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


CONCLUSIONS
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.
Andersson Grönlund
M
M
Institute of Neuroscience and Physiology/Ophthalmology, The Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden. marita.gronlund@neuro.gu.se
Dahlgren
J
J
Aring
E
E
Kraemer
M
M
Hellström
A
A
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004596:Electroretinography / Q000379:methods; D005260:Female; D006801:Humans; D008297:Male; D011446:Prospective Studies; D012029:Refraction, Ocular / Q000502:physiology; D056730:Silver-Russell Syndrome / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology; D013285:Strabismus / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D013548:Sweden / Q000453:epidemiology; D014792:Visual Acuity / Q000502:physiology*