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      A novel characterization of posterior keratoconus using anterior segment optical coherence tomography in an infant: a case report

      case-report

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          Abstract

          Background

          Posterior keratoconus is a rare cause of a corneal opacity in an infant. It is characterized by thinning of the posterior cornea without ectasia of the anterior cornea. Imaging of this condition with anterior segment optical coherence tomography (AS-OCT) has not been reported in the literature.

          Case presentation

          A six week old African-American male presented with a congenital corneal opacity of the right eye. He underwent an examination under anesthesia in which photography and AS-OCT were performed. AS-OCT confirmed the diagnosis of posterior keratoconus. The patient subsequently underwent an optical iridectomy for visual development.

          Conclusion

          AS-OCT is a useful tool in cases when a child presents with a corneal opacity of unknown or unclear etiology. In our patient, AS-OCT showed the classic description of central corneal thinning seen in this condition. Additionally, it revealed an associated detached Descemet membrane, a feature which has not been previously described in posterior keratoconus.

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          Most cited references6

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          Posterior keratoconus.

          Posterior keratoconus is a rare corneal disorder characterized by a total or localized noninflammatory thinning of the cornea. In front of a conical protrusion of the posterior corneal curvature, there is a thinned stroma and nonprotruding anterior surface. A corneal button from a patient with bilateral posterior keratoconus was studied by light and electron microscopy. Descemet's membrane, particularly in the area of stromal thinning, demonstrated abnormal anterior banding, a multilaminar configuration, and localized posterior excrescences. These alterations suggest an early pathogenetic mechanism, which probably originated prior to the fifth or sixth month of gestation.
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            Corneal topography of posterior keratoconus.

            Posterior keratoconus is an unusual abnormality of the cornea generally classified as one of the anterior chamber cleavage anomalies. It is characterized clinically by the presence of a circumscribed or generalized corneal thinning with posterior depression of the cornea and is considered distinct from keratoconus. Although patients with posterior keratoconus may have visual complaints clearly related to their abnormal corneas, the surface topography of these corneas has not been studied in detail. Keratometry and photokeratoscopy provide an incomplete picture of the surface geometry of posterior keratoconus. We utilized computer assisted topographic analysis to study the cornea of a patient with posterior keratoconus. The Topographic Modeling System demonstrated that the patient's cornea showed a central steepened "cone" coincident with the area of circumscribed posterior keratoconus as well as paracentral flattening. This report documents the topographic abnormality in this rare disorder.
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              Acquired posterior keratoconus.

              A case of acquired posterior keratoconus is presented. As in developmental cases the vision was moderately reduced and the condition was not progressive. Posterior keratoconus is usually developmental in origin, but trauma has been implicated in some cases.
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                Author and article information

                Contributors
                nisha.garg@med.miami.edu
                t.chang@med.miami.edu
                Bibiana.Reiser@med.usc.edu
                305-326-6324 , kcavuoto@med.miami.edu
                Journal
                BMC Ophthalmol
                BMC Ophthalmol
                BMC Ophthalmology
                BioMed Central (London )
                1471-2415
                4 November 2015
                4 November 2015
                2015
                : 15
                : 158
                Affiliations
                [ ]Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, 900 NW 17th St, Miami, FL 33136 USA
                [ ]Children’s Hospital Los Angeles, 4650 Sunset Blvd., Los Angeles, CA 90027 USA
                [ ]University of Southern California Eye Institute, 1450 San Pablo Street, Los Angeles, CA 90033-4682 USA
                Article
                139
                10.1186/s12886-015-0139-3
                4634598
                26537455
                fe6c8a21-39da-481e-ad45-b3221876fda0
                © Garg et al. 2015

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 3 July 2015
                : 17 October 2015
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2015

                Ophthalmology & Optometry
                posterior keratoconus,corneal opacity,infant,anterior segment optical coherence tomography

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