Pigmented corneal rings in neonates with liver disease

We performed eye examinations in neonates to determine whether pigmented corneal rings were present in infants with hepatic disease, as in adults. None of 16 control infants had pigmented corneal rings, but 10 (71%) of 14 infants with cholestatic liver disease had such rings. Most rings could be seen without slit lamp by direct inspection of the cornea. Furthermore, the rings appeared to resolve over time. Liver dysfunction tended to be more severe in patients with corneal rings. We speculate that abnormal tissue accumulation of copper may be present in many infants with cholestatic liver disease. Ophthalmologic examination for pigmented corneal rings or determination of serum copper levels may need to be performed in patients at high risk with hepatic disease to monitor for excessive copper accumulation.