title: Changing patterns in paediatric optic atrophy aetiology: 1979 to 2015.
abstract: BACKGROUND
  To evaluate the aetiology of paediatric optic atrophy in an Australian population.
  
  
  METHODS
  Retrospective review of medical records was conducted at The Children's Hospital at Westmead, Sydney, Australia.
  
  
  METHODS
  Two hundred twenty-seven subjects <16 years who were diagnosed with optic atrophy on fundoscopic examination between 1979 and 2015 were included in the study.
  
  
  METHODS
  Subjects were obtained from the hospital database, which codes diagnoses for all admissions, as well as the orthoptic department database, which codes diagnoses for ophthalmology department outpatients.
  
  
  METHODS
  The predominant cause for optic atrophy was assigned to each patient. Clinical presentation was defined as the principal reason for evaluation. Demographic data included gender, affected eye and age at diagnosis. Data on medical comorbidities (cerebral palsy, developmental delay, microcephaly and seizures) and ocular comorbidities (strabismus and nystagmus) were collected.
  
  
  RESULTS
  The mean age at initial eye review was 4.7 ± 4.4 years. There was bilateral optic atrophy in 81.5% of cases. Unilateral optic atrophy was largely due to tumours. When analysing over the three time periods, (1979-1990, 1991-2003 and 2004-2015), perinatal events (3.0%, 22.7% and 22.6%) and neurodegenerative disease (3.0%, 14.9% and 15.1%) are slowly replacing tumours (39.4%, 24.8% and 15.1%) as the top causes for paediatric optic atrophy. The incidence of other causes has remained fairly stable over time, albeit an increase in idiopathic causes.
  
  
  CONCLUSIONS
  There has been shift in the etiological profile of optic atrophy. Whilst tumours are still an important cause of paediatric optic atrophy for an Australian population, perinatal events and neurodegenerative disease are becoming more significant.
authors.affiliation: Department of Ophthalmology, The Children's Hospital at Westmead, Westmead, Australia. lindazhengs@gmail.com.
authors.forename: Linda
authors.initials: L
authors.lastname: Zheng
authors.affiliation: Discipline of Ophthalmology, Save Sight Institute, University of Sydney, Sydney, New South Wales, Australia.
authors.forename: Helen Hyun-Jin
authors.initials: HH
authors.lastname: Do
authors.affiliation: Discipline of Ophthalmology, Save Sight Institute, University of Sydney, Sydney, New South Wales, Australia.
authors.forename: Trent
authors.initials: T
authors.lastname: Sandercoe
authors.affiliation: Eye Genetics Research Group, Children's Medical Research Institute, The Children's Hospital at Westmead, Westmead, Australia.|Discipline of Ophthalmology, Save Sight Institute, University of Sydney, Sydney, New South Wales, Australia.
authors.forename: Robyn V
authors.initials: RV
authors.lastname: Jamieson
authors.affiliation: Department of Ophthalmology, The Children's Hospital at Westmead, Westmead, Australia.|Eye Genetics Research Group, Children's Medical Research Institute, The Children's Hospital at Westmead, Westmead, Australia.|Discipline of Ophthalmology, Save Sight Institute, University of Sydney, Sydney, New South Wales, Australia.
authors.forename: John R
authors.initials: JR
authors.lastname: Grigg
mesh_terms: D001315:Australia / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D009369:Neoplasms; D009896:Optic Atrophy / Q000453:epidemiology* / Q000209:etiology*; D012189:Retrospective Studies; D012307:Risk Factors
keywords: aetiology; child; optic atrophy; paediatric