Abstract
Abstract: :
Purpose: To evaluate ophthalmologic findings in children with severe intrauterine growth retardation e.g Silver Russell Syndrome (SRS). The syndrome is characterized by pre– and postnatal growth retardation, triangular face, micrognathia, lateral asymmetry, clinodactylia and mutations in the 11 and 7 gene are reported. Methods: Eighteen children (8 girls, 10 boys; mean age 11.6 yrs) with SRS were examined by a multidiciplinary team. An ophthalmologic evaluation was performed including visual acuity, refraction, motility and strabismus, slitlamp examination, ophthalmoscopy, axial length measurements, a structured history taking regarding visuoperceptual problems and visual evoked potential (VEP). Data was compared with data for an age– and sex–matched reference group ("ref") of Swedish school children (n= 99). Results: Visual acuity of the best eye was ≤20/60 in one child (ref n=0);n.s, between 20/50 and 20/30 in six children (ref n=1); p<0.0001, and ≥ 20/25 in 11 children (ref n=98); p<0.0001. Three children (ref n=5) were hyperopic (≥2.0 D SE); n.s, four children (ref n=9) were myopic (≥0.5 D SE); n.s. Astigmatism (≥0.75 D) was found in nine children (ref n=25); n.s. Anisometropia (≥1 D) was noted in four children (ref 3); p=0.02. Eight children wore glasses (ref n=6); p=0.0002. 3/16 children (ref n=3) had strabismus; n.s. Six children had small optic discs and six had increased tortuosity of their retinal vessels. The total axial length in both right and left eye was shorter compare with controls (p=0.006; p<0.0001). Signs of visuoperceptual problems were recorded in 38% (ref 4%); p=0.0009. The VEP was normal in 4/14 children. The pathological changes showed side asymmetries (albinism, n=2; albinoic changes, n=3), poor cortical activation after monocular stimulation (n=3) and delayed latencies (n=2). Conclusions: Children with SRS which is an extremly group of children born small for gestatinal age (SGA), show signs of subnormal visual acuity, a high number of refractive errors, anisometropia, short eyes, visuoperceptual problems and pathological changes registered by VEP. On basis of our findings, we recommend that an ophthalmologic examination should be performed in children with severe intrauterine growth retardation.
Keywords: clinical (human) or epidemiologic studies: natural history • electrophysiology: clinical • perception