The microstructure of cornea verticillata in Fabry disease and amiodarone-induced keratopathy: a confocal laser-scanning microscopy study

Abstract

The microstructural corneal changes typically seen in cornea verticillata in both Fabry disease and in amiodarone-induced keratopathy can be successfully visualized by confocal in-vivo microscopy at the level of the basal cell layer. By analogy, with the grading system for cornea verticillata based on slit-lamp microscopy, staging of these deposits in the basal cell layer can also be performed following in-vivo CLSM. The microdots in the anterior stroma as well as the changes observed in the tarsal conjunctiva should be regarded as having less diagnostic value because such changes may also occur in normal subjects. The utility of CLSM as a tool for monitoring ERT in Fabry disease over time needs to be confirmed in studies with larger sample sizes conducted over a longer period.

Confocal laser-scanning microscopy allows differentiation between Fabry disease and amiodarone-induced keratopathy

“…Another drawback limiting the utility of CLSM for follow-up is that it is currently virtually impossible to measure identical areas of tissue on repeated investigations. To our knowledge, only one study has compared the microstructure of Fabry and amiodarone keratopathy using CLSM [12]. The investigators did not find any significant differences between the morphology of basal epithelial inclusions in Fabry and amiodarone-induced corneal changes.…”

“…The images were recorded digitally and stored on the system's hard drive in order to select pictures of the best quality and analyze corneal morphology. The degree of epithelial changes in groups A and B detected with CLSM was classified according to the grading system proposed by Falke et al [12] as follows: grade I: <25% of cells with hyper-reflective inclusions, grade II: 25-50%, grade III: 50-75%, and grade IV: >75% cells with hyper-reflective inclusions.…”

Oküler Bulguların Fabry Hastalığı Tanısındaki Önemi

“…9,10 Bu bulgulardan farklı olarak olgumuzda yüzeyel stromadaki sinir liflerinde kalınlaşma ve kıvrım artışı belirledik. Fabry hastalarında bildirilmiş olan subepitelyal sinir sayısında azalmayı olgumuzda gözlemlemedik.…”

Early Detection of Fabry Disease – The Important Role of Ophthalmology

“…16,17 Corneal verticillata are present in approximately 77 % of females and 73 % of males (age range, 3-71 years) with FD. 15 However should be exercised as a variety of drugs may result in corneal verticillata formation (see Table 1).…”

“…19 Care must be taken as amiodarone used for cardiac disease may be prescribed in addition to an underlying diagnosis of FD. 17 There is some evidence that in vivo confocal laser-scanning microscopy can detect differences between amiodarone-related corneal verticillata and FD. 20 Other rare ocular conditions can result in vortextype lesions at the level of …”