title: Visual outcome in patients with isolated autosomal dominant congenital cataract.
abstract: OBJECTIVE
  To determine the visual outcome and surgical complication rates of patients with isolated inherited congenital cataract.
  
  
  METHODS
  Retrospective noncomparative case series.
  
  
  METHODS
  Patients and their families were ascertained from the genetic eye clinic and outpatient databases of Moorfields Eye Hospital, London, and invited to participate in the study. Four hundred twenty-two individuals from 72 pedigrees with this form of autosomal dominant cataract underwent ophthalmologic assessment.
  
  
  METHODS
  Visual acuity and surgical complications (glaucoma, retinal detachment, amblyopia).
  
  
  RESULTS
  In this study 49.4% of patients (46.8% of those operated) achieved a visual acuity (VA) of 20/40 or better, 35.9% (36.1% of those operated) a VA between 20/50 and 20/200, and 14.7% (17.1% of those operated) worse than 20/200. Opacities that were more diffuse or did not lie close to the visual axis were associated with a better prognosis for vision; 6.6% had glaucoma and 5.0% had retinal detachment develop.
  
  
  CONCLUSIONS
  Patients with isolated inherited congenital cataract have a better visual and surgical outcome than those with coexisting ocular and systemic abnormalities. The improved prognosis is related in part to the lack of other developmental abnormalities of the eye, and, because inherited cataracts are often partial at birth, surgery may be delayed to later infancy and childhood when there is a lower incidence of surgical complications and refractive correction is easier. Certain inherited phenotypes (lamellar, pulverulent, polymorphic, coralliform, and cortical) also seem to have a better prognosis, and this should be borne in mind when counseling these families. A large number of the patients in this study underwent surgery many years previously, when surgical outcomes were less favorable, and thus the results of this study establish only a minimum acuity dataset for the purposes of counseling.
authors.affiliation: Institute of Ophthalmology, London, England, UK.
authors.forename: P J
authors.initials: PJ
authors.lastname: Francis
authors.forename: A
authors.initials: A
authors.lastname: Ionides
authors.forename: V
authors.initials: V
authors.lastname: Berry
authors.forename: S
authors.initials: S
authors.lastname: Bhattacharya
authors.forename: A T
authors.initials: AT
authors.lastname: Moore
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002386:Cataract / Q000151:congenital* / Q000235:genetics; D002387:Cataract Extraction; D002648:Child; D002675:Child, Preschool; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007431:Intraoperative Complications; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011183:Postoperative Complications; D011379:Prognosis; D012189:Retrospective Studies; D014792:Visual Acuity