Document Detail


Surgical and Visual Outcomes of the Type I Boston Keratoprosthesis for the Management of Aniridic Fibrosis Syndrome in Congenital Aniridia.
MedLine Citation:
PMID:  22265154     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
PURPOSE: To report the clinical features and surgical management of aniridic fibrosis syndrome using the type I Boston Keratoprosthesis (KPro). DESIGN: 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. CONCLUSION: 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:
Pejman Bakhtiari; Clara Chan; Jeffrey D Welder; Jose de la Cruz; Edward J Holland; Ali R Djalilian
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-1-19
Journal Detail:
Title:  American journal of ophthalmology     Volume:  -     ISSN:  1879-1891     ISO Abbreviation:  -     Publication Date:  2012 Jan 
Date Detail:
Created Date:  2012-1-23     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370500     Medline TA:  Am J Ophthalmol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 Elsevier Inc. All rights reserved.
Affiliation:
Illinois Eye and Ear Infirmary, University of Illinois at Chicago, Chicago, Illinois.
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