Examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study
Abstract Background Presence of corneal cystine crystals is the main ocular manifestation of cystinosis, although controversial findings concerning the corneal layer with the highest density have been reported. The aim of this study was the analysis of the characteristics of crystal arrangement in d...
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doaj-09e2a94caa7c43849e85a307af0bed382020-11-25T02:11:51ZengBMCBMC Ophthalmology1471-24152020-02-012011810.1186/s12886-020-01336-wExamination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional studyAnita Csorba0Erika Maka1Otto Alexander Maneschg2Attila Szabó3Nóra Szentmáry4Mária Csidey5Miklós Resch6László Imre7Krisztina Knézy8Zoltán Zsolt Nagy9Department of Ophthalmology, Semmelweis UniversityDepartment of Ophthalmology, Semmelweis UniversityDepartment of Ophthalmology, Semmelweis University1st Department of Pediatrics, Semmelweis UniversityDepartment of Ophthalmology, Semmelweis UniversityDepartment of Ophthalmology, Semmelweis UniversityDepartment of Ophthalmology, Semmelweis UniversityDepartment of Ophthalmology, Semmelweis UniversityDepartment of Ophthalmology, Semmelweis UniversityDepartment of Ophthalmology, Semmelweis UniversityAbstract Background Presence of corneal cystine crystals is the main ocular manifestation of cystinosis, although controversial findings concerning the corneal layer with the highest density have been reported. The aim of this study was the analysis of the characteristics of crystal arrangement in different corneal layers and the assessment of corneal morphological changes with age. Methods A cross sectional study was carried out in three children and three adults who had nephropathic cystinosis and corneal cystine depositions. All patients underwent a comprehensive ophthalmological examination including best corrected distance visual acuity, slit-lamp examination, in vivo confocal microscopy and anterior segment optical coherence tomography. An evaluation of the depth of crystal deposits and crystal density in different corneal layers was also performed. Due to the low number of subjects no statistical comparison was performed. Results Anterior segment optical coherence tomography images revealed deposition of hyperreflective crystals from limbus to limbus in each patient. Crystals appeared as randomly oriented hyperreflective, elongated structures on in vivo confocal microscopy images in all corneal layers except the endothelium. In children the deposits occurred predominantly in the anterior stroma, while in adults, the crystals were mostly localized in the posterior corneal stroma with the depth of crystal deposition showing an increasing tendency with age (mean depth of crystal density was 353.17 ± 49.23 μm in children and it was 555.75 ± 25.27 μm in adults). Mean crystal density of the epithelium was 1.47 ± 1.17 (median: 1.5; interquartile range: 0.3–2.4). Mean crystal density of the anterior and posterior stroma of children and adults was 3.37 ± 0.34 (median: 3.4; interquartile range: 3.25–3.55) vs. 1.23 ± 0.23 (median: 1.2; interquartile range: 1.05–1.35) and 0.76 ± 0.49 (median: 0.7; interquartile range: 0.4–1.15) vs. 3.63 ± 0.29 (median: 3.7; interquartile range: 3.45–3.8), respectively. Endothelium had intact structure in all cases. Some hexagonal crystals were observed in two subjects. Conclusions In vivo confocal microscopy and anterior segment optical coherence tomography confirmed an age-related pattern of crystal deposition. In children, crystals tend to locate anteriorly, while in adults, deposits are found posteriorly in corneal stroma.http://link.springer.com/article/10.1186/s12886-020-01336-wCorneaNephropathic cystinosisCorneal crystalsIn vivo confocal microscopyAnterior segment optical coherence tomography |
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DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Anita Csorba Erika Maka Otto Alexander Maneschg Attila Szabó Nóra Szentmáry Mária Csidey Miklós Resch László Imre Krisztina Knézy Zoltán Zsolt Nagy |
spellingShingle |
Anita Csorba Erika Maka Otto Alexander Maneschg Attila Szabó Nóra Szentmáry Mária Csidey Miklós Resch László Imre Krisztina Knézy Zoltán Zsolt Nagy Examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study BMC Ophthalmology Cornea Nephropathic cystinosis Corneal crystals In vivo confocal microscopy Anterior segment optical coherence tomography |
author_facet |
Anita Csorba Erika Maka Otto Alexander Maneschg Attila Szabó Nóra Szentmáry Mária Csidey Miklós Resch László Imre Krisztina Knézy Zoltán Zsolt Nagy |
author_sort |
Anita Csorba |
title |
Examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study |
title_short |
Examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study |
title_full |
Examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study |
title_fullStr |
Examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study |
title_full_unstemmed |
Examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study |
title_sort |
examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study |
publisher |
BMC |
series |
BMC Ophthalmology |
issn |
1471-2415 |
publishDate |
2020-02-01 |
description |
Abstract Background Presence of corneal cystine crystals is the main ocular manifestation of cystinosis, although controversial findings concerning the corneal layer with the highest density have been reported. The aim of this study was the analysis of the characteristics of crystal arrangement in different corneal layers and the assessment of corneal morphological changes with age. Methods A cross sectional study was carried out in three children and three adults who had nephropathic cystinosis and corneal cystine depositions. All patients underwent a comprehensive ophthalmological examination including best corrected distance visual acuity, slit-lamp examination, in vivo confocal microscopy and anterior segment optical coherence tomography. An evaluation of the depth of crystal deposits and crystal density in different corneal layers was also performed. Due to the low number of subjects no statistical comparison was performed. Results Anterior segment optical coherence tomography images revealed deposition of hyperreflective crystals from limbus to limbus in each patient. Crystals appeared as randomly oriented hyperreflective, elongated structures on in vivo confocal microscopy images in all corneal layers except the endothelium. In children the deposits occurred predominantly in the anterior stroma, while in adults, the crystals were mostly localized in the posterior corneal stroma with the depth of crystal deposition showing an increasing tendency with age (mean depth of crystal density was 353.17 ± 49.23 μm in children and it was 555.75 ± 25.27 μm in adults). Mean crystal density of the epithelium was 1.47 ± 1.17 (median: 1.5; interquartile range: 0.3–2.4). Mean crystal density of the anterior and posterior stroma of children and adults was 3.37 ± 0.34 (median: 3.4; interquartile range: 3.25–3.55) vs. 1.23 ± 0.23 (median: 1.2; interquartile range: 1.05–1.35) and 0.76 ± 0.49 (median: 0.7; interquartile range: 0.4–1.15) vs. 3.63 ± 0.29 (median: 3.7; interquartile range: 3.45–3.8), respectively. Endothelium had intact structure in all cases. Some hexagonal crystals were observed in two subjects. Conclusions In vivo confocal microscopy and anterior segment optical coherence tomography confirmed an age-related pattern of crystal deposition. In children, crystals tend to locate anteriorly, while in adults, deposits are found posteriorly in corneal stroma. |
topic |
Cornea Nephropathic cystinosis Corneal crystals In vivo confocal microscopy Anterior segment optical coherence tomography |
url |
http://link.springer.com/article/10.1186/s12886-020-01336-w |
work_keys_str_mv |
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