title: In vivo morphology of the limbal palisades of vogt correlates with progressive stem cell deficiency in aniridia-related keratopathy.
abstract: OBJECTIVE
  To investigate morphologic alterations in the limbal palisades of Vogt in a progressive form of limbal stem cell deficiency.
  
  
  METHODS
  Twenty Norwegian subjects (40 eyes) with congenital aniridia and 9 healthy family members (18 eyes) without aniridia were examined. Clinical grade of aniridia-related keratopathy (ARK) was assessed by slit-lamp biomicroscopy, and tear production and quality, corneal thickness, and sensitivity were additionally measured. The superior and inferior limbal palisades of Vogt and central cornea were examined by laser scanning in vivo confocal microscopy (IVCM).
  
  
  RESULTS
  In an aniridia patient with grade 0 ARK, a transparent cornea and normal limbal palisade morphology were found. In grade 1 ARK, 5 of 12 eyes had degraded palisade structures. In the remaining grade 1 eyes and in all 20 eyes with stage 2, 3, and 4 ARK, palisade structures were absent by IVCM. Increasing ARK grade significantly correlated with reduced visual acuity and corneal sensitivity, increased corneal thickness, degree of degradation of superior and inferior palisade structures, reduced peripheral nerves, increased inflammatory cell invasion, and reduced density of basal epithelial cells and central subbasal nerves. Moreover, limbal basal epithelial cell density and central corneal subbasal nerve density were both significantly reduced in aniridia compared to healthy corneas (P = 0.002 and 0.003, respectively).
  
  
  CONCLUSIONS
  Progression of limbal stem cell deficiency in aniridia correlates with degradation of palisade structures, gradual transformation of epithelial phenotype, onset of inflammation, and a corneal nerve deficit. IVCM can be useful in monitoring early- to late-stage degenerative changes in stem cell-deficient patients.
authors.affiliation: Department of Clinical and Experimental Medicine, Faculty of Health Sciences, Linköping University, Linköping, Sweden. neil.lagali@liu.se
authors.forename: Neil
authors.initials: N
authors.lastname: Lagali
authors.forename: Ulla
authors.initials: U
authors.lastname: Edén
authors.forename: Tor Paaske
authors.initials: TP
authors.lastname: Utheim
authors.forename: Xiangjun
authors.initials: X
authors.lastname: Chen
authors.forename: Ruth
authors.initials: R
authors.lastname: Riise
authors.forename: Anette
authors.initials: A
authors.lastname: Dellby
authors.forename: Per
authors.initials: P
authors.lastname: Fagerholm
mesh_terms: D000293:Adolescent; D000328:Adult; D015783:Aniridia / Q000150:complications* / Q000473:pathology; D002452:Cell Count; D003316:Corneal Diseases / Q000473:pathology*; D018450:Disease Progression; D019573:Epithelium, Corneal / Q000473:pathology*; D005260:Female; D006801:Humans; D016850:Limbus Corneae / Q000473:pathology*; D008297:Male; D033401:Microscopy, Acoustic; D018613:Microscopy, Confocal; D008875:Middle Aged; D013234:Stem Cells / Q000473:pathology*; D055815:Young Adult
keywords: aniridia; corneal epithelium; corneal stem cells; in vivo confocal microscopy; limbal palisades of Vogt; stem cell deficiency; subbasal nerves