Advanced

Dystrophia Helsinglandica - corneal morphology, topography and sensitivity in a hereditary corneal disease with recurrent erosive episodes

Neira, Waldir; Hammar, Björn LU ; Holopainen, Juha M.; Tuisku, Ilpo; Dellby, Anette; Tervo, Timo and Fagerholm, Per (2010) In Acta Ophthalmologica 88(4). p.401-406
Abstract
Purpose: The aim of this study was to describe the morphology, corneal topography and sensitivity in individuals with Dystrophia Helsinglandica. This autosomal dominant corneal disease is characterized by recurrent corneal erosive episodes and progressive subepithelial fibrosis not significantly affecting visual acuity. Methods: The corneas of nine affected and nine unaffected individuals were examined using slit-lamp biomicroscopy, in vivo confocal microscopy (IVCM) and videokeratography. Corneal mechanical sensitivity was also measured using a non-contact esthesiometer. Results: Slit-lamp biomicroscopy revealed that the affected individuals represented different stages of corneal changes, from a nearly normal cornea to subepithelial... (More)
Purpose: The aim of this study was to describe the morphology, corneal topography and sensitivity in individuals with Dystrophia Helsinglandica. This autosomal dominant corneal disease is characterized by recurrent corneal erosive episodes and progressive subepithelial fibrosis not significantly affecting visual acuity. Methods: The corneas of nine affected and nine unaffected individuals were examined using slit-lamp biomicroscopy, in vivo confocal microscopy (IVCM) and videokeratography. Corneal mechanical sensitivity was also measured using a non-contact esthesiometer. Results: Slit-lamp biomicroscopy revealed that the affected individuals represented different stages of corneal changes, from a nearly normal cornea to subepithelial fibrosis of the central cornea. Corneal changes in affected individuals did not significantly decrease the best spectacle-corrected visual acuity. In vivo confocal microscopy detected morphological changes in the epithelium and stroma. Subepithelial opacity formation including altered keratocytes could be found in the anterior stroma in all affected eyes. With the exception of two eyes (one affected and one unaffected), all videokeratographies showed irregular astigmatism. Corneal sensitivity was significantly lower in affected individuals (p = 0.01). Age and corneal sensitivity showed no correlation. Conclusion: The main morphological findings in affected individuals were discrete and progressive subepithelial fibrosis, in the in vivo confocal microscope corresponding to optically dense extracellular matrix and activated keratocytes. Subbasal nerve morphology was changed in the affected family members who also showed a decreased corneal sensitivity. The findings are per se not specific to the disease. The changes probably reflect a healing response to erosive events on the corneal surface influenced by the genotype. (Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
corneal sensitivity, corneal dystrophies, confocal microscopy, cornea, videokeratography, morphology
in
Acta Ophthalmologica
volume
88
issue
4
pages
401 - 406
publisher
Wiley-Blackwell
external identifiers
  • wos:000278182000004
  • scopus:77952797287
ISSN
1755-3768
DOI
10.1111/j.1755-3768.2009.01844.x
language
English
LU publication?
yes
id
595b2c33-24c5-4a03-988f-fd7488d73d22 (old id 1632565)
date added to LUP
2010-07-22 13:10:07
date last changed
2018-05-29 11:01:20
@article{595b2c33-24c5-4a03-988f-fd7488d73d22,
  abstract     = {Purpose: The aim of this study was to describe the morphology, corneal topography and sensitivity in individuals with Dystrophia Helsinglandica. This autosomal dominant corneal disease is characterized by recurrent corneal erosive episodes and progressive subepithelial fibrosis not significantly affecting visual acuity. Methods: The corneas of nine affected and nine unaffected individuals were examined using slit-lamp biomicroscopy, in vivo confocal microscopy (IVCM) and videokeratography. Corneal mechanical sensitivity was also measured using a non-contact esthesiometer. Results: Slit-lamp biomicroscopy revealed that the affected individuals represented different stages of corneal changes, from a nearly normal cornea to subepithelial fibrosis of the central cornea. Corneal changes in affected individuals did not significantly decrease the best spectacle-corrected visual acuity. In vivo confocal microscopy detected morphological changes in the epithelium and stroma. Subepithelial opacity formation including altered keratocytes could be found in the anterior stroma in all affected eyes. With the exception of two eyes (one affected and one unaffected), all videokeratographies showed irregular astigmatism. Corneal sensitivity was significantly lower in affected individuals (p = 0.01). Age and corneal sensitivity showed no correlation. Conclusion: The main morphological findings in affected individuals were discrete and progressive subepithelial fibrosis, in the in vivo confocal microscope corresponding to optically dense extracellular matrix and activated keratocytes. Subbasal nerve morphology was changed in the affected family members who also showed a decreased corneal sensitivity. The findings are per se not specific to the disease. The changes probably reflect a healing response to erosive events on the corneal surface influenced by the genotype.},
  author       = {Neira, Waldir and Hammar, Björn and Holopainen, Juha M. and Tuisku, Ilpo and Dellby, Anette and Tervo, Timo and Fagerholm, Per},
  issn         = {1755-3768},
  keyword      = {corneal sensitivity,corneal dystrophies,confocal microscopy,cornea,videokeratography,morphology},
  language     = {eng},
  number       = {4},
  pages        = {401--406},
  publisher    = {Wiley-Blackwell},
  series       = {Acta Ophthalmologica},
  title        = {Dystrophia Helsinglandica - corneal morphology, topography and sensitivity in a hereditary corneal disease with recurrent erosive episodes},
  url          = {http://dx.doi.org/10.1111/j.1755-3768.2009.01844.x},
  volume       = {88},
  year         = {2010},
}