Australian children with cerebral visual impairment: using what we know now to improve future approaches.

PURPOSE: Little has been reported on Australian children with Cerebral Vision Impairment (CVI). This paper aims to present the outcome of an audit focussed on children with the primary diagnosis of CVI, using findings from the Australian Childhood Vision Impairment Register (ACVIR). METHODS: Records on 132 children (49% girls, 51% boys) from ACVIR data gathered from both the child's parent/guardian and their eye health professional were reviewed. The child's demographics, level of vision impairment, birth history, diagnostic journey, secondary ocular diagnoses, comorbidities and low vision support were analyzed. Several correlations were investigated using a Kendall's tau-b analysis including the relationship between vision and age of diagnosis; level of vision and developmental delay; and age of suspicion of visual impairment and age of diagnosis. RESULTS: The most common level of visual impairment was blindness (39%), and most children were suspected and diagnosed of visual impairment in the first 6 months of life. The majority of children were born full term (72%), weighing >2000gms (84%). Nearly half of the cohort of children (48%) had a secondary ocular diagnosis with 44% having nystagmus. The majority of children (80%) had additional health problems, and 85% of children had additional disabilities, with 79% having developmental delay. CONCLUSION: While the findings of this audit cannot be generalized to a wider population of Australian children with CVI, the outcome encourages continued discussion on CVI, to explore comprehensive assessment approaches which facilitate timely and accurate diagnosis.

Reporting on Australian childhood visual impairment: the first 10 years.

BACKGROUND: Visual impairment is rare but has significant impact on the neurobehavioural development and quality of life of children. This paper presents the key findings from the Australian Childhood Vision Impairment Register, which commenced in 2008 to report on children diagnosed with permanent visual impairment. SUBJECTS/METHODS: Families consent to completing a data form related to their child and for contact with the child's ophthalmologist. Ophthalmologists complete and return a comprehensive data form on the child's primary and secondary ocular diagnoses, associated disabilities and health conditions, visual acuity and visual fields. Data is stored on a secure database and anonymised data is available to researchers and for planning purposes. RESULTS: Nine-hundred four children and their families provided informed consent for participation, with 57% males and 43% females. Most children spoke English in their home. Eighty-three percent of children were born full term, with a birth weight of >2500 g (81%). Children were commonly suspected to have visual impairment by a parent, with 68% of families receiving a diagnosis of visual impairment by their child's first birthday. The most common primary diagnoses were retinal dystrophy (17%), CVI (15%) and Albinism (11%). A secondary diagnosis of infantile nystagmus occurred in 33% of children. Additional disabilities and/or developmental delay were reported for 44% of children. Corrected binocular visual acuity was reported for 75% of children, with moderate visual impairment being most common. CONCLUSIONS: These findings contribute to knowledge of rare diseases affecting the eye and visual pathway and represent Australian childhood visual impairment.

Exploring the Functional Impact of Childhood Vision Impairment: An e-Delphi Study.

The principles of the World Health Organization's International Classification of Function, Disability and Health have been embraced globally as a framework to support people with disability. This framework recognizes the functional needs of a person, as well as their medical diagnosis. However, the challenge of assessing the functional impact of disability to determine a person's needs and eligibility for support remains an issue. One example is that of childhood vision impairment, where a child's eligibility is often gauged from their visual performance in a clinical environment, a setting that is far removed from the child's real-world environment. Such clinical environments fail to reveal those factors that have a functional impact and affect the child's continuous visual function in their everyday situation. To examine this issue a three round modified e-Delphi technique was applied to explore the functional impact of childhood vision impairment. A panel of experienced Australian orthoptists and specialist teachers in vision impairment was convened, and the recently revised vision classifications in the World Health Organization International Statistical Classification of Diseases and Health Related Problems Version 11 were reviewed and modified for their application to children. This paper describes the e-Delphi technique and the major study outcomes including the modifications proposed by the study's participants, and the emerging paradigm that progresses an understanding of the functional impact of childhood vision impairment.