title: Congenital eyelid ptosis: onset and prevalence of amblyopia, associations with systemic disorders, and treatment outcomes.
abstract: OBJECTIVE
  To determine the age at onset of amblyopia, the response to occlusion therapy, and the association with systemic disorders in children with congenital eyelid ptosis.
  
  
  METHODS
  Retrospective chart review of children seen at Seattle Children's Hospital with moderate or severe congenital ptosis. Assessments were longitudinal visual acuity development using objective methods, definition of ptosis severity by eyelid margin to pupillary light reflex distance (margin reflex distance [MRD]), age at amblyopia diagnosis, correlation between amblyopia and MRD, and associated systemic disorders.
  
  
  RESULTS
  Eighty-four children with moderate-to-severe congenital ptosis met inclusion criteria; the mean longitudinal follow-up was 49.1 months. Fifteen (18%) of these children had amblyopia, of which 9 had deprivation amblyopia (mean age 17.3 months ± 11.2) and 6 had anisometropic or strabismic amblyopia (mean age 60 months ± 11.8). Eleven (73%) of the children with amblyopia were successfully treated with occlusion therapy. Amblyopia was not correlated with MRD. A systemic disorder was identified in 29 (35%) of the children, the most common being genetic, chromosomal, or neurologic conditions. Patients with systemic disorders and developmental delay have significantly lower visual acuity bilaterally compared with patients without systemic disorders (P ≤ .003).
  
  
  CONCLUSIONS
  Using longitudinal and objective visual acuity assessments, the incidence of amblyopia was 18% in children with moderate to severe congenital ptosis. Visual deprivation was the predominant risk factor that was reliably distinguished by its earlier onset in young children. The best indicator of amblyopia in children is visual acuity rather than MRD measurements. Systemic disorders are frequent in children with moderate to severe congenital ptosis.
authors.affiliation: Roger Johnson Clinical Vision Laboratory, Division of Ophthalmology, Seattle Children's Hospital, Seattle, WA; Department of Ophthalmology, University of Washington Medical Center, Seattle, WA.
authors.forename: August
authors.initials: A
authors.lastname: Stein
authors.affiliation: Roger Johnson Clinical Vision Laboratory, Division of Ophthalmology, Seattle Children's Hospital, Seattle, WA; Department of Ophthalmology, University of Washington Medical Center, Seattle, WA.
authors.forename: John P
authors.initials: JP
authors.lastname: Kelly
authors.affiliation: Roger Johnson Clinical Vision Laboratory, Division of Ophthalmology, Seattle Children's Hospital, Seattle, WA; Department of Ophthalmology, University of Washington Medical Center, Seattle, WA. Electronic address: avery.weiss@seattlechildrens.org.
authors.forename: Avery H
authors.initials: AH
authors.lastname: Weiss
mesh_terms: D017668:Age of Onset; D000550:Amblyopia / Q000150:complications / Q000453:epidemiology* / Q000628:therapy*; D001763:Blepharoptosis / Q000150:complications* / Q000151:congenital; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005143:Eyelids / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D014785:Vision, Ocular; D014792:Visual Acuity