title: Long-term visual prognosis of corneal and ocular surface surgery in patients with congenital aniridia.
abstract: OBJECTIVE
  To evaluate the long-term visual prognosis of corneal and ocular surface surgery in patients with congenital aniridia.
  
  
  METHODS
  Retrospective comparative interventional case series on 88 eyes of 45 patients with congenital aniridia treated and/or operated on from 1956 to present. Corneal and ocular surface findings were identified and patients were classified into operated (group I) or not operated (group II). Long-term best-ever best-corrected visual acuity (BCVA), final BCVA and long-term delta BCVA (long-term best-ever BCVA - final BCVA) were recorded and compared between the two groups, and between the limbal transplant (LT) (group I-A) and the penetrating keratoplasty (PK) (group I-B) patients. Postoperative results were also compared.
  
  
  RESULTS
  Limbal insufficiency was present in 58% of eyes and dense central corneal opacities were present in 27% of eyes. As a primary surgery, limbal allograft was performed in 10 eyes and PK in 13 eyes. The mean long-term follow-up times were 23 years in group I and 16 years in group II. The mean long-term delta BCVA was 0.032 in group I and 0.028 in group II. Comparisons of the VA means were insignificant (long-term best-ever, final BCVA and long-term delta BCVA). When comparing the LT and PK groups, mean long-term delta BCVA was 0.0328 in group I-A and 0.0382 in group I-B. Mean postoperative delta BCVA was 0.028 in group I-A and 0.048 in group I-B. We found no statistical significance between the LT and the PK groups as regards long-term postoperative BCVA results.
  
  
  CONCLUSIONS
  Long-term visual prognosis does not differ whether or not the patient undergoes surgery for aniridic keratopathy. LT and PK have comparable results over 5 years of follow-up because of the eventual failure of transplanted allografts.
authors.affiliation: Barraquer Ophthalmology Centre, Barcelona, Spain. mpaz@barraquer.com
authors.forename: María Fideliz
authors.initials: MF
authors.lastname: de la Paz
authors.forename: Juan
authors.initials: J
authors.lastname: Alvarez de Toledo
authors.forename: Rafael Ignacio
authors.initials: RI
authors.lastname: Barraquer
authors.forename: Joaquín
authors.initials: J
authors.lastname: Barraquer
mesh_terms: D000293:Adolescent; D000328:Adult; D015783:Aniridia / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D003316:Corneal Diseases / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D015948:Keratoplasty, Penetrating; D016850:Limbus Corneae / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D011184:Postoperative Period; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D014184:Transplantation, Homologous; D014792:Visual Acuity; D055815:Young Adult