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The Multidisciplinary Guidelines for Diagnosis and Referral in Cerebral Visual Impairment
Frouke N Boonstra1,2,3, Daniëlle G M Bosch4, Christiaan J A Geldof1
1Royal Dutch Visio, National Foundation for the Visually Impaired and Blind, Huizen, Netherlands.
Insights
Guidelines for diagnosing cerebral visual impairment (CVI) in children were developed using the GRADE method. This research provides evidence-based diagnostic tools and referral criteria for CVI, improving early detection and management.
Area of Science:
- Ophthalmology
- Neurology
- Pediatrics
- Genetics
Background:
- Cerebral visual impairment (CVI) is a leading cause of vision loss in children, often linked to perinatal hypoxic-ischemic damage, genetic disorders, cerebral palsy, or developmental delay.
- Accurate and timely diagnosis of CVI is critical for effective management, yet consensus on evidence-based diagnostic tools and referral criteria is lacking.
- Existing diagnostic approaches for CVI require standardization to ensure consistency and reliability in clinical practice.
Purpose of the Study:
- To develop evidence-based guidelines for the diagnosis and referral of children with cerebral visual impairment (CVI) using the GRADE (Grading of Recommendations Assessment, Development and Evaluation) method.
- To establish consensus on diagnostic tools and referral criteria for CVI to facilitate early and accurate identification.
- To provide a structured framework for clinicians managing children suspected of having CVI.
Main Methods:
- Systematic literature searches were conducted across Medline, Embase, and Psychinfo databases, focusing on five key topics related to CVI in children up to 18 years of age.
- Articles were selected based on evidence related to medical history and questionnaires, ophthalmological and orthoptic assessments, neuropsychological evaluations, neuroradiological imaging (including MRI), and genetic assessments.
- The GRADE methodology was applied to synthesize evidence and develop recommendations for CVI diagnosis and referral.
Main Results:
- Key historical factors for CVI include prematurity, low birth weight, and low APGAR scores. Specific questionnaires are recommended for different age groups and risk categories.
- Ophthalmological assessments should evaluate eye movements (saccades), accommodation, crowding, contrast sensitivity, and visual fields. OCT and eye-tracking can provide objective measures.
- MRI findings in CVI associated with cerebral palsy can be categorized into five groups, with lesion severity correlating to visual impairment. Genetic and neurological assessments may be necessary.
Conclusions:
- The developed guidelines provide a structured, evidence-based approach to diagnosing and referring children with CVI, addressing the need for consensus on diagnostic tools.
- Comprehensive evaluation integrating medical history, ophthalmological findings, neuroimaging, and potentially genetic testing is crucial for accurate CVI diagnosis and etiology determination.
- These guidelines aim to improve the consistency and quality of CVI care, ultimately benefiting children with visual impairment due to neurological conditions.
Introduction:
Cerebral visual impairment (CVI) is an important cause of visual impairment in western countries. Perinatal hypoxic-ischemic damage is the most frequent cause of CVI but CVI can also be the result of a genetic disorder. The majority of children with CVI have cerebral palsy and/or developmental delay. Early diagnosis is crucial; however, there is a need for consensus on evidence based diagnostic tools and referral criteria. The aim of this study is to develop guidelines for diagnosis and referral in CVI according to the grade method.
Patients And Methods:
We developed the guidelines according to the GRADE method 5 searches on CVI (children, developmental age ≤ 18 years) were performed in the databases Medline, Embase, and Psychinfo, each with a distinct topic.
Results:
Based on evidence articles were selected on five topics: 1. Medical history and CVI-questionnaires 23 (out of 1,007). 2. Ophthalmological and orthoptic assessment 37 (out of 816). 3. Neuropsychological assessment 5 (out of 716). 4. Neuroradiological evaluation and magnetic resonance imaging (MRI) 9 (out of 723). 5. Genetic assessment 5 (out of 458).
Conclusion:
In medical history taking, prematurity low birth weight and APGAR (Appearance, Pulse, Grimace, Activity, Respiration) Scores (<5) are important. Different questionnaires are advised for children under the age of 3 years, older children and for specific risk groups (extremely preterm). In ophthalmological examination, eye movements, specially saccades, accommodation, crowding, contrast sensitivity and visual fields should be evaluated. OCT can show objective signs of trans-synaptic degeneration and abnormalities in fixation and saccades can be measured with eye tracking. Screening of visual perceptive functioning is recommended and can be directive for further assessment. MRI findings in CVI in Cerebral Palsy can be structured in five groups: Brain maldevelopment, white and gray matter lesions, postnatal lesions and a normal MRI. In children with CVI and periventricular leukomalacia, brain lesion severity correlates with visual function impairment. A differentiation can be made between cortical and subcortical damage and related visual function impairment. Additional assessments (neurological or genetic) can be necessary to complete the diagnosis of CVI and/or to reveal the etiology.
