Clinical and demographic associations with optic nerve hypoplasia in New Zealand.
OBJECTIVE
To identify the clinical features of optic nerve hypoplasia (ONH) and prevalence within a population of New Zealand children with severe visual impairment.


METHODS
Retrospective review of medical records of children with severe visual impairment registered with Blind and Low Vision Educational Network New Zealand.


RESULTS
Of 1500 children with severe visual impairment, 94 (6.3%) exhibited ONH, and 91 (97%) cases were bilateral. Of these 94 cases, 52 (55%) were males and ethnicities were European Caucasian (52%), Maori (40%), Pasifika (6%) and other (2%). Most children with ONH had poor vision, with 60% having ≤ 6/60 Snellen visual acuity equivalent. The median maternal age was 20.0 years old with 52% ≤ 20 years. There was a statistically significant over-representation of Maori ethnicity (40%) and young maternal age with age less than 20 years old (44%) in our cohort compared to the general population (14.6% and 7.4%, respectively; p<0.0001). Half had hypopituitarism, while neuroimaging abnormalities were detected in 60% cases. Cerebral neuroradiographic abnormalities were found to be associated with higher rate of developmental delay (OR 9.764 95% CI 3.246 to 29.373).


CONCLUSIONS
This is the first major study of visual impairment in New Zealand children, and it demonstrates that ONH is an important cause of severe visual disability; with an over-representation of Maori children and younger maternal age.
Department of Ophthalmology, Faculty of Medical and Health Sciences, New Zealand National Eye Centre, University of Auckland, Auckland, New Zealand.
Yi Wei
YW
Goh
Department of Ophthalmology, Faculty of Medical and Health Sciences, New Zealand National Eye Centre, University of Auckland, Auckland, New Zealand.
Dale
D
Andrew
Department of Ophthalmology, Faculty of Medical and Health Sciences, New Zealand National Eye Centre, University of Auckland, Auckland, New Zealand.
Charles
C
McGhee
Department of Ophthalmology, Faculty of Medical and Health Sciences, New Zealand National Eye Centre, University of Auckland, Auckland, New Zealand.
Shuan
S
Dai
D001921:Brain / Q000002:abnormalities; D002658:Developmental Disabilities / Q000175:diagnosis / Q000453:epidemiology; D005006:Ethnicity; D005260:Female; D006801:Humans; D007018:Hypopituitarism / Q000175:diagnosis / Q000453:epidemiology; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008423:Maternal Age; D009520:New Zealand / Q000453:epidemiology; D009901:Optic Nerve Diseases / Q000151:congenital* / Q000175:diagnosis / Q000453:epidemiology; D015995:Prevalence; D012042:Registries; D012189:Retrospective Studies; D014786:Vision Disorders / Q000175:diagnosis / Q000453:epidemiology; D014792:Visual Acuity / Q000502:physiology
Imaging; Optic Nerve