In Vivo Corneal Confocal Microscopy In Marfan Syndrome Patients

Abstract

Purpose: : To examine and investigate using a new–generation in vivo confocal microscope the cornea of patients with Marfan syndrome in comparison with a control group.

Methods: : In this case–control study, sixteen eyes of 8 patients with Marfan syndrome had their corneas examined using the in vivo confocal microscope Heidelberg Retina Tomograph (HRT) II / Rostock Cornea Module. The control group included sixteen normal eyes of 8 subjects matched according to age and degree of myopia. Morphological corneal differences were looked for and anterior and posterior keratocyte densities were compared between the two groups.

Results: : Epithelium and neural plexus examination did not show any difference between the two groups. Examination of the stroma showed no significant differences concerning the morphology of keratocytes or the anterior or posterior keratocyte density between the two groups. The extracellular matrix of 11 out of the 16 eyes of the Marfan group was very clearly visible and showed very thin highly reflective interconnected lines between keratocytes. In the normal eye group, reflective lines were observed in only 5 out of the 16 eyes. A grading scale from 1 to 4 was used, with 1 meaning null and 4 maximum stromal extracellular reflectivity. In Marfan group 11 patients showed grade 4 reflectivity and on the contrary no patient of the control group showed such reflectivity. Finally, endothelial cell morphology was similar in both groups. However, the endothelium of 8 corneas of the Marfan group showed brightly reflective particles. In no cornea of the control group such particles were observed.

Conclusions: : Highly reflective extracellular matrix of the stroma and brightly reflective particles among the endothelial cells were the two main corneal findings observed using the in vivo corneal confocal microscopy technique in Marfan patients when compared with a control group. Further investigations need to be made to confirm these findings and eventually find new criteria for Marfan syndrome based on the examination of in vivo corneal confocal microscopy.

Keywords: microscopy: confocal/tunneling • cornea: stroma and keratocytes • imaging/image analysis: clinical