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Ophthalmological findings in children and adolescents with Silver-Russell syndrome.

Journal article
Authors Marita Andersson Grönlund
Jovanna Dahlgren
Eva Aring
Maria Kraemer
Ann Hellström
Published in The British journal of ophthalmology
Volume 95
Issue 5
Pages 637-41
ISSN 1468-2079
Publication year 2011
Published at Institute of Neuroscience and Physiology, Department of Clinical Neuroscience and Rehabilitation
Institute of Clinical Sciences, Department of Pediatrics
Pages 637-41
Language en
Keywords Adolescent, Child, Child, Preschool, Electroretinography, methods, Female, Humans, Male, Prospective Studies, Refraction, Ocular, physiology, Silver-Russell Syndrome, complications, epidemiology, physiopathology, Strabismus, diagnosis, etiology, physiopathology, Sweden, epidemiology, Visual Acuity, physiology
Subject categories Medical and Health Sciences


Aim To evaluate ophthalmological findings in children with Silver-Russell syndrome (SRS). Methods An ophthalmological evaluation including visual acuity (VA), refraction, strabismus, near point of convergence (NPC), slit-lamp examination, ophthalmoscopy, axial length measurements and full-field electroretinogram was performed on 18 children with SRS (8 girls, 10 boys; mean age 11.6 years). Fundus photographs were taken for digital image analysis. Data were compared with data on an age- and gender-matched reference group (ref) of school children (n=99). Results Seventeen out of 18 children with SRS had ophthalmological abnormalities. Best corrected VA of the best eye was <0.1 log of the minimal angle of resolution in 11 children (ref n=98) (p<0.0001), and 11 children had refractive errors (ref n=33) (p=0.05). Anisometropia (>/=1 dioptre) was noted in three of the children (ref n=3) (p=0.046). Subnormal stereo acuity and NPC were found in 2/16 (ref=0) (p=0.02). The total axial length in both eyes was shorter compared with that in controls (p<0.006 and p<0.001). Small optic discs were found in 3/16, large cup in 3/16 and increased tortuosity of retinal vessels in 4/13 children with SRS. Conclusion Children with SRS, who are severely intrauterine growth retarded, show significant ophthalmological abnormalities. Based on the present findings, ophthalmological examination is recommended in children with SRS.

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