Literature DB >> 10632018

Management of oculus fragilis in Ehlers-Danlos type VI.

M S Macsai1, H L Lemley, T Schwartz.   

Abstract

PURPOSE: To describe an alternative, novel surgical approach to the repair of a ruptured globe in the second reported patient with keratoglobus, Ehlers-Danlos type VI, and normal lysyl hydroxylase.
METHODS: Under general anesthesia, a 360 degrees conjunctival peritomy was performed and the epithelium was removed from the keratoglobus cornea. Descemet's membrane and endothelium were removed from a fresh donor corneoscleral ring. An onlay epikeratoplasty was then performed and the entire donor corneoscleral button was secured to the sclera with 9-0 nylon suture. The conjunctiva was brought into position and tacked down over the edge of the donor graft. After 4 months, a full-thickness penetrating keratoplasty was then performed.
RESULTS: The patient ultimately had a ruptured globe in the fellow eye and thus required the identical procedure OU. Both eyes were successfully repaired with a final visual acuity OU of 20/100.
CONCLUSION: The described surgical approach allowed successful surgical repair of bilateral ruptured globes in a patient with keratoglobus, Ehlers-Danlos type VI, and normal lysyl hydroxylase levels. In the previous report of such a patient by Judisch et al. (1), the attempt at surgical repair was unsuccessful and ended in enucleation. The described surgical technique may be used to treat advanced keratoglobus or oculus fragilis.

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Year:  2000        PMID: 10632018     DOI: 10.1097/00003226-200001000-00020

Source DB:  PubMed          Journal:  Cornea        ISSN: 0277-3740            Impact factor:   2.651


  8 in total

1.  A novel technique to treat traumatic corneal perforation in a case of presumed brittle cornea syndrome.

Authors:  Hussin Mohamed Hussin; Suman Biswas; Mohamed Majid; Richard Haynes; Derek Tole
Journal:  Br J Ophthalmol       Date:  2007-03       Impact factor: 4.638

Review 2.  Keratoglobus.

Authors:  B S Wallang; S Das
Journal:  Eye (Lond)       Date:  2013-06-28       Impact factor: 3.775

3.  Unusual case of globe perforation: the brittle cornea without systemic manifestations.

Authors:  Shilpa Ajit Joshi; Shalomith Uppapalli; Pranav More; Madan Deshpande
Journal:  BMJ Case Rep       Date:  2016-10-07

4.  [Brittle cornea syndrome type 1 caused by compound heterozygosity of two mutations in the ZNF469 gene].

Authors:  Johannes Menzel-Severing; Ralph Meiller; Cornelia Kraus; Regina Trollmann; Deniz Atalay
Journal:  Ophthalmologe       Date:  2019-08       Impact factor: 1.059

Review 5.  Brittle cornea syndrome: recognition, molecular diagnosis and management.

Authors:  Emma M M Burkitt Wright; Louise F Porter; Helen L Spencer; Jill Clayton-Smith; Leon Au; Francis L Munier; Sarah Smithson; Mohnish Suri; Marianne Rohrbach; Forbes D C Manson; Graeme C M Black
Journal:  Orphanet J Rare Dis       Date:  2013-05-04       Impact factor: 4.123

6.  Surgical Management of Corneal Hydrops: Case Series

Authors:  Gökçen Özcan; Ömür Özlenen Uçakhan
Journal:  Turk J Ophthalmol       Date:  2022-02-23

7.  Collagen V insufficiency in a mouse model for Ehlers Danlos-syndrome affects viscoelastic biomechanical properties explaining thin and brittle corneas.

Authors:  Sabine Kling; Emilio A Torres-Netto; Hormoz Abdshahzadeh; Edgar M Espana; Farhad Hafezi
Journal:  Sci Rep       Date:  2021-08-30       Impact factor: 4.379

8.  Brittle cornea syndrome: current perspectives.

Authors:  Andrew Walkden; Emma Burkitt-Wright; Leon Au
Journal:  Clin Ophthalmol       Date:  2019-08-12
  8 in total

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