Ophthalmological findings in children and adolescents with Silver–Russell syndrome. Issue 5 (30th August 2010)

• Record Type: Journal Article
• Authors: Andersson Grönlund, M; Dahlgren, J; Aring, E; Kraemer, M; Hellström, A
• Is Part Of: British journal of ophthalmology. Volume 95:Issue 5(2011)
• Page Start: 637
• Page End: 641
• Publication Date: 2010-08-30
• Subjects: Visual function -- ocular findings -- Silver–Russell syndrome (SRS) -- prenatal programming -- intrauterine growth-retarded (IUGR) -- optic nerve -- vision -- optics and refraction; Ophthalmology -- Periodicals; 617.7
• DOI: 10.1136/bjo.2010.184457
• Languages: English
• ISSNs: 0007-1161

Abstract

Abstract : 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.