Kaitlyn L Ohden1, Susanne Pitz2, Jane Ashworth3, Augusto Magalhães4, Diane R Marinho5, Päivi Lindahl6, Kristina Teär Fahnehjelm7, C Gail Summers8. 1. Medical School, University of Minnesota, Minneapolis, Minnesota, USA. 2. Department of Ophthalmology, University Medical Center, Johnannes Gutenberg-University, Mainz, Germany. 3. Department of Ophthalmology, Manchester Royal Eye Hospital, Manchester, UK. 4. Department of Ophthalmology, S. João Hospital, Porto, Portugal. 5. Department of Ophthalmology, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil. 6. Helsinki University Eye Hospital, Helsinki, Finland. 7. Department of Clinical Neuroscience and Rehabilitation, Institute of Neuroscience and Physiology, University of Gothenburg St. Erik Eye Hospital, Stockholm, Sweden. 8. Departments of Ophthalmology & Visual Neurosciences and Pediatrics, University of Minnesota, Minneapolis, Minnesota, USA.
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. 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/.
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. 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/.
Entities:
Keywords:
Child health (paediatrics); Cornea; Genetics; Treatment Surgery; Vision
Authors: Mehmet Umut Akyol; Tord D Alden; Hernan Amartino; Jane Ashworth; Kumar Belani; Kenneth I Berger; Andrea Borgo; Elizabeth Braunlin; Yoshikatsu Eto; Jeffrey I Gold; Andrea Jester; Simon A Jones; Cengiz Karsli; William Mackenzie; Diane Ruschel Marinho; Andrew McFadyen; Jim McGill; John J Mitchell; Joseph Muenzer; Torayuki Okuyama; Paul J Orchard; Bob Stevens; Sophie Thomas; Robert Walker; Robert Wynn; Roberto Giugliani; Paul Harmatz; Christian Hendriksz; Maurizio Scarpa Journal: Orphanet J Rare Dis Date: 2019-06-13 Impact factor: 4.123
Authors: Liujiang Song; Jacquelyn J Bower; Telmo Llanga; Jacklyn H Salmon; Matthew L Hirsch; Brian C Gilger Journal: Mol Ther Methods Clin Dev Date: 2020-05-22 Impact factor: 6.698