In vivo confocal microscopy as a diagnostic tool in Schnyder Corneal Dystrophy’s case

Débora Biazim,Diego Casagrande,Paula Kataguiri

doi:10.5935/0034-7280.20190142

Abstract

Schnyder’s corneal dystrophy (SCD) is a rare corneal condition characterized by cholesterol and phospholipids deposition in the stroma and Bowman’s layer. We present a case report of a patient who had a progressive corneal stromal haze in both eyes since he was 15 years old. Etiological diagnosis of SCD was well established by In Vivo Confocal Microscopy (IVCM). In vivo confocal microscopy as a diagnostic tool in Schnyder Corneal Dystrophy’s case

Highlights

Schnyder’s corneal dystrophy (SCCD) is a rare autosomal dominant disorder characterized by the appearance of discshaped corneal opacity, which begins in the first decade of life and has a gradual progression of its intensity
The main systemic finding is hypercholesterolemia, it has already been reported in the literature that there is no correlation between blood cholesterol levels and the severity of the disease [11] and that its systemic reduction does not prevent the progression of the disease
(12) In addition, we have a description of genuvalgum as another possible component of the clinical picture of SCCD, [5] both of which are present in our report

Summary

Introduction

Schnyder’s corneal dystrophy (SCCD) is a rare autosomal dominant disorder characterized by the appearance of discshaped corneal opacity, which begins in the first decade of life and has a gradual progression of its intensity. A 55-year-old male, a trader, born in São Paulo, was admitted to our ser-vice with complaint of opacification of both eyes (BE) for 20 years, with progressive worsening in recent months It reported con-striction of the peripheral field without complaint of central visual acuity (VA), and in the right eye (RE) the symptomatology was more exacerbat-ed. Due to the diagnostic doubt, we submitted the patient to the in vivo con-focal microscopy (IVCM) (Heidelberg Retina Tomograph 3 with Rostock Cornea Module, Heidelberg EngineeringGmbH, Heidelberg, Germany), in which we obtained normal images of the transplanted eye (Figure 4) and alterations in LE (Figure 5), such as: reduction of subepithelial nerve plexus density, marked reduction of keratocytes in the stroma and accumulation of needle-shaped material in anterior stroma, signaling a second, more accurate and reliable diagnosis: Schnyder’s Corneal Dystrophy, which fit better into the clinical-epidemiological aspect of this report

Discussion

Conclusion