Skip to main content
PMC home page
The British Journal of Ophthalmology logoLink to The British Journal of Ophthalmology
. 1999 Jun;83(6):670–675. doi: 10.1136/bjo.83.6.670

Chronic cortical visual impairment in children: aetiology, prognosis, and associated neurological deficits

R Huo 1, S Burden 1, C Hoyt 1, W Good 1
PMCID: PMC1723072  PMID: 10340973

Abstract

BACKGROUND/AIMS—To evaluate prevalence, aetiology, prognosis, and associated neurological and ophthalmological problems in children with cortical visual impairment (CVI).
METHODS—The records of 7200 outpatients seen in the paediatric ophthalmology practice over the past 15 years were reviewed in order to compile data concerning CVI. In addition, the authors devised and applied a system for grading visual recovery in order to assess prognosis.
RESULTS—CVI occurred in 2.4% of all patients examined. The four most common causes of CVI were perinatal hypoxia (22%), cerebral vascular accident (14%), meningitis (12%), and acquired hypoxia (10%). Most children with CVI had associated neurological abnormalities. The most common were seizures (53%), cerebral palsy (26%) hemiparesis (12%), and hypotonia (5%). Associated ophthalmological problems were esotropia (19%), exotropia (18%), optic nerve atrophy (16%), ocular motor apraxia (15%), nystagmus (11%), and retinal disease (3%). On average, CVI patients improved by two levels as measured by the authors' scale.
CONCLUSION—The majority of children with CVI showed at least some recovery. In this group of children, CVI is often accompanied by additional ophthalmological problems and is nearly always associated with other, serious neurological abnormalities.



Full Text

The Full Text of this article is available as a PDF (102.9 KB).

Figure 1  .

Figure 1  

Open in a new tab

Deficits associated with CVI patients within the study population (n=170).

Figure 2  .

Figure 2  

Open in a new tab

Overall vision outcomes for follow up population (n=96).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Baker-Nobles L., Rutherford A. Understanding cortical visual impairment in children. Am J Occup Ther. 1995 Oct;49(9):899–903. doi: 10.5014/ajot.49.9.899. [DOI] [PubMed] [Google Scholar]
  2. Birch E. E., Bane M. C. Forced-choice preferential looking acuity of children with cortical visual impairment. Dev Med Child Neurol. 1991 Aug;33(8):722–729. doi: 10.1111/j.1469-8749.1991.tb14951.x. [DOI] [PubMed] [Google Scholar]
  3. Casteels I., Demaerel P., Spileers W., Lagae L., Missotten L., Casaer P. Cortical visual impairment following perinatal hypoxia: clinicoradiologic correlation using magnetic resonance imaging. J Pediatr Ophthalmol Strabismus. 1997 Sep-Oct;34(5):297–305. doi: 10.3928/0191-3913-19970901-09. [DOI] [PubMed] [Google Scholar]
  4. Cioni G., Fazzi B., Ipata A. E., Canapicchi R., van Hof-van Duin J. Correlation between cerebral visual impairment and magnetic resonance imaging in children with neonatal encephalopathy. Dev Med Child Neurol. 1996 Feb;38(2):120–132. doi: 10.1111/j.1469-8749.1996.tb12083.x. [DOI] [PubMed] [Google Scholar]
  5. Clarke M. P., Mitchell K. W., Gibson M. The prognostic value of flash visual evoked potentials in the assessment of non-ocular visual impairment in infancy. Eye (Lond) 1997;11(Pt 3):398–402. doi: 10.1038/eye.1997.84. [DOI] [PubMed] [Google Scholar]
  6. Eken P., van Nieuwenhuizen O., van der Graaf Y., Schalij-Delfos N. E., de Vries L. S. Relation between neonatal cranial ultrasound abnormalities and cerebral visual impairment in infancy. Dev Med Child Neurol. 1994 Jan;36(1):3–15. doi: 10.1111/j.1469-8749.1994.tb11760.x. [DOI] [PubMed] [Google Scholar]
  7. Fielder A. R., Evans N. M. Is the geniculostriate system a prerequisite for nystagmus? Eye (Lond) 1988;2(Pt 6):628–635. doi: 10.1038/eye.1988.116. [DOI] [PubMed] [Google Scholar]
  8. Flodmark O., Jan J. E., Wong P. K. Computed tomography of the brains of children with cortical visual impairment. Dev Med Child Neurol. 1990 Jul;32(7):611–620. [PubMed] [Google Scholar]
  9. Foster A. Childhood blindness. Eye (Lond) 1988;2 (Suppl):S27–S36. doi: 10.1038/eye.1988.131. [DOI] [PubMed] [Google Scholar]
  10. Goggin M., O'Keefe M. Childhood blindness in the Republic of Ireland: a national survey. Br J Ophthalmol. 1991 Jul;75(7):425–429. doi: 10.1136/bjo.75.7.425. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Good W. V., Jan J. E., DeSa L., Barkovich A. J., Groenveld M., Hoyt C. S. Cortical visual impairment in children. Surv Ophthalmol. 1994 Jan-Feb;38(4):351–364. doi: 10.1016/0039-6257(94)90073-6. [DOI] [PubMed] [Google Scholar]
  12. Granet D. B., Hertle R. W., Quinn G. E., Breton M. E. The visual-evoked response in infants with central visual impairment. Am J Ophthalmol. 1993 Oct 15;116(4):437–443. doi: 10.1016/s0002-9394(14)71401-1. [DOI] [PubMed] [Google Scholar]
  13. Lambert S. R., Hoyt C. S., Jan J. E., Barkovich J., Flodmark O. Visual recovery from hypoxic cortical blindness during childhood. Computed tomographic and magnetic resonance imaging predictors. Arch Ophthalmol. 1987 Oct;105(10):1371–1377. doi: 10.1001/archopht.1987.01060100073030. [DOI] [PubMed] [Google Scholar]
  14. Mackie R. T., McCulloch D. L., Saunders K. J., Ballantyne J., Day R. E., Bradnam M. S., Dutton G. N. Comparison of visual assessment tests in multiply handicapped children. Eye (Lond) 1995;9(Pt 1):136–141. doi: 10.1038/eye.1995.23. [DOI] [PubMed] [Google Scholar]
  15. Pike M. G., Holmstrom G., de Vries L. S., Pennock J. M., Drew K. J., Sonksen P. M., Dubowitz L. M. Patterns of visual impairment associated with lesions of the preterm infant brain. Dev Med Child Neurol. 1994 Oct;36(10):849–862. doi: 10.1111/j.1469-8749.1994.tb11776.x. [DOI] [PubMed] [Google Scholar]
  16. Rodriguez A., Lozano J. A., del Pozo D., Homar Paez J. Post-traumatic transient cortical blindness. Int Ophthalmol. 1993 Oct;17(5):277–283. doi: 10.1007/BF01007796. [DOI] [PubMed] [Google Scholar]
  17. Rogers M. Vision impairment in Liverpool: prevalence and morbidity. Arch Dis Child. 1996 Apr;74(4):299–303. doi: 10.1136/adc.74.4.299. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Rosenberg T., Flage T., Hansen E., Riise R., Rudanko S. L., Viggosson G., Tornqvist K. Incidence of registered visual impairment in the Nordic child population. Br J Ophthalmol. 1996 Jan;80(1):49–53. doi: 10.1136/bjo.80.1.49. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Schenk-Rootlieb A. J., van Nieuwenhuizen O., van Waes P. F., van der Graaf Y. Cerebral visual impairment in cerebral palsy: relation to structural abnormalities of the cerebrum. Neuropediatrics. 1994 Apr;25(2):68–72. doi: 10.1055/s-2008-1071588. [DOI] [PubMed] [Google Scholar]
  20. Silverman I. E., Galetta S. L., Gray L. G., Moster M., Atlas S. W., Maurer A. H., Alavi A. SPECT in patients with cortical visual loss. J Nucl Med. 1993 Sep;34(9):1447–1451. [PubMed] [Google Scholar]
  21. Sonksen P. M., Petrie A., Drew K. J. Promotion of visual development of severely visually impaired babies: evaluation of a developmentally based programme. Dev Med Child Neurol. 1991 Apr;33(4):320–335. doi: 10.1111/j.1469-8749.1991.tb14883.x. [DOI] [PubMed] [Google Scholar]
  22. Sonksen P. M. The assessment of vision in the preschool child. Arch Dis Child. 1993 Apr;68(4):513–516. doi: 10.1136/adc.68.4.513. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Taylor M. J., McCulloch D. L. Prognostic value of VEPs in young children with acute onset of cortical blindness. Pediatr Neurol. 1991 Mar-Apr;7(2):111–115. doi: 10.1016/0887-8994(91)90006-7. [DOI] [PubMed] [Google Scholar]
  24. Thun-Hohenstein L., Schmitt B., Steinlin H., Martin E., Boltshauser E. Cortical visual impairment following bacterial meningitis: magnetic resonance imaging and visual evoked potentials findings in two cases. Eur J Pediatr. 1992 Oct;151(10):779–782. doi: 10.1007/BF01959090. [DOI] [PubMed] [Google Scholar]
  25. Tsay C. H., Partridge J. C., Villarreal S. F., Good W. V., Ferriero D. M. Neurologic and ophthalmologic findings in children exposed to cocaine in utero. J Child Neurol. 1996 Jan;11(1):25–30. doi: 10.1177/088307389601100106. [DOI] [PubMed] [Google Scholar]
  26. Wong V. C. Cortical blindness in children: a study of etiology and prognosis. Pediatr Neurol. 1991 May-Jun;7(3):178–185. doi: 10.1016/0887-8994(91)90081-u. [DOI] [PubMed] [Google Scholar]
  27. de Sa L., Hoyt C. S., Good W. V. Complications of pediatric ophthalmic surgery. Int Ophthalmol Clin. 1992 Fall;32(4):31–39. doi: 10.1097/00004397-199223000-00003. [DOI] [PubMed] [Google Scholar]
  28. van Hof-van Duin J., Cioni G., Bertuccelli B., Fazzi B., Romano C., Boldrini A. Visual outcome at 5 years of newborn infants at risk of cerebral visual impairment. Dev Med Child Neurol. 1998 May;40(5):302–309. doi: 10.1111/j.1469-8749.1998.tb15381.x. [DOI] [PubMed] [Google Scholar]

Articles from The British Journal of Ophthalmology are provided here courtesy of BMJ Publishing Group

RESOURCES