Abstract

BackgroundTo investigate the topographic measurements and densitometry of corneas in Wilson’s disease (WD) patients with or without a Kayser-Fleischer ring (KF-r) compared to healthy individuals.MethodsThis cross-sectional study included 20 WD patients without a KF-r (group I), 18 WD patients with a KF-r (group II), and 20 age-matched controls (group III). The Pentacam high resolution imaging system is used to determine corneal topographic measurements and densitometry.ResultsMean age for groups I, II and III was 25.40 ± 6.43 years (14–36 years), 25.38 ± 6.96 years (16–39 years), 23.60 ± 6.56 years (17–35 years), respectively (P = 0.623). There was no significant difference between the groups in terms of the anterior corneal densitometry values (P > 0.05), while the 6–10 mm and 10–12 mm mid stroma and the 2–6 mm, 6–10 mm, and 10–12 mm posterior corneal densitometry values in group II were significantly higher than those in groups I and III (for all values, P < 0.05). However, the 10–12 mm posterior corneal densitometry values in group I were also significantly higher than those in group III (P = 0.038). The central corneal thickness (CCT), thinnest corneal thickness (tCT), and corneal volume (CV) values in groups I and II were significantly lower than those in group III (for CCT values, P = 0.011 and P = 0.009; for tCT values, P = 0.010 and P = 0.005; for CV values, P = 0.043 and P = 0.029).ConclusionIn WD patients with a KF-r, corneal transparency decreased in the peripheral posterior and mid stromal corneal layers; for these patients, corneal transparency may be impaired not only in the peripheral cornea but also in the paracentral cornea.

Highlights

  • To investigate the topographic measurements and densitometry of corneas in Wilson’s disease (WD) patients with or without a Kayser-Fleischer ring (KF-r) compared to healthy individuals

  • While the Flat keratometry (K1), Steep keratometry (K2), and Maximum keratometry (Kmax) values were similar between the three groups (P > 0.05 for all values), the central corneal thickness (CCT), thinnest corneal thickness (tCT), and corneal volume (CV) values were significantly different between the groups (P = 0.003, P = 0.002 and P = 0.037, respectively)

  • When corneal densitometry values were assessed, there was no significant difference between the groups in terms of the anterior corneal densitometry values (P > 0.05 for all values)

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Summary

Introduction

To investigate the topographic measurements and densitometry of corneas in Wilson’s disease (WD) patients with or without a Kayser-Fleischer ring (KF-r) compared to healthy individuals. Wilson’s disease (WD) is a hereditary autosomal recessive disease which is a hepatic copper metabolism dysfunction that results in copper accumulation in the hepatic tissues and extrahepatic tissues, such as the Ophthalmological manifestations of WD include the Kayser-Fleischer ring (KF-r), which is caused by the granular deposition of copper in the peripheral corneal Descemet membrane [2, 4]. The KF-r appears as a granular golden-greenish layer near the limbus. It first (2021) 8:8 occurs at the top of the cornea and inferiorly and appears circular, like a ring. Other less common findings are night blindness, exotropic strabismus, optic neuritis, and pallor of the optic disc [5, 6]

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