Congenital aniridia variant: minimally abnormal irides with severe limbal stem cell deficiency.

ABSTRACT:

Purpose

To clinically and molecularly characterize a group of patients with progressive limbal stem cell deficiency (LSCD) due to aniridic keratopathy (AK), but with minimally affected irides.

Design

Retrospective case series.

Participants

A total of 12 eyes of 6 patients who underwent keratolimbal allograft (KLAL) for AK in the absence of the classic stigmata of aniridia at the Cincinnati Eye Institute/University of Cincinnati between 2000 and 2007.

Methods

Retrospective chart review.

Main outcome measures

Ocular surface stability after KLAL and change in visual acuity.

Results

Subjects' mean age was 32.57 years, 66% were female, and mean follow-up was 64.4 months (range, 20-115 months). All patients presented with a decline in their vision secon

SUBMITTER: Skeens HM 

PROVIDER: S-EPMC11492833 | biostudies-literature | 2011 Jul

REPOSITORIES: biostudies-literature