Ophthalmological findings in children and adolescents with Silver Russell Syndrome

Marita Andersson Gronlund (1) , Jovanna Dahlgren (2) , Eva Aring (1) , Maria Kraemer (1) , Ann Hellstrom (1)

1 Institute of Neuroscience and Physiology/Ophthalmology, The Sahlgrenska Academy at the University of
2 Institute for the Health of Women and Children, Gothenburg Paediatric Growth Research Centre (GP-GRC

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 (≥1D) 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 intra-uterine growth-retarded, show significant ophthalmological abnormalities. Based on our findings, we recommend ophthalmological examination in children with SRS.

Mots clés: Optic Nerve; Vision; Optics and Refraction

Marita Andersson Gronlund, Jovanna Dahlgren, Eva Aring, Maria Kraemer, Ann Hellstrom. Ophthalmological findings in children and adolescents with Silver Russell Syndrome. British Journal of Ophthalmology, 2010, 95 (5), pp.637. ⟨10.1136/bjo.2010.184457⟩. ⟨hal-00588358⟩