Outcomes of keratoplasty in the mucopolysaccharidoses: An international perspective

Kaitlyn L. Ohden, Susanne Pitz, Jane Ashworth, Augusto Magalhães, Diane R. Marinho, Päivi Lindahl, Kristina Teär Fahnehjelm, C. Gail Summers

Research output: Contribution to journalArticlepeer-review

11 Scopus citations

Abstract

Objective: To describe visual outcomes after penetrating keratoplasty and deep anterior lamellar keratoplasty in patients with mucopolysaccharidoses. Methods: This is a retrospective review of keratoplasty in consecutive patients from Brazil, England, Finland, Germany, Portugal, Sweden and the USA. All patients had corneal clouding due to mucopolysaccharidoses. Preoperative and postoperative visual outcome and ocular comorbidities were identified. Success was arbitrarily defined as any improvement in visual acuity or best-corrected visual acuity better than logarithm of the minimum angle of resolution 0.30 (20/40). Statistical analysis included only data from first operated eyes in the 16 patients who underwent bilateral keratoplasty. Results: Forty-eight eyes from 32 patients with mucopolysaccharidoses I, IV or VI are reported. Mean follow-up was 70 months (range: 5-186). Penetrating keratoplasty was performed in 45 eyes and deep anterior lamellar keratoplasty in 3 eyes. At last follow-up, a successful visual outcome for penetrating keratoplasty in first operated/only operated eyes was found in 63%. Rejection episodes occurred in 23% of grafts; however, a clear graft was recorded at last follow-up in 94%. Ocular pathway comorbidities were identified in 63% of eyes transplanted. Conclusions: Clear corneal grafts can be obtained for patients with corneal clouding due to mucopolysaccharidosis with improvement in visual acuity in the majority.

Original languageEnglish (US)
Pages (from-to)909-912
Number of pages4
JournalBritish Journal of Ophthalmology
Volume101
Issue number7
DOIs
StatePublished - Jul 1 2017

Bibliographical note

Publisher Copyright:
© Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Keywords

  • Child health (paediatrics)
  • Cornea
  • Genetics
  • Treatment Surgery
  • Vision

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