Scheimpflug Backscatter Imaging of the Fibrillar Layer in Fuchs Endothelial Corneal Dystrophy.

A central collagen-rich subendothelial fibrillar layer (FL) correlates with areas of accentuated loss of corneal endothelial cells in advanced Fuchs endothelial corneal dystrophy (FECD). The present study sought to investigate whether the FL may be visualized by en face Scheimpflug backscatter imaging in vivo. DESIGN: Retrospective analysis of a prospective observational case series. METHODS: A total of 34 eyes (34 subjects) undergoing Descemet membrane endothelial keratoplasty (DMEK) surgery with preoperative high-quality Scheimpflug backscatter imaging data were included. The Descemet endothelium complex (DEC) was retrieved during DMEK surgery, and immunofluorescence staining was performed for collagens I, III, and IV. The FL morphology in en face Scheimpflug backscatter and immunofluorescence imaging was compared and agreement of FL parameters was analyzed using intraclass correlation coefficients (ICC) and Bland-Altman plots. RESULTS: Scheimpflug backscatter imaging delineated the FL in 26 eyes and was FL negative in 8 eyes with deviation compared to immunofluorescence in 1 case and good agreement of morphology characteristics. Horizontal caliper diameter ± SD was 4.84 ± 0.85 mm, vertical caliper diameter was 3.92 ± 0.78 mm, maximum caliper diameter was 5.12 ± 0.82 mm, and surface area was 12.43 ± 4.74 mm2. Compared to immunofluorescence imaging, mean difference (95% limits of agreement) and intraclass correlation coefficients were for horizontal caliper diameter 0.13 mm (-0.81 to 1.1 mm) and 0.88, vertical caliper diameter 0.23 mm (-0.76 to 1.2 mm) and 0.81, maximum caliper diameter 0.06 mm (-1.1 to 1.2 mm) and 0.86, and surface area 1.4 mm2 (-3.9 to 6.7 mm2) and 0.85. CONCLUSIONS: Scheimpflug backscatter imaging facilitates visualization of the FL in advanced FECD eyes, offering the potential to identify particularly diseased areas of the FECD endothelium in vivo.

Fibrillar Layer as a Marker for Areas of Pronounced Corneal Endothelial Cell Loss in Advanced Fuchs Endothelial Corneal Dystrophy.

PURPOSE: We sought to assess the correlation of corneal endothelial cell (CEC) density to alterations of collagen composition of Descemet membrane (DM) in advanced Fuchs endothelial corneal dystrophy (FECD) and to image such changes by slit-lamp biomicroscopy in vivo. DESIGN: Prospective, observational consecutive case series. METHODS: Fifty eyes (50 subjects) with advanced FECD were enrolled. After slit-lamp biomicroscopy and corneal Scheimpflug imaging, the Descemet endothelium complex (DEC) was retrieved during DM endothelial keratoplasty (DMEK) surgery. The expression of collagens I, III, and IV (COL I, COL III, and COL IV) and corresponding CEC density were analyzed by immunofluorescence flat mount-staining. Presence, diameter and surface area of collagen expression, and CEC density served as the main outcome measures. RESULTS: Immunofluorescence staining revealed central coherent collagen positive areas (mean surface area = 10 mm2 ± 6 mm2) corresponding to a fibrillar layer burying the guttae of DM in 84% (42/50) of DECs. CEC density overlying the fibrillar layer compared with the periphery was significantly reduced (-54.8%, P < .0001) with a steep decline of CEC density at its borders. Subgroup analysis revealed that the fibrillar layer may be imaged by slit-lamp biomicroscopy in vivo with significant positive correlation of mean maximum diameter detected by slit-lamp biomicroscopy (dSL max = 4.1 mm ± 0.9 mm) and by immunofluorescence staining (dIF max = 4.7 mm ± 1.1 mm; r = 0.76; P = .001). CONCLUSION: A fibrillar layer with a clear geographic pattern marks areas of pronounced loss of CEC density in advanced FECD eyes and may be imaged by slit-lamp biomicroscopy in vivo.

Fuchs Endothelial Corneal Dystrophy: Clinical, Genetic, Pathophysiologic, and Therapeutic Aspects.

Fuchs endothelial corneal dystrophy (FECD) is a bilateral corneal endothelial disorder and the most common cause of corneal transplantation worldwide. Professor Ernst Fuchs described the first 13 cases of FECD more than 100 years ago. Since then, we have seen far-reaching progress in its diagnosis and treatment. In the field of diagnostics, new technologies enable the development of more accurate classification systems and the more detailed breakdown of the genetic basis of FECD. Laboratory studies help in deciphering the molecular pathomechanisms. The development of minimally invasive surgical techniques leads to a continuous improvement of the postoperative result. This review highlights and discusses clinical, genetic, pathophysiologic, and therapeutic aspects of this common and important corneal disorder.