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In vivo confocal microstructural analysis of corneas presenting Kayser-Fleischer rings in patients with Wilson's disease.
Arq Bras Oftalmol 2018; 81(2):137-143AB

Abstract

PURPOSE

To evaluate microstructural differences between corneas with and without Kayser-Fleischer rings in age-matched subjects with Wilson's disease with neurological symptoms, using confocal laser scanning microscopy.

METHODS

The study included 12 subjects with Wilson's disease with neurological symptoms. Twelve corneas presented clinically with classic Kayser-Fleischer rings, visible on slit lamp examination; the other 12 served as controls. The subjects underwent a comprehensive clinical examination. Microstructural analysis using confocal laser scanning microscopy evaluated increased corneal thickness, decreased number of cells, increased debris or specific deposits, and unusual microstructures.

RESULTS

Clinically, the subjects with Kayser-Fleischer rings had similar corneal findings and normal intraocular pressure; two had typical sunflower cataracts and decreased visual acuity. The control eyes all presented normal visual acuity, intraocular pressure, and corneal appearance. The microstructural analysis demonstrated similar findings in all the affected corneas. Compared with the control corneas, there were fewer keratocytes in the anterior stroma (17.380 vs. 22.380/mm3). Round, "hollow" dark areas were observed between the keratocytes; these were universal and similar in appearance in all affected corneas and all cornea layers. In the peripheral posterior stroma, there were dust-like, bright, granular deposits that tended to increase in number and density toward Descemet's membrane, masking the peripheral endothelium. The control corneas presented a normal microstructure apart from dust-like granular deposits in the periphery.

CONCLUSIONS

In vivo confocal microscopy is a useful tool for evaluating the corneal microstructure when a Kayser-Fleischer ring is clinically present. The ring consists of granular, bright particles that increase in density toward Descemet's membrane, and is associated with a decreased number of keratocytes and peculiar dark, round areas in all stromal layers, probably a sign of corneal damage. When the ring is not visible in subjects with Wilson's disease, changes to the corneal microstructure are insignificant.

Authors+Show Affiliations

Medical University of Varna, Varna, Bulgaria.Medical University of Varna, Varna, Bulgaria.Medical University of Varna, Varna, Bulgaria.Medical University of Varna, Varna, Bulgaria.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

29846416

Citation

Grupchev, Dimitar Ivanov, et al. "In Vivo Confocal Microstructural Analysis of Corneas Presenting Kayser-Fleischer Rings in Patients With Wilson's Disease." Arquivos Brasileiros De Oftalmologia, vol. 81, no. 2, 2018, pp. 137-143.
Grupchev DI, Radeva MN, Georgieva M, et al. In vivo confocal microstructural analysis of corneas presenting Kayser-Fleischer rings in patients with Wilson's disease. Arq Bras Oftalmol. 2018;81(2):137-143.
Grupchev, D. I., Radeva, M. N., Georgieva, M., & Grupcheva, C. N. (2018). In vivo confocal microstructural analysis of corneas presenting Kayser-Fleischer rings in patients with Wilson's disease. Arquivos Brasileiros De Oftalmologia, 81(2), pp. 137-143. doi:10.5935/0004-2749.20180030.
Grupchev DI, et al. In Vivo Confocal Microstructural Analysis of Corneas Presenting Kayser-Fleischer Rings in Patients With Wilson's Disease. Arq Bras Oftalmol. 2018;81(2):137-143. PubMed PMID: 29846416.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - In vivo confocal microstructural analysis of corneas presenting Kayser-Fleischer rings in patients with Wilson's disease. AU - Grupchev,Dimitar Ivanov, AU - Radeva,Mladena Nikolaeva, AU - Georgieva,Miglena, AU - Grupcheva,Christina N, PY - 2017/05/24/received PY - 2017/10/16/accepted PY - 2018/5/31/entrez PY - 2018/5/31/pubmed PY - 2018/10/3/medline SP - 137 EP - 143 JF - Arquivos brasileiros de oftalmologia JO - Arq Bras Oftalmol VL - 81 IS - 2 N2 - PURPOSE: To evaluate microstructural differences between corneas with and without Kayser-Fleischer rings in age-matched subjects with Wilson's disease with neurological symptoms, using confocal laser scanning microscopy. METHODS: The study included 12 subjects with Wilson's disease with neurological symptoms. Twelve corneas presented clinically with classic Kayser-Fleischer rings, visible on slit lamp examination; the other 12 served as controls. The subjects underwent a comprehensive clinical examination. Microstructural analysis using confocal laser scanning microscopy evaluated increased corneal thickness, decreased number of cells, increased debris or specific deposits, and unusual microstructures. RESULTS: Clinically, the subjects with Kayser-Fleischer rings had similar corneal findings and normal intraocular pressure; two had typical sunflower cataracts and decreased visual acuity. The control eyes all presented normal visual acuity, intraocular pressure, and corneal appearance. The microstructural analysis demonstrated similar findings in all the affected corneas. Compared with the control corneas, there were fewer keratocytes in the anterior stroma (17.380 vs. 22.380/mm3). Round, "hollow" dark areas were observed between the keratocytes; these were universal and similar in appearance in all affected corneas and all cornea layers. In the peripheral posterior stroma, there were dust-like, bright, granular deposits that tended to increase in number and density toward Descemet's membrane, masking the peripheral endothelium. The control corneas presented a normal microstructure apart from dust-like granular deposits in the periphery. CONCLUSIONS: In vivo confocal microscopy is a useful tool for evaluating the corneal microstructure when a Kayser-Fleischer ring is clinically present. The ring consists of granular, bright particles that increase in density toward Descemet's membrane, and is associated with a decreased number of keratocytes and peculiar dark, round areas in all stromal layers, probably a sign of corneal damage. When the ring is not visible in subjects with Wilson's disease, changes to the corneal microstructure are insignificant. SN - 1678-2925 UR - https://www.unboundmedicine.com/medline/citation/29846416/In_vivo_confocal_microstructural_analysis_of_corneas_presenting_Kayser_Fleischer_rings_in_patients_with_Wilson's_disease_ L2 - http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27492018000200137&lng=en&nrm=iso&tlng=en DB - PRIME DP - Unbound Medicine ER -