Q: 32 year old male is transferred from outside hospital for transplant evaluation for acute liver failure. Medical student on exam wrote "unusual ring in the eyes". Diagnosis?
Answer: Kayser-Fleischer rings
Kayser-Fleischer ring is a hallmark of Wilson disease. The dense brown copper deposits encircle the iris. It requires slit-lamp examination. Though very highly suggestive of Wilson disease, Kayser-Fleischer rings are not specific for Wilson disease as they have been reported in cholestatic diseases, like primary biliary cirrhosis.
Clinical significance: Kayser-Fleischer rings gradually disappear with medical treatment for Wilson disease or if liver transplantation carried out. Their reappearance suggests either noncompliance or failed treatment.