will not progress as it is to improve the visual acuity. Therefore, many studies have shown combined techniques using ICRS implantation and CXL. Also, further limitations of ICRS implantation can be addressed when associated with phakic intraocular lens implantation and photorefractive keratectomy. ICRS implantation has shown effectiveness and safety in most cases, including combined procedures. In properly selected eyes, it can improve both refraction and vision in patients with keratoconus. [J Refract Surg. 2016;32(12):829-839.]. Copyright 2016, SLACK Incorporated.

  5. Surgery for scoliosis in Duchenne muscular dystrophy.

    Science.gov (United States)

    Cheuk, Daniel K L; Wong, Virginia; Wraige, Elizabeth; Baxter, Peter; Cole, Ashley

    2013-02-28

    Scoliosis in people with Duchenne muscular dystrophy is usually progressive and treated with surgery. However, it is unclear whether the existing evidence is sufficiently scientifically rigorous to support a recommendation for spinal surgery for most people with Duchenne muscular dystrophy and scoliosis. This is an updated review and an updated search was undertaken in which no new studies were found. To determine the effectiveness and safety of spinal surgery in people with Duchenne muscular dystrophy with scoliosis. We intended to test whether spinal surgery is effective in increasing survival, improving respiratory function, improving quality of life and overall functioning; and whether spinal surgery is associated with severe adverse effects. We searched the specialized registers of the Cochrane Neuromuscular Disease Group (31 July 2012), MEDLINE (January 1966 to July 2012), EMBASE (January 1947 to July 2012), CENTRAL (2012, Issue 7 in the Cochrane Library), CINAHL Plus(January 1937 to July 2012), Proquest Dissertation and Thesis Database (January 1980 to July 2012), and the National Institute of Health Clinical Trials Database (July 2012). No language restrictions were imposed. We planned to include controlled clinical trials using random or quasi-random allocation of treatment evaluating all forms of spinal surgery for scoliosis in people with Duchenne muscular dystrophy in the review. The control interventions would have been no treatment, non-operative treatment, or a different form of spinal surgery. Two authors independently examined the search results and evaluated the study characteristics against inclusion criteria to decide which ones would be included in the review. On searching, 47 studies were relevant but none met the inclusion criteria for the review, because they were not clinical trials but prospective or retrospective reviews of case series. Since there were no randomized controlled clinical trials available to evaluate the effectiveness of

  6. Duchenne and Becker muscular dystrophy prevalence in South ...

    African Journals Online (AJOL)

    1993-07-28

    Jul 28, 1993 ... A genetic service for Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) was initiated in. Cape Town in 1987. Of the 143 DMD patients diagnosed during the period 1987-1992, 66 had a familial pattern of inheritance and 77 were apparently sporadic. Twenw BMD patients were ...

  7. Limb girdle muscular dystrophy: reappraisal of a rejected entity

    NARCIS (Netherlands)

    van der Kooi, A. J.; de Visser, M.; Barth, P. G.

    1994-01-01

    The term limb girdle muscular dystrophy (LGMD) has been introduced to delineate a distinct form of muscular dystrophy with predominantly proximal upper and lower extremity weakness. Families with evidence of both autosomal recessive and autosomal dominant modes of inheritance have been described.

  8. A unifying genetic model for facioscapulohumeral muscular dystrophy.

    NARCIS (Netherlands)

    Lemmers, R.J.; Vliet, P.J.C. Van; Klooster, R.; Sacconi, S.; Camano, P.; Dauwerse, J.G.; Snider, L.; Straasheijm, K.R.; Ommen, G.J.B. van; Padberg, G.W.A.M.; Miller, D.G.; Tapscott, S.J.; Tawil, R.; Frants, R.R.; Maarel, S.M. van der

    2010-01-01

    Facioscapulohumeral muscular dystrophy (FSHD) is a common form of muscular dystrophy in adults that is foremost characterized by progressive wasting of muscles in the upper body. FSHD is associated with contraction of D4Z4 macrosatellite repeats on chromosome 4q35, but this contraction is pathogenic

  9. Dysphagia is present but mild in myotonic dystrophy type 2

    NARCIS (Netherlands)

    S. Knuijt; R. Ensink; J. van Vliet; A. Tieleman; Bert de Swart; Baziel van Engelen

    2009-01-01

    The phenotype of myotonic dystrophy type 2 (DM2) shows similarities as well as differences to that of myotonic dystrophy type 1 (DM1). Dysphagia, a predominant feature in DM1, has not yet been examined in DM2. In a recent nationwide questionnaire survey of gastrointestinal symptoms in DM2, 12 out of

  10. Resistance training in patients with limb-girdle and becker muscular dystrophies

    DEFF Research Database (Denmark)

    Sveen, Marie-Louise; Andersen, Søren P; Ingelsrud, Lina H

    2013-01-01

    In this study we investigated the effect of strength training in patients with limb-girdle muscular dystrophy (LGMD) and Becker muscular dystrophy (BMD).......In this study we investigated the effect of strength training in patients with limb-girdle muscular dystrophy (LGMD) and Becker muscular dystrophy (BMD)....

  11. Relationship among Corneal Biomechanics, Anterior Segment Parameters, and Geometric Corneal Parameters

    Directory of Open Access Journals (Sweden)

    Sadık Görkem Çevik

    2016-01-01

    Full Text Available Purpose. To investigate the relationship between corneal biomechanical parameters, anterior segment parameters, and geometric corneal parameters in a healthy Caucasian group. Methods. This retrospective study included the healthy eyes with best corrected visual acuity of at least 20/40 of 122 Caucasian subjects. The anterior segment parameters and geometric corneal parameters such as corneal volume, central corneal thickness, horizontal and vertical corneal radii, anterior and posterior steep, and flat keratometric values were measured with a Scheimpflug camera. The biomechanical properties were measured with Ocular Response Analyzer. Results. One hundred and twenty-two healthy Caucasian subjects (67 males, 55 females with a mean age of 45.32±20.23 were enrolled. Both corneal hysteresis and corneal resistance factor were positively correlated with CCT (r=0.529, p<0.001; r=0.638, p<0.001 and CV (r=0.635, p<0.001; r=0.579, p<0.001 and negatively correlated with age (r=-0.373, p<0.001; r=-0.249, p<0.001. Both in age-gender and multivariate models, CH and CRF had statistically significant negative association with the posterior steep K value. Conclusions. CH and CRF are negatively correlated with posterior steep and average posterior K values.

  12. The scale of substratum topographic features modulates proliferation of corneal epithelial cells and corneal fibroblasts.

    Science.gov (United States)

    Liliensiek, S J; Campbell, S; Nealey, P F; Murphy, C J

    2006-10-01

    The cornea is a complex tissue composed of different cell types, including corneal epithelial cells and keratocytes. Each of these cell types are directly exposed to rich nanoscale topography from the basement membrane or surrounding extracellular matrix. Nanoscale topography has been shown to influence cell behaviors, including orientation, alignment, differentiation, migration, and proliferation. We investigated whether proliferation of SV40-transformed human corneal epithelial cells (SV40-HCECs), primary human corneal epithelial cells (HCECs), and primary corneal fibroblasts is influenced by the scale of topographic features of the substratum. Using basement membrane feature sizes as our guide and the known dimensions of collagen fibrils of the corneal stroma (20-60 nm), we fabricated polyurethane molded substrates, which contain anisotropic feature sizes ranging from 200-2000 nm on pitches ranging from 400 to 4000 nm (pitch = ridge width + groove width). The planar regions separating each of the six patterned regions served as control surfaces. Primary corneal and SV40-HCEC proliferation decreased in direct response to decreasing nanoscale topographies down to 200 nm. In contrast to corneal epithelial cells, corneal fibroblasts did not exhibit significantly different response to any of the topographies when compared with planar controls at 5 days. However, decreased proliferation was observed on the smallest feature sizes after 14 days in culture. Results from these experiments are relevant in understanding the potential mechanisms involved in the control of proliferation and differentiation of cells within the cornea. (c) 2006 Wiley Periodicals, Inc

  13. [The influence of corneal hysteresis and corneal resistance factor on the measurement of intraocular pressure].

    Science.gov (United States)

    Hager, A; Schroeder, B; Sadeghi, M; Grossherr, M; Wiegand, W

    2007-06-01

    The influence of central corneal thickness (CCT) on the measurement of intraocular pressure (IOP) has been discussed extensively in recent years. The problem, however, has not been solved so far. In addition to CCT there are probably further biomechanical properties that play a role in IOP measurement. We wanted to find out whether these properties are related to Goldmann applanation tonometry (GAT), noncontact tonometry (NCT), or CCT. Biomechanical properties of the cornea such as corneal hysteresis (CH) and corneal resistance factor (CRF) can be measured with the Ocular Response Analyzer (ORA, Reichert Ophthalmic Instruments, Depew, NY, USA). Furthermore, a corneal compensated IOP (IOPcc) is given. We examined 156 normal eyes of 80 patients who did not show corneal pathology nor glaucoma. In each eye GAT, NCT, and ORA data as well as CCT were measured. Data were statistically analyzed with respect to agreement and the influence of CH and CRF on IOP measurement. In our patients the following average values were calculated: GAT 14.8+/-3.0 mmHg, NCT 16.4+/-3.9 mmHg, IOPcc 16.2+/-4.1 mmHg, CH 10.6+/-2.3 mmHg, CRF 10.9+/-2.4 mmHg, and CCT 557+/-36 microm. IOPcc was not related to CCT in normal eyes and the only IOP value related to CH (pcorneal thickness. Corneal hysteresis and corneal resistance factor provide further information about biomechanical properties of the cornea beyond central corneal thickness.

  14. Corneal Thickness During Corneal Collagen Cross-Linking with Isotonic Riboflavin Solution without Dextran

    Directory of Open Access Journals (Sweden)

    Refik Oltulu

    2014-08-01

    Full Text Available Objectives: To monitor the corneal thickness change during the dextran-free isotonic riboflavin solution-aided corneal collagen crosslinking procedure in keratoconus patients. Materials and Methods: Corneal thickness measurements during the corneal collagen cross-linking (CXL treatment for progressive keratoconus were evaluated. The corneal thickness measurements were obtained with ultrasonic pachymetry device at five different time points: 0, 15, and 30 minutes after epithelial removal and 15 and 30 minutes after the initiation of UVA irradiation. Results: Twenty-four eyes of 24 patients with progressive keratoconus were included in the study. The thinnest pachymetric values obtained at the 0, 15, and 30 minute measurements after corneal deepithelisation were 409.38±10.43 µm (383-435 µm, 434.56±17.68 µm (400-485 µm, and 457.44±21.78 µm (428-516 µm, respectively. Pachymetric values obtained at 15 and 30 minutes after UVA application to the cornea were 471.69±23.38 µm (439-526 µm and 482.63±23.69 µm (436-524 µm, respectively. The gradual increase was found to be statistically significant when each measurement was compared with the previous values (p<0.001. Conclusion: We found that the corneal thickness was not decreased during the CXL with dextran-free isotonic riboflavin solution; on the contrary, corneal thickness was increased regularly during the procedure. (Turk J Ophthalmol 2014; 44: 272-4

  15. Spatial and temporal variations in extracellular matrix of periocular and corneal regions during corneal stromal development.

    Science.gov (United States)

    Doane, K J; Ting, W H; McLaughlin, J S; Birk, D E

    1996-03-01

    The development of the avian corneal stroma occurs in discrete developmental stages. During this sequence of events, the neural crest-derived corneal fibroblast precursor cells are surrounded by distinct extracellular matrices which change both spatially and temporally. To elucidate the role of these matrices, extracellular matrix components in the periocular mesenchyme and cornea were analysed prior to and during migration and differentiation of corneal fibroblasts using antibodies against collagens, proteoglycans and glycoproteins. Previous work has concentrated on the matrix of the corneal stroma rather than the matrix of the periocular mesenchyme. Since the precursors of the corneal fibroblasts are present within the must migrate through the periocular mesenchyme prior to entry into the cornea proper, this environment was fully evaluated. The present study documents the matrix composition of both the cornea and periocular mesenchyme at developmental stages that are prior to and after initiation of corneal invasion by the corneal fibroblast precursors. Variations in matrix molecules comprising both the periocular mesenchyme and cornea were demonstrated. These include changes in the distribution of collagen types I, II, III, IV and VI; the proteoglycans decorin and lumican; as well as the adhesive glycoproteins tenascin, fibronectin and laminin. It is hypothesized that the variations in matrix localization are important in the regulation of cell migration and differentiation during normal corneal development. Any regulation is likely to involve a combination of components found in the extracellular matrices and therefore, a consideration of the matrix rather than isolated components is required.

  16. [Study of vitreoretinal dystrophies in a Mexican population].

    Science.gov (United States)

    Orozco-Gómez, Luis Porfirio; Castellanos-Pérez Bolde, Carmen Guadalupe; Moguel-Ancheita, Silvia; Lambarri-Arroyo, Andrés

    2008-01-01

    We undertook this study to demonstrate the incidence of vitreoretinal dystrophies in a Mexican population. This was a retrospective, observational, descriptive, transverse study. We analyzed the files of patients treated at the Retina Department of a medical center for state employees (ISSSTE) from January 1991 to December 2006 to obtain the incidence of vitreoretinal dystrophies. We studied 36,300 patient files. We found an incidence of 0.008% for familial exudative vitreoretinal dystrophy, 0.008% for X-linked juvenile retinoschisis, 0.005% for Wagner disease and 0.005% for Goldmann-Favre disease. We present here a representative case of each type of dystrophy. Vitreoretinal dystrophies are uncommon diseases and are difficult to diagnose. Even though their incidence is low, the poor evolution to blindness requires identification of early signs in order to offer timely and opportune treatment.

  17. Genetic modifiers of Duchenne and facioscapulohumeral muscular dystrophies.

    Science.gov (United States)

    Hightower, Rylie M; Alexander, Matthew S

    2018-01-01

    Muscular dystrophy is defined as the progressive wasting of skeletal muscles that is caused by inherited or spontaneous genetic mutations. Next-generation sequencing has greatly improved the accuracy and speed of diagnosis for different types of muscular dystrophy. Advancements in depth of coverage, convenience, and overall reduced cost have led to the identification of genetic modifiers that are responsible for phenotypic variability in affected patients. These genetic modifiers have been postulated to explain key differences in disease phenotypes, including age of loss of ambulation, steroid responsiveness, and the presence or absence of cardiac defects in patients with the same form of muscular dystrophy. This review highlights recent findings on genetic modifiers of Duchenne and facioscapulohumeral muscular dystrophies based on animal and clinical studies. These genetic modifiers hold great promise to be developed into novel therapeutic targets for the treatment of muscular dystrophies. Muscle Nerve 57: 6-15, 2018. © 2017 Wiley Periodicals, Inc.

  18. Cardiac involvement in patients with limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy

    DEFF Research Database (Denmark)

    Sveen, Marie-Louise; Thune, Jens Jakob; Køber, Lars

    2008-01-01

    OBJECTIVE: To investigate the extent of cardiac involvement in patients with 1 of the 12 groups of recessively inherited limb-girdle muscular dystrophy type 2 (LGMD2A-L) and Becker muscular dystrophy (BMD). DESIGN: Prospective screening. SETTING: Neuromuscular Clinic and Department of Cardiology ...... of dystrophic changes on muscle biopsy. CONCLUSIONS: This study demonstrates a high prevalence of cardiac involvement in patients with LGMD2I, LGMD2E, and BMD. Patients with LGMD2A, LGMD2D, and unclassified LGMD2 have a much lower and milder prevalence of cardiac involvement.......OBJECTIVE: To investigate the extent of cardiac involvement in patients with 1 of the 12 groups of recessively inherited limb-girdle muscular dystrophy type 2 (LGMD2A-L) and Becker muscular dystrophy (BMD). DESIGN: Prospective screening. SETTING: Neuromuscular Clinic and Department of Cardiology......-I and in 14 of 30 patients (47%) with BMD. Only a few patients with LGMD2A and unclassified LGMD2 had mild cardiac involvement, whereas 29% and 67% of patients with LGMD2I and LGMD2E, respectively, had cardiac involvement. Cardiac involvement was not correlated with age, muscle strength, or the level...

  19. Psychiatric disorders appear equally in patients with myotonic dystrophy, facioscapulohumeral dystrophy, and hereditary motor and sensory neuropathy type I.

    NARCIS (Netherlands)

    Kalkman, J.S.; Schillings, M.L.; Zwarts, M.J.; Engelen, B.G.M. van; Bleijenberg, G.

    2007-01-01

    OBJECTIVES: To study the presence of psychiatric comorbidity assessed by the use of a structured clinical interview and self-reported questionnaires in a large sample of patients with adult-onset myotonic dystrophy (DM), facioscapulohumeral muscular dystrophy (FSHD), and hereditary motor and sensory

  20. Signs and symptoms of Duchenne muscular dystrophy and Becker muscular dystrophy among carriers in the Netherlands : a cohort study

    NARCIS (Netherlands)

    Hoogerwaard, EM; Bakker, E; Ippel, PF; Oosterwijk, JC; Majoor-Krakauer, DF; Leschot, NJ; Van Essen, AJ; Brunner, HG; van der Wouw, PA; Wilde, AAM; de Visser, Marianne

    1999-01-01

    Background Carriers of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) may show muscle weakness or dilated cardiomyopathy. Studies focusing on skeletal-muscle involvement were done before DNA analysis was possible. We undertook a cross-sectional study in a population of

  1. Signs and symptoms of Duchenne muscular dystrophy and Becker muscular dystrophy among carriers in The Netherlands: a cohort study

    NARCIS (Netherlands)

    Hoogerwaard, E. M.; Bakker, E.; Ippel, P. F.; Oosterwijk, J. C.; Majoor-Krakauer, D. F.; Leschot, N. J.; van Essen, A. J.; Brunner, H. G.; van der Wouw, P. A.; Wilde, A. A.; de Visser, M.

    1999-01-01

    BACKGROUND: Carriers of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) may show muscle weakness or dilated cardiomyopathy. Studies focusing on skeletal-muscle involvement were done before DNA analysis was possible. We undertook a cross-sectional study in a population of

  2. The corneal volume and biomechanical corneal factors: Is there any orrelation?

    Directory of Open Access Journals (Sweden)

    Mohammad Reza Sedaghat

    2012-01-01

    Full Text Available Background: The aim of this study was to determine the correlation between corneal hysteresis (CH and the corneal resistance factor (CRF, which are both novel methods of analyzing ocular rigidity/elasticity, and various corneal cha-racteristics, mainly corneal volume in normal subjects. Methods: This cross-sectional study included 500 normal eyes of volunteers. An ocular response analyzer (ORA was used to measure CH and CRF. Patient age and the Pentacam-measured corneal volume (CV, posterior elevation, ante-rior elevation, corneal curvature, central corneal thickness (CCT, corneal thickness of apex (CTA, and corneal thinnest thickness (CTT were compared with CH and CRF. Statistical significance was defined at p < 0.05. Results: The mean CH and CRF for all eyes were 9.9 ± 1.4 mmHg and 10.1 ± 1.6 mmHg, respectively. The mean CVs of the 3, 5, 7, and 10 mm zones for all eyes were 3.8 ± 0.2 mm3, 11.2 ± 0.6 mm3, 24.3 ± 1.4 mm3, and 60.1 ± 3.5 mm3, respectively. The correlations between CV and the hysteresis or CRF were significant in all zones. The CV of the 7-mm zone had the strongest correlation with CH (r = 0.438 and the CV of the 5-mm zone had the strongest correlation with CRF (r = 0.574. Conclusions: CH and CRF correlate with CV. Moreover, the correlation between CV and CRF is stronger than that between CV and CH. The CV may be valuable for determining patient′s qualification for and predicting the outcome of refractive surgery. It would also be helpful in other cases in which corneal biomechanics are important.

  3. Reliability of the Effect of Artificial Anterior Chamber Pressure and Corneal Drying on Corneal Graft Thickness.

    Science.gov (United States)

    Romano, Vito; Steger, Bernhard; Chen, Jern Y; Hassaan, Sherif; Batterbury, Mark; Willoughby, Colin E; Ahmad, Sajjad; Elsheikh, Ahmed; Kaye, Stephen B

    2015-08-01

    To investigate the effect of artificial anterior chamber (AAC) pressure and corneal drying on the graft thickness in preparation for Descemet stripping automated endothelial keratoplasty. Twenty-seven corneoscleral discs were placed in an AAC. The AAC pressure (15, 45, 92, 109, and 198 mm Hg) was controlled using the height of an infusion bottle and a roller clamp. The endothelium was removed in 1 subgroup. Corneas were exposed to room air or repeatedly dried using cellulose spears. Central corneal thickness was measured every 90 seconds for the first 15 minutes and again at 20 minutes using an ultrasound pachymeter (SP-100, Tomey). There was a significant linear relationship between the corneal thickness and both AAC pressure and corneal drying. Very high coefficients of determination and narrow 95% confidence intervals were present, in particular for high pressures and drying. The rate of thinning increased with increasing pressure and drying to 1.6% per minute. At the maximum rate of thinning, a 10% reduction in corneal thickness occurred in 6 minutes or 100 μm in 8.8 minutes. Removal of the corneal endothelium reduced the rate of thinning to 0.3% per minute (R = 0.72). Increasing AAC pressure and corneal drying reduced the graft thickness at a very predictable rate. Adequate corneal thinning can be achieved by increasing the pressure in the AAC by closing the clamp followed by removal of the residual corneal epithelium and repeated drying with a cellulose spear for 5 to 10 minutes, depending on the initial corneal thickness. This method is simple and is both suitable for use in the eye bank and by the surgeon.

  4. Human tears reveal insights into corneal neovascularization.

    Science.gov (United States)

    Zakaria, Nadia; Van Grasdorff, Sigi; Wouters, Kristien; Rozema, Jos; Koppen, Carina; Lion, Eva; Cools, Nathalie; Berneman, Zwi; Tassignon, Marie-José

    2012-01-01

    Corneal neovascularization results from the encroachment of blood vessels from the surrounding conjunctiva onto the normally avascular cornea. The aim of this study is to identify factors in human tears that are involved in development and/or maintenance of corneal neovascularization in humans. This could allow development of diagnostic tools for monitoring corneal neovascularization and combination monoclonal antibody therapies for its treatment. In an observational case-control study we enrolled a total of 12 patients with corneal neovascularization and 10 healthy volunteers. Basal tears along with reflex tears from the inferior fornix, superior fornix and using a corneal bath were collected along with blood serum samples. From all patients, ocular surface photographs were taken. Concentrations of the pro-angiogenic cytokines interleukin (IL)-6, IL-8, Vascular Endothelial Growth Factor (VEGF), Monocyte Chemoattractant Protein 1 (MCP-1) and Fas Ligand (FasL) were determined in blood and tear samples using a flow cytometric multiplex assay. Our results show that the concentration of pro-angiogenic cytokines in human tears are significantly higher compared to their concentrations in serum, with highest levels found in basal tears. Interestingly, we could detect a significantly higher concentration of IL- 6, IL-8 and VEGF in localized corneal tears of patients with neovascularized corneas when compared to the control group. This is the first study of its kind demonstrating a significant difference of defined factors in tears from patients with neovascularized corneas as compared to healthy controls. These results provide the basis for future research using animal models to further substantiate the role of these cytokines in the establishment and maintenance of corneal neovascularization.

  5. Development of a rabbit corneal equivalent using an acellular corneal matrix of a porcine substrate.

    Science.gov (United States)

    Xu, Yong-Gen; Xu, Yong-Sheng; Huang, Chen; Feng, Yun; Li, Ying; Wang, Wei

    2008-01-01

    The tissue equivalent that mimics the structure and function of normal tissue is a major bioengineering challenge. Tissue engineered replacement of diseased or damaged tissue has become a reality for some types of tissue such as skin and cartilage. The tissue engineered corneal epithelium, stroma, and endothelium scaffold are promising concepts in overcoming the current limitations of a cornea replacement with an allograft. The acellular corneal matrix from porcine (ACMP) was examined as a potential corneal cell sheet frame. The physical and mechanical properties of strength, expansion, transparency, and water content of the ACMP were measured. The major antigens of the cell components were completely removed with series of extraction methods, the major antigens of the cell components were identified by hematoxylin and eosin (HE), immunofluorescence staining, and scanning electron microscopy. The structural properties were investigated by HE stain and scanning electron microscopy. The three types of rabbit corneal cells were cultured in vitro, and characteristics were investigated by colony formation efficiency (CFE), BrdU staining, immunofluorescence staining, and western blot assay of keratin 3 (K3), vimentin, and aquaporin A. The biocompatibility of the ACMP was investigated for one month using rabbit corneal stroma and three types of cultured corneal cells both in vivo and in vitro. The three types of cultured rabbit corneal cells were seeded onto ACMP of each side at a cell density of 5.0 x 10(3) cells/mm(2). The optical and mechanical properties of the ACMP were similar to the normal porcine cornea. The collagen fiber interconnected to the network, formed regular collagen bundles of the ACMP, and was parallel to the corneal surface. The ACMP was transferred to the rabbit cornea stroma, which showed an intact epithelium and keratocytes in the implant region. There were no inflamed cells or new vessel invasion one month after transplantation. The three types of

  6. Corneal biomechanical properties and intraocular pressure in high myopic anisometropia.

    Science.gov (United States)

    Xu, Suzhong; Xu, Aiqin; Tao, Aizhu; Wang, Jianhua; Fan, Fan; Lu, Fan

    2010-07-01

    To investigate corneal biomechanical properties and intraocular pressure (IOP) in patients with high myopic anisometropia. Patients with high myopic anisometropia (n = 23) and emmetropic subjects (n = 55) were enrolled. Corneal hysteresis (CH), Goldmann-correlated intraocular pressure (IOPg), corneal resistance factor, and corneal-compensated IOP (IOPcc) were measured with Ocular Response Analyzer. Central corneal thickness was measured by optical coherence tomography. Zeiss IOL-Master determined the values of corneal refractive power and ocular axial length. Significant differences were presented in CH, IOPg, and IOPcc among the high myopic, contralateral, and normal eyes (analysis of variance, Panisometropia. High myopic eyes showed decreased CH, but not corneal resistance factor, which indicates that some aspects of corneal biomechanical properties may be altered in high myopic eye of anisometropia. It is also suggested that anisometropic eyes with different refractive errors do not share the same biomechanical properties, which may impact IOP measurement.

  7. Changes on the corneal thickness and curvature after orthokeratology

    Science.gov (United States)

    Mitsui, Iwane; Yamada, Yoshiya

    2004-07-01

    To evaluate the corneal thickness and curvature changes after Orthokeratology contact lens wear, using the ORBSCAN II corneal topography system, corneal thickness and corneal curvature were measured on one hundred and twenty eyes of sixty patients before and after wearing the custom rigid gas permeable contact lenses for Orthokeratology. The contact lenses were specially designed for each eye. The subjects wore the orthokeratology lenses for approximately Four hours with their eyes closed. The corneal thickness of the subjects was increased on fifty-five eyes at not only the peripheral zone but also the center of the cornea. The average increase of central and peripheral corneal thickness was 18 micrometer and 22micrometer, respectively. The mean anterior curvature of corneal surface changed 1.25D. The mean posterior curvature of corneal endothelium side changed 0.75D.

  8. [Use of the confocal laser scanning method for determining corneal topography and corneal tissue effects in refractive corneal surgery].

    Science.gov (United States)

    Koop, N; Brinkmann, R; Schirner, G

    1996-06-01

    Refraction of the cornea head been generally measured with ophthalmometers or computer disk keratometers. We therefore used a confocal laser scanning system for measurement of the corneal topography. Enucleated tonicized pig eyes were measured before and after laser thermokeratoplasty (LTK). The topographical data were used to determine refraction and refractive change; the data were stored digitally. The single images and their differences were displayed on a PC. Unlike conventional ophthalmometry, confocal laser scanning can demonstrate the topographical shape, showing the overall topography of the cornea and local corneal effects, e.g., coagulation, mechanical lesions or high-energy laser effects. Topographical laser scanning has proven to be a generally useful method of determining refraction and surface alterations in corneal refractive surgery.

  9. Reflex sympathetic dystrophy and cigarette smoking.

    Science.gov (United States)

    An, H S; Hawthorne, K B; Jackson, W T

    1988-05-01

    Although the cause of reflex sympathetic dystrophy (RSD) remains unknown, hyperactivity of the sympathetic nerves and secondary vasospasm may be pathogenic in this syndrome. A retrospective epidemiologic study of RSD was done on 53 in-patients from 1978-1985. Cigarette smoking was strikingly increased in patient frequency in RSD (68% versus 37% of hospitalized controls, p less than 0.0001). Eighty-seven percent of the patients had a history of trauma or surgery, and 38% had other associated diseases. Cigarette smoking is statistically linked to RSD and may be involved in its pathogenesis by enhancing sympathetic activity, vasoconstriction, or by some other unknown mechanism.

  10. A molecular protocol for diagnosing myotonic dystrophy.

    Science.gov (United States)

    Guida, M; Marger, R S; Papp, A C; Snyder, P J; Sedra, M S; Kissel, J T; Mendell, J R; Prior, T W

    1995-01-01

    Myotonic dystrophy (DM) is an autosomal dominant genetic disease caused by an unstable CTG repeat sequence in the 3' untranslated region of the myotonin protein kinase gene. The CTG repeat is present 5-30 times in the normal population, whereas DM patients have CTG expansions of 50 to several thousand repeats. The age of onset of the disorder and the severity of the phenotype is roughly correlated with the size of the CTG expansion. We developed a molecular protocol for the diagnosis of DM based on an initial polymerase chain reaction screen to detect normal-sized alleles and small expansions, followed by an improved Southern protocol to detect larger expansions.

  11. [Facioscapulohumeral muscle dystrophy and heart disease].

    Science.gov (United States)

    Emmrich, P; Ogunlade, V; Gradistanac, T; Daneschnejad, S; Koch, M C; Schober, R

    2005-05-01

    Cardiac involvement is well known in a number of skeletomuscular diseases but not in facio-scapulohumeral muscular dystrophy (FSHD). We report on a 71 year old woman with progressive cardiac insufficiency in FSHD, which was also confirmed by molecular analysis in one of the two daughters affected by the disease. Autopsy of the deceased patient showed the typical changes in skeletal muscles including focal inflammatory infiltrates in the diaphragm and, in addition, cardiac muscular involvement. The histological changes resembled those seen in primary cardiomyopathy despite the normal muscle mass volume. Both clinically and morphologically, the cardiac disease was the cause of death in this patient with FSHD.

  12. Rehabilitation therapy of Duchenne muscular dystrophy

    Directory of Open Access Journals (Sweden)

    ZHANG Cheng

    2012-06-01

    Full Text Available It is very important that the rehabilitation therapy of Duchenne muscular dystrophy (DMD can improve the quality of life and delay the disease progression. There are the guidelines for DMD rehabilitation therapy in some countries, but it is not emphasized by clinical doctors in our country. According to our experiences to DMD rehabilitation therapy, we reviewed the progress of DMD rehabilitation therapy. It includes the clinical stages and characteristics of DMD, the general principle and the common therapy methods of DMD rehabilitation. We hope this review may increase recognizing to DMD rehabilitation therapy for the clinical doctors and DMD family members.

  13. Corneal reconstruction by stem cells and bioengineering

    Directory of Open Access Journals (Sweden)

    Arjamaa O

    2012-09-01

    Full Text Available Olli ArjamaaDepartment of Biology, University of Turku, Turku, FinlandAbstract: Almost 300 million people are visually impaired worldwide due to various eye diseases such as cataracts, glaucoma, age-related macular degeneration, diabetic retinopathy, and corneal diseases. Notably, ten million people are blind because of severe ocular surface diseases and the majority of cases occur in developing countries. Blinding ocular surface diseases have, however, become treatable by grafting of surface layers, or by full-thickness transplantation of the cornea. As the demand for human corneal tissue for surface reconstruction and transplantation far exceeds the supply, methods are being developed to supplement tissue donation. Xenotransplantation of the cornea or cells from genetically modified pigs may become one of the solutions. Transplantation of limbal stem cells within tissue biopsies, to restore the transparency of the cornea is another remarkable method, which has shown its potential in several clinical studies. The combination of stem cell technology and engineering of biocompatible tissue equivalent, still at preclinical stage, has shown us how synthetic corneal tissue is able to guide cultured corneal stromal stem cells of human origin, to become native-like stroma, the most important layer of the cornea. These findings give hope for a large-quantity production of biomaterial for corneal reconstruction. As such, clinical ophthalmologists should become more familiar with the methods of laboratory science.Keywords: eye, grafting, keratoplasty, xenotransplantation, cell reservoir, biocompatible tissue equivalent

  14. Corneal collagen cross-linking outcomes: review.

    Science.gov (United States)

    Jankov Ii, Mirko R; Jovanovic, Vesna; Delevic, Sladjana; Coskunseven, Efekan

    2011-02-11

    Keratoconus is a condition characterized by biomechanical instability of the cornea, presenting in a progressive, asymmetric and bilateral way. Corneal collagen cross-linking with riboflavin and UVA (CXL) is a new technique of corneal tissue strengthening that combines the use of riboflavin as a photo sensitizer and UVA irradiation. The studies showed that CXL was effective in halting the progression of keratoconus over a period of up to four years. The published studies also revealed a reduction of max K readings by more than 2 D, while the postoperative SEQ was reduced by an average of more than 1 D, and refractive cylinder decreased by about 1 D. No eyes lost any line of BCDVA. Moreover, there was no significant decrease in endothelial cell density. It was also found that CXL treatment was effective with reducing corneal and total wavefront aberrations. Corneal cross-linking has also led to an arrest and/or even a partial reversal of keratectasia in the treatment of iatrogenic ectasia after excimer laser ablation. A primary intervention such as CXL should be considered to potentially increase the biomechanical stability of the corneal tissue and postpone the need of lamellar or penetrating keratoplasty.

  15. Trends in corneal transplantation: indications and techniques.

    Science.gov (United States)

    Ple-Plakon, Patricia A; Shtein, Roni M

    2014-07-01

    To describe trends in corneal transplantation surgery, including indications for surgery, evolution of lamellar keratoplasty, current surgical techniques, and future directions. Over the past decade, anterior and posterior lamellar keratoplasty have begun to supplant penetrating keratoplasty. Surgical techniques continue to change and improve outcomes. In recent years, Descemet membrane endothelial keratoplasty (DMEK) has gained interest as it eliminates the corneal stromal interface, which may limit visual acuity after Descemet stripping automated endothelial keratoplasty. Despite the promising results with improved visual acuity and decreased rejection, the technical challenges associated with DMEK have limited widespread acceptance. With technical refinements and more eye banks providing precut tissue for both Descemet stripping automated endothelial keratoplasty and DMEK, it is likely both procedures will continue to increase over time. Corneal transplantation has evolved rapidly over the past decade, from full-thickness penetrating keratoplasty towards lamellar keratoplasty to only remove and replace damaged layers of the cornea. Achieving minimal induced astigmatism with excellent visual acuity remains a challenge in corneal transplantation. Further refinements in surgical technique may help improve technical challenges and visual outcomes. In this article, we review changing trends in corneal transplantation and highlight developing medical treatments that may be available in the future.

  16. Corneal transplantation: A new view of life.

    Science.gov (United States)

    Amiri, Fardin; Ghiyasvandian, Shahrzad; Navab, Elham; Zakerimoghadam, Masoumeh

    2017-04-01

    The consequences of a corneal transplant are evaluated and classified by care providers, but understanding and interpretation of the results varies between patients, and creates different views for them and influences their lives in different ways while these influences are largely unknown. This study aimed to explore understanding of new life in patients after corneal transplantation. This qualitative study was conducted using a hermeneutic phenomenological approach in Tehran in 2016. Twelve corneal transplant recipients (7 men, 5 women) who were chosen purposefully from penetrating corneal transplant recipients, participated in this study. Semi-structured interviews were used to collect data. The content of the interviews was transcribed and analyzed using Van Manen's methodology. Data analysis led to the emergence of several main themes, among which "having a new sense" and "fear and hope" were two of the most important themes. It can be inferred from the overall participants' experiences that corneal transplant has brought about a new look at life for patients. However, transplant-related issues are endless and continuous, and a sense of fear and hope has always surrounded them.

  17. MicroRNA-184 Regulates Corneal Lymphangiogenesis.

    Science.gov (United States)

    Grimaldo, Sammy; Yuen, Don; Theis, Jaci; Ng, Melissa; Ecoiffier, Tatiana; Chen, Lu

    2015-11-01

    MicroRNAs are a class of small noncoding RNAs that negatively regulate gene expression by binding to complimentary sequences of target messenger RNA. Their roles in corneal lymphangiogenesis are largely unknown. This study was to investigate the specific role of microRNA-184 (mir-184) in corneal lymphangiogenesis (LG) in vivo and lymphatic endothelial cells (LECs) in vitro. Standard murine suture placement model was used to study the expressional change of mir-184 in corneal inflammatory LG and the effect of synthetic mir-184 mimic on this process. Additionally, a human LEC culture system was used to assess the effect of mir-184 overexpression on cell functions in vitro. Expression of mir-184 was significantly downregulated in corneal LG and, accordingly, its synthetic mimic suppressed corneal lymphatic growth in vivo. Furthermore, mir-184 overexpression in LECs inhibited their functions of adhesion, migration, and tube formation in vitro. These novel findings indicate that mir-184 is involved critically in LG and potentially could be used as an inhibitor of the process. Further investigation holds the promise for divulging new therapies for LG disorders, which occur inside and outside the eye.

  18. In-vitro corneal transparency measuring system

    Science.gov (United States)

    Ventura, Liliane; da Costa Vieira, Marcelo A.; Isaac, Flavio; Chiaradia, Caio; Faria de Sousa, Sidney J.

    2001-06-01

    A system for measuring the average corneal transparency of preserved corneas has been developed in order to provide a more accurate and standard report of the corneal tissue. The donated cornea transparency is one of the features to be analyzed previously to its indication for the transplant. The small portable system consists of two main parts: the optical and the electronic parts. The optical system consists of a white light, lenses and pin-holes that collimate white light beams that illuminates the cornea in its preservative medium. The light that passes through the cornea is detected by a resistive detector and the average corneal transparency is shown in a display. In order to obtain just the tissue transparency, the electronic circuit was built in a way that there is a baseline input of the preservative medium, previous to the measurement of the corneal transparency. Manipulating the system consists of three steps: (1) Adjusting the zero percentage in the absence of light (at this time the detectors in the dark); (2) Placing the preservative medium in the system and adjusting the 100% value (this is the baseline input); (3) Preserving the cornea and placing it in the system. The system provides the tissue transparency. The system is connected to an endothelium evaluation system for Slit Lamp, that we have developed, and statistics about the relationship of the corneal transparency and density of the endothelial cells will be provided in the next years. The system is being used in a public Eye Bank in Brasil.

  19. Riboflavin for corneal cross-linking.

    Science.gov (United States)

    O'Brart, D P S

    2016-06-01

    Corneal collagen cross-linking (CXL) with riboflavin and ultraviolet A (UVA) radiation is the first therapeutic modality that appears to arrest the progression of keratoconus and other corneal ectasias. Riboflavin is central to the process, acting as a photosensitizer for the production of oxygen singlets and riboflavin triplets. These free radicals drive the CXL process within the proteins of the corneal stroma, altering its biomechanical properties. Riboflavin also absorbs the majority of the UVA radiation, which is potentially cytotoxic and mutagenic, within the anterior stroma, preventing damage to internal ocular structures, such as the corneal endothelium, lens and retina. Clinical studies report cessation of ectatic progression in over 90% of cases and the majority document significant improvements in visual, keratometric and topographic parameters. Clinical follow-up is limited to 5-10 years, but suggests sustained stability and enhancement in corneal shape. Sight-threatening complications are rare. The optimal stromal riboflavin dosage for CXL is as yet undetermined. Copyright 2016 Prous Science, S.A.U. or its licensors. All rights reserved.

  20. Primary central corneal hemangiosarcoma in a dog.

    Science.gov (United States)

    Haeussler, David J; Rodríguez, Laura Muñoz; Wilkie, David A; Premanandan, Chris

    2011-03-01

    To report a case of primary central corneal hemangiosarcoma in the dog. An 11-year-old, neutered, female, German shepherd mixed breed dog was referred to the Hospital Veterinario Sierra de Madrid (Spain) for evaluation of an enlarging corneal mass of the left eye (OS). The dog was predominantly housed outdoors and was diagnosed with a history of chronic superficial keratitis of both eyes (OU) by the referring veterinarian. The corneal mass was resected by routine superficial keratectomy and submitted for histopathology and Factor VIII immunohistochemical staining. The mass was diagnosed as a corneal hemangiosarcoma with complete excision. Postoperatively, the keratectomy site healed without complication and there was no evidence of recurrence three and a half months postoperatively. Complete systemic evaluation, including abdominal ultrasound and CT scan of the head and thorax, indicated no other detectable neoplasia in the dog. Outdoor housing and ultraviolet exposure, breed, and chronic superficial keratitis were all suspected as contributing factors to the development of a primary corneal hemangiosarcoma. Surgical removal and postoperative treatment for chronic superficial keratitis provided effective therapy. © 2011 American College of Veterinary Ophthalmologists.

  1. Corneal Biomechanics in Ectatic Diseases: Refractive Surgery Implications

    OpenAIRE

    Ambr?sio, Jr, Renato; Correia, Fernando Faria; Lopes,Bernardo; Salom?o, Marcella Q.; Luz,Allan; Daniel G Dawson; Elsheikh, Ahmed; Vinciguerra, Riccardo; Vinciguerra, Paolo; Roberts, Cynthia J.

    2017-01-01

    Background: Ectasia development occurs due to a chronic corneal biomechanical decompensation or weakness, resulting in stromal thinning and corneal protrusion. This leads to corneal steepening, increase in astigmatism, and irregularity. In corneal refractive surgery, the detection of mild forms of ectasia pre-operatively is essential to avoid post-operative progressive ectasia, which also depends on the impact of the procedure on the cornea. Method: The advent of 3D tomography is proven as a ...

  2. The value of corneal transplantation in reducing blindness.

    Science.gov (United States)

    Garg, P; Krishna, P V; Stratis, A K; Gopinathan, U

    2005-10-01

    To analyse the role of keratoplasty in reducing world blindness due to corneal diseases. Review of published literature. We collected and analysed articles published in the English language literature related to the prevalence and causes of blindness in different parts of the world, causes of corneal blindness, and outcome of corneal transplantation for various corneal diseases. A total of 80% of the world's blind live in developing countries. Retinal diseases are the most important causes of blindness (40-54%) in established economy nations while cataract (44-60%) and corneal diseases (8-25%) are the most common causes of blindness in countries with less developed economies. Keratitis during childhood, trauma, and keratitis during adulthood resulting in a vascularized corneal scar and adherent leucoma are the most frequent causes of corneal blindness in developing countries. Corneal diseases are responsible for 20% of childhood blindness. Nearly 80% of all corneal blindness is avoidable. The outcome of keratoplasty for vascularized corneal scar and adherent leucoma is unsatisfactory, necessitating repeat surgery in a high proportion of these cases. Other barriers for keratoplasty in these nations are suboptimal eye banking, lack of trained human resources, and infrastructure. Since the developing world carries most of the load of corneal blindness and the major causes of corneal blindness are corneal scar and active keratitis, development of corneal transplantation services need a comprehensive approach encompassing medical standards in eye banking, training of cornea specialists and eye banking personnel and exposure of ophthalmologists to care of corneal transplants for better follow-up care. However, concerted efforts should be made to develop and implement prevention strategies since most corneal blindness is preventable.

  3. Corneal hysteresis, corneal resistance factor, and intraocular pressure measurements in eyes implanted with a small aperture corneal inlay.

    Science.gov (United States)

    Agca, Alper; Demirok, Ahmet; Celik, Haci Ugur; van de Pol, Corina; Cankaya, Kadir Ilker; Celik, Nimet Burcu; Yasa, Dilek; Yilmaz, Ihsan; Yilmaz, Omer Faruk

    2014-12-01

    To compare the postoperative corneal hysteresis (CH) and corneal resistance factor (CRF) of eyes implanted with a small aperture corneal inlay versus fellow eyes. Medical records of patients who underwent small aperture corneal inlay (KAMRA; AcuFocus, Inc., Irvine, CA) implantation were retrospectively reviewed. There were two groups: the implanted and non-implanted. Main outcome measures were CH, CRF, Goldmann-correlated intraocular pressure (IOPg), corneal-compensated IOP (IOPcc), and Goldmann applanation tonometry measurements performed preoperatively and at postoperative week 1 and months 1, 3, and 6. The study included 68 eyes of 34 patients. CH was higher in the implanted group when compared with the non-implanted group at postoperative week 1 (12.2 ± 3.1 vs 10.9 ± 1.7 mm Hg; P = .007) and month 1 (12.3 ± 2.5 vs 10.9 ± 1.8 mm Hg; P = .001). CRF was higher in the implanted group when compared with the non-implanted group at postoperative week 1 (11.9 ± 2.9 vs 10.7 ± 1.6 mm Hg; P = .003) and month 1 (12.5 ± 2.5 vs 10.4 ± 1.8 mm Hg; P .05). At the 3-month postoperative visit, all parameters had returned to baseline and there was no change at the 6-month visit. Implantation of the KAMRA corneal inlay does not induce a permanent change in CH or CRF. A transient increase in both was seen in the early postoperative period. Copyright 2014, SLACK Incorporated.

  4. What does the future hold for the treatment of Fuchs endothelial dystrophy; will ‘keratoplasty' still be a valid procedure?

    Science.gov (United States)

    Bruinsma, M; Tong, C M; Melles, G R J

    2013-01-01

    Fuchs endothelial corneal dystrophy (FECD) is a well recognized corneal disorder characterized by the presence of collagenous warts extending from Descemet membrane (guttae) and endothelial cellular dysfunction due to cell loss and/or degeneration. Because of the characteristic abnormal cell morphology as seen with specular microscopy as well as the limited regenerative capacity in vivo, the endothelial cells were considered to be ‘dystrophic'. Hence, FECD is commonly managed by replacement of the endothelium with donor tissue by means of a penetrating or endothelial keratoplasty. The latter procedure has now been refined to the isolated transplantation of a donor Descemet membrane and its endothelium, referred to as Descemet membrane endothelial keratoplasty (DMEK). Unexpectedly, clinical observation made after DMEK seemed to challenge the current concept of the state of the endothelium in FECD; we actually observed an important role for the ‘dystrophic' host endothelium in re-endothelialization of the denuded DM, and subsequent corneal clearance. In addition, recent studies regarding the pathophysiology of FECD made us realize that the endothelial cells are not ‘dystrophic' per se, but in the course of time may have acquired a dysfunction instead. This paper describes the rationale behind this new concept and based on this, discusses the possibilities for future, less invasive treatment modalities for FECD. PMID:23846374

  5. Corneal Stroma Regeneration with Acellular Corneal Stroma Sheets and Keratocytes in a Rabbit Model.

    Science.gov (United States)

    Ma, Xiao Yun; Zhang, Yun; Zhu, Dan; Lu, Yang; Zhou, Guangdong; Liu, Wei; Cao, Yilin; Zhang, Wen Jie

    2015-01-01

    Acellular corneal stroma matrix has been used for corneal stroma engineering. However, because of its compact tissue structure, regrowth of keratocytes into the scaffold is difficult. Previously, we developed a sandwich model for cartilage engineering using acellular cartilage sheets. In the present study, we tested this model for corneal stroma regeneration using acellular porcine corneal stroma (APCS) sheets and keratocytes. Porcine corneas were decellularized by NaCl treatment, and the APCS was cut into 20-μm-thick sheets. A rabbit corneal stroma defect model was created by lamellar keratoplasty and repaired by transplantation of five pieces of APCS sheets with keratocytes. Six months after transplantation, transparent corneas were present in the experimental group, which were confirmed by anterior segment optical coherence tomography examination and transmittance examination. The biomechanical properties in the experimental group were similar to those of normal cornea. Histological analyses showed an even distribution of keratocytes and well-oriented matrix in the stroma layer in the experimental group. Together, these results demonstrated that the sandwich model using acellular corneal stroma sheets and keratocytes could be potentially useful for corneal stroma regeneration.

  6. Value of recombinant human epidermal growth factor in corneal wound repair after corneal foreign body elimination

    Directory of Open Access Journals (Sweden)

    Hong-Jie Han

    2013-11-01

    Full Text Available AIM: To investigate the repair efficacy of recombinant human epidermal growth factor on corneal epithelium after corneal foreign body eliminating operation. METHODS: There were 102 patients with corneal foreign body(188 affected eyeschosen for the study. All patients were divided into treatment group and control group according to the random number table. Both groups received corneal foreign body elimination by slit lamp. Postoperatively, the treatment group was given eye drops containing epidermal growth factor(JinYinShucombined with tobramycin while the control group was only administrated with tobramycin. Treatment effects were compared 3d after treatment. RESULTS: Three days after treatment, the cure rate in the treatment group(93.7%, was significantly higher than that in the control group(76.6%(PPCONCLUSION: The recombinant human epidermal growth factor is capable of integrating with corneal epithelial cells and endothelial cell receptor, shortening healing time of corneal epithelial wound, thus making it an effective treatment of traumatic corneal epithelial defect.

  7. Randomized Trial Comparing Amniotic Membrane Transplantation with Lamellar Corneal Graft for the Treatment of Corneal Thinning.

    Science.gov (United States)

    de Farias, Charles C; Allemann, Norma; Gomes, José Á P

    2016-04-01

    There are few studies comparing different surgical procedures for the treatment of corneal thinning. Lamellar corneal transplantation (LCT) has been reported to be efficient, but its results can be jeopardized by allograft rejection, opacification, or high astigmatism. Amniotic membrane transplantation (AMT) has been considered a good alternative, but it is not as resistant as LCT and the tissue can be reabsorbed after surgery. A prospective, randomized, interventional, and comparative study of consecutive patients with corneal thinning over 6 months was performed. Ophthalmological examination was performed before transplant surgery and then repeated 1, 7, 15, 30, 90, and 180 days after surgery and ultrasound biomicroscopy was performed before and then 30, 90, and 180 days after surgery to assess corneal thinning. Herpes simplex infection was the main cause of corneal thinning (9 eyes), followed by surgery (cataract, glaucoma, 5 cases), rheumatoid arthritis (1), chemical burn (1), perforating trauma (1), previous band keratopathy treatment (1), and Stevens-Johnson syndrome (1). Although all patients showed significant increase in final thickness in the area of thinning, it was higher in those submitted to LCT at 180 days postoperatively. Regardless of the surgical technique, all patients showed epithelialization. Patients undergoing AMT showed an 89% decrease in neovascularization. Final corrected distance visual acuity was better in patients submitted to AMT. LCT proved to be the best option for treating corneal thinning. AMT represents an alternative that allows good visual recovery but does not restore corneal thickness as efficiently as LCT.

  8. [Evaluation of corneal biomechanical properties in glaucoma and control patients by dynamic Scheimpflug corneal imaging technology].

    Science.gov (United States)

    Coste, V; Schweitzer, C; Paya, C; Touboul, D; Korobelnik, J-F

    2015-06-01

    To compare corneal biomechanical properties measured with Corvis Scheimpflug technology (Corvis ST) between a group of patients with chronic open-angle glaucoma and a group of control patients. Prospective observational case-control study. This study enrolled 56 right eyes of 56 patients (G1 [chronic open-angle glaucoma] n=37/G2 [control] n=19). Each patient underwent measurement of corneal biomechanical properties by dynamic Scheimpflug (Corvis ST) camera and the Ocular Response Analyser (ORA), then a measurement of intraocular pressure (IOP) by Goldmann applanation tonometry (GAT) and measurement of central corneal thickness (CCT) by optical coherence tomography during the same visit, by a single clinician. The parameters determined by Corvis ST are: Corvis IOP (IOP Corvis ST), the corneal deformation amplitude (CDA), corneal velocity, the time at highest concavity (TIME CONCAV), the lengths of applanation and their corresponding applanation time. Those studied by ORA are: compensated IOP (IOPcc), non-compensated IOL (IOPg), corneal hysteresis (CH) and corneal resistance factor (CRF). IOP measured on all patients by Corvis ST was positively correlated to GAT (Spearman r=0.569, Pcorneal biomechanical properties between glaucoma and control patients. The cornea of glaucoma patients appears less deformable. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  9. Relationship Between Corneal Hysteresis and Corneal Resistance Factor with Other Ocular Parameters.

    Science.gov (United States)

    Rosa, Nicola; Lanza, Michele; De Bernardo, Maddalena; Signoriello, Giuseppe; Chiodini, Paolo

    2015-01-01

    To evaluate the relationship between corneal hysteresis (CH) and corneal resistance factor (CRF) with age, central corneal thickness (CCT), corneal curvature (KM), corneal volume (CV), and refractive error in naïve eyes. 105 healthy subjects (58 male and 47 female) were included in this study. The ages ranged from 19 to 82 years (mean 43.1 ± 15.4 years) and refraction between -11 D and +6 D (mean -0.79 ± 2.95 D). CH and CRF obtained with the Ocular Response Analyzer (ORA) were correlated with age, refractive error, Goldmann Applanation Tonometry (GAT), and with CCT, KM, CV obtained with the Pentacam, and with Corneal-Compensated Intraocular Pressure (IOPcc) and Goldmann-correlated intraocular pressure measurement (IOPg) obtained with ORA. A multivariable mixed effect model was used to evaluate associations among these parameters. CH ranged from 6.9 to 14.6 mmHg (mean 10.26 ± 1.49 mmHg); CRF ranged from 5.8 to 17 mmHg (mean 10.38 ± 1.64 mmHg). Multivariate analysis showed a statistically significant correlation between CH with CCT (p corneal shape and thickness, and show a decrease of CH with age.

  10. Clinical significance of central corneal thickness and comparison of central corneal thickness

    Directory of Open Access Journals (Sweden)

    Özgür Çakıcı

    2014-03-01

    Full Text Available Significance of central corneal thickness has been increasing in ophthalmology practice. It is an important clinical evaluation tool especially prior to refractive surgery and in diagnosis of glaucoma and keratoconus. Refractive surgery is planned according to preoperative central corneal thickness measurements. Besides, in order to determine actual intraocular pressure, central corneal thickness is measured and corrected eye pressure is obtained. Today, devices used in measurement of central corneal thickness do the measurement according to two principles. First and relatively older method is ultrasonic method. Optic method is the second and more recent method. In daily practice, the most commonly used clinical method in measurement of central corneal thickness is ultrasound pachymeter. However, this measurement technique requires contact between cornea and probe and gives thinner measurement results compared to the methods that use optic principle. Recently, several technologic methods based on optics have been put in use; they provide advantages of non-contact technique and objective determination of central corneal thickness. Of these methods, most commonly used include Specular Microscopy, Optical coherence tomography, Laser Doppler Interferometry, Optical low coherence reflectometry pachymetry, Optic based topographic mapping (also called screening section pachymetry and Pentacam. In this article, it was aimed to evaluate importance of central corneal thickness in clinical use and compare measurement methods. J Clin Exp Invest 2014; 5 (1: 153-158

  11. Analysis of the horizontal corneal diameter, central corneal thickness, and axial length in premature infants

    Directory of Open Access Journals (Sweden)

    Ozdemir Ozdemir

    2014-08-01

    Full Text Available Purpose: To determine the horizontal corneal diameter, central corneal thickness, and axial length in premature infants. Methods: Infants with a birth weight of less than 2,500 g or with a gestation period of less than 36 weeks were included in the study. Infants with retinopathy of prematurity (ROP were allocated to Group 1 (n=138, while those without ROP were allocated to Group 2 (n=236. All infants underwent a complete ophthalmologic examination, including corneal diameter measurements, pachymetry, biometry, and fundoscopy. Between-group comparisons of horizontal corneal diameter, central corneal thickness, and axial lengths were performed. Independent sample t-tests were used for statistical analysis. Results: Data was obtained from 374 eyes of 187 infants (102 female, 85 male. The mean gestational age at birth was 30.7 ± 2.7 weeks (range 25-36 weeks, the mean birth weight was 1,514 ± 533.3 g (range 750-1,970 g, and the mean postmenstrual age at examination was 40.0 ± 4.8 weeks. The mean gestational age and the mean birth weight of Group 1 were statistically lower than Group 2 (p0.05. Conclusions: The presence of ROP in premature infants does not alter the horizontal corneal diameter, central corneal thickness, or axial length.

  12. Corneal Stroma Regeneration with Acellular Corneal Stroma Sheets and Keratocytes in a Rabbit Model.

    Directory of Open Access Journals (Sweden)

    Xiao Yun Ma

    Full Text Available Acellular corneal stroma matrix has been used for corneal stroma engineering. However, because of its compact tissue structure, regrowth of keratocytes into the scaffold is difficult. Previously, we developed a sandwich model for cartilage engineering using acellular cartilage sheets. In the present study, we tested this model for corneal stroma regeneration using acellular porcine corneal stroma (APCS sheets and keratocytes. Porcine corneas were decellularized by NaCl treatment, and the APCS was cut into 20-μm-thick sheets. A rabbit corneal stroma defect model was created by lamellar keratoplasty and repaired by transplantation of five pieces of APCS sheets with keratocytes. Six months after transplantation, transparent corneas were present in the experimental group, which were confirmed by anterior segment optical coherence tomography examination and transmittance examination. The biomechanical properties in the experimental group were similar to those of normal cornea. Histological analyses showed an even distribution of keratocytes and well-oriented matrix in the stroma layer in the experimental group. Together, these results demonstrated that the sandwich model using acellular corneal stroma sheets and keratocytes could be potentially useful for corneal stroma regeneration.

  13. [Evaluation of corneal endothelium morphology in diabetic patients].

    Science.gov (United States)

    Wesołek-Czernik, Agata; Bartela, Joanna; Zamojska, Ewa; Omulecki, Wojciech

    2007-01-01

    To evaluate the influence of diabetes mellitus and type of hipoglicemic therapy on corneal endothelium cell morphology. In 68 diabetic patients' eyes with non proliferative diabetic retinopathy (29 males and 39 females), corneal endothelium was studied. Patients age was between 50 and 82 years (mean 63.28). As age-matched control group we analyzed 58 eyes of non diabetic patients. Corneal endothelium density, percentage of corneal endothelium hexagonal cells, average size of corneal endothelium cells and corneal thickness were imaged by non-contact specular microscope TOPCON SP-2000P. The mean corneal endothelium cell density was: 2467 cells/mm2 in diabetic patients, and 2573 cells/mm2 in control group. The mean percentage of corneal endothelium hexagonal cells was: 55.3% in diabetic patients treated with insulin and 52.9% in diabetic patients treated with oral antidiabetic drugs, and 54.4% in the control group. The mean size of corneal endothelium cells was: 414.6 microm2 in diabetic patients, and 395.9 microm2 in the control group. The mean corneal thickness was: 0.556 mm in diabetic patients and 0.545 mm in the control group. Corneal endothelium was thicker in diabetic patients than in non diabetic patients. The duration of diabetes mellitus had no influence on corneal endothelium cell morphology. Diabetic patients treated with oral antidiabetic drugs had larger percentage of hexagonal cells than those treated with insulin.

  14. Pattern of corneal opacity in Ibadan, Nigeria | Ashaye | Annals of ...

    African Journals Online (AJOL)

    Background: The prevalence and causes of corneal blindness vary from one region of the world to another. There is even variation within the developing countries of Africa. Method: A retrospective review of 675 patients with corneal scarring out of the 3,753 new patients corneal scarring in patients attending the eye clinic of ...

  15. Replacing the endothelium without corneal surface incisions or sutures: the first United States clinical series using the deep lamellar endothelial keratoplasty procedure.

    Science.gov (United States)

    Terry, Mark A; Ousley, Paula J

    2003-04-01

    To report the 6- and 12-month results of the first United States clinical series of deep lamellar endothelial keratoplasty (DLEK) in the treatment of endothelial dysfunction. Prospective, noncomparative, interventional case series. Eight eyes of eight patients with corneal edema from Fuchs' dystrophy and pseudophakia. A 9.0-mm limbal, scleral, partial-depth incision provided access for a deep lamellar corneal pocket dissection. A 7.5- to 8.0-mm posterior lamellar disc of recipient tissue was then excised and replaced through the pocket with a same size donor disc containing healthy endothelium. A temporary air bubble in the anterior chamber was used for donor tissue adherence, and no surface corneal incisions or sutures were necessary. Preoperative and postoperative best spectacle-corrected visual acuity (BSCVA), manifest refraction astigmatism, TMS-1 topography, ultrasonic pachymetry, Orbscan topography, and endothelial cell density were evaluated. Intraoperative and postoperative complications are reported. At 6 and 12 months after surgery, all eight corneas were clear and the grafts were healed in good position. At 6 months, the BSCVA varied between 20/30 and 20/70, the average change in astigmatism from before surgery was +1.13 diopters (D; +/-1.50 D), the average change in corneal power was -0.4 D (+/-1.7 D), the average pachymetry was 648 micro m (+/-134 micro m), and the average endothelial cell count was 2290 cells/mm(2) (+/-372 cells/mm(2)). At 12 months, three of the four eyes reaching this time gate were 20/40 or better, with a change in astigmatism from before surgery of only +0.81 D (+/- 0.55 D), a corneal power change of -1.3 D (+/- 0.4 D), and an endothelial density of 2409 cells/mm(2) (+/- 154 cells/mm(2)). One of the original nine eyes entered into this study required conversion to standard penetrating keratoplasty as a result of a microperforation during recipient pocket dissection and has experienced no ill effects. The DLEK procedure, with its

  16. Mechanical methods in refractive corneal surgery.

    Science.gov (United States)

    Hoffmann, F; Kruse, H; Schüler, A

    1993-08-01

    Keratorefractive surgery has developed rapidly over the past decade. For patients with aphakia, however, posterior chamber lenses are safer and more predictable than refractive corneal surgery; myopia is the greatest challenge to this type of surgery. No technique as yet has an accuracy that is adequate for the general treatment of myopia. Keratotomy is the most common procedure currently performed for the correction of mild myopia and astigmatism. Results are less accurate and less stable with myopic than with aphakic epikeratoplasty. Although it is technically more difficult, keratomileusis seems to be more predictable than epikeratophakia. Nonfreeze lamellar corneal surgery accelerates wound healing. Synthetic lenses and ring-shaped implants are also being developed to modify corneal refraction. Current research is directed toward laser stromal keratomileusis on discs that are removed from the cornea using a microkeratome.

  17. Cataract phacoemulsification and corneal endothelial cell damage

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    Ni Zhu

    2013-07-01

    Full Text Available Phacoemulsification with small incision, reduced number of inflammation cells, and better postoperative recovery has been recognized as the world's most popular option for cataract surgery. Modern cataract surgery is developing gradually from sight rehabilitating to refractive surgery with better vision acuity. Being the most important part of the eye refractive system, maintenance of the cornea's transparency relies heavily upon the healthy endothelial cells. It is well known that there will be endothelial cell loss after phacoemulsification and the damage of the endothelial cells may lead to corneal swellings and opacity, or even the corneal descompensation, which often severely influenced the postoperative vision recovery. This is a review of phacoemulsification and the risk factors of corneal endothelial damage pre-and postoperation.

  18. XENOTRANSPLANTATION – THE FUTURE OF CORNEAL TRANSPLANTATION?

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    Hara, Hidetaka; Cooper, David K.C.

    2010-01-01

    Although corneal transplantation is readily available in the USA and certain other regions of the developed world, the need for human donor corneas worldwide far exceeds supply. There is currently renewed interest in the possibility of using corneas from other species, especially pigs, for transplantation into humans (xenotransplantation). The biomechanical properties of human and pig corneas are similar. Studies in animal models of corneal xenotransplantation have documented both humoral and cellular immune responses that play roles in xenograft rejection. The results obtained from the Tx of corneas from wild-type (i.e., genetically-unmodified) pigs into nonhuman primates have been surprisingly good and encouraging. Recent progress in the genetic manipulation of pigs has led to the prospect that the remaining immunological barriers will be overcome. There is every reason for optimism that corneal xenoTx will become a clinical reality within the next few years. PMID:21099407

  19. Applications of biomaterials in corneal wound healing.

    Science.gov (United States)

    Tsai, I-Lun; Hsu, Chih-Chien; Hung, Kuo-Hsuan; Chang, Chi-Wen; Cheng, Yung-Hsin

    2015-04-01

    Disease affecting the cornea is a common cause of blindness worldwide. To date, the amniotic membrane (AM) is the most widely used clinical method for cornea regeneration. However, donor-dependent differences in the AM may result in variable clinical outcomes. To overcome this issue, biomaterials are currently under investigation for corneal regeneration in vitro and in vivo. In this article, we highlight the recent advances in hydrogels, bioengineered prosthetic devices, contact lenses, and drug delivery systems for corneal regeneration. In clinical studies, the therapeutic effects of biomaterials, including fibrin and collagen-based hydrogels and silicone contact lenses, have been demonstrated in damaged cornea. The combination of cells and biomaterials may provide potential treatment in corneal wound healing in the future. Copyright © 2014. Published by Elsevier Taiwan.

  20. Applications of biomaterials in corneal wound healing

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    I-Lun Tsai

    2015-04-01

    Full Text Available Disease affecting the cornea is a common cause of blindness worldwide. To date, the amniotic membrane (AM is the most widely used clinical method for cornea regeneration. However, donor-dependent differences in the AM may result in variable clinical outcomes. To overcome this issue, biomaterials are currently under investigation for corneal regeneration in vitro and in vivo. In this article, we highlight the recent advances in hydrogels, bioengineered prosthetic devices, contact lenses, and drug delivery systems for corneal regeneration. In clinical studies, the therapeutic effects of biomaterials, including fibrin and collagen-based hydrogels and silicone contact lenses, have been demonstrated in damaged cornea. The combination of cells and biomaterials may provide potential treatment in corneal wound healing in the future.

  1. Myotonic dystrophy mimicking postpolio syndrome in a polio survivor.

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    Lim, Jae-Young; Kim, Kyoung-Eun; Choe, Gheeyoung

    2009-02-01

    We describe a 38-yr-old polio survivor with newly developed weakness from myotonic dystrophy. He suffered muscle atrophy and weakness in his legs as a result of poliomyelitis at the age of 3 yrs. After a stable interval of about 30 yrs, he felt new weakness and fatigue in his legs. Electromyography revealed generalized myotonic discharges, early recruitment, and findings of chronic denervation in his left leg. Genetic testing was consistent with myotonic dystrophy type 1. A biopsy from the right gastrocnemius revealed findings of both myotonic dystrophy and chronic denervation. This case report shows the importance of considering other uncommon conditions in the differential diagnoses of postpolio syndrome.

  2. MR imaging of fukuyama congenital muscular dystrophy; a case report

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    Yoo, Jeong Hyun; Kim, Yoo Kyung; Koo, Hae Soo; Park, Ki Deuk [Ewha Womans Univ. College of Medicine, Seoul (Korea, Republic of)

    2000-11-01

    Fukuyama congenital muscular dystrophy is a genetic disease and common in Japan. The typical clinical features are hypotonia with an early infantile onset and severe developmental delay. The diagnosis is based on pathologic evidence of muscular dystrophy revealed by biopsy or an increased serum creatine kinase levels. Involvement of the brain is characterized by abnormal cerebral cortical dysplasia, cerebellar dysplasia, and white matter changes. We encountered a case of Fukuyama congenital muscular dystrophy in which brain MRI findings were typical, and present this case together with a review of the literature.

  3. Genetics and emerging treatments for Duchenne and Becker muscular dystrophy.

    Science.gov (United States)

    Wein, Nicolas; Alfano, Lindsay; Flanigan, Kevin M

    2015-06-01

    Mutations in the DMD gene result in Duchenne or Becker muscular dystrophy due to absent or altered expression of the dystrophin protein. The more severe Duchenne muscular dystrophy typically presents around ages 2 to 5 with gait disturbance, and historically has led to the loss of ambulation by age 12. It is important for the practicing pediatrician, however, to be aware of other presenting signs, such as delayed motor or cognitive milestones, or elevated serum transaminases. Becker muscular dystrophy is milder, often presenting after age 5, with ambulation frequently preserved past 20 years and sometimes into late decades. Copyright © 2015 Elsevier Inc. All rights reserved.

  4. Corneal nerve microstructure in Parkinson's disease.

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    Misra, Stuti L; Kersten, Hannah M; Roxburgh, Richard H; Danesh-Meyer, Helen V; McGhee, Charles N J

    2017-05-01

    Ocular surface changes and blink abnormalities are well-established in Parkinson's disease. Blink rate may be influenced by corneal sub-basal nerve density, however, this relationship has not yet been investigated in Parkinson's disease. This case-control study examined the ocular surface in patients with moderately severe Parkinson's disease, including confocal microscopy of the cornea. Fifteen patients with moderately severe Parkinson's disease (modified Hoehn and Yahr grade 3 or 4) and fifteen control participants were recruited. Ophthalmic assessment included slit-lamp examination, blink rate assessment, central corneal aesthesiometry and in vivo corneal confocal microscopy. The effect of disease laterality was also investigated. Of the 15 patients with Parkinson's disease, ten were male and the mean age was 65.5±8.6years. The corneal sub-basal nerve plexus density was markedly reduced in patients with Parkinson's disease (7.56±2.4mm/mm 2 ) compared with controls (15.91±2.6mm/mm 2 ) (pParkinson's disease (0.79±1.2mBAR) and the control group (0.26±0.35mBAR), p=0.12. Sub-basal nerve density was not significantly different between the eye ipsilateral to the side of the body with most-severe motor symptoms, and the contralateral eye. There was a significant positive correlation between ACE-R scores and sub-basal corneal nerve density (R 2 =0.66, p=0.02). This is the first study to report a significant reduction in corneal sub-basal nerve density in Parkinson's disease and demonstrate an association with cognitive dysfunction. These results provide further evidence to support the involvement of the peripheral nervous system in Parkinson's disease, previously thought to be a central nervous system disorder. Copyright © 2017 Elsevier Ltd. All rights reserved.

  5. Diagnosis and etiology of congenital muscular dystrophy.

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    Peat, R A; Smith, J M; Compton, A G; Baker, N L; Pace, R A; Burkin, D J; Kaufman, S J; Lamandé, S R; North, K N

    2008-07-29

    We aimed to determine the frequency of all known forms of congenital muscular dystrophy (CMD) in a large Australasian cohort. We screened 101 patients with CMD with a combination of immunofluorescence, Western blotting, and DNA sequencing to identify disease-associated abnormalities in glycosylated alpha-dystroglycan, collagen VI, laminin alpha2, alpha7-integrin, and selenoprotein. A total of 45% of the CMD cohort were assigned to an immunofluorescent subgroup based on their abnormal staining pattern. Abnormal staining for glycosylated alpha-dystroglycan was present in 25% of patients, and approximately half of these had reduced glycosylated alpha-dystroglycan by Western blot. Sequencing of the FKRP, fukutin, POMGnT1, and POMT1 genes in all patients with abnormal alpha-dystroglycan immunofluorescence identified mutations in one patient for each of these genes and two patients had mutations in POMT2. Twelve percent of patients had abnormalities in collagen VI immunofluorescence, and we identified disease-causing COL6 mutations in eight of nine patients in whom the genes were sequenced. Laminin alpha2 deficiency accounted for only 8% of CMD. alpha7-Integrin staining was absent in 12 of 45 patients studied, and ITGA7 gene mutations were excluded in all of these patients. We define the distribution of different forms of congenital muscular dystrophy in a large cohort of mixed ethnicity and demonstrate the utility and limitations of current diagnostic techniques.

  6. Muscle MRI findings in facioscapulohumeral muscular dystrophy

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    Gerevini, Simonetta; Caliendo, Giandomenico; Falini, Andrea [IRCCS San Raffaele Scientific Institute, Neuroradiology Unit, Head and Neck Department, Milan (Italy); Scarlato, Marina; Previtali, Stefano Carlo [IRCCS San Raffaele Scientific Institute, Department of Neurology, INSPE and Division of Neuroscience, Milan (Italy); Maggi, Lorenzo; Pasanisi, Barbara; Morandi, Lucia [Fondazione IRCCS Istituto Neurologico ' ' Carlo Besta' ' , Neuromuscular Diseases and Neuroimmunology Unit, Milan (Italy); Cava, Mariangela [IRCCS San Raffaele Scientific Institute, Department of Radiology and Center for Experimental Imaging, Milan (Italy)

    2016-03-15

    Facioscapulohumeral muscular dystrophy (FSHD) is characterized by extremely variable degrees of facial, scapular and lower limb muscle involvement. Clinical and genetic determination can be difficult, as molecular analysis is not always definitive, and other similar muscle disorders may have overlapping clinical manifestations. Whole-body muscle MRI examination for fat infiltration, atrophy and oedema was performed to identify specific patterns of muscle involvement in FSHD patients (30 subjects), and compared to a group of control patients (23) affected by other myopathies (NFSHD). In FSHD patients, we detected a specific pattern of muscle fatty replacement and atrophy, particularly in upper girdle muscles. The most frequently affected muscles, including paucisymptomatic and severely affected FSHD patients, were trapezius, teres major and serratus anterior. Moreover, asymmetric muscle involvement was significantly higher in FSHD as compared to NFSHD patients. In conclusion, muscle MRI is very sensitive for identifying a specific pattern of involvement in FSHD patients and in detecting selective muscle involvement of non-clinically testable muscles. Muscle MRI constitutes a reliable tool for differentiating FSHD from other muscular dystrophies to direct diagnostic molecular analysis, as well as to investigate FSHD natural history and follow-up of the disease. (orig.)

  7. Congenital muscular dystrophy with inflammation: Diagnostic considerations

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    Kaumudi Konkay

    2016-01-01

    Full Text Available Background and Purpose: Muscle biopsy features of congenital muscular dystrophies (CMD vary from usual dystrophic picture to normal or nonspecific myopathic picture or prominent fibrosis or striking inflammatory infiltrate, which may lead to diagnostic errors. A series of patients of CMD with significant inflammatory infiltrates on muscle biopsy were correlated with laminin α 2 deficiency on immunohistochemistry (IHC. Material and Methods: Cryostat sections of muscle biopsies from the patients diagnosed as CMD on clinical and muscle biopsy features from 1996 to 2014 were reviewed with hematoxylin and eosin(H&E, enzyme and immunohistochemistry (IHC with laminin α 2. Muscle biopsies with inflammatory infiltrate were correlated with laminin α 2 deficiency. Results: There were 65 patients of CMD, with inflammation on muscle biopsy in 16. IHC with laminin α 2 was available in nine patients, of which six showed complete absence along sarcolemma (five presented with floppy infant syndrome and one with delayed motor milestones and three showed discontinuous, and less intense staining. Conclusions: CMD show variable degrees of inflammation on muscle biopsy. A diagnosis of laminin α 2 deficient CMD should be considered in patients of muscular dystrophy with inflammation, in children with hypotonia/delayed motor milestones.

  8. Upper Girdle Imaging in Facioscapulohumeral Muscular Dystrophy

    Science.gov (United States)

    Tasca, Giorgio; Monforte, Mauro; Iannaccone, Elisabetta; Laschena, Francesco; Ottaviani, Pierfrancesco; Leoncini, Emanuele; Boccia, Stefania; Galluzzi, Giuliana; Pelliccioni, Marco; Masciullo, Marcella; Frusciante, Roberto; Mercuri, Eugenio; Ricci, Enzo

    2014-01-01

    Background In Facioscapulohumeral muscular dystrophy (FSHD), the upper girdle is early involved and often difficult to assess only relying on physical examination. Our aim was to evaluate the pattern and degree of involvement of upper girdle muscles in FSHD compared with other muscle diseases with scapular girdle impairment. Methods We propose an MRI protocol evaluating neck and upper girdle muscles. One hundred-eight consecutive symptomatic FSHD patients and 45 patients affected by muscular dystrophies and myopathies with prominent upper girdle involvement underwent this protocol. Acquired scans were retrospectively analyzed. Results The trapezius (100% of the patients) and serratus anterior (85% of the patients) were the most and earliest affected muscles in FSHD, followed by the latissimus dorsi and pectoralis major, whilst spinati and subscapularis (involved in less than 4% of the patients) were consistently spared even in late disease stages. Asymmetry and hyperintensities on short-tau inversion recovery (STIR) sequences were common features, and STIR hyperintensities could also be found in muscles not showing signs of fatty replacement. The overall involvement appears to be disease-specific in FSHD as it significantly differed from that encountered in the other myopathies. Conclusions The detailed knowledge of single muscle involvement provides useful information for correctly evaluating patients' motor function and to set a baseline for natural history studies. Upper girdle imaging can also be used as an additional tool helpful in supporting the diagnosis of FSHD in unclear situations, and may contribute with hints on the currently largely unknown molecular pathogenesis of this disease. PMID:24932477

  9. Lower limb surgery in Duchenne muscular dystrophy.

    Science.gov (United States)

    Forst, J; Forst, R

    1999-05-01

    Two hundred and thirteen of 428 patients with Duchenne muscular dystrophy (DMD) of a prospective and open study were operated on bilaterally with hip and knee release, aponeurectomy of the iliotibial band and Achilles tendon lengthening. In 87 patients the operation was carried out during early restrictions of the lower limb joint mobility at an average age of 6.56 years (4.02-8.26, SD 1.42). The follow-up was on average 5.4 years (0.25-9.01, SD 2.7). This group was compared to a control group (natural history) consisting of 100 non-operated DMD patients. A significant (P contrast to the patients of the control group all treated patients between ages 6 and 8 years could walk independently. The positive influence of early lower limb surgery could also be shown by the development of Hammersmith motor ability score, CIDD (Council of Investigation of Duchenne Dystrophy) grading and Vignos scale. Nevertheless, in consideration of the well-known course of DMD not only the prolongation of ambulation but also the achieved prolongation of assisted standing ability with no or mild contractures are aims of lower limb surgery. Since no improvement of muscle strength could be observed after lower limb surgery, further studies have to investigate if additionally administered steroids can prolong ambulation after early lower limb surgery.

  10. Emerging drugs for Duchenne muscular dystrophy.

    Science.gov (United States)

    Malik, Vinod; Rodino-Klapac, Louise R; Mendell, Jerry R

    2012-06-01

    Duchenne muscular dystrophy (DMD) is the most common, severe childhood form of muscular dystrophy. Treatment is limited to glucocorticoids that have the benefit of prolonging ambulation by approximately 2 years and preventing scoliosis. Finding a more satisfactory treatment should focus on maintaining long-term efficacy with a minimal side effect profile. Authors discuss different therapeutic strategies that have been used in pre-clinical and clinical settings. Multiple treatment approaches have emerged. Most attractive are molecular-based therapies that can express the missing dystrophin protein (exon skipping or mutation suppression) or a surrogate gene product (utrophin). Other approaches include increasing the strength of muscles (myostatin inhibitors), reducing muscle fibrosis and decreasing oxidative stress. Additional targets include inhibiting NF-κB to reduce inflammation or promoting skeletal muscle blood flow and muscle contractility using phosphodiesterase inhibitors or nitric oxide (NO) donors. The potential for each of these treatment strategies to enter clinical trials is a central theme of discussion. The review emphasizes that the goal of treatment should be to find a product at least as good as glucocorticoids with a lower side effect profile or with a significant glucocorticoid sparing effect.

  11. The superhealing MRL background improves muscular dystrophy

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    Heydemann Ahlke

    2012-12-01

    Full Text Available Abstract Background Mice from the MRL or “superhealing” strain have enhanced repair after acute injury to the skin, cornea, and heart. We now tested an admixture of the MRL genome and found that it altered the course of muscle pathology and cardiac function in a chronic disease model of skeletal and cardiac muscle. Mice lacking γ-sarcoglycan (Sgcg, a dystrophin-associated protein, develop muscular dystrophy and cardiomyopathy similar to their human counterparts with limb girdle muscular dystrophy. With disruption of the dystrophin complex, the muscle plasma membrane becomes leaky and muscles develop increased fibrosis. Methods MRL/MpJ mice were bred with Sgcg mice, and cardiac function was measured. Muscles were assessed for fibrosis and membrane leak using measurements of hydroxyproline and Evans blue dye. Quantitative trait locus mapping was conducted using single nucleotide polymorphisms distinct between the two parental strains. Results Introduction of the MRL genome reduced fibrosis but did not alter membrane leak in skeletal muscle of the Sgcg model. The MRL genome was also associated with improved cardiac function with reversal of depressed fractional shortening and the left ventricular ejection fraction. We conducted a genome-wide analysis of genetic modifiers and found that a region on chromosome 2 was associated with cardiac, diaphragm muscle and abdominal muscle fibrosis. Conclusions These data are consistent with a model where the MRL genome acts in a dominant manner to suppress fibrosis in this chronic disease setting of heart and muscle disease.

  12. Research on inhibition of corneal neovascularization

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    Zhang-Hui Yang

    2015-12-01

    Full Text Available Corneal transparency is the basis of the normal physiological functions.However, corneal neovascularization(CNVmay occur in the infection, mechanical and chemical injury or under other pathological conditions,which make the cornea lose original transparency and severe visual impairment. In recent years, along with the development of immunology, molecular biology, biochemistry and other disciplines, there is more in-depth understanding on the CNV, and clinical treatment of CNV has made new breakthroughs. This article provides an overview of the inhibition of CNV.

  13. Corneal epithelial inclusion cyst in a dog

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    Campos Carla de Freitas

    2002-01-01

    Full Text Available An unilateral corneal epithelial inclusion cyst in a 7-year-old male Boxer dog is reported. The cyst had been observed for thirty days, was unique, not congenital and only one eye was involved. Seven months prior to the referral the dog had manifested indolent corneal ulcer treated with grade keratotomy and third eyelid flap. The cyst was removed by superficial keratectomy followed by a conjunctival pedicle graft. Recovery was uncomplicated and there wasn?t recurrence seven months after the surgery.

  14. The corneal stroma during contact lens wear.

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    Jalbert, Isabelle; Stapleton, Fiona

    2005-03-01

    Recent technological advances have lead to novel descriptions of the microanatomy of the corneal stroma. In the first section of this review, these findings and the role they play in the maintenance of vital properties such as corneal transparency, mechanical strength, homeostasis, wound-healing response and metabolism are described. In the second part, contact lens induced stromal alterations such as acidosis, oedema, striae, thinning and opacities are reviewed as well as the more recently described phenomenon of microdot deposits and keratocyte loss with an emphasis on how lens wearing stromal effects can be minimised.

  15. The molecular basis of corneal transparency.

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    Hassell, John R; Birk, David E

    2010-09-01

    The cornea consists primarily of three layers: an outer layer containing an epithelium, a middle stromal layer consisting of a collagen-rich extracellular matrix (ECM) interspersed with keratocytes and an inner layer of endothelial cells. The stroma consists of dense, regularly packed collagen fibrils arranged as orthogonal layers or lamellae. The corneal stroma is unique in having a homogeneous distribution of small diameter 25-30 nm fibrils that are regularly packed within lamellae and this arrangement minimizes light scattering permitting transparency. The ECM of the corneal stroma consists primarily of collagen type I with lesser amounts of collagen type V and four proteoglycans: three with keratan sulfate chains; lumican, keratocan, osteoglycin and one with a chondroitin sulfate chain; decorin. It is the core proteins of these proteoglycans and collagen type V that regulate the growth of collagen fibrils. The overall size of the proteoglycans are small enough to fit in the spaces between the collagen fibrils and regulate their spacing. The stroma is formed during development by neural crest cells that migrate into the space between the corneal epithelium and corneal endothelium and become keratoblasts. The keratoblasts proliferate and synthesize high levels of hyaluronan to form an embryonic corneal stroma ECM. The keratoblasts differentiate into keratocytes which synthesize high levels of collagens and keratan sulfate proteoglycans that replace the hyaluronan/water-rich ECM with the densely packed collagen fibril-type ECM seen in transparent adult corneas. When an incisional wound through the epithelium into stroma occurs the keratocytes become hypercellular myofibroblasts. These can later become wound fibroblasts, which provides continued transparency or become myofibroblasts that produce a disorganized ECM resulting in corneal opacity. The growth factors IGF-I/II are likely responsible for the formation of the well organized ECM associated with transparency

  16. Collagen Fibrils and Proteoglycans of Macular Dystrophy Cornea: Ultrastructure and 3D Transmission Electron Tomography.

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    Akhtar, Saeed; Alkatan, Hind M; Kirat, Omar; Khan, Adnan A; Almubrad, Turki

    2015-06-01

    We report the ultrastructure and 3D transmission electron tomography of collagen fibrils (CFs), proteoglycans (PGs), and microfibrils within the CF of corneas of patients with macular corneal dystrophy (MCD). Three normal corneas and three MCD corneas from three Saudi patients (aged 25, 31, and 49 years, respectively) were used for this study. The corneas were processed for light and electron microscopy studies. 3D images were composed from a set of 120 ultrastructural images using the program "Composer" and visualized using the program "Visuliser Kai". 3D image analysis of MCD cornea showed a clear organization of PGs around the CF at very high magnification and degeneration of the microfibrils within the CF. Within the MCD cornea, the PG area in the anterior stroma was significantly larger than in the middle and posterior stroma. The PG area in the MCD cornea was significantly larger compared with the PG area in the normal cornea. The CF diameter and inter-fibrillar spacing of the MCD cornea were significantly smaller compared with those of the normal cornea. Ultrastructural 3D imaging showed that the production of unsulfated keratin sulfate (KS) may lead to the degeneration of micro-CFs within the CFs. The effect of the unsulfated KS was higher in the anterior stroma compared with the posterior stroma.

  17. Corneal endothelial cytotoxicity of the Calotropis procera (ushaar) plant.

    Science.gov (United States)

    Al-Mezaine, Hani S; Al-Amry, Mohammed A; Al-Assiri, Abdullah; Fadel, Talal S; Tabbara, Khalid F; Al-Rajhi, Ali A

    2008-05-01

    To report 6 eyes of 5 patients with transient corneal edema after exposure to the milky latex of Calotropis procera (ushaar). Interventional case series. Intracorneal penetration of ushaar latex can lead to permanent endothelial cell loss with morphologic alteration. Corneal edema resolved completely in approximately 2 weeks in all cases, despite reduced endothelial cell count and abnormal morphology. Corneal endothelial toxicity of ushaar latex is caused by its ability to penetrate the corneal stroma and induce permanent loss of endothelial cells. Corneal edema resolves if sufficient endothelial cell viability is still present after resolution of ushaar keratitis.

  18. Reversible Corneal Toxicity of Retained Intracameral Perfluoro-n-octane.

    Science.gov (United States)

    Alharbi, Saad S; Asiri, Mohammed S

    2016-01-01

    A 58-year-old female presented with intracameral retained perfluoro-n-octane (PFO) following previous retinal reattachment surgery. After 4 years of follow-up without related sequelae, the patient complained of a gradual decrease in vision secondary to corneal edema with whitish corneal precipitate inferiorly corresponding to the area of retained PFO. Three weeks after anterior chamber washout, corneal edema resolved and the patient obtained 20/40 visual acuity. Even though PFO considered to have a relatively good safety profile, early anterior chamber washout may prevent corneal toxicity and avoid later persistent corneal decompensation.

  19. Current status of accelerated corneal cross-linking

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    Michael Mrochen

    2013-01-01

    Full Text Available Corneal cross-linking with riboflavin is a technique to stabilize or reduce corneal ectasia, in diseases such as keratoconus and post-laser-assisted in situ keratomileusis (LASIK ectasia. There is an interest by patient as well as clinicians to reduce the overall treatment time. Especially, the introduction of corneal cross-linking in combination with corneal laser surgery demands a shorter treatment time to assure a sufficient patient flow. The principles and techniques of accelerated corneal cross-linking is discussed.

  20. Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans

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    Julieta Rodini Engrácia de Moraes

    2017-09-01

    Full Text Available ABSTRACT: Golden Retriever muscular dystrophy (GRMD is the most representative model for studying Duchenne muscular dystrophy (DMD in humans, owing its phenotypic expression. DMD is a recessive disorder linked to the X chromosome in which the loss of dystrophin induces progressive weakness and degeneration of the skeletal and cardiac muscles, which lead to replacement by connective and adipose tissues. Onset of clinical signs occurs between 2 and 5 years of age, and many patients die from heart or respiratory failure. The main studies concerning dystrophic Golden Retrievers (DGR sought to elucidate the pathophysiology of the disease and its clinical implications to develop therapies and alternative treatments to improve the quality of life and increase longevity of DMD patients. This review presents an overview of relevant contributions of the DGR model for elucidating DMD in humans.

  1. [Measurement of viscoelastic corneal parameters (corneal hysteresis) in patients with primary open angle glaucoma].

    Science.gov (United States)

    Schroeder, B; Hager, A; Kutschan, A; Wiegand, W

    2008-10-01

    The ocular response analyzer (ORA) uses an air-pressure-triggered, dynamic, bi-directional corneal applanation method to measure biomechanical parameters of the cornea. Corneal hysteresis (CH) is defined as the difference in intraocular pressure recorded during inward and outward applanation. CH is therefore an indicator for the viscoelastic properties of the cornea. CH was recorded in non-glaucoma patients (80 eyes) as well as in patients with primary open angle glaucoma (POAG, 82 eyes). The correlation between CH and central corneal thickness (CCT) was analyzed. Mean CH was 10.6+/-2.2 mmHg in the non-glaucoma group and 9.3+/-2.2 mmHg in patients with POAG (pcorneal parameters with a significant decrease in corneal hysteresis. A positive correlation between CH and CCT, which was seen in the non-glaucoma group could not be detected in the POAG group.

  2. Quantitative evaluation of corneal epithelial injury caused by n-heptanol using a corneal resistance measuring device in vivo

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    Fukuda M

    2012-04-01

    Full Text Available Masamichi Fukuda1, Hiroshi Sasaki11Department of Ophthalmology, Kanazawa Medical University, Uchinada, JapanPurpose: We attempted to develop a device for measuring electrical corneal resistance (CR using corneal contact lens electrodes to quantitatively evaluate corneal injury in vivo. In the present study, full-thickness detachment of the corneal epithelium was induced by n-heptanol, and the feasibility of the quantitative evaluation of this injury by corneal contact lens electrodes was evaluated in vivo.Methods: The central area of an albino rabbit's cornea was exposed to a filter paper pre-immersed in n-heptanol for 1 minute to induce injury of the corneal epithelium. After induction of injury, the electrical CR was measured and the percentage of CR (%CR was calculated. Fluorescein solution (3 µL was applied to the wound/affected area of the corneal epithelium for photography with a slit-lamp biomicroscope. The wound/affected area was measured using an image analysis system. The correlation between the %CR and the wound/affected area was analyzed.Results: As the size of the wound/affected area of the corneal epithelium increased, the %CR decreased after corneal epithelium detachment. Thus, a close correlation was found between the area of corneal epithelium detachment and the %CR.Conclusion: The corneal resistance device that we developed was capable of quantitatively evaluating n-heptanol-induced full-thickness injuries of the corneal epithelium.Keywords: eyes in vivo, corneal injury, corneal contact lens electrode corneal resistance device, n-heptanol

  3. Normative values for corneal nerve morphology assessed using corneal confocal microscopy: a multinational normative data set.

    Science.gov (United States)

    Tavakoli, Mitra; Ferdousi, Maryam; Petropoulos, Ioannis N; Morris, Julie; Pritchard, Nicola; Zhivov, Andrey; Ziegler, Dan; Pacaud, Danièle; Romanchuk, Kenneth; Perkins, Bruce A; Lovblom, Leif E; Bril, Vera; Singleton, J Robinson; Smith, Gordon; Boulton, Andrew J M; Efron, Nathan; Malik, Rayaz A

    2015-05-01

    Corneal confocal microscopy is a novel diagnostic technique for the detection of nerve damage and repair in a range of peripheral neuropathies, in particular diabetic neuropathy. Normative reference values are required to enable clinical translation and wider use of this technique. We have therefore undertaken a multicenter collaboration to provide worldwide age-adjusted normative values of corneal nerve fiber parameters. A total of 1,965 corneal nerve images from 343 healthy volunteers were pooled from six clinical academic centers. All subjects underwent examination with the Heidelberg Retina Tomograph corneal confocal microscope. Images of the central corneal subbasal nerve plexus were acquired by each center using a standard protocol and analyzed by three trained examiners using manual tracing and semiautomated software (CCMetrics). Age trends were established using simple linear regression, and normative corneal nerve fiber density (CNFD), corneal nerve fiber branch density (CNBD), corneal nerve fiber length (CNFL), and corneal nerve fiber tortuosity (CNFT) reference values were calculated using quantile regression analysis. There was a significant linear age-dependent decrease in CNFD (-0.164 no./mm(2) per year for men, P < 0.01, and -0.161 no./mm(2) per year for women, P < 0.01). There was no change with age in CNBD (0.192 no./mm(2) per year for men, P = 0.26, and -0.050 no./mm(2) per year for women, P = 0.78). CNFL decreased in men (-0.045 mm/mm(2) per year, P = 0.07) and women (-0.060 mm/mm(2) per year, P = 0.02). CNFT increased with age in men (0.044 per year, P < 0.01) and women (0.046 per year, P < 0.01). Height, weight, and BMI did not influence the 5th percentile normative values for any corneal nerve parameter. This study provides robust worldwide normative reference values for corneal nerve parameters to be used in research and clinical practice in the study of diabetic and other peripheral neuropathies. © 2015 by the American Diabetes Association

  4. The Effect of Corneal Epithelium on Corneal Curvature in Patients with Keratoconus.

    Science.gov (United States)

    Akcay, Emine Kalkan; Uysal, Betul Seher; Sarac, Ozge; Ugurlu, Nagehan; Yulek, Fatma; Cagil, Nurullah; Aslan, Nabi

    2015-01-01

    To investigate the effects of corneal epithelium on corneal curvature in patients with keratoconus. This is a prospective, nonrandomized study. Fifty-nine eyes of 47 patients diagnosed as keratoconus and for whom corneal collagen crosslinking (CXL) was recruited in this study. This study is a single-center clinical trial. Pregnancy, lactation, connective tissue disease, corneal thickness below 350 μm, severe dry eyes, or scar of corneal surgery were exclusion criteria. Before and during CXL procedure after removing the corneal epithelium, maximum values of corneal apical curvature, simulated keratometry 1 (Sim-K1), simulated keratometry 2 (Sim-K2), temporal and inferior curvature values, all of which are 1.5 mm from the corneal center, were calculated. These values before and after removal of epithelium were compared statistically. Mean age of patients was 23.30 ± 5.5 (12-38) years. Twenty-eight (59%) were male while 19 (41%) were female. Mean values measured before and after removing the corneal epithelium were: apical curvature; 59.19 ± 7.2 (47.06-82.40) diopter (D) and 61.70 ± 8.8 (49.19-92.66) D (p = 0.001), SimK1; 47.57 ± 4.3 (39.14-64.57) D and 48.23 ± 4.3 (41.89-66.70) D (p = 0.001), SimK2; 52.04 ± 5.3 (43.56-69.34) D and 53.34 ± 5.6 (43.73-70.89) D (p = 0.001), inferior curvature; 53,85 ± 5.2 (43.47-76.56) D and 55.05 ± 5.8 (44.56-81.93) D (p = 0.002), temporal curvature 49.49 ± 5.1 (41.50-71.03) D and 51.53 ± 5.4 (41.58-73.34) D (p = 0.001), respectively. In keratoconus patients during CXL treatment, after removing the corneal epithelium, more steepness is detected in the curvature of the steeper area of the cornea. When evaluating patients with keratoconus, the masking effect of corneal epithelium on values of curvature should be taken into consideration.

  5. The scale of substratum topographic features modulates proliferation of corneal epithelial cells and corneal fibroblasts

    OpenAIRE

    Liliensiek, S.J.; Campbell, S.; Nealey, P. F.; Murphy, C J

    2006-01-01

    The cornea is a complex tissue composed of different cell types, including corneal epithelial cells and keratocytes. Each of these cell types are directly exposed to rich nanoscale topography from the basement membrane or surrounding extracellular matrix. Nanoscale topography has been shown to influence cell behaviors, including orientation, alignment, differentiation, migration, and proliferation. We investigated whether proliferation of SV40-transformed human corneal epithelial cells (SV40-...

  6. Corneal Biomechanical Assessment Using Corneal Visualization Scheimpflug Technology in Keratoconic and Normal Eyes

    OpenAIRE

    Lei Tian; Yi-Fei Huang; Li-Qiang Wang; Hua Bai; Qun Wang; Jing-Jing Jiang; Ying Wu; Min Gao

    2014-01-01

    Purpose. To compare the corneal biomechanical properties of keratoconic patients and age-matched controls using corneal visualization Scheimpflug technology (Corvis ST). Methods. Sixty keratoconic eyes from 47 keratoconus patients and 60 normal eyes from 60 controls were enrolled in this prospective study. Tomography and biomechanical parameters of all eyes were obtained with the Pentacam and Corvis ST, respectively. Intraocular pressure was measured using a Goldmann applanation tonometer. ...

  7. Increased corneal hysteresis after corneal collagen crosslinking: a study based on applanation resonance technology.

    Science.gov (United States)

    Beckman Rehnman, Jeannette; Behndig, Anders; Hallberg, Per; Lindén, Christina

    2014-12-01

    A reliable tool for quantification of the biomechanical status of the cornea in conjunction with corneal collagen crosslinking (CXL) treatment is needed. To quantify the biomechanical effects of CXL in vivo. A prospective, open, case-control study was conducted at the Department of Ophthalmology, Umeå University, Umeå, Sweden. Participants included 28 patients (29 eyes) aged 18 to 28 years with progressive keratoconus and corresponding age- and sex-matched healthy individuals serving as controls. All participants were monitored during a 6-month period between October 13, 2009, and November 5, 2012. Corneal hysteresis after CXL for keratoconus. A difference in corneal hysteresis between the control group and the patients with keratoconus was found at baseline, both with an applanation resonance tonometer (ART) and an ocular response analyzer (ORA), at mean (SD) values of -1.09 (1.92) mm Hg (99% CI, -2.26 to 0.07; P = .01) and -2.67 (2.55) mm Hg (99% CI, -4.05 to -1.32; P corneal hysteresis was demonstrated with an ART 1 and 6 months after CXL, at 1.2 (2.4) mm Hg (99% CI,-0.1 to 2.5; P = .02) and 1.1 (2.7) mm Hg (99% CI, -0.3 to 2.6; P = .04), respectively, but not with ORA. A decrease in corneal thickness was seen 1 and 6 months after treatment (-24 [26] µm, P corneal flattening of -0.6 (0.7) diopters was seen at 6 months (P corneal hysteresis after CXL treatment. Given the large-scale use of CXL in modern keratoconus treatment, a tool with this capacity has a great potential value. Refinement of the ART method of measuring and quantifying corneal biomechanical properties will be a subject of further studies.

  8. Fibrin glue-assisted for the treatment of corneal perforationsusing glycerin-cryopreserved corneal tissue

    Directory of Open Access Journals (Sweden)

    Nuo Dong

    2014-04-01

    Full Text Available AIM: To evaluate the outcomes and safety of lamellar keratoplasty (LK assisted by fibrin glue in corneal perforations.METHODS: Six eyes of 6 patients affected by different corneal pathologies (2 posttraumatic corneal scar and 3 bacterial keratitis underwent LK procedures by using fibrin glue. The mean corneal perforation diameter was 1.35±0.64mm (range, 0.7-2.5mm, and the greatest diameter of the ulcerative stromal defect was 2.47±0.77mm in average (range, 1.5-3.5mm. The donor corneal lamella diameters were 0.20-mm larger and thicker than the recipient to restore a physiologic corneal thickness and shape:mean donor diameter was 8.34±0.28mm (range, 8.2-8.7mm and mean thickness was 352±40.27mm (range, 220-400mm. Mean follow-up was 7.33±1.97 months (range, 6-11 months. Postoperatively, the graft status, graft clarity, anterior chamber response, the visual prognosis, intraocular pressures, and postoperative complications were recorded.RESULTS: All the corneal perforations were successfully healed after the procedure. The best-corrected visual acuity (BCVA ranged from 20/1 000 to 20/50 in their initial presentation, and from 20/100 to 20/20 in their last visit, showed increase in all the patients. No major complications such as graft dislocation and graft failure were noted. Neovascularization developed in the superficial stroma of donor graft in 1 case. High intraocular pressure developed on day 2 after surgery, while was remained in normal range after application of anti-glaucomatous eyedrops for 1 week in 1 case.CONCLUSION: Fibrin glue-assisted sutureless LK is valuable for maintaining the ocular integrity in the treatment of corneal perforations.

  9. Intraoperative corneal thickness change and clinical outcomes after corneal collagen crosslinking: Standard crosslinking versus hypotonic riboflavin.

    Science.gov (United States)

    Rosenblat, Elan; Hersh, Peter S

    2016-04-01

    To determine intraoperative changes in corneal thickness and outcomes of corneal collagen crosslinking (CXL) using 2 intraoperative regimens: riboflavin-dextran or hypotonic riboflavin. Cornea and refractive surgery practice, Teaneck, New Jersey, USA. Prospective randomized case series. Eyes with keratoconus or corneal ectasia were treated. All eyes received preloading with riboflavin 0.1% in 20% dextran. During ultraviolet-A (UVA) exposure, patients were randomly assigned to 1 of 2 study arms; that is, riboflavin-dextran or hypotonic riboflavin. Intraoperative pachymetry was measured before and after the corneal epithelium was removed, after initial riboflavin loading, and after UVA light exposure. Patients were evaluated for maximum keratometry (K), uncorrected distance visual acuity (UDVA), corrected distance visual acuity, corneal thickness, and endothelial cell count (ECC). Forty-eight eyes were treated. After removal of the epithelium and riboflavin loading, the mean pachymetry was 430 μm and 432 μm in the standard group and hypotonic group, respectively. Immediately after 30-minute UVA administration, the mean pachymetry was 302 μm and 342 μm, respectively. There was no statistically significant difference in the postoperative maximum K change, UDVA, corneal thickness, or ECC between the 2 groups. The cornea thinned substantially during the CXL procedure. The use of hypotonic riboflavin rather than riboflavin-dextran during UVA administration decreased the amount of corneal thinning during the procedure by 30%, from 128 μm to 90 μm. However, there were no significant differences in clinical efficacy or changes in ECC or function between groups postoperatively. In general, corneal thinning during CXL did not seem to compromise the safety of the endothelium. Dr. Hersh is a consultant to Avedro, Inc. Dr. Rosenblat has no financial or proprietary interest in any material or method mentioned. Copyright © 2016 ASCRS and ESCRS. Published by Elsevier

  10. Astigmatism induced by intrastromal corneal suture after small incision phacoemulsification.

    Science.gov (United States)

    Chipont-Benabent, E; Artola Roig, A; Pérez-Santonja, J J; Guisbert Medel, M; Alió Sanz, J L

    1998-04-01

    To evaluate the course of astigmatic evolution and complications after clear corneal incisions using an intrastromal corneal suture. Instituto Oftalmologico de Alicante, University of Alicante, Spain. Eighty eyes of 62 patients had endocapsular phacoemulsification. A foldable intraocular lens was implanted through a 4.0 mm clear corneal incision. A 10-0 nylon intrastromal corneal suture was used in all eyes. Change sin corneal astigmatism were calculated by vector analysis; follow-up was 6 months. Early and late suture-related complications were also evaluated. Mean induced cylinder was 1.25 diopters (D) +/- 1.24 (SD) with the wound 1 day postoperatively and 0.19 +/- 0.81 D against the wound at 6 months. There were no incision- or suture-related complications postoperatively. Use of the intrastromal corneal suture led to astigmatically neutral closure of multiplanar corneal incisions.

  11. [Clinical observation of astigmatism induced by corneal incision after phacoemulsification].

    Science.gov (United States)

    Xie, L; Zhu, G; Wang, X

    2001-03-01

    To evaluate the changes of astigmatism induced by corneal incision after phacoemulsification. Phacoemulsification was performed on 62 cases (78 eyes) without suture for a corneal incision. The changes of corneal astigmatism before and after operation in group A (incision at the superior nasal or superior temporal) and B (incision at the steepest corneal meridian) were compared. Three months after the operation, the surgical induced astigmatism was (0.83 plus minus 0.65) D and (0.72 plus minus 0.55) D in group A and B respectively. There was no significant difference, statistically (P > 0.05). The changes of corneal astigmatism were 0.11 D and 0.39 D in group A and B respectively, the difference being statistically significant (P astigmatism is very small after a corneal incision in phacoemulsification without a suture. If the incision is placed on the steepest meridian, the corneal astigmatism can be significantly reduced postoperatively.

  12. Immediately observation on post-LASIK corneal flap

    Directory of Open Access Journals (Sweden)

    Kun Wu

    2014-08-01

    Full Text Available AIM:To immediately observe the complication of corneal flap after LASIK surgery.METHODS:A retrospective case series were studies.Totally 2 040 cases(4 080 eyesfrom January 2010 to October 2012 in our hospital were collected, the corneal flap was observed using lamp microscope after LASIK within 30min. Corneal flap dislocation, corneal flap strial and intraface debris were examined after operation, the effective treatment and controlled measure should be taken for these complications.RESULTS: Postoperative complications were corneal flap dislocation 102 eyes(2.5%, corneal flapstriae 95 eyes(2.33%, interface debris 105 eyes(2.57%. No failure case was seen. There had no corneal flap-related complications, which seriously impact the visual quality after the surgery.CONCLUSION: Carefully postoperative examination at the first-time is an effective way to manage some complications of post-LASIK. Thus promoting the diagnosis and treatment of post-LASIK complications.

  13. Mothers' psychological adaptation to Duchenne/Becker muscular dystrophy

    National Research Council Canada - National Science Library

    Peay, Holly L; Meiser, Bettina; Kinnett, Kathleen; Furlong, Pat; Porter, Kathryn; Tibben, Aad

    2016-01-01

    Duchenne and Becker muscular dystrophy (DBMD) cause significant emotional and care-related burden on caregivers, but no studies have evaluated predictors of positive caregiver outcomes, including disorder-specific psychological adaptation...

  14. Nonmuscular involvement in merosin-negative congenital muscular dystrophy.

    NARCIS (Netherlands)

    Gilhuis, H.J.; Donkelaar, H.J. ten; Tanke, R.B.; Vingerhoets, D.M.; Zwarts, M.J.; Verrips, A.; Gabreëls, F.J.M.

    2002-01-01

    The spectrum of nonmuscular involvement in six children with merosin-negative congenital muscular dystrophy is described. In all children, biochemical, neuroradiologic, cardiac, and neurophysiologic studies were performed. Cerebral structures that were myelinated at gestation, including internal

  15. Clinical and molecular genetic analysis of best vitelliform macular dystrophy.

    NARCIS (Netherlands)

    Boon, C.J.F.; Theelen, T.; Hoefsloot, L.H.; Schooneveld, M.J. van; Keunen, J.E.E.; Cremers, F.P.M.; Klevering, B.J.; Hoyng, C.B.

    2009-01-01

    PURPOSE: To describe the phenotype of Best vitelliform macular dystrophy (BVMD) and to evaluate genotype-phenotype and histopathologic correlations. METHODS: Retrospective analysis of patients with BVMD who underwent an extensive ophthalmic examination, including best-corrected Snellen visual

  16. Ocular, bulbar, limb, and cardiopulmonary involvement in oculopharyngeal muscular dystrophy

    DEFF Research Database (Denmark)

    Witting, N; Mensah, A; Køber, L

    2014-01-01

    OBJECTIVES: To assess skeletal muscle weakness and progression as well as the cardiopulmonary involvement in oculopharyngeal muscular dystrophy (OPMD). MATERIALS AND METHODS: Cross-sectional study including symptomatic patients with genetically confirmed OPMD. Patients were assessed by medical...

  17. Strong association between myotonic dystrophy type 2 and autoimmune diseases.

    NARCIS (Netherlands)

    Tieleman, A.A.; Broeder, A. den; Logt, A. van de; Engelen, B.G.M. van

    2009-01-01

    BACKGROUND: Myotonic dystrophy type 2 (DM2) is a dominantly inherited multisystem disorder, characterised by progressive proximal weakness, myotonia, cataracts and cardiac conduction abnormalities. Our clinical impression of an association between DM2 and autoimmune diseases or autoantibody

  18. Strength training and albuterol in facioscapulohumeral muscular dystrophy

    NARCIS (Netherlands)

    van der Kooi, EL; Vogels, OJM; van Asseldonk, RJGP; Lindeman, E; Hendriks, JCM; Wohlgemuth, M; van der Maarel, SM; Padberg, GW

    2004-01-01

    Background: In animals and healthy volunteers beta2-adrenergic agonists increase muscle strength and mass, in particular when combined with strength training. In patients with facioscapulohumeral muscular dystrophy (FSHD) albuterol may exert anabolic effects. The authors evaluated the effect of

  19. Strength training and albuterol in facioscapulohumeral muscular dystrophy.

    NARCIS (Netherlands)

    Kooi, E.L. van der; Vogels, O.J.M.; Asseldonk, R.J. van; Lindeman, E.J.M.; Hendriks, J.C.M.; Wohlgemuth, M.; Maarel, S.M. van der; Padberg, G.W.A.M.

    2004-01-01

    BACKGROUND: In animals and healthy volunteers beta2-adrenergic agonists increase muscle strength and mass, in particular when combined with strength training. In patients with facioscapulohumeral muscular dystrophy (FSHD) albuterol may exert anabolic effects. The authors evaluated the effect of

  20. Predictive factors for masticatory performance in Duchenne muscular dystrophy

    NARCIS (Netherlands)

    Bruggen, H.W. van; Engel-Hoek, L. van den; Steenks, M.H.; Bronkhorst, E.M.; Creugers, N.H.; Groot, I.J.M. de; Kalaykova, S.

    2014-01-01

    Patients with Duchenne muscular dystrophy (DMD) report masticatory and swallowing problems. Such problems may cause complications such as choking, and feeling of food sticking in the throat. We investigated whether masticatory performance in DMD is objectively impaired, and explored predictive