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Scheimpflug Imaging of the Danish Cohort of Patients With Wilson Disease.
Cornea 2019; 38(8):998-1002C

Abstract

PURPOSE

The purpose of this study was to investigate the use of anterior segment imaging in diagnosing Kayser-Fleischer rings in patients with Wilson disease.

METHODS

In a tertiary center for Wilson disease, patients were examined with a Pentacam HR Scheimpflug-based tomography device in addition to conventional slit-lamp examination. The inferior part of the cornea was analyzed using both a built-in densitometry module and ImageJ.

RESULTS

Thirty-one patients with Wilson disease (78% of all Danish patients) were included, resulting in 83 examinations over a 5-year period. Ten had a manifest Kayser-Fleischer ring in the inferior part of the cornea on at least 1 examination, 5 had other causes of peripheral corneal scatter, and 16 had normal examinations. The built-in densitometry module performed poorly in discriminating between the presence and absence of a Kayser-Fleischer ring. However, analysis of the images in ImageJ and calculation of a normalized signal (peak posterior value/peak anterior value) with a cutoff value set to 1 detected 28 of 31 Kayser-Fleischer rings and resulted in 96% sensitivity and 95% specificity. In 12 patients who underwent 3 or more examinations during the period, changes in the normalized signal seemed to reflect the efficiency of the treatment, although more studies are needed for this conclusion.

CONCLUSIONS

ImageJ-based analysis of Pentacam images has a high sensitivity in detecting Kayser-Fleischer rings and can be used as a diagnostic procedure for Wilson disease and may be a tool to monitor the disease in an objective manner.

Authors+Show Affiliations

Department of Ophthalmology, Aarhus University Hospital, Aarhus, Denmark.Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark.Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark.Department of Ophthalmology, Aarhus University Hospital, Aarhus, Denmark.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31276461

Citation

Telinius, Niklas, et al. "Scheimpflug Imaging of the Danish Cohort of Patients With Wilson Disease." Cornea, vol. 38, no. 8, 2019, pp. 998-1002.
Telinius N, Ott P, Sandahl T, et al. Scheimpflug Imaging of the Danish Cohort of Patients With Wilson Disease. Cornea. 2019;38(8):998-1002.
Telinius, N., Ott, P., Sandahl, T., & Hjortdal, J. (2019). Scheimpflug Imaging of the Danish Cohort of Patients With Wilson Disease. Cornea, 38(8), pp. 998-1002. doi:10.1097/ICO.0000000000001959.
Telinius N, et al. Scheimpflug Imaging of the Danish Cohort of Patients With Wilson Disease. Cornea. 2019;38(8):998-1002. PubMed PMID: 31276461.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Scheimpflug Imaging of the Danish Cohort of Patients With Wilson Disease. AU - Telinius,Niklas, AU - Ott,Peter, AU - Sandahl,Thomas, AU - Hjortdal,Jesper, PY - 2019/7/6/entrez PY - 2019/7/6/pubmed PY - 2019/8/24/medline SP - 998 EP - 1002 JF - Cornea JO - Cornea VL - 38 IS - 8 N2 - PURPOSE: The purpose of this study was to investigate the use of anterior segment imaging in diagnosing Kayser-Fleischer rings in patients with Wilson disease. METHODS: In a tertiary center for Wilson disease, patients were examined with a Pentacam HR Scheimpflug-based tomography device in addition to conventional slit-lamp examination. The inferior part of the cornea was analyzed using both a built-in densitometry module and ImageJ. RESULTS: Thirty-one patients with Wilson disease (78% of all Danish patients) were included, resulting in 83 examinations over a 5-year period. Ten had a manifest Kayser-Fleischer ring in the inferior part of the cornea on at least 1 examination, 5 had other causes of peripheral corneal scatter, and 16 had normal examinations. The built-in densitometry module performed poorly in discriminating between the presence and absence of a Kayser-Fleischer ring. However, analysis of the images in ImageJ and calculation of a normalized signal (peak posterior value/peak anterior value) with a cutoff value set to 1 detected 28 of 31 Kayser-Fleischer rings and resulted in 96% sensitivity and 95% specificity. In 12 patients who underwent 3 or more examinations during the period, changes in the normalized signal seemed to reflect the efficiency of the treatment, although more studies are needed for this conclusion. CONCLUSIONS: ImageJ-based analysis of Pentacam images has a high sensitivity in detecting Kayser-Fleischer rings and can be used as a diagnostic procedure for Wilson disease and may be a tool to monitor the disease in an objective manner. SN - 1536-4798 UR - https://www.unboundmedicine.com/medline/citation/31276461/Scheimpflug_Imaging_of_the_Danish_Cohort_of_Patients_With_Wilson_Disease_ L2 - http://Insights.ovid.com/pubmed?pmid=31276461 DB - PRIME DP - Unbound Medicine ER -