Quantitative Analysis of the Association Between Follow-Up Duration and Severity of Limbal Stem Cell Deficiency or Visual Acuity in Aniridia

Abstract

This study aimed to quantitatively analyze the association between follow-up duration and the severity of limbal stem cell deficiency (LSCD) or visual acuity in patients with aniridia. METHODS. A total of 52 eyes of 27 patients with aniridia were enrolled at Osaka University Hospital. Medical records were retrospectively reviewed to obtain information on the severity of LSCD and corrected distance visual acuity (CDVA). LSCD severity was based on a modified severity grading scale. We used an ordered logistic regression model to examine the association between follow-up duration and LSCD severity, and a linear regression model with a generalized linear mixed model for the association between follow-up duration and visual acuity. RESULTS. The mean follow-up duration was 5.2 ± 6.3 years. The mean age at the last follow-up visit was 40.5 ± 18.9 years. The mean CDVA was 1.52 ± 1.09 logMAR. At the last follow-up, 1 examined eye (1.9%) was categorized as stage 0, 7 (13.5%) as Ia, 9 (17.3%) as Ib, 5 (9.6%) as Ic, 2 (3.8%) as IIb, 12 (23.1%) as IIc, and 11 (21.2%) as III. Five eyes (9.6%) were unclassifiable. There was a significant association between follow-up duration and LSCD severity (odds ratio per +1 year, 1.41; P < 0.001). CDVA significantly decreased as follow-up duration increased. Each increase of 1 year in the follow-up duration was associated with a mean difference of +0.021 logMAR (95% confidence interval [CI] 0.01-0.03; P < 0.001). CONCLUSIONS. We quantitatively demonstrate that LSCD severity and visual impairment significantly progress as follow-up duration increases.

An attempt to optimize the outcome of penetrating keratoplasty in congenital aniridia-associated keratopathy (AAK)

“…A prospective randomized study is not possible for ethical reasons. As our proposed therapeutic scheme does not directly address the cause of AAK, it could be combined with a prior transplantation of allogenic limbal stem cells, even though limbal stem cell transplantation alone in patient with AAK has been significantly associated with progression of limbal stem cell deficiency severity and visual impairment [ 30 ]. However, the survival curve looks promising, with a major separation after only 2 years of follow-up.…”

Profile of biological characterizations and clinical application of corneal stem/progenitor cells

“…Thermal burns, acid burns, radiation burns and others account for only a small percentage[ 78 , 79 ]. The conditions that lead mainly to LSCD include two main categories, hereditary LSCD and acquired LSCD, as shown in Table 3 [ 80 - 115 ].…”