Thomas Damgaard Sandahl

Scheimpflug Imaging of the Danish Cohort of Patients with Wilson Disease

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Scheimpflug Imaging of the Danish Cohort of Patients with Wilson Disease. / Telinius, Niklas; Ott, Peter; Sandahl, Thomas; Hjortdal, Jesper.

In: Cornea, Vol. 38, No. 8, 08.2019, p. 998-1002.

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@article{9418270cdfbb4c85bc9f58624e88b668,
title = "Scheimpflug Imaging of the Danish Cohort of Patients with Wilson Disease",
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.",
keywords = "hepatolenticular degeneration, Kayser-Fleischer ring, Wilson disease, Wilson's disease",
author = "Niklas Telinius and Peter Ott and Thomas Sandahl and Jesper Hjortdal",
year = "2019",
month = aug,
doi = "10.1097/ICO.0000000000001959",
language = "English",
volume = "38",
pages = "998--1002",
journal = "Cornea",
issn = "0277-3740",
publisher = "LIPPINCOTT WILLIAMS & WILKINS",
number = "8",

}

RIS

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 - 2019/8

Y1 - 2019/8

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.

AB - 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.

KW - hepatolenticular degeneration

KW - Kayser-Fleischer ring

KW - Wilson disease

KW - Wilson's disease

UR - http://www.scopus.com/inward/record.url?scp=85069267687&partnerID=8YFLogxK

U2 - 10.1097/ICO.0000000000001959

DO - 10.1097/ICO.0000000000001959

M3 - Journal article

C2 - 31276461

AN - SCOPUS:85069267687

VL - 38

SP - 998

EP - 1002

JO - Cornea

JF - Cornea

SN - 0277-3740

IS - 8

ER -