Healthcare professionals, neonatal units, hospitals, follow-up teams, and health services
Statement of standard
Standardised visual assessment is conducted by age 3.5 to 4 years and repeated by age 5 to 6, at which age additional attention is payed to visual information processing dysfunctions.
Rationale
The goal is to assess and evaluate the development of visual and visual information processing functions in order to identify those who could benefit from additional support. Preterm born infants have an increased risk of visual dysfunctions, in particular those with severe brain injury and those who suffered from severe and/or treated retinopathy of prematurity (ROP). Long-term follow-up showed that an adverse ophthalmological outcome (AOO) (reduced acuity, strabismus, high myopia, colour defect, field defect and/or subnormal contrast sensitivity) is present in 25-50% of preterm infants with a birth weight <1500 g. (1,2) Infants who suffered from grade 2 or 3 hypoxic ischaemic encephalopathy or meningoencephalitis have an increased risk of (cerebral) visual impairment (7-11% and 17% respectively). (3,4) Impairments include dysfunctions in visual sensory, oculomotor and perceptive (such as object recognition and spatial processing) functioning. Both visual sensory and visual perceptive dysfunctions exert a negative effect on neuropsychological outcome and academic skills such as reading, writing and maths achievement. (5–8)
Severe visual sensory and oculomotor deficits mostly become visible at early ages. However, visual screening is most reliable at the age of 3.5 to 4 years. At 5 to 6 years, most visual sensory and oculomotor problems have become apparent. If there is suspicion of visual perceptive dysfunctions, standardised examinations can be done from 5 years of age onwards.
Refractive error can often be corrected. Strabismic amblyopia needs to be corrected at an early stage with patching. The treatment or support of visual perceptual deficits, aims to offer the child the best environment to improve its visual functioning and to learn strategies to cope with its specific deficits.
Benefits
Short-term benefits
N/A
Long-term benefits
Early diagnosis of visual impairment promotes timely interventions (9)
Promotes realistic expectations in those with severe impairment (consensus)
Improved decision making for schooling and learning support (consensus)
Provides feedback to perinatal and neonatal services and healthcare officials (consensus)
Reduced risk of misdiagnoses (e.g. reading difficulties) (consensus)
Improved parent-infant interaction adapted to visual ability (consensus)
Improved academic outcome (10)
Improved social integration and quality of life (2)
Reduced social burden and costs (consensus)
Components of the standard
Component
Grading of evidence
Indicator of meeting the standard
For parents and family
Parents are informed about and invited by healthcare professionals to attend follow-up programme including visual assessments (including ages at which visual follow-up takes place and the provider thereof). (2)
A (High quality) B (High quality)
Patient information sheet1
Parents receive standardised feedback about the results of their child’s visual health screening in a language that is accessible to them.
B (High quality)
Parent feedback
Parents are informed about the need for early intervention and support in case of visual impairments.
B (High quality)
Patient information sheet1
Parents are asked for permission to allow their child’s medical and educational information to be used for outcome measures.
B (Low quality)
Parent consent, patient information sheet1
Parents are asked to consent to share the results of their child’s visual screening tests with education services.
B (Moderate quality)
Parent consent
For healthcare professionals
A guideline on follow-up programme including visual assessment is adhered to by all healthcare professionals.
B (High quality)
Guideline
Training on standardised visual assessment in high-risk infants in which gestational age, ROP status, and brain damage are taken into account is attended by all responsible healthcare professionals. (1,2,11–13)
A (High quality) B (High quality)
Training documentation
Children with ROP grade ≤2 undergo ophthalmologic screening at 3.5-4 years and assessment of visual acuity at 4-5 years; at younger ages, children with signs of adverse visual development are referred directly to the ophthalmologist. (1,2,10,13)
A (High quality) B (High quality)
Guideline
Children with ROP grades 3 and 4 (or treated for any grade of ROP) and with severe brain damage have regular follow-up assessments at the discretion of the ophthalmologist and are at least screened for strabismus and refractive errors at 12 months. (14)
A (High quality)
Guideline
Children with clinical suspicion for visual perception dysfunctions are assessed at 5 years of age onwards. (15)
A (High quality)
Audit report2
For neonatal unit, hospital and follow-up team
A guideline on follow-up programme including visual assessment is available and regularly updated.
B (High quality)
Guideline
A follow-up programme after discharge including visual assessment is funded and supported.
B (Moderate quality)
Audit report2
Training on standardised visual assessment in high-risk infants is ensured.
B (High quality)
Training documentation
For health service
A national guideline on follow-up programme including visual assessment is available and regularly updated.
B (High quality)
Guideline
A follow-up service including visual assessment is specified, funded and monitored.
B (Moderate quality)
Audit report2
1The indicator “patient information sheet” is an example for written, detailed information, in which digital solutions are included, such as web-based systems, apps, brochures, information leaflets, and booklets.
2The indicator “audit report” can also be defined as a benchmarking report.
Where to go
Further development
Grading of evidence
For parents and family
Offer visual follow-up until adult age. (16)
B (Moderate quality)
Families are supported by case manager in order to ensure follow-up programme including visual assessments.
B (High quality)
For healthcare professionals
N/A
For neonatal unit and follow-up team
Establish an integrated electronic system with follow-up provider to schedule follow-up visits.
B (Moderate quality)
For hospital and follow-up team
Establish multidisciplinary teams, including opthalmologist/neuropsychologist specialised in visual perception, to evaluate high-risk children. (2)
B (Moderate quality)
For health service
Support the development of reliable and valid instruments to assess cerebral visual deficits with country specific norms and facilitate differential diagnosis. (11,15)
A (High quality) B (High quality)
Develop a national network for benchmarking of follow-up quality.
B (Moderate quality)
Getting started
Initial steps
For parents and family
Parents are informed by healthcare professionals about the risks to vision after high-risk birth and about the follow-up programme.
For healthcare professionals
Attend appropriate training on standardised visual assessment.
Establish a structure of communication with other healthcare institutions, providing follow-up care.
For neonatal unit and follow-up team
Develop and implement a guideline on follow-up programme including visual assessment.
Develop information material about importance of visual follow-up assessment for parents.
Establish at least a formal system of keeping track of families.
Develop a structure of follow-up locally.
For hospital and follow-up team
Support healthcare professionals to participate in training on standardised visual assessments.
Ensure ophthalmologists are available and trained in visual sequelae of high-risk births.
For health service
Develop and implement a national guideline on follow-up programme including visual assessment.
Make a policy decision that visual follow-up services is standard of care for all infants.
Retinopathy of prematurity (ROP) is an important cause of visual impairment in the preterm infant, and is due to disorganized vascular development of the retina usually after retinal ischaemia consequent to oxygen exposure. Infants who develop ROP are at increased risk of ophthalmological deficits such as refractive error (up to 64%), amblyopia and strabismus (36-44%). (17) However, these disorders are also prevalent in those born under 32 weeks without ROP, in whom refractive errors are present in 26% of infants, amblyopia in 21% and strabismus in 16-20%. (11) In preterm children attending mainstream school, decreased visual acuity was reported to occur two to three times more frequently than in term-born peers, principally due to refractive errors. High myopia and anisometropia, in particular, confer a risk for developing amblyopia and strabismus. Such early reductions of visual acuity are reportedly subject to “catch-up” by age 5 years, following timely treatment. (17) Weight at birth, head circumference at birth and head circumference at 5,5 years seem to be important contributing factors. (18)
Premature infants are born in a phase of rapid brain growth and organisation. Alterations of brain development have been shown in the neonatal period but can last into adulthood, both in structure, altered networks and function, also in the visual areas of the brain. (19–24) Visual impairments caused by adverse brain development are collectively referred to as cerebral visual impairment (CVI) and include both visual sensory impairment and deficient visual perception. CVI nowadays is the most frequent cause of visual impairment in children in developed countries, in contrast to the visual sequelae of ROP (25) , and is associated with deficiencies in the development of cognition and motor abilities. (11,26,27) CVI covers a wide range of deficits, from children merely suffering from spatial processing dysfunctions to deficits in object recognition and scene identification, and also cortically blind children, having no visual perception at all. (11)
In preterm born children, CVI is typically diagnosed in children with periventricular white matter disease, thus particularly in those born <32 weeks of gestation, although its prevalence is not exactly known. (28) However, CVI can also emerge in children without evident/overt brain pathology. The clinical profile of visual perceptive deficits can change during childhood. (11) Once CVI is suspected, regular follow-up of visual functioning is therefore advised.
Stephenson T, Wright S, O’Connor A, Fielder A, Johnson A, Ratib S, et al. Children born weighing less than 1701 g: visual and cognitive outcomes at 11-14 years. Arch Dis Child Fetal Neonatal Ed. 2007 Jul;92(4):F265-270.
Holmström G, Larsson E. Long-term follow-up of visual functions in prematurely born children–a prospective population-based study up to 10 years of age. J AAPOS Off Publ Am Assoc Pediatr Ophthalmol Strabismus. 2008 Apr;12(2):157–62.
Stevens JP, Eames M, Kent A, Halket S, Holt D, Harvey D. Long term outcome of neonatal meningitis. Arch Dis Child Fetal Neonatal Ed. 2003 May;88(3):F179-184.
Azzopardi D, Strohm B, Marlow N, Brocklehurst P, Deierl A, Eddama O, et al. Effects of hypothermia for perinatal asphyxia on childhood outcomes. N Engl J Med. 2014 Jul 10;371(2):140–9.
Molloy CS, Di Battista AM, Anderson VA, Burnett A, Lee KJ, Roberts G, et al. The contribution of visual processing to academic achievement in adolescents born extremely preterm or extremely low birth weight. Child Neuropsychol J Norm Abnorm Dev Child Adolesc. 2017 Apr;23(3):361–79.
Rapin I. Dyscalculia and the Calculating Brain. Pediatr Neurol. 2016;61:11–20.
Downie ALS, Jakobson LS, Frisk V, Ushycky I. Periventricular brain injury, visual motion processing, and reading and spelling abilities in children who were extremely low birthweight. J Int Neuropsychol Soc JINS. 2003 Mar;9(3):440–9.
Beligere N, Perumalswamy V, Tandon M, Mittal A, Floora J, Vijayakumar B, et al. Retinopathy of prematurity and neurodevelopmental disabilities in premature infants. Semin Fetal Neonatal Med. 2015 Oct;20(5):346–53.
Chavda S, Hodge W, Si F, Diab K. Low-vision rehabilitation methods in children: a systematic review. Can J Ophthalmol J Can Ophtalmol. 2014 Jun;49(3):e71-73.
Holmström G, Larsson E. Outcome of retinopathy of prematurity. Clin Perinatol. 2013 Jun;40(2):311–21.
Ortibus EL, De Cock PP, Lagae LG. Visual perception in preterm children: what are we currently measuring? Pediatr Neurol. 2011 Jul;45(1):1–10.
Ricci D, Romeo DM, Gallini F, Groppo M, Cesarini L, Pisoni S, et al. Early visual assessment in preterm infants with and without brain lesions: correlation with visual and neurodevelopmental outcome at 12 months. Early Hum Dev. 2011 Mar;87(3):177–82.
Hellström A, Källén K, Carlsson B, Holmström G, Jakobsson P, Lundgren P, et al. Extreme prematurity, treated retinopathy, bronchopulmonary dysplasia and cerebral palsy are significant risk factors for ophthalmological abnormalities at 6.5 years of age. Acta Paediatr Oslo Nor 1992. 2018 May;107(5):811–21.
AMERICAN ACADEMY OF PEDIATRICS Section on Ophthalmology, AMERICAN ACADEMY OF OPHTHALMOLOGY, AMERICAN ASSOCIATION FOR PEDIATRIC OPHTHALMOLOGY AND STRABISMUS, AMERICAN ASSOCIATION OF CERTIFIED ORTHOPTISTS. Screening Examination of Premature Infants for Retinopathy of Prematurity. PEDIATRICS. 2013 Jan 1;131(1):189–95.
Geldof CJA, van Wassenaer-Leemhuis AG, Dik M, Kok JH, Oosterlaan J. A functional approach to cerebral visual impairments in very preterm/very-low-birth-weight children. Pediatr Res. 2015 Aug;78(2):190–7.
Darlow BA, Elder MJ, Kimber B, Martin J, Horwood LJ. Vision in former very low birthweight young adults with and without retinopathy of prematurity compared with term born controls: the NZ 1986 VLBW follow-up study. Br J Ophthalmol. 2017 Dec 6;
Fierson WM, American Academy of Pediatrics Section on Ophthalmology, American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Association of Certified Orthoptists. Screening examination of premature infants for retinopathy of prematurity. Pediatrics. 2013 Jan;131(1):189–95.
Raffa L, Aring E, Dahlgren J, Karlsson A-K, Andersson Grönlund M. Ophthalmological findings in relation to auxological data in moderate-to-late preterm preschool children. Acta Ophthalmol (Copenh). 2015 Nov;93(7):635–41.
Brumbaugh JE, Conrad AL, Lee JK, DeVolder IJ, Zimmerman MB, Magnotta VA, et al. Altered brain function, structure, and developmental trajectory in children born late preterm. Pediatr Res. 2016;80(2):197–203.
Groppo M, Ricci D, Bassi L, Merchant N, Doria V, Arichi T, et al. Development of the optic radiations and visual function after premature birth. Cortex. 2014 Jul 1;56:30–7.
Kelly CE, Cheong JLY, Molloy C, Anderson PJ, Lee KJ, Burnett AC, et al. Neural Correlates of Impaired Vision in Adolescents Born Extremely Preterm and/or Extremely Low Birthweight. PLOS ONE. 2014 Mar 24;9(3):e93188.
Pavaine J, Young JM, Morgan BR, Shroff M, Raybaud C, Taylor MJ. Diffusion tensor imaging-based assessment of white matter tracts and visual-motor outcomes in very preterm neonates. Neuroradiology. 2016 Mar;58(3):301–10.
Ramenghi LA, Ricci D, Mercuri E, Groppo M, De Carli A, Ometto A, et al. Visual performance and brain structures in the developing brain of pre-term infants. Early Hum Dev. 2010 Jul;86 Suppl 1:73–5.
Thompson DK, Thai D, Kelly CE, Leemans A, Tournier J-D, Kean MJ, et al. Alterations in the optic radiations of very preterm children-Perinatal predictors and relationships with visual outcomes. NeuroImage Clin. 2014;4:145–53.
Bunce C, Xing W, Wormald R. Causes of blind and partial sight certifications in England and Wales: April 2007-March 2008. Eye Lond Engl. 2010 Nov;24(11):1692–9.
Geldof CJA, van Hus JWP, Jeukens-Visser M, Nollet F, Kok JH, Oosterlaan J, et al. Deficits in vision and visual attention associated with motor performance of very preterm/very low birth weight children. Res Dev Disabil. 2016 Jul;53–54:258–66.
Geldof CJA, van Wassenaer AG, de Kieviet JF, Kok JH, Oosterlaan J. Visual perception and visual-motor integration in very preterm and/or very low birth weight children: a meta-analysis. Res Dev Disabil. 2012 Apr;33(2):726–36.
Dutton GN, McKillop ECA, Saidkasimova S. Visual problems as a result of brain damage in children. Br J Ophthalmol. 2006 Aug;90(8):932
November 2018 / 1st edition / next revision: 2023
Recommended citation
EFCNI, Ortibus E, Leemhuis AG et al., European Standards of Care for Newborn Health: Assessment of visual function. 2018.
