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Original article
Ophthalmological findings in children and adolescents with Silver–Russell syndrome
  1. M Andersson Grönlund1,
  2. J Dahlgren2,
  3. E Aring1,
  4. M Kraemer1,
  5. A Hellström1
  1. 1Institute of Neuroscience and Physiology/Ophthalmology, The Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden
  2. 2Institute for the Health of Women and Children, Gothenburg Paediatric Growth Research Centre (GP-GRC), The Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden
  1. Correspondence to Marita Andersson Grönlund, Department of Paediatric Ophthalmology, The Queen Silvia Children's Hospital, Sahlgrenska University Hospital/Östra, SE 416 85 Gothenburg, Sweden; marita.gronlund{at}neuro.gu.se

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.

  • Visual function
  • ocular findings
  • Silver–Russell syndrome (SRS)
  • prenatal programming
  • intrauterine growth-retarded (IUGR)
  • optic nerve
  • vision
  • optics and refraction

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Footnotes

  • 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.

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