Assessment of corneal and lens clarity in children with Wilson disease

J AAPOS. 2019 Jun;23(3):147.e1-147.e8. doi: 10.1016/j.jaapos.2018.12.010. Epub 2019 May 9.


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

Methods: 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.

Results: 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).

Conclusion: 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.

Publication types

  • Comparative Study
  • Multicenter Study

MeSH terms

  • Adolescent
  • Child
  • Cornea / pathology*
  • Corneal Topography / methods*
  • Cross-Sectional Studies
  • Densitometry / methods*
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Hepatolenticular Degeneration / diagnosis*
  • Humans
  • Lens, Crystalline / pathology*
  • Male
  • Prospective Studies