title: Congenital aniridia: long-term clinical course, visual outcome, and prognostic factors.
abstract: OBJECTIVE
  To describe the clinical course of congenital aniridia and to evaluate prognostic factors for visual outcome after long-term follow-up.
  
  
  METHODS
  The medical records of 120 eyes from 60 patients with congenital aniridia were retrospectively reviewed. The prevalence and clinical course of ophthalmic characteristics, systemic disease, refractive errors, and visual acuity were assessed. Prognostic factors for final visual outcomes were analyzed.
  
  
  RESULTS
  Aniridic keratopathy developed in 82 (69%) of 119 eyes. Macular hypoplasia was observed in 70 eyes of 35 patients (91%). Cataract was observed in 63 of 120 eyes (53%). Nystagmus was present in 41 patients (68% of 60 patients) at the initial visit but decreased in five patients (8% of 60 patients). Ocular hypertension was detected in 19 eyes (20% of 93 eyes), six (32% of 19 eyes) of which developed secondarily after cataract surgery. The mean changes in spherical equivalent and astigmatism during the follow-up period were -1.10 and 1.53 diopter, respectively. The mean final visual acuity was 1.028 logarithm of minimal angle of resolution. Nystagmus and ocular hypertension were identified as prognostic factors for poor visual outcome.
  
  
  CONCLUSIONS
  Identification of nystagmus and ocular hypertension was important to predict final visual outcome. Based on the high rate of secondary ocular hypertension after cataract surgery, careful management is needed.
authors.affiliation: Department of Ophthalmology, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea.
authors.forename: Ji Woong
authors.initials: JW
authors.lastname: Chang
authors.affiliation: Department of Ophthalmology, Seoul National University Hospital, Seoul, Korea. ; Seoul Artificial Eye Center, Seoul National University Hospital Clinical Research Institute, Seoul, Korea.
authors.forename: Jeong Hun
authors.initials: JH
authors.lastname: Kim
authors.affiliation: Department of Ophthalmology, Seoul National University Hospital, Seoul, Korea. ; Seoul Artificial Eye Center, Seoul National University Hospital Clinical Research Institute, Seoul, Korea.
authors.forename: Seong-Joon
authors.initials: SJ
authors.lastname: Kim
authors.affiliation: Department of Ophthalmology, Seoul National University Hospital, Seoul, Korea. ; Seoul Artificial Eye Center, Seoul National University Hospital Clinical Research Institute, Seoul, Korea.
authors.forename: Young Suk
authors.initials: YS
authors.lastname: Yu
mesh_terms: D000293:Adolescent; D000328:Adult; D015783:Aniridia / Q000175:diagnosis* / Q000503:physiopathology; D002386:Cataract / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D003316:Corneal Diseases / Q000175:diagnosis; D005124:Eye Abnormalities / Q000175:diagnosis; D005128:Eye Diseases / Q000175:diagnosis* / Q000503:physiopathology; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D009759:Nystagmus, Pathologic / Q000175:diagnosis; D009798:Ocular Hypertension / Q000175:diagnosis; D011379:Prognosis; D012160:Retina / Q000002:abnormalities; D012189:Retrospective Studies; D014792:Visual Acuity / Q000502:physiology*; D055815:Young Adult
keywords: Aniridia; Eye abnormalities; Prognosis