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Outcomes of keratoplasty in the mucopolysaccharidoses: an international perspective
  1. Kaitlyn L Ohden1,
  2. Susanne Pitz2,
  3. Jane Ashworth3,
  4. Augusto Magalhães4,
  5. Diane R Marinho5,
  6. Päivi Lindahl6,
  7. Kristina Teär Fahnehjelm7,
  8. C Gail Summers8
  1. 1Medical School, University of Minnesota, Minneapolis, Minnesota, USA
  2. 2Department of Ophthalmology, University Medical Center, Johnannes Gutenberg-University, Mainz, Germany
  3. 3Department of Ophthalmology, Manchester Royal Eye Hospital, Manchester, UK
  4. 4Department of Ophthalmology, S. João Hospital, Porto, Portugal
  5. 5Department of Ophthalmology, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil
  6. 6Helsinki University Eye Hospital, Helsinki, Finland
  7. 7Department of Clinical Neuroscience and Rehabilitation, Institute of Neuroscience and Physiology, University of Gothenburg St. Erik Eye Hospital, Stockholm, Sweden
  8. 8Departments of Ophthalmology & Visual Neurosciences and Pediatrics, University of Minnesota, Minneapolis, Minnesota, USA
  1. Correspondence to Dr C Gail Summers, Departments of Ophthalmology & Visual Neurosciences and Pediatrics, University of Minnesota, 701 25th Ave. S., #300, Minneapolis, MN 55454, USA; summe001{at}


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.

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

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