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.
- Visual function
- ocular findings
- Silver–Russell syndrome (SRS)
- prenatal programming
- intrauterine growth-retarded (IUGR)
- optic nerve
- optics and refraction
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Funding This study was supported by the Gothenburg Medical Society, the W & M Lundgrens Vetenskapsfond II, the Sven Jerring Foundation, the Swedish Research Society (grant #10863 and grant 522-2005-7238), and Research and Development of Region Västra Götaland (grant 2002–2004).
Competing interests None.
Patient consent Obtained.
Ethics approval This study was conducted with the approval of the Ethical Committee at the Medical Faculty, Sahlgrenska Academy at the University of Gothenburg, Sweden.
Provenance and peer review Not commissioned; externally peer reviewed.