Multimodal Imaging Features of Schnyder Corneal Dystrophy

Objective. To describe the multimodal imaging of Schnyder corneal dystrophy. Methods. Seven eyes of seven patients (5 female and 2 male patients) aged 52 to 92 years were included in this prospective observational study. Diagnosis of SCD was confirmed by histology after keratoplasty. In vivo multimo...

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Main Authors: Wassim Ghazal, Cristina Georgeon, Kate Grieve, Nacim Bouheraoua, Vincent Borderie
Format: Article
Language:English
Published: Wiley 2020-01-01
Series:Journal of Ophthalmology
Online Access:http://dx.doi.org/10.1155/2020/6701816
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author Wassim Ghazal
Cristina Georgeon
Kate Grieve
Nacim Bouheraoua
Vincent Borderie
author_facet Wassim Ghazal
Cristina Georgeon
Kate Grieve
Nacim Bouheraoua
Vincent Borderie
author_sort Wassim Ghazal
collection DOAJ
description Objective. To describe the multimodal imaging of Schnyder corneal dystrophy. Methods. Seven eyes of seven patients (5 female and 2 male patients) aged 52 to 92 years were included in this prospective observational study. Diagnosis of SCD was confirmed by histology after keratoplasty. In vivo multimodal imaging consisted of spectral domain-optical coherence tomography with cross sections, en face scans, corneal pachymetry, and epithelial mapping, and in vivo confocal microscopy was recorded. Ex vivo full-field optical coherence tomography scans of two corneal buttons were analyzed. The seven corneal buttons obtained during penetrating or deep anterior lamellar keratoplasty were processed for light microscopy. Results. Slit-lamp examination showed central stromal opacities, arcus lipoides, and midperipheral haze. Corneal crystals were found in 2 out of 7 eyes. SD-OCT cross sections and en face scans showed diffuse hyperreflectivity of the anterior, mid, and posterior stroma with a maximum in the anterior stroma, hyporeflective stromal striae, and epithelial hyperreflectivity. Central corneal thickness ranged from 507 to 635 μm. IVCM revealed hyperreflective deposits in the epithelium and throughout the stroma, thin subepithelial nerves, and needle-shaped and rectangular crystals. Keratocyte nuclei were rare or undetectable. FF-OCT scans confirmed the presence of small round and needle-shaped hyperreflective deposits in the epithelium and stroma. Histology revealed vacuolization of the basal epithelial cells and empty interlamellar stromal vacuoles. Conclusion. High-resolution multimodal imaging demonstrates the characteristic features of SCD which involve both the corneal epithelium and stroma, and it provides diagnosis confirmation even in eyes with no visible corneal crystals at slit-lamp examination.
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spelling doaj-art-b4559b495b4941e7a2245c2c2acf2df42025-08-20T02:23:12ZengWileyJournal of Ophthalmology2090-004X2090-00582020-01-01202010.1155/2020/67018166701816Multimodal Imaging Features of Schnyder Corneal DystrophyWassim Ghazal0Cristina Georgeon1Kate Grieve2Nacim Bouheraoua3Vincent Borderie4Centre Hospitalier National d’Ophtalmologie des 15-20, Paris, FranceCentre Hospitalier National d’Ophtalmologie des 15-20, Paris, FranceCentre Hospitalier National d’Ophtalmologie des 15-20, Paris, FranceCentre Hospitalier National d’Ophtalmologie des 15-20, Paris, FranceCentre Hospitalier National d’Ophtalmologie des 15-20, Paris, FranceObjective. To describe the multimodal imaging of Schnyder corneal dystrophy. Methods. Seven eyes of seven patients (5 female and 2 male patients) aged 52 to 92 years were included in this prospective observational study. Diagnosis of SCD was confirmed by histology after keratoplasty. In vivo multimodal imaging consisted of spectral domain-optical coherence tomography with cross sections, en face scans, corneal pachymetry, and epithelial mapping, and in vivo confocal microscopy was recorded. Ex vivo full-field optical coherence tomography scans of two corneal buttons were analyzed. The seven corneal buttons obtained during penetrating or deep anterior lamellar keratoplasty were processed for light microscopy. Results. Slit-lamp examination showed central stromal opacities, arcus lipoides, and midperipheral haze. Corneal crystals were found in 2 out of 7 eyes. SD-OCT cross sections and en face scans showed diffuse hyperreflectivity of the anterior, mid, and posterior stroma with a maximum in the anterior stroma, hyporeflective stromal striae, and epithelial hyperreflectivity. Central corneal thickness ranged from 507 to 635 μm. IVCM revealed hyperreflective deposits in the epithelium and throughout the stroma, thin subepithelial nerves, and needle-shaped and rectangular crystals. Keratocyte nuclei were rare or undetectable. FF-OCT scans confirmed the presence of small round and needle-shaped hyperreflective deposits in the epithelium and stroma. Histology revealed vacuolization of the basal epithelial cells and empty interlamellar stromal vacuoles. Conclusion. High-resolution multimodal imaging demonstrates the characteristic features of SCD which involve both the corneal epithelium and stroma, and it provides diagnosis confirmation even in eyes with no visible corneal crystals at slit-lamp examination.http://dx.doi.org/10.1155/2020/6701816
spellingShingle Wassim Ghazal
Cristina Georgeon
Kate Grieve
Nacim Bouheraoua
Vincent Borderie
Multimodal Imaging Features of Schnyder Corneal Dystrophy
Journal of Ophthalmology
title Multimodal Imaging Features of Schnyder Corneal Dystrophy
title_full Multimodal Imaging Features of Schnyder Corneal Dystrophy
title_fullStr Multimodal Imaging Features of Schnyder Corneal Dystrophy
title_full_unstemmed Multimodal Imaging Features of Schnyder Corneal Dystrophy
title_short Multimodal Imaging Features of Schnyder Corneal Dystrophy
title_sort multimodal imaging features of schnyder corneal dystrophy
url http://dx.doi.org/10.1155/2020/6701816
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AT nacimbouheraoua multimodalimagingfeaturesofschnydercornealdystrophy
AT vincentborderie multimodalimagingfeaturesofschnydercornealdystrophy