title: Surgical and visual outcomes of the type I Boston Keratoprosthesis for the management of aniridic fibrosis syndrome in congenital aniridia.
abstract: OBJECTIVE
  To report the clinical features and surgical management of aniridic fibrosis syndrome using the type I Boston Keratoprosthesis (KPro).
  
  
  METHODS
  Interventional case series.
  
  
  METHODS
  Retrospective chart review of 9 eyes in 9 patients with congenital aniridia that developed aniridic fibrosis syndrome.
  
  
  RESULTS
  All patients had clinical diagnosis of congenital aniridia. Previously, all patients had undergone cataract surgery with posterior chamber intraocular lens (IOL) implantation and 7 patients had existing tube shunts. In all cases, fibrosis presented as progressive retrocorneal and retrolenticular membrane formation causing displacement of the IOL and secondary corneal decompensation. Two eyes had tractional folds in the retina with posterior extension of the membrane. The management included IOL explantation in 7 of 9 cases, removal of fibrosis with pars plana vitrectomy in all 9 patients, and implantation of a type I Boston KPro in all eyes. At a mean final follow-up of 26.1 months (range 6 to 48 months), vision remained improved in all patients. No patient had recurrence of the fibrotic membrane after KPro implantation.
  
  
  CONCLUSIONS
  This study represents another case series describing aniridic fibrosis syndrome and the largest study to report utilization of the type I Boston KPro in such patients. As the fibrosis can cause IOL dislocation, corneal decompensation, hypotony, and retinal detachment, monitoring for aniridic fibrosis syndrome in congenital aniridia with early surgical intervention is recommended. Type I Boston KPro may be considered in the surgical treatment of this condition.
authors.affiliation: Illinois Eye and Ear Infirmary, University of Illinois at Chicago, 60612, USA.
authors.forename: Pejman
authors.initials: P
authors.lastname: Bakhtiari
authors.forename: Clara
authors.initials: C
authors.lastname: Chan
authors.forename: Jeffrey D
authors.initials: JD
authors.lastname: Welder
authors.forename: Jose
authors.initials: J
authors.lastname: de la Cruz
authors.forename: Edward J
authors.initials: EJ
authors.lastname: Holland
authors.forename: Ali R
authors.initials: AR
authors.lastname: Djalilian
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015783:Aniridia / Q000503:physiopathology / Q000601:surgery*; D001187:Artificial Organs; D002387:Cataract Extraction; D002648:Child; D003315:Cornea; D005260:Female; D005355:Fibrosis; D006801:Humans; D007498:Iris / Q000473:pathology*; D019654:Lens Implantation, Intraocular; D008297:Male; D008875:Middle Aged; D019736:Prostheses and Implants; D012189:Retrospective Studies; D013577:Syndrome; D016896:Treatment Outcome; D014792:Visual Acuity / Q000502:physiology*; D014821:Vitrectomy; D055815:Young Adult