Assessment of corneal and lens clarity in children with Wilson disease

To investigate the effect of copper accumulation on corneal and lens clarity in children with Wilson disease (WD) compared to healthy children.

This multicenter cross-sectional study included 24 subjects with WD and 25 age-matched controls. Clinical and laboratory characteristics of the WD subjects were recorded. The Pentacam HR imaging system was used both for lens densitometry and corneal densitometry.

Corneal densitometry values were higher in the posterior 6-10 mm (P = 0.021), posterior 10-12 mm (P < 0.001), posterior total diameter (P = 0.037), total thickness 10-12 mm (P = 0.032), and total thickness 6-10 mm zones and layers (P = 0.040) in the WD eyes than in control eyes. The lens densitometry values of zone 1 were higher in WD eyes (P < 0.001). There was a significant relationship between corneal densitometry values in the posterior 10-12 mm zones (P = 0.012; r = 0.527) and the duration of WD and liver copper content (P = 0.016; r = 0.507). A statistically significant correlation was also detected between lens densitometry values in zone 1 and WD duration (P = 0.018; r = 0.426).

In this study cohort, children with WD had decreased corneal and lens clarity even in cases without Kayser-Fleischer rings and sunflower cataracts. Densitometry measurements using Scheimpflug imaging provided detection of corneal and lens involvement in the early stages of WD.