Abstract

P 142

Cornea verticillata in fabry disease and amiodaron-induced keratopathy – comparison of the microstructure in a confocal laser-scanning microscopy

Joanna Wasielica-Poslednik, Susanne Pitz, Norbert Pfeiffer
Universitäts-Augenklinik Mainz

Objective
Cornea verticillata is the most common ophthalmic abnormality in Fabry’s disease. It also occurs in patients treated with amiodarone. Our aim was to investigate and to compare the corneal microstructure in both cohorts using in vivo confocal laser-scanning microscopy and to evaluate eventual discrepancies between findings assessed by means of confocal and slit-lamp microscopy.
Methods
Ten eyes of ten patients affected with Fabry disease, nine eyes of seven patients receiving amiodarone therapy and ten eyes of healthy control-subjects were examined by use of conventional slit-lamp microscopy and in vivo confocal laser-scanning microscopy. All Fabry patients were treated with enzyme replacement therapy (ERT).
Results
Four Fabry patients and five patients receiving amiodarone showed a slit-lamp microscopy phenomenon of cornea verticillata (bilateral, whorl-like pattern of white, yellow or brown corneal deposits). All Fabry patients who presented cornea vertcillata in slit-lamp microscopy revealed hyper-reflective intracellular inclusions in form of patches, streaks or as hazy pattern in the corneal epithelium in confocal microscopy. Three of them revealed stromal microdots and none of them showed endothelial changes. One patient affected with Fabry disease exhibited a massive epithelial intracellular accumulation in confocal microscopy but no slit-lamp-detectable cornea verticillata. All amiodarone patients with cornea verticillata phenomenon showed epithelial intracellular deposits in confocal microscopy. These inclusions were more reflective than those by Fabry disease, presented no regular pattern and varied in size strongly. All amiodarone-receiving patients showed stromal microdots. The endothelial microdots were observed only in amiodarone patients. One healthy control subject revealed stromal microdots in confocal microscopy.
Conclusions
In vivo confocal microscopy allows differentiation between Fabry-induced cornea verticillata and amiodarone-keratopathy. Both phenomena present similar findings in conventional slit-lamp microscopy. In vivo confocal microscopy reveales corneal changes, which cannot be detected in slit-lamp examination. In conclusion, in vivo confocal microscopy might be an attractive alternative to conventional slit-lamp microscopy as diagnostic device in early stages of Fabry disease.

Copyright: 2009 Porstmann Kongresse GmbH. All rights reserved. Impressum, Disclaimer.