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      Bilateral Keratectasia 34 Years after Corneal Transplant

      case-report

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          Abstract

          We report the clinical findings of a patient with severe bilateral keratectasia 34 years after a penetrating keratoplasty (PK) in both eyes. An otherwise healthy 67-year-old man complained of deterioration of the eyesight in both eyes over the last 6 months. The patient was diagnosed with bilateral keratoconus at the age of 32 years, and he underwent a bilateral PK. At presentation, visual acuity was 20/200 in the right eye and light perception in the left eye. A Pentacam pachymetric map revealed a central pachymetry of 720 μm in the right eye and of 710 μm in the left eye, as well as an average paracentral pachymetry of 436 and 270 μm in the 9-mm zone in the right and the left eye, respectively. Corneal topography revealed bilateral irregular and asymmetric bowing with generalized steepening and high corneal power. We describe a case of bilateral keratectasia 34 years after PK in a patient who was originally diagnosed with bilateral keratoconus.

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          Most cited references10

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          Leber congenital amaurosis and its association with keratoconus and keratoglobus.

          M Elder (2016)
          Leber congenital amaurosis has been associated with keratoconus and it has been postulated that this is due to eye rubbing, the oculo-digital sign, because of poor vision. Six schools for the blind were visited, and 174 children with a visual acuity of less than 3/60 examined. Thirty-five children had Leber amaurosis, and 10 of these had keratoconus (29%) and one had keratoglobus (3%). The six pedigrees of the cases with Leber amaurosis and keratoconus are presented in detail. Only 3 of the 139 other blind children had keratoconus (P < .05) and 1 had keratoglobus. Keratoconus seems specifically associated with Leber amaurosis, probably due to genetic factors, rather than poor visual acuity per se.
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            Topographic analysis in pellucid marginal corneal degeneration and keratoglobus.

            Pellucid marginal corneal degeneration (PMCD) is an uncommon cause of inferior peripheral corneal ectasia, affecting patients between the ages of 20 and 40 years. Although histopathologically it is considered a variant of keratoconus, it differs in that the marked corneal steepening occurs more inferiorly, above a narrow band of corneal stromal thinning concentric to the inferior limbus. Here we present two cases. The first case is a clinically typical bilateral PMCD with a characteristic pattern of irregular against-the-rule astigmatism on corneal topography. The second case had an uncommon presentation of hydrops in a clinically keratoglobic eye which showed a marked steepening of the inferior corneal periphery on corneal topography. The other eye showed both clinically and topographically the features of PMCD. Corneal topography suggested that in the second patient, PMCD may have preceded the development of keratoglobus. Keratoconus, PMCD and keratoglobus are considered to be associated as part of the spectrum of non-inflammatory corneal thinning disorders. However, although the finding of PMCD and keratoconus in fellow eyes has been reported, to the best of our knowledge progression from PMCD to keratoglobus has not previously been shown.
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              Keratoglobus and blue sclera.

              Five patients from two families had similar features including keratoglobus, blue scleras, hyperextensibility of the hand, wrist, and ankle joints, sensorineural conduction hearing alterations, and mottling of the teeth. Keratoglobus had been observed in all patients at, or shortly after, birth. Corneal perforations developed in seven of the ten eyes after minimal trauma. Repair of these perforations was complicated by the extremely thin corneas and six eyes had to be either enucleated or eviscerated. Histopathological examination of two of the enucleated eyes showed the corneal stromas of both eyes to be estremely thin, Bowman's membrane was absent, and Descemet's membrane was unusually thick. This condition has an autosomal-recessive inheritance pattern.
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                Author and article information

                Journal
                Case Report Ophthalmol
                COP
                Case Reports in Ophthalmology
                S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch )
                1663-2699
                May-Aug 2010
                02 July 2010
                02 July 2010
                : 1
                : 1
                : 24-29
                Affiliations
                [1] aDepartment of Ophthalmology, Hospital Universitari Germans Trias i Pujol, Badalona, Spain
                [2] bSt Paul's Eye Unit, Royal Liverpool University Hospital, Liverpool, UK
                [3] cDepartment of Ophthalmology, Campus Biomedico University, Rome, Italy
                [4] dDepartment of Ophthalmology, S. Sebastiano Hospital, Caserta, Italy
                [5] eDepartment of Ophthalmology, Istituto Clinico Humanitas, Rozzano, Italy
                Author notes
                *Xavier Valldeperas, MD, PhD, FEBO, Department of Ophthalmology, Hospital Universitari Germans Trias i Pujol, Carretera de Canyet s/n, ES–08916 Badalona (Spain), E-Mail xvalldeperas@ 123456gmail.com
                Article
                cop0001-0024
                10.1159/000316919
                2914445
                20737056
                63e6b6cf-8cb1-4b34-be44-ac3e7acf2f02
                Copyright © 2010 by S. Karger AG, Basel

                This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License ( http://creativecommons.org/licenses/by-nc-nd/3.0/). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.

                History
                Page count
                Figures: 3, References: 9, Pages: 6
                Categories
                Published: July 2010

                Ophthalmology & Optometry
                bilateral keratoconus,penetrating keratoplasty,bilateral keratectasia

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