title: Clinical characteristics of children with severe visual impairment but favorable retinal structural outcomes from the Early Treatment for Retinopathy of Prematurity (ETROP) study.
abstract: OBJECTIVE
  To describe visual function and associated characteristics at the 6-year examination in children enrolled in the Early Treatment for Retinopathy of Prematurity Study who had unfavorable visual outcomes despite favorable structural outcomes in one or both eyes.
  
  
  METHODS
  The clinical examination records of children completing the 6-year follow-up examination were retrospectively reviewed. Eligible subjects were those with visual acuity of ≤20/200 in each eye (where recordable) and a normal fundus or straightening of the temporal retinal vessels with or without macular ectopia in at least one eye. Data regarding visual function, retinal structure, presence of nystagmus, optic atrophy, optic disk cupping, seizures/shunts, and Functional Independence Measure for Children (ie, WeeFIM: pediatric functional independence measure) developmental test scores were reviewed.
  
  
  RESULTS
  Of 342 participants who completed the 6-year examination, 39 (11%) met inclusion criteria. Of these, 29 (74%) had normal retinal structure, 18 (46%) had optic atrophy, and 3 (8%) had increased cupping of the optic disk in at least one eye. Latent and/or manifest nystagmus occurred in 30 children (77%). The presence of nystagmus was not related to the presence of optic atrophy. Of the 39 children, 28 (72%) had a below-normal WeeFIM score.
  
  
  CONCLUSIONS
  In 25 participants (7%) completing the 6-year examination, cortical visual impairment was considered the primary cause of visual loss. The remainder likely had components of both anterior and posterior visual pathway disease. Clinical synthesis of ocular anatomy and visual and neurologic function is required to determine the etiology of poor vision in these children.
authors.affiliation: Dean McGee Eye Institute, Department of Ophthalmology, University of Oklahoma, Oklahoma City, Oklahoma 73104, USA. RMichael-Siatkowski@dmei.org
authors.forename: R Michael
authors.initials: RM
authors.lastname: Siatkowski
authors.forename: William V
authors.initials: WV
authors.lastname: Good
authors.forename: C Gail
authors.initials: CG
authors.lastname: Summers
authors.forename: Graham E
authors.initials: GE
authors.lastname: Quinn
authors.forename: Betty
authors.initials: B
authors.lastname: Tung
mesh_terms: D002648:Child; D015331:Cohort Studies; D005260:Female; D005654:Fundus Oculi; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D012164:Retinal Diseases / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D012171:Retinal Vessels / Q000473:pathology; D012178:Retinopathy of Prematurity / Q000150:complications / Q000503:physiopathology*; D014792:Visual Acuity / Q000502:physiology*