Assessment of visual function

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Authors

Ortibus E, Huening B, Wolke D, Khonyongwa-Fernandez G, Cassiman C, Geldof C, Schalij-Delfos N

Click on the image to read the standard in brief.

Target group

Infants born very preterm or those infants with risk factors (see preamble Follow-up & continuing care) and parents

User group

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 (1), 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 to identify those who could benefit from additional support and early intervention.

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. (2,3)Infants who suffered from grade 2 or 3 hypoxic ischaemic encephalopathy or meningoencephalitis have an increased risk of (cerebral) visual impairment (CVI) (7-11% and 17% respectively). (4–6)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 visuomotor, neuropsychological outcome and academic skills such as reading, writing and maths achievement and on quality of life. (7–11)

Benefits

Short-term benefits

In infants with co-morbidities, early assessment and diagnosis could lead to early visual intervention, proactive treatment, and support.

Long-term benefits

Early diagnosis of visual impairment promotes timely interventions (12,13)
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 parental awareness and parent-infant interaction adapted to visual ability (consensus)
Improved academic outcome (14)
Improved social integration and quality of life (3,11)
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, in a timely manner, by healthcare professionals to attend follow-up programme, which includes visual assessments (define ages at which visual follow-up should take place and the provider thereof). (3)

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 treatment and support options 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 by healthcare professionals 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

Regular 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. (2,3,15–17)

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 paediatric ophthalmologist. (2,3,14,17)

A (High quality)
B (High quality)

Guideline

Children with ROP grades 3 and 4 (or treated for any grade of ROP) and/or 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. (18)

A (High quality)

Guideline

Children with clinical suspicion for visual perception dysfunctions are assessed at 3 years of age onwards, based on psychometrically sound assessment tools. (11,19)

A (High quality)

Audit report², guideline

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, and referral and signposting to supportis funded, provided,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 and intervention is specified, provided, 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. (20)

B (Moderate quality)

Families are supported by case manager in order to ensure follow-up programme, which includes visual assessments, and signposting to regular support is provided.

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 ophthalmologist/neuropsychologist specialised in visual perception, to evaluate high-risk children. (3)

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. (15,19)

A (High quality)
B (High quality)

Develop a national network for benchmarking of follow-up quality and provision of care.

B (Moderate quality)

Getting started

Initial steps

For parents and family

Parents are informed as soon as possible, by healthcare professionals about the risks to vision after high-risk birth and about the follow-up programme.

For healthcare professionals

Attend appropriate and regular training on standardised visual assessment.
Establish a structure of communication with other healthcare institutions, who are providing follow-up care.

For neonatal unit and follow-up team

Develop and implement a guideline on follow-up programmes, which include visual assessment.
Develop information material for parents to raise awareness about the importance of visual follow-up assessment.
Establish at least a standardised formal system of keeping track of families.
Develop a structure for local follow-up care.

For hospital and follow-up team

Support healthcare professionals to participate in regular training on standardised visual assessments.
Ensure ophthalmologists are available and regularly trained in visual sequelae of high-risk births.

For health service

Develop and implement a national guideline on follow-up programmes which include 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%). (21)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%. (15) 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 3.5-4 years, following timely treatment. (21) Weight at birth, head circumference at birth and head circumference at 5,5 years seem to be important contributing factors. (22)

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. (23–28) 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 (29) , and is associated with deficiencies in the development of cognition and motor abilities. (15,30,31) 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. (11,15)

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. (32) However, CVI can also emerge in children without evident/overt brain pathology. The clinical profile of visual perceptive deficits can change during childhood. (15)Once CVI is suspected, regular follow-up of visual functioning is therefore advised.

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. In case of suspicion of visual perceptive dysfunctions, screening is feasible in infants.(33) Neuropsychological batteries for diagnosing CVI from the age of 3 years onward have been developed. (34) Furthermore, (inter)national guidelines for diagnosis and referral in case of suspicion of CVI, have recently been published. (35,36) At least those children affected by brain damage, should undergo the described work-up for CVI. 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. Micheletti et al nicely summarised the evidence for early identification of visual dysfunctions, enabling access to early visual intervention, which may support visual and overall development. (13)

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